Atlas of Uncommon Pain Syndromes [4th Edition] 9780323640770

Table of contents :
Title page......Page 2
Table of Contents......Page 4
Copyright......Page 56
Dedication......Page 59
Preface......Page 61
section 1. Headache and Facial Pain Syndromes......Page 65
1. Ice Pick Headache......Page 68
Clinical Syndrome......Page 69
Signs and Symptoms......Page 70
Testing......Page 71
Differential Diagnosis......Page 74
Treatment......Page 75
Complications and Pitfalls......Page 76
2. Supraorbital Neuralgia......Page 80
Clinical Syndrome......Page 81
Signs and Symptoms......Page 82
Testing......Page 83
Differential Diagnosis......Page 84
Treatment......Page 85
Complications and Pitfalls......Page 89
3. Chronic Paroxysmal Hemicrania......Page 98
Clinical Syndrome......Page 99
Signs and Symptoms......Page 100
Testing......Page 101
Differential Diagnosis......Page 105
Treatment......Page 106
Complications and Pitfalls......Page 108
4. Hemicrania Continua......Page 112
Clinical Syndrome......Page 113
Signs and Symptoms......Page 114
Testing......Page 115
Differential Diagnosis......Page 117
Treatment......Page 118
Complications and Pitfalls......Page 119
5. Charlin Syndrome......Page 125
Clinical Syndrome......Page 126
Signs and Symptoms......Page 127
Testing......Page 128
Differential Diagnosis......Page 129
Treatment......Page 130
Complications and Pitfalls......Page 133
6. Sexual Headache......Page 137
The Clinical Syndrome......Page 138
Signs and Symptoms......Page 139
Testing......Page 142
Differential Diagnosis......Page 143
Treatment......Page 146
Complications and Pitfalls......Page 147
7. Cough Headache......Page 151
The Clinical Syndrome......Page 152
Signs and Symptoms......Page 153
Testing......Page 155
Differential Diagnosis......Page 158
Treatment......Page 159
Complications and Pitfalls......Page 160
8. Sudden Unilateral Neuralgiform Conjunctival Injection Tearing Headache......Page 164
The Clinical Syndrome......Page 165
Signs and Symptoms......Page 166
Testing......Page 167
Differential Diagnosis......Page 172
Treatment......Page 177
Complications and Pitfalls......Page 178
9. Primary Thunderclap Headache......Page 183
The Clinical Syndrome......Page 184
Signs and Symptoms......Page 185
Testing......Page 186
Differential Diagnosis......Page 193
Treatment......Page 194
Complications and Pitfalls......Page 195
10. Hypnic Headache......Page 200
The Clinical Syndrome......Page 201
Signs and Symptoms......Page 202
Testing......Page 203
Differential Diagnosis......Page 207
Treatment......Page 208
Complications and Pitfalls......Page 209
11. Nummular Headache......Page 213
The Clinical Syndrome......Page 214
Signs and Symptoms......Page 215
Testing......Page 216
Differential Diagnosis......Page 217
Treatment......Page 220
Complications and Pitfalls......Page 221
12. Headache Associated With Temporal Arteritis......Page 225
The Clinical Syndrome......Page 226
Signs and Symptoms......Page 227
Testing......Page 228
Differential Diagnosis......Page 231
Treatment......Page 232
Complications and Pitfalls......Page 234
13. Post–Dural Puncture Headache......Page 237
The Clinical Syndrome......Page 238
Signs and Symptoms......Page 239
Testing......Page 240
Differential Diagnosis......Page 241
Treatment......Page 243
Complications and Pitfalls......Page 244
14. Ramsay Hunt Syndrome......Page 249
Signs and Symptoms......Page 251
Testing......Page 254
Differential Diagnosis......Page 257
Treatment......Page 258
Nerve Blocks......Page 260
Opioid Analgesics......Page 261
Adjuvant Analgesics......Page 262
Antiviral Agents......Page 263
Adjunctive Treatments......Page 264
Complications and Pitfalls......Page 265
15. Eagle Syndrome......Page 269
The Clinical Syndrome......Page 270
Signs and Symptoms......Page 271
Testing......Page 272
Differential Diagnosis......Page 273
Treatment......Page 274
Complications and Pitfalls......Page 279
16. Atypical Odontalgia......Page 283
The Clinical Syndrome......Page 284
Signs and Symptoms......Page 285
Testing......Page 286
Differential Diagnosis......Page 291
Treatment......Page 292
Complications and Pitfalls......Page 293
17. Burning Mouth Syndrome......Page 297
The Clinical Syndrome......Page 298
Signs and Symptoms......Page 299
Testing......Page 300
Differential Diagnosis......Page 301
Treatment......Page 304
Complications and Pitfalls......Page 307
18. Nervus Intermedius Neuralgia......Page 311
The Clinical Syndrome......Page 312
Signs and Symptoms......Page 313
Testing......Page 314
Differential Diagnosis......Page 319
Treatment......Page 320
Complications and Pitfalls......Page 324
19. Red Ear Syndrome......Page 328
The Clinical Syndrome......Page 329
Testing......Page 331
Differential Diagnosis......Page 334
Treatment......Page 335
Complications and Pitfalls......Page 336
20. Glossopharyngeal Neuralgia......Page 340
The Clinical Syndrome......Page 341
Signs and Symptoms......Page 342
Testing......Page 343
Differential Diagnosis......Page 346
Treatment......Page 347
Complications and Pitfalls......Page 354
section 2. Neck and Brachial Plexus Pain Syndromes......Page 359
21. Clival Chordoma Syndrome......Page 361
The Clinical Syndrome......Page 362
Signs and Symptoms......Page 363
Testing......Page 364
Differential Diagnosis......Page 367
Treatment......Page 368
Complications and Pitfalls......Page 369
22. Spasmodic Torticollis......Page 373
The Clinical Syndrome......Page 374
Signs and Symptoms......Page 375
Testing......Page 376
Differential Diagnosis......Page 377
Treatment......Page 380
Complications and Pitfalls......Page 381
23. Cervicothoracic Interspinous Bursitis......Page 385
The Clinical Syndrome......Page 386
Signs and Symptoms......Page 387
Testing......Page 388
Differential Diagnosis......Page 393
Treatment......Page 394
Complications and Pitfalls......Page 397
24. Scapulocostal Syndrome......Page 402
The Clinical Syndrome......Page 403
Signs and Symptoms......Page 404
Testing......Page 405
Differential Diagnosis......Page 406
Treatment......Page 408
Complications and Pitfalls......Page 409
The Clinical Syndrome......Page 413
Signs and Symptoms......Page 414
Differential Diagnosis......Page 415
Testing......Page 416
Treatment......Page 419
Complications and Pitfalls......Page 426
26. Hyoid Syndrome......Page 430
The Clinical Syndrome......Page 431
Signs and Symptoms......Page 432
Testing......Page 433
Differential Diagnosis......Page 434
Treatment......Page 435
Complications and Pitfalls......Page 441
27. Omohyoid Syndrome......Page 446
The Clinical Syndrome......Page 447
Signs and Symptoms......Page 448
Testing......Page 449
Differential Diagnosis......Page 450
Treatment......Page 451
Complications and Pitfalls......Page 455
28. Acute Calcific Prevertebral Tendinitis......Page 460
The Clinical Syndrome......Page 461
Signs and Symptoms......Page 462
Testing......Page 464
Differential Diagnosis......Page 465
Treatment......Page 466
Complications and Pitfalls......Page 467
29. Neck-Tongue Syndrome......Page 475
The Clinical Syndrome......Page 476
Signs and Symptoms......Page 477
Testing......Page 478
Differential Diagnosis......Page 479
Treatment......Page 480
Complications and Pitfalls......Page 481
30. Sternohyoid Syndrome......Page 485
The Clinical Syndrome......Page 486
Signs and Symptoms......Page 487
Testing......Page 488
Differential Diagnosis......Page 489
Treatment......Page 490
Complications and Pitfalls......Page 493
section 3. Shoulder Pain Syndromes......Page 500
31. Supraspinatus Tendinitis......Page 502
The Clinical Syndrome......Page 503
Signs and Symptoms......Page 504
Testing......Page 506
Differential Diagnosis......Page 507
Treatment......Page 510
Complications and Pitfalls......Page 514
32. Infraspinatus Tendinitis......Page 519
The Clinical Syndrome......Page 520
Signs And Symptoms......Page 521
Testing......Page 522
Differential Diagnosis......Page 523
Treatment......Page 526
Complications and Pitfalls......Page 528
33. Subacromial Impingement Syndrome......Page 533
The Clinical Syndrome......Page 534
Signs and Symptoms......Page 535
Testing......Page 536
Differential Diagnosis......Page 544
Treatment......Page 545
Complications and Pitfalls......Page 549
34. Os Acromiale Pain Syndrome......Page 553
The Clinical Syndrome......Page 554
Signs and Symptoms......Page 558
Testing......Page 560
Differential Diagnosis......Page 562
Treatment......Page 563
Complications and Pitfalls......Page 564
35. Glomus Tumor of the Shoulder......Page 568
The Clinical Syndrome......Page 569
Signs and Symptoms......Page 570
Testing......Page 571
Differential Diagnosis......Page 572
Treatment......Page 573
Complications and Pitfalls......Page 575
36. Pectoralis Major Tear Syndrome......Page 579
Pectoralis Muscle Tear......Page 580
Pectoralis Muscle Tendon Rupture......Page 581
The Clinical Syndrome......Page 582
Signs and Symptoms......Page 587
Testing......Page 591
Differential Diagnosis......Page 593
Treatment......Page 594
Complications and Pitfalls......Page 595
37. Suprascapular Nerve Entrapment......Page 599
The Clinical Syndrome......Page 600
Signs and Symptoms......Page 601
Testing......Page 602
Differential Diagnosis......Page 604
Treatment......Page 605
Complications and Pitfalls......Page 608
38. Snapping Scapula Syndrome......Page 612
The Clinical Syndrome......Page 613
Signs and Symptoms......Page 614
Testing......Page 617
Differential diagnosis......Page 622
Treatment......Page 623
Complications and Pitfalls......Page 625
39. Quadrilateral Space Syndrome......Page 633
The Clinical Syndrome......Page 634
Signs and Symptoms......Page 638
Testing......Page 640
Differential Diagnosis......Page 641
Treatment......Page 645
Complications and Pitfalls......Page 646
section 4. Elbow Pain Syndromes......Page 649
40. Pronator Syndrome......Page 651
The Clinical Syndrome......Page 652
Signs and Symptoms......Page 653
Testing......Page 654
Differential Diagnosis......Page 658
Treatment......Page 661
Complications and Pitfalls......Page 663
41. Cubital Bursitis......Page 668
The Clinical Syndrome......Page 669
Signs and Symptoms......Page 670
Testing......Page 671
Differential Diagnosis......Page 672
Treatment......Page 674
Complications and Pitfalls......Page 677
42. Anconeus Epitrochlearis......Page 681
The Clinical Syndrome......Page 682
Signs and Symptoms......Page 683
Testing......Page 688
Differential Diagnosis......Page 689
Treatment......Page 690
Complications and Pitfalls......Page 691
43. Os Supratrochleare-Related Elbow Pain......Page 695
The Clinical Syndrome......Page 696
Signs and Symptoms......Page 697
Testing......Page 698
Differential Diagnosis......Page 699
Treatment......Page 703
Complications and Pitfalls......Page 704
44. Osteonecrosis of the Elbow Joint......Page 708
The Clinical Syndrome......Page 709
Signs and Symptoms......Page 710
Testing......Page 711
Differential Diagnosis......Page 716
Treatment......Page 717
Complications and Pitfalls......Page 718
45. Triceps Tendinitis......Page 722
The Clinical Syndrome......Page 723
Signs and Symptoms......Page 724
Testing......Page 727
Differential Diagnosis......Page 728
Treatment......Page 729
Complications and Pitfalls......Page 730
46. Radial Tunnel Syndrome......Page 734
The Clinical Syndrome......Page 735
Signs and Symptoms......Page 736
Testing......Page 738
Differential Diagnosis......Page 742
Treatment......Page 743
Complications and Pitfalls......Page 744
47. Cubital Tunnel Syndrome......Page 748
The Clinical Syndrome......Page 749
Signs and Symptoms......Page 750
Testing......Page 751
Differential Diagnosis......Page 752
Treatment......Page 756
Complications and Pitfalls......Page 757
48. Driver’s Elbow......Page 765
The Clinical Syndrome......Page 766
Signs and Symptoms......Page 767
Testing......Page 768
Differential Diagnosis......Page 769
Treatment......Page 772
Complications and Pitfalls......Page 773
49. Anterior Interosseous Syndrome......Page 778
The Clinical Syndrome......Page 779
Signs and Symptoms......Page 780
Testing......Page 781
Differential Diagnosis......Page 782
Treatment......Page 788
Complications and Pitfalls......Page 789
section 5. Wrist and Hand Pain Syndromes......Page 792
50. Ulnar Tunnel Syndrome......Page 795
The Clinical Syndrome......Page 796
Signs and Symptoms......Page 797
Testing......Page 801
Differential Diagnosis......Page 802
Treatment......Page 804
Complications and Pitfalls......Page 805
51. Cheiralgia Paresthetica......Page 810
The Clinical Syndrome......Page 811
Signs and Symptoms......Page 812
Testing......Page 813
Differential Diagnosis......Page 814
Treatment......Page 816
Complications and Pitfalls......Page 818
52. Secretan Syndrome......Page 826
The Clinical Syndrome......Page 827
Signs and Symptoms......Page 828
Testing......Page 829
Differential Diagnosis......Page 830
Treatment......Page 831
Complications and Pitfalls......Page 833
53. Foreign Body Synovitis......Page 837
The Clinical Syndrome......Page 838
Signs and Symptoms......Page 839
Testing......Page 841
Differential Diagnosis......Page 842
Treatment......Page 843
Complications and Pitfalls......Page 847
54. Glomus Tumor of the Hand......Page 851
The Clinical Syndrome......Page 852
Signs and Symptoms......Page 853
Testing......Page 854
Differential Diagnosis......Page 855
Treatment......Page 858
Complications and Pitfalls......Page 859
55. Erythromelalgia......Page 863
The Clinical Syndrome......Page 864
Testing......Page 866
Differential Diagnosis......Page 872
Treatment......Page 873
Complications and Pitfalls......Page 874
56. Boxer’s Knuckle......Page 878
The Clinical Syndrome......Page 879
Signs and Symptoms......Page 880
Testing......Page 881
Differential Diagnosis......Page 882
Treatment......Page 883
Complications and Pitfalls......Page 888
57. Triangular Fibrocartilage Tear Syndrome......Page 892
The Clinical Syndrome......Page 893
Signs and Symptoms......Page 894
Testing......Page 900
Differential Diagnosis......Page 902
Treatment......Page 904
Complications and Pitfalls......Page 905
58. Scapholunate Ligament Tear Syndrome......Page 909
The Clinical Syndrome......Page 910
Signs and Symptoms......Page 913
Testing......Page 914
Differential Diagnosis......Page 917
Treatment......Page 918
Complications and Pitfalls......Page 921
59. Lunotriquetral Instability Pain Syndrome......Page 926
The Clinical Syndrome......Page 927
Signs and Symptoms......Page 929
Testing......Page 932
Differential Diagnosis......Page 933
Treatment......Page 934
Complications and Pitfalls......Page 935
60. Kienböck Disease......Page 941
The Clinical Syndrome......Page 942
Signs and Symptoms......Page 943
Testing......Page 944
Differential Diagnosis......Page 947
Treatment......Page 948
Complications and Pitfalls......Page 949
61. Avascular Necrosis of the Scaphoid......Page 954
The Clinical Syndrome......Page 955
Signs and Symptoms......Page 957
Testing......Page 961
Differential Diagnosis......Page 963
Treatment......Page 964
Complications and Pitfalls......Page 965
62. Extensor Carpi Ulnaris Tendinitis......Page 969
The Clinical Syndrome......Page 970
Signs and Symptoms......Page 972
Testing......Page 975
Differential Diagnosis......Page 976
Treatment......Page 977
Complications and Pitfalls......Page 978
63. Flexor Carpi Radialis Tendinitis......Page 982
The Clinical Syndrome......Page 983
Signs and Symptoms......Page 984
Testing......Page 985
Differential Diagnosis......Page 990
Treatment......Page 991
Complications and Pitfalls......Page 992
64. Trigger Wrist......Page 996
The Clinical Syndrome......Page 997
Signs and Symptoms......Page 998
Testing......Page 999
Differential Diagnosis......Page 1005
Treatment......Page 1006
Complications and Pitfalls......Page 1007
section 6. Thoracic Pain Syndromes......Page 1010
The Clinical Syndrome......Page 1012
Signs and Symptoms......Page 1013
Testing......Page 1014
Treatment......Page 1018
Complications and Pitfalls......Page 1019
66. Sternoclavicular Syndrome......Page 1024
The Clinical Syndrome......Page 1025
Signs and Symptoms......Page 1027
Testing......Page 1028
Differential Diagnosis......Page 1032
Treatment......Page 1035
Complications and Pitfalls......Page 1037
67. Postmastectomy Pain......Page 1041
The Clinical Syndrome......Page 1042
Signs and Symptoms......Page 1043
Testing......Page 1046
Differential Diagnosis......Page 1047
Treatment......Page 1048
Complications and Pitfalls......Page 1050
68. Sternalis Syndrome......Page 1054
The Clinical Syndrome......Page 1055
Signs and Symptoms......Page 1056
Testing......Page 1057
Differential Diagnosis......Page 1058
Treatment......Page 1059
Complications and Pitfalls......Page 1063
The Clinical Syndrome......Page 1068
Signs and Symptoms......Page 1069
Testing......Page 1070
Differential Diagnosis......Page 1071
Treatment......Page 1074
Complications and Pitfalls......Page 1077
The Clinical Syndrome......Page 1081
Signs and Symptoms......Page 1082
Testing......Page 1083
Differential Diagnosis......Page 1087
Treatment......Page 1088
Complications and Pitfalls......Page 1089
The Clinical Syndrome......Page 1093
Signs and Symptoms......Page 1094
Testing......Page 1095
Differential Diagnosis......Page 1096
Treatment......Page 1097
Complications and Pitfalls......Page 1100
The Clinical Syndrome......Page 1104
Signs and Symptoms......Page 1105
Testing......Page 1106
Differential Diagnosis......Page 1107
Treatment......Page 1110
Complications and Pitfalls......Page 1111
73. Winged Scapula Syndrome......Page 1115
The Clinical Syndrome......Page 1116
Signs and Symptoms......Page 1117
Testing......Page 1118
Differential Diagnosis......Page 1119
Treatment......Page 1120
Complications and Pitfalls......Page 1123
section 7. Abdominal and Groin Pain Syndromes......Page 1126
The Clinical Syndrome......Page 1128
Signs and Symptoms......Page 1129
Testing......Page 1130
Differential Diagnosis......Page 1132
Treatment......Page 1133
Complications and Pitfalls......Page 1137
75. Acute Intermittent Porphyria......Page 1141
The Clinical Syndrome......Page 1142
Signs and Symptoms......Page 1143
Testing......Page 1144
Differential Diagnosis......Page 1145
Treatment......Page 1147
Complications and Pitfalls......Page 1148
76. Radiation Enteritis......Page 1154
The Clinical Syndrome......Page 1155
Signs and Symptoms......Page 1156
Testing......Page 1157
Differential Diagnosis......Page 1158
Treatment......Page 1159
Complications and Pitfalls......Page 1161
77. Liver Pain......Page 1167
The Clinical Syndrome......Page 1168
Signs and Symptoms......Page 1169
Testing......Page 1172
Differential Diagnosis......Page 1173
Treatment......Page 1175
Complications and Pitfalls......Page 1176
78. Abdominal Angina......Page 1180
The Clinical Syndrome......Page 1181
Signs and Symptoms......Page 1182
Testing......Page 1183
Differential Diagnosis......Page 1187
Treatment......Page 1190
Complications and Pitfalls......Page 1191
section 8. Lumbar Spine and Sacroiliac Joint Pain Syndromes......Page 1194
79. Epidural Abscess......Page 1196
The Clinical Syndrome......Page 1197
Signs and Symptoms......Page 1198
Testing......Page 1199
Differential Diagnosis......Page 1200
Treatment......Page 1202
Complications and Pitfalls......Page 1203
80. Multiple Myeloma......Page 1210
The Clinical Syndrome......Page 1211
Signs and Symptoms......Page 1212
Testing......Page 1215
Differential Diagnosis......Page 1216
Treatment......Page 1217
Complications and Pitfalls......Page 1218
81. Foix-Alajouanine Syndrome......Page 1223
The Clinical Syndrome......Page 1224
Signs and Symptoms......Page 1225
Testing......Page 1226
Differential Diagnosis......Page 1230
Treatment......Page 1232
Complications and Pitfalls......Page 1233
82. Paget Disease......Page 1240
The Clinical Syndrome......Page 1241
Signs and Symptoms......Page 1243
Testing......Page 1244
Differential Diagnosis......Page 1247
Treatment......Page 1248
Complications and Pitfalls......Page 1251
83. Diffuse Idiopathic Skeletal Hyperostosis......Page 1255
The Clinical Syndrome......Page 1256
Signs and Symptoms......Page 1257
Testing......Page 1258
Differential Diagnosis......Page 1259
Treatment......Page 1264
Complications and Pitfalls......Page 1265
84. Spondylolisthesis......Page 1271
The Clinical Syndrome......Page 1272
Signs and Symptoms......Page 1273
Testing......Page 1274
Differential Diagnosis......Page 1281
Treatment......Page 1282
Complications and Pitfalls......Page 1285
85. Ankylosing Spondylitis......Page 1289
The Clinical Syndrome......Page 1290
Signs and Symptoms......Page 1291
Testing......Page 1292
Differential Diagnosis......Page 1293
Treatment......Page 1296
Complications and Pitfalls......Page 1301
86. Superior Cluneal Nerve Entrapment Syndrome......Page 1305
The Clinical Syndrome......Page 1306
Signs and Symptoms......Page 1307
Testing......Page 1310
Differential Diagnosis......Page 1311
Treatment......Page 1313
Complications and Pitfalls......Page 1315
87. Lumbar Myofascial Pain Syndrome......Page 1319
The Clinical Syndrome......Page 1320
Signs and Symptoms......Page 1321
Testing......Page 1323
Differential Diagnosis......Page 1324
Treatment......Page 1325
Complications and Pitfalls......Page 1328
88. Lumbar Paraspinal Compartment Syndrome......Page 1332
The Clinical Syndrome......Page 1333
Signs and Symptoms......Page 1334
Testing......Page 1335
Differential Diagnosis......Page 1341
Treatment......Page 1343
Complications and Pitfalls......Page 1344
section 9. Pelvic Pain Syndromes......Page 1347
89. Gluteus Maximus Pain Syndrome......Page 1349
The Clinical Syndrome......Page 1350
Signs and Symptoms......Page 1354
Testing......Page 1355
Differential Diagnosis......Page 1356
Treatment......Page 1357
Complications and Pitfalls......Page 1359
90. Gluteus Medius Syndrome......Page 1363
The Clinical Syndrome......Page 1364
Signs and Symptoms......Page 1367
Testing......Page 1368
Differential Diagnosis......Page 1369
Treatment......Page 1370
Complications and Pitfalls......Page 1372
91. Gluteal Bursitis......Page 1376
The Clinical Syndrome......Page 1377
Signs and Symptoms......Page 1378
Testing......Page 1379
Differential Diagnosis......Page 1380
Treatment......Page 1383
Complications and Pitfalls......Page 1385
92. Clunealgia......Page 1389
The Clinical Syndrome......Page 1390
Signs and Symptoms......Page 1391
Testing......Page 1392
Differential Diagnosis......Page 1396
Treatment......Page 1397
Complications and Pitfalls......Page 1398
93. Nutcracker Syndrome......Page 1407
The Clinical Syndrome......Page 1408
Signs and Symptoms......Page 1412
Testing......Page 1413
Differential Diagnosis......Page 1415
Treatment......Page 1416
Complications and Pitfalls......Page 1419
94. Orchialgia......Page 1427
The Clinical Syndrome......Page 1428
Signs and Symptoms......Page 1429
Testing......Page 1430
Differential Diagnosis......Page 1434
Treatment......Page 1438
Complications and Pitfalls......Page 1439
95. Clitoral Priapism......Page 1443
The Clinical Syndrome......Page 1444
Signs and Symptoms......Page 1445
Testing......Page 1446
Differential Diagnosis......Page 1449
Treatment......Page 1450
Complications and Pitfalls......Page 1451
96. Vulvodynia......Page 1459
The Clinical Syndrome......Page 1460
Signs and Symptoms......Page 1461
Testing......Page 1462
Differential Diagnosis......Page 1468
Treatment......Page 1469
Complications and Pitfalls......Page 1472
97. Prostatodynia......Page 1477
The Clinical Syndrome......Page 1478
Signs and Symptoms......Page 1479
Testing......Page 1480
Differential Diagnosis......Page 1481
Treatment......Page 1483
Complications and Pitfalls......Page 1484
98. Levator Ani Pain Syndrome......Page 1491
The Clinical Syndrome......Page 1492
Signs and Symptoms......Page 1494
Testing......Page 1497
Differential Diagnosis......Page 1498
Treatment......Page 1499
Complications and Pitfalls......Page 1500
99. Proctalgia Fugax......Page 1506
The Clinical Syndrome......Page 1507
Signs and Symptoms......Page 1508
Testing......Page 1512
Differential Diagnosis......Page 1513
Treatment......Page 1514
Complications and Pitfalls......Page 1516
100. Paroxysmal Extreme Pain Disorder......Page 1520
The Clinical Syndrome......Page 1521
Signs and Symptoms......Page 1522
Testing......Page 1523
Differential Diagnosis......Page 1524
Treatment......Page 1528
Complications and Pitfalls......Page 1529
section 10. Hip and Lower Extremity Pain Syndromes......Page 1532
101. Avascular Necrosis of the Hip......Page 1534
The Clinical Syndrome......Page 1535
Signs and Symptoms......Page 1536
Testing......Page 1537
Differential Diagnosis......Page 1540
Treatment......Page 1541
Complications and Pitfalls......Page 1545
102. Transient Regional Osteoporosis......Page 1549
The Clinical Syndrome......Page 1550
Signs and Symptoms......Page 1551
Testing......Page 1552
Differential Diagnosis......Page 1553
Treatment......Page 1555
Complications and Pitfalls......Page 1557
103. Psoas Bursitis......Page 1563
The Clinical Syndrome......Page 1564
Signs and Symptoms......Page 1565
Testing......Page 1566
Differential Diagnosis......Page 1567
Treatment......Page 1568
Complications and Pitfalls......Page 1570
104. Iliopsoas Tendon Rupture......Page 1574
The Clinical Syndrome......Page 1575
Signs and Symptoms......Page 1576
Testing......Page 1577
Differential Diagnosis......Page 1578
Treatment......Page 1579
Complications and Pitfalls......Page 1584
105. Femoral Neuropathy......Page 1588
The Clinical Syndrome......Page 1589
Signs and Symptoms......Page 1590
Testing......Page 1591
Differential Diagnosis......Page 1592
Treatment......Page 1597
Complications and Pitfalls......Page 1598
106. Saphenous Neuralgia......Page 1602
The Clinical Syndrome......Page 1603
Signs and Symptoms......Page 1604
Testing......Page 1605
Differential Diagnosis......Page 1606
Treatment......Page 1607
Complications and Pitfalls......Page 1611
107. Obturator Neuralgia......Page 1617
The Clinical Syndrome......Page 1618
Signs and Symptoms......Page 1619
Testing......Page 1620
Differential Diagnosis......Page 1621
Treatment......Page 1622
Complications and Pitfalls......Page 1626
108. Adductor Tendinitis......Page 1630
The Clinical Syndrome......Page 1631
Signs and Symptoms......Page 1632
Testing......Page 1633
Differential Diagnosis......Page 1637
Treatment......Page 1638
Complications and Pitfalls......Page 1639
109. Iliopectinate Bursitis......Page 1643
The Clinical Syndrome......Page 1644
Signs and Symptoms......Page 1645
Differential Diagnosis......Page 1648
Treatment......Page 1649
Complications and Pitfalls......Page 1652
110. Snapping Hip Syndrome......Page 1656
The Clinical Syndrome......Page 1657
Signs and Symptoms......Page 1658
Testing......Page 1659
Differential Diagnosis......Page 1660
Treatment......Page 1661
Complications and Pitfalls......Page 1663
section 11. Knee Pain Syndromes......Page 1674
111. Tibiofibular Pain Syndrome......Page 1676
The Clinical Syndrome......Page 1677
Signs and Symptoms......Page 1678
Testing......Page 1679
Differential Diagnosis......Page 1683
Treatment......Page 1685
Complications and Pitfalls......Page 1686
112. Jumper’s Knee......Page 1690
The Clinical Syndrome......Page 1691
Signs and Symptoms......Page 1692
Testing......Page 1693
Differential Diagnosis......Page 1694
Treatment......Page 1695
Complications and Pitfalls......Page 1698
113. Semimembranosus Insertion Syndrome......Page 1705
The Clinical Syndrome......Page 1706
Signs and Symptoms......Page 1709
Testing......Page 1710
Differential Diagnosis......Page 1714
Treatment......Page 1715
Complications and Pitfalls......Page 1716
114. Coronary Ligament Strain......Page 1720
The Clinical Syndrome......Page 1721
Signs and Symptoms......Page 1722
Testing......Page 1724
Differential Diagnosis......Page 1725
Treatment......Page 1726
Complications and Pitfalls......Page 1727
115. Breaststroker’s Knee......Page 1731
The Clinical Syndrome......Page 1732
Signs and Symptoms......Page 1733
Testing......Page 1734
Differential Diagnosis......Page 1737
Treatment......Page 1738
Complications and Pitfalls......Page 1741
The Clinical Syndrome......Page 1745
Signs and Symptoms......Page 1746
Testing......Page 1747
Differential Diagnosis......Page 1748
Treatment......Page 1751
Complications and Pitfalls......Page 1752
117. Runner’s Knee......Page 1756
The Clinical Syndrome......Page 1757
Signs and Symptoms......Page 1758
Testing......Page 1759
Differential Diagnosis......Page 1763
Treatment......Page 1764
Complications and Pitfalls......Page 1767
118. Snapping Pes Anserinus Syndrome......Page 1771
The Clinical Syndrome......Page 1772
Signs and Symptoms......Page 1773
Testing......Page 1774
Differential Diagnosis......Page 1781
Treatment......Page 1782
Complications and Pitfalls......Page 1783
119. Glomus Tumor of the Knee......Page 1787
The Clinical Syndrome......Page 1788
Signs and Symptoms......Page 1789
Testing......Page 1790
Differential Diagnosis......Page 1791
Treatment......Page 1796
Complications and Pitfalls......Page 1797
120. Iliotibial Band Bursitis......Page 1802
The Clinical Syndrome......Page 1803
Signs and Symptoms......Page 1804
Testing......Page 1807
Differential Diagnosis......Page 1808
Treatment......Page 1809
Complications and Pitfalls......Page 1811
121. Fabella Syndrome......Page 1815
The Clinical Syndrome......Page 1816
Signs and Symptoms......Page 1817
Testing......Page 1818
Differential Diagnosis......Page 1819
Treatment......Page 1828
Complications and Pitfalls......Page 1830
122. Hamstring Tendinitis......Page 1834
The Clinical Syndrome......Page 1835
Signs and Symptoms......Page 1836
Testing......Page 1837
Differential Diagnosis......Page 1838
Treatment......Page 1839
Complications and Pitfalls......Page 1844
123. Pes Anserine Bursitis......Page 1848
The Clinical Syndrome......Page 1849
Signs and Symptoms......Page 1850
Testing......Page 1851
Treatment......Page 1855
Complications and Pitfalls......Page 1856
section 12. Ankle and Foot Pain Syndromes......Page 1861
124. Subtalar Joint Pain......Page 1863
The Clinical Syndrome......Page 1864
Signs and Symptoms......Page 1865
Testing......Page 1866
Differential Diagnosis......Page 1868
Treatment......Page 1870
Complications and Pitfalls......Page 1873
125. Midtarsal Joint Pain......Page 1877
The Clinical Syndrome......Page 1878
Signs and Symptoms......Page 1879
Testing......Page 1882
Differential Diagnosis......Page 1883
Treatment......Page 1884
Complications and Pitfalls......Page 1885
126. Posterior Tibial Tendinitis......Page 1889
The Clinical Syndrome......Page 1890
Signs and Symptoms......Page 1891
Testing......Page 1894
Differential Diagnosis......Page 1895
Treatment......Page 1899
Complications and Pitfalls......Page 1900
127. Achilles Bursitis......Page 1904
The Clinical Syndrome......Page 1905
Signs and Symptoms......Page 1906
Testing......Page 1907
Differential Diagnosis......Page 1908
Treatment......Page 1909
Complications and Pitfalls......Page 1914
128. Anterior Talofibular Pain Syndrome......Page 1918
The Clinical Syndrome......Page 1919
Signs and Symptoms......Page 1920
Testing......Page 1921
Differential Diagnosis......Page 1922
Treatment......Page 1924
Complications and Pitfalls......Page 1925
129. Accessory Navicular Pain Syndrome......Page 1929
The Clinical Syndrome......Page 1930
Signs and Symptoms......Page 1931
Testing......Page 1932
Differential Diagnosis......Page 1933
Treatment......Page 1936
Complications and Pitfalls......Page 1937
130. Fibulocalcaneal Pain Syndrome......Page 1944
The Clinical Syndrome......Page 1945
Signs and Symptoms......Page 1946
Testing......Page 1947
Differential Diagnosis......Page 1948
Treatment......Page 1952
Complications and Pitfalls......Page 1953
131. Os Trigonum Pain Syndrome......Page 1957
The Clinical Syndrome......Page 1958
Signs and Symptoms......Page 1959
Testing......Page 1960
Differential Diagnosis......Page 1963
Treatment......Page 1964
Complications and Pitfalls......Page 1965
132. Bunionette Pain......Page 1971
The Clinical Syndrome......Page 1972
Signs and Symptoms......Page 1973
Testing......Page 1974
Differential Diagnosis......Page 1975
Treatment......Page 1976
Complications and Pitfalls......Page 1979
133. Sesamoiditis......Page 1983
The Clinical Syndrome......Page 1984
Signs and Symptoms......Page 1985
Testing......Page 1987
Differential Diagnosis......Page 1988
Treatment......Page 1991
Complications and Pitfalls......Page 1992
134. Metatarsalgia......Page 1996
The Clinical Syndrome......Page 1997
Signs and Symptoms......Page 1998
Testing......Page 1999
Differential Diagnosis......Page 2000
Treatment......Page 2003
Complications and Pitfalls......Page 2004
135. Submetatarsal Adventitial Bursitis......Page 2008
The Clinical Syndrome......Page 2009
Signs and Symptoms......Page 2010
Testing......Page 2011
Differential Diagnosis......Page 2012
Treatment......Page 2015
Complications and Pitfalls......Page 2016
Index......Page 2020

Citation preview

Atlas of Uncommon Pain Syndromes FOURTH EDITION

Steven D. Waldman, MD, JD Vice Dean, Chairman and Professor, Department of Humanities and Bioethics, University of Missouri—Kansas City, Kansas City, Missouri

Table of Contents Cover image Title page Copyright Dedication Preface

section 1. Headache and Facial Pain Syndromes 1. Ice Pick Headache Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 2. Supraorbital Neuralgia Clinical Syndrome

Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 3. Chronic Paroxysmal Hemicrania Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 4. Hemicrania Continua Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 5. Charlin Syndrome

Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 6. Sexual Headache The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 7. Cough Headache The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls

8. Sudden Unilateral Neuralgiform Conjunctival Injection Tearing Headache The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 9. Primary Thunderclap Headache The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 10. Hypnic Headache The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment

Complications and Pitfalls 11. Nummular Headache The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 12. Headache Associated With Temporal Arteritis The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 13. Post–Dural Puncture Headache The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis

Treatment Complications and Pitfalls 14. Ramsay Hunt Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Nerve Blocks Opioid Analgesics Adjuvant Analgesics Antiviral Agents Adjunctive Treatments Complications and Pitfalls 15. Eagle Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment

Complications and Pitfalls 16. Atypical Odontalgia The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 17. Burning Mouth Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 18. Nervus Intermedius Neuralgia The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis

Treatment Complications and Pitfalls 19. Red Ear Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 20. Glossopharyngeal Neuralgia The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls

section 2. Neck and Brachial Plexus Pain Syndromes 21. Clival Chordoma Syndrome

The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 22. Spasmodic Torticollis The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 23. Cervicothoracic Interspinous Bursitis The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls

24. Scapulocostal Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 25. Parsonage-Turner Syndrome The Clinical Syndrome Signs and Symptoms Differential Diagnosis Testing Treatment Complications and Pitfalls 26. Hyoid Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment

Complications and Pitfalls 27. Omohyoid Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 28. Acute Calcific Prevertebral Tendinitis The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 29. Neck-Tongue Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis

Treatment Complications and Pitfalls 30. Sternohyoid Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls

section 3. Shoulder Pain Syndromes 31. Supraspinatus Tendinitis The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 32. Infraspinatus Tendinitis

The Clinical Syndrome Signs And Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 33. Subacromial Impingement Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 34. Os Acromiale Pain Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls

35. Glomus Tumor of the Shoulder The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 36. Pectoralis Major Tear Syndrome Pectoralis Muscle Tear Pectoralis Muscle Tendon Rupture The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 37. Suprascapular Nerve Entrapment The Clinical Syndrome Signs and Symptoms Testing

Differential Diagnosis Treatment Complications and Pitfalls 38. Snapping Scapula Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential diagnosis Treatment Complications and Pitfalls 39. Quadrilateral Space Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls

section 4. Elbow Pain Syndromes

40. Pronator Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 41. Cubital Bursitis The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 42. Anconeus Epitrochlearis The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment

Complications and Pitfalls 43. Os Supratrochleare-Related Elbow Pain The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 44. Osteonecrosis of the Elbow Joint The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 45. Triceps Tendinitis The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis

Treatment Complications and Pitfalls 46. Radial Tunnel Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 47. Cubital Tunnel Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 48. Driver ’s Elbow The Clinical Syndrome Signs and Symptoms Testing

Differential Diagnosis Treatment Complications and Pitfalls 49. Anterior Interosseous Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls

section 5. Wrist and Hand Pain Syndromes 50. Ulnar Tunnel Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls

51. Cheiralgia Paresthetica The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 52. Secretan Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 53. Foreign Body Synovitis The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment

Complications and Pitfalls 54. Glomus Tumor of the Hand The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 55. Erythromelalgia The Clinical Syndrome Testing Differential Diagnosis Treatment Complications and Pitfalls 56. Boxer ’s Knuckle The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment

Complications and Pitfalls 57. Triangular Fibrocartilage Tear Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 58. Scapholunate Ligament Tear Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 59. Lunotriquetral Instability Pain Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis

Treatment Complications and Pitfalls 60. Kienböck Disease The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 61. Avascular Necrosis of the Scaphoid The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 62. Extensor Carpi Ulnaris Tendinitis The Clinical Syndrome Signs and Symptoms Testing

Differential Diagnosis Treatment Complications and Pitfalls 63. Flexor Carpi Radialis Tendinitis The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 64. Trigger Wrist The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls

section 6. Thoracic Pain Syndromes

65. Devil’s Grip The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 66. Sternoclavicular Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 67. Postmastectomy Pain The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment

Complications and Pitfalls 68. Sternalis Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 69. Manubriosternal Joint Pain The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 70. Xiphodynia The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis

Treatment Complications and Pitfalls 71. Serratus Anterior Muscle Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 72. Slipping Rib Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 73. Winged Scapula Syndrome The Clinical Syndrome Signs and Symptoms Testing

Differential Diagnosis Treatment Complications and Pitfalls

section 7. Abdominal and Groin Pain Syndromes 74. Anterior Cutaneous Nerve Entrapment The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 75. Acute Intermittent Porphyria The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls

76. Radiation Enteritis The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 77. Liver Pain The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 78. Abdominal Angina The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment

Complications and Pitfalls

section 8. Lumbar Spine and Sacroiliac Joint Pain Syndromes 79. Epidural Abscess The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 80. Multiple Myeloma The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 81. Foix-Alajouanine Syndrome The Clinical Syndrome

Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 82. Paget Disease The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 83. Diffuse Idiopathic Skeletal Hyperostosis The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 84. Spondylolisthesis

The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 85. Ankylosing Spondylitis The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 86. Superior Cluneal Nerve Entrapment Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls

87. Lumbar Myofascial Pain Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 88. Lumbar Paraspinal Compartment Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls

section 9. Pelvic Pain Syndromes 89. Gluteus Maximus Pain Syndrome The Clinical Syndrome Signs and Symptoms Testing

Differential Diagnosis Treatment Complications and Pitfalls 90. Gluteus Medius Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 91. Gluteal Bursitis The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 92. Clunealgia The Clinical Syndrome Signs and Symptoms

Testing Differential Diagnosis Treatment Complications and Pitfalls 93. Nutcracker Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 94. Orchialgia The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 95. Clitoral Priapism The Clinical Syndrome

Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 96. Vulvodynia The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 97. Prostatodynia The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 98. Levator Ani Pain Syndrome

The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 99. Proctalgia Fugax The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 100. Paroxysmal Extreme Pain Disorder The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls

section 10. Hip and Lower Extremity Pain Syndromes 101. Avascular Necrosis of the Hip The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 102. Transient Regional Osteoporosis The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 103. Psoas Bursitis The Clinical Syndrome Signs and Symptoms Testing

Differential Diagnosis Treatment Complications and Pitfalls 104. Iliopsoas Tendon Rupture The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 105. Femoral Neuropathy The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 106. Saphenous Neuralgia The Clinical Syndrome Signs and Symptoms

Testing Differential Diagnosis Treatment Complications and Pitfalls 107. Obturator Neuralgia The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 108. Adductor Tendinitis The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 109. Iliopectinate Bursitis The Clinical Syndrome

Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 110. Snapping Hip Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls

section 11. Knee Pain Syndromes 111. Tibiofibular Pain Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment

Complications and Pitfalls 112. Jumper ’s Knee The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 113. Semimembranosus Insertion Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 114. Coronary Ligament Strain The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis

Treatment Complications and Pitfalls 115. Breaststroker ’s Knee The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 116. Quadriceps Expansion Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 117. Runner ’s Knee The Clinical Syndrome Signs and Symptoms Testing

Differential Diagnosis Treatment Complications and Pitfalls 118. Snapping Pes Anserinus Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 119. Glomus Tumor of the Knee The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 120. Iliotibial Band Bursitis The Clinical Syndrome Signs and Symptoms

Testing Differential Diagnosis Treatment Complications and Pitfalls 121. Fabella Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 122. Hamstring Tendinitis The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 123. Pes Anserine Bursitis The Clinical Syndrome

Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls

section 12. Ankle and Foot Pain Syndromes 124. Subtalar Joint Pain The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 125. Midtarsal Joint Pain The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment

Complications and Pitfalls 126. Posterior Tibial Tendinitis The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 127. Achilles Bursitis The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 128. Anterior Talofibular Pain Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis

Treatment Complications and Pitfalls 129. Accessory Navicular Pain Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 130. Fibulocalcaneal Pain Syndrome The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 131. Os Trigonum Pain Syndrome The Clinical Syndrome Signs and Symptoms Testing

Differential Diagnosis Treatment Complications and Pitfalls 132. Bunionette Pain The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 133. Sesamoiditis The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls 134. Metatarsalgia The Clinical Syndrome Signs and Symptoms

Testing Differential Diagnosis Treatment Complications and Pitfalls 135. Submetatarsal Adventitial Bursitis The Clinical Syndrome Signs and Symptoms Testing Differential Diagnosis Treatment Complications and Pitfalls Index

Copyright 1600 John F. Kennedy Blvd. Ste 1800 Philadelphia, PA 19103-2899 ATLAS OF UNCOMMON PAIN SYNDROMES, FOURTH EDITION 978-0-323-64077-0 Copyright © 2020 by Elsevier Inc. All rights reserved.

ISBN:

No part of this publication may be reproduced or transmitted in any form or by any means, electronic or mechanical, including photocopying, recording, or any information storage and retrieval system, without permission in writing from the publisher. Details on how to seek permission, further information about the Publisher ’s permissions policies and our arrangements with organizations such as the Copyright Clearance Center and the Copyright Licensing Agency, can be found at our website: www.elsevier.com/permissions. This book and the individual contributions contained in it are protected under copyright by the Publisher (other than as may be noted herein).

Notices Practitioners and researchers must always rely on their own experience and knowledge in evaluating and using any information, methods, compounds or experiments described herein. Because of rapid advances in the medical sciences, in particular, independent verification of diagnoses and drug dosages should be made. To the fullest extent of the law, no responsibility is assumed by Elsevier, authors, editors or contributors for any injury and/or damage to persons or property as a matter of products liability, negligence or otherwise, or from any use or operation of any methods, products, instructions, or ideas contained in the material herein.

Previous editions copyrighted 2014, 2008, 2003. Library of Congress Control Number: 2019939394 Executive Content Strategist: Michael Houston Senior Content Development Manager: Luke Held Senior Content Development Specialist: Ann Ruzycka Anderson Publishing Services Manager: Catherine Jackson Senior Project Manager: Sharon Corell Design Direction: Patrick Ferguson Printed in China Last digit is the print number:

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Dedication This book is dedicated to David Mayo My little boy yesterday, my friend today, my son forever. SDW 2019

Preface

What Occam, KISS, Zebras, and Mickey Gilley All Have In Common It has been said that the three most dangerous things in medicine are: (1) a medical student with a sharp object; (2) a resident armed with a recently published study from NEJM, and; (3) an attending physician with an anecdote. One must suspect that #2 was at play when in the 1940’s, while on rounds at the University of Maryland Hospital in Baltimore, Maryland, Theodore Woodward, MD, stated that “If you hear hoof beats out on Green Street, don’t look for zebras”! How this admonition to aspiring physicians morphed into “when you hear hoof beats, look for horses, not zebras” is anybody’s guess. (My son who was an ophthalmology resident in Baltimore suggests that this sage piece of advice was most likely accompanied by a long-winded and confusing anecdote—see #3 above). On the surface, most of us would agree with Dr. Woodward’s logic that the most common things are the most common. Occam agreed, when in the 14th century he put forth the philosophical tenant of parsimony that proposed that simpler explanations are, all things being equal, almost always better than more complex ones. He used a razor to “shave away” unnecessary or extraneous data to get to the simplest solution. When you think about it, a razor was all the rage as a medical instrument in the 14th century, so it is not surprising that Occam chose it as his preferred medical device. Occam’s razor certainly has a nice ring to it—better than Occam’s MRI—which would no doubt be the name of his maxim if he had lived in the 21st century, given that the MRI is certainly our most current popular medical device for “shaving away” extraneous data. Which brings us to KISS. Not the Gene Simmons’ rock band KISS, but the admonition “Keep It Simple Stupid.” KISS was set forth by Lockhead aeronautical engineer Kelly Johnson when he handed his design team a few simple tools and challenged them to design military jets that could be easily fixed with the simple tools that were available in combat situations. It is still not exactly clear to me who was “stupid,” but I certainly hope it is not the guys who fix the jets I fly on. KISS makes sense when designing jet engines, but one has to ask what KISS has to do with the individual patient—the sick one—the scared one—the one you worry about in the middle of the night. Unfortunately, very little. Because for the individual patient with a difficult

diagnosis, it turns out that a guy named Harry Hickam was probably more correct than Occam. While on teaching rounds at Duke University, Harry Hickam, MD, admonished his students and residents that “patients can have as many diseases as they damn well please”! (See also #3 above.) He correctly posited that when diagnosing the individual patient, using Occam’s razor often provides the correct diagnosis. But more often than we would care to admit, when dealing with a patient presenting with a perplexing constellation of signs and symptoms, it can just as easily provide the wrong one. In fact, overreliance on Occam’s razor can be downright dangerous for both the patient and physician. Often the simplest or, in the case of medical diagnosis, the most common illness is exactly what is causing the patient’s symptoms. But sometimes, in our almost obsessive desire to make the diagnosis, simplicity is our enemy. In our haste to make the patient fit the diagnosis, we get it wrong. Uncommon diseases are called uncommon diseases because they are uncommon—they are not called unknown diseases (see below). Since the beginning of time healers have recognized that the correct diagnosis is the key to getting the patient well, and as a corollary, they also realized that the wrong diagnosis is not a “practice builder.” Which brings us to country music legend Mickey Gilley. In 1976 Mickey Gilley recorded the classic country ballad “The Girl’s All Get Prettier At Closing Time. (FYI, 12 straight weeks charted at #1). This song is a plaintive lament about loneliness and late-night desperation. It explores how one’s perception of things can change as circumstances change. What turns an unknown and undiagnosable disease into an uncommon disease is knowledge. What changes our perception of what a constellation of symptoms and physical findings mean when we are confronted with a sick patient with an elusive diagnosis is knowledge. As we gain more clinical experience, things that were once unknown become known—even commonplace. The more we hone our clinical acumen, the easier it is to put the pieces together—the jumble of disparate signs and symptoms come into focus—and then all of a sudden, we have a diagnosis—a diagnosis we will never miss again! The Atlas of Uncommon Pain Syndromes, Fourth Edition, seeks to accomplish three things: The first is to familiarize the clinician with a group of

uncommon pain syndromes that occur with enough frequency that they merit serious study—not rare or orphan diseases—just uncommon ones that are often misdiagnosed. Second, this text is written with the goal of helping the clinician reinforce his or her knowledge of common pain syndromes to help in those situations when Occam is relatively correct—when the pieces of the puzzle don’t quite fit the simple diagnosis. The third goal is more about the clinician and a little less about the patient. It is about what attracted many of us to medicine to begin with. It is the irresistible charm of being presented with a difficult clinical problem and getting it right. What a great feeling that is! I hope you enjoy reading Atlas of Uncommon Pain Syndromes, Fourth Edition, as much as I did writing it. Steven D. Waldman, MD, JD PS: The following ad recently ran in a local Kansas newspaper. Perhaps the hoof beats were zebras after all! Tamed Young Male and Female Zebras for Sale ($2,000.00) I have available young Male and Female zebra babies for sale to good and lovely homes who knows about the tamed zebras, feel free to send us emails for more details and pictures, please only serious inquiries. Category: Pets »Horses Ad ID: 1564632 Date: May 16, 2018

SECT ION 1

Headache and Facial Pain Syndromes OUT LINE 1. Ice Pick Headache 2. Supraorbital Neuralgia 3. Chronic Paroxysmal Hemicrania 4. Hemicrania Continua 5. Charlin Syndrome 6. Sexual Headache 7. Cough Headache 8. Sudden Unilateral Neuralgiform Conjunctival Injection Tearing Headache 9. Primary Thunderclap Headache 10. Hypnic Headache 11. Nummular Headache 12. Headache Associated With Temporal Arteritis 13. Post–Dural Puncture Headache 14. Ramsay Hunt Syndrome 15. Eagle Syndrome 16. Atypical Odontalgia

17. Burning Mouth Syndrome 18. Nervus Intermedius Neuralgia 19. Red Ear Syndrome 20. Glossopharyngeal Neuralgia

1

Ice Pick Headache

Abstract Ice pick headache is a constellation of symptoms consisting of paroxysms of stabbing jabs and jolts that occur primarily in the first division of the trigeminal nerve. These paroxysms of pain may occur as a single jab or a series of jabs that last for a fraction of a second followed by relatively pain-free episodes. The pain of ice pick headache occurs in irregular intervals of hours to days. Similar to cluster headache, ice pick headache is an episodic disorder that is characterized by “clusters” of painful attacks followed by pain-free periods. Synonyms for ice pick headache include jabs and jolts headache, primary stabbing headache, ophthalmodynia periodica, and idiopathic stabbing headache.

Keywords autonomic trigeminal cephalgia; cluster headache; headache; ophthalmodynia periodica; primary stabbing headache; trigeminal neuralgia

ICD-10 CODE R51

Clinical Syndrome Ice pick headache is a constellation of symptoms consisting of paroxysms of stabbing jabs and jolts that occur primarily in the first division of the trigeminal nerve. These paroxysms of pain may occur as a single jab or a series of jabs that lasts for a fraction of a second followed by relatively painfree episodes. The pain of ice pick headache occurs in irregular intervals of hours to days. Similar to cluster headache, ice pick headache is an episodic disorder that is characterized by clusters of painful attacks followed by painfree periods. Episodes of ice pick headache usually occur on the same side, but in rare patients the pain may move to the same anatomical region on the contralateral side. Ice pick headache occurs more commonly in women and is generally not seen before the fourth decade of life, but rare reports of children suffering from ice pick headache sporadically appear in the literature. Synonyms for ice pick headache include jabs and jolts headache, primary stabbing headache, ophthalmodynia periodica, and idiopathic stabbing headache.

Signs and Symptoms A patient suffering from ice pick headache complains of jolts or jabs of pain in the orbit, temple, or parietal region (Fig. 1.1). Some patients describe the pain of ice pick headache as a sudden smack or slap on the side of the head. Similar to patients suffering from trigeminal neuralgia, a patient suffering from ice pick headache may exhibit involuntary muscle spasms of the affected area in response to the paroxysms of pain. In contrast to trigeminal neuralgia, involving the first division of the trigeminal nerve, there are no trigger areas that induce the pain of ice pick headache. The neurological examination of a patient suffering from ice pick headache is normal. Some patients exhibit anxiety and depression because the intensity of pain associated with ice pick headache leads many patients to believe they have a brain tumor.

Testing Magnetic resonance imaging (MRI) of the brain provides the best information regarding the cranial vault and its contents. MRI is highly accurate and helps to identify abnormalities that may put the patient at risk for neurological disasters secondary to intracranial and brainstem pathological conditions, including tumors and demyelinating disease (Fig. 1.2). Magnetic resonance angiography (MRA) also may be useful in helping identify aneurysms, which may be responsible for the patient’s neurological findings. In patients who cannot undergo MRI, such as a patient with a pacemaker, computed tomography (CT) is a reasonable second choice. Radionuclide bone scanning and plain radiography are indicated if fracture or bony abnormality, such as metastatic disease, is considered in the differential diagnosis.

FIG. 1.1 Ice pick headache is characterized by jabs or jolts in the orbit, temple, or parietal region.

FIG. 1.2 Diffuse Pachymeningeal and Calvarial Metastasis From Carcinoma of the BreastAxial T1-weighted postgadolinium MRI shows diffuse nodular and bandlike contrast-enhanced thickening of the dura over the high right frontoparietal convexity (arrow). From Haaga JR, Lanzieri CF, Gilkeson RC, eds. CT and MR Imaging of the Whole Body. 4th ed. Philadelphia: Mosby; 2003:198.

Screening laboratory tests consisting of complete blood cell count, erythrocyte sedimentation rate, and automated blood chemistry should be performed if the diagnosis of ice pick headache is in question. Intraocular pressure should be measured if glaucoma is suspected.

Differential Diagnosis Ice pick headache is a clinical diagnosis supported by a combination of clinical history, normal physical examination, radiography, and MRI. Pain syndromes that may mimic ice pick headache include trigeminal neuralgia involving the first division of the trigeminal nerve, demyelinating disease, and chronic paroxysmal hemicrania. Trigeminal neuralgia involving the first division of the trigeminal nerve is uncommon and is characterized by trigger areas and tic-like movements. Demyelinating disease is generally associated with other neurological findings, including optic neuritis and other motor and sensory abnormalities. The pain of chronic paroxysmal hemicrania lasts much longer than the pain of ice pick headache and is associated with redness and watering of the ipsilateral eye.

Treatment Ice pick headache uniformly responds to treatment with indomethacin. Failure to respond to indomethacin puts the diagnosis of ice pick headache in question. A starting dosage of 25 mg daily for 2 days and titrating to 25 mg three times per day is a reasonable treatment approach. This dose may be carefully increased to 150 mg per day. Indomethacin must be used carefully, if at all, in patients with peptic ulcer disease or impaired renal function. Anecdotal reports of a positive response to cyclooxygenase-2 (COX-2) inhibitors in the treatment of ice pick headache have been noted in the headache literature. Underlying sleep disturbance and depression are best treated with a tricyclic antidepressant compound, such as nortriptyline, which can be started at a single bedtime dose of 25 mg.

Complications and Pitfalls Failure to correctly diagnose ice pick headache may put the patient at risk if intracranial pathological conditions or demyelinating disease, which may mimic the clinical presentation of chronic paroxysmal hemicrania, is overlooked. MRI is indicated in all patients thought to be suffering from ice pick headache. Failure to diagnose glaucoma, which also may cause intermittent ocular pain, may result in permanent loss of sight.

Clinical Pearls The diagnosis of ice pick headache is made by obtaining a thorough, targeted headache history. Patients suffering from ice pick headache should have a normal neurological examination. If the results of the neurological examination are abnormal, the diagnosis of ice pick headache should be discarded and a careful search for the cause of the neurological findings should be undertaken.

Suggested Readings Cutrer F.M, Boes C.J. Cough, exertional, and sex headaches. Neurol Clin. 2004;22:133–149. Dafer R.M. Neurostimulation in headache disorders. Neurol Clin. 2010;28:835–841. Loulwah O, Jan M.M.S. Primary stabbing “ice-pick” headache. Pediatric Neurology. 2011;45(4):268. Matthew N.T. Indomethacin responsive headache syndromes: headache. J Head Face Pain. 1981;21:147–150. Pascual J. Other primary headaches. Neurol Clin. 2009;27:557–571. Rampello L, Malaguarnera M, Rampello L, et al. Stabbing headache in patients with autoimmune disorders. Clin Neurol Neurosurg. 2012;114(6):751–753. Tuğba T, Serap Ü, Esra O, et al. Features of stabbing, cough, exertional and sexual headaches in a Turkish population of headache patients. J Clin Neurosci. 2008;15:774–777.

2

Supraorbital Neuralgia

Abstract The pain of supraorbital neuralgia is characterized as persistent pain in the supraorbital region and forehead with occasional sudden, shock-like paresthesias in the distribution of the supraorbital nerves. Sinus headache involving the frontal sinuses, which is much more common than supraorbital neuralgia, can mimic the pain of supraorbital neuralgia. Supraorbital neuralgia is the result of compression or trauma of the supraorbital nerves as the nerves exit the supraorbital foramen. Such trauma can be in the form of blunt trauma directly to the nerve, such as when the forehead hits the steering wheel during a motor vehicle accident, or repetitive microtrauma resulting from wearing welding or swim goggles that are too tight. This clinical syndrome also is known as swimmer ’s headache.

Keywords chronic paroxysmal hemicrania; first division trigeminal nerve; headache; neuralgia; sinus headache; supraorbital nerve block; supraorbital neuralgia; swimmer’s headache; ultrasound guided nerve block

ICD-10 CODE G50.0

Clinical Syndrome The pain of supraorbital neuralgia is characterized as persistent pain in the supraorbital region and forehead with occasional sudden, shock-like paresthesias in the distribution of the supraorbital nerves. Sinus headache involving the frontal sinuses, which is much more common than supraorbital neuralgia, can mimic the pain of supraorbital neuralgia. Supraorbital neuralgia is the result of compression or trauma of the supraorbital nerves as the nerves exit the supraorbital foramen. Such trauma can be in the form of blunt trauma directly to the nerve, such as when the forehead hits the steering wheel during a motor vehicle accident, or repetitive microtrauma resulting from wearing welding or swim goggles that are too tight. This clinical syndrome also is known as swimmer ’s headache.

Signs and Symptoms The supraorbital nerve arises from fibers of the frontal nerve, which is the largest branch of the ophthalmic nerve. The frontal nerve enters the orbit via the superior orbital fissure and passes anteriorly beneath the periosteum of the roof of the orbit. The frontal nerve gives off a larger lateral branch, the supraorbital nerve, and a smaller medial branch, the supratrochlear nerve. Both exit the orbit anteriorly. The supraorbital nerve sends fibers all the way to the vertex of the scalp and provides sensory innervation to the forehead, upper eyelid, and anterior scalp (Fig. 2.1). The pain of supraorbital neuralgia is characterized as persistent pain in the supraorbital region and forehead with occasional sudden, shock-like paresthesias in the distribution of the supraorbital nerves. Occasionally, a patient suffering from supraorbital neuralgia complains that the hair on the front of the head hurts (Fig. 2.2). Supraorbital nerve block is useful in the diagnosis and treatment of supraorbital neuralgia.

Testing Magnetic resonance imaging (MRI) of the brain provides the best information regarding the cranial vault and its contents. MRI is highly accurate and helps identify abnormalities that may put the patient at risk for neurological disasters secondary to intracranial and brainstem pathological conditions, including tumors and demyelinating disease (Fig. 2.3). Magnetic resonance angiography (MRA) also may be useful in helping identify aneurysms, which may be responsible for the patient’s neurological findings. In patients who cannot undergo MRI, such as a patient with a pacemaker, computed tomography (CT) is a reasonable second choice. Radionuclide bone scan, CT, and plain radiography are indicated if sinus disease, fracture, or bony abnormality such as metastatic disease is considered in the differential diagnosis. Screening laboratory tests consisting of complete blood cell count, erythrocyte sedimentation rate, and automated blood chemistry testing should be performed if the diagnosis of supraorbital neuralgia is in question. Intraocular pressure should be measured if glaucoma is suspected (Fig. 2.4).

Differential Diagnosis Supraorbital neuralgia is a clinical diagnosis supported by a combination of clinical history, normal physical examination, radiography, CT, and MRI. Pain syndromes that may mimic supraorbital neuralgia include ice pick headache, trigeminal neuralgia involving the first division of the trigeminal nerve, demyelinating disease, and chronic paroxysmal hemicrania. Trigeminal neuralgia involving the first division of the trigeminal nerve is uncommon and is characterized by trigger areas and tic-like movements. Demyelinating disease is generally associated with other neurological findings, including optic neuritis and other motor and sensory abnormalities. The pain of chronic paroxysmal hemicrania lasts much longer than the paroxysmal pain of supraorbital neuralgia and is associated with redness and watering of the ipsilateral eye.

Treatment The primary treatment intervention for supraorbital neuralgia is the identification and removal of anything causing compression of the supraorbital nerves (e.g., tight welding or swim goggles). A brief trial of simple analgesics alone or in combination with gabapentin also should be considered. For patients who do not respond to these treatments, supraorbital nerve block with local anesthetic and a steroid is a reasonable next step. Ultrasound guidance for needle placement may be useful when performing supraorbital nerve block.

FIG. 2.1 The supraorbital nerve sends fibers all the way to the vertex of the scalp and provides sensory innervation to the forehead, upper eyelid, and anterior scalp. n., Nerve.

FIG. 2.2 Occasionally a patient with supraorbital neuralgia complains that the hair on the front of the head hurts. The supraorbital nerve sends fibers all the way to the vertex of the scalp and provides sensory innervation to the forehead, upper eyelid, and anterior scalp.

To perform supraorbital nerve block, the patient is placed in the supine position. Using a 10-mL sterile syringe, 3 mL of local anesthetic is drawn up. When treating supraorbital neuralgia with supraorbital nerve block, 80 mg of depot steroid is added to the local anesthetic with the first block, and 40 mg of depot steroid is added with subsequent blocks.

The supraorbital notch on the affected side is identified by palpation. The skin overlying the notch is prepared with antiseptic solution, with care taken to avoid spillage into the eye. A 25-gauge, 1½-inch needle is inserted at the level of the supraorbital notch and is advanced medially approximately 15 degrees off the perpendicular to avoid entering the foramen. The needle is advanced until it approaches the periosteum of the underlying bone (Fig. 2.5). A paresthesia may be elicited, and the patient should be warned of such. The needle should not enter the supraorbital foramen; if this occurs, the needle should be withdrawn and redirected slightly more medially. Because of the loose alveolar tissue of the eyelid, a gauze sponge should be used to apply gentle pressure on the upper eyelid and supraorbital tissues before injection of solution to prevent the injectate from dissecting inferiorly into these tissues. This pressure should be maintained after the procedure to avoid periorbital hematoma and ecchymosis. After gentle aspiration, 3 mL of solution is injected in a fanlike distribution. If blockade of the supratrochlear nerve also is desired, the needle is redirected medially and, after careful aspiration, an additional 3 mL of solution is injected in a fanlike manner. In rare cases, destruction of the supraorbital nerve by radiofrequency lesioning or supraorbital nerve stimulation may be required to provide long-lasting relief (Fig. 2.6). Underlying sleep disturbance and depression associated with the pain of supraorbital neuralgia are best treated with a tricyclic antidepressant compound, such as nortriptyline. The tricyclic antidepressant can be started at a single bedtime dose of 25 mg.

Complications and Pitfalls Failure to diagnose supraorbital neuralgia correctly may put the patient at risk if an intracranial pathological condition or demyelinating disease, which may mimic the clinical presentation of supraorbital neuralgia, is overlooked. MRI is indicated in all patients thought to have supraorbital neuralgia. Failure to diagnose glaucoma, which also may cause intermittent ocular pain, may result in permanent loss of sight. The forehead and scalp are highly vascular, and when performing supraorbital nerve block the clinician should carefully calculate the total milligram dosage of local anesthetic that may be given safely, especially if bilateral nerve blocks are being performed. This vascularity gives rise to an increased incidence of postblock ecchymosis and hematoma formation. Despite the vascularity of this anatomical region, this technique can be performed safely in the presence of anticoagulation by using a 25- or 27gauge needle, albeit at increased risk for hematoma, if the clinical situation dictates a favorable risk-to-benefit ratio. These complications can be decreased if manual pressure is applied to the area of the block immediately after injection. Application of cold packs for 20-minute periods after the block also decreases the amount of postprocedure pain and bleeding.

FIG. 2.3 Subdural Empyema in a Patient With Sinusitis(A) T2-weighted MRI shows high-signal-intensity extraaxial fluid collection in the right frontal convexity and along the falx on the right side. (B and C) Gadolinium-enhanced MRI shows extraaxial fluid collections in the right frontal convexity and along the falx with intense peripheral enhancement. The signal intensity of the fluid collection is slightly higher than that of cerebrospinal fluid. From Haaga JR, Lanzieri CF, Gilkeson RC, eds. CT and MR Imaging of the Whole Body. 4th ed. Philadelphia: Mosby; 2003:209.

FIG. 2.4 Acute Angle Closure Resulting From an Intumescent Cataractous LensThe eye is red with a hazy view of the anterior segment from corneal edema, with a fixed, irregular, semidilated pupil from iris infarction. The slit image shows the corneal edema and a very shallow anterior chamber. Some uveitis may be present because of ischemia, and this must be differentiated from the larger accumulations of lens material and macrophages seen with phacolytic glaucoma. From Spalton DJ, Hitchings RA, Hunter P. Atlas of Clinical Ophthalmology. 3rd ed. London: Mosby; 2005:225.

FIG. 2.5 Injection Technique for Relieving the Pain of Supraorbital Neuralgian., Nerve. From Waldman SD. Atlas of Pain Management Injection Techniques. 2nd ed. Philadelphia: Saunders; 2007.

FIG. 2.6 A three-dimensional CT reconstruction, taken prior to the procedure, showing the position of the supraorbital foramen (arrow). From Huibin Q, Jianxing L, Guangyu H, et al. The treatment of first division idiopathic trigeminal neuralgia with radiofrequency thermocoagulation of the peripheral branches compared to conventional radiofrequency. J Clin Neurosci. 2009;16(11):1425–1429. ISSN 0967-5868, https://doi.org/10.1016/j.jocn.2009.01.021.

Clinical Pearls Supraorbital nerve block is especially useful in the diagnosis and palliation of pain secondary to supraorbital neuralgia. The first step in the management of this unusual cause of headache is the correct fitting of swimming goggles that do not compress the supraorbital nerves. Coexistent frontal sinusitis should be ruled out in patients who do not respond rapidly to a change in swim goggles and a series of the previously mentioned nerve blocks. Any patient with headaches severe enough to require neural

blockade as part of the treatment plan should undergo MRI of the head to rule out unsuspected intracranial pathological conditions.

Suggested Readings Hillerup S, Jensen R.H, Ersbøll B.K. Trigeminal nerve injury associated with injection of local anesthetics: needle lesion or nneurotoxicity? J Am Dental Assoc. 2011;142(5):531–539. Levin M. Nerve blocks and nerve stimulation in headache disorders. Tech Reg Anesth Pain Manage. 2009;13:42–49. Levin M. Nerve blocks in the treatment of headache. Neurotherapeutics. 2010;7:197–203. Waldman S.D. Swimmer ’s headache. In: Waldman S.D, ed. Atlas of Pain Management Injection Techniques. Philadelphia: Saunders; 2007:7–10. Waldman S.D. The trigeminal nerve. In: Waldman S.D, ed. Pain Review. Philadelphia: Saunders; 2009:15–17.

3

Chronic Paroxysmal Hemicrania

Abstract Chronic paroxysmal hemicrania, which is also known as Sjaastad syndrome, shares many characteristics of its more common analogue, cluster headache, but has several important differences. Similar to cluster headache, chronic paroxysmal hemicrania is a severe, episodic, unilateral headache that affects the periorbital and retroorbital regions. In contrast to cluster headache, which occurs 10 times more commonly in men, chronic paroxysmal hemicrania occurs primarily in women. The duration of pain associated with chronic paroxysmal hemicrania is shorter than that of cluster headache, lasting 5 to 45 minutes. This pain does not follow the chronobiological pattern seen in patients with cluster headache. Patients with chronic paroxysmal hemicrania usually experience more than five attacks per day. Chronic paroxysmal hemicrania uniformly responds to indomethacin, whereas cluster headache does not.

Keywords autonomic trigeminal cephalgias; chronic paroxysmal hemicranias; cluster headache; Horner syndrome; indomethacin; indomethacin responsive headaches; Sjaastad syndrome

ICD-10 CODE R51

Clinical Syndrome Chronic paroxysmal hemicrania, which is also known as Sjaastad syndrome, shares many characteristics of its more common analogue, cluster headache, but has several important differences (Table 3.1). Similar to cluster headache, chronic paroxysmal hemicrania is a severe, episodic, unilateral headache that affects the periorbital and retroorbital regions. In contrast to cluster headache, which occurs 10 times more commonly in men, chronic paroxysmal hemicrania occurs primarily in women (Fig. 3.1). The duration of pain associated with chronic paroxysmal hemicrania is shorter than that of cluster headache, lasting 5 to 45 minutes. This pain does not follow the chronobiological pattern seen in patients with cluster headache. Patients with chronic paroxysmal hemicrania usually experience more than five attacks per day. Chronic paroxysmal hemicrania uniformly responds to indomethacin, whereas cluster headache does not.

Signs and Symptoms During attacks of chronic paroxysmal hemicrania, patients exhibit the following physical findings suggestive of Horner syndrome on the ipsilateral side of the pain: • Conjunctival and scleral injection • Lacrimation • Nasal congestion • Rhinorrhea • Ptosis of the eyelid As in cluster headache, the patient may become agitated during attacks, rather than seeking dark and quiet as does the patient with migraine. In contrast to cluster headache, alcohol consumption does not seem to trigger attacks of chronic paroxysmal hemicrania. Between attacks, the neurological examination of a patient with chronic paroxysmal hemicrania should be normal.

Testing Magnetic resonance imaging (MRI) of the brain provides the best information regarding the cranial vault and its contents. MRI is highly accurate and helps to identify abnormalities that may put the patient at risk for neurological disasters secondary to intracranial and brainstem pathological conditions, including tumors and demyelinating disease (Fig. 3.2). Magnetic resonance angiography (MRA) also may be useful in identifying aneurysms, which may be responsible for the patient’s neurological findings. In patients who cannot undergo MRI, such as a patient with a pacemaker, computed tomography (CT) is a reasonable second choice. Radionuclide bone scanning and plain radiography are indicated if fracture or bony abnormality such as metastatic disease is considered in the differential diagnosis. TABLE 3.1 Comparison of Cluster Headache and Chronic Paroxysmal Hemicrania Comparison Factors Gender predominance

Cluster Headache Male

Chronic Paroxysmal Hemicrania Female

Response to indomethacin

Negative

Positive

Chronobiological pattern

Positive

Negative

Alcohol trigger

Positive

Negative

Length of attacks

Longer

Shorter

Horner syndrome

Present

Present

FIG. 3.1 In contrast to cluster headache, which occurs primarily in men, chronic paroxysmal hemicrania occurs primarily in women.

FIG. 3.2 Sagittal (A) and semiaxial (B) T2-weighted images of a massive prolactinoma in a 41-year-old man with chronic daily headache. From Benitez-Rosario MA, McDarby G, Doyle R, et al. Chronic cluster-like headache secondary to prolactinoma: uncommon cephalalgia in association with brain tumors. J Pain Symptom Manage. 2009;37:271–276.

Screening laboratory tests consisting of complete blood cell count, erythrocyte sedimentation rate, and automated blood chemistry testing should be performed if the diagnosis of chronic paroxysmal hemicrania is in question. Intraocular pressure should be measured if glaucoma is suspected.

Differential Diagnosis Chronic paroxysmal hemicrania is a clinical diagnosis supported by a combination of clinical history, abnormal physical examination during attacks, radiography, and MRI. Pain syndromes that may mimic chronic paroxysmal hemicrania include cluster headache, trigeminal neuralgia involving the first division of the trigeminal nerve, demyelinating disease, and ice pick headache. Trigeminal neuralgia involving the first division of the trigeminal nerve is uncommon and is characterized by trigger areas and ticlike movements. Demyelinating disease is generally associated with other neurological findings, including optic neuritis and other motor and sensory abnormalities. The pain of cluster headache lasts much longer than the pain of chronic paroxysmal hemicrania, and cluster headache has a male predominance, a chronobiological pattern of attacks, and a lack of response to treatment with indomethacin (Fig. 3.3).

Treatment Chronic paroxysmal hemicrania uniformly responds to treatment with indomethacin. Failure to respond to indomethacin puts the diagnosis of chronic paroxysmal hemicrania in question. A starting dose of 25 mg daily for 2 days and titrating to 25 mg three times per day is a reasonable treatment approach. This dose may be carefully increased up to 150 mg per day. Indomethacin must be used carefully, if at all, in patients with peptic ulcer disease or impaired renal function. Anecdotal reports of a positive response to cyclooxygenase-2 (COX-2) inhibitors in the treatment of chronic paroxysmal hemicrania have been noted in the headache literature. Underlying sleep disturbance and depression are best treated with a tricyclic antidepressant compound, such as nortriptyline, which can be started at a single bedtime dose of 25 mg.

FIG. 3.3 Overlap between attack duration in trigeminal autonomic cephalalgias. The duration of each trigeminal autonomic cephalalgia is specified by the International Classification of Headache Disorders. From Silberstein SD, Vodovskaia N. Trigeminal autonomic cephalalgias other than cluster headache. Med Clin North Am. 2013;97(2):321–328.

TABLE 3.2 Characteristics of the Trigeminal Autonomic Cephalgias • Unilateral • Short duration • High frequency • Orbital, periorbital, or temporal • Associated autonomic symptoms • Lacrimation • Conjunctival injection • Nasal congestion • Ptosis • Eyelid edema

Complications and Pitfalls Failure to diagnose chronic paroxysmal hemicrania correctly may put the patient at risk if intracranial pathological conditions or demyelinating disease that may mimic the clinical presentation of chronic paroxysmal hemicrania is overlooked. MRI is indicated in all patients thought to have chronic paroxysmal hemicrania. Failure to diagnose glaucoma, which may cause intermittent ocular pain, may result in permanent loss of sight.

Clinical Pearls Chronic paroxysmal hemicrania is classified as a trigeminal autonomic cephalgia. The trigeminal autonomic cephalgias are a group of distinct headache syndromes that share a number of common and often overlapping clinical characteristics (Table 3.2). The diagnosis of chronic paroxysmal hemicrania is made by obtaining a thorough, targeted headache history. Between attacks, patients with chronic paroxysmal hemicrania should have a normal neurological examination. If the neurological examination is abnormal between attacks, the diagnosis of chronic paroxysmal hemicrania should be discarded and a careful search for the cause of the patient’s neurological findings should be undertaken.

Suggested Readings Benitez-Rosario M.A, McDarby G, Doyle R, Fabby C. Chronic cluster-like headache secondary to prolactinoma: uncommon cephalalgia in association with brain tumors. J Pain Symptom Manage. 2009;37:271–276. Benoliel R, Sharav Y. Paroxysmal hemicrania: case studies and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endodontol. 1998;85:285–292. Camarda C, Camarda R, Monastero R. Chronic paroxysmal hemicrania and hemicrania continua responding to topiramate: two case reports. Clin Neurol Neurosurg. 2008;110:88–91. Klasser G.D, Balasubramaniam R. Trigeminal autonomic cephalalgias. II. Paroxysmal hemicrania. Oral Surg Oral Med Oral Pathol Oral Radiol Endodontol. 2007;104:640–646. Schytz H.W, Hargreaves R, Ashina M. Challenges in developing drugs for primary headaches. Prog Neurobiol. 2017;152:70–88. Sjaastad O. Chronic paroxysmal hemicrania: clinical aspects and controversies. In: Blau J.N, ed. Migraine: Clinical, Therapeutic, Conceptual and Research Aspects. London: Chapman & Hall; 1987:135–152. Silberstein S.D, Vodovskaia N. Trigeminal autonomic cephalalgias other than cluster headache. Med Clin North Am. 2013;97(2):321–328. Talvik I, Koch K, Kolk A, Talvik T. Chronic paroxysmal hemicrania in a 3-year, 10-month-old female. Pediatr Neurol. 2006;34:225–227.

4

Hemicrania Continua

Abstract Hemicrania continua is a primary headache disorder that is classified as a trigeminal autonomic cephalgia that shares characteristics of both cluster headache and migraine headache (Table 4.1). Similar to cluster headache, hemicrania continua is a severe, unilateral headache with associated signs of autonomic dysfunction including lacrimation, scleral injection, eyelid ptosis, nasal stuffiness syndrome. In contrast to cluster headache, which occurs 10 times more commonly in men, hemicrania continua occurs more commonly in women, a characteristic it shares with migraine. Like migraine headache, hemicranias continua is associated with nausea and vomiting as well as sonophobia and phonophobia. Unlike the pain of cluster and migraine headache, the pain of hemicraina continua is continuous with intermittent severe exacerbations of pain. This pain is unilateral and is side-locked, that is that it does not change sides like migraine headache occasionally does. Hemicrania continua is an indomethacin responsive headache, with complete resolution of headache and fassociated symptoms with therapeutic doses of indomethacin.

Key words autonomic trigeminal cephalgias; chronic paroxysma hemicranias; cluster headache; hemicrania continuas; horner’s syndrome; indomethacin; indomethacin responsive headaches; sjaastad syndrome; trigeminal autonomic cephalgias; unilateral headaches

ICD-10 CODE G44.51

Clinical Syndrome Hemicrania continua is a primary headache disorder that is classified as a trigeminal autonomic cephalgia that shares characteristics of both cluster headache and migraine headache (Table 4.1). Similar to cluster headache, hemicrania continua is a severe, unilateral headache with associated signs of autonomic dysfunction, including lacrimation, scleral injection, eyelid ptosis, and nasal stuffiness syndrome (Fig. 4.1). In contrast to cluster headache, which occurs 10 times more commonly in men, hemicrania continua occurs more commonly in women, a characteristic it shares with migraine. Like migraine headache, hemicrania continua is associated with nausea and vomiting, as well as sonophobia and phonophobia. Unlike the pain of cluster and migraine headache, the pain of hemicrania continua is continuous with intermittent severe exacerbations of pain. This pain is unilateral and is side locked (i.e., it does not change sides like migraine headache occasionally does). Hemicrania continua is an indomethacin responsive headache, with complete resolution of headache and associated symptoms with therapeutic doses of indomethacin. The cause of hemicrania continua is unknown, but like other trigeminal autonomic cephalgias, functional magnetic resonance scanning and positron emission tomography reveal activation in the posterior hypothalamus during exacerbation of headaches.

Signs and Symptoms During attacks of hemicrania continua, patients exhibit the following physical findings suggestive of Horner syndrome on the ipsilateral side of the pain: • Conjunctival and scleral injection • Lacrimation • Nasal congestion • Rhinorrhea • Ptosis of the eyelid As in cluster headache, the patient may become agitated during attacks, rather than seeking dark and quiet as does the patient with migraine. In contrast to cluster headache, alcohol consumption does not seem to trigger attacks of hemicrania continua. Between attacks, the neurological examination of a patient with hemicrania continua should be normal.

Testing Magnetic resonance imaging (MRI) of the brain provides the best information regarding the cranial vault and its contents. MRI is highly accurate and helps identify abnormalities that may put the patient at risk for neurological disasters secondary to intracranial and brainstem pathological conditions, including tumors and demyelinating disease (Fig. 4.2). Magnetic resonance angiography (MRA) also may be useful in identifying aneurysms, which may be responsible for the patient’s neurological findings. In patients who cannot undergo MRI, such as a patient with a pacemaker, computed tomography (CT) is a reasonable second choice. Radionuclide bone scanning and plain radiography are indicated if fracture or bony abnormality such as metastatic disease is considered in the differential diagnosis. Positron emission tomography may help further delineate and characterize tumors responsible for the patient’s pain and neurological symptoms. Screening laboratory tests consisting of complete blood cell count, erythrocyte sedimentation rate, and automated blood chemistry testing should be performed if the diagnosis of hemicrania continua is in question. Intraocular pressure should be measured if glaucoma is suspected. TABLE 4.1 Comparison of Cluster Headache and Hemicrania Continua

FIG. 4.1 Hemicrania continua is a unilateral side-locked headache with associated signs of autonomic dysfunction. In contrast to cluster headache, which occurs primarily in men, hemicrania continua occurs primarily in women.

Differential Diagnosis Hemicrania continua is a clinical diagnosis supported by a combination of clinical history, abnormal physical examination during exacerbation of baseline headache, radiography, and MRI. Pain syndromes that may be confused for hemicrania continua include cluster headache, chronic paroxysmal hemicrania, trigeminal neuralgia involving the first division of the trigeminal nerve, demyelinating disease, ice pick headache, and other indomethacin responsive headaches. Trigeminal neuralgia involving the first division of the trigeminal nerve is uncommon and is characterized by trigger areas and tic-like movements. Demyelinating disease is generally associated with other neurological findings, including optic neuritis and other motor and sensory abnormalities. The pain of cluster headache is episodic, whereas the pain of hemicrania continua is continuous with acute severe exacerbations (Fig. 4.3). Cluster headache also has a male predominance, a chronobiological pattern of attacks, and a lack of response to treatment with indomethacin.

Treatment Hemicrania continua uniformly responds to treatment with indomethacin. Failure to respond to indomethacin puts the diagnosis of hemicrania continua in question. A starting dose of 25 mg daily for 2 days and titrating to 25 mg three times per day is a reasonable treatment approach. This dose may be carefully increased up to 150 mg per day. Indomethacin must be used carefully, if at all, in patients with peptic ulcer disease or impaired renal function. Anecdotal reports of a positive response to cyclooxygenase-2 (COX2) inhibitors in the treatment of hemicrania continua have been noted in the headache literature. Underlying sleep disturbance and depression are best treated with a tricyclic antidepressant compound, such as nortriptyline, which can be started at a single bedtime dose of 25 mg.

Complications and Pitfalls Failure to diagnose hemicrania continua correctly may put the patient at risk if intracranial pathological conditions or demyelinating disease, which may mimic the clinical presentation of hemicrania continua, is overlooked. MRI is indicated in all patients thought to have hemicrania continua. Failure to diagnose glaucoma, which may cause intermittent ocular pain, may result in permanent loss of sight.

Clinical Pearls Hemicrania continua is classified as a trigeminal autonomic cephalgia. The trigeminal autonomic cephalgias are a group of distinct headache syndromes that share a number of common and often overlapping clinical characteristics (Table 4.2). The diagnosis of hemicrania continua is made by obtaining a thorough, targeted headache history. Between attacks, patients with hemicrania continua should have a normal neurological examination. If the neurological examination is abnormal between attacks, the diagnosis of hemicrania continua should be discarded and a careful search for the cause of the patient’s neurological findings should be undertaken.

FIG. 4.2 A 57-Year-Old Woman With Recurrent GBMPostcontrast T1-weighted image (A) and rCBV map (B) demonstrate two adjacent necrotic, peripherally enhancing lesions with elevated rCBV (white arrow) consistent with recurrent GBM. After subtotal reresection of the peripheral lesion and 2 weeks of treatment with TMZ

and bevacizumab, postcontrast T1-weighted image (C) and rCBV map (D) show reduced size and decreased intensity of enhancement of the residual medial necrotic rim-enhancing lesion with no significant rCBV elevation (white arrow). Findings are consistent with response to treatment. The patient had a relatively long OS (>17 months) after bevacizumab initiation. From Boxerman JL, Shiroishi MS, Ellingson BM, et al. Dynamic susceptibility contrast MR imaging in glioma: review of current clinical practice. Magn Reson Imaging Clin N Am. 2016;24(4):649–670. rCBV, Cerebral blood volume; GBM, glioblastoma multiforme; OS, overall survival rate; TMZ, temozolomide.

FIG. 4.3 The Temporal Relationships of Trigeminal Autonomic Cephalalgias. Modified from Silberstein SD, Vodovskaia N. Trigeminal autonomic cephalalgias other than cluster headache. Med Clin North Am. 2013;97(2):321–328.

TABLE 4.2 Characteristics of the Trigeminal Autonomic Cephalgias • Unilateral • Short duration • High frequency • Orbital, periorbital, or temporal • Associated autonomic symptoms • Lacrimation • Conjunctival injection • Nasal congestion • Ptosis • Eyelid edema

Suggested Readings Benitez-Rosario M.A, McDarby G, Doyle R, Fabby C. Chronic cluster-like headache secondary to prolactinoma: uncommon cephalalgia in association with brain tumors. J Pain Symptom Manage. 2009;47:271–276. Evans R.W. Case studies of uncommon and rare headache disorders. Neurol Clin. 2016;34(3):631–650. Jay G.W, Barkin R.L. Primary headache disorders, Part I: migraine and the trigeminal autonomic cephalalgias. Dis Mon. 2017;63(11):308–338. Schytz H.W, Hargreaves R, Ashina M. Challenges in developing drugs for primary headaches. Prog Neurobiol. 2017;152:70–88. Silberstein S.D, Vodovskaia N. Trigeminal autonomic cephalalgias other than cluster headache. Med Clin North Am. 2014;97(2):421–428.

5

Charlin Syndrome

Abstract Charlin’s syndrome, also known as nasociliary neuralgia and CharlinSluder cephalgia, is an uncommon cause of head and face pain. As with most headache syndromes, the exact cause of the pain of Charlin’s syndrome is unknown. However, the pathogenesis of this uncommon cause of head and face pain is thought to be dysfunction of the nasociliary ganglion in a manner analogous to the dysfunction of the sphenopalatine ganglion thought to be the source of cluster headache. The pain of Charlin’s syndrome has a rapid onset to peak, with attacks lasting 45 to 60 minutes. In some patients, these attacks can be triggered by sensory stimulation of the affected areas. Although in many ways similar to cluster headache (e.g., retroorbital location of pain, profuse unilateral rhinorrhea, rapid onset to peak, and short duration of attacks), many dissimilarities also exist. In contrast to cluster headache, alcohol consumption does not appear to trigger attacks of Charlin’s syndrome and the seasonal and chronobiological patterns so characteristic of cluster headache do not seem to be a factor.

Key Words Charlin’s syndrome; Charlin-Sluder syndrome; eye pain; facial pain; headache; nasociliary ganglion; nasociliary nerve block cluster headache; nasociliary neuralgia

ICD-10 CODE G50.0

Clinical Syndrome Charlin syndrome, also known as nasociliary neuralgia and Charlin-Sluder cephalgia, is an uncommon cause of head and face pain. As with most headache syndromes, the exact cause of the pain of Charlin syndrome is unknown. However, the pathogenesis of this uncommon cause of head and face pain is thought to be dysfunction of the nasociliary ganglion in a manner analogous to the dysfunction of the sphenopalatine ganglion thought to be the source of cluster headache. The pain of Charlin syndrome has a rapid onset to peak, with attacks lasting 45 to 60 minutes. In some patients, these attacks can be triggered by sensory stimulation of the affected areas. Although in many ways similar to cluster headache (e.g., retroorbital location of pain, profuse unilateral rhinorrhea, rapid onset to peak, and short duration of attacks), many dissimilarities also exist. In contrast to cluster headache, alcohol consumption does not appear to trigger attacks of Charlin syndrome and the seasonal and chronobiological patterns so characteristic of cluster headache do not seem to be a factor (Table 5.1). Blockade of the sphenopalatine ganglion, which is so effective in the treatment of cluster headache, is of little value in the treatment of Charlin syndrome. Patients suffering from Charlin syndrome uniformly respond to daily nasociliary nerve blocks with local anesthetic, as described subsequently.

Signs and Symptoms Patients suffering from Charlin syndrome present with the complaint of severe paroxysms of ocular or retroorbital pain that radiates into the ipsilateral forehead, nose, and maxillary region. This pain is associated with voluminous ipsilateral rhinorrhea and congestion of the nasal mucosa and significant inflammation of the affected eye (Fig. 5.1).

Testing Magnetic resonance imaging (MRI) of the brain provides the best information regarding the cranial vault and its contents. MRI is highly accurate and helps identify abnormalities that may put the patient at risk for neurological disasters secondary to intracranial and brainstem pathological conditions, including tumors and demyelinating disease (Fig. 5.2). Magnetic resonance angiography (MRA) also may be useful in helping identify aneurysms, which may be responsible for the patient’s neurological findings. In patients who cannot undergo MRI, such as a patient with a pacemaker, computed tomography (CT) is a reasonable second choice. Radionuclide bone scanning and plain radiography are indicated if fracture or bony abnormality such as metastatic disease is considered in the differential diagnosis. Screening laboratory tests consisting of complete blood cell count, erythrocyte sedimentation rate, and automated blood chemistry testing should be performed if the diagnosis of Charlin syndrome is in question. Intraocular pressure should be measured if glaucoma is suspected.

Differential Diagnosis Charlin syndrome is a clinical diagnosis supported by a combination of clinical history, normal physical examination, radiography, and MRI. Pain syndromes that may mimic Charlin syndrome include cluster headache, temporal arteritis, trigeminal neuralgia involving the first division of the trigeminal nerve, demyelinating disease, and chronic paroxysmal hemicrania (see Table 5.1). Trigeminal neuralgia involving the first division of the trigeminal nerve is uncommon and is characterized by trigger areas and ticlike movements. Demyelinating disease is generally associated with other neurological findings, including optic neuritis and other motor and sensory abnormalities. The pain of chronic paroxysmal hemicrania lasts much longer than the pain of Charlin syndrome.

Treatment The treatment of Charlin syndrome is analogous to the treatment of trigeminal neuralgia. The use of anticonvulsants such as carbamazepine and gabapentin represents a reasonable starting point. High-dose steroids tapered over 10 days also have been anecdotally reported to provide relief. For patients who do not respond to the previously mentioned treatments, daily nasociliary ganglion block with local anesthetic and steroid is a reasonable next step. Underlying sleep disturbance and depression associated with the pain of supraorbital neuralgia are best treated with a tricyclic antidepressant compound, such as nortriptyline, which can be started at a single bedtime dose of 25 mg. Table 5.1 Comparison of Cluster Headache and Charlin Syndrome Comparison Factors Ocular and retroorbital location

Cluster Headache Yes

Charlin Syndrome Yes

Unilateral

Yes

Yes

Rapid onset to peak

Yes

Yes

Severe intensity

Yes

Yes

Attacks occur in paroxysms

Yes

Yes

Duration of attacks short

Yes

Yes

Pain free between attacks

Yes

Yes

Significant rhinorrhea during attacks

Yes

Yes

Alcohol triggers attacks

Yes

No

Tactile trigger areas

No

Yes

Seasonal pattern of attacks

Yes

No

Chronobiological pattern of attacks

Yes

No

Significant eye inflammation

No

Yes

Responds to sphenopalatine ganglion block

Yes

No

Responds to nasociliary block

No

Yes

FIG. 5.1 Patients suffering from Charlin syndrome present with the complaint of severe paroxysms of ocular or retroorbital pain that radiates into the ipsilateral forehead, nose, and maxillary region. The pain is associated with voluminous ipsilateral rhinorrhea and congestion of the nasal mucosa and significant inflammation of the affected eye.

FIG. 5.2 Multiple SclerosisFluid-attenuated inversion recovery (FLAIR) parasagittal MR image depicts the extensive demyelinated plaques of progressive multiple sclerosis. From Haaga JR, Lanzieri CF, Gilkeson RC, eds. CT and MR Imaging of the Whole Body. 4th ed. Philadelphia: Mosby; 2003:466.

Complications and Pitfalls Failure to diagnose Charlin syndrome correctly may put the patient at risk if an intracranial pathological condition or demyelinating disease, which may mimic the clinical presentation of Charlin syndrome, is overlooked. MRI is indicated in all patients thought to have Charlin syndrome. Failure to diagnose glaucoma or temporal arteritis, which also may cause intermittent ocular pain, may result in permanent loss of sight.

Clinical Pearls Nasociliary nerve block via the medial orbital approach is especially useful in the diagnosis and palliation of pain secondary to Charlin syndrome. Given the uncommon nature of this headache syndrome and its overlap with the symptoms of cluster headache and other neurological problems, including cavernous sinus thrombosis and intracranial and retroorbital tumors, Charlin syndrome must remain a diagnosis of exclusion. All patients suspected to have Charlin syndrome require MRI of the brain with and without gadolinium contrast material and thorough ophthalmological and neurological evaluation. Nasociliary nerve block via the medial orbital approach should be performed only by clinicians familiar with the regional anatomy.

Suggested Readings Becker M, Kohler R, Vargas M.I, Viallon M, Delavelle J. Pathology of the trigeminal nerve. Neuroimaging Clin N Am. 2008;18:283–307. Craven J. Anatomy of the cranial nerves. Anaesth Intensive Care Med. 2010;11:529–534. Lewis D.W, Gozzo Y.F, Avner M.T. The “other ” primary headaches in children and adolescents [review]. Pediatr Neurol. 2005;33:303–313. Waldman S.D. The trigeminal nerve. In: Waldman S.D, ed. Pain Review. Philadelphia: Saunders; 2009:15–17. Waldman S.D. Charlin’s syndrome. In: Waldman S.D, ed. Atlas of Pain Management Injection Techniques. Philadelphia: Saunders; 2007:20–24.

6

Sexual Headache

Abstract Sexual headache, which is also known as primary headache associated with sexual activity, is a term used to describe a group of headaches associated with sexual activity. Clinicians have identified the following three general types of headache associated with sexual activity: (1) Explosive type; (2) Dull type; (3)Postural type.In general, sexual headache includes a benign group of disorders, but a rare patient may have acute subarachnoid hemorrhage during sexual activity, which may be erroneously diagnosed as the benign explosive type of sexual headache. There is no gender predilection for sexual headache, and the occurrence of all types of sexual headache may be episodic rather than chronic. Rarely, more than one type of sexual headache occurs in the same patient. Sexual headaches have been associated with the use of cannabis, pseudoephedrine, oral contraceptives, and amiodarone.

Key words dull sexual headache; explosive sexual headache; indomethacin; magnesium; posterual sexual headache; primary headache associated with sexual activity; propranolol; sexual headache; thunderclap headache

ICD-10 CODE R51

The Clinical Syndrome Sexual headache, which is also known as primary headache associated with sexual activity, is a term used to describe a group of headaches associated with sexual activity. Clinicians have identified the following three general types of headache associated with sexual activity: • Explosive type • Dull type • Postural type Each of these sexual headache types was previously called benign coital headache, but this term has been replaced by sexual headache because each may occur with sexual activity other than coitus (Fig. 6.1). In general, sexual headache includes a benign group of disorders, but a rare patient may have acute subarachnoid hemorrhage during sexual activity, which may be erroneously diagnosed as the benign explosive type of sexual headache. There is no gender predilection for sexual headache, and the occurrence of all types of sexual headache may be episodic rather than chronic. Rarely, more than one type of sexual headache occurs in the same patient. Sexual headaches have been associated with the use of cannabis, pseudoephedrine, oral contraceptives, and amiodarone.

Signs and Symptoms Patients with sexual headache present differently depending on the type of sexual headache experienced. Each clinical presentation is discussed subsequently.

Explosive Type of Sexual Headache The explosive type of sexual headache is the most common type of sexual headache encountered in clinical practice. The patient usually fears he or she has had a stroke. The patient may be less forthcoming about the circumstances surrounding the onset of headache, and tactful questioning may be required to ascertain the actual clinical history. The explosive type of sexual headache occurs suddenly, with an almost instantaneous onset to peak just before or during orgasm. The intensity of the explosive type of sexual headache is severe and has been likened to the pain of acute subarachnoid hemorrhage. The location of pain is usually occipital, but some patients volunteer that the pain felt “like the top of my head was going to blow off.” The pain is usually bilateral, but isolated cases of unilateral explosive sexual headache have been reported. The pain usually remains intense for 10 to 15 minutes and then gradually abates. Some patients note some residual headache pain for 2 days.

Dull Type of Sexual Headache The dull type of sexual headache begins during the early portion of sexual activity. This headache type has an aching character and begins in the occipital region. The headache becomes holocranial as sexual activity progresses toward orgasm. It may peak at orgasm, but in contrast to the explosive type of sexual headache, the dull type disappears rapidly after orgasm. Ceasing sexual activity usually aborts the dull type of sexual headache. Some headache specialists think the dull type of sexual headache is simply a milder version of the explosive type of sexual headache.

Postural Type of Sexual Headache The postural type of sexual headache is similar to the explosive type of sexual headache in that it occurs just before or during orgasm. Its rapid onset to peak and severe intensity also are similar to that of the explosive type. It

differs from the explosive type of headache in that the headache symptoms recur when the patient stands up, in a manner analogous to postdural puncture headache. The postural component of this type of sexual headache is thought to be due to minute tears in the dura that may occur during intense sexual activity.

FIG. 6.1 Sexual headaches show no gender predilection and are generally benign.

FIG. 6.2 Brain magnetic resonance angiography performed 1 month after sexual headache onset showing multifocal vasoconstriction in the left posterior cerebral artery (arrows). From Hu CM, Lin YJ, Fan YK, et al. Isolated thunderclap headache during sex: orgasmic headache or reversible cerebral vasoconstriction syndrome? J Clin Neurosci. 2010;17[10]:1349–1351, fig. 1, ISSN 0967-5868, https://doi.org/10.1016/j.jocn.2010.01.052. http://www.sciencedirect.com/science/article/pii/S0967586810002419.

Testing Magnetic resonance imaging (MRI) of the brain provides the best information regarding the cranial vault and its contents. MRI is highly accurate and helps identify abnormalities that may put the patient at risk for neurological disasters secondary to intracranial and brainstem pathological conditions, including tumors, demyelinating disease, and hemorrhage. More importantly, MRI helps identify bleeding associated with leaking intracranial aneurysms. Magnetic resonance angiography (MRA) and cerebral arteriography may be useful in helping identify aneurysms or other arterial abnormalities responsible for the patient’s neurological symptoms (Figs 6.2 and 6.3). In patients who cannot undergo MRI, such as patients with pacemakers, computed tomography (CT) is a reasonable second choice. Even if blood is not present on MRI or CT, if intracranial hemorrhage is suspected, lumbar puncture should be performed. Screening laboratory tests consisting of complete blood cell count, erythrocyte sedimentation rate, and automated blood chemistry testing should be performed if the diagnosis of sexual headache is in question. Intraocular pressure should be measured if glaucoma is suspected.

Differential Diagnosis Sexual headache is a clinical diagnosis supported by a combination of clinical history, normal physical examination, radiography, MRI, and MRA. Pain syndromes that may mimic sexual headache include trigeminal neuralgia involving the first division of the trigeminal nerve, demyelinating disease, cluster headache, migraine, and chronic paroxysmal hemicrania. Trigeminal neuralgia involving the first division of the trigeminal nerve is uncommon and is characterized by trigger areas and tic-like movements. Demyelinating disease is generally associated with other neurological findings, including optic neuritis and other motor and sensory abnormalities. The pain of chronic paroxysmal hemicrania and cluster headache is associated with redness and watering of the ipsilateral eye, nasal congestion, and rhinorrhea during the headache. These findings are absent in all types of sexual headache. Migraine headache may or may not be associated with nonpainful neurological findings known as aura, but the patient almost always reports some systemic symptoms, such as nausea or photophobia, not typically associated with sexual headache.

FIG. 6.3 Cerebral angiogram revealed segmental (boundaries denoted by large arrows) irregularities (small arrows) of the basilar artery in both anterior-posterior (A) and lateral (B) views. From Delasobera BE, Osborn SR, Davis JE. Thunderclap headache with orgasm: a case of basilar artery dissection associated with sexual intercourse. J Emerg Med. 2012;43[1]:e43–e47, fig. 1.

Treatment It is generally thought that avoiding the inciting activity for a few weeks decreases the propensity to trigger sexual headaches. If this avoidance technique fails or is impractical because of patient preference, a trial of propranolol is a reasonable next step. A low dose of 20 to 40 mg as a daily dose and titrating in 20-mg increments to 200 mg as a divided daily dose until prophylaxis occurs treats most patients suffering from sexual headache. Propranolol should be used with caution in patients with asthma or cardiac failure and patients with brittle diabetes. If propranolol is ineffective, indomethacin may be tried. A starting dose of 25 mg daily for 2 days and titrating to 25 mg three times per day is a reasonable treatment approach. This dose may be carefully increased to 150 mg per day. Indomethacin must be used carefully, if at all, in patients with peptic ulcer disease or impaired renal function. Anecdotal reports of a positive response to cyclooxygenase-2 (COX-2) inhibitors and magnesium in the treatment of sexual headache have been noted in the headache literature. Underlying sleep disturbance and depression are best treated with a tricyclic antidepressant compound, such as nortriptyline, which can be started at a single bedtime dose of 25 mg.

Complications and Pitfalls Failure to diagnose sexual headache correctly may put the patient at risk if intracranial pathology or demyelinating disease, which may mimic the clinical presentation of sexual headache, is overlooked. MRI, MRA, and occasionally cerebral angiography are indicated in all patients thought to have sexual headache. Failure to diagnose glaucoma, which also may cause intermittent ocular pain, may result in permanent loss of sight.

Clinical Pearls The diagnosis of sexual headache is made by obtaining a thorough, targeted headache history. As mentioned earlier, patients may not be forthcoming about the events surrounding the onset of their headache, and the clinician should be sensitive to this fact. Patients suffering from sexual headache should have a normal neurological examination. If the neurological examination is abnormal, the diagnosis of sexual headache should be discarded and a careful search for the cause of the patient’s neurological findings should be undertaken.

Suggested Readings Delasobera B.E, Osborn S.R, Davis J.E. Thunderclap headache with orgasm: a case of basilar artery dissection associated with sexual intercourse. J Emerg Med. 2012;43(1):e43–e47. Evans R.W. Diagnostic testing for migraine and other primary headaches. Neurol Clin. 2009;27:393–415. Hu C.M, Lin Y.J, Fan Y.K, et al. Isolated thunderclap headache during sex: orgasmic headache or reversible cerebral vasoconstriction syndrome? J Clin Neurosci. 2010;17:1349–1351. Jolobe O.M.P. The differential diagnosis includes reversible cerebral vasoconstrictor syndrome. Am J Emerg Med. 2010;28:637. Kim H.J, Seo S.Y. Recurrent emotion-triggered headache following primary headache associated with sexual activity. J Neurol Sci. 2008;273:142–143. Tuğba T, Serap Ü, Esra O, et al. Features of stabbing, cough, exertional and sexual headaches in a Turkish population of headache patients. J Clin Neurosci. 2008;15:774–777.

7

Cough Headache

Abstract Cough headache is a term used to describe headaches triggered by coughing and other activities associated with a Valsalva maneuver, such as laughing, straining at stool, lifting, and bending the head toward the ground (Figure 6.1). Clinicians have identified the following two types of cough headache: (1)Benign primary cough headache; (2) Symptomatic cough headache. Initially, both types of cough headache were thought to be related to sexual and exertional headaches, but they are now considered distinct clinical entities. A strong male predilection is seen for benign cough headache and no gender predilection for symptomatic cough headache.

Key Words Arnold-Chiari Type I malformation; Cough Headache; exertional headache; indomethacin; sexual headache; symptomatic cough headache; Valsalva maneuver

ICD-10 CODE R51

The Clinical Syndrome Cough headache is a term used to describe headaches triggered by coughing and other activities associated with a Valsalva maneuver, such as laughing, straining at stool, lifting, and bending the head toward the ground (Fig. 7.1). Clinicians have identified the following two types of cough headache: • Benign primary cough headache • Symptomatic cough headache Initially, both types of cough headache were thought to be related to sexual and exertional headaches, but they are now considered distinct clinical entities. A strong male predilection is seen for benign cough headache and no gender predilection for symptomatic cough headache.

Signs and Symptoms Patients suffering from cough headache present differently depending on the type of cough headache experienced. Each clinical presentation is discussed.

Benign Cough Headache Benign cough headache is not associated with obvious neurological or musculoskeletal disease. More than 80% of patients with benign cough headache are males, in contradistinction to symptomatic cough headache, in which no gender predilection is seen. The onset of benign cough headache is abrupt, occurring immediately after coughing or other activities that cause a Valsalva maneuver. Although the intensity of pain is severe and peaks rapidly, it lasts only seconds to minutes. The character of the pain associated with benign cough headache is splitting or sharp, and the pain is in the occipital region bilaterally and occasionally the vertex of the skull. No accompanying neurological or systemic symptoms are seen, as with cluster and migraine headaches. The age of onset of benign cough headache is generally in the late fifth or sixth decade of life. If such headaches occur before age 50, there should be strong clinical suspicion that the patient either has symptomatic cough headache or a pathological condition in the posterior fossa, such as Arnold-Chiari malformation or tumor. Tumors of the foramen magnum also may mimic the presentation of benign cough headache even if no neurological symptoms are present.

Symptomatic Cough Headache Symptomatic cough headache is almost always associated with structural abnormalities of the cranium, such as Arnold-Chiari malformation I and II or intracranial tumors (Fig. 7.2). The symptoms associated with symptomatic cough headache are thought to be due to herniation of the cerebellar tonsil through the foramen magnum into the space normally occupied by the upper portion of the cervical spinal cord. Similar to benign cough headache, the onset of pain associated with symptomatic cough headache is abrupt, occurring immediately after coughing or other activities that cause a Valsalva maneuver. Although the intensity of pain is severe and peaks rapidly, it lasts only seconds to minutes. In contrast to benign cough headache, associated neurological symptoms may be present, including difficulty swallowing, faintness, and numbness in the face and upper extremities. These associated

symptoms should be taken very seriously because they are indicative of increased intracranial pressure and herniation of the intracranial contents. The character of the pain associated with symptomatic cough headache is splitting or sharp, and pain is in the occipital region bilaterally and occasionally the vertex of the skull. The age of onset of symptomatic cough headache is generally in the third decade of life, although, depending on the amount of neurological compromise, it may occur at any age. In contrast to benign cough headache, which occurs predominantly in men, symptomatic cough headache occurs with equal prevalence in both genders.

Testing Magnetic resonance imaging (MRI) of the brain provides the best information regarding the cranial vault and its contents. MRI is highly accurate and helps identify abnormalities that may put the patient at risk for neurological disasters secondary to intracranial and brainstem pathological conditions, including tumors and demyelinating disease. Special attention to the foramen magnum may help identify more subtle abnormalities responsible for posterior fossa neurological signs and symptoms. MRI helps identify bleeding associated with leaking intracranial aneurysms, which may mimic the symptoms of both types of cough headache. Magnetic resonance angiography (MRA) may be useful in helping identify aneurysms responsible for the patient’s neurological symptoms. In patients who cannot undergo MRI, such as patients with pacemakers, computed tomography (CT) is a reasonable second choice. Lumbar puncture should be performed if intracranial hemorrhage is suspected, even if blood is not present on MRI or CT. Plain radiographs of the cervical spine also may be useful in the evaluation of Arnold-Chiari malformations and should be included in the evaluation of all patients with cough headache.

FIG. 7.1 Symptomatic cough headache is often associated with structural abnormalities, such as Arnold-Chiari malformation, and usually occurs in the third decade of life.

FIG. 7.2 Low-lying cerebellar tonsils (straight arrows) of a Chiari malformation are shown deforming the medulla (curved arrow) in a sagittal T1-weighted spin echo image. 4, Fourth ventricle. From Stark DD, Bradley WG Jr, eds. Magnetic Resonance Imaging. 3rd ed. St Louis: Mosby; 1999:1841.

Screening laboratory tests consisting of complete blood cell count, erythrocyte sedimentation rate, and automated blood chemistry testing should be performed if the diagnosis of cough headache is in question. Intraocular pressure should be measured if glaucoma is suspected.

Differential Diagnosis Cough headache is a clinical diagnosis supported by a combination of clinical history, physical examination, radiography, MRI, and MRA. Pain syndromes that may mimic cough headache include benign exertional headache, ice pick headache, sexual headache, trigeminal neuralgia involving the first division of the trigeminal nerve, demyelinating disease, cluster headache, and chronic paroxysmal hemicrania. Trigeminal neuralgia involving the first division of the trigeminal nerve is uncommon and is characterized by trigger areas and tic-like movements. Demyelinating disease is generally associated with other neurological findings, including optic neuritis and other motor and sensory abnormalities. The pain of chronic paroxysmal hemicrania and cluster headache is associated with redness and watering of the ipsilateral eye, nasal congestion, and rhinorrhea during the headache. These findings are absent in all types of cough headache. Migraine headache may or may not be associated with painless neurological findings known as aura, but the patient almost always reports some systemic symptoms, such as nausea or photophobia, not typically associated with cough headache.

Treatment Indomethacin is the treatment of choice for benign cough headache. A starting dose of 25 mg daily for 2 days and titrating to 25 mg three times per day is a reasonable treatment approach. This dose may be carefully increased up to 150 mg per day. Indomethacin must be used carefully, if at all, in patients with peptic ulcer disease or impaired renal function. Headache specialists have noted anecdotal reports of a positive response to cyclooxygenase-2 (COX-2) inhibitors in the treatment of benign cough headache. Underlying sleep disturbance and depression are best treated with a tricyclic antidepressant compound, such as nortriptyline, which can be started at a single bedtime dose of 25 mg. The only uniformly effective treatment for symptomatic cough headache is surgical decompression of the foramen magnum. This surgery is usually done via suboccipital craniectomy. Surgical decompression prevents the lowlying cerebellar tonsils from obstructing the flow of spinal fluid from the cranium to the spinal subarachnoid space during a Valsalva maneuver.

Complications and Pitfalls Failure to diagnose cough headache correctly may put the patient at risk if intracranial pathology or demyelinating disease, which may mimic the clinical presentation of cough headache, is overlooked. MRI and MRA are indicated in all patients thought to have cough headache. Failure to diagnose glaucoma, which also may cause intermittent ocular pain, may result in permanent loss of sight.

Clinical Pearls Any patient presenting with headaches associated with exertion or Valsalva maneuver should be taken very seriously. Although statistically most of these headaches ultimately are proved to be of benign cause, a few patients have potentially life-threatening disease. The diagnosis of cough headache is made by obtaining a thorough, targeted headache history and performing a careful physical examination. The clinician must separate patients suffering from benign cough headache from patients suffering from symptomatic cough headache. Patients with benign cough headache should have a normal neurological examination. If the neurological examination is abnormal, the diagnosis of benign cough headache should be discarded and a careful search for the cause of the patient’s neurological findings should be undertaken.

Suggested Readings Berciano J, Poca M.-A, García A, Sahuquillo J. Paroxysmal cervicobrachial cough-induced pain in a patient with syringomyelia extending into spinal cord posterior gray horns. J Neurol. 2007;54:678–681. Chen Y.Y, Lirng J.F, Fuh J.L, et al. Primary cough headache is associated with posterior fossa crowdedness: a morphometric MRI study. Cephalalgia. 2004;24:694–699. Rothrock J.F. Headaches caused by vascular disorders. Neurol Clin. 2014;32(2):305–319. Cutrer M, DeLange F.J. Cough, exercise, and sex headaches. Neurol Clin. 2014;32(2):433–450. Langridge B, Phillips E, Choi D. Chiari malformation type 1: a systematic review of natural history and conservative management. World Neurosurg. 104:213–219 Pascual J, Rubén Martín A, Oterino A. Headaches precipitated by cough, prolonged exercise or sexual activity: a prospective etiological and clinical study. J Headache Pain. 2008;9:259–266. Pascual J. Primary cough headache. Curr Pain Headache Rep. 2005;9:272–276. Pascual J. Cough and exertional headache, primary. In. Encyclopedia of the Neurological Sciences. 2nd ed. Oxford: Academic Press; 2014:881–884. Waldman S.D. Arnold Chiari malformation type I. In: Waldman S.D, Campbell R.S, eds. Imaging of Pain. Philadelphia: Saunders; 2011:27–28. Waldman S.D. Arnold Chiari malformation type II. In: Waldman S.D, Campbell R.S, eds. Imaging of Pain. Philadelphia: Saunders; 2011:29–30.

8

Sudden Unilateral Neuralgiform Conjunctival Injection Tearing Headache

Abstract Sudden unilateral neuralgiform conjunctival injection tearing (SUNCT) headache is an uncommon primary headache disorder that is one of a group of three headache syndromes known as the trigeminal autonomic cephalgias Whether SUNCT headache is in fact a distinct headache entity or simply a constellation of symptoms that occurs on a continuum along with the other trigeminal autonomic cephalgias is a point of ongoing debate among headache and pain management specialists The pain of SUNCT headache has a rapid onset to peak, with attacks lasting 5 seconds to 4 minutes and the frequency of attacks ranging from 20 to 200 attacks per day.

Key Words anticonvulsants; autonomic dysfunction; short-lasting unilateral neuralgiform headache attack with conjunctival injection and tearing; SUNCT; trigeminal autonomic cephagias; trigeminal neuralgia

ICD-10 CODE G50.0

The Clinical Syndrome Sudden unilateral neuralgiform conjunctival injection tearing (SUNCT) headache is an uncommon primary headache disorder that is one of a group of three headache syndromes known as the trigeminal autonomic cephalgias (Table 8.1). Whether SUNCT headache is in fact a distinct headache entity or simply a constellation of symptoms that occurs on a continuum along with the other trigeminal autonomic cephalgias is a point of ongoing debate among headache and pain management specialists (Fig. 8.1). As with most headache syndromes, the exact cause of the pain of SUNCT headache is unknown; however, the pathogenesis of this uncommon cause of head and face pain is thought to be dysfunction of the trigeminal autonomic reflex. The pain of SUNCT headache has a rapid onset to peak, with attacks lasting 5 seconds to 4 minutes and the frequency of attacks ranging from 20 to 200 attacks per day. In some patients, these attacks can be triggered by sensory stimulation of the affected areas, such as when washing the face, brushing the teeth, and so forth. Although in many ways similar to cluster headache (e.g., unilateral, periorbital, and frontal location of pain, sclera injection, rapid onset to peak, short duration of attacks, and pain-free periods between attacks), SUNCT exhibits many dissimilarities as well. In contrast to cluster headache, alcohol consumption does not seem to trigger attacks of SUNCT headache, and there do not seem to be the seasonal and chronobiological patterns so characteristic of cluster headache, although SUNCT headache occurs most frequently in the morning and afternoon (Table 8.2). Blockade of the sphenopalatine ganglion, which is so effective in the treatment of cluster headache, is of little value in the treatment of SUNCT headache. Patients suffering from SUNCT headache may respond to daily trigeminal nerve blocks with local anesthetic, as described subsequently.

Signs and Symptoms Patients with SUNCT headache present with the complaint of severe paroxysms of ocular or periorbital pain that radiate into the ipsilateral temple, forehead, nose, cheek, throat, and maxillary region. This pain is associated with significant inflammation of the affected eye and associated autonomic signs and symptoms (Figs 8.2 and 8.3). The pain is neuralgiform and severe to excruciating in intensity (Table 8.3). SUNCT occurs on the right side 70% of the time in a manner analogous to trigeminal neuralgia. Like trigeminal neuralgia, rare cases of bilateral SUNCT headache have been reported. Also, like trigeminal neuralgia, the pain of SUNCT headache rarely switches sides. SUNCT headache occurs slightly more frequently in males. It can occur at any age, with a peak incidence in the fifth decade.

Testing Magnetic resonance imaging (MRI) of the brain provides the clinician with the best information regarding the cranial vault, vasculature, and its contents (Fig. 8.4). MRI is highly accurate and helps identify abnormalities that may put the patient at risk for neurological disasters secondary to intracranial and brainstem pathological conditions, including tumors and demyelinating disease. Magnetic resonance angiography (MRA) also may be useful in helping identify aneurysms, which may be responsible for the patient’s neurological findings. In patients who cannot undergo MRI, such as patients with pacemakers, computed tomography (CT) is a reasonable second choice. Radionuclide bone scanning and plain radiography are indicated if fracture or bony abnormality such as metastatic disease is considered in the differential diagnosis.

TABLE 8.1 Trigeminal Autonomic Cephalgias Cluster headache Chronic paroxysm hemicranias Sudden unilateral neuralgiform conjunctival injection tearing headache

FIG. 8.1 Overlap Between Attack Duration in Trigeminal Autonomic CephalalgiasSUNCT, Sudden unilateral neuralgiform conjunctival injection tearing. From Leone M, Bussone G. Pathophysiology of trigeminal autonomic cephalalgias. Lancet Neurol. 2009;8:755–774.

TABLE 8.2 Comparison of Cluster Headache and Sudden Unilateral Neuralgiform Conjunctival Injection Tearing Headache Comparison Factors Ocular and retroorbital location

Cluster Headache Yes

SUNCT Headache Yes

Unilateral

Yes

Yes

Rapid onset to peak

Yes

Yes

Severe intensity

Yes

Yes

Attacks occur in paroxysms

Yes

Yes

Duration of attacks short

Yes

Yes

Pain free between attacks

Yes

Yes

Significant rhinorrhea during attacks

Yes

No

Alcohol triggers attacks

Yes

No

Tactile trigger areas

No

Yes

Seasonal pattern of attacks

Yes

No

Chronobiological pattern of attacks

Yes

No

Significant eye inflammation

No

Yes

Responds to sphenopalatine ganglion block

Yes

No

Responds to trigeminal nerve block

No

Yes

SUNCT, Sudden unilateral neuralgiform conjunctival injection tearing.

FIG. 8.2 Patients with sudden unilateral neuralgiform conjunctival injection tearing headache present with severe paroxysms of ocular or periorbital pain that radiates into the ipsilateral temple, forehead, nose, cheek, throat, and maxillary region that is associated with significant inflammation of the affected eye.

Screening laboratory tests consisting of complete blood cell count, erythrocyte sedimentation rate, and automated blood chemistry testing should be performed if the diagnosis of SUNCT headache is in question. Intraocular pressure should be measured if glaucoma is suspected.

FIG. 8.3 Autonomic signs and symptoms, ipsilateral to the pain, observed in a patient with sudden unilateral neuralgiform conjunctival injection tearing. CI, Conjunctival injection; EO, eyelid edema; FF, forehead and facial flushing; La, lacrimation; Mi, miosis; NC, nasal congestion; Pt, ptosis; Rh, rhinorrhea. From Kitahara I, Fukuda A, Imamura Y, et al. Pathogenesis, surgical treatment, and cure for SUNCT syndrome. World Neurosurg. 2015;84[4]:1080–1083, fig. 1.

TABLE 8.3 Descriptors of Pain Associated With Sudden Unilateral Neuralgiform Conjunctival Injection Tearing Headache Stabbing Shooting Lancinating Shock-like Sharp Piercing Pricking Staccato-like

Differential Diagnosis SUNCT headache is a clinical diagnosis supported by a combination of clinical history, normal physical examination, radiography, and MRI. Pain syndromes that may mimic SUNCT headache include cluster headache, temporal arteritis, trigeminal neuralgia involving the first division of the trigeminal nerve, demyelinating disease, primary stabbing headache, hypnic headache, and chronic paroxysmal hemicrania, although because of the overlapping features of all headache and facial pain syndromes, SUNCT headache can be easily mistaken for another type of headache or facial pain (Fig. 8.5; Table 8.4). Trigeminal neuralgia involving the first division of the trigeminal nerve is uncommon and is characterized by trigger areas and ticlike movements. Demyelinating disease is generally associated with other neurological findings, including optic neuritis and other motor and sensory abnormalities. The pain of chronic paroxysmal hemicrania lasts much longer than the pain of SUNCT headache.

FIG. 8.4 Magnetic resonance imaging (MRI) with right-sided short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing syndromes, demonstrating an aberrant loop of the causative vessels abutting the right trigeminal nerve. Right (R) and left (L) sides of the patient are indicated on the MRI images. MRI (A) and operative (B) images for case 1. Axial (A, upper panel) and sagittal (lower panel) images are shown. The side of the first branch of the right trigeminal nerve (RTN) was compressed by the vertebral artery (VA). MRI (C) and operative (D) images for case 2. Axial (C, top panel) and 2 sagittal images are shown. Compression of the side of the first branch of the right trigeminal nerve by the superior cerebellar artery (SCA) was suspected (C, middle panel). The side of the third branch of the right trigeminal nerve was compressed by the anterior inferior cerebellar artery (AICA) (C, bottom panel). FTN, The first branch of the trigeminal nerve; LTN, left trigeminal nerve; PV, petrosal vein; RC, right cerebellum; TTN, the third branch of the trigeminal nerve; VII–VIII N, VII and VIII nerves. From Kitahara I, Fukuda A, Imamura Y, et al. Pathogenesis, surgical treatment, and cure for SUNCT syndrome. World Neurosurg. 2015;84[4]:1080–1083, fig. 2.

FIG. 8.5 Pain Location in the Trigeminal Autonomic Cephalgias and Neurovascular Orofacial Pain (NVOP)The TACs are characterized by orbital and periorbital pain. In paroxysmal hemicrania and hemicrania continua, large adjacent areas are affected. Migraine is largely unilateral but may be bilateral in up to 30% of cases (this has been marked by a lighter shaded area contralaterally). Neurovascular orofacial pain is characterized by its location in the lower two-thirds of the face with intraoral and perioral areas frequently involved as primary sites. Two-headed arrow above diagram indicates the side shift that occurs in specific headache. SUNCT, Sudden unilateral neuralgiform conjunctival injection tearing. Modified from Benoliel R, Sharav Y. The trigeminal autonomic cephalgias (TACs). In: Sharav Y, ed. Orofacial Pain and Headache. Edinburgh: Elsevier; 2008:225–254.

TABLE 8.4 Differential Diagnosis: Sudden Unilateral Neuralgiform Conjunctival Injection Tearing Headache Cluster headache Temporal arteritis Trigeminal neuralgia Demyelinating disease Primary stabbing headache Hypnic headache Chronic paroxysmal hemicrania

Treatment The treatment of SUNCT headache is analogous to the treatment of trigeminal neuralgia, although the pharmacological management of this uncommon headache disorder is disappointing. The use of anticonvulsants such as lamotrigine and gabapentin represents a reasonable starting point. High-dose steroids tapered over 10 days also have been anecdotally reported to provide relief. For patients who do not respond to the previously mentioned treatments, daily trigeminal nerve block with a local anesthetic and steroid is a reasonable next step. Occasionally, retrogasserian injection of glycerol, balloon compression of the gasserian ganglion, and microvascular decompression of the trigeminal nerve root are required to provide palliation of pain. Underlying sleep disturbance and depression associated with the pain of SUNCT headache are best treated with a tricyclic antidepressant compound, such as nortriptyline, which can be started at a single bedtime dose of 25 mg.

Complications and Pitfalls Failure to diagnose SUNCT headache correctly may put the patient at risk if an intracranial pathological condition or demyelinating disease, which may mimic the clinical presentation of SUNCT headache, is overlooked. MRI is indicated in all patients thought to have SUNCT headache. Failure to diagnose glaucoma or temporal arteritis, which also may cause intermittent ocular pain, may result in permanent loss of sight.

Clinical Pearls Trigeminal nerve block with local anesthetic is especially useful in the diagnosis and palliation of pain secondary to SUNCT headache. Given the uncommon nature of this headache syndrome and its overlap with the symptoms of cluster headache and other neurological problems, including cavernous sinus thrombosis and intracranial and retroorbital tumors, SUNCT headache must remain a diagnosis of exclusion. All patients suspected to have SUNCT headache require MRI of the brain with and without gadolinium contrast material and thorough ophthalmological and neurological evaluation. Trigeminal nerve block should be performed only by clinicians familiar with the regional anatomy.

Suggested Readings Kitahara I, Fukuda A, Imamura Y, et al. Pathogenesis, surgical treatment, and cure for SUNCT syndrome. World Neurosurg. 2015;84(4):1080–1083. Klasser G.D, Balasubramaniam R. Trigeminal autonomic cephalalgias. III. Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing. Oral Surg Oral Med Oral Pathol Oral Radiol Endodontol. 2007;104 773–771. Leone M, Bussone G. Pathophysiology of trigeminal autonomic cephalalgias. Lancet Neurol. 2009;8:755–774. Levin M. Nerve blocks and nerve stimulation in headache disorders. Tech Reg Anesth Pain Manage. 2009;13:42–49. Levin M. Nerve blocks in the treatment of headache. Neurotherapeutics. 2010;7:197–203. Nagel M.A, Burns T.M, Gilden D. SUNCT headaches after ipsilateral ophthalmic-distribution zoster. J Neurol Sci. 2016;366:207–208. Nobuhiro, Yoshiharu T, Takamasa N, et al. Four patients with short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) successfully treated with lamotrigine. J Neurol Sci. 2017;381(suppl):941. Pulido Fontes L, Mayor Gómez S. SUNCT (Short-lasting unilateral neuralgiform headache attack with conjunctival injection and tearing) associated with pituitary lesion. Neurología (English Edition). 2015;30(7):458–459. Rozen T.D. Trigeminal autonomic cephalalgias. Neurol Clin. 2009;27:537–557. Waldman S.D. Gasserian ganglion block. In: Waldman S.D, ed. Atlas of Interventional Pain Management. 3rd ed. Philadelphia: Saunders; 2009:32–38. Waldman S.D. Gasserian ganglion block: balloon compression technique. In: Waldman S.D, ed. Atlas of Interventional Pain Management. 3rd ed. Philadelphia: Saunders; 2009:43–47. Waldman S.D. The trigeminal nerve. In: Waldman S.D, ed. Pain Review. Philadelphia: Saunders; 2009:15–17. Waldman S.D. Trigeminal nerve block: coronoid approach. In: Waldman S.D, ed. Atlas of Interventional Pain Management. 3rd ed. Philadelphia: Saunders; 2009:47–50. Williams M.H, Broadley S.A. SUNCT and SUNA: clinical features and

medical treatment. J Clin Neurosci. 2008;15:527–534.

9

Primary Thunderclap Headache

Abstract One of the most severe headaches encountered in clinical practice, thunderclap headache is characterized by a very rapid onset to peak of less than 1 minute. The headache may last from 1 to 10 days and, because of its intensity, almost always provokes an urgent trip to the emergency department, where the headache is invariably initially misdiagnosed as the sentinel headache of acute subarachnoid hemorrhage or other potentially catastrophic headache syndromes. Thunderclap headache may be the result of an underlying vascular or nonvascular intracranial abnormality or may represent a primary headache syndrome of unknown cause. The more benign, though no less painful, primary thunderclap headache occurs over three times more frequently than the serious secondary thunderclap headache. Because of the often-threatening causes of the less common secondary thunderclap headache (e.g., subarachnoid hemorrhage, cerebral venous thrombosis), urgent evaluation including computed tomography (CT) and/or magnetic resonance imaging of the brain and cerebrospinal fluid analysis are indicated in all patients suspected of having thunderclap headache.

Key words computerized tomography; lone acute severe headache; lumbar puncture; pneumopcephalus; subarachnoid hemorrhage; thunderclap headache

ICD-10 CODE G44.53

The Clinical Syndrome Thunderclap headache, which is also known as lone acute severe headache, is an uncommon type of headache that may be the result of an underlying vascular or nonvascular intracranial abnormality or may represent a primary headache syndrome of unknown cause. Common and uncommon causes of thunderclap headache are listed in Table 9.1. The more benign, though no less painful, primary thunderclap headache occurs over three times more frequently than the serious secondary thunderclap headache. Because of the often-threatening causes of the less common secondary thunderclap headache (e.g., subarachnoid hemorrhage, cerebral venous thrombosis), urgent evaluation including computed tomography (CT) and/or magnetic resonance imaging (MRI) of the brain and cerebrospinal fluid analysis are indicated in all patients suspected of having thunderclap headache. One of the most severe headaches encountered in clinical practice, thunderclap headache is characterized by a very rapid onset to peak of less than 1 minute. The headache may last from 1 to 10 days and, because of its intensity, almost always provokes an urgent trip to the emergency department, where the headache is invariably initially misdiagnosed as the sentinel headache of acute subarachnoid hemorrhage or other potentially catastrophic headache syndromes (Tables 9.2 and 9.3). This is not surprising in that primary thunderclap headache is virtually indistinguishable clinically from subarachnoid hemorrhage, one of the most neurologically devastating forms of cerebrovascular accident. Thus, because of the serious consequences of misdiagnosis, by necessity primary thunderclap headache is a diagnosis of exclusion.

Signs and Symptoms As mentioned earlier, primary thunderclap headache is characterized by a very rapid onset to peak of less than 1 minute without obvious inciting factors (e.g., sexual activity, coughing, straining at stool). The patient with primary thunderclap headache is almost always convinced that he or she is having a stroke and often appears frightened and anxious. The headache of primary thunderclap headache can be located anywhere in the head or neck. Nausea and vomiting are present approximately 75% of the time. However, the nuchal rigidity and other focal neurological signs often associated with acute subarachnoid hemorrhage and other neurologically devastating syndromes in which severe headache of acute onset are a prominent feature are uniformly absent.

Testing Testing in patients suspected of having primary thunderclap headache has two immediate goals: (1) to identify occult intracranial pathological conditions or other diseases that may mimic primary thunderclap headache and may require specific urgent treatment and (2) to identify the presence of subarachnoid hemorrhage. All patients with a recent onset of severe headache thought to be secondary to primary thunderclap headache should undergo emergent CT of the brain to rule out any pathological condition that could be responsible for the patient’s symptoms (Fig. 9.1). Modern multidetector CT scanners have a diagnostic accuracy approaching 100% for subarachnoid hemorrhage if CT angiography of the cerebral vessels is part of the scanning protocol. Cerebral angiography may also be required if surgical intervention is being considered and the site of bleeding cannot be accurately identified. TABLE 9.1 Main and Rare Causes of Thunderclap Headache

Modified from Linn FHH. Primary thunderclap headache. In: Aminoff MJ, ed. Handbook of Clinical Neurology. Vol. 97. New York: Elsevier; 2010:473–481.

TABLE 9.2 Comparison of Primary Thunderclap Headache and Subarachnoid Hemorrhage Comparison Factors Severe headache

Primary Thunderclap Headache Yes

Subarachnoid Hemorrhage Yes

Nausea and vomiting

Yes

Yes

Focal neurological signs

No

Yes

Nuchal rigidity

No

Yes

Photophobia

No

Yes

Vertigo

No

Yes

Neck and back pain

No

Yes

TABLE 9.3 Diseases That May Mimic Primary Thunderclap Headache Hemorrhagic Ischemic Neoplasm Infection Meningitis Encephalitis Abscess Parasitic Hypertensive crisis Loss of spinal fluid Postdural puncture headache Spontaneous spinal fluid leak Collagen-vascular disease Lupus cerebritis Vasculitis Polymyositis Headache Cluster headache Primary exertional headache Primary cough headache Migraine Ice pick headache Primary sexual headache

MRI of the brain and magnetic resonance angiography (MRA) may be useful if an aneurysm is not identified on CT studies and may be more accurate in the diagnosis of arteriovenous malformations (AVMs) (Fig. 9.2). Screening laboratory tests, including an erythrocyte sedimentation rate, complete blood count, coagulation studies, and automated blood chemistry, should be performed in patients with subarachnoid hemorrhage. Blood typing and crossmatching should be considered in any patient in whom surgery is being contemplated or who has preexisting anemia. Careful serial ophthalmological examination should be performed on all patients with subarachnoid hemorrhage to chart the course of papilledema.

FIG. 9.1 Computed Tomography Scan Showing Subarachnoid Hemorrhage (SAH)Right middle cerebral artery aneurysm in a 58-year-old man with SAH and intracranial hematoma (IH). (A) Volume rendering image from computed tomography angiography (CTA) clearly displays the relationship of the aneurysm to bone structures, adjacent branch vessels, and aneurysmal neck (arrow). (B) Maximum intensity projection (MIP) image from CTA clearly demonstrates the relationship of the aneurysm (arrow). (C) Thin-MIP image from CTA shows the relationship of the aneurysm to IH (arrowhead), and the ruptured aneurysm has a small nipple (arrow). From Chen W, Yang Y, Xing W, et al. Applications of multislice CT angiography in the surgical clipping and endovascular coiling of intracranial aneurysms. J Biomed Res. 2010;24:467–473.

Lumbar puncture is useful in revealing the presence or absence of blood in the spinal fluid, but its utility may be limited by the presence of increased intracranial pressure, making lumbar puncture too dangerous. Electrocardiographic abnormalities are common in patients with subarachnoid hemorrhage and are thought to be due to abnormally high levels of circulating catecholamines and hypothalamic dysfunction; however, they are rarely present in patients with primary thunderclap headache.

FIG. 9.2 Magnetic Resonance Imaging Showing Arteriovenous MalformationPatient with aneurysm-related false aneurysm (FA) in right parietal region. Preangiographic T1-weighted magnetic resonance axial image (A) and T2-weighted magnetic resonance coronal image (B) show round lesion (arrow) with flow void and mixed signal in the center and mixed signal on the periphery. Fluid-attenuated inversion recovery image (C) reveals small area of surrounding edema (arrow). (D) Flow in the center of FA (arrow) on two-dimensional time-of-flight magnetic resonance angiography. (E) Preembolization digital subtraction angiography image. (F) Residual inflow to FA (arrow) on postembolization digital subtraction angiography (DSA) image (arrow indicates arteriovenous malformation). From Brzozowski K, Frankowska E, Piasecki P, et al. The use of routine imaging data in diagnosis of cerebral pseudoaneurysm prior to angiography. Eur J Radiol. 2011;80:e401–e409.

Differential Diagnosis The differential diagnosis of primary thunderclap headache generally can be thought of as the diagnosis of the lesser of two evils because most of the diseases that mimic primary thunderclap headache are also associated with significant mortality and morbidity. Table 9.3 lists diseases that may be mistaken for primary thunderclap headache. Prominent among them is subarachnoid hemorrhage, stroke, collagen-vascular disease, infection, neoplasm, hypertensive crisis, spinal fluid leaks, and a variety of more benign causes of headache.

Treatment Although no generally accepted treatment for primary thunderclap headache has been defined, the following guidelines may be useful for the clinician when faced with a patient thought to have this uncommon headache syndrome. First and foremost, if test results reveal no evidence of intracranial pathology or other serious, life-threatening diseases, constant reassurance that the patient does not have a stroke or brain tumor is indicated. In general, drugs used to treat headaches whose primary mechanism of action is vasoconstriction (e.g., ergots, triptans) should be avoided. Anecdotal reports indicate that intravenous nimodipine may help abort acute attacks and prevent recurrent headache episodes. Gabapentin also has been advocated as a reasonable treatment for primary thunderclap headache and, given its favorable risk-to-benefit ratio, may be a reasonable therapeutic option.

Complications and Pitfalls Complications and pitfalls in the diagnosis and treatment of primary thunderclap headache generally fall into three categories. The first category involves the failure to recognize a sentinel bleed associated with subarachnoid hemorrhage and evaluate and treat the patient before significant morbidity or mortality occurs. The second category involves misdiagnosis that results in unnecessary testing, specifically, cerebral angiography, which itself is associated with significant morbidity and rarely death. The third category involves iatrogenic morbidity and rarely mortality from the use of medications to treat primary thunderclap headache (e.g., triptans, ergots) that not only do not treat this primary headache syndrome but also have significant side effects.

Clinical Pearls Primary thunderclap headache is a diagnosis of exclusion. It is frequently misdiagnosed as the sentinel headache of subarachnoid hemorrhage, causing the treating physician to order urgent diagnostic testing, which is associated with its own significant mortality and morbidity. The lack of focal neurological findings in a patient with acute headache should point the clinician toward the diagnosis of benign primary headaches including primary thunderclap headache, cough headache, exertional headache, atypical migraine, and headache associated with sexual activity. This does not mean that urgent computerized scanning of the brain and analysis of the patient’s cerebrospinal fluid are not indicated.

Suggested Readings Arenaza-Basterrechea N, Iglesias Díez. F, López Sarnago P. Thunderclap headache secondary to pneumocephalus. Neurología (English Ed). 2017;32(2):132–133. Chih-Ming H, Ya-Ju L, Yang-Kai F, Shih-Pin C, Tzu-Hsien L. Isolated thunderclap headache during sex: orgasmic headache or reversible cerebral vasoconstriction syndrome? J Clin Neurosci. 2010;17:1349–1351. de Bruijn S.F.T.M, Stam J, Kappelle L.J. CVST Study Group: thunderclap headache as first symptom of cerebral venous sinus thrombosis. Lancet. 1996;348:1623–1625. Esmanhotto B.B, Piovesan E.J, Moreschi F.A, et al. Thunderclap headache prevalence and modification of preexisting headache patterns after endovascular embolization in patients with ruptured intracranial aneurysm. J Neurol Sci. 2015;357(suppl 1):e166–e167. Janardhan V, Biondi A, Riina H.A, et al. Vasospasm in aneurysmal subarachnoid hemorrhage: diagnosis, prevention, and management. Neuroimaging Clin N Am. 2006;6:483–496. Linn F.H.H. Primary thunderclap headache. In: Aminoff M.J, ed. Handbook of Clinical Neurology. Vol. 97. New York: Elsevier; 2010:473–481. Manno E.M. Subarachnoid hemorrhage. Neurol Clin. 2004;22:347–366. Hennessy M. All thunderclap headaches are equal but some are more equal than others. Clin Radiol. 2015;70(suppl 1):S6–S7. Newfield P. Intracranial aneurysms: vasospasm and other issues. In: Atlee J.L, ed. Complications in Anesthesia. 2nd ed. Philadelphia: Saunders; 2007:719–723. Palestrant D, Connolly E.S. Subarachnoid hemorrhage. Neurobiol Dis. 2007:265–270. Pouration N, Dumont A.S, Kassell N.F. Subarachnoid hemorrhage. In: Alves W, Skolnick B, eds. Handbook of Neuroemergency Clinical Trials. New York: Elsevier; 2006:17–44. Yamakawa M, Hashimoto Y, Sakamoto T, et al. Clinical features of patients presenting reversible cerebral vasoconstriction without thunderclap headache. J Neurol Sci. 2017;381(suppl):1124.

10

Hypnic Headache

Abstract Hypnic headache is a term used to describe an uncommon headache syndrome characterized by its propensity to wake the person at the same time each night. Always occurring during sleep, hypnic headache is of short duration and rarely lasts more than 15 minutes after the patient is awakened by the pain. Research suggests that hypnic headache occurs most commonly during rapid eye movement (REM) sleep and it has been suggested that hypothalamic dysregulation may be the pathologic mechanism that triggers this rare headache syndrome. Hypnic headache occurs frequently, with a mean incidence of at least 15 attacks per month. The location of the headache pain is variable and the intensity of pain described as moderate with an aching character. Unlike as in cluster headache, which also occurs after sleep, patients with hypnic headache exhibit no autonomic signs or symptoms. Hypnic headache is a disease of the late fifth and sixth decades, with a mean age of onset of 63 years, although cases in the pediatric and adloscent population have been reported. It occurs more commonly in females.

Key words alarm clock headache; hypnic headache; indomethacin; lithium carbonate; nocturnal headache; pediatric headache; rapid eye movement sleep

ICD-10 CODE G44.81

The Clinical Syndrome Also known as alarm clock headache, hypnic headache is a term used to describe an uncommon headache syndrome characterized by its propensity to wake the person at the same time each night. Always occurring during sleep, hypnic headache is of short duration and rarely lasts more than 15 minutes after the patient is awakened by the pain (Fig. 10.1). Research suggests that hypnic headache occurs most commonly during rapid eye movement (REM) sleep and it has been suggested that hypothalamic dysregulation may be the pathologic mechanism that triggers this rare headache syndrome (Fig. 10.2). Hypnic headache occurs frequently, with a mean incidence of at least 15 attacks per month. The location of the headache pain varies, and the intensity of pain described as moderate with an aching character. Unlike cluster headache, which also occurs after sleep, patients with hypnic headache exhibit no autonomic signs or symptoms. Hypnic headache is a disease of the late fifth and sixth decades, with a mean age of onset of 63 years, although cases in the pediatric and adolescent population have been reported. It occurs more commonly in females.

Signs and Symptoms Hypnic headache is associated with no obvious neurological or musculoskeletal disease. Specifically, there are no autonomic signs or symptoms as are often seen with cluster headache. Furthermore, no accompanying focal neurological signs or systemic symptoms occur as with cluster headache and migraine headache, although rarely nausea can occur. The age of onset of benign hypnic headache is generally in the late fifth or sixth decade of life. Although no specific location is seen in hypnic headache, they are bilateral in 66% of patients. When the headaches are unilateral, they tend to occur on the same side night after night. What is fascinating to the treating physician and frustrating to the patient is the fact that hypnic headache wakes the patient from a sound sleep at almost the same time each night. Because the onset of hypnic headache occurs later in life, it must be considered a diagnosis of exclusion as with the other uncommon primary headache syndromes, for example, cough headache and thunderclap headache. The clinician must assiduously search for other explanations for the patient’s headache symptomatology, including intracranial pathological conditions and systemic disease.

Testing Magnetic resonance imaging (MRI) of the brain provides the best information regarding the cranial vault and its contents. MRI is highly accurate and helps identify abnormalities that may put the patient at risk for neurological disasters secondary to intracranial and brainstem pathological conditions, including tumors and demyelinating disease. MRI helps identify bleeding associated with leaking intracranial aneurysms, which may mimic the symptoms of both types of hypnic headache. Magnetic resonance angiography (MRA) may be useful in identifying aneurysms responsible for the patient’s neurological symptoms. In patients who cannot undergo MRI, such as patients with pacemakers, computed tomography (CT) is a reasonable second choice. Lumbar puncture should be performed if intracranial hemorrhage is suspected even if blood is not present on MRI or CT. Plain radiographs of the cervical spine also may be useful in the evaluation of Arnold-Chiari malformations and should be included in the evaluation of all patients with hypnic headache.

FIG. 10.1 Hypnic headache is also known as alarm clock headache due to its propensity to wake the person up at the same time each night.

FIG. 10.2 Gray Matter Decrease in the Hypothalamus in Hypnic HeadacheVoxelbased morphometry shows decreased gray matter in the hypothalamus. These structural data suggest an involvement of the hypothalamus in the pathophysiology of hypnic headache. From Obermann M, Holle D. Headache, hypnic. In: Aminoff MJ, Daroff RB, eds. Encyclopedia of the Neurological Sciences. 2nd ed. Oxford: Academic Press; 2014:516–519, fig. 1.

TABLE 10.1 Nocturnal Headaches That May Be Confused With Hypnic Headache Cluster headache Headache associated with sleep apnea Headache associated with nocturnal arterial hypertension Headache associated with increased intracranial pressure Analgesic rebound headache

Screening laboratory tests consisting of complete blood cell count,

erythrocyte sedimentation rate, and automated blood chemistry testing should be performed if the diagnosis of hypnic headache is in question. Intraocular pressure should be measured if glaucoma is suspected.

Differential Diagnosis Hypnic headache is a clinical diagnosis supported by a combination of clinical history, physical examination, radiography, MRI, and MRA. Pain syndromes that may mimic hypnic headache include the uncommon primary headaches benign exertional headache, ice pick headache, and sexual headache, although the unique same-time nocturnal occurrence should help the clinician easily identify the patient’s symptoms as hypnic headache. The clinician must consider other types of headache that occur more frequently at night, including cluster headache and headaches associated with sleep apnea, nocturnal arterial hypertension, analgesic rebound, and increased intracranial pressure (Table 10.1). Given that hypnic headache occurs in an older population, temporal arteritism must also be considered. Less commonly, hypnic headache may be confused with trigeminal neuralgia (involving the first division of the trigeminal nerve) or demyelinating disease. Trigeminal neuralgia involving the first division of the trigeminal nerve is uncommon and is characterized by trigger areas and tic-like movements. Demyelinating disease is generally associated with other neurological findings, including optic neuritis and other motor and sensory abnormalities. The pain of chronic paroxysmal hemicrania and cluster headache is associated with redness and watering of the ipsilateral eye, nasal congestion, and rhinorrhea during the headache. These findings are absent in hypnic headache. Migraine headache may or may not be associated with painless neurological findings known as aura, but patients almost always report some systemic symptoms, such as nausea or photophobia, not typically associated with hypnic headache.

Treatment Indomethacin and lithium carbonate are the treatments of choice for hypnic headache, with indomethacin being slightly more effective for the unilateral form of the syndrome. Indomethacin at a starting dose of 25 mg daily for 2 days and titrating to 25 mg three times per day is a reasonable treatment approach. This dose may be carefully increased up to 150 mg per day. Indomethacin must be used carefully, if at all, in patients with peptic ulcer disease or impaired renal function. Headache specialists have noted anecdotal reports of a positive response to cyclooxygenase-2 (COX-2) inhibitors in the treatment of benign hypnic headache. Lithium carbonate is used in the same manner as in the treatment of cluster headache and has its basis in use in its proven efficacy in the treatment of other diseases thought to have a chronobiological basis, such as cluster headache and bipolar disorders. However, the therapeutic window of lithium carbonate is small, and this drug should be used with caution. A starting dose of 300 mg at bedtime may be increased after 48 hours to 300 mg twice per day. If no side effects are noted after 48 hours, the dose may be increased again to 300 mg three times per day. Anecdotal reports exist that gabapentin and pregabalin also may be useful in decreasing the frequency and intensity of attacks of hypnic headache. Unlike with cluster headache, oxygen inhalation has been ineffective in aborting attacks of hypnic headache once the patient has been awakened by the pain.

Complications and Pitfalls Failure to diagnose hypnic headache correctly may put the patient at risk if an intracranial pathological condition or demyelinating disease, which may rarely mimic the clinical presentation of hypnic headache, is overlooked. MRI and MRA are indicated in all patients thought to have hypnic headache. Failure to diagnose glaucoma, which also may cause intermittent ocular pain, may result in permanent loss of sight.

Clinical Pearls Any patient presenting with nocturnal headaches should be taken very seriously. Although statistically most of these headaches ultimately are proved to be of benign cause, a few patients have potentially life-threatening disease. The diagnosis of hypnic headache is made by obtaining a thorough, targeted headache history and performing a careful physical examination. The clinician must separate patients with hypnic headache from patients with headaches caused by an intracranial pathological condition such as tumors or systemic disease such as nocturnal arterial hypertension. Patients with hypnic headache should have a normal neurological examination. If the neurological examination is abnormal, the diagnosis of benign hypnic headache should be discarded and a careful search for the cause of the patient’s neurological findings should be undertaken.

Suggested Readings Alberti A. Headache and sleep. Sleep Med Rev. 2006;10:431–437. Berciano J, Poca M.-A, García A, Sahuquillo J. Paroxysmal cervicobrachial hypnic-induced pain in a patient with syringomyelia extending into spinal cord posterior gray horns. J Neurol. 2007;254:678–681. Chen Y.Y, Lirng J.F, Fuh J.L, et al. Primary hypnic headache is associated with posterior fossa crowdedness: a morphometric MRI study. Cephalalgia. 2004;24:694–699. Fowler M.V, Capobianco D.J, Dodick D.W. Headache in the elderly. Semin Pain Med. 2004;2:123–128. Manni R, Ghiotto N. In: Aminoff M, ed. Handbook of Clinical Neurology. New York: Elsevier; 2010:469–472. Obermann M. Holle D. Headache, hypnic. Encyclopedia of the Neurological Sciences. 6th ed. Oxford: Academic Press; 2014:516–519. Pascual J, González-Mandly A, Martín R, Oterino A. Headaches precipitated by cough, prolonged exercise or sexual activity: a prospective etiological and clinical study. J Headache Pain. 2008;9:259–266. Pascual J. Primary hypnic headache. Curr Pain Headache Rep. 2005;9:272–276. Silva-Néto R.P, Almeida K.A. Hypnic headache: a descriptive study of 25 new cases in Brazil. J Neurol Sci. 2014;338(1–2):166–168. Silva-Néto R.P, Almeida K.A. Hypnic headache in childhood: a literature review. J Neurol Sci. 2015;356(1–2):45–48. Waldman S.D. Arnold Chiari malformation type I. In: Waldman S.D, Campbell R.S, eds. Imaging of Pain. Philadelphia: Saunders; 2011:27–28. Waldman S.D. Arnold Chiari malformation type II. In: Waldman S.D, Campbell R.S, eds. Imaging of Pain. Philadelphia: Saunders; 2011:29–30.

11

Nummular Headache

Abstract: Nummular headache is an uncommon chronic headache syndrome characterized by constant localized pain with superimposed paroxysms of stabbing jabs and jolts of mild to moderate intensity that occur in a coin-shaped localized area of the scalp. Most commonly located in the parietal region, the pain of nummular headache is unilateral and localized to a single area. It rarely if ever switches sides. The scalp overlying the area may be tender to touch and stimulation of the area; for example, the brushing of hair may exacerbate the pain. Nummular headache occurs slightly more commonly in women and is generally not seen before the fourth decade of life, but rare reports of children suffering from nummular headache sporadically appear in the literature. Nummular headache has been associated with coexistant migraine headache and occipital neuralgia. A high prevalence of autoimmune indicies and disorders have also been identified in patients suffering from nummular headache.

Keywords Autoimmune diseases; coin-shaped headache; headache; magnetic resonance imaging; migraine headache; nummular headache; occipital neuralgia

ICD-10 CODE R51

The Clinical Syndrome Nummular headache is an uncommon chronic headache syndrome characterized by constant localized pain with superimposed paroxysms of stabbing jabs and jolts of mild to moderate intensity that occur in a coinshaped localized area of the scalp. Most commonly located in the parietal region, the pain of nummular headache is unilateral and localized to a single area. It rarely if ever switches sides. The scalp overlying the area may be tender to touch and stimulation of the area; for example, the brushing of hair may exacerbate the pain. Nummular headache occurs slightly more commonly in women and is generally not seen before the fourth decade of life, but rare reports of children suffering from nummular headache sporadically appear in the literature. Nummular headache has been associated with coexistent migraine headache and occipital neuralgia. A high prevalence of autoimmune indices and disorders have also been identified in patients suffering from nummular headache. Nummular headache is also known as coin-shaped headache.

Signs and Symptoms A patient with nummular headache complains of a unifocal region of pain and sensitivity most commonly occurring in the vertex of the parietal region (Fig. 11.1). The pain is almost always unilateral and does not switch sides, although rare reports exist of bilateral nummular headache. Some patients describe the pain of nummular headache as a constant dull ache or sensitivity in the affected area with superimposed paroxysms of lancinating pain. The pain is chronic, although spontaneous remissions have been rarely reported. Some patients with nummular headache exhibit anxiety and depression because the intensity of the associated pain leads many patients to believe they have a brain tumor.

Testing Magnetic resonance imaging (MRI) of the brain provides the best information regarding the cranial vault and its contents. MRI is highly accurate and helps identify abnormalities that may put the patient at risk for neurological disasters secondary to intracranial and brainstem pathological conditions, including tumors and calvarial lesions (Fig. 11.2). Magnetic resonance angiography (MRA) also may be useful in helping identify aneurysms, which may be responsible for the patient’s pain. In patients who cannot undergo MRI, such as patients with pacemakers, computed tomography (CT) is a reasonable second choice. Radionuclide bone scanning and plain radiography are indicated if fracture or bony abnormality, such as metastatic disease, is considered in the differential diagnosis. Screening laboratory tests consisting of complete blood cell count, erythrocyte sedimentation rate, and automated blood chemistry should be performed if the diagnosis of nummular headache is in question.

Differential Diagnosis Nummular headache is a clinical diagnosis supported by a combination of clinical history, normal physical examination, radiography, and MRI. Pain syndromes that may mimic nummular headache include chronic paroxysmal hemicrania and jolts and jabs headache. Trigeminal neuralgia involving the first division of the trigeminal nerve is uncommon and is characterized by trigger areas and tic-like movements. Demyelinating disease is generally associated with other neurological findings, including optic neuritis and other motor and sensory abnormalities. The pain of chronic paroxysmal hemicrania lasts much longer than the pain of nummular headache and is associated with redness and watering of the ipsilateral eye.

FIG. 11.1 Patients Suffering From Nummular Headache Complain of Unifocal Area of Pain and Scalp Sensitivity.

FIG. 11.2 Calvarial Metastases(A) Abnormal enhancement (arrows) is present within the diploë on this gadolinium-enhanced T1-weighted image. Expansion of the left parietal bone occurs, affecting the inner table more than the outer table. (B) Heterogeneous hyperintensity (arrows) persists within the calvaria on this T2weighted image. The right parietal lesion is no longer imaged on this more superior section. From Edelman RR, Hesselink JR, Zlatkin MB, Crues JV III, eds. Clinical Magnetic Resonance Imaging. 3rd ed. Philadelphia: Saunders; 2005.

Treatment Nummular headache uniformly responds to treatment with indomethacin. Failure to respond to indomethacin puts the diagnosis of nummular headache in question. A starting dosage of 25 mg daily for 2 days and titrating to 25 mg three times a day is a reasonable treatment approach. This dose may be carefully increased to 150 mg/day. Indomethacin must be used carefully, if at all, in patients with peptic ulcer disease or impaired renal function. Anecdotal reports of a positive response to cyclooxygenase-2 (COX2) inhibitors in the treatment of nummular headache have been noted in the headache literature, as well as a successful treatment with gabapentin. Underlying sleep disturbance and depression are best treated with a tricyclic antidepressant compound, such as nortriptyline, which can be started at a single bedtime dose of 25 mg.

Complications and Pitfalls Failure to diagnose nummular headache correctly may put the patient at risk if an intracranial pathological condition or calvarial disease, which may mimic the clinical presentation of nummular headache, is overlooked. MRI is indicated in all patients thought to have nummular headache.

Clinical Pearls The diagnosis of nummular headache is made by taking a careful, targeted headache history. Patients with nummular headache should have a normal neurological examination. If the results of the neurological examination are abnormal, the diagnosis of nummular headache should be discarded and a careful search for the cause of the patient’s neurological findings should be undertaken.

Suggested Readings Cohen G.L. Nummular headache: what denomination? Headache. 2005;10:1417–1418. Evans R.W, Pareja J.A. Nummular headache. Headache. 2005;45:164–165. Mathew N.T. Indomethacin responsive headache syndromes. Headache. 1981;21:147–150. Pareja J.A, Caminero A.B. Serra J, et al. Nummular headache: a coin-shaped cephalgia. Neurology. 2002;58:1678–1679. Pareja J.A, Pareja J, Barriga F.J, et al. Nummular headache: a prospective series of 14 new cases. Headache. 2004;44:611–614. Pareja J.A, Pareja J, Yangüela J. Nummular headache, trochleitis, supraorbital neuralgia, and other epicranial headaches and neuralgias: the epicranias. J Headache Pain. 2003;4:125–131. Iwanowski P, Kozubski W, Losy J. Nummular headache in a patient with ipsilateral occipital neuralgia—a case report. Neurol Neurochir Pol. 2014;48(2):141–143. Chen WH, Chen YT, Lin CS, Li TH, Lee LH, Chen CJ. A high prevalence of autoimmune indices and disorders in primary nummular headache. J Neurol Sci. 2012;320(1–2):127–130.

12

Headache Associated With Temporal Arteritis

Abstract As the name suggests, headache associated with temporal arteritis is located primarily in the temples, with secondary pain often located in the frontal and occipital regions. A disease of the sixth decade and beyond, temporal arteritis affects whites almost exclusively, and there is a 3:1 female gender predominance. Temporal arteritis is also known as giant cell arteritis because of the finding of giant multinucleated cells that infiltrate arteries containing elastin, including the temporal, ophthalmic, and external carotid arteries. Approximately half of patients with temporal arteritis also have polymyalgia rheumatica.

Key words giant cell arteritis; halo sign; jaw claudication; polymyalgia rheumatic; prednisone; temporal arteritis; ultrasound imaging

ICD-10 CODE M31.6

The Clinical Syndrome As the name suggests, headache associated with temporal arteritis is located primarily in the temples, with secondary pain often located in the frontal and occipital regions. A disease of the sixth decade and beyond, temporal arteritis affects whites almost exclusively, and there is a 3:1 female gender predominance. Temporal arteritis is also known as giant cell arteritis because of the finding of giant multinucleated cells that infiltrate arteries containing elastin, including the temporal, ophthalmic, and external carotid arteries (Fig. 12.1A). Approximately half of patients with temporal arteritis also have polymyalgia rheumatica.

Signs and Symptoms Headache is seen in most patients with temporal arteritis. The headache is in the temples and is usually continuous. The character of the headache pain associated with temporal arteritis is aching and has a mild to moderate level of intensity. A patient with temporal arteritis also may complain of soreness of the scalp, making the combing of hair or resting the head on a firm pillow extremely uncomfortable. Although temporal headache is present in almost all patients with temporal arteritis, the finding of intermittent jaw claudication is pathognomonic for the disease (see Fig. 12.1B). In an elderly patient, jaw pain while chewing should be considered secondary to temporal arteritis until proved otherwise. In the presence of strong clinical suspicion that the patient has temporal arteritis, immediate treatment with corticosteroids is indicated (see discussion of treatment). The reason immediate treatment is needed is the potential for sudden painless deterioration of vision in one eye secondary to ischemia of the optic nerve. In addition to the signs and symptoms mentioned previously, patients with temporal arteritis experience myalgia and morning stiffness. Muscle weakness associated with inflammatory muscle disease and many other collagen-vascular diseases is absent in temporal arteritis, unless the patient has been treated with prolonged doses of corticosteroids for other systemic disease, such as polymyalgia rheumatica. The patient also may experience nonspecific systemic symptoms, including malaise, weight loss, night sweats, and depression. On physical examination, a swollen, indurated, nodular temporal artery is present. Diminished pulses are often noted, as is tenderness to palpation. Scalp tenderness to palpation is often seen. Funduscopic examination may reveal a pale, edematous optic disc. The patient with temporal arteritis often appears chronically ill, depressed, or both.

Testing Erythrocyte sedimentation rate should be obtained in all patients suspected to have temporal arteritis. In temporal arteritis, the erythrocyte sedimentation rate is greater than 50 mm/h in more than 90% of patients. Less than 2% of patients with biopsy-proved temporal arteritis have normal erythrocyte sedimentation rates. Ideally, the blood for the erythrocyte sedimentation rate should be obtained before beginning corticosteroid therapy because the initial level of elevation of this test is useful not only to help diagnose the disease but also as a mechanism to establish the efficacy of therapy. The erythrocyte sedimentation rate is a nonspecific test, and other diseases that may manifest clinically in a manner similar to temporal arteritis, such as malignancy or infection, also may markedly elevate the erythrocyte sedimentation rate. Confirmation of the clinical diagnosis of temporal arteritis requires ultrasound imaging and/or a temporal artery biopsy. Ultrasound imaging can provide confirmation by identification of a halo sign surrounding the affected temporal artery. The finding of a positive halo sign strongly supports the diagnosis of temporal arteritis (Fig. 12.2). Given the simplicity and safety of temporal artery biopsy, it probably should be performed on all patients suspected of having temporal arteritis. The presence of an inflammatory infiltrate with giant cells in the biopsied artery is characteristic of the disease. Edema of the intima and disruption of the internal elastic lamina strengthen the diagnosis. A small percentage of patients with clinical signs and symptoms strongly suggestive of temporal arteritis who also exhibit a significantly elevated erythrocyte sedimentation rate have a negative temporal artery biopsy result. As mentioned, in the presence of a strong clinical impression that the patient has temporal arteritis, an immediate blood sample for erythrocyte sedimentation rate testing should be obtained and the patient started on corticosteroids. Complete blood cell count and automated chemistries, including thyroid testing, are indicated in all patients with suspected temporal arteritis to help rule out other systemic disease that may mimic the clinical presentation of temporal arteritis.

FIG. 12.1 (A) Temporal arteritis is a disease of the sixth decade that occurs almost exclusively in whites, with a predilection of 3:1 for women. (B) The sine qua non of temporal arteritis is jaw claudication.

FIG. 12.2 Positive Halo Sign on Transverse Ultrasound Image in a Patient With Temporal Arteritis The patient’s sedimentation rate was 98.

If the diagnosis of temporal arteritis is in doubt, magnetic resonance imaging (MRI) of the brain provides the best information regarding the cranial vault and its contents. MRI is highly accurate and helps identify abnormalities that may put the patient at risk for neurological disasters secondary to intracranial and brainstem pathological conditions, including tumors and demyelinating disease. More importantly, MRI helps identify bleeding associated with leaking intracranial aneurysms. Magnetic resonance angiography (MRA) may be useful to help identify aneurysms responsible for neurological symptoms. In patients who cannot undergo MRI, such as patients with pacemakers, computed tomography (CT) is a reasonable second choice. If intracranial hemorrhage is suspected, lumbar puncture should be performed, even if blood is not present on MRI or CT. Case reports of the utility of positron emission tomography (PET)/computerized tomography scans in the diagnosis of giant cell arteritis of the temporal, occipital, and vertebral arteries may offer additional diagnostic options (Fig. 12.3). Intraocular pressure should be measured if glaucoma is suspected.

Differential Diagnosis Headache associated with temporal arteritis is a clinical diagnosis supported by a combination of clinical history, abnormal findings on physical examination of the temporal artery, normal radiography, MRI findings, an elevated erythrocyte sedimentation rate, and a positive temporal artery biopsy result. Pain syndromes that may mimic temporal arteritis include tension type headache, brain tumor, other forms of arteritis, trigeminal neuralgia involving the first division of the trigeminal nerve, demyelinating disease, migraine headache, cluster headache, and chronic paroxysmal hemicrania. Trigeminal neuralgia involving the first division of the trigeminal nerve is uncommon and is characterized by trigger areas and tic-like movements. Demyelinating disease is generally associated with other neurological findings, including optic neuritis and other motor and sensory abnormalities. The pain of chronic paroxysmal hemicrania and cluster headache is associated with redness and watering of the ipsilateral eye, nasal congestion, and rhinorrhea during the headache. These findings are absent in all types of sexual headache. Migraine headache may or may not be associated with painless neurological findings known as aura, but the patient almost always reports some systemic symptoms, such as nausea or photophobia, not typically associated with the headache of temporal arteritis.

Treatment The mainstay of treatment for temporal arteritis and its associated headaches and other systemic symptoms is the immediate use of corticosteroids. If visual symptoms are present, an initial dose of 80 mg of prednisone is indicated. This dose should be continued until the symptoms of temporal arteritis have completely abated. At this point, the dose may be decreased by 5 mg/week if the symptoms remain quiescent and the erythrocyte sedimentation rate does not increase. Cytoprotection of the stomach mucosa should be considered because ulceration and gastrointestinal bleeding are possible. If the patient cannot tolerate corticosteroids, or the maintenance dose of steroids remains so high as to produce adverse effects, azathioprine is a reasonable next choice.

FIG. 12.3 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) scan (axial slices), PET brain protocol —spectrum color scale, CT (same slice) and fusion PET/CT. High 18F-FDG uptake is evident in temporal arteries (red arrows), in their branches (yellow arrows), occipital arteries (green arrows), and vertebral arteries (white arrows). From Rehak Z, Vasina J, Ptacek J, et al. PET/CT in giant cell arteritis: high 18F-FDG uptake in the temporal, occipital and vertebral arteries. Rev Esp Med Nucl Imagen Mol. 2016;35[6]:398–401, fig. 2. ISSN 2253-654X, https://doi.org/10.1016/j.remn.2016.03.007. http://www.sciencedirect.com/science/article/pii/S2253654X16300051.

Complications and Pitfalls Failure to recognize, diagnose, and treat temporal arteritis promptly may result in the permanent loss of vision. Failure to diagnose the headache associated with temporal arteritis correctly may put the patient at risk if an intracranial pathological condition or demyelinating disease, which may mimic the clinical presentation of temporal arteritis, is overlooked. MRI of the brain is indicated in all patients thought to have headaches associated with temporal arteritis. Failure to diagnose glaucoma, which also may cause intermittent ocular pain, may result in permanent loss of sight.

Clinical Pearls The diagnosis of headache associated with temporal arteritis is made by obtaining a thorough, targeted headache history. As mentioned, jaw claudication is pathognomonic for temporal arteritis, and its presence should be sought in all elderly patients presenting with headache. Failure to recognize, diagnose, and treat temporal arteritis promptly may result in the permanent loss of vision.

Suggested Readings Bajkó Z, Bălaşa R, Szatmári S, et al. The role of ultrasound in the diagnosis of temporal arteritis. Neurol Neurochir Pol. 2015;49(2):139–143. Dickason A, McArdle P. Jaw claudication in the presentation of temporal arteritis: a review of the epidemiology and presenting symptoms of 207 patients at Derriford Hospital, Plymouth. Br J Oral Maxillofac Surg. 2015;53(10):e87. Hazelman B.L. Polymyalgia rheumatica. In: Waldman S.D, ed. Pain Management. Philadelphia: Saunders; 2009:449–454. Paget S.A, Spiera R.F. Polymyalgia rheumatica and temporal arteritis. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia: Saunders; 2007:1123–1127. Rehak Z, Vasina J, Ptacek J, et al. PET/CT in giant cell arteritis: high 18F-FDG uptake in the temporal, occipital and vertebral arteries. Rev Esp Med Nucl Imagen Mol. 2016;35(6):398–401. Sloane J, Rice N, Kergozou E, et al. Temporal artery biopsy for giant cell arteritis: an audit of 471 cases – what have we learnt? Br J Oral Maxillofac Surg. 2015;53(10):e44. Waldman SD. Connective tissue diseases. In: Waldman SD, ed. Pain Review. 2nd ed. Philadelphia: Saunders Elsevier; 200916:43167–44468. Waldman SD. Temporal arteritis. In: Waldman SD, ed. Pain Review. 2nd ed. Philadelphia: Saunders Elsevier; 200916:22236–22328.

13

Post–Dural Puncture Headache

Abstract The symptoms and rare physical findings associated with post–dural puncture headache are due to low cerebrospinal fluid pressure resulting from continued leakage of spinal fluid out of the subarachnoid space. The symptoms of post–dural puncture headache begin almost immediately after the patient moves from a horizontal to an upright position. The intensity peaks within 1 or 2 minutes and abates within several minutes of the patient again assuming the horizontal position. The headache is pounding in character, and its intensity is severe, with the intensity increasing the longer the patient remains upright. The headache is almost always bilateral and located in the frontal, temporal, and occipital regions. Nausea and vomiting and dizziness frequently accompany the headache pain, especially if the patient remains upright for long periods. If cranial nerve palsy occurs, visual disturbance may occur.

Key words cough headache; low pressure hydrocephalus; magnetic resonance imaging; postdural puncture headache; postural headache; spinal headache epidural blood patch

ICD-10 CODE G97.1

The Clinical Syndrome When the dura is intentionally or accidentally punctured, the potential for headache exists. The clinical presentation of post–dural puncture headache is classic and makes the diagnosis straightforward if considering this diagnostic category of headache. The diagnosis may be obscured if the clinician is unaware that dural puncture may have occurred or in the rare instance when this type of headache occurs spontaneously after a bout of sneezing or coughing. The symptoms and rare physical findings associated with post– dural puncture headache are due to low cerebrospinal fluid pressure resulting from continued leakage of spinal fluid out of the subarachnoid space. The symptoms of post–dural puncture headache begin almost immediately after the patient moves from a horizontal to an upright position. The intensity peaks within 1 or 2 minutes and abates within several minutes of the patient again assuming the horizontal position. The headache is pounding in character, and its intensity is severe, with the intensity increasing the longer the patient remains upright. The headache is almost always bilateral and located in the frontal, temporal, and occipital regions. Nausea and vomiting and dizziness frequently accompany the headache pain, especially if the patient remains upright for long periods. If cranial nerve palsy occurs, visual disturbance may occur. Post–dural puncture headache is also known as spinal headache.

Signs and Symptoms The diagnosis of post–dural puncture headache is most often made on the basis of clinical history rather than physical findings on examination. The neurological examination in most patients suffering from post–dural puncture headache is normal. If the spinal fluid leak is allowed to persist, or if the patient remains in the upright position for long periods despite the headache, cranial nerve palsies may occur, with the sixth cranial nerve affected most commonly. This complication may be transient, but may become permanent, especially in patients with vulnerable nerves, such as those with diabetes. If the neurological examination is abnormal, other causes of headache should be considered, including subarachnoid hemorrhage. The onset of headache pain and other associated symptoms such as nausea and vomiting that occurs when the patient moves from the horizontal to the upright position and then abates when the patient resumes a horizontal position is the sine qua non of post–dural puncture headache (Fig. 13.1). A history of intentional dural puncture, such as lumbar puncture, spinal anesthesia, or myelography, or accidental dural puncture, such as failed epidural block or dural injury during spinal surgery, strongly points to the diagnosis of post–dural puncture headache. As mentioned, a spontaneous postural headache that manifests identically to headache after dural puncture can occur after bouts of heavy sneezing or coughing and is thought to be due to traumatic rents in the dura. In this setting, a diagnosis of post– dural puncture headache is one of exclusion.

Testing Magnetic resonance imaging (MRI) with and without gadolinium is highly accurate in helping confirm the diagnosis of post–dural puncture headache. Enhancement of the dura with low-lying cerebellar tonsils invariably is present. Poor visualization of the cisterns and subdural and epidural fluid collections also may be identified (Fig. 13.2). No additional testing is indicated for a patient who has undergone dural puncture and then develops a classic postural headache, unless infection or subarachnoid hemorrhage is suspected. In this setting, lumbar puncture, complete blood cell count, and erythrocyte sedimentation rate are indicated on an emergent basis.

Differential Diagnosis If the clinician is aware that the patient has undergone dural puncture, the diagnosis of post–dural puncture headache is usually made. Delayed diagnosis most often occurs in settings in which dural puncture is not suspected. Occasionally, post–dural puncture headache is misdiagnosed as migraine headache because of the associated nausea and vomiting coupled with visual disturbance. In any patient with dural puncture, infection remains an ever-present possibility. If fever is present, immediate lumbar puncture and blood cultures should be obtained and the patient started on antibiotics that cover resistant strains of Staphylococcus. MRI to rule out epidural abscess also should be considered if fever is present. Subarachnoid hemorrhage may mimic post–dural puncture headache but should be identified on MRI of the brain.

FIG. 13.1 The onset of headache that occurs when the patient moves from the horizontal to the upright position is the sine qua non of post–dural puncture headache.

FIG. 13.2 Lumbar Spine Magnetic Resonance Image in a Patient With Post–Dural Puncture Headache Before the Administration of an Epidural Blood PatchThe static collection of fluid at L2 to L3 (arrows) corresponds to leakage of cerebrospinal fluid from the dural puncture site. From Feuer MP, Liu SS. Spinal anesthesia: post–dural puncture headache. In: Atlee JL, ed. Complications in Anesthesia. 2nd ed. Philadelphia: WB Saunders; 2007: chap 54, 223–226, fig. 54-1. ISBN 9781416022152, https://doi.org/10.1016/B978-14160-2215-2.50059-4. https://www.sciencedirect.com/science/article/pii/B9781416022152500594.

Treatment The mainstay of treatment of post–dural puncture headache is the administration of autologous blood into the epidural space. This technique is known as epidural blood patch and is highly successful in the treatment of post–dural puncture headache. A volume of 12 to 18 mL of autologous blood is injected slowly into the epidural space at the level of dural puncture under strict aseptic precautions. The patient should remain in the horizontal position for the next 12 to 24 hours. Relief occurs within 2 to 3 hours in more than 90% of patients. Approximately 10% of patients experience temporary relief and then a recurrence of symptoms when assuming the upright position. These patients should undergo a second epidural blood patch within 24 hours. If the patient has experienced significant nausea and vomiting, antiemetics combined with intravenous fluids help speed recovery. Some clinicians have advocated the use of alcoholic beverages to suppress the secretion of antidiuretic hormone and increase cerebrospinal fluid production. Caffeine and systemic glucocorticoids also have been reported to be helpful in treating the headache pain.

Complications and Pitfalls Failure to recognize, diagnose, and treat post–dural puncture headache promptly may result in considerable pain and suffering for the patient. If the low cerebrospinal fluid pressure is allowed to persist, cranial nerve deficits may occur. In most instances, the cranial nerve deficits are temporary, but in rare instances, these deficits may become permanent, especially in patients with vulnerable nerves, such as those with diabetes. MRI of the brain is indicated in all patients thought to be suffering from headaches associated with dural puncture. Failure to diagnose central nervous system infection correctly can result in significant mortality and morbidity.

FIG. 13.3 Comparison of Dural Puncture Holes Made by Quincke and Sprotte NeedlesThe Sprotte atraumatic needle is above the cutting Quincke needle. From Sladky JH, Piwinski SE. Lumbar puncture technique and lumbar drains. Atlas Oral Maxillofac Surg Clin North Am. 2015;23[2]:169–176, fig. 2.

Clinical Pearls The diagnosis of post–dural puncture headache is made by obtaining a thorough, targeted headache history and performing a careful physical examination. The postural nature is pathognomonic for post–dural puncture headache, and its presence should lead the clinician to strongly consider the

diagnosis of post–dural puncture headache. The incidence of post–dural puncture headache after lumbar puncture, myelography, or spinal anesthesia can be decreased by using needles with a smaller diameter and placing the needle bevel parallel to the dural fibers. Special noncutting needles may decrease further the incidence of post–dural puncture headache (Fig. 13.3).

Suggested Readings Candido K.D, Stevens R.A. Post-dural puncture headache: pathophysiology, prevention and treatment. Best Pract Res Clin Anaesthesiol. 2003;17(3):451– 469. Feuer M.P, Liu S.S. Chapter 54 - spinal anesthesia: post–dural puncture headache. In: Atlee John L, ed. Complications in Anesthesia. 2nd ed. Philadelphia: W.B. Saunders; 2007:223–226. Ghaleb A, Pablo C, Mandoff V.L, Albataniah J, Candido K. Postdural puncture cephalgia. Semin Pain Med. 2004;2:215–219. Harrington B.E. Postdural puncture headache. Adv Anesth. 2010;28:121–146. Neal J.M. Update on postdural puncture headache. Tech Reg Anesth Pain Manage. 1998;2:202–210. Sladky J.H, Piwinski S.E. Lumbar puncture technique and lumbar drains. Atlas Oral Maxillofac Surg Clin. 2015;23(2):169–176. Waldman S.D, Feldstein G.S, Allen M.L. Cervical epidural blood patch for treatment of cervical dural puncture headache. Anesth Rev. 1987;14:23–25.

14

Ramsay Hunt Syndrome

Abstract Ramsay Hunt syndrome is the eponym given to acute herpes zoster involvement of the geniculate ganglion. The syndrome results from reactivation of the varicella-zoster virus (VZV) within the geniculate ganglion. VZV is also the causative agent of chickenpox (varicella). Primary infection in the nonimmune host manifests itself clinically as the childhood disease chickenpox. It is postulated that during the course of primary infection with VZV, the virus invades the geniculate ganglia. The virus remains dormant in the ganglia, producing no clinically evident disease. In some individuals, the dormant virus reactivates and travels along the pathways of the geniculate ganglion, producing the pain and skin lesions characteristic of shingles. The reason that reactivation occurs in only some individuals is not fully understood, but it is theorized that a decrease in cell-mediated immunity may play an important role in the evolution of this disease by allowing the virus to multiply in the ganglia and spread to the corresponding sensory nerves, producing clinical disease. Patients with malignancies (particularly lymphoma), patients who are receiving immunosuppressive therapy (chemotherapy, steroids, radiation), and patients with chronic diseases are generally debilitated and much more likely than healthy individuals to develop acute herpes zoster. These patients all have in common a decreased cell-mediated immune response, which may be the reason for their propensity to develop shingles. This decreased immune response may also explain why the incidence of shingles increases dramatically in individuals older than 60 years and is uncommon in individuals younger than age 20.

Key words Acyclovir; Bell’s palsy; geniculate ganglion; herpes zoster oticus; Peripheral facial palsy; postherpetic neuralgia; Ramsay Hunt syndrome; shingles; stellate ganglion block; steroid therapy; varicellazoster virus

ICD-10 CODE B02.21

The Clinical Syndrome Ramsay Hunt syndrome is the eponym given to acute herpes zoster involvement of the geniculate ganglion. The syndrome results from reactivation of the varicella-zoster virus (VZV) within the geniculate ganglion. VZV is also the causative agent of chickenpox (varicella). Primary infection in the nonimmune host manifests itself clinically as the childhood disease chickenpox. It is postulated that during the course of primary infection with VZV, the virus invades the geniculate ganglia. The virus remains dormant in the ganglia, producing no clinically evident disease. In some individuals, the dormant virus reactivates and travels along the pathways of the geniculate ganglion, producing the pain and skin lesions characteristic of shingles. The reason that reactivation occurs in only some individuals is not fully understood, but it is theorized that a decrease in cell-mediated immunity may play an important role in the evolution of this disease by allowing the virus to multiply in the ganglia and spread to the corresponding sensory nerves, producing clinical disease. Patients with malignancies (particularly lymphoma), patients who are receiving immunosuppressive therapy (chemotherapy, steroids, radiation), and patients with chronic diseases are generally debilitated and much more likely than healthy individuals to develop acute herpes zoster. These patients all have in common a decreased cell-mediated immune response, which may be the reason for their propensity to develop shingles. This decreased immune response may also explain why the incidence of shingles increases dramatically in individuals older than 60 years and is uncommon in individuals younger than age 20. The first division of the trigeminal nerve is the second-most common site for the development of acute herpes zoster after the thoracic dermatomes. Rarely, the virus may attack the geniculate ganglion, resulting in facial pain, hearing loss, vertigo, vesicles in the ear, and pain. This constellation of symptoms is called Ramsay Hunt syndrome and must be distinguished from acute herpes zoster involving the first division of the trigeminal nerve.

Signs and Symptoms As viral reactivation occurs, ganglionitis and peripheral neuritis cause pain, which is generally localized to the segmental distribution of the geniculate ganglion. This pain may be accompanied by flu-like symptoms and generally progresses from a dull, aching sensation to dysesthetic neuritic pain in the distribution of the geniculate ganglion. In most patients, the pain of acute herpes zoster precedes the eruption of rash by 3 to 7 days, often leading to erroneous diagnosis (see discussion of differential diagnosis). The clinical diagnosis of shingles is readily made, however, in most patients when the characteristic rash appears. Similar to chickenpox, the rash of herpes zoster appears in crops of macular lesions, which rapidly progress to papules and then to vesicles (Fig. 14.1).

FIG. 14.1 Ramsay Hunt syndrome results from infection of the geniculate ganglion by the varicella zoster virus.

FIG. 14.2 Classic herpes zoster oticus lesions involving the patient’s left ear in a patient with Ramsey Hunt syndrome. From Angles EM, Nelson SW, Higgins GL 3rd. A woman with facial weakness: a classic case of Ramsay Hunt syndrome. J Emerg Med. 2013;44[1]:e137–e138, fig. 1. https://doi.org/10.1016/j.jemermed.2012.02.061. http://www.sciencedirect.com/science/article/pii/S0736467912003630.

As the disease progresses, the vesicles coalesce, and crusting occurs (Fig. 14.2). The area affected by the disease can be extremely painful, and the pain tends to be exacerbated by any movement or contact (e.g., with clothing or sheets). As healing occurs, the crusts fall away, leaving pink scars in the distribution of the rash that gradually become hypopigmented and atrophic. In most patients, the hyperesthesia and pain generally resolve as the skin lesions heal. In some patients, pain and neurological findings may persist beyond lesion healing (Fig. 14.3). This most common and feared complication of acute herpes zoster is postherpetic neuralgia. Elderly patients are affected at a higher rate than the general population suffering from acute herpes zoster. The symptoms of postherpetic neuralgia can vary from a mild, selflimited problem to a debilitating, constantly burning pain exacerbated by light touch, movement, anxiety, or temperature change. This unremitting pain may be so severe that it completely devastates the patient’s life, even leading ultimately to suicide. To avoid these disastrous sequelae to a usually benign self-limited disease, the clinician must use all possible therapeutic efforts for the patient suffering from acute herpes zoster in the geniculate ganglion.

Testing Although in most instances the diagnosis of acute herpes zoster involving the geniculate ganglion is easily made on clinical grounds, confirmatory testing is occasionally required. Such testing may be desirable in patients with other skin lesions that confuse the clinical picture, such as patients with acquired immunodeficiency syndrome who have Kaposi’s sarcoma. In such patients, the diagnosis of acute herpes zoster may be confirmed by obtaining a Tzanck smear from the base of a fresh vesicle, which reveals multinucleated giant cells and eosinophilic inclusions. To differentiate acute herpes zoster from localized herpes simplex infection, the clinician can obtain fluid from a fresh vesicle and submit it for immunofluorescent testing.

FIG. 14.3 Neurological examination revealed a flattened right nasolabial fold (black arrows) and ptosis of the right angle of the mouth (white arrow).

From Taguchi T, Ueda S, Kudo T, et al. Ramsay-Hunt syndrome. J Infect. 2011;62:180–181.

Differential Diagnosis Careful initial evaluation, including a thorough history and physical examination, is indicated in all patients suffering from acute herpes zoster involving the geniculate ganglion to rule out occult malignancy or systemic disease that may be responsible for the patient’s immunocompromised state and allow early recognition of changes in clinical status that may presage the development of complications, including myelitis or dissemination of the disease. Other causes of pain in the distribution of the geniculate ganglion include trigeminal neuralgia, sinus disease, glaucoma, retroorbital tumors, inflammatory diseases such as Tolosa-Hunt syndrome, and intracranial pathology, including tumors.

Treatment The therapeutic challenge of a patient with acute herpes zoster involving the geniculate ganglion is twofold: (1) to provide immediate relief of acute pain and symptoms and (2) to prevent complications, including postherpetic neuralgia. It is the consensus of most pain specialists that the earlier in the natural course of the disease that treatment is initiated, the less likely it is that the patient will develop postherpetic neuralgia. Because older patients are at highest risk for developing postherpetic neuralgia, early aggressive treatment of these patients is mandatory.

FIG. 14.4 Transverse ultrasound view demonstrating the position of the stellate ganglion (blue star) just anterior to the longus colli muscle. Note the relationship of Chassaignac’s tubercle of the C6 vertebral body and the carotid artery. SCM, Sternocleidomastoid muscle.

Nerve Blocks Sympathetic neural blockade with local anesthetics and steroids via stellate ganglion block seems to be the treatment of choice to relieve the symptoms of acute herpes zoster involving the geniculate ganglion and to prevent the occurrence of postherpetic neuralgia. Sympathetic nerve block is thought to achieve these goals by blocking the profound sympathetic stimulation that results from the viral inflammation of the nerve and geniculate ganglion. If untreated, this sympathetic hyperactivity can cause ischemia secondary to decreased blood flow of the intraneural capillary bed. If this ischemia is allowed to persist, endoneural edema forms, increasing endoneural pressure and causing a further reduction of endoneural blood flow with irreversible nerve damage. As vesicular crusting occurs, the addition of steroids to the local anesthetic may decrease neural scarring and decrease further the incidence of postherpetic neuralgia. These sympathetic blocks should be continued aggressively until the patient is pain free and should be reimplemented at the return of pain. Failure to use sympathetic neural blockade immediately and aggressively, especially in elderly patients, may sentence the patient to a lifetime of suffering from postherpetic neuralgia. Occasionally, some patients suffering from acute herpes zoster involving the geniculate ganglion may not experience pain relief from stellate ganglion block, but they do respond to blockade of the trigeminal nerve. Ultrasound needle guidance may improve the accuracy of needle placement and decrease needle-related complications when performing stellate ganglion block (Fig. 14.4).

Opioid Analgesics Opioid analgesics may be useful in relieving the aching pain that is often present during the acute stages of herpes zoster as sympathetic nerve blocks are being implemented. They are less effective in the relief of the neuritic pain that is often present. Careful administration of potent, long-acting opioid analgesics (e.g., oral morphine elixir or methadone) on a timecontingent rather than as-needed basis may represent a beneficial adjunct to the pain relief provided by sympathetic neural blockade. Because many patients with acute herpes zoster are elderly or may have severe multisystem disease, close monitoring for the potential side effects of potent opioid analgesics (e.g., confusion or dizziness, which may cause a patient to fall) is warranted. Daily dietary fiber supplementation and Milk of Magnesia should be started along with opioid analgesics to prevent the side effect of constipation.

Adjuvant Analgesics The anticonvulsant gabapentin represents a first-line treatment in the palliation of neuritic pain of acute herpes zoster involving the geniculate ganglion. Studies suggest that gabapentin also may help prevent the development of postherpetic neuralgia. Treatment with gabapentin should begin early in the course of the disease, and this drug may be used concurrently with neural blockade, opioid analgesics, and other adjuvant analgesics, including the antidepressant compounds if care is taken to avoid central nervous system side effects. Gabapentin is started at a bedtime dose of 300 mg and is titrated in 300-mg increments to a maximum dose of 3600 mg given in divided doses as side effects allow. Carbamazepine should be considered in patients with severe neuritic pain who have failed to respond to nerve blocks and gabapentin. If this drug is used, rigid monitoring for hematological parameters is indicated, especially in patients receiving chemotherapy or radiation therapy. Phenytoin also may be beneficial to treat neuritic pain, but it should not be used in patients with lymphoma because the drug may induce a pseudolymphoma state that is difficult to distinguish from the actual lymphoma itself. Antidepressants also may be useful adjuncts in the initial treatment of patients with acute herpes zoster. On an acute basis, these drugs help alleviate the significant sleep disturbance that is commonly seen with acute herpes zoster. In addition, antidepressants may be valuable in helping ameliorate the neuritic component of the pain, which is treated less effectively with opioid analgesics. After several weeks of treatment, the antidepressants may exert a mood-elevating effect that may be desirable in some patients. Patients must be observed closely for central nervous system side effects. These drugs may cause urinary retention and constipation that may be mistakenly attributed to herpes zoster myelitis.

Antiviral Agents A few antiviral agents, including famciclovir and acyclovir, have been shown to shorten the course of, and may help prevent the development of, acute herpes zoster. They are probably useful in attenuating the disease in patients with immunosuppression. These antiviral agents can be used in conjunction with the treatment modalities mentioned earlier. Careful monitoring for side effects is mandatory with these drugs.

Adjunctive Treatments The application of ice packs to the lesions of acute herpes zoster may provide relief in some patients. Application of heat increases pain in most patients, presumably because of increased conduction of small fibers, but it is beneficial occasionally and may be worth trying if application of cold is ineffective. Transcutaneous electrical nerve stimulation and vibration also may be effective in a few patients. The favorable risk-to-benefit ratio of all these modalities makes them reasonable alternatives for patients who cannot or will not undergo sympathetic neural blockade and do not tolerate pharmacological interventions. Topical application of aluminum sulfate as a tepid soak provides excellent drying of the crusting and weeping lesions of acute herpes zoster, and most patients find these soaks soothing. Zinc oxide ointment also may be used as a protective agent, especially during the healing phase when temperature sensitivity is a problem. Disposable diapers can be used as an absorbent padding to protect healing lesions from contact with clothing and sheets.

Complications and Pitfalls In most patients, acute herpes zoster involving the geniculate ganglion is a self-limited disease. In elderly and immunosuppressed patients, complications may occur, however. Cutaneous and visceral dissemination may range from a mild rash resembling chickenpox to an overwhelming, lifethreatening infection in patients already suffering from severe multisystem disease. Myelitis may cause bowel, bladder, and lower extremity paresis. Ocular complications from trigeminal nerve involvement may range from severe photophobia to keratitis with loss of sight.

Clinical Pearls Because the pain of herpes zoster usually precedes the eruption of skin lesions by 5-7 days, erroneous diagnosis of other painful conditions (e.g., trigeminal neuralgia, glaucoma) may be made. In this setting, the astute clinician advises the patient to call immediately if a rash appears because the diagnosis of acute herpes zoster is a possibility. Some pain specialists believe that in a few immunocompetent patients, when reactivation of virus occurs, a rapid immune response may attenuate the natural course of the disease and the characteristic rash of acute herpes zoster may not appear. This pain in the distribution of the geniculate ganglion without associated rash is termed zoster sine herpete and is, by necessity, a diagnosis of exclusion. Other causes of head pain must be ruled out first before invoking this diagnosis. Because of the potential for hearing loss in Ramsay Hunt syndrome, patients should be warned of this possibility to avoid erroneously blaming this complication on a therapeutic intervention, such as stellate ganglion block.

Suggested Readings Angles E.M, Nelson S.W, Higgins G.L. A woman with facial weakness: a classic case of Ramsay Hunt syndrome. J Emerg Med. 2013;;44(1):e137–e138. Bhagra A, Stead L.G. Ramsay Hunt syndrome: a rare entity. Ann Emerg Med. 2006;47:579. Gantz B.J, Redleaf M.I, Perry B.P, Gubbels S.P. Management of Bell’s palsy and Ramsay Hunt syndrome. In: Brackmann D.E, et al., ed. Otologic Surgery. 3rd ed. Philadelphia: Saunders; 2010:335–346. Persson A, Bergström T, Lindh M, Namvar L, Studahl M. Varicella-zoster virus CNS disease: viral load, clinical manifestations and sequels. J Clin Virol. 2009;46:249–253. Ryu E.W, Lee H.Y, Lee S.Y, et al. Clinical manifestations and prognosis of patients with Ramsay Hunt syndrome. Am J Otolaryngol. 2012;;33(3):313– 318. Taguchi T, Ueda S, Kudo T, et al. Ramsay-Hunt syndrome. J Infect. 2011;62:180– 181. Ulusoy Ç, Özkan G, Bektaç D, et al. Ramsay Hunt syndrome in renal transplantation recipient: a case report. Transplant Proc. 2010;42:1986–1988. Zainine R, Sellami M, Charfeddine A, et al. Ramsay Hunt syndrome. Eur Ann Otorhinolaryngol Head Neck Dis. 2012;;129(1):22–25.

15

Eagle Syndrome

Abstract Eagle syndrome (also known as stylohyoid syndrome) is caused by pressure on the internal carotid artery and surrounding structures, including branches of the glossopharyngeal nerve, by an abnormally elongated styloid process, a calcified stylohyoid ligament, or both. The pain of Eagle syndrome is sharp and stabbing and occurs with movement of the mandible or turning of the neck. The pain starts below the angle of the mandible and radiates into the tonsillar fossa, temporomandibular joint, and base of the tongue. A trigger point may be present in the tonsillar fossa. Injection of the attachment of the stylohyoid ligament to the styloid process with local anesthetic and steroid serves as a diagnostic maneuver and a therapeutic maneuver. Eagle syndrome is most often a diagnosis of exclusion.

Key words Eagle syndrome; glossopharyngeal neuralgia; stylohyoid ligament; stylohyoid syndrome; styloid process; tonsillar fossa; ultrasound guided stylohyoid ligament block

ICD-10 CODE M62.89

The Clinical Syndrome An uncommon cause of facial pain, Eagle syndrome (also known as stylohyoid syndrome) is caused by pressure on the internal carotid artery and surrounding structures, including branches of the glossopharyngeal nerve, by an abnormally elongated styloid process, a calcified stylohyoid ligament, or both. The pain of Eagle syndrome is sharp and stabbing and occurs with movement of the mandible or turning of the neck. The pain starts below the angle of the mandible and radiates into the tonsillar fossa, temporomandibular joint, and base of the tongue. A trigger point may be present in the tonsillar fossa. Injection of the attachment of the stylohyoid ligament to the styloid process with local anesthetic and steroid serves as a diagnostic maneuver and a therapeutic maneuver.

Signs and Symptoms Eagle syndrome is most often a diagnosis of exclusion. Patients suffering from Eagle syndrome present with a history of sudden, sharp neuritic pain that begins below the angle of the mandible and radiates into the tonsillar fossa, temporomandibular joint, and base of the tongue. The pain is triggered by swallowing, movement of the mandible, or turning of the neck (Fig. 15.1). The intensity of pain is moderate to severe and unpleasant. The neurological examination is normal. The pain of Eagle syndrome may be triggered by palpation of the tonsillar fossa.

Testing In patients with Eagle syndrome, radiographs and computed tomography (CT) scans of the region of the styloid process show an elongated styloid process that is often associated with a calcified stylohyoid ligament (Fig. 15.2). The diagnosis of Eagle syndrome may be strengthened by a diagnostic injection of the attachment of the stylohyoid ligament to the styloid process with local anesthetic. Pain relief after this injection suggests a local cause for the pain rather than a more distant cause, such as glossopharyngeal neuralgia or retropharyngeal tumor (Fig. 15.3).

Differential Diagnosis Eagle syndrome can be distinguished from glossopharyngeal neuralgia because the pain of glossopharyngeal neuralgia is characterized by paroxysms of shock-like pain in a manner analogous to trigeminal neuralgia, rather than the sharp, shooting pain on movement that is associated with Eagle syndrome. Because glossopharyngeal neuralgia may be associated with serious cardiac bradyarrhythmias and syncope, the clinician must distinguish the two syndromes. The clinician should always evaluate a patient with pain in this anatomical region for occult malignancy. Tumors of the larynx, hypopharynx, and anterior triangle of the neck may manifest with clinical symptoms identical to those of Eagle syndrome. Because of the low incidence of Eagle syndrome relative to pain secondary to malignancy in this anatomical region, Eagle syndrome must be considered a diagnosis of exclusion.

Treatment Many patients with Eagle syndrome respond to a series of therapeutic injections of the attachment of the stylohyoid ligament to the styloid process with local anesthetic and steroid. To perform this procedure, an imaginary line is visualized running from the mastoid process to the angle of the mandible (Fig. 15.4). The styloid process should lie just below the midpoint of this line. The skin is prepared with antiseptic solution. A 22-gauge, 1½inch needle attached to a 14-mL syringe is advanced at this midpoint location in a plane perpendicular to the skin. The styloid process should be encountered within 3 cm. After contact is made, the needle is withdrawn slightly out of the periosteum or substance of the calcified ligament. After careful aspiration reveals no blood or cerebrospinal fluid, 5 mL of 0.5% preservative-free lidocaine combined with 80 mg of methylprednisolone is injected in incremental doses. Subsequent daily nerve blocks are performed in a similar manner, substituting 40 mg of methylprednisolone for the initial 80-mg dose. Ultrasound-guided needle placement may improve the accuracy of needle placement and decrease the incidence of needle-related complications. Surgical excision of the styloid process and calcified stylohyoid ligament may be required in some cases.

FIG. 15.1 The pain of Eagle syndrome is triggered by swallowing, movement of the mandible, or turning of the neck.

FIG. 15.2 Three-dimensional computed tomography clearly shows a stylohyoid ligament ossified from the base of the skull all the way into the anterolateral hyoid bone and stylohyoid joint. From Ata-Ali J, Ata-Ali F, Melo M, et al. Eagle syndrome compared with stylohyoid syndrome: complete ossification of the stylohyoid ligament and joint. Br J Oral Maxillofac Surg. 2017;55[2];218–219, fig. 1. https://doi.org/10.1016/j.bjoms.2016.07.002.

FIG. 15.3 Tumor (T) of the Piriform SinusThe lesion protrudes through the thyroarytenoid gap between thyroid cartilage and arytenoid (arrow). The tumor invades the paraglottic space (arrowhead) of the supraglottic larynx. Compare with the fat in the paraglottic space on the normal side. C, Carotid artery. From Haaga JR, Lanzieri CF, Gilkeson RC, eds. CT and MR Imaging of the Whole Body. 4th ed. Philadelphia: Mosby; 2003.

FIG. 15.4 An imaginary line from the mastoid process to the angle of the mandible is an aid in needle placement for injection in a patient with Eagle syndrome.

The sharp, shooting pain associated with Eagle syndrome also may be treated with gabapentin. Gabapentin is started at a single nighttime dose of 300 mg and titrated by 300-mg increments every 2 days in divided doses until pain relief is achieved or a total daily dose of 3600 mg is reached. Alternatively, carbamazepine or phenytoin may be tried.

Complications and Pitfalls The major complications associated with this injection technique are related to trauma to the internal jugular and carotid artery. Hematoma formation and intravascular injection of local anesthetic with subsequent toxicity are common complications of this technique. Inadvertent blockade of the motor portion of the glossopharyngeal nerve can result in dysphagia secondary to weakness of the stylopharyngeus muscle. If the vagus nerve is inadvertently blocked, dysphonia secondary to paralysis of the ipsilateral vocal cord may occur. A reflex tachycardia secondary to vagal nerve block also is observed in some patients. Inadvertent block of the hypoglossal and spinal accessory nerves during glossopharyngeal nerve block results in weakness of the tongue and trapezius muscle.

Clinical Pearls Eagle syndrome is an uncommon cause of facial pain. Because of the low incidence of Eagle syndrome relative to pain secondary to malignancy in this anatomical region, Eagle syndrome must be considered a diagnosis of exclusion. The clinician should always evaluate a patient with pain in this anatomical region for occult malignancy. Tumors of the larynx, hypopharynx, and anterior triangle of the neck may manifest with clinical symptoms identical to those of Eagle syndrome.

Suggested Readings Al Weteid A.S, Miloro M. Transoral endoscopic-assisted styloidectomy: how should Eagle syndrome be managed surgically? Int J Oral Maxillofac Surg. 2015;44(9):1181–1187. Ata-Ali J, Ata-Ali F, Melo M, Andrés-Teruel J.C, Soto-Sarrion C. Eagle syndrome compared with stylohyoid syndrome: complete ossification of the stylohyoid ligament and joint. Br J Oral Maxillofac Surg. 2017;55(2):218– 219. Blythe J.N, Matthews N.S, Connor S. Eagle’s syndrome after fracture of the elongated styloid process. Br J Oral Maxillofac Surg. 2009;47:233–235. Callahan B, Kang J, Dudekula A, Eusterman V, Rabb C.H. New Eagle’s syndrome variant complicating management of intracranial pressure after traumatic brain injury. Injury Extra. 2010;41:41–44. Johnson G.M, Rosdy N.M, Horton S.J. Manual therapy assessment findings in patients diagnosed with Eagle’s syndrome: a case series. Man Ther. 2011;16:199–202. Klécha A, Hafian H, Devauchelle B, Lefévre B. A report of post-traumatic Eagle’s syndrome. Int J Oral Maxillofac Surg. 2008;37:970–972. Chen R, Liang F, Han P, Cai Q, Yu S, Huang X. Endoscope-assisted resection of elongated styloid process through a retroauricular incision: a novel surgical approach to eagle syndrome. Br J Oral Maxillofac Surg. 2017;75(7):1442–1448.

16

Atypical Odontalgia

Abstract Atypical odontalgia (also known as persistent orodental pain syndrome) describes a heterogeneous group of pain syndromes that share in common the fact that the odontalgia cannot be classified as classic trigeminal neuralgia. The pain is continuous but may vary in intensity. It is almost always unilateral and may be characterized as aching or cramping rather than the shocklike neuritic pain typical of trigeminal neuralgia. The vast majority of patients suffering from atypical odontalgia are female. Atypical odontalgia can occur at any age but has a peak incidence in the fifth decade of life. The pain is felt in a single tooth or its surrounding area and occurs most commonly in the maxillary region.

Keywords atypical facial pain; atypical odontalgia; chronic trigeminal neuropathy; neuroma; noiciceptive pain; osteomyelitis; persistent orodental pain syndrome; phantom tooth pain; pulpitis

ICD-10 CODE K08.9l

The Clinical Syndrome Atypical odontalgia (also known as persistent orodental pain syndrome) describes a heterogeneous group of pain syndromes that share in common the fact that the odontalgia cannot be classified as classic trigeminal neuralgia. The pain is continuous but may vary in intensity. It is almost always unilateral and may be characterized as aching or cramping rather than the shock-like neuritic pain typical of trigeminal neuralgia. The vast majority of patients suffering from atypical odontalgia are female. Atypical odontalgia can occur at any age, but has a peak incidence in the fifth decade of life. The pain is felt in a single tooth or its surrounding area and occurs most commonly in the maxillary region (Fig. 16.1). Headache may occur with atypical odontalgia and is clinically indistinguishable from the tension type of headache. Stress is often the precipitating, or an exacerbating, factor in the development of atypical odontalgia. Depression and sleep disturbance are also present in a significant number of patients. A history of dental or facial trauma, including dental extractions, root canal treatment, infection, or tumor of the head and neck may be elicited in some patients with atypical odontalgia, but in many cases no precipitating event can be identified.

Signs and Symptoms Table 16.1 compares atypical odontalgia with trigeminal neuralgia. Unlike trigeminal neuralgia, which is characterized by sudden paroxysms of neuritic shock-like pain, atypical odontalgia is constant and has a dull, aching quality but may vary in intensity. The pain of trigeminal neuralgia is almost always within the distribution of one division of the trigeminal nerve, whereas atypical odontalgia invariably involves just a single tooth, its surrounding gingival tissue, or underlying bone. The trigger areas characteristic of trigeminal neuralgia are absent in patients with atypical odontalgia. Most important, no findings of pathological condition of the painful tooth or adjacent gingival tissues are seen on physical examination.

Testing Radiographs of the head are usually within normal limits in patients suffering from atypical odontalgia, but they may be useful to identify a tumor or bony abnormality. Magnetic resonance imaging (MRI) of the brain and sinuses can help the clinician identify intracranial pathology such as tumor, sinus disease, and infection (Fig. 16.2). A complete blood count, erythrocyte sedimentation rate, and antinuclear antibody testing are indicated if inflammatory arthritis or temporal arteritis is suspected. Injection of the painful tooth with small amounts of local anesthetic can serve as a diagnostic maneuver to determine whether the tooth or adjacent structures are the source of the patient’s pain. Differential neural blockade can help distinguish primary tooth pathology from atypical odontalgia and reflex sympathetic dystrophy of the face (Table 16.2). Complete relief of pain after injection of the painful tooth with local anesthetic suggests a local pathological process, whereas incomplete pain relief suggests the pathological process is more central. Thus, the diagnosis of atypical odontalgia is a strong possibility of underlying pathological condition of the trigeminal nerve, adjacent bone, brain, or brainstem. Complete relief of pain after ipsilateral stellate ganglion block is highly suggestive of reflex sympathetic dystrophy of the face. Psychological evaluation should be considered if significant coexistent depression or sleep disturbance is present.

FIG. 16.1 Patients with atypical odontalgia often rub the affected area; those with trigeminal neuralgia do not.

TABLE 16.1 Comparison of Trigeminal Neuralgia and Atypical Odontalgia Pain Factor Temporal pattern of pain

Trigeminal Neuralgia Sudden and intermittent

Atypical Odontalgia Constant

Character of pain

Shock-like and neuritic

Dull, aching, cramping

Pain-free interval

Usual

Rare

Distribution of pain

One division of the trigeminal nerve

One tooth and surrounding area

Trigger areas

Present

Uncommon

Underlying psychopathology

Rare

Common

FIG. 16.2 Computed Tomography (CT) Scan and Magnetic Resonance Imaging (MRI) of the LesionCT shows a well-defined expansile lesion with thin cortical margin and high-density area (A). MRI of the lesion revealed the well-circumscribed lesion (B) to be homogeneously and relatively hypointense on T2-weighted imaging (C). The lesion was weakly enhanced by gadolinium (D). From Nozaki S, Yamazaki M, Koyama T, et al. Primary extracranial meningioma of the maxillary sinus presenting as buccal swelling. Asian J Oral Maxillofac Surg. 2011;23:134–137.

TABLE 16.2 Differential Nerve Block in the Diagnosis of Atypical Odontalgia 1. Record the patient’s pain level on a visual analogue scale of 0–10.

2. Isolate the painful area with cotton rolls and cheek retractor.

3. Dry the painful area with gauze.

4. Apply 20% topical benzocaine gel to the painful area.

5. Record the patient’s pain level on a visual analogue scale of 0–10 every 3 min for 15 min.

6. If the patient experiences incomplete pain relief, perform localized block of the painful tooth with 1% lidocaine 1.5 mL.

7. Record the patient’s pain level on a visual analogue scale of 0–10 every 3 min for 15 min.

8. If the patient experiences incomplete relief, perform ipsilateral stellate ganglion block with 0.5% preservative-free lidocaine 7–10 mL.

9. Record the patient’s pain level on a visual analogue scale of 0–10 every 3 min for 15 min.

10. Repeat this sequence on a separate visit to confirm the results.

Differential Diagnosis The clinical symptoms of atypical odontalgia may be confused with pain of dental or sinus origin or may be erroneously characterized as trigeminal neuralgia. Careful questioning and physical examination usually allow the clinician to distinguish these overlapping pain syndromes. Tumors of the zygoma, maxilla, and mandible, as well as posterior fossa and retropharyngeal tumors, may produce ill-defined pain that is attributed to atypical odontalgia. These potentially life-threatening diseases must be excluded in any patient with odontalgia (see Fig. 16.2). Reflex sympathetic dystrophy of the face should also be considered in any patient with illdefined odontalgia after trauma, infection, or central nervous system injury. As noted, atypical odontalgia is dull and aching, whereas reflex sympathetic dystrophy of the face causes burning pain and significant allodynia is often present. Stellate ganglion block may help distinguish these two pain syndromes; the pain of reflex sympathetic dystrophy of the face readily responds to this sympathetic nerve block, whereas atypical odontalgia does not. Atypical odontalgia must also be distinguished from the pain of jaw claudication associated with temporal arteritis.

Treatment The mainstay of therapy is a combination of drug treatment with tricyclic antidepressants and physical modalities such as oral orthotic devices and physical therapy. Trigeminal nerve block and intraarticular injection of the temporomandibular joint with small amounts of local anesthetic and steroid also may be of value. Antidepressants such as nortriptyline at a single bedtime dose of 25 mg can help alleviate sleep disturbance and treat any underlying myofascial pain syndrome. Orthotic devices help the patient avoid jaw clenching and bruxism, which may exacerbate the clinical syndrome. Management of underlying depression and anxiety is also mandatory.

Complications and Pitfalls The major pitfall in caring for patients thought to have atypical odontalgia is failure to diagnose underlying pathology that may be responsible for the patient’s pain. Atypical odontalgia is essentially a diagnosis of exclusion. If trigeminal nerve block or intraarticular injection of the temporomandibular joint is being considered as part of the treatment plan, it must be remembered that the region’s vascularity and proximity to major blood vessels can lead to an increased incidence of postblock ecchymosis and hematoma formation, and the patient should be warned of this potential complication.

Clinical Pearls Atypical odontalgia requires careful evaluation to design an appropriate treatment plan. Infection and inflammatory causes, including collagenvascular diseases, must be ruled out. Stress and anxiety often accompany atypical odontalgia, and these factors must be addressed and treated. The myofascial pain component of atypical odontalgia is best treated with tricyclic antidepressants such as amitriptyline. Dental malocclusion and nighttime bruxism should be treated with an acrylic bite appliance. Opioid analgesics and benzodiazepines should be avoided in patients with atypical odontalgia.

Suggested Readings Clark G.T. Persistent orodental pain, atypical odontalgia, and phantom tooth pain: when are they neuropathic disorders? J Calif Dent Assoc. 2006;34:599– 609. Greene C.S, Murray G.M. Atypical odontalgia: an oral neuropathic pain phenomenon. J Am Dent Assoc. 2011;142(9):1031–1032. Marbach J.J. Is phantom tooth pain a deafferentation (neuropathic) pain syndrome? Oral Surg Oral Med Oral Pahtol. 1993;75:95–105. Marbach J.J. Orofacial phantom pain: theory and phenomenology. JADA. 1996;127:221–229. Marbach J.J, Raphael K.G. Phantom tooth pain: a new look at an old dilemma. Pain Med. 2000;1:68–77. Matwychuk M.J. Diagnostic challenges of neuropathic tooth pain. J Can Dent Assoc. 2004;70:542–546. McQuay H.J, Tramér M, Nye B.A, et al. A systematic review of antidepressants in neuropathic pain. Pain. 1996;68:217–227. Melis M, Lobo S.L, Ceneviz C, et al. Atypical odontalgia: a review of the literature. Headache. 2003;43:1060–1074. Pertes R.A, Bailey D.R, Milone A.S. Atypical odontalgia: a nondental toothache. J N J Dent Assoc. 1995;66:29–31 33. Ram S, Teruel A, Kumar S.K.S, Clark G. Clinical characteristics and diagnosis of atypical odontalgia: implications for dentists. J Am Dent Assoc. 2009;140(2):223–228.

17

Burning Mouth Syndrome

Abstract Burning mouth syndrome is an infrequent but serious cause of oral pain. Although mouth pain has many causes with readily demonstrable pathological conditions, such as herpes simplex infections and aphthous ulcers, burning mouth syndrome is the diagnosis given to patients who complain of mouth and tongue pain in the presence of a completely normal physical examination. Therefore burning mouth syndrome is by definition a diagnosis of exclusion. Included in the diagnosis of burning mouth syndrome are the clinical syndromes of burning tongue syndrome, glossalgia, glossodynia, stomatodynia, and oral dysesthesia syndrome. Affecting females 7 to 8 times more frequently than men, burning mouth syndrome is a disease of the fifth decade and beyond. The pain of burning mouth syndrome is characterized as a burning, hot, or scalded sensation of the mouth and tongue that may be accompanied by tingling. Most commonly the anterior two thirds of the tongue, palate, gingiva of the upper and lower alveolar region, and lips are involved, with the sublingual region less commonly affected. The exact pathophysiology responsible for burning mouth syndrome remains elusive, and the putative causes in most cases are multifactorial. Underlying nutritional disorders, psychiatric illness, allergic stomatitis, xerostomia, diabetes mellitus, menopause, and other endocrinopathies are often identified in patients with burning mouth syndrome, even though the oral examination is completely negative.

Key words burning mouth syndrome; burning tongue syndrome gloosodynia; glossalgia; oral dysesthesia syndrome; stomatodynia; xerostomia

ICD-10 CODE K14.6

The Clinical Syndrome Burning mouth syndrome is an infrequent but serious cause of oral pain. Although mouth pain has many causes with readily demonstrable pathological conditions, such as herpes simplex infections and aphthous ulcers, burning mouth syndrome is the diagnosis given to patients who complain of mouth and tongue pain in the presence of a completely normal physical examination. Therefore burning mouth syndrome is by definition a diagnosis of exclusion. Included in the diagnosis of burning mouth syndrome are the clinical syndromes of burning tongue syndrome, glossalgia, glossodynia, stomatodynia, and oral dysesthesia syndrome. Affecting females 7 to 8 times more frequently than men, burning mouth syndrome is a disease of the fifth decade and beyond. The pain of burning mouth syndrome is characterized as a burning, hot, or scalded sensation of the mouth and tongue that may be accompanied by tingling. Most commonly the anterior two-thirds of the tongue, palate, gingiva of the upper and lower alveolar region, and lips are involved, with the sublingual region less commonly affected. The exact pathophysiology responsible for burning mouth syndrome remains elusive, and the putative causes in most cases are multifactorial. Underlying nutritional disorders, psychiatric illness, allergic stomatitis, xerostomia, diabetes mellitus, menopause, and other endocrinopathies are often identified in patients with burning mouth syndrome, even though the oral examination is completely negative. Magnetic resonance imaging (MRI) and functional MRI has identified altered structure and function of the hippocampus and medial prefrontal cortex in some patients with burning mouth syndrome.

Signs and Symptoms The hallmark of burning mouth syndrome is mouth and tongue burning pain in the absence of clinically demonstrable oral pathology. Depressive affect or a phobic preoccupation with occult cancer is often present, as is xerostomia. The classic oral findings of nutritional deficiencies such as iron and zinc deficiency, pernicious anemia, and vitamin B complex deficiency may be absent in patients with burning mouth syndrome and must be confirmed with appropriate laboratory testing. The clinician should observe the patient closely for abnormal tongue and mouth movements, such as bruxism, tongue thrusting, and repetitive running of the tongue against the teeth, because these are suggestive of behavioral abnormalities that may contribute to the patient’s pain symptomatology (Fig. 17.1).

Testing No specific test exists for burning mouth syndrome, and a presumptive diagnosis can be made only if (1) the clinical examination is normal and (2) a workup for all underlying pathological findings fails to identify a specific cause for the patient’s pain symptomatology. A suggested workup based on the experience at the Mayo Clinic is outlined in Table 17.1 and should always include laboratory testing for vitamin deficiencies and diabetes and a culture for Candida infection.

Differential Diagnosis Myriad causes of burning mouth and tongue pain have been identified, many of which are readily treatable (Table 17.2). It is therefore imperative that the clinician faced with a patient with burning mouth and tongue pain obtain an extremely thorough history and perform an oral examination with these diseases in mind. It should be kept in mind that more often than not the patient with burning mouth syndrome has more than one pathological condition contributing to the pain, and the possibility of multiple diagnosis should always be considered.

FIG. 17.1 The hallmark of burning mouth syndrome is mouth and tongue burning pain in the absence of clinically demonstrable oral pathology.

TABLE 17.1 Workup of Burning Mouth Syndrome Thorough history and review of symptoms Medications causing xerostomia Dental or denture work Oral care, oral products Oral habits or parafunctional behavior History of depression, anxiety, cancerphobia Family history of oral cancer, psychiatric diagnoses, and connective tissue disease Oral examination Erythema, candidiasis, xerostomia, or other mucosal abnormalities Tongue disorders, such as a geographic, fissured, or atrophic tongue Dental work or dentures Laboratory tests Complete blood count Iron, total iron binding capacity, iron saturation, ferreting Vitamin B 12 , folate, zinc Glucose, glycosylated hemoglobin Culture for Candida Patch testing Include standard series, metal series, oral flavors, and preservatives Further consultation if indicated by history and review of systems Psychometric testing and psychiatric consultation Dentistry Neurology Otorhinolaryngology

From Drage LA, Rogers RSR III. Burning mouth syndrome. Dermatol Clin. 2003;21:135–145.

Treatment The successful treatment of burning mouth syndrome requires the clinician to endeavor to identify the underlying pathology responsible for the patient’s pain. All underlying medical conditions (e.g., diabetes, deficiency syndromes) must be treated, along with the removal of any local irritants such as mouth washes, spicy foods, and cinnamon and mint products. Providing the patient with a supportive and positive emotional environment and reassurance that cancer is not the cause of the pain is paramount if symptom relief is to be achieved. Coexistent behavioral and psychiatric abnormalities also must be addressed in a positive therapeutic milieu. Empirical treatments, including anticandidal agents, vitamin B complex supplementation, and low-dose antidepressants, are also worthy of consideration. Treatment often involves some combination of elimination of any local irritants, treatment of underlying medical conditions, pharmacological therapy, and behavioral therapy. First, any nidus of tissue trauma that is contributing to the ongoing sympathetic dysfunction responsible for the symptoms must be identified and removed. Second, interruption of the sympathetic innervation of the face by stellate ganglion block with local anesthetic must be implemented. This may require daily stellate ganglion block for a considerable period. Occupational therapy consisting of tactile desensitization of the affected mucosa also may be of value. Underlying depression and sleep disturbance are best treated with a tricyclic antidepressant such as nortriptyline, given as a single 25-mg dose at bedtime. Gabapentin may help palliate any neuritic pain component and is best started slowly with a single bedtime dose of 300 mg, titrating the dosage upward in divided doses to a maximum dose of 3600 mg per day. Pregabalin is a reasonable alternative to gabapentin and is better tolerated in some patients. Pregabalin is started at 50 mg three times per day and may be titrated upward to 100 mg three times per day as disease effects allow. Pregabalin is excreted primarily by the kidneys, and the dosage should be decreased in patients with compromised renal function.

TABLE 17.2 Causes of Burning Mouth and Tongue Pain Systemic

Local

Deficiencies

Denture factors

Psychogenic, Psychiatric, and Idiopathic Psychiatric

Iron

Dental work

Depression

Vitamin B12

Mechanical

Anxiety

Folate

Obsessive-compulsive disorder

Zinc

Oral habit or parafunctional behavior Clenching

B complex vitamins

Bruxism

Cancerphobia

Endocrine

Tongue thrusting

Psychosocial stressors

Diabetes mellitus

Myofascial pain

Hypothyroidism

Allergic contact stomatitis

Menopause or hormonal

Connective tissue disease

Dental restoration or denture materials Foods Preservative, additives, flavorings Neurologic

Sjögren syndrome

Referred from tonsils or teeth

Sicca syndrome

Lingual nerve neuropathy

Drug related

Glossopharyngeal neuropathy

Anxiety or stress

Acoustic neuroma

Medication

Infection

Angiotensin-converting enzyme inhibitor

Candidiasis

Esophageal reflux

Antibiotic related

Anemia

Denture related

Xerostomia

Somatoform disorder

Local trauma Corticosteroid Diabetes mellitus Fusospirochetal Xerostomia Irradiation Local disease

From Drage LA, Rogers RSR III. Burning mouth syndrome. Dermatol Clin. 2003;21:135–145. Opioid analgesics and benzodiazepines should be avoided to prevent

iatrogenic chemical dependence.

Complications and Pitfalls The main complications surrounding the treatment of burning mouth syndrome are those associated with its misdiagnosis. Chemical dependence, depression, and multiple failed therapeutic procedures are the rule rather than the exception. A diagnosis of a psychiatric basis for the patient’s pain should be made only after all somatic causes of burning mouth syndrome have carefully been ruled out.

Clinical Pearls The key to diagnosing burning mouth syndrome is a high index of clinical suspicion. Once causes of burning mouth and tongue that have clinically identifiable pathological processes have been ruled out, a rational treatment plan addressing the often multifactorial nature of the patient’s pain can be initiated. A supportive therapeutic environment is crucial if symptom reduction is to be achieved.

Suggested Readings Drage L.A, Rogers III. R.S.R. Burning mouth syndrome. Dermatol Clin. 2003;21:135–145. Fenelon M, Quinque E, Arrive E, et al. Pain-relieving effects of clonazepam and amitriptyline in burning mouth syndrome: a retrospective study. Int J Oral Maxillofacial Surg. 2017;46(11):1505–1511. Khan S.A, Keaser M.L, Meiller T.F, Seminowicz D.A. Altered structure and function in the hippocampus and medial prefrontal cortex in patients with burning mouth syndrome. Pain. 2014;155(8):1472–1480. Miziara I.D, Araújo Filho B.C, Oliveira R. Rodrigues dos Santos RM. Group psychotherapy: an additional approach to burning mouth syndrome. Psychosom Res. 2009;67:443–448. Mock D. Burning tongue/mouth syndrome. J Oral Maxillofac Surg. 2009;67(suppl 1):5. Moghadam-Kia S, Fazel N. A diagnostic and therapeutic approach to primary burning mouth syndrome. Clini Dermatol. 2017;35(5):453–460. Moore P.A, Guggenheimer J, Orchard T. Burning mouth syndrome and peripheral neuropathy in patients with type 1 diabetes mellitus. J Diabetes Complications. 2007;21:397–402. Scully C. Burning mouth syndrome (oral dysaesthesia). In: Oral and Maxillofacial Medicine: The Basis of Diagnosis and Treatment. Edinburgh: Churchill Livingstone (Elsevier); 2013:249–253.

18

Nervus Intermedius Neuralgia

Abstract Nervus intermedius neuralgia causes severe, episodic pain afflicting the area of the acoustic auditory meatus supplied by the nervus intermedius. The pain is unilateral and characterized by paroxysms of electric shock– like pain lasting from several seconds to less than 2 minutes. The progression from onset to peak is essentially instantaneous. Attacks can be triggered by daily activities involving contact with the external acoustic meatus or auricle. Patients have also noted that attacks of nervus intermedius neuralgia can be triggered by lying on the affected side. Disorders of lacrimation, salivation and taste have also been reported in patients suffering from nervus intermedius neuralgia. Pain can be controlled with medication in some patients, but microvascular decompression or surgical resection of the nervus intermedius is required in approximately 50% of cases.

Key words facial nerve; geniculate neuralgia; magnetic resonance imaging; microvascular decompression; nervus intermedius; nervus intermedius neuralgia; posterior fossa surgery; ultrasound guided facial nerve block

ICD-10 CODE B02.21

The Clinical Syndrome Nervus intermedius neuralgia is an uncommon cause of primary otalgia. Also known as geniculate neuralgia, nervus intermedius neuralgia is believed to be caused by compression of the nervus intermedius portion of the cranial nerve VII (facial) by aberrant blood vessels or tumor in a manner analogous to trigeminal and glossopharyngeal neuralgia (Fig. 18.1). Although cranial nerve VII is primarily a motor nerve comprising special visceral efferent fibers that innervate the facial muscles, a small number of sensory and parasympathetic fibers are also present. These sensory fibers provide sensory innervations to the skin of the external auditory meatus, portions of the nasal and nasopharyngeal mucosa, and the anterior two-thirds of the tongue. When the nervus intermedius and its associated geniculate ganglion are infected with herpes zoster virus, a clinical syndrome known as Ramsey Hunt syndrome occurs (see Chapter 14). The pain of nervus intermedius neuralgia is severe and is rivaled only by that of trigeminal and glossopharyngeal neuralgia and cluster headache. The pain has been described as like having an ice pick repeatedly jabbed into the ear. Uncontrolled pain of this severity has been associated with suicide and should therefore be treated as an emergency. Attacks can be triggered by daily activities involving contact with the external acoustic meatus or auricle. Patients have also noted that attacks of nervus intermedius neuralgia can be triggered by lying on the affected side (Fig. 18.2). Disorders of lacrimation, salivation, and taste have also been reported in patients suffering from nervus intermedius neuralgia. Pain can be controlled with medication in some patients, but microvascular decompression or surgical resection of the nervus intermedius is required in approximately 50% of cases. The association between multiple sclerosis and trigeminal neuralgia does not appear to be strong in patients with nervus intermedius neuralgia, but a single case has been reported.

Signs and Symptoms Nervus intermedius neuralgia causes severe, episodic pain afflicting the area of the acoustic auditory meatus supplied by the nervus intermedius. The pain is unilateral and characterized by paroxysms of electric shock–like pain lasting from several seconds to less than 2 minutes. The progression from onset to peak is essentially instantaneous. Patients with nervus intermedius neuralgia go to great lengths to avoid any contact with trigger areas. In contrast, persons with other types of facial pain, such as temporomandibular joint dysfunction, tend to constantly rub the affected area or apply heat or cold to it. Patients with uncontrolled nervus intermedius neuralgia frequently require hospitalization for rapid control of pain. Between attacks, patients are relatively pain free. A dull ache remaining after the intense pain subsides may indicate persistent compression of the nerve by a structural lesion. Disorders of lacrimation, salivation, and taste may also be present. This disease is almost never seen in persons younger than 30 years. Patients with nervus intermedius neuralgia often have severe depression (sometimes to the point of being suicidal), with high levels of superimposed anxiety during acute attacks. Both of these problems may be exacerbated by the sleep deprivation that often accompanies painful episodes.

Testing All patients with a new diagnosis of nervus intermedius neuralgia should undergo magnetic resonance imaging (MRI) of the brain and brainstem, with and without gadolinium contrast medium, to rule out posterior fossa or brainstem lesions and demyelinating disease (Fig. 18.3). Magnetic resonance angiography is also useful to confirm vascular compression of the nervus intermedius or geniculate ganglion by aberrant blood vessels. Functional MRI may also provide useful information as to the anatomical location of the pathophysiology responsible for the patient’s symptoms (Fig. 18.4). Additional imaging of the sinuses should be considered in the case of any question of occult or coexisting sinus disease. If the first division of the trigeminal nerve is affected, ophthalmological evaluation to measure intraocular pressure and rule out intraocular pathological conditions is indicated. Screening laboratory tests consisting of a complete blood count, erythrocyte sedimentation rate, and automated blood chemistry should be performed if the diagnosis of trigeminal neuralgia is in question. A complete blood count is required for baseline comparisons before starting treatment with carbamazepine (see discussion of treatment).

FIG. 18.1 Arterial loop that was in close contact with the lower cranial nerves at the level of the brainstem in a patient with nervus intermediate neuralgia. From Tubbs RS, Mosier KM, Cohen-Gadol AA. Geniculate neuralgia: clinical, radiologic, and intraoperative correlates. World Neurosurg. 2013;80[6]:e353–e357, fig. 6. ISSN 1878-8750, https://doi.org/10.1016/j.wneu.2012.11.053. http://www.sciencedirect.com/science/article/pii/S1878875012013526.

FIG. 18.2 Nervus intermedius neuralgia is characterized by severe episodic neuritic pain affecting the area of the acoustic auditory meatus.

FIG. 18.3 Gadolinium-Enhanced Magnetic Resonance ImagingImages show a centrally enhancing lesion in the geniculate ganglion (arrow), measuring 5 mm × 10 mm in diameter. From Miyashita T, Hoshikawa H, Kagawa M, et al. A case report of facial nerve hemangioma. Auris Nasus Larynx. 2007;34:519–522.

Differential Diagnosis Nervus intermedius neuralgia is generally a diagnosis of exclusion, although the clinical presentation makes it a straightforward clinical diagnosis that can be made on the basis of a targeted history and physical examination. Diseases of the eyes, ears, nose, throat, and teeth may mimic nervus intermedius neuralgia or may coexist and confuse the diagnosis. Atypical facial pain or temporomandibular joint dysfunction is sometimes confused with nervus intermedius neuralgia, but it can be distinguished by the character of the pain—atypical facial pain is dull and aching, whereas the pain of nervus intermedius neuralgia is sharp and neuritic. Additionally, the pain of nervus intermedius neuralgia occurs in the distribution of the nervus intermedius, whereas the pain of atypical facial pain does not follow a specific nerve distribution. Multiple sclerosis should be considered in all patients who present with nervus intermedius neuralgia before the fifth decade of life.

Treatment Drug Therapy Carbamazepine Carbamazepine is considered first-line treatment for nervus intermedius neuralgia. In fact, a rapid response to this drug helps confirm the clinical diagnosis. Despite the safety and efficacy of carbamazepine, some confusion and anxiety exist surrounding its use. This medication, which may be the patient’s best chance for pain control, is sometimes discontinued because of laboratory abnormalities erroneously attributed to it. Therefore baseline measurements consisting of a complete blood count, urinalysis, and automated blood chemistry profile should be obtained before starting the drug. Carbamazepine should be initiated slowly if the pain is not out of control, with a starting dose of 100 to 200 mg at bedtime for 2 nights. The patient should be cautioned about side effects, including dizziness, sedation, confusion, and rash. The drug is increased in 100- to 200-mg increments given in equally divided doses over 2 days, as side effects allow, until pain relief is obtained or a total dose of 1200 mg per day is reached. Careful monitoring of laboratory parameters is mandatory to avoid the rare possibility of a life-threatening blood dyscrasia. At the first sign of blood count abnormality or rash, this drug should be discontinued. Failure to monitor patients on carbamazepine can be disastrous, because aplastic anemia can occur. When pain relief is obtained, the patient should be kept at that dosage of carbamazepine for at least 6 months before tapering of the medication is considered. The patient should be informed that under no circumstances should the drug dosage be changed or the drug refilled or discontinued without the physician’s knowledge.

FIG. 18.4 Zoomed and cropped axial images from functional magnetic resonance imaging of cranial nerve (CN) VII stimulation paradigm. Activation is seen as red or yellow areas in the region of the right spinotrigeminothalamic tract and mesencephalic nucleus of CN V (A), right spinal tract of the trigeminal nerve (B), and left spinal tract of the trigeminal and CN V sensory nucleus (C). From Tubbs RS, Mosier KM, Cohen-Gadol AA. Geniculate neuralgia: clinical, radiologic, and intraoperative correlates. World Neurosurg. 2013;80[6]:e353–e357, fig. 2. ISSN 1878-8750, https://doi.org/10.1016/j.wneu.2012.11.053. http://www.sciencedirect.com/science/article/pii/S1878875012013526.

Gabapentin In the uncommon event that carbamazepine does not adequately control a patient’s pain, gabapentin may be considered. As with carbamazepine, baseline blood tests should be obtained before starting therapy and the patient should be cautioned about potential side effects, including dizziness, sedation, confusion, and rash. The initial dose is 300 mg at bedtime for 2

nights. The drug is then increased in 300-mg increments given in equally divided doses over 2 days, as side effects allow, until pain relief is obtained or a total dose of 2400 mg per day is reached. At this point, if the patient has experienced only partial pain relief, blood values are measured and the drug is carefully titrated upward using 100-mg tablets. Rarely is a dosage greater than 3600 mg per day required.

Pregabalin Pregabalin represents a reasonable alternative to gabapentin and is better tolerated in some patients. Pregabalin is started at 50 mg three times per day and may be titrated upward to 100 mg three times per day as side effects allow. Pregabalin is excreted primarily by the kidneys, and thus the dosage should be decreased in patients with compromised renal function.

Baclofen Baclofen may be of value in some patients who fail to obtain relief from carbamazepine, gabapentin, or pregabalin. As with those drugs, baseline laboratory tests should be obtained before beginning baclofen therapy and the patient should be warned about the same potential adverse effects. Start with a 10-mg dose at bedtime for 2 nights, then increase the drug in 10-mg increments given in equally divided doses over 7 days, as side effects allow, until pain relief is obtained or a total dose of 100 mg per day is reached. This drug has significant hepatic and central nervous system side effects, including weakness and sedation. As with carbamazepine, careful monitoring of laboratory values is indicated when using baclofen. When treating individuals with any of these drugs, the physician should make sure the patient knows that premature tapering or discontinuation of the medication may lead to the recurrence of pain, which will be more difficult to control.

Invasive Therapy Microvascular Decompression of the Nervus Intermedius and/or Geniculate Ganglion Compression of the nervus intermedius and the geniculate ganglion by aberrant arteries can cause nervus intermedius neuralgia. Microvascular decompression of these neural structures in a manner analogous to the

Janneta procedure may provide dramatic symptomatic relief.

Section of the Nervus Intermedius This neurosurgical technique is the invasive treatment of choice for those patients with nervus intermedius neuralgia who have failed to respond to conservative pharmacological management and microvascular decompression procedures. To perform this procedure, the nervus intermedius and geniculate ganglion are identified and isolated and the nervus intermedius is sectioned in two places. Some surgeons also advocate extirpation of the geniculate ganglion. Section of the nervus intermedius alone provides excellent palliation of pain in 75% to 90% of cases.

Complications and Pitfalls The pain of nervus intermedius neuralgia is severe and can lead to suicide; therefore it must be considered a medical emergency, and strong consideration should be given to hospitalizing such patients. If a dull ache remains between the intense paroxysms of pain, the clinician should have a high index of suspicion that the nidus of the patient’s pain is persistent compression of the nerve by a structural lesion such as a brainstem tumor or schwannoma.

Clinical Pearls Nervus intermedius neuralgia is an uncommon cause of otalgia. Because of the potential for disastrous clinical outcome should a more common cause of otic pain be overlooked (e.g., tumor or the temporal bone, brainstem, or nasopharynx), the diagnosis of nervus intermedius neuralgia must by necessity be one of exclusion. Because of the severity of the pain associated with this syndrome, aggressive pharmacological management in an inpatient setting may be required. Surgical treatment consisting of the sectioning of the nervus intermedius is often the patient’s best option for complete and long-lasting pain relief.

Suggested Readings Alcaraz N, King W.A, Wackym P.A. Endoscopy during neurotomy of the nervus intermedius for geniculate neuralgia. Otolaryngol Head Neck Surg. 1999;121:334–336. Bhagra A, Stead L.G. Nervus intermedius neuralgia: a rare entity. Ann Emerg Med. 2006;47:579–584. Dorsch J.N. Neurologic syndromes of the head and neck. Prim Care Clin Office Pract. 2014;41(1):133–149. Gantz B.J, Redleaf M.I, Perry B.P, Gubbels S.P. Management of Bell’s palsy and nervus intermedius neuralgia. In: Brackmann D.E, Shelton C, Arriaga M.A, eds. Otologic Surgery. 3rd ed. Philadelphia: Elsevier; 2010:335–346. Persson A, Bergström T, Lindh M, Namvar L, Studahl M. Varicella-zoster virus CNS disease: viral load, clinical manifestations and sequels. J Clin Virol. 2009;46:249–253. Taguchi T, Ueda S, Kudo T, et al. Ramsay-Hunt syndrome. J Infect. 2011;62:180– 181. Tubbs R.S, Steck D.T, Mortazavi M.M, Cohen-Gadol A.A. The nervus intermedius: a review of its anatomy, function, pathology, and role in neurosurgery. World Neurosurg. 2013;79(5–6):763–767 878–8750. Tubbs R.S, Mosier K.M, Cohen-Gadol A.A. Geniculate neuralgia. Clinical, radiologic, and intraoperative correlates. World Neurosurg. 2013;80(6):e353– e357. Ulusoy Ş, Özkan G, Bektaş D, et al. Nervus intermedius neuralgia in renal transplantation recipient: a case report. Transplant Proc. 2010;42:1986–1988.

19

Red Ear Syndrome

Abstract Red ear syndrome is an uncommon primary pain disorder thought to be a variant of one of a group of three headache syndromes known as the trigeminal autonomic cephalgias. As its name implies, the pathognomonic finding of red ear syndrome is in fact a unilateral red ear redness involves the entire ear, including the pinna, and is associated with neuralgia-like pain reminiscent of sudden unilateral neuralgiform conjunctival injection tearing (SUNCT) headache The pain and erythema associated with red ear syndrome have a rapid onset to peak, with attacks lasting 15 seconds to 5 minutes and the frequency of attacks ranging from 20 to 200 attacks per day. In some patients, these attacks can be triggered by sensory stimulation of the affected area, such as when brushing the hair. Although in many ways similar to SUNCT headache (i.e., unilateral, rapid onset to peak, short duration of attacks, pain-free periods between attacks), many dissimilarities also exist, including the location and pronounced autonomic phenomenon manifested by the red ear.

Key words facet block; facial pain; greater auricular nerve; nervus intermedius neuralgiaotalgia; red ear syndrome; relapsing polychondritis; trigeminal autonomic cephalgias

ICD-10 CODE G50.0

The Clinical Syndrome Red ear syndrome is an uncommon primary pain disorder thought to be a variant of one of a group of three headache syndromes known as the trigeminal autonomic cephalgias (Table 19.1). Whether red ear syndrome is in fact a distinct pain syndrome resulting from auriculo-autonomic dysfunction or simply a constellation of symptoms that occurs on a continuum along with the other trigeminal autonomic cephalgias is a point of ongoing debate among headache and pain management specialists. As with most headache and facial pain syndromes, the exact cause of the pain of red ear syndrome is unknown; however, the pathogenesis of this uncommon cause of head and face pain is thought to be dysfunction of the trigeminal autonomic reflex. The rapid onset of ear redness and associated pain may be caused by an antidromic release of vasoactive peptides from the terminal afferent fibers of the third cervical nerve root, which provides sensory innervations to the pinna of the ear. As its name implies, the pathognomonic finding of red ear syndrome is in fact a unilateral red ear (Fig. 19.1). This redness involves the entire ear, including the pinna, and is associated with neuralgia-like pain reminiscent of sudden unilateral neuralgiform conjunctival injection tearing (SUNCT) headache (see Chapter 8). The pain and erythema associated with red ear syndrome have a rapid onset to peak, with attacks lasting 15 seconds to 5 minutes and the frequency of attacks ranging from 20 to 200 attacks per day. In some patients, these attacks can be triggered by sensory stimulation of the affected area, such as when brushing the hair. Although in many ways similar to SUNCT headache (i.e., unilateral, rapid onset to peak, short duration of attacks, pain-free periods between attacks), many dissimilarities also exist, including the location and pronounced autonomic phenomenon manifested by the red ear.

TABLE 19.1 The Trigeminal Autonomic Cephalgias • Cluster headache • Paroxysmal hemicranias • Short-lasting unilateral neuralgiform headache with conjunctival injection tearing

Signs and Symptoms Patients with red ear syndrome present with the complaint of severe paroxysms of sudden onset of unilateral ear redness associated with pain involving the ipsilateral ear (Fig. 19.2). The pain is neuralgiform in quality and severe to excruciating in intensity. Like trigeminal neuralgia, the pain of red ear syndrome rarely switches sides. Red ear syndrome occurs slightly more frequently in males. It can occur at any age, with a peak incidence in the fifth decade.

Testing Magnetic resonance imaging (MRI) of the brain provides the clinician with the best information regarding the cranial vault and its contents. MRI is highly accurate and helps identify abnormalities that may put the patient at risk for neurological disasters secondary to intracranial and brainstem pathology, including tumors and demyelinating disease. Magnetic resonance angiography (MRA) also may be useful in helping identify aneurysms that may be responsible for the patient’s neurological findings. In patients who cannot undergo MRI, such as patients with pacemakers, computed tomography (CT) is a reasonable second choice. Radionuclide bone scan and plain radiography are indicated if a fracture or bony abnormality such as metastatic disease is considered in the differential diagnosis.

FIG. 19.1 Red ear syndrome is characterized by the complaint of severe paroxysms of sudden onset of unilateral ear redness associated with ipsilateral ear pain.

FIG. 19.2 Left-Sided Red Ear Syndrome in a 5-Year-Old Boy From Moitri MO, Banglawala SM, Archibald J. Red ear syndrome: literature review and a pediatric case report. Int J Pediatr Otorhinolaryngol. 2015;79[3]:281–285, fig. 1. ISSN 0165-5876, https://doi.org/10.1016/j.ijporl.2014.12.023. http://www.sciencedirect.com/science/article/pii/S0165587614006910.

Screening laboratory tests consisting of complete blood cell count, erythrocyte sedimentation rate, and automated blood chemistry should be performed if the diagnosis of red ear syndrome is in question. Additional testing to rule out collagen-vascular disease is indicated if polychondritis is suspected.

Differential Diagnosis Red ear syndrome is a clinical diagnosis supported by a combination of clinical history, normal physical examination, radiography, and MRI. Pain syndromes that may mimic red ear syndrome include erythromelalgia of the ear, polychondritis, cluster headache, temporal arteritis, trigeminal neuralgia, demyelinating disease, primary stabbing headache, SUNCT, and chronic paroxysmal hemicranias. However, because of the overlapping features of all headache and facial pain syndromes, red ear syndrome easily can be mistaken for another type of headache or facial pain. Trigeminal neuralgia is more common and is characterized by trigger areas and tic-like movements. Demyelinating disease is generally associated with other neurological findings, including optic neuritis and other motor and sensory abnormalities. The pain of chronic paroxysmal hemicrania lasts much longer than the pain of red ear syndrome.

Treatment The treatment of red ear syndrome is analogous to the treatment of trigeminal neuralgia, although the pharmacological management of this uncommon headache disorder is disappointing. The use of anticonvulsants such as lamotrigine and gabapentin represents a reasonable starting point. Anecdotal reports have reported improvement in symptomatology with amitriptyline, verapamil, and propranolol. High-dose steroids tapered over 10 days also have been anecdotally reported to provide relief. For patients who do not respond to these treatments, a few case reports suggest that daily ipsilateral C2 to C3 facet joint blocks with local anesthetic and steroid may provide relief of both the pain and the autonomic dysfunction. Underlying sleep disturbance and depression associated with the pain of red ear syndrome are best treated with a tricyclic antidepressant compound, such as nortriptyline, which can be started at a single bedtime dose of 25 mg.

Complications and Pitfalls Failure to diagnose red ear syndrome correctly may put the patient at risk if intracranial pathology or demyelinating disease, which may mimic the clinical presentation of red ear syndrome, is overlooked. MRI is indicated in all patients thought to have red ear syndrome. A careful evaluation of the ear to rule out localized pathological conditions is also indicated, as is laboratory testing for collagen-vascular disease if polychondritis is suspected.

Clinical Pearls Given the poor response to treatment with drugs traditionally used to treat trigeminal neuralgia, facet block of the ipsilateral C2–C3 facet joints with local anesthetic and steroids should be considered in patients thought to have red ear syndrome. Given the uncommon nature of this headache syndrome and its overlap with the other trigeminal autonomic cephalgias and other more serious forms of intracranial pathological conditions such as tumors and vascular abnormalities, red ear syndrome must remain a diagnosis of exclusion. All patients thought to have red ear syndrome require MRI of the brain with and without gadolinium contrast material and thorough otic and neurological evaluation. Cervical facet block should be performed only by clinicians familiar with the regional anatomy.

Suggested Readings Flicinski J, Wigowska-Sowinska J, Winczewska-Wiktor A, Steinborn B. Red ear syndrome – case report and review of literature. Neurol Neurochir Pol. 2015;49(1):74–77. Kumar N, Swanson J.W. The ‘red ear syndrome’ revisited: two cases and a review of literature. Cephalalgia. 2004;24:305–308. Lambru G, Miller S, Matharu M.S. The red ear syndrome. J Headache Pain. 2013;14:83. Lance J.W. The red ear syndrome. Neurology. 1996;47:617–620. Leone M, Bussone G. Pathophysiology of trigeminal autonomic cephalalgias. Lancet Neurol. 2009;8:1855–1884. Moitri M.O, Banglawala S.M, Archibald J. Red ear syndrome: literature review and a pediatric case report. Int J Pediatr Otorhinolaryngol. 2015;79(3):281– 285. Purdy R.A, Dodick D.W. Red ear syndrome. Curr Pain Headache Rep. 2007;11:313–316. Raieli V, Compagno A, D’Amelio M. Red ear syndrome. Curr Pain Headache Rep. 2016;20(3):19. Waldman S.D. Cervical facet block. In: Waldman S.D, ed. Atlas of Interventional Pain Management. 3rd ed. Philadelphia: Saunders; 2009:165– 168.

20

Glossopharyngeal Neuralgia

Abstract The pain of glossopharyngeal neuralgia is in the distribution of cranial nerve IX. In some patients, overflow pain may occur in areas innervated by the trigeminal nerve, upper cervical segments, or both. The pain is neuritic and is unilateral in 98% of patients. It is often described as shooting or stabbing, with a severe intensity level. The pain of glossopharyngeal neuralgia is often triggered by swallowing, chewing, coughing, or talking. With the exception of trigger areas in the distribution of cranial nerve IX, the patient’s neurological examination should be normal. Because tumors at the cerebellopontine angle may produce symptoms identical to those of glossopharyngeal neuralgia, an abnormal neurological examination is cause for serious concern. Dull, aching pain that persists between the paroxysms of pain normally associated with glossopharyngeal neuralgia is highly suggestive of a space-occupying lesion and requires thorough evaluation.

Key words anticonvulsants; bradycardia; geniculate neuralgia; gloospharyngeal neuralgia; glossopharyngeal nerve block; microvascular decompression; nervus intermedius neuralgia; syncope; trigeminal neuralgia; ultrasound guided glossopharyngeal nerve block

ICD-10 CODE G52.10

The Clinical Syndrome Glossopharyngeal neuralgia is a rare condition characterized by paroxysms of pain in the sensory division of the cranial nerve IX. Although the pain of glossopharyngeal neuralgia is similar to that of trigeminal neuralgia, it occurs 100 times less frequently. Glossopharyngeal neuralgia occurs more commonly in patients older than 50 years. The pain is located in the tonsil, laryngeal region, and posterior tongue. The pain is unilateral in most patients, but can occur bilaterally 2% of the time. Rarely, the pain of glossopharyngeal neuralgia is associated with bradyarrhythmias; in some patients, it is associated with syncope. These cardiac symptoms are thought to be due to overflow of neural impulses from the glossopharyngeal nerve to the vagus nerve. Although rare, this unusual combination of pain and cardiac arrhythmia can be lethal.

Signs and Symptoms The pain of glossopharyngeal neuralgia is in the distribution of cranial nerve IX (Fig. 20.1). In some patients, overflow pain may occur in areas innervated by the trigeminal nerve, upper cervical segments, or both. The pain is neuritic and is unilateral in 98% of patients. It is often described as shooting or stabbing, with a severe intensity level. The pain of glossopharyngeal neuralgia is often triggered by swallowing, chewing, coughing, or talking. With the exception of trigger areas in the distribution of cranial nerve IX, the patient’s neurological examination should be normal. Because tumors at the cerebellopontine angle may produce symptoms identical to those of glossopharyngeal neuralgia, an abnormal neurological examination is cause for serious concern (Fig. 20.2). Dull, aching pain that persists between the paroxysms of pain normally associated with glossopharyngeal neuralgia is highly suggestive of a space-occupying lesion and requires thorough evaluation.

Testing Magnetic resonance imaging (MRI) of the brain and brainstem should be performed in all patients thought to have glossopharyngeal neuralgia. MRI of the brain provides the best information regarding the cranial vault and its contents. MRI is highly accurate and helps identify abnormalities that may put the patient at risk for neurological disasters secondary to intracranial and brainstem pathology, including tumors and demyelinating disease (see Fig. 20.2). MRI can also aid in the identification of blood vessels that may be compressing the glossopharyngeal nerve (Fig. 20.3). Magnetic resonance angiography (MRA) may be helpful in identifying aneurysms responsible for neurological symptoms. In patients who cannot undergo MRI, such as patients with pacemakers, computed tomography (CT) is a reasonable second choice. Clinical laboratory tests consisting of complete blood cell count, automated chemistry profile, and erythrocyte sedimentation rate are indicated to rule out infection, temporal arteritis, and malignancy that may mimic glossopharyngeal neuralgia. Endoscopy of the hypopharynx with special attention to the piriform sinuses also is indicated to rule out occult malignancy. Differential neural blockade of the glossopharyngeal nerve may help strengthen the diagnosis of glossopharyngeal neuralgia.

FIG. 20.1 The Pain of Glossopharyngeal Neuralgia is in the Distribution of Cranial Nerve IX.

FIG. 20.2 Mixed Cystic and Solid Acoustic Nerve Schwannoma in Association With a Solid Schwannoma of the Geniculate Ganglion(A) Axial enhanced image with fat saturation. A large mass with solid and cystic enhancing components is seen in the right cerebellopontine angle. A separate solid erosive tumor is seen in the region of the right geniculate ganglion (arrowhead). (B) Coronal enhanced image with fat saturation. The characteristic mushroom appearance of an intracanalicular acoustic schwannoma with extension into the adjacent cerebellopontine angle is well seen. This more anterior section through the internal auditory canal does not show the cystic portion of the tumor, but it does show the compression of the adjacent brainstem. From Stark DD, Bradley WG Jr, eds. Magnetic Resonance Imaging. 3rd ed. St Louis: Mosby; 1999:1219.

Differential Diagnosis Glossopharyngeal neuralgia is generally a straightforward clinical diagnosis that can be made on the basis of a targeted history and physical examination. Diseases of the eye, ears, nose, throat, and teeth may mimic trigeminal neuralgia or may coexist and confuse the diagnosis. Tumors of the hypopharynx, including the tonsillar fossa and piriform sinuses, may mimic the pain of glossopharyngeal neuralgia, as may tumors at the cerebellopontine angle. Occasionally, demyelinating disease may produce a clinical syndrome identical to glossopharyngeal neuralgia. The jaw claudication associated with temporal arteritis also sometimes confuses the clinical picture, as does trigeminal neuralgia.

Treatment Pharmacological Treatment Carbamazepine Carbamazepine is considered first-line treatment for glossopharyngeal neuralgia. Rapid response to this drug essentially confirms a clinical diagnosis of glossopharyngeal neuralgia. Despite the safety and efficacy of carbamazepine compared with other treatments for glossopharyngeal neuralgia, much confusion and unfounded anxiety surround its use. This medication, which may be the patient’s best chance for pain control, is sometimes discontinued because of laboratory abnormalities erroneously attributed to it. Baseline screening laboratory tests, consisting of a complete blood cell count, urinalysis, and automated chemistry profile, should be obtained before starting the drug. Carbamazepine should be started slowly, if the pain is not out of control, at a starting dose of 100 to 200 mg at bedtime for 2 nights; the patient should be cautioned regarding side effects, including dizziness, sedation, confusion, and rash. The drug is increased in 100- to 200-mg increments, given in equally divided doses over 2 days, as side effects allow, until pain relief is obtained or a total dose of 1200 mg per day is reached. Careful monitoring of laboratory parameters is mandatory to avoid the rare possibility of life-threatening blood dyscrasia. At the first sign of blood count abnormality or rash, this drug should be discontinued. Failure to monitor patients started on carbamazepine can be disastrous because aplastic anemia can occur. When pain relief is obtained, the patient should be kept at that dosage of carbamazepine for at least 6 months before considering tapering of this medication. The patient should be informed that under no circumstances should the dosage of drug be changed or the drug refilled or discontinued without the physician’s knowledge.

FIG. 20.3 Magnetic Resonance Imagings of Glossopharyngeal Neuralgia(A) A vertebral artery (VA) compressed the left ninth and tenth (IX–X) nerves. (B) The vertebral artery moved to the left side, and there were no space-occupying lesions in posterior cranial fossa. From Zhao H, Zhang X, Zhu J, et al. Microvascular decompression for glossopharyngeal neuralgia: long-term follow-up. World Neurosurg. 2017;102:151– 156, fig. 1. ISSN 1878-8750, https://doi.org/10.1016/j.wneu.2017.02.106. http://www.sciencedirect.com/science/article/pii/S1878875017302796.

Gabapentin In the uncommon event that carbamazepine does not control a patient’s pain adequately, gabapentin may be considered. As with carbamazepine, baseline blood tests should be obtained before starting therapy. Gabapentin should be started with a 300-mg dose at bedtime for 2 nights; the patient should be cautioned about potential side effects, including dizziness, sedation, confusion, and rash. The drug is increased in 300-mg increments, given in equally divided doses over 2 days, as side effects allow, until pain relief is obtained or a total dose of 2400 mg per day is reached. At this point, if the patient has experienced partial pain relief, blood values are measured and the drug is carefully titrated using 100-mg tablets. More than 3600 mg per day is rarely required.

Baclofen Baclofen has been reported to be of value in some patients who fail to obtain relief from carbamazepine and gabapentin. Baseline laboratory tests should be obtained before starting baclofen. The drug is started with a 10-mg dose at bedtime for 2 nights; the patient should be cautioned about potential adverse effects, which are the same as those of carbamazepine and gabapentin. The drug is increased in 10-mg increments, given in equally divided doses over 7 days, as side effects allow, until pain relief is obtained or a total dose of 80 mg daily is reached. This drug has significant hepatic and central nervous system side effects, including weakness and sedation. As with carbamazepine, careful monitoring of laboratory values is indicated during the initial use of this drug.

FIG. 20.4 Proper Needle Placement for Glossopharyngeal Nerve Block From Waldman SD, ed. Atlas of Interventional Pain Management Techniques. 3rd ed. Philadelphia: Saunders; 2009.

When treating patients with any of the drugs mentioned, the clinician should inform the patient that premature tapering or discontinuation of the medication may lead to the recurrence of pain. It becomes more difficult to control pain thereafter.

Interventional Treatment Glossopharyngeal Nerve Block The use of glossopharyngeal nerve block with local anesthetic and a steroid serves as an excellent adjunct to drug treatment of glossopharyngeal neuralgia (Fig. 20.4). This technique rapidly relieves pain while medications are being titrated to effective levels. The initial block is performed with preservative-free bupivacaine combined with methylprednisolone. Subsequent daily nerve blocks are done in a similar manner, substituting a lower dose of methylprednisolone. This approach also may be used to obtain control of breakthrough pain. Ultrasound guided needle placement may improve the efficacy of this technique and help avoid needle-related complications (Fig. 20.5).

Radiofrequency Destruction of the Glossopharyngeal Nerve The destruction of the glossopharyngeal nerve can be carried out by creating a radiofrequency lesion under biplanar fluoroscopic guidance. This procedure is reserved for patients who have failed to respond to all the treatments mentioned for intractable glossopharyngeal neuralgia and who are not candidates for microvascular decompression of the glossopharyngeal root. Gamma knife ablation has also been used in this patient population. Ultrasound guided electrode placement may improve the efficacy of this technique and help avoid needle-related complications.

FIG. 20.5 Ultrasound Image Demonstrating the Relationship of the Glossopharyngeal Nerve to the Styloid Process

Microvascular Decompression of the Glossopharyngeal Root Microvascular decompression of the glossopharyngeal root, also referred to as the Jannetta procedure, is the major neurosurgical procedure of choice for intractable glossopharyngeal neuralgia. It is based on the theory that glossopharyngeal neuralgia is a compressive mononeuropathy analogous to trigeminal neuralgia. The operation consists of identifying the glossopharyngeal root close to the brainstem and isolating the offending compressing blood vessel (Fig. 20.6Aa). A sponge is interposed between the vessel and nerve, relieving the compression and the pain (see Fig. 20.6B).

Complications and Pitfalls The pain of glossopharyngeal neuralgia is severe and can lead to suicide; therefore it must be considered a medical emergency, and strong consideration should be given to hospitalizing such patients. If a dull ache remains after the intense, paroxysmal pain of glossopharyngeal neuralgia subsides, this is highly suggestive of persistent compression of the nerve by a structural lesion such as a brainstem tumor or schwannoma. Glossopharyngeal neuralgia is almost never seen in persons younger than 30 years unless it is associated with multiple sclerosis, and all such patients should undergo MRI to identify demyelinating disease. The major complications associated with glossopharyngeal nerve block are related to trauma to the internal jugular and carotid artery. Hematoma formation and intravascular injection of local anesthetic with subsequent toxicity are significant problems for the patient. Blockade of the motor portion of the glossopharyngeal nerve can result in dysphagia secondary to weakness of the stylopharyngeus muscle. If the vagus nerve is inadvertently blocked, as it often is during glossopharyngeal nerve block, dysphonia secondary to paralysis of the ipsilateral vocal cord may occur. Reflex tachycardia secondary to vagal nerve block is also observed in some patients. Inadvertent block of the hypoglossal and spinal accessory nerves during glossopharyngeal nerve block will result in weakness of the tongue and trapezius muscle.

FIG. 20.6 Intraoperative Photograph of a Patient Suffering From Left Glossopharyngeal Neuralgia(A) We retracted the cerebellum without using cerebellar retractors to expose the entire course of the ninth and tenth (IX–X) nerves and found the neurovascular conflict between the IX and X nerves and the posterior inferior cerebellar artery (PICA). (B) Teflon felts were put between the IX and X nerves and the PICA. From Zhao H, Zhang X, Zhu J, et al. Microvascular decompression for glossopharyngeal neuralgia: long-term follow-up. World Neurosurg. 2017;102:151– 156, fig. 2. ISSN 1878-8750, https://doi.org/10.1016/j.wneu.2017.02.106. http://www.sciencedirect.com/science/article/pii/S1878875017302796.

The glossopharyngeal nerve is susceptible to trauma from the needle, hematoma, or compression during injection procedures. Such complications, although usually transitory, can be quite upsetting to the patient. Although uncommon, risk for infection is ever present, especially in patients who have cancer and are immunocompromised. Early detection of infection is crucial to avoiding potentially life-threatening sequelae.

Clinical Pearls The pain of glossopharyngeal neuralgia is among the most severe pain that humans can experience and must be considered a medical emergency. The uncontrolled pain of glossopharyngeal neuralgia has led to suicide, and hospitalization of such patients should be strongly considered. Between attacks of glossopharyngeal neuralgia, the patient is relatively pain free. If a dull ache remains after the intense pain subsides, this is highly suggestive of a persistent compression of the nerve by a structural lesion, such as a brainstem tumor or schwannoma. Glossopharyngeal neuralgia is almost never seen in individuals younger than 30 years unless it is associated with multiple sclerosis, and all such patients should undergo MRI with sequences

designed to identify demyelinating disease.

Suggested Readings Albahkaly S, Alshehabi M, Saleh Al-Shmasi H, Ali Altaleb O. Reappraisal of glossopharyngeal neuralgia. Interdisciplinary Neurosurg. 2018;11:34–36. Benoliel R, Eliav E. Neuropathic orofacial pain. Oral Maxillofac Surg Clin North Am. 2008;20:237–254. den Hartog A.W, Jansen E, Kal J.E, et al. Recurrent syncope due to glossopharyngeal neuralgia. Heart Rhythm Case Rep. 2017;3(1):73–77. Franzini A, Ferroli P, Messina G, Broggi G. Surgical treatment of cranial neuralgias. In: Bruyn G, Vinken P, eds. Handbook of Clinical Neurology. 97. New York: Elsevier; 2010:679–692. Khan N.U, Iyer A. Glossopharyngeal neuralgia associated with anomalous glossopharyngeal nerve. Otolaryngol Head Neck Surg. 2007;136:502–503. Rao S, Rao S. Glossopharyngeal neuralgia: a mystery explored!. Int J Oral Maxillofacial Surg. 2015;44(1):e128–e129. Waldman S.D. Glossopharyngeal nerve block. In: Waldman S.D, ed. Atlas of Interventional Pain Management. 4th ed. Philadelphia: Elsevier; 2015:87–91. Zhao H, Zhang X, Zhu J, et al. Microvascular decompression for glossopharyngeal neuralgia: long-term follow-up. World Neurosurg. 2017;102:151–156 ISSN 18788750. https://doi.org/10.1016/j.wneu.2017.02.106.

SECT ION 2

Neck and Brachial Plexus Pain Syndromes OUT LINE 21. Clival Chordoma Syndrome 22. Spasmodic Torticollis 23. Cervicothoracic Interspinous Bursitis 24. Scapulocostal Syndrome 25. Parsonage-Turner Syndrome 26. Hyoid Syndrome 27. Omohyoid Syndrome 28. Acute Calcific Prevertebral Tendinitis 29. Neck-Tongue Syndrome 30. Sternohyoid Syndrome

21

Clival Chordoma Syndrome

Abstract Clival chordoma is a rare neoplasm that arises from embryological remnants of the notochord along the spinal axis. Clival chordomas are usually benign, although aggressive clival chordomas have been reported. Comprising one third of central nervous system chordomas, clival chordomas tend to be slow growing and produce symptoms by compression of the adjacent brainstem and cranial nerves. In spite of this, the long-term outcome of patients diagnosed with clival chordoma remains poor because of the location of these tumors and their tendency to recur regardless of the treatment method chosen. Clival chordomas can occur at any age, further complicating the diagnosis. These uncommon tumors occur slightly more commonly in men. Early diagnosis of clival chordoma is important to avoid acute neurological disasters; however, because of the slow growth of these tumors, the average time from onset of symptoms to diagnosis averages 2 years.

Keywords chondrosarcoma; chordoma; clival chordoma; clival lesion; clival tumor; clivus; endoscopic endonasal approach; skull base surgery

ICD-10 CODE D16.4

The Clinical Syndrome Clival chordoma is a rare neoplasm that arises from embryological remnants of the notochord along the spinal axis. Clival chordomas are usually benign, although aggressive clival chordomas have been reported. Comprising onethird of central nervous system chordomas, clival chordomas tend to be slow growing and produce symptoms by compression of the adjacent brainstem and cranial nerves. In spite of this, the long-term outcome of patients diagnosed with clival chordoma remains poor because of the location of these tumors and their tendency to recur regardless of the treatment method chosen. Clival chordomas can occur at any age, further complicating the diagnosis. These uncommon tumors occur slightly more often in men. Early diagnosis of clival chordoma is important to avoid acute neurological disasters; however, because of the slow growth of these tumors, the average time from onset of symptoms to diagnosis averages 2 years.

Signs and Symptoms Headache is the most common presenting complaint in patients with clival chordoma. Other common symptoms associated with clival chordoma reflect the propensity of this tumor to compress adjacent neural structures, causing facial pain, facial numbness, facial paresthesias, and diplopia (Table 21.1). Ataxia, dysphagia, visual disturbance, hoarseness, and extremity weakness also commonly occur. Findings on neurological examination (e.g., cranial nerve deficits, pyramidal tract dysfunction, hemiparesis, hyperreflexia, clonus, a positive Babinski sign, and cerebellar signs, including ataxia) also reflect compression of neural structures by this slow-growing tumor (Fig. 21.1). Occasionally, papilledema and optic nerve atrophy are identified.

Testing Magnetic resonance imaging (MRI) of the brain and brainstem should be performed in all patients thought to have clival chordoma (see Fig. 21.1). MRI of the brain provides the best information regarding the cranial vault and its contents. MRI is highly accurate and helps identify abnormalities that may put the patient at risk for neurological disasters secondary to intracranial and brainstem pathological conditions, including tumors and demyelinating disease (Fig. 21.2). Magnetic resonance angiography (MRA) may be helpful in identifying aneurysms responsible for neurological symptoms. In patients who cannot undergo MRI, such as patients with pacemakers, computed tomography (CT) is a reasonable second choice. Clinical laboratory tests consisting of a complete blood cell count, automated chemistry profile, and erythrocyte sedimentation rate are indicated to rule out infection, temporal arteritis, and other malignancies that may mimic clival chordoma. Endoscopy of the nasopharynx and hypopharynx with special attention to the piriform sinuses also is indicated to rule out occult malignancy.

TABLE 21.1 Common Symptoms Associated With Clival Chordoma Headache Facial numbness Facial pain Facial paresthesias Diplopia Dysarthria Dysphagia Ataxia Extremity weakness Hoarseness Visual disturbance

FIG. 21.1 Patients suffering from clival chordoma will often complain of headaches and associated facial pain, numbness, and diplopia.

FIG. 21.2 Sagittal (A) and axial (B) T2-weighted magnetic resonance imaging of the clival chordoma showing significant compression of the spinal cord and brainstem plus destruction of cervical vertebra. From Chau T, Lazzaro A, Mobbs RJ, et al. Surgical treatment of cranial neuralgias: combined endoscopic endonasal and posterior cervical approach to a clival chordoma. J Clin Neurosci. 2010;17:1463–1465.

Differential Diagnosis Clival chordoma is generally a straightforward clinical diagnosis in retrospect. Given that the time between the onset of neurological signs and symptoms and definitive diagnosis is an average of 2 years, a high index of clinical suspicion is necessary to avoid misdiagnosis. Obtaining a targeted history and performing a careful physical examination are essential. Diseases of the eye, ears, nose, throat, and teeth may mimic trigeminal neuralgia or may coexist and confuse the diagnosis. Tumors of the nasopharynx and hypopharynx, including the tonsillar fossa and piriform sinus, may mimic the pain of clival chordoma, as may tumors at the cerebellopontine angle. Occasionally, demyelinating disease may produce a clinical syndrome identical to clival chordoma. The jaw claudication associated with temporal arteritis also sometimes confuses the clinical picture, as does trigeminal neuralgia.

Treatment Treatment of clival chordoma requires surgery, radiation therapy, or both. Although clival chordomas are almost always benign and rarely metastasize, the critical location of clival chordomas relative to adjacent neural structures makes both forms of treatment challenging. Often, complete tumor resection is impossible because of the location, and postoperative radiation therapy, gamma knife stereotactic surgery, and implantation of radioactive seeds may be required.

Complications and Pitfalls Because of the slow-growing nature of clival chordomas, delayed diagnosis is an ever-present possibility complicating an already difficult treatment regimen. Further confusing the clinical presentation of this tumor of embryological origin is the fact that many of the clinical syndromes that mimic the signs and symptoms of clival chordoma are also difficult to diagnose.

Clinical Pearls Clival chordoma is a rare neoplasm that is usually benign, although aggressive clival chordomas have been reported. Clival chordomas tend to be slow growing and produce symptoms by compression of the adjacent brainstem and cranial nerves. In spite of this fact, the long-term outcome of patients diagnosed with clival chordoma remains poor because of the location of these tumors and their tendency to recur regardless of the treatment method chosen. Clival chordomas can occur at any age.

Suggested Readings Chau T, Lazzaro A, Mobbs R.J, Teo C. Surgical treatment of cranial neuralgias: combined endoscopic endonasal and posterior cervical approach to a clival chordoma. J Clin Neurosci. 2010;17:1463–1465. Chugh R, Tawbi H, Lucas D.R, et al. Chordoma: the nonsarcoma primary bone tumor. Oncologist. 2007;12:1344–1350. Feng K, Qiuhang Z, Qiuyi Q. Transclival cerebrospinal fluid rhinorrhea as the initial presenting symptom of a tiny intradural chordoma. J Clin Neurosci. 2010;17:1083–1085. Folbe A.J, Svider P.F, Liu J.K, Eloy J.A. Endoscopic resection of clival malignancies. Otolaryngol Clin North Am. 2017;50(2):315–329. Menezes A.H. Clival and craniovertebral junction chordomas. World Neurosurg. 2014;81(5–6):690–692. Neelakantan A, Rana A.K. Benign and malignant diseases of the clivus. Clini Radiol. 2014;69(12):1295–1303. Vilela M.D, Pedrosa H.A.S, Filho M.A.D. A hemorrhagic clival chordoma with a long progression-free survival. World Neurosurg. 2017;105:1042.e1–1042.e4.

22

Spasmodic Torticollis

Abstract Spasmodic torticollis is a rare condition characterized by involuntary movement of the head. It is classified as a focal or segmental dystonia and occurs in approximately 3 in 10,000 people. It begins in early adult life. The three varieties of spasmodic torticollis are as follows: (1) tonic, which involves involuntary turning of the head to one side; (2) clonic, which involves involuntary shaking of the head; and (3) tonic/clonic, which involves both types of involuntary movement. Spasmodic torticollis also can be subclassified based on the specific movement of the head: (1) rotation, which involves the turning of the head to the side; (2) laterocollis, which involves the leaning of the head against the shoulder; (3) retrocollis, which involves the leaning of the head toward the back; and (4) anterocollis, which involves the leaning of the head toward the chest. The disease occurs more commonly in women and often is initially diagnosed as a hysterical reaction or tic.

Keywords cervical dystonia; cervical plexus; microvascular decompression; neck pain; spasmodic torticollis; spinal accessory nerve

ICD-10 CODE G24.8

The Clinical Syndrome Spasmodic torticollis is a rare condition characterized by involuntary movement of the head. It is classified as a focal or segmental dystonia and occurs in approximately 3 in 10,000 people. It begins in early adult life. The three varieties of spasmodic torticollis are as follows: • Tonic, which involves involuntary turning of the head to one side • Clonic, which involves involuntary shaking of the head • Tonic/clonic, which involves both types of involuntary movement Spasmodic torticollis also can be subclassified based on the specific movement of the head: (1) rotation, which involves the turning of the head to the side; (2) laterocollis, which involves the leaning of the head against the shoulder; (3) retrocollis, which involves the leaning of the head toward the back; and (4) anterocollis, which involves the leaning of the head toward the chest. The disease occurs more commonly in women and often is initially diagnosed as a hysterical reaction or tic. Thought to be due to dysfunction centrally, rather than a disease of the affected muscles, spasmodic torticollis often begins as a subtle involuntary movement of the head. Early in the disease, the dystonia is often intermittent. As the disease progresses, the symptoms become more severe and harder for the patient to hide. The dystonic movements may become more sustained and associated with constant, aching pain in the affected muscles. The pain often becomes the primary reason for the patient to seek medical attention, with the patient almost indifferent to the dystonic movements. The dystonia often disappears with sleep and becomes less pronounced on first awakening, with the dystonic movements and pain worsening as the day progresses. Spontaneous recovery has been reported, but, overall, treatment is difficult and of limited success.

Signs and Symptoms A patient with spasmodic torticollis exhibits involuntary, dystonic movements of the head. In extreme cases, the dystonia is continuous and the laterocollis so marked that the patient’s ear rests on the ipsilateral shoulder (Figs. 22.1 and 22.2). Pain may be a predominant feature of the syndrome, and spasms of the cervical paraspinous musculature, the strap muscles of the neck, and the sternocleidomastoid are often present. Hypertrophy of the affected muscles may occur occasionally. Other than the dystonic movements, the neurological examination is normal. As mentioned previously, the patient may seem indifferent to the abnormal head movements or position. Touching the opposite side of the face or chin often causes the dystonia to cease momentarily.

Testing Magnetic resonance imaging (MRI) of the brain and brainstem should be performed in all patients thought to have spasmodic torticollis. MRI of the brain provides the best information regarding the cranial vault and its contents. MRI is highly accurate and helps identify abnormalities that may put the patient at risk for neurological disasters secondary to intracranial and brainstem pathological conditions, including tumors and demyelinating disease. Magnetic resonance angiography (MRA) may be useful in identifying aneurysms responsible for neurological symptoms. In patients who cannot undergo MRI, such as patients with pacemakers, computed tomography (CT) is a reasonable second choice. Clinical laboratory tests consisting of a complete blood cell count, automated chemistry profile, and erythrocyte sedimentation rate are indicated to rule out infection and malignancy.

Differential Diagnosis Spasmodic torticollis is generally a straightforward clinical diagnosis that can be made on the basis of a targeted history and physical examination. The involuntary nature of this movement disorder is the hallmark of the disease and helps distinguish it from tics and habit spasms that are voluntary and worsen when the patient is tense. Tics and habit spasms resemble volitional movement. Behavioral abnormalities, such as hysterical conversion reactions, also must be considered. Acute spasm and pain of the muscles of the neck or wry neck can mimic spasmodic torticollis, but its onset is acute, and the symptoms usually resolve within days to a week. Occasionally, patients with clonic spasmodic torticollis are initially diagnosed as having Parkinson disease.

FIG. 22.1 The dystonia of spasmodic torticollis causes significant pain and functional disability.

FIG. 22.2 Anterocollis, Right Laterocaput and Laterocollis From Le Goff F, Duparc F, Lefaucheur R, et al. Clavicle fracture mimicking droppedhead syndrome in a patient with multiple system atrophy. Parkinsonism Relat Disord. 2017;35:98–99, fig. 1A. ISSN 1353-8020, https://doi.org/10.1016/j.parkreldis.2016.11.008. http://www.sciencedirect.com/science/article/pii/S1353802016304394.

Treatment In general, the treatment of spasmodic torticollis is disappointing. Physical interventions including biofeedback and patient self-performed geste antagoniste, for example, touching one’s chin, may provide some symptomatic relief. Pharmacological treatment with skeletal muscle relaxants, including drugs that act at the spinal cord level, such as baclofen, and centrally acting drugs, such as the anticonvulsants and levodopa, may provide some symptomatic relief in mild cases. Trihexyphenidyl, dopamine agonists, and diazepam also have been advocated. In patients for whom pharmacological treatment fails, injection of the affected muscles with botulinum toxin is a reasonable next step. Frequent injections may result in the development of antibodies against the toxin, which makes the toxin less effective. By changing to different subtypes of toxin, efficacy may be restored. For intractable cases, microvascular decompression of the spinal accessory nerve, neurodestruction of the spinal accessory nerve, deep brain stimulation, and bilateral thalamotomy has been advocated. The results of treatments are variable at best.

Complications and Pitfalls Although spasmodic torticollis is usually a straightforward clinical diagnosis, the clinician must always rule out other pathological processes involving the central nervous system. Treatment of this syndrome is difficult, and treatment of concurrent depression is often required.

Clinical Pearls Spasmodic torticollis is a devastating disease that responds poorly to treatment. Injection of the affected muscles with botulinum toxin to effect chemodenervation is probably the best therapeutic option for most patients. The diagnosis of the disease is straightforward. MRI of the brain is indicated in all patients thought to have spasmodic torticollis.

Suggested Readings Del Sorbo F, Albanese A. Botulinum neurotoxins for the treatment of focal dystonias: review of rating tools used in clinical trials. Toxicon. 2015;107:89– 97 (Part A). Maia F.M, Kanashiro A.K, Chien H.F, Gonçalves L.R, Barbosa E.R. Clinical changes of cervical dystonia pattern in long-term botulinum toxin treated patients. Parkinsonism Relat Disord. 2010;16:8–11. Nicot R, Schlund M, Konopnicki S, Ferri J. Spasmodic torticollis after orthognathic surgery. J Stomatol Oral Maxillofac Surg. 2017;118(6):393–396. Ochudło S, Drzyzga K, Drzyzga L.R, Opala G. Various patterns of gestes antagonistes in cervical dystonia. Parkinsonism Relat Disord. 2007;13:417– 420. Takeuchi N, Chuma T, Mano Y. Phenol block for cervical dystonia: effects and side effects. Arch Phys Med Rehabil. 2004;85:1117–1120. Truong D, Brodsky M, Lew M, et al. Global Dysport Cervical Dystonia Study Group: long-term efficacy and safety of botulinum toxin type A (Dysport) in cervical dystonia. Parkinsonism Relat Disord. 2010;16:316–323. Xinyuan L, Shiting L, Benfang P, Chunhui H. Comparison of 2 operative methods for treating laterocollis and torticollis subtypes of spasmodic torticollis: follow-up of 121 cases. World Neurosurg. 2017;108:636–641. Yasumoto A, Nakao N, Doyu M. Neuropsychological backgrounds of the patients with botulinum toxin ineffective spasmodic torticollis. J Neurol Sci. 2017;381(suppl):1051.

23

Cervicothoracic Interspinous Bursitis

Abstract Cervicothoracic interspinous bursitis is an uncommon cause of pain in the lower cervical and upper thoracic spine. The interspinous ligaments of the lower cervical and upper thoracic spine and their associated muscles are susceptible to the development of acute and chronic pain symptoms after overuse. Bursitis is believed to be responsible for this pain syndrome. Frequently, the patient presents with midline pain after prolonged activity requiring hyperextension of the neck, such as painting a ceiling or prolonged use of a computer monitor with too high of a focal point. The pain is localized to the interspinous region between C7 and T1 and does not radiate. It is constant, dull, and aching. The patient may attempt to relieve the constant ache by assuming a posture of dorsal kyphosis with a thrusting forward of the neck. The pain of cervicothoracic interspinous bursitis often improves with activity and worsens with rest and relaxation.

Keywords cervicalgia; cervicothoracic interspinous bursitis; diagnostic ultrasound; magnetic resonance imaging; neck pain; overuse syndromes; ultrasound guided injection

ICD-10 CODE M71.50

The Clinical Syndrome Cervicothoracic interspinous bursitis is an uncommon cause of pain in the lower cervical and upper thoracic spine. The interspinous ligaments of the lower cervical and upper thoracic spine and their associated muscles are susceptible to the development of acute and chronic pain symptoms after overuse. Bursitis is believed to be responsible for this pain syndrome. Frequently, the patient presents with midline pain after prolonged activity requiring hyperextension of the neck, such as painting a ceiling or prolonged use of a computer monitor with too high of a focal point. The pain is localized to the interspinous region between C7 and T1 and does not radiate. It is constant, dull, and aching. The patient may attempt to relieve the constant ache by assuming a posture of dorsal kyphosis with a thrusting forward of the neck (Fig. 23.1). The pain of cervicothoracic interspinous bursitis often improves with activity and worsens with rest and relaxation.

Signs and Symptoms A patient with cervicothoracic bursitis presents with the complaint of dull, poorly localized pain in the lower cervical and upper thoracic region. The pain spreads from the midline to the adjacent paraspinous area, but is nonradicular. The patient often holds the cervical spine rigid, with the head thrust forward to splint the affected ligament and bursae. Flexion and extension of the lower cervical spine and upper thoracic spine tend to cause more pain than rotation of the head. The neurological examination of patients with cervicothoracic bursitis should be normal. Focal or radicular neurological findings suggest a central or spinal cord origin of pain symptoms and should be followed with magnetic resonance imaging (MRI) of the appropriate anatomical regions.

Testing MRI of the lower cervical and upper thoracic spine should be performed in all patients thought to have cervicothoracic bursitis (Fig. 23.2). Electromyography of the brachial plexus and upper extremities is indicated if neurological findings or pain that radiates into the arms are present. Clinical laboratory tests, including a complete blood cell count, automated chemistry profile, antinuclear antibody testing, and erythrocyte sedimentation rate, are indicated to rule out infection; collagen-vascular disease, including ankylosing spondylitis; and malignancy that may mimic the clinical presentation of cervicothoracic bursitis. Injection of the affected interspinous bursae with local anesthetic and steroid may serve as a diagnostic and therapeutic maneuver and may help strengthen the diagnosis of cervicothoracic bursitis. Plain radiography of the sacroiliac joints is indicated if ankylosing spondylitis is being considered in the differential diagnosis.

FIG. 23.1 Patients with cervicothoracic interspinous bursitis attempt to relieve pain by assuming a position of dorsal kyphosis with a thrusting forward of the neck.

FIG. 23.2 Magnetic Resonance Imaging (T2) of an Interspinous Bursa Measuring 2 × 2 × 2.5 cm Between C6 and C7 From Perka C, Schneider SV, Buttgereit F, et al. Development of cervical interspinous bursitis after prolonged sports trauma: a case report. Joint Bone Spine. 2006;73:118–120.

Differential Diagnosis The diagnosis of cervicothoracic bursitis is usually made on clinical grounds as a diagnosis of exclusion. The clinician needs to rule out intrinsic disease of the spinal cord, including syringomyelia and tumor, which may mimic the clinical presentation of cervicothoracic bursitis. Ankylosing spondylitis also may manifest in a manner similar to that of cervicothoracic bursitis. Fibromyalgia may coexist with cervicothoracic bursitis and should be identifiable by its characteristic trigger points and positive jump sign.

Treatment Initial treatment of the pain and functional disability associated with cervicothoracic bursitis should include a combination of nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and physical therapy. The local application of heat and cold also may be beneficial. For patients who do not respond to these treatment modalities, the following injection technique with a local anesthetic and steroid may be a reasonable next step.

FIG. 23.3 Proper Needle Placement for Injection for Treatment of Cervicothoracic Interspinous Bursitis Pain From Waldman SD. Atlas of Pain Management Injection Techniques. Philadelphia: Saunders; 2000;33.

The skin overlying the C7 to T1 interspace is prepared with antiseptic solution. A syringe containing 20 mL of 0.25% preservative-free bupivacaine and 40 mg of methylprednisolone is attached to a 25-gauge, inch needle. The needle is carefully advanced through the supraspinal ligament into the interspinous ligament (Fig. 23.3). Care must be taken to keep the needle in the midline and not to advance it too deeply, or inadvertent epidural, subdural, or subarachnoid injection could occur. After careful aspiration, a

volume of 2 to 3 mL is gently injected into the ligament. The patient should be informed that two to five treatment sessions may be required to abolish the symptoms of cervicothoracic bursitis completely. Ultrasound needle guidance may help improve the accuracy of needle placement and decrease the incidence of needle-related complications.

Complications and Pitfalls The proximity to the spinal cord and exiting nerve roots makes it imperative that this procedure be performed only by clinicians well versed in the regional anatomy and experienced in performing injection techniques. The proximity to the vertebral artery combined with the vascular nature of this anatomical region makes the potential for intravascular injection high. Even small amounts of a local anesthetic injected into the vertebral arteries result in seizures. Given the proximity of the brain and brainstem, ataxia after trigger point injection as a result of vascular uptake of local anesthetic is common. Many patients also complain of a transient increase in pain after injection in this anatomical area. If long needles are used, pneumothorax also may occur. Because of the proximity of the epidural, subdural, and subarachnoid space, placement of a needle too deeply could result in inadvertent neuraxial block. Failure to recognize inadvertent epidural, subdural, or dural puncture can result in significant motor and sensory block with the potential for associated loss of consciousness, hypotension, and apnea. If subdural placement is unrecognized, and the previously mentioned doses of local anesthetics are administered, the signs and symptoms are similar to those of subarachnoid injection, although the resulting motor and sensory block may be spotty.

Clinical Pearls The aforementioned injection technique is extremely effective in the treatment of cervicothoracic bursitis. This technique is a safe procedure if careful attention is paid to the clinically relevant anatomy in the areas to be injected. Care must be taken to use sterile technique to avoid infection and universal precautions to avoid risk to the operator. Most side effects of the injection technique for cervicothoracic bursitis are related to needle-induced trauma to the injection site and underlying tissues. The incidence of ecchymosis and hematoma formation can be decreased if pressure is placed on the injection site immediately after injection. The avoidance of overly long needles helps decrease the incidence of trauma to underlying structures. Special care must be taken to avoid pneumothorax given the proximity to the underlying pleural space.

The use of physical modalities, including local heat and gentle stretching exercises, should be introduced several days after the patient undergoes this injection technique for cervicothoracic bursitis. Vigorous exercises should be avoided because they would exacerbate the symptoms. Cognitive functional therapy may provide symptom relief in some patients. Simple analgesics, NSAIDs, and antimyotonic agents such as tizanidine may be used concurrently with this injection technique.

Suggested Readings Ferreira M.L, de Luca K. Spinal pain and its impact on older people. Best Pract Res Clin Rheumatol. 2017;31(2):192–202. Hull J.J, Tomaski S.M. Osteomyelitis of the cervical spine: case report and literature review. Otolaryngol Head Neck Surg. 1995;113:193. Meziat-Filho N, Lima M, Fernandez J, Reis F.J.J. Cognitive functional therapy (CFT) for chronic non-specific neck pain. J Bodyw Mov Ther. 2018;22(1):32– 36 Chronic pain. Perka C, Schneider S.V, Buttgereit F, Matziolis G. Development of cervical interspinous bursitis after prolonged sports trauma: a case report. Joint Bone Spine. 2006;73:118–120. Reckelhoff K.E, Green M.N, Kettner N.W. Cervical spine osteochondroma: rare presentation of a common lesion. J Manipulative Physiol Ther. 2010;33:711–715. Waldman S.D. Cervicothoracic interspinous bursitis. In: Waldman S.D, ed. Pain Review. Philadelphia: Saunders; 2009:238–239.

24

Scapulocostal Syndrome

Abstract Scapulocostal syndrome is a clinical syndrome characterized by pain and paresthesias over the medial border of the scapula that radiate into the neck, upper triceps, chest wall, and distal upper extremity. The pain is burning and aching. The intensity level of pain associated with scapulocostal syndrome is moderate. Also known as traveling salesman shoulder, the scapulocostal syndrome is thought to be an overuse syndrome resulting from repetitive use of the shoulder stabilizing muscles, including the serratus anterior, levator scapulae, pectoralis minor, and rhomboid, when carrying out activities such as reaching backward over a car seat for samples and prolonged use of the telephone cradled between the shoulder and neck. Racquet sports also have been implicated in the evolution of scapulocostal syndrome.

Keywords amitriptyline; Scapulocostal syndrome; sports injuries; traveling salaesman’s shoulder; trigger point; trigger point injection

ICD-10 CODE M75.80

The Clinical Syndrome Scapulocostal syndrome is a clinical syndrome characterized by pain and paresthesias over the medial border of the scapula that radiate into the neck, upper triceps, chest wall, and distal upper extremity. The pain is burning and aching. The intensity level of pain associated with scapulocostal syndrome is moderate. Also known as traveling salesman shoulder, the scapulocostal syndrome is thought to be an overuse syndrome resulting from repetitive use of the shoulder stabilizing muscles, including the serratus anterior, levator scapulae, pectoralis minor, and rhomboid, when carrying out activities such as reaching backward over a car seat for samples and prolonged use of the telephone cradled between the shoulder and neck (Fig. 24.1). Racquet sports also have been implicated in the evolution of scapulocostal syndrome.

Signs and Symptoms Physical examination reveals myofascial trigger points in the rhomboid, infraspinatus, and subscapularis muscles. These trigger points are best shown by having the patient reach across the chest and place his or her hand on the uninvolved shoulder. Palpation of trigger points along the medial border of the scapula produces a positive jump sign and causes pain to radiate into the ipsilateral upper extremity. The neurological examination of the upper extremity is normal in scapulocostal syndrome. Untreated, patients with scapulocostal syndrome develop decreased range of motion of the shoulder and scapula, resulting in functional disability and pain.

Testing Plain radiographs are indicated in all patients with scapulocostal syndrome. Based on the clinical presentation, additional tests, including complete blood cell count, erythrocyte sedimentation rate, and antinuclear antibody level, may be indicated. Magnetic resonance imaging (MRI) of the shoulder is indicated if rotator cuff tear is suspected. Radionuclide bone scanning is indicated if metastatic disease or primary tumor involving the shoulder is being considered. Chest radiographs with apical lordotic views should be obtained if superior sulcus tumor of the lung is a possibility. Electromyography and nerve conduction velocity testing help rule out radiculopathy, brachial plexopathy, and entrapment neuropathy.

Differential Diagnosis Scapulocostal syndrome is most commonly misdiagnosed as cervical radiculopathy. In contrast to cervical radiculopathy, however, which is associated with numbness and weakness in the affected dermatomes, the upper extremity neurological examination in scapulocostal syndrome is normal. Osteoarthritis, rheumatoid arthritis, posttraumatic arthritis, and rotator cuff tear arthropathy also are common causes of shoulder pain secondary to arthritis that may be confused with scapulocostal syndrome. Less common causes of arthritis-induced shoulder pain include the collagenvascular diseases, infection, villonodular synovitis, and Lyme disease. Acute infectious arthritis usually is accompanied by significant systemic symptoms, including fever and malaise, and should be easily recognized by an astute clinician and treated appropriately with culture and antibiotics, rather than injection therapy. The collagen-vascular diseases generally manifest as a polyarthropathy rather than a monarthropathy limited to the shoulder joint, and the pain does not radiate into the upper extremity. Pancoast tumor and brachial plexopathy also may mimic the clinical presentation of scapulocostal syndrome.

FIG. 24.1 Scapulocostal syndrome is due to repetitive use of the shoulder stabilizing muscles.

Treatment Initial treatment of the pain and functional disability associated with scapulocostal syndrome should include a combination of nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and physical therapy. The local application of heat and cold as well as the use of massage and scapular stabilization exercises also may be beneficial. Repetitive movements that incite the syndrome should be avoided. For patients who do not respond to these treatment modalities, injection of myofascial trigger points with local anesthetic and steroid may be a reasonable next step.

Complications and Pitfalls The major complication in the care of a patient thought to have scapulocostal syndrome is misdiagnosis. Tumors of the superior sulcus of the lung or primary or metastatic tumors of the shoulder and scapula must be included in the differential diagnosis.

Clinical Pearls Scapulocostal syndrome is a less common cause of shoulder and upper extremity pain encountered in clinical practice, with cervical radiculopathy occurring much more commonly. This painful condition must be separated from other causes of shoulder pain, including rotator cuff tears. Coexistent bursitis and tendinitis also may contribute to shoulder pain and may require additional treatment with more localized injection of local anesthetic and depot steroid. Trigger point injections are a safe procedure if careful attention is paid to the clinically relevant anatomy in the areas to be injected. Care must be taken to use sterile technique to avoid infection and universal precautions to avoid risk to the operator. The incidence of ecchymosis and hematoma formation can be decreased if pressure is placed on the injection site immediately after injection. The use of physical modalities, including local heat and gentle range-of-motion exercises, should be introduced several days after the patient undergoes trigger point injections for scapulocostal syndrome. Avoidance of activities responsible for the evolution of the disease must be considered or the syndrome will recur. Vigorous exercises should be avoided because they would exacerbate symptoms. Simple analgesics and NSAIDs or a COX-2 inhibitor may be used concurrently with an injection technique.

Suggested Readings Bradley L.A. Pathophysiology of fibromyalgia. Am J Med. 2009;122(suppl 1):S22–S30. Buttagat V, Eungpinichpong W, Kaber D, et al. Acute effects of traditional Thai massage on electroencephalogram in patients with scapulocostal syndrome. Complement Ther Med. 2012;20(4):167–174. Buttagat V, Taepa N, Suwannived N, Rattanachan N. Effects of scapular stabilization exercise on pain related parameters in patients with scapulocostal syndrome. A randomized controlled trial. J Bodyw Mov Ther. 2016;20(1):115–122. Dommerholt J, Hooks T, Chou L.-W, Finnegan M. A critical overview of the current myofascial pain literature – January 2018. J Bodyw Mov Ther. 2018;22(1):184–191. Ge H.U, Nie H, Madeleine P. Contribution of the local and referred pain from active myofascial trigger points in fibromyalgia syndrome. Pain. 2009;147:233–240. Monach P.A. Shoulder pain. In: Mushlin S.B, Greene II. H.L, eds. Decision Making in Medicine. 3rd ed. New York: Elsevier; 2010:522–523. Waldman SD. Scapulocostal syndrome. In: Waldman SD. ed. Atlas of Pain Management Injection Techniques. 2nd ed. Philadelphia: Saunders; 123–126.

25

Parsonage-Turner Syndrome Keywords brachial plexopathy; brachial plexus; brachial plexus block; Pancost tumor; ultrasound guided nerve block; Parsonage-Turner syndrome

ICD-10 CODE G54.0

The Clinical Syndrome Parsonage and Turner described the painful condition of the shoulder and upper extremity that bears their name and was first identified as a distinct clinical entity in 1948. It is also known by a number of other names, including Kiloh-Nevin syndrome, idiopathic brachial plexopathy, and paralytic brachial neuritis. The pain of Parsonage-Turner syndrome is of acute onset and is severe in intensity. The pain is burning and involves the shoulder and upper arm, preceding the onset of muscle weakness by hours to days (Fig. 25.1). Sleep disturbance is common, and weakness of the muscles of the shoulder and upper extremity, including the deltoid, infraspinatus, supraspinatus, and biceps, occurs as the syndrome progresses. In some patients, this weakness can be severe, progressing to complete flaccidity. A viral cause of ParsonageTurner syndrome has been suggested, as has the belief that this painful condition is an immunological disease. Neither theory has been proven, although two patients on anti–programmed cell death-1 checkpoint inhibitor therapies developed the syndrome.

Signs and Symptoms Patients with Parsonage-Turner syndrome first experience a sudden onset of pain that begins in the shoulder and radiates down the arm. The pain is severe and is followed by the development of weakness. The skin examination is normal, with no evidence of acute herpes zoster. Range of motion of the cervical spine generally does not affect the pain or numbness, in contrast to cervical radiculopathy. Weakness of the muscles of the shoulder and upper extremity, including the deltoid, infraspinatus, supraspinatus, and biceps, increases as the syndrome progresses. Scapular winging may be present (Fig. 25.2). Flaccidity of these muscles may occur. Usually, more than one portion of the brachial plexus is affected, although isolated single-nerve involvement can occur.

Differential Diagnosis Brachial plexopathy has many causes. In common to all of them is the constellation of symptoms consisting of neurogenic pain and associated weakness that radiates into the supraclavicular region and upper extremity. More common causes of brachial plexopathy include compression of the plexus by cervical ribs or abnormal muscles (e.g., thoracic outlet syndrome), invasion of the plexus by tumor (e.g., Pancoast syndrome), direct trauma to the plexus (e.g., stretch injuries and avulsions), inflammatory causes (e.g., Parsonage-Turner syndrome), and postradiation plexopathy. Cervical radiculopathy is a much more common cause of upper extremity pain and weakness relative to Parsonage-Turner syndrome. Table 25.1 differentiates these two painful conditions. In patients in whom Parsonage-Turner syndrome affects only an isolated nerve, the syndrome may be misdiagnosed as entrapment neuropathy. Electromyography is the cornerstone in sorting out the differential diagnosis in patients with the acute onset of shoulder and upper extremity pain. Diseases of the cervical spinal cord, bony cervical spine, and disc can mimic Parsonage-Turner syndrome. Appropriate testing, including magnetic resonance imaging (MRI) and electromyography, helps sort out the myriad possibilities, but the clinician also should be aware that more than one pathological process may coexist and contribute to the patient’s symptoms. Syringomyelia, tumors of the cervical spinal cord, and tumors of the cervical nerve roots as they exit the spinal cord, such as schwannomas, can be of insidious onset and quite difficult to diagnose. Pancoast tumor should be high on the list of diagnostic possibilities in all patients with brachial plexopathy in the absence of clear antecedent trauma, especially in the presence of a history of tobacco abuse. Lateral herniated cervical disc, metastatic tumor, or cervical spondylosis that results in significant nerve root compression also may manifest as a brachial plexopathy. Rarely, infection involving the apex of the lung may compress and irritate the plexus.

Testing All patients presenting with Parsonage-Turner syndrome must undergo MRI of the cervical spine and the brachial plexus (Figs. 25.2 and 25.3). Computed tomography (CT) is a reasonable second choice if MRI is contraindicated. Electromyography and nerve conduction velocity testing are extremely sensitive, and a skilled electromyographer can help delineate the specific portion of the plexus that is abnormal. If an inflammatory basis for the plexopathy is suspected, serial electromyography is indicated. If Pancoast tumor or other tumors of the brachial plexus are suspected, chest radiographs with apical lordotic views may be helpful.

FIG. 25.1 The pain of Parsonage-Turner syndrome involves the shoulder and upper arm, preceding the onset of muscle weakness by hours to days.

Screening laboratory tests consisting of complete blood cell count, erythrocyte sedimentation rate, antinuclear antibody testing, and automated blood chemistry testing should be performed if the diagnosis of brachial plexopathy is in question, to help rule out other causes of pain.

Treatment Pharmacological Therapy Gabapentin Gabapentin is the first-line treatment for the neuritic pain of ParsonageTurner syndrome. The drug is started with a 300-mg dose at bedtime for 2 nights; the patient should be cautioned about potential side effects, including dizziness, sedation, confusion, and rash. The drug is increased in 300-mg increments, given in equally divided doses over 2 days, as side effects allow, until pain relief is obtained or a total dose of 2400 mg per day is reached. At this point, if the patient has experienced partial pain relief, blood values are measured, and the drug is carefully titrated upward using 100-mg tablets. More than 3600 mg daily rarely is required.

Carbamazepine Carbamazepine is useful in patients with Parsonage-Turner syndrome who do not experience pain relief with gabapentin. Despite the safety and efficacy of carbamazepine compared with other treatments for Parsonage-Turner syndrome, much confusion and unfounded anxiety surround its use. This medication, which may be the best chance for pain control, is sometimes discontinued because of laboratory abnormalities erroneously attributed to it. Baseline screening laboratory tests, consisting of a complete blood cell count, urinalysis, and automated chemistry profile, should be obtained before starting the drug.

FIG. 25.2 Parsonage-Turner Syndrome in a 19-Year-Old Male Sailing Teacher, With

a 3-Week History of Right PneumopathyHe presented with right shoulder pain, severe muscle weakness, and a winging scapula less than 2 weeks previously. The dorsal scapular nerve and the accessory nerve were affected but the suprascapular nerve was preserved. (A) Photography of the scapular winging. (B) Sagittal FSE T2 weighted image demonstrates muscle edema of the inferior part of the rhomboids major (arrow). (C and D) Axial IDEAL sequence with water only images highlights edema in the trapezius (arrow head) and rhomboids minor and major muscles (arrows), which was hardly depicted with the previous sequence. From Blum A, Lecocq S, Louis M, et al. The nerves around the shoulder. Eur J Radiol. 2013;82[1]:2–16, fig 18.

TABLE 25.1 Comparison of Parsonage-Turner Syndrome and Cervical Radiculopathy

FIG. 25.3 Pancoast Tumor in a 46-Year-Old ManSagittal T1-weighted (A) and sagittal T1-weighted gadolinium-enhanced with fat saturation (B) sequences show left apical bronchogenic carcinoma (white arrow) invading the supraclavicular fossa, involving the brachial plexus, and encasing the subclavian artery (black arrow). From Knisely BL, Broderick LS, Kuhlman JE. MR imaging of the pleura and chest wall. Magn Reson Imaging Clin N Am. 2000;8:125.

Carbamazepine should be started slowly if the pain is not out of control. The drug is started with a 100- to 200-mg dose at bedtime for 2 nights; the patient should be cautioned regarding side effects, including dizziness, sedation, confusion, and rash. The drug is increased in 100- to 200-mg increments, given in equally divided doses over 2 days, as side effects allow, until pain relief is obtained or a total dose of 1200 mg daily is reached. Careful monitoring of laboratory parameters is mandatory to avoid the rare possibility of life-threatening blood dyscrasia. At the first sign of blood count abnormality or rash, carbamazepine should be discontinued. Failure to monitor patients started on carbamazepine can be disastrous because aplastic anemia can occur. When pain relief is obtained, the patient should be kept at that dosage of carbamazepine for at least 6 months before considering tapering of this medication. The patient should be informed that

under no circumstances should the dosage of drug be changed or the drug refilled or discontinued without the physician’s knowledge.

Baclofen Baclofen has been reported to be valuable in some patients who fail to obtain relief from gabapentin and carbamazepine. Baseline laboratory tests should be obtained before starting baclofen. The drug is started with a 10-mg dose at bedtime for 2 nights; the patient should be cautioned about potential adverse effects, which are the same as those of carbamazepine and gabapentin. Baclofen is increased in 10-mg increments, given in equally divided doses over 7 days, as side effects allow, until pain relief is obtained or a total dose of 80 mg per day is reached. This drug has significant hepatic and central nervous system side effects, including weakness and sedation. As with carbamazepine, careful monitoring of laboratory values is indicated during the initial use of this drug. When treating patients with any of the drugs mentioned, the physician should inform the patient that premature tapering or discontinuation of the medication may lead to the recurrence of pain. The pain becomes more difficult to control thereafter.

FIG. 25.4 Ultrasound Image of the Trunks of the Brachial Plexus (TBP)Note the relationship of the vertebral artery to the trunks. TVP, Transverse vertebral process. Image property of Steven D. Waldman, MD.

Interventional Treatment Brachial Plexus Block The use of brachial plexus block with a local anesthetic and steroid is an excellent adjunct to drug treatment of Parsonage-Turner syndrome. This technique rapidly relieves pain while medications are being titrated to effective levels. The initial block is performed with preservative-free bupivacaine combined with methylprednisolone. Subsequent daily nerve blocks are done in a similar manner, substituting a lower dose of methylprednisolone. This approach also may be used to obtain control of breakthrough pain. Ultrasound guidance may improve the accuracy of needle placement and decrease the incidence of needle-related complications (Fig. 25.4).

Physical Modalities The use of physical and occupational therapy to maintain function and help

palliate pain is a crucial part of the treatment plan for patients with Parsonage-Turner syndrome. Shoulder abnormalities, including subluxation and adhesive capsulitis, must be aggressively searched for and treated. Occupational therapy to assist in activities of daily living also is important to avoid further deterioration of function.

Complications and Pitfalls The pain of Parsonage-Turner syndrome is difficult to treat. It responds poorly to opioid analgesics and may respond poorly to the previously mentioned medications. The uncontrolled pain of Parsonage-Turner syndrome has led to suicide, and hospitalization of such patients should be strongly considered. Correct diagnosis is crucial to successfully treat the pain and dysfunction associated with brachial plexopathy because stretch injuries and contusions of the plexus may respond with time, but plexopathy secondary to tumor or avulsion of the cervical roots requires aggressive treatment.

Clinical Pearls Brachial plexus block with a local anesthetic and steroid represents an excellent stop-gap measure for patients with the uncontrolled pain of Parsonage-Turner syndrome while waiting for pharmacological treatments to take effect. As mentioned, correct diagnosis is paramount to allow the clinician to design a logical treatment plan.

Suggested Readings Alhammad R.M, Dronca R.S, Kottschade L.A, et al. Brachial plexus neuritis associated with anti–programmed cell death-1 antibodies: report of 2 cases. Mayo Clin Proc Innov Qual Outcomes. 2017;1(2):192–197. Blum A, Lecocq S, Louis M, et al. The nerves around the shoulder. Eur J Radiol. 2013;82(1):2–16. Marshall G.B, McKenna E, Mahallati H. Parsonage–Turner syndrome. Eur J Radiol Extra. 2005;6:51–53. Mileto A, Gaeta M. Calcific tendonitis of supraspinatus simulating acute brachial neuritis (Parsonage-Turner syndrome). Clin Radiol. 2011;66:578– 581. Smith C.C, Bevelaqua A.C. Challenging pain syndromes: Parsonage-Turner syndrome. Phy MedRehabilitation Clin Nor Am. 2014;25(2):265–277. Stutz C.M. Neuralgic amyotrophy: Parsonage-Turner syndrome. J Hand Surg. 2010;35:2104–2106. Wendling D, Sevrin P, Bouchaud-Chabot A, et al. Parsonage–Turner syndrome revealing Lyme borreliosis. Joint Bone Spine. 2009;76:202–204.

26

Hyoid Syndrome

Abstract Hyoid syndrome is caused by calcification and inflammation of the attachment of the stylohyoid ligament to the hyoid bone. The stylohyoid ligament’s cephalad attachment is to the styloid process, and its caudal attachment is to the hyoid bone. Tendinitis of the other muscular attachments to the hyoid bone also may contribute to this painful condition. Hyoid syndrome also may be seen in conjunction with Eagle’s syndrome. The pain of hyoid syndrome is sharp and stabbing and occurs with movement of the mandible, turning of the neck, or swallowing.

Keywords anterior neck pain; dysphagia; Eagle’s syndrome; hyoid muscle dystonia; Hyoid syndrome; otalgia

ICD-10 CODE M62.89

The Clinical Syndrome Hyoid syndrome is caused by calcification and inflammation of the attachment of the stylohyoid ligament to the hyoid bone. The stylohyoid ligament’s cephalad attachment is to the styloid process, and its caudal attachment is to the hyoid bone. Tendinitis of the other muscular attachments to the hyoid bone also may contribute to this painful condition. Hyoid syndrome also may be seen in conjunction with Eagle syndrome (see Chapter 15). The pain of hyoid syndrome is sharp and stabbing and occurs with movement of the mandible, turning of the neck, or swallowing.

Signs and Symptoms The pain of hyoid syndrome starts below the angle of the mandible and radiates into the anterolateral neck. It is triggered or worsened with chewing, rotation of the cervical spine, and swallowing (Fig. 25.1). The pain of hyoid syndrome is sharp and stabbing and often is referred to the ipsilateral ear. Some patients also may complain of a foreign body sensation in the pharynx. Injection of the attachment of the stylohyoid ligament to the greater cornu of hyoid bone with local anesthetic and steroid serves as a diagnostic and therapeutic maneuver.

Testing A 22-gauge, 1 1⁄2-inch needle is attached to a 10 mL syringe. At a point 1 inferior to the angle of the mandible in a plane perpendicular to the skin, the needle is advanced toward the hyoid bone. Injection of the ligament with local anesthetic can serve as a diagnostic maneuver to help strengthen the diagnosis.

Differential Diagnosis Soft tissue injuries to the region may mimic styloid syndrome. Because trauma is invariably involved in the evolution of the painful condition, the strain and sprain of other soft tissues, such as omohyoid syndrome, often exist concurrently with hyoid syndrome (see Chapter 27). Hyoid muscle dystonia may also mimic the signs and symptoms of hyoid syndrome. Primary or metastatic tumors of the neck and hypopharynx and mass effect from thyroglossal duct cyst also may mimic the clinical presentation of hyoid syndrome and should be high on the list of diagnostic possibilities if the history of trauma is weak or absent. Although clinically similar, glossopharyngeal neuralgia can be distinguished from hyoid syndrome in that the pain of glossopharyngeal neuralgia is characterized by paroxysms of shock-like pain in a manner analogous to trigeminal neuralgia, rather than the sharp, shooting pain that occurs on movement associated with hyoid syndrome. Because glossopharyngeal neuralgia may be associated with serious cardiac bradyarrhythmias and syncope, the clinician must distinguish the two syndromes.

Treatment Nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors represent a reasonable first step in the treatment of hyoid syndrome. The use of tricyclic antidepressants, such as nortriptyline, at a single bedtime dose of 25 mg titrating as side effects allow also is useful, especially if sleep disturbance is present. If symptoms persist, injection of the caudad attachment of the stylohyoid ligament is a reasonable next step. To perform this injection, the patient is placed in the supine position. The angle of the mandible on the affected side is identified. The greater cornu of the hyoid bone should lie approximately 1 inch inferior to the angle of the mandible. Gentle pressure at the same point on the contralateral side of the neck steadies the hyoid bone and makes identification of the greater cornu and subsequent injection easier (Fig. 26.2). The skin is prepared with antiseptic solution. A 22-gauge, 1½-inch needle attached to a 10-mL syringe is advanced at this point 1 inch inferior to the angle of the mandible in a plane perpendicular to the skin. The greater cornu of the hyoid bone should be encountered within 2.5 to 3 cm (Fig. 26.3). After contact is made, the needle is withdrawn slightly out of the periosteum or substance of the calcified ligament. After careful aspiration reveals no blood or cerebrospinal fluid, 5 mL of 0.5% preservative-free lidocaine combined with 80 mg of methylprednisolone is injected in incremental doses. Subsequent daily nerve blocks are done in a similar manner, substituting 40 mg of methylprednisolone for the initial 80-mg dose. Ultrasound and color Doppler guidance may improve the accuracy of needle placement and decrease the incidence of needle-related complications (see Figs. 26.3 and 26.4).

FIG. 26.1 The pain of hyoid syndrome starts below the angle of the mandible and radiates into the anterolateral neck. It is triggered or worsened with chewing, rotation of the cervical spine, or swallowing.

FIG. 26.2 Identification of the Greater Cornu of the Hyoid Bone. From Waldman SD. Atlas of Pain Management Injection Techniques. 2nd ed. Philadelphia: Saunders; 2007:18.

FIG. 26.3 Injection Technique for Relieving the Pain of Hyoid Syndrome. From Waldman SD. Atlas of Pain Management Injection Techniques. 2nd ed. Philadelphia: Saunders; 2007:17.

FIG. 26.4 Color Doppler image demonstrating the relationship of the hyoid bone to the internal carotid artery.

Complications and Pitfalls The major complication in the treatment of patients thought to have hyoid syndrome is wrong diagnosis. Occult cervical spine fracture or instability after trauma remains an ever-present possibility. Failure to diagnose such injuries can put the patient at significant risk for permanent neurological sequelae. As mentioned earlier, if the patient is thought to have a history of trauma, the diagnosis of hyoid syndrome should become one of exclusion. A careful search for tumors of the neck, apex of the lung, anterior triangle of the neck, and hypopharynx is indicated. If a significant history of vomiting is ascertained, esophageal tear should be considered. Although the injection technique for hyoid syndrome is safe, complications can occur. In addition to the potential for complications involving the vasculature, if the needle is placed too laterally, the proximity of the brachial plexus, the central neuraxial structures, and the phrenic nerve can result in side effects and complications. Although these complications should be rare if proper technique is observed, the potential for inadvertent epidural, subdural, or subarachnoid injection remains. Phrenic nerve block also can occur when using this injection technique to treat hyoid syndrome if the needle placement is too posterolateral. In the absence of significant pulmonary disease, unilateral phrenic nerve block should rarely create respiratory embarrassment. Blockade of the recurrent laryngeal nerve with its attendant vocal cord paralysis combined with paralysis of the diaphragm may make the clearing of pulmonary and upper airway secretions difficult. Because of the proximity of the apex of the lung, pneumothorax is a distinct possibility, and the patient should be informed of this.

Clinical Pearls The clinician should always evaluate a patient who has pain in this anatomical region for occult malignancy. Tumors of the larynx, hypopharynx, and anterior triangle of the neck may manifest clinical symptoms identical to those of hyoid syndrome. Given the low incidence of hyoid syndrome relative to pain secondary to malignancy in this anatomical region, hyoid syndrome must be considered a diagnosis of exclusion. The injection technique described for hyoid syndrome is a simple technique that can produce dramatic relief for patients with the previously

mentioned pain problems. As discussed earlier, the proximity of the greater cornu of the hyoid bone to major vasculature makes postblock hematoma and ecchymosis a distinct possibility. Although these complications are usually transitory, their dramatic appearance can be quite upsetting to the patient; therefore the patient should be warned of this possibility before the procedure. The vascularity of this region also increases the incidence of inadvertent intravascular injection. Even small amounts of local anesthetic injected into the carotid artery at this level can result in local anesthetic toxicity and seizures. Incremental dosing while carefully monitoring the patient for signs of local anesthetic toxicity helps avoid this complication.

Suggested Readings Auvenshine R.C. Anatomy of the airway: an overview. Sleep Med Clin. 2010;5:45–57. Ernest III. E.A, Salter E.G. Hyoid bone syndrome: a degenerative injury of the middle pharyngeal constrictor muscle with photomicroscopic evidence of insertion tendinosis. J Prosthet Dentist. 1991;66:78–83. Nir D, Hefer T, Joachims H.Z. Hyoid bone syndrome and its treatment with nonsteroidal anti-inflammatory drugs. Am J Otolaryngol. 1998;19:296–300. Norby E, Orbelo D, Strand E, et al. Hyoid muscle dystonia: a distinct focal dystonia syndrome. Parkinsonism Relat Disord. 2015;21(10):1210–1213 ISSN 1353– 8020. https://doi.org/10.1016/j.parkreldis.2015.08.022. http://www.sciencedirect.com Waldman S.D. Hyoid syndrome. In:. Atlas Of Pain Management Injection Techniques. 2nd ed. Philadelphia: Saunders; 2007:16–19.

27

Omohyoid Syndrome

Abstract Patients suffering from omohyoid syndrome presents with pain in the supraclavicular region at a point just lateral and superior to the attachment of the sternocleidomastoid muscle to the clavicle. The pain often radiates into the anterolateral neck and increases with movement of the omohyoid muscle. A baseline level of pain is present even without movement of the muscle. The pain intensity ranges from minor to moderate. A trigger point in the belly of the omohyoid muscle is often present. The pain of omohyoid syndrome is often exacerbated by swallowing. The neurological examination of a patient with omohyoid syndrome is normal, unless trauma has occurred to the cervical nerve roots or brachial plexus.

Keywords brachial plexopathy; dysphagia; neck pain; Omohyoid syndrome; phrenic nerve; pneumothorax; recurrent laryngeal nerve; trigger point injection; trigger point; ultrasound guided injection

ICD-10 CODE M79.7

The Clinical Syndrome Trauma is the common denominator in patients with omohyoid syndrome. The syndrome is most often seen in patients who have recently experienced a bout of intense vomiting or sustained a flexion/extension injury to the cervical spine and the musculature of the anterior neck. The pain of omohyoid syndrome is the result of damage to the fibers of the inferior belly of the omohyoid muscle. This pain manifests as myofascial. It is constant and exacerbated with movement of the affected muscle. A trigger point in the inferior belly of the omohyoid muscle is often present and provides a basis for treatment. The pain of omohyoid syndrome starts just above the clavicle at the lateral aspect of the clavicular attachment of the sternocleidomastoid muscle. The pain may radiate into the anterolateral neck. Injection of the trigger point in the inferior muscle of the omohyoid muscle with local anesthetic and steroid serves as a diagnostic and therapeutic maneuver.

Signs and Symptoms Patients suffering from omohyoid syndrome present with pain in the supraclavicular region at a point just lateral and superior to the attachment of the sternocleidomastoid muscle to the clavicle (Fig. 27.1). The pain often radiates into the anterolateral neck and increases with movement of the omohyoid muscle. A baseline level of pain is present even without movement of the muscle. The pain intensity ranges from minor to moderate. A trigger point in the belly of the omohyoid muscle is often present. The pain of omohyoid syndrome is often exacerbated by swallowing. The neurological examination of a patient with omohyoid syndrome is normal, unless trauma has occurred to the cervical nerve roots or brachial plexus.

Testing Magnetic resonance imaging (MRI) and/or ultrasound imaging of the soft tissues of the neck may reveal hematoma formation of the omohyoid muscle acutely and calcification, fibrosis, or both as the syndrome becomes more chronic. Injection of the belly of the omohyoid muscle with local anesthetic can serve as a diagnostic maneuver to help strengthen the diagnosis.

Differential Diagnosis Soft tissue injuries to the region may mimic omohyoid syndrome. Because trauma is invariably involved in the evolution of the painful condition, strain and sprain of other soft tissues often exist concurrently with omohyoid syndrome. Sternohyoid syndrome as well as primary or metastatic tumors of the neck and hypopharynx also may mimic the clinical presentation of omohyoid syndrome and should be high on the list of diagnostic possibilities if the history of trauma is weak or absent.

Treatment Nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors represent a reasonable first step in the treatment of omohyoid syndrome. The use of tricyclic antidepressants, such as nortriptyline, at a single bedtime dose of 25 mg, titrating upward as side effects allow also is helpful, especially if sleep disturbance is present. The injection of trigger points in the inferior belly of the omohyoid muscle often produces dramatic improvement in pain symptoms.

FIG. 27.1 The pain of omohyoid syndrome is localized in the supraclavicular region at a point just lateral and superior to the attachment of the sternocleidomastoid muscle to the clavicle.

FIG. 27.2 Injection Site for the Treatment of Omohyoid Syndrome.

The key landmark for injecting when treating omohyoid syndrome is the lateral aspect of the clavicular head of the sternocleidomastoid muscle (Fig. 27.2). The omohyoid muscle is located slightly lateral and deep to the clavicular head of the sternocleidomastoid muscle approximately ¾ to 1 inch above the superior margin of the clavicle. Given the relationship of the great vessels of the neck to the omohyoid muscle, care must be taken when placing needles in this anatomical area. The patient is placed in the supine position, with the head turned away from the side to be blocked. Using a 5-mL sterile syringe, 3 mL of local anesthetic is drawn up. When treating omohyoid syndrome, 80 mg of depot steroid is added to the local anesthetic with the first block, and 40 mg of depot steroid is added with subsequent blocks. The patient is asked to raise the head against the resistance of the pain specialist’s hand to aid in identification of the posterior border of the sternocleidomastoid muscle. The point at which the lateral border of the sternocleidomastoid attaches to the clavicle is identified. At this point, slightly lateral and approximately 1 inch

above the clavicle, after preparation of the skin with antiseptic solution, a 1½inch needle is inserted directly perpendicular to the table top (see Fig. 27.2). The needle should be advanced slowly because of proximity of the great vessels and brachial plexus. A “pop” often is felt as the fascia of the omohyoid muscle is pierced; this should occur at a depth of ½ to ¾ of an inch. If strict attention to technique is observed, and the needle is not placed or directed too laterally, the brachial plexus should not be encountered. Because of the proximity of the brachial plexus, the patient should be warned that a paresthesia could occur; the patient should be instructed to say “There!” if a paresthesia is felt. The needle should never be directed in a more inferior medial trajectory because pneumothorax is likely to occur. After the muscle is identified, gentle aspiration is done to identify blood or cerebrospinal fluid. If the aspiration test is negative, and no paresthesia into the distribution of the brachial plexus is encountered, 3 mL of solution is slowly injected, with the patient being monitored closely for signs of local anesthetic toxicity or inadvertent neuraxial injection. This technique can also be utilized for injection of botulinum toxin into the omohyoid muscle. Ultrasound guidance may improve the accuracy of needle placement and decrease the incidence of needle-related complications.

Complications and Pitfalls The major complication in the treatment of patients thought to have omohyoid syndrome is wrong diagnosis. Occult cervical spine fractures or instability after trauma remain an ever-present possibility. Failure to diagnose such injuries can put the patient at significant risk for permanent neurological sequelae. As mentioned earlier, if the history of trauma is suspect, the diagnosis of omohyoid syndrome should become one of exclusion. A careful search for tumors of the neck, apex of the lung, anterior triangle of the neck, and hypopharynx is indicated. If a significant history of vomiting is ascertained, esophageal tear also should be considered. Although the injection technique for omohyoid syndrome is safe, complications can occur. In addition to the potential for complications involving the vasculature, if the needle is placed too laterally, the proximity of the brachial plexus, the central neuraxial structures, and the phrenic nerve can result in side effects and complications. Although these complications should be rare if proper technique is observed, the potential for inadvertent epidural, subdural, or subarachnoid injection remains a possibility. Inadvertent phrenic nerve block also can occur when using this injection technique to treat omohyoid syndrome if the needle placement is too far posterolaterally. In the absence of significant pulmonary disease, unilateral phrenic nerve block should rarely create respiratory embarrassment. Inadvertent blockade of the recurrent laryngeal nerve with its attendant vocal cord paralysis combined with paralysis of the diaphragm may make the clearing of pulmonary and upper airway secretions difficult, however. Because of the proximity of the apex of the lung, pneumothorax is a distinct possibility and the patient should be informed of this.

Clinical Pearls Although an uncommon cause of pain, omohyoid syndrome is a clinically distinct and easily recognizable pain syndrome. Because of its excellent response to the injection technique described, the diagnosis of omohyoid syndrome should be considered in the presence of a history of trauma or after prolonged or forceful vomiting. If the patient has severe, acute pain after vomiting, esophageal tear is a more likely diagnosis. More chronic pain after a significant episode of vomiting is more likely to indicate omohyoid

syndrome. The key to performing this injection technique safely is a clear understanding of the anatomy and careful identification of the anatomical landmarks necessary to perform the block. The brachial plexus is quite superficial at the level at which this block is performed. The needle should rarely be inserted deeper than ¾ of an inch in all but the most obese patients. If strict adherence to technique is observed, and the needle is never advanced medially from the lateral border of the insertion of the sternocleidomastoid muscle on the clavicle, the incidence of pneumothorax should be less than 0.5%. In the absence of well-documented trauma to the anterior neck, omohyoid syndrome is a diagnosis of exclusion. The clinician should always evaluate a patient with pain in this anatomical region for occult malignancy. Tumors of the larynx, hypopharynx, and anterior triangle of the neck may manifest with clinical symptoms identical to omohyoid syndrome. In the setting of flexion/extension injuries or other forceful trauma to the soft tissues of the neck, cervical spine, or both, the clinician also should evaluate the patient for trauma to the brachial plexus by careful physical examination and electromyography.

Suggested Readings Bradley L.A. Pathophysiology of fibromyalgia. Am J Med. 2009;122(Suppl 1):S22–S30. Ge H.U, Nie H, Madeleine P, et al. Contribution of the local and referred pain from active myofascial trigger points in fibromyalgia syndrome. Pain. 2009;147:233–240. Guo-Hua W, Xiao-Ling J, Rong W, et al. Doubled omohyoid muscle in human: a case report and literature review. Clin Anat. 2009;22:868–870. Kim L, Kwon H, Pyun S.B. Pseudodysphagia due to omohyoid muscle syndrome. Dysphagia. 2009;24:357–361. Kim J.S, Hong K.H, Hong Y.T, Han B.H. Sternohyoid muscle syndrome. Am J Otolaryngol. 2015;36(2):190–194. Lapegue F, Faruch-Bilfeld M, Demondion X, et al. Ultrasonography of the brachial plexus, normal appearance and practical applications. Diag Intervent Imaging. 2014;95(3):259–275. Su P.H, Wang T.G, Wang Y.C. Ultrasound-guided injection of botulinum toxin in a patient with omohyoid muscle syndrome: a case report. J Clin Ultrasound. 2013;41:373–376. Waldman S.D. Omohyoid syndrome. In: Waldman S.D, ed. Atlas of Pain Management Injection Techniques. ed 4. Philadelphia: Saunders; 2017:29–31. Wong D.S.Y, Li H.J.C. The omohyoid sling syndrome. Am J Otolaryngol. 2000;21:318–322.

28

Acute Calcific Prevertebral Tendinitis

Abstract The tendons of the longus colli, longus capitis, anterior rectus capitis, and rectus muscles lie in the anterior cervical space and are prone to the development of tendinitis. Tendinitis of these muscles is usually caused either by repetitive trauma to the musculotendinous apparatus or by the deposition of calcium hydroxyapatite crystals. This crystal deposition usually occurs in the superior fibers of the musculotendinous apparatus and is easily identified on a lateral plain radiograph of the neck. The onset of calcific prevertebral tendinitis is generally acute, and it is often misdiagnosed as acute pharyngitis or retropharyngeal abscess because the acute onset of retropharyngeal pain is frequently accompanied by a mild elevation in temperature and leukocytosis. The longus colli muscle is most often affected. Calcific prevertebral tendinitis is most often seen in the third to sixth decades of life.

Keywords acute calcific prevertebral tendinitis; acute calcific retropharyngeal tendinitis; calcium hydroxyapatite; calcific tendonitis; crowned dens syndrome; discitis; dysphagia; odynophagia; pharyngitis; retropharyngeal abscess; ultrasound guided injection

ICD-10 CODE M65.20

The Clinical Syndrome The tendons of the longus colli, longus capitis, anterior rectus capitis, and rectus muscles lie in the anterior cervical space and are prone to the development of tendinitis. Tendinitis of these muscles is usually caused either by repetitive trauma to the musculotendinous apparatus or by the deposition of calcium hydroxyapatite crystals. This crystal deposition usually occurs in the superior fibers of the musculotendinous apparatus and is easily identified on a lateral plain radiograph of the neck. The onset of calcific prevertebral tendinitis is generally acute, and it is often misdiagnosed as acute pharyngitis or retropharyngeal abscess because the acute onset of retropharyngeal pain is frequently accompanied by a mild elevation in temperature and leukocytosis. The longus colli muscle is most often affected. Calcific prevertebral tendinitis is most often seen in the third to sixth decades of life.

Signs and Symptoms The pain of calcific prevertebral tendinitis is constant and severe and is localized to the retropharyngeal area. It is made worse by swallowing (Fig. 28.1). The patient may complain of acute anterior neck pain in addition to the pain on swallowing. Referred pain from the inflamed muscles into the anterior and posterior neck often occurs. A mild fever is often present, as is mild leukocytosis. Intraoral palpation of the superior attachment of the muscles usually reproduces the symptoms. Thickening of the affected muscles may also be appreciated.

FIG. 28.1 The pain of acute calcific prevertebral tendinitis is constant and severe and is localized to the retropharyngeal area. It is made worse by swallowing. From Waldman SD. Waldman’s Comprehensive Atlas of Diagnostic Ultrasound of Painful Conditions. Philadelphia: Wolters Kluwer; 2016:215–221.

FIG. 28.2 This image shows the contrast neck computed tomography scan, revealing globular calcific density to the right of midline, anterior to the vertebral body of the longus colli tendon (black arrow). From Kanzaria H, Stein JC. A severe sore throat in a middle-aged man: calcific tendonitis of the longus colli tendon. J Emerg Med. 2011;41[2]:151–153, fig 1.

Testing Plain radiographs are indicated for all patients who present with retropharyngeal pain. Characteristic amorphous calcification of the superior attachment of the musculotendinous unit just below the anterior arch of atlas is highly suggestive of calcific prevertebral tendinitis. Computed tomographic scanning, magnetic resonance imaging, and/or ultrasound imaging may further delineate the problem (Figs. 28.2–28.4). The finding of a smooth, linear prevertebral fluid collection is considered pathognomonic for this disease (Fig. 28.5). Unlike in a retropharyngeal or prevertebral abscess, the wall of the fluid-containing structure does not enhance with contrast administration. Additional testing may be indicated, including a complete blood count, erythrocyte sedimentation rate, and complete blood chemistry tests, in patients suspected of suffering from calcific prevertebral tendinitis.

Differential Diagnosis Acute calcific prevertebral tendinitis is often misdiagnosed as acute pharyngitis or retropharyngeal abscess. Occasionally, the patient is diagnosed with an early peritonsillar abscess. This delay in diagnosis can often subject the patient to unnecessary antibiotic therapy and occasionally surgical drainage of the suspected “abscess.” In some clinical situations, consideration should be given to primary or secondary tumors involving this anatomical region.

Treatment Initial treatment of the pain and functional disability associated with acute calcific prevertebral tendinitis should include nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 inhibitors. Local application of heat and cold and deep sedative massage may also be beneficial. For patients who do not respond to these treatment modalities, injection of the superior portion of the affected musculotendinous units with local anesthetic and steroid is a reasonable next step. Such injection should be considered only if the clinician is certain that no occult infection in this anatomical region exists. Ultrasound needle guidance may simplify needle placement and avoid injury to surrounding structures, including the thyroid gland, carotid artery, jugular vein, and exiting cervical nerve roots (Fig. 28.6).

Complications and Pitfalls The main pitfalls in the treatment of longus colli tendinitis are failure to diagnose this painful condition in a timely manner and mistaking it for a disease requiring more intensive treatment (e.g., retropharyngeal abscess or peritonsillar abscess). Rapid institution of treatment with NSAIDs and reassurance is often all that is required. For more recalcitrant cases, injection with local anesthetic and steroid almost always results in prompt resolution of symptoms. Use of this injection technique is safe if careful attention is paid to the clinically relevant anatomy. Sterile technique must be used to avoid infection, along with universal precautions to minimize any risk to the operator. The incidence of ecchymosis and hematoma formation can be decreased if pressure is applied to the injection site immediately after injection. Trauma to the tendon from the injection itself is also a possibility. Tendons that are highly inflamed or previously damaged are subject to rupture if they are injected directly. This complication can often be avoided if the clinician uses a gentle technique and stops injecting immediately on encountering significant resistance. Approximately 25% of patients complain of a transient increase in pain after injection, and patients should be warned of this possibility.

Clinical Pearls The musculotendinous unit of the muscles are susceptible to the development of tendinitis. Also known as crowned dens syndrome and retropharyngeal calcific tendinitis, calcific prevertebral tendinitis is often misdiagnosed as retropharyngeal abscess. Calcium hydroxyapatite deposition around the tendon may occur, thus making subsequent treatment more difficult. NSAIDs usually provide excellent palliation of the patient’s pain. If they do not, properly performed injection of the affected inflamed musculotendinous units with local anesthetic and steroid is a reasonable next step.

FIG. 28.3 Axial (A), coronal (B), and sagittal (C) enhanced computed tomography images of the neck at the bone window (left to right) demonstrating linear calcifications (white arrows) adjacent to the C1 arch and the odontoid process of C2. Note that there is no evidence of destruction of adjacent vertebral-body bony tissue or narrowing of intervertebral disc space suggestive of underlying discitis. From Alamoudi U, Al-Sayed AA, Al-Sallumi Y, et al. Acute calcific tendinitis of the longus colli muscle masquerading as a retropharyngeal abscess: a case report and review of the literature. Int J Surg Case Rep. 2017;41:343–346, fig 1.

FIG. 28.4 MRI Soft Tissue Neck Showing Edema of the Left Longus Colli Muscle With No Defined Enhancement. From Patel TK, Weis JC. Acute neck pain in the ED: consider longus colli calcific tendinitis vs meningitis. Am J Emerg Med. 2017;35[6]:943.e3–943.e4, fig 2.

FIG. 28.5 Sagittal ReconstructionThin arrow indicates area of inflammation, which may be mistaken for retropharyngeal abscess. Thick arrow indicates ectopic calcification on the ligament of the longus colli muscle. From Zapolsky N, Heller M, Felberbaum M, et al. Calcific tendonitis of the longus colli: an uncommon but benign cause of throat pain that closely mimics retropharyngeal abscess. J Emerg Med. 2017;52[3]:358–360, fig 1.

FIG. 28.6 Transverse Ultrasound Image of the Longus Colli Muscle.The blue star is the stellate ganglion. SCM, Sternocleidomastoid. Image property of Steven D. Waldman, MD.

Suggested Readings Alamoudi U, Al-Sayed A.A, Al-Sallumi Y, et al. Acute calcific tendinitis of the longus colli muscle masquerading as a retropharyngeal abscess: a case report and review of the literature. Int J Surg Case Rep. 2017;41:343–346. Ea H.-K, Lioté F. Diagnosis and clinical manifestations of calcium pyrophosphate and basic calcium phosphate crystal deposition diseases. Rheum Dis Clin North Am. 2014;40(2):207–229. Javanshir K, Rezasoltani A, Mohseni-Bandpei M.A, et al. Ultrasound assessment of bilateral longus colli muscles in subjects with chronic bilateral neck pain. Am J Phys Med Rehabil. 2011;90:293–301. Roldan C.J, Carlson P.J. Longus colli tendonitis, clinical consequences of a misdiagnosis. Am J Emerg Med. 2013;31(10):1538.e1–1538.e2. Suyama Y, Kishimoto M, Nozaki T, et al. Acute calcific tendinitis of the longus colli muscle. Arthritis Rheumatol. 2015;67:2446. Trendel D, Bonfort G, Lapierre-Combes M, et al. Acute severe neck pain and dysphagia following cervical maneuver: diagnostic approach. Eur Ann Otorhinolaryngol Head Neck Dis. 2014;131(2):135–138. Torbati S.S, Vos E.M, Bral D, et al. Calcific tendinitis of the longus colli muscle. Ear Nose Throat J. 2014;93:492–493.

29

Neck-Tongue Syndrome

Abstract Neck-tongue syndrome is a rare condition characterized by pain in the neck associated with numbness of the ipsilateral half of the tongue that is aggravated by movement of the upper cervical spine. This unusual constellation of symptoms is thought to be due to compression of the C2 nerve root by compromise of the atlantoaxial joint. This compression can be caused by joint instability that allows subluxation of the lateral joint, bony abnormality such as congenital fusions or stenosis, or tubercular infection. The tongue numbness is thought to be due to damage or intermittent compression of the lingual afferent fibers that pass via the hypoglossal nerve to innervate the tongue. The bulk of the fibers are proprioceptive, and patients with neck-tongue syndrome also may exhibit pseudoathetosis of the tongue. Neck-tongue syndrome occurs most commonly in patients older than 50 years, although the syndrome has been reported in a few pediatric patients.

Keywords atlatonaxial joint; cervicalgia; neck pain; neck-tongue; numbness; pseudoathetosis

ICD-10 CODE M79.2

The Clinical Syndrome Neck-tongue syndrome is a rare condition characterized by pain in the neck associated with numbness of the ipsilateral half of the tongue that is aggravated by movement of the upper cervical spine. This unusual constellation of symptoms is thought to be due to compression of the C2 nerve root by compromise of the atlantoaxial joint. This compression can be caused by joint instability that allows subluxation of the lateral joint, bony abnormality such as congenital fusions or stenosis, or tubercular infection. The tongue numbness is thought to be due to damage or intermittent compression of the lingual afferent fibers that pass via the hypoglossal nerve to innervate the tongue. The bulk of the fibers are proprioceptive, and patients with neck-tongue syndrome also may exhibit pseudoathetosis of the tongue. Neck-tongue syndrome occurs most commonly in patients older than 50 years, although the syndrome has been reported in a few pediatric patients.

Signs and Symptoms The pain of neck-tongue syndrome is in the distribution of the C2 nerve root. It is intermittent but is reproducible with certain neck movements. The physical findings associated with this pain are ill defined, with some patients with neck-tongue syndrome exhibiting a decreased range of motion of the cervical spine or tenderness of the upper paraspinous musculature. The main objective finding in neck-tongue syndrome is decreased sensation of the ipsilateral half of the tongue (Fig. 29.1). Often associated with this finding are pseudoathetoid movements of the tongue resulting from an impairment of the proprioceptive fibers.

Testing Magnetic resonance imaging (MRI) of the brain and brainstem should be performed in all patients thought to have neck-tongue syndrome. MRI of the brain provides the best information regarding the cranial vault and its contents. MRI is highly accurate and helps identify abnormalities that may put the patient at risk for neurological disasters secondary to intracranial and brainstem pathology, including tumors and demyelinating disease. Magnetic resonance angiography (MRA) may be useful to help identify aneurysms responsible for the patient’s neurological symptoms. In patients who cannot undergo MRI, such as patients with pacemakers, computed tomography (CT) is a reasonable second choice. Clinical laboratory tests consisting of a complete blood cell count, automated chemistry profile, and erythrocyte sedimentation rate are indicated to rule out infection, temporal arteritis, and malignancy that may mimic neck-tongue syndrome. Endoscopy of the hypopharynx with special attention to the piriform sinuses also is indicated to rule out occult malignancy. Differential neural blockade of the C2 nerve may help strengthen the diagnosis of neck-tongue syndrome.

Differential Diagnosis Neck-tongue syndrome is a clinical diagnosis that can be made on the basis of a targeted history and physical examination. Because of the rarity of this syndrome, the clinician must consider neck-tongue syndrome to be a diagnosis of exclusion. Diseases of the eyes, ears, nose, throat, and teeth may coexist and confuse the diagnosis. Tumors of the hypopharynx, including the tonsillar fossa and piriform sinus, may mimic the pain of neck-tongue syndrome, as may tumors at the cerebellopontine angle. Occasionally, demyelinating disease may produce a clinical syndrome identical to necktongue syndrome. The jaw claudication associated with temporal arteritis also may confuse the clinical picture, as may glossopharyngeal neuralgia.

Treatment The initial treatment of neck-tongue syndrome should consist of immobilization of the cervical spine with a soft cervical collar. A trial of nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors represents a reasonable next step. Case reports suggest that myofascial release, exercise, and spinal manipulative therapy may provide symptomatic relief. Blockade of the atlantoaxial joint and C2 nerve root with a local anesthetic and steroid also should be considered. For refractory cases, cervical fusion of the upper cervical segments may be required.

FIG. 29.1 The pain and numbness of the ipsilateral half of the tongue are aggravated by movement of the upper cervical spine.

Complications and Pitfalls Because of the rarity of neck-tongue syndrome, it is often misdiagnosed. Further complicating the confusion surrounding the diagnosis of this painful condition is the fact that many of the pathological processes that mimic necktongue syndrome are also difficult to diagnose, especially diseases of the hypopharynx. For these reasons, the diagnosis of neck-tongue syndrome should be approached with extreme caution.

Clinical Pearls Neck-tongue syndrome is a unique and uncommon cause of neck pain. The associated ipsilateral tongue numbness is pathognomonic for the syndrome and is unusual in character. An analogous type of proprioceptive numbness is seen in patients with Bell palsy. Given the rarity of this painful condition, the clinician should search carefully for other causes of the symptoms before attributing them to neck-tongue syndrome.

Suggested Readings Bogduk N. An anatomical basis for the neck-tongue syndrome. J Neurol Neurosurg Psychiatry. 1981;44:202–208. Borody C. Neck-tongue syndrome. J Manipulative Physiol Ther. 2004;29:367. Chedrawi A.K, Fishman M.A, Miller G. Neck-tongue syndrome. Pediatr Neurol. 2000;22:397–399. Niethamer L, Myers R. Manual therapy and exercise for a patient with necktongue syndrome: a case report. Orthop Sports Phys Ther. 2016;46(3):217– 224. Orrell R.W, Marsden C.D. The neck-tongue syndrome. J Neurol Neurosurg Psychiatry. 1994;57:348–352. Roberts C.S. Chiropractic management of a patient with neck-tongue syndrome: a case report. J Chiropr Med. 2016;15(4):321–324.

30

Sternohyoid Syndrome

Abstract While trauma is the common denominator in patients with omohyoid syndrome, there is usually no history of trauma in patients suffering from sternohyoid syndrome. The clinical presentation of sternohyoid syndrome mirrors that of omohyoid syndrome. Both painful conditions present as anterior supraclavicular neck pain with associated dysphagia and a foreign body sensation in throat. The pain of sternohyoid syndrome appears to be the result of damage to the fibers of the of the belly of sternohyoid muscle or as the result of abnormal attachment of the muscle to the mid-clavical rather than to the more medial clavical.

Keywords brachial plexopathy; dysphagia; neck pain; omohyoid syndrome; phrenic nerve; pneumothorax; recurrent laryngeal nerve; sternohyoid muscle; Sternohyoid syndrome; trigger point injection; trigger point; ultrasound guided injection

ICD-10 CODE M62.9

The Clinical Syndrome Although trauma is the common denominator in patients with omohyoid syndrome, there is usually no history of trauma in patients suffering from sternohyoid syndrome. The clinical presentation of sternohyoid syndrome mirrors that of omohyoid syndrome. Both painful conditions present as anterior supraclavicular neck pain with associated dysphagia and a foreign body sensation in the throat (Fig. 30.1). The pain of sternohyoid syndrome appears to be the result of damage to the fibers of the belly of the sternohyoid muscle or as the result of abnormal attachment of the muscle to the mid-clavicle rather than to the more medial clavicle. This pain manifests as myofascial. It is constant and exacerbated with movement of the affected muscle. A trigger point in the affected sternohyoid muscle is often present and provides a basis for treatment. The pain of omohyoid syndrome starts just above the clavicle at the mid to medial aspect of the clavicular attachment of the sternocleidomastoid muscle. The pain may radiate into the anterolateral neck and an abnormal mass adjacent to the anterior border of the sternocleidomastoid muscle may be palpable and in some cases visible. This abnormal muscle mass may be seen to move superiorly along with the larynx when the patient swallows (Fig. 30.2). Injection of the trigger point in the belly of the sternohyoid muscle with local anesthetic and steroid serves as a diagnostic and therapeutic maneuver.

Signs and Symptoms Patients suffering from sternohyoid syndrome present with pain in the supraclavicular region at a point just medial and superior to the attachment of the sternocleidomastoid muscle to the clavicle (see Fig. 30.1). A baseline level of pain is present even without movement of the muscle. The pain intensity ranges from minor to moderate. The pain often radiates into the anterolateral neck and increases with swallowing and movement of the sternohyoid muscle. An abnormal mass adjacent to the anterior border of the sternocleidomastoid muscle may be palpable and, in some cases, visible. This abnormal muscle mass may be seen to move superiorly along with the larynx when the patient swallows. A trigger point in the belly of the sternohyoid muscle is often present. The pain of sternohyoid syndrome is often exacerbated by swallowing. The neurological examination of a patient with sternohyoid syndrome is normal.

Testing Magnetic resonance imaging (MRI), computerized tomography, and/or ultrasound imaging of the soft tissues of the neck may reveal hematoma formation of the sternohyoid muscle acutely if trauma has occurred and calcification, fibrosis, or both as the syndrome becomes more chronic. These imaging modalities may also aid in the identification of abnormalities of the muscle and its insertion (Fig. 30.3). Injection of the belly of the sternohyoid muscle with local anesthetic can serve as a diagnostic maneuver to help strengthen the diagnosis. Occasionally, surgical section of the sternohyoid muscle may be required to provide long-lasting relief.

Differential Diagnosis Soft tissue injuries to the region may mimic sternohyoid syndrome. Because trauma is invariably involved in the evolution of the painful condition, strain and sprain of other soft tissues often exist concurrently with omohyoid syndrome. Omohyoid syndrome as well as primary or metastatic tumors of the neck and hypopharynx also may mimic the clinical presentation of omohyoid syndrome and should be high on the list of diagnostic possibilities if the history of trauma is weak or absent.

Treatment Nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors represent a reasonable first step in the treatment of omohyoid syndrome. The use of tricyclic antidepressants, such as nortriptyline, at a single bedtime dose of 25 mg, titrating upward as side effects allow also is helpful, especially if sleep disturbance is present. The injection of trigger points in the belly of the sternohyoid muscle often produces dramatic improvement in pain symptoms. The key landmark for injecting when treating omohyoid syndrome is the medial aspect of the clavicular extent of the sternocleidomastoid muscle (Fig. 30.4). The sternohyoid muscle is located just lateral to the trachea and the trigger point and/or abnormal mass can usually be identified approximately ¾ to 1 inch above the superior margin of the clavicle, medial to the sternocleidomastoid. The muscle finds its origin on the medial portion of the clavicle and the sternum and its tendon inserts onto the hyoid bone, slightly lateral and deep to the clavicular head of the sternocleidomastoid muscle. Given the relationship of the great vessels of the neck to the sternohyoid muscle, care must be taken when placing needles in this anatomical area.

FIG. 30.1 The pain of sternohyoid syndrome is localized in the supraclavicular region at a point just medial and superior to the attachment of the sternocleidomastoid muscle to the clavicle.

The patient is placed in the supine position, with the head turned away from the side to be blocked. Using a 5-mL sterile syringe, 3 mL of local anesthetic is drawn up. When treating sternohyoid syndrome, 80 mg of depot steroid is added to the local anesthetic with the first block, and 40 mg of depot steroid is added with subsequent blocks. The patient is asked to raise the head against the resistance of the pain specialist’s hand to aid in identification of the anteromedial border of the sternocleidomastoid muscle. The point at which the medial border of the sternocleidomastoid attaches to the clavicle is identified. At this point, slightly medial and approximately 1 inch above the clavicle, after preparation of the skin with antiseptic solution, a 1½-inch needle is inserted directly perpendicular to the table top (see Fig. 30.2). The needle should be advanced slowly because of proximity of the great vessels and brachial plexus. A “pop” often is felt as the fascia of the sternohyoid muscle is pierced; this should occur at a depth of ½ to ¾ of an inch. If strict attention to technique is observed, and the needle is not placed or directed too laterally, the brachial plexus should not be encountered.

Because of the proximity of the brachial plexus, the patient should be warned that a paresthesia could occur; the patient should be instructed to say “There!” if a paresthesia is felt. The needle should never be directed in a more inferior medial trajectory because pneumothorax is likely to occur. After the muscle is identified, gentle aspiration is done to identify blood or cerebrospinal fluid. If the aspiration test is negative, and no paresthesia into the distribution of the brachial plexus is encountered, 3 mL of solution is slowly injected, with the patient being monitored closely for signs of local anesthetic toxicity or inadvertent neuraxial injection. This technique can also be utilized for injection of botulinum toxin into the sternohyoid muscle. Ultrasound guidance may improve the accuracy of needle placement and decrease the incidence of needle-related complications.

Complications and Pitfalls The major complication in the treatment of patients thought to have sternohyoid syndrome is wrong diagnosis. Occult cervical spine fractures or instability after trauma remain an ever-present possibility. Failure to diagnose such injuries can put the patient at significant risk for permanent neurological sequelae. As mentioned earlier, if the history of trauma is suspect, the diagnosis of sternohyoid syndrome should become one of exclusion. A careful search for tumors of the neck, apex of the lung, anterior triangle of the neck, and hypopharynx is indicated. If a significant history of vomiting is ascertained, esophageal tear also should be considered.

FIG. 30.2 (A–D) Two patients with sternohyoid syndrome. Neck photograph of the resting state (A and C) and swallowing (B and D). X-shaped mass showed more medial to omohyoid muscles (B and D: white arrow head). From Kim JS, Hong KH, Hong YT, et al. Sternohyoid muscle syndrome. Am J Otolaryngol. 2015;36[2]:190–194. ISSN 0196-0709, https://doi.org/10.1016/j.amjoto.2014.10.028. http://www.sciencedirect.com/science/article/pii/S0196070914002555.

FIG. 30.3 Enhanced Neck Computed TomographyNormal anatomy was shown near the hyoid bone (A). Slightly thick sternohyoid muscle was noted on the left side (B) (white arrow). Normal sternohyoid (Right: blue arrow head) and abnormal attachment to midportion of clavicle (white arrow) were noted (C). From Kim JS, Hong KH, Hong YT, et al. Sternohyoid muscle syndrome. Am J Otolaryngol. 2015;36[2]:190–194. ISSN 0196-0709, https://doi.org/10.1016/j.amjoto.2014.10.028.

Although the injection technique for sternohyoid syndrome is safe, complications can occur. In addition to the potential for complications involving the vasculature, if the needle is placed too laterally, the proximity of the brachial plexus, the central neuroaxial structures, and the phrenic nerve can result in side effects and complications. Although these complications should be rare if proper technique is observed, the potential for inadvertent epidural, subdural, or subarachnoid injection remains a possibility. Inadvertent phrenic nerve block also can occur when using this injection technique to treat sternohyoid syndrome if the needle placement is too far posterolaterally. In the absence of significant pulmonary disease, unilateral phrenic nerve block should rarely create respiratory embarrassment. Inadvertent blockade of the recurrent laryngeal nerve with its attendant vocal cord paralysis combined with paralysis of the diaphragm from phrenic nerve block may make the clearing of pulmonary and upper airway secretions difficult, however. Because of the proximity of the apex of the lung, pneumothorax is a distinct possibility and the patient should be informed of this.

FIG. 30.4 Injection Site for the Treatment of Sternohyoid Syndrome.

Clinical Pearls Although an uncommon cause of pain, sternohyoid syndrome is a clinically distinct and easily recognizable pain syndrome. Because of the similarities in presentation of the more common omohyoid syndrome and the rarer sternohyoid syndrome, careful physical examination and imaging may be required to distinguish the two uncommon causes of anterior neck pain. The key to performing this injection technique safely is a clear understanding of the anatomy and careful identification of the anatomical landmarks necessary to perform the block. The brachial plexus is quite superficial at the level at which this block is performed. The needle should rarely be inserted deeper than ¾ of an inch in all but the most obese patients. If strict adherence to technique is observed, and the needle is never advanced medially from the lateral border of the insertion of the sternocleidomastoid

muscle on the clavicle, the incidence of pneumothorax should be less than 0.5%. Sternohyoid syndrome is a diagnosis of exclusion. The clinician should always evaluate a patient with pain in this anatomical region for occult malignancy. Tumors of the larynx, hypopharynx, and anterior triangle of the neck may manifest with clinical symptoms identical to sternohyoid syndrome. In the setting of flexion/extension injuries or other forceful trauma to the soft tissues of the neck, cervical spine, or both, the clinician also should evaluate the patient for trauma to the cervical spine and brachial plexus by careful physical examination and electromyography.

Suggested Readings Bradley L.A. Pathophysiology of fibromyalgia. Am J Med. 2009;122(suppl 1):S22–S30. Colombo J.R, Dagher W, Wein R.O. Benign proliferative myositis of the sternohyoid muscle: review and case report. Am J Otolaryngol. 2015;36(1):87–89. Ge H.U, Nie H, Madeleine P, et al. Contribution of the local and referred pain from active myofascial trigger points in fibromyalgia syndrome. Pain. 2009;147:233–240. Guo-Hua W, Xiao-Ling J, Rong W, et al. Doubled omohyoid muscle in human: a case report and literature review. Clin Anat. 2009;22:868–870. Kim J.S, Hong K.H, Hong Y.T, Han B.H. Sternohyoid muscle syndrome. Am J Otolaryngol. 2015;36(2):190–194. Kim L, Kwon H, Pyun S.B. Pseudodysphagia due to omohyoid muscle syndrome. Dysphagia. 2009;24:357–361. Lapegue F, Faruch-Bilfeld M, Demondion X, et al. Ultrasonography of the brachial plexus, normal appearance and practical applications. Diagn Interv Imaging. 2014;95(3):259–275. Su P.H, Wang T.G, Wang Y.C. Ultrasound-guided injection of botulinum toxin in a patient with omohyoid muscle syndrome: a case report. J Clin Ultrasound. 2013;41:373–376. Waldman S.D. Omohyoid syndrome. In: Waldman S.D, ed. Atlas of Pain Management Injection Techniques. 4th ed. Philadelphia: Saunders; 2017:29–31. Wong D.S.Y, Li H.J.C. The omohyoid sling syndrome. Am J Otolaryngol. 2000;21:318–322.

SECT ION 3

Shoulder Pain Syndromes OUT LINE 31. Supraspinatus Tendinitis 32. Infraspinatus Tendinitis 33. Subacromial Impingement Syndrome 34. Os Acromiale Pain Syndrome 35. Glomus Tumor of the Shoulder 36. Pectoralis Major Tear Syndrome 37. Suprascapular Nerve Entrapment 38. Snapping Scapula Syndrome 39. Quadrilateral Space Syndrome

31

Supraspinatus Tendinitis

Abstract Supraspinatus tendinitis can manifest as an acute or chronic painful condition of the shoulder. Acute supraspinatus tendinitis usually occurs in younger patients after overuse or misuse of the shoulder joint. Inciting factors may include carrying heavy loads in front of and away from the body, throwing injuries, or the vigorous use of exercise equipment. Chronic supraspinatus tendinitis tends to occur in older patients and to manifest in a more gradual or insidious manner, without a single specific event of antecedent trauma. The pain of supraspinatus tendinitis is constant and severe, with sleep disturbance often reported. The pain of supraspinatus tendinitis is felt primarily in the deltoid region. It is moderate to severe and may be associated with a gradual loss of range of motion of the affected shoulder. The patient often awakens at night when he or she rolls over onto the affected shoulder.

Keywords calcific tendinopathy; crystal deposition disease; rotator cuff tear; shoulder pain; subdeltoid bursitis; Supraspinatus tendinitis; ultrasound guided injection sports injury

ICD-10 CODE M75.10

The Clinical Syndrome Supraspinatus tendinitis can manifest as an acute or chronic painful condition of the shoulder. Acute supraspinatus tendinitis usually occurs in younger patients after overuse or misuse of the shoulder joint. Inciting factors may include carrying heavy loads in front of and away from the body, throwing injuries, or the vigorous use of exercise equipment. Chronic supraspinatus tendinitis tends to occur in older patients and to manifest in a more gradual or insidious manner, without a single specific event of antecedent trauma. The pain of supraspinatus tendinitis is constant and severe, with sleep disturbance often reported. The pain of supraspinatus tendinitis is felt primarily in the deltoid region. It is moderate to severe and may be associated with a gradual loss of range of motion of the affected shoulder. The patient often awakens at night when he or she rolls over onto the affected shoulder.

Signs and Symptoms A patient with supraspinatus tendinitis may attempt to splint the inflamed tendon by elevating the scapula to remove tension from the ligament, giving the patient a “shrugging” appearance (Fig. 31.1). Point tenderness is usually present over the greater tuberosity. The patient exhibits a painful arc of abduction and complains of a catch or sudden onset of pain in the midrange of the arc resulting from impingement of the humeral head onto the supraspinatus tendon. A patient with supraspinatus tendinitis exhibits a positive Dawbarn sign, which is pain to palpation over the greater tuberosity of the humerus when the arm is hanging down that disappears when the arm is fully abducted. The empty can test can help confirm the diagnosis (Fig. 31.2). Early in the course of the disease, passive range of motion is full and without pain.

FIG. 31.1 Patients with supraspinatus tendinitis exhibit point tenderness of the greater tuberosity and a painful arc of abduction.

FIG. 31.2 The empty can test for supraspinatus tendonitis is performed by having the patient assume the standing position. The affected arm is then gradually elevated to 90 degrees in the scapular plane with the elbow fully extended, the arm in full internal rotation, and the forearm pronated, as if the patient is trying to shake the last few drops out of an empty can. The examiner then exerts downward pressure to the affected arm. The test is considered positive if the patient experiences a significant increase in pain or demonstrates weakness. From Waldman SD. Physical Diagnosis of Pain. 3rd ed. Philadelphia: Elsevier; 2015:98.

As the disease progresses, patients often experience a gradual decrease in functional ability with decreasing shoulder range of motion, making simple everyday tasks, such as combing hair, fastening a brassiere, or reaching overhead, quite difficult. With continued disuse, muscle wasting may occur and a frozen shoulder may develop.

Testing Plain radiographs are indicated in all patients who present with shoulder pain. Based on the patient’s clinical presentation, additional testing, including complete blood cell count, sedimentation rate, and antinuclear antibody testing, may be indicated. Magnetic resonance imaging (MRI) of the shoulder is indicated if rotator cuff tear is suspected and to confirm the diagnosis of supraspinatus tendinitis (Fig. 31.3). The injection technique described here serves as a diagnostic and therapeutic maneuver.

Differential Diagnosis Because supraspinatus tendinitis may occur after seemingly minor trauma or develop gradually over time, the diagnosis often is delayed. Tendinitis of the musculotendinous unit of the shoulder frequently coexists with bursitis of the associated bursae of the shoulder joint, creating additional pain and functional disability. This ongoing pain and functional disability can cause the patient to splint the shoulder group with resultant abnormal movement of the shoulder, which puts additional stress on the rotator cuff. This stress can lead to further trauma to the entire rotator cuff. With rotator cuff tears, passive range of motion is normal but active range of motion is limited, in contrast to frozen shoulder, in which passive and active range of motion are limited. Rotator cuff tear rarely occurs before age 40 except in cases of severe acute trauma to the shoulder. Cervical radiculopathy rarely may cause pain limited to the shoulder, although in most instances, associated neck and upper extremity pain and numbness are present.

FIG. 31.3 (A) AP radiograph of a patient with calcific tendinitis affecting the supraspinatus tendon. (B) The corresponding longitudinal US image shows the area of calcification within the tendon substance (small white arrows) with posterior acoustic shadowing. The subdeltoid fat plane superficial to the tendon (large white arrows) and the tendon insertion on the greater tuberosity (broken white arrows) can both be clearly seen. From Waldman SD, Campbell RSD. Imaging of Pain. Philadelphia: Elsevier; 2010:233, fig 3.

Treatment Initial treatment of the pain and functional disability associated with supraspinatus tendinitis should include a combination of nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and physical therapy. The local application of heat and cold also may be beneficial. For patients who do not respond to these treatment modalities, the following injection technique may be a reasonable next step. The use of physical therapy, including gentle range-of-motion exercises, should be introduced several days after the patient undergoes this injection technique for shoulder pain. Vigorous exercises should be avoided because they would exacerbate the symptoms.

FIG. 31.4 Correct Needle Placement for Injection Into the Supraspinatus Tendon.

To inject the supraspinatus tendon, the patient is placed in the supine position, with the forearm medially rotated behind the back. This positioning of the upper extremity places the lateral epicondyle of the elbow in an anterior position and makes its identification easier. After identification of the lateral epicondyle of the elbow, the humerus is traced superiorly to the anterior edge of the acromion. A slight indentation just below the anterior edge of the acromion marks the point of insertion of the supraspinatus tendon into the upper facet of the greater tuberosity of the humerus. The

point is marked with a sterile marker. Proper preparation with antiseptic solution of the skin overlying the shoulder, subacromial region, and joint space is carried out. A sterile syringe containing 1 mL of 0.25% preservative-free bupivacaine and 40 mg of methylprednisolone is attached to a 25-gauge, 1½-inch needle using strict aseptic technique. With strict aseptic technique, the previously marked point is palpated, and the indentation indicating the insertion of the supraspinatus tendon is identified again with the gloved finger. The needle is carefully advanced perpendicularly at this point through the skin and subcutaneous tissues and through the joint capsule until it impinges on bone (Fig. 31.4). The needle is withdrawn 1 to 2 mm out of the periosteum of the humerus, and the contents of the syringe are gently injected. Slight resistance to injection should be felt. If no resistance is encountered, either the needle tip is in the joint space itself or the supraspinatus tendon is ruptured. If significant resistance to injection is detected, the needle tip is probably in the substance of a ligament or tendon and should be advanced or withdrawn slightly until the injection proceeds without significant resistance. The needle is removed, and a sterile pressure dressing and ice pack are placed at the injection site. Ultrasound guidance may improve the accuracy of needle placement and decrease the incidence of needle-related complications (Fig. 31.5).

FIG. 31.5 Ultrasound Image Demonstrating Grade IV Calcific Tendinitis of the Supraspinatus Tendon. Image owned by Steven D. Waldman.

Complications and Pitfalls The major complication of this injection technique is infection. This complication should be exceedingly rare if strict aseptic technique is followed. The possibility of trauma to the supraspinatus tendon from the injection itself remains an ever-present possibility. Tendons that are highly inflamed or previously damaged are subject to rupture if they are directly injected. This complication can be greatly decreased if the clinician uses gentle technique and stops injecting immediately if significant resistance to injection is encountered. Approximately 25% of patients complain of a transient increase in pain after this injection technique; patients should be warned of this possibility.

Clinical Pearls The musculotendinous unit of the shoulder joint is susceptible to the development of tendinitis for several reasons. First, the joint is subjected to a wide range of repetitive motions. Second, the space in which the musculotendinous unit functions is restricted by the coracoacromial arch, making impingement a likely possibility with extreme movements of the joint. Third, the blood supply to the musculotendinous unit is poor, making healing of microtrauma more difficult. All of these factors can contribute to tendinitis of one or more of the tendons of the shoulder joint. Calcium deposition around the tendon may occur if the inflammation continues, making subsequent treatment more difficult. Tendinitis of the musculotendinous unit of the shoulder frequently coexists with bursitis of the associated bursae of the shoulder joint, creating additional pain and functional disability. The injection technique described is extremely effective in the treatment of pain secondary to the causes of shoulder pain mentioned earlier. Coexistent bursitis and arthritis also may contribute to shoulder pain and may require additional treatment with a more localized injection of local anesthetic and depot steroid. This technique is a safe procedure if careful attention is paid to the clinically relevant anatomy in the areas to be injected. Care must be taken to use sterile technique to avoid infection and universal precautions to avoid risk to the operator. The incidence of ecchymosis and hematoma formation can be decreased if pressure is placed on the injection

site immediately after injection.

Suggested Readings Chen SK, Chou PH, Lue YL, Lu YM. Treatment for frozen shoulder combined with calcific tendinitis of the supraspinatus. Kaohsiung J Med Sci. 3080(24):78–84. Clements N. Ultrasound-guided therapy of calcifying tendinitis in the supraspinatus tendon. Ultrasound Med Biol. 2011;378(suppl):S139–S140. Mileto A, Gaeta M. Calcific tendonitis of supraspinatus simulating acute brachial neuritis (Parsonage-Turner syndrome). Clin Radiol. 2011;66(6):578– 581. Vinanti G.B, Pavan D, Rossato A, Carlo B. Atypical localizations of calcific deposits in the shoulder. Int J Surg Case Rep. 2015;10:206–210. Waldman S.D. Functional anatomy of the shoulder joint. In: Waldman S.D, ed. Pain Review. 2nd ed. Philadelphia: Elsevier; 2017:94–95. Waldman S.D. Supraspinatus tendinitis. In: Waldman S.D, ed. Atlas of Pain Management Injection Techniques. 4th ed. Philadelphia: Elsevier; 2017 94–67.

32

Infraspinatus Tendinitis

Abstract Infraspinatus tendinitis can manifest as an acute or chronic painful condition of the shoulder. Acute infraspinatus tendinitis usually occurs in a younger group of patients after overuse or misuse of the shoulder joint. Inciting factors include activities that require repeated abduction and lateral rotation of the humerus, such as installing brake pads during assembly line work. The vigorous use of exercise equipment also has been implicated. The pain of infraspinatus tendinitis is constant, severe, and localized to the deltoid area. Significant sleep disturbance is often reported. Patients with infraspinatus tendinitis exhibit pain with lateral rotation of the humerus and on active abduction. Chronic infraspinatus tendinitis tends to occur in older patients and to manifest in a more gradual or insidious manner, without a single specific event of antecedent trauma. The pain of infraspinatus tendinitis may be associated with a gradual loss of range of motion of the affected shoulder. The patient often awakens at night when he or she rolls over onto the affected shoulder.

Keywords Infraspinatus tendinitis; shoulder pain; sports injury; subdeltoid bursitis; tendinosis; ultrasound guided injection

ICD-10 CODE M75.10

The Clinical Syndrome Infraspinatus tendinitis can manifest as an acute or chronic painful condition of the shoulder. Acute infraspinatus tendinitis usually occurs in a younger group of patients after overuse or misuse of the shoulder joint. Inciting factors include activities that require repeated abduction and lateral rotation of the humerus, such as installing brake pads during assembly line work. The vigorous use of exercise equipment also has been implicated. The pain of infraspinatus tendinitis is constant, severe, and localized to the deltoid area. Significant sleep disturbance is often reported. Patients with infraspinatus tendinitis exhibit pain with lateral rotation of the humerus and on active abduction. Chronic infraspinatus tendinitis tends to occur in older patients and to manifest in a more gradual or insidious manner, without a single specific event of antecedent trauma. The pain of infraspinatus tendinitis may be associated with a gradual loss of range of motion of the affected shoulder. The patient often awakens at night when he or she rolls over onto the affected shoulder.

Signs And Symptoms The patient may attempt to splint the inflamed infraspinatus tendon by rotating the scapula anteriorly to remove tension from the tendon (Fig. 32.1). Point tenderness is usually present over the greater tuberosity. The patient exhibits a painful arc of abduction and complains of a catch or sudden onset of pain in the midrange of the arc. Early in the course of the disease, passive range of motion is full and painless. As the disease progresses, patients with infraspinatus tendinitis often experience a gradual decrease in functional ability with decreasing shoulder range of motion, making simple everyday tasks, such as combing hair, fastening a brassiere, or reaching overhead, quite difficult. With continued disuse, muscle wasting may occur and a frozen shoulder may develop.

FIG. 32.1 Patients with infraspinatus tendinitis exhibit posterior point tenderness and a painful arc of abduction.

Testing Plain radiographs are indicated in all patients with shoulder pain. Based on the patient’s clinical presentation, additional testing, including complete blood cell count, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. Magnetic resonance imaging (MRI) of the shoulder is indicated if rotator cuff tear is suspected and to confirm the diagnosis of infraspinatus tendinitis (Fig. 32.2). The injection technique discussed here serves as a diagnostic and therapeutic maneuver.

Differential Diagnosis Because infraspinatus tendinitis may occur after seemingly minor trauma or develop gradually over time, the diagnosis is often delayed. Tendinitis of the musculotendinous unit of the shoulder frequently coexists with bursitis of the associated bursae of the shoulder joint, creating additional pain and functional disability. This ongoing pain and functional disability can cause the patient to splint the shoulder group, with resultant abnormal movement of the shoulder that puts additional stress on the rotator cuff. This stress can lead to further trauma to the entire rotator cuff. With rotator cuff tears, passive range of motion is normal, but active range of motion is limited, in contrast to frozen shoulder, in which passive and active range of motion are limited. Rotator cuff tear rarely occurs before age 40 except in cases of severe acute trauma to the shoulder. Cervical radiculopathy rarely may cause pain limited to the shoulder, although in most instances associated neck and upper extremity pain and numbness are present.

FIG. 32.2 Periarticular Crystal Deposition: Shoulder—Infraspinatus, Teres Minor, and Subscapularis Tendon Calcification(A) In internal rotation, calcific deposits in the infraspinatus and teres minor tendons appear lateral to the humeral head (arrow) and deposits in the subscapularis tendon are located near the lesser tuberosity, overlying the joint space (arrowhead). (B and C) Radiograph and photograph of a section of the humeral head outline these same calcifications (arrows, arrowhead). From Resnick D, ed. Diagnosis of Bone and Joint Disorders. 4th ed. Philadelphia: Saunders; 2002.

FIG. 32.3 Correct Needle Placement for Injection Into the Infraspinatus Tendon.

Treatment Initial treatment of the pain and functional disability associated with rotator cuff tear should include a combination of nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and physical therapy. The local application of heat and cold also may be beneficial. For patients who do not respond to these treatment modalities, the following injection technique may be a reasonable next step. The use of physical therapy, including gentle range-of-motion exercises, should be introduced several days after the patient undergoes this injection technique for shoulder pain. Vigorous exercises should be avoided because they would exacerbate the symptoms. To inject the infraspinatus tendon, the skin overlying the posterior shoulder is prepared with antiseptic solution. A sterile syringe containing 1 mL of 0.25% preservative-free bupivacaine and 40 mg of methylprednisolone is attached to a 25-gauge, 1½-inch needle using strict aseptic technique. With strict aseptic technique, the previously marked point is palpated, and the insertion of the infraspinatus tendon is identified again with the gloved finger. The needle is carefully advanced at this point through the skin and subcutaneous tissues and the margin of the deltoid muscle and underlying infraspinatus muscle until it impinges on bone (Fig. 32.3). The needle is withdrawn 1 to 2 mm out of the periosteum of the humerus, and the contents of the syringe are gently injected. There should be slight resistance to injection. If no resistance is encountered, either the needle tip is in the joint space itself or the infraspinatus tendon is ruptured. If significant resistance to injection is felt, the needle tip is probably in the substance of a ligament or tendon and should be advanced or withdrawn slightly until the injection proceeds without significant resistance. The needle is removed, and a sterile pressure dressing and ice pack are placed at the injection site. Ultrasound guidance may improve the accuracy of needle placement and decrease the incidence of needle-related complications (Fig. 32.4).

FIG. 32.4 Longitudinal Ultrasound Image Demonstrating a Full Thickness Tear of the Infraspinatus Musculotendinous Unit.

Complications and Pitfalls The major complication of this injection technique is infection. This complication should be exceedingly rare if strict aseptic technique is followed. The possibility of trauma to the infraspinatus tendon from the injection itself remains an ever-present possibility. Tendons that are highly inflamed or previously damaged are subject to rupture if they are directly injected. This complication can be greatly decreased if the clinician uses gentle technique and stops injecting immediately if significant resistance to injection is encountered. Approximately 25% of patients complain of a transient increase in pain after this injection technique; patients should be warned of this possibility.

Clinical Pearls The musculotendinous unit of the shoulder joint is susceptible to the development of tendinitis for several reasons. First, the joint is subjected to a wide range of often repetitive motions. Second, the space in which the musculotendinous unit functions is restricted by the coracoacromial arch, making impingement a likely possibility with extreme movements of the joint. Third, the blood supply to the musculotendinous unit is poor, making healing of microtrauma more difficult. These factors can contribute to tendinitis of one or more of the tendons of the shoulder joint. Calcium deposition around the tendon may occur if the inflammation continues, making subsequent treatment more difficult. Tendinitis of the musculotendinous unit of the shoulder frequently coexists with bursitis of the associated bursae of the shoulder joint, creating additional pain and functional disability. The injection technique described is extremely effective in the treatment of pain secondary to the causes of shoulder pain mentioned. Coexistent bursitis and arthritis also may contribute to shoulder pain and may require additional treatment with a more localized injection of a local anesthetic and depot steroid. This technique is a safe procedure if careful attention is paid to the clinically relevant anatomy in the areas to be injected. Care must be taken to use sterile technique to avoid infection and universal precautions to avoid risk to the operator. The incidence of ecchymosis and hematoma formation can be decreased if pressure is placed on the injection site

immediately after injection.

Suggested Readings Guerini H, Pluot E, Pessis E, et al. Tears at the myotendinous junction of the infraspinatus: ultrasound findings, diagnostic and interventional imaging. 2015;96(4):349–356. Golshani K, Cinque ME, O’Halloran P, Softness K, Keeling L, Macdonell JR. Upper extremity weightlifting injuries: diagnosis and management. J Orthop. 2018;15(1):24–27. Walch G, Nové-Josserand L, Liotard J.P, Noël E. Musculotendinous infraspinatus ruptures: an overview. Orthop Traumatol Surg Res. 2009;95(7):463–470. Waldman S.D. Functional anatomy of the shoulder joint. In: Waldman S.D, ed. Pain Review. 2nd ed. Philadelphia: Elsevier; 2017:88–90. Waldman S.D. Infraspinatus tendinitis. In: Waldman S.D, ed. Atlas of Pain Management Injection Techniques. 3rd ed. Philadelphia: Saunders; 2007:71–75. Waldman S.D. Infraspinatus tendinitis. In: Waldman S.D, ed. Atlas of Pain Management Injection Techniques. 4th ed. Philadelphia: Elsevier; 2017:97–99.

33

Subacromial Impingement Syndrome

Abstract Patients with subacromial impingement syndrome present with diffuse shoulder pain, with an associated feeling of weakness combined with loss of range of motion. Pain is often worse at night, and patients often complain that they are unable to sleep on the affected shoulder. Although subacromial impingement syndrome can occur as a result of acute trauma, the usual clinical presentation is more insidious, without a clear-cut history of trauma to the affected shoulder. Untreated, subacromial impingement syndrome can lead to progressive tendinopathy of the rotator cuff and gradually increasing shoulder instability and functional disability. In patients older than 50 years, progression of impingement often leads to rotator cuff tear.

Key Words Hawkins test; magnetic resonance imaging; Neer test; shoulder pain rotator cuff tear; Subacromial impingement syndrome; subacromial injection; tendinopathy; ultrasound guided injection; Yocum test

ICD-10 CODE M25.519

The Clinical Syndrome The subacromial space lies directly inferior to the acromion, coracoid process, acromioclavicular joint, and coracoacromial ligament (Fig. 33.1). Lubricated by the subacromial bursa, the subacromial space in health is narrow, and the anatomical structures surrounding it are responsible for maintaining static and dynamic shoulder stability. The space between the acromion and the superior aspect of the humeral head is called the impingement interval, and abduction of the arm narrows the space further (Fig. 33.2). Any pathological condition that further narrows this space (e.g., osteophyte, abnormal acromial anatomy, ligamentous calcification, or congenital defects of the acromion) increases the incidence of impingement (Fig. 33.3). The most common causes of subacromial impingement are listed in Table 33.1. Similar to the congenital anatomical variant of the trefoil spinal canal being associated with a statistically significantly higher incidence of spinal stenosis, several common normal anatomical variants of the acromion often contribute to development of subacromial impingement syndrome. These include type 2 and type 3 acromions (Fig. 33.4). Although the “normal” type 1 acromion is relatively flat, the type 2 acromion curves downward and the type 3 acromion hooks downward in the shape of a scimitar. The downward curve of the type 2 and type 3 acromions markedly narrow the subacromial space (Fig. 33.5). In addition to these anatomical variations, a congenitally unfused acromial apophysis, the os acromiale, is often associated with subacromial impingement syndrome (Fig. 33.6). Patients with subacromial impingement syndrome present with diffuse shoulder pain, with an associated feeling of weakness combined with loss of range of motion. Pain is often worse at night, and patients often complain that they are unable to sleep on the affected shoulder. Although subacromial impingement syndrome can occur as a result of acute trauma, the usual clinical presentation is more insidious, without a clear-cut history of trauma to the affected shoulder. Untreated, subacromial impingement syndrome can lead to progressive tendinopathy of the rotator cuff and gradually increasing shoulder instability and functional disability. In patients older than 50 years, progression of impingement often leads to rotator cuff tear.

Signs and Symptoms A patient with subacromial impingement syndrome reports increasing shoulder pain with any activities that abduct or forward flex the shoulder, such as putting in a light bulb or reaching for dishes in a cabinet above shoulder height (Fig. 33.7). Patients with subacromial impingement syndrome have a positive Neer test, which is performed by having the patient assume a sitting position while the examiner applies firm forward pressure on the patient’s scapula and simultaneously raises the patient’s arm to an overhead position (Fig. 33.8). Neer test is considered positive when the patient exhibits pain or apprehension when the arm moves about 60 degrees. Although not completely diagnostic of subacromial impingement syndrome, a positive Neer test should prompt the examiner to order magnetic resonance imaging (MRI) of the affected shoulder to clarify and strengthen the diagnosis.

Testing MRI and dynamic ultrasound imaging of the shoulder provides the best information regarding any pathological process of the shoulder (Fig. 33.9). MRI is highly accurate and helps identify abnormalities that may put the patient at risk for continuing damage to the rotator cuff and humeral head. MRI of the shoulder also helps rule out unsuspected pathological conditions that may harm the patient, such as primary and metastatic tumors of the shoulder joint and surrounding structures. In patients who cannot undergo MRI, such as patients with pacemakers, computed tomography (CT) is a reasonable second choice. Radionuclide bone scanning and plain radiography are indicated if fracture or bony abnormality such as metastatic disease is considered in the differential diagnosis.

FIG. 33.1 The subacromial space lies directly inferior to the acromion, the coracoid process, the acromioclavicular joint, and the coracoacromial ligament.

FIG. 33.2 The space between the acromion and the superior aspect of the humeral head is the impingement interval, and abduction of the arm narrows the space further.

FIG. 33.3 Coronal T1W MR image demonstrating lateral downsloping of the acromion, a pattern of acromial morphology thought to be associated with impingement. From Waldman SD, Campbell RSD. Subacromial impingement. In: Imaging of Pain. Philadelphia: Elsevier; 2011:chap 98, 251.

TABLE 33.1 Causes of Subacromial Impingement Syndrome

FIG. 33.4 Anatomical Variants of the Acromion.

FIG. 33.5 (A) Lateral downsloping (LD) of the anterior acromion as seen on coronal section (arrow). (B) Coronal T2-weighted MR image with fat suppression revealing LD (arrow). Note the corresponding alterations on the bursal surface of the rotator cuff and the thickened subdeltoid bursa filled with fluid (arrowheads). From Zlatkin MB. MRI of the Shoulder. 2nd ed. Philadelphia: Lippincott Williams & Wilkins; 2003:1639.

Screening laboratory tests consisting of complete blood cell count, erythrocyte sedimentation rate, and automated blood chemistry testing

should be performed if the diagnosis of subacromial impingement syndrome is in question. Arthrocentesis of the glenohumeral joint may be indicated if septic arthritis or crystal arthropathy is suspected.

FIG. 33.6 Os AcromialeTransaxial intermediate-weighted (TR/TE, 1000/20) spin echo MRI shows a triangular os acromiale (arrows) articulating with the clavicle and, in an irregular fashion (arrowhead), with the acromion. From Resnick D, ed. Diagnosis of Bone and Joint Disorders. 4th ed. Philadelphia: Saunders; 2002:4577.

Differential Diagnosis Subacromial impingement syndrome is a clinical diagnosis supported by a combination of clinical history, physical examination, radiography, and MRI. Pain syndromes that may mimic subacromial impingement syndrome include subacromial bursitis, tendinopathy and tendinitis of the rotator cuff, calcification and thickening of coracoacromial ligament, and arthritis affecting any of the shoulder joints. Adhesive capsulitis or frozen shoulder may confuse the diagnosis, as may idiopathic brachial plexopathy (Parsonage-Turner syndrome; see Chapter 25). Primary and metastatic tumors of the shoulder and surrounding structures remain an ever-present possibility and should always be part of the differential diagnosis of patients presenting with shoulder pain.

Treatment Initial treatment of the pain and functional disability associated with subacromial impingement syndrome should include a combination of nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and gentle physical therapy. Local application of heat and cold also may be beneficial. For patients who do not respond to these treatment modalities, injection of the subacromial space with local anesthetic and steroid is a reasonable next step while obtaining MRI and other appropriate testing to clarify further the working clinical diagnosis. Ultrasound guidance may improve the accuracy of needle placement and decrease the incidence of needle related complications. The use of physical therapy, including gentle range-of-motion exercises, should be introduced several days after the patient undergoes this injection technique for shoulder pain. Vigorous exercises should be avoided because they would exacerbate the patient’s symptoms. For patients who do not respond to these treatment modalities or radiographically have shown anatomical subacromial impingement that is producing ongoing damage to the rotator cuff, open or arthroscopic acromioplasty is required.

FIG. 33.7 Patients with subacromial impingement syndrome typically complain of increasing shoulder pain with activities that abduct or forward flex the shoulder.

FIG. 33.8 Neer Test for Subacromial Impingement Syndrome. From Waldman SD. Physical Diagnosis of Pain: An Atlas of Signs and Symptoms. 3rd ed. Philadelphia: Saunders; 2016:68.

FIG. 33.9 Longitudinal Ultrasound Image of Abducted Right Shoulder Demonstrating Subacromial Impingement. Image owned by Stephen D. Waldman.

Complications and Pitfalls Failure to diagnose subacromial impingement syndrome correctly puts the patient at risk for the missed diagnosis of other syndromes that may result in ongoing damage to the shoulder or lead to overlooked pathological processes in this anatomical region that may harm the patient, such as Pancoast tumor or primary or metastatic tumors of the shoulder. MRI is indicated in all patients thought to have subacromial impingement syndrome, and aggressive treatment of surgically correctable causes of such impingement is generally indicated sooner rather than later to avoid ongoing irreversible shoulder damage.

Clinical Pearls The musculotendinous unit of the shoulder joint is susceptible to the development of tendinitis for several reasons. First, the joint is subjected to a wide range of often repetitive motions. Second, the space in which the musculotendinous unit functions is restricted by the coracoacromial arch, making impingement a likely possibility with extreme movements of the joint. Third, the blood supply to the musculotendinous unit is poor, making healing of microtrauma more difficult. These factors can contribute to tendinitis of one or more of the tendons of the shoulder joint. Calcium deposition around the tendon may occur if the inflammation continues, making subsequent treatment more difficult. Tendinitis of the musculotendinous unit of the shoulder frequently coexists with bursitis of the associated bursae of the shoulder joint, creating additional pain and functional disability. Patients with untreated subacromial impingement syndrome continue to experience pain and functional disability and may continue to cause ongoing irreversible shoulder damage culminating in damage to the humeral head and rotator cuff tear.

Suggested Readings Cuff Andrew, Littlewood Chris. Subacromial impingement syndrome – What does this mean to and for the patient? A qualitative study. Musculoskelet Sci Pract. 2018;33:24–28. Antoine Ferenczi, Yelnik Alain, Orcel Philippe, Beaudreuil Johann. Reliability study of sub-acromial impingement tests including a new clinical manoeuver. Ann Phys Rehabil Med. 2017;60(suppl):e29. Mahajan A. Comparing platelet-rich plasma with steroid injection in patients with subacromial impingement syndrome. Sports Med Arthrosc Rehabil Ther Technol. 2017;9:118. Dickens V.A, Williams J.L, Bhamra M.S. Role of physiotherapy in the treatment of subacromial impingement syndrome: a prospective study. Physiotherapy. 2005;91:159–164. Michener L.A, McClure P.W, Karduna A.R. Anatomical and biomechanical mechanisms of subacromial impingement syndrome. Clin Biomech. 2003;18:369–379. Neagle C.E, Bennett J.B. Subacromial anatomy and biomechanics related to the impingement syndrome. Oper Tech Sports Med. 1994;2:82–88. Susmita S, Neelam V, Aneja P.S, Saxena A. Acromial morphology and subacromial impingement syndrome: a clinicoanatomical study. J Anat Soc India. 2017;66(suppl 1):S48. Waldman S.D. An overview of impingement Syndromes. In: Waldman S.D, ed. Physical Diagnosis of Pain: An Atlas of Signs and Symptoms. 3rd ed. Philadelphia: Saunders; 2016:64–67. Waldman S.D. Functional anatomy of the shoulder joint. In: Waldman S.D, ed. Pain Review. 2nd ed. Philadelphia: Saunders; 2009:808–8190. Waldman S.D. The Neer test. In: Waldman S.D, ed. Physical Diagnosis of Pain: An Atlas of Signs and Symptoms. 3rd ed. Philadelphia: Saunders; 2016:68.

34

Os Acromiale Pain Syndrome

Abstract Patients suffering from os acromiale have diffuse shoulder pain, with an associated feeling of weakness combined with loss of range of motion. Pain is often worse at night, and patients often complain that they are unable to sleep on the affected shoulder. The clinical presentation is usually insidious, without a clear-cut history of trauma to the affected shoulder. Affected patients tend to be younger than those with other causes of shoulder impingement syndromes. Untreated, os acromiale can lead to progressive tendinopathy of the rotator cuff and gradually increasing shoulder instability and functional disability. In patients older than 50 years, progression of impingement often leads to rotator cuff tear.

Keywords Hawkins test; magnetic resonance imaging; Neer test; Os acromiale; shoulder pain rotator cuff tear; subacromial impingement syndrome; subacromial injection; tendinopathy; ultrasound guided injection; Yocum test

ICD-10 CODE M25.519

The Clinical Syndrome The subacromial space lies directly inferior to the acromion, the coracoid process, the acromioclavicular joint, and the coracoacromial ligament (Fig. 34.1). Lubricated by the subacromial bursa, the subacromial space in health is narrow and the anatomical structures surrounding it are responsible for maintaining static and dynamic shoulder stability. The space between the acromion and the superior aspect of the humeral head is the impingement interval, and abduction of the arm narrows the space further (Fig. 34.2). Any pathological condition that further narrows this space (e.g., osteophyte, abnormal acromial anatomy, ligamentous calcification, or congenital defects of the acromion) increases the incidence of impingement (Fig. 34.3). One such congenital defect is caused by failure of the distal ossification center of the acromion to fuse (see Fig. 34.1). This failure to fuse is termed os acromiale and essentially results in a second acromial joint. This “second joint” can lead to impingement syndromes and exacerbate shoulder instability.

FIG. 34.1 Os acromiale is a congenital defect caused by failure of the distal ossification center of the acromion to fuse.

FIG. 34.2 Patients with os acromiale complain of increasing shoulder pain with any activities that abduct or forward flex the shoulder.

Patients suffering from os acromiale have diffuse shoulder pain, with an associated feeling of weakness combined with loss of range of motion. Pain is often worse at night, and patients often complain that they are unable to sleep on the affected shoulder. The clinical presentation is usually insidious, without a clear-cut history of trauma to the affected shoulder. Affected patients tend to be younger than those with other causes of shoulder impingement syndromes. Untreated, os acromiale can lead to progressive tendinopathy of the rotator cuff and gradually increasing shoulder instability and functional disability. In patients older than 50 years, progression of impingement often leads to rotator cuff tear.

Signs and Symptoms A patient with os acromiale reports increasing shoulder pain with any activities that abduct or forward flex the shoulder, such as putting in a light bulb or reaching for dishes in a cabinet above shoulder height (see Fig. 34.2). Patients with os acromiale have positive tests for shoulder impingement, such as Neer and Hawkins tests (see Fig. 34.3). Although not completely diagnostic of os acromiale, a positive Neer or Hawkins test should prompt the examiner to order magnetic resonance imaging (MRI) of the affected shoulder to clarify and strengthen the diagnosis.

FIG. 34.3 Hawkins Test for Shoulder Impingement. From Waldman SD. Physical Diagnosis of Pain: An Atlas of Signs and Symptoms. Philadelphia: Saunders; 2006:72.

Testing MRI of the shoulder provides the best information regarding any pathological condition of the shoulder (Fig. 34.4). MRI is highly accurate and helps identify abnormalities that may put the patient at risk for continuing damage to the rotator cuff and the humeral head. MRI of the shoulder also helps rule out unsuspected pathology that may harm the patient, such as primary and metastatic tumors of the shoulder joint and surrounding structures. In patients who cannot undergo MRI, such as patients with pacemakers, computed tomography (CT) is a reasonable second choice. Radionuclide bone scanning and plain radiography are indicated if fracture or bony abnormality such as metastatic disease is considered in the differential diagnosis. Screening laboratory tests consisting of complete blood cell count, erythrocyte sedimentation rate, and automated blood chemistry testing should be performed if the diagnosis of subacromial impingement syndrome is in question. Arthrocentesis of the glenohumeral joint may be indicated if septic arthritis or crystal arthropathy is suspected.

FIG. 34.4 (A) Subaxial radiograph of an os acromiale (broken white arrow). The pseudoarticulation can be clearly seen (white arrow), and the lateral clavicle is outlined (dotted line). The axial gradient echo (B) and coronal oblique T1-weighted (C) MR images clearly demonstrate the os acromiale (white arrow). Care must be taken not to mistake an os acromiale for the acromioclavicular joint on coronal MR images. From Haaga JR, Lanzieri CF, Gilkeson RC, eds. CT and MR Imaging of the Whole Body. 4th ed. Philadelphia: Mosby; 2003:1955.

Differential Diagnosis Os acromiale is a clinical diagnosis supported by a combination of clinical history, physical examination, radiography, and MRI. Pain syndromes that may mimic os acromiale include subacromial impingement syndrome, subacromial bursitis, tendinopathy and tendinitis of the rotator cuff, calcification and thickening of the coracoacromial ligament, and arthritis affecting any of the shoulder joints. Adhesive capsulitis or frozen shoulder may confuse the diagnosis, as may idiopathic brachial plexopathy (Parsonage-Turner syndrome; see Chapter 25). Acromial stress fractures and undiagnosed clavicular fractures also may mimic the clinical presentation of os acromiale, as may impingement syndromes caused by aberrant subacromial blood vessels. Primary and metastatic tumors of the shoulder and surrounding structures are an ever-present possibility and should remain as part of the differential diagnosis of patients with shoulder pain.

Treatment Initial treatment of the pain and functional disability associated with os acromiale should include a combination of nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and gentle physical therapy. Local application of heat and cold also may be beneficial. For patients who do not respond to these treatment modalities, injection of the subacromial space with local anesthetic and steroid is a reasonable next step while obtaining MRI and other appropriate testing to clarify the working clinical diagnosis further. Ultrasound guidance may improve the accuracy of needle placement and decrease the incidence of needle-related complications. The use of physical therapy, including gentle range-of-motion exercises, should be introduced several days after the patient undergoes this injection technique for shoulder pain. Vigorous exercises should be avoided because they would exacerbate the symptoms. For patients who do not respond to these treatment modalities or have radiographically shown anatomical subacromial impingement that is producing ongoing damage to the rotator cuff, open or arthroscopic acromioplasty is required.

Complications and Pitfalls Failure to diagnose os acromiale correctly puts the patient at risk for the missed diagnosis of other syndromes, which may result in ongoing damage to the shoulder or lead to an overlooked pathological condition in this anatomical region that may harm the patient, such as Pancoast tumor or primary or metastatic tumors of the shoulder. MRI is indicated in all patients thought to have os acromiale, and aggressive treatment of surgically correctable causes of such impingement is generally indicated sooner rather than later to avoid ongoing irreversible shoulder damage.

Clinical Pearls The acromion has three distinct ossification centers: (1) the basiacromionmetacromion, which is most proximal; (2) the metacromion-mesoacromion, which is in the middle; and (3) the mesoacromion-preacromion, which is most distal. Lack of fusion of the mesoacromion-preacromion is responsible for the development of os acromiale. The musculotendinous unit of the shoulder joint is susceptible to the development of tendinitis for several reasons. First, the joint is subjected to a wide range of often repetitive motions. Second, the space in which the musculotendinous unit functions is restricted by the coracoacromial arch, making impingement a likely possibility with extreme movements of the joint. Third, the blood supply to the musculotendinous unit is poor, making healing of microtrauma more difficult. These factors can contribute to tendinitis of one or more of the tendons of the shoulder joint. Calcium deposition around the tendon may occur if the inflammation continues, making subsequent treatment more difficult. Tendinitis of the musculotendinous unit of the shoulder frequently coexists with bursitis of the associated bursae of the shoulder joint, creating additional pain and functional disability. Patients with untreated os acromiale continue to experience pain and functional disability and may continue to cause ongoing irreversible shoulder damage culminating in damage to the humeral head and rotator cuff tear.

Suggested Readings Barbier O, Block D, Dezaly C, et al. Os acromiale, a cause of shoulder pain, not to be overlooked. Orthop Traumatol Surg Res. 2013;99(4):465–472. Case D.T, Burnett S.E, Nielsen T. Os acromiale: population differences and their etiological significance. HOMO. 2006;57:1–18. Nicholson G.P, Goodman D.A, Flatow E.L, Bigliani LU. The acromion: morphologic condition and age-related changes—a study of 420 scapulas. J Shoulder Elbow Surg. 1996;5:1–11. Nissen C.W. The acromion: fractures and os acromiale. Oper Tech Sports Med. 2004;12:32–34. Pagnani M.J, Mathis C.E, Solman C.G. Painful os acromiale (or unfused acromial apophysis) in athletes. J Shoulder Elbow Surg. 2006;15(4):432–435. Waldman S.D, Campbell R.S.D. Os acromiale. In: Waldman S.D, Campbell R.S.D, eds. Imaging of Pain. Philadelphia: W.B. Saunders; 2011:229–230.

35

Glomus Tumor of the Shoulder

Abstract The diagnosis of glomus tumor of the shoulder is based primarily on three points in the patient’s clinical history: (1) excruciating pain that is localized to the area of the tumor, (2) ability to trigger the pain by palpating the area (Love’s test), and (3) marked intolerance to cold (Posner ’s cold induction test). Hildreth’s test is also useful in the diagnosis of glomus tumor. It is performed by placing a tourniquet proximal to the area of suspected tumor. As the distal area becomes ischemic, the sharp lancinating pain characteristic of glomus tumor will occur. If the tumor is superficial enough, the examiner may identify it beneath the skin. The patient with glomus tumor of the shoulder often will guard or protect the area of the tumor to avoid stimulating the pain.

Keywords Ganglion cyst; glomus tumor; impingement syndrome; magnetic resonance imaging; neoplasm; shoulder pain

ICD-10 CODE D18.00

The Clinical Syndrome Glomus tumor of the shoulder is an uncommon cause of shoulder pain. It is the result of tumor formation of the glomus body, which is a neuromyoarterial apparatus whose function is to regulate peripheral blood flow in the dermis. Glomus tumors occur most commonly in the subungual region of the fingers but may also occur in areas of the body that are not richly endowed with glomus apparatus (e.g., muscle, bone, blood vessels, nerves). Glomus tumors tend to be solitary, small tumors, but occasionally can become quite large. Most patients with glomus tumor are women 30 to 50 years of age. The pain associated with glomus tumor is severe, lancinating, and boring. Patients suffering from glomus tumor exhibit the classic triad of intermittent, excruciating pain, cold intolerance, and tenderness to palpation. If the tumor is located superficially, a bluish discoloration under the skin may be visible and the patient may experience an exacerbation of pain with exposure to cold. Because of the rarity of glomus tumor in areas other than the digits, diagnosis is often delayed.

Signs and Symptoms The diagnosis of glomus tumor of the shoulder is based primarily on three points in the patient’s clinical history: (1) excruciating pain that is localized to the area of the tumor, (2) ability to trigger the pain by palpating the area (Love test), and (3) marked intolerance to cold (Posner cold induction test). Hildreth test is also useful in the diagnosis of glomus tumor. It is performed by placing a tourniquet proximal to the area of suspected tumor. As the distal area becomes ischemic, the sharp lancinating pain characteristic of glomus tumor will occur. If the tumor is superficial enough, the examiner may identify it beneath the skin. The patient with glomus tumor of the shoulder often will guard or protect the area of the tumor to avoid stimulating the pain.

Testing Magnetic resonance imaging (MRI) of the affected area often reveals the actual glomus tumor and may reveal erosion or a perforating lesion of the phalanx beneath the tumor. The tumor appears as a very high and homogeneous signal on T2-weighted images (Fig. 35.1). The bony changes associated with glomus tumor of the shoulder also may appear on plain radiographs if a careful comparison of the corresponding contralateral shoulder is made. Radionuclide bone scan also may reveal localized bony destruction. If the tumor is superficial, pain may be reproduced by placing an ice pack over the affected area. Based on the patient’s clinical presentation, additional tests, including complete blood cell count, uric acid level, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. Electromyography is indicated if coexistent plexopathy or radiculopathy is suspected. Surgical exploration of the affected area bed often is necessary to confirm the diagnosis.

Differential Diagnosis The triad of localized, intermittent, lancinating excruciating pain, tenderness to palpation, and cold intolerance makes the diagnosis apparent to an astute clinician. Glomus tumor of the shoulder must be distinguished from other causes of localized shoulder pain. If a history of trauma is present, fracture, osteomyelitis, tenosynovitis, and foreign body synovitis should be considered. If there is no history of trauma, tumors or diseases of the glenohumeral joint and associated soft tissues should be considered. Reflex sympathetic dystrophy should be distinguishable from glomus tumor of the shoulder because the pain of reflex sympathetic dystrophy is less localized and is associated with distal trophic skin and nail changes and vasomotor and sudomotor abnormalities.

Treatment The mainstay of treatment of glomus tumor is surgical removal. Medication management is uniformly disappointing. Injection of the affected area in the point of maximal tenderness may provide temporary relief of the pain of glomus tumor and blocks Posner cold induction test response, further strengthening the diagnosis.

FIG. 35.1 Glomus Tumor of the ShoulderMR showing tail sign indicative of a lesion close to a neurovascular structure—suprascapular nerve. From Singh R, Malhotra A, Cribbz G, et al. Unusual lesions mimicking impingement syndrome in the shoulder joint—think medially. Ann Med Surg. 2016;10:88–91, fig 5. ISSN 2049-0801, https://doi:org/10.1016/j.amsu.2016.08.006. http://www.sciencedirect.com/science/article/pii/S2049080116301121.

Complications and Pitfalls The main complication associated with glomus tumor of the shoulder involves problems associated with delayed diagnosis, mainly ongoing destruction of the bone and soft tissues adjacent to the glomus tumor. Although usually localized and well encapsulated, and they rarely metastasize, these tumors can exhibit aggressive invasive tendencies making the complete excision of the tumor and careful follow-up mandatory.

Clinical Pearls The diagnosis of glomus tumor of the shoulder is usually straightforward if the clinician identifies the unique nature of its clinical presentation. Because of the rare potential for aggressive, invasive behavior, complete excision and careful follow-up are important.

Suggested Readings Abela M, Cole A.S, Hill G.A, Carr A.J. Glomus tumor of the scapular region. J Shoulder Elbow Surg. 2000;9:532–533. Boretto J.G, Lazerges C, Coulet B, Baldet P, Chammas M. Calcified glomus tumor of the shoulder: a case report. Chir Main. 2008;27:183–186. Ghaly R.F, Ring A.M. Supraclavicular glomus tumor, 20-year history of undiagnosed shoulder pain: a case report. Pain. 1999;83:379–382. Nebreda C.L, Urban B.J, Taylor A.E. Upper extremity pain of 10 years duration caused by a glomus tumor. Reg Anesth Pain Med. 2000;25:69–71. Roberts S.N, Carter C, Brown J.N, Hayes M.G, Saies A. Enormous glomus tumor of the shoulder. J Shoulder Elbow Surg. 1999;8:365–366. Singh R, Malhotra A, Cribb G, et al. Unusual lesions mimicking impingement syndrome in the shoulder joint—think medially. Ann Med Surg. 2016;10:88– 91. Yoshikawa I, Murakami M, Ishizawa M, Matsumoto K, Hukuda S. Glomus tumor of the musculotendinous junction of the rotator cuff. Clin Orthop. 1996;326:250–253.

36

Pectoralis Major Tear Syndrome

Abstract The clinical presentation of pectoralis major tear syndrome is varied because of its several causes, with the severity of symptoms directly proportional to the amount of trauma sustained by the muscle, its tendons, or both. Patients with pectoralis major tear syndrome present with the acute onset of anterior chest wall pain after trauma to the muscle sustained while performing activities such as bench pressing or rappelling down cliffs. The severity of pain is proportional to the amount of trauma sustained. A patient with pectoralis muscle tear syndrome also may complain of varying degrees of weakness with internal rotation of the humerus. If complete tear of the muscle or rupture of the tendon occurs, the anterior chest wall bulges acutely with contraction of the muscle in a manner analogous to the Popeye’s bulge of Ludington’s sign associated with rupture of the biceps tendon. If the rupture is not repaired promptly, further muscle retraction and calcification occur, worsening the functional disability and cosmetic deformity.

Keywords chest wall pain; diagnostic ultrasound; magnetic resonance imaging; Pectoralis major rupture; pectoralis major tear; seat belt injury; Sports injury; weight lifiting

Pectoralis Muscle Tear ICD-10 CODE S46.919A

Pectoralis Muscle Tendon Rupture ICD-10 CODE T14.90

The Clinical Syndrome The pectoralis major muscle is susceptible to trauma ranging from microscopic tears of the muscle substance after heavy exertion to macroscopic partial tearing of the muscle or, in extreme cases, full-thickness tearing with associated hematoma formation and cosmetic deformity (Figs 36.1–36.3). Additionally, the pectoralis major tendon can rupture at its point of insertion into the crest of the greater tubercle of the humerus (Fig. 36.4). A broad, thick, fan-like muscle, the pectoralis major arises from the anterior surface of the proximal clavicle, the anterior surface of the sternum, the cartilaginous attachments of the second through sixth and occasionally seventh ribs, and the aponeurotic band of the obliquus externus abdominis. These muscle fibers overlap, with some running upward and laterally, others running horizontally, and others running downward and laterally, all ending in a broad flat tendon that inserts into the crest of the greater tubercle of the humerus. The clinical presentation of pectoralis major tear syndrome is varied because of its several causes, with the severity of symptoms directly proportional to the amount of trauma sustained by the muscle, its tendons, or both. Patients with pectoralis major tear syndrome present with the acute onset of anterior chest wall pain after trauma to the muscle sustained while performing activities such as bench pressing or rappelling down cliffs (Fig. 36.5). The severity of pain is proportional to the amount of trauma sustained. A patient with pectoralis muscle tear syndrome also may complain of varying degrees of weakness with internal rotation of the humerus. If complete tear of the muscle or rupture of the tendon occurs, the anterior chest wall bulges acutely with contraction of the muscle in a manner analogous to the Popeye’s bulge of Ludington sign associated with rupture of the biceps tendon (Figs 36.6 and 36.7). If the rupture is not repaired promptly, further muscle retraction and calcification occur, worsening the functional disability and cosmetic deformity.

FIG. 36.1 Microscopic Tear of the Pectoralis Muscle With Mild Bleeding and Slight Edema.

FIG. 36.2 Full-Thickness Tear of the Pectoralis Muscle With Associated Hematoma Formation and Cosmetic Deformity.

FIG. 36.3 Clinical image of an acute (2 weeks) left pectoralis major rupture demonstrate swelling and medialization of the muscle belly. Ecchymosis has resolved. Also noted is a “dropped nipple sign,” which is associated with pectoralis major rupture. From Haley CA, Zacchilli MA. Pectoralis major injuries: evaluation and treatment. Clin Sports Med. 2014;33[4]:739–756, fig. 3. https://doi.org/10.1016/j.csm.2014.06.005. http://www.sciencedirect.com/science/article/pii/S0278591914000544.

FIG. 36.4 Pectoralis major tendon rupture at its point of insertion into the crest of the greater tubercle of the humerus.

Signs and Symptoms A patient with pectoralis major tear syndrome complains of the acute onset of pain in the anterior chest after trauma to the pectoralis major muscle, tendon, or both. If the trauma is significant, hematoma formation is clearly visible. With rupture of the tendon at its insertion site into the humerus, impressive ecchymosis of the arm and anterior chest wall that may seem out of proportion to the amount of trauma perceived by the patient is present. Active internal rotation of the humerus against examiner resistance may reveal weakness. If significant disruption of the muscle or rupture of the tendon occurs, the patient is unable to reach behind his or her back (Fig. 36.8). As mentioned previously, if complete tear of the muscle or rupture of the tendon occurs, the anterior chest wall bulges with contraction of the pectoralis major against the unopposed torn distal muscle, tendon, or both. Although not completely diagnostic of pectoralis major tear syndrome, this physical finding should prompt the examiner to order magnetic resonance imaging (MRI) of the affected proximal humerus and shoulder and anterior chest wall to further clarify and strengthen the diagnosis.

FIG. 36.5 Patients with pectoralis major tear syndrome present with acute onset of anterior chest wall pain after trauma to the muscle sustained while performing activities such as bench pressing.

FIG. 36.6 (A) Resting axial gradient-recalled echo (GRE) MRI in a patient with a known pectoralis tendon tear adjacent to the left humerus at rest shows mild asymmetry of the pectoralis major muscles, with apparent discontinuity of the left pectoralis major muscle at the axillary line (arrow). (B) Axial GRE MRI in the same patient with sustained maximal contraction of the injured muscle shows a prominent

bulge in the medial aspect of the left pectoralis major muscle (arrow). From Edelman RR, Hesselink JR, Zlatkin MB, et al, eds. Clinical Magnetic Resonance Imaging. 3rd ed. Philadelphia: Saunders; 2006: 3468.

FIG. 36.7 Popeye’s Sign Associated With Rupture of the Biceps Tendon. From Waldman SD. Physical Diagnosis of Pain: An Atlas of Signs and Symptoms. Philadelphia: Saunders; 2006: 83.

Testing MRI and ultrasound imaging of the shoulder, proximal humerus, and anterior chest wall provides the best information regarding pathological processes of these anatomical regions. Both MRI and ultrasound imaging are highly accurate and help identify abnormalities that may require urgent surgical repair, such as large complete muscle tears, tendon rupture, or both. MRI and ultrasound imaging of the affected anatomy also helps the clinician rule out unsuspected pathological conditions that may harm the patient, such as primary and metastatic tumors. In patients who cannot undergo MRI, such as patients with pacemakers, computed tomography (CT) is a reasonable second choice. Radionuclide bone scanning and plain radiography are indicated if fracture or bony abnormality such as metastatic disease of the proximal humerus, shoulder, or anterior chest wall is being considered in the differential diagnosis. Screening laboratory tests consisting of complete blood cell count, erythrocyte sedimentation rate, and automated blood chemistry testing should be performed if the diagnosis of pectoralis major tear syndrome is in question.

FIG. 36.8 Active Internal Rotation of the Humerus May Reveal WeaknessIf significant disruption of the muscle or rupture of the tendon occurs, the patient is unable to reach behind his or her back. From Waldman SD. Physical Diagnosis of Pain: An Atlas of Signs and Symptoms. Philadelphia: Saunders; 2006:50.

Differential Diagnosis Pectoralis major tear syndrome is a clinical diagnosis supported by a combination of clinical history, physical examination, radiography, and MRI. Pain syndromes that may mimic pectoralis major tear syndrome include injuries to the pectoralis minor, subscapularis, or latissimus dorsi muscles and inferior glenohumeral ligament injuries. Dislocation of the manubrium from the body of the sternum after acceleration/deceleration injuries also may confuse the diagnosis. Fractures of all the bony origins of the pectoralis major muscles (e.g., the sternum and ribs and fractures of the anatomical or surgical neck of the humerus) may mimic the clinical presentation of pectoralis major tear syndrome. Primary and metastatic tumors of the shoulder, humerus, and anterior chest wall and their surrounding structures remain an ever-present possibility and should be included as part of the differential diagnosis of patients with symptoms thought to result from pectoralis major tear syndrome.

Treatment Although the pain and functional disability associated with mild microscopic tears of the pectoralis major muscle may be treated conservatively with a combination of the nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and gentle physical therapy, more extensive tears and rupture of the pectoralis major tendon require urgent surgical repair if permanent cosmetic deformity and functional disability are to be avoided.

Complications and Pitfalls Failure to diagnose pectoralis major tear syndrome correctly puts the patient at risk for the missed diagnosis of other syndromes that may result in ongoing damage to the shoulder or lead to overlooked pathological processes in this anatomical region that may harm the patient, such as Pancoast tumor or primary or metastatic tumors of the shoulder, humerus, or anterior chest wall. MRI is indicated in all patients thought to have pectoralis major tear syndrome, and aggressive treatment of surgically correctable causes of the symptoms is indicated on an urgent basis to avoid irreversible cosmetic deformity and functional disability.

Clinical Pearls Pectoralis major tear syndrome is an uncommon but easily recognized cause of anterior chest wall and shoulder pain. A patient with complete pectoralis major muscle tear, tendon rupture, or both may present with hematoma and ecchymosis formation that seems out of proportion to the patient’s perception of the amount of trauma sustained; the patient often requires reassurance that he or she will not bleed to death. On rare occasions, the pectoralis major may rupture at its sternal origin. Regardless of the site of rupture, patients with complete rupture of the pectoralis major musculotendinous unit should undergo urgent surgical repair and careful postoperative rehabilitation to avoid permanent cosmetic deformity and functional disability.

Suggested Readings Beloosesky Y, Grinblat J, Katz M, Hendel D, Sommer R. Pectoralis major rupture in the elderly: clinical and sonographic findings. Clin Imaging. 2003;27:261–264. Butt U, Mehta S, Funk L, Monga P. Pectoralis major ruptures: a review of current management. J Shoulder Elbow Surg. 2015;24(4):655–662. Chang E.S, Zou J, Costello J.M, Lin A. Accuracy of magnetic resonance imaging in predicting the intraoperative tear characteristics of pectoralis major ruptures. J Shoulder Elbow Surg. 2016;25(3):463–468. Cordasco F.A, Mahony G.T, Tsouris N, Degen R.M. Pectoralis major tendon tears: functional outcomes and return to sport in a consecutive series of 40 athletes. J Shoulder Elbow Surg. 2017;26(3):458–463. El Maraghy A.R, Devereaux M.W. A systematic review and comprehensive classification of pectoralis major tears. J Shoulder Elbow Surg. 2012;21:412– 422. Haley C.A, Zacchilli M.A. Pectoralis major injuries: evaluation and treatment. Clin Sports Med. 2014;33(4):739–756. Keizer A.A, Leferink V.J. Pectoralis major tear at its sternal origin after a seatbelt trauma. J Plast Reconstr Aesthet Surg. 2015;68(12):e205–e206. Weaver J.S, Jacobson J.A, Jamadar DA, et al. Sonography of the pectoralis major tear. Ultrasound Med Biol. 2003;29:374–383 S15.

37

Suprascapular Nerve Entrapment

Abstract The most important finding in patients with suprascapular nerve entrapment is weakness of the supraspinatus and infraspinatus muscles. This weakness manifests itself as weakness of abduction and external rotation of the ipsilateral shoulder. With significant compromise of the suprascapular nerve, atrophy of the infraspinatus muscle is apparent as it lies superficially. The pain of suprascapular nerve entrapment can be exacerbated by abducting the ipsilateral scapula by reaching across the chest and simultaneously rotating the neck away from the involved shoulder. Tenderness to palpation of the suprascapular notch is often present.

Keywords diagnostic ultrasound; magnetic resonance imaging; shoulder pain cervical radiculopathy; sports injuries; Suprascapular nerve entrapment; suprascapular nerve

ICD-10 CODE G58.9

The Clinical Syndrome Suprascapular nerve entrapment is an uncommon cause of shoulder pain that is being encountered more frequently in clinical practice with the increasing use of backpacks instead of briefcases. Suprascapular nerve entrapment syndrome is caused by compression of the suprascapular nerve as it passes through the suprascapular notch. The most common causes of compression of the suprascapular nerve at this anatomical location include the prolonged wearing of heavy backpacks and direct blows to the nerve such as occur in football injuries and in falls from trampolines (Fig. 37.1). Suprascapular nerve entrapment syndrome also is seen in baseball pitchers and quarterbacks. This entrapment neuropathy manifests most commonly as a severe, deep, aching pain that radiates from the top of the scapula to the ipsilateral shoulder. Tenderness over the suprascapular notch is usually present. Shoulder movement, especially reaching across the chest, may increase the pain. Untreated, weakness and atrophy of the supraspinatus and infraspinatus muscles occur.

Signs and Symptoms The most important finding in patients with suprascapular nerve entrapment is weakness of the supraspinatus and infraspinatus muscles. This weakness manifests itself as weakness of abduction and external rotation of the ipsilateral shoulder. With significant compromise of the suprascapular nerve, atrophy of the infraspinatus muscle is apparent as it lies superficially. The pain of suprascapular nerve entrapment can be exacerbated by abducting the ipsilateral scapula by reaching across the chest and simultaneously rotating the neck away from the involved shoulder. Tenderness to palpation of the suprascapular notch is often present.

FIG. 37.1 Suprascapular nerve entrapment is caused by compression of the suprascapular nerve as it passes through the suprascapular notch. m, Muscle.

Testing Electromyography helps distinguish cervical radiculopathy and ParsonageTurner syndrome from suprascapular nerve entrapment syndrome. Plain radiographs are indicated in all patients who present with suprascapular nerve entrapment syndrome to rule out occult bony pathology. Ultrasound imaging may also aid in the identification of this uncommon cause of shoulder pain (Fig. 37.2). Based on the patient’s clinical presentation, additional testing, including complete blood cell count, uric acid level, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. Magnetic resonance imaging (MRI) of the shoulder is indicated if a primary joint pathological process or space-occupying lesion is suspected (Fig. 37.3). The injection technique described here is a diagnostic and therapeutic maneuver.

FIG. 37.2 Transverse color Doppler image of the scapular notch spinoglenoid ligament, the suprascapular artery, and the suprascapular nerve. Image property Steven D. Waldman, MD.

Differential Diagnosis Suprascapular nerve entrapment syndrome is often misdiagnosed as bursitis, tendinitis, or arthritis of the shoulder. Cervical radiculopathy of the C5 nerve root also may mimic the clinical presentation of suprascapular nerve entrapment syndrome. Parsonage-Turner syndrome, also known as idiopathic brachial neuritis, may manifest as sudden onset of shoulder pain and can be confused with suprascapular nerve entrapment. Tumor involving the superior scapular nerve, shoulder, or both also should be considered in the differential diagnosis of suprascapular nerve entrapment syndrome.

Treatment Nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors represent a reasonable first step in the treatment of suprascapular nerve entrapment syndrome. The use of tricyclic antidepressants, such as nortriptyline, at a single bedtime dose of 25 mg titrating upward as side effects allow also is useful, especially if sleep disturbance also is present. Avoidance of repetitive trauma thought to be contributing to this entrapment neuropathy also is important, especially in professional athletes. If these maneuvers fail to produce rapid symptomatic relief, injection of the suprascapular nerve with local anesthetic and steroid is a reasonable next step (Fig. 37.4). Ultrasound guidance may improve the accuracy of needle placement and a decrease in needle-related complications. If symptoms persist, surgical exploration and release of the suprascapular nerve are indicated.

FIG. 37.3 (A) Coronal oblique T2W with fat suppression (FST2W) MR image of a patient with a high signal intensity (SI) paralabral cyst in the spinoglenoid notch (white arrow). (B) A sagittal oblique FST2W MR image shows the cyst. There is also high SI within the infraspinatus muscle belly (broken white arrows) in comparison with the supraspinatus, teres minor, and deltoid muscle bellies. This finding indicates muscle denervation edema due to compression of the suprascapular nerve by the cyst. D, Deltoid; SST, supraspinatus; TM, teres minor. Image courtesy Dr. Phil Robinson, Leeds, United Kingdom.

FIG. 37.4 Injection of Suprascapular Nerve for Relief of Painm, Muscle. From Waldman SD. Atlas of Pain Management Injection Techniques. Philadelphia: Saunders; 2000:169.

Complications and Pitfalls The proximity to the suprascapular artery and vein suggests the potential for inadvertent intravascular injection or local anesthetic toxicity from intravascular absorption or both. The clinician should carefully calculate the total milligram dosage of local anesthetic that may be given safely when performing this injection technique. Because of proximity of the lung, if the needle is advanced too deeply through the suprascapular notch, pneumothorax is possible. Care must be taken if surgical decompression of the suprascapular nerve is undertaken to avoid inadvertent trauma to the spinal accessory nerve as it runs along the ventral surface of the trapezius.

Clinical Pearls Avoidance techniques of the repetitive movements responsible for suprascapular nerve entrapment are often forgotten in the rush to treatment. The use of rolling briefcases instead of backpacks may help avoid continued trauma to the nerve. This injection technique renders the shoulder joint insensate. It is important that the clinician ensures that the physical and occupational therapists caring for a patient who has undergone suprascapular nerve block understand that the shoulder girdle as well as the shoulder joint have been rendered insensate after this injection technique. Deep heat modalities and range-of-motion exercises must be monitored carefully to avoid burns or damage to the shoulder.

Suggested Readings Agrawal D, Singh B, Dixit S.G, et al. Morphometry and variations of the human suprascapular notch. Morphologie. 2015;99(327):132–140. Blum A, Lecocq S, Louis M, et al. The nerves around the shoulder. Eur J Radiol. 2013;82(1):2–16. Fehrman D.A, Orwin J.F, Jennings R.M. Suprascapular nerve entrapment by ganglion cysts: a report of six cases with arthroscopic findings and review of the literature. Arthroscopy. 1995;11:727–736. Moore T.P, Hunter R.E. Suprascapular nerve entrapment. Oper Tech Sports Med. 1996;4:8–14. Plancher K.D, Petterson S.C. Posterior shoulder pain and arthroscopic decompression of the suprascapular nerve at the transverse scapular ligament. Oper Tech Sports Med. 2014;22(1):58–72. Toussaint C.P, Zager E.L. What’s new in common upper extremity entrapment neuropathies. Neurosurg Clin North Am. 2008;19:573–581. Waldman S.D. Suprascapular nerve block. In: Waldman S.D, ed. Pain Review. 2nd ed. Philadelphia: Elsevier; 2017:460–462. Waldman S.D. Suprascapular nerve block. In: Waldman S.D, Campbell R.S.D, eds. Imaging of Pain. Philadelphia: Saunders; 2011:256–259.

38

Snapping Scapula Syndrome

Abstract Snapping scapula syndrome, which is also known as jumped shoulder blade occurs when there is dysfunction of the normal smooth sliding of the concave anterior scapula over the convex posterior thorax. When this abnormal scapulothoracic motion occurs, a snapping, cracking, grating, or thumping sound in the region of the superiomedial border of the scapula occurs. These sounds can be quite distressing to the patient and those in proximity as they can be very loud as the sounds are amplified by the patient’s air containing thorax. While there are numerous causes of snapping scapula syndrome, the etiopathology of snapping scapula syndrome can be divided into five major categories: (1) scapulothoracic bursitis; (2) muscular abnormalities; (3) soft issue abnormalities; (4) bony abnormalities, and (5) idiopathic.

ICD-10 CODE M89.8X1

The Clinical Syndrome An uncommon cause of shoulder pain, snapping scapula syndrome, which is also known as jumped shoulder blade, occurs when there is dysfunction of the normal smooth sliding of the concave anterior scapula over the convex posterior thorax. When this abnormal scapulothoracic motion occurs, a snapping, cracking, grating, or thumping sound in the region of the superomedial border of the scapula occurs. These sounds can be quite distressing to the patient and those in proximity, as they can be very loud, as the sounds are amplified by the patient’s air-containing thorax. While there are numerous causes of snapping scapula syndrome, the etiopathology of snapping scapula syndrome can be divided into five major categories: (1) scapulothoracic bursitis; (2) muscular abnormalities; (3) soft issue abnormalities; (4) bony abnormalities; and (5) idiopathic (Table 38.1, Fig. 38.1).

Signs and Symptoms The onset of snapping scapular syndrome may be acute or chronic depending on the etiopathology of the syndrome. Common acute inciting factors are overhead activities such as painting a ceiling, overhand throwing, swimming, repetitive overuse of the shoulder (e.g., raking leaves, pushups), and scapulothoracic trauma (Fig. 38.2). However, regardless of the cause, it is invariably the abnormal sound on shoulder movement rather than the associated pain that prompts the patient to seek medical attention. Descriptions of sounds associated with snapping scapula syndrome include snapping, cracking, grating, thumping, clinking, crunching, and popping. These sounds can be quite loud, as they are amplified by the air-filled thorax. The intensity of the pain associated with snapping scapula syndrome ranges from irritating to excruciating. On physical examination, crepitus, and at times a grating sensation, can be appreciated on palpation as the scapula is moved anteriorly. This sensation can be magnified if the examiner exerts firm pressure to the superior angle of the scapula during movement. Tenderness to palpation, most commonly at the superomedial border and inferior pole of the scapula, is present. Pain and scapular snapping are often reproduced by moving the affected extremity into the abducted position and then having the patient abduct the affected shoulder and then fully externally rotate the affected extremity with the thumb in a hitchhiking position. This maneuver is known as the armed arm test and the test is positive if the maneuver reproduces the patient’s pain and elicits an audible snapping sound (Fig. 38.3). Pain and scapular snapping can be reduced by having the patient cup the hand of the affected extremity over the contralateral shoulder (Fig. 38.4). This maneuver effectively lifts the affected scapula away from the underlying thorax. If there is significant bursitis and associated edema, pseudo-winging of the scapula may be present and compression of the scapula may produce a boggy sensation. If there is neural compromise, true winging of the scapula may be present (Fig. 38.5).

TABLE 38.1 Etiopathology of Snapping Scapula Syndrome Scapular Bursitis Overuse injury • Reactive bursitis of the infraserratus bursa • Reactive bursitis of the supraserratus bursa • Reactive bursitis of the scaphotrapezial bursa Chronic Bursitis • Scarring of bursae and periscapular musculature • Fibrosis • Impingement • Pain • Snapping sound Muscular Abnormalities • Congenital muscle abnormalities • Abnormal muscle insertions • Periscapular muscle avulsions • Muscular injury • Muscular weakness • Muscle atrophy • Post-inflammatory muscular fibrosis • Post-traumatic muscular fibrosis Soft Tissue Lesions • Elastoma dorsi • Lipomas • Glomus tumors • Chondrosarcomas • Osteochondromas Bony Abnormalities • Scapular anatomic variations • Winged scapula • Pseudo-winged scapula • Scapular fractures • Hypertrophic callus formation • Displacement of fracture • Malunion of healing fracture • Abnormal Luschka tubercle • Fractured posterior ribs • Hypertrophic callus formation • Displacement of fracture • Malunion of healing fracture • Osteophyte formation • Excessive thoracic kyphosis • Scoliosis • Joint hypermobility syndrome • Glenohumeral joint dysfunction • Idiopathic

FIG. 38.1 How untreated reactive scapulothoracic bursitis can progress to snapping scapula syndrome.

Testing Electromyography may help identify neural compromise (e.g., damage to the long thoracic nerve of Bell), although the test may be normal in mild cases even though significant neurapraxia is present (see Fig. 38.5). Plain radiographs of the scapula, posterior ribs, and shoulder are indicated in all patients who present with snapping scapula syndrome to rule out fractures and occult bony pathological processes. Based on the patient’s clinical presentation, additional testing, including complete blood cell count, uric acid level, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. MRI and ultrasound imaging of the shoulder and scapula is indicated in all patients thought to have snapping scapula syndrome, given the large range of pathological processes responsible for this uncommon cause of shoulder pain (Fig. 38.6). Computerized tomography (CT) may also help identify bony abnormalities responsible for the patient’s symptomatology (Fig. 38.7).

FIG. 38.2 The pain and snapping sound associated with snapping scapula syndrome occur with movement of the scapula, especially during overhead activities.

FIG. 38.3 The armed arm test is performed by having the patient fully abduct the affected shoulder and then fully externally rotating the affected extremity with the thumb in a hitchhiking position. The test is positive if the maneuver reproduces the patient’s pain and elicits an audible snapping sound.

FIG. 38.4 Pain and scapular snapping can be reduced by having the patient cup the hand of the affected extremity over the contralateral shoulder.

FIG. 38.5 A Patient Who Experienced a Winged Scapula as a Result of Inadvertent Injury to the Long Thoracic Nerve. From Waldman SD. Atlas of Pain Management Injection Techniques. 3rd ed. Philadelphia: Elsevier; 2013.

Differential diagnosis Snapping syndrome is often initially misdiagnosed as bursitis, tendinitis, or arthritis of the shoulder. Parsonage-Turner syndrome, or idiopathic brachial neuritis, also may manifest as sudden onset of shoulder pain and can confuse the clinical picture. Tumor involving this anatomical region also should always be considered in the differential diagnosis of snapping scapula syndrome, as should occult fractures of the scapula and other mass lesions such as large bursa, cysts, and lipomas.

Treatment Nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors represent a reasonable first step in the treatment of mild, selflimited snapping scapula syndrome. The use of tricyclic antidepressants, such as nortriptyline, at a single bedtime dose of 25 mg, titrating upward as side effects allow, also is useful, especially if sleep disturbance is present. Avoidance of repetitive trauma thought to be contributing to this uncommon pain syndrome also is important, especially in professional athletes. If these maneuvers fail to produce rapid symptomatic relief, local injection of the superomedial and inferior pole of the affected scapula and any inflamed bursae with local anesthetic and steroid is a reasonable next step (Figs. 38.8 and 38.9) For persistent cases, arthroscopic debridement of the anterior scapula may be indicated.

FIG. 38.6 (A) An axial short tau inversion recovery sequence of the proximal right scapula of a 3.0-T magnetic resonance imaging (MRI) scan showing a straight scapula bony morphology, which was defined as a type I scapula. (B) Sample of an axial MRI scan, using the short tau inversion recovery sequence, of a left scapula showing a wave-shaped scapula bony morphology, which represents a type II scapula. (C) Sample of an axial MRI scan, using the proton density fast spin echo fat-suppressed sequence, of a left scapula showing a concave superior scapula corpus. This morphology was defined as a type III scapula. All type III scapulae were associated with snapping scapula syndrome. From Spiegl UJ, Petri M, Smith SW, et al. Association between scapula bony morphology and snapping scapula syndrome. J Shoulder Elbow Surg. 2015;24[8]:1289–1295. https://doi.org/10.1016/j.jse.2014.12.034. http://www.sciencedirect.com/science/article/pii/S1058274615000208.

FIG. 38.7 Computed tomographic scan of the chest wall, demonstrating malunited fractures of ribs 3 to 8. From Ten Duis K, IJpma FF. Surgical treatment of snapping scapula syndrome due to malunion of rib fractures. Ann Thorac Surg. 2017;103[2]:e143–e144, fig 2. https://doi.org/10.1016/j.athoracsur.2016.07.061. http://www.sciencedirect.com/science/article/pii/S000349751630964X.

Complications and Pitfalls Failure to diagnose snapping scapula syndrome correctly puts the patient at risk for the missed diagnosis of other syndromes that may result in ongoing damage to the shoulder or lead to overlooked pathological processes in this anatomical region that may harm the patient, such as primary or metastatic tumors of the shoulder, posterior chest wall, and scapula. MRI and ultrasound imaging are indicated in all patients thought to have snapping scapula, and aggressive treatment of surgically correctable causes is generally indicated sooner rather than later to avoid ongoing irreversible shoulder damage.

Clinical Pearls Avoidance techniques of the repetitive movements responsible for snapping scapula syndrome often are forgotten in the rush to treatment. Mild cases of snapping scapula syndrome are usually self-limited, but more severe cases may require injection with local anesthetic and steroid. Occasionally, surgical intervention is indicated. As with other uncommon pain syndromes, snapping scapula syndrome should be considered a diagnosis of exclusion and the clinician should ensure that no potentially harmful occult spaceoccupying lesions are present before attributing symptoms to other benign causes.

FIG. 38.8 Location of scapular bursal spaces in posterior (A) and superior (B) cross-sectional views. Modified from Kuhne M, Boniquit N, Ghodadra N, et al. The snapping scapula: diagnosis and treatment. Arthroscopy. 2009;25[11]:1298–1311, fig 3. https://doi.org/10.1016/j.arthro.2008.12.022. http://www.sciencedirect.com/science/article/pii/S0749806309000346.

FIG. 38.9 The Two Common Locations Of Scapular Bursal Injections Are At The Superomedial Border And At The Inferior Tip. Modified from Kuhne M, Boniquit N, Ghodadra N, et al. The snapping scapula: diagnosis and treatment. Arthroscopy. 2009;25[11]:1298–1311, fig 5. https://doi.org/10.1016/j.arthro.2008.12.022.http://www.sciencedirect.com/science/article/pii/S0749806309

Suggested Readings Abat F, Trullols L, Álvarez C, et al. The snapping scapula as a symptom of a tumour in the scapulothoracic region. Rev Esp Cir Ortop Traumatol. 2013;57(2):123–128 ISSN 19888856. https://doi.org/10.1016/j.recote.2013.04.002. Clarke D.O, Crichlow A, Christmas M, et al. The unusual osteochondroma: a case of snapping scapula syndrome and review of the literature. Orthop Traumatol Surg Res. 2017;103(8):1295–1298. Kadri F, Konzelmann M. Joint hypermobility syndrome must be investigated in case of snapping scapula syndrome. Ann Phys Rehabil Med. 2015;58(suppl1):e42. Menge T.J, Horan M.P, Tahal D.S, et al. Arthroscopic treatment of snapping scapula syndrome: outcomes at minimum of 2 years. Arthroscopy. 2017;33(4):726–732. Patzkowski J.C, Owens B.D, Burns T.C. Snapping scapula syndrome in the military. Clin Sports Med. 2014;33(4):757–766. Saper M, Kasik C, Dietzel D. Arthroscopic scapulothoracic decompression for snapping scapula syndrome. Arthroscopy Tech. 2015;4(6):e631–e636. Spiegl U.J, Petri M, Smith S.W, et al. Association between scapula bony morphology and snapping scapula syndrome. J Shoulder Elbow Surg. 2015;24(8):1289–1295. Ten Duis K, IJpma F.F.A. Surgical treatment of snapping scapula syndrome due to malunion of rib fractures. Ann Thor Surg. 2017;103(2):e143–e144.

39

Quadrilateral Space Syndrome

Abstract Quadrilateral space syndrome is caused by compression of the axillary nerve as it passes through the quadrilateral space. The onset of quadrilateral space syndrome is usually insidious, with the patient often not reporting any obvious antecedent trauma. A patient suffering from quadrilateral space syndrome complains of ill-defined pain in the shoulder and paresthesias radiating into the posterior upper arm and lateral shoulder. This pain and associated paresthesias frequently are worsened with abduction and external rotation of the affected upper extremity. As the syndrome progresses, the patient may note increasing weakness of the affected arm and difficulty with abduction and external rotation. Most cases of quadrilateral space syndrome have occurred in young athletes in their early second decade to third decade who are involved in throwing activities.

Keywords axillary nerve; electromyography; entrapment neuropathy; neuropraxia; Quadrilateral space syndrome; quadrilateral space; subclavian arteriography

ICD-10 CODE G58.9

The Clinical Syndrome Quadrilateral space syndrome is an uncommon cause of shoulder and posterior upper arm pain first described by Cahill and Palmer in 1983. It is now being encountered more frequently in clinical practice because magnetic resonance imaging (MRI) makes confirmation of the clinical diagnosis much easier than the previously required arteriography of the shoulder and upper extremity. Quadrilateral space syndrome is caused by compression of the axillary nerve as it passes through the quadrilateral space (Figs. 39.1 and 39.2). The onset of quadrilateral space syndrome is usually insidious, with the patient often not reporting any obvious antecedent trauma. A patient suffering from quadrilateral space syndrome complains of ill-defined pain in the shoulder and paresthesias radiating into the posterior upper arm and lateral shoulder. This pain and associated paresthesias frequently are worsened with abduction and external rotation of the affected upper extremity. As the syndrome progresses, the patient may note increasing weakness of the affected arm and difficulty with abduction and external rotation. Most cases of quadrilateral space syndrome have occurred in young athletes in their early second to third decade who are involved in throwing activities (Fig. 39.3). The syndrome may be seen occasionally in older patients as a result of other causes of compression of the axillary nerve as it travels through the quadrilateral space, such as glenolabral cysts or tumor. Mild cases of quadrilateral space syndrome resolve over time, but more severe cases, if left untreated, result in permanent atrophy of the deltoid and teres minor muscles (Fig. 39.4).

FIG. 39.1 Anatomy of Axillary Nerve As It Travels Through the Quadrilateral Space.

FIG. 39.2 The Axillary Nerve and Posterior Circumflex Humeral Artery Course Posteriorly Through the Quadrilateral Space. From Zurkiya O, Walker TG. Quadrilateral space syndrome. J Vasc Interv Radiol. 2014;25[2]:229, fig 1.

FIG. 39.3 Most cases of quadrilateral space syndrome have occurred in young athletes in their early second to third decade who are involved in throwing activities.

Signs and Symptoms The most important finding in patients with quadrilateral space syndrome is weakness of the supraspinatus and infraspinatus muscles. This manifests as weakness of abduction and external rotation of the ipsilateral shoulder. With significant compromise of the axillary nerve, atrophy of the deltoid and teres minor muscle is apparent on physical examination. The pain of quadrilateral space syndrome can be exacerbated by abducting and externally rotating the ipsilateral upper extremity. Tenderness to palpation of the quadrilateral space often is present.

FIG. 39.4 If left untreated, quadrilateral space syndrome may result in permanent atrophy of the deltoid and teres minor muscles (arrow). From Zurkiya O, Walker TG. Quadrilateral space syndrome. J Vasc Interv Radiol. 2014;25[2]:229, fig 2.

Testing Electromyography may help identify entrapment of the axillary nerve, although the test may be normal in mild cases even though significant neurapraxia is present. Electromyography helps distinguish cervical radiculopathy and Parsonage-Turner syndrome from quadrilateral space syndrome. Plain radiographs are indicated in all patients who present with quadrilateral space syndrome to rule out occult bony pathological processes. Based on the patient’s clinical presentation, additional testing, including complete blood cell count, uric acid level, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. MRI and ultrasound imaging of the shoulder is indicated in all patients thought to have quadrilateral space syndrome because this test is highly specific for this disorder (Figs. 39.5 and 39.6). In the rare patient in whom MRI is nondiagnostic, subclavian arteriography to show occlusion of the posterior humeral circumflex artery may be considered because this finding is highly suggestive of a diagnosis of quadrilateral space syndrome (Figs. 39.7 and 39.8).

Differential Diagnosis Quadrilateral space syndrome is often initially misdiagnosed as bursitis, tendinitis, or arthritis of the shoulder. Cervical radiculopathy of the lower nerve roots also may mimic the clinical presentation of quadrilateral space syndrome.

FIG. 39.5 (A) Sagittal oblique T1 magnetic resonance image medial to the suprascapular notch shows teres minor (TM) muscle atrophy. (B) Sagittal oblique T1 magnetic resonance image in the plane of the quadrilateral space (QS). Infra, infraspinatus; Subsc, subscapularis, Supra, supraspinatus. From Flynn LS, Wright TW, King JJ:L Quadrilateral space syndrome: a review. J Shoulder Elbow Surg 27(5);2018: 950-956.

FIG. 39.6 Ultrasound Image of Another Case of Quadrilateral Space SyndromeThe teres minor muscle (white arrows) is small and echogenic, in comparison with the infraspinatus muscle (broken white arrows), because of fat infiltration and atrophy. From Waldman SD, Campbell RSD. Quadrilateral space syndrome. In: Imaging of Pain. Philadelphia: Elsevier; 2011:255–256, fig 100-2.

FIG. 39.7 The posterior humeral circumflex artery fills normally (white arrow) with the arm neutrally positioned in a patient with quadrilateral space syndrome.

FIG. 39.8 When the arm is abducted, the posterior humeral circumflex artery is truncated (black arrow). These pathognomonic findings allow angiographic diagnosis of this syndrome.

Parsonage-Turner syndrome, or idiopathic brachial neuritis, also may manifest as sudden onset of shoulder pain and can be confused with quadrilateral space syndrome. Tumor involving this anatomical region also should be considered in the differential diagnosis of quadrilateral space syndrome, as should occult fractures of the proximal humerus and other mass lesions, such as cysts and lipomas, which may compress the axillary nerve as it traverses the quadrilateral space.

Treatment Nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors represent a reasonable first step in the treatment of mild, selflimited quadrilateral space syndrome. The use of tricyclic antidepressants, such as nortriptyline, at a single bedtime dose of 25 mg, titrating upward as side effects allow, also is useful, especially if sleep disturbance is present. Gabapentin or carbamazepine also can be considered. Avoidance of repetitive trauma thought to be contributing to this entrapment neuropathy also is important, especially in professional athletes. If these maneuvers fail to produce rapid symptomatic relief, surgical exploration and release of the axillary nerve are indicated.

Complications and Pitfalls Failure to diagnose quadrilateral space syndrome correctly puts the patient at risk for the missed diagnosis of other syndromes that may result in ongoing damage to the shoulder or lead to overlooked pathological processes in this anatomical region that may harm the patient, such as Pancoast tumor or primary or metastatic tumors of the shoulder. MRI and ultrasound imaging are indicated in all patients thought to have quadrilateral space syndrome, and aggressive treatment of surgically correctable causes is generally indicated sooner rather than later to avoid ongoing irreversible shoulder damage.

Clinical Pearls Avoidance techniques of the repetitive movements responsible for quadrilateral space syndrome often are forgotten in the rush to treatment. Mild cases of quadrilateral space syndrome are usually self-limited, but more severe cases require urgent surgical intervention. As with other uncommon pain syndromes, quadrilateral space syndrome should be considered a diagnosis of exclusion and the clinician should ensure that no potentially harmful occult space-occupying lesions are present before attributing symptoms to other benign causes.

Suggested Readings Brown S.N, Doolittle D.A, Bohanon C.J, et al. Quadrilateral space syndrome: the Mayo Clinic experience with a new classification system and case series. Mayo Clin Proc. 2015;90(3):382–394. Chautems R.C, Glauser T, WaeberFey M.C, Rostan O, Barraud G.E. Quadrilateral space syndrome: case report and review of the literature. Ann Vasc Surg. 2000;14:673–676. McClelland D, Paxinos A. The anatomy of the quadrilateral space with reference to quadrilateral space syndrome. J Shoulder Elbow Surg. 2008;17:162–164. Nishimura M, Kobayashi M, Hamagashira K, et al. Quadrilateral space syndrome: a rare complication of thoracic surgery. Ann Thorac Surg. 2008;86:135370–135371. Sanders T.G, Tirman P.F.J. Paralabral cyst: an unusual cause of quadrilateral space syndrome. Arthroscopy. 1999;15:632–637. Waldman S.D. Quadrilateral space syndrome. In: Waldman S.D, Campbell R.S.D, eds. Imaging of Pain. Philadelphia: Saunders; 2011:254–256. Zurkiya O, Walker T.G. Quadrilateral space syndrome. J Vasc Interv Radiol. 2014;25(2):229.

SECT ION 4

Elbow Pain Syndromes OUT LINE 40. Pronator Syndrome 41. Cubital Bursitis 42. Anconeus Epitrochlearis 43. Os Supratrochleare-Related Elbow Pain 44. Osteonecrosis of the Elbow Joint 45. Triceps Tendinitis 46. Radial Tunnel Syndrome 47. Cubital Tunnel Syndrome 48. Driver’s Elbow 49. Anterior Interosseous Syndrome

40

Pronator Syndrome

Abstract The physical findings in pronator syndrome include tenderness over the forearm in the region of the pronator teres muscle. Unilateral hypertrophy of the pronator teres muscle may be identified. A positive Tinel’s sign over the median nerve as it passes beneath the pronator teres muscle also may be present. Weakness of the intrinsic muscles of the forearm and hand that are innervated by the median nerve may be identified with careful manual muscle testing. A positive pronator syndrome test, which is pain on forced pronation of the patient’s fully supinated arm, is highly suggestive of compression of the median nerve by the pronator teres muscle.

Keywords diagnostic ultrasound ultrasound guided injection; electromyography; lacertus fibrosus; ligament of Struther’s flexor digitorum superficialis; magnetic resonance imaging; median nerve; pronator syndrome; pronator teres muscle Tinel’s sign

ICD-10 CODE G56.00

The Clinical Syndrome Several sites of entrapment of the median nerve exist in the forearm. The median nerve may be entrapped at the lacertus fibrosus, at the lateral edge of the flexor digitorum superficialis, by fibrous bands of the superficial head of the pronator teres muscle, or, most commonly, by the pronator teres muscle itself (Fig. 40.1). Pronator syndrome is the compression of the median nerve by the pronator teres muscle. The onset of symptoms is usually after repetitive elbow motions, such as chopping wood, sculling, and cleaning fish, although occasionally the onset is more insidious, without apparent antecedent trauma. Clinically, pronator syndrome manifests as a chronic aching sensation localized to the forearm with pain occasionally radiating into the elbow. Patients with pronator syndrome may complain of a tired or heavy sensation in the forearm with minimal activity and clumsiness of the affected extremity. The sensory symptoms of pronator syndrome are identical to symptoms of carpal tunnel syndrome. In contrast to carpal tunnel syndrome, nighttime symptoms are unusual with pronator syndrome.

Signs and Symptoms The physical findings in pronator syndrome include tenderness over the forearm in the region of the pronator teres muscle. Unilateral hypertrophy of the pronator teres muscle may be identified. A positive Tinel sign over the median nerve as it passes beneath the pronator teres muscle also may be present (Fig. 40.2). Weakness of the intrinsic muscles of the forearm and hand that are innervated by the median nerve may be identified with careful manual muscle testing. A positive pronator syndrome test, which is pain on forced pronation of the patient’s fully supinated arm, is highly suggestive of compression of the median nerve by the pronator teres muscle (Fig. 40.3). The middle finger flexor superficialis test may also help confirm the diagnosis. The test is positive if a paresthesia in the median nerve is elicited when with the back of the hand and arm against the examining table, the examiner extends the index, ring, and little fingers while the patient forcefully flexes the middle finger at the proximal interphalangeal joint against examiner resistance (Fig. 40.4).

Testing Electromyography helps distinguish cervical radiculopathy, thoracic outlet syndrome, and carpal tunnel syndrome from pronator syndrome. Plain radiographs are indicated in all patients who present with pronator syndrome to rule out occult bony pathological processes. Based on the patient’s clinical presentation, additional testing, including complete blood cell count, uric acid level, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. Magnetic resonance imaging (MRI) and ultrasound imaging of the forearm is indicated if a primary elbow pathological condition or space-occupying lesion is suspected. The injection of the median nerve at the elbow may serve as a diagnostic and therapeutic maneuver.

FIG. 40.1 Intraoperative photograph showing compression of the median nerve by tendinous fascia off the deep head of pronator teres and the fibrous arch of the flexor digitorum superficialis proximal to it. From Aljawder A, Faqi MK, Mohamed A, et al. Anterior interosseous nerve syndrome diagnosis and intraoperative findings: a case report. Int J Surg Case Rep. 2016;21:44–47, fig 3.

FIG. 40.2 The symptoms of pronator syndrome are due to compression of the median nerve by the pronator teres muscle.

Differential Diagnosis Median nerve entrapment by the ligament of Struthers manifests clinically as unexplained persistent forearm pain caused by compression of the median nerve by an aberrant ligament that runs from a supracondylar process to the medial epicondyle. Clinically, it is difficult to distinguish from pronator syndrome. The diagnosis is made by electromyography and nerve conduction velocity testing that show compression of the median nerve at the elbow combined with the radiographic finding of a supracondylar process. Both these entrapment neuropathies can be differentiated from isolated compression of the anterior interosseous nerve that occurs approximately 6 to 8 cm below the elbow. These syndromes also should be differentiated from cervical radiculopathy involving the C6 or C7 roots, which sometimes may mimic median nerve compression. Cervical radiculopathy and median nerve entrapment may coexist as the “double crush” syndrome. The double crush syndrome is seen most commonly with median nerve entrapment at the wrist or carpal tunnel syndrome. Thoracic outlet syndrome also may cause forearm pain to be confused with pronator syndrome. The pain of thoracic outlet syndrome radiates into the ulnar rather than the median portion of the hand, however.

FIG. 40.3 A positive pronator syndrome test is highly indicative of pronator syndrome.

Treatment Nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors represent a reasonable first step in the treatment of pronator syndrome. The use of tricyclic antidepressants, such as nortriptyline, at a single bedtime dose of 25 mg, titrating upward as side effects allow, also is useful, especially if sleep disturbance is present. Avoidance of repetitive trauma thought to be contributing to this entrapment neuropathy also is important. If these maneuvers fail to produce rapid symptomatic relief, injection of the median nerve at the elbow with a local anesthetic and steroid is a reasonable next step. Ultrasound guidance may help improve the accuracy of needle placement and decrease the incidence of needle-related complications. If symptoms persist, surgical exploration and release of the median nerve are indicated.

FIG. 40.4 (A–C) The middle finger flexor superficialis test may also help confirm the diagnosis of pronator syndrome. The test is positive if a paresthesia in the median nerve is elicited when with the back of the hand and arm against the examining table, the examiner extends the index, ring, and little fingers while the patient forcefully flexes the middle finger at the proximal interphalangeal joint against examiner resistance. From Spinner RJ. Nerve entrapment syndromes. In: Morrey BF, Sancez-Sotelo J, Morrey ME, eds. Morrey’s the Elbow and Its Disorders. 5th ed. Philadelphia: Elsevier; 2018:679–701, fig 72.21C.

Complications and Pitfalls Median nerve block at the elbow is a safe block, with the major complications being inadvertent intravascular injection and persistent paresthesia secondary to needle trauma to the nerve. This technique can be performed safely in the presence of anticoagulation by using a 25- or 27-gauge needle, although at increased risk for hematoma, if the clinical situation dictates a favorable risk-to-benefit ratio. These complications can be decreased if manual pressure is applied to the area of the block immediately after injection. Application of cold packs for 20-minute periods after the block also decreases the amount of post-procedure pain and bleeding.

Clinical Pearls Avoidance techniques of the repetitive movements responsible for pronator syndrome are often forgotten in the rush to treatment. Median nerve block at the elbow is a simple and safe technique in the evaluation and treatment of the aforementioned painful conditions. Careful neurological examination to identify preexisting neurological deficits that may later be attributed to the nerve block should be performed in all patients before beginning median nerve block at the elbow. Median nerve compression by the ligament of Struthers manifests clinically as unexplained persistent forearm pain caused by compression of the median nerve by an aberrant ligament that runs from a supracondylar process to the medial epicondyle. The diagnosis is made by electromyography and nerve conduction velocity testing that show compression of the median nerve at the elbow combined with the radiographic finding of a supracondylar process. The pronator syndrome is characterized by unexplained persistent forearm pain with tenderness to palpation over the pronator teres muscle. A positive Tinel sign also may be present. Median nerve compression by the ligament of Struthers and pronator syndrome must be differentiated from isolated compression of the anterior interosseous nerve that occurs approximately 6 to 8 cm below the elbow. These syndromes also should be differentiated from cervical radiculopathy involving the C6 or C7 roots that may sometimes mimic median nerve compression. Cervical radiculopathy and median nerve entrapment may coexist as the double crush syndrome. The double crush

syndrome is seen most commonly with median nerve entrapment at the wrist or carpal tunnel syndrome.

Suggested Readings Caetano E.B, Vieira L.A, Sprovieri F.A.A, et al. Anatomical variations of pronator teres muscle: predispositional role for nerve entrapment. Rev Bras Ortop. (English Edition). 2017;52(2):169–175. Caetano E.B, Sabongi Neto J.J, Ribas L.A.A, Milanello E.V. Accessory muscle of the flexor digitorum superficialis and its clinical implications. Rev Bras Ortop. (English Edition). 2017;52(6):731–734. Gurses I.A, Altinel L, Gayretli O, et al. Morphology and morphometry of the ulnar head of the pronator teres muscle in relation to median nerve compression at the proximal forearm. Orthop Traumatol Surg Res. 2016;102(8):1005–1008. Horak B.T, Kuz J.T. An unusual case of pronator syndrome with ipsilateral supracondylar process and abnormal muscle mass. J Hand Surg. 2008;33:79–82. Hsiao C.-W, Shih J.-T, Hung S.-T. Concurrent carpal tunnel syndrome and pronator syndrome: a retrospective study of 21 cases. Orthop Traumatol Surg Res. 2017;103(1):101–103. Lacey S.H, Soldatis J.J. Bilateral pronator syndrome associated with anomalous heads of the pronator teres muscle: a case report. J Hand Surg. 1993;18:349–351. Presciutti S, Rodner C.M. Pronator syndrome. J Hand Surg. 2011;39:907–909. Rehak D.C. Pronator syndrome. Clin Sports Med. 2001;20:531–540.

41

Cubital Bursitis

Abstract An uncommon cause of elbow pain, cubital bursitis is being seen in clinical practice more frequently because of the increasing number of people using exercise equipment. The cubital bursa lies in the anterior aspect of the elbow and produces anterior elbow pain when inflamed. Also known as the bicipitoradial bursa, the cubital bursa may exist as a single bursal sac or in some patients may exist as a multisegmented series of sacs that may be loculated. The cubital bursa is vulnerable to injury from acute trauma and repeated microtrauma. Acute injuries frequently take the form of direct trauma to the anterior aspect of the elbow. Repetitive movements of the elbow, including repeated bicepsstrengthening exercises and throwing of javelins and baseballs, may result in inflammation and swelling of the cubital. Gout or rheumatoid arthritis rarely may precipitate acute cubital bursitis. If the inflammation of the cubital bursa becomes chronic, calcification of the bursa may occur.

Keywords bursitis; cubital bursitis; diagnostic ultrasound; elbow pain; median nerve; repetitive stress injury; Rice bodies; septic bursitis; sports injury; ultrasound guided injection

ICD-10 CODE M70.20

The Clinical Syndrome An uncommon cause of elbow pain, cubital bursitis is being seen in clinical practice more frequently because of the increasing number of people using exercise equipment. The cubital bursa lies in the anterior aspect of the elbow and produces anterior elbow pain when inflamed. Also known as the bicipitoradial bursa, the cubital bursa may exist as a single bursal sac or in some patients may exist as a multisegmented series of sacs that may be loculated. The cubital bursa is vulnerable to injury from acute trauma and repeated microtrauma. Acute injuries frequently take the form of direct trauma to the anterior aspect of the elbow. Repetitive movements of the elbow, including repeated biceps-strengthening exercises and throwing of javelins and baseballs, may result in inflammation and swelling of the cubital bursa (Fig. 41.1). Gout or rheumatoid arthritis rarely may precipitate acute cubital bursitis. If the inflammation of the cubital bursa becomes chronic, calcification of the bursa may occur.

Signs and Symptoms A patient with cubital bursitis frequently reports pain and swelling with any movement of the elbow (see Fig. 41.1). The pain is localized to the cubital area, with referred pain often noted in the forearm and hand. Physical examination reveals point tenderness in the anterior aspect of the elbow over the cubital bursa and swelling of the bursa. Passive extension and resisted flexion of the elbow reproduce the pain, as does any pressure over the bursa.

Testing The diagnosis of cubital bursitis usually can be made on clinical grounds and easily confirmed with ultrasound imaging (Fig. 41.2). Plain radiographs of the elbow may reveal calcification of the bursa and associated structures consistent with chronic inflammation. Magnetic resonance imaging (MRI) is indicated if the patient is thought to have a joint mouse or primary pathological process of the elbow joint. Laboratory testing to rule out hyperuricemia and collagen-vascular disease also should be considered in appropriate patients. Electromyography and nerve conduction velocity testing rule out nerve entrapment syndromes at the elbow. Injection of the cubital bursa with a local anesthetic and steroid is a diagnostic and therapeutic maneuver.

Differential Diagnosis The most common causes of elbow pain are arthritis of the elbow joint, tennis elbow, golfer ’s elbow, and olecranon bursitis. Arthritis of the elbow joint may mimic cubital bursitis because both painful conditions are associated with movement of the joint. The anterior point tenderness seen in cubital bursitis is absent in arthritis of the elbow, however. Tennis elbow and golfer ’s elbow are distinct clinical entities that should not be confused with cubital bursitis because the point tenderness seen in these painful conditions is identified over the lateral and medial epicondyles, rather than at the midline, as is seen with cubital bursitis. Acute gout affecting the elbow manifests as a diffuse acute inflammatory condition that may be difficult to distinguish from infection of the joint, rather than as a localized musculoskeletal pain syndrome.

FIG. 41.1 A patient with cubital bursitis reports pain and swelling on any movement of the elbow. From Waldman SD. Injection technique for cubital bursitis pain. In: Waldman SD, ed. Pain Review. 2nd ed. Philadelphia: Saunders; 2016:487–488.

Treatment Initial treatment of the pain and functional disability associated with cubital bursitis should include a combination of nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and physical therapy. Local application of heat and cold also may be beneficial. The repetitive movements that incite the syndrome should be avoided. For patients who do not respond to these treatment modalities, injection of the cubital bursa with a local anesthetic and steroid may be a reasonable next step (Fig. 41.3). To inject the cubital bursa, the patient is placed in the supine position, with the arm fully adducted at the patient’s side, elbow extended, and the dorsum of the hand resting on a folded towel. Using a 5-mL sterile syringe, 2 mL of local anesthetic and 40 mg of methylprednisolone is drawn.

FIG. 41.2 Cubital or bicipitoradial bursitis longitudinal extended field of view image demonstrating a sausage-shaped heterogeneous distended cubital bursa (arrowheads). From James JJ. Ultrasound of the elbow. In: Allan PL, Baxter GM, Weston MJ, eds. Clinical Ultrasound. 3rd ed. New York: Elsevier; 2011:1043–1054.

FIG. 41.3 Proper needle placement for injection for treatment of cubital bursitis.

After sterile preparation of skin overlying the anterior aspect of the joint, the clinician identifies the pulsations of the brachial artery at the crease of the elbow. After preparation of the skin with antiseptic solution, a 25-gauge, 1-inch needle is inserted just lateral to the brachial artery at the crease and slowly advanced in a slightly medial and cephalad trajectory through the skin and subcutaneous tissues. If bone is encountered, the needle is withdrawn back into the subcutaneous tissue. The contents of the syringe are gently injected. Little resistance to injection should be felt. If resistance is encountered, the needle is probably in the tendon and should be withdrawn back until the injection proceeds without significant resistance. The needle is removed, and a sterile pressure dressing and ice pack are placed at the injection site. Ultrasound guidance may improve the accuracy of needle placement and decrease the incidence of needle-related complications.

Complications and Pitfalls The major complication associated with cubital diagnosis is misdiagnosis. Failure of the clinician to recognize an acute inflammatory or infectious arthritis of the elbow may result in permanent damage to the joint and chronic pain and functional disability. Injection of the cubital bursa at the elbow is a safe block, with the major complications being inadvertent intravascular injection and persistent paresthesia secondary to needle trauma to the median nerve. This technique can be performed safely in the presence of anticoagulation by using a 25- or 41-gauge needle, although at increased risk for hematoma, if the clinical situation dictates a favorable risk-to-benefit ratio. These complications can be decreased if manual pressure is applied to the area of the block immediately after injection. Application of cold packs for 20-minute periods after the block also decreases the amount of postprocedure pain and bleeding.

Clinical Pearls Bursae are formed from synovial sacs whose purpose is to allow easy sliding of muscles and tendons across one another at areas of repeated movement. These synovial sacs are lined with a synovial membrane invested with a network of blood vessels that secrete synovial fluid. Inflammation of the bursa results in an increase in the production of synovial fluid with swelling of the bursal sac. With overuse or misuse, these bursae may become inflamed, enlarged, and, rarely, infected. Coexistent tendinitis and epicondylitis also may contribute to elbow pain and may require additional treatment with more localized injection of local anesthetic and depot steroid. This technique is a safe procedure if careful attention is paid to the clinically relevant anatomy in the areas to be injected, in particular avoiding the median nerve by keeping the needle lateral to the brachial artery. Care must be taken to use sterile technique to avoid infection and universal precautions to avoid risk to the operator. The incidence of ecchymosis and hematoma formation can be decreased if pressure is placed on the injection site immediately after injection. The use of physical modalities, including local heat and gentle range-of-motion exercises, should be introduced several days after the patient undergoes this injection technique for elbow pain. Vigorous exercises should be avoided because they exacerbate the patient’s

symptoms. Simple analgesics and NSAIDs may be used concurrently with this injection technique.

Suggested Readings Chung C.B, Kim H.J. Sports injuries of the elbow. Magn Res Imaging Clin N Am. 2003;11:239–253. De Maeseneer M, Marcelis S, Cattrysse E, et al. Ultrasound of the elbow: a systematic approach using bony landmarks. Eur J Radiol. 2012;81(5):919– 922. De Maeseneer M, Brigido M.K, Antic M, et al. Ultrasound of the elbow with emphasis on detailed assessment of ligaments, tendons, and nerves. Eur J Radiol. 2015;84(4):671–681. Draghi F, Gregoli B, Sileo C. Sonography of the bicipitoradial bursa: a short pictorial essay. J Ultrasound. 2012;15(1):39–41. Hayter C.L, Giuffre B.M. Overuse and traumatic injuries of the elbow. Magn Res Imaging Clin N Am. 2009;17:617–638. Howard T.M, Shaw J.L, Phillips J. Physical examination of the elbow. In: Seidenberg P.H, Beutler A.I, eds. The Sports Medicine Resource Manual. Philadelphia: Saunders; 2008:71–78. Sellards R, Kuebrich C. The elbow: diagnosis and treatment of common injuriesprimary care. Clin Office Pract. 2005;32:1–16.

42

Anconeus Epitrochlearis

Abstract Anconeus epitrochlearis is caused by entrapment and compression of the ulnar nerve at the elbow by an accessory anconeus muscle. This entrapment neuropathy manifests as pain and associated paresthesias in the lateral forearm that radiates to the wrist and ring and little fingers in a manner analogous to tardy ulnar palsy. The symptoms often are aggravated by prolonged flexion of the elbow. The pain of anconeus epitrochlearis has been characterized as unpleasant and dysesthetic. The onset of symptoms is usually after repetitive elbow motions or from repeated pressure on the elbow, such as using the elbows to arise from bed. Anconeus epitrochlearis also is seen in throwing athletes such as baseball pitchers and quarterbacks. Direct trauma to the ulnar nerve as it enters the cubital tunnel may result in a similar clinical presentation, as can compression of the ulnar nerve as it passes through the cubital tunnel by osteophytes, lipomas, ganglions, and aponeurotic bands. Untreated, progressive motor deficit and ultimately flexion contracture of the affected fingers can result.

Keywords anconeus epitrochlearis; cubital tunnel; entrapment neuropathy; sports injury; throwing injury; ulnar nerve

ICD-10 CODE G56.20

The Clinical Syndrome Anconeus epitrochlearis is an uncommon cause of lateral forearm pain and weakness that can be quite distressing to the patient. Anconeus epitrochlearis is caused by entrapment and compression of the ulnar nerve at the elbow by an accessory anconeus muscle (Fig. 42.1). This entrapment neuropathy manifests as pain and associated paresthesias in the lateral forearm that radiate to the wrist and ring and little fingers in a manner analogous to tardy ulnar palsy. The symptoms often are aggravated by prolonged flexion of the elbow. The pain of anconeus epitrochlearis has been characterized as unpleasant and dysesthetic. The onset of symptoms is usually after repetitive elbow motions or from repeated pressure on the elbow, such as using the elbows to arise from bed. Anconeus epitrochlearis also is seen in throwing athletes such as baseball pitchers and quarterbacks. Direct trauma to the ulnar nerve as it enters the cubital tunnel may result in a similar clinical presentation, as can compression of the ulnar nerve as it passes through the cubital tunnel by osteophytes, lipomas, ganglions, and aponeurotic bands. Untreated, progressive motor deficit and ultimately flexion contracture of the affected fingers can result.

Signs and Symptoms Physical findings include tenderness over the ulnar nerve at the elbow. A positive Tinel sign over the ulnar nerve as it passes beneath the aponeuroses is usually present. Weakness of the intrinsic muscles of the forearm and hand that are innervated by the ulnar nerve may be identified with careful manual muscle testing, although early in the course of the evolution of anconeus epitrochlearis, the only physical finding other than tenderness over the nerve may be the loss of sensation on the ulnar side of the little finger. As the syndrome progresses, the affected hand may have a claw-like appearance. A positive Wartenberg sign indicative of weakness of voluntary adduction results in involuntary abduction of the little finger. A positive Froment sign also may be present (Fig. 42.2).

FIG. 42.1 Anconeus epitrochlearis is caused by entrapment and compression of the ulnar nerve at the elbow by an accessory anconeus muscle. m, Muscle.

FIG. 42.2 Eliciting Froment’s Sign.The patient is asked to hold an object, usually a flat object such as a piece of paper, between the thumb and index finger (pinch grip). The examiner then attempts to pull the object out of the subject’s hands. (A) A normal individual will be able to maintain a hold on the object without difficulty. (B) The patient with ulnar nerve palsy will experience difficulty maintaining a hold on the paper and will compensate by flexing the flexor pollicis longus of the thumb to maintain grip pressure causing a pinching effect. Clinically, this compensation manifests as flexion of the interphalangeal joint of the thumb (rather than adduction, as would occur with correct use of the adductor pollicis). The compensation of the affected hand results in a weak pinch grip with the tips of the thumb and index finger; therefore, the thumb moves into obvious flexion. From Waldman SD. Physical Diagnosis of Pain: An Atlas of Signs and Symptoms. Philadelphia: Saunders; 2006:126.

FIG. 42.3 Anconeus Epitrochlearis Muscle Replacing the Cubital Tunnel Retinaculum.A T2-weighted axial image reveals the ulnar nerve (white arrow) deep to an anomalous anconeus epitrochlearis muscle (black arrow) and superficial to the posterior bundle of the medial collateral ligament (curved arrow). From Edelman RR, Hesselink JR, Zlatkin MB, et al, eds. Clinical Magnetic Resonance Imaging. 3rd ed. Philadelphia: Saunders; 2006:3303.

FIG. 42.4 Ultrasound Image of the Muscle Demonstrating Anconeus Epitrochlearis (Arrows)The muscle is seen to cover ulnar nerve (arrowhead). H, Humerus; U, ulna. From De Maeseneer M, Brigido MK, Antic M, et al. Ultrasound of the elbow with emphasis on detailed assessment of ligaments, tendons, and nerves. Eur J Radiol. 2015;84[4]:671–681, fig 27.

Testing Electromyography helps distinguish cervical radiculopathy and anconeus epitrochlearis from golfer ’s elbow. Plain radiographs are indicated in all patients who present with anconeus epitrochlearis to rule out occult bony pathology, such as osteophytes impinging on the ulnar nerve. Based on the patient’s clinical presentation, additional testing, including complete blood cell count, uric acid level, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. Magnetic resonance imaging (MRI) of the elbow is indicated if joint instability is suspected and clearly identifies whether the compression of the ulnar nerve is caused by an accessory anconeus muscle (Fig. 42.3). Ultrasound imaging will help assess the status of the ulnar nerve at the elbow. Injection of the ulnar nerve serves as a diagnostic and therapeutic maneuver (Fig. 42.4).

Differential Diagnosis Anconeus epitrochlearis is often misdiagnosed as golfer ’s elbow, and this fact accounts for the many patients with golfer ’s elbow who fail to respond to conservative measures. In anconeus epitrochlearis, the maximal tenderness to palpation is over the ulnar nerve 1 inch below the medial epicondyle, whereas in golfer ’s elbow, the maximal tenderness to palpation is directly over the medial epicondyle. Anconeus epitrochlearis also should be differentiated from cervical radiculopathy involving the C7 or C8 roots and golfer ’s elbow. Cervical radiculopathy and ulnar nerve entrapment may coexist as the “double crush” syndrome. The double crush syndrome is seen most commonly with median nerve entrapment at the wrist or carpal tunnel syndrome.

Treatment Initial treatment of the pain and functional disability associated with anconeus epitrochlearis should include a combination of nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and physical therapy. Local application of heat and cold also may be beneficial. The repetitive movements that incite the syndrome should be avoided. For patients who do not respond to these treatment modalities, injection of the ulnar nerve at the elbow with a local anesthetic and steroid may be a reasonable next step. If the symptoms of anconeus epitrochlearis persist, surgical exploration, resection of the accessory anconeus muscle, and decompression of the ulnar nerve are indicated.

Complications and Pitfalls The major complications associated with anconeus epitrochlearis fall into two categories: (1) iatrogenically induced complications resulting from persistent and overaggressive treatment of “resistant golfer ’s elbow” and (2) the potential for permanent neurological deficits resulting from prolonged untreated entrapment of the ulnar nerve. Failure of the clinician to recognize an acute inflammatory or infectious arthritis of the elbow may result in permanent damage to the joint and chronic pain and functional disability.

Clinical Pearls An accessory anconeus muscle is present in approximately 11% of the adult population. Anconeus epitrochlearis is a distinct clinical entity that is often misdiagnosed as golfer ’s elbow, and this fact accounts for the many patients with “golfer ’s elbow” who fail to respond to conservative measures. With anconeus epitrochlearis, the maximal tenderness to palpation is over the ulnar nerve and a positive Tinel sign is present, whereas with golfer ’s elbow, the maximal tenderness to palpation is over the medial epicondyle. If anconeus epitrochlearis is suspected, injection of the radial nerve at the elbow with a local anesthetic and steroid gives almost instantaneous relief. Careful examination to identify preexisting neurological deficits that may later be attributed to the nerve block should be performed on all patients before beginning ulnar nerve block at the elbow.

Suggested Readings De Maeseneer M, Brigido M.K, Antic M, et al. Ultrasound of the elbow with emphasis on detailed assessment of ligaments, tendons, and nerves. Eur J Radiol. 2015;84(4):671–681. Fernandez J, Camuzard O, Gauci M.O, Winter M. A rare cause of ulnar nerve entrapment at the elbow area illustrated by six cases: the anconeus epitrochlearis muscle. Chir Main. 2015;34(6):294–299. Kojima T. Ulnar compression neuropathy secondary to the anconeus epitrochlearis muscle. J Hand Surg. 1989;14:918–919. Law K.-K. Acute cubital tunnel syndrome secondary to anconeus epitrochlearis muscle. J Orthop Traumatol Rehabil. 2015;19(2):111–113. Maslow J.I, Johnson D.J., Block J.J., Lee D.H., Desai M.J. Prevalence and clinical manifestations of the anconeus epitrochlearis and cubital tunnel syndrome: level 4 evidence. J Hand Surg. 2017;42(9):S9–S10. Waldman S.D. The ulnar nerve. In: Waldman S.D, ed. Pain Review. ed 2. Philadelphia: Elsevier; 2016:86–87.

43

Os Supratrochleare-Related Elbow Pain

Abstract Elbow pain secondary to os supratrochleare is being seen with increasing frequency in clinical practice owing to the increased interest in physical fitness and the use of exercise machines. Os supratrochleare is the name given to an accessory ossicle occasionally found in the posterior elbow. This accessory ossicle often is found adjacent to the proximal aspect of the olecranon process. It is thought that accessory ossicles such as os supratrochleare bones serve to decrease the friction and pressure of tendons as they pass in proximity to a joint. Similar accessory ossicles are found in the feet, hands, and wrists. Elbow pain secondary to os supratrochleare is characterized by tenderness and pain over the posterior elbow. Patients often describe a feeling of having gravel in their elbow and may report a grating sensation with flexion and extension of the elbow.

Keywords accessory ossicle; elbow pain; excersize-related injury; olecranon process; os surpatrochleare

ICD-10 CODE M89.8x9

The Clinical Syndrome Elbow pain secondary to os supratrochleare is being seen with increasing frequency in clinical practice owing to the increased interest in physical fitness and the use of exercise machines. Os supratrochleare is the name given to an accessory ossicle occasionally found in the posterior elbow. This accessory ossicle often is found adjacent to the proximal aspect of the olecranon process. It is thought that accessory ossicles such as os supratrochleare bones serve to decrease the friction and pressure of tendons as they pass in proximity to a joint. Similar accessory ossicles are found in the feet, hands, and wrists. Elbow pain secondary to os supratrochleare is characterized by tenderness and pain over the posterior elbow. Patients often describe a feeling of having gravel in their elbow and may report a grating sensation with flexion and extension of the elbow (Fig. 43.1). The pain of os supratrochleare worsens with activities that require repeated flexion and extension of the elbow or with forceful overhead throwing. Os supratrochleare is often associated with loose bodies in the elbow joint and may coexist with olecranon bursitis.

Signs and Symptoms On physical examination, pain can be reproduced by pressure on the os supratrochleare. In contrast to olecranon bursitis, in which the tender area remains over the olecranon bursa, with os supratrochleare, the area of maximal tenderness is just above the olecranon process. A creaking or grating sensation may be appreciated by the examiner and locking or catching on extension and flexion of the elbow occasionally may be present.

Testing Plain radiographs are indicated in all patients with os supratrochleare to rule out fractures and identify accessory ossicles that may have become inflamed (Fig. 43.2). Plain radiographs also often identify loose bodies or joint mice frequently seen in patients with elbow pain secondary to os supratrochleare (Fig. 43.3). Based on the patient’s clinical presentation, additional testing, including complete blood cell count, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. Magnetic resonance imaging (MRI) and ultrasound imaging of the elbow joint is indicated if joint instability, occult mass, or tumor is suspected and to clarify the diagnosis further. Radionucleotide bone scanning may be useful in identifying stress fractures or tumors of the elbow and distal humerus that may be missed on plain radiographs.

Differential Diagnosis Primary pathological processes of the elbow, including gout and occult fractures, may mimic the pain and disability associated with os supratrochleare. Entrapment neuropathies, such as ulnar tunnel syndrome, also may confuse the diagnosis, as may bursitis, tendinitis, and epicondylitis of the elbow, which may coexist with os supratrochleare. Osteochondritis dissecans, Panner disease, and synovial chondromatosis also may mimic the pain associated with os supratrochleare. Primary and metastatic tumors of the elbow may manifest in a manner similar to elbow pain secondary to os supratrochleare.

FIG. 43.1 Elbow pain secondary to os supratrochleare is characterized by tenderness and pain over the posterior elbow.

FIG. 43.2 A smooth, rounded ossicle in the olecranon fossa (arrow) is consistent with the diagnosis of os supratrochleare. From Morrey BF. Loose bodies and ossification centers about the elbow. In: Morrey’s the Elbow and Its Disorders. 5th ed. Philadelphia: Elsevier; 2018: chap 84, 795–804. ISBN 9780323341691, https://doi.org/10.1016/B978-0-323-34169-1.00084-X. https://www.sciencedirect.com/science/article/pii/B978032334169100084X.

FIG. 43.3 The os supratrochleare (arrow) may sometimes take on an irregular shape, which can mislead the examiner to suspect that the cause is trauma, or even osteochondritis dissecans of the trochlea. Multiple loose bodies in the olecranon fossa and coronoid osteophytosis suggest primary arthrosis as another possible cause. From Morrey BF. Loose bodies and ossification centers about the elbow. In: Morrey’s the Elbow and Its Disorders. 5th ed. Philadelphia: Elsevier; 2018: chap 84, 795–804. ISBN 9780323341691, https://doi.org/10.1016/B978-0-323-34169-1.00084-X. https://www.sciencedirect.com/science/article/pii/B978032334169100084X.

Treatment Initial treatment of the pain and functional disability associated with os supratrochleare should include a combination of nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and physical therapy. The local application of heat and cold also may be beneficial. Avoidance of repetitive activities that aggravate the patient’s symptoms also may provide relief. For patients who do not respond to these treatment modalities, injection of the os supratrochleare with a local anesthetic and steroid may be a reasonable next step. Ultrasound guidance may improve the accuracy of needle placement and decrease the incidence of needle-induced complications. For pain that persists, or if the os supratrochleare is causing damage to the elbow joint, surgical removal is indicated.

Complications and Pitfalls The major complication of injection of os supratrochleare is infection. This complication should be exceedingly rare if strict aseptic technique is followed. Approximately 25% of patients report a transient increase in pain after injection of the os supratrochleare and should be warned of this possibility. Another potential risk of this injection technique is trauma to the extensor tendons from the injection itself.

Clinical Pearls Pain emanating from the elbow is a common problem encountered in clinical practice. Os supratrochleare must be distinguished from fractures of the elbow, fractures of the os supratrochleare itself, entrapment neuropathies of the ulnar nerve, bursitis, tendinitis, and epicondylitis. Less common causes of posterior elbow pain are osteochondritis dissecans, Panner disease, and synovial chondromatosis.

Suggested readings Gudmundsen E, Østensen H. Accessory ossicles in the elbow. Acta Orthop Scand. 1987;58:130–132. McFarland E.G, Gill H.S, Laporte D.M, Streiff M. Miscellaneous conditions about the elbow in athletes [review]. Clin Sports Med. 2004;23:743–763. Waldman SD. In: Supratrochlear os, Waldman SD, Campbell RSD, eds. Imaging of Pain. Philadelphia: Saunders; 2011:285–286. Waldman S.D. Functional anatomy of the elbow. In: Waldman S.D, ed. Pain Review. Philadelphia: Saunders Elsevier; 2009:98–100. Waldman S.D. The ulnar nerve. In: Waldman S.D, ed. Pain Review. 2nd ed. Philadelphia: Elsevier; 2016:86–87. Wood V.E, Campbell G.S. The supratrochleare dorsale accessory ossicle in the elbow. J Shoulder Elbow Surg. 1994;3:39435–39438.

44

Osteonecrosis of the Elbow Joint

Abstract Patients with osteonecrosis of the elbow joint report pain over the affected elbow joint or joints that may radiate into the upper extremity. The pain is deep and aching, and patients often report a catching sensation with range of motion of the affected elbow joint or joints. Range of motion decreases as the disease progressesFactors predisposing to osteonecrosis of the elbow joint include trauma to the joint; corticosteroid use; Cushing’s disease; alcohol abuse; connective tissue diseases, especially systemic lupus erythematosus; osteomyelitis; human immunodeficiency virus infection; organ transplantation; hemoglobinopathies, including sickle cell disease; hyperlipidemia; gout; renal failure; pregnancy; sickle cell disease; and radiation therapy involving the femoral head.

Keywords aseptic necrosis; collagen-vascular disease; Cushings; disease; elbow pain; human immunodeficiency virus; Osteonecrosis of the elbow; sickle cell disease; ultrasound guided injection

ICD-10 CODE M87.03

The Clinical Syndrome Osteonecrosis of the elbow joint is an often-missed diagnosis. Like the scaphoid bone of the wrist, the elbow joint is extremely susceptible to this disease because of the tenuous blood supply of the articular cartilage. This blood supply is easily disrupted, often leaving the proximal portion of the bone without nutrition and leading to osteonecrosis (Fig. 44.1). A disease of the fourth and fifth decades, with the exception of patients with osteonecrosis of the elbow joint secondary to collagen-vascular disease, osteonecrosis of the elbow joint is more common in men. In younger patients, sickle cell disease is the most common cause of osteonecrosis of the elbow. The disease is bilateral in 45% to 50% of cases. Factors predisposing to osteonecrosis of the elbow joint are listed in Table 44.1. They include trauma to the joint; corticosteroid use; Cushing disease; alcohol abuse; connective tissue diseases, especially systemic lupus erythematosus; osteomyelitis; human immunodeficiency virus infection; organ transplantation; hemoglobinopathies, including sickle cell disease; hyperlipidemia; gout; renal failure; pregnancy; and radiation therapy involving the femoral head. Patients with osteonecrosis of the elbow joint report pain over the affected elbow joint or joints that may radiate into the upper extremity. The pain is deep and aching, and patients often report a catching sensation with range of motion of the affected elbow joint or joints. Range of motion decreases as the disease progresses.

Signs and Symptoms Physical examination of patients with osteonecrosis of the elbow joint reveals pain to deep palpation of the elbow joint. The pain can be worsened by passive and active range of motion. A click or crepitus also may be appreciated by the examiner when ranging the elbow joint. A decreased range of motion is invariably present.

Testing Plain radiographs are indicated in all patients with osteonecrosis of the elbow joint to rule out underlying occult bony pathological processes and identify sclerosis and fragmentation of the osseous support of the articular surface. However, early in the course of the disease, plain radiographs can be notoriously unreliable; magnetic resonance imaging (MRI) reveals articular changes before significant changes are evident on plain radiographs (Fig. 44.2). Based on the patient’s clinical presentation, additional testing, including complete blood cell count, uric acid level, erythrocyte sedimentation rate, and antinuclear antibody testing, also may be indicated. MRI and ultrasound imaging of the elbow joint is indicated in all patients thought to have osteonecrosis of the elbow joint; if other causes of joint instability, infection, or tumor are suspected; or if plain radiographs are nondiagnostic (Fig. 44.3). Computed tomography (CT) may be useful in early diagnosis, especially with three-dimensional reconstruction (Fig. 44.4). Administration of gadolinium followed by post-contrast imaging may help delineate the adequacy of blood supply, with contrast enhancement of the elbow joint being a good prognostic sign. Electromyography is indicated if coexistent cervical radiculopathy or brachial plexopathy is suspected. A very gentle intraarticular injection of the elbow joint with small volumes of local anesthetic will provide immediate improvement of the pain and help demonstrate the nidus of the pain is in fact the elbow joint. Ultimately, total joint replacement will be required in most patients with osteonecrosis of the elbow joint, although newer joint preservation techniques are becoming more popular in younger, more active patients, given the short life expectancy of total shoulder prosthesis.

FIG. 44.1 The blood supply to the elbow is easily disrupted, often leaving the proximal portion of the bone without nutrition and leading to osteonecrosis.

TABLE 44.1 Predisposing Factors for Osteonecrosis of the Elbow Joint Trauma to the elbow joint Steroids Cushing disease Alcohol abuse Connective tissue diseases, especially systemic lupus erythematosus Osteomyelitis Human immunodeficiency virus Organ transplantation Hemoglobinopathies, including sickle cell disease Hyperlipidemia Gout Renal failure Pregnancy Radiation therapy Sickle cell disease

FIG. 44.2 (A) Coronal T2-weighted with fat suppression (FST2W) MRI demonstrating an area of high-signal intensity marrow edema in the capitellum (solid arrow) of an adolescent with elbow pain. An area of low signal intensity (SI) is seen in the subchondral bone plate (dashed arrow), suggestive of an osteochondral defect. (B) The sagittal FST2W MRI more clearly shows the low-SI osteochondral defect (curved arrow), with a linear area of high SI at its base, indicating that the lesion is likely to be unstable. These appearances are typical of Panner disease (osteochondritis dissecans). From Waldman SD. Osteonecrosis of the elbow. In: Waldman SD, Campbell RSD, eds. Imaging of Pain. New York: Elsevier; 2011:282.

FIG. 44.3 Ultrasound image demonstrating a significant joint effusion in a patient with osteonecrosis of the elbow.

Differential Diagnosis Coexistent arthritis and gout of the elbow joint, bursitis, and tendinitis may coexist with osteonecrosis of the elbow joints and exacerbate the pain and disability. Tears of the ligaments, bone cysts, bone contusions, and fractures may mimic the pain of osteonecrosis of the elbow joint, as can occult metastatic disease.

FIG. 44.4 (A-C) Multifocal Aspect of Elbow Osteonecrosis A, Anterior; P, posterior. From Mukaza MM, Manicom O, Fillipini P, et al. Elbow osteonecrosis in sickle cells anemia: a study of six cases. Orthop Traumatol. 2009;95:82–84.

Treatment Initial treatment of the pain and functional disability associated with osteonecrosis of the elbow joint should include a combination of the nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and decreased weight bearing of the affected elbow joint or joints. Local application of heat and cold may be beneficial. For patients who do not respond to these treatment modalities, an injection of a local anesthetic into the elbow joint may be a reasonable next step to provide palliation of acute pain. Ultrasound guidance may improve the accuracy of needle placement and decrease the incidence of needle-related complications. Vigorous exercises should be avoided because they will exacerbate the symptoms. Ultimately, surgical repair in the form of total joint arthroplasty is the treatment of choice.

Complications and Pitfalls Failure to surgically treat significant osteonecrosis of the elbow joint usually will result in continued pain and disability and in most patients will lead to ongoing damage to the elbow joint (see Fig. 44.2). Injection of the joint with local anesthetic is a relatively safe technique if the clinician is attentive to detail, specifically using small amounts of local anesthetic and avoiding high injection pressures, which may further damage the joint. Another complication of this injection technique is infection. This complication should be exceedingly rare if strict aseptic technique is followed. Approximately 25% of patients report a transient increase in pain after this injection technique and should be warned of this possibility.

Clinical Pearls Osteonecrosis of the elbow joint is a diagnosis that is often missed, leading to considerable unnecessary pain and disability. The clinician should include osteonecrosis of the elbow joint in the differential diagnosis in all patients with shoulder joint pain, especially if any of the predisposing factors listed in Table 44.1 are present. Coexistent arthritis, tendinitis, and gout may contribute to the pain and may require additional treatment. The use of physical modalities, including local heat and cold and decreased weight bearing, may provide symptomatic relief. Vigorous exercises should be avoided because they will exacerbate the symptoms and may cause further damage to the wrist. Simple analgesics and NSAIDs may be used concurrently with this injection technique.

Suggested Readings Mukaza M.M, Manicom O, Fillipini P, Hernigou P. Elbow osteonecrosis in sickle cells anemia: a study of six cases. Orthop Traumatol. 2009;95:82–84. Savini C.J, James C.W. HIV infection and osteonecrosis. J Assoc Nurse AIDS Care. 2001;12:83–85. Waldman S.D. Functional anatomy of the elbow. In: Waldman S.D, ed. Pain Review. Philadelphia: Saunders; 2009:76–77. Waldman S.D. Osteonecrosis of the elbow. In: Waldman S.D, Campbell R.S.D, eds. Imaging of pain. New York: Elsevier; 2011:281–283. Watanabe R, Sato K, Nakamura T, et al. Steroid-induced osteonecrosis of bilateral distal humerus treated by arthroplasty using costal osteochondral graft: case report. J Hand Surg. 2011;36:816–819.

45

Triceps Tendinitis

Abstract Triceps tendinitis is being seen with increasing frequency in clinical practice as exercising and the use of exercise equipment have increased in popularity. The triceps tendon is susceptible to the development of tendinitis at its distal portion and its insertion on the ulna. The triceps tendon is subject to repetitive motion that may result in microtrauma, which heals poorly because of the tendon’s avascular nature. Exercise is often implicated as the inciting factor of acute triceps tendinitis. Tendinitis of the triceps tendon frequently coexists with bursitis of the associated bursae of the tendon and elbow joint, creating additional pain and functional disability. Calcium deposition around the tendon may occur if the inflammation continues, making subsequent treatment more difficult. Continued trauma to the inflamed tendon ultimately may result in tendon rupture.

Keywords bursitis; elbow pain; excersize-related injury; tendinopathy; triceps tendinitis; triceps tendon

ICD-10 CODE M65.80

The Clinical Syndrome Triceps tendinitis is being seen with increasing frequency in clinical practice as exercising and the use of exercise equipment have increased in popularity. The triceps tendon is susceptible to the development of tendinitis at its distal portion and its insertion on the ulna. The triceps tendon is subject to repetitive motion that may result in microtrauma, which heals poorly because of the tendon’s avascular nature. Exercise is often implicated as the inciting factor of acute triceps tendinitis. Tendinitis of the triceps tendon frequently coexists with bursitis of the associated bursae of the tendon and elbow joint, creating additional pain and functional disability. Calcium deposition around the tendon may occur if the inflammation continues, making subsequent treatment more difficult (Fig. 45.1). Continued trauma to the inflamed tendon ultimately may result in tendon rupture (Fig. 45.2).

Signs and Symptoms The onset of triceps tendinitis is usually acute, occurring after overuse or misuse of the elbow joint. Inciting factors include playing tennis and aggressive use of exercise machines. Improper stretching of triceps muscle and triceps tendon before exercise also has been implicated in the development of triceps tendinitis and acute tendon rupture. Injuries ranging from partial to complete tears of the tendon can occur when the distal tendon sustains direct trauma while it is fully flexed under load or when the elbow is forcibly flexed while the arm is fully extended. The pain of triceps tendinitis is constant and severe and is localized in the posterior elbow (Fig. 45.3). Significant sleep disturbance is often reported. Patients with triceps tendinitis exhibit pain with resisted extension of the elbow. A creaking or grating sensation may be palpated when passively extending the elbow. As mentioned, a chronically inflamed triceps tendon may rupture suddenly with stress or during vigorous injection procedures inadvertently injected into the substance of the tendon. With triceps tendon rupture, the patient is unable to fully and forcefully extend the affected arm.

FIG. 45.1 Tendon and Soft Tissue CalcificationCalcified deposits are visualized in the triceps tendon (T) and soft tissues (ST) around the proximal end of the radius. From Resnick D, ed. Diagnosis of Bone and Joint Disorders. 4th ed. Philadelphia: Saunders; 2002:1581.

FIG. 45.2 Triceps Tendon Rupture Imaged in FlexionThis patient was unable to extend the elbow because of discomfort. The images were obtained on a high-field scanner with the patient prone and the arm flexed overhead. Proton density (A) and fat-suppressed T2-weighted (B) coronal images reveal a fluid-filled tear of the distal triceps tendon (arrows) from the olecranon (O). From Edelman RR, Hesselink JR, Zlatkin MB, et al, eds. Clinical Magnetic Resonance Imaging. 3rd ed. Philadelphia: Saunders; 2006:3302.

FIG. 45.3 The pain of triceps tendinitis is constant and severe and is localized in the posterior elbow.

Testing Plain radiographs, ultrasound imaging, and magnetic resonance imaging (MRI) are indicated for all patients who present with posterior elbow pain (see Figs. 45.1 and 45.2). Based on the patient’s clinical presentation, additional tests, including complete blood count, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. MRI and ultrasound imaging of the elbow (Fig. 45.4) are indicated if joint instability is suspected and to confirm the diagnosis. Radionuclide bone scanning is useful to identify stress fractures of the elbow not seen on plain radiographs.

FIG. 45.4 Ultrasound image demonstrating the insertion of the distal triceps tendon with tearing. Note the cortical defect (∗). From Waldman SD. Triceps tendon injection. In: Waldman SD, ed. Atlas of Pain Management Injection Techniques. 4th ed. Philadelphia: Elsevier; 2017:206, fig 577.

Differential Diagnosis Triceps tendinitis generally is easily identified on clinical grounds, but coexistent bursitis may confuse the diagnosis. Stress fractures of the olecranon also may mimic triceps tendinitis and may be identified on plain radiographs or radionuclide bone scanning.

Treatment Initial treatment of the pain and functional disability associated with triceps tendinitis should include a combination of nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and physical therapy. The local application of heat and cold also may be beneficial. Patients should be encouraged to avoid repetitive activities responsible for the evolution of the tendinitis. For patients who do not respond to these treatment modalities, injection with local anesthetic and steroid may be a reasonable next step.

Complications and Pitfalls Trauma to the triceps tendon from the injection itself is possible. Tendons that are highly inflamed or previously damaged are subject to rupture if they are directly injected. This complication can be greatly decreased if the clinician uses gentle technique and stops injecting immediately if significant resistance to injection is encountered. Approximately 25% of patients report a transient increase in pain after this injection technique, and patients should be warned of this possibility.

Clinical Pearls The triceps tendon is a very strong tendon, but it is also very susceptible to rupture. Coexistent bursitis and arthritis also may contribute to posterior elbow pain and may require additional treatment with a more localized injection of local anesthetic and methylprednisolone acetate. Injection of the triceps tendon is a safe procedure if careful attention is paid to the clinically relevant anatomy in the areas to be injected. The use of physical modalities, including local heat and gentle range-of-motion exercises, should be introduced several days after the patient undergoes this injection technique for elbow pain. Vigorous exercises should be avoided because they would exacerbate the patient’s symptoms. Simple analgesics and NSAIDs may be used concurrently with this injection technique.

Suggested Readings Badia A, Stennett C. Sports-related injuries of the elbow. J Hand Ther. 2006;19:206–227. Jafarnia K, Gabel G.T, Morrey B.F. Triceps tendinitis. Oper Tech Sports Med. 2001;9:217–221. Potter H.G, Schachar J, Jawetz S. Imaging of the elbow. Oper Tech Orthop. 2009;19:199–208. Waldman S.D. Functional anatomy of the elbow. In: Waldman S.D, ed. Pain Review. Philadelphia: Elsevier; 2016:98–100.

46

Radial Tunnel Syndrome

Abstract In radial tunnel syndrome, the posterior interosseous branch of the radial nerve is entrapped by a variety of mechanisms that have in common a similar clinical presentation. These mechanisms include aberrant fibrous bands in front of the radial head, anomalous blood vessels that compress the nerve, extrinsic masses, or a sharp tendinous margin of the extensor carpi radialis brevis. These entrapments may exist alone or in combination. The lateral elbow pain of radial tunnel syndrome is aching and localized to the deep extensor muscle mass. The pain may radiate proximally and distally into the upper arm and forearm). The intensity of the pain of radial tunnel syndrome is mild to moderate, but it may produce significant functional disability.

Keywords diagnostic ultrasound; entrapment neuropathy; extensor carpi radialis brevis; persistent tennis elbow; pronator syndrome tennis elbow; radial nerve; Radial tunnel syndrome; superficial radial nerve arcade of Froshe; ultrasound guided injection

ICD-10 CODE G56.90

The Clinical Syndrome Radial tunnel syndrome is an uncommon cause of lateral elbow pain that has the unique distinction among entrapment neuropathies of almost always being initially misdiagnosed. The incidence of misdiagnosis of radial tunnel syndrome is so common that it is often incorrectly referred to as resistant tennis elbow (Table 46.1). As seen from the following discussion, the only major similarity that radial tunnel syndrome and tennis elbow share is the fact that both clinical syndromes produce lateral elbow pain. The lateral elbow pain of radial tunnel syndrome is aching and localized to the deep extensor muscle mass. The pain may radiate proximally and distally into the upper arm and forearm (Fig. 46.1). The intensity of the pain of radial tunnel syndrome is mild to moderate, but it may produce significant functional disability. In radial tunnel syndrome, the posterior interosseous branch of the radial nerve is entrapped by a variety of mechanisms that have in common a similar clinical presentation (Fig. 46.2). These mechanisms include aberrant fibrous bands in front of the radial head, anomalous blood vessels that compress the nerve, extrinsic masses, or a sharp tendinous margin of the extensor carpi radialis brevis. These entrapments may exist alone or in combination.

Signs and Symptoms Regardless of the mechanism of entrapment of the radial nerve, the common clinical feature of radial tunnel syndrome is pain just below the lateral epicondyle of the humerus. The pain of radial tunnel syndrome may develop after an acute twisting injury or direct trauma to the soft tissues overlying the posterior interosseous branch of the radial nerve, or the onset may be more insidious, without an obvious inciting factor. The pain is constant and worsens with active supination of the wrist. Patients often note the inability to hold a coffee cup or hammer. Sleep disturbance is common. On physical examination, elbow range of motion is normal. Grip strength on the affected side may be diminished. In the classic text on entrapment neuropathies, Dawson and colleagues note three important signs that allow the clinician to distinguish radial tunnel syndrome from tennis elbow: (1) tenderness to palpation distal to the radial head in the muscle mass of the extensors, rather than over the more proximal lateral epicondyle, as in tennis elbow; (2) increasing pain on active resisted supination of the forearm owing to compression of the radial nerve by the arcade of Frohse as a result of contraction of the muscle mass; and (3) a positive result on the middle finger test. The middle finger test is performed by having the patient extend the forearm, wrist, and middle finger and sustain this action against resistance. Patients with radial tunnel syndrome exhibit increased lateral elbow pain secondary to fixation and compression of the radial nerve by the extensor carpi radialis brevis muscle (Fig. 46.3).

TABLE 46.1 Characteristics of Radial Tunnel Syndrome and Lateral Epicondylitis Characteristic

Radial Tunnel Syndrome

Lateral Epicondylitis (Tennis Elbow)

Frequency

Rare (2% of all peripheral nerve compressions of the upper limb) Compression of the radial nerve

Common cause of lateral elbow pain

Cause

Anybody with repetitive, stressful pronation and supination (e.g., tennis players, Frisbee players, swimmers, powerlifters) Pain over the neck of the radius and lateral Pain location aspect of the proximal forearm over the extensor muscles themselves (distal to where the pain is located in lateral epicondyle) Pain can radiate proximally and (more Pain radiation commonly) distally Provocative tests Pain with resisted extension of the middle finger with the forearm pronated and the elbow (much extended. Pain with resisted forearm supination overlap with the elbow fully extended between the two entities) Characteristic patient

Caused by overuse of the extensor and supinator muscles Tennis players Pain and tenderness over the lateral epicondyle and immediately distal to it (at the origin of the extensor muscles) Usually localized without radiation Pain with resisted wrist extension or elbow supination with the elbow extended. Pain with forceful wrist flexion or forearm pronation

Modified from Mileti J, Largacha M, O’Driscoll SW. Radial tunnel syndrome caused by ganglion cyst: treatment by arthroscopic cyst decompression. Arthroscopy. 2004;20:e39–e44.

Testing Because of the ambiguity and confusion surrounding this clinical syndrome, testing is important to help confirm the diagnosis of radial tunnel syndrome. Electromyography helps distinguish cervical radiculopathy and radial tunnel syndrome from tennis elbow. Plain radiographs are indicated in all patients who present with radial tunnel syndrome to rule out occult bony pathology. Based on the patient’s clinical presentation, additional testing, including complete blood cell count, uric acid, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated.

FIG. 46.1 The pain of radial tunnel syndrome is localized to the deep extensor muscle mass and may radiate proximally and distally into the upper arm and forearm.

FIG. 46.2 Recurrent radial tunnel syndrome with posterior interosseous nerve (PIN) entrapment in a 44-year-old man with painful forearm and elbow following prior radial tunnel release. Axial T2 spectral attenuated inversion recovery (SPAIR) (A) image shows normal radial nerve (large arrow) and abnormal ulnar nerve (small arrow) at the level of elbow, consistent with cubital tunnel syndrome. Axial T2 SPAIR (B) and T1-weighted (C) images at proximal forearm show the postoperative scarring (large arrows), mildly hyperintense superficial radial nerve (small arrows), and markedly abnormal PIN (double small arrows). From Chalian M, Behzadi AH, Williams EH, et al. High-resolution magnetic resonance neurography in upper extremity neuropathy. Neuroimaging Clin N Am. 2014;24[1]:109–125, fig 13.

FIG. 46.3 Ultrasound image demonstrating the relationship of the radial nerve to the arcade of Frohse.

Magnetic resonance imaging (MRI) of the elbow is indicated if internal derangement of the joint is suspected and may help identify the factors responsible for the nerve entrapment, such as ganglion cysts or lipomas (see Fig. 46.2–C). The injection technique of the radial nerve at the elbow with a local anesthetic and steroid may help confirm the diagnosis and treat the syndrome.

Differential Diagnosis Cervical radiculopathy and tennis elbow can mimic radial tunnel syndrome. Radial tunnel syndrome can be distinguished from tennis elbow because with radial tunnel syndrome, the maximal tenderness to palpation is distal to the lateral epicondyle over the posterior interosseous branch of the radial nerve, whereas with tennis elbow, the maximal tenderness to palpation is over the lateral epicondyle. Increased pain with active supination and a positive middle finger test (see earlier discussion) helps strengthen the diagnosis of radial tunnel syndrome. Acute gout affecting the elbow manifests as a diffuse acute inflammatory condition that may be difficult to distinguish from infection of the joint, rather than a localized nerve entrapment.

Treatment Initial treatment of the pain and functional disability associated with radial tunnel syndrome should include a combination of nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and physical therapy. The local application of heat and cold also may be beneficial. Patients should avoid the repetitive movements that incite the syndrome. For patients who do not respond to these treatment modalities, injection of the radial nerve at the elbow with a local anesthetic and steroid may be a reasonable next step. If the symptoms of radial tunnel syndrome persist, surgical exploration and decompression of the radial nerve are indicated.

Complications and Pitfalls The major complications associated with radial tunnel syndrome fall into two categories: (1) iatrogenically induced complications resulting from persistent and overaggressive treatment of “resistant tennis elbow” and (2) the potential for permanent neurological deficits as a result of prolonged untreated entrapment of the radial nerve. Failure of the clinician to recognize an acute inflammatory or infectious arthritis of the elbow may result in permanent damage to the joint and chronic pain and functional disability.

Clinical Pearls Radial tunnel syndrome is a distinct clinical entity that is often misdiagnosed as tennis elbow, and this fact accounts for the many patients with “tennis elbow” who fail to respond to conservative measures. Radial tunnel syndrome can be distinguished from tennis elbow because with radial tunnel syndrome, the maximal tenderness to palpation is over the radial nerve, whereas with tennis elbow, the maximal tenderness to palpation is over the lateral epicondyle. If radial tunnel syndrome is suspected, injection of the radial nerve at the humerus with a local anesthetic and steroid gives almost instantaneous relief. Careful neurological examination to identify preexisting neurological deficits that may later be attributed to the nerve block should be performed on all patients before beginning radial nerve block at the humerus.

Suggested Readings Clavert P, Lutz J.C, Adam P. Frohse’s arcade is not the exclusive compression site of the radial nerve in its tunnel. Orthop Traumatol Surg Res. 2009;95:114–118. Deniel A, Causeret A, Moser T, Rolland Y, Dréano T, Guillin R. Entrapment and traumatic neuropathies of the elbow and hand: an imaging approach. Diagn Interv Imaging. 2015;96(12):1261–1278. Huisstede B, Miedema H.S, van Opstal T. Interventions for treating the radial tunnel syndrome: a systematic review of observational studies. J Hand Surg. 2008;33:e1–72.e10 72. Kumar S.D, Bourke G. Nerve compression syndromes at the elbow. Orthop Trauma. 2016;30(4):355–362. Lee J.T, Azari K, Ford Jones N. Long term results of radial tunnel release: the effect of co-existing tennis elbow, multiple compression syndromes and workers’ compensationa. J Plast Reconstr Aesthet Surg. 2008;61:1095–1099. Marchese J, Wolf J.M, Coyle K, Cote M. Prospective evaluation of single corticosteroid injection in radial tunnel syndrome: level 4 evidence. J Hand Surg. 2017;42(9):S51 (suppl). Tennent T.D, Woodgate A. Posterior interosseous nerve dysfunction in the radial tunnel. Curr Orthop. 2008;22:226–232. Thurston A. Radial tunnel syndrome. Orthop Trauma. 2013;27(6):403–408. Waldman S.D. Radial tunnel syndrome. In: Waldman S.D, Campbell R.S.D, eds. Imaging of Pain. Philadelphia: Saunders; 2011:287–288. Waldman S.D. Radial tunnel syndrome. In: Waldman S.D, ed. Pain Review. 2nd ed. Philadelphia: Elsevier; 2016:2252–2254.

47

Cubital Tunnel Syndrome

Abstract Cubital tunnel syndrome is an uncommon cause of lateral forearm pain and weakness that can be quite distressing to the patient. This entrapment neuropathy manifests as pain and associated paresthesias in the lateral forearm that radiates to the wrist and ring and little fingers. The symptoms are often aggravated by prolonged flexion of the elbow. The pain of cubital tunnel syndrome has been characterized as unpleasant and dysesthetic. The onset of symptoms is usually after repetitive elbow motions or from repeated pressure on the elbow, such as using the elbows to arise from bed. Direct trauma to the ulnar nerve as it enters the cubital tunnel may result in a similar clinical presentation as can compression of the ulnar nerve as it travels thtough the cubital tunnel.(Fig. 47.1) Untreated, progressive motor deficit and ultimately flexion contracture of the affected fingers can result. Cubital tunnel syndrome is most often caused by compression of the ulnar nerve by an aponeurotic band that runs from the medial epicondyle of the humerus to the medial border of the olecranon.

Key Words Cubital tunnel syndrome; cubital tunnel; diagnostic ultrasound; electromyography; entrapment neuropathy; forearm pain; gout; ulnar neuropathy; ultrasound guided injection; upper extremity pain

ICD-10 CODE G56.2

The Clinical Syndrome Cubital tunnel syndrome is an uncommon cause of lateral forearm pain and weakness that can be quite distressing to the patient. This entrapment neuropathy manifests as pain and associated paresthesias in the lateral forearm that radiate to the wrist and ring and little fingers. The symptoms are often aggravated by prolonged flexion of the elbow. The pain of cubital tunnel syndrome has been characterized as unpleasant and dysesthetic. The onset of symptoms is usually after repetitive elbow motions or from repeated pressure on the elbow, such as using the elbows to arise from bed. Direct trauma to the ulnar nerve as it enters the cubital tunnel may result in a similar clinical presentation. Untreated, progressive motor deficit and ultimately flexion contracture of the affected fingers can result. Cubital tunnel syndrome is most often caused by compression of the ulnar nerve by an aponeurotic band that runs from the medial epicondyle of the humerus to the medial border of the olecranon (Fig. 47.1).

Signs and Symptoms Physical findings include tenderness over the ulnar nerve at the elbow. A positive Tinel sign over the ulnar nerve as it passes beneath the aponeuroses is usually present. Weakness of the intrinsic muscles of the forearm and hand that are innervated by the ulnar nerve may be identified with careful manual muscle testing, although early in the course of the evolution of cubital tunnel syndrome, the only physical finding other than tenderness over the nerve may be the loss of sensation on the ulnar side of the little finger. As the syndrome progresses, the affected hand may take on a claw-like appearance (Fig. 47.2). A positive Wartenberg sign indicative of weakness of the adduction of the fifth digit is often present (Figs. 47.3 and 47.4). A positive scratch collapse test is often present (Fig. 47.5).

Testing Electromyography helps distinguish cervical radiculopathy and cubital tunnel syndrome from golfer ’s elbow. Plain radiographs are indicated in all patients with cubital tunnel syndrome to rule out occult bony pathological processes, such as osteophytes impinging on the ulnar nerve. Based on the patient’s clinical presentation, additional tests, including complete blood cell count, uric acid level, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. Magnetic resonance imaging (MRI) of the elbow is indicated if joint instability is suspected and to identify the cause of ulnar nerve entrapment (Fig. 47.6). Ultrasound evaluation is also useful if the diagnosis is in question (Fig. 47.7). Injection of the ulnar nerve serves as a diagnostic maneuver and a therapeutic maneuver.

Differential Diagnosis Cubital tunnel syndrome is often misdiagnosed as golfer ’s elbow, which accounts for the many patients with “golfer ’s elbow” who fail to respond to conservative measures. Cubital tunnel syndrome can be distinguished from golfer ’s elbow, because in cubital tunnel syndrome, the maximal tenderness to palpation is over the ulnar nerve 1 inch below the medial epicondyle, whereas with golfer ’s elbow, the maximal tenderness to palpation is directly over the medial epicondyle. Cubital tunnel syndrome also should be differentiated from cervical radiculopathy involving the C7 or C8 roots and golfer ’s elbow. Cervical radiculopathy and ulnar nerve entrapment may coexist as the “double crush” syndrome. The double crush syndrome is seen most commonly with median nerve entrapment at the wrist or carpal tunnel syndrome.

FIG. 47.1 Intraoperative photograph of the extraneural ganglion, which is approximately 1.0 cm × 0.9 cm × 1.1 cm in size, compressing and flattening the ulnar nerve in the cubital tunnel. From Chang KW, Li YP, Zhang DF, et al. The cubital tunnel syndrome caused by the intraneural or extraneural ganglion cysts: case report and review of the literature. J Plast Reconstr Aesthet Surg. 2017;70[10]:1404–1408, fig 3. https://doi.org/10.1016/j.bjps.2017.05.006.

FIG. 47.2 Patients with cubital tunnel syndrome exhibit weakness of the intrinsic muscles of the forearm, and the hand may take on a claw-like appearance.

FIG. 47.3 Involuntary Abduction Is Often Present in Cubital Tunnel Syndrome. From Waldman SD. Physical Diagnosis of Pain: An Atlas of Signs and Symptoms. Philadelphia: Saunders; 2006:127.

Treatment Initial treatment of the pain and functional disability associated with cubital tunnel syndrome should include a combination of nonsteroidal anti inflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and physical therapy. Local application of heat and cold also may be beneficial. The repetitive movements that incite the syndrome should be avoided. For patients who do not respond to these treatment modalities, injection of the ulnar nerve at the elbow with a local anesthetic and steroid may be a reasonable next step. If the symptoms of cubital tunnel syndrome persist, surgical exploration and decompression of the ulnar nerve are indicated.

Complications and Pitfalls The major complications associated with cubital tunnel syndrome fall into two categories: (1) iatrogenically induced complications resulting from persistent and overaggressive treatment of “resistant golfer ’s elbow” and (2) the potential for permanent neurological deficits as a result of prolonged untreated entrapment of the ulnar nerve. Failure of the clinician to recognize an acute inflammatory or infectious arthritis of the elbow may result in permanent damage to the joint and chronic pain and functional disability.

FIG. 47.4 A Positive Wartenberg Sign Is Indicative of Cubital Tunnel Syndrome. From Waldman SD. Physical Diagnosis of Pain: An Atlas of Signs and Symptoms. Philadelphia: Saunders; 2006:128.

FIG. 47.5 Scratch Collapse Test of the Right Arm for Ulnar Nerve Impingement at the Cubital Tunnel.(A) Patient resists internal rotation with arm adducted at the side and the elbow flexed to 90 degrees. (B) Examiner scratches over the ulnar nerve at the cubital tunnel. (C) Positive test is an inability to resist internal rotation. From Kancherla VK, Caggiano NM, Matullo KS. Elbow injuries in the throwing athlete. Orthop Clin North Am. 2014;45[4]:571–585, fig 4.

FIG. 47.6 Thickening of the Cubital Tunnel Retinaculum.(A) T1-weighted axial MR image reveals the ulnar nerve (white arrow) deep to a thickened cubital tunnel retinaculum (arrowheads) and superficial to the posterior bundle of the medial collateral ligament (curved arrow). (B) Axial image further distally in the same patient reveals the ulnar nerve (white arrow) deep to a normal, thin aponeurosis of the flexor carpi ulnaris (small black arrows) and superficial to a mildly thickened medial joint capsule (open arrow). From Edelman RR, Hesselink JR, Zlatkin MB, et al., eds. Clinical Magnetic Resonance Imaging. 3rd ed. Philadelphia: Saunders; 2006:3303.

FIG. 47.7 Longitudinal Ultrasound Image Demonstrating Severe Cubital Tunnel Syndrome Caused by a Large Gouty Tophus. From Wiesler ER, Chloros GD, Cartwright MS, et al. Ultrasound in the diagnosis of ulnar neuropathy at the cubital tunnel. J Hand Surg. 2006;31:1088–1093.

Clinical Pearls Cubital tunnel syndrome is a distinct clinical entity that is often misdiagnosed as golfer ’s elbow, which accounts for the many patients with “golfer ’s elbow” who fail to respond to conservative measures. Cubital tunnel syndrome can be distinguished from golfer ’s elbow because in cubital tunnel syndrome, the maximal tenderness to palpation is over the ulnar nerve and a positive Tinel sign is present, whereas with golfer ’s elbow, the maximal tenderness to palpation is over the medial epicondyle. If cubital tunnel syndrome is suspected, injection of the radial nerve at the elbow with a local anesthetic and steroid gives almost instantaneous relief. Careful neurological examination to identify preexisting neurological deficits that may later be attributed to the nerve block should be performed on all patients before beginning ulnar nerve block at the elbow.

Suggested Readings Chang W.K, Li Y.P, Zhang D.F, Liang B.S. The cubital tunnel syndrome caused by the intraneural or extraneural ganglion cysts: case report and review of the literature. J Plast Reconstr Aesthet Surg. 2017;70(10):1404–1408. Hariri S, McAdams T.R. Nerve injuries about the elbow. Clin Sports Med. 2010;29:655–675. Heithoff S.J. Cubital tunnel syndrome: ulnar nerve subluxation. J Hand Surg. 2010;35:1556. Kancherla V.K, Caggiano N.M, Matullo K.S. Elbow injuries in the throwing athlete. Orthop Clin North Am. 2014;45(4):571–585. Lauretti L, D’Alessandris Q.G, De Simone C, et al. Ulnar nerve entrapment at the elbow. A surgical series and a systematic review of the literature. J Clin Neurosci. 2017;46:99–108. . Maslow J.I, Johnson D.J, Block J.J, et al. Prevalence and clinical manifestations of the anconeus epitrochlearis and cubital tunnel syndrome: level 4 evidence. J Hand Surg. 2017;42(9):S9–S10 Supplement. Osma-Rueda J.L, Amaya-Mujica J. Entrapment of the ulnar nerve in cubital tunnel by free intra-articular body—a case report. JSES Open Access. 2017;1(2):109–112. Palmer B.A, Hughes T.B. Cubital tunnel syndrome. J Hand Surg. 2010;35:153– 163. Schertz M, Mutschler C, Masmejean E, Silvera J. High-resolution ultrasound in etiological evaluation of ulnar neuropathy at the elbow. Eur J Radiol. 2017;95:111–117. Terlemez R, Yilmaz F, Dogu B, Kuran B. Comparison of ultrasonography and short-segment nerve conduction study in ulnar neuropathy at the elbow. Arch Phys Med Rehabil. 2018;99(1):116–120.

48

Driver’s Elbow

Abstract The ulnar nerve is susceptible to compression when a driver or passenger rests his or her elbow on the lower sill of the vehicle window while the shoulder is abducted and the elbow flexed. When the elbow is flexed, the proximal edge of the arcuate ligament becomes taut and the total volume of the cubital tunnel is decreased, resulting in increased intratunnel pressure further compromising the ulnar nerve. Vibration transmitted from the car body to the elbow also may further contribute to compromise of the ulnar nerve. This entrapment neuropathy presents as pain and associated paresthesias in the lateral forearm that radiate to the wrist and ring and little finger. Untreated, progressive motor deficit and, ultimately, flexion contracture of the affected fingers can result.

Key Words diagnostic ultrasound; Driver’s elbow; electromyography; entrapment neuropathy; Froment’s sign; Jeanne’s sign; magnetic resonance imaging; Tinel sign; ulner neuropathy; ultrasound guided injection

ICD-10 CODE G56.20

The Clinical Syndrome The ulnar nerve is susceptible to compression when a driver or passenger rests his or her elbow on the lower sill of the vehicle window while the shoulder is abducted and the elbow flexed. When the elbow is flexed, the proximal edge of the arcuate ligament becomes taut and the total volume of the cubital tunnel is decreased, resulting in increased intratunnel pressure further compromising the ulnar nerve. Vibration transmitted from the car body to the elbow also may further contribute to compromise of the ulnar nerve. This entrapment neuropathy presents as pain and associated paresthesias in the lateral forearm that radiate to the wrist and ring and little finger. Untreated, progressive motor deficit and, ultimately, flexion contracture of the affected fingers can result.

Signs and Symptoms Physical findings associated with driver ’s elbow include tenderness over the ulnar nerve at the elbow. A positive Tinel sign over the ulnar nerve as it passes beneath the aponeuroses is usually present (Fig. 48.1). Weakness of the intrinsic muscles of the forearm and hand that are innervated by the ulnar nerve may be identified with careful manual muscle testing (Table. 48.1). It should be noted that the possibility always exists that a patient with driver ’s elbow also may have a coexistent ulnar, median, or radial nerve lesion distal to the elbow that may confuse the clinical picture. Furthermore, it should be remembered that cervical radiculopathy and ulnar nerve entrapment may coexist as the “double crush” syndrome. The double crush syndrome is seen most commonly with median nerve entrapment at the wrist or with carpal tunnel syndrome. The clinician should be aware that early in the course of the evolution of driver ’s elbow, the only physical finding other than tenderness over the nerve may be the loss of sensation on the ulnar side of the little finger.

Testing Driver ’s elbow should be differentiated from cervical radiculopathy involving the C7 or C8 roots and golfer ’s elbow. Electromyography helps distinguish cervical radiculopathy and driver ’s elbow from golfer ’s elbow. Ultrasound imaging of the elbow may be useful in assessing the status of the ulnar nerve and can provide important anatomical information when combined with the neurophysiological data obtained from electromyography. Plain radiographs and magnetic resonance imaging (MRI) are indicated in all patients with driver ’s elbow to rule out intrinsic pathological conditions of the elbow joint (Fig. 48.2). Based on the patient’s clinical presentation, additional testing, including complete blood count, uric acid level, sedimentation rate, and antinuclear antibody testing, may be indicated. The injection technique described in this chapter serves as both a diagnostic and therapeutic maneuver.

Differential Diagnosis Driver ’s elbow is an entrapment neuropathy resulting from external compression of the ulnar nerve that clinically mimics cubital tunnel syndrome. It is often misdiagnosed as golfer ’s elbow, which accounts for the many patients with “golfer ’s elbow” who fail to respond to conservative measures. Driver ’s elbow can be distinguished from golfer ’s elbow in that in driver ’s elbow, the maximal tenderness to palpation is over the ulnar nerve 1 inch below the medial epicondyle, whereas with golfer ’s elbow, the maximal tenderness to palpation is directly over the medial epicondyle.

FIG. 48.1 Tinel Sign at Elbow. From Waldman SD. Atlas of Pain Management Injection Techniques. 3rd ed. Philadelphia: Saunders; 2013:129.

TABLE. 48.1 Summary of Ulnar Nerve Motor Signs and Tests Grouped by Affected Musculature

DIP, Distal interphalangeal; EDC, extensor digitorum communis; FDP, flexor digitorum profundus; IP, interphalangeal; MP, metacarpophalangeal.

Modified from Goldman SB, Brininger TL, Schrader JW, et al. A review of clinical tests and signs for the assessment of ulnar neuropathy. J Hand Ther. 2009;22:209–220.

FIG. 48.2 (A) Axial T1-weighted magnetic resonance imaging (MRI) of a patient with symptoms of ulnar nerve compression. Soft tissue is seen within the region of the cubital tunnel (white arrow); it is isointense, with normal muscle, and represents an accessory anconeus muscle. The ulnar nerve is not clearly visible. (B) Compare this axial T1-weighted image of a normal elbow with high signal intensity fat suppression (FS) within the cubital tunnel around the ulnar nerve (broken black arrow) and no accessory muscle tissue. The axial (C) and sagittal (D) FS T2weighted MRI demonstrate high signal intensity within the nerve (white arrows) resulting from compression neuritis. LE, Lateral epicondyle; ME, medial epicondyle; O, olecranon. From Waldman SD, Campbell RSD, eds. Imaging of Pain. Philadelphia: Saunders; 2011:290.

Treatment Initial treatment of the pain and functional disability associated with driver ’s elbow should include a combination of nonsteroidal anti inflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and physical therapy. Local application of heat and cold also may be beneficial. The repetitive movements that incite the syndrome should be avoided. For patients who do not respond to these treatment modalities, injection of the ulnar nerve at the elbow with a local anesthetic and steroid may be a reasonable next step. If the symptoms of cubital tunnel syndrome persist, surgical exploration and decompression of the ulnar nerve are indicated.

Complications and Pitfalls The major complications associated with the diagnosis and treatment of patients with driver ’s elbow fall into two categories: (1) iatrogenically induced complications resulting from persistent and overaggressive treatment of “resistant golfer ’s elbow” and (2) the potential for permanent neurological deficits as a result of prolonged untreated entrapment of the ulnar nerve. Failure of the clinician to recognize acute inflammatory or infectious arthritis of the elbow may result in permanent damage to the joint, chronic pain, or functional disability.

Clinical Pearls Driver ’s elbow is a distinct clinical entity often misdiagnosed as golfer ’s elbow, which accounts for the many patients with “golfer ’s elbow” who fail to respond to conservative measures. Driver ’s elbow can be distinguished from golfer ’s elbow because, with cubital tunnel syndrome, the maximal tenderness to palpation is over the ulnar nerve and a positive Tinel sign is present, whereas with golfer ’s elbow, the maximal tenderness to palpation is over the medial epicondyle. Driver ’s elbow also should be differentiated from cervical radiculopathy involving the C8 spinal root, which may at times mimic ulnar nerve compression. Furthermore, it should be remembered that cervical radiculopathy and ulnar nerve entrapment may coexist in double crush syndrome. The double crush syndrome is seen most commonly with median nerve entrapment at the wrist or with carpal tunnel syndrome. Pancoast tumor invading the medial cord of the brachial plexus may also mimic an isolated ulnar nerve entrapment and should be ruled out by apical lordotic chest radiograph. Careful neurological examination to identify preexisting neurological deficits that may later be attributed to the nerve block should be performed on all patients before beginning ulnar nerve block at the elbow. Ulnar nerve entrapment at the elbow is often misdiagnosed as golfer ’s elbow, and this fact accounts for the many patients whose “golfer ’s elbow” fails to respond to conservative measures. Driver ’s elbow can be distinguished from golfer ’s elbow in that in driver ’s elbow, the maximal tenderness to palpation is over the ulnar nerve 1 inch below the medial epicondyle, whereas with golfer ’s elbow, the maximal tenderness to

palpation is directly over the medial epicondyle. If cubital tunnel syndrome is suspected, injection of the ulnar nerve at the elbow with local anesthetic and steroid gives almost instantaneous relief.

Suggested Readings Abdel-Salam A, Eyres K.S, Cleary J. Drivers’ elbow: a cause of ulnar neuropathy. J Hand Surg. 1991;16:436–437. Palmer B.A, Hughes T.B. Cubital tunnel syndrome. J Hand Surg. 2010;35:153– 163. Schertz M, Mutschler C, Masmejean E, Silvera J. High-resolution ultrasound in etiological evaluation of ulnar neuropathy at the elbow. Eur J Radiol. 2017;95:111–117. Terlemez R, Yilmaz F, Dogu B, Kuran B. Comparison of ultrasonography and short-segment nerve conduction study in ulnar neuropathy at the elbow. Arch Phys Med Rehabil. 2018;99(1):116–120. Szabo R.M, Kwak C. Natural history and conservative management of cubital tunnel syndrome. Hand Clin. 2007;23:311–318. Lauretti L, D’Alessandris QG, De Simone C, et al. Ulnar nerve entrapment at the elbow. A surgical series and a systematic review of the literature. J Clin Neurosci. 2017;46:99–108. Waldman S.D. Golfer ’s elbow. In: Waldman S.D, ed. Pain Review. 2nd ed. Philadelphia: Elsevier; 2016:267–268. Waldman S.D. The ulnar nerve. In: Waldman S.D, ed. Pain Review. 2nd ed. Philadelphia: Elsevier; 2016:83–84. Waldman S.D. Ulnar nerve entrapment at the elbow. In: Waldman S.D, ed. Pain Review. 2nd ed. Philadelphia: Elsevier; 2016:254–256.

49

Anterior Interosseous Syndrome

Abstract Anterior interosseous syndrome is an uncommon cause of forearm and wrist pain. The onset of symptoms in patients with anterior interosseous syndrome is usually after acute trauma to the forearm or after repetitive forearm and elbow motions, such as using an ice pick. In this setting, the pain and muscle weakness of anterior interosseous syndrome are thought to be secondary to compression of the anterior interosseous nerve just below the elbow by the tendinous origins of the pronator teres muscle and flexor digitorum superficialis muscle of the long finger or by aberrant blood vessels. In some patients, no antecedent trauma is identified, and an inflammatory cause analogous to Parsonage-Turner syndrome has been suggested as the cause of anterior interosseous syndrome in the absence of trauma.

Key Words anterior interosseous nerve; Anterior interosseous syndrome; diagnostic ultrasound; electromyography; entrapment neuropathy; flexor digitorum superficialis muscle; median nerve; pronator teres muscle; ultrasound guided injection

ICD-10 CODE G56.90

The Clinical Syndrome Anterior interosseous syndrome is an uncommon cause of forearm and wrist pain. The onset of symptoms in patients with anterior interosseous syndrome is usually after acute trauma to the forearm or after repetitive forearm and elbow motions, such as using an ice pick. In this setting, the pain and muscle weakness of anterior interosseous syndrome are thought to be secondary to compression of the anterior interosseous nerve just below the elbow by the tendinous origins of the pronator teres muscle and flexor digitorum superficialis muscle of the long finger or by aberrant blood vessels (Fig. 49.1). In some patients, no antecedent trauma is identified, and an inflammatory cause analogous to Parsonage-Turner syndrome has been suggested as the cause of anterior interosseous syndrome in the absence of trauma. Clinically, anterior interosseous syndrome manifests as acute pain in the proximal forearm and deep in the wrist. As the syndrome progresses, patients with anterior interosseous syndrome may report a tired or heavy sensation in the forearm with minimal activity and the inability to pinch items between the thumb and index finger because of paralysis of the flexor pollicis longus and the flexor digitorum profundus (Fig. 49.2).

Signs and Symptoms Physical findings include the inability to flex the interphalangeal joint of the thumb and the distal interphalangeal joint of the index finger resulting from paralysis of the flexor pollicis longus and the flexor digitorum profundus. A positive Playboy and Spinner sign may also be present (Figs. 49.3 and 49.4). Tenderness over the forearm in the region of the pronator teres muscle is seen in some patients with anterior interosseous syndrome. A positive Tinel sign over the anterior interosseous branch of the median nerve approximately 6 to 8 cm below the elbow also may be present.

Testing Electromyography helps distinguish cervical radiculopathy, thoracic outlet syndrome, and carpal tunnel syndrome from anterior interosseous syndrome. Plain radiographs are indicated in all patients who present with anterior interosseous syndrome to rule out occult bony pathology. Based on the patient’s clinical presentation, additional tests, including complete blood cell count, uric acid level, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. Magnetic resonance imaging (MRI) and ultrasound imaging of the forearm is indicated to help clarify the diagnosis and if a primary elbow pathological process or a space-occupying lesion is suspected (Fig. 49.5). Injection of the median nerve at the elbow serves as a diagnostic and therapeutic maneuver.

Differential Diagnosis The anterior interosseous syndrome also should be differentiated from cervical radiculopathy involving the C6 or C7 roots, which sometimes may mimic median nerve compression. Cervical radiculopathy and median nerve entrapment may coexist as the double crush syndrome. The double crush syndrome is seen most commonly with median nerve entrapment at the wrist or carpal tunnel syndrome. Anterior interosseous syndrome can be distinguished from pronator syndrome and median nerve compression by the ligament of Struthers, because the pain of anterior interosseous syndrome occurs more distally and is accompanied by the characteristic loss of ability to pinch items between the thumb and index finger.

FIG. 49.1 Intraoperative photograph showing compression of the median nerve by tendinous fascia off the deep head of pronator teres and the fibrous arch of the flexor digitorum superficialis proximal to it. From Aljawder A, Faqi MK, Mohamed A, et al. Anterior interosseous nerve syndrome diagnosis and intraoperative findings: a case report. Int J Surg Case Rep. 2016;21:44–47.

FIG. 49.2 Patients with anterior interosseous syndrome exhibit acute forearm pain and progressive weakness of pinch.

FIG. 49.3 The “Playboy Bunny” sign is positive when instead of the classic OK sign as seen on the right, the extension of the distal interphalangeal joint and thumb interphalangeal joint on the left forms the elongated nose of a bunny. From Waldman SD. Atlas of Pain Management Injection Techniques. Philadelphia: Elsevier; 2015:245.

FIG. 49.4 The Spinner sign is positive when the index finger of the affected extremity cannot achieve flexion to the palmar crease as the little, ring, and middle fingers can. From Waldman SD. Atlas of Pain Management Injection Techniques. Philadelphia: Elsevier; 2015:245.

FIG. 49.5 (A) Axial T1-weighted magnetic resonance imaging (MRI) of the midforearm in a patient with weakness in muscles in the distribution of the anterior interosseous nerve. The forearm appears normal on the T1-weighted image, but the axial FST2-weighted image (B) shows high signal intensity within the muscles of the flexor pollicis longus (FPL), index finger tendon (FDP2), and middle finger tendon (FDP3) (arrows), which are significantly reduced in bulk. This pattern is typical of anterior interosseous syndrome denervation edema and atrophy. (C) The axial FST2-weighted image of the distal forearm shows similar high signal intensity denervation edema in the pronator quadratus muscle (arrows). From Waldman SD. In: Waldman SD, Campbell RSD, eds. Imaging of Pain. Philadelphia: Saunders; 2011:291.

Treatment Nonsteroidal anti inflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX2) inhibitors represent a reasonable first step in the treatment of anterior interosseous syndrome. The use of the tricyclic antidepressants, such as nortriptyline, at a single bedtime dose of 25 mg, titrating upward as side effects allow, also is useful, especially if sleep disturbance is present. It is important for the patient to avoid repetitive trauma thought to be contributing to this entrapment neuropathy. If these maneuvers fail to produce rapid symptomatic relief, injection of the median nerve at the elbow with a local anesthetic and steroid is a reasonable next step. If symptoms persist, surgical exploration and release of the anterior interosseous branch of the median nerve are indicated.

Complications and Pitfalls Median nerve block below the elbow is a relatively safe block, with major complications being inadvertent intravascular injection and persistent paresthesia secondary to needle trauma to the nerve. This technique can be performed safely in the presence of anticoagulation by using a 25- or 27gauge needle, albeit at increased risk for hematoma, if the clinical situation dictates a favorable risk-to-benefit ratio. These complications can be decreased if manual pressure is applied to the area of the block immediately after injection. Application of cold packs for 20-minute periods after the block also decreases the amount of post-procedure pain and bleeding the patient may experience.

Clinical Pearls Avoidance techniques for the repetitive movements responsible for anterior interosseous syndrome are often forgotten in the rush to treatment. Median nerve block at the elbow is a simple and safe technique in the evaluation and treatment of the aforementioned painful conditions. Careful neurological examination to identify preexisting neurological deficits that may later be attributed to the nerve block should be performed in all patients before beginning median nerve block at the elbow.

Suggested Readings Akman Y.E, Yalcinkaya M, Arikan Y, Kabukcuoglu Y. Atypically localized glomus tumor causing anterior interosseous nerve syndrome: a case report. Acta Orthop Traumatol Turc. 2017;51(6):492–494. Aljawder A, Faqi M.K, Mohamed A, Alkhalifa F. Anterior interosseous nerve syndrome diagnosis and intraoperative findings: a case report. Int J Surg Case Rep. 2016;21:44–47. Chi Y, Harness N.G. Anterior interosseous nerve syndrome. J Hand Surg. 2010;35:2078–2080. Douglas H, Chin C.L, Meals R.A. Anterior interosseous nerve syndrome. J Am Soc Surg Hand. 2001;1:249–257. Feldman M.I, Muhammad K, Beltran J. Preoperative diagnosis of anterior interosseous nerve syndrome resulting in complete recovery. Eur J Radiol Extra. 2009;69:e73–e76. Waldman S.D. Anterior interosseous syndrome. In: Waldman S.D, ed. Pain Review. 2nd ed. Philadelphia: Elsevier; 2016:256–257. Waldman S.D. Anterior interosseous syndrome. In: Waldman S.D, Campbell R.S.D, eds. Imaging of Pain. Philadelphia: Saunders; 2011:291–293.

SECT ION 5

Wrist and Hand Pain Syndromes OUT LINE 50. Ulnar Tunnel Syndrome 51. Cheiralgia Paresthetica 52. Secretan Syndrome 53. Foreign Body Synovitis 54. Glomus Tumor of the Hand 55. Erythromelalgia 56. Boxer’s Knuckle 57. Triangular Fibrocartilage Tear Syndrome 58. Scapholunate Ligament Tear Syndrome 59. Lunotriquetral Instability Pain Syndrome 60. Kienböck Disease 61. Avascular Necrosis of the Scaphoid 62. Extensor Carpi Ulnaris Tendinitis 63. Flexor Carpi Radialis Tendinitis 64. Trigger Wrist

50

Ulnar Tunnel Syndrome

Abstract Ulnar tunnel syndrome is an entrapment neuropathy of the ulnar nerve characterized by pain, numbness, and paresthesias of the wrist that radiate into the ulnar aspect of the palm and dorsum of the hand and the little finger and the ulnar half of the ring finger. These symptoms also may radiate proximal to the nerve entrapment into the forearm. The pain of ulnar tunnel syndrome is often described as aching or burning, with associated “pins and needles” paresthesias. Similar to carpal tunnel syndrome, ulnar tunnel syndrome occurs more commonly in women than in men. Also similar to carpal tunnel syndrome, the pain of ulnar tunnel syndrome is frequently worse at night and worsened by vigorous flexion and extension of the wrist. The onset of symptoms usually follows repetitive wrist motions or from direct trauma to the wrist, such as wrist fractures, or direct trauma to the proximal hypothenar eminence, such as may occur when the hand is used to hammer on hubcaps or from handlebar compression during long-distance cycling. Ulnar tunnel syndrome also is seen in patients with rapid weight gain, rheumatoid arthritis, or Dupuytren’s disease or during pregnancy.

Keywords complications of pregnancy; Dupuytren’s contracture; rheumatoid arthritis; sports injury; ulnar nerve entrapment neuropathy; ulnar tunnel syndrome

ICD-10 CODE G56.20

The Clinical Syndrome Ulnar tunnel syndrome is an entrapment neuropathy of the ulnar nerve characterized by pain, numbness, and paresthesias of the wrist that radiate into the ulnar aspect of the palm and dorsum of the hand and the little finger and the ulnar half of the ring finger. These symptoms also may radiate proximal to the nerve entrapment into the forearm. The pain of ulnar tunnel syndrome is often described as aching or burning, with associated “pins and needles” paresthesias. Similar to carpal tunnel syndrome, ulnar tunnel syndrome occurs more commonly in women than in men. Also similar to carpal tunnel syndrome, the pain of ulnar tunnel syndrome is frequently worse at night and worsened by vigorous flexion and extension of the wrist. The onset of symptoms usually follows repetitive wrist motions or from direct trauma to the wrist, such as wrist fractures, or direct trauma to the proximal hypothenar eminence, such as may occur when the hand is used to hammer on hubcaps or from handlebar compression during long-distance cycling. Ulnar tunnel syndrome also is seen in patients with rapid weight gain, rheumatoid arthritis, or Dupuytren disease or during pregnancy. Untreated, progressive motor deficit and ultimately flexion contracture of the affected fingers can result. Ulnar tunnel syndrome is caused by compression of the ulnar nerve as it passes through Guyon canal at the wrist (Fig. 50.1). The most common causes of compression of the ulnar nerve at this anatomical location include spaceoccupying lesions, such as ganglion cysts and ulnar artery aneurysms; fractures of the distal ulna and carpals; and repetitive motion injuries that compromise the ulnar nerve as it passes through this closed space. This entrapment neuropathy manifests most commonly as a pure motor neuropathy without pain, which is due to compression of the deep palmar branch of the ulnar nerve as it passes through Guyon canal. This pure motor neuropathy manifests as painless paralysis of the intrinsic muscles of the hand. Ulnar tunnel syndrome also may manifest as a mixed sensory and motor neuropathy. Clinically, this mixed neuropathy manifests as pain and the previously described motor deficits.

Signs and Symptoms Physical findings include tenderness over the ulnar nerve at the wrist. A positive Tinel sign over the ulnar nerve as it passes beneath the transverse carpal ligament is usually present. If the sensory branches are involved, decreased sensation occurs into the ulnar aspect of the hand and the little finger and the ulnar half of the ring finger. Depending on the location of neural compromise, the patient may have weakness of the intrinsic muscles of the hand as evidenced by the inability to spread the fingers, weakness of the hypothenar eminence, or both.

FIG. 50.1 The ulnar nerve can be divided into sensory (palmar) and motor (dorsal) branches. Note the fibrous arch of the hypothenar muscles under which the deep motor branch passes on its way out of the ulnar tunnel. The ulnar artery travels along the radial side of the nerve through the tunnel, after which it splits and becomes the deep and superficial palmar arches. Blue tag, Sensory branch; black tag, motor branch; red tag, ulnar artery. From Waugh RP, Pellegrini VD Jr. Ulnar tunnel syndrome. Hand Clin. 2007;23:301– 310.

FIG. 50.2 Entrapment of the ulnar nerve: Guyon canal syndrome (ulnar tunnel syndrome). (A) Ganglion cyst. Transverse T2-weighted (TR/TE, 2000/80) spin echo magnetic resonance imaging shows a ganglion cyst (arrow) adjacent to the ulnar nerve and vessels (arrowhead). (B and C) Anomalous muscle. This accessory muscle (i.e., accessory abductor digiti minimi muscle) (arrows) is well shown in transverse T1-weighted (TR/TE, 550/12) spin echo (B) and fat-suppressed fast spin echo (TR/TE, 3000/11) (C) images. Note the abnormal high signal intensity in the muscle and subjacent Guyon canal in (C). From Resnick D, ed. Diagnosis of Bone and Joint Disorders. 4th ed. Philadelphia: Saunders; 2002:3527.

Testing Electromyography helps distinguish cervical radiculopathy, diabetic polyneuropathy, and Pancoast tumor from ulnar tunnel syndrome. Plain radiographs are indicated in all patients who present with ulnar tunnel syndrome to rule out occult bony pathological processes. Based on the patient’s clinical presentation, additional tests, including complete blood cell count, uric acid level, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. Magnetic resonance imaging (MRI) and ultrasound imaging of the wrist is indicated to help confirm the diagnosis and whether joint instability or a space-occupying lesion is suspected (Figs. 50.2–50.4). The injection technique described here serves as a diagnostic and therapeutic maneuver.

Differential Diagnosis Ulnar tunnel syndrome often is misdiagnosed as arthritis of the carpometacarpal joints, cervical radiculopathy, Pancoast tumor, and diabetic neuropathy. Patients with arthritis of the carpometacarpal joint usually have radiographic evidence and physical findings suggestive of arthritis. Most patients with a cervical radiculopathy have reflex, motor, and sensory changes associated with neck pain, whereas patients with ulnar tunnel syndrome have no reflex changes, and motor and sensory changes are limited to the distal ulnar nerve. Diabetic polyneuropathy generally manifests as symmetrical sensory deficit involving the entire hand, rather than limited in the distribution of the ulnar nerve. Cervical radiculopathy and ulnar nerve entrapment may coexist as the “double crush” syndrome. Because ulnar tunnel syndrome is commonly seen in patients with diabetes, diabetic polyneuropathy usually occurs in patients with diabetes with ulnar tunnel syndrome. Pancoast tumor invading the medial cord of the brachial plexus also may mimic an isolated ulnar nerve entrapment and should be ruled out by apical lordotic chest radiographs.

FIG. 50.3 (A) Axial T2-weighted magnetic resonance imaging through the level of the proximal carpal row in a patient with symptoms of ulnar nerve compression. A high signal intensity lesion (white arrow) adjacent to the ulnar artery and vein (broken white arrows) displaces the ulnar nerve (curved white arrow). (B) The postcontrast (obtained after administration of a contrast agent) T1-weighted image shows low signal intensity within the lesion (white arrow) without enhancement, and the displaced ulnar nerve (curved white arrow) is again demonstrated. The appearances are consistent with a ganglion within Guyon canal. (C) The cystic nature of the lesion is further confirmed on the transverse Doppler ultrasound image, on which the ganglion can be seen as an anechoic mass (white arrow) with flow evident in the ulnar artery and vein (black arrows). L, Lunate; P, pisiform; S, scaphoid; T, triquetrum. Reproduced with permission from Spratt JD, Stanley AJ, Grainger AJ, et al. The role of diagnostic radiology in compressive and entrapment neuropathies. Eur Radiol. 2002;12:2352–2364.

Treatment Initial treatment of the pain and functional disability associated with ulnar tunnel syndrome should include a combination of nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and physical therapy. Local application of heat and cold also may be beneficial. The repetitive movements that incite the syndrome should be avoided. For patients who do not respond to these treatment modalities, injection of the ulnar nerve at the ulnar tunnel with a local anesthetic and steroid may be a reasonable next step. Ultrasound guidance may improve the accuracy of needle placement and decrease the incidence of needle-related complications. If the symptoms of ulnar tunnel syndrome persist, surgical exploration and decompression of the ulnar nerve are indicated.

Complications and Pitfalls The major complication associated with ulnar tunnel syndrome is due to delayed diagnosis and treatment of the disease. This delay can cause permanent neurological deficits resulting from prolonged untreated entrapment of the ulnar nerve. Failure of the clinician to recognize an acute inflammatory or infectious arthritis of the wrist may result in permanent damage to the joint and chronic pain and functional disability.

FIG. 50.4 Transverse Ultrasound Image Demonstrating Compression of the Ulnar Nerve at the Ulnar Tunnel.

Clinical Pearls Ulnar tunnel syndrome should be differentiated from cervical radiculopathy involving the C8 spinal root, which sometimes may mimic ulnar nerve compression. Cervical radiculopathy and ulnar nerve entrapment may coexist in the double crush syndrome. The double crush syndrome is seen most commonly with ulnar nerve entrapment at the wrist or carpal tunnel syndrome. Pancoast tumor invading the medial cord of the brachial plexus also may mimic isolated ulnar nerve entrapment and should be ruled out by apical lordotic chest radiographs.

Suggested Readings Lauretti L, D’Alessandris Q.G, De Simone C, et al. Ulnar nerve entrapment at the elbow. A surgical series and a systematic review of the literature. J Clin Neurosci. 2017;46:99–108. Moghtaderi A, Ghafarpoor M. The dilemma of ulnar nerve entrapment at wrist in carpal tunnel syndrome. Clin Neurol Neurosurg. 2009;111:151–155. Schertz M, Mutschler C, Masmejean E, Silvera J. High-resolution ultrasound in etiological evaluation of ulnar neuropathy at the elbow. Eur J Radiol. 2017;95:111–117. Waldman S.D. Ulnar tunnel syndrome. In: Waldman S.D, Campbell R.S.D, eds. Imaging of Pain. Philadelphia: Saunders; 2011:323–324. Waldman S.D. Injection technique for ulnar tunnel syndrome. In: Waldman S.D, ed. Pain Review. 2nd ed. Philadelphia: Elsevier; 2016:439–440. Waldman S.D. The ulnar tunnel. In: Waldman S.D, ed. Pain Review. 2nd ed. Philadelphia: Elsevier; 2016:108. Waugh R.P, Pellegrini Jr. V.D. Ulnar tunnel syndrome. Hand Clin. 2007;23:301– 310.

51

Cheiralgia Paresthetica

Abstract Cheiralgia paresthetica is an uncommon cause of wrist and hand pain and numbness. It also is known as handcuff neuropathy and Wartenberg syndrome. The onset of symptoms usually occurs after compression of the sensory branch of the radial nerve. Radial nerve dysfunction secondary to compression by tight handcuffs, wristwatch bands, or casts is a common cause of cheiralgia paresthetica. Direct trauma to the nerve may result in a similar clinical presentation. Fractures or lacerations frequently disrupt the nerve completely, resulting in sensory deficit in the distribution of the radial nerve. The sensory branch of the radial nerve also may be damaged during surgical treatment of de Quervain tenosynovitis.

Keywords cheiralgia paresthetica; de Quervain tenosynovitis; diagnostic ultrasound; electromyography; handcuff neuropathy; magnetic resonance imaging; radial nerve; superficial radial nerve; ultrasound guided procedure; Wartenberg syndrome; wristwatch palsy

ICD-10 CODE G58.9

The Clinical Syndrome Cheiralgia paresthetica is an uncommon cause of wrist and hand pain and numbness. It also is known as handcuff neuropathy and Wartenberg syndrome. The onset of symptoms usually occurs after compression of the sensory branch of the radial nerve. Radial nerve dysfunction secondary to compression by tight handcuffs, wristwatch bands, or casts is a common cause of cheiralgia paresthetica. Direct trauma to the nerve may result in a similar clinical presentation. Fractures or lacerations frequently disrupt the nerve completely, resulting in sensory deficit in the distribution of the radial nerve. The sensory branch of the radial nerve also may be damaged during surgical treatment of de Quervain tenosynovitis. Cheiralgia paresthetica manifests as pain and associated paresthesias and numbness of the radial aspect of the dorsum of the hand to the base of the thumb (Fig. 51.1). Because significant interpatient variability exists in the distribution of the sensory branch of the radial nerve owing to overlap of the lateral antebrachial cutaneous nerve, the signs and symptoms of cheiralgia paresthetica may vary from patient to patient.

Signs and Symptoms Physical findings include tenderness over the radial nerve at the wrist. A positive Tinel sign over the radial nerve at the distal forearm is usually present (Fig. 51.2). Decreased sensation in the distribution of the sensory branch of the radial nerve is often present, although, as mentioned, the overlap of the lateral antebrachial cutaneous nerve may result in a confusing clinical presentation. A positive wristwatch sign also may be present (Fig. 51.3). Flexion and pronation of the wrist and ulnar deviation often cause paresthesias in the distribution of the sensory branch of the radial nerve in patients with cheiralgia paresthetica.

Testing Electromyography can help identify the exact source of neurological dysfunction and clarify the differential diagnosis; this should be the starting point of the evaluation of all patients thought to have cheiralgia paresthetica. Plain radiographs are indicated in all patients who present with cheiralgia paresthetica to rule out occult bony pathological processes. Ultrasound imaging is also useful in identifying abnormalities of the superficial radial nerve (Fig. 51.4). Based on the patient’s clinical presentation, additional tests, including complete blood cell count, uric acid level, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. Magnetic resonance imaging (MRI) of the wrist is indicated if joint instability or abnormal mass is suspected. Injection of the sensory branch of the radial nerve at the wrist serves as a diagnostic and therapeutic maneuver and may be used as an anatomical differential neural blockade to distinguish lesions of the sensory branch of the radial nerve from lesions involving the lateral antebrachial cutaneous nerve.

Differential Diagnosis Cheiralgia paresthetica is often misdiagnosed as lateral antebrachial cutaneous nerve syndrome. Cheiralgia paresthetica also should be differentiated from cervical radiculopathy involving the C6 or C7 roots, although patients with cervical radiculopathy generally present not only with pain and numbness but also with reflex and motor changes. Cervical radiculopathy and radial nerve entrapment may coexist as the “double crush” syndrome. The double crush syndrome is seen most commonly with median nerve entrapment at the wrist or carpal tunnel syndrome. It should be remembered that radial nerve compression has many causes and the nerve can be compressed anywhere along its path (Table 51.1).

FIG. 51.1 Cheiralgia paresthetica manifests as pain, paresthesias, and numbness of the radial aspect of the dorsum of the hand to the base of the thumb.

Treatment The first step in the treatment of cheiralgia paresthetica is the removal of the cause of pressure on the radial nerve. A trial of nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors represents a reasonable next step. For patients for whom these treatment modalities fail, injection of the sensory branch of the radial nerve at the wrist with a local anesthetic and steroid should be considered. Ultrasound guidance may improve the accuracy of needle placement and decrease the incidence of needle-related complications.

FIG. 51.2 A positive Tinel sign over the radial nerve at the distal forearm is usually present in patients with cheiralgia paresthetica. From Waldman SD. Physical Diagnosis of Pain: An Atlas of Signs and Symptoms. Philadelphia: Saunders; 2006:168.

For persistent symptoms, surgical exploration and decompression of the nerve are indicated.

Complications and Pitfalls Radial nerve block at the wrist is a relatively safe block, with the major complications being inadvertent intravascular injection and persistent paresthesia secondary to needle trauma to the nerve. This technique can be performed safely in the presence of anticoagulation by using a 25- or 27gauge needle, albeit at increased risk for hematoma, if the clinical situation dictates a favorable risk-to-benefit ratio. These complications can be decreased if manual pressure is applied to the area of the block immediately after injection. Application of cold packs for 20-minute periods after the block also decreases the amount of post-procedure pain and bleeding the patient may experience.

FIG. 51.3 Positive Wristwatch Sign Suggests Cheiralgia Paresthetica. From Waldman SD. Physical Diagnosis of Pain: An Atlas of Signs and Symptoms. Philadelphia: Saunders; 2006:169.

FIG. 51.4 Ultrasound Image Demonstrating the Superficial Radial Nerve.

TABLE 51.1 Causes of Compressive Radial Neuropathies Site High radial nerve

Radial nerve

Posterior interosseous nerve

Superficial branch

Cause Trauma Fractures: Diaphyseal, distal third of the humerus Aneurysms Tumors Infection Inflammation: Local Anomalous muscles and arteries Idiopathic: Nerve torsion or localized constrictions Muscular effort: Lateral triceps Muscular hypertrophy Hereditary neuropathies External compression: Casts, crutches, braces, sleeping positions, tourniquets, walkers Radial tunnel: Pain without muscular weakness Anatomy: (1) Fibrous band, (2) vasculature leash (of Henry), (3) extensor carpi radialis brevis, (4) arcade of Frohse, (5) distal edge of supinator Musculature compression: Rowers, tennis players, weightlifters Metabolic: Pseudogout (joint swelling), rheumatoid arthritis Tumor: Synovial chondromatosis, ganglion, bicipital bursitis Infection: Septic arthritis External compression: Casts Same sites as the radial tunnel

Surgical: Arthroscopy portals Tumor: Scapholunate ganglion, lipoma, intramuscular myxoma, ganglion Metabolic: Pseudogout Arteriovenous malformation, vasculitis Trauma: Dislocated radial head External compression: Casts, weight Idiopathic nerve constriction Wrist ganglion Anatomical: Fascia at brachioradialis/extensor carpi radialis brevis External compression: Casts, watch bands Crush injury

Modified from Markiewitz AD, Merryman J. Radial nerve compression in the upper extremity. J Am Soc Surg Hand. 2005;5:87–99.

Clinical Pearls Radial nerve block at the wrist is an effective treatment for the symptoms of cheiralgia paresthetica. Careful neurological examination to identify preexisting neurological deficits that may later be attributed to the nerve block should be performed in all patients before beginning radial nerve block at the wrist when treating cheiralgia paresthetica. If cheiralgia paresthetica is identified early, removal of the offending pressure and radial nerve block with a local anesthetic and steroid should lead to marked improvement in most patients.

Suggested Readings Arnold W.D, Elsheikh B.H. Entrapment neuropathies. Neurol Clin. 2013;31(2):405–424. Massey E.W, Pleet A.B. Handcuffs and cheiralgia paresthetica. Neurology. 1978;28:1312–1313. Smith M.S. Handcuff neuropathy. Ann Emerg Med. 1981;10:668. Trivedi J.R, Silvestri N.J, Wolfe G.I. Treatment of painful peripheral neuropathy. Neurol Clin. 2013;31(2):377–403. Waldman S.D. Cheiralgia paresthetica. In: Waldman S.D, ed. Pain Review. Philadelphia: Saunders; 2009:275–276. Wang L.H, Weiss M.D. Anatomical, clinical, and electrodiagnostic features of radial neuropathies. Phys Med Rehabil Clin N Am. 2013;24(1):33–47.

52

Secretan Syndrome

Abstract Secretan syndrome, also known as posttraumatic edema syndrome, is caused by a peritendinous fibrosis that occurs after trauma to the dorsum of the hand. Often, the trauma is seemingly minor, such as hitting the back of the hand on the corner of a desk. Initially, the swelling and tenderness may be attributed to the trauma, but instead of improvement with time, the dorsum of the hand becomes more indurated, with the edema becoming brawny. Without treatment, peritendinous fibrosis and an almost myxedematous hardening of the soft tissues of the dorsum of the hand occur. Similar to the pain of Dupuytren contracture, the pain of Secretan syndrome seems to burn itself out as the disease progresses.

Keywords chronic regional pain syndrome; factitious hand injury; magnetic resonance imaging; peritendinous fibrosis; posttraumatic edema syndrome; reflex sympathetic dystrophy; Secretan syndrome

ICD-10 CODE F68.1

The Clinical Syndrome Secretan syndrome, also known as posttraumatic edema syndrome, is caused by a peritendinous fibrosis that occurs after trauma to the dorsum of the hand. Often, the trauma is seemingly minor, such as hitting the back of the hand on the corner of a desk. Initially, the swelling and tenderness may be attributed to the trauma, but instead of improvement with time, the dorsum of the hand becomes more indurated with the edema becoming brawny. Without treatment, peritendinous fibrosis and an almost myxedematous hardening of the soft tissues of the dorsum of the hand occur (Fig. 52.1). Similar to the pain of Dupuytren contracture, the pain of Secretan syndrome seems to burn itself out as the disease progresses.

Signs and Symptoms Brawny edema with associated loss of extensor function of the hand after seemingly minor trauma is the sine qua non of Secretan syndrome. In contrast to reflex sympathetic dystrophy, which can mimic Secretan syndrome, no sudomotor, vasomotor, or trophic nail changes occur, although the skin changes can appear identical to the skin changes of reflex sympathetic dystrophy.

Testing Plain radiographs are indicated in all patients who present with Secretan syndrome to rule out underlying occult bony pathological processes. Based on the patient’s clinical presentation, additional tests, including complete blood cell count, uric acid level, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. Magnetic resonance imaging (MRI) and ultrasound imaging of the hand will help confirm the diagnosis and also are indicated if joint instability, infection, or tumor is suspected. Electromyography is indicated if coexistent ulnar or carpal tunnel syndrome is suspected. Injection of the areas of fibrosis provides improvement of the pain and disability of this disease if implemented early.

Differential Diagnosis Coexistent arthritis, gout of the metacarpal and interphalangeal joints, and tendinitis also may coexist with Secretan syndrome and exacerbate the pain and disability of Secretan syndrome. Reflex sympathetic dystrophy may manifest in a similar clinical manner but can be distinguished from Secretan syndrome because the pain of reflex sympathetic dystrophy responds to sympathetic neural blockade and the pain of Secretan syndrome does not. Given the uncommon nature and unusual presentation of this syndrome, factitious pathoetiology should be considered.

Treatment Initial treatment of the pain and functional disability associated with Secretan syndrome should include a combination of nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and physical therapy. Local application of heat and cold also may be beneficial. For patients who do not respond to these treatment modalities, an injection of a local anesthetic and steroid into the areas of the peritendinous fibrosis may be a reasonable next step. The use of physical therapy, including gentle range-of-motion exercises, should be introduced several days after the patient undergoes this injection technique. Vigorous exercises should be avoided because they would exacerbate the patient’s symptoms.

FIG. 52.1 Secretan syndrome is caused by a peritendinous fibrosis that occurs after trauma to the dorsum of the hand.

Complications and Pitfalls Injection of the peritendinous fibrosis responsible for Secretan syndrome is a relatively safe technique if the clinician is attentive to detail. Such tendons may rupture if directly injected, and needle position should be confirmed outside the tendon before injection to avoid this complication. Another complication of this injection technique is infection. This complication should be exceedingly rare if strict aseptic technique is followed. Approximately 25% of patients report a transient increase in pain after this injection technique, and patients should be warned of this possibility.

Clinical Pearls This injection technique is extremely effective in the treatment of pain and dysfunction secondary to Secretan syndrome. Coexistent arthritis, tendinitis, and gout also may contribute to the pain and may require additional treatment with more localized injection of a local anesthetic and depot steroid. Given the uncommon nature and unusual presentation of this syndrome, factitious pathoetiology should be considered. This technique is a safe procedure if careful attention is paid to the clinically relevant anatomy in the areas to be injected. Care must be taken to use sterile technique to avoid infection and universal precautions to avoid risk to the operator. The incidence of ecchymosis and hematoma formation can be decreased if pressure is placed on the injection site immediately after injection. The use of physical modalities, including local heat, massage, and gentle range-of-motion exercises, should be introduced several days after the patient undergoes this injection technique. Vigorous exercises should be avoided because they would exacerbate the patient’s symptoms. Simple analgesics and NSAIDs may be used concurrently with this injection technique.

Suggested Readings de Oliveira R.K, Bayer L.R, Lauxen D, et al. Factitious lesions of the hand. Rev Bras Ortop (English Edition). 2013;50(4):381–386. Moretta D.N, Cooley R.D. Jr. Secretan’s disease: a unique case report and literature review. Am J Orthop. 2002;31:524–527. O’Connor E.A, Grunert B.K, Matloub H.S, Eldridge M.P. Factitious hand disorders: review of 29 years of multidisciplinary care. J Hand Surg. 2013;38(8):1590–1598. Reading G. Secretan’s syndrome: hard edema of the dorsum of the hand. Plast Reconstr Surg. 1980;65:182–187. Whitney T.M, Jones N.F. Magnetic resonance imaging findings in Secretan’s disease. J Hand Surg. 1995;20:464–466. Winkelmann R.K, Barker S.M. Factitial traumatic panniculitis. J Am Acad Dermatol. 1985;13:988–994.

53

Foreign Body Synovitis

Abstract Foreign body synovitis is an uncommon cause of joint or soft tissue pain encountered in clinical practice. Although foreign body synovitis can occur anywhere in the body when a foreign material is introduced into or near a joint, tendon sheath, or soft tissue surrounding a joint, the hand is most often affected. When this occurs, a chronic, inflammatory monarthritis or tenosynovitis results. Plant thorns, wood splinters, glass, and sea urchin spines are most commonly implicated.

Keywords autoimmune reaction; color Doppler; foreign body synovitis; joint pain; magnetic resonance imaging; monoarthritis; tenosynovitis; ultrasound imaging

ICD-10 CODE M65.9

The Clinical Syndrome Foreign body synovitis is an uncommon cause of joint or soft tissue pain encountered in clinical practice. Although foreign body synovitis can occur anywhere in the body when a foreign material is introduced into or near a joint, tendon sheath, or soft tissue surrounding a joint, the hand is most often affected. When this occurs, a chronic, inflammatory monoarthritis or tenosynovitis results. Plant thorns, wood splinters, glass, and sea urchin spines are most commonly implicated. After the initial injury, a patient with foreign body tenosynovitis may note localized pain in and around the joint. If the patient realizes a foreign body is present, he or she may try to remove it. If a portion of the foreign body is left behind, foreign body synovitis can occur. After the acute injury, a period of quiescence may occur, lasting weeks to months. After this latent period, the patient begins to experience pain and loss of function in the area of the retained foreign body and an inflammatory monoarthritis or tenosynovitis may result.

Signs and Symptoms The diagnosis of foreign body synovitis is easy if the antecedent trauma is recognized. This is not usually the case, however. A patient with foreign body synovitis presents with a localized monoarthritis or synovitis without obvious cause (Fig. 53.1). The patient also may report myalgias or flu-like symptoms in some cases. Examination of other joints fails to reveal evidence of inflammatory arthritis, and the targeted history is negative. A high index of suspicion in any patient with monoarthritis combined with appropriate testing leads the clinician to a correct diagnosis.

FIG. 53.1 Foreign body synovitis manifests as a monoarthritis without apparent cause.

FIG. 53.2 Ultrasound image of the elbow in a patient with a fragment of nonradiopaque cactus thorn in the anterior joint indicated by arrows. The piece of thorn is surrounded by hypoechoic synovitis, with increased flow on color Doppler. From Stevens KJ, McNally EG. Magnetic resonance imaging of the elbow in athletes. Clin Sports Med. 2010;29[4]:521–553, fig 4.

Testing Magnetic resonance imaging (MRI) and ultrasound imaging of the affected joint often reveal the offending foreign body (Fig. 53.2). Vegetable matter such as plant thorns, wood, and glass are not radiopaque and do not show up on plain radiographs; failure to obtain MRI results in a missed diagnosis (Figs. 53.3 and 53.4). Sea urchin spines have a high calcium content and may appear on plain radiographs. Based on the patient’s clinical presentation, additional tests, including complete blood cell count, uric acid level, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. Electromyography is indicated if coexistent ulnar or carpal tunnel syndrome is suspected. Joint aspiration and synovial biopsy may be required to make the diagnosis of foreign body synovitis. Arthroscopy or arthrotomy may be the mechanism by which the diagnosis is finally made.

Differential Diagnosis The recognition of the possibility of antecedent trauma with the introduction of a foreign body makes the diagnosis apparent. Foreign body synovitis must be distinguished from other causes of monoarthritis and synovitis. Table 53.1 lists common causes of monoarthritis. The ultimate differential diagnosis usually requires a careful targeted history and physical examination combined with appropriate laboratory and radiographic testing.

Treatment Initial treatment of the pain and functional disability associated with foreign body synovitis should include a combination of nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and physical therapy. Local application of heat and cold also may be beneficial. For patients who do not respond to these treatment modalities, an injection into the affected area with a local anesthetic and steroid may be a reasonable next step. The use of physical therapy, including gentle range-ofmotion exercises, should be introduced several days after the patient undergoes this injection technique. Vigorous exercises should be avoided because they would exacerbate the patient’s symptoms. Surgical removal of the offending foreign body often is the only intervention that successfully treats foreign body synovitis.

FIG. 53.3 T1-Weighted Axial Post-Gadolinium ImageWhite arrow indicates possible foreign body within area of enhancement. From Yewlett A, Oakley J, Makwana N, et al. Retained blackthorn causing peroneal tendonitis: a case report. Foot Ankle Surg. 2009;15:205–206.

FIG. 53.4 T2-weighted sagittal foot MRI depicting foreign body as a longitudinal structure plantar to first metatarsal similar in appearance to flexor hallucis brevis tendon. Other findings include synovitis with erosions and nonspecific marrow edema of the first metatarsal head and proximal phalanx. From Bode KS, Haggerty CJ, Krause J. Latent foreign body synovitis. J Foot Ankle Surg. 2007;46:291–296.

TABLE 53.1 Common Causes of Monoarthritis Gout Traumatic arthritis Gonococcal arthritis Charcot joint Other crystal arthropathies Sarcoidosis Amyloidosis Osteoarthritis Osteonecrosis Villonodular synovitis Neoplasm Foreign body synovitis

Complications and Pitfalls The main complication associated with foreign body synovitis is the risk for permanent joint damage resulting from delayed diagnosis. Injection of the area of synovitis syndrome is a safe technique if the clinician is attentive to detail. Inflamed tendons may rupture if directly injected, and needle position should be confirmed outside the tendon before injection to avoid this complication. Another complication of this injection technique is infection. This complication should be exceedingly rare if strict aseptic technique is followed. Approximately 25% of patients report a transient increase in pain after this injection technique, and patients should be warned of this possibility.

Clinical Pearls The diagnosis of foreign body synovitis is easy if the clinician thinks of it. By including foreign body synovitis in the differential diagnosis of patients with monoarthritis or tenosynovitis, the diagnosis is more easily recognized. The early use of MRI and ultrasound imaging of the affected area also help increase the diagnostic accuracy of the clinician.

Suggested Readings Bharti A, Mohan K, Kumar S, Kumar V. Plant thorn synovitis of elbow in children. J Clin Orthop Trauma. 2014;5(4):266–269. Bode K.S, Haggerty C.J, Krause J. Latent foreign body synovitis. J Foot Ankle Surg. 2007;46:291–296. Kandel L, Friedman A, Chaimski G, et al. Foreign-body synovitis mimicking septic arthritis of the knee. Arthroscopy. 2001;17:993–996. Olenginski T.P, Bush D.C, Harrington T.M. Plant thorn synovitis: an uncommon cause of monoarthritis. Semin Arthritis Rheum. 1991;21:40–46. Stevens K.J, McNally E.G. Magnetic resonance imaging of the elbow in athletes. Clin Sports Med. 2010;29(4):521–553. Yewlett A, Oakley J, Makwana N, Patel H.J. Retained blackthorn causing peroneal tendonitis: a case report. Foot Ankle Surg. 2009;15:205–206.

54

Glomus Tumor of the Hand

Abstract Glomus tumor of the hand is an uncommon cause of distal finger pain. It is the result of tumor formation of the glomus body, which is a neuromyoarterial apparatus whose function is to regulate peripheral blood flow in the digits. Most patients with glomus tumor are women 30 to 52 years of age. The pain is severe in intensity, lancinating, and boring. The tumor frequently involves the nail bed and may invade the distal phalanx. Patients with glomus tumor of the hand exhibit the classic triad of excruciating distal finger pain, cold intolerance, and tenderness to palpation of the affected digit. Multiple glomus tumors are present in approximately 25% of patients diagnosed with this disease. Glomus tumors also can occur in the foot and occasionally in other parts of the body. Glomus tumors may recur in spite of surgical extirpation.

Keywords finger pain; glomus body; glomus tumor; hand pain; Hildreth test; Love test; neuromyoarterial apparatus

ICD-10 CODE D18.00

The Clinical Syndrome Glomus tumor of the hand is an uncommon cause of distal finger pain. It is the result of tumor formation of the glomus body, which is a neuromyoarterial apparatus whose function is to regulate peripheral blood flow in the digits. Most patients with glomus tumor are women 30 to 52 years of age. The pain is severe in intensity, lancinating, and boring. The tumor frequently involves the nail bed and may invade the distal phalanx. Patients with glomus tumor of the hand exhibit the classic triad of excruciating distal finger pain, cold intolerance, and tenderness to palpation of the affected digit. Multiple glomus tumors are present in approximately 25% of patients diagnosed with this disease. Glomus tumors also can occur in the foot and occasionally in other parts of the body. Glomus tumors may recur despite surgical extirpation.

Signs and Symptoms The diagnosis of glomus tumor of the hand is based primarily on three points in the patient’s clinical history: (1) excruciating pain localized to a distal digit, (2) the ability to trigger the pain by palpating the area (Love test), and (3) marked intolerance to cold. The pain of glomus tumor can be reproduced by placing the affected digit in a glass of ice water. If glomus tumor is present, the characteristic lancinating, boring pain occurs within 30 to 60 seconds. Placing other unaffected fingers of the same hand in ice water does not trigger the pain in the affected finger. Hildreth test also is useful in the diagnosis of glomus tumor. It is performed by placing a tourniquet proximal to the area of suspected tumor. As the distal area becomes ischemic, the sharp lancinating pain characteristic of glomus tumor will occur. Nail bed ridging is present in many patients with glomus tumor of the hand, and a small blue or dark red spot at the base of the nail is visible in 10% to 15% of patients with the disease (Fig. 54.1). The patient with glomus tumor of the hand frequently wears a finger protector on the affected digit and guards against hitting the digit on anything to avoid triggering the pain.

Testing Magnetic resonance imaging (MRI) of the affected digit often reveals the actual glomus tumor and may reveal erosion or a perforating lesion of the phalanx beneath the tumor (Fig. 54.2). The tumor appears as a very high and homogeneous signal on T2-weighted images. Color Doppler imaging will help confirm the diagnosis (Fig. 54.3). The bony changes associated with glomus tumor of the hand also may appear on plain radiographs if a careful comparison of the corresponding contralateral digit is made. Radionuclide bone scan also may reveal localized bony destruction. The ice water test mentioned earlier helps the clinician strengthen the diagnosis. Based on the patient’s clinical presentation, additional tests, including complete blood cell count, uric acid level, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. Electromyography is indicated if coexistent ulnar or carpal tunnel syndrome is suspected. Surgical exploration of the affected digit and nail bed often is necessary to confirm the diagnosis.

Differential Diagnosis The triad of localized excruciating distal digit pain, tenderness to palpation, and cold intolerance makes the diagnosis apparent to an astute clinician. Glomus tumor of the hand must be distinguished from other causes of localized hand pain, including subungual melanoma and osteoid osteoma. If a history of trauma is present, fracture, osteomyelitis, tenosynovitis, and foreign body synovitis should be considered. If there is no history of trauma, gout, other crystal monoarthropathies, tumors, and diseases of the nail and nail bed should be considered. Reflex sympathetic dystrophy should be distinguishable from glomus tumor of the hand because the pain of reflex sympathetic dystrophy is less localized and is associated with trophic skin and nail changes and vasomotor and sudomotor abnormalities. Raynaud syndrome usually involves the entire hand, and the ice water test mentioned typically triggers pain if the “unaffected” finger is tested.

FIG. 54.1 The classic bluish discoloration of the nail plate is seen on the right proximal corner of the nail. From McDermott EM, Weiss AP. Glomus tumors. J Hand Surg Am. 2006;31:1397– 1400.

FIG. 54.2 Glomus Tumor: MRI Abnormalities(A) On a sagittal T1-weighted (TR/TE, 352/25) spin echo magnetic resonance image, a glomus tumor (arrows) led to subtle erosion of the dorsal surface of the distal phalanx. Its signal intensity is identical to that of the nail bed (arrowhead). (B) After intravenous gadolinium administration, a sagittal fat-suppressed T1-weighted (TR/TE, 520/25) spin echo image shows the glomus tumor (arrows) and nail bed (arrowhead) as regions of

high signal intensity. From Resnick D, ed. Diagnosis of Bone and Joint Disorders. 4th ed. Philadelphia: Saunders; 2002:3999.

FIG. 54.3 Color Doppler Image of a Digital Glomus Tumor.

Treatment The mainstay of treatment of glomus tumor is surgical removal. Medication management is uniformly disappointing. Injection of the affected digit in the point of maximal tenderness may provide temporary relief of the pain of glomus tumor and blocks the positive ice water test response, further strengthening the diagnosis.

Complications and Pitfalls The main complication associated with glomus tumor of the hand involves the problems associated with delayed diagnosis, mainly ongoing destruction of the bone and nail bed. Although usually localized and well encapsulated, rarely, these tumors can exhibit aggressive invasive tendencies, making complete excision of the tumor and careful follow-up mandatory.

Clinical Pearls The diagnosis of glomus tumor of the hand is usually straightforward if the clinician identifies the unique nature of its clinical presentation. Glomus tumors are usually solitary tumors, but a rare autosomal dominant condition characterized by multiple glomus tumors affecting more than one body part has been described. The clinician should remember that glomus tumors have been known to recur. Because of the rare potential for aggressive, invasive behavior, complete excision and careful follow-up are important.

Suggested Readings Abou Jaoude J.F, Roula Farah A, Sargi Z, Khairallah S, Fakih C. Glomus tumors: report on eleven cases and a review of the literature. Chirurg Main. 2000;19:243–252. Constantinesco A, Arbogast S, Foucher G, et al. Detection of glomus tumor of the finger by dedicated MRI at 0.1 T. Magn Reson Imaging. 1994;12:1131– 1134. Gandhi J, Yang S.S, Hurd J. The anatomic location of digital glomus tumor recurrences. J Hand Surg. 2010;35(6):986–989. Gandon F, Legaillard Ph, Brueton R, Le Viet D, Foucher G. Forty-eight glomus tumours of the hand: retrospective study and four-year follow-up. Ann Chir Main Memb Super. 1992;11:401–405. Gombos Z, Fogt F, Zhang P.J. Intraosseous glomus tumor of the great toe: a case report with review of the literature. J Foot Ankle Surg. 2008;47:299–301. McDermott E.M, Weiss A.P.C. Glomus tumors. J Hand Surg Am. 2006;31:1397– 1400. Trehan S.K, Athanasian E.A, DiCarlo E.F, Mintz D.N, Daluiski A. Characteristics of glomus tumors in the hand not diagnosed on magnetic resonance imaging. J Hand Surg. 2015;40(3):542–545.

55

Erythromelalgia

Abstract Erythromelalgia is an uncommon pain syndrome that is characterized by the presence of a clinical triad consisting of periodic excruciating extremity pain, redness, and increased temperature. Erythromelalgia is also known as Mitchell’s disease, after Silas Weir Mitchell, the civil war surgeon who is best known for his description of causalgia. The symptoms associated with erythromelalgia are triggered by the exposure of the affected areas to increased temperature, pressure, exertion, insomnia, stress, or mild to moderate activity. Occurring as both a primary and secondary pain syndrome, erythromelalgia most commonly affects the extremities, but may also affect the face and ears. Primary erythromelalgia is an autosomomal dominant condition that is caused by a ion channel channelopathy mutation in the gene SCN9A encoding the voltage-gated sodium channel α-subunit of NaV1.7sodium channel.

Keywords burning pain; erythema; erythromelalgia; Gerhardt disease; Mitchell disease; peripheral vascular disease; SCN9A gene

ICD-10 CODE I73.81

The Clinical Syndrome Erythromelalgia is an uncommon pain syndrome that is characterized by the presence of a clinical triad consisting of periodic excruciating extremity pain, redness, and increased temperature (Fig. 55.1). Erythromelalgia is also known as Mitchell disease, after Silas Weir Mitchell, the civil war surgeon who is best known for his description of causalgia. The symptoms associated with erythromelalgia are triggered by the exposure of the affected areas to increased temperature, pressure, exertion, insomnia, stress, or mild to moderate activity. Occurring as both a primary and secondary pain syndrome, erythromelalgia most commonly affects the extremities, except the face and ears. Primary erythromelalgia is an autosomal dominant condition that is caused by an ion channel channelopathy mutation in the gene SCN9A encoding the voltage-gated sodium channel α-subunit of NaV1.7 (Fig. 55.2). These specific voltage-gated sodium channel subunits are found in the C fibers of peripheral nerves as well as throughout the sympathetic nervous system. Secondary erythromelalgia is associated with a variety of neuropathies and vasculopathies including small fiber peripheral neuropathy, polycythemia vera, essential thrombocytosis as well as autoimmune disorders, paraneoplastic syndrome, and multiple sclerosis. Secondary erythromelalgia has also been associated with mercury intoxication, mushroom poisoning, and hypercholesterolemia, and is a side effect of the calcium channel blocking medications verapamil and nifedipine.

Signs and Symptoms Erythromelalgia is characterized by the presence of a clinical triad consisting of periodic excruciating extremity pain, redness, and increased temperature (Fig. 55.3). It occurs in the lower extremities more commonly than in the upper extremities, face, and ears. In most patients, the symptoms of erythromelalgia present symmetrically, with the patient describing intense burning pain. The pain may also be deep and aching in nature and often has a radiating or lancinating component. Elevation of the affected body part may result in a reduction of the intense rubor. The duration of attacks of erythromelalgia vary from hours to days and occur with varying frequency. The symptoms associated with erythromelalgia are triggered by the exposure of the affected areas to increased temperature, pressure, exertion, insomnia,

stress, or mild to moderate activity. Spicy foods, alcohol, and caffeine are known to trigger attacks as is the wearing of socks or shoes. Application of cold will often provide symptomatic relief in patients suffering from erythromelalgia. Often, ulcers and chronic scarring will be observed in affected areas due to cold injury (Fig. 55.4).

Testing The diagnosis of erythromelalgia is made on clinical grounds as there is no specific test that will accurately diagnose this painful condition. Given the rarity of erythromelalgia, it must be a diagnosis of exclusion. Tests that may help solidify the clinical diagnosis include biopsy of the affected skin, which may reveal decreased capillary density. Quantitative sensory nerve testing, sudomotor testing, laser evoked potentials, and epidermal sensory nerve fiber density testing may all be abnormal due to abnormalities in peripheral nerve C fibers and small sympathetic fibers. Routine testing for autoimmune diseases should be undertaken in all patients suspected of suffering from erythromelalgia. Genetic testing may help elucidate the sodium channelopathy associated with primary erythromelalgia.

FIG. 55.1 The Clinical Elements of Erythromelalgia.

FIG. 55.2 Clinical Characteristics and Genetic and Biophysical Features of Inherited Erythromelalgia and Small-Fiber Neuropathy(A) Patient with inherited erythromelalgia and erythema cools feet in cold water in an attempt to relieve pain, which is exacerbated by warmth. (B) Schematic diagram of the Nav1.7 α subunit. Two mutations are shown in the fourth domain—Trp1538Arg in transmembrane segment 2 and Ala1746Gly in transmembrane segment 6—that cause erythromelalgia with varying clinical phenotypes and that have been characterized biophysically. (C) Whole-cell voltage-clamp recordings in HEK293 cells expressing wild-type Nav1.7 or mutant channels. From a holding potential of −120 mV, currents were evoked by voltage increments of 5 mV from −80 mV to 40 mV. (D) Normalized current–voltage plots from recordings in (C) show a hyperpolarizing shift in the voltage dependence of activation. This shift is more pronounced in the mutation

causing early onset (first decade) of inherited erythromelalgia (Ala1746Gly) rather than late onset (sixth decade; Trp1538Arg). (E) Inherited erythromelalgia is not associated with sensory nerve-fiber degeneration, by contrast with small-fiber neuropathy. Epidermal innervations in a healthy individual are detected by immunostaining with the pan-neuronal marker PGP9.5 (red). Intraepidermal fibers (arrows) and dermal fibers (arrowheads) are seen. (F) Gain-of-function mutations in Nav1.7 (distinct from mutations causing inherited erythromelalgia) or Nav1.8 can cause small-fiber neuropathy characterized by degeneration and loss of intraepidermal nerve fibers as shown here. From Bennett DL, Woods CG. Painful and painless channelopathies. Lancet Neurol. 2014;13(6):587–599.

FIG. 55.3 Erythromelalgia is characterized by the presence of a clinical triad consisting of periodic excruciating extremity pain, redness, and increased temperature. The symptoms associated with erythromelalgia are triggered by the exposure of the affected areas to increased temperature, pressure, exertion, insomnia, stress, or mild to moderate activity.

Differential Diagnosis As mentioned previously erythromelalgia is a diagnosis of exclusion. Infection and reflex sympathetic dystrophy and causalgia may mimic erythromelalgia, although these complex regional pain syndromes are almost always unilateral, whereas erythromelalgia is almost always bilateral. Furthermore, heat rarely triggers the pain associated with chronic regional pain syndromes and cold, rather than relieving the pain as in erythromelalgia, worsens the pain of chronic regional pain syndromes. Factitious disease should also be considered, given the rarity and unusual presentation of this uncommon pain syndrome.

FIG. 55.4 Erythromelalgia of the feet: erythema of the skin is observed with erosions and ulcerations caused by frequent immersion of the feet in cold water and cooling by application of ice. From Adamec I, Lakoš Jukic´ I, Habek M. Erythromelalgia as a manifestation of autonomic nervous system involvement in multiple sclerosis. Mult Scler Relat Disord. 2016;8:1–3.

Treatment Initial treatment of the pain and symptoms of erythromelalgia should be focused on symptomatic relief by elevation of the affected area and cooling of the ambient temperature. Autonomic dysfunction and neuropathy associated with erythromelalgia makes the application of cold problematic. By the time the patient seeks medical attention, cold induced injury to the affected area has already occurred as the patient is desperate to obtain pain relief. If the patient insists on immersing the affected areas in cold water, the application of a plastic bag as a barrier to the affected body part may decrease the incidence of cold induced tissue injury. Anecdotal reports suggest that aspirin, mexiletine, misoprostol, intravenous lidocaine, magnesium sulfate, gabapentin, and venlafaxine may provide some amelioration of the pain and symptoms associated with erythromelalgia. A careful search for underlying diseases, medications, substances, and other triggers of the patient’s pain and symptoms should be immediately undertaken and, when these triggers are identified, promptly removed and/or treated.

Complications and Pitfalls Misdiagnosis of erythromelalgia is a problem as is the failure to identify underlying treatable diseases that may be triggering the disease. Mercury poisoning is notoriously hard to diagnosis on purely clinical grounds and is often missed. Cold induced injury is the rule rather than the exception in patients suffering from both primary and secondary erythromelalgia as patients are desperate to obtain pain relief. Factitious disease must always be considered and can be extremely difficult to diagnose.

Clinical Pearls Erythromelalgia is a rare uncommon pain syndrome that is difficult to diagnose and difficult to treat. The identification of a primary form of the disease that is the result of a sodium channelopathy may lead to more targeted treatment options. Another mutation of the same gene responsible for erythromelalgia is thought to be responsible for a heritable inability to feel pain.

Suggested Readings Adamec I, Lakoš Jukić I, Habek M. Erythromelalgia as a manifestation of autonomic nervous system involvement in multiple sclerosis. Mult Scler Relat Disord. 2016;8:1–3. Bennett D.L, Woods C.G. Painful and painless channelopathies. Lancet Neurol. 2014;13(6):587–599. Breivik H. Erythromelalgia – a dramatic pain of genetic origin, revealing pain mechanisms with implications for neuropathic pain in general. Scand J Pain. 2014;5(4):215–216. Pagani-Estévez GL, Sandroni P, et al. Erythromelalgia: identification of a corticosteroid-responsive subset. J Am Acad Dermatol. 2017;76(3):506– 511 e1. Patel N, Femia A.N, Eastham A.B, et al. Facial erythromelalgia: a rare entity to consider in the differential diagnosis of connective tissue diseases. J Am Acad Dermatol. 2014;71(6):e250–e251. Senet P. Diagnostic des acrosyndromes vasculaires. Annales de Dermatologie et de Vénéréologie. 2015;142(8–9):513–518.

56

Boxer’s Knuckle

Abstract It is not surprising that given the tremendous forces placed on a boxer ’s clenched fist when throwing a punch that traumatic injury can occur. Along with fractures of the metacarpals and phalanges, carpal boss and boxer ’s knuckle are the most common hand injuries of boxers seen in clinical practice. Boxer ’s knuckle is characterized by localized tenderness and sharp pain over metacarpophalangeal joints with subluxation or dislocation of the longitudinal central tendon following to rupture of the extensor hood mechanism and associated longitudinal central tendon dysfunction. Along with the sagittal bands, the longitudinal central tendon serves as a shock absorber that protects the underlying articular capsule and surfaces. When these structures are damaged by the trauma of a punch, the central tendon subluxes or dislocates, leaving the underlying joint unprotected.

Keywords boxer’s knuckle; carpometacarpal joint arthrodesis; combat sports; martial arts; dorsal capsular rupture of the metacarpophalangeal joint; plain radiography; sports injury

ICD-10 CODE M20.019

The Clinical Syndrome It is not surprising that given the tremendous forces placed on a boxer ’s clenched fist when throwing a punch that traumatic injury can occur. Along with fractures of the metacarpals and phalanges, carpal boss and boxer ’s knuckle are the most common hand injuries of boxers seen in clinical practice. Boxer ’s knuckle is characterized by localized tenderness and sharp pain over metacarpophalangeal joints with subluxation or dislocation of the longitudinal central tendon leading to rupture of the extensor hood mechanism and associated longitudinal central tendon dysfunction (Fig. 56.1). Along with the sagittal bands, the longitudinal central tendon serves as a shock absorber that protects the underlying articular capsule and surfaces. When these structures are damaged by the trauma of a punch, the central tendon subluxes or dislocates, leaving the underlying joint unprotected (Fig. 56.2).

Signs and Symptoms On physical examination, the patient with boxer ’s knuckle will exhibit swelling over the affected joint with a decreased range of motion. The examiner may detect lag of extension of the affected digit in contrast to the adjacent untraumatized fingers. The pain associated with boxer ’s knuckle can be reproduced by applying pressure to the affected knuckle and by active flexion and extension. Patients with boxer ’s knuckle often demonstrate ulnar deviation of the central tendon (see Fig. 56.1). With acute trauma to the dorsum of the hand, ecchymosis over the affected joint or joints may be present.

Testing Plain radiographs are indicated in all patients with boxer ’s knuckle to rule out fractures and identify subchondral cysts, which are often associated with osteochondral fracture (Fig. 56.3). Based on the patient’s clinical presentation, additional testing may be warranted to rule out inflammatory arthritis, including a complete blood count, erythrocyte sedimentation rate, uric acid level, and antinuclear antibody testing. Magnetic resonance imaging (MRI) and ultrasound imaging of the fingers and wrist are indicated to confirm the diagnosis and if joint instability, occult mass, occult fracture, infection, or tumor is suspected. Radionuclide bone scanning may be useful to identify stress fractures.

Differential Diagnosis The tentative diagnosis of boxer ’s knuckle is made on clinical grounds and confirmed by radiographic testing. Arthritis, tenosynovitis, or gout of the affected digits may accompany boxer ’s knuckle and exacerbate the patient’s pain. Occult fractures occasionally confuse the clinical presentation.

Treatment Initial treatment of the pain and functional disability associated with boxer ’s knuckle consists of nonsteroidal antiinflammatory drugs (NSAIDs), simple analgesics, or cyclooxygenase-2 (COX-2) inhibitors. Physical modalities, including local heat and gentle range-of-motion exercises, should be introduced to avoid loss of function. Vigorous exercises should be avoided, because they will exacerbate the patient’s symptoms. A nighttime splint to protect the fingers may be helpful. If sleep disturbance is present, low-dose tricyclic antidepressants are indicated. Ultimately, surgical repair is required to alleviate the patient’s pain and functional disability.

FIG. 56.1 Subluxation of the central tendon and rupture of the extensor hood mechanism create the classic deformity associated with boxer’s knuckle.

FIG. 56.2 Normal anatomy of the metacarpophalangeal joint extensor hood mechanism and the pathoanatomy of boxer’s knuckle. Despite variations in extent and exact location, the characteristic lesion consistently comprises rupture of the sagittal band with subluxation or overt dislocation of the central extensor tendon. From Melone CP Jr, Polatsch DB, Beldner S. Disabling hand injuries in boxing: boxer’s knuckle and traumatic carpal boss. Clin Sports Med. 2009;28:609–621.

FIG. 56.3 Radiograph demonstrating a subchondral cyst at the head of the index metacarpal highly suggestive of boxer’s knuckle associated with osteochondral fracture (arrow). From Polatsch DB, Beldner S. Disabling hand injuries in boxing: boxer’s knuckle and traumatic carpal boss. Clin Sports Med. 2009;28:609–621.

Complications and Pitfalls The clinician should always keep in mind that occult fracture or tumor may mimic the clinical symptoms of boxer ’s knuckle. Radiographic imaging is important to avoid misdiagnosis. Given the amount of trauma sustained with the sport of boxing, coexistent arthritis is usually present.

Clinical Pearls Pain emanating from the hand is a common problem. Boxer ’s knuckle must be distinguished from stress fracture, arthritis, and other occult pathological conditions of the wrist and hand. Although NSAIDs may palliate the pain of boxer ’s knuckle, patients often require surgical repair to obtain long-lasting relief and restore functionality. Coexistent arthritis, bursitis, and tendinitis may contribute to the patient’s pain, necessitating additional treatment with more localized injection of local anesthetic and steroid.

Suggested Readings Aronowitz A.R, Leddy J.P. Closed tendon injuries of the hand and wrist in athletes. Clin Sports Med. 1998;17:449–467. Kelly M. Role of the ringside physician and medical preparticipation evaluation of boxers. Clin Sports Med. 2009;28(4):515–519. Kelly M, Posner M.A, Ambrose L. Boxer ’s knuckle: dorsal capsular rupture of the metacarpophalangeal joint of a finger. J Hand Surg Am. 1989;14:229–236. Melone Jr. C.P, Polatsch D.B, Beldner S. Disabling hand injuries in boxing: boxer ’s knuckle and traumatic carpal boss. Clin Sports Med. 2009;28:609– 621. Waldman S.D. Painful Conditions of the Wrist And Hand. Physical Diagnosis of Pain: An Atlas of Signs and Symptoms. 2nd ed. Philadelphia: Saunders; 2010 156–154.

57

Triangular Fibrocartilage Tear Syndrome

Abstract Patients with triangular fibrocartilage tear syndrome usually give a history of trauma to the affected wrist, although older patients may report ulnar-side wrist pain in the absence of trauma, often attributing their symptoms to arthritis. Reports of increased pain when stirring coffee or other activities that require rotation of the distal radioulnar joint are common with triangular fibrocartilage tear syndrome. Some patients also may report a catching or clicking sensation with movement of the wrist and a feeling of weakness. Occasionally, patients note that the bones beneath their little finger have sunken in. This finding is due to the loss of support of the carpal bones on the ulnar side of the wrist resulting from disruption of the TFCC.

Keywords degenerative arthritis; hand deformity; magnetic resonance imaging; sports injury; triangular fibrocartilage tear; triangular fibrocartilage complex; ulnar sided wrist pain; ultrasound imaging; wrist pain

ICD-10 CODE M24.139

The Clinical Syndrome Triangular fibrocartilage tear syndrome, also known as triangular fibrocartilage complex (TFCC) lesion, is caused by trauma or degenerative changes to the wrist. The TFCC is a group or complex of ligament and cartilage structures that together serve four major functions related to the function of the human wrist, as follows: (1) The TFCC helps suspend the distal radius and ulnar carpus from the distal ulna; (2) the TFCC is the major ligamentous stabilizer of the distal radioulnar joint; (3) the TFCC provides a continuous gliding surface across the entire distal face of the radius and ulna to allow smooth flexion/extension and translational movements of the wrist; and (4) the TFCC acts as a shock absorber for forces transmitted over the ulnocarpal axis (Fig. 57.1 and Table 57.1). Degeneration of the TFCC begins to occur as part of the natural aging process in the third decade. This degenerative process predisposes the TFCC to traumatic injury. Common injuries that lead to TFCC tear syndrome are listed in Table 57.2. These injuries include falls onto a fully pronated and hyperextended wrist; waterskiing and horseback riding injuries, in which the patient is dragged by the wrist by a tangled ski rope or reins, causing critical distraction forces to be applied to the volar forearm and wrist; power drill injuries, in which the drill bit binds and the drill handle forcibly rotates the wrist rather than the drill bit; and distal radius fractures (Fig. 57.2). Fractures of the distal radius usually affect the radial side of the TFCC, and the clinical symptoms, as described subsequently, may be less clear-cut. Patients with triangular fibrocartilage tear syndrome usually give a history of trauma to the affected wrist, although older patients may report ulnar-side wrist pain in the absence of trauma, often attributing their symptoms to arthritis. Reports of increased pain when stirring coffee or other activities that require rotation of the distal radioulnar joint are common with triangular fibrocartilage tear syndrome. Some patients also may report a catching or clicking sensation with movement of the wrist and a feeling of weakness. Occasionally, patients note that the bones beneath their little finger have sunken in. This finding is due to the loss of support of the carpal bones on the ulnar side of the wrist resulting from disruption of the TFCC.

Signs and Symptoms Physical examination of patients with triangular fibrocartilage tear syndrome reveals pain on rotation of the wrist with a marked exacerbation of this pain with stress loading of the distal radioulnar joint with the wrist in pronation and supination. Hyperpronation may also reproduce the pain. A clicking sensation may be appreciated by the examiner on range of motion and depression or sag of the carpals on the ulnar side of the unsupported wrist. Instability of the distal radioulnar joint often can be shown by shucking or pressing one’s fingers between the distal radius and ulna. Similar instability may be shown between the lunotriquetral interval. A positive piano key sign is often present and can be elicited by pressing down on the ulnar styloid as if it were a piano key. If the ulnar styloid readily depresses, the test is considered positive (Fig. 57.3).

FIG. 57.1 Anatomy of the Triangular Fibrocartilage Complex.

Table 57.1 Function of the Triangular Fibrocartilage Complex Helps suspend distal radius and ulnar carpus from distal ulna Acts as major ligamentous stabilizer of distal radioulnar joint Provides continuous gliding surface across entire distal face of radius and ulna Allows for smooth flexion/extension and translational movements of wrist Acts as shock absorber for forces transmitted over ulnocarpal axis

Table 57.2 Common Causes of Triangular Fibrocartilage Tear Syndrome Falls onto fully pronated and hyperextended wrist Waterskiing and horseback riding dragging injuries causing critical distraction forces to be applied to volar forearm and wrist Power drill injuries in which the drill bit binds and the drill handle forcibly rotates the wrist rather than the drill bit Distal radius fractures Degenerative changes

FIG. 57.2 Common injuries that lead to triangular fibrocartilage tear syndrome include waterskiing and horseback riding injuries, in which the patient is dragged by the wrist by a tangled ski rope or reins, causing critical distraction forces to be applied to the volar forearm and wrist.

FIG. 57 3 Piano key sign is elicited by pressing down on the ulnar styloid as if it were a piano key. If the ulnar styloid readily depresses, the test is considered positive.

FIG. 57.4 Ulnar-Sided Triangular Fibrocartilage Complex (TFCC) Tears(A) Coronal two-dimensional T2 gradient echo magnetic resonance imaging. A large amount of fluid signal is seen replacing the ulnar attachment of the TFCC, extending beyond the ulnar capsule, along the extensor carpi ulnaris tendon sheath more proximally (long arrows). A tear of the scapholunate ligament also is present (short arrow). (B) Coronal T2 fast spin echo image with fat saturation reveals fluid signal and altered morphology indicating disruption of the ulnar attachment (arrow) of the TFCC. (C) Coronal short tau inversion recovery MR image. The ulnar aspect of the TFCC is torn and detached (white arrows). A fracture of the ulnar styloid tip can be seen (black arrow). From Edelman RR, Hesselink JR, Zlatkin MB, et al, eds. Clinical Magnetic Resonance Imaging. 3rd ed. Philadelphia: Saunders; 2006:3325.

Testing Plain radiographs are indicated in all patients who present with triangular fibrocartilage tear syndrome to rule out underlying occult bony pathological processes. Based on the patient’s clinical presentation, additional tests, including complete blood cell count, uric acid level, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. Magnetic resonance imaging (MRI) of the wrist is indicated in all patients suspected to have triangular fibrocartilage tear syndrome or if other causes of joint instability, infection, or tumor are suspected (Figs. 57.4 and 57.5). Magnetic resonance arthrography also will help confirm the diagnosis of fibrocartilage tear syndrome in questionable cases, as will arthroscopy of the wrist (Fig. 57.6). Ultrasound imaging will also help confirm the diagnosis and identify other causes of ulnar-sided pain (Fig. 57.7). Electromyography is indicated if coexistent ulnar or carpal tunnel syndrome is suspected. A very gentle injection of the radioulnar joint with small volumes of local anesthetic and steroid provides immediate improvement of the pain, but ultimately surgical repair is required.

FIG. 57.5 Radial-Sided Triangular Fibrocartilage Complex (TFCC) TearsCoronal two-dimensional T2∗ gradient echo magnetic resonance imaging (A) and T2 fast spin echo image with fat saturation (B) show fluid signal intensity in the radial aspect of the TFCC (arrow), which extends to the radiocarpal and the distal radioulnar joint articular surfaces. Fluid is seen in the distal radioulnar joint. (C) Coronal highresolution T1 fast spin echo image after intraarticular contrast injection into the radiocarpal joint in another patient illustrates high signal intensity contrast extending through a TFCC defect into the distal radioulnar joint (arrow). (From Edelman RR, Hesselink JR, Zlatkin MB, et al, eds. Clinical Magnetic Resonance Imaging. 3rd ed. Philadelphia: Saunders; 2006:3324.)

Differential Diagnosis Coexistent arthritis, gout of the radioulnar joint, carpometacarpal and interphalangeal joints, and tendinitis also may coexist with triangular fibrocartilage tear syndrome and exacerbate the patient’s pain and disability. Ulnocarpal abutment syndrome, Kienböck disease, and extensor carpi ulnaris tendinitis also may mimic the pain of triangular fibrocartilage tear syndrome.

FIG. 57.6 (A) Digital subtraction magnetic resonance arthrogram image demonstrating a leak of contrast agent from the radiocarpal joint into the distal radioulnar joint (DRUJ) (broken black arrow) resulting from a triangular fibrocartilage complex (TFCC) tear. (B) The post-injection radiograph also shows contrast agent within the DRUJ. In addition, contrast agent is seen in the midcarpal joint because of a leak through an asymptomatic central perforation of the scapholunate ligament. (C) The coronal gradient echo magnetic resonance arthrogram image shows the tear of the TFC (white arrow). The articular cartilage of the wrist is well demonstrated and normal. From Waldman SD, Campbell RSD, eds. Imaging of Pain. Philadelphia: Saunders; 2011:310.

FIG. 57.7 Ultrasound Image of the Triangular Fibrocartilage Complex (TFCC).

Treatment Initial treatment of the pain and functional disability associated with triangular fibrocartilage tear syndrome should include a combination of nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and short-term immobilization of the wrist. Local application of heat and cold also may be beneficial. For patients who do not respond to these treatment modalities, an injection of a local anesthetic and steroid into the radioulnar joint may be a reasonable next step. Vigorous exercises should be avoided because they would exacerbate the patient’s symptoms. Ultimately, surgical repair is the treatment of choice.

Complications and Pitfalls Failure to treat significant triangular fibrocartilage tear syndrome surgically usually results in continued pain and disability and in some patients leads to ongoing damage to the wrist. Injection of the radioulnar joint with local anesthetic and steroid is a safe technique if the clinician is attentive to detail, specifically using small amounts of local anesthetic and steroid and avoiding high injection pressures, which may disrupt the complex further. Another complication of this injection technique is infection. This complication should be exceedingly rare if strict aseptic technique is followed. Approximately 25% of patients report a transient increase in pain after this injection technique, and patients should be warned of this possibility.

Clinical Pearls Triangular fibrocartilage tear syndrome is a straightforward diagnosis in the presence of obvious antecedent trauma. The diagnosis is less obvious in the absence of trauma, however, unless the clinician includes it in the differential diagnosis with all patients with ulnar-sided wrist pain. Coexistent arthritis, tendinitis, and gout also may contribute to the pain and may require additional treatment with more localized injection of a local anesthetic and depot steroid. The use of physical modalities, including local heat and cold and immobilization of the wrist, may provide symptomatic relief. Vigorous exercises should be avoided because they would exacerbate the patient’s symptoms and may cause further damage to the wrist. Simple analgesics and NSAIDs may be used concurrently with this injection technique.

Suggested Readings Coggins C.A. Imaging of ulnar-sided wrist pain. Clin Sports Med. 2006;25:505– 546. Henderson C.J, Kobayashi K.M. Ulnar-sided wrist pain in the athlete. Orthop Clin North Am. 2016;47(4):789–798. Kovachevich R, Elhassan B.T. Arthroscopic and open repair of the TFCC. Hand Clin. 2010;26:485–494. Pirolo J.M, Yao J. Minimally invasive approaches to ulnar-sided wrist disorders. Hand Clin. 2014;30(1):77–89. Sachar K. Ulnar-sided wrist pain: evaluation and treatment of triangular fibrocartilage complex tears, ulnocarpal impaction syndrome, and lunotriquetral ligament tears. J Hand Surg Br. 2008;33:1669–1679. Tiedeken N.C, Baratz M.E. Ulnocarpal impingement and triangular fibrocartilage complex tears. Oper Tech Sports Med. 2016;24(2):131–138. Wolf M.R, Avery D, Wolf J.M. Upper extremity injuries in gymnasts. Hand Clin. 2017;33(1):187–197.

58

Scapholunate Ligament Tear Syndrome

Abstract Scapholunate ligament tear syndrome is caused by trauma or, rarely, degenerative changes to the wrist. The scapholunate ligament serves as a stabilizer of the scaphoid’s palmarward rotational force against the opposite dorsalward rotational force of the lunate. The ligament also maintains the spacing of the scapulolunate gap, keeping the proximal pole of the scaphoid in proper position relative to the lunate.

Keywords David Letterman sign; ligamentous injury; magnetic resonance imaging; scapholunate ligament tear; scapholunate ligament complex; scapholunate advanced collapse; sports injury; Terry Thomas sign; ultrasound imaging; wrist pain

ICD-10CODE M24.139

The Clinical Syndrome Scapholunate ligament tear syndrome is caused by trauma or, rarely, degenerative changes to the wrist. The scapholunate ligament serves as a stabilizer of the scaphoid’s palmarward rotational force against the opposite dorsalward rotational force of the lunate. The ligament also maintains the spacing of the scapulolunate gap, keeping the proximal pole of the scaphoid in proper position relative to the lunate (Fig. 58.1). Degeneration of the scapholunate ligament complex begins to occur as part of the natural aging process in the third decade. This degenerative process predisposes the scapholunate ligament complex to traumatic injury. Common injuries that lead to scapholunate ligament tear include falls onto a hyperextended wrist (Fig. 58.2). If the tear is partial, the patient reports dorsoradial wrist pain. If the tear is complete, instability of the wrist accompanies the pain. Some patients report an audible click with any ulnar to radial deviation of the wrist.

FIG. 58.1 Anatomy of the Scapholunate Ligament Complex.

FIG. 58.2 Common Injuries That Lead to Scapholunate Ligament Tear Include Falls Onto a Hyperextended Wrist.

Signs and Symptoms Physical examination of patients with scapholunate ligament tear reveals pain on ulnar deviation of the wrist with the pain worsened by having the patient tightly clench the fist, which places stress on the carpal bones. Pain is present on palpation of the anatomical snuffbox, and a widening of the scapholunate gap may be appreciated. A clicking sensation may be appreciated by the examiner on range of motion. A positive Watson’s test also is present when the wrist is moved from the ulnar to radial position while the patient tightly clutches the fist (Fig. 58.3). If left untreated, degeneration of the radioscaphoid, midcarpal, and radiolunate joints results in a deformity termed scapholunate advanced collapse, which is also referred to as a scaphoid lunate advanced collapse (SLAC) wrist.

Testing Plain radiographs are indicated in all patients who present with scapholunate ligament tear syndrome to rule out underlying occult bony pathological conditions and identify widening of the scapholunate gap (also known as a positive Terry Thomas or David Letterman sign after the space between the teeth of these celebrities), palmar flexion of the scaphoid, and dorsiflexion of the lunate, which is termed scapholunate dissociation with dorsal intercalary segment instability (Fig. 58.4). Based on the patient’s clinical presentation, additional tests, including complete blood cell count, uric acid level, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. Magnetic resonance imaging (MRI) and ultrasound imaging of the wrist is indicated in all patients thought to have scapholunate ligament tear or if other causes of joint instability, infection, or tumor are suspected (Figs. 58.5 and 58.6). Scaphoid dorsal subluxation identified on these imaging modalities will strengthen the diagnosis of scapholunate ligament tear. Electromyography is indicated if coexistent ulnar or carpal tunnel syndrome is suspected. A very gentle injection of the radioulnar joint with small volumes of local anesthetic and steroid provides immediate improvement of the pain, but ultimately surgical repair is required.

FIG. 58.3 Watson’s test (scaphoid displacement test) for the diagnosis of scapholunate dissociation is performed by pushing upward on the scaphoid tuberosity while the hand is in ulnar deviation. This action tends to cause the scaphoid to ride out of the radial fossa over the dorsal rim, at times producing a painful snap. The test might be positive in loose-jointed individuals and should always be compared with the contralateral side.

From Manuel J, Moran SL. The diagnosis and treatment of scapholunate instability. Hand Clin. 2010;26:129–144.

Differential Diagnosis Coexistent arthritis and gout of the radioulnar, carpal, metacarpal, and interphalangeal joints; dorsal wrist ganglion; de Quervain stenosing tenosynovitis; and tendinitis may coexist with scapholunate ligament tear syndrome and exacerbate the patient’s pain and disability. Kienböck disease, avascular necrosis of the scaphoid, and scaphoid fractures also may mimic the pain of scapholunate ligament tear.

Treatment Initial treatment of the pain and functional disability associated with scapholunate ligament tear syndrome should include a combination of nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and short-term immobilization of the wrist. Local application of heat and cold also may be beneficial. For patients who do not respond to these treatment modalities, an injection of a local anesthetic and steroid into the scapholunate joint may be a reasonable next step. Ultrasound guidance may improve the accuracy of needle placement and decrease the incidence of needle-related complications. Vigorous exercises should be avoided because they would exacerbate the patient’s symptoms. Ultimately, surgical repair is the treatment of choice.

FIG. 58.4 Posteroanterior radiograph of the wrist depicting severe scapholunate dissociation with increased scapholunate gap, also referred to as a positive Terry Thomas sign. The scaphoid is foreshortened with respect to the longitudinal axis of

the forearm. The scaphoid tuberosity is seen in profile providing the ring sign. The lunate appears to be trapezoidal in shape because the palmar pole is rotated under the capitate. From Manuel J, Moran SL. The diagnosis and treatment of scapholunate instability. Hand Clin. 2010;26:129–144.

Complications and Pitfalls Failure to treat significant scapholunate ligament tear surgically usually results in continued pain and disability and, in some patients, leads to ongoing damage to the wrist. Injection of the scapholunate joint with local anesthetic and steroid is a safe technique if the clinician is attentive to detail, specifically using small amounts of local anesthetic and steroid and avoiding high injection pressures, which may disrupt the ligament further. Another complication of this injection technique is infection. This complication should be exceedingly rare if strict aseptic technique is followed. Approximately 25% of patients report a transient increase in pain after this injection technique, and patients should be warned of this possibility.

Clinical Pearls Scapholunate ligament tear and other disorders of the scaphoid are a straightforward diagnosis in the presence of obvious antecedent trauma. The diagnosis is less obvious in the absence of trauma, however, unless the clinician includes it in the differential diagnosis with all patients reporting radial-sided wrist pain. Coexistent arthritis, tendinitis, and gout also may contribute to the pain and may require additional treatment with more localized injection of a local anesthetic and depot steroid. The use of physical modalities, including local heat and cold and immobilization of the wrist, may provide symptomatic relief. Vigorous exercises should be avoided because they would exacerbate the patient’s symptoms and may cause further damage to the wrist. Simple analgesics and NSAIDs may be used concurrently with this injection technique.

FIG. 58.5 Intercarpal ligaments: three-dimensional Fourier transform gradient recalled magnetic resonance imaging. Normal and abnormal scapholunate interosseous ligament. (A) Normal scapholunate interosseous ligament. Coronal three-dimensional Fourier transform (TR/TE, 60/11; flip angle, 10 degrees) MRI shows the low signal intensity and linear morphology that characterize normal scapholunate (arrow) and lunotriquetral (arrowhead) interosseous ligaments. The triangular fibrocartilage also is normal. (B) Communicating defect of the scapholunate interosseous ligament. Coronal oblique three-dimensional Fourier transform (TR/TE, 60/10; flip angle, 30 degrees) MRI shows altered morphology (arrow) of the scapholunate interosseous ligament. From Resnick D, ed. Diagnosis of Bone and Joint Disorders. 4th ed. Philadelphia: Saunders; 2002:3039.

FIG. 58.6 A 42-year-old man with dorsal SLL tear of the left wrist confirmed on arthrogram (not shown). (Arrows reveal tear of the ligament.) From Harish S, O’Neill J, Finlay K, et al. Ultrasound of wrist pain. Curr Probl Diagn Radiol. 2009:111–125.

Suggested Readings Gras M, Mathoulin C. Dorsal capsule tears of the wrist. Chirurgie de la Main. 2015;34(6):363–364. Goldberg S.H, Riansuwan K, Rosenwasser M.P. Arthroscopic treatment of scapholunate ligament tears. In: Slutsky D.J, Osterman A.L, eds. Fractures and Injuries of the Distal Radius and Carpus. Philadelphia: Elsevier; 2009:463– 474. Hafezi-Nejad N, Carrino J.A, Eng J, et al. Scapholunate interosseous ligament tears: diagnostic performance of 1.5T, 3T MRI, and MR arthrography—a systematic review and meta-analysis. Acad Radiol. 2016;23(9):1091–1103. Manuel J, Moran S.L. The diagnosis and treatment of scapholunate instability. Orthop Clin North Am. 2007;38:261–277. Manuel J, Moran S.L. The diagnosis and treatment of scapholunate instability. Hand Clin. 2010;26:129–144. Meister D.W, Hearns K.A, Carlson M.G. Dorsal scaphoid subluxation on sagittal magnetic resonance imaging as a marker for scapholunate ligament tear. J Hand Surg. 2017;42(9):717–721. Messina J.C. Identification and treatment of scapho-lunate injuries. Chirurgie de la Main. 2015;34(6):363. O’Meeghan C.J, Stuart W, Mamo V, Stanley J.K, Trail I.A. The natural history of an untreated isolated scapholunate interosseus ligament injury. J Hand Surg Br. 2003;28:307–310.

59

Lunotriquetral Instability Pain Syndrome

Abstract Lunotriquetral instability pain syndrome is caused by trauma or, rarely, degenerative changes to the wrist. The lunotriquetral ligament stabilizes the wrist and helps maintain the proper spacing of the lunotriquetral gap. Physical examination of patients with lunotriquetral instability pain syndrome reveals pain on ulnar or radial deviation of the wrist with the pain worsened by having the patient tightly clench the fist, which places stress on the carpal bones. Pain is felt on palpation of the lunate and triquetrum, and a widening of the lunotriquetral gap may be appreciated. A clicking sensation may be appreciated by the examiner on range of motion. A positive lunotriquetral shear test is often present. This test is performed by displacing the triquetrum dorsally, while displacing the lunate palmarly. The test is considered positive if the examiner demonstrates increased excursion of the lunotriquetral joint over the normal side.

Keywords diagnostic ultrasound; lunotriquetral instability pain syndrome; lunotriquetral gap; lunotriquetral ligament complex; lunotriquetral ligament; lunotriquetral shear test; magnetic resonance imaging; sports injuries

ICD-10 CODE M24.139

The Clinical Syndrome Lunotriquetral instability pain syndrome is caused by trauma or, rarely, degenerative changes to the wrist. The lunotriquetral ligament stabilizes the wrist and helps maintain the proper spacing of the lunotriquetral gap (Fig. 59.1). Degeneration of the lunotriquetral ligament complex begins to occur as part of the natural aging process in the third decade. This degenerative process predisposes the lunotriquetral ligament complex to traumatic injury. Common injuries that lead to lunotriquetral instability pain syndrome include backward falls onto a hyperextended wrist (Fig. 59.2). If the tear is partial, the patient reports dorsoulnar wrist pain. If the tear is complete, instability of the wrist accompanies the pain. Some patients report an audible click with any ulnar deviation of the wrist.

FIG. 59.1 Anatomy of the Lunotriquetral Ligament Complex.

Signs and Symptoms Physical examination of patients with lunotriquetral instability pain syndrome reveals pain on ulnar or radial deviation of the wrist with the pain worsened by having the patient tightly clench the fist, which places stress on the carpal bones. Pain is felt on palpation of the lunate and triquetrum, and a widening of the lunotriquetral gap may be appreciated. A clicking sensation may be appreciated by the examiner on range of motion. A positive lunotriquetral shear test is often present. This test is performed by displacing the triquetrum dorsally while displacing the lunate palmarly. The test is considered positive if the examiner demonstrates increased excursion of the lunotriquetral joint over the normal side. With complete lunotriquetral disruption, static collapse can occur causing a classic dinner fork deformity (Fig. 59.3).

FIG. 59.2 Common Injuries That Lead to Lunotriquetral Instability Pain Syndrome Include Backward Falls Onto a Hyperextended Wrist.

FIG. 59.3 Dinner Fork DeformityPreoperative clinical image of a 25-year-old male worker presenting 6 weeks after a sustained fall with a prominent dinner fork deformity and prominent distal ulna. From Nicoson MC, Moran SL. Diagnosis and treatment of acute lunotriquetral ligament injuries. Hand Clin. 2015;31[3]:467–476.

Testing Plain radiographs are indicated in all patients who present with lunotriquetral instability pain syndrome to rule out underlying occult bony pathological processes and identify widening of the lunotriquetral gap. Based on the patient’s clinical presentation, additional tests, including complete blood cell count, uric acid level, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. Magnetic resonance imaging (MRI) and ultrasound imaging of the wrist is indicated in all patients suspected to have lunotriquetral instability pain syndrome or if other causes of joint instability, infection, or tumor are suspected (Figs. 59.4 and 59.5). Electromyography is indicated if coexistent ulnar or carpal tunnel is suspected. A gentle injection of the lunotriquetral joint with small volumes of local anesthetic and steroid provides immediate improvement of the pain, but ultimately surgical repair is required.

Differential Diagnosis Coexistent arthritis and gout of the radioulnar, carpometacarpal, and interphalangeal joints; dorsal wrist ganglion; and tendinitis may coexist with lunotriquetral instability pain syndrome and exacerbate the patient’s pain and disability. Kienböck disease and lunate fractures also may mimic the pain of lunotriquetral instability pain syndrome, as can tear of the triangular fibrocartilage complex and ulnar impaction syndrome.

Treatment Initial treatment of the pain and functional disability associated with lunotriquetral instability pain syndrome should include a combination of nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and short-term immobilization of the wrist. Local application of heat and cold also may be beneficial. For patients who do not respond to these treatment modalities, an injection of a local anesthetic and steroid into the lunotriquetral joint may be a reasonable next step. Ultrasound guidance may improve the accuracy of needle placement and decrease the incidence of needle-related complications. Vigorous exercises should be avoided because they would exacerbate the patient’s symptoms. Ultimately, surgical repair is the treatment of choice.

Complications and Pitfalls Failure to treat significant lunotriquetral instability pain syndrome surgically usually results in continued pain and disability and in some patients leads to ongoing damage to the wrist. Injection of the joint with local anesthetic and steroid is a safe technique if the clinician is attentive to detail, specifically using small amounts of local anesthetic and steroid and avoiding high injection pressures, which may disrupt the ligament further. Another complication of this injection technique is infection. This complication should be exceedingly rare if strict aseptic technique is followed. Approximately 25% of patients report a transient increase in pain after this injection technique, and patients should be warned of this possibility.

Clinical Pearls Lunotriquetral instability pain syndrome and other disorders of the lunate and triquetrum are a straightforward diagnosis in the presence of obvious antecedent trauma. The diagnosis is less obvious in the absence of trauma, however, unless the clinician included it in the differential diagnosis with all patients with ulnar-sided wrist pain. Coexistent arthritis, tendinitis, and gout also may contribute to the pain and may require additional treatment with more localized injection of a local anesthetic and depot steroid. The use of physical modalities, including local heat and cold and immobilization of the wrist, may provide symptomatic relief. Vigorous exercises should be avoided because they would exacerbate the patient’s symptoms and may cause further damage to the wrist. Simple analgesics and NSAIDs may be used concurrently with this injection technique.

FIG. 59.4 (A) Lateral radiograph demonstrating the lunate (white arrows) with its axis (white line) tilted in a volar direction compared with the axis of capitate and radius (broken white line); this tilting is consistent with a volar intercalated segment instability deformity. (B) A digital subtraction arthrogram of the same patient after injection of contrast agent into the radiocarpal joint demonstrates contrast agent passing through the lunotriquetral (LT) joint (black arrow) and into the metacarpal joint (broken black arrow), indicating a tear of the LT ligament. The coronal T1weighted (C) and T2-weighted with fat suppression (D) magnetic resonance arthrogram images show sclerosis, subchondral cyst formation, and marrow edema within the lunate resulting from secondary osteoarthritis change in the LT joint. (E) The gradient echo magnetic resonance image best demonstrates absence of the LT ligament (white arrow) and loss of articular cartilage. Compare with the normal appearance of the scapholunate ligament (broken white arrow). From Waldman SD, Campbell RSD, eds. Lunotriquetral instability pain syndrome. In: Imaging of Pain. Philadelphia: Saunders, 2011:307–308.

FIG. 59.5 Lunotriquetral LigamentThis ligament extends between the lunate and the triquetrum and has both volar and a dorsal band connected at their proximal pole, thus separating the radiocarpal and midcarpal joint spaces. (A) Schematic drawing of the volar band of the lunotriquetral ligament: the ligament is shown in yellow. (B) US scan of the volar band of the lunotriquetral ligament (arrowheads). (C) Probe positioning on the volar wrist, with the wrist in supination and slight extension. (D) Schematic drawing of the dorsal band of the lunotriquetral ligament: the ligament is shown in yellow. (E) US scan of the dorsal band of the lunotriquetral ligament (arrowheads). (F) Probe position on the dorsal wrist, with the wrist in pronation and slight flexion. L, Lunate; T, triquetrum. From Gitto S, Messina C, Mauri G, et al. Dynamic high-resolution ultrasound of intrinsic and extrinsic ligaments of the wrist: how to make it simple. Eur J Radiol. 2017;87:20–35.

Suggested Readings Andersson J.K, Andernord D, Karlsson J, Fridén J. Efficacy of magnetic resonance imaging and clinical tests in diagnostics of wrist ligament injuries: a systematic review, arthroscopy. J Arthroscopic Related Surg. 2015;31(10):2014–2020. Gitto S, Messina C, Mauri G, Aliprandi A, Sardanelli F, Sconfienza L.M. Dynamic high-resolution ultrasound of intrinsic and extrinsic ligaments of the wrist: how to make it simple. Euro J Radiol. 2017;87:20–35. Goldberg S.H, Strauch R.E, Rosenwasser M.P. Scapholunate and lunotriquetral instability in the athlete: diagnosis and management. Oper Tech Sports Med. 2006;14:108–121. Lee D.J, Elfar J.C. Carpal ligament injuries, pathomechanics, and classification. Hand Clin. 2015;31(3):389–398. Lindau T. Arthroscopic evaluation of associated soft tissue injuries in distal radius fractures. Hand Clin. 2017;33(4):651–658. Nicoson M.C, Moran S.L. Diagnosis and treatment of acute lunotriquetral ligament injuries. Hand Clin. 2015;31(3):467–476. Pulos N, Bozentka D.J. Carpal ligament anatomy and biomechanics. Hand Clin. 2015;31(3):381–387. Sachar K. Ulnar-sided wrist pain: evaluation and treatment of triangular fibrocartilage complex tears, ulnocarpal impaction syndrome, and lunotriquetral ligament tears. J Hand Surg Am. 2008;33:1669–1679. Lunotriquetral instability pain syndrome. In: Waldman S.D, Campbell R.S.D, eds. Imaging of Pain. Philadelphia: Saunders; 2011:307–308.

60

Kienböck Disease

Abstract Kienböck disease, or lunatomalacia, is caused by avascular necrosis of the lunate after repeated microfractures or major fractures to the lunate following trauma to the wrist. Repetitive microtrauma to the wrist from repetitive compressive loading and unloading such as use of a jackhammer and recurrent compression of the lunate by the capitate and distal radius resulting from extreme wrist positions also has been implicated in the evolution of this painful condition of the wrist and forearm. A patient with Kienböck disease reports unilateral dorsal wrist pain over the lunate that radiates into the forearm and decreasing range of motion of the wrist. Weakened grip strength also may be noticed. Kienböck disease usually affects one wrist; incidence of bilateral disease is extremely low. The disease is most common in the second through fourth decades of life.

Keywords avascular necrosis; Kienböck disease; lunatomalacia; magnetic resonance imaging of the wrist; microfractures; ulnar variance; ultrasound guided injection; ultrasound imaging; wrist instability; wrist pain

ICD-10 CODE M92.30

The Clinical Syndrome Kienböck disease, or lunatomalacia, is caused by avascular necrosis of the lunate after repeated microfractures or major fractures to the lunate following trauma to the wrist. Repetitive microtrauma to the wrist from repetitive compressive loading and unloading such as use of a jackhammer and recurrent compression of the lunate by the capitate and distal radius resulting from extreme wrist positions also has been implicated in the evolution of this painful condition of the wrist and forearm (Fig. 60.1). A patient with Kienböck disease reports unilateral dorsal wrist pain over the lunate that radiates into the forearm and decreasing range of motion of the wrist. Weakened grip strength also may be noticed. Kienböck disease usually affects one wrist; incidence of bilateral disease is extremely low. The disease is most common in the second through fourth decades of life with the wrist of the dominant hand affected in the majority of cases.

Signs and Symptoms Physical examination of patients with Kienböck disease reveals pain on ulnar or radial deviation of the wrist, with the pain worsened by passively dorsiflexing the middle phalanx on the affected side. Pain is felt on palpation of the lunate, and a click or crepitus may be appreciated by the examiner when putting the wrist through range of motion.

Testing Plain radiographs are indicated in all patients who present with Kienböck disease to rule out underlying occult bony pathological conditions and identify sclerosis and fragmentation of the lunate. Based on the patient’s clinical presentation, additional tests, including complete blood cell count, uric acid level, erythrocyte sedimentation rate, and antinuclear antibody testing, may also be indicated. Magnetic resonance imaging (MRI) of the wrist and ultrasound imaging is indicated in all patients suspected to have Kienböck disease or if other causes of joint instability, infection, or tumor are suspected (Fig. 60.2). Computed tomography may help ascertain the condition of the articular cartilage. Electromyography is indicated if coexistent ulnar or carpal tunnel syndrome is suspected. A very gentle injection of the lunotriquetral joint with small volumes of local anesthetic and steroid provides immediate improvement of the pain, but ultimately surgical repair is required. Low-intensity pulsed ultrasound may help increase vascular growth factor, which is believed to increase blood flow to the affected bones of the wrist.

FIG. 60.1 Repetitive microtrauma to the wrist from repetitive compressive loading and unloading and recurrent compression of the lunate by the capitate and distal radius owing to extreme wrist positions have been implicated in the evolution of this painful condition of the wrist and forearm.

FIG. 60.2 Kienböck Disease and Nonunion of a Scaphoid Fracture: Magnetic Resonance Imaging (MRI).(A) Conventional tomography shows cystic changes and sclerosis in the lunate bone and an ununited fracture of the midportion of the scaphoid bone. The fracture lines are smooth with sclerotic margins. Mild negative ulnar variance is seen. (B) Coronal T1-weighted (TR/TE, 800/20) spin echo MRI reveals low signal intensity throughout the lunate bone and in the fracture gap of the scaphoid bone. (C) Coronal T2-weighted (TR/TE, 2500/60) spin echo MRI shows foci of high signal intensity (arrowhead) in the lunate bone. Fluid of high signal intensity (arrow) is evident in a portion of the fracture gap in the scaphoid bone. From Resnick D, ed. Diagnosis of Bone and Joint Disorders. 4th ed. Philadelphia: Saunders; 2002:3044.

Differential Diagnosis Coexistent arthritis and gout of the radioulnar, carpometacarpal, and interphalangeal joints; dorsal wrist ganglion; and tendinitis may coexist with Kienböck disease and exacerbate the pain and disability of the patient. Lunate cysts, contusions, and fractures also may mimic the pain of Kienböck disease, as can tear of the triangular fibrocartilage complex and ulnar impaction syndrome (Fig. 60.3).

Treatment Initial treatment of the pain and functional disability associated with Kienböck disease should include a combination of nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and short-term immobilization of the wrist. Local application of heat and cold also may be beneficial. For patients who do not respond to these treatment modalities, an injection of a local anesthetic and steroid into the lunotriquetral joint may be a reasonable next step to provide palliation of acute pain. Ultrasound guidance may improve the accuracy of needle placement and decrease the incidence of needle-related complications. Vigorous exercises should be avoided because they would exacerbate the patient’s symptoms. Ultimately, surgical repair is the treatment of choice.

Complications and Pitfalls Failure to treat significant Kienböck disease surgically usually results in continued pain and disability and in some patients leads to ongoing damage to the wrist. Injection of the joint with local anesthetic and steroid is a safe technique if the clinician is attentive to detail, specifically using small amounts of local anesthetic and steroid and avoiding high injection pressures, which may damage the joint further. Another complication of this injection technique is infection. This complication should be exceedingly rare if strict aseptic technique is followed. Approximately 25% of patients report a transient increase in pain after this injection technique, and patients should be warned of this possibility.

FIG. 60.3 Kienböck Disease MimicsPosteroanterior x-ray (A) and (B) T1-weighted coronal magnetic resonance imaging of a patient with stage 1 Kienböck disease (B). From Beredjiklian PK. Kienböck’s disease. J Hand Surg Am. 2009;34:167–175.

Clinical Pearls Kienböck disease and other disorders of the lunate are a relatively straightforward diagnosis in the presence of obvious antecedent trauma. The diagnosis is less obvious in the absence of trauma, however, unless the clinician included it in the differential diagnosis with all patients with dorsoulnar wrist pain that radiated into the forearm. Coexistent arthritis, tendinitis, and gout also may contribute to the pain and may require additional treatment with more localized injection of a local anesthetic and depot steroid. The use of physical modalities, including local heat and cold and immobilization of the wrist, may provide symptomatic relief. Vigorous exercises should be avoided because they would exacerbate the patient’s symptoms and may cause further damage to the wrist. Simple analgesics and NSAIDs may be used concurrently with this injection technique.

Suggested Readings Beredjiklian P.K. Kienböck’s disease. J Hand Surg Am. 2009;34:167–175. Fontaine C. Kienböck’s disease. Chir Main. 2015;34(1):4–17. Heire P, Temperley D, Murali R. Radiological imaging of the wrist joint. Orthop Trauma. 2017;31(4):248–256. Innes L, Strauch R.J. Systematic review of the treatment of Kienböck’s disease in its early and late stages. J Hand Surg. 2010;35(5) 713–717.e1–4. Lichtman D.M, Pientka W.F, Bain G.I. Kienböck disease: moving forward. J Hand Surg. 2016;41(5):630–638. Lutsky K, Beredjiklian P.K. Kienböck disease. J Hand Surg. 2012;37(9):1942– 1952. Taniguchi Y, Yoshida M, Iwasaki H, et al. Kienböck’s disease in elderly patients. J Hand Surg Am. 2003;28:779–783. Wagner J.P, Chung K.C. A historical report on Robert Kienböck (1871–1953) and Kienböck’s disease. J Hand Surg Am. 2005;30:1117–1121. Yazaki N, Nakamura R, Nakao E, et al. Bilateral Kienböck’s disease. J Hand Surg Br. 2005;30:133–136.

61

Avascular Necrosis of the Scaphoid

Abstract Avascular necrosis of the scaphoid is a common sequela to scaphoid fracture. Second only to the hip in the incidence of avascular necrosis, the scaphoid is extremely susceptible to this disease because of the tenuous blood supply of the scaphoid, which enters the bone through its distal half. The dorsal blood supply and the volar blood supply are easily disrupted by fracture of the scaphoid, often leaving the proximal portion of the bone without nutrition, leading to osteonecrosis.

Keywords anatomic snuffbox; avascular necrosis; avascular necrosis of the scaphoid; carpal fractures; scaphoid; scaphoid fractures; sports injury

ICD-10 CODE M84.1

The Clinical Syndrome Avascular necrosis of the scaphoid is a common sequela to scaphoid fracture. Second only to the hip in the incidence of avascular necrosis, the scaphoid is extremely susceptible to this disease because of the tenuous blood supply of the scaphoid, which enters the bone through its distal half. The dorsal blood supply and the volar blood supply are easily disrupted by fracture of the scaphoid, often leaving the proximal portion of the bone without nutrition, leading to osteonecrosis. Common causes of scaphoid fracture include trauma to the scaphoid from falls on a hyperextended wrist and from steering wheel injuries during motor vehicle accidents, although an idiopathic form of the disease, known as Preiser disease, can occur (Fig. 61.1). A patient with avascular necrosis of the scaphoid reports unilateral wrist pain over the anatomical snuffbox that may radiate into the radial aspect of the forearm and decreasing range of motion of the wrist. Weakened grip strength also may be noticed. Movement of the thumb usually exacerbates the patient’s pain.

FIG. 61.1 Common causes of scaphoid fractures include trauma to the scaphoid from falls on a hyperextended wrist and from steering wheel injuries during motor vehicle accidents.

Signs and Symptoms Physical examination of patients reports avascular necrosis of the scaphoid reveals pain on palpation of the anatomical snuffbox (Fig. 61.2). The pain can be worsened by passively moving the wrist from ulnar to radial position or by moving the thumb of the affected side. A click or crepitus also may be appreciated by the examiner when putting the wrist through range of motion. Weakness of dorsiflexion is common, as is weakness of grip strength in contrast to the nonaffected side.

FIG. 61.2 Physical examination of patients with avascular necrosis of the scaphoid reveals pain on palpation of the anatomical snuffbox.

FIG. 61.3 (A) Radiograph obtained 12 weeks after a scaphoid fracture. There is an apparent cyst in the scaphoid but no fracture line. (B) The computed tomography scan, however, confirms fracture nonunion. From Waldman SD, Campbell RSD, eds. Imaging of Pain. Philadelphia: Saunders; 2011:313–315.

FIG. 61.4 Osteonecrosis of the Scaphoid Bone After a Fracture(A) Four months after a scaphoid fracture, coronal T1-weighted (TR/TE, 500/14) spin echo magnetic resonance imaging (MRI) reveals nonunion of the bone and low signal intensity at the fracture line and in the proximal pole of the scaphoid. (B) After intravenous gadolinium administration, fat-suppressed coronal T1-weighted (TR/TE, 550/14) spin echo MRI image shows enhancement in both portions of the scaphoid, a good prognostic sign. From Resnick D, ed. Diagnosis of Bone and Joint Disorders. 4th ed. Philadelphia: Saunders; 2002:3045.

Testing Plain radiographs are indicated in all patients who present with avascular necrosis of the scaphoid to rule out underlying occult bony pathological conditions and identify sclerosis and fragmentation of the scaphoid, although early in the course of the disease plain radiographs can be notoriously unreliable (Fig. 61.3). Based on the patient’s clinical presentation, additional tests, including complete blood cell count, uric acid level, erythrocyte sedimentation rate, and antinuclear antibody testing, also may be indicated. Computed tomography (CT) and magnetic resonance imaging (MRI) of the wrist are indicated in all patients thought to have avascular necrosis of the scaphoid or if other causes of joint instability, infection, or tumor are suspected. Administration of gadolinium followed by postcontrast imaging may help delineate the adequacy of blood supply, with contrast enhancement of the proximal scaphoid being a good prognostic sign (Fig. 61.4). Ultrasound imaging of the scaphoid also may aid in the diagnosis (Fig. 61.5). Electromyography is indicated if coexistent ulnar or carpal tunnel syndrome is suspected. A very gentle injection of the radial aspect of the distal radioulnar joint with small volumes of local anesthetic and steroid provides immediate improvement of the pain, but ultimately surgical repair is required.

FIG. 61.5 A split-screen side-by-side comparison of the fractured scaphoid (right) and normal scaphoid (left). The arrows identify two cortical fractures in the palmar cortex. From Senall JA, Failla JM, Bouffard A, et al. Ultrasound for the early diagnosis of clinically suspected scaphoid fracture. J Hand Surg Am. 2004;29:400–405.

Differential Diagnosis Coexistent arthritis and gout of the radioulnar, carpometacarpal, and interphalangeal joints; dorsal wrist ganglion; and tendinitis may coexist with avascular necrosis of the scaphoid and exacerbate the patient’s pain and disability. Distal fractures of the radius, de Quervain stenosis, tenosynovitis, scapholunate ligament tears, scaphoid cysts, contusions, and fractures also may mimic the pain of avascular necrosis of the scaphoid, as can tear of the triangular fibrocartilage complex.

Treatment Initial treatment of the pain and functional disability associated with avascular necrosis of the scaphoid should include a combination of nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and short-term immobilization of the wrist. Local application of heat and cold also may be beneficial. For patients who do not respond to these treatment modalities, an injection of a local anesthetic and steroid into the radial aspect of the distal radioulnar joint may be a reasonable next step to provide palliation of acute pain. Vigorous exercises should be avoided because they would exacerbate the patient’s symptoms. Ultimately, surgical repair is the treatment of choice.

Complications and Pitfalls Failure to treat significant avascular necrosis of the scaphoid surgically usually results in continued pain and disability and in some patients leads to ongoing damage to the wrist. Injection of the joint with local anesthetic and steroid is a safe technique if the clinician is attentive to detail, specifically using small amounts of local anesthetic and steroid and avoiding high injection pressures, which may damage the joint further. Another complication of this injection technique is infection. This complication should be exceedingly rare if strict aseptic technique is followed. Approximately 25% of patients report a transient increase in pain after this injection technique, and patients should be warned of this possibility.

Clinical Pearls Avascular necrosis of the scaphoid is a diagnosis that is often missed, leading to much unnecessary pain and disability. The clinician should include avascular necrosis of the scaphoid in the differential diagnosis in all patients with radial-sided wrist pain after trauma to the wrist. Coexistent arthritis, tendinitis, and gout also may contribute to the pain and may require additional treatment with more localized injection of a local anesthetic and depot steroid. The use of physical modalities, including local heat and cold and immobilization of the wrist, may provide symptomatic relief. Vigorous exercises should be avoided because they would exacerbate the patient’s symptoms and may cause further damage to the wrist. Simple analgesics and NSAIDs may be used concurrently with this injection technique.

Suggested Readings Adey L, Souer J.S, Lozano-Calderon S. Computed tomography of suspected scaphoid fractures. J Hand Surg Am. 2007;32:61–66. Kawamura K, Chung K.C. Treatment of scaphoid fractures and nonunions. J Hand Surg Am. 2008;33:988–997. Senall J.A, Failla J.M, Bouffard A, Holsbeeck M. Ultrasound for the early diagnosis of clinically suspected scaphoid fracture. J Hand Surg Am. 2004;29:400–405.

62

Extensor Carpi Ulnaris Tendinitis

Abstract The onset of extensor carpi ulnaris tendinitis is usually acute, occurring after overuse or misuse of the wrist joint. Inciting factors include playing tennis, playing golf, and prolonged use of a heavy hammer. Injuries ranging from partial to complete tears of the tendon can occur when the distal tendon sustains direct trauma while the wrist is in full radial deviation under load or when the wrist is forced into full radial deviation while under load. The pain of extensor carpi ulnaris tendinitis is constant, severe, and localized in the dorsoulnar aspect of the wrist. Significant sleep disturbance is often reported. Patients with extensor carpi ulnaris tendinitis exhibit pain with resisted radial deviation of the wrist. A creaking or grating sensation may be palpated when the wrist is passively deviated radially. As mentioned, the chronically inflamed extensor carpi ulnaris tendon may rupture suddenly with stress or during vigorous injection procedures inadvertently injected into the substance of the tendon.

Keywords diagnostic ultrasound; extensor carpi ulnaris tendinitis; extensor carpi ulnaris; hand pain; magnetic resonance imaging; sports injury; tendinitis; tendinopathy; ultrasound guided injection

ICD-9 CODE M65.849

The Clinical Syndrome Extensor carpi ulnaris tendinitis is being seen with increasing frequency in clinical practice as golf and racquet sports have increased in popularity. The extensor carpi ulnaris tendon is susceptible to the development of tendinitis at the distal portion. The extensor carpi ulnaris tendon is subject to repetitive motion that may result in microtrauma, which heals poorly because of the tendon’s avascular nature. Exercise is often implicated as the inciting factor of acute extensor carpi ulnaris tendinitis, with improper grip of golf clubs and tennis racquets a common inciting cause (Fig. 62.1). Tendinitis of the extensor carpi ulnaris tendon frequently coexists with bursitis, creating additional pain and functional disability. Calcium deposition around the tendon may occur if the inflammation continues, making subsequent treatment more difficult. Continued trauma to the inflamed tendon ultimately may result in tendon rupture.

FIG. 62.1 Improper grip of golf clubs and tennis racquets is often implicated as the inciting cause of acute extensor carpi ulnaris tendinitis.

Signs and Symptoms The onset of extensor carpi ulnaris tendinitis is usually acute, occurring after overuse or misuse of the wrist joint. Inciting factors include playing tennis, playing golf, and prolonged use of a heavy hammer. Injuries ranging from partial to complete tears of the tendon can occur when the distal tendon sustains direct trauma while the wrist is in full radial deviation under load or when the wrist is forced into full radial deviation while under load. The pain of extensor carpi ulnaris tendinitis is constant, severe, and localized in the dorsoulnar aspect of the wrist. Significant sleep disturbance is often reported. Patients with extensor carpi ulnaris tendinitis exhibit pain with resisted radial deviation of the wrist and will exhibit an ECU synergy test (Fig. 62.2). A creaking or grating sensation may be palpated when the wrist is passively deviated radially. As mentioned, the chronically inflamed extensor carpi ulnaris tendon may rupture suddenly with stress or during vigorous injection procedures inadvertently injected into the substance of the tendon.

FIG. 62.2 The extensor carpi ulnaris (ECU) synergy test is performed by having the patient radially deviate the thumb against resistance. Note that the ECU tendon bowstrings against the skin (large arrow). From Ruland RT, Hogan CJ. The ECU synergy test: an aid to diagnose ECU tendonitis. J Hand Surg. 2008;33[10]:1777–1782, fig 1.

FIG. 62.3 Axial T1W (A) and FST2W (B) MR arthrogram images of a patient being investigated for ulnar-sided wrist pain and exclusion of a triangular fibrocartilage tear. The extensor carpi ulnaris (ECU) tendon (white arrows) is thickened and rounded with increased SI consistent with tendinopathy. There is also thickening of the tendon sheath. Compare with the normal ECU tendon on T1W (C) and FST2W (D) MR images (white arrows) in another subject, in whom the tendon is more ovoid and smaller with uniformly low SI. The small amount of high-SI fluid within the tendon sheath on the second FST2W MR image is within normal limits and is visible in other tendon groups. From Waldman SD. Extensor carpi ulnaris tendinitis. In: Waldman SD, Campbell RSD, eds. Imaging of Pain. Philadelphia: Saunders; 2011:329–330, fig 129.2.

Testing Plain radiographs, ultrasound imaging, and magnetic resonance imaging (MRI) are indicated for all patients who present with ulnar-sided wrist pain. Based on the patient’s clinical presentation, additional tests, including complete blood count, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. MRI of the wrist is indicated if joint instability is suspected and to confirm the diagnosis further (Fig. 62.3). Ultrasound imaging is also useful in aiding in diagnosis (Fig. 62.4). Radionuclide bone scanning is useful to identify stress fractures of the wrist not seen on plain radiographs.

Differential Diagnosis Extensor carpi ulnaris tendinitis is generally easily identified on clinical grounds; however, coexistent bursitis may confuse the diagnosis. Fractures of the ulnar styloid and lunate and tears of the triangular fibrocartilage complex, ulnocarpal abutment syndrome, and Kienböck disease also may mimic extensor carpi ulnaris tendinitis.

Treatment Initial treatment of the pain and functional disability associated with extensor carpi ulnaris tendinitis should include a combination of nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and physical therapy. Local application of heat and cold also may be beneficial. Repetitive activities responsible for the evolution of the tendinitis should be avoided. For patients who do not respond to these treatment modalities, injection with local anesthetic and steroid may be a reasonable next step. The use of ultrasound guidance may improve the accuracy of needle placement and decrease the incidence of needle-induced complications.

FIG. 62.4 Longitudinal Ultrasound Image of a Patient With Simple Tenosynovitis of the Extensor Carpi Ulnaris (ECU) TendonThere is anechoic fluid (white arrows) within the ECU tendon sheath. The ECU tendon (asterisks) is not thickened. From Waldman SD. Extensor carpi ulnaris tendinitis. In: Waldman SD, Campbell RSD, eds. Imaging of Pain. Philadelphia: Saunders; 2011:329–330.

Complications and Pitfalls Trauma to the extensor carpi ulnaris tendon from the injection itself is an ever-present possibility. Tendons that are highly inflamed or previously damaged are subject to rupture if they are directly injected. This complication can be greatly decreased if the clinician uses gentle technique and stops injecting immediately if significant resistance to injection is encountered. Approximately 25% of patients report a transient increase in pain after this injection technique, and patients should be warned of this possibility.

Clinical Pearls The extensor carpi ulnaris is a very strong tendon, but it is also very susceptible to rupture. Coexistent bursitis and arthritis also may contribute to wrist pain and may require additional treatment with a more localized injection of local anesthetic and methylprednisolone acetate. Injection of the extensor carpi ulnaris tendon is a safe procedure if careful attention is paid to the clinically relevant anatomy in the areas to be injected. The use of physical modalities, including local heat and gentle range-ofmotion exercises, should be introduced several days after the patient undergoes this injection technique for elbow pain. Vigorous exercises should be avoided because they would exacerbate the patient’s symptoms. Simple analgesics and NSAIDs may be used concurrently with this injection technique.

Suggested Readings Allende C, Le Viet D. Extensor carpi ulnaris problems at the wrist: classification, surgical treatment and results. J Hand Surg Br. 2005;30:265– 272. Conroy C, Ruchelsman D.E, Vitale M.A. Extensor carpi ulnaris instability in athletes - diagnosis and treatment. Operative Techniques Sports Med. 2016;24(2):139–147. Henderson C.J, Kobayashi K.M. Ulnar-sided wrist pain in the athlete. Orthop Clin North Am. 2016;47(4):789–798. Jeantroux J, Becce F, Guerini H, et al. Athletic injuries of the extensor carpi ulnaris subsheath: MRI findings and utility of gadolinium-enhanced fatsaturated T1-weighted sequences with wrist pronation and supination. Eur Radiol. 2011;21:160–166. Stewart S.K, Singleton J.A.G. Spontaneous extensor carpi ulnaris compartment syndrome. J Hand Surg. 2016;41(6):e143–e145. Waldman S.D. Extensor carpi ulnaris tendinitis. In: Waldman S.D, Campbell R.S.D, eds. Imaging of Pain. Philadelphia: Saunders; 2011:329–330. Watanabe A, Souza F, Vezeridis P.S, Blazar P, Yoshioka H. Ulnar-sided wrist pain. II. Clinical imaging and treatment. Skeletal Radiol. 2010;39:837–859.

63

Flexor Carpi Radialis Tendinitis

Abstract The onset of flexor carpi radialis tendinitis is usually acute, occurring after overuse or misuse of the wrist joint. Inciting factors include playing tennis, playing golf, and prolonged use of a heavy hammer. Injuries ranging from partial to complete tears of the tendon can occur when the distal tendon sustains direct trauma while the wrist is in full ulnar deviation under load or when the wrist is forced into full ulnar deviation while under load. The pain of flexor carpi radialis tendinitis is constant and severe and is localized in the dorsoradial aspect of the wrist. Significant sleep disturbance is often reported. Patients with flexor carpi radialis tendinitis exhibit pain with resisted ulnar deviation of the wrist. A creaking or grating sensation may be palpated when the wrist is passively deviated radially. As mentioned, the chronically inflamed flexor carpi radialis tendon may rupture suddenly with stress or during vigorous injection procedures inadvertently injected into the substance of the tendon.

Keywords diagnostic ultrasound; flexor carpi radialis tendinitis; flexor carpi radialis; magnetic resonance imaging; sports injuries; tendinitis; tendinopathy; ultrasound guided injections; wrist pain

ICD-10 CODE M65.849

The Clinical Syndrome Flexor carpi radialis tendinitis is being seen with increasing frequency in clinical practice as golf and racquet sports have increased in popularity. The flexor carpi radialis tendon is susceptible to the development of tendinitis at its distal portion. The flexor carpi radialis tendon is subject to repetitive motion that may result in microtrauma, which heals poorly because of the tendon’s avascular nature. Exercise and repetitive trauma are often implicated as inciting factors of acute flexor carpi radialis tendinitis, with improper grip of golf clubs or tennis racquets and the prolonged use of a heavy hammer as the common inciting causes (Fig 63.1). Tendinitis of the flexor carpi radialis tendon frequently coexists with bursitis, creating additional pain and functional disability. Calcium deposition around the tendon may occur if the inflammation continues, making subsequent treatment more difficult. Continued trauma to the inflamed tendon ultimately may result in tendon rupture.

Signs and Symptoms The onset of flexor carpi radialis tendinitis is usually acute, occurring after overuse or misuse of the wrist joint. Inciting factors include playing tennis, playing golf, and prolonged use of a heavy hammer. Injuries ranging from partial to complete tears of the tendon can occur when the distal tendon sustains direct trauma while the wrist is in full ulnar deviation under load or when the wrist is forced into full ulnar deviation while under load. The pain of flexor carpi radialis tendinitis is constant and severe and is localized in the dorsoradial aspect of the wrist. Significant sleep disturbance is often reported. Patients with flexor carpi radialis tendinitis exhibit pain with resisted ulnar deviation of the wrist. A creaking or grating sensation may be palpated when the wrist is passively deviated radially. As mentioned, the chronically inflamed flexor carpi radialis tendon may rupture suddenly with stress or during vigorous injection procedures inadvertently injected into the substance of the tendon.

Testing Plain radiographs, ultrasound imaging, and magnetic resonance imaging (MRI) are indicated for all patients who present with ulnar-sided wrist pain. Based on the patient’s clinical presentation, additional tests, including complete blood count, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. MRI and ultrasound imaging of the wrist are indicated if joint instability or occult mass is suspected and to further confirm the diagnosis (Figs. 63.2–63.4). Radionuclide bone scanning is useful to identify stress fractures of the wrist not seen on plain radiographs.

FIG 63.1 Exercise and repetitive trauma, such as prolonged use of a heavy hammer, are often implicated as inciting factors of acute flexor carpi radialis tendinitis.

FIG 63.2 Tenosynovitis of the Flexor Carpi Radialis Tendon Sheath: Magnetic Resonance Imaging (MRI)Coronal T1-weighted (TR/TE, 600/14) spin echo MRI of the volar aspect of the wrist shows the enlarged tendon sheath (arrow) containing fluid or inflammatory tissue. From Resnick D, ed. Diagnosis of Bone and Joint Disorders. 4th ed. Philadelphia: Saunders; 2002:3049.

FIG 63.3 Flexor Carpi Radialis (FCR) Tendinopathy in a 65-Year-Old Woman With a Painful Palpable Lump Over the Ventral Radial Aspect of the Right WristThe patient was referred for ultrasound examination for a suspected ventral ganglion cyst. (A) Anteroposterior radiograph reveals scapholunate diastasis and advanced triscaphe arthritis (arrows). (B) Transverse ultrasound image over the lump demonstrates a swollen and heterogeneous FCR tendon (arrows) stabilized over the scaphoid tubercle by a thickened retinaculum (white arrowhead). (C) Longitudinal ultrasound image shows bony spurs (hollow arrowhead) from the ventral aspect of the scaphoid and the trapezium (tra) impinging on the undersurface of the abnormal tendon. The retinaculum is thickened (solid arrowheads). From Allen PL, Baxter GM, Weston MJ. Clinical Ultrasound. 3rd ed, vol. 2. New York: Churchill Livingstone; 2011:1060.

FIG 63.4 Longitudinal Ultrasound Image of the Flexor Carpi Radialis (FCR) Demonstrating Peritendinous Fluid Consistent With Tendinitis.

Differential Diagnosis Flexor carpi radialis tendinitis is generally easily identified on clinical grounds; however, coexistent bursitis may confuse the diagnosis. Fractures of the distal radius and scaphoid and tears of the triangular fibrocartilage complex and avascular necrosis of the scaphoid also may mimic flexor carpi radialis tendinitis.

Treatment Initial treatment of the pain and functional disability associated with flexor carpi radialis tendinitis should include a combination of nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and physical therapy. Local application of heat and cold also may be beneficial. Repetitive activities responsible for the evolution of the tendinitis should be avoided. For patients who do not respond to these treatment modalities, injection with local anesthetic and steroid may be a reasonable next step. Use of ultrasound guidance may improve the accuracy of needle placement and decrease the incidence of needle-related complications.

Complications and Pitfalls Trauma to the flexor carpi radialis tendon from the injection itself is an everpresent possibility. Tendons that are highly inflamed or previously damaged are subject to rupture if they are directly injected. This complication can be greatly decreased if the clinician uses gentle technique and stops injecting immediately if significant resistance to injection is encountered. Approximately 25% of patients report a transient increase in pain after this injection technique, and patients should be warned of this possibility.

Clinical Pearls The flexor carpi radialis is a very strong tendon, yet it is also very susceptible to rupture. Coexistent bursitis and arthritis may contribute to wrist pain and may require additional treatment with a more localized injection of local anesthetic and methylprednisolone acetate. Injection of the flexor carpi radialis tendon is a safe procedure if careful attention is paid to the clinically relevant anatomy in the areas to be injected. The use of physical modalities, including local heat and gentle range-ofmotion exercises, should be introduced several days after the patient undergoes this injection technique for elbow pain. Vigorous exercises should be avoided because they would exacerbate the patient’s symptoms. Simple analgesics and NSAIDs may be used concurrently with this injection technique.

Suggested Readings Bishop A.T, Gabel G, Carmichael S.W. Flexor carpi radialis tendinitis. I. Operative anatomy. J Bone Joint Surg Am. 1994;76:1009–1014. Cowey A.J, Carmont M.R, Tins B, Ford D.J. Flexor carpi radialis rupture reined in!. Injury Extra. 2007;38:90–93. Fitton J.M, Shea F.W, Goldie W. Lesions of flexor carpi radialis tendon and sheath causing pain at the wrist. J Bone Joint Surg Br. 1968;50:359–363. Gabel G, Bishop A.T, Wood M.B. Flexor carpi radialis tendinitis. II. Results of operative treatment. J Bone Joint Surg Am. 1994;76:1015–1018. Gaston R.G, Loeffler B.J. Sports-specific injuries of the hand and wrist. Clini Sports Med. 2015;34(1):1–10. Kosiyatrakul A, Luenam S, Prachaporn S. Symptomatic flexor carpi radialis brevis: case report. J Hand Surg. 2010;35:633–635. Trehan S.K, Weiland A.J. Baseball and softball injuries: elbow, wrist, and hand. J Hand Surg. 2015;40(4):826–830.

64

Trigger Wrist

Abstract Trigger wrist is a rare cause of wrist pain and functional disability caused by inflammation and swelling of the wrist flexor tendon apparatus or by tumors or masses affecting the wrist flexor tendons. Commonly, the tendinopathy associated with trigger wrist is due to compression by the carpal bones, especially the hook of the hamate bone. Sesamoid bones in this region may also compress and cause trauma to the tendons. Trauma is usually the result of repetitive motion or pressure on the tendon as it passes over these bony prominences. If the inflammation and swelling become chronic, the tendon sheath may thicken, resulting in constriction. Frequently, nodules develop on the tendon, and they can often be palpated when the patient flexes and extends the wrists. Such nodules may catch in the tendon sheath as they pass under the transverse palmar ligament in a manner analogous to the more common trigger finger phenomenon, producing a triggering that causes the wrist to catch or lock. Trigger wrist occurs in patients engaged in repetitive activities such as playing the drums.

Keywords diagnostic ultrasound; hand pain; musician diseases; sesamoid bones; tendinitis; tendinopathy; tendon sheath; trigger wrist; triggering phenomenon; ultrasound guided procedure; wrist flexor tendon apparatus

ICD-10 CODE M65.849

The Clinical Syndrome Trigger wrist is a rare cause of wrist pain and functional disability caused by inflammation and swelling of the wrist flexor tendon apparatus or by tumors or masses affecting the wrist flexor tendons. Commonly, the tendinopathy associated with trigger wrist is due to compression by the carpal bones, especially the hook of the hamate bone. Sesamoid bones in this region may also compress and cause trauma to the tendons. Trauma is usually the result of repetitive motion or pressure on the tendon as it passes over these bony prominences. If the inflammation and swelling become chronic, the tendon sheath may thicken, resulting in constriction. Frequently, nodules develop on the tendon, and they can often be palpated when the patient flexes and extends the wrists. Such nodules may catch in the tendon sheath as they pass under the transverse palmar ligament in a manner analogous to the more common trigger finger phenomenon, producing a triggering that causes the wrist to catch or lock. Other causes of trigger wrist include rheumatoid nodules, anomalous muscles (especially of the flexor digitorum superficialis), ganglion cysts, and pigmented villonodular synovitis (Box 64.1). Trigger wrist occurs in patients engaged in repetitive activities such as playing the drums (Fig. 64.1).

Signs and Symptoms The pain of trigger wrist is localized to the distal wrist and proximal palm, and tender nodules often can be palpated. The pain is constant and is made worse with active flexion/extension of the wrist. Patients note significant stiffness when flexing the wrists. Sleep disturbance is common, and patients often awaken to find that the wrist has become locked in a flexed position. On physical examination, tenderness and swelling are noted over the tendon, with maximal point tenderness over the carpal bones. Many patients with trigger wrist experience a creaking or snapping sensation with flexion and extension of the wrists. Range of motion of the wrists may be decreased because of pain, and a triggering phenomenon may be noted. A catching tendon sign may be elicited by having the patient flex the affected wrist for 30 seconds and then relax but not unflex the wrist. The examiner then passively extends the affected wrist, and if a locking, popping, or catching of the tendon is appreciated as the wrist is straightened, the sign is positive (Fig. 64.2).

Testing Plain radiographs are indicated in all patients who present with trigger wrist to rule out occult bony pathological processes. Based on the patient’s clinical presentation, additional testing, including a complete blood count, uric acid level, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. Magnetic resonance imaging (MRI) and ultrasound imaging of the hand is indicated if joint instability, mass, tumor, or some other abnormality is suspected (Figs. 64.3 and 64.4). The injection technique described later serves as both a diagnostic and therapeutic maneuver. Occasionally, surgical exploration is required to accurately ascertain the cause of trigger wrist (Fig. 64.5).

Box 64. 1 C om m on C a use s of Trigge r Wrist • Tenosynovitis • Anomalous muscles • Rheumatoid nodules • Fibromas of the tendon sheath • Pigmented villonodular synovitis • Ganglion cysts

FIG. 64.1 Trigger wrist is the result of repetitive microtrauma from repeated flexion and extension of the wrist.

FIG. 64.2 The Catching Tendon Sign for Trigger Wrist(A) The patient is asked to actively flex the affected wrist for 30 seconds. (B) The examiner passively extends the affected wrist while palpating the flexor tendons.

FIG. 64.3 (A and B) Coronal and axial magnetic resonance imaging shows a softtissue mass (white arrows) wrapped around the flexor tendon sheath at the distal border of the carpal tunnel. (C and D) Intraoperatively, a pale brown oval mass (M) was identified between the flexor tendons (∗) and median nerve (∗∗). (E) A 1.0- × 2.5-cm mass was resected. (F) Microscopic examination revealed hypertrophy of fibrotic tissue, confirming a fibroma of the tendon sheath (hematoxylin and eosin [H– E] staining, ×200 high-power field). From Park I-J, Lee Y-M, Kim H-M, et al. Multiple etiologies of trigger wrist. J Plastic Reconstr Aesthet Surg. 2016;69[3]:335–340, fig 1.

FIG. 62.4 (A and B) Ultrasonography shows a hypoechoic mass (white arrows) around the flexor tendon at the proximal portion. (C) Intraoperatively, an anomalous muscle belly (∗) of the flexor digitorum superficialis (FDS) of the index/middle finger is shown within the carpal tunnel. (D) Surgical excision of the anomalous FDS muscle belly was performed, allowing its tendon to move freely within the carpal tunnel. (E) Histology shows normal skeletal muscle (H–E stain, ×200 high-power field). From Park I-J, Lee Y-M, Kim H-M, et al. Multiple etiologies of trigger wrist. J Plastic Reconstr Aesthet Surg. 2016;69[3]:335–340, fig 2.

FIG. 62.5 Giant Cell Tumor of the Flexor Sheath Compressing the Median Nerve From Chalmers RL, Mandalia M, Contreras R, et al. Acute trigger wrist and carpal tunnel syndrome due to giant-cell tumour of the flexor sheath. J Plast Reconstr Aesthet Surg. 2008;61:1557.

Differential Diagnosis The diagnosis of trigger wrist is usually made on clinical grounds. Arthritis or gout of the carpal or radioulnar joint may accompany trigger wrist and exacerbate the patient’s pain. Occult fractures occasionally confuse the clinical presentation. Trigger finger and carpal tunnel syndrome frequently coexist with the much less commonly occurring trigger wrist.

Treatment Initial treatment of the pain and functional disability associated with trigger wrist includes a combination of nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and physical therapy. A nighttime splint to protect the wrists also may help relieve the symptoms. If these treatments fail, the following injection technique is a reasonable next step. Injection of trigger wrist is carried out by placing the patient in the supine position with the arm fully adducted at the patient’s side and the dorsal surface of the hand resting on a folded towel. A total of 2 mL local anesthetic and 40 mg methylprednisolone is drawn up in a 5-mL sterile syringe. After sterile preparation of the skin overlying the affected tendon, the carpal bone beneath the tendon is identified. Using strict aseptic technique, at a point just proximal to the joint, a 1-inch, 25-gauge needle is inserted at a 45-degree angle parallel to the affected tendon through the skin and into the subcutaneous tissue overlying the tendon, with care taken to avoid the median nerve as it passes under the transverse carpal ligament and radial nerve and artery. If bone is encountered, the needle is withdrawn into the subcutaneous tissue. The contents of the syringe are then gently injected. The tendon sheath should distend as the injection proceeds. Little resistance to injection should be felt; if resistance is encountered, the needle is probably in the tendon and should be withdrawn until the injection can be accomplished without significant resistance. The needle is then removed, and a sterile pressure dressing and ice pack are applied to the injection site. Ultrasound guidance may improve the accuracy of needle placement and decrease the incidence of needle-related complications. Physical modalities, including local heat and gentle range-of-motion exercises, should be introduced several days after the patient undergoes injection. Vigorous exercises should be avoided, because they will exacerbate the patient’s symptoms. Surgical treatment should be considered for patients who fail to respond to the aforementioned treatment modalities.

Complications and Pitfalls Failure to adequately treat trigger wrist early in its course can result in permanent pain and functional disability because of continued trauma to the tendon and tendon sheath. The major complications associated with injection are related to trauma to the inflamed and previously damaged tendon. The tendon may rupture if it is injected directly, so a needle position outside the tendon should be confirmed before proceeding with the injection. Furthermore, the radial artery and superficial branch of the radial nerve are susceptible to damage if the needle is placed too far medially. Another complication of injection is infection, although it should be exceedingly rare if strict aseptic technique is used, along with universal precautions to minimize any risk to the operator. The incidence of ecchymosis and hematoma formation can be decreased if pressure is applied to the injection site immediately after injection. Approximately 25% of patients report a transient increase in pain after injection, and they should be warned of this possibility.

Clinical Pearls The injection technique described is extremely effective in the treatment of pain secondary to trigger wrist. Coexistent arthritis or gout may contribute to the patient’s pain, necessitating additional treatment with more localized injection of local anesthetic and methylprednisolone. A hand splint to protect the wrists also may help relieve the symptoms of trigger wrist. Simple analgesics and NSAIDs can be used concurrently with the injection technique, although surgical treatment may ultimately be required to provide permanent relief.

Suggested Readings Al-Qattan M.M, Elshamma N.A, Alqabbani A. Trigger wrist and carpal tunnel syndrome caused by a flexor tendon-related ganglion in a teenager: a case report. Int J Surg Case Reports. 2017;30:86–88. Chalmers R.L, Mandalia M, Contreras R, Schreuder F. Acute trigger wrist and carpal tunnel syndrome due to giant-cell tumour of the flexor sheath. J Plast Reconstr Aesthet Surg. 2008;61:1557. Colio S.W, Smith J, Pourcho A.M. Ultrasound-guided interventional procedures of the wrist and hand: anatomy, indications, and techniques. Phy Med Rehabil Clin North Am. 2016;27(3):589–605. Giannikas D, Karabasi A, Dimakopoulos P. Trigger wrist. J Hand Surg Br. 2007;32:214–216. Park I.-J, Lee Y.-M, Kim H.-M, et al. Multiple etiologies of trigger wrist. J Plastic Reconstructive Aesthetic Surg. 2016;69(3):335–340. Pople I.K. Trigger wrist due to idiopathic synovial hypertrophy. J Hand Surg Br. 1986;11:453–454. Ragheb D, Stanley A, Gentili A, Hughes T, Chung C.B. MR imaging of the wrist tendons: normal anatomy and commonly encountered pathology. Eur J Radiol. 2005;56:296–306. Sonoda H, Takasita M, Taira H, Higashi T, Tsumura H. Carpal tunnel syndrome and trigger wrist caused by a lipoma arising from flexor tenosynovium: a case report. J Hand Surg Am. 2002;27:1056–1058. Waldman S.D. Painful Conditions of the Wrist and Hand. Physical Diagnosis of Pain: An Atlas of Signs and Symptoms. 3rd ed. Philadelphia: Elsevier; 2016:166–167. Waldman S.D. Trigger Finger. Atlas of Pain Management Injection Techniques. 4th ed. Philadelphia: Elsevier; 2017:301–303. Woo H.-H, Lee Y.-K, Kim J.-M, Cheon H.-J, Chung W.H. Hand and wrist injuries in golfers and their treatment. Hand Clin. 2017;33(1):81–96.

SECT ION 6

Thoracic Pain Syndromes OUT LINE 65. Devil’s Grip 66. Sternoclavicular Syndrome 67. Postmastectomy Pain 68. Sternalis Syndrome 69. Manubriosternal Joint Pain 70. Xiphodynia 71. Serratus Anterior Muscle Syndrome 72. Slipping Rib Syndrome 73. Winged Scapula Syndrome

65

Devil’s Grip ICD-10 CODE R07.81

The Clinical Syndrome Devil’s grip is an uncommon cause of chest pain. Also known as Bornholm disease, the grip of the phantom, dry pleurisy, and Sylvest disease, devil’s grip is caused by acute infection with coxsackievirus and less frequently by echovirus. This virus is transmitted via the fecal–oral route and is highly contagious, owing to a long period of viral shedding of 6 weeks. In some patients, their immune system limits the infection to a mild fever or flulike illness called summer fever. In others, a full-fledged infection with resultant pleurodynia and cough develops. Devil’s grip has a seasonal variation in occurrence, with 90% of cases occurring in the summer and fall, with August being the peak month. No gender predilection is seen, but the disease occurs more commonly in young adults and occasionally in children. The pain is severe and described as sharp or pleuritic. The pain occurs in paroxysms that can last 30 minutes.

Signs and Symptoms Physical examination of a patient with devil’s grip reveals a patient who appears acutely ill (Fig. 65.1). Pallor and fever are invariably present, as is tachycardia. Patients may report of malaise, sore throat, and arthralgia, which may confuse the clinical picture. Examination of the chest wall reveals minimal physical findings, although a friction rub is sometimes present. During the paroxysms of pain, the patient suffering from devil’s grip attempts to splint or protect the affected area. Deep inspiration or movement of the chest wall markedly increases the pain of devil’s grip.

Testing Plain radiographs are indicated in all patients with pain thought to be the result of infection with coxsackievirus to rule out occult chest wall pathology, pulmonary tumors, pneumonia, or empyema (Fig. 65.2). Ventilation/perfusion studies of the lungs are indicated if pulmonary embolism is being considered in the differential diagnosis. Based on the patient’s clinical presentation, additional tests, including complete blood cell count, sedimentation rate, and throat cultures for Streptococcus spp., may be indicated. Computed tomography (CT) scan of the thoracic contents is indicated if occult mass or empyema is suspected.

FIG. 65.1 Deep Inspiration Markedly Increases the Pain of Devil’s Grip.

FIG. 65.2 (A) This patient presented with a right upper lobe pneumonia (∗) and a pleural effusion (arrow). (B) Chest computed tomography shows the effusion (∗) that appears to be free flowing as it is dependent. (C) An ultrasound shows multiple septations in the pleural fluid (arrows). (D) Radiograph after image-guided insertion of a small-bore chest tube and fibrinolytic therapy. The empyema is nearly resolved, with persistent pneumonia (∗) causing persistent fevers. (E) Minimal residual pleural thickening (arrow) seen after removal of the chest tube and completion of antibiotics. From Hogan MJ, Coley BD. Interventional radiology treatment of empyema and lung abscesses. Paediatr Respir Rev. 2008;9:77–84.

Differential Diagnosis As is the case with costochondritis, costosternal joint pain, Tietze syndrome, and rib fractures, many patients with devil’s grip first seek medical attention because they believe they are having a heart attack. If the area innervated by the subcostal nerve is involved, patients may believe they have gallbladder disease. Statistically, children with devil’s grip have abdominal pain more often than do adults, and such pain may be attributed to appendicitis, leading to unnecessary surgery. In contradistinction to most other causes of pain involving the chest wall, which are musculoskeletal or neuropathic, the pain of devil’s grip is infectious. The constitutional symptoms associated with devil’s grip may lead the clinician to consider pneumonia, empyema, and occasionally pulmonary embolus as the most likely diagnosis. As mentioned earlier, the pain of devil’s grip is often mistaken for pain of cardiac or gallbladder origin and can lead to visits to the emergency department and unnecessary cardiac and gastrointestinal workups. If trauma has occurred, devil’s grip may coexist with fractured ribs or fractures of the sternum itself, which can be missed on plain radiographs and may require radionucleotide bone scanning for proper identification. Tietze syndrome, which is painful enlargement of the upper costochondral cartilage associated with viral infection, can be confused with devil’s grip. Neuropathic pain involving the chest wall also may be confused or coexist with costosternal syndrome. Examples of such neuropathic pain include diabetic polyneuropathies and acute herpes zoster involving the thoracic nerves. The possibility of diseases of the structures of the mediastinum is ever present, and such disease sometimes can be difficult to diagnose. Pathological processes that inflame the pleura, such as pulmonary embolus, infection, and tumor, also need to be considered.

Treatment Initial treatment of devil’s grip should include a combination of simple analgesics and nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors. If these medications do not control the patient’s symptoms adequately, opioid analgesics may be added during the period of acute pain. Local application of heat and cold also may be beneficial to provide symptomatic relief of the pain of devil’s grip. The use of an elastic rib belt may help provide symptomatic relief in some patients. For patients who do not respond to the aforementioned treatment modalities, the following injection technique using a local anesthetic and steroid may be a reasonable next step. The patient is placed in the prone position with the patient’s arms hanging loosely off the side of the cart. Alternatively, this block can be done with the patient in the sitting or lateral position. The rib to be blocked is identified by palpating its path at the posterior axillary line. The index and middle fingers are placed on the rib, bracketing the site of needle insertion. The skin is prepared with antiseptic solution. A 22-gauge, 11⁄2-inch needle is attached to a 12-mL syringe and is advanced perpendicular to the skin, aiming for the middle of the rib between the index and middle fingers. The needle should impinge on bone after being advanced approximately 3/4 inch. After bony contact is made, the needle is withdrawn into the subcutaneous tissues and the skin and subcutaneous tissues are retracted with the palpating fingers inferiorly; this allows the needle to be walked off the inferior margin of the rib. As soon as bony contact is lost, the needle is slowly advanced approximately 2 mm deeper; this places the needle in proximity to the costal groove, which contains the intercostal nerve and the intercostal artery and vein (Fig. 65.3). After careful aspiration reveals no blood or air, 3 to 5 mL of 1% preservative-free lidocaine is injected. If the pain has an inflammatory component, the local anesthetic is combined with 80 mg of methylprednisolone and is injected in incremental doses. Subsequent daily nerve blocks are done in a similar manner, substituting 40 mg of methylprednisolone for the initial 80-mg dose. Because of the overlapping innervation of the chest and upper abdominal wall, the intercostal nerves above and below the nerve suspected of subserving the painful condition need to be blocked. Ultrasound guidance may improve the accuracy of needle placement and decrease the incidence of needle-related complications.

Complications and Pitfalls The major problem in the care of patients believed to have devil’s grip is the failure to identify potentially serious pathological conditions of the thorax or upper abdomen. Given the proximity of the pleural space, pneumothorax after intercostal nerve block is a distinct possibility. The incidence of the complication is less than 1%, but it occurs with greater frequency in patients with chronic obstructive pulmonary disease. Because of the proximity to the intercostal nerve and artery, the clinician should calculate carefully the total milligram dosage of local anesthetic administered, because vascular uptake by these vessels is high. Although uncommon, infection is an ever-present possibility, especially in an immunocompromised patient with cancer. Early detection of infection is crucial to avoid potentially life-threatening sequelae.

Clinical Pearls Devil’s grip is an uncommon cause of chest pain that is frequently misdiagnosed. Correct diagnosis is necessary to treat this painful condition properly and to avoid overlooking serious intrathoracic or intra abdominal pathology. Intercostal nerve block is a simple technique that can produce dramatic relief for patients with devil’s grip. As mentioned, the proximity of the intercostal nerve to the pleural space makes careful attention to technique mandatory.

FIG. 65.3 Injection Technique to Relieve the Pain of Devil’s Grip.a, Artery; n, nerve; v, vertebra.

Suggested Readings Connolly J.H, O’Neill H.J. Bornholm disease associated with coxsackie A9 virus infection. Lancet. 1971;298:1035. Cotterill J.A. The devil’s grip. Lancet. 1973;301:1308–1309. Huang W.-T, Lee P.-I, Chang L.-Y, et al. Epidemic pleurodynia caused by Coxsackievirus B3 at a medical center in northern Taiwan. J Microbiol Immunol Infection. 2010;43(6):515–518. Ikeda R.M, Kondracki S.F, Drabkin P.D, Birkhead G.S, Morse D.L. Pleurodynia among football players at a high school: an outbreak associated with coxsackievirus B1. JAMA. 1993;270:2205–2206. Leendertse M, van Vugt M, Benschop K.S, et al. Pleurodynia caused by an echovirus 1 brought back from the tropics. J Clin Virol. 2013;58(2):490–493. Stalkup J.R, Chilukuri S. Enterovirus infections: a review of clinical presentation, diagnosis, and treatment. Dermatol Clin. 2002;20:217–223. Waldman S.W. Chest wall pain syndromes. In: Pain Management. Philadelphia: W.B. Saunders; 2011:632–645 Chapter 73.

66

Sternoclavicular Syndrome

Abstract The sternoclavicular joint is often traumatized during acceleration/deceleration injuries and blunt trauma to the chest. With severe trauma, the joint may sublux or dislocate in association with fractures of adjacent structures. Overuse or misuse also can result in acute inflammation of the sternoclavicular joint, which can be debilitating. Because the sternoclavicular joint is a true joint, it is susceptible to the development of arthritis, including osteoarthritis, rheumatoid arthritis, ankylosing spondylitis, Reiter syndrome, infection, and psoriatic arthritis (Fig. 66.1). The joint also is subject to invasion by tumor from either primary malignancies, including thymoma, or metastatic disease. Pain emanating from the sternoclavicular joint often mimics the pain of cardiac origin.

Keywords chest wall pain; computed tomography; diagnostic ultrasound; magnetic resonance imaging; seatbelt injury; septic arthritis; sternoclavicular syndrome; vehicular trauma; ultrasound guided injection

ICD-10 CODE R07.89

The Clinical Syndrome With the advent of seat belts that cross the chest, sternoclavicular syndrome is being seen with greater frequency by clinicians. The joint is often traumatized during acceleration/deceleration injuries and blunt trauma to the chest. With severe trauma, the joint may sublux or dislocate in association with fractures of adjacent structures. Overuse or misuse also can result in acute inflammation of the sternoclavicular joint, which can be debilitating. Because the sternoclavicular joint is a true joint, it is susceptible to the development of arthritis, including osteoarthritis, rheumatoid arthritis, ankylosing spondylitis, Reiter syndrome, infection, and psoriatic arthritis (see Fig. 66.1). The joint also is subject to invasion by tumor from either primary malignancies, including thymoma, or metastatic disease. Pain emanating from the sternoclavicular joint often mimics the pain of cardiac origin.

FIG. 66.1 Tubercular Infection of the Sternoclavicular Joint From Meena UK, Saibaba B, Behera P, et al. Sternoclavicular joint tuberculosis: a series of 9 cases. Ind J Tubercul. 64[3]:2017;221–224.

Signs and Symptoms On physical examination, obvious physical deformity may be present and the patient vigorously attempts to splint the joint by keeping the shoulders stiffly in neutral position (Figs. 66.2 and 66.3). Pain is reproduced with active protraction or retraction of the shoulder and full elevation of the arm. Shrugging of the shoulder also may reproduce the pain. The sternoclavicular joint may be tender to palpation and feel hot and swollen if acutely inflamed. The patient also may report a clicking sensation with movement of the joint.

Testing Plain radiographs are indicated in all patients who have pain thought to emanate from the sternoclavicular joint to rule out occult bony pathological processes, including tumor. Based on the patient’s clinical presentation, additional tests, including complete blood cell count, prostate-specific antigen level, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. Computed tomography (CT), ultrasound imaging, and magnetic resonance imaging (MRI) of the joint is indicated if joint instability, tumor, or infection is suspected and to clarify the diagnosis (Figs. 66.4 and 66.5). Injection of the sternoclavicular joint with a local anesthetic, steroid, or both serves as a diagnostic and therapeutic maneuver.

FIG. 66.2 Acute Protraction or Retraction of the Shoulder Reproduces the Pain of Sternoclavicular Syndrome.

FIG. 66.3 (A) Anterior view shows anterior dislocation of the right sternoclavicular joint. (B) Superior view shows posterior dislocation of the acromioclavicular joint. From Schemitsch LA, Schemitsch EH, McKee MD. Bipolar clavicle injury: posterior dislocation of the acromioclavicular joint with anterior dislocation of the sternoclavicular joint—a report of two cases. J Shoulder Elbow Surg. 2011;20:e18– e22.

Differential Diagnosis As mentioned earlier, the pain of sternoclavicular syndrome is often mistaken for pain of cardiac origin and can lead to visits to the emergency department and unnecessary cardiac workups. If trauma has occurred, sternoclavicular syndrome may coexist with fractured ribs or fractures of the sternum itself, which can be missed on plain radiographs and may require radionucleotide bone scanning for proper identification. Tietze syndrome, which is painful enlargement of the upper costochondral cartilage associated with viral infections, can be confused with sternoclavicular syndrome.

FIG. 66.4 (A and B) Three-dimensional computed tomography images show bipolar dislocation of the clavicle. From Schemitsch LA, Schemitsch EH, McKee MD. Bipolar clavicle injury: posterior dislocation of the acromioclavicular joint with anterior dislocation of the sternoclavicular joint—a report of two cases. J Shoulder Elbow Surg. 2011;20:e18– e22.

Neuropathic pain involving the chest wall also may be confused or coexist with sternoclavicular syndrome. Examples of such neuropathic pain include diabetic polyneuropathies and acute herpes zoster involving the thoracic nerves. The possibility of diseases of the structures of the mediastinum is ever present and these diseases sometimes can be difficult to diagnose. Pathological processes that inflame the pleura, such as pulmonary embolus, infection, and Bornholm disease, also should be considered.

Treatment Initial treatment of the pain and functional disability associated with sternoclavicular syndrome should include a combination of nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors. Local application of heat and cold also may be beneficial. The use of an elastic clavicle splint may help provide symptomatic relief and help protect the sternoclavicular joints from additional trauma. For patients who do not respond to these treatment modalities, injection of the sternoclavicular joint using a local anesthetic and steroid may be a reasonable next step. Ultrasound guidance may improve the accuracy of needle placement and decrease the incidence of needle-related complications.

FIG. 66.5 Advanced Imaging of Sternoclavicular Joint Dislocation(A) Ultrasound with linear array probe (10 to 15 mHz) showing posterior sternoclavicular dislocation (SCD) where clavicle (arrow) is angled posteriorly relative to sternum (star). (B) Ultrasound of patient’s contralateral unaffected and normal sternoclavicular joint (SCJ) where the clavicle (arrow) is aligned normally with the sternum (star). (C) CT scan showing the corresponding view of the right clavicle displacement posteriorly by 1.04 cm and hematoma. From Bengtzen RR, Petering RC. Point-of-care ultrasound diagnosis of posterior sternoclavicular joint dislocation. J Emerg Med. 2017;52[4]:513–515.

Complications and Pitfalls Because of the many pathological processes that may mimic the pain of sternoclavicular syndrome, the clinician must be careful to rule out underlying cardiac disease and diseases of the lung and structures of the mediastinum. Failure to do so could lead to disastrous results. The major complication of this injection technique is pneumothorax if the needle is placed too laterally or deeply and invades the pleural space. Infection, although rare, can occur if strict aseptic technique is not followed. The possibility of trauma to the contents of the mediastinum remains an everpresent possibility. This complication can be greatly decreased if the clinician pays close attention to accurate needle placement.

Clinical Pearls Patients with pain emanating from the sternoclavicular joint often attribute their pain symptoms to a heart attack. Reassurance is required, although it should be remembered that this musculoskeletal pain syndrome and coronary artery disease can coexist. Tietze syndrome, which is painful enlargement of the upper costochondral cartilage associated with viral infections, can be confused with sternoclavicular syndrome, although both respond to the injection technique described. The use of physical modalities, including local heat and gentle range-of-motion exercises, should be introduced several days after the patient undergoes this injection technique for sternoclavicular joint pain. Vigorous exercises should be avoided because they would exacerbate the patient’s symptoms. Simple analgesics and NSAIDs may be used concurrently with this injection technique. Laboratory evaluation for collagen-vascular disease is indicated in patients with sternoclavicular joint pain in whom other joints are involved.

Suggested Readings Bengtzen R.R, Petering R.C. Point-of-care ultrasound diagnosis of posterior sternoclavicular joint dislocation. J Emerg Med. 2017;52(4):513–515. Bicos J, Nicholson G.P. Treatment and results of sternoclavicular joint injuries. Clin Sports Med. 2003;22:359–370. Crisostomo R.A, Laskowski E.R, Bond J.R, Agerter D.C. Septic sternoclavicular joint: a case report. Arch Phys Med Rehabil. 2008;89:884– 886. . Meena U.K, Saibaba B, Behera P, Meena R.C. Sternoclavicular joint tuberculosis: a series of 9 cases. Indian J Tuberculosis. 2017;64(3):221–224. Qureshi M.Z, Gorczyca J.T, Doyle A.J, Gestring M.L. Posterior sternoclavicular joint dislocation: a rare manifestation of seatbelt injury. Surgery. 2017;162(4):958–960. Puri V, Meyers B.F, Kreisel D, et al. Sternoclavicular joint infection: a comparison of two surgical approaches. Ann Thorac Surg. 2011;91:257–264. Schemitsch L.A, Schemitsch E.H, McKee M.D. Bipolar clavicle injury: posterior dislocation of the acromioclavicular joint with anterior dislocation of the sternoclavicular joint—a report of two cases. J Shoulder Elbow Surg. 2011;20:e18–e22. Schipper P, Tieu B.H. Acute chest wall infections: surgical site infections, necrotizing soft tissue infections, and sternoclavicular joint infection. Thorac Surg Clin. 2017;27(2):73–86.

67

Postmastectomy Pain

Abstract Postmastectomy pain syndrome is a constellation of symptoms that includes pain in the anterior chest, breast, axilla, and medial upper extremity after surgical procedures on the breast. Postmastectomy pain is a misnomer because the clinical syndrome includes the pain mentioned here even if the patient has only a lumpectomy or if another, less extensive surgical procedure is performed on the breast. The pain is often described as constricting, with a continuing dull ache. In addition to these symptoms, many patients with postmastectomy pain syndrome also report sudden paresthesia radiating into the breast, axilla, or both. In some patients, a burning, allodynic pain reminiscent of reflex sympathetic dystrophy may be the principal manifestation. The intensity of postmastectomy pain is moderate to severe. The onset of postmastectomy pain may be immediately after surgery and initially be confused with expected postsurgical pain, or the onset may be more insidious, occurring gradually 2 to 6 weeks after the inciting surgical procedure. If complete mastectomy is performed, phantom breast pain may confound the diagnosis further, as may associated lymphedema. Sleep disturbance is a common finding in patients with postmastectomy pain.

Keywords antidepressants; breast cancer; breast pain; chest wall pain; gabapentin; hypnosis; neuropathic pain; phantom pain; postmastectomy pain

ICD-10 CODE N64.4

The Clinical Syndrome Postmastectomy pain syndrome is a constellation of symptoms that includes pain in the anterior chest, breast, axilla, and medial upper extremity after surgical procedures on the breast. Postmastectomy pain is a misnomer because the clinical syndrome includes the pain mentioned here even if the patient has only a lumpectomy or if another, less extensive surgical procedure is performed on the breast. The pain is often described as constricting, with a continuing dull ache. In addition to these symptoms, many patients with postmastectomy pain syndrome also report sudden paresthesia radiating into the breast, axilla, or both. In some patients, a burning, allodynic pain reminiscent of reflex sympathetic dystrophy may be the principal manifestation. The intensity of postmastectomy pain is moderate to severe. The onset of postmastectomy pain may be immediately after surgery and initially be confused with expected postsurgical pain, or the onset may be more insidious, occurring gradually 2 to 6 weeks after the inciting surgical procedure. If complete mastectomy is performed, phantom breast pain may confound the diagnosis further, as may associated lymphedema. Sleep disturbance is a common finding in patients with postmastectomy pain.

Signs and Symptoms Evaluation of a patient with postmastectomy syndrome requires that the clinician take a careful history designed to delineate the various components that make up the patient’s pain to help guide the physical examination. The clinician should question the patient specifically about the presence of phantom breast pain, which may be quite distressing to the patient when superimposed on the pain of postmastectomy syndrome. Typical physical findings in patients with postmastectomy syndrome include areas of decreased sensation, hyperpathia, and dysesthesia in the distribution of the intercostobrachial nerve, which is a branch of the second intercostal nerve (Fig. 67.1). This nerve is frequently damaged during breast surgery (Fig. 67.2). Allodynia outside the distribution of the intercostobrachial nerve also is often present. Movement of the arm and axilla often exacerbates the pain, which leads to splinting and disuse of the affected shoulder and upper extremity. This disuse often worsens any lymphedema that is present. If disuse of the upper extremity continues, frozen shoulder may develop, further complicating the clinical picture. The clinician should always be alert to the possibility of metastatic disease or direct extension of tumor into the chest wall, which may mimic the pain of postmastectomy syndrome. The findings of the targeted history and physical examination assist the clinician in making an assessment of the sympathetic, neuropathic, and musculoskeletal components of the pain and designing a rational treatment plan.

FIG. 67.1 The pain of postmastectomy syndrome is due to damage of the intercostobrachial nerve.

FIG. 67.2 Relationship of the intercostobrachial nerve (ICNB), the lateral thoracic vein (LTV), the axillary vein (AV), and the breast within the left axilla during mastectomy. From Ivanovic N, Granic M, Randjelovic T, et al. Fragmentation of axillary fibrofatty tissue during dissection facilitates preservation of the intercostobrachial nerve and the lateral thoracic vein. Breast. 2008;17:293–295.

Testing Plain radiographs are indicated in all patients who present with pain thought to be due to postmastectomy syndrome to rule out occult bony pathology, including tumor. Electromyography helps rule out damage to the intercostobrachial nerve or plexopathy that may be contributing to the patient’s pain. Radionucleotide bone scanning may be useful to rule out occult pathological fractures of the ribs, sternum, or both. Based on the patient’s clinical presentation, additional tests, including complete blood cell count, prostate-specific antigen level, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. Computed tomography (CT) scan of the thoracic contents is indicated if occult mass is suspected. Magnetic resonance imaging (MRI) of the brachial plexus also should be considered if plexopathy secondary to tumor involvement is a consideration.

Differential Diagnosis As mentioned earlier, the pain of postmastectomy syndrome is often mistaken for postoperative pain. If the breast surgery was performed for malignancy, a careful search for metastatic disease or tumor invasion of the chest wall is mandatory. Postmastectomy syndrome may coexist with pathological rib fractures or pathological fractures of the sternum itself, which can be missed on plain radiographs and may require radionucleotide bone scanning for proper identification. Neuropathic pain involving the chest wall also may be confused or coexist with postmastectomy syndrome. Examples of such neuropathic pain include diabetic polyneuropathies and acute herpes zoster involving the thoracic nerves. The possibility of diseases of the structures of the mediastinum is ever present, and these diseases sometimes can be difficult to diagnose. Pathological processes that inflame the pleura, such as pulmonary embolus, infection, and Bornholm disease, also may mimic the pain of postmastectomy syndrome.

Treatment Initial treatment of postmastectomy syndrome should include a combination of simple analgesics and nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors. If these medications do not control the patient’s symptoms adequately, a tricyclic antidepressant or gabapentin should be added. Traditionally, tricyclic antidepressants have been a mainstay in the palliation of pain secondary to postmastectomy syndrome. Controlled studies have shown the efficacy of amitriptyline for this indication. Other tricyclic antidepressants, including nortriptyline and desipramine, also have been shown to be clinically useful. This class of drugs is associated with significant anticholinergic side effects, however, including dry mouth, constipation, sedation, and urinary retention. These drugs should be used with caution in patients with glaucoma, cardiac arrhythmia, and prostatism. To minimize side effects and encourage compliance, the primary care physician should start amitriptyline or nortriptyline at a 10-mg dose at bedtime. The dose can be titrated to 25 mg at bedtime as side effects allow. Upward titration of dosage in 25-mg increments can be done each week as side effects allow. Even at lower doses, patients generally report a rapid improvement in sleep disturbance and begin to experience pain relief in 10 to 14 days. If the patient does not experience any improvement in pain as the dose is being titrated upward, the addition of gabapentin alone or in combination with nerve blocks of the intercostal nerves with local anesthetics, steroid, or both is recommended. Selective serotonin reuptake inhibitors, such as fluoxetine, also have been used to treat the pain of diabetic neuropathy, and although better tolerated than tricyclic antidepressants, they seem to be less efficacious. If the antidepressant compounds are ineffective or contraindicated, gabapentin represents a reasonable alternative. Gabapentin should be started with a 300-mg dose at bedtime for 2 nights. The patient should be cautioned about potential side effects, including dizziness, sedation, confusion, and rash. The drug is increased in 300-mg increments, given in equally divided doses over 2 days, as side effects allow, until pain relief is obtained or a total dose of 2400 mg daily is reached. At this point, if the patient has experienced partial pain relief, blood values are measured and the drug is carefully titrated upward using 100-mg tablets. Rarely is more than 3600 mg daily required. Pregabalin may be a consideration if gabapentin

is ineffective. Intravenous infusions of lidocaine and topical lidocaine patches have been reported to provide symptomatic relief of postmastectomy pain. Spinal cord stimulation may also provide symptomatic relief of postmastectomy pain. Local application of heat and cold, as well as topical capsaicin, also may be beneficial to provide symptomatic relief of the pain of postmastectomy syndrome. The use of an elastic rib belt may help provide symptomatic relief. Hypnosis may provide adjunctive relief of pain. For patients who do not respond to these treatment modalities, injection of the affected intercostal nerves or thoracic epidural nerve block using local anesthetic and steroid may be a reasonable next step.

Complications and Pitfalls The major problem in the care of patients thought to have postmastectomy syndrome is the failure to identify potentially serious pathological conditions of the thorax or upper abdomen secondary to metastatic disease or invasion of the chest wall and thorax by tumor. Given the proximity of the pleural space, pneumothorax after intercostal nerve block is a distinct possibility. The incidence of the complication is less than 1%, but it occurs with greater frequency in patients with chronic obstructive pulmonary disease. Although uncommon, infection is an ever-present possibility, especially in an immunocompromised patient with cancer. Early detection of infection is crucial to avoid potentially life-threatening sequelae.

Clinical Pearls Postmastectomy syndrome is a cause of chest wall and thoracic pain that should not be overlooked in patients after breast surgery. Correct diagnosis is necessary to treat this painful condition properly and to avoid overlooking serious intrathoracic or intra-abdominal pathological processes. The use of the pharmacological agents mentioned, including gabapentin, allows the clinician to control the pain of postmastectomy syndrome adequately. Intercostal nerve block is a simple technique that can produce dramatic relief for patients with postmastectomy syndrome. As mentioned, the proximity of the intercostal nerve to the pleural space makes careful attention to technique mandatory. Recent research has identified genetic and epigenetic factors that may predispose patients to the development of postmastectomy pain.

Suggested Readings Berlière M, Roelants F, Watremez C, et al. The advantages of hypnosis intervention on breast cancer surgery and adjuvant therapy. Breast. 2018;37:114–118. Björkman B, Arnár S, Hydén L.-C. Phantom breast and other syndromes after mastectomy: eight breast cancer patients describe their experiences over time—a 2-year follow-up study. J Pain. 2008;9:1018–1025. Chang S.H, Mehta V, Langford R.M. Acute and chronic pain following breast surgery. Acute Pain. 2009;11:1–14. Hetta D.F, Mohamed M.A, Mohammad M.F. Analgesic efficacy of pregabalin in acute postmastectomy pain: placebo controlled dose ranging study. J Clin Anesthesia. 2016;34:303–309. Katz J, Poleshuck E.L, Andrus C.H, et al. Risk factors for acute pain and its persistence following breast cancer surgery. Pain. 2005;119:16–25. Kumar A, Bullock W.M, Dooley J. Use of local anesthetic a key tenet in multimodal analgesia to modulate chronic post-mastectomy pain. J Clin Anesthesia. 2017;38:26. Stephens K.E, Levine J.D, Aouizerat B.E, et al. Associations between genetic and epigenetic variations in cytokine genes and mild persistent breast pain in women following breast cancer surgery. Cytokine. 2017;99:203–213. Watson C.P, Evans R.J, Watt V.R. The post-mastectomy pain syndrome and the effect of topical capsaicin. Pain. 1989;38:177–186. Wisotzky E, Hanrahan N, Lione T.P, Maltser S. Deconstructing postmastectomy syndrome: implications for physiatric management. Phy Med Rehabil Clin N Am. 2017;28(1):153–169.

68

Sternalis Syndrome

Abstract Sternalis syndrome is an infrequent cause of anterior chest wall pain. Sternalis is a constellation of symptoms affecting the midline anterior chest wall that can radiate to the retrosternal area and the medial aspect of the arm. Sternalis syndrome can mimic the pain of myocardial infarction and is frequently misdiagnosed as such. Sternalis syndrome is a myofascial pain syndrome and is characterized by trigger points in the midsternal area. In contrast to costosternal syndrome, which also manifests as midsternal pain, the pain of sternalis syndrome is not exacerbated by movement of the chest wall and shoulder.

Keywords accessory muscle; anomalous muscle; chest wall pain; diagnostic ultrasound; magnetic resonance imaging; midsternal pain; myocardial infarction; sternalis syndrome; ultrasound guided injection

ICD-10 CODE R07.1

The Clinical Syndrome Chest wall pain syndromes are commonly encountered in clinical practice. Some occur with relatively greater frequency and are more readily identified by the clinician, such as costochondritis and Tietze syndrome. Others occur so infrequently that they are often misdiagnosed, resulting in a suboptimal outcome. Sternalis syndrome is one such infrequent cause of anterior chest wall pain. Sternalis is a constellation of symptoms affecting the midline anterior chest wall that can radiate to the retrosternal area and the medial aspect of the arm. Sternalis syndrome can mimic the pain of myocardial infarction and is frequently misdiagnosed as such. Sternalis syndrome is a myofascial pain syndrome and is characterized by trigger points in the midsternal area. In contrast to costosternal syndrome, which also manifests as midsternal pain, the pain of sternalis syndrome is not exacerbated by movement of the chest wall and shoulder. The intensity of the pain associated with sternalis syndrome is mild to moderate and described as having a deep, aching character. The pain of sternalis syndrome is intermittent.

Signs and Symptoms On physical examination, a patient with sternalis syndrome exhibits myofascial trigger points at the midline over the sternum (Fig. 68.1). Occasionally, a coexistent trigger point is located in the pectoralis muscle or sternal head of the sternocleidomastoid muscle. Pain is reproduced with palpation of these trigger points, rather than movement of the chest wall and shoulders. A positive jump sign is present when these trigger points are stimulated. Trigger points at the lateral border of the scapula also may be present and amenable to injection therapy. As mentioned, movement of the shoulders and chest wall does not exacerbate the pain.

Testing Plain radiographs are indicated in all patients thought to have sternalis syndrome to rule out occult bony pathological processes, including metastatic lesions. Based on the patient’s clinical presentation, additional tests, including complete blood cell count, prostate-specific antigen level, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. Computed tomography (CT) and magnetic resonance imaging (MRI) of the chest are indicated if a retrosternal mass, such as thymoma, is suspected, as well as to help confirm the presence of a sternalis muscle or other anterior chest wall mass (Fig. 68.2 and68.3). Ultrasound imaging may help further characterize the nature of masses involving the anterior chest wall. Electromyography is indicated in patients with sternalis syndrome to help rule out cervical radiculopathy or plexopathy that may be considered because of the referred arm pain. Injection of the sternalis muscle with a local anesthetic and steroid serves as a diagnostic and therapeutic maneuver.

Differential Diagnosis As mentioned earlier, the pain of sternalis syndrome is often mistaken for pain of cardiac origin and can lead to visits to the emergency department and unnecessary cardiac workups. If trauma has occurred, sternalis syndrome may coexist with fractured ribs or fractures of the sternum itself, which can be missed on plain radiographs and may require radionucleotide bone scanning for proper identification. Tietze syndrome, which is painful enlargement of the upper costochondral cartilage associated with viral infections, can be confused with sternalis syndrome, as can costosternal syndrome and congenital abnormalities of the sternum (Fig. 68.4). Neuropathic pain involving the chest wall also may be confused or coexist with costosternal syndrome. Examples of such neuropathic pain include diabetic polyneuropathies and acute herpes zoster involving the thoracic nerves. The possibility of diseases of the structures of the anterior chest wall and mediastinum is ever present, and these diseases sometimes can be difficult to diagnose. Pathological processes that inflame the pleura, such as pulmonary embolus, infection, and tumor, also should be considered.

Treatment Initial treatment of sternalis syndrome should include a combination of simple analgesics and nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors. Local application of heat and cold also may be beneficial to provide symptomatic relief of the pain of sternalis syndrome. The use of an elastic rib belt may help provide symptomatic relief in some patients. For patients who do not respond to these treatment modalities, injection of the trigger areas located in the sternalis muscle using a local anesthetic and steroid may be a reasonable next step. Ultrasound guidance may improve the accuracy of needle placement and decrease the incidence of needle-related complications.

FIG. 68.1 Patients with sternalis syndrome exhibit myofascial trigger points at the midline over the sternum.

FIG. 68.2 Left sternalis muscle is incidentally discovered anterior to the left pectoralis major muscle in this young adult man. From Alpert JB, Naidich DP. Imaging of incidental findings on thoracic computed tomography. Radiol Clin North Am. 2011;49:267–289.

FIG. 68.3 Well-encapsulated, fat density lesion associated with the left serratus anterior muscle is consistent with chest wall lipoma, the most common benign chest wall neoplasm. From Alpert JB, Naidich DP. Imaging of incidental findings on thoracic computed tomography. Radiol Clin North Am. 2011;49:267–289.

Complications and Pitfalls The major problem in the care of patients thought to have sternalis syndrome is the failure to identify potentially serious pathological conditions of the thorax, mediastinum, or both. Given the proximity of the pleural space, pneumothorax after injection of the sternalis muscle is a possibility, as is injury to the mediastinal and intrathoracic structures. Approximately 25% of patients report a transient increase in pain after this injection technique and should be warned about this.

Clinical Pearls Patients with sternalis syndrome often present to the emergency department, fearing they are having a heart attack. The syndrome also is misdiagnosed frequently as cervical radiculopathy secondary to the referred arm pain. Electromyography helps delineate the cause and extent of neural compromise. The injection technique is extremely effective in the treatment of sternalis syndrome. Coexistent costosternal or manubriosternal arthritis also may contribute to anterior chest wall pain and may require additional treatment with a more localized injection of a local anesthetic and depot steroid. This technique is a safe procedure if careful attention is paid to the clinically relevant anatomy in the areas to be injected. Pneumothorax can be avoided if shorter needles are used and the needle is not advanced too deeply. Care must be taken to use sterile technique to avoid infection, and universal precautions should be taken to avoid risk to the operator. The incidence of ecchymosis and hematoma formation can be decreased if pressure is placed on the injection site immediately after injection. The use of physical modalities, including local heat and gentle range-of-motion exercises, should be introduced several days after the patient undergoes this injection technique for shoulder pain. Vigorous exercises should be avoided because they would exacerbate symptoms. Simple analgesics and NSAIDs may be used concurrently with this injection technique.

FIG. 68.4 A 21-year-old woman with bifid sternum. Findings: volume rendering reconstruction of noncontrast chest computed tomography (CT). (A and B) Two views of cleft of the upper part of the sternum with “U-shape” variant. The superior sternal cleft may be “V-shaped” when the cleft reaches the xiphoid process, or “Ushaped,” with a bony bridge joining the two edges ending at the third or fourth costal cartilages, as seen in this case. From Manenti G, Bozzi A, Ferrazzoli V, et al. Bifid sternum in a young woman: multimodality imaging features. Radiol Case Rep. 2017;12[4]:672–677.

Suggested Readings Alpert J.B, Naidich D.P. Imaging of incidental findings on thoracic computed tomography. Radiol Clin North Am. 2011;49:267–289. Ayloo A, Cvengros T, Marella S. Evaluation and treatment of musculoskeletal chest pain. Prim Care. 2013;40(4):863–887. Baldry P. The chest wall. In: Baldry P, ed. Myofascial Pain Fibromyalgia Syndromes. London: Churchill Livingstone; 2001:303–327. Baldry P.E, Thompson J.W, eds. Acupuncture, Trigger Points and Musculoskeletal Pain. 3rd ed. London: Churchill Livingstone; 2005:165–185. Bennett R. Myofascial pain syndromes and their evaluation. Best Pract Res Clin Rheumatol. 2007;21:427–445. Bergeron E.J, Meguid R.A, Mitchell J.D. Chronic infections of the chest wall. Thorac Surg Clin. 2017;27(2):87–97. Manenti G, Bozzi A, Ferrazzoli V, et al. Bifid sternum in a young woman: multimodality imaging features. Radiol Case Rep. 2017;12(4):672–677. Shah J.P, Thaker N, Heimur J, Aredo J.V, et al. Myofascial trigger points then and now: a historical and scientific perspective. PM R. 2015;7(7):746–761.

69

Manubriosternal Joint Pain ICD-10 CODE R07.1

The Clinical Syndrome The manubriosternal joint can serve as a source of pain that often may mimic pain of cardiac origin. The manubriosternal joint is susceptible to the development of arthritis, including osteoarthritis, rheumatoid arthritis, ankylosing spondylitis, Reiter syndrome, and psoriatic arthritis (Fig. 69.1). The joint is often traumatized during acceleration/deceleration injuries and blunt trauma to the chest. With severe trauma, the joint may sublux or dislocate (Fig. 69.2). Overuse or misuse can result in acute inflammation of the manubriosternal joint, which can be quite debilitating. The joint is subject to invasion by tumor from primary malignancies, including thymoma, metastatic disease, and infection (Fig. 69.3).

Signs and Symptoms On physical examination, the physical deformity of joint subluxation after traumatic injury may be obvious on inspection (Fig. 69.4). The patient vigorously attempts to splint the joint by keeping the shoulders stiffly in neutral position. Pain is reproduced by active protraction or retraction of the shoulder, deep inspiration, and full elevation of the arm. Shrugging of the shoulder also may reproduce the pain. The manubriosternal joint may be tender to palpation and feel hot and swollen if acutely inflamed or infected. The patient may report a “clicking” sensation with movement of the joint.

Testing Plain radiographs are indicated for all patients who present with pain thought to be emanating from the manubriosternal joint to rule out occult bony pathological processes, including tumor. Based on the patient’s clinical presentation, additional testing may be indicated, including complete blood count, prostate-specific antigen level, erythrocyte sedimentation rate, and antinuclear antibody testing. Computed tomography (CT), ultrasound imaging, or magnetic resonance imaging (MRI) of the joint is indicated if infection, tumor, or joint instability is suspected (Fig. 69.5 and 69.6). Injection of the manubriosternal joint with local anesthetic and steroid serves as a diagnostic maneuver and a therapeutic maneuver. Ultrasound guidance may improve the accuracy of needle placement and decrease the incidence of needle-related complications.

Differential Diagnosis As mentioned earlier, manubriosternal joint pain is often mistaken for cardiac pain. A careful search for metastatic disease or tumor invasion of the chest wall is mandatory in all patients with manubriosternal joint pain, because this pain may coexist with pathological rib fractures or pathological fractures of the sternum itself, which can be missed on plain radiographs and may require radionucleotide bone scanning for proper identification.

FIG. 69.1 Abnormalities of the Manubriosternal JointRadiographic abnormalities of the manubriosternal joint are illustrated in this coronal section of the sternum. They include osseous erosions and sclerosis. Note the irregularity of the costosternal joints (arrow). From Resnick D, ed. Diagnosis of Bone and Joint Disorders. 4th ed. Philadelphia: Saunders; 2002:924.

FIG. 69.2 With severe trauma, the manubriosternal joint may sublux or dislocate.

FIG. 69.3 (A) Obvious soft-tissue swelling (arrow) of the anterior chest wall, before surgical intervention. The patient lies faced up on the surgery table (head on the right side of the picture, lateral view). (B) Transverse incision demonstrating a disruption of the manubriosternal joint. From Carnevale A, Righi R, Maniscalco P, et al. Primary septic arthritis of the manubriosternal joint in an immunocompetent young patient: a case report. Radiol Case Rep. 2017;12[4]:682–685.

Neuropathic pain involving the chest wall and sternum also may be confused or coexist with manubriosternal joint pain. Examples of such neuropathic pain include diabetic polyneuropathies and acute herpes zoster involving the thoracic nerves. The possibility of diseases of the structures of the mediastinum is ever present, and these diseases can be sometimes difficult to diagnose. Pathological processes that inflame the pleura, such as pulmonary embolus, infection, and Bornholm disease, also may mimic the pain emanating from the manubriosternal joint.

Treatment Initial treatment of manubriosternal joint pain should include a combination of simple analgesics and nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors. If these medications do not control the patient’s symptoms adequately, or if considerable sleep disturbance exists, a tricyclic antidepressant should be added.

FIG. 69.4 Obvious Step in Manubriosternal Joint From Lyons I, Saha S, Arulampalam T. Manubriosternal joint dislocation: an unusual risk of trampolining. J Emerg Med. 2010;39:596–598.

FIG. 69.5 Lateral Chest CT Scan Showing Posterior Dislocation of the Sternum From Lyons I, Saha S, Arulampalam T. Manubriosternal joint dislocation: an unusual risk of trampolining. J Emerg Med. 2010;39:596–598.

Traditionally, tricyclic antidepressants have been a mainstay in the palliation of sleep disturbance associated with painful conditions. Controlled studies have shown the efficacy of amitriptyline for this indication. Other tricyclic antidepressants, including nortriptyline and desipramine, also have been shown to be clinically useful. This class of drugs is associated with considerable anticholinergic side effects, including dry mouth, constipation, sedation, and urinary retention. These drugs should be used with caution in patients with glaucoma, cardiac arrhythmia, and prostatism. To minimize side effects and encourage compliance, the primary care physician should start amitriptyline or nortriptyline at a 10-mg dose at bedtime. The dose can be titrated upward to 25 mg at bedtime as side effects allow. Upward titration of dosage in 25-mg increments can be done each week as side effects allow. Even at lower doses, patients generally report a rapid improvement in sleep disturbance and begin to experience pain relief in 10 to 14 days. Selective serotonin reuptake inhibitors, such as fluoxetine, also have been used to treat the pain of diabetic neuropathy, and although better tolerated than tricyclic

antidepressants, they seem to be less efficacious than the tricyclic antidepressants.

FIG. 69.6 Soft-tissue ultrasonography, longitudinal plane, demonstrating a lobulated heterogeneously hypoechoic mass (straight arrows) over the manubriosternal joint (curved arrow). From Carnevale A, Righi R, Maniscalco P, et al. Primary septic arthritis of the manubriosternal joint in an immunocompetent young patient: a case report. Radiol Case Rep. 2017;12[4]:682–685.

Local application of heat and cold may be beneficial to provide symptomatic relief of the pain of manubriosternal joint pain. The use of an elastic rib belt may help provide symptomatic relief. For patients who do not respond to these treatment modalities, injection of the manubriosternal joint using local anesthetic and steroid may be a reasonable next step.

Complications and Pitfalls The major problem in the care of patients thought to have manubriosternal pain is the failure to identify potentially serious pathology of the thorax or upper abdomen secondary to metastatic disease or invasion of the chest wall and thorax by tumor. Given the proximity of the pleural space, pneumothorax after injection of the manubriosternal joint is a possibility. The incidence of the complication is less than 1%, but it occurs with greater frequency in patients with chronic obstructive pulmonary disease. Although uncommon, infection is an ever-present possibility, especially in an immunocompromised patient with cancer. Early detection of infection is crucial to avoid potentially life-threatening sequelae.

Clinical Pearls Patients with pain emanating from the manubriosternal joint often attribute their pain symptoms to a heart attack. Reassurance is required, although it should be remembered that this musculoskeletal pain syndrome and coronary artery disease can coexist. Care must be taken to use sterile technique to avoid infection and universal precautions to avoid risk to the operator. The incidence of ecchymosis and hematoma formation can be decreased if pressure is placed on the injection site immediately after injection. The use of physical modalities, including local heat and gentle range-of-motion exercises, should be introduced several days after the patient undergoes this injection technique for manubriosternal joint pain. Vigorous exercise should be avoided because it exacerbates the symptoms. Simple analgesics and NSAIDs may be used concurrently with this injection technique. Laboratory evaluation for collagen-vascular disease is indicated for patients who have manubriosternal joint pain with other joints involved.

Suggested Readings Al-Dahiri A, Pallister I. Arthrodesis for osteoarthritis of the manubriosternal joint. Eur J Cardiothorac Surg. 2006;29:119–121. Carnevale A, Righi R, Maniscalco P, et al. Primary septic arthritis of the manubriosternal joint in an immunocompetent young patient: a case report. Radiol Case Rep. 2017;12(4):682–685. da Silva Herrero C.F.P, Porto M.A, Nogueira-Barbosa M.H. Defino HLA: occult manubriosternal joint injury associated with fracture of the thoracic spine. Revista Brasileira de Ortopedia (English Edition). 2011;46(2):211–214. Ellis H. The superior mediastinum. Anaesth Intens Care Med. 2009;10:360–361. Gorospe L, Ayala-Caronero A.M, Rodríguez-Díaz R, et al. Tuberculosis of the manubriosternal joint and concurrent asymptomatic active pulmonary tuberculosis in a patient presenting with a chest wall mass. Clin Imaging. 2015;39(2):311–314. Lyons I, Saha S, Arulampalam T. Manubriosternal joint dislocation: an unusual risk of trampolining. J Emerg Med. 2010;39:596–598. Stochkendahl M.J, Christensen H.W. Chest pain in focal musculoskeletal disorders. Med Clin North Am. 2010;94:259–273. Waldman S.D. Manubriosternal joint syndrome. In: Waldman S.D, ed. Pain Review. 2nd ed. Philadelphia: Elsevier; 2016:231–232.

70

Xiphodynia Keywords anterior chest wall pain; computed tomography; diagnostic ultrasound; magnetic resonance imaging; septic arthritis; ultrasound guided injection; xiphodynia; xiphoid process

ICD-10 CODE M94.9

The Clinical Syndrome An uncommon cause of anterior chest wall pain, xiphodynia is often misdiagnosed as pain of cardiac or upper abdominal origin. Xiphodynia syndrome is a constellation of symptoms consisting of severe intermittent anterior chest wall pain in the region of the xiphoid process that worsens with overeating, stooping, and bending. The patient may report a nauseated feeling associated with the pain of xiphodynia syndrome. This xiphisternal joint seems to serve as the nidus of pain for xiphodynia syndrome. The xiphisternal joint is often traumatized during acceleration/deceleration injuries and blunt trauma to the chest. With severe trauma, the joint may sublux or dislocate. The xiphisternal joint also is susceptible to the development of arthritis, including osteoarthritis, rheumatoid arthritis, ankylosing spondylitis, Reiter syndrome, and psoriatic arthritis. The joint is subject to invasion by tumor from either primary malignancies, including thymoma or metastatic disease.

Signs and Symptoms Physical examination reveals that the pain of xiphodynia syndrome is reproduced with palpation or traction on the xiphoid. The xiphisternal joint may feel swollen (Fig. 70.1). Stooping or bending may reproduce the pain. Coughing may be difficult, and this may lead to inadequate pulmonary toilet in patients who have sustained trauma to the anterior chest wall. The xiphisternal joint and adjacent intercostal muscles also may be tender to palpation. The patient may report a clicking sensation with movement of the joint. Furthermore, patients with a prominent xiphoid process on visual inspection indicating an xiphisternal angle less than 160 degrees are more prone to the development of xiphodynia (Fig. 70.2).

Testing Plain radiographs are indicated in all patients with pain thought to be emanating from the xiphisternal joint to rule out occult bony pathological conditions, including tumor. Based on the patient’s clinical presentation, additional tests, including complete blood cell count, prostate-specific antigen level, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. Computed tomography (CT), ultrasound imaging, or magnetic resonance imaging (MRI) of the joint is indicated if joint instability or an occult mass is suspected (Figs 70.3 and 70.4). The following injection technique serves as a diagnostic and therapeutic maneuver.

FIG. 70.1 The Xiphisternal Joint is Swollen in Patients with Xiphodynia.

FIG. 70.2 Visible Prominence of the Xyphoid Process From Maigne J-Y, Vareli M, Rousset P, et al. Xiphodynia and prominence of the xyphoid process: value of xiphosternal angle measurement—three case reports. Joint Bone Spine. 2010;77:474–476.

FIG. 70.3 The xiphisternal angle was 105 degrees. The curved shape of the xyphoid process hindered the measurement of the angle. From Maigne J-Y, Vareli M, Rousset P, et al. Xiphodynia and prominence of the xyphoid process: value of xiphosternal angle measurement—three case reports. Joint Bone Spine. 2010;77:474–476.

Differential Diagnosis As with costochondritis, costosternal joint pain, devil’s grip, Tietze syndrome, and rib fractures, many patients with xiphodynia first seek medical attention because they believe they are having a heart attack. Patients also may believe they have ulcer or gallbladder disease. In contrast to most other causes of pain involving the chest wall that are musculoskeletal or neuropathic in origin, the pain of devil’s grip results from infection. The constitutional symptoms associated with devil’s grip may lead the clinician to consider pneumonia, empyema, and occasionally pulmonary embolus as the most likely diagnosis.

FIG. 70.4 Ultrasound image of the xiphisternal joint and a patient with anterior chest wall pain following a motor vehicle accident. Note the fracture of the sternal body.

Treatment Initial treatment of xiphodynia should include a combination of simple analgesics and nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors. If these medications do not control the patient’s symptoms adequately, opioid analgesics may be added during the period of acute pain. Local application of heat and cold may be beneficial to provide symptomatic relief of the pain of xiphodynia. The use of an elastic rib belt may help provide symptomatic relief in some patients. For patients who do not respond to these treatment modalities, the injection of the xiphisternal joint using a local anesthetic and steroid may be a reasonable next step.

Complications and Pitfalls The major problem in the care of patients thought to have xiphodynia is the failure to identify potentially serious pathology of the thorax or upper abdomen. The major complication of injection of the xiphisternal joint is pneumothorax if the needle is placed too laterally or deeply and invades the pleural space. Infection, although rare, can occur if strict aseptic technique is not followed. Trauma to the contents of the mediastinum is an ever-present possibility. This complication can be greatly decreased if the clinician pays close attention to accurate needle placement.

Clinical Pearls Patients with pain emanating from the xiphisternal joint often attribute their pain symptoms to a heart attack or ulcer disease. Reassurance is required, although it should be remembered that this musculoskeletal pain syndrome, ulcer disease, and coronary artery disease can coexist. The xiphoid process articulates with the sternum via the xiphisternal joint. The xiphoid process is a plate of cartilaginous bone that becomes calcified in early adulthood. The xiphisternal joint is strengthened by ligaments, but it can be subluxed or dislocated by blunt trauma to the anterior chest. The xiphisternal joint is innervated by the T4-7 intercostal nerves and the phrenic nerve. It is thought that this innervation by the phrenic nerve is responsible for the referred pain associated with xiphodynia syndrome. Tietze syndrome, which is painful enlargement of the upper costochondral cartilage associated with viral infections, can be confused with xiphisternal syndrome, although both respond to the injection technique described. The use of physical modalities, including local heat and gentle range-ofmotion exercises, should be introduced several days after the patient undergoes this injection technique for xiphisternal joint pain. Vigorous exercises should be avoided because they would exacerbate the patient’s symptoms. Simple analgesics and NSAIDs may be used concurrently with this injection technique. Laboratory evaluation for collagen-vascular disease is indicated in patients with xiphisternal joint pain in whom other joints are involved.

Suggested Readings Howell J. Xiphodynia: an uncommon cause of exertional chest pain. Am J Emerg Med. 1990;8:176. Howell J.M. Xiphodynia: a report of three cases. J Emerg Med. 1992;10:435–438. Jelenko III. C, Cowan Jr. G.S.M. Perichondritis (Tietze’s syndrome) at the xiphisternal joint: a mimic of severe disease. J Am Coll Emerg Physicians. 1977;6:536–542. Koren W, Shahar A. Xiphodynia masking acute myocardial infarction: a diagnostic cul-de-sac. Am J Emerg Med. 1998;16:177–178. Stochkendahl M.J, Christensen H.W. Chest pain in focal musculoskeletal disorders. Med Clin North Am. 2010;94:259–273. Waldman S.D. Xiphisternal joint injection. In: Waldman S.D, ed. Atlas of Pain Management Injection Techniques. 4th ed. Philadelphia: Elsevier; 2017:360– 362.

71

Serratus Anterior Muscle Syndrome ICD-10 CODE M79.7

The Clinical Syndrome Chest wall pain syndromes are commonly encountered in clinical practice. Some occur with relatively greater frequency and are more readily identified by the clinician, such as costochondritis and Tietze syndrome. Others occur so infrequently that they are often misdiagnosed, resulting in a less-thanoptimal outcome. Serratus anterior muscle syndrome is one such infrequent cause of anterior chest wall pain. The syndrome is a constellation of symptoms consisting of pain overlying the fifth to the seventh ribs in the midaxillary line, with referred pain that may radiate down the ipsilateral upper extremity into the palmar aspect of the ring and little finger. Serratus anterior muscle syndrome can mimic the pain of myocardial infarction and is frequently misdiagnosed as such. It is a myofascial pain syndrome. The intensity of the pain associated with serratus anterior muscle syndrome is mild to moderate and is described as having a deep, aching character. The pain of serratus anterior muscle syndrome is intermittent.

Signs and Symptoms On physical examination, the patient with serratus anterior muscle syndrome will exhibit myofascial trigger points overlying the fifth to seventh ribs in the midaxillary line, with referred pain that may radiate down the ipsilateral upper extremity into the palmar aspect of the ring and little fingers (Fig. 71.1). Pain is reproduced with palpation of these trigger points rather than with movement of the chest wall and shoulders. A positive jump sign will be present when these trigger points are stimulated. Trigger points at the lateral border of the scapula may be present and amenable to injection therapy. As mentioned, movement of the shoulders and chest wall will not exacerbate the pain.

Testing Plain radiographs are indicated in all patients with suspected serratus anterior muscle syndrome to rule out occult bony pathological processes, including metastatic lesions. Based on the patient’s clinical presentation, additional testing may be indicated, including complete blood cell count, prostate-specific antigen level, sedimentation rate, and antinuclear antibody testing. Magnetic resonance imaging (MRI) of the chest is indicated if a retrosternal mass such as thymoma is suspected or if trauma to the serratus anterior muscle itself has occurred (Fig. 71.2). Electromyography is indicated in patients with serratus anterior muscle syndrome to help rule out cervical radiculopathy or plexopathy that may be considered because of the referred arm pain. Injection of the serratus anterior muscle with a local anesthetic and steroid serves as both a diagnostic and therapeutic maneuver.

Differential Diagnosis As mentioned, the pain of serratus anterior muscle syndrome is often mistaken for pain of cardiac origin and can lead to visits to the emergency department and unnecessary cardiac workups. If trauma has occurred, serratus anterior muscle syndrome may coexist with fractured ribs or fractures of the sternum itself, which can be missed on plain radiographs and may require radionucleotide bone scanning for proper identification. Tietze syndrome, which is painful enlargement of the upper costochondral cartilage associated with viral infections, can be confused with sternalis syndrome, as can costosternal syndrome. Neuropathic pain involving the chest wall may be confused or coexist with costosternal syndrome. Examples of such neuropathic pain include diabetic polyneuropathies and acute herpes zoster involving the thoracic nerves. The possibility of diseases of the structures of the mediastinum remains ever present and at times can be difficult to diagnose. Pathological processes that inflame the pleura, such as pulmonary embolus, infection, and tumor, also should be considered.

Treatment Initial treatment of serratus anterior muscle syndrome should include a combination of simple analgesics and the nonsteroidal antiinflammatory agents or the cyclooxygenase-2 (COX-2) inhibitors. The local application of heat and cold may be beneficial to provide symptomatic relief of the pain of serratus anterior muscle syndrome. The use of an elastic rib belt may help provide symptomatic relief in some patients. For patients who do not respond to these treatment modalities, injection of the trigger areas located in the sternalis muscle using a local anesthetic and steroid may be a reasonable next step.

FIG. 71.1 Serratus anterior muscle syndrome is a constellation of symptoms consisting of anterior chest wall pain that can radiate to the retrosternal area and the medial aspect of the arm.

FIG. 71.2 T2-weighted magnetic resonance image showing the hematoma (∗) in the right chest wall after traumatic avulsion of the serratus anterior muscle. (A) Axial view. (B) Coronal view. From Otoshi K, Itoh Y, Tsujino A, et al. Avulsion injury of the serratus anterior muscle in a high-school underhand pitcher: a case report. J Shoulder Elbow Surg. 2007;16:e45–e47.

Complications and Pitfalls The major problem in the care of patients thought to have serratus anterior muscle syndrome is the failure to identify potentially serious pathological conditions of the thorax or mediastinum. Given the proximity of the pleural space, pneumothorax after injection of the serratus anterior muscle is a distinct possibility, as is injury to the mediastinal and intrathoracic structures. Approximately 25% of patients will report a transient increase in pain after this injection technique and should be warned of this.

Clinical Pearls Patients with serratus anterior muscle syndrome will often go the emergency department, fearing they are having a heart attack. The syndrome is also frequently misdiagnosed as a cervical radiculopathy because of the referred arm pain. Electromyography will help delineate the cause and extent of neural compromise. This injection technique is extremely effective in the treatment of serratus anterior muscle syndrome. Coexistent costosternal or manubriosternal arthritis may contribute to anterior chest wall pain and may require additional treatment with a more localized injection of an anesthetic and depot steroid. This technique is a safe procedure if careful attention is paid to the clinically relevant anatomy in the areas to be injected. Pneumothorax can be avoided if shorter needles are used and the needle is not advanced too deeply. Care must be taken to use sterile technique to avoid infection, and universal precautions must be used to avoid risk to the operator. The incidence of ecchymosis and hematoma formation can be decreased if pressure is placed on the injection site immediately after injection. The use of physical modalities, including local heat and gentle range-of-motion exercises, should be introduced several days after the patient undergoes this injection technique for shoulder pain. Vigorous exercises should be avoided because they will exacerbate the symptoms. Simple analgesics and nonsteroidal anti inflammatory agents may be used concurrently with this injection technique.

Suggested Readings Al-Qadi M.O. Disorders of the chest wall: clinical manifestations. Clin Chest Med. 2018;39(2):361–375. Carter B.W, Gladish G.W. MR imaging of chest wall tumors. Magn Reson Imaging Clin N Am. 2015;23(2):197–215. Häuser W, Perrot S, Clauw J.D, Fitzcharles M.-A. Unravelling fibromyalgia— steps toward individualized management. J Pain. 2018;19(2):125–134. Higgs J.B. Fibromyalgia in primary care. Prim Care. 2018;45(2):325–341. Rushton S, Carman M.J. Chest pain: if it is not the heart, what is it? Nurs Clin North Am. 2018;53(3):421–431.

72

Slipping Rib Syndrome ICD-10 CODE R07.82

The Clinical Syndrome Encountered more frequently in clinical practice since the increased use of across-the-chest seat belts and airbags, slipping rib syndrome is often misdiagnosed, leading to prolonged suffering and excessive testing for intraabdominal and intrathoracic pathological conditions. Slipping rib syndrome is a constellation of symptoms consisting of severe knife-like pain emanating from the lower costal cartilages associated with hypermobility of the anterior end of the lower costal cartilages. The tenth rib is most commonly involved, but the eighth and ninth ribs also can be affected. This syndrome is also known as the rib-tip syndrome. Slipping rib syndrome is almost always associated with trauma to the costal cartilage of the lower ribs. These cartilages are often traumatized during acceleration/deceleration injuries and blunt trauma to the chest. With severe trauma, the cartilage may sublux or dislocate from the ribs. Patients with slipping rib syndrome may report a clicking sensation with movement of the affected ribs and associated cartilage.

Signs and Symptoms On physical examination, the patient vigorously attempts to splint the affected costal cartilage joints by keeping the thoracolumbar spine slightly flexed (Fig. 72.1). Pain is reproduced with pressure on the affected costal cartilage. Patients with slipping rib syndrome exhibit a positive hooking maneuver test. The hooking maneuver test is performed by having the patient lie in the supine position with the abdominal muscles relaxed while the clinician hooks his or her fingers under the lower rib cage and pulls gently outward. Pain and a clicking or snapping sensation of the affected ribs and cartilage indicate a positive test.

Testing Plain radiographs are indicated in all patients who present with pain thought to be emanating from the lower costal cartilage and ribs to rule out occult bony pathological processes, including rib fracture and tumor. Based on the patient’s clinical presentation, additional tests, including complete blood cell count, prostate-specific antigen level, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. Magnetic resonance imaging (MRI) of the affected ribs and cartilage is indicated if joint instability or occult mass is suspected. The injection technique discussed in this chapter serves as a diagnostic and therapeutic maneuver.

Differential Diagnosis As mentioned earlier, the pain of slipping rib syndrome is often mistaken for pain of cardiac or gallbladder origin and can lead to visits to the emergency department and unnecessary cardiac and gastrointestinal workups. If trauma has occurred, slipping rib syndrome may coexist with rib fractures or fractures of the sternum, which can be missed on plain radiographs and may require radionucleotide bone scanning for proper identification. Tietze syndrome, which is painful enlargement of the upper costochondral cartilage associated with viral infections, can be confused with slipping rib syndrome, as can devil’s grip, which is a pleura-based pain syndrome of infectious origin.

FIG. 72.1 Patients with slipping rib syndrome exhibit pain on “hooking” of the affected costochondral cartilage.

Neuropathic pain involving the chest wall also may be confused or coexist

with slipping rib syndrome. Examples of such neuropathic pain include diabetic polyneuropathies and acute herpes zoster involving the thoracic nerves. The possibility of diseases of the structures of the mediastinum is ever present, and these diseases sometimes can be difficult to diagnose. Pathological processes that inflame the pleura, such as pulmonary embolus, infection, and tumor, also should be considered.

Treatment Initial treatment of the pain and functional disability associated with slipping rib syndrome should include a combination of nonsteroidal anti inflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and physical therapy. The local application of heat and cold may be beneficial. The repetitive movements that incite the syndrome should be avoided. For patients who do not respond to these treatment modalities, injection of the affected costochondral cartilages with a local anesthetic and steroid may be a reasonable next step. To inject the slipping ribs, the patient is placed in the supine position, and proper preparation with antiseptic solution of the skin overlying the affected costal cartilage and rib is done. A sterile syringe containing 1 mL of 0.25% preservative-free bupivacaine for each joint to be injected and 40 mg of methylprednisolone is attached to a 25-gauge, 1½-inch needle using strict aseptic technique. With strict aseptic technique, the distal rib and costal cartilage are identified. The lower margin of each affected distal rib is identified and marked with a sterile marker. The needle is carefully advanced at the point marked through the skin and subcutaneous tissues until the needle tip impinges on the periosteum of the underlying rib. The needle is withdrawn back into the subcutaneous tissues and walked inferiorly off the inferior rib margin. The needle should be advanced just beyond the inferior rib margin, but no farther, or pneumothorax or damage to the abdominal viscera could result. After careful aspiration to ensure that the needle tip is not in an intercostal vein or artery, 1 mL of solution is gently injected. There should be limited resistance to injection. If significant resistance is encountered, the needle should be withdrawn slightly until the injection proceeds with only limited resistance. This procedure is repeated for each affected rib and associated cartilage. The needle is removed, and a sterile pressure dressing and ice pack are placed at the injection site.

Complications and Pitfalls The major problem in the care of patients thought to have slipping rib syndrome is the failure to identify potentially serious pathological conditions of the thorax or upper abdomen. Given the proximity of the pleural space, pneumothorax after the injection technique described is a possibility. The incidence of the complication is less than 1%, but it occurs with greater frequency in patients with chronic obstructive pulmonary disease. Because of the proximity to the intercostal nerve and artery, the clinician should calculate carefully the total milligram dosage of local anesthetic administered, in consideration of the high vascular uptake via these vessels. Although uncommon, infection is an ever-present possibility, especially in an immunocompromised patient with cancer. Early detection of infection is crucial to avoid potentially life-threatening sequelae.

Clinical Pearls Patients with pain from slipping rib syndrome often attribute their pain symptoms to a gallbladder attack or ulcer disease. Reassurance is required, although it should be remembered that this musculoskeletal pain syndrome and intra-abdominal pathological conditions can coexist. Care must be taken to use sterile technique to avoid infection and universal precautions to avoid risk to the operator. The incidence of ecchymosis and hematoma formation can be decreased if pressure is placed on the injection site immediately after injection. The use of physical modalities, including local heat and gentle range-of-motion exercises, should be introduced several days after the patient undergoes this injection technique for slipping rib syndrome. Vigorous exercises should be avoided because they could exacerbate the symptoms. Simple analgesics and NSAIDs may be used concurrently with this injection technique. Laboratory evaluation for collagen-vascular disease is indicated in patients with costal cartilage pain in whom other joints are involved.

Suggested Readings Fares M.Y, Dimassi Z, Baydoun H, Musharrafieh U. Slipping rib syndrome: solving the mystery of the shooting pain. Am J Med Sci. 2019;357(2):168–173. McMahon L.E. Slipping rib syndrome: a review of evaluation, diagnosis and treatment. Semin Pediatr Surg. 2018;27(3):183–188. Turcios N.L. Slipping rib syndrome: an elusive diagnosis. Paediatr Respir Rev. 2017;2:44–46. van Delft E.A.K, van Pul K.M, Bloemers F.W. The slipping rib syndrome: a case report. Int J Surg Case Rep. 2016;23:23–24.

73

Winged Scapula Syndrome

Abstract Winged scapula syndrome is an uncommon cause of musculoskeletal pain of the shoulder and posterior chest wall. Caused by paralysis of the serratus anterior muscle, winged scapula syndrome begins as a painless weakness of the muscle with the resultant pathognomonic finding of winged scapula. As a result of dysfunction secondary to paralysis of the muscle, musculoskeletal pain often results. Winged scapula syndrome is often initially misdiagnosed as strain of the shoulder groups and muscles of the posterior chest wall because the onset of the syndrome often occurs after heavy exertion, most commonly after carrying heavy backpacks. The syndrome may coexist with entrapment of the suprascapular nerve.

Keywords brachial plexopathy; long thoracic nerve of Bell; posterior chest wall pain; serratus anterior muscle; suprascapular nerve; winged scapula; Winged Scapula syndrome

ICD-10 CODE M21.80

The Clinical Syndrome Winged scapula syndrome is an uncommon cause of musculoskeletal pain of the shoulder and posterior chest wall. Caused by paralysis of the serratus anterior muscle, winged scapula syndrome begins as a painless weakness of the muscle with the resultant pathognomonic finding of winged scapula. As a result of dysfunction secondary to paralysis of the muscle, musculoskeletal pain often results. Winged scapula syndrome is often initially misdiagnosed as strain of the shoulder groups and muscles of the posterior chest wall because the onset of the syndrome often occurs after heavy exertion, most commonly after carrying heavy backpacks. The syndrome may coexist with entrapment of the suprascapular nerve. Trauma to the long thoracic nerve of Bell is most often responsible for the development of winged scapula syndrome. Arising from the fifth, sixth, and seventh cervical nerves, the nerve is susceptible to stretch injuries and direct trauma. The nerve is often injured during first rib resection for thoracic outlet syndrome. Injuries to the brachial plexus or cervical roots also may cause scapular winging, but usually in conjunction with other neurological findings. Trauma to the serratus anterior muscle and facial entrapment of the long thoracic nerve of Bell can also cause winging of the scapula. The pain of winged scapula syndrome is aching and is localized to the muscle mass of the posterior chest wall and scapula. The pain may radiate into the shoulder and upper arm. The intensity of the pain of winged scapula syndrome is mild to moderate, but it may produce significant functional disability, which, if untreated, continues to exacerbate the musculoskeletal component of the pain.

Signs and Symptoms Regardless of the mechanism of injury to the long thoracic nerve of Bell, the common clinical feature of winged scapula syndrome is paralysis of the scapula resulting from weakness of the serratus anterior muscle. The pain of winged scapula syndrome generally develops after the onset of acute muscle weakness, but it is often erroneously attributed to overuse during vigorous exercise. On physical examination, the last 30 degrees of overhead arm extension is lost and the scapular rhythm is disrupted. By having the patient press the outstretched arms against a wall, the scapular winging is easily viewed by the clinician observing the patient from behind (Fig. 73.1 and 73.2). The remainder of the patient’s neurological examination should be within normal limits

Testing Owing to the ambiguity and confusion surrounding this clinical syndrome, testing is important to help confirm the diagnosis of winged scapula syndrome. Electromyography helps distinguish isolated damage to the long thoracic nerve of Bell associated with winged scapula syndrome from brachial plexopathy. Plain radiographs are indicated in all patients who present with winged scapula syndrome to rule out occult bony pathological processes. Based on the patient’s clinical presentation, additional tests, including complete blood cell count, uric acid level, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. Magnetic resonance imaging (MRI) and ultrasound imaging of the brachial plexus, cervical spine, or both is indicated if the patient exhibits other neurological deficits.

Differential Diagnosis Lesions of the cervical spinal cord, brachial plexus, and cervical nerve roots can produce clinical symptoms that include winging of the scapula. Such lesions also should produce additional neurological findings that allow the clinician to distinguish these pathological conditions from the isolated neurological findings seen in winged scapula syndrome. Pathology of the scapula or shoulder group also may confuse the clinical diagnosis.

Treatment No specific treatment for winged scapula syndrome exists other than removal of the cause of nerve entrapment (e.g., heavy backpacks or tumor compressing a nerve) and use of an orthotic device to help stabilize the scapula to allow normal shoulder function. Initial symptomatic relief of the pain and functional disability associated with winged scapula should include a combination of nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and physical therapy. Local application of heat and cold also may be beneficial. Repetitive movements or movements that incite the syndrome should be avoided.

FIG. 73.1 Scapular winging is best viewed by having the patient push his or her hands against the wall.

FIG. 73.2 A patient who experienced a winged scapula as a result of inadvertent injury to the long thoracic nerve. From Waldman SD. Long thoracic nerve block. In: Atlas of Pain Management Injection Techniques. 3rd ed. Philadelphia: Elsevier; 2013:341–343.

Complications and Pitfalls The major complications associated with winged scapula syndrome fall into two categories: (1) damage to the shoulder resulting from the functional disability associated with the syndrome and (2) failure to recognize that the cause of winging of the scapula is the result not of an isolated lesion of the long thoracic nerve of Bell but rather a part of a larger neurological problem.

Clinical Pearls Winged scapula syndrome is a distinct clinical entity that is difficult to treat. Early removal of the offending cause of nerve entrapment should allow rapid recovery of nerve function with resultant improvement in pain and shoulder dysfunction. A careful search for other causes of winging of the scapula should occur before attributing this neurological finding to winged scapula syndrome.

Suggested Readings Akgun K, Aktas I, Terzi Y. Winged scapula caused by a dorsal scapular nerve lesion: a case report. Arch Phys Med Rehabil. 2008;89:2017–2020. Belville R.G, Seupaul R.A. Winged scapula in the emergency department: a case report and review. J Emerg Med. 2005;29:279–282. . Nakatsuchi Y, Saitoh S, Hosaka M, Uchiyama S. Long thoracic nerve paralysis associated with thoracic outlet syndrome. J Shoulder Elbow Surg. 1994;3:28– 33. Salah S, Migaou H, Belaaj Z, et al. The winged scapula; a muscle rupture or a nerve paralysis? A case series. Ann Phys Rehabil Med. 2016;59:e114–e115. Sherman S.C, O’Connor M. An unusual cause of shoulder pain: winged scapula. J Emerg Med. 2005;28:329–331. Silva J.B, Gerhardt S, Pacheco I. Syndrome of fascial incarceration of the long thoracic nerve: winged scapula. Revista Brasileira de Ortopedia (English Edition). 2015;50(5):573–577. Singh V.K, Vargaonkar G.S. Winging of scapula due to serratus anterior tear. Chin J Traumatol. 2014;17(5):305–306. Tittal P, Pawar I, Kapoor S.K. Pseudo-winging of scapula due to benign lesions of ventral surface of scapula – two unusual causes. J Clin Orthop Trauma. 2015;6(1):30–35.

SECT ION 7

Abdominal and Groin Pain Syndromes OUT LINE 74. Anterior Cutaneous Nerve Entrapment 75. Acute Intermittent Porphyria 76. Radiation Enteritis 77. Liver Pain 78. Abdominal Angina

74

Anterior Cutaneous Nerve Entrapment ICD-10 CODE G58.9

The Clinical Syndrome Anterior cutaneous nerve entrapment is an uncommon cause of anterior abdominal wall pain that is a frequently overlooked clinical diagnosis. Anterior cutaneous nerve entrapment syndrome is a constellation of symptoms consisting of severe knife-like pain emanating from the anterior abdominal wall, associated with the physical finding of point tenderness over the affected anterior cutaneous nerve. The pain radiates medially to the linea alba but in almost all cases does not cross the midline. Anterior cutaneous nerve entrapment syndrome occurs most commonly in young women. The patient can often localize the source of pain accurately by pointing to the spot at which the anterior cutaneous branch of the affected intercostal nerve pierces the fascia of the abdominal wall at the lateral border of the abdominus rectus muscle (Fig. 74.1). At this point, the anterior cutaneous branch of the intercostal nerve turns sharply in an anterior direction to provide innervation to the anterior wall. The nerve passes through a firm fibrous ring as it pierces the fascia, and at this point the nerve becomes subject to entrapment. The nerve is accompanied through the fascia by an epigastric artery and vein. The potential exists for small amounts of abdominal fat to herniate through this fascial ring and become incarcerated, which results in further entrapment of the nerve. The pain of anterior cutaneous nerve entrapment is moderate to severe in intensity.

Signs and Symptoms As mentioned earlier, the patient often can point to the exact spot that the anterior cutaneous nerve is entrapped. Palpation of this point often elicits sudden sharp, lancinating pain in the distribution of the affected anterior cutaneous nerve. Voluntary contraction of the abdominal muscles puts additional pressure on the nerve and may elicit the pain. The patient attempts to splint the affected nerve by keeping the thoracolumbar spine slightly flexed to avoid increasing tension on the abdominal musculature (Fig. 74.2). Having the patient do a sit-up often reproduces the pain, as does a Valsalva maneuver. Patients with anterior cutaneous nerve entrapment will also exhibit a positive Carnett test when the patient is asked to tense his or her abdominal musculature, which is indicative of abdominal wall pain rather than pain with an intra-abdominal nidus (Fig. 74.3).

FIG. 74.1 The course of the anterior cutaneous nerve within the abdominal wall.

Testing Plain radiographs are indicated in all patients with pain thought to be emanating from the lower costal cartilage and ribs to rule out occult bony pathological conditions, including rib fracture and tumor. Radiographic evaluation of the gallbladder is indicated if cholelithiasis is suspected. Based on the patient’s clinical presentation, additional tests, including complete blood cell count, rectal examination with stool guaiac, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. Ultrasonography and computed tomography (CT) scan of the abdomen are indicated if intra-abdominal pathological process or occult mass is suspected. Injection of the anterior cutaneous nerve with or without ultrasound guidance at the point at which it pierces the fascia serves as a diagnostic and therapeutic maneuver (Fig. 74.4).

FIG. 74.2 Patients with anterior cutaneous nerve entrapment often attempt to splint the affected nerve by keeping the thoracolumbar spine slightly flexed to avoid increasing tension on the abdominal musculature.

Differential Diagnosis The differential diagnosis of anterior cutaneous nerve entrapment syndrome should consider ventral hernia, peptic ulcer disease, cholecystitis, intermittent bowel obstruction, renal calculi, angina, mesenteric vascular insufficiency, diabetic polyneuropathy, and pneumonia (Table 74.1). Rarely the collagen-vascular diseases, including systemic lupus erythematosus and polyarteritis nodosa, may cause intermittent abdominal pain; porphyria also may cause intermittent abdominal pain. Because the pain of acute herpes zoster may precede the rash by 24 to 72 hours, the pain may be attributed erroneously to anterior cutaneous nerve entrapment.

Treatment Initial treatment of the pain and functional disability associated with anterior cutaneous entrapment syndrome should include a combination of nonsteroidal antiinflammatory drugs (NSAIDs) or the cyclooxygenase-2 (COX-2) inhibitors and physical therapy. Local application of heat and cold may be beneficial. The repetitive movements that incite the syndrome should be avoided. For patients who do not respond to these treatment modalities, injection of the anterior cutaneous nerve at the point at which the nerve pierces the fascia with a local anesthetic and steroid may be a reasonable next step. If the symptoms of anterior cutaneous entrapment syndrome persist, surgical exploration and decompression of the anterior cutaneous nerve are indicated.

FIG. 74.3 (A) The patient is asked to completely relax the abdominal muscles and point with one finger to the most painful area. (B) The patient is then asked to maximally tense the abdominal muscles. The Carnett test is positive if the localized pain increases at the previously identified painful area.

FIG. 74.4 Transverse ultrasound image demonstrating the linea alba, rectus muscles, and the skin and subcutaneous tissues.

TABLE 74.1 The Differential Diagnosis of Anterior Cutaneous Nerve Entrapment Syndrome Differential Diagnosis Anterior cutaneous nerve entrapment syndrome

Investigations and Characteristics Carnett test, injection of local anesthetics

Thoracic lateral cutaneous nerve entrapment

History of previous surgery, clinical examination

Ilioinguinal or iliohypogastric nerve entrapment

History of previous groin surgery, clinical examination, injection of local anesthetics

Endometriosis

History of cyclic abdominal pain, laparoscopy

Myofascial pain syndrome

Clinical examination, myofascial strain

Slipping rib syndrome

Hypermobile, luxating eighth to tenth ribs, clinical examination

Diabetic radiculopathy

Paraspinal EMG, patient with diabetes mellitus

Abdominal wall tear

History of acute pain related to lifting or stretching, athletes

Abdominal wall or rectus sheath hematoma

Abdominal ultrasound or CT scan, after laparoscopy, after coughing in anticoagulated patient

Herpes zoster

History and clinical examination, dermatomal

Abdominal wall tumor (lipoma, desmoid, metastasis)

History and clinical examination, abdominal CT scan

Spinal nerve irritation

Referred pain by thoracic spine pathological condition

Hernia

Abdominal ultrasound, clinical examination

Traction symphysitis or pubalgia

Athletes, positive findings on MRI or scintigraphy

CT, Computed tomography; EMG, electromyography; MRI, magnetic resonance imaging.

Complications and Pitfalls The major complications associated with anterior cutaneous entrapment syndrome fall into two categories: (1) iatrogenically induced complications secondary to incorrect diagnosis and (2) failure of the clinician to recognize that a hernia coexists with the nerve entrapment until bowel ischemia occurs.

Clinical Pearls Patients with pain from anterior cutaneous nerve entrapment syndrome often attribute their pain symptoms to a gallbladder attack or ulcer disease. Reassurance is required, although it should be remembered that this musculoskeletal pain syndrome and intra-abdominal pathological conditions can coexist. The use of physical modalities, including local heat and gentle range-of-motion exercises, should be introduced several days after the patient undergoes this injection technique for anterior cutaneous nerve entrapment syndrome. Vigorous exercises should be avoided because they would exacerbate the symptoms. Simple analgesics and NSAIDs may be used concurrently with the aforementioned injection technique. Radiographic evaluation for intra-abdominal pathological conditions is indicated in patients with anterior abdominal pain of unclear origin.

Suggested Readings Armstrong L.B, Dinakar P, Mooney D.P. Neurectomy for anterior cutaneous nerve entrapment syndrome in children. J Pediatr Surg. 2018;53(8):1547– 1549. Hall M.W, Sowden D.S, Gravestock H, et al. Abdominal wall tenderness test. Lancet. 1991;7:1606–1607. Kanakarajan S, High K, Nagaraja R. Chronic abdominal wall pain and ultrasound-guided abdominal cutaneous nerve infiltration: a case series. Pain Med. 2011;12:382–386. Mol F.M.U, Lataster A, Scheltinga M, Roumen R. Anatomy of abdominal anterior cutaneous intercostal nerves with respect to the pathophysiology of anterior cutaneous nerve entrapment syndrome (ACNES): a case study. Transl Res Anat. 2017:8–9 6–10. Siawash M, Maatman R, Tjon W, et al. Anterior neurectomy in children with a recalcitrant anterior cutaneous nerve entrapment syndrome is safe and successful. J Pediatr Surg. 2017;52(3):478–480. Sweetser S. Abdominal wall pain: a common clinical problem. Mayo Clin Proc. 2019;94(2).

75

Acute Intermittent Porphyria

Abstract Acute intermittent porphyria is an autosomal dominant trait with variable clinical expression. The incidence of the gene responsible for acute intermittent porphyria is thought to be 1 in 100,000 individuals. The disease rarely manifests before puberty. Acute abdominal pain is usually the first clinical expression of the disease. As the name implies, the pain of acute intermittent porphyria is intermittent and colicky. The pain may be localized to the abdomen or may radiate to the flanks. The patient also may exhibit neurological symptoms, suggesting dysfunction of the central and peripheral nervous systems. Port wine urine, which is characteristic of hepatic porphyrias, including acute intermittent porphyria, is often seen during acute attacks.

Keywords abdominal pain; acute intermittent porphyria; enzyme deficiency; heme biosynthetic pathway; hepatic porphyrias; liver transplant; photosensitivity; porphobilinogen; port wine urine

ICD-10 CODE E802.9

The Clinical Syndrome Acute intermittent porphyria is an uncommon cause of abdominal pain that frequently confounds the diagnostic efforts of even the most astute clinician. The porphyrias are disorders of heme synthesis that can produce a wide range of clinical symptoms. Many different types of porphyrias occur, each of which manifests in a distinct clinical manner that reflects the specific enzyme deficiency of the heme biosynthetic pathway. The porphyrias can be inherited or acquired. The main clinical manifestations of the porphyrias are neurological dysfunction and the unique clinical finding of cutaneous sensitivity to sunlight. Acute intermittent porphyria is an autosomal dominant trait with variable clinical expression. The incidence of the gene responsible for acute intermittent porphyria is thought to be 1 in 100,000 individuals. This enzyme responsible for the clinical manifestation of acute intermittent porphyria is hydroxymethylbilane synthase, which is the third enzyme in the heme synthesis pathway. The disease rarely manifests before puberty. Acute abdominal pain is usually the first clinical expression of the disease. As the name implies, the pain of acute intermittent porphyria is intermittent and colicky. The pain may be localized to the abdomen or may radiate to the flanks. The patient also may exhibit neurological symptoms, suggesting dysfunction of the central and peripheral nervous systems. Port wine urine, which is characteristic of hepatic porphyrias, including acute intermittent porphyria, is often seen during acute attacks (Fig. 75.1).

Signs and Symptoms Although the reports of abdominal pain are often quite impressive in patients with acute intermittent porphyria, the abdominal examination is often nondescript (Fig. 75.2). Vomiting occasionally occurs. Tachycardia and autonomic dysfunction, including sweating, are common, as is labile hypertension. Occasionally urinary retention may occur and may confuse the clinical diagnosis, especially if port wine urine is present. Neurological findings, including attenuated deep tendon reflexes and decreased distal sensation suggestive of peripheral neuropathy, are often present and seizures often occur. Cranial nerve involvement is less common, but can be severe. Mental disturbance ranging from agitation to frank psychosis can occur in one-third of patients with acute intermittent porphyria. The presence of nervousness and anxiety often makes the care of these patients difficult. Alcohol, smoking, pregnancy, barbiturates, and oral contraceptives may precipitate attacks of acute intermittent porphyria, as may dehydration, calorie restriction, hormonal alterations, and infection.

Testing Given the usual delay in the diagnosis of acute intermittent porphyria, a considerable amount of testing is done. Most standard laboratory testing does not point the clinician toward a diagnosis of acute intermittent porphyria, however. Specifically, liver function tests are normal. A mild normocytic, normochromic anemia is sometimes present. Freshly passed urine is colorless, but it turns a port wine color if exposed to light. Given the low incidence of porphyria, qualitative urine screening tests, such as the Watson-Schwartz and Hoesch tests, are a reasonable first step in diagnosing porphyria. If the qualitative tests are positive, quantitative testing, such as gas chromatographic measurements for aminolevulinic acid, should be performed. Magnetic resonance imaging may reveal transient abnormalities during attacks of acute intermittent porphyria (Fig. 75.3).

Differential Diagnosis Essentially all causes of acute intermittent abdominal pain must be included in the differential diagnosis. The clinician needs to take a detailed history and perform a careful physical examination to rule out life-threatening causes of acute, intermittent abdominal pain, such as ischemic bowel, volvulus, and acute appendicitis. The key distinguishing factor in acute intermittent porphyria is that the patient’s report of severe abdominal pain and the benign abdominal examination do not correlate. Given the high incidence of psychiatric abnormalities in patients with acute intermittent porphyria, psychogenic causes of abdominal pain must be included in the differential diagnosis.

FIG. 75.1 Port wine urine in patient with acute intermittent porphyria. From Mumoli N, Vitale J, Cei M. Acute intermittent porphyria. Ann Emerg Med. 2014;63[2]: 267–273.

FIG. 75.2 Patients with acute intermittent porphyria often report very severe abdominal pain.

Treatment Attacks of acute intermittent porphyria can be aborted by the intravenous administration of large quantities of carbohydrates, such as glucose. Hematin can be given intravenously and seems to be well tolerated. Cimetidine, a histamine-2 inhibitor, also may be useful in ameliorating acute attacks. Avoidance of barbiturates, anticonvulsants, and alcohol is imperative to avoid exacerbating the symptoms of acute intermittent porphyria attacks. Careful attention to fluid and electrolyte balance also is important. Despite careful treatment, fatalities during attacks do occur. Orthotopic liver transplant has the potential to actually correct the enzyme defect responsible for the disease and provide both biochemical and clinical remission.

Complications and Pitfalls The major complications surrounding acute intermittent porphyria relate to misdiagnosis and failure to correct metabolic and electrolyte abnormalities during acute attacks. Barbiturates and anticonvulsants are often erroneously given to control seizures associated with acute intermittent porphyria, which worsen the porphyria, creating a vicious negative feedback cycle that ultimately may kill the patient.

Clinical Pearls The cause of abdominal pain in acute intermittent porphyria is thought to be the result of intermittent autonomic dysfunction, causing abnormal gut motility with alternating spasm and obstruction. The incidence of psychiatric abnormalities in patients with acute intermittent porphyria often confounds the clinician and complicates treatment. It has been said that to make a diagnosis, the clinician must think of it first. Nowhere is this statement more true than in the case of acute intermittent porphyria.

FIG. 75.3 Axial fluid-attenuated inversion recovery (FLAIR) images (2016-12-24) show bilateral cortical and subcortical hyperintense lesions (A to C). Follow-up FLAIR (2016-7-3) images show complete resolution of hyperintense lesions (D to F). From Yang J, Yang H, Chen Q, et al. Reversible MRI findings in a case of acute intermittent porphyria with a novel mutation in the porphobilinogen deaminase gene. Blood Cells Mol Dis. 2017;63:21–24.

Suggested Readings Besur S, Schmeltzer P, Bonkovsky H.L. Acute porphyrias. J Emerg Med. 2015;49(3):305–312. Crimlisk H.L. The little imitator: porphyria—a neuropsychiatric disorder. J Neurol Neurosurg Psychiatry. 1997;62:319–328. González-Parra S, Aparicio Reinoso T, Leonor M, et al. Psychiatric manifestations in porphyria: a case report of psychosis. Eur Psychiatr. 2016;33(suppl):S378–S379. Herrero C, Badenas C, Aguilera P, To-Figueras J. Acute intermittent porphyria: long-term follow up of 35 patients. Med Clín (English Edition). 2015;145(8):332–337. Herrick A.L, McColl K.E.L. Acute intermittent porphyria. Best Pract Res Clin Gastroenterol. 2005;19:235–249. Karim Z, Lyoumi S, Nicolas G, et al. Porphyrias: a 2015 update. Clin Res Hepatol Gastroenterol. 2015;39(4):412–425. Kuo H.C, Lee M.J, Chuang W.L, Huang C.C. Acute intermittent porphyria with peripheral neuropathy: a follow-up study after hematin treatment. J Neurol Sci. 2007;260:231–235. Lin C.N, Huang Y.C, Ro L.S, et al. Validation and evaluation of two porphobilinogen deaminase activity assays for diagnosis of acute intermittent porphyria. Clin Chim Acta. 2018;479:1–6. Peters T.J, Deacon A.C. International air travel: a risk factor for attacks in acute intermittent porphyria. Clin Chim Acta. 2003;335:59–63. Shen F.C, Hsieh C.H, Huang C.R. Acute intermittent porphyria presenting as acute pancreatitis and posterior reversible encephalopathy syndrome. Acta Neurol Taiwan. 2008;17:177–183.

76

Radiation Enteritis

Abstract Early symptoms of radiation enteritis are due to mucosal edema and ulceration, and include abdominal pain, nausea, vomiting, and a sensation of needing to move the bowels, tenesmus, or both. Late symptoms that are more related to radiation-induced scarring and narrowing of the bowel include small-caliber stools, rectal burning, and mucoid stools. The intensity of pain is mild to moderate and cramping. The onset of the early symptoms of radiation enteritis can begin within 1 week to 10 days after the completion of radiation therapy, and the late symptoms can occur months to years later.

Keywords abdominal pain; capsule endoscopy; colonoscopy; computed tomography; magnetic resonance imaging; radiation enteritis; radiation therapy; small caliber stools

ICD-10 CODE K52.0

The Clinical Syndrome As cancer patients live longer, clinicians are being called on with greater frequency to manage the side effects and complications of cancer therapy. One such complication is radiation enteritis. This complication of radiation therapy can occur after radiation to the abdomen or pelvis. Early symptoms of radiation enteritis are due to mucosal edema and ulceration, and include abdominal pain, nausea, vomiting, and a sensation of needing to move the bowels, tenesmus, or both. Late symptoms that are more related to radiationinduced scarring and narrowing of the bowel include small-caliber stools, rectal burning, and mucoid stools. The intensity of pain is mild to moderate and includes cramping. The onset of the early symptoms of radiation enteritis can begin within 1 week to 10 days after the completion of radiation therapy, and the late symptoms can occur months to years later. A variety of factors can predispose the patient to the development of radiation enteritis, including preexisting systemic disease such as diabetes and treatmentrelated factors. Recent studies suggest that genetic tissue specific hypoxiainducible factor alpha may modify the acute gastrointestinal tissue response to radiation (Table 76.1).

Signs and Symptoms Physical examination of a patient with radiation enteritis reveals diffuse abdominal tenderness and hyperactive bowel sounds. Mild abdominal distention may be present. Signs of acute peritoneal irritation suggestive of perforated viscus, such as rebound tenderness, are absent. The patient may exhibit frequent mucoid stools, diarrhea, and vomiting. The patient appears systemically ill, but not septic (Fig. 76.1).

Testing Colonoscopy provides definitive evidence of radiation enteritis, while helping to exclude other causes of abdominal pain that may mimic this clinical syndrome. Capsule endoscopy may also aid in the diagnosis. Based on the patient’s clinical presentation, additional tests, including complete blood cell count, erythrocyte sedimentation rate, and stool and blood cultures for infectious enteritis, may be indicated (Fig. 76.2). Computed tomography (CT) of the abdomen with oral and intravenous contrast material is indicated if occult mass or abscess is suspected. Magnetic resonance imaging (MRI) of the abdomen also helps confirm the diagnosis of radiation enteritis (Fig. 76.3).

Differential Diagnosis A history of previous radiation therapy is necessary to consider the diagnosis of radiation enteritis. The very problem that necessitated radiation therapy in the first place—malignancy—can recur, however, and produce clinical symptoms indistinguishable from those of radiation enteritis. Given the immunocompromised state of most patients who have received radiation therapy, the possibility of infectious enteritis or intra-abdominal abscess always must be included in the differential diagnosis. Other causes of abdominal pain, including diverticulitis, bowel obstruction, and appendicitis, also may occur in conjunction with radiation enteritis.

Treatment Symptom management is the primary thrust of the treatment of radiation enteritis. Careful attention to the patient’s fluid and metabolic status during the acute phases of the disease is crucial to avoid complications. Psyllium helps the patient with diarrhea and with mucoid stools, and may decrease the sensations of needing to move the bowels frequently. Anticholinergics such as dicyclomine and antiperistaltics such as loperamide can help decrease diarrhea. Zinc oxide ointment and sitz baths with aluminum acetate soaks help ease the symptoms of tenesmus and rectal pain. Steroid and sucralfate enemas have also been reported to provide symptomatic relief in difficult cases of radiation enteritis. TABLE 76.1 Risk Factors Associated With Chronic Radiation Enteritis Patient Factors

Comorbidities (e.g., diabetes mellitus, hypertension, inflammatory bowel disease)

Treatment Factors Volume of small bowel in radiotherapy field Radiotherapy dose and fractionation

Smoking

Radiotherapy technique

Reduced body mass index

Previous intestinal surgery

Concomitant chemotherapy use Hypoxia-inducible factor alpha effect

Modified from Theis VS, Sripadam R, Ramani V, et al. Chronic radiation enteritis. Clin Oncol. 2010;2:70–83.

FIG. 76.1 Patients with radiation enteritis typically exhibit diffuse abdominal tenderness, hyperactive bowel sounds, and mild abdominal distention, with frequent mucoid stools, diarrhea, and vomiting. They appear systemically ill but are not septic.

Complications and Pitfalls The potential for complications after radiation therapy is high. Spontaneous bowel perforation, stenosis, fistula formation, bleeding, and malabsorption occur with sufficient frequency to complicate the management of this painful condition. As mentioned, the potential for recurrence of tumor and infectious complications is ever present.

Clinical Pearls Treatment of the symptoms associated with radiation enteritis should be part of the overall management of a patient with cancer. The recognition and treatment of symptoms other than pain are often delayed while the clinician focuses on pain control, further compounding the patient’s suffering. Vigilance for life-threatening complications of radiation enteritis, including bowel perforation, is mandatory to avoid disaster.

FIG. 76.2 Radiation Enteritis Diagnosed With Capsule Endoscopy.(A) Mucosal edema of the terminal ileum with superficial whitish spots and nonspecific chronic inflammation in the histology report. (B) The presence of multiple ulcers that distorted and strictured the intestinal lumen. (C) Distal segments through which the device passed with no difficulty. (D) Postradiation enteritis is shown. From Juanmartiñena Fernández JF, Elosua-González A, Casanova-Ortiz L, et al. Radiation enteritis diagnosed through capsule endoscopy, an uncommon cause of iron-deficiency anemia. Revista de Gastroenterología de México (English Edition). 2017;82[4]:339–340.

FIG. 76.3 Radiation Enteritis.Gadolinium-enhanced spoiled gradient echo image after pelvic radiation shows segmental mural thickening and enhancement (arrows) representing evidence of radiation enteritis. From Edelman RR, Hesselink JR, Zlatkin MB, et al, eds. Clinical Magnetic Resonance Imaging. 3rd ed. Philadelphia: Saunders; 2006:2701.

Suggested Readings Andreyev H.J. Gastrointestinal problems after pelvic radiotherapy: the past, the present and the future. Clin Oncol. 2007:19790–19799. Chon B.H, Loeffler J.S. The effect of nonmalignant systemic disease on tolerance to radiation therapy. Oncologist. 2002;7:136–143. Grabenbauer G.G, Holger G. Management of radiation and chemotherapy related acute toxicity in gastrointestinal cancer. Best Pract Res Clin Gastroenterol. 2016;30(4):655–664. Juanmartiñena Fernández J.F, Elosua-González A, Casanova-Ortiz L, et al. Radiation enteritis diagnosed through capsule endoscopy, an uncommon cause of iron-deficiency anemia. Rev Gastroenterol México (English Edition). 2017;82(4):339–340. Martínez Hernández Magro P. Bowel obstruction secondary to radiation enteritis: a case report. Rev Gastroenterol México (English Edition). 2015;80(1):111–113. Theis V.S, Sripadam R, Ramani V, Lal S. Chronic radiation enteritis. Clin Oncol. 2010;22:70–83. Toullec A, Buard V, Rannou E, et al. HIF-1α deletion in the endothelium, but not in the epithelium, protects from radiation-induced enteritis. Cell Mol Gastroenterol Hepatol. 2018;5(1):15–30. Waddell B.E, RodriguezBigas M.A, Lee R.J, Weber T.K, Petrelli N.J. Prevention of chronic radiation enteritis. J Am Coll Surg. 1999;189:611–624.

77

Liver Pain

Abstract Liver pain is a common clinical occurrence, but it is often poorly diagnosed and treated. The liver can serve as a source of pain in and of itself through the sympathetic nervous system and via referred pain secondary to peritoneal irritation through the intercostal and subcostal nerves. Pain that emanates from the liver itself tends to be ill defined and may be referred primarily to the epigastrium. It is dull and aching and is mild to moderate in severity. The pain can be related to swelling of the liver and concomitant stretching of the liver capsule or distention of the veins, as is seen with portal obstruction. This pain is carried via sympathetic fibers from the celiac ganglion that enter the liver along with the hepatic artery and vein. This type of liver pain responds poorly to adjuvant analgesics. Occasionally, hepatic enlargement causes diaphragmatic irritation, which produces pain that is referred to the ipsilateral supraclavicular and shoulder region. This referred pain is known as the Kehr sign, and is transmitted via the phrenic nerve and often misdiagnosed.

Keywords intercostal block; Kehr sign; liver pain; liver tumor; peritoneal irritation; pleurisy; sympathetic pain; ultrasound guided nerve block

ICD-10 CODE K76.8

The Clinical Syndrome Liver pain is a common clinical occurrence, but it is often poorly diagnosed and treated. The liver can serve as a source of pain in and of itself through the sympathetic nervous system and via referred pain secondary to peritoneal irritation through the intercostal and subcostal nerves. Pain that emanates from the liver itself tends to be ill defined and may be referred primarily to the epigastrium. It is dull and aching, and is mild to moderate in severity. The pain can be related to swelling of the liver and concomitant stretching of the liver capsule or distention of the veins, as is seen with portal obstruction. This pain is carried via sympathetic fibers from the celiac ganglion that enter the liver along with the hepatic artery and vein. This type of liver pain responds poorly to adjuvant analgesics. Occasionally, hepatic enlargement causes diaphragmatic irritation, which produces pain that is referred to the ipsilateral supraclavicular and shoulder region. This referred pain is known as the Kehr sign, and is transmitted via the phrenic nerve and often misdiagnosed. Referred liver pain is caused by mechanical irritation and inflammation of the inferior pleura and peritoneum. This pain is somatic and carried primarily by the lower intercostal and subcostal nerves. This somatic pain is sharp and pleuritic and is moderate to severe in intensity. It responds more favorably to nonsteroidal antiinflammatory drugs (NSAIDs) and opioid analgesics in contrast to sympathetically mediated liver pain.

Signs and Symptoms The clinical presentation of liver pain is directly related to whether the pain is mediated via the sympathetic or somatic nervous system or both. In patients with sympathetically mediated pain, the abdominal examination may reveal hepatomegaly with tenderness to palpation of the liver. Primary tumor or metastatic disease may also be identified. The remainder of the abdominal examination is nondescript. Auscultation over the liver fails to reveal a friction rub in most cases. As mentioned, the patient may report illdefined pain in the supraclavicular region (Fig. 77.1). Patients with somatically mediated liver pain present in an entirely different manner. The patient often splints the right lower chest wall and abdomen and takes small, short breaths to avoid exacerbating the pain. The patient may avoid coughing because of the pain and accumulated upper airway secretions, and atelectasis may be a problem. The abdominal examination may reveal signs of peritoneal irritation over the right upper quadrant. A friction rub is often present with auscultation over the liver. The liver may be extremely tender to palpation. Primary tumor or metastatic disease or both may be present.

FIG. 77.1 Patients with liver pain may report ill-defined pain in the supraclavicular region.

FIG. 77.2 Gallbladder carcinoma (small arrows) manifesting as thickening of the gallbladder wall with a gallstone (large arrow) and metastasis to lymph nodes (n). From Haaga JR, Lanzieri CF, Sartoris UJ, et al. Computed Tomography and Magnetic Resonance Imaging of the Whole Body. 3rd ed. St Louis: Mosby; 1994:1359.

Testing Testing for patients with liver pain should be aimed at identifying the primary source of liver disease responsible for the pain and ruling out other pathological processes that may be responsible for the pain. Plain radiographs of the chest and abdomen, including an upright abdominal film, are indicated in all patients with pain thought to be emanating from the liver. Radiographs of the ribs are indicated to rule out occult bony pathological conditions, including tumors. Based on the patient’s clinical presentation, additional tests, including complete blood cell count, automated chemistries, liver function test, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. Computed tomography (CT) and magnetic resonance imaging (MRI) of the lower thoracic contents and abdomen are indicated in most patients with liver pain to rule out occult pulmonary and intra-abdominal pathological processes, including cancer of the gallbladder and pancreas (Figs. 77.2 and 77.3). Positron emission tomography may help identify occult lesions both above and below the diaphragm. Differential neural blockade on an anatomical basis can serve as a diagnostic and therapeutic maneuver (see discussion of treatment).

Differential Diagnosis Pain of hepatic origin must be taken seriously. It is often the result of an underlying serious disease, such as biliary malignancy, portal hypertension, or hepatic metastatic disease. Pain emanating from the liver is often mistaken for pain of cardiac or gallbladder origin and can lead to visits to the emergency department and unnecessary cardiac and gastrointestinal workups. If trauma has occurred, liver pain may coexist with rib fractures or fractures of the sternum itself that can be missed on plain radiographs and may require radionucleotide bone scanning for proper identification.

FIG. 77.3 Axial precontrast T1-weighted magnetic resonance imaging shows numerous bright metastases within the liver and vertebral body. From Edelman RR, Hesselink JR, Zlatkin MB, et al., eds. Clinical Magnetic Resonance Imaging. 3rd ed. Philadelphia: Saunders; 2006:2577.

Neuropathic pain involving the chest wall may be confused or coexist with liver pain. Examples of neuropathic pain include diabetic polyneuropathies and acute herpes zoster involving the lower thoracic and upper lumbar nerves. The possibility of diseases of the structures of the inferior mediastinum and retroperitoneum is ever present, and these diseases

sometimes can be difficult to diagnose. Pathological processes that inflame the pleura, such as pulmonary embolus, infection, and Bornholm disease, may mimic or coexist with pain of hepatic origin.

Treatment Initial treatment of liver pain should include a combination of simple analgesics and NSAIDs or cyclooxygenase-2 (COX-2) inhibitors. If these medications do not control the patient’s symptoms adequately, an opioid analgesic may be added. Local application of heat and cold may be beneficial to provide symptomatic relief of liver pain. The use of an elastic rib belt over the liver may help provide symptomatic relief. For patients who do not respond to these treatment modalities, an intercostal nerve block using a local anesthetic and steroid may be a reasonable next step. If the pain is thought to be sympathetically mediated, a celiac plexus block is a reasonable next step. This technique provides diagnostic and therapeutic benefit. If the pain is thought to be somatic, intercostal nerve blocks should be the next step. Pain of hepatic origin may be somatic and sympathetic, and require celiac plexus and intercostal nerve block for complete control. The use of ultrasound guidance may improve the accuracy of needle placement and decrease the incidence of needle-related complications.

Complications and Pitfalls The major problem in the care of patients thought to have liver pain is the failure to identify potentially serious pathological processes of the thorax or upper abdomen. Given the proximity of the pleural space, pneumothorax after intercostal nerve block is a possibility. The incidence of the complication is less than 1%, but it occurs with greater frequency in patients with chronic obstructive pulmonary disease. Although uncommon, infection, including liver abscess, remains an ever-present possibility, especially in an immunocompromised patient with cancer. Early detection of infection is crucial to avoid potentially life-threatening sequelae.

Clinical Pearls Liver pain is often poorly diagnosed and treated. Correct diagnosis of the cause of liver pain and the nerves subserving the pain is necessary to treat this painful condition properly and to avoid overlooking serious intrathoracic or intra-abdominal pathological processes. Intercostal nerve block is a simple technique that can produce dramatic relief for patients with liver pain thought to be somatically mediated. Celiac plexus block is technically more demanding and should be performed only by clinicians well versed in the technique and potential complications.

Suggested Readings Erol O, Unsar S, Yacan L, Pelin M, Kurt S, Erdogan B. Pain experiences of patients with advanced cancer: a qualitative descriptive study. Eur J Oncol Nurs. 2018;33:28–34. Goodman C.C. Screening for medical problems in patients with upper extremity signs and symptoms. J Hand Ther. 2010;23:105–126. Hansen L, Sasaki A, Zucker B. End-stage liver disease: challenges and practice implications. Nurs Clin North Am. 2010;45:411–426. Khoury G.F, Stein C, Ramming K.P. Neck and shoulder pain associated with hepatic arterial chemotherapy using an implantable infusion pump. Pain. 1988;32:275–277. Maurice J.B, Yu D, Watkins J. A 41-year-old man with abdominal pain, deranged liver function, and fevers. Gastroenterology. 2018;154(1):26–27. Tsunekawa K, Matsuda R, Ohgushi N, Ohnishi Ogasawara A. Basic problems of the pain from the gallbladder and liver. Pain. 1987;30(suppl 1):S24. Yeager K.A, Quest T.E, Vena C, Sterk C.E. Living with symptoms: a qualitative study of black adults with advanced cancer living in poverty. Pain Manag Nurs. 2018;19(1):34–45. Waldman S.W, Feldstein G.S, Donohoe C.D, Waldman K.A. The relief of body wall pain secondary to malignant hepatic metastases by intercostal nerve block with bupivacaine and methylprednisolone. J Pain Symptom Manage. 1988;3:39–43.

78

Abdominal Angina

Abstract The cause of abdominal angina is arterial vascular insufficiency. The term angina is used because the pain occurs only after eating, when the insufficient fixed arterial supply is unable to meet the increased demands needed to support digestion. The most common cause of abdominal angina is stenosis of the celiac artery with inadequate collateralization. Aneurysms of the superior mesenteric artery, the vasculitides, fibromuscular hyperplasia, and tumor encroachment on the celiac artery have also been implicated as causes of abdominal angina.

Keywords abdominal angina; abdominal pain; arterial vascular insufficiency; celiac artery; malabsorption; superior mesenteric artery; vascular disease; vasculitides; weight loss

ICD-10 CODE K55.1

The Clinical Syndrome Abdominal angina is an uncommon cause of intermittent abdominal pain. Patients with abdominal angina report severe cramping abdominal pain that begins 15 to 30 minutes after eating (Fig. 78.1). This postprandial pain persists for 2 to 3 hours. Additional ingestion of food aggravates the patient’s pain, forcing the patient to stop eating. Weight loss is common. As the disease progresses, malabsorption and diarrhea occur as a result of mucosal and mural injury, which further exacerbates the patient’s weight loss. The cause of abdominal angina is arterial vascular insufficiency. The term angina is used because the pain occurs only after eating, when the insufficient fixed arterial supply is unable to meet the increased demands needed to support digestion. The most common cause of abdominal angina is stenosis of the celiac artery with inadequate collateralization. Aneurysms of the superior mesenteric artery, the vasculitides, fibromuscular hyperplasia, and tumor encroachment on the celiac artery also have been implicated as causes of abdominal angina.

Signs and Symptoms Physical examination of a patient with abdominal angina reveals diffuse abdominal tenderness. Mild abdominal distention may be present. Signs of acute peritoneal irritation suggestive of perforated viscus, such as rebound tenderness, are absent. The patient may exhibit frequent defecation of mucoid stools, diarrhea, and vomiting. The patient appears systemically ill, but not septic.

Testing The diagnosis of abdominal angina is based on clinical history. Angiography of the celiac artery provides proof of vascular insufficiency and often identifies the cause of the problem. Barium enema shows the classic finding of thumbprinting that is strongly suggestive of mucosal ischemia (Fig. 78.2). Colonoscopy reveals localized hemorrhage and ulceration of the affected mucosa. Based on the patient’s clinical presentation, additional tests, including complete blood cell count, erythrocyte sedimentation rate, and stool and blood cultures for infectious enteritis, may be indicated. Given the possibility that the patient’s abdominal angina is due to vasculitis, a collagenvascular workup is indicated in all patients with abdominal angina. Computed tomography (CT) of the abdomen with oral and intravenous contrast material is indicated if an occult mass or abscess is suspected. Magnetic resonance angiography (MRA) of the celiac and mesenteric vessels also can help clarify the diagnosis and aid in planning a treatment strategy, as can ultrasonographic and Doppler flow studies (Figs. 78.3 and 78.4).

FIG. 78.1 Abdominal angina is an uncommon cause of intermittent abdominal pain. Patients with abdominal angina report severe cramping abdominal pain that begins 15 to 30 minutes after eating.

FIG. 78.2 Thumbprinting in Acute Ischemic Colitis at the Splenic Flexure. From Grainger RG, Allison D. Grainger and Allison’s Diagnostic Radiology: A Textbook of Medical Imaging. 3rd ed. New York: Churchill Livingstone; 1997:1036.

FIG. 78.3 Severe chronic mesenteric ischemia secondary to superior mesenteric artery occlusion and a high-grade celiac stenosis (arrow) depicted by contrastenhanced magnetic resonance angiography (left). Anterior projection shows a large inferior mesenteric artery (arrow) that supplies the entire abdomen via large mesenteric and retroperitoneal collaterals (right). From Edelman RR, Hesselink JR, Zlatkin MB, et al, eds. Clinical Magnetic Resonance Imaging. 3rd ed. Philadelphia: Saunders; 2006:805.

Differential Diagnosis Any disease process that can produce ischemic bowel can mimic the pain of abdominal angina. The vasculitides, including polyarteritis nodosum and Henoch-Schönlein purpura, also can cause the symptoms of abdominal angina. Embolic disease that may cause occlusion of the vascular supply to the gut also should be considered. The possibility of infectious enteritis always must be included in the differential diagnosis. Other causes of abdominal pain, including diverticulitis, bowel obstruction, and appendicitis, may occur in conjunction with abdominal angina.

FIG. 78.4 Ischemic Colitis.(A) Longitudinal view shows thickened descending colon with absence of blood flow. The mural stratification is maintained. (B) Transverse view shows diffuse, poorly reflective thickening (arrow), loss of the mural stratification, and absence of blood flow. Focal area of pneumatosis is seen (arrow) with edema of the paracolic fat and ascites (arrowhead). From Allen PL, Baxter GM, Weston MJ. Clinical Ultrasound. vol 1, 3rd ed. New York: Churchill Livingstone; 2011:402.

Treatment The only definitive treatment of abdominal angina is correction of the arterial insufficiency via either angioplasty, stent placement, and/or surgical revascularization. Careful attention to the patient’s fluid and metabolic status is crucial to avoid complications. Anticholinergics such as dicyclomine and antiperistaltics such as loperamide can help decrease diarrhea. Small, frequent feedings also may help palliate the postprandial pain.

Complications and Pitfalls The potential for complications in patients with abdominal angina is high. Spontaneous bowel perforation, stenosis, fistula formation, bleeding, and malabsorption occur with sufficient frequency to complicate the management of this painful condition. Untreated, abdominal angina frequently progresses to bowel infarction.

Clinical Pearls The treatment of the symptoms associated with abdominal angina is difficult, and ultimately, correction of the vascular insufficiency is required. Vigilance for life-threatening complications of abdominal angina, including bowel infarction, is mandatory to avoid disaster.

Suggested Readings Blauw J.T.M, Bulut T, Oderich G.S, Geelkerken B.R.H. Mesenteric vascular treatment 2016: from open surgical repair to endovascular revascularization. Best Pract Res Clin Gastroenterol. 2017;31(1):75–84. Kärkkäinen J.M, Acosta S. Acute mesenteric ischemia (part I) – incidence, etiologies, and how to improve early diagnosis. Best Pract Res Clin Gastroenterol. 2017;31(1):15–25. Kärkkäinen J.M, Acosta S. Acute mesenteric ischemia (Part II) – vascular and endovascular surgical approaches. Best Pract Res Clin Gastroenterol. 2017;31(1):27–38. Naganuma T, Fujino Y, Mitomo S, et al. One-year follow-up optical coherence tomography after endovascular treatment with a new-generation zotarolimus-eluting stent for chronic mesenteric ischemia. Hell J Cardiol. 2017;58(3):233–235. van Dijk L.J.D, van Petersen A.S, Moelker A. Vascular imaging of the mesenteric vasculature. Best Pract Res Clin Gastroenterol. 2017;31(1):3–14.

SECT ION 8

Lumbar Spine and Sacroiliac Joint Pain Syndromes OUT LINE 79. Epidural Abscess 80. Multiple Myeloma 81. Foix-Alajouanine Syndrome 82. Paget Disease 83. Diffuse Idiopathic Skeletal Hyperostosis 84. Spondylolisthesis 85. Ankylosing Spondylitis 86. Superior Cluneal Nerve Entrapment Syndrome 87. Lumbar Myofascial Pain Syndrome 88. Lumbar Paraspinal Compartment Syndrome

79

Epidural Abscess

Abstract A patient with epidural abscess initially presents with ill-defined pain in the segment of the spine affected (e.g., cervical, thoracic, or lumbar). This pain becomes more intense and localized as the abscess increases in size and compresses neural structures. Low-grade fever and vague constitutional symptoms, including malaise and anorexia, progress to frank sepsis with a high-grade fever, rigors, and chills. At this point, the patient begins to experience sensory and motor deficits and bowel and bladder symptoms as the result of neural compromise. As the abscess continues to expand, compromise of the vascular supply to the affected spinal cord and nerve occurs with resultant ischemia and, if untreated, infarction and permanent neurological deficits.

Keywords Babinski sign; computerized tomography; epidural abscess; epidural; epidural steroid injection; infection; magnetic resonance imaging; sepsis

ICD-10 CODE G06.1

The Clinical Syndrome Epidural abscess is an uncommon cause of spine pain that, if undiagnosed, can result in paralysis and life-threatening complications. Epidural abscess can occur anywhere in the spine and intracranially. It can occur spontaneously via hematogenous seeding, most frequently as a result of urinary tract infections that spread to the spinal epidural space via Batson plexus. More commonly, epidural abscess occurs after instrumentation of the spine, including surgery and epidural nerve blocks. The literature suggests that the administration of steroids into the epidural space results in immunosuppression, with a resultant increase in the incidence of epidural abscess. Although theoretically plausible, the statistical evidence—given the thousands of epidural steroid injections performed around the United States on a daily basis—calls this belief into question. A patient with epidural abscess initially presents with ill-defined pain in the segment of the spine affected (e.g., cervical, thoracic, or lumbar) (Fig. 79.1). This pain becomes more intense and localized as the abscess increases in size and compresses neural structures. Low-grade fever and vague constitutional symptoms, including malaise and anorexia, progress to frank sepsis with a high-grade fever, rigors, and chills. At this point, the patient begins to experience sensory and motor deficits and bowel and bladder symptoms as the result of neural compromise. As the abscess continues to expand, compromise of the vascular supply to the affected spinal cord and nerve occurs with resultant ischemia and, if untreated, infarction and permanent neurological deficits.

Signs and Symptoms A patient with epidural abscess initially has ill-defined pain in the general area of the infection. At this point, mild pain may occur on range of motion of the affected segments. The neurological examination is within normal limits. A low-grade fever, night sweats, or both may be present. Theoretically, if the patient has received steroids, these constitutional symptoms may be attenuated or their onset may be delayed. As the abscess increases in size, the patient appears acutely ill, with fever, rigors, and chills. The clinician may be able to identify neurological findings suggestive of spinal nerve root compression, spinal cord compression, or both. Subtle findings that point toward the development of myelopathy (e.g., Babinski sign, clonus, and decreased perineal sensation) may be overlooked if not carefully sought. As compression of the involved neural structures continues, the patient’s neurological status may deteriorate rapidly. If the diagnosis is not made, irreversible motor and sensory deficit occurs.

Testing Myelography is still considered the best test to ascertain compromise of the spinal cord and exiting nerve roots by an extrinsic mass such as an epidural abscess. In this era of readily available magnetic resonance imaging (MRI) and high-speed computed tomography (CT), it may be more prudent to perform this noninvasive testing first, rather than wait for a radiologist or spine surgeon to perform a myelogram (Fig. 79.2). MRI and CT are highly accurate in the diagnosis of epidural abscess and are probably more accurate than myelography in the diagnosis of intrinsic disease of the spinal cord and spinal tumor. All patients suspected to have epidural abscess should undergo laboratory testing consisting of complete blood cell count, erythrocyte sedimentation rate, and automated blood chemistries. Blood and urine cultures should be performed immediately in all patients thought to have epidural abscess to allow immediate implementation of antibiotic therapy while the workup is in progress. Gram stains and cultures of the abscess material also should be performed, but antibiotic treatment should not be delayed waiting for this information.

Differential Diagnosis The diagnosis of epidural abscess should be strongly considered in any patient with spine pain and fever, especially if the patient has undergone spinal instrumentation or epidural nerve blocks for either surgical anesthesia or pain control. Other pathological processes that must be considered in the differential diagnosis include intrinsic disease of the spinal cord, such as demyelinating disease and syringomyelia, and other processes that can result in compression of the spinal cord and exiting nerve roots, such as metastatic tumor, Paget disease, and neurofibromatosis. As a general rule, unless the patient has concomitant infection, these diseases are routinely associated with only back pain and not with fever.

FIG. 79.1 Patients with epidural abscess initially present with ill-defined pain in the affected segment of the spine.

Treatment The rapid initiation of treatment of epidural abscess is mandatory if the patient is to avoid the sequelae of permanent neurological deficit or death. The treatment of epidural abscess has two goals: (1) treatment of the infection with antibiotics and (2) drainage of the abscess to relieve compression on neural structures. Because most epidural abscesses are caused by Staphylococcus aureus, antibiotics such as vancomycin that treat staphylococcal infection should be started immediately after blood and urine culture samples are taken. Antibiotic therapy can be tailored to the culture and sensitivity reports as they become available. As mentioned, antibiotic therapy should not be delayed while waiting for definitive diagnosis if epidural abscess is being considered as part of the differential diagnosis. Antibiotics alone rarely treat an epidural abscess successfully unless the diagnosis is made very early in the course of the disease; drainage of the abscess is required to effect full recovery. Drainage of the epidural abscess is usually accomplished via decompression laminectomy and evacuation of the abscess (Fig. 79.3). More recently, interventional radiologists have been successful in draining epidural abscesses percutaneously using drainage catheters placed with the use of CT or MRI guidance. Serial CT or MRI scans are useful in following the resolution of epidural abscess; scans should be repeated immediately at the first sign of negative change in the patient’s neurological status.

Complications and Pitfalls Failure to diagnose and treat epidural abscess rapidly and accurately can result in disaster for the clinician and patient alike. The insidious onset of neurological deficit associated with epidural abscess can lull the clinician into a sense of false security that can result in permanent neurological damage to the patient. If epidural abscess or other causes of spinal cord compression is suspected, the algorithm shown in Box 79.1 should be followed.

FIG. 79.2 Sagittal (A and B) and axial (C and D) T2-weighted magnetic resonance imaging (MRI) of discitis at the LF-S1 disk level showing high-signal-intensity fluid within the disk. High-signal-intensity fluid collections (white arrows) are seen in the epidural space, consistent with abscesses. The sagittal postcontrast T1-weighted MRI with fat saturation (E and F) show the abscesses as low-signal-intensity areas with only peripheral enhancement (broken white arrows). From Waldman SD, Campbell RSD, eds. Imaging of Pain. Philadelphia, PA: Saunders; 2011:152.

FIG. 79.3 Intraoperative photograph demonstrating a purulent epidural abscess. From Kumar A, Kumar V, Dhatt SS, et al. Spontaneous spinal epidural abscess in a normoglycemic diabetic patient—keep it as a differential. J Clin Orthop Trauma. 2017;8[2]:178–180.

BO X 79. 1 Algorit hm for Eva lua t ion of Spina l C ord

C om pre ssion C a use d by Epidura l Absce ss Immediately obtain blood and urine samples for cultures. Immediately start high-dose antibiotics that cover Staphylococcus aureus. Immediately order the most readily available spinal imaging technique (computed tomography, magnetic resonance imaging, myelography) that can confirm the presence of spinal cord compression (e.g., abscess, tumor). Simultaneously obtain emergency consultation from a spine surgeon. Continuously and carefully monitor patient’s neurological status. If any of the measures listed here are unavailable, arrange emergency transfer of patient to tertiary care center by the most rapidly available transportation. Repeat imaging and obtain repeat surgical consultation if any deterioration occurs in the patient’s neurological status.

Clinical Pearls

Delay in diagnosis puts the patient and clinician at tremendous risk for a poor outcome. The clinician should assume that all patients who present with fever and back pain have an epidural abscess until proved otherwise and should treat accordingly. Overreliance on a single negative or equivocal imaging test is a mistake. Serial CT or MRI scans are indicated should there be any deterioration in the patient’s neurological status.

Suggested Readings Bridges K.J, Than KD. Holospinal epidural abscesses – institutional experience. J Clin Neurosci. 2018;48:18–27. Farber S.H, Murphy KR, Suryadevara CM, et al. Comparing outcomes of early, late, and non-surgical management of intraspinal abscess. J Clin Neurosci. 2017;36:64–71. Fong J, Roantree RA, Spano C. Lumbar l3-4 and l4-5 epidural abscesses from Peptostreptococcus micros with tethering of the cauda equina. Visual J Emerg Med. 2018;10:125–126. Schneider B.J, Maybin S, Sturos E. Safety and complications of cervical epidural steroid injections. Phys Med Rehabil Clin N Am. 2018;29(1):155–169. Shrivastava A, Tadepalli K, Goel G, Gupta K, Kumar Gupta P. Melanized fungus as an epidural abscess: a diagnostic and therapeutic challenge. Med Mycol Case Reports. 2017;16:20–24. Vakili M. Crum-Cianflone NF. Spinal epidural abscess: a series of 101 cases. Am J Med. 2017;130(12):1458–1463.

80

Multiple Myeloma

Abstract The most common clinical presentation of multiple myeloma is back and rib pain. It occurs in more than 70% of patients ultimately diagnosed with the disease. These bone lesions are osteolytic and are best diagnosed with plain radiography rather than with radionucleotide bone scanning. Pain with movement is common, and hypercalcemia occurs with sufficient frequency to be the presenting symptom in many patients with multiple myeloma. Life-threatening infection, anemia, bleeding, and renal failure are often present in conjunction with the symptoms of pain. Hyperviscosity of the serum that is the result of the products of tumor production may lead to cerebrovascular accidents.

Keywords bence Jones protein; back pain; Chvostek sign; computerized tomography; hypercalcemia; multiple myeloma; protein electrophoresis; radiography; RNA virus; Trousseau sign

ICD-10 CODE C90.00

The Clinical Syndrome Multiple myeloma is an uncommon cause of back pain that is frequently initially misdiagnosed. It is a unique disease in that it may cause pain via several mechanisms that can act alone or in concert. These mechanisms include invasion or compression of pain-sensitive structures (1) by the tumor itself, (2) by the products that the tumor produces, and (3) by the host response to the tumor and its products. Although the exact cause of multiple myeloma is unknown, the following facts have been elucidated. There seems to be a genetic predisposition to the development of myeloma. It also is known that exposure to radiation increases the incidence of the disease, as witnessed in survivors of the nuclear bombs used in World War II. RNA viruses also have been implicated in the evolution of multiple myeloma. There also appear to be abnormalities in signaling pathways. The disease is rare in individuals younger than 40 years, with a median age of diagnosis of 60 years. A male gender predilection is seen, and blacks have twice the incidence of multiple myeloma compared with whites. Worldwide, the incidence of multiple myeloma is 3 per 100,000 people. The most common clinical presentation of multiple myeloma is back and rib pain. It occurs in more than 70% of patients ultimately diagnosed with the disease. These bone lesions are osteolytic and are best diagnosed with plain radiography rather than with radionucleotide bone scanning. Pain with movement is common, and hypercalcemia occurs with sufficient frequency to be the presenting symptom in many patients with multiple myeloma. Lifethreatening infection, anemia, bleeding, and renal failure are often present in conjunction with the symptoms of pain. Hyperviscosity of the serum that is the result of the products of tumor production may lead to cerebrovascular accidents.

Signs and Symptoms Pain is the most common clinical symptom that ultimately leads the clinician to the diagnosis of multiple myeloma (Fig. 80.1). Seemingly minor trauma may cause pathological vertebral compression or rib fractures. Pain on movement of the affected bones is a common finding on physical examination, as is the finding of tumor mass on palpation of the skull and other affected bones. Neurological findings, either based on neural compression secondary to tumor or fracture or as a result of cerebrovascular accident, are often present. Positive Trousseau and Chvostek signs secondary to hypercalcemia also may be elicited. Anasarca resulting from renal failure, if present, is an ominous prognostic sign.

FIG. 80.1 Pain is the most common clinical symptom that ultimately leads to the diagnosis of multiple myeloma.

FIG. 80.2 (A) Peripheral blood smear showing erythrocyte rouleaux formation; (B) skull x-ray (lateral view) showing multiple punched-out lytic lesions. From Dalfardi B. A man with generalized weakness. Eur J Intern Med. 2018;51:e7– e8.

Testing The presence of Bence Jones protein in the urine, anemia, and increased M protein on serum protein electrophoresis point strongly to the diagnosis of multiple myeloma. Peripheral blood smears will often reveal erythrocyte rouleaux formation (Fig. 80.2). Classic punched-out bone lesions in the skull and spine on plain radiographs are pathognomonic for the disease (Fig. 80.3). Because little osteoclastic activity is present in patients with multiple myeloma, radionucleotide bone scanning can be negative in the face of diffuse bony destruction. Magnetic resonance imaging (MRI) is indicated in any patient thought to have multiple myeloma who exhibits signs of spinal cord compression. Serum creatine testing and automated blood chemistries that include serum calcium determinations are indicated in all patients with multiple myeloma.

Differential Diagnosis A variety of other abnormalities of the bone marrow, including the heavy chain diseases and Waldenström macroglobulinemia, can mimic the clinical presentation of multiple myeloma. Amyloidosis also shares many common clinical signs and symptoms. Metastatic disease from prostate and breast cancer can produce pathological fractures of the spine and ribs and calvarial metastases that may be mistaken for multiple myeloma. A small group of patients have benign monoclonal gammopathy, which in most patients requires no therapy but can mimic the laboratory findings of multiple myeloma.

Treatment Management of multiple myeloma is aimed at the treatment of progressive bone lesions and reduction in serum myeloma proteins. These goals are accomplished with radiation therapy and chemotherapy, alone or in combination. High-dose pulsed steroids have been shown to provide symptomatic relief and to extend life expectancy in patients with multiple myeloma. Initial treatment of the pain associated with multiple myeloma should include either nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors. It may be necessary to add opioid analgesics to control the more severe pain of pathological fractures. Orthotic devices such as the Cash brace and rib belts may help stabilize the spine and ribs and should be considered in the presence of pathological fractures. Local application of heat and cold may be beneficial. The repetitive movements that incite the syndrome should be avoided. For patients who do not respond to these treatment modalities, injection of the affected areas with a local anesthetic and steroid using either intercostal or epidural nerve blocks is a reasonable next step. Spinal administration of opioids may be beneficial in selected cases. Ultimately, radiation therapy is often required to provide adequate pain control if significant bony involvement occurs. Stem cell transplant and gene therapy are emerging as exciting new options in the treatment of this disease.

Complications and Pitfalls Approximately 15% of patients with multiple myeloma die within the first 3 months after diagnosis despite aggressive treatment. An additional 15% of patients die each successive year. The most common causes of death are renal failure, overwhelming sepsis, hypercalcemia, bleeding, the development of acute leukemia, and stroke. Nonfatal complications such as pathological fractures can make life for patients with multiple myeloma quite difficult. Failure to recognize and treat these complications of multiple myeloma early can increase patient suffering and hasten death.

Clinical Pearls Careful evaluation of patients with the triad of proteinuria, spine or rib pain, and abnormal serum protein electrophoresis is mandatory to help avoid the inevitable complications of a delayed diagnosis of multiple myeloma. The clinician and patient must understand that despite early treatment, most patients with multiple myeloma die within 2–5 years of the time the disease is diagnosed. Epidural and intercostal injection of local anesthetics and steroids can provide excellent palliation of the pain associated with multiple myeloma.

FIG. 80.3 Elderly Patient With Low Back Pain.Anteroposterior (A) and lateral (B) radiographs show a presumed insufficiency fracture of L2 with minor end-plate collapse of L3. The sagittal T1-weighted (C), T2-weighted (D), and short tau inversion recovery (STIR) (E) magnetic resonance images acquired a few months later show multilevel vertebral fractures. Diffuse abnormalities of the bone marrow are seen, with a generally patchy appearance and some rounded areas of high signal intensity on the T2-weighted and STIR images. The appearances are strongly suspicious for disorders such as plasma cell dyscrasias and other reticuloendothelial disorders. Immunoglobulin testing yielded results positive for myeloma, and a subsequent skeletal survey showed lytic lesions in the skull (F) typical of multiple myeloma. From Waldman SD, Campbell RSD, eds. Imaging of Pain. Philadelphia, PA: Saunders; 2011:192.

Suggested Readings Ailawadhi S, Patel R, Meghji Z, Cochuyt J, Hodge D, Vivek R. Characteristics, time trend and outcomes of patients undergoing autologous stem cell transplant for multiple myeloma. Biol Blood Marrow Transplant. 2018;24(3):S140–S141. Andres M, Feller A, Arndt V. Trends of incidence, mortality, and survival of multiple myeloma in Switzerland between 1994 and 2013. Cancer Epidemiol. 2018;53:105–110. Hu J, Hu WX. Targeting signaling pathways in multiple myeloma: pathogenesis and implication for treatments. Cancer Lett. 2018;414:214–221. Vangsted A.J, Helm-Petersen S, Cowland J.B, et al. Drug response prediction in high-risk multiple myeloma. Gene. 2018;644:80–86. Willrich MAV, Murray DL, Kyle RA. Laboratory testing for monoclonal gammopathies: focus on monoclonal gammopathy of undetermined significance and smoldering multiple myeloma. Clin Biochem. 2018;51:38– 47.

81

Foix-Alajouanine Syndrome

Abstract Foix-Alajouanine syndrome is an uncommon cause of lower thoracic and lumbosacral pain. It is caused by an arteriovenous malformation of the lower thoracic and lumbosacral spinal cord, with the grey matter of the spinal cord more severely compromised when compared with the white matter. Also known as subacute necrotizing myelopathy, angiodysgenetic necrotizing myelopathy, and venous congestive myelopathy. Foix-Alajouanine syndrome is extremely rare with an incidence of four to five occurrences per 1 million patients.

Keywords arteriovenous malformation; cauda equine syndrome; cauda equine syndrome; computed tomography; epidural block; Foix-Alajouanine; lumbar myelopathy; lumbar radiculopathy; lumbar spine; lumbar vertebra; magnetic resonance imaging; paraplegia; pseudoclaudication; spinal cord infarction; vascular myelopathy

ICD-10 CODE G95.19

The Clinical Syndrome Foix-Alajouanine syndrome is an uncommon cause of lower thoracic and lumbosacral pain. It is caused by an arteriovenous malformation of the lower thoracic and lumbosacral spinal cord, with the grey matter of the spinal cord more severely compromised when compared with the white matter. Also known as subacute necrotizing myelopathy, angiodysgenetic necrotizing myelopathy, and venous congestive myelopathy. Foix-Alajouanine syndrome is extremely rare with an incidence of four to five occurrences per 1 million patients. The etiopathology of Foix-Alajouanine syndrome is not fully understood, but it has been postulated that intradural arteriovenous fistulas allow higher arterial pressure from dural arteries to be transmitted to the intradural veins (Fig. 81.1). Over time, this chronic increase in the intradural venous pressure leads to compromised perfusion of the spinal cord and ultimately spinal cord infarction. This uncommon cause of pain and spinal cord compromise is usually diagnosed in the fifth decade, although the initial case reports by Foix and Alajouanine described two patients in their late twenties. Foix-Alajouanine syndrome occurs five times more commonly in males.

Signs and Symptoms Patients with Foix-Alajouanine syndrome report the gradual onset nonradiating back and sacrococcygeal pain with associated lower extremity weakness, dysesthesias, and numbness. Heaviness of the lower extremities as is seen in patients suffering from spinal stenosis may occur after walking a short distance. This pseudoclaudication improves with rest. As the spinal cord compromise continues, bowel, bladder, and sexual dysfunction may occur. Gait abnormalities are not uncommon. On physical examination, central cognitive functions are preserved and examination of the lumbosacral spine is unremarkable. An unsteady halting, rather than antalgic, gait is seen, and increased deep tendon reflexes, clonus, and Babinski signs will be present as the spinal cord compromise progresses (Fig. 81.2). With spinal cord infarction, sensory and motor deficit below the level of infarction is easily identified. Paraparesis to complete paraplegia is usually the final result of cord infarction.

Testing Because the neurological symptoms of vitamin B12 deficiency, Lyme disease, syphilis myelopathy secondary to human immunodeficiency and T-cell leukemia virus presents in a similar manner to Foix-Alajouanine syndrome, laboratory testing to measure serum B12 levels as well as testing for syphilis and human immunodeficiency and T-cell leukemia virus should be immediately undertaken. Magnetic resonance imaging and magnetic resonance angiography of the spinal cord are indicated, although these tests as well as computed tomographic myelography may be nondiagnostic early in the course of the disease. With disease progression, on magnetic resonance imaging, hyperintense central spinal cord lesions are readily identifiable with spinal cord edema and decreased signal intensity identified on T1-weighted sequences. With contrast administration, serpentine vascular enhancement as well as vessel enlargement with associated flow void phenomena within the subarachnoid space are often identified (Fig. 81.3). Selective spinal angiography remains the gold standard for diagnosis as this imaging modality can best identify the extent of spinal-dural arteriovenous fistulas and may help identify specific offending arteries and veins (Fig. 81.4). Somatosensory evoked potential testing can help identify the presence and localize the level of spinal cord compromise, and electromyography and nerve conduction testing can help identify motor neuron disease such as amyotrophic lateral sclerosis.

FIG. 81.1 Artist’s Interpretation of a Dural Arteriovenous Fistula and Associated Vascular MetamorphosisBlood is diverted through the fistula under high pressure (a) into the arterialized medullary vein and results in vascular changes along three major axes: axial length (b), circumference (c), and radius (d). Macroscopically, these changes are translated into elongation, dilation, and tortuosity of the vessels of the coronal venous plexus, which is herein visualized. From Lagman C, Chung LK, Chitale RV, et al. Dural arteriovenous fistula and FoixAlajouanine syndrome: assessment of functional scores with review of pathogenesis. World Neurosurg. 2017;106:206–210.

FIG. 81.2 Foix-Alajouanine Syndrome.

Differential Diagnosis In its early stages, Foix-Alajouanine syndrome is difficult to diagnose on clinical grounds alone as its presentation mimics many much more common causes of lumbosacral pain and lower extremity pain and dysfunction (Box 81.1). Early in the course of the disease, the patient’s clinical symptomatology is often attributed to lumbar disc disease, spinal stenosis, cervical disc disease, spinal cord tumors, arachnoiditis, or lumbar spondylosis and treated as such (Fig. 81.5). As the disease progresses, spinal cord tumors, anterior horn cell disorders such as amyotrophic lateral sclerosis, poliomyelitis/postpolio syndrome, and polyradiculopathies such as GuillainBarré disease are often diagnosed. As the upper motor neurons and the sacral segments are affected, the diagnosis becomes more obvious, but given its rarity, Foix-Alajouanine syndrome remains a diagnosis of exclusion and one that is confirmed on radiographic grounds. As mentioned previously, human immunodeficiency virus, syphilis, human T-cell leukemia virus infection, and vitamin B12 deficiency can present in a similar manner to FoixAlajouanine syndrome.

FIG. 81.3 T2-Weighted Spinal Cord MRI Centered on the Lumbosacral Region: Sagittal Slices.Centromedullary edema of the conus medullaris (arrow). Dilated perimedullary vessels, visible as flow voids with T2-weighting (arrowheads). From Amanieu C, Hermier M, Peyron N, et al. Spinal dural arteriovenous fistula. Diagn Interv Imaging. 2014;95[9]:897–902.

Treatment Foix-Alajouanine syndrome is best treated by closure of dural arteriovenous fistulas using either surgical ligation of the vein draining the fistula or endovascular embolization with liquid polymers such as isobutyl 2cyanoacrylate or n-butyl 2-cyanoacrylate. The use of intraoperative Doppler may improve surgical results. Postoperatively, dexamethasone may improve neurological deficits due to spinal cord edema. An aggressive rehabilitation program with a multimodality approach to optimize function is always indicated. Neuropathic pain can be treated with gabapentin, pregabalin antidepressants, and anticonvulsants. Heat modalities and deep sedative massage may help decrease aching lower extremity pain. Underlying sleep disturbance and depression are best treated with a tricyclic antidepressant compound, such as nortriptyline, which can be started as a single bedtime dose of 25 mg.

Complications and Pitfalls Failure to diagnose Foix-Alajouanine syndrome early in the course of the disease worsens patient outcome. The early use of magnetic resonance imaging and selective angiography in any patient with pseudoclaudication of the lower extremities in the absence of spinal stenosis or in whom neurological findings seem out of proportion to the pain complaint will speed diagnosis and treatment.

Clinical Pearls The diagnosis of Foix-Alajouanine syndrome should be considered in any patient reporting back pain, symptoms of pseudoclaudication, and lower extremity weakness. Imaging of the spine and its contents should be done earlier rather than later. After definitive treatment with surgical ligation or embolization, physical therapy to improve function is crucial to improve outcomes.

FIG. 81.4 (A and B) T2-weighted magnetic resonance imaging (inset: axial view at T10) and magnetic resonance angiogram in a 31-year-old male with deteriorating

lower limb weakness showing a T9-10 spinal arteriovenous fistula (