Textbook of Paediatric Emergency Medicine [3rd ed.] 9780702073038, 9780702073045

Table of contents :
Title page......Page 2
Table of Contents......Page 4
Copyright......Page 57
Preface to third edition......Page 59
Preface to second edition......Page 61
Contributors......Page 63
Acknowledgements......Page 76
Section 1. Approach to the Paediatric Patient......Page 77
1.1. Approach to the paediatric patient......Page 78
Introduction......Page 79
Children with fever......Page 81
Evolving illness in children......Page 82
The environment......Page 83
Triage......Page 84
Front loading care......Page 85
The paediatric approach......Page 86
History......Page 88
Examination......Page 90
Gentle, distraction, painful last......Page 92
The parents......Page 97
Management of febrile children......Page 99
The role of the GP in paediatric emergency management......Page 103
Reflection on the Practice of Paediatric Emergency......Page 109
Introduction......Page 113
Cerebral palsy......Page 115
Cystic fibrosis......Page 122
The ex-premature infant......Page 128
Autism spectrum disorder......Page 130
Section 2. Resuscitation......Page 134
2.1. Paediatric cardiopulmonary arrest......Page 135
Aetiology......Page 136
Preventing cardiac arrest......Page 137
Differences compared to adults......Page 138
Ethics of paediatric resuscitation......Page 139
2.2. Paediatric basic life support......Page 146
Paediatric versus adult basic life support......Page 147
Basic life support sequence......Page 148
Relief of foreign body airway obstruction......Page 161
2.3. Paediatric advanced life support......Page 167
Diagnosing cardiac arrest......Page 168
Oxygen, ventilation and advanced airway support......Page 169
Ventilation......Page 171
Advanced airway support......Page 174
Laryngeal mask airway......Page 177
Management of the difficult airway......Page 178
Monitoring......Page 183
Vascular access......Page 184
Fluid therapy......Page 186
Management of pulseless arrhythmias......Page 191
Management of pulsatile dysrhythmias......Page 194
Post-resuscitation management......Page 197
Cessation of cardiopulmonary resuscitation......Page 199
Avoiding cardiac arrest during critical care management......Page 202
Asthma......Page 203
Drowning......Page 204
Toxicological emergencies......Page 205
Envenomation......Page 207
Marine envenomation......Page 208
Introduction......Page 210
Diagnosis and assessment......Page 212
Initial management......Page 214
Further management......Page 216
Introduction......Page 221
Background......Page 222
Definition......Page 223
Diagnosis......Page 224
Initial emergency management......Page 225
Disposition......Page 227
Section 3. Neonatal Emergencies......Page 228
Definition and introduction......Page 229
Common reasons for healthy neonates to present to the emergency department......Page 233
Summary......Page 236
Introduction......Page 237
Recurrent crying......Page 238
Acute crying......Page 240
Disposition......Page 241
Skin in the Neonatal Period......Page 243
Neonatal erythroderma......Page 245
Red scaly rashes......Page 247
Vesicles and blisters......Page 250
Pustular lesions......Page 255
Birthmarks......Page 257
Blue/purple lesions......Page 260
Vascular lesions in the neonatal period......Page 262
3.4. Acute neonatal emergencies......Page 264
Neonatal resuscitation......Page 265
Assessment of the neonate......Page 266
The neonate with vomiting......Page 274
The neonate with seizures......Page 278
The neonate with breathing difficulty......Page 281
The neonate with prolonged jaundice......Page 285
3.5. Neonatal resuscitation......Page 291
Aetiology and pathophysiology......Page 292
Preparation......Page 293
Ventilation......Page 294
Heart rate......Page 296
Colour......Page 297
Medications......Page 298
Specific resuscitation situations......Page 301
Post-resuscitation stabilisation......Page 303
Prognosis......Page 305
Section 4. Trauma in Children......Page 308
4.1. Introduction to paediatric trauma......Page 309
Prevention......Page 310
Succinct treatment (salvage)......Page 311
Primary survey......Page 312
Paediatric differences......Page 313
Other issues during initial stabilisation......Page 317
Secondary survey......Page 318
Orthopaedic trauma......Page 319
Rehabilitation......Page 321
4.2. Paediatric neurotrauma......Page 323
Pathophysiology......Page 324
Classification......Page 326
Assessment......Page 328
4.3. Spinal injury......Page 350
Developmental anatomy and physiology......Page 351
Initial assessment......Page 352
Spinal immobilisation......Page 353
Cervical spine injuries......Page 356
Thoracic and lumbar spine injuries......Page 367
Spinal cord injury......Page 372
Spinal cord injury without radiographic abnormality......Page 375
4.4. Thoracic injuries in childhood......Page 380
Initial approach in the emergency department......Page 381
Chest wall injury......Page 383
Pulmonary injury......Page 384
Tracheobronchial injuries......Page 388
Mediastinal injury......Page 389
Cardiac injuries......Page 390
Diaphragmatic injury......Page 391
Emergency department thoracotomy......Page 392
Introduction......Page 395
Examination......Page 397
Investigations......Page 400
General management......Page 403
Surgical issues......Page 404
Hollow viscus injuries......Page 405
Pancreatic and renal injuries......Page 406
Pelvic fractures......Page 407
Disposition......Page 409
Introduction......Page 412
Pathophysiology......Page 413
Classification......Page 414
History......Page 416
Examination......Page 417
Management......Page 418
Management of burns......Page 421
Electrical burns......Page 424
Chemical burns......Page 427
Introduction......Page 429
Phases of disaster response......Page 430
4.8. Wound management......Page 434
Introduction......Page 435
Classification of wounds......Page 436
Evaluation of the patient with a laceration......Page 437
Treatment of wounds......Page 440
Wound closure......Page 444
Post-wound-closure care......Page 456
Treatment of selected injuries......Page 458
Section 5. Cardiovascular......Page 467
Introduction......Page 468
History......Page 469
Physical examination......Page 470
Chest X-ray......Page 471
Pathological murmurs......Page 478
Disposition......Page 479
Introduction......Page 480
Immediate approach......Page 481
General approach......Page 482
Summary......Page 485
Aetiology......Page 486
Typical presentations......Page 487
Clinical......Page 490
Investigations within the emergency department......Page 493
Further investigations of syncope......Page 494
Summary......Page 495
5.4. Cyanotic heart disease and tetralogy of Fallot spells......Page 497
Cyanotic congenital heart disease......Page 498
Clinical features......Page 499
Investigations......Page 500
Management......Page 501
Tetralogy Spells......Page 502
Investigations......Page 503
Disposition......Page 504
Definition......Page 507
Clinical manifestations and investigations......Page 508
Management......Page 511
Introduction......Page 516
Undiagnosed congenital heart disease......Page 517
Complications/residua of congenital heart disease and its treatment......Page 525
Congenital heart disease and intercurrent illness......Page 528
Introduction......Page 530
Investigations......Page 535
Treatment......Page 537
Prevention and prophylaxis......Page 538
Introduction......Page 541
Microbiology......Page 542
Modified Duke criteria......Page 543
Investigations......Page 544
Treatment......Page 545
Prevention......Page 546
Introduction......Page 550
Pathophysiology......Page 551
Clinical features......Page 552
Differential diagnosis......Page 553
Investigations......Page 554
Treatment......Page 555
Prognosis......Page 557
5.10. Cardiac arrhythmias......Page 561
Normal conduction system......Page 562
The cardiac action potential......Page 563
Pathogenesis of arrhythmias......Page 567
General principles for arrhythmia management......Page 569
Role of ‘molecular autopsy’ in sudden unexplained cardiac death in the young......Page 578
Section 6. Respiratory......Page 581
Introduction......Page 582
History......Page 583
Common causes of acute stridor in children......Page 584
Common causes of chronic stridor in children......Page 586
Introduction......Page 590
Nasopharyngitis......Page 591
Stomatitis......Page 593
Pharyngitis/tonsillitis......Page 595
Introduction......Page 598
Upper airway foreign bodies......Page 599
Treatment......Page 600
Lower airway foreign body......Page 601
Prevention......Page 603
Introduction......Page 604
Presentation......Page 605
Investigations......Page 607
Differential diagnosis......Page 608
Treatment and disposition......Page 609
Prognosis......Page 611
Prevention......Page 612
Introduction......Page 614
Diagnosis of asthma......Page 615
Risk factors for mortality......Page 617
Clinical assessment......Page 618
Treatment......Page 619
Discharge from hospital......Page 622
Prevention......Page 624
Future directions/research......Page 629
Introduction......Page 633
History......Page 634
Examination......Page 635
Treatment......Page 636
Prognosis......Page 638
Introduction......Page 640
Aetiology......Page 641
Clinical findings......Page 642
Investigations......Page 643
Management......Page 644
Prevention......Page 645
Conclusion......Page 646
6.8. Bronchiolitis......Page 649
Introduction......Page 650
Clinical assessment......Page 651
Treatment......Page 654
Prognosis......Page 656
Prevention......Page 657
Section 7. Gastroenterology and Hepatology......Page 660
7.1. Abdominal pain......Page 661
Pathophysiology......Page 662
Assessment......Page 663
History......Page 666
Examination......Page 668
Investigations......Page 670
Management......Page 671
Acute Appendicitis......Page 672
Differential diagnoses......Page 673
Investigations......Page 674
Management......Page 675
Differential diagnoses......Page 676
Chronic Abdominal Pain......Page 677
Management......Page 678
Non-Surgical Vomiting......Page 683
Definitions......Page 684
Clinical evaluation......Page 685
Examination......Page 686
Differential diagnoses......Page 688
Investigations......Page 689
Management......Page 692
Surgical (Bilious) Vomiting......Page 694
Causes......Page 695
Complications......Page 698
Treatment......Page 699
Introduction......Page 703
Aetiology......Page 704
History......Page 705
Examination......Page 706
Investigations......Page 708
Initial medical therapy......Page 710
Treatment......Page 712
Dispositon......Page 714
Introduction......Page 717
History......Page 718
Complications......Page 719
Investigations......Page 720
Treatment......Page 721
Follow-up......Page 723
Introduction......Page 725
Clinical presentation......Page 726
Examination findings......Page 727
Imaging studies......Page 728
Management......Page 729
Complications......Page 730
Introduction......Page 733
History......Page 734
Investigations......Page 735
Treatment......Page 737
Prevention......Page 742
Introduction......Page 746
Aetiology......Page 748
Pathophysiology......Page 753
Presentation......Page 754
Investigations......Page 755
Management......Page 756
Disposition......Page 758
Prevention......Page 759
Definitions......Page 764
Clinical evaluation......Page 765
Examination......Page 766
Differential diagnoses......Page 768
Investigations......Page 769
Management......Page 771
Conclusions......Page 772
Introduction......Page 774
Examination......Page 775
Investigations......Page 776
Viral hepatitis......Page 778
Drug- and toxin-induced liver injury......Page 779
Chronic liver disease presenting as acute hepatitis......Page 780
Other causes of chronic liver disease presenting in childhood......Page 781
Introduction......Page 782
Clinical......Page 783
Investigations......Page 785
Management......Page 786
Outcome......Page 787
Introduction......Page 791
Types of herniae......Page 792
Complications......Page 793
Treatment......Page 794
7.12. Gastroenteritis......Page 797
History......Page 798
Examination......Page 800
Differential diagnosis......Page 802
Investigations......Page 803
Treatment......Page 804
Other treatments......Page 809
Prognosis......Page 811
7.13. Constipation......Page 816
Pathophysiology......Page 817
Management basics......Page 818
Constipation in babies......Page 819
Acute constipation......Page 820
Management......Page 821
Medications......Page 822
Introduction......Page 828
New diagnoses......Page 829
Known IBD patients......Page 833
Common treatments and their complications......Page 836
Section 8. Neurology......Page 838
Introduction......Page 839
Clinical presentation......Page 841
Examination......Page 844
Investigations......Page 845
Introduction......Page 852
Particular issues in children......Page 855
Clinical features of raised intracranial pressure......Page 856
Other examination findings in raised intracranial pressure......Page 859
Investigations......Page 860
Some particular causes of raised intracranial pressure......Page 863
Introduction......Page 866
Classification of seizures......Page 867
Febrile seizures......Page 868
Presentation post a possible seizure......Page 869
Presentation of a seizure......Page 875
8.4. Acute weakness......Page 881
Primary survey approach......Page 882
History......Page 884
Examination......Page 885
Investigations......Page 886
Specific conditions causing acute weakness......Page 887
Introduction......Page 904
Pathophysiology......Page 905
Differential diagnosis......Page 907
Chronic ataxia......Page 914
Clinical evaluation of the patient......Page 916
Examination......Page 917
Investigations......Page 918
Disposition......Page 919
Introduction......Page 923
Pathophysiology......Page 924
Clinical assessment......Page 927
Disposition......Page 934
Pathophysiology......Page 935
Clinical features......Page 936
Treatment......Page 937
Conclusions......Page 938
8.7. Central nervous system infections: Meningitis and encephalitis......Page 943
Meningitis......Page 944
Encephalitis......Page 952
Conclusion......Page 954
Section 9. Infectious Diseases......Page 958
9.1. Infectious diseases......Page 959
Fever......Page 960
Empiric antibiotic therapy......Page 965
Common infectious exanthems......Page 967
What specimens, when should they be ordered and what tests?......Page 968
Needlestick injury......Page 973
Immunisation......Page 974
Section 10. Metabolic Emergencies......Page 978
Introduction......Page 979
Physiology and pathogenesis......Page 980
Clinical features......Page 981
Investigation......Page 982
Management......Page 984
Conclusion......Page 989
Introduction......Page 990
Causes of hypoglycaemia......Page 991
Treatment of hypoglycaemia......Page 994
Diabetic ketoacidosis......Page 996
Hypoglycaemia......Page 1001
The child with an insulin pump......Page 1002
The child with diabetes and intercurrent illness......Page 1003
Long-term management......Page 1005
Thyrotoxicosis......Page 1007
Neonatal thyrotoxicosis......Page 1009
Congenital hypothyroidism......Page 1010
Hashimoto’s thyroiditis......Page 1011
10.5. Adrenal emergencies......Page 1013
Adrenal Crisis......Page 1014
Clinical presentation......Page 1015
Cushing’s Syndrome......Page 1018
Presenting symptoms......Page 1019
Diagnostic testing......Page 1020
Treatment......Page 1021
10.6. Disorders of fluids, electrolytes and acid–base......Page 1023
Physiology......Page 1024
Clinical assessment......Page 1025
Investigations......Page 1029
Treatment......Page 1030
Investigation and management of fluids in different conditions......Page 1033
Acid–base disorders......Page 1042
Section 11. Haematology and oncology......Page 1050
11.1. The use of blood products in children......Page 1051
Introduction......Page 1052
Packed red blood cells......Page 1054
Platelets......Page 1056
Fresh frozen plasma......Page 1057
Albumin......Page 1058
Normal human immunoglobulin......Page 1059
Risks of blood component use......Page 1060
11.2. Anaemia......Page 1064
Introduction......Page 1065
Acute management......Page 1066
Neonatal anaemia......Page 1071
Anaemias of childhood......Page 1074
Haemolytic anaemias......Page 1076
Haemophilia......Page 1088
von Willebrand disease......Page 1091
Introduction......Page 1094
Immune thrombocytopaenia......Page 1095
Chronic immune thrombocytopaenia......Page 1099
Introduction......Page 1101
Anti-cytoplasmic-antibody-associated vasculitis......Page 1103
Medium-Vessel Vasculitis......Page 1106
Polyarthritis nodosa......Page 1107
Large-Vessel Vasculitis......Page 1108
Secondary Vasculitis and Vasculitis Mimics......Page 1109
Introduction......Page 1113
Clinical presentation......Page 1114
Investigations......Page 1115
Prognosis......Page 1116
Complications......Page 1117
Management......Page 1118
Introduction......Page 1120
Presentation......Page 1121
Investigations......Page 1122
Treatment......Page 1123
11.8. Emergencies in paediatric oncology......Page 1126
Fever and infection......Page 1127
Gastrointestinal emergencies......Page 1128
Blood product use in oncology......Page 1131
Cardiothoracic emergencies......Page 1132
Metabolic emergencies......Page 1135
Genitourinary emergencies......Page 1138
Neurological emergencies......Page 1139
Brain and Spinal Cord Tumours in Children......Page 1141
Brain tumours......Page 1142
Spinal cord compression......Page 1143
Section 12. Dermatology......Page 1146
Introduction......Page 1147
Erythroderma and skin failure......Page 1149
Vesiculobullous rashes......Page 1156
Pustular rashes......Page 1177
Papular (raised) rashes......Page 1184
Red scaly (papulosquamous) rashes......Page 1194
Eczematous rashes......Page 1202
Red blanching rashes (erythematous)......Page 1213
Purpuric rashes......Page 1228
Vascular tumours – haemangiomas and haemangioma variants......Page 1236
Vascular malformations......Page 1241
Hyperpigmentation......Page 1245
Hypopigmentation......Page 1252
Skin texture......Page 1254
Mouth disorders......Page 1255
Anogenital rashes......Page 1258
Zinc and other nutritional deficiencies......Page 1263
Immunodeficiency states......Page 1265
Hair problems......Page 1266
Nail problems......Page 1270
Itch without rash......Page 1272
Section 13. Eyes......Page 1275
Introduction......Page 1276
Examination......Page 1277
The red eye in paediatrics......Page 1279
Strabismus......Page 1288
Ocular tumours......Page 1289
Neuroblastoma......Page 1290
Introduction......Page 1292
Globe trauma......Page 1294
Eyelid trauma......Page 1298
Orbital trauma......Page 1300
Section 14. Ent and Dental......Page 1303
14.1. The ear......Page 1304
Otitis externa......Page 1305
Acute otitis media......Page 1307
Discharging otitis media – chronic suppurative otitis media......Page 1309
Otitis media with effusion......Page 1310
Mastoiditis......Page 1311
Trauma......Page 1313
14.2. The nose......Page 1317
Rhinitis and sinusitis......Page 1318
Epistaxis......Page 1321
Nasal trauma......Page 1322
14.3. The mouth and throat......Page 1326
Stomatitis......Page 1327
Differential diagnosis......Page 1328
Pharyngitis/tonsillitis......Page 1329
Peritonsillar abscess......Page 1331
Post-tonsillectomy haemorrhage......Page 1332
Oral/dental trauma......Page 1334
Oral/dental infection......Page 1337
Other dental issues......Page 1338
Introduction......Page 1341
Investigation......Page 1342
Treatment......Page 1343
Nasal foreign bodies......Page 1345
Aural foreign bodies......Page 1347
Caustic ingestion......Page 1349
Section 15. Obstetrics and Gynaecology......Page 1353
15.1. Paediatric gynaecology......Page 1354
Infant and prepubescent gynaecology......Page 1355
Adolescent gynaecology......Page 1363
Introduction......Page 1376
Available medicines......Page 1377
Medicine contraindications......Page 1378
Copper intrauterine devices......Page 1379
Section 16. Renal......Page 1382
Introduction......Page 1383
Clinical presentation......Page 1386
Treatment......Page 1387
Acute presentation of chronic renal failure......Page 1390
Acute kidney injury in the renal transplant recipient......Page 1392
16.2. Haematuria......Page 1395
History......Page 1396
Investigation......Page 1397
Disposition......Page 1398
Introduction......Page 1399
Examination......Page 1400
Emergency department management......Page 1401
Introduction......Page 1404
History and examination......Page 1405
Treatment......Page 1407
Prognosis......Page 1410
Prevention......Page 1411
Introduction......Page 1413
Definition of haemolytic uraemic syndrome......Page 1414
Pathophysiology of haemolytic uraemic syndrome......Page 1415
Investigations......Page 1417
Differential diagnosis......Page 1418
Treatment......Page 1419
Prognosis......Page 1421
Prevention......Page 1422
Pathophysiology of proteinuria......Page 1424
Mechanisms of proteinuria......Page 1426
Definition of nephrotic syndrome......Page 1428
Classification of paediatric nephrotic syndrome......Page 1429
Idiopathic nephrotic syndrome: epidemiology......Page 1434
Clinical features of idiopathic nephrotic syndrome......Page 1439
Investigations......Page 1440
Complications of idiopathic nephrotic syndrome......Page 1443
Treatment of initial nephrotic phase......Page 1446
Prognosis......Page 1450
Introduction......Page 1452
Pathogenesis......Page 1453
Clinical features......Page 1457
Diagnosis and investigation......Page 1460
Differential diagnosis......Page 1461
Treatment......Page 1463
Long-term prognosis and follow-up......Page 1464
Section 17. Psychiatric......Page 1466
General approach......Page 1467
Suicidal patients......Page 1469
Anxiety disorders......Page 1475
Psychosis......Page 1476
Other psychiatric presentations......Page 1478
Acknowledgement......Page 1479
Introduction......Page 1481
The principles of psychiatric triage......Page 1482
The ABCC of rapid psychiatric assessment......Page 1486
Managing the ABCC including restraint and acute sedation......Page 1488
Conclusion......Page 1497
17.3. Autism and behavioural disturbance in the pre-adolescent child......Page 1499
Introduction......Page 1500
Approach to assessment......Page 1501
Approach to management......Page 1504
Management......Page 1505
Conclusion......Page 1508
Section 18. Crisis Intervention......Page 1513
Introduction......Page 1514
Attitudes/myths surrounding child sexual assault......Page 1515
Epidemiology of child sexual assault......Page 1516
Signs and symptoms......Page 1517
Diagnostic considerations......Page 1519
Role of the emergency physician......Page 1520
Introduction......Page 1523
Definition......Page 1524
The community response to the child at risk......Page 1532
Section 19. Administration In EMS......Page 1535
19.1. Managing the death of a child in the emergency department: Bereavement issues......Page 1536
Introduction......Page 1537
The resuscitation process......Page 1539
Talking to parents and families......Page 1540
Laying out of the child......Page 1542
The grief response......Page 1543
Cultural implications......Page 1545
Legal issues......Page 1546
Organ and tissue donation and collection......Page 1547
Debriefing and support for emergency department staff......Page 1548
The concept of a good death......Page 1549
Conclusion......Page 1550
Section 20. Analgesia and Sedation......Page 1555
Introduction......Page 1556
Management......Page 1558
Non-pharmacological methods......Page 1559
Pharmacological methods......Page 1560
Introduction......Page 1581
Pre-procedure......Page 1582
Intra-procedure......Page 1586
Post-procedure......Page 1587
Non-pharmacological methods......Page 1588
Pharmacological methods......Page 1590
Section 21. Poisoning......Page 1600
21.1. General approach to poisoning......Page 1601
Diagnosis......Page 1602
Risk assessment......Page 1604
Investigations......Page 1605
Decontamination......Page 1606
Antidotes......Page 1608
Supportive care......Page 1609
Consultation and disposition......Page 1610
Common poisons......Page 1612
Rare and dangerous poisons......Page 1622
Section 22. Environmental......Page 1639
22.1. Envenomation......Page 1640
Snakebite......Page 1641
Scorpion stings......Page 1668
Spiderbite......Page 1672
Tick bite paralysis......Page 1680
Jellyfish stings......Page 1681
Venomous fish stings......Page 1685
Venomous marine molluscs......Page 1688
Introduction......Page 1692
Epidemiology......Page 1693
Aetiology......Page 1694
Pathophysiology......Page 1695
History......Page 1697
Examination......Page 1698
Investigations......Page 1699
Treatment......Page 1700
Disposition......Page 1703
Prognosis......Page 1704
Prevention......Page 1705
Introduction......Page 1709
Causes of heat-related illness......Page 1710
Management......Page 1715
Prognosis and disposition......Page 1717
Introduction......Page 1722
Normal physiology: a review......Page 1723
Hypothermia......Page 1724
Localised cold injuries......Page 1732
Frostbite......Page 1733
Hypothermia not due to environmental causes......Page 1735
22.5. Anaphylaxis......Page 1741
Pathophysiology......Page 1742
Aetiology......Page 1743
Clinical features......Page 1744
Investigations......Page 1745
Treatment......Page 1746
Diagnosis......Page 1749
Prevention......Page 1751
Section 23. Ultrasound......Page 1755
23.1. Ultrasound......Page 1756
23.2. Diagnostic ultrasound in paediatric emergency medicine......Page 1764
Ultrasound guidance for procedures......Page 1770
23.4. Incorporating ultrasound into paediatric resuscitation......Page 1777
Section 24. Common Procedures......Page 1785
Age-based tools to estimate body weight......Page 1786
Tips......Page 1787
Background......Page 1790
Oropharyngeal and Nasopharyngeal Airways......Page 1791
Preparation......Page 1792
Procedure......Page 1794
Tips......Page 1797
Positioning......Page 1800
Procedure......Page 1801
Tips......Page 1802
Contraindications......Page 1803
Positioning......Page 1804
Procedure......Page 1805
Tips......Page 1809
Equipment......Page 1810
Procedure......Page 1811
Humidified High-Flow Nasal Prong Oxygenation......Page 1815
Procedure......Page 1816
T-Piece Ventilation Device......Page 1818
Procedure......Page 1819
Equipment......Page 1822
Contraindications......Page 1823
Tips......Page 1824
Background......Page 1826
Equipment......Page 1828
Preparation......Page 1829
Procedure......Page 1833
Tips......Page 1836
Confirmation of Intubation......Page 1839
Contraindications......Page 1841
Procedure......Page 1842
Tips......Page 1843
Background......Page 1845
Needle cricothyroidotomy......Page 1847
Surgical cricothyroidotomy......Page 1854
Needle Thoracostomy......Page 1861
Contraindications......Page 1863
Preparation......Page 1864
Complications......Page 1867
Tube Thoracostomy......Page 1868
Equipment......Page 1869
Preparation......Page 1870
Procedure......Page 1871
Complications......Page 1872
Tips......Page 1873
Three-Sided Dressing......Page 1874
Indications......Page 1877
Pericardiocentesis......Page 1878
Indications......Page 1879
Equipment......Page 1880
Standard preparation......Page 1881
Procedure......Page 1884
Tips......Page 1885
Background......Page 1887
Equipment......Page 1888
Preparation......Page 1889
Procedure......Page 1891
Tips......Page 1895
Background......Page 1897
Peripheral venous catheter placement......Page 1899
Central venous line placement......Page 1906
Background......Page 1916
Equipment......Page 1917
Procedure......Page 1918
Complications......Page 1921
Tips......Page 1922
Indications......Page 1924
Preparation......Page 1925
Procedure......Page 1926
Complications......Page 1934
Tips......Page 1935
Background......Page 1937
Indications for synchronous cardioversion......Page 1940
Standard preparation......Page 1941
Standard procedure......Page 1942
Automated external defibrillators procedure......Page 1945
Tips......Page 1946
Background......Page 1949
Transurethral catheterisation......Page 1950
Suprapubic aspiration......Page 1956
Indications......Page 1960
Equipment......Page 1961
Preparation and positioning......Page 1962
Procedure......Page 1964
Tips......Page 1966
Introduction......Page 1969
Procedure......Page 1970
Inguinal hernias in girls......Page 1971
Complications......Page 1972
Contraindications......Page 1974
Procedures......Page 1975
Tips......Page 1978
Background......Page 1980
Percutaneous endoscopic gastrostomy displacement......Page 1982
Buried bumper......Page 1983
Gastrocolocutaneous fistula......Page 1984
Mechanical problems – blockage......Page 1985
Jejunal feeding devices......Page 1986
Granulation tissue......Page 1987
Section 25. Orthopaedics and Rheumatology......Page 1989
Introduction......Page 1990
The child with acute musculoskeletal pain or dysfunction......Page 1991
Generalised or multifocal bone/joint pain......Page 1999
Other important subacute paediatric musculoskeletal presentations......Page 2008
Acknowledgement......Page 2011
Introduction......Page 2014
History......Page 2016
Examination......Page 2019
Clinical decision making in a child with a limp......Page 2020
Specific conditions......Page 2024
Fracture patterns in childhood......Page 2046
Initial assessment and management......Page 2049
Upper limb and shoulder girdle injuries......Page 2057
Lower limb and pelvis injuries......Page 2082
Conclusions......Page 2090
Introduction......Page 2093
Adverse events in acute paediatric orthopaedics......Page 2094
Section 26. Male Genitalia......Page 2098
The acute scrotum......Page 2099
Idiopathic scrotal oedema......Page 2101
Acute hydrocele......Page 2102
Phimosis of the foreskin......Page 2103
Balanitis......Page 2104
Priapism......Page 2105
Section 27. Transport and Retrieval......Page 2108
Paediatric retrieval......Page 2109
What’s different about children?......Page 2110
Paediatric emergency referrals......Page 2111
Paediatric referral and retrieval – roles and expectations......Page 2112
Paediatric retrieval staff......Page 2113
Paediatric retrieval equipment......Page 2114
Criteria for transport......Page 2116
Degrees of urgency......Page 2118
Transport platforms......Page 2121
Stabilisation......Page 2123
Framework for communications during paediatric retrieval......Page 2124
Parents......Page 2126
Paediatric retrieval and end-of-life situations......Page 2127
Quality......Page 2128
Summary......Page 2129
Introduction......Page 2131
Challenges in the rural setting......Page 2132
Caring for the critically ill child......Page 2134
What can be done to assist care in remote environments?......Page 2136
Acknowledgement......Page 2139
Section 28. Teaching Paediatric Emergency Medicine......Page 2141
Needs of paediatric emergency medicine staff......Page 2142
Educational tools available online......Page 2143
Social media......Page 2144
Pitfalls of online content......Page 2145
Introduction......Page 2148
Desirable attitudes in paediatric emergency medicine......Page 2149
Skill set for paedicatric emergency......Page 2151
Putting knowledge into perspective......Page 2154
Helping others acquire knowledge, skills and attitudes: modes of learning......Page 2155
Learning resources......Page 2156
Conclusions......Page 2157
Section 29. Paediatric Research in the Emergency Department......Page 2159
29.1. Research in children in the emergency department......Page 2160
Research science......Page 2161
The ethics of medical research......Page 2163
The practice and governance of research......Page 2168
Implementation research: an emerging research field......Page 2172
Funding research......Page 2174
Section 30. Adolescent Medicine in the Emergency Department......Page 2179
Introduction......Page 2180
Adolescent health problems in the emergency department......Page 2181
The approach to the adolescent in the emergency department......Page 2183
Summary......Page 2188
Introduction......Page 2192
Investigations......Page 2193
Complications......Page 2195
Management......Page 2196
Prognosis......Page 2198
Index......Page 2200

Citation preview

Textbook of Paediatric Emergency Medicine THIRD EDITION

Editors

Peter Cameron, MBBS MD FACEM Professor of Emergency Medicine, The Alfred Hospital and Monash University, Melbourne, Australia

Gary Browne, MD MBBS MSpMed FRACP FACEM Professor of Emergency Medicine, The Children’s Hospital at Westmead Chair of Discipline of Emergency Medicine, The University of Sydney Head of Academic Emergency Medicine, The Children’s Hospital at Westmead, Sydney, Australia

Biswadev Mitra, MBBS MHSM PhD FACEM Professor of Emergency Medicine, The Alfred Hospital and Monash University, Melbourne, Australia

Stuart Dalziel, MBChB FRACP PhD Director of Emergency Medicine Research, The University of Auckland and Starship Children’s Hospital, Auckland, New Zealand

Simon Craig, MBBS MHPE MPH FACEM Adjunct Clinical Associate Professor, Monash University; Emergency Physician, Monash Medical Centre, Monash Health, Melbourne, Australia

For additional online content visit ExpertConsult.com

Table of Contents Cover image Title page Copyright Preface to third edition Preface to second edition Contributors Acknowledgements

Section 1. Approach to the Paediatric Patient 1.1. Approach to the paediatric patient Introduction Children with fever Evolving illness in children The environment

Triage Front loading care The paediatric approach History Examination Gentle, distraction, painful last When to investigate The parents Management of febrile children The role of the GP in paediatric emergency management Reflection on the Practice of Paediatric Emergency

1.2. Common chronic paediatric conditions Introduction Cerebral palsy Cystic fibrosis The ex-premature infant Autism spectrum disorder

Section 2. Resuscitation 2.1. Paediatric cardiopulmonary arrest Epidemiology Aetiology Preventing cardiac arrest Outcome Differences compared to adults

Development of resuscitation guidelines Ethics of paediatric resuscitation

2.2. Paediatric basic life support Introduction Paediatric versus adult basic life support Basic life support techniques and age Preparation and equipment Basic life support sequence Precautions and complications Relief of foreign body airway obstruction

2.3. Paediatric advanced life support Introduction Diagnosing cardiac arrest Epidemiology Oxygen, ventilation and advanced airway support Ventilation Advanced airway support Laryngeal mask airway Management of the difficult airway Monitoring Vascular access Fluid therapy Management of pulseless arrhythmias Management of pulsatile dysrhythmias Post-resuscitation management

Cessation of cardiopulmonary resuscitation

2.4. Paediatric resuscitation in specific circumstances Avoiding cardiac arrest during critical care management Anaphylaxis Asthma Drowning Traumatic cardiac arrest Toxicological emergencies Envenomation Marine envenomation

2.5. Shock Introduction Diagnosis and assessment Initial management Further management

2.6. Sepsis recognition and initial management Introduction Background Definition Aetiology Pathophysiology Diagnosis Initial emergency management Disposition

Section 3. Neonatal Emergencies 3.1. The normal neonate Definition and introduction Common reasons for healthy neonates to present to the emergency department Summary

3.2. The crying infant Introduction Recurrent crying Acute crying Disposition

3.3. Neonatal Dermatology Skin in the Neonatal Period Neonatal erythroderma Red scaly rashes Vesicles and blisters Pustular lesions Birthmarks Blue/purple lesions Vascular lesions in the neonatal period

3.4. Acute neonatal emergencies The Neonatal Period Neonatal resuscitation Assessment of the neonate

The neonate with vomiting The neonate with seizures The neonate with breathing difficulty The neonate with prolonged jaundice

3.5. Neonatal resuscitation Introduction Aetiology and pathophysiology Preparation Assessment at birth Ventilation Heart rate Colour Muscle tone and reflex irritability Medications Specific resuscitation situations Post-resuscitation stabilisation Prognosis

Section 4. Trauma in Children 4.1. Introduction to paediatric trauma Prevalence Prevention Succinct treatment (salvage) Primary survey Paediatric differences

Other issues during initial stabilisation Secondary survey Orthopaedic trauma Rehabilitation

4.2. Paediatric neurotrauma Introduction Epidemiology Pathophysiology Classification Assessment

4.3. Spinal injury Introduction Developmental anatomy and physiology Initial assessment Spinal immobilisation Cervical spine injuries Thoracic and lumbar spine injuries Spinal cord injury Spinal cord injury without radiographic abnormality

4.4. Thoracic injuries in childhood Introduction Initial approach in the emergency department Chest wall injury Pulmonary injury

Tracheobronchial injuries Mediastinal injury Cardiac injuries Diaphragmatic injury Emergency department thoracotomy

4.5. Abdominal and pelvic trauma Introduction History Examination Investigations General management Surgical issues Hollow viscus injuries Pancreatic and renal injuries Penetrating trauma Pelvic fractures Disposition

4.6. Burns Introduction Pathophysiology Classification History Examination Investigations Management

Management of burns Electrical burns Chemical burns

4.7. Children in a disaster response Introduction Phases of disaster response

4.8. Wound management Introduction Classification of wounds Evaluation of the patient with a laceration Treatment of wounds Wound closure Post-wound-closure care Treatment of selected injuries

Section 5. Cardiovascular 5.1. Cardiovascular assessment and murmurs Introduction History Physical examination Chest X-ray The child with an asymptomatic murmur Pathological murmurs Disposition

5.2. Chest pain Introduction Immediate approach General approach Summary

5.3. Syncope Introduction Aetiology Typical presentations Clinical Investigations within the emergency department Further investigations of syncope Management of syncope within the emergency department Summary

5.4. Cyanotic heart disease and tetralogy of Fallot spells Introduction Cyanotic congenital heart disease Clinical features Investigations Management Disposition Tetralogy Spells Investigations Treatment Disposition

5.5. Heart failure Definition Causes of congestive heart failure Clinical manifestations and investigations Management

5.6. Congenital heart disease Introduction Undiagnosed congenital heart disease Complications/residua of congenital heart disease and its treatment Congenital heart disease and intercurrent illness

5.7. Acute rheumatic fever Introduction Investigations Treatment Prevention and prophylaxis

5.8. Infective endocarditis Introduction Epidemiology Pathophysiology Microbiology History Examination Modified Duke criteria Investigations

Differential diagnosis Treatment Prognosis Prevention

5.9. Kawasaki disease Introduction Pathophysiology Clinical features Incomplete Kawasaki disease Differential diagnosis Complications Investigations Treatment Refractory Kawasaki disease Prognosis

5.10. Cardiac arrhythmias Introduction Normal conduction system The cardiac action potential Pathogenesis of arrhythmias General principles for arrhythmia management Role of ‘molecular autopsy’ in sudden unexplained cardiac death in the young

Section 6. Respiratory

6.1. Stridor and noisy breathing Introduction Initial assessment History Examination Common causes of acute stridor in children Common causes of chronic stridor in children

6.2. Upper respiratory tract infections Introduction Nasopharyngitis Stomatitis Pharyngitis/tonsillitis

6.3. Inhaled foreign body Introduction Upper airway foreign bodies Treatment Lower airway foreign body Prevention

6.4. Croup Introduction Presentation Investigations Differential diagnosis Treatment and disposition

Prognosis Prevention

6.5. Acute asthma Introduction Diagnosis of asthma Risk factors for mortality Clinical assessment Differential diagnosis Treatment Discharge from hospital Prognosis Prevention Future directions/research

6.6. Pertussis Introduction Pathophysiology Epidemiology History Examination Investigations Differential diagnosis Complications Treatment Prognosis

6.7. Community-acquired pneumonia Introduction Definition Aetiology Clinical findings Investigations Management Complications Prevention Conclusion

6.8. Bronchiolitis Introduction Clinical assessment Treatment Prognosis Prevention

Section 7. Gastroenterology and Hepatology 7.1. Abdominal pain Introduction Pathophysiology Aetiology Assessment History Examination

Investigations Management Disposition Acute Appendicitis Clinical features Differential diagnoses Investigations Management Meckel’s Diverticulum Clinical features Differential diagnoses Investigations Management Chronic Abdominal Pain Assessment Diagnosis Management

7.2. Vomiting Non-Surgical Vomiting Definitions Clinical evaluation Examination Differential diagnoses Investigations Management Conclusions

Surgical (Bilious) Vomiting Causes Complications Investigations Treatment

7.3. Gastrointestinal bleeding Introduction Aetiology History Examination Investigations Initial medical therapy Surgery Treatment Dispositon

7.4. Gastro-oesophageal reflux Introduction Pathophysiology History Examination Differential diagnosis Complications Investigations Treatment Follow-up

7.5. Pyloric stenosis Introduction Epidemiology Clinical presentation Examination findings Imaging studies Differential diagnosis Management Complications

7.6. Ingested foreign bodies Introduction History Examination Investigations Treatment Disposition Prevention

7.7. Acute liver failure Introduction Aetiology Pathophysiology Presentation Investigations Management Disposition

Prognosis Prevention

7.8. Diarrhoea Introduction Definitions Clinical evaluation Examination Differential diagnoses Investigations Management Conclusions

7.9. Management of acute hepatitis in children presenting to the emergency department Introduction Aetiology History Examination Investigations Viral hepatitis Drug- and toxin-induced liver injury Chronic liver disease presenting as acute hepatitis Other causes of chronic liver disease presenting in childhood

7.10. Intussusception Introduction Aetiology

Epidemiology Clinical Investigations Management Outcome

7.11. Herniae Introduction Types of herniae Complications Treatment

7.12. Gastroenteritis Introduction Aetiology History Examination Differential diagnosis Investigations Treatment Severely dehydrated Other treatments Disposition Prognosis

7.13. Constipation Introduction

Definitions Pathophysiology Management basics Constipation in babies Acute constipation Chronic constipation Investigations Management Medications

7.14. Inflammatory bowel disease Introduction New diagnoses Known IBD patients Common treatments and their complications

Section 8. Neurology 8.1. Cerebrospinal fluid shunt complications Introduction Clinical presentation Examination Investigations

8.2. Raised intracranial pressure Introduction Particular issues in children

Clinical features of raised intracranial pressure Other examination findings in raised intracranial pressure Investigations Some particular causes of raised intracranial pressure

8.3. Seizures and non-epileptic events Introduction General comments Classification of seizures Febrile seizures Presentation to Emergency Department Presentation post a possible seizure Presentation of a seizure

8.4. Acute weakness Introduction Presentation Primary survey approach History Examination Investigations Specific conditions causing acute weakness

8.5. Acute ataxia Introduction Pathophysiology Differential diagnosis

Chronic ataxia Clinical evaluation of the patient Examination Investigations Management Disposition

8.6. Headache Introduction Incidence Pathophysiology Clinical assessment Management Disposition Migraine Pathophysiology Clinical features Treatment Disposition Conclusions

8.7. Central nervous system infections: Meningitis and encephalitis Introduction Meningitis Brain abscess Encephalitis Conclusion

Section 9. Infectious Diseases 9.1. Infectious diseases Fever Empiric antibiotic therapy Common infectious exanthems What specimens, when should they be ordered and what tests? Infection control in the emergency department Needlestick injury Immunisation

Section 10. Metabolic Emergencies 10.1. Inborn errors of metabolism Introduction Physiology and pathogenesis Clinical features Investigation Management Chronic presentations Extended newborn screening Conclusion

10.2. Hypoglycaemia in the non-diabetic child Introduction The hypoglycaemia screen Causes of hypoglycaemia

Treatment of hypoglycaemia

10.3. Diabetic emergencies in children Diagnosis Diabetic ketoacidosis Hypoglycaemia The child with an insulin pump The child with diabetes and intercurrent illness Diabetic child and surgery Long-term management

10.4. Thyroid emergencies Thyrotoxicosis Neonatal thyrotoxicosis Hypothyroidism Congenital hypothyroidism Hashimoto’s thyroiditis

10.5. Adrenal emergencies Adrenal Crisis Clinical presentation Disposition Prevention Cushing’s Syndrome Presenting symptoms Diagnostic testing Treatment

10.6. Disorders of fluids, electrolytes and acid–base Introduction Physiology Clinical assessment Investigations Treatment Investigation and management of fluids in different conditions Acid–base disorders

Section 11. Haematology and oncology 11.1. The use of blood products in children Introduction Packed red blood cells Platelets Fresh frozen plasma Cryoprecipitate Clotting factor concentrates Albumin Normal human immunoglobulin Hyperimmune immunoglobulins Risks of blood component use

11.2. Anaemia Introduction Acute management Neonatal anaemia

Anaemias of childhood Haemolytic anaemias

11.3. Disorders of coagulation Haemophilia von Willebrand disease

11.4. Platelet disorders Introduction Immune thrombocytopaenia Chronic immune thrombocytopaenia

11.5. Vasculitis Introduction Clinical presentation Classification Small-Vessel Vasculitis Anti-cytoplasmic-antibody-associated vasculitis Medium-Vessel Vasculitis Polyarthritis nodosa Large-Vessel Vasculitis Secondary Vasculitis and Vasculitis Mimics

11.6. Acute leukaemia Introduction Classification Clinical presentation

Differential diagnosis Investigations Prognosis Complications Management

11.7. Febrile neutropaenia Introduction Presentation Investigations Treatment

11.8. Emergencies in paediatric oncology Emergency Complications of Paediatric Malignancy Fever and infection Gastrointestinal emergencies Blood product use in oncology Cardiothoracic emergencies Metabolic emergencies Genitourinary emergencies Neurological emergencies Brain and Spinal Cord Tumours in Children Brain tumours Spinal cord compression

Section 12. Dermatology

12.1. Dermatology Introduction Erythroderma and skin failure Vesiculobullous rashes Pustular rashes Papular (raised) rashes Red scaly (papulosquamous) rashes Eczematous rashes Red blanching rashes (erythematous) Purpuric rashes Vascular tumours – haemangiomas and haemangioma variants Vascular malformations Management of vascular malformations Hyperpigmentation Hypopigmentation Skin texture Mouth disorders Anogenital rashes Zinc and other nutritional deficiencies Anogenital papules and lumps Immunodeficiency states Hair problems Nail problems Itch without rash

Section 13. Eyes

13.1. Ophthalmological emergencies Introduction History Examination The red eye in paediatrics

13.2. Congenital, developmental and neoplastic conditions of the eye Deficits in visual acuity Strabismus Paediatric cataracts Congenital nasolacrimal duct obstruction Infantile glaucoma Ocular tumours Retinoblastoma Rhabdomyosarcoma Neuroblastoma

13.3. Ocular trauma Introduction Globe trauma Eyelid trauma Orbital trauma

Section 14. Ent and Dental 14.1. The ear Otitis externa

Acute otitis media Discharging otitis media – chronic suppurative otitis media Otitis media with effusion Mastoiditis Trauma

14.2. The nose Rhinitis and sinusitis Epistaxis Nasal trauma

14.3. The mouth and throat Stomatitis Differential diagnosis Pharyngitis/tonsillitis Peritonsillar abscess Post-tonsillectomy haemorrhage Oral/dental trauma Oral/dental infection Other dental issues

14.4. Retropharyngeal abscess Introduction History Examination Investigation Treatment

14.5. Foreign bodies and caustic ingestion Nasal foreign bodies Aural foreign bodies Complications Caustic ingestion

Section 15. Obstetrics and Gynaecology 15.1. Paediatric gynaecology Infant and prepubescent gynaecology Adolescent gynaecology

15.2. Emergency contraception Introduction Clinical assessment Available medicines Medicine interactions Medicine contraindications Medicine adverse effects Medicine outcomes Copper intrauterine devices

Section 16. Renal 16.1. Acute kidney injury Introduction

Pathophysiology Clinical presentation Treatment Acute presentation of chronic renal failure Acute kidney injury in the renal transplant recipient

16.2. Haematuria Isolated microscopic haematuria History Examination Investigation Disposition

16.3. Hypertension Introduction History Examination Emergency department management

16.4. Urinary tract infection in pre-school children Introduction History and examination Diagnosis Treatment Prognosis Prevention

16.5. Haemolytic uraemic syndrome Introduction Definition of haemolytic uraemic syndrome Classification of haemolytic uraemic syndrome Pathophysiology of haemolytic uraemic syndrome History Examination Investigations Differential diagnosis Treatment Prognosis Complications Prevention

16.6. Idiopathic nephrotic syndrome Introduction Pathophysiology of proteinuria Mechanisms of proteinuria Definition of nephrotic syndrome Classification of paediatric nephrotic syndrome Idiopathic nephrotic syndrome: epidemiology Clinical features of idiopathic nephrotic syndrome Investigations Differential diagnosis Complications of idiopathic nephrotic syndrome Treatment of initial nephrotic phase Prognosis

16.7. Henoch–Schönlein purpura Introduction Epidemiology Pathogenesis Diagnostic criteria for Henoch–Schönlein purpura Clinical features Diagnosis and investigation Differential diagnosis Treatment Long-term prognosis and follow-up

Section 17. Psychiatric 17.1. Paediatric psychiatric emergencies Introduction General approach Common Paediatric Psychiatric Presentations Suicidal patients Anxiety disorders Psychosis Other psychiatric presentations Acknowledgement

17.2. The treatment of the behaviourally disturbed adolescent Introduction The principles of psychiatric triage The ABCC of rapid psychiatric assessment

Managing the ABCC including restraint and acute sedation Conclusion

17.3. Autism and behavioural disturbance in the pre-adolescent child Introduction Underlying mechanisms in austistic spectrum of disorders/neurodevelopmental disorders Approach to assessment Challenges in emergency department presentations Approach to management Management Ongoing management in the community Conclusion

Section 18. Crisis Intervention 18.1. Sexual assault Introduction Definitions Attitudes/myths surrounding child sexual assault Epidemiology of child sexual assault Child sexual assault and emergency medicine Recognition of child sexual assault Signs and symptoms Genitoanal anatomy Sexually transmitted infections Diagnostic considerations Role of the emergency physician

18.2. Child at risk Introduction Definition The community response to the child at risk

Section 19. Administration In EMS 19.1. Managing the death of a child in the emergency department: Bereavement issues Introduction The resuscitation process Talking to parents and families Laying out of the child Viewing the body – quiet suite The grief response Support of the family Cultural implications Legal issues Organ and tissue donation and collection Debriefing and support for emergency department staff Collaboration with paediatric palliative care services The concept of a good death Conclusion

Section 20. Analgesia and Sedation 20.1. Analgesia

Introduction Assessment of pain Management Non-pharmacological methods Pharmacological methods

20.2. Paediatric procedural sedation within the emergency department Introduction Pre-procedure Intra-procedure Post-procedure Non-pharmacological methods Pharmacological methods

Section 21. Poisoning 21.1. General approach to poisoning Introduction and epidemiology Diagnosis Risk assessment Investigations Resuscitation Decontamination Antidotes Enhanced elimination Supportive care Consultation and disposition

21.2. Specific poisons Common poisons Rare and dangerous poisons

Section 22. Environmental 22.1. Envenomation Introduction Snakebite Scorpion stings Spiderbite Tick bite paralysis Jellyfish stings Venomous fish stings Venomous marine molluscs

22.2. Drowning Introduction Epidemiology Aetiology Pathophysiology History Examination Investigations Differential diagnosis Treatment Disposition

Prognosis Prevention

22.3. Heat-induced illness Introduction Causes of heat-related illness Investigations Management Prognosis and disposition

22.4. Cold injuries Introduction Normal physiology: a review Hypothermia Localised cold injuries Frostbite Hypothermia not due to environmental causes

22.5. Anaphylaxis Introduction Pathophysiology Aetiology Clinical features Investigations Treatment Diagnosis Prevention

Section 23. Ultrasound 23.1. Ultrasound 23.2. Diagnostic ultrasound in paediatric emergency medicine 23.3. Ultrasound guidance for procedures Ultrasound guidance for procedures

23.4. Incorporating ultrasound into paediatric resuscitation

Section 24. Common Procedures 24.1. Estimating the weight of infants and children Background Age-based tools to estimate body weight Length- and body-habitus–based tools Tips

24.2. Basic airway management techniques Background Oropharyngeal and Nasopharyngeal Airways Contraindications Equipment Preparation Positioning Procedure

Complications Tips Laryngeal Mask Airway Equipment Preparation Positioning Procedure Complications Tips Bag-Valve-Mask and Flow-Inflating Mask Ventilation Contraindications Bag-Valve-Mask Equipment Preparation Positioning Procedure Complications Tips Flow-Inflating Bag Equipment Preparation Procedure

24.3. Non-Invasive Ventilation Non-Invasive Continuous Positive Pressure Ventilation Contraindications Humidified High-Flow Nasal Prong Oxygenation Equipment

Procedure Complications Tips T-Piece Ventilation Device Equipment Procedure Complications Tips Continuous Positive Airway Pressure/Biphasic Positive Airway Pressure Equipment Contraindications Relative contraindications Procedure Complications Tips

24.4. Endotracheal intubation Background Indications Contraindications Equipment Preparation Positioning Procedure Complications Tips Confirmation of Intubation

Indications Contraindications Equipment Preparation and positioning Procedure Complications Tips

24.5. The surgical airway Background Indications Contraindications Needle cricothyroidotomy Surgical cricothyroidotomy

24.6. Chest procedures Introduction Needle Thoracostomy Indications Contraindications Equipment Preparation Procedure Complications Tips Tube Thoracostomy Indications

Contraindications Equipment Preparation Procedure Complications Tips Three-Sided Dressing Indications Contraindications Equipment Preparation Procedure Pericardiocentesis Indications Contraindications Equipment Standard preparation Procedure Complications Tips

24.7. Removing and replacing a tracheostomy tube Background Indications for emergent replacement Contraindications Equipment Preparation

Procedure Complications Tips

24.8. Central and peripheral intravenous lines Background Indications Contraindications Peripheral venous catheter placement Central venous line placement

24.9. Intraosseous infusions Background Indications Contraindications Equipment Preparation Procedure Complications Tips

24.10. Umbilical vessel cannulation Background Indications Contraindications Equipment Preparation

Procedure Complications Tips

24.11. Defibrillation Background Indications for defibrillation (asynchronous) Indications for synchronous cardioversion Contraindications Equipment Standard preparation Standard procedure Automated external defibrillators procedure Complications Tips

24.12. Transurethral catheterisation and suprapubic bladder aspiration Background Indications Contraindications Transurethral catheterisation Suprapubic aspiration

24.13. Lumbar puncture Background Indications Contraindications

Equipment Preparation and positioning Procedure Complications Tips

24.14. Reduction of paediatric inguinal hernias Introduction Preparation Procedure Inguinal hernias in girls Complications

24.15. Paraphimosis Introduction Indications Contraindications Procedures Complications Tips

24.16. Gastrostomies and other enteral feeding devices – trouble shooting in the emergency department Background Anatomy of a percutaneous endoscopic gastrostomy tube Percutaneous endoscopic gastrostomy insertion method Complications Percutaneous endoscopic gastrostomy displacement

Buried bumper Gastrocolocutaneous fistula Peristomal leak Peristomal infection Mechanical problems – blockage Jejunal feeding devices Granulation tissue

Section 25. Orthopaedics and Rheumatology 25.1. Orthopaedics and rheumatology Introduction The child with acute musculoskeletal pain or dysfunction Generalised or multifocal bone/joint pain Other important subacute paediatric musculoskeletal presentations Conclusion Acknowledgement

25.2. Child with a limp Introduction History Examination Investigation Clinical decision making in a child with a limp Specific conditions

25.3. Fractures and dislocations

Fracture patterns in childhood Initial assessment and management Upper limb and shoulder girdle injuries Lower limb and pelvis injuries Conclusions

25.4. Risk management in acute paediatric orthopaedics Introduction Non-identification or delayed identification of paediatric fractures Adverse events in acute paediatric orthopaedics

Section 26. Male Genitalia 26.1. Male genitalia The acute scrotum Epididymo-orchitis Idiopathic scrotal oedema Testicular tumours Irreducible inguinal hernia Rupture of the testis Acute hydrocele Acute problems of the penis and foreskin Introduction Phimosis of the foreskin Balanitis Priapism

Section 27. Transport and Retrieval 27.1. Paediatric emergency retrieval Paediatric retrieval What’s different about children? Paediatric emergency referrals Paediatric referral and retrieval – roles and expectations Paediatric retrieval staff Paediatric retrieval equipment Criteria for transport Degrees of urgency Transport platforms While waiting Stabilisation Communication and retrieval leadership Framework for communications during paediatric retrieval Interface with adult retrieval Parents Paediatric retrieval and end-of-life situations Quality Summary

27.2. Sick child in a rural hospital Introduction Challenges in the rural setting Caring for the critically ill child What can be done to assist care in remote environments?

Relations between rural and urban hospitals Acknowledgement

Section 28. Teaching Paediatric Emergency Medicine 28.1. Availing web-based resources Accessing web-based resources Needs of paediatric emergency medicine staff Educational tools available online Social media Pitfalls of online content

28.2. Teaching paediatric emergency medicine Introduction Desirable attitudes in paediatric emergency medicine Skill set for paedicatric emergency Putting knowledge into perspective Helping others acquire knowledge, skills and attitudes: modes of learning Learning resources Conclusions

Section 29. Paediatric Research in the Emergency Department 29.1. Research in children in the emergency department Introduction Research science The ethics of medical research

The practice and governance of research Implementation research: an emerging research field Multicentre research Funding research

Section 30. Adolescent Medicine in the Emergency Department 30.1. Adolescent medicine in the emergency department Introduction Adolescent health problems in the emergency department The approach to the adolescent in the emergency department Summary

30.2. Eating disorders and anorexia Introduction History Examination Investigations Complications Differential diagnosis Management Prognosis

Index

Copyright © 2019, Elsevier Limited. All rights reserved. First edition 2006 Second edition 2012 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). Chapter 3.3 Neonatal Skin Disorders and Chapter 12.1 Dermatology © Roderic Phillips, Mike Starr, David Orchard and Diane Purvis. All rights reserved. Published with permission of Roderic Phillips, Mike Starr, David Orchard and Diane Purvis 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. ISBN: 978-0-7020-7305-2 Content Strategist: Laurence Hunter Content Development Specialist: Alexandra Mortimer Content Coordinator: Kirsty Guest Senior Project Manager: Manchu Mohan Design: Amy Buxton Illustration Manager: Amy Naylor

Printed in China Last digit is the print number:

9 8 7 6 5 4 3 2 1

Preface to third edition It is now more than 10 years since the publication of the first edition and interest in paediatric emergency medicine continues to grow at a local and international level. For generalist clinicians, who see both adult and paediatric patients, children account for a minority of the daily workload, however the anxiety associated with providing safe and effective care to this population ensures a keen interest in updating skills and knowledge. The principles in managing paediatric patients are the same as adults; however, there are significant differences in patterns of illness and responses to illness. In addition, the therapies available vary widely between adult and paediatric practice. Not all of the differences have been evidence based; however, the last couple of decades have seen a major improvement in research supporting guidelines for the management of paediatric emergencies. The formation of research groups such as PREDICT (Paediatric Research in Emergency Departments International Collaborative) and PERN (Pediatric Emergency Research Network) has generated solid evidence to underpin some of the new guidelines for assessment and management of paediatric patients. The assessment of head injuries, oxygen therapy, fluid management, sedation and analgesia are just some of the areas that have had major changes in approach over the last few years due to excellent research by the paediatric research networks. New technologies and better application of older techniques have also led to changes in practice. For example, ultrasound is now being used more routinely in clinical practice for placement of lines, incision and drainage, resuscitation of critically ill patients and assessment of minor injuries. More research needs to be done into what place bedside imaging takes in specific circumstances, such as FAST in trauma patients. It is likely that management algorithms in children are significantly different to adults, because of different patterns of injury and response to injury.

This new third edition attempts to capture the major changes in guidelines across the specialty, whilst refining established approaches to practice in most subject areas. The authors have significantly updated the resuscitation and trauma sections, clinical applications of bedside ultrasound, analgesia and sedation. There is also a new focus on the teaching and research sections. This revision has involved the focused input of more than 100 clinicians and academics for over 1 year. The dedication to purpose and attention to detail is reflected in the quality of the book. I would also like to thank the publication staff at Elsevier, in particular Ms Alexandra Mortimer, and my Personal Assistant, Ms Angela Hodges, for coordinating the many people involved and keeping focus. 2018 P. C. G. B. B. M. S. D. S. C.

Preface to second edition Following the successful launch of the first edition as a companion to the Adult Textbook of Emergency Medicine, we have had considerable feedback on the content and layout from doctors, nurses and paramedics working in paediatric emergency practice from around the world. The feedback has been positive, particularly regarding the importance of a text with a standardised, easily accessible format. Despite major advances in computerisation, most clinicians studying detailed clinical material, still prefer a well-presented book with carefully edited text to on-line material. It is likely that further advances in technology will enable electronic versions of this text shortly and it is intended for this to occur after the printed version has been released. In this edition we have reviewed each chapter and updated guidelines and management protocols where appropriate. Material has been reviewed by chapter authors and editors to ensure that it is consistent with best practice internationally. The structure has remained the same to enable easy access for readers. Since the first edition, there has been consolidation of paediatric emergency medicine as a specialised domain of clinical expertise. Standards for paediatric patient care in emergency departments have been published in the United Kingdom and elsewhere and training programmes have been developed in many countries. There is a high degree of cooperation within the international emergency paediatric community and international networks for research (e.g. PERN – Paediatric Emergency Research Network) and other activities are being considered. Hopefully texts such as this can further consolidate the convergence of clinical knowledge and practice internationally. This edition was developed over approximately 18 months with contributions from authors around the world including Australia, New Zealand, United Kingdom, Hong Kong, and the United States. The commitment and effort required to coordinate and cajole the many people involved, required dedication

from all involved but we would particularly like to thank Helen Leng from Elsevier who remained focused throughout. 2012 P. C. G. J. I. E. G. B. J. R.

Contributors Richard Aickin, MBChB FRACP FACEM, Paediatric Emergency Specialist, Starship Children’s Hospital, Auckland, New Zealand David Armstrong, MBChB FRACP MD Adjunct Associate Professor, Paediatrics, Monash University Head, Department of Respiratory Medicine, Monash Children’s Hospital, Clayton, Australia Franz E. Babl, MD MPH DMedSc FRACP FAAP FACEP, Associate Professor of Paediatrics, Royal Children’s Hospital, Murdoch Children’s Research Institute and University of Melbourne, Melbourne, Australia Bindu Bali, BMedSci BMBS MRCPCH FRACP MMEd Paediatrician, Emergency Department, Sunshine Hospital Paediatrician, Royal Children’s Hospital, Melbourne, Australia Peter L.J. Barnett, MBBS FRACP MSc FACEM MSpMed Consultant, Emergency Department, Royal Children’s Hospital Associate Professor, Department of Paediatrics, University of Melbourne Honorary Research Fellow, Emergency Services, Murdoch Children’s Research Institute, Parkville, Australia Katherine Barton, MBBS (Hons) FRACP, Paediatric Emergency Physician, Princess Margaret Hospital, Perth, Australia Tom Beattie, MB BAO BCh MSc FRCPE FRCSE FFSEM RCS&PI, Codirector MSc Paediatric Emergency Medicine, Child Life & Health, University of Edinburgh, Edinburgh, UK Nader Beshay, MMed MBBS, Registrar, Emergency Medicine, Royal North Shore Hospital, Sydney, Australia Robyn Brady, FRACP FACEM LCCH CCPU Paediatric Emergency Physician and Ultrasound Lead

Lady Cilento Children’s Hospital, Brisbane, Australia Adrian Mark Bonsall, MA MBBS, Paediatric Emergency Hospitalist, Emergency Department, Children’s Hospital at Westmead, Sydney, Australia Christine Brabyn, BSc MBChB FRACP FACEM Emergency Physician, Paediatric Emergency Physician, Waikato Emergency Department, Hamilton, New Zealand Drago Bratkovic, MBBS FRACP, Unit Head, Metabolic Clinic, Women’s and Children’s Hospital, North Adelaide, Australia Simon G.A. Brown, PhD FACEM Director, Aeromedical and Retrieval (AMR), Ambulance Tasmania Emergency Physician, Royal Hobart Hospital, Hobart, Tasmania Clinical Professor, Emergency Medicine, University of Western Australia, Hobart, Australia David H.F. Buckley, BSc MBChB FANZCA FCICM, Paediatric Anaesthesia and Intensive Care, Starship Children’s Health, Auckland, New Zealand Jim P. Buttery, MBBS FRACP MSc MD Head, Infection and Immunity, Monash Children’s Hospital Professor of Paediatric Epidemiology, Epidemiology and Preventive Medicine & Paediatrics, Monash University, Melbourne, Australia Adam Bystrzycki, MBBS FACEM PGDipEcho Emergency Physician, Emergency & Trauma Centre, Alfred Hospital Senior Lecturer, Department of Medicine, Monash University, Sandringham, Australia Gar Ming Chan, MD FACEM, Specialist, Emergency Medicine Calvary Hospital, Lenah Valley, Tasmania, Australia Annette Chang, MBBS MMed Surgery, Registrar, Paediatric Surgery Department, Monash Children’s Hospital, Melbourne, Australia John A. Cheek, MBBS FACEM, Deputy Director, Performance and Improvement, Emergency Department, The Royal Children’s Hospital, Melbourne, Australia Nicholas Cheng, MBBS BSc(Med) DCH FRACP

Paediatric Emergency Consultant, Emergency Department, Children’s Hospital at Westmead Conjoint Lecturer, Faculty of Medicine, University of NSW, Conjoint Lecturer, Paediatrics, Western Sydney University, Westmead, Australia Robin Choong, MBBS FRACP FCICM, Senior Staff Specialist, Paediatric Intensive Care, The Children’s Hospital at Westmead, Sydney, Australia Jane Cocks, MBBS DCH FRACP FACEM FCICM, PGCert Aeromedical Retrieval, Paediatric Emergency Physician, Paediatric Emergency Department, The Women’s and Children’s Hospital, North Adelaide, Australia Elizabeth Cotterell, MBBS MPH FRACP, Associate Professor of Paediatrics, School of Rural Medicine, University of New England, Armidale and Armidale Rural Referral Hospital, Armidale, Australia John Craven, BSc (Hons) BMBS FRACP FACEM Head of Unit, SAAS MedSTAR Kids Retrieval Service Paediatric Emergency Physician, Flinders Medical Centre & Women’s and Children’s Hospital, Adelaide, Australia Nigel W. Crawford, MBBS MPH PhD FRACP, General Paediatrician, The Royal Children’s Hospital and the Murdoch Children’s Research Institute, Melbourne, Australia John Cronin, MB BAO BCh AFRCSI DCH FRCEM, Consultant in Emergency Medicine, Emergency Department, St Vincent’s University Hospital, Dublin, Ireland Sarah Davidson, MBBS FACEM PEM, Paediatric Emergency Medicine Physician, The Sunshine Coast University Hospital and Health District, Queensland, Australia Tessa Davis, MBChB BSc(Hons) MA MRPCH FRACP, Co-Founder, Clinical Fellow in Paediatric Emergency Medicine, Royal London Hospital, Don’t Forget The Bubbles, London, UK Anthony Delaney, MBBS MSc PhD FACEM FCICM Senior Staff Specialist, Malcolm Fisher Department of Intensive Care Medicine, Royal North Shore Hospital Associate Professor, Sydney Medical School, University of Sydney Adjunct Senior Research Fellow, School of Public Health and Preventative

Medicine, Monash University, St. Leonards, Australia Peter Downie, MD FRACP Head, Paediatric Haematology-Oncology, Monash Children’s Hospital Director, Centre for Cancer and Blood Diseases, Monash Children’s Hospital Senior Lecturer, Department of Paediatrics, Monash University, Clayton, Australia Evelyn Doyle, MB Bch BAO MRCP FRACP, Consultant Paediatric Emergency Physician, Paediatric Emergency Department, Women’s and Children’s Hospital, Adelaide, Australia Linas Dziukas, MBBS MD FRACP FACEM, Emergency Physician, Emergency and Trauma Centre, Alfred Hospital, Melbourne, Australia Daryl Efron, MBBS FRACP MD Consultant Paediatrician, General Medicine, Royal Children’s Hospital Senior Research Fellow, Murdoch Children’s Research Institute Senior Lecturer, Paediatrics, University of Melbourne, Parkville, Australia Tom Everitt, MBBS FRACGP, General Practitioner, Cranbourne, Victoria, Australia Michael Fairley, MBBS FRANZCP Consultant Child Psychiatrist, Child Protection Unit, Sydney Children’s Hospital Child and Adolescent Psychiatrist, Youth and Family Mental Health, Lismore Base Hospital, Strathfield, Australia Bruce Fasher, MBBS DCH (RCP) DRCOG (Lond) FRCP (Lond) FRACP, Physician, Emergency Department, Royal Alexandra Hospital for Children, Sydney, Australia Dominic Fleischer, MBChB FACEM, Specialist Emergency Physician, Emergency Department, Christchurch Hospital, Christchurch, New Zealand Jeremy Furyk, MBBS FACEM MSc MPH&TM Emergency Physician, University Hospital Geelong, Victoria, Australia Associate Professor (Adjunct), James Cook University, Townsville, Australia Lalith Gamage, MMBS DCH DCH MD(PAEDS) FRACP Staff Specialist, Emergency Department, Women’s and Children’s Hospital Staff Specialist, Paediatric Department, Port Augusta Hospital Lecturer, Rural School of Medicine, University of Adelaide, Auldana, Australia

Gary Geelhoed, MBBS FRACP FACEM MD, Clinical Professor, Chief Medical Officer Western Australia, University of Western Australia, Perth Australia Ed Giles, MBBS FRACP MRCPCH PhD Consultant Paediatric Gastroenterology, Paediatrics, Monash Children’s Hospital Research Fellow, Paediatrics, Monash University, Clayton, Australia Laura Graley, BMBS MRCPCH, Melbourne, Australia Joanne Grindlay, MBBS FACEM FRACGP FARGP DA(UK) Grad Dip(Rural GP) EMDM HOSM Deputy Director, Emergency Medicine, The Royal Children’s Hospital Clinical Associate Professor, University of Melbourne, Department of Paediatrics Research Associate, Murdoch Children’s Research, Melbourne, Australia Sonia R. Grover, MBBS FRANZCOG MD FFPMANZCA Director, Gynaecology Department, Royal Children’s Hospital Clinical Professor, Dept Paediatrics, University of Melbourne Head of Family Planning Unit, Austin Health Head of Gynaecology Unit, Mercy Hospital for Women, Austin Health, Clifton Hill, Australia Naren Gunja, MBBS MSc FACEM FACMT Clinical & Forensic Toxicologist, Westmead Hospital Clinical Toxicologist, NSW Poisons Information Centre Clinical Associate Professor, Discipline of Emergency Medicine, Sydney Medical School, Australia Wayne Hazell, MBBS DipObs GCCT MCLINED FACEM, Emergency Physician, The Prince Charles Hospital Emergency Department, Deputy Head of the Prince Charles Clinical School, University of Queensland, Brisbane, Australia Malcolm Higgins, BM BS FRACP, Medical Unit Head, Paediatric Emergency Department, Women’s and Children’s Hospital, Adelaide, Australia Rupert Hinds, MBBS MRCP MRCPCH FRACP, Consultant Gastroenterologist and Head of Department of Gastroenterology at Monash Children’s Hospital and Senior Lecturer in Paediatrics at Monash University, Clayton, Australia

Andrew John Anderson Holland, BSc (Hons) MBBS PhD Grad Cert Ed Studies FRCS (Eng) FRACS (Paed) FACS Professor of Paediatric Surgery, Discipline of Paediatrics and Child Health, The University of Sydney Senior Clinical Academic, Douglas Cohen Department of Paediatric Surgery, The Children’s Hospital at Westmead, Westmead, Australia Jason Hort, MBBS MRCP FRACP Staff Specialist, Emergency, The Children’s Hospital at Westmead Clinical Senior Lecturer, Emergency, Sydney Medical School, Westmead, Australia Sarah Jamison, BHB MBChB FRACP, Paediatric Emergency Specialist, Children’s Emergency Department, Starship Children’s Hospital, Auckland, New Zealand Shefali Jani, FRACP(PEM) MD(Paed) MClinEpid MBBS Paediatric Emergency Physician, Children’s Hospital at Westmead Clinical Associate lecturer, University of Sydney, Sydney, Australia Sheena Kaul, MBBS, Paediatric Registrar, Monash Children’s Hospital, Clayton, Australia Colin S. Kikiros, MBBS FRACS, Senior Consultant Surgeon, Paediatric Surgery, Perth Children’s Hospital, Perth, Australia Erica Kreismann, MD FACEM, Director of Emergency Medicine, Calvary Hospital, Lenah Valley, Tasmania, Australia David M. Krieser, MBBS FRACP Paediatric Emergency Physician, Emergency Department, Sunshine Hospital Clinical Associate Professor, Department of Paediatrics, University of Melbourne Honorary Fellow, Murdoch Children’s Research Institute Honorary Fellow, Joseph Epstein Centre for Emergency Medicine Research, St Albans, Australia Ben Lawton, MBChB BSc(Hons) FRACP(PEM), Paediatric Emergency Physician, Logan Hospital, Lady Cilento Children’s Hospital and the University of Queensland, Brisbane, Australia Richard Lennon, MBBS FRACP(Paed) FACEM MBioeth, Cherrybrook,

Australia Damir Ljuhar, BBioMed MBBS MPHTM, Paediatric Surgery Registrar, Monash Children’s Hospital, Melbourne, Australia Elliot Long, FRACP Consultant, Emergency Medicine, The Royal Children’s Hospital Research Fellow, Critical Care, Murdoch Children’s Research Institute PhD Candidate, Paediatrics, University of Melbourne, Parkville, Australia Niki Talić, MBBS FACEM-PEM, Dr, Paediatric Emergency Consultant, Royal Children’s Hospital, Parkville Australia and Monash Children’s Hospital, Clayton, Australia Jeanette Marchant, MBChB DCH MRCPCH(UK) FRACP, Specialist, Paediatric Emergency and Retrieval, The Children’s Hospital, Westmead and NSW Newborn and Paediatric Transport Service, Sydney, Australia Susan Marks, MD M Forensic Medicine FRACP FRCPA, Staff Specialist, Child Protection Unit, Children’s Hospital at Westmead, Westmead, Australia Jennie Martin, MBBS (Hons) DipCH FACEM, Emergency Physician, Emergency, Royal North Shore Hospital, St Leonards NSW, Australia Sarah Martin, MBBS MSc DTM&H FRACP FACEM, Paediatric Emergency Physician, Emergency Department, Lady Cilento Children’s Hospital, Brisbane, Australia Mary Elinor McCaskill, MBBS BSc(med) DipPaeds MBA FACEM, Paediatric Emergency Physician, Emergency Department, The Children’s Hospital at Westmead, Sydney, Australia Dermot Thomas McDowell, MB BAO BCh MRCS, Dr, Department of Paediatric Surgery, Children’s Hospital at Westmead, Westmead, Australia Shona McIntyre, MbChB FACEM, Emergency Physician, Emergency Department, Sunshine Hospital, Melbourne, Australia Damien McKay, MBBS BAppSc (Physio) MSpMed FRACP Staff Specialist Paediatrician, Children’s Hospital at Westmead Paediatric Rheumatologist, Liverpool Hospital, Sydney, Australia Ben McKenzie, MBBS FACEM AFRACMA Emergency Physician, Bendigo Health, Victoria, Australia

Critical Care Coordinator and Retrieval Physician, ARV, Ambulance Victoria Adjunct Senior Lecturer, Monash University, Victoria, Australia Christopher McKinlay, MBChB PhD DipProfEthics FRACP CCPU Senior Lecturer, Liggins Institute and Department of Paediatrics, Child and Youth Health, University of Auckland Neonatologist, Kidz First Neonatal Care, Counties Manukau Health, Auckland, New Zealand Robert Melvin, FACEM DTMH MBBS MA(Cantab) BA Hons, Deputy Director of Emergency Medicine, Clinical Lead for Paediatric Emergency Medicine, Emergency Physician, Alfred Health, Melbourne, Australia Alastair D. McR. Meyer, BSc(Hons) BMedSci MBBS FACEM FRCEM(Lond) FRCP(Edin) FRACGP Director of Emergency Medicine, Emergency Department, Casey Hospital Adjunct Associate Professor, Medicine, Nursing and Health Sciences, Monash University, Melbourne, Australia Erin Mills, MBBS (Hons) BMedSci FRACP PEM, Paediatric Emergency Physician, Paediatric Emergency Department, Monash Medical Centre, Clayton, Australia Yuresh Naidoo, MbChb FACEM GradCert HPE(UWA), Emergency Medicine Specialist, Director of Clinical Training, Clinical Lead Service Improvement Unit, Joondalup Health Campus, Western Australia Murali Narayanan, FRACP DNB (Ped) DCH MBBS, Consultant Paediatrician, Emergency Department, Fiona Stanley Hospital, Murdoch, Australia Ramesh Nataraja, BSc(Hons) MBBS FRCSEd(PaedsSurg) GCCS (Hons) FFSTEd FRACS(Paeds) Consultant Paediatric Surgeon, Director of Simulation, Monash Children’s Hospital Senior Lecturer, Paediatric, Monash University, Melbourne, Australia Jocelyn Neutze, MBChB FRACP FACEM, Paediatric Emergency Specialist, Kidz First, Middlemore Hospital, Auckland, New Zealand Kenneth Patrick Nunn, MBBS (Hons) PhD FRACPsych FRANZCP, Senior Paediatric Neuropsychiatrist, Psychological Medicine,

Children’s Hospital Westmead, Westmead, Australia Felix Oberender, MRCPCH PhD FCICM Director, Paediatric Intensive Care Unit, Monash Children’s Hospital Honorary Consultant, Paediatric Intensive Care Unit, The Royal Children’s Hospital Adjunct Senior Lecturer, Monash University, Melbourne, Australia Adam O’Brien, MBBS DDU FACEM, Emergency Physician, The Royal Children’s Hospital, Melbourne, Australia Fenton O’Leary, MBBS FACEM, Clinical Associate Professor Emergency Medicine, The Children’s Hospital at Westmead and University of Sydney, Sydney, Australia Kim Lian Ong, MBBS FRCS (Edin) FHKCEM FHKAM (Emergency Medicine) FAMS Consultant, Accident and Emergency Department, Pok Oi Hospital Honorary Clinical Associate Professor, Accident and Emergency Medicine Academic Unit, Chinese University of Hong Kong, Hong Kong SAR, China David Orchard, MBBS FACD, Director of Dermatology and Associate Professor, Royal Children’s Hospital and Melbourne University, Melbourne, Australia Michael Osborn, MBBS FRACP FRCPA, Haematologist/Paediatric, Adolescent and Young Adult Oncologist, Women’s and Children’s Hospital and Royal Adelaide Hospital, Adelaide, Australia Colin Parker, MBChB DCH(Lon) MRCPCH FACEM Deputy Director, Emergency Medicine, Joondalup Health Campus Emergency Physician, Perth Children’s Hospital Clinical Senior Lecturer, Emergency Medicine, University of Western Australia, Perth, Australia Jacqueline E.L. Parkinson, BPharm (Hons) MPharm GradCert HlthServManagement BCGP MSHP, Senior Pharmacist, Pharmacy Department, Monash Health, Melbourne, Australia Sameer A. Pathan, MBBS CABEM MRCEM EM Consultant, Emergency Department, Hamad General Hospital, Hamad Medical Corporation

Department of Epidemiology & Preventive Medicine, Monash University, Mesaieed, Qatar Scott Pearson, MB ChB FACEM, Emergency Physician, Emergency, Christchurch Hospital, Christchurch, New Zealand Ioannis Pegiazoglou, Dr. med. Deputy Director, Interdisciplinary Pediatric Emergency Department, University Children’s Hospital Basel (UKBB) Basel, Switzerland Clinical Associate Lecturer, Medical School, University of Sydney, Sydney, Australia Roderic Phillips, MBBS FRACP PhD, Clinical Associate Professor, Paediatric Dermatology, Royal Children’s Hospital and Monash University, Melbourne, Australia Susan Phin, MBBS FRACP, Paediatric Emergency Physician, Emergency Department, The Children’s Hospital at Westmead, Westmead, Australia Colin Victor Eric Powell, MB ChB FRACP FRCPCH MRCP(UK) MD, Consultant Paediatrician Noah’s Ark Children’s Hospital for Wales Cardiff and Reader in Paediatrics and Deputy Dean for Research, Division of Population Medicine, School of Medicine, Cardiff University, Wales, United Kingdom Harsh Priyadarshi, MS, Complex Spine Fellow, Macquarie Neurosurgery, Associate Lecturer, Macquarie University, Sydney, Australia Diana Purvis, MBChB FRACP, Paediatric Dermatologist, Paediatric Dermatology, Starship Children’s Hospital, Auckland, New Zealand Karen Quay, BHB MBChB FACEM, Paediatric Emergency Specialist, Childrens Emergency Department, Starship Childrens Hospital, Auckland, New Zealand Kottayam Radhakrishnan, MBBS FRACP FRCPA, Consultant Paediatric Haematologist, Children’s Cancer Centre, Monash Children’s Hospital, Melbourne, Australia Damayanthi Rasanathan, FRACP, Paediatric Emergency Specialist, Children’s Emergency Department, Starship Hospital, Auckland, New Zealand Meenakshi Rattan, MBBS MD (Paediatrics) DCH(UniSyd) FRACP MPH

MHM, Consultant Paediatrician, Wyong and Campbelltown Hospital; Associate Clinical Lecturer, University of Sydney, Wahroonga, Australia Felix Regenfelder, MD Dr, Orthopaedics, The Children’s Hospital at Westmead, Sydney, Australia Dr, Orthopaedie und Unfallchirurgie, Buergerspital Solothurn, Solothurn, Switzerland Sasha Rossaye, MBBCh BSc FRACP, Paediatric Emergency Specialist, Paediatric Emergency Department, Women’s and Children’s Hospital, Adelaide, Australia John M. Ryan, FCEM FRCSEd(A&E) FFSEM DipSportsMed DCH, Professor, Emergency Department, St Vincent’s University Hospital, Dublin, Ireland Vered Schildkraut, BSc MD FRACP Consultant Paediatric Gastroenterologist, Paediatric Gastroenterology Children’s Program, Monash Children’s Hospital Consultant Paediatric Gastroenterologist, Gastroenterology and Clinical Nutrition, The Royal Children’s Hospital, Melbourne, Australia Scott Schofield, MBBS FRACP, Paediatric Emergency Physician, Sunshine Coast Hospital and Health Service, Queensland, Australia Robert Seith, MBChB MRCPCH FRACP, Paediatric Emergency Consultant, Director of Emergency Medicine Training, Monash Children’s Hospital/Monash Medical Centre, Melbourne, Australia Michael Shepherd, MBChB FRACP MPH, Paediatric Emergency Specialist, Children’s ED, Starship Hospital, Auckland, New Zealand James Tibballs, BMedSci (Hons) MBBS MEd MBA MHlth&MedLaw MD FCICM FACLM DALF Associate Professor, Departments of Paediatrics and Pharmacology, The University of Melbourne Senior Intensivist, Paediatric Intensive Care Unit, Royal Children’s Hospital Melbourne, Melbourne, Australia Loren Sher, MBBCh DCH FACEM Paediatric Emergency Physician, Head of Paediatric Emergency, The Northern Hospital

Honorary Clinical Lecturer, The University of Melbourne, Melbourne, Australia Kam King Sinn, MBBS FRACP, Emergency Paediatrician, Senior Specialist, Emergency Department, The Canberra Hospital, Canberra, Australia Holly Smith, MDCM FRACP, Paediatric Emergency Specialist, Emergency Department, Royal North Shore Hospital, St Leonards, Sydney, Australia Soundappan S.V. Soundappan, MBBS MS(Gen Surg) MCh(Paed Surg) FRACS(Paeds) Head of Trauma, The Children’s Hospital at Westmead Senior Lecturer, University of Sydney, Sydney, Australia Mike Starr, MBBS FRACP, Paediatrician, Infectious Diseases Physician, Consultant in Emergency Medicine, Director of Paediatric Education, Honorary Clinical Associate Professor, University of Melbourne, The Royal Children’s Hospital Melbourne, Melbourne, Australia Greg Stevens, BHB MBChB FACEM, Specialist, Emergency Medicine, Waikato Hospital, Hamilton, New Zealand Emma Tavender, BSc (Hons) MA PhD Knowledge Translation Co-ordinator, Emergency Research Group, Murdoch Children’s Research Institute Honorary Fellow, Department of Paediatrics, University of Melbourne, Melbourne, Australia James Tilleard, MBBS FACEM MEd, Senior Staff Specialist, Emergency Department, Sunshine Coast Hospital and Health Service, QLD, Australia Joseph Ting, MBBS BMedSc MSc (Lond) PGDipEpi DipLSTHM FACEM Senior Staff Specialist, Emergency Medicine, Mater Hospital Brisbane Senior Staff Specialist, Emergency Medicine, Ipswich General Hospital Adj Associate Prof, School of Public Health & Social Work, Queensland University of Technology Clinical Senior Lecturer, School of Anaesthesiology & Critical Care, University of Queensland, Australia Henry Patrick John Walsh, MBchB MCNorth FRCS FRACS, Associate Professor, Department of Paediatric Orthopaedics, Lady Cilento Hospital and University of Queensland, Brisbane, Australia Donald Payne, MD MBBChir, Adolescent Medicine, Princess Margaret

Hospital, Subiaco, Western Australia Rachel H. Webb, MBChB MPH&TM FRACP Senior Lecturer in Paediatrics, Paediatrics, University of Auckland Paediatrician, Kidz First, Counties Manukau Health Paediatric Infectious Diseases Specialist, Starship Children’s Hospital, Auckland, New Zealand Christopher Webber, MBBS Dip RACOG FRACP Head of Department & Paediatric Emergency Physician, Emergency Department, Sydney Children’s Hospital Consultant, Neoanatal & Paediatric Retrieval, NETS New South Wales, Randwick, Australia Adam West, MBBS DRANZCOG FACEM Director Paediatric Emergency Medicine, Monash Emergency, Monash Health Adjunct Lecturer, Department of Medicine, Monash University, Clayton, Australia Julian White, MBBS MD FACTM, Professor & Department Head, Toxinology Department, Women’s & Children’s Hospital, North Adelaide, Australia Gary David Williams, MBBS FRACP, Paediatric Intensivist, Paediatric ICU, Sydney Children’s Hospital, Randwick, Australia Simon Vincent Wood, MBBS(Syd) DipPaed(NSW) FACEM, Emergency Physician, Joondalup Health Campus, Joondalup, Australia Kim Yates, MBChB MMedSci PGDipClinEd FACEM, Emergency Medicine Specialist, Emergency Department, North Shore & Waitakere Hospitals, Auckland, New Zealand

Acknowledgements The editors would like to acknowledge and offer grateful thanks for the input of all those who have contributed to previous editions of this textbook; without them this new edition would not have been possible. In particular we acknowledge and thank the following who have stepped down after contributing to the Second Edition:

Jason Acworth, Navid Adib, Philip Aplin, Roger Barkin, Andrew Berry, Meredith Borla G. Cooksley, Lisa Coutts, Maree Crawford, Sarah Dalton, Andrew J. Davidson, Conor Deasy, Ronald A. Dieckmann, Linda Durojaiye, Ian Everitt, Toby Fogg, Peter Francis, Padraic Grattan Smith, Anthony Harrington, Andrew Harris, Robert Henning, Andrew Jan, Andrew Stewart Kemp, Barbara King, Judith Klein, Stuart Lewena, Michelle Lin, Kevin Mackw Jones, Elly Marillier, Karen McCarthy, Helen Mead, Alistair Murray, Matthew O’Mear P. Widmer, Barry Wilkins, Frank Willis, and Simon Young

SECTION 1

Approach to the Paediatric Patient OUTLINE 1.1. Approach to the paediatric patient 1.2. Common chronic paediatric conditions

1.1

Approach to the paediatric patient Adam West, and Tom Everitt

ESSENTIALS 1 Gaining rapport with the child and the confidence of the parents is the key to assessing children. Never underestimate the power of distraction and entertainment. 2 A child needs to be approached according to chronological and developmental age. 3 Observation is a vital diagnostic tool, which is vastly more important in children than in adult patients. 4 The need for investigations is a balance between an invasive stress on a child and the potential gain of information to aid decision making. 5 Always back up discharge with a concrete action plan and definitive follow-up. 6 It is often more important to exclude serious illnesses than make a definitive diagnosis. This may be more easily achieved with timely review. 7 Addressing parental concerns is an important part of the therapeutic process. 8 Emergency physicians should stay within their comfort zone and when in doubt, consult. 9 A febrile child should be considered as potentially sick until one can confidently conclude that he/she is well following a thorough assessment and period of observation.

10 Always reflect on the potential fears of the child and parents.

Introduction Who sees paediatric emergencies? It is essential that all doctors are familiar with the recognition and management of the seriously ill child. The majority of children presenting to emergency departments (EDs) are taken to mixed departments that see both adults and children, while a number present to tertiary paediatric centres. Occasionally, children will arrive, due to close proximity in an emergency, at adult departments where staff may be less familiar with their management. Likewise, paediatric emergencies occur remote from EDs and may require stabilisation by general practitioners, paramedical staff or laypersons prior to subsequent referral. It is an important role of EDs to be an available resource to support the community in the management of paediatric emergencies. This function may occur through liaison, education, and the provision of advice. Some critically ill children will arrive in a more predictable fashion via ambulance, and some preparation can occur to plan for their initial treatment. On the other hand, a child in extremis may arrive unannounced, rushed in from a family car. Systems of preparedness for these situations are critical for the immediate assessment and optimal early management of children by ED staff (see Section 2).

Identifying the potentially sick child Of the vast number of children attending EDs, approximately 2–5% are classified as immediate emergencies (Australasian Triage Scale (ATS) 1 and 2) that require urgent assessment and management.1 Importantly, while children can present with less urgent triage categories they may rapidly deteriorate from evolving sepsis or airway compromise. The majority of paediatric presentations consist of less emergent problems involving a wide spectrum of injuries and illness. Of this group, there is a subset where the diagnosis is not immediately apparent. Thus, paediatric patients can generally be divided into three broad groups: the obviously well, the obviously sick, or the potentially sick child. One of the major

tasks for the emergency physician is to identify the ‘sick child’ from a large, undifferentiated group of children who may present as potentially sick. It is by a ‘filtering process’ via history, observation, examination, appropriate investigation and consultation that one identifies the potentially sick child (Fig. 1.1.1). This group of patients includes: those children who have progressed to a severe form of a usually benign illness; those with early, subtle signs of a serious disease; or those who on initial assessment appear unwell but require observation or investigation to help rule out serious disease. It is often through observation of a child that one is able to more accurately assess each of these possibilities.2 With experience, the ability to appreciate a ‘sick child’ improves. Serial observation and considered investigation can be of use in identifying children with life-threatening conditions. Ongoing uncertainty of the underlying problem may provide justification for admission for a more prolonged period of observation. If a life-threatening problem like sepsis is considered, treatment should be initiated after collection of appropriate pathology samples where this can be done in a suitable time frame.

FIG. 1.1.1 Algorithm of paediatric decision making.

Children with fever

The concept of ‘occult bacteraemia’ (OB) highlights the difficulties in detecting significant illness in febrile young children. With the introduction of widespread vaccination to the common agents of OB (HiB, Pneumococcus) the prevalence of paediatric sepsis has diminished significantly, and the clinical experience of managing septic children has been diluted in developed countries. Hence, one needs to have a planned approach to the assessment of febrile children at various ages. Bacteraemia in its most obvious form presents as a febrile, pale, pasty, mottled child, centrally warm but with cool peripheries. Some young children with bacteraemia, however, can appear completely well apart from fever. Investigations may demonstrate a high white cell count or elevated C-reactive protein (CRP), but these inflammatory markers are unfortunately often nondiscriminatory between benign and serious causes. The problem is not so much that children with OB are sick at the time of initial assessment, but the possibility of the later development of serious bacterial sequelae necessitates timely treatment. However, many bacteraemic children will spontaneously clear the organism without therapy. Therefore, these children remain in the potentially sick category of patients and should have either admission for observation or discharge with frequent planned reviews for sequelae and a definitive action plan for their parents should the condition change.

Evolving illness in children Due to differences in anatomy, physiology, development and psychology, children’s diseases are age-specific, with serious illness often taking time to evolve.3 Many children present to an ED in the early stage of an illness, and making a definitive diagnosis may require time and repeated review. The clinical status of paediatric patients may also change rapidly as they can compensate remarkably well during serious illness. Deterioration can occur in response to prior trauma, evolving sepsis, toxin absorption or a seizure and necessitate a change in the initial priority to receive treatment. The younger the child, the greater the potential for rapid deterioration as the early manifestations of a serious illness may be subtle and non-specific. For these reasons clinicians must be vigilant for the early signs of compensated shock such as tachycardia, decreased capillary refill, mottled skin, cool peripheries, decreased urine output, or drowsiness. Early detection and fluid

resuscitation at this point may prevent hypotension in a child with evolving sepsis. Children with severe and deteriorating respiratory illness will manifest fatigue. It is the early recognition of children with serious illness or the potential to deteriorate that is critical to the timely initiation of effective treatment.2 An important principle in emergency paediatrics is to be proactive. One must be aware of the importance of regularly reviewing a child’s response to a given therapy, escalate treatment if required and be vigilant for subtle signs of deterioration.

The environment The physical environment of the ED needs to be child and family friendly with appropriately equipped cubicles for the reception of children accompanied by their carers. Despite the noise inherent in a busy department of sick children, the environment should be as calm and relaxed as possible. Ideally, the care of children is managed in an area separate to adult patients thus shielding children from what can at times be a confronting environment in the emergency department. Wall or ceiling posters, mobiles, and a selection of toys and books are useful to distract younger children from the distress and threat of an unfamiliar hospital environment. Familiar characters from current movies and television shows can provide distraction as well as facilitating central nervous system (CNS) assessment through recognition. A few initial moments gaining a child’s confidence with a toy or bubbles will usually reward the clinician with a more rapid and thorough assessment of the reluctant child. Stickers or bravery certificates are excellent rewards to have on hand for young frightened children who have undergone imaging or blood tests. Even in a mixed department children should be completely separated from adult patients. A separate waiting area set up for children and families is also highly recommended. Adult patients who are behaviourally disturbed, severely ill, or covered in blood will be distressing for a child and family to see or hear in a nearby cubicle. Departments should be designed such that this is a very unlikely scenario. Likewise, if a child is to undergo a procedure during which he/she may become distressed, such as intravenous insertion or laceration repair, it is best performed in a closed dedicated procedure room. This will avoid visual or auditory distress to other children and parents. A mounted television/video

monitor in this setting can be an excellent distraction during procedures, as an adjunct to analgesia and sedation. If available, play therapists provide an invaluable asset to assist explaining procedures in an age-appropriate manner to children and parents as well as assisting in distraction during the procedure. Comfort positioning with a parent is preferable to the former practice of wrapping children in a sheet for the procedure. For neonates and small infants, a radiant heater over the examination bed will aid temperature stability, examination and often the discovery of veins for cannulation. The paediatric resuscitation area should include wall charts, which refer to emergency algorithms and drug dose guidelines, which can be rapidly referred to during the resuscitation of critically ill children. Although white boards may be useful for pre-sizing and dose calculations for the imminent arrival of a sick child, this is prone to error, particularly during a stressful paediatric resuscitation. Where possible, resources which have pre-calculated doses and equipment sizes should be available to clinicians. These include the Broselow tape, medication books, software programs or smartphone apps. Hospitals should have agreed and readily accessible weight-based protocols on preparation and administration of emergency medications and infusions for children. Updated electronic and hard-copy clinical guidelines for the management of common paediatric emergency conditions are useful. State or national guidelines and pathways can ensure consistency of management from all levels of clinical staff, as well as improving continuity of care in children who require admission to an inpatient unit. Patient/parent information resources such as handouts, leaflets and information videos should also be available. They should cover a range of common paediatric conditions and ideally be translated into languages which reflect the demographics of the hospital’s local community.

Triage Paediatric patients arriving in the ED undergo triage according to the standardised Australasian Triage Scale (ATS 1–5) or equivalent so that they are seen in a prioritised fashion according to urgency. In mixed EDs where triage nurses may have had less paediatric experience, there has been a tendency to uptriage paediatric patients.1 The use of scoring systems for specific conditions or a Triage Observation Tool may be helpful in improving the reliability of triage in young children, who may present with non-specific symptomatology.3

Children with life-threatening conditions may not be easily recognised during brief triage assessments. A secondary nursing assessment should occur as early as possible, with further observations performed at the bedside, so that any change in condition can be detected early and acted on promptly. The senior doctor in the department should immediately be informed of children triaged as ATS 1 or 2 to direct timely management. The senior doctor and nurse should also be informed of any child with vital signs that are in the range to otherwise trigger a medical emergency team (MET) call. In times of high workload, children with an ATS 3 may not be definitively assessed within 30 minutes and should have a senior doctor or nurse rapidly assess status and initiate therapy, if required. It may be necessary to modify normal triage systems when ED numbers are affected by surges in demand such as during significant influenza outbreaks or similar events.

Front loading care Some initiation of treatment is appropriate during the triage process or soon after. This can include the provision of analgesia for pain or an antipyretic in a miserable febrile child. It is important that all patients with pain are given early and appropriate analgesia or have injuries splinted when required. This will facilitate a more comfortable, reliable and expeditious assessment. The use of opiates, when required, will only enhance, rather than detract from, the subsequent physician’s physical examination.4 The use of visual analogue scales such as the Wong–Baker faces5 may assist the assessment of a child’s response to analgesia as may asking the parents if their child is in pain. A process of fast tracking appropriate children with isolated limb injuries for an X-ray prior to definitive medical review may improve efficiency through the department. Other interventions which may be instituted early in a child’s ED visit include: topical anaesthesia for open wounds, topical anaesthetic creams for those likely to require intravenous access or venipuncture, oral ondansetron for those who are actively vomiting, oral rehydration fluids or icypoles for gastroenteritis, corticosteroids for croup, commencement of bronchodilators for asthma, and provision of relevant information leaflets. Febrile children who present with a rash, not clearly due to a viral exanthema or benign phenomena, should be fast tracked to be seen by a senior doctor to consider the possibility of meningococcaemia. It is useful to have documented management plans for children who may

repeatedly present to the department. This includes children with complex healthcare needs, brittle asthma, cyclical vomiting, metabolic conditions or recalcitrant seizures where a clear plan of management can expedite care by ED staff. These management plans are often developed as a result of cooperative efforts between the ED and the child’s usual specialists.

The paediatric approach The evaluation process of a child in the ED involves history, observation, examination and sometimes investigations. Each of these components needs to be considered in the formulation of a diagnosis and disposition plan. A child needs to be considered in the context of the family. The assessment of children in the ED setting can be both challenging and very rewarding. It is a challenge to modify the clinical approach according to the chronological and developmental level of the individual child. Likewise, treating paediatric patients is a rewarding area of emergency medicine, as children will often respond rapidly to management within the time frame of the ED attendance.

Gaining rapport Efforts to gain initial rapport with a child and the confidence of the parents are the key to assessing children in the ED setting. An unrushed, gentle and caring manner will rapidly settle the fears and anxieties of most children and their parents. Playing simple games with a child can often provide useful information as well as building trust and rapport. This usually allows the examination to proceed in a non-threatening fashion and improves the reliability of clinical signs. It will take time, experience and the observation of colleagues’ techniques for every emergency physician to develop his/her own individual approach to children. A thorough examination without causing distress to a child is very reassuring to a parent. Many children arrive at an ED miserable, in pain, fearful, or with some trepidation of what lies ahead. With a child-friendly approach by all staff, most will leave feeling much better and, hopefully, even having enjoyed the experience.

Age appropriate

The approach to any child in the ED is dictated by the child’s age and developmental level. It is useful to have a modified approach to suit newborns, infants, toddlers, preschoolers, school-children and adolescents. An understanding of the range of normal neonatal behaviours, which will often precipitate ED visits, can assist in reassuring new parents that their baby is in fact well (see Chapter 3.1). This understanding will also assist in recognising when behaviour falls outside of the expected normal range and may indicate an underlying problem requiring further evaluation. A preverbal or developmentally delayed child won’t tell you of pain which has shifted to the right iliac fossa. An unwell 14-month-old clinging to his/her mother may actively resist a stranger’s initial attempts to examine him/her. The absence of familiarity with a family or child that his/her usual doctor may have may further impede the assessment of anxious children. When explaining procedures to children it is important to be age appropriate and above all honest. Explain in age-appropriate terms what it may feel like, but also create an environment to suitably reassure and distract the child.6 Maintaining a child’s trust at all times is crucial and will positively influence any subsequent medical contacts the child may have. The demonstration of a procedure on a doll may decrease the anticipatory trepidation in a child. Additional resources include online videos and procedure-specific handouts. When available, play therapists and experienced nursing staff are excellent sources of information for parents and children prior to procedures. The assessment of a child should always be carried out in the presence of the parent or carer, unless the child arrives by ambulance or other means without the parent/carer present and the child’s medical needs warrant immediate attention. Otherwise, it is prudent in the non-urgent situation to provide a staff member to support the child and defer the assessment until carers are present. Non-urgent procedures in younger children are best carried out with the child in a comfortable position (usually the parent’s lap), with suitable distraction and good anaesthesia/analgesia.

Developmentally appropriate Infants particularly benefit from the constant presence of their parent in their visual field in order to avoid stranger distress. It is often easier to examine children in their parent’s arms. Neonates can be examined on the ED bed as long as they are kept warm. It is a useful sign of illness or other pathology to note

when young children do not exhibit these normal stranger anxieties. The preschooler who enjoys a sense of play and imagination can usually be relaxed during an examination or procedure by storytelling or engaging in play with a toy. An anxious early school-aged child may respond to participation in the examination or being asked about school or other favoured activities. Adolescents, on the other hand, need to be approached in a more adult fashion and should be offered confidentiality and the opportunity to choose whether their parents are present (see Chapter 30.1). In the event of an uncooperative child resisting any examination, one may have to modify the approach to gain essential clinical findings in a gentle, sensitive manner. It is unusual, however, for a child to remain ‘unexaminable’ if appropriate analgesia is given and the child is left undisturbed for a period of time. Often observation will provide as much or more useful information than a traditional examination.

Parental involvement In order to provide emotional support, parents should be encouraged to remain close to their child during any procedures. Ideally, children can sit on their parent’s laps on the bed or trolley during the procedure. Parents who appear at risk of vagal syncope need to be safely positioned in the procedure room. This is another advantage of having them cuddle their children on the trolley where possible. Children’s behaviour often mirrors that of their parents, so gaining the confidence of the parent will often make an anxious child relax prior to procedures. The use of a confident, calm, caring approach will be rewarded by a child who will allow a more reliable examination. It is very reassuring to the parents to see that the doctor is experienced and comfortable in dealing with children and anticipates the expected anxieties and reluctance to examination that a child may have when unwell.

History The initial contact with the family must include an introduction of who you are and your role. The parents should be addressed and the child greeted by name, in an age-appropriate manner. It is important to consider one’s approach in terms of the needs of both the child’s illness and the parental concerns. The history is generally elicited from the parent or caregiver, but it is appropriate, in a verbal

child, to augment this information by directly questioning the child. Referral letters and/or ambulance documentation should be reviewed to ensure a complete understanding of the child’s visit to the ED. Other useful sources of information include hospital medical records, specialist letters and the child’s baby record book which contains details of the child’s immunisation, growth and development.

Critically ill child Sometimes the normal routine of history, followed by examination, will need to be altered in a child who arrives critically ill. The management will need to be expeditious and occur simultaneously with the gathering of pertinent information from the parents. Parents must be given the opportunity to remain at the bedside of their critically ill child undergoing resuscitation, with a capable support person.

Parental issues The clarity of the history given by parents can be affected by parental distress, anxiety or sleep deprivation. One should begin the history in a focused manner according to the presenting complaint. Later it may be useful to explore individual parental anxieties. One of the most important questions to a parent is ‘What is your biggest worry or fear?’ Some parents may have specific concerns such as a fear their febrile child has meningococcal disease when community alertness to this condition is heightened. Addressing this concern may occupy most of the doctor’s time. The parent of a child who has sustained an accidental scald or injury may be feeling distressed or guilty, and sensitivity to this is required. Again, addressing guilt will involve much of the doctor’s time.

Child-specific issues In younger children, certain symptoms are less specific. The report of vomiting in an infant may be due to meningitis, pneumonia, tonsillitis or urinary sepsis rather than gastroenteritis. The assessment of wellness or otherwise in infants can be more challenging due to their limited psychomotor activities. Indeed, their spectrum of normal behaviours involves sleeping and waking to cry or demand a feed, followed by a return to sleep. Hence, it is important to enquire

into their feeding status and sleep/activity pattern as an indicator of compromise due to illness. One needs to carefully clarify what their current intake is compared to their normal breast- or bottle-feeding. An infant who is feeding less than 50% of normal may have significant compromise. Urine production and the frequency of nappy changes can be used as a rough guide to the adequacy of intake. Box 1.1.1 History warning bells • Child taking less than 50% of normal fluids • The child with prolonged lethargy • No urine output for 6 hours • Prolonged irritability or inconsolability • Report of cyanosis, pallor, seizures or significant apnoea • The child who has not smiled over a period of hours • Nursing staff feel the child is ‘just not right’ • Unplanned re-presentations • Parental concerns out of proportion to child’s illness • Brought in by ambulance • History not compatible with injury/non-accidental injury It is important to note the report of a young febrile child who remains lethargic and fails to smile or interact with parents. In the otherwise well-looking infant, who appears mottled, clarify with parents whether this may be usual for their child (i.e. physiological cutis marmoratum versus sepsis). In assessing young children with trauma, a thorough history of the timing and mechanism of injury, noting the child’s developmental capabilities, is paramount to detecting possible non-accidental injuries (see Chapter 18.2 on Child at Risk). Nonaccidental injuries must always be actively considered when assessing a child who has presented with trauma. Other useful information to cover in the paediatric patient history is shown in Boxes 1.1.1 and 1.1.2.

Examination

Age appropriate The examination technique used in paediatric patients depends on the age and developmental level of the child. The key is to gain the confidence and then the cooperation of the child. Older children are generally examined in a systematic fashion similar to adult patients. However, younger children usually need to be examined in a less formalised and opportunistic manner, whilst maintaining a high degree of vigilance. The order of the examination frequently needs to be adapted according to the individual child’s responses and presenting problem. In a reluctant child, clinical findings may be achieved by surreptitiously examining through play as the opportunity arises. This is often an enjoyable and informative process for the clinician. Much can be ascertained in this situation by careful observation rather than hurrying to complete a clinical examination. Entertaining a young child in a professional manner during examination will generally allow the confounding influence of anxiety to diminish. Potentially significant examination findings are outlined in Box 1.1.3. Box 1.1.2 Important elements of the paediatric

history Presenting complaint Pregnancy Delivery – gestational age, prolonged rupture of membranes, delivery type, APGARS, birth weight, need for resuscitation or special care nursery admission Development – in a CNS problem, compatibility with injury mechanism Immunisation status – need to clarify carefully Previous illnesses/surgery/admissions/medications Allergies Infectious contacts/recent travel Family history Social history – family circumstances may influence a child’s disposition significantly Fasting status if relevant Feeds – normal bottle or breast feeds for comparison Urine output – number of wet nappies

Box 1.1.3 Examination warning bells The pale, pasty child The floppy child The child who appears drowsy Alteration in vital signs, SpO2 Early signs of compensated shock The tiring child with respiratory distress The child who never smiles despite appropriate prompting The child who looks sicker than the usual child with gastroenteritis/croup/bronchiolitis/URTI Other specific signs Non-blanching rash – petechiae/purpura-sepsis Widespread blanching rash – toxin-mediated illness, including toxic shock Bulging or full fontanelle – raised intracranial pressure Bilious (green) vomiting – bowel obstruction High-pitched cry – meningitis Grunting – respiratory distress

Gentle, distraction, painful last Children are usually reluctant to have any painful area disturbed. Confirming tenderness needs to be gentle and unhurried to minimise any distress, with appropriate prior analgesia. Many young children will respond to ageappropriate verbal banter during the examination, which distracts from the perceived threat of the examining hand. Alternatively, one may need to gently palpate a tender right iliac fossa, whilst using distraction such as the counting of the child’s fingers. Sometimes a child may prefer their tender abdomen to be palpated with the examiner’s hand ‘through their own hand’. The examination needs to be adapted to the child’s responses, deferring distressing phases until the final moment of examination. Time used initially to gain a child’s confidence will make subsequent assessment more rewarding and the clinical signs more reliable. It can be difficult and sometimes impossible to accurately make an assessment in an upset and distressed child.

Often the most reliable method of excluding peritonism or other serious problems in a child does not involve any palpation of the abdomen or specific examination. Asking a child to cough, walk, run, jump or climb the trolley is a useful manoeuvre to help exclude peritoneal irritation and will give a lot of useful information in other scenarios too. The examination of ears and throat, a tender abdomen or a painful injury is best left until last in order to minimise upsetting a child and make the remaining routine examination difficult. If one detects that a child has an unfortunate fearful memory of a stethoscope or the like, a preliminary auscultation of a child’s soft toy and warming the diaphragm will often allow this to subside.

Improvise The examination of infants and young children is best done in the least threatening position. This is usually with the child being held comfortably in the parent’s arms or on the knee. If a young child is sleeping, the opportunity should be taken to perform auscultation and palpate a fontanelle/abdomen, which will be altered in the crying state, prior to disturbing the child to wakefulness. A neonate examined supine needs to be kept warm with a blanket or a radiant heater. Hands should be warm. The crying fractious baby may be settled by offering a feed or a pacifier before the examination. The symmetry of normal infant reflexes is a useful screen for any focal motor problem or as a localiser of a painful limb that will modify normal symmetry of response. If possible, it is best to avoid waking a sleeping child. Instead and with parental agreement, return later and complete your assessment if this is clinically appropriate.

Observation Your examination through observation begins prior to introducing oneself to the family and continues after the traditional examination, whilst writing up notes or between seeing another patient. It also includes reading and acknowledging nursing remarks and vital signs recorded in order to obtain additional information about the child’s presenting problem. The trends of vital signs over time are useful indicators to detect evidence of disease progression or response to therapy. This ‘ongoing triage’, in effect, is particularly important to detect ‘evolving illness’ that may otherwise remain undetected. Subtle features that may be missed on examination include persistent tachycardia or tachypnoea that

is not clearly related to fever. Highly abnormal vital signs that fail to improve should not be attributed to fear, pain or distress and should raise concern of serious underlying pathology. One needs to be alert to the spectrum of stigmata of non-accidental injuries that may present to the ED (Chapter 18.2).

Observational variables The general appearance of a child should include noting his/her level of alertness, eye contact, activity, quality of cry, posture, interaction with the environment, irritability, colour, hydration, perfusion, general growth and nutrition, respiratory distress and presence of any unusual smell (e.g. ketones). The lack of normal resistance to examination or a procedure expected of a child is an important observation to note. The sick child may make none of the resistance expected to examination or venipuncture. Observational variables have been shown to be more predictive of serious disease than historical information in young children.7 Likewise, clinical examination, considered alone, is a poor predictor of serious illness. Observation of a child needs to be performed as a separate process from the examination and may require a period of time for re-evaluation to detect disease progress. Researchers have used formalised scales such as the McCarthy Observation Scales to aid this assessment in febrile children.8 In the ED setting, discussion of a child with a colleague can be a rewarding aid to decision making. A child’s posture, undisturbed, can be a useful clue to systemic illness, abnormal neuromuscular function or a painful limb or joint. Children with sepsis or meningitis may be floppy or flaccid. In other cases, the only sign of meningeal irritation may be a child who is holding his/her neck in a slightly extended position.

Observing breathing When observing tachypnoea in a child, it is useful to determine whether a child has ‘quiet tachypnoea’ (breathing fast and quietly) with no evidence of increased work of breathing, such as may occur in conditions of fever, acidosis or cyanotic heart disease. This should serve as a trigger to look more broadly for the source of illness. Children with ‘noisy tachypnoea’ (breathing fast and hard) demonstrate increased work of breathing, due to conditions such as airway obstruction, pneumonia or heart failure.

Confounders There are several observational confounders that can influence initial decision making, when children can appear transiently sicker than they really are. Frequently, an initially sick-appearing child can pick up and appear well again over a short period of time. This may be due to a response from analgesics or antipyretics and generally occurs within an hour or so of administration. The vomiting child will often look pale and ‘pasty’ for up to 20 minutes post vomiting. Children initially emerging from a simple febrile convulsion can look well again in 20 to 30 minutes. A young child’s physiological sleep can mimic septic drowsiness or somnolence resulting from head trauma. A fearful child, during examination, can escalate the examiner’s perception of his illness.

Re-evaluate This reinforces the power of a period of observation (Box 1.1.4). It allows time for a trial of fluids, reducing fever with an antipyretic, or seeing if a child responds to distraction. Subsequent re-evaluation of the child often allows one to differentiate whether a child is sick or well. The use of observation really allows one to identify the persistence of the initial abnormal examination findings. A child with intussusception may intermittently appear well, and observation may be required to observe the episode to prompt the appropriate diagnostic investigation. Box 1.1.4 Observation warning bells Decreased level of alertness, activity, eye contact Drowsiness or decreased interaction with the environment/parents Abnormal posture Abnormal quality of cry Prolonged irritability or inconsolability Ongoing pallor Decreased peripheral perfusion or hydration appearance Persistence of abnormal recorded vital signs Respiratory distress/tachypnoea (‘quiet’ or ‘noisy’) Persistence of examination warning bells Confounders – post vomit/seizure/head injury, high fever, normal sleep,

anxiety

Respiratory examination Noisy breathing in children can sometimes be difficult to determine whether it is due to airway obstruction of intra- (lower airway) or extra-thoracic (upper airway) origin. The localisation of airway obstruction to a particular segment of airway can often be aided by successive auscultation over the nares, mouth, larynx and peripheral airways. Remember, young children may manifest both upper and lower airway involvement (‘asthma/croup/bronchiolitis’) with inflammatory involvement of both segments of the respiratory tract. Younger children are often easier to auscultate by listening through clothes (avoiding the ‘stethoscope–cry reflex’) from behind whilst being held by the parent. Modern stethoscopes tend not to be cold when placed on skin. Detection of ‘occult’ asthma in a child with suggestive symptoms but no wheeze may be aided by comparing the diminished volume and rate of airflow in expiration compared to inspiration or alternatively re-auscultation after exercising the child in the ED. Wheeze may also be unmasked through a therapeutic trial of salbutamol. Young children with throat discomfort will be reluctant to volunteer a cough, but a gentle tickle of the axilla or palpating the anterior larynx will usually produce a bark to clarify suspicion of croup. Recognising the pattern of respiratory distress in a child from the end of the bed will often differentiate upper and lower airway obstruction, prior to any auscultation. Children with upper airway obstruction have slower inspiration, whereas gas-trapped wheezers will have diminished flow and speed of expiration on observation.

Abdominal examination The abdominal examination needs to always conclude with the nappy area for otherwise occult torsions, hernias, skin problems and for stool examination, if present. Rectal examination in children is not routine and should only be performed with clear indication and almost always by a paediatric surgeon. Privacy and dignity must be maintained, particularly when examining older children and adolescents. The examination of a child with possible sexual abuse is outlined in Chapter 18.1.

ENT last In preschool age and younger children examination of the ears and throat is best deferred to last. A gentle but rapid approach is necessary to achieve an accurate assessment of the oropharynx, followed by a cuddle from the parent. Despite the potential difficulty, the source of fever will often be overlooked if the throat is inadequately visualised in children. In infants, the throat is best examined with the child sitting in his/her parent’s lap with both arms cuddled by one of the parent’s arms whilst his/her other arm secures the child’s head. A young child who is fearful of throat examination needs to be held as still as possible for a rapid ‘one gag, one look’ approach. Be careful though as this is a great technique for creating an infective aerosol that you may come to regret in several days’ time. ‘Let’s count your teeth’ is a less threatening signal to most children to open the mouth, rather than mentioning ‘the tonsil or throat’ words, particularly if parents warn you that ‘Nobody has been able to get a look at his throat’. As children get older, this examination becomes easier to perform. Following any distressing procedure, it is important to acknowledge bravery in a frightened child. Likewise, giving a child an honest, developmentally appropriate explanation of what to expect prior to any procedure, such as an IV insertion, must always occur. This is best done immediately prior to the procedure so that an anxious child’s fears don’t escalate in the intervening period.6

When to investigate Investigations in children in the ED should be judicious and considered. Investigations serve more than one purpose. They might help confirm or refute clinical suspicions. Occasionally, parents may appear to initially want more reassurance than simple clinical assessment and explanation. In this situation, the utility of investigations (along with the associated distress and discomfort obtaining them) needs to be placed into context for the parents. Serial review over time may often prove a more effective and less distressing technique to evaluate a child for a serious illness. Investigation for parental reassurance should not be a routine practice unless also clinically appropriate.

The parents Parents who accompany their child to an ED are often anxious and fearful

regarding the safety of their child. It is important to consider that the parents are entrusting the doctor with the wellbeing of their most cherished and precious possession. The management of their fears and the identification of their needs and expectations are important roles of the doctor attending to their child. Listening to and addressing the parents’ concerns in a sympathetic and unhurried fashion is often the main therapeutic strategy to reassure an anxious parent that a child with a relatively minor illness is safe. Many parents may be sleep deprived due to attending to their sick child, and this will influence their ability to convey a lucid history or to receive new information. The time spent at triage or in the waiting area in a busy ED can frustrate the most patient parent. This needs to be anticipated and acknowledged at the start of the consultation. Sensitivity to potential cultural issues is important in all interactions with carers.

Managing the parents The ED visit may follow previous medical consultation(s) where their concerns may not have been addressed or unfairly amplified, and it is important to explore these. Always acknowledge the parents’ fears and anxieties; however, medical judgement should allow an objective decision about whether a child is sick or not. An exception to this is the parents of children with a chronic illness or special needs (Chapter 1.2). They are usually correct when they judge that their child is sick. Aggressive and unreasonable carers will usually respond to a professional, polite and courteous senior doctor. There is usually a reason behind their behaviour that needs to be explored. Even the most anxious parent will usually respond to a thorough assessment of his/her child followed by an explanation of diagnosis and management. In unplanned second presentations where parents demand admission, it is usually best to admit.

Communication issues Explanations to parents as a general rule should be appropriate to their level of understanding and education. Provision of appropriate handouts – in the parents’ preferred language – early in the ED attendance allows parents to improve their knowledge while waiting to be seen, for investigations to occur, or for a period

of observation. This early information provision saves time at the end of the visit. Once a plan is determined, a verbal explanation reinforced by written instructions is useful to ensure optimal understanding. Reviewing a parent’s understanding of instructions prior to discharge will allow clarification and avoid communication problems. Parents may have fears related to anecdotal advice from family/friends, misinterpretation of media reporting, the internet and social media, or other sources, which need to be explored.Gaining the confidence of parents before they leave the department is an essential part of the therapeutic process and has a positive effect on compliance to therapy. It is useful to explain to parents the likely natural history of their child’s illness and encourage review should significant deviations from this occur. Where an ED assessment differs significantly from that of the referring clinician, great care must be taken to address differences in opinion and management plans. In addition, timely communication with the referring clinician should occur, in order to clarify any differences in clinical assessment and to provide an update on the child’s ED course. Parents will return to primary care doctors for ongoing treatment, and it is important for emergency physicians to maintain and build confidence in the community healthcare providers.

Management of febrile children The management of febrile, young children is a large part of emergency paediatric practice. Children less than 1 month old require a full septic evaluation if rectal temperature is greater than 38.0oC. This should include FBE and cultures of blood, urine and cerebrospinal fluid (CSF), followed by empirical antibiotics. A chest X-ray (CXR) may be required if symptoms and examination findings suggest chest infection. Febrile children between 1 and 3 months require a graded approach with FBE, blood culture and urine culture assisting to risk stratify the child. Collection of CSF for culture and administration of empirical antibiotics should occur if the child looks unwell. Children older than 3 months can be managed to a greater extent based upon clinical findings. A well, febrile child with a clear focus of infection can be managed as clinically indicated. Unwell children with a clear focus require further evaluation and admission for treatment. Children older than 3 months without a clear focus of infection who look well should have a clean urine sample collected for microscopy and culture. This well group should have

review arranged for the following day to assess progress and to check on laboratory results. Unwell-looking children in this age group without a clear focus should have a septic workup including FBE and blood and urine culture. A CXR and lumbar puncture (LP) may be indicated based upon clinical symptoms or signs. Admission for observation and potentially antibiotics can be arranged after discussion with the admitting paediatric service. Parents whose child is discharged home should be clearly instructed to return to the department if their child deteriorates. The discharge action plan should give clear and understandable instructions on when to return. For example, in the febrile child, this should include if the child becomes more unwell, with a decrease in oral intake to less than 50% normal, with no urine output for 6 hours, or the child becomes drowsy beyond his/her normal sleeping. Parents should be alerted to potential complications such as becoming limp, fitting or appearance of a rash, which warrant urgent review. Parents should always leave the ED feeling empowered to return for further medical care in the ED or with the GP if they feel their child is not improving or worsening. Never minimise or trivialise parental concern, as this may lead to reluctance to seek further medical advice. The presence of fever itself provokes considerable parental anxiety, often more pronounced in parents (and other family members) from specific cultural groups. This is important to address with explanation and provision of culturally appropriate written information which can be taken home and shared with the extended family. Additionally, it is important to avoid routine administration of antipyretics to all febrile children at triage, a practice which reinforces caregiver ‘fever phobia’.

Reasonable expectations Managing children is often about understanding the natural history of the illness and predicting when the child will improve. Presenting parents with a reasonable expectation of when they might expect their child to improve is one way of ensuring the child is safely managed and parents are reassured. This approach will not avoid the need for serial review in either the ED or by the GP, but it may provide some reassurance. A recent systematic review found that the expected duration of symptoms for some common childhood respiratory illnesses is quite prolonged: ear ache – up to 8 days; sore throat – up to 7 days; common cold – up to 15 days; acute cough – up to 25 days.9

Decision making Making decisions in paediatric emergency medicine is a balance of history, examination, intuition, knowing when to trust the parents and maintaining objectivity. If you feel uneasy with your diagnosis regarding a child, respect that feeling, and gain support until you do feel comfortable with your decision. There are many strategies to do this: 1. Consider early consultation with an emergency or paediatric consultant colleague. 2. Organise early follow-up in the ED or with a paediatrician or general practitioner. 3. Have a colleague on the floor listen to the story or examine the child. 4. Phone review the patient’s family yourself later that day or the next morning. 5. Provide a concrete action discharge plan. 6. Admit the patient for observation to either the ED observation ward or to the paediatric ward. 7. Empower the parents to return to the ED if they are concerned after discharge.

When to admit The decision to admit or discharge a child from the ED is easily made when the child requires medical care that is only available in the hospital setting. The receiving ward will need to have appropriate resources for the ongoing management of the child, which should be clarified by discussion with the receiving inpatient unit/paediatrician. Some children may need to be discussed with and transferred to a tertiary paediatric environment when they require, or have the potential to require, paediatric intensive care facilities or paediatric subspecialty management.

Factors influencing disposition However, many other factors need to be considered in the disposition decision (Box 1.1.5). The threshold to admit a child is influenced by the child’s age, availability of appropriate follow-up, assessment of parents’ ability to provide care and ongoing monitoring, the natural history of the illness and likelihood to

deteriorate, social factors, comorbidity, distance from hospital, time of day, parental anxiety levels, availability of an early paediatric opinion, and the possibility that a child may be at risk. One needs to assess in a non-judgmental fashion the ability of the parents to carry out any ongoing treatment and consider admission if there appears to be a need for ongoing support. When in doubt regarding whether or not to discharge a child, err on the side of caution. It may be prudent to consult, consider a period of observation in the ED, or admit the child to hospital.

Continuity of care It is important for ED staff to liaise closely with the admitting paediatrician to provide continuity of care and to ensure ongoing care is expedited in the ED. Ongoing management and monitoring of the patient are important roles of medical and nursing staff after this decision has been made, particularly if there is a delay in the transfer process. Any significant change in a child’s previous status or treatment needs to be communicated to the appropriate receiving team. The development of unstable vital signs or other evidence of severe illness requires an appropriate escalation in treatment, including activation of a medical emergency team or arranging inter-hospital transfer. Box 1.1.5 Factors influencing admission threshold Age of child Availability of appropriate follow-up/review Parental ability to provide care and monitoring, social factors including the care of other children Comorbidity Distance from hospital and ease of returning Time of presentation Parental anxiety levels To enable further observation or obtain a paediatrician opinion Possible child at risk outside hospital

Observation ward

Significant compromise from many childhood illnesses is often transient and will often respond rapidly to interventions commenced in the ED followed by a period of observation. Parents can often be reassured during this period of observation in hospital that their child has remained well and will respond to management strategies that subsequently can be continued at home. Studies have shown that many children admitted to hospital only require a limited period of in-patient therapy and are discharged in less than 24 hours.10 In a tertiary paediatric environment an effective way to manage these children is by admission to a short-stay observation ward. The ED needs to be appropriately resourced with staff to provide ongoing care and regular review of patients to expedite timely discharge. Conditions suitable for consideration of an observation ward admission will vary with local resources and may include asthma, croup, gastroenteritis, febrile convulsion, presumptive viral illnesses, non-surgical abdominal pain, minor head injuries and other trauma, post-sedation recovery or ingestions.11 In mixed departments, without the facility of a short-stay ward, it is often appropriate to use the paediatric ward to admit patients who would benefit from a period of observation.

Making a diagnosis Not every child leaving the ED will do so with a specific diagnosis. The unwell febrile child needs to have serious diagnoses such as meningitis considered, before making a diagnosis of viral illness. Many children without a clear diagnosis can be managed expectantly and safely discharged home with organised review by a local doctor or paediatrician or return to the department. Giving the parents clear instructions to return should the state of their child not follow an expected course is essential. Children are often seen early in the natural history of their illness, and a diagnosis will only become clear with time. It is important to communicate clearly, verbally or in writing, with the doctor who will be following up the child. Close liaison with a local doctor who has referred a patient to the ED is essential. One should always respect the concerns raised by a referring local doctor who usually has the advantage of familiarity with the child and family.

The role of the GP in paediatric emergency

management Introduction GPs are the cornerstone of the Australasian health system and often the child’s first point of contact when unwell or injured. They are uniquely placed to have an intimate working knowledge of the biological, psychological and social dynamics that impact on a child’s illness. GPs are involved in the long-term care of family members, often for many years and in some cases several generations. It is this continuity of care and ongoing relationship with a family that are invaluable in assessing and triaging presenting medical conditions in children and their subsequent management. This is particularly important when caring for the health of children, who are often seen in the early prodromal phase of serious illnesses. It is the skill of the GP to differentiate the possibility of a serious illness in a child, particularly during the seasonal peaks of febrile presentations. This child may be nonspecifically ‘different’ to similarly febrile children, but the experienced GP may just have a ‘gut feeling’ that a second opinion may be warranted and refer to the ED. There are two distinct areas where the GP plays a vital role in paediatric emergency management: 1. Assessment, initial stabilisation and transfer of the child to the paediatric ED of the clearly ‘unwell’ or ‘potentially unwell’ child 2. Ongoing management and follow-up of the child after discharge from a hospital encounter, particularly when a diagnosis is yet to be made.

Management prior to hospital care The GP is more often than not the first point of contact for the potentially unwell child. The fundamental clinical medical tools of history taking and examination are used to make an initial assessment of whether the child can be treated in the community or requires referral to an ED for further opinion and management. This can be a challenging task as the GP is not afforded the luxury of observation over time, readily available ancillary testing such as pathology and imaging, or an immediate further opinion from a specialist colleague. Particularly in the case of early or undifferentiated illness, the GP will need to make a judgement call on whether or not a child can be safely managed at home.

Experienced GPs will not only use traditional methods of history and examination but will also listen to their ‘gut feeling’ when assessing children. This may involve attaching importance to red flag symptoms or signs or heeding the warning signs reported by an anxious yet appropriately worried parent. This may depend on not only the medical status of the child but also the assessment of the social circumstances, education and competence of the parents/carers to detect their child is failing to ‘turn the corner’ or deteriorating. Often there is significant parental anxiety with an unwell child which cannot always be allayed by sound advice from an experienced GP when a child clearly has a self-limiting viral illness. There may be parental demands for pathology testing to ensure ‘nothing is missed’ even though these may be deemed inappropriate by the family doctor. Parents may also report significant symptoms such as fever, an infant not feeding normally, cough or stridor which may no longer be present at the time of presentation to the GP. Some auscultatory chest findings are dynamic and therefore have a fluctuating presence, such as wheezing in bronchiolitis, so may vary greatly between the time of the GP and ED visit. It is this complex interaction of medical and environmental factors which must be processed by the GP, often in the context of a 15-minute appointment. The outcome of this assessment may be the subsequent referral to hospital-level care. Remember that the GP’s decision is carefully considered with all the aforementioned factors coming into play. Some of the more common reasons for referral to the ED may include the following: • A serious time-critical illness which requires ambulance transfer, such as severe asthma, sepsis or meningitis • Non-time critical illness which may not be responding to communitybased treatment and requires further investigation or consideration of parenteral antibiotics, such as evolving pneumonia • Illness or injuries which are beyond the level of facilities available to the GP to manage, for example unstable limb fractures or those that require a period of supervised acute treatment and/or prolonged observation • Parental concern and anxiety which cannot be sufficiently allayed by the GP • Social factors whereby the child cannot be adequately cared for or progress if monitored in the home setting due to lack of family

resources. It is imperative once the decision has been made to refer the child on to the ED, that the clinical assessment and concerns of the GP are adequately communicated to the physician who will be the next link in the management chain. This is best done with a phone call to the ED outlining the reasons for referral. In potentially serious illnesses the ED clinician can provide initial phone advice. A referral letter which contains the child’s past medical history, allergies, immunisation status, list of current medications and any relevant investigations should accompany the child to the ED. This gives the ED doctor a head start in managing the child and avoids wasted time, effort and cost in repeating already established findings.

Management after hospital care Once the child has been managed and discharged from the ED the circle of communication should include verbal and written feedback to the referring GP. This timely discharge communication has several benefits. First and foremost, it ensures continuity of care for the child. If a treatment plan has been commenced by the hospital staff, the GP is then responsible for its implementation through continuing clinical assessment and adjustment of management according to progress. The natural history of illness and convalescence are dynamic processes which will vary from patient to patient and may require vigilant monitoring. This is most likely to be successful if the discharge plan is well communicated to the family doctor. It is vital that communication is not mislaid compromising patient care. The GP should receive information directly (fax or electronic) as well as via the patient or family as a backup if the usual communication systems fail. Second, medical practitioners continue to accumulate knowledge and expertise throughout their careers so that reflective and sensitive feedback concerning outcomes of their referred patients is useful. This helps the GP to analyse and reflect upon their decision-making processes and contribute to their evolving clinical acumen, which is a career-long journey for all doctors. This is particularly so in the case of paediatrics as recognising the potentially unwell child can sometimes be as much art as science. GPs can arrange further monitoring of the recovering child and are well

placed to arrange further tests (for example chest X-ray following complicated pneumonia) or specialist follow-up if needed. Often the busy ED is not the easiest place, especially after hours, to arrange such important steps in the child’s follow-up care. The GP is also able to assess any psychological impact of the child’s illness and offer ongoing support to the child and the family. These potential issues may not be evident at the time of the ED visit. Integral to the communication process is a mutual respect between the GP and the ED physician with both having an understanding and appreciation of the environment and challenges that each is working under. GPs have strong attachments to their patient and families and will appreciate a follow-up phone call and/or letter advising of the status of a referred child. The letter should be timely, with appropriate information including diagnosis, medication and results of investigations with an access phone number for any results pending. It should be presented in a clear concise form with a structured plan of management. Computer-generated letters are often more legible than hand-written ones and reduce the chance of miscommunication in the discharge process. Some GP clinics now have secure email availability and may prefer to receive information this way. If these strategies are implemented within a spirit of cooperation between GPs and EDs, this will ensure improved continuity of care and therefore better patient outcomes in the care of sick children.

Developmental milestones It is important to have an understanding of the major developmental milestones throughout childhood for the provision of care to paediatric patients. These can be rapidly confirmed by examination or parental enquiry. This allows the use of an appropriate age-modified approach in the child’s evaluation. Some specific behaviours, such as stranger anxiety in a 12-month-old, may challenge assessment, so it is important to adapt an approach to these expected behaviours. Significant deviations from normal warrant consideration of paediatric referral. Useful early milestones are shown in Table 1.1.1.

Growth It is essential to measure a child’s current weight on every ED visit in order to accurately dose any therapeutic drug and to quantify recent weight loss. Where a

child is critically unwell and unable to be weighed, estimation can be made via Broselow or other charts. Between the ages of 1 and 10 years an estimation of weight is 2 × (age + 4) kg. Standardised percentile growth charts are useful to confirm suspicion of failure to thrive or discrepancy in linear or cranial growth. The trend of growth plotted on a growth chart over time is more important than a single measurement. As a general rule, birth weight doubles by 5 months and triples by 1 year. Newborns are often discharged from hospital in the first few days of life and may present in the first week to an ED. Following the expected initial weight loss, term babies should normally regain their birth weight by the end of the first week. Appropriate neonatal weight gain is an important index of wellness and can be tracked on the growth or centile charts that all babies receive after birth. Head circumference increases by 2 cm in the first 3 months, 1 cm in the next 3 months, followed by 0.5 cm per month thereafter (Table 1.1.2). Table 1.1.1 Normal milestones in first 2 years of life Neonate

Lift head, visually fix for period

6 weeks

Smile, follow past midline

4 months

Roll over

6 months

Sit, transfer toy between hands

9 months

Stand holding on, crawl, stranger anxiety

12 months

Walking, single words

18 months

Explorer (trauma/poisons), tantrums, several words

2 years

Combine words, run, jump

Table 1.1.2

Adapted from Gary R. Fleisher, Stephen Ludwig, Textbook of Pediatric Emergency Medicine, 6E, Lippincott Williams & Wilkins, 2010.

Immunisation It is not the role of the ED to provide routine immunisations to children. It is useful, however, to clarify a child’s immunisation status with regard to the possibility of a particular infection such as epiglottitis, whooping cough or measles. Despite immunisation, children may still acquire these diseases but usually manifest a modified form of these infections. In children who are found to be incompletely or non-immunised, it is opportunistic to respectfully provide information regarding the normal vaccination schedule and refer to the local doctor or appropriate community facility for follow-up (Table 1.1.3). Being disrespectful or rude about parental choice to not vaccinate their child risks the loss of any therapeutic relationship with the family.

Vital signs It is necessary to interpret the vital signs according to the age of a particular child. A wall chart in the paediatric resuscitation area is a useful reference as a guide to these parameters. A good rule to remember is any child with a persistent respiratory rate >60 or heart rate >160 is definitely abnormal (Table 1.1.4). An important aspect of the care of children in the hospital setting is the increasing use and availability of ‘early warning tools’. These identify and flag abnormal age-appropriate vital signs, in order to escalate the level of care for children with potentially critical illness.

Reflection on the Practice of Paediatric Emergency The practice of paediatric emergency can be immensely rewarding. The opportunity to play games in the context of assessing patients is a wonderful way to spend a working day. However, great care and attention are required to identify those children with serious or life-threatening illness from those who have a viral illness. An unhurried, calm approach with serial review and close, careful follow-up can minimise the likelihood of missing a serious diagnosis. Involvement of the child’s carers is vital to ensure they are comfortable with the assessment made in the ED, have had an opportunity to ask questions and have been provided with clear follow-up instructions.

Table 1.1.3 Australian standard vaccination schedule (0–5 years) Age

Vaccine

Birth

HepB

2 months

HepB-DTPa-Hib-IPV; 13vPCV; Rotavirus

4 months

HepB-DTPa-Hib-IPV; 13vPCV; Rotavirus

6 months

HepB-DTPa-Hib-IPV; 13vPCV; Rotavirus

12 months

Hib-MenC; MMR

18 months

DTPa; MMRV

4 years

DTPa-IPV

Adapted from www.immunise.health.gov.au, Accessed December 2016. The most up to date schedule is available from the Immunise Australia website at www.immunise.health.gov.au., The footnote is as follows and is included in the online NIP schedule.∗ Rotavirus vaccine: third dose of vaccine is dependent on vaccine brand used. Contact your State or Territory Health Department for details.

Table 1.1.4

Adapted from Royal Children’s Hospital. Clinical practice guidelines resuscitation: emergency drug and fluid calculator. Melbourne, Australia, 2003.

It is a humbling situation to encounter a child with a life-threatening illness. In the event of an adverse outcome it is important to not only review the event but to care for the family and staff involved. The impact and distress caused by the death of a child are significant. By reading this textbook you are on a journey to be well positioned to recognise and manage the sick child and hopefully change the outcome for the better. For that, the child’s parents will be forever grateful.

Acknowledgements The contributions of Lisa Coutta, Ian Everitt, Andrew Jan and Andrew Harris as authors in previous editions of the textbook are hereby acknowledged.

References

1. Durojaiye L, O’Meara M. A study of triage of paediatric patients in Australia. Emerg Med. 2002;14:67–76. 2. Luten R.C. Recognition of the sick child. Problems in paediatric emergency medicine. New York: Churchill Livingstone; 1988:1–12. 3. Browne G.L, Gaudry P.L. A triage observation tool improves the reliability of the National Triage Scale in children. Emerg Med. 1997;9:283–338. 4. Browne G.J, Chong R.K.C, Gaudry P.L, eds. Principles and practice of children’s emergency care. Sydney: McLennan and Petty; 1997:1–5. 5. Wong-Baker Faces Foundation. http://wongbakerfaces.org/. 6. Royal Childrens Hospital, Melbourne. Clinical Practice Guideline: Communicating procedures to families. http://www.rch.org.au/clinicalguide/guideline_index/Communicating_procedures_to_ 7. Waskerwitz S, Berkelhamer J.E. Outpatient bacteraemia: clinical findings in children under two years with initial temperatures of 39.5°C or higher. J Paediatr. 1981;99(2):231–233. 8. McCarthy P.L, Sharpe M.R, Spiesel S.Z, et al. Predictive observation scales to identify serious illness in febrile children. Paediatrics. 1982;70(5):802–809. 9. Thompson M, Vodicka T.A, Blair P.S, et al. Duration of symptoms of respiratory tract infections in children: systematic review. BMJ. 2013;347:f7027. doi: 10.1136/bmj.f7027. 10. Browne G, Penna A. Short stay facilities. The future of efficient paediatric emergency services. Arch Dis Child. 1996;74:309–313. 11. Scribano P.V, Wiley J.F, Platt K. Use of an observation unit by a paediatric emergency department for common paediatric illnesses. Pediatr Emerg Care. 2001;17(5):321–323. 12. Fleischer G.R, Ludwig S. Understanding and meeting the unique needs of children. In: Fleischer G.R, Ludwig S, eds. Textbook of Paediatric Emergency Medicine. 3rd ed. Baltimore: Williams & Wilkins; 1993.

13. Royal Children’s Hospital. Clinical practice guidelines resuscitation: Emergency drug and fluid calculator. Melbourne: Australia; 2003. http://www.rch.org.au/clinicalguide/cpg.cfm? doc-id=5162.

1.2

Common chronic paediatric conditions Erin Mills

ESSENTIALS 1 Children with chronic medical conditions make up 10–20% of paediatric emergency department (ED) presentations. 2 These patients have a longer ED stay and have increased rates of hospital and intensive care unit admission. 3 Trust the parents/caregivers: if they are worried, you should be worried. 4 Minimise pain and anxiety associated with procedures.

Introduction Paediatric patients with chronic medical conditions (Table 1.2.1) make up 10– 20% of presentations to tertiary paediatric emergency departments (EDs).1,2 Compared to children without chronic medical conditions, these patients have a longer ED stay and are more likely to be admitted to both the ward and the paediatric intensive care unit (PICU).1–3 Therefore it is essential that the emergency physician is equipped to deal with this potentially complex group of patents. Table 1.2.1 Common chronic childhood conditions

Neurological

Epilepsy Cerebral palsy Ventriculoperitoneal shunt Spina bifida

Cardiac

Congenital heart disease Arrhythmias

Respiratory

Asthma Cystic fibrosis Bronchopulmonary dysplasia

Renal/urological

Vesico-ureteric reflux Chronic renal insufficiency Nephrotic syndrome

Haemato-oncological

Haemoglobinopathies Coagulation disorders Cancer Immunodeficiency

Endocrine

Diabetes mellitus Obesity

Developmental

Autism Attention-deficit/hyperactivity disorder

Other

Psychiatric disorders Gastroenterological problems Ear, nose and throat problems

The emergency physician may be involved in the initial diagnosis but is mostly required to recognise and manage disease complications as well as acute illness unrelated to the chronic condition. This group of patients can be very complex, with numerous medical and psychosocial issues and a baseline abnormal examination. It can be extremely difficult for the emergency physician, in a time-pressured ED scenario, to gain a thorough understanding of the patient and the level of deterioration. It is very important to listen to the parent or caregiver’s concerns, as well as involve the patient’s primary care team if possible. The parents are the best advocate for their child, and they know their child better than anybody else. If they are worried, the clinician should take this seriously. Chronic illness brings with it a range of stressors for the child and family, including those surrounding painful procedures such as venous access. As with any child, but especially in this population who are likely to be subjected to multiple procedures over time, it is critical to minimise the trauma surrounding potentially painful procedures. Tools used may include distraction, parental presence, play therapy, positioning on a parent, topical anaesthesia, pharmaceutical analgesia, or procedural sedation. In the appropriate clinical setting, oral midazolam 0.5 mg kg up to 20 mg can be mixed with sucrose and

administered 20–30 minutes prior to attempts at intravenous access. Inadequate pain management is known to have long-term negative effects on children, including diminishing effects of adequate analgesia for subsequent procedures or needle phobia, which make future procedures more traumatic for the child and more difficult for the clinician.4,5 Table 1.2.2

This chapter will provide an overview of some of the more commonly encountered chronic paediatric conditions, including cerebral palsy (CP), spina bifida, cystic fibrosis (CF), the ex-premature infant and autism spectrum disorder.

Cerebral palsy Introduction CP is not a single entity but rather a heterogenous collection of clinical syndromes characterised by abnormal motor patterns and postures (Table 1.2.2). It is the most common chronic motor disability of children, with a prevalence of approximately 1/500 live births.6 CP is caused by an insult to the developing brain, such as infection, preterm birth, intrauterine growth restriction, ischaemia, congenital malformations or head trauma. The degree of motor function may vary widely and can be communicated quickly using the GMFCS (Table 1.2.3). Children may have associated impairments including epilepsy or intellectual, speech, visual or hearing impairment. However, it is important to remember that many children have normal cognition.

Complications seen in the emergency department

Children with CP are susceptible to frequent illness because of their increased risk of epilepsy, respiratory complications and feeding difficulties, as well as complications associated with increased technology dependence. Increasing CP severity and complexity are associated with increased frequency of presentations, higher urgency of presentation and higher rates of hospital admission. The majority of presentations to the ED are due to respiratory or neurological causes, followed by gastrointestinal and musculoskeletal complications.7,8

Respiratory complications Respiratory illness is the leading cause of mortality in individuals with CP, and chest infections are the most common reason for children with CP to require hospital admission.9 History Predisposing factors include the following: • Oropharyngeal motor incoordination (leading to aspiration of food and secretions) • Gastro-oesophageal reflux • Poor cough and airway clearance • Immobility • Kyphoscoliosis • Sleep apnoea • Malnutrition, leading to atrophy and weakness of respiratory muscles. Causative organisms of pneumonia are similar to other children, though anaerobes may also be involved in the setting of possible aspiration. Risk factors for serious illness include previous ICU admission and severe scoliosis. Examination and investigation Assessment is similar to other children with respiratory illness: • Respiratory distress (respiratory rate, intercostal/subcostal recession, grunting) • Oxygen requirement • Respiratory failure (conscious state, pH and CO2 on venous blood gas)

• Wheeze and bronchodilator responsiveness. Note that chest X-rays will be difficult to interpret in those with severe scoliosis. Table 1.2.3 Gross motor function classification scale (GMFCS) I

Can run, jump and climb stairs, but speed, balance or coordination is impaired

II

Can walk, run and climb in most settings but may need mobility equipment for safety or travelling long distances

III

Can walk using assistance and can self-propel manual or powered wheelchairs

IV

Capable of walking with a walking frame but mostly relies on wheeled mobility

V

Little or no voluntary control of movement and requires extensive support in all situations

Management Oxygen and respiratory support • Give oxygen if the saturations are 60 mmol L. Sweat testing is only performed in specialised secondary and tertiary centres. Newborn screening programmes using the Guthrie blood spot test screen all newborns for CF. The initial screen is for raised concentrations of immunoreactive trypsinogen, with further testing as indicated. This screening is only about 95% sensitive, which means some cases will be missed. Antenatal screening is also offered. Early diagnosis and aggressive nutritional support improve growth and allow genetic counselling for the family; however, it may not improve pulmonary outcomes.

Management Most patients have care coordinated by a tertiary CF centre, which has been shown to improve outcomes.

Management principles • Segregation to prevent cross-infection: • Acquisition of some organisms, such as B. cepacia and P. aeruginosa are known to cause respiratory morbidity and accelerate respiratory decline. • Some of these organisms may be transferred from one patient with CF to another. Therefore children with CF should not share bays or waiting areas with other children with CF or with immunocompromised or other at-risk patients. • Airway clearance techniques: • Physiotherapy, mucolytics, hypertonic saline, bronchodilators. • Prevent respiratory infection: • Consider use of prophylactic antibiotics • Influenza vaccination. • Treat infective exacerbations: • Oral or IV antibiotics appropriate for culture. • Treat allergic bronchopulmonary aspergillosis (ABPA): • Prednisolone. • Reduce airway inflammation:

• Macrolide antibiotics. • Consider insertion of an indwelling IV access device: • Many patients requiring multiple courses of IV antibiotics will have poor venous access. • Manage exocrine insufficiency (malabsorption, steatorrhoea, poor growth): • Supplement with pancreatic enzymes and fat-soluble vitamins. • Manage constipation: • Dietary advice, laxatives. • Manage insulin deficiency or diabetes: • Insulin, high-fat diet, occasionally oral hypoglycaemic agents • Prevent osteopenia and pathological fractures: • Weight-bearing exercise, high dairy intake, vitamin D, consider bisphosphonates. • Manage infertility: • Assisted fertilisation techniques. Social and psychological support for patient and family:

Complications managed in the emergency department Respiratory Prompt and aggressive treatment of infective exacerbations is crucial to maintaining lung function, improving quality of life, and prolonging survival.14 History and examination • Suspect an infective exacerbation in the patient presenting with: • change in sputum volume or colour • increased cough • increased lethargy • anorexia or weight loss • increased dyspnoea • chest pain (consider pneumothorax). • Know results of recent sputum cultures: the presence or absence of particular colonising organisms (particularly Pseudomonas) will alter management.

• Consider aspergillus if there is new onset wheeze, pleuritic chest pain or new chest X-ray infiltrates. • Ask about associated viral symptoms. Investigations • Chest X-ray looking for new infiltrates, pneumothorax or effusion • Nasopharyngeal aspirate for viral aetiologies • Sputum culture for bacterial aetiologies • Pathology: FBE, UEC, CRP, BSL as baseline. LFT and IgE if indicated • Lung function tests if available (particularly VC and FEV1 to compare to baseline). Management • Oxygen and respiratory support as required • The decisions surrounding antibiotic treatment (route, number of agents, choice of agent, duration of treatment) are complex and should be made in conjunction with the usual treating physician. • Nebulised hypertonic saline and mucolytics may be beneficial • Chest physiotherapy • Nutritional support.

Gastrointestinal Intestinal obstruction syndromes Etiology • Meconium ileus, distal intestinal obstruction syndrome (DIOS) and constipation are all due to increased viscosity of intestinal mucus and increased transit time in CF.15 Clinical • Meconium ileus, unique to CF, is complete intestinal obstruction in the neonatal period due to accumulation of meconium. This presents with a clinical picture of bowel obstruction and no history of passing

meconium. • DIOS is partial or complete intestinal obstruction due to faecal accumulation in the terminal ileum and proximal colon. Patients usually present with abdominal pain, distension, vomiting and a right lower quadrant faecal mass which is palpable or visible on plain X-rays. If obstruction is complete they may have bilious vomiting and small intestinal air-fluid levels on abdominal X-rays. • Constipation may present in a similar way to DIOS, but the symptoms are usually milder and longer standing. Management • Meconium ileus requires referral to a paediatric surgeon who may treat conservatively with an enema or surgically. • Most DIOS episodes are treated conservatively with intensive inpatient laxative treatment, with surgical intervention rarely required. • Constipation can be managed with laxatives as an outpatient.

Endocrine Cystic fibrosis–related diabetes • The etiology of cystic fibrosis–related diabetes (CFRD) is complex and likely due to a combination of insulin deficiency and insulin resistance • Patients with CFRD have an accelerated decline in clinical status, including lung function, and have a higher mortality • Diagnosis is usually made via routine screening, usually with an oral glucose tolerance test • It is usually treated aggressively with insulin, though sulphonylureas may be used to delay insulin therapy in some cases • Diabetic ketoacidosis (DKA) is rare in CFRD, and if a CF patient presents to the ED with DKA he/she should be screened for type 1 diabetes mellitus (T1DM) Table 1.2.4 Nomenclature

Gestational age

The elapsed time between the last menstrual period and the date of delivery

Chronological age

The time elapsed since birth

Corrected age

Chronological age minus the number of weeks premature

Table 1.2.5 Complications of prematurity Neurological

Intraventricular haemorrhage Periventricular leukomalacia

Respiratory

Respiratory distress syndrome Chronic neonatal lung disease

Cardiac

Patent ductus arteriosus

Gastrointestinal

Necrotising enterocolitis Gastrointestinal reflux disease

Other

Developmental delay Growth reduction Hearing impairment Nosocomial infection Retinopathy of prematurity Iron deficiency anaemia Osteopenia

• Acute hypoglycaemia should be managed in the same way as in T1DM, with oral glucose if tolerated, or intramuscular glucagon or intravenous dextrose.

The ex-premature infant Introduction Advances in neonatal care have improved survival at the extreme of prematurity, and it is common to see ex-premature infants in the ED. Prematurity is defined as birth before 37 weeks’ gestation, with extreme prematurity describing those born before 28 weeks’ gestation. In Australia approximately 8% of babies are born prematurely, with extreme prematurity accounting for 1%.

Complications of prematurity There are many complications of prematurity (Table 1.2.5). This chapter will focus on longer-term complications of prematurity relevant to ED care.

Chronic neonatal lung disease Chronic lung disease (CLD) is defined as supplemental oxygen requirement past 36 weeks of gestational age and affects up to 40% those born at 6% by day 3), spherocytes or red cell fragments. Adapted from Management of hyperbilirubinemia in the newborn infant 35 or more weeks of gestation. Pediatrics. 2004;114(1):297–316.

Table 3.4.8

∗

Risk factors include haemolysis, sepsis, asphyxia, hypoalbuminaemia. Immediate exchange should be considered with bilirubin concentration >430 μmol L-1 510 μmol L-1 ≥48 hours or if the rate of rise is >8.5 μmol L-1 hr -1 despite intensive phototherapy (two overhead units and a BiliBed).

References 1. Riviere D, McKinlay C.J.D, Bloomfield F.H. Adaptation for life after birth: a review of neonatal physiology. Anaesth Intensive Care Med. 2016 In Press. 2. Calado C.S, Pereira A.G, Santos V.N, Castro M.J, Maio J.F. What brings newborns to the emergency department?: a 1-year study. Pediatr Emerg Care. 2009;25(4):244–248. 3. Perlman J.M, Wyllie J, Kattwinkel J, et al. Part 7: Neonatal resuscitation: 2015 International Consensus on Cardiopulmonary Resuscitation and Emergency Cardiovascular Care Science with Treatment Recommendations. Pediatrics. 2015;95:e169–e201. 4. Schmolzer G.M, O’Reilly M, Labossiere J, et al. 3:1 compression to ventilation ratio versus continuous chest compression with asynchronous ventilation in a porcine model of neonatal resuscitation. Resuscitation. 2014;85(2):270–275. 5. Solevag A.L, Dannevig I, Wyckoff M, Saugstad O.D, Nakstad B. Return of spontaneous circulation with a compression:ventilation ratio of 15:2 versus 3:1 in newborn pigs with cardiac arrest due to asphyxia. Arch Dis Child Fetal Neonatal Ed. 2011;96(6):F417–F421. 6. de Caen A.R, Maconochie I.K, Aickin R, et al. Part 6: Pediatric basic life support and pediatric advanced life support: 2015 International Consensus on Cardiopulmonary Resuscitation and Emergency Cardiovascular Science with Treatment Recommendations. Pediatrics. 2015;136(Suppl 2):S88–S119. 7. Brousseau T, Sharieff G.Q. Newborn emergencies: the first 30 days of life. Pediatr Clin North Am. 2006;53(1):69–84 vi. 8. Miller M.J, Martin R.J, Carlo W.A, Fouke J.M, Strohl K.P, Fanaroff A.A. Oral breathing in newborn infants. J Pediatr. 1985;107(3):465–469. 9. Olney R.S, Ailes E.C, Sontag M.K. Detection of critical congenital heart defects: review of contributions from prenatal and newborn screening. Semin Perinatol. 2015;39(3):230–237. 10. Thangaratinam S, Brown K, Zamora J, Khan K.S, Ewer A.K. Pulse oximetry screening for critical congenital heart defects in asymptomatic newborn babies: a systematic review and meta-

analysis. Lancet. 2012;379(9835):2459–2464. 11. Verma A, Rattan K.N, Yadav R. Neonatal intestinal obstruction: A 15 year experience in a tertiary care hospital. J Clin Diagn Res. 2016;10(2) Sc10-3. 12. Godbole P, Stringer M.D. Bilious vomiting in the newborn: How often is it pathologic? J Pediatr Surg. 2002;37(6):909–911. 13. Lilien L.D, Srinivasan G, Pyati S.P, Yeh T.F, Pildes R.S. Green vomiting in the first 72 hours in normal infants. Am J Dis Child. 1986;140(7):662–664. 14. de Lorijn F, Kremer L.C, Reitsma J.B, Benninga M.A. Diagnostic tests in Hirschsprung disease: a systematic review. J Pediatr Gastroenterol Nutr. 2006;42(5):496–505. 15. Krogh C, Fischer T.K, Skotte L, et al. Familial aggregation and heritability of pyloric stenosis. JAMA. 2010;303(23):2393–2399. 16. Oakley E.A, Barnett P.L. Is acid base determination an accurate predictor of pyloric stenosis? J Paediatr Child Health. 2000;36(6):587– 589. 17. Miozzari H.H, Tonz M, von Vigier R.O, Bianchetti M.G. Fluid resuscitation in infantile hypertrophic pyloric stenosis. Acta Paediatr. 2001;90(5):511–514. 18. Shah D.K, Wusthoff C.J, Clarke P, et al. Electrographic seizures are associated with brain injury in newborns undergoing therapeutic hypothermia. Arch Dis Child Fetal Neonatal Ed. 2014;99(3):F219– F224. 19. Murray D.M, Boylan G.B, Ali I, Ryan C.A, Murphy B.P, Connolly S. Defining the gap between electrographic seizure burden, clinical expression and staff recognition of neonatal seizures. Arch Dis Child Fetal Neonatal Ed. 2008;93(3):F187–F191. 20. Ferreira E, Carceller A.M, Agogue C, et al. Effects of selective serotonin reuptake inhibitors and venlafaxine during pregnancy in term and preterm neonates. Pediatrics. 2007;119(1):52–59. 21. Maurer V.O, Rizzi M, Bianchetti M.G, Ramelli G.P. Benign neonatal sleep myoclonus: a review of the literature. Pediatrics. 2010;125(4):e919–e924. 22. Filan P, Boylan G.B, Chorley G, et al. The relationship between the onset of electrographic seizure activity after birth and the time of

cerebral injury in utero. Br J Obstet Gynaecol. 2005;112(4):504–507. 23. Loman A.M, ter Horst H.J, Lambrechtsen F.A, Lunsing R.J. Neonatal seizures: aetiology by means of a standardized work-up. Eur J Paediatr Neurol. 2014;18(3):360–367. 24. Looney C.B, Smith J.K, Merck L.H, et al. Intracranial hemorrhage in asymptomatic neonates: prevalence on MR images and relationship to obstetric and neonatal risk factors. Radiology. 2007;242(2):535–541. 25. Wu Y.W, Hamrick S.E, Miller S.P, et al. Intraventricular hemorrhage in term neonates caused by sinovenous thrombosis. Ann Neurol. 2003;54(1):123–126. 26. Abend N.S, GutierrezColina A.M, Monk H.M, Dlugos D.J, Clancy R.R. Levetiracetam for treatment of neonatal seizures. J Child Neurol. 2011;26(4):465–470. 27. Berardi A, Rossi C, Lugli L, et al. Group B streptococcus late-onset disease: 2003-2010. Pediatrics. 2013;131(2):e361–e368. 28. Isaac A, Zhang H, Soon S.R, Campbell S, El-Hakim H. A systematic review of the evidence on spontaneous resolution of laryngomalacia and its symptoms. Int J Pediatr Otorhinolaryngol. 2016;83:78–83. 29. Carter J, Rahbar R, Brigger M, et al. International Pediatric ORL Group (IPOG) laryngomalacia consensus recommendations. Int J Pediatr Otorhinolaryngol. 2016;86:256–261. 30. Lister G, Hellenbrand W.E, Kleinman C.S, Talner N.S. Physiologic effects of increasing hemoglobin concentration in left-to-right shunting in infants with ventricular septal defects. N Engl J Med. 1982;306(9):502–506. 31. Smits-Wintjens V.E, Walther F.J, Rath M.E, et al. Intravenous immunoglobulin in neonates with rhesus hemolytic disease: a randomized controlled trial. Pediatrics. 2011;127(4):680–686. 32. Management of hyperbilirubinemia in the newborn infant 35 or more weeks of gestation. Pediatrics. 2004;114(1):297–316.

3.5

Neonatal resuscitation Gary David Williams

ESSENTIALS 1 Occasionally deliveries occur in the emergency department (ED) when labour cannot be delayed to enable transfer of the patient to a labour ward setting. 2 The ED should have available appropriate equipment and guidelines for the resuscitation of the newborn. 3 The unique physiological events at birth affect the resuscitative interventions in the newborn. 4 Ventilation is the main priority in the resuscitation of the newborn. 5 The heart rate is a reliable indicator of the newborn baby’s degree of compromise and response to resuscitation. The easiest way to monitor newborn pulses is by palpation of the umbilicus. 6 Chest compressions are indicated if: (1) absent pulse; or (2) heart rate 1.5 seconds) may be required for the first several (≈5) breaths. Initial peak inflating pressures required are variable and unpredictable. In general, the minimum pressure required to achieve an increase in the heart rate should be used. Visible chest wall movement and an increase in the heart rate are the best indicators of adequate ventilation. Ventilations should be administered at a rate of 40 to 60 breaths min, and after 30 seconds of effective ventilation, the heart rate should be evaluated.

Heart rate Assessment of the heart rate can be done by palpating the umbilical stump, brachial or femoral pulse or auscultation of the apical heart sounds. Clinical assessment of heart rate by these methods can be intermittent and inaccurate, and prompt commencement of oximetry or application of three chest ECG leads can provide a continuous and more accurate number: • The heart rate is a reliable indicator of the degree of compromise and

response to resuscitation. It should be assessed at least every 30 seconds in the first 2 minutes if necessary, until the baby’s required level of support is established. • If the heart rate is less than 60 beats min despite adequate assisted ventilation, compressions are required. When the heart rate is greater than 100 beats min, only ventilation is continued. If the heart rate is between these two points, the level of intervention should be increased or decreased depending on the serial change in the heart rate. • If compressions are initiated it is recommended that inspired oxygen concentration be increased to 100%. • If the heart rate is not rising after 30 seconds of effective ventilation with 100% oxygen (preferably through an ET tube) combined with chest compressions, then adrenaline (epinephrine) should be administered. • Once a slow heart rate has increased above 60 beats min and is rising, cardiac compressions may be discontinued.

Compression technique If required, compressions should use the two-thumbs-encircling-hands technique. The lower third of the sternum (just below an imaginary intermammary line) should be depressed one-third of the depth of the chest. These should be coordinated with ventilations (to avoid simultaneous delivery) in a ratio of 3:1 with about 90 compressions and 30 breaths each minute. The xiphoid portion of the sternum should not be compressed because such compression may damage the neonate’s liver.

Colour Once the heart rate has been evaluated, the infant’s colour should be assessed by examining the trunk and mucosae. It is noteworthy that clinical assessment of colour in isolation is an unreliable indicator of oxygenation or the effectiveness of resuscitation:3 • Peripheral cyanosis (acrocyanosis) is common in the first few minutes after birth and is not pathological. • Central cyanosis reflects inadequate oxygenation and may be pulmonary or cardiac in origin. If present despite adequate ventilation and a heart

rate greater than 100 beats per minute, pulse oximetry should be commenced. • Oximetry should guide oxygen administration to limit hyperoxia, especially in the premature. The aim should be to target SpO2 levels achieved in healthy term infants during the first minutes of life remembering that an SpO2 of 70% is at the lower end of the normal range at 5 minutes of age in a healthy term baby (Table 3.5.4). • Pallor is suggestive of a decreased cardiac output and may be due to myocardial dysfunction, severe anaemia, hypovolaemia, hypothermia or acidosis. Table 3.5.4 Preductal SpO2 targets after birth Age

SpO2 target

1 min

60–65%

2 min

65–70%

3 min

70–75%

4 min

75–80%

5 min

80–85%

10 min

85–95%

Muscle tone and reflex irritability These physical signs are valuable composite reflections of the adequacy of cerebral perfusion and oxygenation. As such, they constitute two of the five components of the Apgar score (see Table 3.5.3) used to assess a newborn’s condition after birth.

Medications Medications are rarely required during neonatal resuscitation. One study suggested medications were required in only 0.12% of all births, for severe fetal acidosis or ventilatory problems. This reaffirms the primary and critical importance of achieving optimal ventilation before resorting to medications in neonatal resuscitation.

Vascular access Adrenaline may be administered by intravenous (IV), intraosseous (IO) or ET routes. If there is respiratory depression once heart rate and colour have been restored by adequate rescue ventilation, naloxone may be given via the IV or intramuscular (IM) route. The other drugs and volume expansion, detailed below, require emergency vascular access. Once vascular access is achieved, if the child remains in arrest, an adrenaline dose should be immediately administered via the IV route. The preferred site of the vascular access during neonatal resuscitation is the umbilical vein, the larger thin-walled single vessel (in comparison to the paired thicker-walled arteries), which appears when the umbilical cord is trimmed 1 cm above the skin. A 3.5 or 5 French catheter flushed with saline (to remove any air in the tubing) should be inserted only until a good blood return is achieved (usually to a depth of 1 to 4 cm below the skin). Peripheral veins on extremities or scalp may be attempted, or alternatively an IO cannula may be placed on the medial aspect of the tibia just below the tibial tuberosity, if umbilical or other direct venous access is not readily obtainable.

Adrenaline Adrenaline is administered with the aim of producing α-adrenergic-mediated vasoconstriction, an increase in coronary perfusion pressure and myocardial blood flow. Adrenaline is indicated if the heart rate remains less than 60 beats per minute after a minimum of 30 seconds of adequate ventilation and 30 seconds of combined ventilation and chest compressions. The recommended IV dose is 0.1–0.3 mL kg of a 1:10,000 solution (10–30 mcg kg) repeated every 3 to 5 minutes as indicated. ET delivery, though of unproven efficacy, can be considered while IV access is being established and requires a higher dose (up to 100 mcg kg); it should be followed by 1 mL of normal saline and several good inflations to achieve optimal delivery to the pulmonary vascular bed. Most infant animal experimental dosing data supporting adrenaline’s efficacy have been obtained in VF models and as such their value may not be directly applicable to the apparent preterminal bradyarrhythmia in an asphyxiated newborn with markedly elevated pCO2. Through the early 1990s some experimental and human data showed that higher intravenous doses of adrenaline (100 mcg kg–1) were capable of achieving

higher plasma adrenaline levels as well as greater myocardial and cerebral blood flow. However, several subsequent adult and paediatric studies showed no ultimate clinical benefit in survival or neurological outcome, with a significant risk of adverse effects from the higher dose (myocardial dysfunction or necrosis, hyperadrenergic states, reduced cerebral cortical blood flow). Specifically, there is an increase in potential risk of intraventricular haemorrhages (IVH) in preterm infants. For these reasons, the currently recommended initial IV dose remains 10 mcg kg in neonates. There is a paucity of both experimental and human data regarding dosage and efficacy of ET adrenaline in neonates. There are data to suggest a slower onset with a more prolonged and variable effect at higher dosages. For these reasons the dose recommended is 50–100 mcg kg.

Naloxone Naloxone is a narcotic antagonist, recommended for the neonate with respiratory depression secondary to narcotics given to the mother within 4 hours of delivery. Prompt institution of adequate ventilation is the first priority in such a situation, and naloxone is not recommended for newborns whose mothers are suspected narcotic abusers as abrupt withdrawal may be precipitated. Following IV administration, onset of action occurs in 1–2 minutes, although it is variable in duration. The recommended IV dose is 0.1 mg kg. Since the duration of action of narcotics may exceed that of naloxone, continued surveillance and repeat administration are often required. Naloxone may be administered IM, with some adult data suggesting slower onset and more prolonged duration of action via this route. There are no studies examining the ET route of administration in the neonate.

Dextrose Hypoglycaemia is a potential problem for all stressed and asphyxiated babies and should be treated by using a slow bolus of 2 mL kg of 10% dextrose IV, avoiding hyperglycaemia.

Volume expansion If hypovolaemia is present because of known or suspected blood loss or loss of

vascular tone following asphyxia, volume expansion may be appropriate. Isotonic non-dextrose containing crystalloid (normal saline or Ringer’s lactate) 10 mL kg IV over 5 to 10 minutes is recommended. Group O negative packed red cells may be indicated for replacement of large volume blood loss. Albumincontaining solutions are used less frequently because of limited availability, risk of infectious disease and an observed association with increased mortality. A recent randomised controlled comparison of albumin versus normal saline for hypotension in premature newborns showed that those who received albumin required significantly more volume expander to maintain normal blood pressure and had a higher mean percentage weight gain in the first 48 hours after birth.

Bicarbonate Although correcting acidosis during cardiac arrest to improve both myocardial function and adrenaline’s effectiveness makes theoretic sense, there are few supportive experimental data. Most adult studies have found either no difference or that bicarbonate had deleterious effects on myocardial performance. There are no neonatal animal or human studies specifically examining this question. Bicarbonate administration has multiple possible adverse effects (metabolic alkalosis compromising peripheral tissue oxygen delivery, paradoxical intracellular hypercarbic acidosis). Pertinent to neonates are studies demonstrating large increases in plasma osmolality and reductions in cerebral blood flow, both of which may increase the risk of IVH in newborns. Therefore bicarbonate administration is only recommended during prolonged arrests unresponsive to other therapy after establishment of adequate ventilation and perfusion. The dose is 1–2 mEq kg of 4.2% solution by slow IV push over at least 2 minutes.

Specific resuscitation situations Premature neonate Preterm newborns have an increased likelihood of respiratory depression requiring assisted ventilation at birth. This occurs because of diminished lung compliance, weak respiratory muscles and immature respiratory drive and may make it difficult to establish and maintain an adequate FRC. A recent study has shown that CPAP applied promptly to premature babies who breathe at birth may be effective in reducing need for ventilation. Therefore infants born at or before

32 weeks’ gestation should receive prompt CPAP via face mask rather than routine intubation for positive pressure ventilation (PPV). Resuscitation of preterms less than 35 weeks’ gestation should be initiated with low oxygen (21– 30%)4 and the oxygen concentration titrated using oximetry. Also, because preterm infants often have low body fat and a high body surface area to mass ratio, they are more difficult to keep warm and therefore at increased risk of cold stress. For this reason babies 30 minutes

Any airway manoeuvre at any time

Signs

Assisted ventilation

Respiratory rate30 per minute

Chest decompression

Systolic blood pressure 20%. Fluid resuscitation rates should be calculated using the time of the burn, not the time of presenting to the ED (see Fluid resuscitation,

below). Peripheral venous access, preferably through non-burnt skin, is preferred over central venous access for the initial resuscitation. Monitoring of urinary output (via urinary catheter, weighing nappies) is important in determining the adequacy of fluid replacement. A nasogastric tube should also be inserted in children with severe burns, as gastric dilatation can occur, leading to respiratory compromise. Analgesia should be given early, during the stabilisation of the A, B and C. Intranasal fentanyl (1.5–2 mcg kg–1) is a good first up treatment. In severe burns, a morphine infusion should be started after adequate initial intravenous or intranasal analgesia has been given. Large doses of narcotics are sometimes needed in severe burns to control the pain. Intramuscular morphine should never be used given the use of intranasal fentanyl. A careful secondary survey should then be undertaken, looking at the extent, depth and anatomical relevance of the burns. It is important to determine if there are any circumferential burns to the limbs and chest. In superficial or partialthickness burns, careful monitoring of circulation or ventilatory compromise is required. In full-thickness circumferential burns, an urgent escharotomy may be required to restore circulation to the limb or allow for adequate ventilation. A burn specialist should be consulted in this situation. The secondary survey should also include careful examination for any other injuries requiring attention (e.g. head, neck, chest, limbs, pelvis, intraabdominal). Burns to the face should also include fluorescein staining of the eyes to check for corneal involvement.

Fluid resuscitation Fluid resuscitation should be calculated based on the weight of the child and the total surface area of the burn. Several formulas are used to calculate the resuscitation fluid requirement in the first 24 hours. The Parkland formula (BSA affected % × weight (kg) × 4) gives the number of millilitres of resuscitation fluid to be given over the first 24 hours. Half the fluid is given in the first 8 hours and the remainder in the subsequent 16 hours. The 24-hour period should begin from the time of the injury. Thus if a patient has received very little fluid in transfer, and it is 4 hours since initial burn, then the fluid calculated should be given over the next 20 hours and half the calculated fluid given in the first 4 hours. In addition, maintenance fluid for the 24-hour period should also be given.

This is calculated as 100 mL kg–1 for 0–10 kg, 50 mL kg–1 for 11–20 kg, and 25 mL kg–1 for >20 kg. Thus a 30 kg child’s maintenance = 1000 + 500 + 250 = 1750 mL over 24 hours. It is also important to monitor ongoing losses (urinary output, respiratory loss, etc.). The calculated amount of fluid (burn deficient plus maintenance) to be replaced in 24 hours is only a guide and should be adjusted according to the haemodynamic response. Patients need to be maintained in a positive fluid balance for the first 24–48 hours. The adequacy of fluid replacement is monitored by urine output and clinical parameters of perfusion. In children, 0.5– 1 mL kg–1 per hour is the recommended urine output and should be monitored by a urinary catheter in severe burns. A central venous catheter is generally not required in the ED phase of management. The type of fluid used varies between burn specialists, and it is best to be familiar with the preference of the local paediatric burns unit. In the shocked child, a 20 mL kg–1 bolus of normal saline should be given. This can be repeated if necessary to restore peripheral circulation. Ongoing replacement is generally with crystalloid in the first 24 hours, as colloid may leak through the burnt capillaries, causing worsening oedema. After 24 hours, colloid is used as part of the replacement fluids in the intensive care setting. When calculating the initial fluid requirements, it is important to subtract the bolus fluids given from this amount. Additional fluids may be required in severe electrical burns causing muscle damage, as myoglobin may cause renal failure secondary to renal tubular deposition, and therefore maintaining adequate glomerular filtration is very important.

Management of burns (Box 4.6.1) Major burns These patients should be treated in a specialised burns unit. Covering the burn with a sterile dressing is required prior to transfer. Specific dressing type is best decided after discussion with the receiving unit. Within the burn centre, patients are generally dressed with topical silver sulfadiazine (SSD) cream that should be changed each day. At each change, the wound should be cleaned with warm water and debrided to remove any avascular tissue (which may lead to infection). The burns are covered with a non-stick dressing over the SSD cream

(e.g. Melolin) and then wrapped in crepe bandages to prevent contamination of the burn. The face and perineum are generally left open and covered with a water-based gel. SSD should not be used on the face, as the patient may spread it into the eyes. SSD is currently only recommended for inpatient care of patients with significant burns. Box 4.6.1 Admission criteria for paediatric burns

unit • Require admission: • Partial-thickness burns >20% BSA • Full-thickness burns >5–10% BSA • Smoke inhalation or airway burn is suspected • Child abuse suspected • Consider admission: Burn to hands, feet, face, perineum, or joints Burns 2.5 mm (Fig. 5.1.1). • Left atrial enlargement – P wave >0.08 s, may also be plateau or notched (Fig. 5.1.2).

Ventricular enlargement

Right ventricular hypertrophy (Fig. 5.1.3): • R greater than S in V1 after 1 year • T wave upright in V1 after 1 week • SV6 greater than 15 mm at 1 week, 10 mm at 6 months, 5 mm at 1 year • Right axis deviation for the patient’s age • Abnormal R/S ratio in favour of the right ventricle. Left ventricular hypertrophy (Fig. 5.1.4). • SV1 + rV6 greater than 30 mm to 1 year • SV1 + rV6 greater than 40 mm after 1 year • Left axis deviation for the patient’s age • Abnormal R/S ratio in favour of the left ventricle.

FIG. 5.1.1 Right atrial abnormality. Tall narrow P waves may indicate right atrial abnormality or overload (formerly referred to as P pulmonale pattern). From Goldberger: Clinical Electrocardiography: A Simplified Approach, 7th ed. 2006. Copyright © Mosby.

FIG. 5.1.2 Left atrial abnormality. Left atrial abnormality/enlargement may produce the following: (A) wide, sometimes notched P waves in one or more limb leads (formerly referred to as P mitrale pattern); and/or (B) wide biphasic P waves in lead V1. From Goldberger: Clinical Electrocardiography: A Simplified Approach, 7th ed. 2006. Copyright © Mosby.

FIG. 5.1.3 Right ventricular hypertrophy. A tall R wave with an inverted T wave caused by right ventricular overload is seen in lead V1 from a patient with tetralogy of Fallot. Marked right axis deviation is also present. (The R wave in lead III is taller than the R wave in lead II.) From Goldberger: Clinical Electrocardiography: A Simplified Approach, 7th ed. 2006. Copyright © Mosby.

FIG. 5.1.4 Left ventricular hypertrophy. Tall voltages are seen in the chest leads and lead aVL (R = 17 mm). A repolarisation (ST-T) abnormality (arrow), formerly referred to as a ‘strain’ pattern, is also present in these leads. In addition, enlargement of the left atrium is indicated by a biphasic P wave in lead V1 and a broad, notched P wave in lead II. From Goldberger: Clinical Electrocardiography: A Simplified Approach, 7th ed. 2006. Copyright © Mosby.

Table 5.1.1

Table 5.1.2

Timing of pathological cardiac murmurs Systolic

Diastolic

Continuous

VSD

Aortic regurgitation

PDA

ASD

Pulmonary regurgitation

Fistula e.g. coronary

Aortic stenosis

Mitral stenosis

AP window

Pulmonary stenosis

AP collaterals

Valvular regurgitation

VSD, ventricular septal defect; ASD, atrial septal defect; PDA, patent ductus arteriosus; AP, aortopulmonary.

The child with an asymptomatic murmur Asymptomatic murmurs are very common in children. They may be heard at some time in 30% to 50% of normal school-aged children. Innocent murmurs occur when there is normal or increased blood flow through a normal heart and vessels. Innocent murmurs are more obvious during febrile episodes. Recognising common innocent murmurs enables exclusion of organic heart disease and the need for unnecessary investigation and referral. Innocent murmurs with the exception of the venous hum share the characteristics of being systolic, short, soft and usually well localized. The most common innocent murmurs are: 1. vibratory murmur (Still’s murmur) 2. pulmonary flow murmur 3. carotid bruit 4. venous hum (Table 5.1.1). ECG and chest X-ray are poor screening tests for children with asymptomatic murmurs. Clinical assessment by a paediatrician or cardiologist correctly identifies almost all murmurs as innocent or needing further investigation. Echocardiography is not necessary in children identified by a specialist as having an innocent murmur.

Pathological murmurs Pathological murmurs tend to be louder, harsher and longer than innocent

murmurs. They do not vary with respiration. Most diastolic or continuous murmurs are pathological with the exception of the venous hum (Table 5.1.2).

Disposition Referral for specialist consultation is indicated for a child with a murmur who has: • symptoms that may indicate cardiac disease (e.g. breathlessness, cyanosis, chest pain) • abnormalities of the heart sounds (e.g. fixed splitting of the second heart sound) • a murmur that cannot be confidently identified as innocent • a murmur with an associated thrill (grade 4 intensity or greater).

Further reading Park M.K. Pediatric Cardiology for Practitioners. 5th ed. Philadelphia: Mosby; 2008.

5.2

Chest pain John A. Cheek

ESSENTIALS 1 Chest pain is a common reason for children to present to the emergency department. 2 Serious underlying pathology is rare. 3 Most children can be discharged after a history and examination, sometimes supplemented by an ECG or chest X-ray. Other investigations are rarely required.

Introduction Chest pain is a frequent reason for children to present to emergency departments (EDs), although unlike adult patients they rarely have serious underlying organic pathology.1,2 Retrospective studies have demonstrated a cardiac cause for chest pain of between 0% and 5% in children in a variety of settings including EDs.2 Awareness in the community for chest pain being a sinister symptom is well established; parents and children will often seek reassurance. Even in the absence of a sinister cause it can have a significant impact on children’s lives; one-third of children are woken from sleep, one-third miss school, and up to 45% complain of pain for more than 6 months.3 There are, however, a small number of infants, children and adolescents who do present either in extremis or with serious pathology, and as such a systematic approach for all those presenting with chest pain is required. Age is a significant factor in the etiology of chest pain; younger children are more likely to have an organic, cardiorespiratory cause for pain, whereas older children and adolescents

are more likely to have a psychological cause. These should be regarded as a diagnosis of exclusion, and consideration of a much broader differential (Box 5.2.1) should be routine.

Immediate approach Children will rarely present to the ED with undifferentiated cardiovascular collapse or impending arrest. When this occurs, other causes (such as sepsis) are more common; however, a preceding history of chest pain should prompt consideration of differentials such as pneumothorax, arrhythmia, cardiomyopathy or myocarditis. Box 5.2.1 Differential diagnoses of chest pain in

children Cardiac causes • Coronary artery related: • Ischaemia/infarction • Coronary arteritis (Kawasaki disease) • Anomalous origin of coronary arteries • Coronary artery vasospasm • Arrhythmias: • Supraventricular tachycardia • Ventricular tachycardia • Structural abnormalities: • Hypertrophic cardiomyopathy • Dilated cardiomyopathy • Arrythmogenic right ventricular dysplasia • Left ventricular outflow tract obstruction • Pulmonary stenosis • Infection/inflammation: • Pericarditis • Myocarditis

Non-cardiac causes

• Musculoskeletal disorders: • Costochondritis∗ • Trauma, muscle sprain∗ • Scoliosis • Precordial catch∗ • Idiopathic∗ • Respiratory disorders: • Cough∗ • Asthma∗ • Pneumonia/respiratory infection with or without cough∗ • Pneumothorax, pneumomediastinum • Pulmonary embolism • Pleural effusion • Psychological causes: • Anxiety∗ • Other: • Gastro-oesophageal reflux, gastritis∗ • Pancreatitis, biliary disease • Oesophageal foreign body • Shingles • Dissecting aortic aneurysm (e.g. Marfans) • Sickle cell crisis • Lymphoma

∗

Common causes.

General approach (Table 5.2.1) Most children do not need an urgent approach. A thorough history and examination, sometimes supplemented by some simple tests (most often an electrocardiogram or chest X-ray) will enable sinister causes to be excluded. There are some aspects of the history and examination which can be useful to indicate causes more likely in paediatrics.

History Several aspects of history can prompt consideration of a more serious diagnosis (Table 5.2.1). Children with a recent, sudden onset of pain are more likely to have a pathological cause: in an older child, a pneumothorax, pulmonary embolism or arrhythmia; in a younger child also consider ingested oesophageal foreign body – beware button battery ingestion. Precipitation of pain during activity, particularly exercise, is concerning for an arrhythmia or structural lesion that obstructs left ventricular outflow during an attempted increase in cardiac output. Hypertrophic cardiomyopathy is more common in children with a family history of sudden unexpected death. Duration and radiation of pain should be elicited. Association with syncope, near syncope or palpitations are concerning for arrhythmias, structural lesions and myocarditis. Patients with myocarditis or pericarditis often have prodromal viral symptoms, but this is not universal. Unfortunately, concerning historical features do occur far more often than the significant diagnoses they are associated with – in one recent study of cardiology outpatients (none of whom died over the 10 years of the study because of a cardiac condition), 33% of children complained of exertional chest pain and 22% of palpitations.4 In adolescents, a history of recent stressors and social issues can be a pointer to a psychological cause of pain, and younger children can present with cardiacsounding symptoms after close family members have had dramatic cardiac events. Sudden short pains (often left sided) experienced in healthy adolescents are typical of precordial catch syndrome.

Physical examination A structured physical exam focused on the cardiorespiratory systems is essential; several specific pointers to etiology can be found for paediatric conditions. Don’t forget that chest pain can be caused by gastrointestinal disorders and systemic disease, so do not neglect these in your examination. Fever is an important sign; its presence suggests an infective process, such as pneumonia, myocarditis or pericarditis. A cardiac and a respiratory exam should be undertaken. Abnormal cardiac findings – a murmur, rub or muffled heart sounds – may point to specific causes. A murmur that becomes louder during a Valsalva maneuver is a classic sign of hypertrophic cardiomyopathy, although this is not a sensitive finding. Chest palpation can elicit a musculoskeletal cause;

tenderness at the costochondral junction suggests costochondritis. Subcutaneous emphysema at the shoulders and neck can occur in both pneumomediastinum and pneumothorax. Evidence of dyspnea (either resting or with exertion) associated with chest pain is indicative of a more serious cause, particularly pneumonia, myocarditis and cardiomyopathy. Table 5.2.1 Risk factors for serious underlying pathology presenting as chest pain5 Risk factor

Pathology to consider

Prior cardiac disease

Myocardial ischaemia, arrhythmia, pericarditis, pericardial effusion

Major chest trauma

Pneumothorax, haemothorax, cardiac or pulmonary contusion, mediastinal disruption

Sickle cell disease

Acute chest syndrome

Chronic respiratory disease

Pneumothorax

Chronic renal disease

Myocardial ischaemia

Kawasaki disease

Coronary artery aneurysm, myocardial ischaemia, arrhythmia

Family history of sudden death

Prolonged QT syndrome, hypertrophic cardiomyopathy

Hypercoagulable states (clotting disorders, cancer, connective tissue diseases, oral contraceptive use, prolonged immobility, central venous catheters, strong family history of thromboembolic disease)

Pulmonary embolism

Familial hyperlipidaemia syndromes

Myocardial ischaemia

Connective tissue disorders

Pericarditis, pericardial effusion, aortic dissection

Chest pain [Internet]. http://www.rch.org.au/clinicalguide/guideline_index/Chest_pain/.

Investigations Most children require no investigations for chest pain after a history and examination. If a cardiac cause is being considered, an ECG can help reveal arrhythmias, pre-excitation, ischaemia and some secondary characteristics of structural disease (such as hypertrophy with some congenital lesions or small voltages in myocarditis or with cardiac effusions). A chest X-ray can reveal lung

pathology and assist in assessing for gross cardiomegaly. Cardiac markers such as troponin are rarely useful in most children presenting with chest pain. However, these tests should be considered if pathology affecting the myocardium is being contemplated, such as myocarditis or ischaemia (e.g. anomalous left coronary artery arising from the pulmonary artery in neonates/infants, ischaemic heart disease in older teenagers with risk factors). Other investigations, such as an echocardiogram for structural lesions, a holter monitor for arrhythmias, or an exercise ECG for QT prolongation can occasionally be useful. This is usually done after consultation with a paediatric cardiologist dependent on local referral patterns.

Summary Most children in the ED with chest pain have a benign cause and after consideration of more sinister differentials can be discharged home with GP follow-up if required. A small number of children will need the involvement of a paediatric cardiologist.

References 1. Selbst S.M. Approach to the child with chest pain. Pediatr Clin North Am. 2010;57(6):1221–1234. 2. Friedman K.G, Alexander M.E. Chest pain and syncope in children: a practical approach to the diagnosis of cardiac disease. J Pediatr. 2013;163(3):896–901 e1–3. 3. Selbst S.M, Ruddy R.M, Clark B.J, Henretig F.M, Santulli T. Pediatric chest pain: a prospective study. Pediatrics. 1988;82(3):319–323. 4. Thull-Freedman J. Of 3700 children thought to have non-cardiac chest pain at initial paediatric cardiology clinic evaluation, none suffered cardiac death over a median of 4 years follow-up. Evid Based Med. 2012;17(6):190–191. 5. Chest pain [Internet]. http://www.rch.org.au/clinicalguide/guideline_index/Chest_pain/.

5.3

Syncope Kim Yates, and Sarah Jamison

ESSENTIALS 1 The most common cause of syncope in children is vasovagal. 2 A careful and detailed history will usually enable vasovagal syncope to be established with confidence. 3 The main differential diagnoses of syncope in childhood include cardiovascular causes, seizures, migraines, drugs, hypoglycaemia, and psychogenic events. 4 A 12-lead ECG should be done for all children at the initial presentation with syncope. 5 Any child in whom a cardiac cause of syncope is either suspected or diagnosed should be referred to a cardiologist.

Introduction Syncope is defined as an abrupt and transient loss of consciousness and postural tone due to cerebral hypoperfusion, followed by rapid complete recovery. In the first two decades of life 15% to 35% of children experience an episode of syncope, with peak incidence amongst toddlers and adolescents. Syncope accounts for 1% to 3% of emergency department (ED) visits by children. Most causes of paediatric syncope are benign; however, it is important to identify children at risk of the rarer life-threatening causes.

Aetiology

Box 5.3.1 shows causes of syncope in children ranked by incidence. The differential diagnosis of pediatric syncope is wide; however, in childhood and adolescence the major cause of syncope is transient autonomic dysfunction. In toddlers such episodes usually manifest as either blue breath-holding spells or reflex anoxic seizures (pale breathing-holding episode). The mechanism for the cyanosis in blue breath-holding spells is poorly understood but often follows a prolonged period of crying. The precipitant for reflex anoxic seizures may be a noxious stimulus such as a fright or a painful stimulus causing reflex asystole, which leads to an anoxic seizure. In older children and in adolescents such episodes most commonly present as episodes of vasovagal syncope. A combination of hypotension and profound bradycardia or either bradycardia or hypotension alone leads to cerebral hypoxia, but the mechanisms are not completely understood. Other terms used to describe these episodes include neurocardiogenic syncope, vasodepressor syncope or neurally mediated syncope. It should be noted that situational syncope (syncope that occurs during micturition, swallowing cold liquids, defecation or coughing) and carotid sinus sensitivity are rare in the paediatric population.

Typical presentations Vasovagal syncope In vasovagal syncope, the episode typically occurs whilst standing or sitting upright. There may or may not be a stressful antecedent event (this occurs less commonly in frequent recurrent vasovagal syncope). There is a prodrome of nausea, dizziness, visual disturbance and a sensation of warmth, followed by a period of loss of tone and consciousness. Witnesses will describe marked pallor. Seizure activity is unusual, but brief tonic–clonic activity or stiffening is possible, particularly if the patient fails to fall to a recumbent position. Urinary incontinence may also occur. Recovery to a normal level of consciousness is usually prompt once in the supine position. The child may have a headache or be fatigued for minutes to hours after the event.

Breath-holding spells and reflex anoxic seizures Blue breath-holding spells are usually associated with a tantrum or prolonged episode of crying after which the child has a prolonged forced expiration and

apnoea and becomes cyanosed. This may be followed by a brief period of loss of consciousness, with a rapid recovery to full normal activity. They occur in children between the ages of 1 and 5 years, with a peak incidence at the age of 2 years. Reflex anoxic seizure or pale breath-holding episodes occur when an infant is suddenly startled or has a painful injury. The infant may give one or two cries, quiets and becomes pale, then abruptly loses consciousness. Tonic–clonic movements may occur. Episodes usually last less than 1 minute and are immediately followed by normal consciousness and posture. There is an association between iron deficiency and breath-holding spells, so when risk factors such as dietary restrictions or cow’s milk intake of 400–500 mL per day are present, further investigation or empiric treatment can be considered in children with recurrent episodes. Although alarming, these episodes have no long-term sequelae and cease for most children by 6 years of age, although 10% to 20% may have vasovagal syncope in later life. Box 5.3.1 Common causes of childhood syncope Cause (incidence) Vasovagal syncope (64–73%) Breath-holding spell (6.4%) • Reflex anoxic seizures Cardiac (2.9–4.8%) • Primary electrical disturbances: long or short QT syndromes, Wolff–Parkinson–White, ventricular tachycardia, Brugada syndrome, drug induced, sinus node dysfunction, atrioventricular blocks • Structural heart disease: cardiomyopathies, coronary artery anomalies, aortic stenosis or other valve lesions, pulmonary hypertension, myocarditis, congenital heart disease, arrhythmogenic right ventricular dysplasia, cardiac masses, aortic dissection, pulmonary embolus, other outflow obstructions Neurologic (2.1–4.6%) • Seizures • Vascular events: subclavian steal, vertebrobasilar insufficiency • Disrupted cerebrospinal fluid circulation: colloid cyst of 3rd ventricle, posterior fossa tumour

• Vertiginous drop attack • Basilar migraine • Narcolepsy/cataplexy Metabolic (0.8%) • Bleeding, dehydration, hypoglycaemia, electrolyte disturbances, endocrine diseases, carbon monoxide poisoning Psychiatric (2.2–2.3%) • Conversion disorder, somatisation, anxiety, hyperventilation, Munchausen, malingering Unknown or other (8.2–18.9%) • Volume depletion, orthostatic hypotension, pregnancy related, unknown

Cardiac syncope Cardiac syncope should be suspected in a patient with a history of congenital heart disease or with a family history of sudden unexplained death. Cardiac causes should be suspected if episodes occur with no warning, with associated chest pain, during exercise, whilst sitting or supine, or in association with palpitations (though palpitations are frequently described by individuals with vasovagal syncope and with hyperventilation). Long QT syndrome is an ECG diagnosis that is associated with episodes of syncope or seizures caused by episodes of paroxysmal ventricular tachycardia (often torsades de pointes). It may result in sudden death. Syncopal episodes in patients with this diagnosis may be precipitated by exercise or a startle or may be spontaneous. The condition may be congenital or acquired. The ECG in sinus rhythm reveals a prolonged QT interval. The QT prolongation may be minimal, and a high index of suspicion is needed to make the diagnosis.

Hypovolaemic states There will usually be a history suggestive of fluid or blood loss, and obvious signs of shock may be present. Orthostatic hypotension and tachycardia may be the only positive clinical signs. These tend to occur immediately, as distinct from the changes seen in vasovagal syncope, which occur after more prolonged orthostatic stress.

Seizures It may be difficult to differentiate seizures from vasovagal episodes, as they may both be associated with brief convulsions as well as a loss of consciousness, although in seizures motor activity usually occurs before or with the collapse. A history of significant post-event disorientation is helpful in differentiating seizures from other causes of syncope. Seizures are also more likely to be associated with cyanosis, tongue biting, and a more prolonged period of loss of consciousness.

Hyperventilation and conversion syncope Hyperventilation and conversion syncope are rare in children younger than 10 years. Hyperventilation often has a prodrome of apprehension and sighing respiration, then dyspnea, air hunger and chest tightness prior to loss of consciousness. Conversion syncope is a diagnosis to be made once all other possible causes have been excluded but often occurs in the presence of an audience, is not posture dependent, and associated injury is rare. The child may remember surrounding events, and there will be no neurologic, autonomic or cardiovascular changes.

Clinical History A careful and detailed history will usually enable the correct diagnosis of the most common cause of childhood syncope, vasovagal syncope, to be established with confidence. Any unusual features of the history should raise suspicion of an alternate diagnosis: • The prodrome is most important. Nausea, sweating, light-headedness or visual changes (e.g. seeing spots, etc.) strongly suggest a vasovagal cause, although some children with cardiac syncope have also reported preceding light-headedness. An absence of a prodrome suggests a possible cardiac cause. Also enquire about perioral paraesthesia, carpopedal spasms, aura, palpitations, dyspnoea or chest pain. • Circumstances of the event. Recent change of position, poor hydration or eating, a hot environment, phlebotomy, pain or emotional upset suggests

a vasovagal cause. Mid-exertional syncope suggests a cardiac cause. Post-exertional syncope or pre-syncope is typically benign but has been noted in children with cardiac pathology. • Collateral history of event by a witness, if possible. Ask specifically about duration of loss of consciousness, timing of seizure activity, incontinence of urine, pallor or cyanosis, and post-ictal drowsiness or confusion. • Relevant medical history including previous episodes of syncope, cardiac disease, epilepsy, diabetes, medication and drug use, dietary history and past sexual activity. • Family history of sudden collapse or death (including sudden infant death syndrome, single vehicle car crashes or drowning in a competent swimmer) or cardiac disease may suggest cardiac syncope as a cause. Family history of epilepsy, syncope or metabolic disease also gives clues.

Examination The physical examination, although rarely helpful, aims to seek potential diagnostic clues for the cause and identify any secondary trauma related to the syncopal event: • Vital signs are helpful. The peripheral pulse rate, rhythm and character must be noted and the orthostatic blood pressure recorded (this is abnormal if there is a decrease in systolic blood pressure of more than 20 mmHg between measurements taken in the supine and sitting or standing position).

FIG. 5.3.1 Syncope flow chart.

ECG, electrocardiograph; WPW, Wolff–Parkinson–White; PVCs, premature ventricular contractions; AV, atrioventricular; PHx, past history; Hx, history; LOC, loss of consciousness.

• Cardiac and neurological exams aim to identify the rare occurrence of structural cardiac or neurological disease. • Injuries that require treatment or further investigation should be identified. • The history and the initial assessment should direct further examination of the child.

Investigations within the emergency department Figure 5.3.1 shows an approach to working up a child who presents to the ED with syncope. A 12-lead ECG is routinely done for all children at the initial presentation with syncope; however, abnormalities are rare. Paediatric ECGs change with age, so you may need to consult tables to determine whether an ECG is within normal limits for that age group. Long QT syndrome is the most common ion channelopathy so the QT interval should be calculated with Bazett’s formula:

where QTc is the corrected QT interval (normal 85%) may be difficult to detect clinically. Other confounding factors may be acrocyanosis (a normal finding, which may last 72 hours), polycythaemia (giving the appearance of cyanosis) and dark skin (cyanosis is more difficult to detect). Cyanosis is better appreciated in natural light than in artificial light. Arterial oxygen saturation should always be assessed with pulse oximetry when considering cyanosis in the newborn. It is an accurate, reliable, and noninvasive method for monitoring oxygen saturation in infants. In severe cyanosis with respiratory distress, both preductal and post ductal oxygen saturations should be monitored to detect the gradient across the ductus arteriosus by placing the pulse oximeter probes over the right hand and a lower extremity (never the left hand).2 The hyperoxia test was historically used to differentiate between cardiac and pulmonary aetiologies. It is contra-indicated in preterm infants and does not differentiate persistent pulmonary hypertension from cyanotic congenital heart

disease (CHD). With the wide availability of echocardiography, the hyperoxia test is rarely required and should be considered only after discussion with a paediatric cardiologist.2 Box 5.4.1 Causes of cyanosis2 – 6

Differential diagnosis Arterial oxygen desaturation (central cyanosis [pO2 50 mmHg but low O2 saturation)

Clinical features2,3,5,6 A comprehensive cardiac and respiratory examination is essential in any

cyanosed child. The key features are: • pulses – rate, rhythm, volume • blood pressure – all four limbs if the pulse volume is abnormal • precordial impulse – heaves, thrills • auscultation – abnormalities of P2, murmurs • pre- and postductal oximetry (right arm versus left arm/legs) if there is differential cyanosis.

Investigations Investigations are directed at likely causes, after history and clinical examination. They may include arterial blood gas, serum electrolytes, glucose levels, full blood count, haematocrit and cultures. Table 5.4.1 Congenital cyanotic heart disease in the newborn (the 5-Ts mnemonic) Lesion

Features

T-Transposition of the great arteries (TGA)

Switch of the two outflow tracts (two parallel circulations) Most common is D-TGA (aorta anterior and to the right) No. 1 early presenting cyanotic lesion Intracardiac shunt → blood mixes at the PFO ± VSD Life-threatening if no intracardiac shunt → urgent balloon atrial septostomy.

T-Tetrology of Fallot (TOF)

Four components: VSD Overriding aorta Pulmonary stenosis Right ventricular hypertrophy Main cause of cyanosis is pulmonary stenosis or RVOTO (right ventricular outflow tract obstruction).

T-Total anomalous pulmonary venous return (TAPVR)

Pulmonary veins drain into right atrium (instead of left atrium) via superior vena cava, inferior vena cava, or hepatic veins → cyanosis + pulmonary congestion TAPVR can be partial or total, depending on whether all four pulmonary veins drain into right side or not Generally associated with an ASD/PFO for intracardiac shunting.

T-Truncus arteriosus

T-Tricuspid atresia

A single semilunar valve with single outflow tract originating from both ventricles, instead of two vessels (aorta + pulmonary artery) + VSD → cyanosis + increased pulmonary blood flow. All RA blood is shunted R → L to left atrium via ASD/PFO Most have a VSD Stenosis of the tricuspid, and Ebstein’s anomaly = inferiorly displaced tricuspid valve causing atrialisation of part of the right ventricle, a small functional right ventricle and RVOTO.

Others

Pulmonary atresia ± VSD (No VSD → duct-dependent) L → R shunt with pulmonary oedema (consider large VSD or PDA) = less severe cyanosis than in conditions where R → L shunt but more respiratory distress Single ventricle physiology (hypoplastic left or right heart) Low cardiac output states.

PFO, patent foramen ovale; VSD, ventricular septal defect; ASD, Atrial septal defect; R, Right; L, Left.

A chest X-ray (CXR) can be very useful and should be examined carefully for heart size (normal cardiothoracic ratio in AP film is 90%) cause of childhood diabetes.

Diagnosis The classic symptoms of polyuria, polydipsia and weight loss may be present for a few weeks before parental concern is raised. The diagnosis should be confirmed by an elevated random laboratory blood glucose level (>11 mmol L), in addition to urine analysis for glucose and ketones. Once the diagnosis is confirmed, initial management is dictated by the severity of dehydration, presence of shock, degree of acidosis, hyperglycaemia, ketosis, and osmolality. In a child with no past history of diabetes, the initial diagnosis may be misled by non-specific symptoms, such as abdominal pain, weight loss, drowsiness, fever, secondary enuresis and dyspnoea. Beware of tachypnoea due to metabolic acidosis, intercurrent infection in a new diabetic, abdominal pain related to diabetic ketoacidosis (DKA), and drowsiness. In such children, diabetes should be excluded as a possible cause with a random blood glucose.

Diabetic ketoacidosis Diabetic ketoacidosis is the major cause of mortality in diabetic children. It often presents in newly diagnosed type 1 diabetic children. In established diabetics, it occasionally presents in the midst of intercurrent febrile illness, poor adherence

to management, or problems with their insulin pump. Diabetic ketoacidosis is caused by insulin deficiency, leading to hyperglycaemia, osmotic diuresis, hyperosmolar dehydration, lipolysis, ketosis and acidosis. It may be defined by the combination of: hyperglycaemia (BSL >11 mmol L) ketosis and ketonuria acidosis (pH 6 mmol L–1, or 7 mmol L–1 or there are ECG changes, consider giving calcium 0.1–0.15 mmol kg–1, equating to 0.5 mL kg–1 of 10% solution of calcium gluconate or 0.2 mL kg–1 of 10% calcium chloride, which does not change plasma potassium but protects cell membranes. Beware of subcutaneous infiltration of the infusion. Nebulised salbutamol 2.5–5 mg promotes potassium entry into cells as does a glucose/insulin infusion (0.1 units kg–1 Actrapid insulin plus 5 mL kg–1 of 10% dextrose, over 30–60 minutes). Sodium bicarbonate may also be given, especially if the child is acidotic, at a dose of 1–3 mmol kg–1 equating to 1–3 mL kg–1 of 8.4% sodium bicarbonate, over 30–60 minutes. Resonium, 1 g kg–1 every 4–6 hours orally or rectally, may eliminate 1–2 mmol kg–1 of potassium but is more slowly acting. Consider arranging early dialysis. Continuous electrocardiographic monitoring is required as well as discussion with nephrology and intensive care services.33 See Table 10.6.6 for an example of doses required for management of hyperkalaemia in a 10 kg child.

Hypokalaemia Hypokalaemia usually represents with potassium depletion rather than potassium shifts. Causes of potassium depletion include vomiting, diuretics, secretory diarrhoea, ureterosigmoidostomy, renal tubular acidosis, hyperaldosteronism, anorexia nervosa and diabetic ketoacidosis. Causes of hypokalaemia without depletion include salbutamol use, alkalosis and familial periodic paralysis. Mild hypokalaemia is often asymptomatic. This may not need to be actively treated as it will self-correct with illness resolution. Hypokalaemia associated with alkalosis usually corrects itself as the pH corrects. However, more severe hypokalaemia can cause tachyarrhythmias, ileus, and weakness and rhabdomyolysis may occur. If potassium depletion requires treatment, it should be by slow potassium

supplementation. Depending on the situation and severity, oral potassium can be given at maximum 1 mmol kg–1 (5 years). IV potassium can be given at a maximum rate of 0.4 mmol kg–1 hour for 4–6 hours. ECG monitoring and frequent repeat measurement are required. Concentration >40 mmol L–1 of infusion fluid should be given into a central venous catheter.33

Maintenance fluids The concept of maintenance fluids refers to healthy children, where the kidneys are able to conserve or excrete water and salt over a wide range, in each case according to intake and non-renal losses. ‘Maintenance’ is a volume of water intake which maintains urine output in the middle of the normal range with an osmolality about that of extracellular fluid. However, changes in total body water and sodium and other electrolytes are common in many diseases. Insensible skin loss of water may be high because of fever and the higher surface area:weight ratio in infants. Maintenance is only relevant after restoration of circulating volume and total body water and is therefore relevant to ongoing rather than ED care. A maintenance amount should be a starting amount. It may be excessive for any sick child where there may be diminished ability to excrete water. Maintenance fluids should initially be with isotonic fluids, such as saline (0.9%) and 5% dextrose, and then tailored to the child’s needs. Hypotonic solutions should not be used for initial maintenance fluids. A common formula for calculating ‘maintenance’ water requirements in a healthy child is as follows: • For the first 10 kg of body weight: 100 mL kg–1 day–1 or 4 mL kg–1 hour–1 • For the second 10 kg of body weight: 50 mL kg–1 day–1 or 2 mL kg–1 hour–1 • For every subsequent kg of body weight: 25 mL kg–1 day–1 or 1 mL kg–1 hour–1. Remember to not include the dehydration deficit in maintenance; consider this separately and use 0.9% saline or a buffered isotonic solution for replacement. Fluid input from coexisting drug administration and oral intake should also be taken into consideration. Constant monitoring and re-assessment are the key to safe maintenance fluid prescribing. Unwell children are more likely to release ADH as a response to illness; this

in turn reduces the ability of the kidneys to excrete water. Therefore maintenance fluids should be reduced by a one-third initially or even one-half if there is risk of cerebral oedema, e.g. in meningitis or brain injury, especially if hyponatraemia already exists.

Acid–base disorders Disorders of physiological control of acidity of body fluids are common in acutely ill children. The system of defining acid–base state by changes in pCO2 (respiratory) and standardised base excess (metabolic) according to the Copenhagen school remains the most familiar way of analysing acid–base disorders. Base excess is mostly bicarbonate deficit but includes a small amount of buffering by albumin and a larger amount by haemoglobin. Standardised base excess is provided by blood gas machines derived by microprocessor rather than the original nomograms. The philosophy of base excess has been challenged because it does not take into account the actual plasma albumin concentration and assumes a notional haemoglobin concentration of 50 g L–1 across blood and extracellular fluid in all patients. It can be argued that the bicarbonate concentration alone is a sufficient measure of the degree of metabolic acidosis or alkalosis. Table 10.6.7 Causes of acidosis High anion gap M – Metformin U – Uraemia/renal failure D – Diabetic ketoacidosis P – Phenformin, pyroglutamic acidosis I – Infection, inborn errors of metabolism, isoniazid L – Lactic acidosis including sepsis, hepatic failure, hypoxia, shock, leukemia, lymphoma and G6PD deficiency E – Ethanol, ethylene glycol, ethanol ketoacidosis S – Salicylates (especially in babies), solvents starvation/ketosis

Normal anion gap U – Ureteroenterostomy S – Small bowel fistula E – Excessive sodium chloride administration, early acute renal failure D – Diarrhoea C – Carbonic anhydrase inhibitors A – Addison's disease R – Renal tubular acidosis P – Pancreatic fistula

The anion gap remains a useful tool in determining whether any bicarbonate deficit is caused by organic acid (high anion gap) or by chloride excess (normal anion gap). It is obtained from the formula:

The normal is 16 mEq L–1 and is essentially the negative charge on albumin and phosphate. If the potassium is normal this can be deleted from the equation with the normal now being 12 mEq L–1. Any excess is accounted for by abnormal unmeasured acid and/or lactate. Most modern laboratories, including blood gas machines, measure lactate. A more accurate way of determining the unmeasured component is from the formula:

The normal value for unmeasured anion is 5–6 mEq L–1, including 1 mEq L–1 of lactate. Any excess is abnormal. This may be lactate in hypoxia–ischaemia, acetoacetate or β-hydroxybutyrate in diabetic ketoacidosis (DKA), ketones in starvation, organic acids in inborn errors of metabolism, or alcohol poisoning or toluene inhalation. Most metabolic acid–base disturbances do not need treatment. Compensating mechanisms should generally not be treated, otherwise the primary disturbance is exacerbated. It is not necessary to restore pH to normal.

Metabolic acidosis Normal physiology maintains extracellular pH close to 7.4 in health but permits metabolic acidosis at times of anaerobic metabolism without danger. Metabolic acidosis may be advantageous because it is thought to protect cells against the effects of hypoxia and assists oxygen unloading from haemoglobin by shifting the oxygen/haemoglobin dissociation curve to the right. When a metabolic acidosis is present as indicated by a pH 80 and in a 15-year-old >100 μmol L–1. More important is the rate of increase of plasma creatinine in AKI, which takes 12–24 hours to become evident. The plasma creatinine loses its value to assess the kidney function once dialysis is initiated. Unlike creatinine, some of the urea is reabsorbed from renal filtration. Normally 20% of the renal plasma flow is filtered, which may rise up to 50% in case of intravascular hypovolaemia. Thus, in pre-renal AKI, urea to creatinine ratio may rise to >20:1 with Oliguria. In conditions such as gastrointenstinal bleeding, tissue breakdown or low muscle mass in critically ill patients, there may be a falsely high urea/creatinine ratio. In a patient with liver disease or low protein intake, low urea production may give a false normal ratio of urea and creatinine. Therefore in the diagnosis of AKI the urea/creatinine ratio has limited diagnostic utility (see Table 16.1.2).

Clinical presentation AKI in the paediatric population has a wide range of clinical manifestations,

with non-specific presentations to anuric renal failure. One should not rule out AKI simply based on normal creatinine at the time of presentation for the various reasons discussed above. However, a high index of suspicion and a detailed history and physical examination are important to detect AKI or risk of AKI in a paediatric population (Table 16.1.3). Early diagnosis and treatment are important as in-hospital mortality ranges from 1.5–9.5% in non-intensive care settings to as high as 30–50% in patients with severe AKI. The chance of developing chronic kidney disease (CKD) or end-stage renal disease (ESRD) is up to 40–60% in the survivors of paediatric AKI. Suspicion of glomerulonephritis or vasculitis should prompt inpatient referral for further investigation into the cause. Urine electrolytes and osmolality are helpful in differentiating causes of AKI (Table 16.1.4).

Treatment 1. Treat established causes and complications: Life-threatening conditions (e.g. hypoxia, hypovolaemia, hyperkalaemia, and seizures) should be assessed and treated promptly. Ventilation may be needed for pulmonary oedema not responsive to diuretics. Patients should be connected to the monitor for continuous haemodynamic monitoring. 2. Avoid further damage and remove the cause: Avoid excessive fluids and electrolyte supplements (especially Na, K, and PO4). Attempt to establish urine output in oliguria of short duration, but if no response is seen, management with Furosemide should not persist. Mannitol is not recommended as it may worsen the situation by acute intravascular expansion, especially if there is no diuresis. Avoid nephrotoxic drugs. Table 16.1.3

ACE, angiotensin-converting enzyme; AKI, acute kidney injury; BUN, blood urea nitrogen; CRP, C-reactive protein; GN, glomerulonephritis; HSP, Henoch–Schönlein purpura; HUS, haemolytic uraemic syndrome; NSAIDs, non-steroidal anti-inflammatory drugs; SIADH, syndrome of inappropriate antidiuretic hormone secretion; UTI, urinary tract infection.

Table 16.1.4

ADH, antidiuretic hormone; AKI, acute kidney injury; ATN, acute tubular necrosis; GFR, glomerular filtration rate; RT, renal tubular; SIADH, syndrome of inappropriate antidiuretic hormone secretion.

When the GFR falls below 50% of normal, most drugs excreted by the kidney will require modifications in dose or scheduling. However, there is no evidence to support the reduction of the initial dose of antibiotics in the emergency department (ED) if an infection is suspected. 3. Fluid management: • Hypovolaemic: Rapid volume restoration should be performed with 0.9% saline (10–20 mL kg fluid bolus, can be repeated twice) in addition to preventing further fluid loss. Urine output

should be measured to calculate hourly urine output. • Euvolaemic: Care should be given to make up for insensible losses which can be higher in febrile patients (300–500 mL m2 per day). • Hypervolaemic: In critically ill paediatric patients, fluid overload in the setting of AKI was found to be an independent risk factor for mortality (3% increase for each 1% increase in fluid overload). However, the role of diuretics in established renal failure is limited in terms of improving renal or patient survival. Therefore, the main use of diuretics in AKI is to treat fluid overload and not to improve or reverse the kidney function.

A single high-dose bolus (2–5 mg kg) can be tried in children with oliguric AKI with signs of fluid overload (i.e. oliguria of less than 24 hours). If the initial dose is effective in improving urine output, an infusion of Furosemide (0.1–0.3 mg kg h) can be started in consultation with the nephrologist. Diuretic therapy should be used as bridging and not delay the need for renal replacement therapy (RRT) (when overload exceeds 10–15%). 4. Electrolyte management: • Hyperkalaemia is a common and life-threatening electrolyte abnormality seen in AKI. When there is a high index of suspicion, monitoring and immediate treatment of severe hyperkalaemia (K+ >6.5 mEq L or high K+ with ECG changes) are important in preventing cardiac dysrhythmia and deaths. • As fluid overload is common, sodium intake should be restricted to 2–3 mEq kg per day, and caution should be taken in using hyperosmolar fluids. • Potassium or phosphate supplements or replacement should be

avoided unless there is a significant deficit. • Metabolic acidosis is common, and it is secondary to impaired filtration of acids and loss of bicarbonate absorption in the kidney. Use of sodium bicarbonate is reserved to treat severe acidosis. • Hyperphosphataemia can be treated with oral phosphate binders, and calcium gluconate is used to treat hypocalcaemia. 5. Hypertension management: Glomerulonephritis causing AKI in children may end up requiring dialysis in many cases. Since some patients with hypertension may also be fluid overloaded, diuretics can be considered as an adjunct to antihypertensive medications. Specific treatment is further discussed in Chapter 16.3. 6. Nutrition: Children diagnosed with AKI need a high-calorie, high-protein, lowsodium diet. However, these recommendations and their role in AKI are largely expert-opinion based, and further evidence is needed. 7. Renal replacement therapy: Renal replacement therapy (RRT) includes haemodialysis (HD), peritoneal dialysis (PD), and continuous RRT (CRRT). Generally agreed indications to initiate the RRT are as below: • Renal: Uremia/azotaemia – >uraemic encephalopathy or pericarditis or haemorrhage; consider if urea >30–35 mM (no strict cutoff) Fluid overload: oliguria resulting in volume overload and respiratory distress Metabolic acidosis due to renal failure Hyperkalaemia • Non-renal • Toxins/drugs: small, non-protein bound • Sepsis – for removal of cytokines 8. Disposition: All children with AKI should be admitted for inpatient management.

Acute presentation of chronic renal failure

First-time presentation of undiagnosed CRF is a common differential of AKI. Anaemia, poor growth, clinical or radiological evidence of osteodystrophy or small kidneys on ultrasound may suggest CRF (though polycystic kidneys are large). Elevated PCr rising by 80% of cases). It is thus an ascending infection that may affect the bladder (cystitis) or upper renal tract (pyelonephritis). Neonates are unusual as they may also develop UTI following haematogenous dissemination of organisms. Predictors for renal scarring after first UTI include:2 • vesicoureteric reflux (VUR) • abnormal renal ultrasound (either pre-natal or post-natal) • high C-reactive protein >40 mg L • temperature ≥39°C • UTI caused by organism other than E. coli.

History and examination In infants and young children with UTI, the clinical history is frequently nonspecific and may include irritability, jaundice (neonates), poor feeding or fever without apparent source. Symptoms and signs become more specific with increasing age (Table 16.4.2). Previous UTI, increasing pain/crying on passing urine, increasingly smelly urine, and absence of severe cough or ear pain are all suggestive of UTI.3 Table 16.4.1 Prevalence of urinary tract infection in febrile∗ infants and children by demographic group Demographic group 0 to 3 months

Prevalence or pretest probability (95% CI) 7.2% (5.8–8.6)

Girls

7.5% (5.1–10)

Circumcised boys

2.4% (1.4–3.5)

Uncircumcised boys

20.1% (1.7–11.5)

3 to 6 months

6.6% (1.7–11.5)

Girls

5.7% (2.3–9.4)

Boys

3.3% (1.3–5.3)

6 to 12 months

5.4% (3.4–7.4)

Girls

8.3% (3.9–12.7)

Boys

1.7% (0.5–2.9)

12 to 24 months

4.5%∗∗

Girls

2.1% (1.2–3.6)

Boys

4 hours before culture can be performed, the urine sample should be refrigerated. Dipstick urinalysis may be helpful in making a provisional diagnosis of UTI. However, a negative result does not rule out UTI in infancy. One study showed that urinalysis was normal in 50% of infants 5 white blood cells (WBC) per highpower field (centrifuged urine). Another definition is >10 WBC mm–3 (uncentrifuged urine). The definition of significant bacteriuria is guided by the method by which the urine specimen was collected (Table 16.4.3), though on occasion genuine UTI may be present with lower colony counts than would usually be considered significant, especially in babies – interpret results in light of history and clinical findings.

Treatment

ED treatment recommendations for UTI vary.

Age 108

Catheter

>103

Suprapubic

>0

• If not systemically unwell, consider single IM/IV dose of ceftriaxone or gentamicin, followed by oral antibiotics • Arrange GP or ED follow-up at 24–48 hours • Ensure urine culture and sensitivity results are checked at 48–72 hours.

Age >3 years old • Treat on clinical merits, i.e. admit or allow home, parenteral or oral antibiotics • Overnight admission to observation ward for IV/IM antibiotics may be worth considering if unwell • Ensure urine culture and sensitivity results are checked at 48–72 hours.

Antibiotic choice6,7 Empirical antibiotic choice, dose and duration should be guided by local sensitivity patterns and antibiotic guidelines. However, consideration may involve the following:

Parenteral treatment (IV/IM) • Benzylpenicillin 60 mg kg–1 (max 2 g) every 6 hours AND gentamicin 7.5 mg kg–1 (max 360 mg) once daily, or • Ceftriaxone 50 mg kg–1 once daily IV (may not cover enterococci in young infants), or • Gentamicin 7.5 mg kg–1 once daily IV/IM (single drug for initial therapy if penicillin hypersensitivity). Reduce gentamicin to 2.5 mg kg–1 as a single dose in patients with known or suspected renal impairment, and check a level before repeat dosing.

Oral treatment • Amoxicillin + clavulanic acid (22.5 + 3.2 mg kg–1) every 12 hours, or • Cephalexin 12.5 mg kg–1 every 6 hours, or • Trimethoprim + sulfamethoxazole (co-trimoxazole) (4 + 20 mg kg–1) every 12 hours, or • Trimethoprim 4 mg kg–1 (max 150 mg) every 12 hours • Amoxicillin is not recommended.

Duration of treatment? • Infants 20 × 109 L–1) at presentation • Elevated white cell count at presentation and which remains elevated • Age 5 years • Central nervous system involvement • The degree and duration of renal dysfunction.

Complications Complications during the initial episode of HUS include stroke (occurs in 3% to 5% of patients), other central nervous system problems (seizures, coma and cortical blindness), bowel problems (hemorrhagic colitis, bowel necrosis, bowel perforation or intussusception), myocarditis, elevated serum troponin, pancreatitis, glucose intolerance or cholestatic jaundice. Children with STEC-HUS need prolonged follow-up even after apparent full recovery as some can develop late complications. These complications include hypertension, proteinuria, reduced glomerular filtration rate and eventual development of end-stage renal disease (which can develop as late as 15 years to 25 years following recovery). HUS can recur in transplanted kidneys regardless of the aetiological agent.

Prevention STEC-HUS is an infectious disease and the most effective prevention strategy would be to prevent ingestion of the E. coli. Avoidance of undercooked meat can assist in this area. There are, however, other vectors for the transmission of the E. coli, and these include contaminated water and beverages. Food handlers, vendors and consumers must be made aware of proper food-handling techniques. The incubation period for E. coli O157:H7 is usually 3 to 4 days but can range from 1 to 8 days. The infectivity of children colonised with E. coli O157:H7 is up to 3 weeks in children.

Further reading Barbour T, Johnson S, Cohney S, et al. Thrombotic microangiopathy and associated renal disorders. Nephrol Dial Transplant. 2012;27:2673–2685. Corrigan J.J, Boineau F. Hemolytic uremic syndrome. Pediatr Rev. 2001;22:365–368. Elliott E.J, Robins-Browne R.M, O’Loughlin E.V, et al. Nationwide study of haemolytic uraemic syndrome: clinical, microbiological, and epidemiological features. Arch Dis Child. 2001;85:125–131. Garg A.X, Suri R.S, Barrowman N, et al. Long-term renal prognosis of diarrhoea-associated hemolytic uremic syndrome: a systematic review, meta-analysis and meta-regression. JAMA. 2003;290:1360–1370. George J.N, Carla M, Nester C.M. Syndromes of thrombotic microangiopathy. N Engl J Med. 2014;371:654–666. Kaplan B, Meyers K. The pathogenesis of hemolytic uremic syndrome. J Am Soc Nephrol. 1998;9:1126– 1133. Keir L, Coward R.J.M. Advances in our understanding of the pathogenesis of glomerular thrombotic microangiopathy. Pediatr Nephrol. 2011;26:523–533.

Marina Noris M, Remuzzi G. Hemolytic uremic syndrome. J Am Soc Nephrol. 2005;16:1035–1050. Michael M, Elliott E.J, Jonathan C, et al. Interventions for hemolytic uremic syndrome and thrombotic thrombocytopenic purpura: a systematic review of randomized controlled trials. Am J Kidney Dis. 2009;53:259–272. Ray P, Liu X. Pathogenesis of Shiga toxin-induced hemolytic uremic syndrome. Pediatr Nephrol. 2001;16:823–839. Razzaq S. Hemolytic uremic syndrome: an emerging health risk. Am Fam Physician. 2006;74:991–996. Siegler R.L. The hemolytic uremic syndrome. Pediatr Clin N Am. 1995;42:1505–1522. Stewart C.L, Tina L.U. Hemolytic uremic syndrome. Pediatr Rev. 1993;14:218–224. Tarr P.I, Gordon C.A, Chandler W.L. Shiga-toxin-producing Escherichia coli and haemolytic uraemic syndrome. Lancet. 2005;365:1073–1086.

16.6

Idiopathic nephrotic syndrome Linas Dziukas

Introduction Nephrotic syndrome (NS) is a particular combination of clinical findings, urine abnormalities and changes in the blood. The underlying cause is a marked increase in the permeability of the glomerular filtration barrier (GFB) to protein, resulting in the following changes: • Excretion of large quantities of protein in the urine (marked proteinuria), causing a decrease in the serum albumin concentration (hypoalbuminaemia) • Retention of sodium by the kidney, causing an increase in the size of the interstitial compartment that causes oedema • Changes in lipid metabolism with: • Hypercholesterolaemia • Fat droplets in renal tubular cells • Fat in the urine (lipiduria): free fat or fat within renal tubular cells. There are other changes in the concentration of blood components that affect in particular the immune system and the coagulation system. Measurement of the blood concentration of lipids is not as available as other blood tests, so a working diagnosis of NS is the combination of oedema, marked proteinuria and hypoalbuminaemia. Patients without oedema but with marked proteinuria are said to have a nephrotic level of proteinuria.

Pathophysiology of proteinuria

Nephron and glomerular filtration barrier Each human kidney is an aggregate of about one million nephrons. Each nephron has a globular enlargement that is invaginated by capillaries, forming the renal corpuscle (glomerulus) (Fig. 16.6.1). The glomerulus has four structural components: • Mesangium • Glomerular capillaries • Glomerular basement membrane (GBM) • Visceral epithelial cells (VEC) or podocytes (PO). The plasma in the glomerular capillaries crosses three structures (collectively called the glomerular filtration barrier [GFB]) to become the glomerular filtrate. The GFB consists of: • glomerular capillary endothelium • glomerular basement membrane • podocyte slit: the foot processes of the podocytes and the slit diaphragm (a specialised type of intercellular junction) connecting adjacent podocyte foot processes. The GBM contains embedded anionic molecules that retard the filtration of negatively charged molecules in the blood (Fig. 16.6.2). The GFB thus acts as a ‘sieve’ that allows small molecules and water to be filtered across the GBM but retards or prevents the passage of larger molecules based on their size and shape and charge.

Renal handling of albumin Albumin is the most abundant plasma protein, accounting for 55–60% of the measured serum protein. It consists of a single polypeptide chain of 585 amino acids and has a molecular weight of 66500 Da. In solution the molecule is ellipsoid and is negatively charged (Fig. 16.6.3). Albumin has a major role in the maintenance of the normal colloid oncotic pressure, contributing to 80% of the normal oncotic pressure. It is a flexible molecule that binds and transports many substances: long-chain fatty acids, bilirubin, calcium and magnesium. Albumin is also a secondary or tertiary

carrier for substances that have specific binding proteins, e.g. vitamin D and thyroxine. The binding of drugs to albumin affects the delivery of the drug to tissue sites and the metabolism and elimination of the drug. The normal serum albumin concentration is 32–45 g L, i.e. 32–45 mg mL. The amount of albumin entering the (two) kidneys (the ‘renal albumin load’) is determined by the serum albumin concentration and the renal plasma flow. The ‘renal albumin load’ in a healthy child is about 1110 mg min m2. The normal GFB is not completely impermeable to albumin, and the total amount of albumin filtered by the glomeruli of a healthy child is about 0.7 mg min m2, i.e. about 1000 mg day m2. The total protein excreted in the urine of a healthy child is less than 100 mg day m2. The kidneys of a healthy child thus reabsorb nearly all the filtered albumin; the main sites of reabsorption are the proximal convoluted tubule (71%), the loop of Henle and the distal tubule (23%) and the collecting duct (3%).

Mechanisms of proteinuria There are three main processes that can cause proteinuria: 1. Overflow proteinuria: • Excretion of acute phase reactants or cytokines: • Sepsis • Trauma • Myoglobinuria • Haemoglobinuria • Immunoglobulin fragments. 2. Glomerular proteinuria: • Increased permeability of the GFB to proteins and large molecules. 3. Renal tubular disease (tubular proteinuria). Urinary electrophoresis distinguishes between overflow proteinuria, glomerular proteinuria and tubular proteinuria. Urinary electrophoresis that shows a predominance of albumin in comparison to molecules of intermediate molecular weight in the NS (i.e. in glomerular proteinuria) is called a ‘selective

proteinuria’. An increased proportion of molecules of intermediate molecular weight relative to albumin in the urine is called a ‘non-selective’ proteinuria. Selective proteinuria is more common in minimal change (glomerular) disease, and non-selective proteinuria is more common in other types of glomerular damage that produce the NS.

FIG. 16.6.1 A, Diagram of the structure of the glomerulus. B, Diagram of the structures forming part of the glomerulus and the glomerular filtration barrier. Modified from Feehally J, Floege J, Johnson RJ. Comprehensive Nephrology, 3rd ed. London: Mosby Elsevier; 2007.

FIG. 16.6.2 Light microscopy of a normal glomerulus. A, Section passing through the vascular pole (VP), with the dominant feature being the open glomerular capillaries of the glomerular tuft (Silver stain). B, High-power view of part of a normal glomerulus, showing podocytes (Po) and glomerular capillaries (C) arranged round the mesangium (Silver stain). C, Special stain of a normal glomerulus that shows the negatively charged ions in the glomerular filtration barrier as a thin blue line (Colloidal iron stain).

Definition of nephrotic syndrome Peripheral oedema AND marked proteinuria∗ AND hypoalbuminaemia∗∗ AND hyperlipidaemia Accurate timed urine collections are difficult to obtain in children, and the protein/creatinine ratio (Pr/Cr) on an untimed urine specimen is an accepted alternative: • Children aged 6 months to 2 years: normal Pr/Cr is 30 mg mmol of urine albumin to creatinine ratio on a spot morning sample; and/or haematuria, red blood cell casts: ≥5 red cells per high power field or ≥2+ on dipstick or red blood cell casts in the urinary sediment).

Clinical features In two-thirds of the cases, the disease follows an upper respiratory tract infection, with onset an average of 10 days after the start of respiratory symptoms. Despite this association, no single microorganism or environmental exposure has been shown to cause HSP. Other symptoms that can precede the disease include fever or headache. The development of HSP has also been linked to cold exposure, insect bites, drugs and vaccination. The classic presentation is with a tetrad of symptoms or signs: • Rash • Arthritis • Abdominal pain • Kidney involvement.

The onset of purpura, abdominal pain and arthritis may be in any sequence. All patients with HSP will eventually develop the characteristic rash, but sometimes the rash is not part of the initial presentation. Arthritis occurs in 80% of cases, and gastrointestinal involvement occurs in 50–75% of patients and is potentially the most serious initial complication of HSP due to the risk of bleeding and intussusception. The clinical hallmark of HSP nephritis is haematuria or proteinuria or both. HSPN occurs in 50% of older children but in only 25% of children younger than 2 years.

Rash The rash is most commonly symmetrical and is usually located in dependent areas such as the extensor surfaces of the lower extremities or on the buttocks (Fig. 16.7.3). The rash can be accentuated in areas of pressure (such as sock lines and the waistline). Lesions may also be seen on the forearms with involvement of trunk and face described occasionally in younger children. Classic lesions consist of urticarial wheals, erythematous maculopapules and larger, palpable ecchymosis-like lesions. Petechiae and target lesions may be present as well. These lesions are typically non-blanching, as they are due to extravasation of blood into the skin. Blisters or ulcers or both may develop in the affected areas. Subcutaneous oedema that is not associated with a rash may develop in the scalp, face, dorsum of the hands and feet or scrotum. The rash can persist for 3–10 days and progresses in colour from red to purple to brown before fading. Recurrent crops of lesions occur over the next 6 to 16 weeks in up to one-third of cases and may be associated with more severe renal involvement.

Arthritis This usually involves one to four joints, especially the ankles and knees. There is usually prominent periarticular swelling, tenderness and pain; erythema and joint effusion are uncommon. The joint symptoms are transient, may be migratory, and leave no permanent deformity. Joint symptoms may precede the rash in 25% of cases.

Gastrointestinal The gastrointestinal manifestations are due to oedema and haemorrhage, predominantly affecting the proximal small bowel. Gastrointestinal haemorrhage is mostly confined to the mucosa and submucosa, and full-thickness necrosis and perforation of a bowel loop are rare. Abdominal pain is the most common symptom and may mimic that of an acute abdomen. The pain is typically colicky and usually occurs about 1 week after the onset of the rash. The pain may precede the rash in up 15% of cases.

FIG. 16.7.4 Gastrointestinal involvement in Henoch–Schönlein purpura. (A) Endoscopic finding of submucosal haemorrhage and superficial ulceration. (B) Ultrasound showing intussusception. (C) Contrast-enhanced CT scan shows bowel wall thickening of the ileum (∗) with the target sign. (D) Axial contrast-enhanced CT scan showing a focal wall defect area, suggesting perforation. Modified from A: Henoch-Schönlein Purpura – A Case Report and Review of the Literature. Gastroenterology Research and Practice Volume 2010, Article ID 597648; B: Williams H. Imaging and intussusception. Arch Dis Child Educ Pract Ed 2008;93:30–6; C: Ha HK, Lee SH, Rha SE, et al. Radiological features of vasculitis involving the gastrointestinal tract. RadioGraphics 2000;20:779–4; D: Chung DJ, Park YS, Huh KC, Kim

JH. Radiologic findings of gastrointestinal complications in an adult patient with HenochSchönlein purpura. AJR 2006;187:W396–8.

Gastrointestinal bleeding (occult and gross) will develop in 30% of patients. The most severe gastrointestinal complication is intussusception, affecting 3–4% of patients with HSP. The intussusception is limited to the small bowel in twothirds of cases. Perforation of the bowel wall may occur. Pancreatitis and acalculous cholecystitis are rare complications.

Renal In contrast to arthritis and abdominal pain, it is very uncommon for evidence of HSPN to precede the appearance of the rash. The onset of renal involvement may be delayed after the onset of other symptoms. In most cases (80% of patients with HSPN) renal involvement will develop within 4 weeks of the onset of HSP. A small number of cases of HSPN have a later onset, but nearly everyone (97%) with HSPN will develop nephritis within 3 months of the onset of other symptoms. The development of HSPN is often asymptomatic, and the diagnosis depends on urine analysis or blood tests or evidence of hypertension.

Other findings Scrotal involvement can present as oedema or pain. Scrotal ultrasound may show scrotal wall thickening, hydrocele and inflammation of the epididymis and spermatic cord, with or without associated orchitis. Hypertension may be present in 5% of cases.

Diagnosis and investigation Initial investigations There is no diagnostic laboratory test for HSP, although a low platelet count will (by definition) rule out the diagnosis. Blood tests may reveal a normal or elevated white blood cell count and possible eosinophilia. The platelets count may be normal or increased, and the prothrombin time and partial thromboplastin time are normal. Factor XIII activity can be reduced and is associated with more severe disease but is not part of routine assessment. Inflammatory markers (CRP/ESR) are usually elevated, and the initial serum creatinine is normal in nearly all (97%) cases. Autoimmune tests are either

normal (negative antineutrophil cytoplasmic antibody) or the abnormalities are non-specific (serum IgA levels are elevated in half the cases, but there is no correlation with disease severity). Urine analysis may show isolated haematuria (40% of cases) or proteinuria (25%) or both. Macroscopic haematuria is uncommon (7% of cases), as is the presence of the nephrotic syndrome (3% of cases).

Radiology Ultrasound is the initial imaging modality of choice for evaluation of abdominal pain or boys with scrotal oedema or testicular pain. CT scans will be needed in patients with signs of peritonitis or haemodynamic instability. On CT imaging, bowel involvement in HSP is seen as multifocal symmetric, circumferential wall thickening with a target pattern. The target pattern is seen after administration of intravenous contrast and consists of enhancing mucosal and serosal layers with an intervening hypo-dense submucosal layer due to oedema. Other abdominal findings on CT scan of HSP include free intra-peritoneal fluid, ileus of affected loop, vascular engorgement in the adjoining mesentery and non-specific lymphadenopathy.

Biopsy Skin biopsy may be required in cases where the diagnosis of HSP is uncertain. The indications for renal biopsy include: • acute renal impairment/nephritic syndrome at presentation • nephrotic syndrome with normal renal function persisting at 4 weeks • nephrotic range proteinuria (urine protein/creatinine ratio >250 mg mmol) at 4–6 weeks (if not improving spontaneously) • persistent proteinuria – urine protein/creatinine ratio >100 mg mmol for more than 3 months.

Differential diagnosis The diagnosis of HSP is usually obvious due to the characteristic rash. However, in the event of an atypical presentation other conditions should be considered. Patients who are suffering from sepsis, malignancy or hypersensitivity to drugs can present with leucocytosis, thrombocytosis, raised erythrocyte sedimentation

rate (ESR) or C-reactive protein (CRP) associated with a rash (urticaria, petechiae, palpable purpura), unexplained arthritis and renal impairment. Acute haemorrhagic oedema of infancy (AHOI) is a rare type of cutaneous small-vessel vasculitis with a characteristic presentation in infants. It is not clear if it is a mild variant of HSP or a separate condition. AHOI is seen in children between the ages of 4 months and 2 years of age and usually develops after an upper respiratory infection. The child remains in good health but develops a rash on the extremities and face, especially the ears, eyelids and cheeks. The trunk and mucous membranes are usually spared. The rash consists of urticarial wheals, ecchymoses or purpura. The lesions often have a target-like appearance. Oedema may occur around purpuric lesions or involve the hands and feet and extend up the limbs. Joint involvement and renal involvement are uncommon. The diagnosis in patients with HSP where arthritis is the presenting symptom includes juvenile rheumatoid arthritis, rheumatic fever or a reactive arthritis. The diagnosis in patients with HSP where abdominal pain precedes the rash includes surgical causes of an acute abdomen. Kawasaki disease is an acute febrile illness with inflammation of small- and medium-sized blood vessels throughout the body, in particular the coronary arteries. The highest incidence is in children of Asian descent, especially Japanese. Eighty per cent of cases occur in children younger than 5 years of age with a peak incidence between 1 to 2 years. A child with Kawasaki disease has a high swinging fever (greater than 39°C), associated with marked irritability. Other symptoms and signs can include abdominal pain, diarrhoea, joint pain or arthritis. The diagnostic criteria are fever for at least 5 days, the presence of four out of five cardinal signs (peripheral extremity changes – reddening of the palms and soles, indurative oedema and subsequent desquamation; oral signs – redness within the mouth or on the pharynx, strawberry tongue and red or cracked lips; eye signs – redness of the bulbar conjunctivae; lymphadenopathy – often on one side of the neck; peripheral limb signs – firm swelling of the hands and feet, sometimes including the fingers and toes, with redness of the palms and soles; skin rash – morbilliform, maculopapular, erythematous or target-like) and the absence of any other illness to account for the signs and symptoms. The main complication of Kawasaki disease is the development of dilatation and/or narrowing of one or more coronary arteries.

Treatment Symptomatic Treatment is mainly supportive with hydration, rest and analgesics. Outpatient management is appropriate for many patients with HSP. Inpatient management may be required for management of abdominal pain and arthritis/arthralgia, especially if the child is unable to ambulate. Patients with mild to moderate pain (abdominal or arthralgia) can be treated with paracetamol or non-steroidal anti-inflammatory drugs (NSAIDs). NSAIDs should be avoided if there is evidence of gastrointestinal bleeding or there is renal impairment. Prednisolone 1 mg kg daily (maximum dose 50 mg) for 2 weeks with subsequent weaning over 2 weeks can reduce the intensity and duration of severe joint pain and severe abdominal pain.

Complications Patients with severe abdominal pain or significant bleeding (either as haematemesis, melaena or fresh bloody stools) need close clinical monitoring of their haemodynamic status, radiological imaging, consideration of endoscopy and surgical review. The long-term prognosis of HSP is directly dependent on the severity of renal involvement. The following observations are relevant: • The development of HSPN can occur up to 3 months after an initial episode of HSP with non-renal symptoms and signs. • The onset of HSPN may be asymptomatic. • The severity and⁄or duration of extra-renal HSP symptoms and an older age are the most significant risk factors for developing HSPN. • The clinical and histological severity at the initial HSPN episode is, in general, predictive of a long-term renal impairment. The risk of longterm renal impairment is less than 2% in patients with only isolated haematuria or proteinuria but is ten times greater in those with a nephritic syndrome or nephrotic syndrome at initial presentation. • Children with similar presentations of HSPN can experience either complete disappearance of urinary signs or an unexpected late progression to chronic kidney disease – sometimes in spite of an

apparent long-term remission of symptoms. Hence, prolonged observation of persons with HSPN is necessary. • Early prednisone treatment for HSP does not prevent renal disease. • There are limited evidence-based data about the efficacy of corticosteroids, cyclophosphamide or other immunosuppressive agents in patients with established, persistent HSPN nephritis.

Prevention Angiotensin-converting enzyme (ACE) inhibition decreases proteinuria and slows progression of renal impairment in IgAN. Given the similarities in pathology between IgAN and HSPN, ACE inhibition should be considered in the management of persistent proteinuria in HSPN and used as a first-line therapy for hypertension secondary to HSPN.

Long-term prognosis and follow-up The duration of the disease is less than 14 days in approximately one-third of patients, 2–4 weeks in one-third and longer than 4 weeks in the remaining third. HSP has a very good prognosis unless there are severe gastrointestinal complications (which cause early morbidity) or HSPN develops (which may cause long-term morbidity). Patients with HSP and normal findings on urinalysis should undergo regular urine analysis for at least 1 year after the initial presentation. Patients with HSPN that has caused only microscopic haematuria on initial presentation should be assessed regularly for several years. This group is believed to have an excellent long-term prognosis, but some have developed a late deterioration in renal function. Patients with HSPN who have recovered fully from clinical features of the nephritic syndrome or nephrotic syndrome should be monitored closely for 5 years. Patients with persistent haematuria or significant persistent proteinuria or both should be followed by a renal unit. Women with a past history of HSPN are at increased risk of deterioration of renal function during pregnancy.

Acknowledgements

Julia Linaker assisted with the preparation of this manuscript.

Further reading Bogdanovic R. Henoch-Schönlein purpura nephritis in children: risk factors, prevention and treatment. Acta Pædiatrica. 2009;98:1882–1889. Chartapisak W, Opastirakul S, Hodson E.M, et al. Interventions for preventing and treating kidney disease in Henoch-Schönlein Purpura (HSP). Evid Based Child Health. 2010;5:637–700. Chung D.J, Park Y.S, Huh K.C, Kim J.H. Radiologic findings of gastrointestinal complications in an adult patient with Henoch-Schönlein purpura. AJR. 2006;187:W396–W398. Davin J.-C, Coppo R. Henoch-Schönlein purpura nephritis in children. Nat Rev Nephrol. 2014;10:563–573. Davin J.-C. Henoch-Schönlein purpura nephritis: pathophysiology, treatment, and future strategy. Clin J Am Soc Nephrol. 2011;6:679–689. Floege J, Feehally J. Treatment of IgA nephropathy and Henoch-Schönlein nephritis. J Nat Rev Nephrol. 2013;9:320–327. Ha H.K, Lee S.H, Rha S.E, et al. Radiological features of vasculitis involving the gastrointestinal tract. RadioGraphics. 2000;20:779–794. Jennette J.C, Falk R.J, Bacon P.A, et al. 2012 revised international Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheum. 2013;65:1–11. Khanna G, Sargar K, Baszis K.W. Pediatric vasculitis: recognizing multisystemic manifestations at body imaging. RadioGraphics. 2015;35:849–865. Ozen S, Ruperto N, Dillon M, et al. EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis. 2006;65:936–941. Saulsbury F.T. Henoch-Schönlein purpura in children. Report of 100 patients and review of the literature. Medicine. 1999;78:386–409. Tizard E.J, Hamilton-Ayres M.J.J. Henoch-Schönlein purpura. Arch Dis Child Educ Pract Ed. 2008;93:1–8. Trnka P. Henoch-Schönlein purpura in children. J Paediatr Child Health. 2013;49:995–1003. Williams H. Imaging and intussusception. Arch Dis Child Ed Pract. 2008;93:30–36. Yang Y.-H, Yu H.-H, Chiang B.-L. The diagnosis and classification of Henoch-Schönlein purpura: an updated review. Autoimmun Rev. 2014;13:355–358.

SECTION 17

Psychiatric OUTLINE 17.1. Paediatric psychiatric emergencies 17.2. The treatment of the behaviourally disturbed adolescent 17.3. Autism and behavioural disturbance in the pre-adolescent child

17.1

Paediatric psychiatric emergencies Michael Fairley

ESSENTIALS 1 Emergency department intervention can reduce the risk of recurrence for self-harm and attempted suicide. 2 The presentation may be somatic but the problem psychological. The identified patient may be the child but the problem may reside in the parents or their relationship with the child.

Introduction Paediatric patients and their parents can provide complex diagnostic and logistic challenges to staff in a busy emergency department (ED). They often present in a crisis. Thus their social situation overshadows the presenting problem, which is likely to be psychological rather than physical. Though there may have been multiple visits to previous medical practitioners, no clear diagnosis may have been made. Assessment requires time, patience and unique clinical skills. Intent listening with directed questioning is required. It is important to avoid drawing premature conclusions. Emotional factors should be considered, even when the problem appears predominantly organic.

General approach Assessment

Parents generally initiate medical contact for a child. In their view, the problem resides within the child. The possibility that they may have a problem to which the child is reacting or that the difficulty is in their relationship is not considered and can be unwelcome if suggested. This preconception can introduce a bias into the way information is presented. If possible, a history from both the parent and the child should be obtained. To enable a child to give his/her account, measures such as reassurance that the visit will not be painful and is not a punishment are needed. Playing and drawing may put the child at ease. If possible, the child should be seen individually. In adolescents, self-referral for psychological problems is more likely; however, having presented they may still be reluctant to talk. Physical complaints may mask a psychological issue (e.g. headache, abdominal pain and shortness of breath). Reassurance about confidentiality can enhance rapport, but parents will need to be told about safety concerns and authorities notified of abuse. Avoid making undertakings that cannot be honoured.

History Adolescents wish to be understood, but if they are unwilling to talk about their current problems, they may be prepared to tell about their past and gossip about their friends or family, thereby giving an indirect account. Constructing a genogram, even for a small child, can be an enjoyable activity and illuminate a complex family situation. Always have paper and coloured pens or a whiteboard. Many children communicate better with pictures than words. Children with attention deficit hyperactivity disorder (ADHD) may find it easier to talk if they have something to fiddle with or hold. Extremely restless individuals may be better interviewed in a courtyard than a small interview room.

Collateral history 1. Pre-existing psychological disorders, life experiences, medical conditions and family vulnerability 2. Precipitants – trigger for presentation 3. Presentation – why now, who is most troubled by the symptom 4. Perpetuation – factors that operate to prevent recovery 5. Positives – strengths and resources, coping with previous problems 6. Preconceptions – belief system, expectations from medical consultation.

What the child and parent really want (for example, the child has autism or bipolar disorder and a confirmatory letter is sought).

Examination Examination includes obtaining vital signs, physical and mental state examination. The following points are useful headings for recording observations about mental state: • Appearance • Behaviour during interview • Communication (content, themes, structure) • Affect (mood) • Perception and misperceptions • Cognition (orientation level, formal thought disorder) • Insight into present situation • Judgment.

Investigations Special tests should be confined to the differential diagnosis after clinical assessment. Consider a urine drug screen under appropriate circumstances.

Synthesis of assessment It should be possible for the emergency physician to determine why the child is attending, who wants something done, and where the main pathology rests: in the patient, the parent or their relationship.

Common Paediatric Psychiatric Presentations The acutely disturbed child See separate chapters dealing with this presentation in adolescents (Chapter 17.2) and younger children (Chapter 17.3).

Suicidal patients

Introduction In Australia in 2015, 3027 people died from intentional self-harm. The death rate was 12.6 deaths per 100,000 people compared to a rate of 10.2 suicide deaths per 100,000 persons in 2006. The rate for males was three times greater than that for females. Overall, suicide was the 13th leading cause of death; however, among people 15–24 years of age, suicide accounted for one-third of deaths (33.9%) and was the leading cause of death. In 5–17 year olds, the suicide rate was 2.3/100,000. The rate for males was 2.4 and females 2.2. The rate for adolescent girls has risen since 2006.1 The death of a child or adolescent by suicide is a tragedy. It is an irrevocable act, often in response to transient circumstances. It has an enduring effect on the family and can embolden others to follow suit. Suicide is chosen when no other course of action remains; the person feels trapped. There is a failure of problem solving. In children and adolescents, the predicament that produces this feeling may seem trivial or easily resolved to an adult with more resources and life experience. Even in someone determined to commit suicide, there is a struggle between the wish to die and a wish to live. The strength of these forces changes quickly. Fortunately, most suicidal crises are short lived. If lethal means are chosen, fleeting but intense suicidal urges can result in death. Repeated acts of self-harm, such as cutting or taking overdoses, share many common features with suicide attempts, however, there is less desire for death. Supplanting mental pain by physical pain or overcoming a feeling of numbness is the main aim. Self-harm can escalate to suicide, or death can arise from misadventure.

Assessment Suicidal patients are in emotional pain. They should be treated with kindness and sympathy. Attending to their comfort and explaining what is going to happen will improve rapport and thus the quality of assessment.2 A medical history and physical examination are required to identify trauma, intoxication and underlying medical conditions that may affect the presentation. Cognitive and emotional state should be noted. Toxicology screens should be collected, if indicated. Those who are intoxicated should be observed and reassessed. The patient who is suicidal while intoxicated but denies it when

sober makes assessment difficult. It should be remembered that acute or chronic alcohol abuse raises the risk of suicide, and one-third of adults who completed suicide had consumed alcohol. Psychosocial assessment should include an examination of suicidal thoughts and behaviours, personal history, home environment and mental state. Suicidal ideation may not be volunteered. Asking about it will not incite action. Though there seems much information to be obtained, letting the patient tell his/her story, in his/her own time, enables you to discover what is most important to him/her. This process is therapeutic: Suicidal act: Was there an intention to die? Did the wish to die persist afterwards? How lethal was the means chosen? Was the person able to assess the consequences of his/her action (e.g. due to immaturity or low intelligence)? An isolated location, low probability of intervention, precautions against discovery, detailed planning, communication of intent (e.g. social media posts, suicide note or giving away possessions) and rejection of help after discovery indicate determination and serious intent. Trying to gain help during or afterwards, an impulsive act, intoxication at the time and unawareness of irreversibility do not indicate a low risk of recurrence but rather that the person is unpredictable. Personal history: Precipitants such as bereavement, separation, custody dispute, and disciplinary crisis. Immediate past history such as what is happening at school and home. Interpersonal relationships. Social support systems. Family medical history. Role models for suicide amongst family, friends and heroes. Mental state examination: Current suicidal ideation, depression, pervasive hopelessness, psychotic features (delusions, hallucinations) and quality of interaction with the interviewer. Collateral history should be obtained from family, friends, mental health professionals who know the person and police or ambulance that attended. By the end of the assessment, there should be information that is useful in determining whether this young person has an increased risk of further suicide attempts and for making a mental health referral:

• Details of this and previous suicide attempts • Nature and meaning of current crisis • Current or past depression, other psychiatric disorder, drug and alcohol abuse • History of violent behaviour • Social disadvantage: low socioeconomic status, limited educational achievements, low income, poverty and associated factors such as itinerancy • Indigenous • LGBTI status • Parental loss through separation and divorce • Physical or sexual abuse, domestic violence • Impaired parent–child relationships • Parents with a mental illness such as mood disorders, harmful drug use (including alcohol) or problems involving violence • Loss through suicide in his/her family or peer group • Experiencing a greater number of life stresses than normal • Personality strengths and usual coping mechanisms • Social supports and potential source of help.

Hazards during assessment The young person may minimise the seriousness of the event in order to avoid hospital admission so that he/she can make a further attempt, run away, obtain sharp objects for later use or make a further attempt in ED. Parents and carers may deny the seriousness of the attempt. Staff hostility, especially towards frequent attenders, may interfere with developing rapport. Negative attitudes are common in ED staff, especially to patients who repeatedly self-harm.3 Irritation and hostility arise from the belief that hospital is for the physically ill and these patients are attending unnecessarily. A distinction is made between illegitimate and legitimate patients based on overt responsibility. Legitimate are seen to have a greater entitlement to care even when their actions may have caused the presentation, such as ketoacidosis in a non-compliant adolescent diabetic. Staff anxiety arises from anticipation of being hurt emotionally, feelings of failure as nothing can be done, not knowing how to speak to these patients and expectations to do something that is unrealistic.

ED staff are less likely to feel sympathy for self-harming patients than other hospital staff. Doctors hold more negative views than nurses, and those with more experience have more negative attitudes. Poor clinical practice, such as underestimation of risk through attribution of the injury to attention seeking, can result. Patient satisfaction with their ED experience and outcome measures are also affected. Staff training consistently leads to improved attitudes and understanding of the reasons for the behaviour. Risk assessment using a checklist-based algorithm is ineffective at predicting suicide in an individual. Most who are depressed or self-harm do not commit suicide; suicidal ideation is common but in comparison, completed suicide is rare. Pokorny, in a prospective study of psychiatric inpatients, identified a ‘highrisk’ group using rating scales and known predictors such as suicide attempt, depression, social isolation and drug or alcohol abuse.4 The high-risk group had a suicide rate four times higher than the group as a whole. Thus it was possible to predict a group at increased risk. Those who were admitted for a suicide attempt subsequently had a suicide rate of 1.7% per year. This was a very high rate but still of little assistance in predicting suicide in an individual. Sensitivity and specificity of risk scales were low. False positives result in excessive restriction of those who will not commit suicide, and false negatives lead to inadequate treatment of those who will. It is still possible to define a group that is at increased risk and implement effective measures to reduce suicide. Psychiatric admission is clearly not feasible for all. Fortunately, there are effective measures that are not too onerous.

Management following an attempt While et al. investigated measures that lower the suicide rate. Using national suicide data in England and Wales, implementation of key recommendations of the National Confidential Inquiry into Suicide and Homicide by People with Mental Illness was examined. Those services implementing seven to nine recommendations experienced a decreased suicide rate compared to no change in rate for those implementing fewer. A 24-hour crisis team, with a single point of contact providing prompt response and short-term input in the community, led to the biggest fall of suicide rate from 11.44 per 10,000 patient contacts per year to 9.32. Other measures included in the recommendations were assertive outreach in the community for those that were difficult to reach, follow-up within 7 days of discharge, dual diagnosis policy for patients with psychiatric disorder and

alcohol/substance use, multidisciplinary review and information sharing with families following suicide, training of clinical staff in the management of suicide risk, criminal justice sharing and removal of potential ligature points on inpatient wards. The greatest benefit occurred in the most deprived catchment areas and those with the most patients. Follow-up within 7 days was effective in reducing suicide in the 3 months after suicide attempt. Assertive follow-up was most effective for those who were non-compliant with medication and missed appointments.5 A mental health assessment should always occur. Hickey found that deliberate self-harm patients who left ED without psychiatric assessment were twice as likely to self-harm again during the next year.6 Psychiatric admission, however, is not always desirable even when the adolescent cannot assure his/her safety. Chronically suicidal adolescents may react to efforts to keep them safe as a challenge, engaging in more ingenious and dangerous behaviours while in hospital. Relieved of the demands of everyday life, a reluctance to leave hospital can develop, leading to increased suicidal thoughts around the time of discharge. Admission is helpful when there is diagnostic uncertainty or to initiate a new treatment. A safety plan should be developed in conjunction with the patient. Identifying suicidal thoughts, developing ways to cope with them and listing sources of help can be started in ED. Details of the follow-up appointment with a named mental health professional within the next 7 days, the telephone numbers of the 24-hour crisis service, friends and family should be on a card and entered into the patient’s phone. Measures to reduce access to lethal means, such as secure storage of medication, should be discussed with parents. Mental health follow-up, for as few as three sessions, reduces the risk of further self-harm. In a large international randomised control trial following suicide attempters, Fleischmann found that 0.2% of those who received brief intervention and contact committed suicide in the following 18 months compared to 2.2% of the controls.7

Suicidal patients: important considerations In summary, the major considerations for the assessment and management of a child or adolescent presenting to the ED with suicidal ideation include the following:

1. Medical assessment for trauma, drug ingestion and other physical conditions that could affect the mental state. This may lead to admission to a medical unit 2. Assess cognitive and emotional state. Listen to the adolescent’s story to discover the predicament that led him/her to the attempt. Avoid reliance on checklists especially for predicting risk 3. Safety while in ED 4. Safety planning 5. Mental health assessment in ED 6. Mental health follow-up with treatment of depression and other psychiatric condition.

Anxiety disorders Anxiety is a complex mixture of somatic and cognitive symptoms: autonomic arousal, worry about what has happened and apprehension about what will happen. Though experienced as fear, there is no physical danger. The pulmonary, cardiac and other sensations enhance the experience of alarm. It is a ubiquitous condition that varies from the physiological to the pathological in its presentation. Panic disorder often starts in adolescence and has a lifetime prevalence of 1.5–3.5%. Cases of syncopal collapse may manifest as a panic attack. Conversion disorders present with acute paralysis, loss of vision or sensation. Obsessive compulsive disorder (OCD) represents an attempt to reduce anxiety through extreme control of one aspect of the environment. Children are unable to resist the urge to repeat rituals such as tapping a specified number of times. They frequently attempt to enlist family members in performing rituals and become enraged when they resist. This situation may be mistaken for a behaviour disorder. Separation anxiety presents as school refusal. The child often presents with a somatic complaint such as abdominal pain. Other anxiety diagnoses include phobias, post-traumatic stress disorder and generalised anxiety disorder. Early identification can reduce the number of medical investigations and enable mental health referral. Acute anxiety can be treated effectively with a comprehensive approach utilising cognitive therapy, behavioural techniques, psychotherapy, counselling and medication. SSRI antidepressants are first-line treatment but do not give

immediate symptom relief. For OCD, high doses are often required, and the response may take up to 3 months. Beta-blockers effectively reduce autonomic symptoms and interrupt mounting anxiety driven by sensations usually associated with danger. They should be avoided in asthma. Anxiety is contagious. Families present with a sense of urgency, and there is pressure to prescribe medication that provides immediate relief. Benzodiazepines and antipsychotics are often given in this situation. This should be avoided, if possible. Anxiety is chronic, but the benefits of these medications are short lived. There is the risk of dependence, and side effects are disproportionate to the benefits. There are limitations on prescription for antipsychotics that may subsequently place the GP in an awkward position or expose the family to expense. Non-pharmacological treatments, that are hard work and take time, will be eschewed in favour of something that can be taken. Arranging a referral for mental health assessment may help contain parental anxiety.

Psychosis Hearing ‘voices’ is very common in children and often persists in those with intellectual delay. The voice is the child’s own thoughts and may say to do something naughty, thus distancing the child from the impulse. By early adolescence, the world is more complex, but the cognitive maturity may not have been achieved to maintain contradictory positions simultaneously – ambivalence. One voice may say to do something while another warns against it. It can be difficult to differentiate thoughts from auditory hallucinations. They are often experienced within or outside the head and have distinct qualities, such as a man’s voice, that can be described. The content is almost always unpleasant and can be frightening. In depression, there are critical comments on appearance and performance–saying that the person is a burden to the family, does not deserve to live and should commit suicide. In anxiety, the voice is anticipating all the things that will go wrong. Activity and company can reduce the voices. They are often worst when alone of an evening. Self-harm, cutting and burning, can be an attempt to use physical pain to displace mental pain. There is a risk of acting on the voices’ instructions, in order to get relief. Regular marijuana use often leads to the misperception that everyone is staring at the person, knows about him/her and is mocking. This usually settles spontaneously if consumption is ceased. Since marijuana is often used to self-

medicate dysphoria, the emergence of this symptom can result in increased use. The underlying medical condition is usually evident in delirium. The onset is abrupt, and the mental state fluctuates. Visual and olfactory hallucinations occur as well as auditory and can predominate. Unstable mood is one of the commonest psychiatric symptoms and can occur in any condition. It is often mistakenly equated with bipolar affective disorder. The mood swings in bipolar disorder are more prolonged. Excessively elevated mood is more likely to be the effect of antidepressant medication than bipolar disorder. Common symptoms are loud and continual talking, reduced sleep and disinhibited behaviour. A high dose of antidepressant in young children is most likely to produce this effect. Symptoms will usually settle with cessation of the antidepressant. Psychosis is rare before late childhood. The prevalence increases through adolescence. Environmental stress precipitates illness in children with a genetic predisposition. Onset may be abrupt or preceded by a gradual withdrawal, academic decline and altered perceptions. If acute, there are more likely to be hallucinations, perplexity and fearfulness. It can resemble a confusional state. Mood symptoms are common, making the distinction between schizophrenia and bipolar disorder difficult. Schizophrenia is characterised by auditory hallucinations. Delusions may be created to account for the altered experiences (e.g. spirits coming up from underground). Thought disorder is usually prominent with the flow being blocked or disrupted connections between ideas. Idiosyncratic speech, perseveration, ideas of reference and complaints of thought control also occur. Apathy, flattened affect and withdrawal are disabling and difficult to treat.

Treatment Combined psychotropic and psychotherapeutic treatment is required. Depression should have a mental health assessment because of the risk of suicide. There is a delay, sometimes weeks, before antidepressants improve mood so their introduction in ED is not essential, if follow-up is arranged. Bipolar disorder and schizophrenia require antipsychotic medication. Olanzapine (initial dose 2.5–5 mg), risperidone (initial dose 0.5 mg) and quetiapine (initial dose 25 mg) are the most commonly used. They are calming, reduce aggression and promote sleep. Acute dystonic reactions are more likely with risperidone, and all can cause

akathisia, which is distressing for the patient but may be overlooked by the clinician. As use of antipsychotic medications may be prolonged, and metabolic syndrome is a serious side effect, it is important to establish baseline height, weight and girth. Lipids, HbA1c and fasting blood glucose should be collected early in treatment.

Other psychiatric presentations Acute dystonic reactions Antiemetic and antipsychotic medication can induce acute muscle spasm, most commonly torticollis. Oculogyric crisis, opisthotonos, trismus and grimacing can also occur. The experience is painful and frightening to the patient and carers. Laryngeal spasm is rare but can compromise respiration. Onset is usually within days of starting the medication or following a dose increase. Young males are most at risk. Treatment is benztropine 0.02 mg kg to a maximum of 1 mg intramuscularly or by slow intravenous injection. Response is usually rapid. If there is no response, repeat once after 30 minutes. If still no response, review the diagnosis.8 To prevent recurrence, it is often recommended that the same dose be given orally and repeated twice daily for the next 2 days. If possible, the causative medication should be ceased. Occasionally, anticholinergic medication causes a delirium that can be confused with psychosis. Further antipsychotic medication should be avoided.

Night terrors Sleep disruption is a parent’s most frequent concern during the first 2 years of a child’s life. Half of all infants develop a disrupted sleep pattern serious enough to warrant physician assistance. Night terror disorder is characterised by recurrent episodes of intense crying and fear. There are signs of autonomic arousal, and there is difficulty waking the child during episodes. Children do not recall a dream after a night terror and typically do not remember the episode the next morning. Night terrors are frightening episodes that disrupt family life and cause the child significant distress. Usual onset is between 3 to 12 years. The disorder generally resolves during adolescence. An estimated 1–6% of children experience night terror episodes. Recurrent night terror episodes accompanied by significant distress and impairment are less

frequent. Peak frequency in children younger than 3.5 years is at least one episode per week; among older children, peak frequency is one to two episodes per month.

History The most important step toward diagnosing this disorder is to obtain a detailed history: • Approximately 90 minutes after falling asleep, the child sits up in bed and screams. Prominent autonomic activity (e.g. tachycardia, tachypnoea, diaphoresis, flushing) occurs. The child appears awake but confused, disoriented and unresponsive to stimuli. • Most episodes last 1–2 minutes, but the child may remain inconsolable for 5–30 minutes before relaxing and returning to quiet sleep. • If the child awakens during the night terror, only fragmented pieces of the episode may be recalled. • In the morning, the child typically has no memory of the experience.

Management This consists of educating the parents that night terrors are part of the normal development of sleep. The episodes are not harmful, and it is best not to wake the child. This contrasts to nightmares, after which the child often wakes in distress and requires comforting. As lack of sleep predisposes to night terrors, ensure routine use of sleep hygiene measures.

Acknowledgement The contributions of Raymond Chin as author in previous editions of the textbook is hereby acknowledged.

References 1. Australian Bureau of Statistics, 2016. Causes of Death in Australia, 2015. viewed 15 December 2016, http://www.abs.gov.au/ausstats/[email protected]/mf/3303.0 2. Betz M.E, Boudreaux E.D. Managing suicidal patients in the emergency department. Ann Emerg Med. 2016;67:276–282.

3. Saunders K.E.A, Hawton K, Fortune S, Farrell S. Attitudes and knowledge of staff regarding people who self-harm: a systematic review. J Affect Disord. 2012;139:205–216. 4. Pokorny A.D. Prediction of suicide in psychiatric patients: report of a prospective study. Arch Gen Psychiatry. 1983;40:249–257. 5. While D, Bickley H, Roscoe A, et al. Implementation of mental health recommendations in England and Wales and suicide rates 1997–2006: a cross-sectional and before-and-after observational study. Lancet. 2012;379:1005–1012. 6. Hickey L, Hawton K, Fagg J, Weitzel H. Deliberate self-harm patients who leave the accident and emergency department without a psychiatric assessment. A neglected population at risk of suicide. J Psychosom Res. 2001;50:87–93. 7. Fleischmann A, Bertolote J.M, Wasserman D, et al. Effectiveness of brief intervention and contact for suicide attempters: a randomized controlled trial in five countries. Bull World Health Organ. 2008;86:703–709. 8. Campbell D. The management of acute dystonic reactions. Aust Prescr. 2001;24:19–20.

17.2

The treatment of the behaviourally disturbed adolescent (see Fig. 17.3.4) Kenneth Patrick Nunn, and Meenakshi Rattan

ESSENTIALS 1 Emergency psychiatry is treating the underlying neurobehavioural processes NOT the cognitive content or psychiatric diagnosis. 2 The psychiatric ABCC is a rapid assessment of risk that can be done in a few minutes and enables decisions to be made on the need for sedation and whether sedation is cooperative or non-cooperative. 3 When thinking about management it is worthwhile to move back through the ABCC from the most disrupted young people through to the early levels of distress and dysfunction: C – If cognitive processes are very disrupted hospital admission will be necessary irrespective of the eventual diagnosis. C – If containment is threatened – offer cooperative sedation early, and put security on notice. If containment is actually being breached all other treatment must wait until containment is addressed voluntarily or involuntarily. B – If behaviour is extreme – actively offer relief with calm reassurance, nursing presence and medication while consciously preparing for escalation to a containment breach. A – If arousal is high establish whether this can be readily managed by calmness and cooperative use of medication. If calm is not forthcoming be prepared for behavioural escalation.

Introduction Emergencies require routines and procedures that make decision making seamless, effective and professional. Psychiatric emergencies are no different.

Psychiatry, and adolescent psychiatry in particular, requires clear, well-rehearsed routines to avoid chaotic decision making. No area of medicine is more difficult to routinise without practice than psychiatry. No area of psychiatry punishes poorly operationalised routines as much as adolescent psychiatry. As in all paediatrics, the first question is not ‘which illness?’ but ‘how unwell are they?’ The task of clinical stabilisation is much more important than satisfying a particular psychiatric diagnosis. Once the essentials are understood and applied, the emergency physician can provide consistent, high-quality care in triage, initial stabilisation and management of immediate risks before preparing for transfer for definitive management and ongoing treatment where needed. This chapter is written with the underlying premise that psychiatrists, especially child and adolescent psychiatrists, will be in short supply and sometimes not trained in emergency psychiatry. For the present, outside of large paediatric hospitals, emergency adolescent psychiatry largely falls to emergency physicians and general paediatricians.

The principles of psychiatric triage Purpose Triage enables the early identification and treatment of factors that may threaten the immediate safety and wellbeing of the patient, others and staff in triage and the emergency department (ED). The following priorities are important in the below sequence: 1. Safety 2. Management of distress and behaviour 3. Diagnosis. It is critical that the task of definitive diagnosis does not delay acute management. Whether or not a young person has a particular psychiatric disorder is of a lower order priority in the acute setting. Accurately identifying the: • components of threat • complexity of the threat (that is the number of domains of risk) • severity of the threat

• mitigators of threat (for risk management) all within a rapid time frame requires training, skill and experience.

Time is risk The more time in the ED environs taken before treatment begins, the greater the risk.

Why? 1. Impulsivity, agitation and lack of relief of distress converge with the sensory overstimulation of most ED waiting areas. 2. Time passing constitutes an escalating risk of a loss-of-control or loss-ofcontainment event. Table 17.2.1 Early warning signs Threats to safety

Syndromes of distress

If you feel unsafe or moved to protect If you feel distressed at others watching

Organic flags If you can’t make sense, and the child looks unwell

FIG. 17.2.1 Hierarchy of needs.

3. Where agitation is present, motor activity increased and cognitive processes clearly altered, the risk rises dramatically, even after short periods of waiting. 4. The slide down the spectrum of distress to disruption – anxiety, agitation, anger, together with demanding, impatient, impulsive and explosive behaviour – leaves few options once in full progress. On the other hand, the rapid initiation of triage and the commencement of an altogether less disruptive process – seen, relieved, treated, monitored, transitioned – are each associated with a reduced risk of loss of control within the ED.

Pre-triage An immediate threat of patients to others, themselves or to the medical safety of carrying out procedures requires immediate action analogous to a cardiac arrest. Practicing commonly encountered scenarios with acceptance of ‘error and inefficiency’ is essential.

Early warning signs – subjective

Identifying a safety threat to staff and others? Questions such as those listed below may all provide useful clues, if considered (Table 17.2.1). It is worthwhile acting on this as a given until reassured otherwise: • Do I feel unsafe as a clinician in the ED? • Am I anxious for the patient’s welfare? • Is there a sense of threat around the patient? • Are staff members avoiding the patient because they are afraid?

Early warning signs – observed Even before we sit down with the patient and begin to listen to his/her story, some behaviour stands out as ‘out of control’. If unusual, potentially dangerous, combative or distressing behaviour is observed: • A louder than usual voice • An argument between a parent and child • A surly withdrawn father • A seemingly drunken teenager sitting down is a helpful first step to reducing autonomic arousal and activation in the patient.

The hierarchy of needs All triage involves addressing a hierarchy of needs (Fig. 17.2.1). Safety, symptom relief, initial investigation and a provisional diagnosis (SSRI-PD) form the underpinnings of emergency psychological care. Mental state monitoring and the active exclusion of medical contributors commence immediately upon completing the initial stabilisation.

Signs of threat to safety 1. Threats to self 2. Threats to others 3. Threats to the fabric and good working of the ED imperilling the

treatment of others.

Signs of intense distress By way of analogy with the provision of acute life support, it is helpful to employ the ABCC mnemonic.

The ABCC of rapid psychiatric assessment Arousal – autonomic fright, fight, flight arousal Behaviour – behavioural activation and the presence of withdrawal or agitation Containment – behavioural control within a social setting Cognitive processes – the coherent communication of reality-based thinking and feeling. Identifying early changes, rate of change and the extremity of change to each component of the ABCC gives a quick sense of what needs to be done.

Arousal The triad of early signs of change in arousal, volatility of arousal and extremes of arousal is our focus. Thus, early autonomic signs of either sympathetic (crisis emotions) or parasympathetic shift (calming emotions) include pallor, flushing, tachycardia, bradycardia, tremulousness, mydriasis and tachypnoea. Marked hypervigilance and clouding of consciousness or rapid excursions between the two are very helpful in identifying the need to intervene rapidly. Anxieties may be more difficult to assess with lack of fine social and emotional tuning with either unresponsiveness or exquisite responsiveness to the environment or a rapidly changing mixture of both. If the arousal is sufficiently disruptive the child or young person may have a functional overflow of his/her arousal into his/her behaviour.

Behaviour Early signs of change in behaviour, volatility of behaviour and extremes of behaviour are our focus. Sitting with one leg constantly shaking, wringing of hands or stroking of hair as part of anguish or anger all portend an imminent

deterioration in behaviour. Constant pacing or refusal to move, a loud voice or speaking very quietly, swearing excessively and particularly offensively, or refusing to talk, a broader picture of social disinhibition or extreme inhibition, reflects extremes of response and lack of fine social and emotional tuning. If these are sustained they may overflow and have an impact on the other patients, staff and the functioning of the ED. If this impact is sufficiently disruptive, it may constitute a containment threat or demand for containment.

Containment Behavioural control to reduce major threat and disruption, in an acute medical setting, is termed containment. Containment is usually a physical process which reduces the capacity of a patient to disrupt the ED. It aims to reduce risk to others, risk to self and risk to the environment and safe working of the ED. The acute and open nature of the ED means that any disruptive threat may constitute a broader threat to the provision of urgent medical treatment to the young person in question or to other patients. Changes in arousal normally precede changes in behaviour. Changes in behaviour risk loss of containment. Early warning signs of a loss of containment event have already been covered under the headings of arousal and behaviour. Frequently these early features will have happened prior to being seen in the ED, and the demand for containment is the presenting request. Running away, disrupting the ED, damaging property and creating an atmosphere of threat and menace would all constitute a containment threat or a containment failure. In extreme cases this may involve weapons, the police and the clearing of the ED with cessation of medical activities while a local disaster response is put into place. In most cases, some form of containment, such as the police being in attendance, will be in place before a patient is brought to the ED. It is important that this containment is not lost in the transition process into the ED or in transfer from the ED.

Cognitive processes Early signs of thought disruption are changes in arousal, behaviour, especially speech and containment that belie incoherence of thought, and other cognitive processes.

Other cognitive processes such as attention, executive function (planning, judgment, problem solving and insight) and perception underpin thinking and consequent action. This is not to say that thought or speech content is irrelevant. But the process of thought is more relevant to the emergency setting. In the case of psychosis, containment failure is what is seen, and the fragmentation of thought processes is what is heard. Paranoid or self-destructive ideation is seen in the arousal, behaviour, speech and containment assessment. This can be evaluated without a formal mental status assessment.

What they say is not as critical as how they say it The young person may be worried, fearful or self-loathing or behave in a bizarre, threatening or angry manner. However, the processes of perceived threat (in the extreme, paranoia) and the pursuit of relief (in the extreme, self-harm and suicide) are the underpinning cognitive processes foremost in any emergency assessment. Extremes of incoherence or paranoid ‘coherence’ apply here as well. Jumbled, accelerated, guarded, slowed down, rigidly pre-occupied, fixedly convinced or non-communicative speech and thought are all more worrying process variables, irrespective of what or who is upsetting the patient.

Managing the ABCC including restraint and acute sedation It is inappropriate to attempt to obtain a detailed developmental history, systems review or full exploratory psychiatric history from an acutely distressed and very ill young person. When thinking about management it is worthwhile to move back through the ABCC from the most disrupted young people through to the early levels of distress and dysfunction: C – If cognitive processes are very disrupted, hospital admission will be necessary irrespective of the eventual diagnosis, and a sedative antipsychotic such as droperidol is indicated. C – If containment is threatened, offer cooperative sedation early, and put security on notice. If it is actually being breached, all other treatment must wait until containment is addressed, voluntarily or involuntarily. Droperidol will be necessary.

B – If behaviour is extreme actively offer relief with calm reassurance, nursing presence and medication while consciously preparing for escalation to a containment breach. Once agitation is established sedation with olanzapine (voluntary) or droperidol (involuntary) will be needed. A – If arousal is high establish whether this can be readily managed by calmness and cooperative use of medication. If calm is not forthcoming be prepared for behavioural escalation. Quetiapine will usually be sufficient. There are only six essential things which must be attempted: 1. Rapport and respect – even where they cannot be achieved. 2. Identify the immediate issue of concern to the young person and to others while establishing a formulation in medical terms. 3. Identify risks – self and others, including child maltreatment, medical and reputation risks. 4. Symptomatic relief – provide immediate treatment with whatever biological, psychological and social supports and resources are available. The provision of relief is the single biggest factor in reducing risk. 5. Identify psychosocial supports and stressors (immediate), i.e. who is the consenting authority for the young person and where he/she normally lives. None of these may be available in the acute situation. However, he/she will save a great deal of time in determining the disposition of the patient. 6. A plan of action for the next 24 hours.

Sedating the adolescent brain When attempting to reduce hyper-arousal and behavioural agitation, to manage a breach of containment, it is helpful to consider which parts of the brain – or neurobehavioural systems – are being targeted. The neurobehavioural systems are referred to here as sub-brains as a useful shorthand. The thinking brain (the cortex) – easiest to sedate, especially in children – inhibits the other two ‘lower’ brains. When the cortical brain is sedated, the two lower brains (disinhibition) are released. Benzodiazepines are often enough to

sedate this brain. However, paradoxical excitement may result, so quetiapine is preferable: Target: arousal and preoccupation – for example, the inability to stop thinking about distressing events despite sustained talking through and reassurance. The feeling brain (the limbic system) – takes longer and more medication to sedate, especially when distress is established. Quetiapine may be enough, but where psychosis, anger, agitation often olanzapine (voluntary) or droperidol (involuntary) will be needed: Target: arousal and distress – for example, the inability to find emotional relief despite sustained and skilled reassurance. The moving brain (the basal nuclei) – takes longest to sedate and first to awaken from sedation. Olanzapine or droperidol will always be needed to sedate this brain: Target: behaviour and agitation – for example, the inability to be able to move in an emotional paralysis or to sit still due to agitation, despite sustained reassurance.

Tracking the seven stages of sedation 1. Fixation: immobilising the body to enable vascular access or intramuscular injection and a safe medical procedure. 2. Induction: commencing sedation with the steady reduction of consciousness. 3. Disinhibition: loss of emotional and behavioural control associated with loss of cortical inhibition before limbic and basal nuclei are similarly inhibited. This may occur after an initial settling. 4. Stabilisation of arousal depth – titrating to a level of sedation that maintains gag reflexes, pharyngeal patency and adequate breathing and oxygenation. 5. Maintenance: high level observation with clear parameter thresholds and specification of appropriate response when thresholds are breached.

6. Emergence: the period of decremental lowering of medication to allow a transition to the fully conscious state, with an awareness that disinhibition may occur during this process as during the establishment of sedation. 7. After care: the psychological explanation and support required specifically in relation to the sedation process.

Five tips on sedating adolescents 1. Start high (without bolus), and titrate down in an emergency. 2. If there is definite motor agitation do not use a benzodiazepine alone, which may disinhibit the patient. Add droperidol, and monitor for extrapyramidal side effects, such as dystonia. 3. If re-sedation is likely, move to regular doses rather than p.r.n., ‘ebb and flow prescribing’. Less medication is needed if non-p.r.n. 4. Establish sedation away from the main ED (more private and less disruptive), and maintain and monitor the sedation close to the main ED. 5. Plan for emergence risks – ensure security presence during emergence from sedation or preparedness to re-sedate.

Signs of organic dysfunction • Elevated temperature in a psychotic patient • Appearance – looking lost or ‘out of it’ with lowered eyelids, glazed eyes and ‘in a world of his/her own’ • Behaviour – recent personality change with loss of social fine-tuning and reversal of sleep rhythms • Speech – muddled speech and thinking, often with paranoid but fragmented themes • Perception – visual illusions, i.e. misidentifying actual stimuli, e.g. shadows mistaken for people or frank visual and tactile hallucinations (especially with antihistamine or anticholinergic overdose) • Ideation – often paranoid in thinking • Cognition – clouded and fluctuating levels of consciousness and disorientation • Judgment and insight – impaired markedly.

Differential diagnosis – medical 1. Specific toxidromes and syndromes of adverse drug reaction for: • substance abuse • overdose • accidental ingestion 2. Delirium – generalised acute brain syndrome 3. Frontal lobe syndromes (including traumatic brain injury, solvent abuse and intellectual disability) 4. Seizure-related disorders 5. Starvation-related syndromes 6. Cerebellar dysfunction: • slurred speech • ataxic gait • incoordination • substance abuse, e.g. benzodiazepine • toxicity from prescribed medications, e.g. anticonvulsants • ingestion of poisons, e.g. alcohol, phenytoin. 7. Dementia – generalised chronic brain syndrome 8. Neurodevelopmental: autism, fetal alcohol spectrum disorder (FASD) and severe attention deficit hyperactivity disorder (ADHD), intellectual disability with associated respiratory, ear, urinary or other pathology or pain (see Chapter 17.3).

Differential diagnosis – psychiatric 1. Acute on chronic self-harm or aggression in chronic complex posttraumatic stress disorder in abused, neglected and/or out-of-home-care youth. 2. Substance abuse in as yet undiagnosed mentally ill young people – especially major depression or bipolar disorder. 3. Bipolar depression treated with anti-depressants or stimulants. 4. Aggression treated with stimulants or SSRIs or both, worsening aggression. There is no acute indication for SSRIs or stimulants. 5. Psychosis treated with stimulants or weight-loss dopamine agonists. 6. Cryptic abuse in outwardly well-functioning family.

7. Emerging paranoid illness presenting for the first time with aggression.

The management of acute risk including medical risk Managing threat to staff and others • Do not ask medical or nursing staff to expose themselves to unreasonable risks. • Offer cooperative oral sedation early such as quetiapine or olanzapine. • Work closely with security staff, seek other help early and provide sedation backup. • Don’t address long-term issues in an ED setting.

Behaviours that usually de-escalate aggression Nothing works all the time, and nothing works in every case. But some of these are likely to be helpful. Ten DOs 1. Be respectful, friendly and open (single most important strategy, especially respect). 2. Be quicker to listen than to speak. 3. Speak clearly, quietly, gently and calmly with an expectation that they will respond. 4. Use humour that shows we accept we have things about us that are not ideal, perfect or completely ‘respectable’, especially when we are derided or spoken to rudely. 5. Declare desire to be helpful even if it is not always known how to help or what it is they want. 6. Relax posture, voice and face, even if preparing internally for fight and/or flight. 7. Move slowly, predictably and with due respect for distance. 8. Distract to details that they might be interested in as well, especially things about them and what they like. 9. Acknowledge any faults in our behaviour (not someone else) that might be contributing to their being upset.

10. Acknowledge your own tiredness, a hearing problem (if they are withdrawn, hostile, talking quietly), irritability or crabbiness after a busy shift which emphasises our humanity and reduces their fear. Ten DON’Ts 1. Try to shame them into good behaviour by telling them they are childish, silly or stupid. 2. Try to get above them (so called ‘towering’) physically, by position, verbally, intellectually or socially. 3. Talk without listening or ‘nag’. Don’t all talk at once – one person only. 4. Issue ultimatums – ‘do this or else’ – almost always leads to ‘or else’ in this population. 5. Back into corner either physically or psychologically (unless everything else has failed and safety demands we must). 6. Adopt a ‘thou shalt’ tone of voice instead of a reasonable request. 7. Raise voices in a counter ‘arc-up’ to the patients. 8. Mock, criticise or accuse the patients at any time. 9. Rush the process or create time pressure. 10. Hark back to previous behaviour or try to sort out longstanding issues, unless this is a previously agreed treatment goal.

The four main themes 1. Communicate respect. Many of them have not been treated with respect. 2. Communicate our desire to keep everyone safe. We want them, and us, to be safe. Many of our patients have not lived in safe environments. 3. Take responsibility. Show a willingness to acknowledge we may have contributed to their distress by what we have done or not done. Many of the young people who will present have lived with people who have never accepted blame, use denial a great deal and blame others. 4. Appreciate their distress even though it may not be fully understood why they feel like this or how bad they feel. Many of the patients have been told how they feel (even if they did not) and had the legitimacy of their distress invalidated. They may never have been in a situation where high distress is tolerated, validated and appreciated so long as safety is high as well.

Identify patients’ threat to themselves • If patients or those with them feel unsafe, they probably are unsafe. • If your ABCC evaluation says they are unsafe they are unsafe. • If a colleague outside the hospital says they have been behaving unsafely, be very wary of not honouring their assessment.

Managing a threat to themselves • Remain calm and firm, enlist security, maintain constant observation and, if actually attempting, once you are able to ensure staff safety, intervene immediately. • Identify treatable disorder and social predicaments while waiting for suicidality to settle (usually attenuates over hours to days NOT weeks to months).

Identify medical risks • The broader medical needs of the patient need to be recognised. • Psychiatric disorder does not protect against medical disorder. • It is possible to do a great deal with a non-cooperative patient to clarify medical status.

Manage specific medical risks • Obtundation – due to panic sedation in response to a previous failed sedation in adolescents who became disinhibited (see below). • Extrapyramidal side effects – nuchal spasm with headache is common in the young as a form of dystonia. • Respiratory – laryngeal dystonia – especially in younger patients but still rare. Use of benzodiazepines (even in reduced doses) or anticholinergics such as benztropine with droperidol. • Cardiac – prolonged QTc, especially in poor CYP2D6 metabolisers (5% of normal population). • Neurological – delirium (common, especially substance abuse), serotonergic syndrome, neuroleptic malignant syndrome (rare) (NMS). • Concurrent medical disorder – asthma, diabetes, traumatic brain injury,

epilepsy, atopy and anorexia. • Dermatological – lamotrigine-induced Stevens–Johnson syndrome. • Metabolic – lithium toxicity – especially chronic toxicity.

Sentinel nursing observations post intramuscular or intravenous sedation Respiratory rate, O2 saturation, pulse, blood pressure and level of consciousness should be checked continuously during the maintenance of sedation – and documented each 15 minutes for 2 hours, then each 30 minutes for 2 hours. An electrocardiogram to screen for prolonged QT interval should be performed as soon as possible after any sedation with zuclopenthixol acetate or droperidol.

Four tips for monitoring adolescents 1. Expect the medication to be metabolised quicker. 2. They become disinhibited more easily going into sedation and coming out of sedation. 3. Maintaining sedation intravenously (IV) should be done with a slow injection over several minutes – avoid boluses to avoid respiratory depression (benzodiazepines) or marked hypotension (droperidol). 4. Small initial doses because of timidity about sedating young people followed by large doses because of little effect leading to obtundation is to be avoided.

Voluntary/cooperative – oral agents preferred First line Hyper-aroused but not psychotic, aggressive or severely agitated: Quetiapine 5 mg kg–1 Psychotic, aggressive or severely agitated: Olanzapine wafer 40 kg–10 mg

Involuntary/uncooperative – parenteral agents preferred Intramuscular injection (IMI) will often be the route of choice, and the agent

should be relatively quick, effective and safe: Droperidol 0.1–0.2 mg kg–1 (max 10 mg) is first line and may be repeated after 15 minutes. Perform an ECG when able to screen for prolonged QT interval and potential arrhythmias. Midazolam or ketamine is an option which may be used if there is an inadequate response to two doses of droperidol. The patient must be continuously monitored for respiratory depression and other potential complications.

Transfer is a potential escalation of risk • The leaving – are they stable? • The transfer – are they monitored with an adequate response to thresholds strategy? • The arriving – are the recipients prepared?

Conclusion Emergency psychiatry is treating the underlying neurobehavioural processes not the cognitive content or psychiatric diagnosis. Process psychiatry requires a different mindset. Emergency physicians are ideally trained to adopt that mindset.

Further reading Barzman D.H, DelBello M.P, Adler C.M, Stanford K.E, Strakowski S.M. The efficacy and tolerability of quetiapine versus divalproex for the treatment of impulsivity and reactive aggression in adolescents with co-occurring bipolar disorder and disruptive behavior disorder(s). J Child Adolesc Psychopharmacol. 2006;16(6):665–670. Brown E.N, Lydic R, Schiff N.D. General anesthesia, sleep, and coma. N Engl J Med. 2010;363:2638– 2650. Busch A.B, Shore M.F. Seclusion and restraint: a review of recent literature. Harv Rev Psychiatry. 2000;8(5):261–270. Glick R.L, Berlin J.S, Fishkind A.B, Zeller S.L. Emergency Psychiatry – Principles and Practice. Philadelphia: Lippincott Williams & Wilkins; 2008. Haddad F, Gerson R. Helping kids in crisis: managing psychiatric emergencies in children and adolescents. Arlington: American Psychiatric Publishing; 2015. Hillard R, Zitek B. Emergency Psychiatry. New York: McGraw Hill Professional; 2004.

Meehan K, Zhang F, David S, et al. A double-blind, randomized comparison of the efficacy and safety of intramuscular injections of olanzapine, lorazepam, or placebo in treating acutely agitated patients diagnosed with bipolar mania. J Clin Psychopharmacol. 2001;21(4):389–397. Nunn K.P, Dey C, eds. The Clinician’s Guide to Psychotropic Prescribing in Children and Adolescents. Sidney: Child and Adolescent Mental Health Statewide Network (CAMHSNET) Publications; 2003. Nunn K.P. Emergency paediatric psychiatry: an emerging sub-specialty. J Paediatr Child Health. 2017;53(7):628–629. Petit J.R. Handbook of Emergency Psychiatry. Philadelphia: Lippincott Williams & Wilkins; 2004. Prager L.M, Donovan A.L. Suicide by Security Blanket and Other Stories from the Child Psychiatry Emergency Service: What Happens to Children with Acute Mental Illness. Santa Barbara: Praeger Press; 2012. Slaby A.E, Lieb J, Tancredi L.R. Handbook of Psychiatric Emergencies. New York: Medical Examination Publishing Co; 1975. Steiner H, Saxena K, Chang K. Psychopharmacologic strategies for the treatment of aggression in juveniles. CNS Spectrums. 2003;8(4):298–308. Zimbroff D.L, Marcus R.N, Manos G, et al. Management of acute agitation in patients with bipolar disorder: efficacy and safety of intramuscular aripiprazole. J Clin Psychopharmacol. 2007;27(2):171– 176.

17.3

Autism and behavioural disturbance in the pre-adolescent child Meenakshi Rattan, and Kenneth Patrick Nunn

ESSENTIALS 1 Clinicians should aim to create calm, quiet, non-pressured minicontexts for autistic children within the sensory-overloaded, unfamiliar and sometimes frightening environment of the emergency department. 2 Recounting a history of behavioural disturbance in front of a child is likely to escalate behavioural disturbance. 3 Most autistic children do not know why they are upset. ‘Why’ questions are usually unproductive or counterproductive. 4 The non-tactile physical examination of the fully clothed uncooperative child is an essential paediatric skill pending a better available alternative. 5 Medical causes of behavioural disturbance are less common but increasingly important the younger the child, the more severe the intellectual disability, the less his/her speech and the more sensory deficits the child has. 6 Autistic children can be more difficult to restrain and sedate than most adults. 7 Deciding when to proceed to sedation and when to move from voluntary to involuntary sedation must be done calmly and with anticipation, rather than as a consequence of many failed attempts to get control of the child’s behaviour.

8 Long-term management and appropriate community follow-up are of critical importance.

Introduction The emergency physician is often surprised to find that some of his/her biggest behavioural difficulties are encountered with young autistic children who are presented by others in a state of being ‘out of control’ or aggressive. The tiny number of children who are regarded as ‘unsedatable’ without a general anaesthetic includes many with autistic spectrum disorders. They often require more mg kg of medication than adults to achieve a safe and stable level of sedation. Autism and the autistic spectrum of disorders (ASD) affect behaviour and experience and include the following: 1. Social and emotional agnosias (disorders of social and emotional awareness and recognition) 2. Social and emotional dysphasias (disorders of social and emotional communication) 3. Emotional and social dyspraxias (disorders of social and emotional coordination of movement). Children with ASD have trouble making sense of their surroundings and the expectations of others. They have trouble communicating their own condition and why they are feeling what they are feeling. They have trouble implementing problem solving and converting a behavioural request into a coping strategy. The levels of autonomic arousal, both sympathetic and parasympathetic, can be profound, extreme and prolonged. Once hyper-aroused, they often have very little capacity to de-escalate themselves within a crisis and an unfamiliar setting like an emergency department (ED). This chapter gives a simple structure to prepare for dealing with these children as a matter of routine, communicating professionalism and calm, while excluding serious medical and psychiatric disorders. Importantly, they are likely to return to the ED with repeated presentations if appropriate community followup to address the long-term issues of environmental reduction of threat, stimulus management and predictability of nurture are not put in place.

The underlying cause of aggression in autistic children like any other child can be multifactorial as shown in Box 17.3.1.

Underlying mechanisms in austistic spectrum of disorders/neurodevelopmental disorders Most aggression in young people, especially autistic young people, is fear based. Frustration, especially in face of limit setting, is also common. In the rest, sensory overload or under-stimulation (Fig. 17.3.1) accounts the majority of situations where neither a medical disorder nor a discrete psychiatric syndrome is involved. Many unsuitable home settings, classrooms or residential placements have too much of the wrong sort of stimulation and too little planned activities geared to the developmental profile of the child. Reducing fear and frustration through communication of what will happen over the day, what is happening and what has happened is a simple and effective preventive. Reducing sensory overload and understimulation are avoided by always having something for the child to do but never too much to do. In the acute setting reassuring that safety is our goal, that they are not in trouble and reducing the number of people dealing with the child will help much more than threatening with a ‘show of force’, saying what ‘they must not do’ and allowing everyone ‘to have go’ at settling the child with an audience looking on.

Approach to assessment Is sedation necessary? Try to determine the current state of agitation before determining the cause. Untreated persistent agitation in the absence of even passive cooperation is the most likely indicator that involuntary sedation will be necessary. The risk of injury/violence should be based on known past history, present agitation and whether staff and carers are feeling unsafe, until better information is available.

Physical examination Mental state examination should assess for physiological arousal, extremes of motor agitation or withdrawal, containment breaches such as absconding or damaging property and cognitive incoherence and fragmentation (Fig. 17.3.2).

Some children will be extremely uncooperative with a full physical examination. However, a rapid but thorough examination is essential. Even in very uncooperative children, much can be gained from careful observation. Note neurological function and general health (pulling at ear, guarding the abdomen, stridor, respiratory distress, abnormal gait, pale or flushed skin). Box 17.3.1 Differential diagnosis of acute aggression

in autistic children A). General medical conditions Most common medical causes: • Chest infection • Urinary tract infection • Otitis media and/or otitis externa • Abdominal pathology, such as constipation, appendicitis or inflammatory bowel disease • Any other occult cause of pain (dental pathology, fractures, inguino-scrotal conditions). Less common but very serious: • Ictal and peri-ictal events • Head trauma (sometimes self-inflicted head banging, which can cause retinal detachment) • Encephalitis, meningitis, or other inflammatory encephalopathies • Acute delirium • Metabolic derangement (e.g. hypoglycaemia, hyponatraemia, hypocalcaemia, liver or renal failure) • Hypoxia.

B). Drug induced/withdrawal Although substance addictions seem to be less common in autistic children, usage still occurs. However, this is more often a problem with adolescents than younger children. More commonly they involve the following: • Recent commencement of stimulants, SSRIs or antihistamines

• Other drug toxicity, inappropriate ingestion or solvent abuse • Alcohol, hallucinogens, stimulants (amphetamine-type substances and cocaine), cannabis, synthetic opioids, benzodiazepines

C). Mental health conditions Most common psychiatric causes: • Anxiety disorders • Chronic complex post-traumatic stress disorder due to neglect and maltreatment combined with uncertainties of placement and future plans in the out-of-home-care child • Agitated depression • Dysexecutive syndromes – inability to cope with unplanned occurrences, unfamiliar settings and changes to routine • ADHD – especially in foetal-alcohol-related disorders. Less common but very serious: • Mood volatility (emerging bipolar disorder) • Psychotic disorders (emerging schizophrenia).

D). Others • Developmental crises associated with puberty • Psychosocial adjustment – the shift from primary school to high school • Situational crises such as parents fighting, children teasing and loved ones leaving or dying • Impulse control disorders – restricting behavioural addictions, such as games use or repetitive behaviour.

History The history should be taken from a parent or carer who is familiar with the child, with particular emphasis on antecedents to and consequences of any behavioural difficulties (Fig. 17.3.3). It is important not to recount all the child’s behaviour difficulties in front of the child. Looking at phone videos of behavioural crises in

the child’s presence is humiliating and likely to re-escalate his/her behavioural disturbance. Detail should be sought on current medication plans, behaviour support plans, as well as any communication plans/aids and sensory considerations for the patient.

Challenges in emergency department presentations • An ED is generally a strange, noisy and unfamiliar environment with the pervasive threat of separation and needles. • Triage can be inappropriate (time is risk of loss of containment), and waiting times can be long. • There can be idiosyncratic responses to standard interventions. • Staff appreciation of the patient’s level of functioning and unique ways of communicating can vary greatly.

Approach to management The management should follow and implement the underlying principles of autism management: early identification of disturbed and/or aggressive behaviour, the use of de-escalation strategies, early effective medication use, and the minimisation of the use of manual and mechanical restraint: • Effective use of the parent or carer • Use of communication/visual aids if appropriate • Team work and collaboration • Multidisciplinary approach (emergency physician, psychiatrist, allied health, social worker, carer) • Early recognition of escalation • Following policies and guidelines • Medications (avoid oral benzodiazepines due to increased risk of disinhibition) • There is no acute indication for commencement of stimulants or selective serotonin reuptake inhibitors (SSRIs) – this should be done in the community by those following up the child if it is truly indicated. • Proactive crisis management plan.

Management Sedation must be carried out with a definitive plan and clear therapeutic end points. Inadequate planning of sedation can lead to a situation of ‘undersedation–oversedation and obtundation’ where small doses of medication are administered, sufficient only to disinhibit any remaining controls the child has, followed by panic prescribing when these doses fail to help leading to obtundation.

Acute (Fig. 17.3.4) The important components of management of acute behavioural disturbance include the following: • Safety and containment • Aggression minimisation strategies • Verbal de-escalation • Negotiated voluntary oral sedation • Restraint • Involuntary parental sedation • Continued assessment once control of behaviour is achieved. Avoid benzodiazepines, as in autistic children there is high risk of disinhibition, and very large doses are usually required to have any sedative effect. If extrapyramidal side effects occur after administration of antipsychotic medication, administer benztropine 0.02 mg kg (adult dose 1–2 mg) IM or IV. If respiratory depression occurs after benzodiazepines, administer flumazenil 0.02– 0.04 mg kg, titrated to respiratory effort.

FIG. 17.3.1 Underlying causes of aggression in autism. Adapted from Schopler E. Behavioral priorities for autism and related developmental disorders. In: Schopler E, Mesibov GB, editors. Behavioral Issues in Autism. New York: Plenum Press; 1994, p. 55– 75.

FIG. 17.3.2 ABCC (Arousal, Behaviour, Containment, Cognitive processes) of emergency psychiatry.

‘The unsedatable child’ may very occasionally need a general anaesthetic with supported airway with or without an alpha2 adrenergic agonist infusion and management in ICU for 24–48 hours. Seek specialist child psychiatry advice and involve anaesthetic and intensivist colleagues.

Restraint • Five-point restraint (one for each limb and one for head); supine position • Team leader should be at head end, managing airway and monitoring condition. • Avoid prone restraint. • Physical restraint should only be used to facilitate appropriate and effective chemical sedation. • Explain to child and family, and try voluntary holding/cuddling when appropriate and safe.

Monitoring Level of consciousness monitoring and pupil size monitoring are useful to assess medication response and sedation. Monitoring of vital signs should occur every 5 minutes for 20 minutes after each dose of parenteral medication, then every 15 minutes for the next 2 hours.

Ongoing management in the community • Behavioural modification interventions – behavioural support planning/positive behavioural reinforcement • Applied behavioural analysis (ABA) programmes • Picture exchange communication system (social understanding/social story) • Functional/sensory assessment and treatment • Disability services: Aged Care and Disability, National Disability Insurance Scheme • ASPECT (Autism Spectrum Australia) • Private psychologists/psychiatrists • School counsellors • Headspace • CAMHS (Child and Adolescent Mental Health Service).

Conclusion Triage of an autistic child needs to be prompt and appreciative that the whole emergency department can be locked down to deal with a behavioural containment breach if not addressed expeditiously. Autistic children represent the most common cause of ‘unsedatable’ children in EDs. This is particularly problematic if occurring during retrieval in-flight or on-road transport. General EDs, based on size and age, are prone to underestimate the chaos that is possible with these children. Autistic children require medical assessment, including physical examination, even if the method of examining is less than ideal. It can be very reassuring to parents and carers when medical factors have been ruled out in children who cannot communicate their needs. If sustained agitation is present and cooperation is absent, involuntary

sedation needs to occur in an organised and rapid fashion. The sedation team requires a similar level of proficiency and professionalism as one managing a cardiac arrest.

FIG. 17.3.3 Approach to assessment in autism. Adapted from Addabbo L,Bulhak-Paterson D. Behaviour Management Strategies for Individuals with Autism Spectrum Disorders, 2011, Autism Victoria, (http://www.amaze.org.au)

FIG. 17.3.4 Sedation algorithm for children with autism/autistic spectrum disorders and acute severe behavioural disturbance.

It is often the emergency physician/paediatrician who insists on a coherent community response to prevent future unproductive and distressing crises. Adequate and ongoing community follow-up is vital to ensure appropriate care and to reduce the risk of repeated ED visits with behavioural disturbance.

Further reading

Baren J.M, Mace S.E, Hendry P.L, et al. Children’s mental health emergencies-part 2: emergency department evaluation and treatment of children with mental health disorders. Pediatr Emerg Care. 2008;24:485–498. Bassuk E.L, Birk A.W. Emergency Psychiatry: Concepts, Methods, and Practices. New York: Plenum Press; 1984. Bradley E, Lofchy J. Learning disability in the accident and emergency department. APT. 2005;11:45–57. Corson A.H, Barkenbus J.E, Posey D.J, Stigler K.A, McDougle C.J. A retrospective analysis of quetiapine in the treatment of pervasive developmental disorders. J Clin Psychiatry. 2004;65(11):1531–1536. Dossetor D, White D, Whatson L. Mental Health of Children and Adolescents with Intellectual and Developmental Disabilities: A Framework for Professional Practice. Victoria: IP Communications; 2011:249. Farmer C.A, Aman M.G. Pharmacological intervention for disruptive behaviors in intellectual and developmental. Disabil J Am Acad Child Adolesc Psychiatry. 2013:281–325. Fernández-Mayoralas D.M, Fernández-Jaén A, Muñoz-Jareño N, et al. Treatment with paliperidone in children with behavior disorders previously treated with risperidone. Clin Neuropharmacol. 2012;35:227–230. Fitzpatrick S.E, Srivorakiat L, Wink L.K, Pedapati E.V, Erickson C.A. Aggression in autism spectrum disorder: presentation and treatment options. Neuropsychiatr Dis Treat. 2016;12:1525–1538. Isbister G.K, Calver L.A, Page C.B, et al. Randomized controlled trial of intramuscular droperidol versus midazolam for violence and acute behavioral disturbance: the DORM Study. Ann Emerg Med. 2010;56:392–401. Golubchik P, Sever J, Weizman A. Low-dose quetiapine for adolescents with autistic spectrum disorder and aggressive behavior: open-label trial. Clinic Neuropharmacol. 2011;34(6):216–219. Hoyle J.D, White L.J. Treatment of pediatric and adolescent mental health emergencies in the United States: current practices, models, barriers, and potential solutions. Prehosp Emerg Care. 2003;7(1):66–73. McCracken J.T, McGough J, Shah B, et al. for Research Units on Pediatric Psychopharmacology Autism Network. Risperidone in children with autism and serious behavioral problems. N Engl J Med. 2002;347:314–321. Kowalski J.L, Wink L.K, Blankenship K, et al. Paliperidone palmitate in a child with autistic disorder. J Child Adolesc Psychopharmacol. 2011;21(5):491–493. Koegel L.K, Koegel R.L, Hurley C, Frea W.D. Improving social skills and disruptive behavior in children with autism through self-management. J Applied Behavior Analysis. 1992;25(2):341–353. Bertelli M.O, Rossi M, Keller R, Lassi S. Update on psychopharmacology for autism spectrum disorder. Adv Mental Health Intellect Disabil. 2016;10:6–26. Malone R.P, Carter J, Sheikh R.M, Choudhry M.S, Delaney M.A. Olanzapine versus haloperidol in children with autistic disorder: an open pilot study. J Am Acad Child Adolesc Psychiatry. 2001;40(8):887–894. Newton A.S, Ali S, Johnson D.W, et al. A 4 year review of pediatric mental health emergencies in Alberta. CJEM. 2009;11(5):447–454. Nunn K.P, The treatment of the behaviourally disturbed adolescent. In: Cameron P, Jalinek G, Everitt I, Browne G.J, Raftos J, eds. Textbook of Paediatric Emergency Medicine. London: Elsevier; 2012:386–390. Scattone D, Wilczynski S.M, Edwards R.P, Rabian B. Decreasing disruptive behaviors of children with autism using social stories. J Autism Dev Disord. 2002;32(6):535–543. Starling J, Bridgland K, Rose D. Psychiatric emergencies in children and adolescents: an emergency department audit. Australas Psychiatry. 2006;14(4):403–407.

Tanaporn P, Chutima T, Jeerawan J, Worachon Y. Comparison of the effectiveness of oral diazepam and midazolam for the sedation of autistic patients during dental treatment. Pediatr Dentist. 2005;27:198– 206.

SECTION 18

Crisis Intervention OUTLINE 18.1. Sexual assault 18.2. Child at risk

18.1

Sexual assault Susan Marks

ESSENTIALS 1 Sexual assault occurs when a child is engaged in sexual activity that the child cannot comprehend, for which the child is developmentally unprepared and cannot give consent, and/or that violates the law or social taboos of society. 2 Sexual assault includes a spectrum of activities ranging from rape to physically less intrusive sexual activity.1,2 3 Assessment and management of children following alleged or suspected sexual assault is a highly specialised area and requires a multidisciplinary, multiagency team approach.

Introduction Medical assessment of child sexual assault (CSA) requires a dedicated, welltrained and experienced doctor who is able to spend a significant amount of time making an unhurried and thorough assessment and detailed documentation of history and examination findings. The doctor must have an accurate knowledge of genital anatomy and experience in performing genital examinations. Skills and experience in this field are developed through postgraduate studies, significant case numbers, a knowledge of current literature and involvement in peer-review practices.3,4 Inexpert assessment of such cases may have a profound negative influence on the child and family. It may potentially lead to inappropriate removal of the child from the family or wrongful imprisonment5 or, conversely, to fail to protect a

child from further abuse. The roles of the emergency physician in this process are: • recognition of the possibility of sexual assault • emergency treatment of acute physical injury • consideration of the need for urgent toxicology screen6 • referral to the local specialist sexual assault service (SAS) if available • ensuring that the following services are provided if indicated (these services are usually provided by the specialist sexual assault service): • anogenital examination and collection of forensic evidence • provision of emergency contraception and/or antibiotic and antiviral prophylaxis • provision of appropriate psychological support to the child and family • protection of the child and referral to local child protection agencies. In the majority of cases, determination of whether or not sexual assault has occurred is not possible within the emergency department (ED).

Definitions CSA is the use of a child for sexual gratification by an adult or significantly older child/adolescent.7 It may involve a range of activities that vary from exposing the child to sexually explicit materials to anal or vaginal penetration of the child. Central to the definition is that the child cannot provide truly informed consent for sexual activity with adults. Sexual play between children of similar age does not fit into this description. The term ‘assault’ is preferred over ‘abuse’ as it highlights the criminal nature of the activity and avoids minimisation of such abusive acts.

Attitudes/myths surrounding child sexual assault The subject of CSA is an emotive one. Emergency physicians will often have strongly held opinions and attitudes on this subject. These attitudes may be shaped by past experience and/or social taboos. In order to approach CSA in a

calm, non-judgemental and objective manner it is important that emergency physicians are cognisant of their own opinions and emotional responses. In dealing with victims of CSA, expressions of anger, sadness or surprise are not helpful and potentially stigmatising and harmful to the child. With emergency physicians infrequently encountering CSA, it is useful to reflect on the following, sometimes poorly understood, statements: • A broad range of sexual behaviours has been observed in ‘normal’ children. • Most children are not abused by strangers.8 • As historians, children are no less reliable than adults. • CSA is not normally an isolated incident. • CSA uncommonly produces severe genito-anal injury.3,9–12 • CSA often occurs in the context of other family problems, including physical abuse, emotional maltreatment and substance abuse.13

Epidemiology of child sexual assault There has been a significant increase in the recognition of CSA,3,13,14 which has been reflected by a substantial increase in the number of reports made to child protection services across Australia and overseas. Sexual assault of children of all ages and both sexes has been documented and is committed predominantly by men, who are commonly members of the child’s family, family friends or other trusted adults in positions of authority.13 Sexual abuse by family members or acquaintances usually involves multiple episodes over periods ranging from a week to years. Victims of unknown assailants tend to be older than children who are sexually abused by someone they know and are usually only subjected to a single episode of abuse. The estimated proportion of children exposed to some form of sexual assault varies depending on the definition of sexual abuse and methodology used. In the United States, literature surveys provide estimates of 9–52% for females and 3– 10% for males.8 In Australia, between 2010–11 and 2014–15, the rates of child maltreatment have remained steady for all types of abuse and neglect except for emotional abuse, which has increased from 2.2 to 3.4 per 1000 children over this time.15 Sexual abuse was the least common form of substantiated harm (or risk of

harm) from child maltreatment for 2014–15 (12.9% of all substantiations). Girls were significantly more likely to be the subject of substantiation cases of sexual abuse (16.7% of all substantiations) compared to boys (9%).15

Child sexual assault and emergency medicine Children who are victims of sexual assault may present to EDs in a variety of circumstances: 1. They may be seen for an unrelated matter when routine history and physical examination produce information where sexual assault forms part of the differential diagnosis. 2. They are brought by a parent or carer to the ED for evaluation of suspected abuse. 3. They are brought to the ED by social services or the police for a medical evaluation for possible sexual abuse as part of an investigation. 4. They are brought to an ED after a suspected acute sexual assault for evaluation, evidence collection and crisis management.

Recognition of child sexual assault Recognition of the possibility of CSA is dependent on history and examination findings, both of which are normally non-specific. In the majority of cases, physical examination will neither confirm nor refute an allegation of sexual assault. History from the child remains the single most important diagnostic feature in coming to the conclusion that a child has been sexually abused.16

Signs and symptoms Non-specific Children who have been sexually assaulted may develop a variety of emotional and physical complaints, often unrelated to the genital area. These include: • developmentally regressive behaviour • deterioration in school performance • sleep disturbances

• abdominal pain • enuresis, encopresis • phobias • sexualised behaviour.

Specific • Disclosure by child • Genitoanal injury • Sexually transmissible infection • Pregnancy.

Genitoanal injury Only 4% of all children referred for medical evaluation of sexual abuse have abnormal examinations at the time of evaluation. Even with a history of severe abuse, such as vaginal or anal penetration, the rate of abnormal medical findings is only 5.5%.16 The physical examination of sexually abused children should not result in additional emotional trauma. When the alleged sexual abuse has occurred within 5 days or there is bleeding or acute injury forensic examination should be performed within an appropriate time frame by the designated sexual assault service medical officer. In this situation, protocols for CSA victims should be followed to secure biological trace evidence such as epithelial cells, semen, and blood, as well as to maintain a ‘chain of evidence’. When more than 5 days have passed and no acute injuries are present, an emergency examination usually is not necessary. An evaluation, therefore, should be scheduled at the earliest convenient time for the child, physician and investigative team. In the child presenting with genitoanal injury or abnormality, CSA is only one of a number of diagnoses that should be considered. The differential diagnosis of genitoanal injury includes: • accidental injury • falls astride • sexual assault • medical/dermatological condition, e.g. lichen sclerosis/drug reaction.

Genital findings in children are difficult to interpret. Such interpretation is generally beyond the expertise of most emergency physicians17. Whilst acute trauma may be easily recognised, interpretation of such findings may be problematic for the occasional examiner.17

Genitoanal anatomy Knowledge of what constitutes normal and abnormal anatomy has evolved over recent years. This has been driven partly by several highly publicised cases where misinterpretation of normal findings led to inappropriate separation of children from parents and wrongful conviction.

Hymen There is considerable variation in the shape of the hymen. In the prepubertal girl it can be thin and relatively inelastic. In this age group, blunt penetrating trauma to the vagina may result in tearing of the hymen. Such tears when healed may manifest as a notch or defect in the hymenal tissue. It is also possible to have penetrating injury to the hymen without any abnormal findings on examination. As an oestrogen-dependent/responsive tissue, at puberty the hymen becomes thick, irregular and elastic and distensible. It is less likely to sustain injury during penetration than in the prepubertal state.

Sexually transmitted infections The diagnosis of a sexually transmitted infection in a child warrants a thorough assessment by a specialised sexual assault service.18

Diagnostic considerations The diagnosis of CSA can often be made based on a child’s history. Physical examination is infrequently diagnostic in the absence of a history and/or specific laboratory findings. Physical findings are often absent even when the perpetrator admits to penetration of the child’s genitalia. Many types of abuse leave no physical evidence, and mucosal injuries often heal rapidly. On examination, findings which are suggestive, but not diagnostic, of CSA include injuries indicative of acute or healed trauma to the genital/anal tissues. The American Academy of Pediatrics regularly updates their guidelines for the

medical assessment and care of children who may have been sexually abused including a consensus approach to Interpretation of Medical Findings in Suspected Child Sexual Abuse.19 Many cases of alleged sexual abuse involve parents who are in the process of separation or divorce and who allege that their child is being sexually abused by the other parent during custodial visits. Although these cases are generally more time consuming, they should not be dismissed because a custody dispute exists. Allegations of abuse that occur in the context of divorce proceedings should be reported to the child protective services agency.

Role of the emergency physician The emergency physician (EP) should ensure that any physical injuries are detected, accurately documented and correctly treated. In most jurisdictions, medical assessment of CSA will be performed by a specialist sexual assault service. Medical issues, such as sexually transmitted diseases and emergency contraception, should be discussed and managed. The EP should collect, or provide opportunity for collection of, forensic specimens, ensure appropriate psychological support is provided to the child and family, and report the case to protective agencies within the legislation of the local jurisdiction.

Documentation Because the likelihood of civil or criminal court action is high, detailed records and/or drawings should be kept.

Mandatory reporting legislation Some form of mandatory reporting legislation exists in all Australian jurisdictions. Although this legislation varies from state to state, the basic principles are similar. Doctors are mandated to report cases where there is reasonable suspicion that CSA will occur or is occurring. The reporting practitioner has statutory protection from prosecution if a report is made in good faith.

References 1. American Academy of Pediatrics. Committee on Adolescence, . Sexual assault and the adolescent. Paediatrics. 1994;94:761–765. 2. American Academy of Child and Adolescent Psychiatry, . Practice parameters for the forensic evaluation of children and adolescents who may have been physically or sexually abused. J Am Acad Child Adolesc Psychiatry. 1997;36:423–442. 3. Donald T, Wells D. Graduate Diploma in Forensic Medicine, Subject guide. Melbourne: Monash University Centre for Learning and Teaching Support; 2000:253. 4. Jenny C, Crawford-Jakubiak J.E, Committee on Child Abuse and Neglect, , American Academy of Pediatrics, . The evaluation of children in the primary care setting when sexual abuse is suspected. Pediatrics. 2013;132:e558–e567. 5. Butler-Sloss E. Report of the Enquiry into Child Abuse in Cleveland 1987. London: HMSO; 1988. 6. Kaufman M. Care of the adolescent sexual assault victim. Pediatrics. 2008;122:46. 7. Kempe C.H. Sexual abuse, another hidden paediatric problem. The 1977 C. Anderson Aldrich lecture. Paediatrics. 1978;62:382–389. 8. Tomison A. Update on child sexual abuse. National Child Protection Clearinghouse: Issues in child abuse prevention number 5. 1995. http://www.aifs.gov.au/nch/pubs/issues/issues5/issues5.html. 9. Adams J.A, Harper K, Knudson S, Revilla J. Examination findings in legally confirmed child sexual abuse: it’s normal to be normal. Paediatrics. 1994;94:310–317. 10. Finkel M.A. Anogenital trauma in sexually abused children. Paediatrics. 1989;84:317–322. 11. McCann J, Voris J, Simon M. Genital injuries resulting from sexual abuse: a longitudinal study. Paediatrics. 1992;89:307–317. 12. McCann J, Voris J. Perianal injuries resulting from sexual abuse: a longitudinal study. Paediatrics. 1993;91:390–397. 13. Finkelhor D. The international epidemiology of child sexual abuse. Child Abuse Negl. 1994;18(5):409–417. 14. Leventhal J.M. Epidemiology of child sexual abuse. In: Oates R.K, ed. Understanding and Managing Child Sexual

Abuse. Sydney: Harcourt Brace Jovanovich; 1990. 15. Child abuse and neglect statistics CFCA Resource Sheet— October 2016, Australian Institute of Family Studies. 16. Heger A, Ticson L, Velasquez O, Bernier R. Children referred for possible sexual abuse: medical findings in 2384 children. Child Abuse Negl. 2002;26(6–7):645–659. 17. Makoroff K.L, Brauley J.L, Brandner A.M, et al. Genital examinations for alleged sexual abuse of prepubertal girls: findings by pediatric emergency medicine physicians compared with child abuse trained physicians. Child Abuse Negl. 2002;26(12):1235–1242. 18. Hammerschlag M.R. Sexually transmitted diseases in sexually abused children. Adv Pediatr Infect Dis. 1988;3:1–18. 19. Adams J.A, et al. Updated guidelines for the medical assessment and care of children who may have been sexually abused. J Pediatr Adolesc Gynecol. 2016;29:e81–e87.

18.2

Child at risk Susan Marks

ESSENTIALS 1 Concerns of child abuse or neglect arise when an adult responsible for the care of the child either harms the child or fails to protect the child from harm. 2 A child may be at risk from physical abuse, sexual abuse, emotional abuse or neglect. 3 Medical and nursing practitioners have a responsibility to protect children at risk and are often mandated by law to report suspicions that a child is at risk of significant harm

Introduction Child abuse is increasingly recognised as a major public health and social welfare problem with important short-term and long-lasting effects for children and adolescents. As Australian and New Zealand emergency departments (EDs) provide care for many hundreds of thousands of children and adolescents each year, departments and their staff play an important role in the detection of abuse and initiation of a medical and community response. This response is aimed primarily at treating the child, minimising psychological effects and ensuring their safety. Children and adolescents are, by virtue of their developing intellectual, emotional and physical state, a vulnerable group. The environment within which they develop is influenced by many factors outside their control: the economic and social status of their family, the personality and values of family members

and friends, and the extent of physical and intellectual stimulation that they receive may all have profound influence upon their development. The potential variability in these factors and the recognition that negative experiences often have serious short- and long-term implications for the child have led to a general acknowledgment that children and adolescents need protection. The United Nations Convention on the Rights of the Child recognises that: ... for the full and harmonious development of his or her personality, [a child] should grow up in a family environment, in an atmosphere of happiness, love and understanding .... The Convention continues, stating in Article 19 that governments shall: ... take all appropriate legislative, administrative, social and educational measures to protect the child from all forms of physical or mental violence, injury or abuse, neglect or negligent treatment, maltreatment or exploitation, including sexual abuse, while in the care of parent(s), legal guardian(s) or any other person who has the care of the child .... It is this philosophy that has driven the creation of the social and legal framework of child protection. Doctors, nurses and other healthcare workers who deal with children are an integral part of this system that acts to protect children and adolescents. Every health professional that has contact with children needs to be aware of the possibility of child abuse, must be able to detect when it is occurring and know how to act in the best interests of the child once it is suspected.

Definition The child at risk is not a medical diagnosis but rather a description of certain forms of behaviour displayed by adults responsible for the care of a child. The child is at risk when an adult responsible for the care of the child harms, threatens to harm or fails to protect the child from harm. Harm may be physical (e.g. inflicting an injury, causing pain or poisoning), psychological (e.g. causing feelings of being unloved or worthless; exposure to domestic violence) or neglectful (e.g. failure to meet the child’s basic needs and/or medical needs, lack of adequate supervision). A child may be at risk of harm from:

• physical abuse • sexual abuse • emotional abuse • neglect. These are not exclusive, and a child may be subjected to more than one type of abuse.

Physical abuse Many thousands of children present to EDs around Australia each year with a wide range of physical injuries, the vast majority of which are caused accidentally. It is a difficult but essential task to identify children within this group who have been injured as a consequence of abuse. While medical and nursing staff must be alert for the possibility of an inflicted injury, there are specific circumstances that may raise suspicion. These include: • situations where there are direct allegations of violence directed against the child made by the child or any other person • the type and pattern of injury observed at the examination • an explanation being offered for an injury that does not fit the type or pattern of injury or does not fit with the developmental ability of the child • delayed presentation for medical care with an injury that a reasonable person would have recognised as needing care sooner • multiple presentations with injuries, often to different healthcare providers, seeking medical attention. Once the possibility of inflicted injury has been raised the priorities of the treating doctor are: 1. to diagnose, treat and document the child’s injuries 2. to interpret the pattern of injury or behaviour as to the possible causes 3. to notify and involve the agency responsible for ensuring safety of the child 4. to provide a written or verbal report and advice to that agency or the police.

Presentation Physical abuse may present to the ED in many different ways. Most commonly it will present as a child with an obvious injury and a suggestive history, but in some situations it will be more subtle, such as a younger child who presents not using a limb. Young children who present with symptoms such as vomiting, irritability or a decreased conscious state with no obvious cause may have a head injury from a blow or a fall or a ‘shaking’ injury or may have been poisoned.

History It is necessary to collect as much information as possible on the events that led to the child sustaining the injuries. Specifically, enquire as to when, where and how the injury happened, who was present at the time and what happened after the injury. The child’s medical, developmental and social history, with specific information on past injuries, is important. This history must be sought in an open and non-judgemental fashion, which encourages the participants to reveal all the important information. Unfortunately, the ED is often not the ideal place to conduct a lengthy and indepth forensic interview with the parents or carers, and it may be prudent to limit the information gathering to items that will enable specific issues to be addressed. The interview can always be completed by trained investigators at a later time.

Physical examination Prior to commencing the examination the doctor must ensure that the parents and, where appropriate, the child are informed of the nature and extent of the examination and that valid consent has been given. In addition it is ideal to have spent some time with the child, to gain his/her confidence and thus increase the chances of keeping his/her cooperation during the examination. Consent from the parent or legal guardian is necessary to conduct a physical examination, to perform investigations (including photographs) and to release clinical information in the form of a report to a third party. If consent is refused the protective agency or police must seek a court order. If there is an urgent medical problem that needs intervention and such intervention is clearly in the best interests of the child, then the examination and treatment should proceed and not be delayed by the lack of consent.

An adolescent may be able to give consent for his/her own examination as long as he/she is capable of understanding what the examination entails, what the results will be used for and the implications that this may have for him/her. A thorough physical examination of the child should be performed, with observation and palpation of skin, soft tissues, bones and joints and giving specific attention to the eyes, ears and mouth. The examination should look for the following physical findings: Bruising of the skin Bruises are extremely common in children. In the absence of a documented bleeding tendency they are evidence of blunt trauma and may provide some information on the site, the implement or force of an impact. Accidental bruises are commonly found in children once they have learnt to crawl, occurring over bony prominences, usually on the front of the body and are directly related to a child’s increasing motor activity. Babies who are not yet crawling rarely have accidental bruising. Bruises caused by abuse may occur anywhere on a child’s body. Specifically, look in places where accidental bruises are uncommon such as the mouth, behind the ears, on the inner aspect of the upper arm and around the buttocks. Observe the shape and pattern of bruises, looking for features that may suggest a blow from an open hand or single or multiple blows from an implement. Look for a pattern within the bruise that may suggest contact with a specific surface. Whilst it is important to describe the appearance and colour of the bruising, it is not possible to be accurate about the age of a bruise. If a bruise is yellowing in colour then it is likely to be more than 18 hours old. Laceration and abrasion of the skin Lacerations are the tearing of tissues caused by a blow from a blunt object. Abrasions are the disruption of the outer layers of the skin caused when the skin contacts a surface at an angle. They occur at or close to the site of impact and may occur after a blow with an object or after a fall on to a surface. They are frequently associated with bruising. Examine the wound for neurovascular and tendon injury and for foreign bodies. The presence of foreign material such as glass, dirt or gravel should be noted as they may be important in evaluating the injury. Burns or scalds

The appearance of a burn on a child is influenced by many factors: the temperature, size and shape of the causative agent, the depth of skin at the contact site, the length of time of contact and the application of first-aid measures all have the potential to modify its characteristics. Whilst information on all of these factors should be sought from the caregivers it is often difficult to draw accurate conclusions from examination of the wound itself. Associated injuries such as bruises or fractures may help. Although classically described as associated with inflicted injury, cigarette burns are uncommon. These appear as small, deep, round burns, usually on the limbs or back. Healing burns can sometimes be especially difficult to diagnose and interpret. Inflammation can extend beyond the margins of the burn, obscuring the shape and increasing the size of the lesion. The healing area may become flaking or exudative, causing confusion with skin conditions such as impetigo. Scalds are a common form of accidental injury in infants, often caused by a hot liquid being tipped over the upper torso, arms or hands: some features of a scald may suggest an intentional cause. Look at the position, shape and depth of the burn. Circumferential scalds of the hands or feet may be caused by forced immersion. Small round bruises above the burn may represent forcible gripping by a hand. Scalds of the buttocks extending on to the lower back or upper thighs with sparing of the natal cleft may indicate the child has been lowered into hot water. Fractures Fractures of long bones, ribs and skull may occur when a child is intentionally struck, pushed, squeezed or dropped. While any fracture may be caused by inflicted injury, certain fractures have an association with abuse that should alert the ED clinician and prompt further action. Specifically, fractures in children under the age of 18 months, rib fractures, metaphyseal fractures, multiple fractures and fractures of differing ages should be carefully evaluated. A bone scan and skeletal survey may be extremely useful in gathering evidence of multiple bony injuries when a young child, typically under the age of 2 years, presents with suspicious bruising or other features of abuse. Eye injuries Direct blows to the face may cause subconjunctival haemorrhages; intraocular injuries such as retinal haemorrhages may occur as a result of shaking injury. A careful eye examination including visual acuity and fundoscopy may be

necessary. Ear injuries Injuries such as blows to the side of the face or pinching may cause bruising of and behind the pinna. The eardrum may rupture due to the air pressure changes. Head injuries Head injuries are a major source of mortality and morbidity in inflicted injury. Young children have a large head-to-body size ratio, relatively weak neck musculature and compliant skull bones that predispose them to intracranial injuries. The child may be struck, dropped, thrown or shaken, producing an open or closed head wound. Skull fractures, cerebral contusion, intracerebral haemorrhages, extradural haematoma and subdural haematoma are all possible sequelae. Intra-abdominal injury Blows to the abdomen may cause laceration or rupture of either solid abdominal organs, such as the liver or spleen, or of the hollow organs, such as the duodenum. There may or may not be accompanying bruising of the abdominal skin to alert you to this possibility. Liver function tests and lipase may be helpful screening investigations. Investigations Investigations are done for a number of reasons, including the following: • Further assessment of the presenting injuries: • Primarily, the findings of the clinical examination dictate the extent and type of investigations necessary. As would be the case in the investigation of any injured child, plain X-rays, CT scans, MRI, ultrasound and other imaging should be directed at areas where there is clinical suspicion of injury. • Assessment for possible occult injuries: • Other investigations, such as a skeletal survey and bone scan, are used in an attempt to detect injuries that may not be clinically apparent but which will assist in establishing the likelihood of inflicted injury. These are especially useful in children under the age of 2.

• A urine drug screen may be indicated. • Investigation for underlying disease: • In a child with multiple bruises, the possibility of a bleeding disorder should be considered. A full blood examination and coagulation profile may be necessary in these circumstances. • Children with multiple fractures may need screening for underlying bone disease.

Emotional abuse This is the commonest type of abuse that is reported to child protection agencies. Emotional or psychological abuse is difficult to define and even harder to detect, particularly in the ED, when frequently the child presents to a particular health worker on a single occasion. Five possible components of emotional abuse have been described. These are the following behaviours: • Rejecting • Isolating • Terrorising • Ignoring • Corrupting. The incidence of emotional abuse is unknown, but it is likely to be common and underdiagnosed.

History and examination Presentation is subtle and depends upon the age of the child. An infant may present with sleep or feeding problems, irritability or apathy. Older children may present with attention deficit, attention seeking, aggression, school failure, truancy, anxiety, depression and psychosomatic disease. It is likely that emotional abuse accompanies other forms of abuse, such as physical and sexual abuse. Consideration of its possible role is important in all assessments of a child at risk. Detection by all health workers looking after children, including emergency staff, requires a high index of suspicion and vigilance. Diagnosis is suggested by the consequences in the child, as above. Assessment should include that of behavioural, emotional and physical signs

and the child–parent interaction. This will usually require time not available to emergency staff, and therefore, when emotional abuse is suspected, referral is necessary. This may be to the hospital child protection unit, to other health services for further assessment and/or to community social services.

Neglect Neglect may also cause the child to be at risk and can be difficult to define and diagnose. A broad definition is anything that individuals, institutions or processes fail to do, which directly or indirectly harms children or damages their prospects of a safe and healthy development into adulthood. Other definitions have tended to be narrower and therefore target more severe or persistent neglect. It is important to differentiate neglect from poverty or ignorance, as these will require a different intervention. The true incidence of neglect is unknown, and it is probably the most common reason for the child to be at risk. Its diagnosis usually only occurs when harm has occurred, but consideration should be made of potential for harm and longterm effects. Types of neglect include medical neglect, safety neglect, educational neglect, physical neglect and emotional neglect. Non-organic failure to thrive is likely to be due to a combination of a lack of calories and affection.

History and examination Some possible features to look out for are frequent presentation and admission to hospital with accidents or illness, delay or failure to access health care, malnutrition, failure to immunise, poor physical presentation, poor compliance, behaviour disorders, developmental delay and, very importantly, failure to thrive. The assessment of growth and development is clearly an essential part of child health assessment, even in the emergency setting. Measurement of height, weight and head circumference and their plotting on standardised growth charts can be very useful in assessment and follow-up. Emotional and behavioural assessment is difficult in the emergency setting.

Treatment The diagnosis of non-organic failure to thrive usually requires admission to hospital to assess the child’s ability to grow with adequate nutrition and an interdisciplinary approach. Other forms of neglect may require admission or, if not, referral to child protection/abuse unit or community social services.

Medical child abuse Medical child abuse, previously known as Münchausen’s syndrome by proxy (MSBP) and/or factitious illness, is an unusual presentation of the child at risk that may present to an ED. This condition occurs when an adult caregiver, usually the mother, presents a false history to the physician regarding a child. This history may cause the emergency physician to perform unnecessary diagnostic and therapeutic procedures that do not result in any specific diagnosis. In the past, the focus of diagnosis was on an underlying psychiatric diagnosis for the caregiver. Medical child abuse is a diagnosis focused on what is happening for the child. The presentation may involve fabricated or induced symptoms or signs. Children at risk for medical child abuse are typically aged 15 months to 6 years. The emergency physician is often confronted with baffling symptoms. Frequently, the child has been taken to many care providers before the diagnosis is finally established. Warning signs that are suggestive of medical child abuse include the following: • Illness is multisystemic, prolonged, unusual or rare • Symptoms are inappropriate or incongruent • Symptoms disappear when parent or caregiver is absent • General health of patient clashes with results of laboratory tests • Symptoms (e.g. seizure activity) are unresponsive to conventional medical treatment and are witnessed only by parent or caregiver.

The community response to the child at risk Responsibilities to report For medical practitioners working in acute medicine, often the first point of contact with a child at risk is when they present for treatment. Mandatory reporting legislation brought in across all states of Australia has made it compulsory to report cases to the State Child Protection Agency where there are concerns of risk of significant harm due to suspected inflicted injury or neglect. ED staff should familiarise themselves with the mandatory reporting requirements and processes in their jurisdiction. Once notification is made a process of risk management is commenced.

What to do as the medical practitioner, in suspected cases 1. Take a concise history of events and physical examination. 2. Document injuries, and order appropriate investigations. 3. Manage injuries, as usual practice. 4. Notify relevant child protection agency. (Client’s consent is not required in child protection notification.) 5. This may be facilitated by social worker, nurses and paediatric specialists. 6. Arrange appropriate ongoing medical care for the child. This may require hospital admission for further investigations and/or for child protection reasons (to ensure the child’s safety and wellbeing).

Legal responsibilities 1. By law, health services staff must provide all relevant information that they have available when asked (in writing) to do so by the child protection authority. 2. Staff do not have to get permission of the client in order to forward the relevant information. 3. Protection for notifier. Typically there will be legislation safeguarding the identity of the person who makes the report. 4. Neither the report nor its contents are admissible as evidence in any proceedings against the person who made the report. 5. If, as a result of making a report, a person is threatened or fears personal violence, this should be reported to the police, who may apply for, and pursue on his/her behalf, an apprehended violence order.

What happens after notification? 1. The Child Protection Agency determines their response when someone reports that he/she thinks a child or young person under the age of 16 years has been, or is being, injured or neglected. 2. When the agency receives information about suspected inflicted injury, it makes decisions about how to go ahead with investigating the claims and how others may be able to help. For example, it may contact the

child’s teacher, child-care worker, relatives or the police. 3. The police will be contacted and may become involved if the agency thinks the law has been broken. 4. Health professionals should ensure that appropriate heath care plans are in place.

Actions based on risk assessment 1. If the child is in immediate danger, steps will be taken to reduce the level of risk or move the child to a safe place. This may mean admission to hospital or foster care. 2. In many cases, this might mean giving the family practical help, such as organising child care, emergency finance, providing a referral for counselling or information on health or other services. 3. In some cases, the child protection agency takes the matter to the Children’s Court. The court can order a child be placed in agency care for a period of time. The court can also order counselling and other types of support services including health services.

Further reading Browne K, Hanks H, Stratton P, Hamilton C. Early Prediction and Prevention of Child Abuse: a Handbook. Chichester: John Wiley & Sons; 2002. Christian C, AAP Committee on Child Abuse and Neglect, , AAP Section on Child Abuse and Neglect, . Understanding Abusive Head Trauma in Infants and Children. Elk Grove Village, IL: American Academy of Paediatrics; 2015. Flaherty E, Perez-Rossello J.M, Levine M.A, et al. Evaluating children with fractures for child physical abuse. Pediatrics. 2014;133:e477. Gabarino J, Guttman E, Seeley J. The Psychologically Battered Child. San Francisco: Jossey-Bass; 1988. Hobbs C, Hanks G, Wynne J. Child Abuse and Neglect: a Clinician’s Handbook. London: Churchill Livingstone; 1999. Oates K.R. The Spectrum of Child Abuse: Assessment, Treatment and Prevention. New York: Brunner/Mazel; 1996. Reece R. Child Abuse: Medical Diagnosis and Management. Elk Grove Village, IL: American Academy of Pediatrics; 2008. Roesler T, Jenny C. Medical Child Abuse. Elk Grove Village, IL: American Academy of Pediatrics; 2009. Tomison A, Tucci J. Emotional Abuse: the hidden form of maltreatment. Sidney: Australian Institute of Family Studies; 1997. Ward M. The medical assessment of bruising in suspected child maltreatment cases: a clinical perspective. Paediatr Child Health. 2013;18(8):433–437.

SECTION 19

Administration In EMS OUTLINE 19.1. Managing the death of a child in the emergency department: Bereavement issues

19.1

Managing the death of a child in the emergency department Bereavement issues Ioannis Pegiazoglou

ESSENTIALS 1 The death of a child under any circumstances is likely to lead to a significant crisis and grief response in parents. 2 Emergency physicians should be prepared for parental presence in the resuscitation room, anticipate their high level of distress, and ensure that they are kept informed. 3 It is important that the family knows that everything that could have been done was done. 4 Parental questions should be answered honestly and directly, allowing humanity and empathy to show. 5 Personal, compassionate and individualised support should be provided for families, respecting their cultural, religious and social values. 6 Family members are likely to have impaired decision making and communication abilities, and this needs to be taken into consideration around informed consent issues. 7 The needs of the grieving family must be balanced with the legislative requirements of the Coroner’s Act when this is relevant. 8 Relatives should be allowed to spend time with the deceased child if

they want to, preferably in a quiet suite. 9 It is important to be available in the weeks following the death to clarify and answer any further questions from the family. 10 Team members need to be aware of their own likely emotional responses to the death of a child.

Introduction Deaths occurring in the emergency department (ED) present unique challenges for the clinician, particularly if the patient is a child.1,2 The unexpected death of a child undoubtedly brings about the most severe and shattering grief response for the child’s parents.3 Because the loss is unexpected and involves someone so young and so intrinsically a part of self, the grief response of parents may be very painful and prolonged. The death of a child must be viewed as a tragedy for the entire continuum of family and friends. Additionally, paediatric deaths are frequently personalised by ED staff and hence have broad implications for the whole ED clinical team. In large hospital EDs, particularly in urban areas, there is rarely a pre-existing relationship between the health professionals and the patient/family. While this facilitates the professional detachment needed for ED staff to function effectively, it creates inherent voids in the ability to support grieving relatives and friends. In smaller hospitals like those found in rural and regional communities, there may be a pre-existing relationship, potentially lowering communication barriers but bringing out other stresses and strains for ED staff. Good communication with family members must be established early and maintained throughout. This is best left to an experienced member of the staff. There is evidence to suggest that junior medical staff do not feel adequately trained in talking with parents in regards to end-of-life care matters.4 Furthermore, in an AAP (American Academy of Pediatrics) statement published in 2008 it was noted that ‘health care communication is currently learned primarily through trial and error’.5 Simulating scenarios of difficult discussions in paediatric emergency medicine can help the ED providers to develop specific strategies when managing those challenging events.6 Due consideration for the comfort of the family should be at the forefront of the minds of clinical staff at all times.

Box 19.1.1 Essential components of care in the

emergency department when a child dies Clinical Resuscitation best practice Termination of resuscitation: Identifying, validating, and respecting advanced care directives

Operational Staff training in communication Team response (including readily available support staff such as security, child life, chaplaincy, social work) Family presence policy Dealing with media Communication with medical home Defusing/debriefing for team: Private location for family to be with deceased, means and location to conduct rituals

Legal and forensic Organ donation Autopsy Working with police and coroner/medical examiner Child protective services Child fatality review team Documentation in medical record Preservation of evidence

Ethical Resuscitation: how long is too long? Prolongation of resuscitation efforts for family presence/organ donation Practice on newly deceased

Initiation of resuscitation at the border of viability in extreme preterm birth

Spiritual and emotional Needs of family, including saying goodbye, memory making Needs of multidisciplinary team Envisioning a ‘good death’ in the emergency department

Follow-up care for family Helping family to know everything was done Assisting family in explaining to siblings, family, friends Assisting family in locating community support to address grief and bereavement Plan for post-autopsy meeting to answer questions Plan for scheduled follow-ups and marking of meaningful dates

Follow-up care for team Scheduled voluntary defusing/debriefing with all members of the emergency care team who wish to participate Adapted from Death of a Child in the Emergency Department, Joint Statement of American Academy of Pediatrics Committee on Pediatric Emergency Medicine; American College of Emergency Physicians Pediatric Emergency Medicine Committee; Emergency Nurses Association Pediatric Committee. Pediatrics 2014;134(1):e313–30.

In summary, essential components of care in the ED when a child dies include clinical, operational, legal, ethical and spiritual layers (Box 19.1.1).7

The resuscitation process Parents usually benefit from being present during the resuscitation process.8 It is therefore unacceptable to discourage their presence unless they are interfering with, and compromising, the resuscitation itself. Family members watching monitors and seeing the trace ‘go flat’ experience much alarm and distress, but this should not be seen as a reason to exclude them.9 In a study published in

2006, Mangurten et al. reported that 95% of the families they surveyed would again wish to be present during the resuscitation process and felt that it had been helpful to them, and no disruption of care was documented.7,10 In a similar study examining pediatric trauma resuscitation efforts, there also was no difference in time to milestones of care in trauma patients with or without family members present.7,11 The resuscitation process can be traumatic for parents and family members, requiring ongoing communication and interpretation of events. It should be expected that parents will be visibly upset and distressed during this period. A staff member, often a social worker, should be assigned to support the family, to answer any questions about the procedures and responses, and to prevent distraught family members from impeding the resuscitation.12 The ED medical officer in charge must communicate with this staff member and family members about the progress of the resuscitation. Viewing the resuscitation efforts allows the family to see a caring and competent staff, in control of their emotions, doing their best to save the child’s life. Where parents choose not, or feel unable, to be in the resuscitation room, it is essential that they be kept informed of progress. Panic, fear and a sense of isolation have been noted as the main responses of relatives who remain outside the resuscitation room.9 Small, dull rooms with no windows or natural light were seen as heightening the sense of isolation, disconnectedness and fear for those family members unable to bring themselves to view the resuscitation. It is important to be skilled in early recognition of the signs of trauma responses by parents, such as dissociation, as this can affect long-term adjustment. A social worker or other designated professional should ideally be available to provide support for parents and act as an advocate during what is likely to be an overwhelming and bewildering process. The social worker is also likely to be the main staff member to have an ongoing role after death has occurred and the family has left the hospital.

Talking to parents and families When talking with the family about the child’s deteriorating condition, give details in a simple, straightforward and accurate manner. Provide the information using appropriate language. Answer questions and be responsive to needs and concerns. When death has occurred, or is imminent, it is essential to have identified the

relevant family members so that discussions are with the appropriate individuals. At the point of death, the medical officer in charge of the resuscitation should advise those family members present in the resuscitation room or in a private, quiet location. Research has indicated that families appreciated a high level of physician involvement.13 Clear, distinct and accurate information is essential, and medical jargon should be avoided. It is very important to state initially that the child has died. This is the piece of information that the parents will most want clarified. It is then desirable to provide a brief chronology of events, while reassuring the family that everything was done and that the child did not suffer pain. Sometimes family members are not present at the time of death. If practicable it is best to delay notification of death until it can be done in person.14 On the other hand, strict adherence to the goal of family presence at time of death pronouncement may result in the prolongation of otherwise futile resuscitative efforts. An alternative may be to designate a family surrogate, a staff member whose job is simply to be with the child, so when family members do arrive after their child has died, they can be assured that their child was not alone at the time of death.7 If the family cannot readily access the ED, telephone notification may be necessary. A survey of survivors suggested that if delay in personal notification was greater than 1 hour, telephone notification may be appropriate.15 However, it is obviously difficult to be sensitive to the family’s response via a telephone, and there may be limited ability to provide immediate support. Ensure that the family is safe to transport themselves and that ongoing support options have been explored for those family members unable to make it to hospital. If family members were not present at the hospital it is likely that they will have many questions related to the process, potential suffering and any awareness by the child of the event. These may be asked either over the telephone or upon arrival. If parents arrive ‘too late’, this can create a further burden of guilt because they were not present. Family members experiencing significant grief are likely to struggle with the integration of the information that they are being given and with the communication of any questions that they might have. They may need to revisit the same questions and information repeatedly in order to try to make sense of the event.9 It is important to allow parents and family members time to examine the implications of the loss and to begin the process of searching for some answers

and meaning in the midst of the event. It is also important to assist them to mobilise resources from their social, cultural and religious communities to help them to deal with their grief. There can be a temptation to offer sedation to grief-stricken parents. This is often requested by relatives distressed by observing the parents’ pain. Grief is a normal process, which is rarely helped by pharmacological intervention. Junior medical staff are often involved in resuscitations, and it is essential that they have received some training/education to help them handle the unexpected death of a child. A number of programmes have been described, which have been found to be useful in preparing staff to deal with loss in an effective manner, from the perspective of both the family and staff members.16–19

Laying out of the child Where parents want to ‘view’ or spend time with their deceased child, it is important to facilitate their wishes (having due regard for the possibility that the death may need to be referred to the coroner and hence care not to interfere with evidence). All tubes inserted during the resuscitation process (endotracheal tubes, intravenous cannulae, drains, etc.) should be removed, unless the medical officer in charge considers that the placement of a tube may have been associated with an adverse event. All wounds and cannula sites should be dressed to avoid leakage of bodily fluids. The child’s face and exposed areas should be bathed/cleaned and any soiling removed. The impact of the death can often cause an overwhelming sense of numbness and helplessness, diminishing the ability to self-advocate. Therefore it is important to be proactive with family members and ask how much they want to be involved with the bathing and laying out of the child and about any specific cultural or religious practices that they would like observed. It can often be useful to obtain mementos of the child. Photographs, a lock of hair, or a foot/hand print may become important mementos along the grieving journey. It is recommended that hospital EDs have access to such items as a camera, memento books and bereavement packs to give to families. There are specific requirements in place for deaths that must be referred to the coroner. These may limit the process of ‘laying out’ the body and require that family members may not be left unsupervised with the child. ED staff need to balance the needs of grieving family members with their legal responsibilities to the coroner.

Viewing the body – quiet suite Most available evidence strongly suggests that seeing the body of the deceased is an important part of accepting the reality of death.20,21 This includes not only seeing but also being able to touch and hold the loved one. It is helpful to describe to relatives what they are going to see prior to viewing the body, especially if there are trauma-related injuries.9 Viewing the body can also relieve anxieties about mutilation, signs of trauma, or that the person was in pain when he/she died.21 A parent or family member’s preference not to spend time with the child should also be respected. Most large paediatric hospitals have a ‘quiet suite’ or ‘family room’ to facilitate parents spending time with their deceased child. This can allow a private ‘goodbye’ and time to reflect. It can also allow time to create an image of the child as dead, altered from the image of the living child.21 The importance of the family/relatives’ room cannot be overemphasised – privacy and basic facilities are essential. Subsequently, additional relatives/friends may arrive at the hospital. This can often lead to heightened distress for parents as they try to explain the events that have led to the child’s death, hence taking them back to the initial traumatic stages. It can also be a useful process. By reviewing events, parents may build a clearer picture and ‘fill in the blanks’ as they retell their story.

The grief response Grief is a normal reaction accompanying death. The severity of the grief response parallels the severity of the loss. Perhaps the most well-known model of describing the process of grief is the ‘stages’ model with its clearly defined stages of shock, denial and isolation, anger and envy, bargaining, depression and acceptance.22 These stages should not be seen as linear or rigid. Individuals can move back and forth between the stages or may appear ‘stuck’ in a stage. Although the ‘stages’ model is the most well known and can be a useful guide, there are a number of other models of grieving including psychodynamic,23 attachment,24,25 social constructionist,26 cognitive/behavioural,27,28 and personal construct.29 Good practice requires being open and flexible and adapting to the needs of the grieving family as opposed to trying to fit the family into any particular model. It is important not to pathologise individuals whose grief response does not fit neatly into a particular

model of grief.30 The death of a child provokes the most intense form of bereavement. It is certain to alter the course of the parents’ lives and their relationship with each other and with others. Losing a child is more than losing a relationship. For a parent it is losing part of his/her self, his/her present and his/her future. Many parents experience a loss of meaning in their lives and may never fully recover from the impact of their child’s death.25 A child’s death is not a singular loss but produces a ripple effect overwhelming all aspects of the family and environment. Parents, and even the extended family, may feel that they have failed, irrespective of the nature of the death and level of love, nurturing and caring that existed during the child’s life.21,31,32 The parental relationship faces severe stress following the death of a child. It can pull a dysfunctional relationship further apart or glue a functional one closer together. Adverse impacts on the relationship can occur through the real or perceived apportioning of blame by one parent to the other. This can occur where a child died while under the specific supervision of one parent or one parent was simply not present when a critical event occurred. Siblings of the deceased child will also experience a significant grief reaction. Not only must they manage the actual loss of their deceased sibling, but they must also cope with the loss of their normal family environment. Their parents will be struggling to cope with their own grief and thus will be less emotionally available. The cognitive developmental level of a sibling has a significant bearing on his/her capacity to understand concepts of death like permanent, irreversible, inevitable, universal.33 Regardless of how siblings understand and express their grief, it is critically important to remember that they are part of the social context in which the death has occurred. Their needs for explanation and support are just as important as the needs of their parents. The death of a child does not occur in isolation, but rather it occurs in a social context that includes many variables. The main ones are parental coping capacity and skills, family and relationship functionality, social networks, parental physical and mental health issues, education, socioeconomic status and, importantly, any real or perceived parental responsibility in the death of the child. Thus the broader social context will have relevance to how parents and extended family members manage the impact of the child’s death.31 Any available psychosocial assessment or information, such as that provided by the ED social worker, should be factored into the management of the family.

Support of the family Generally, parents are completely unprepared for the impact of their child’s death as they have no prior knowledge or experience to draw on.34 Arranging support is essential, and early social worker involvement is highly desirable. Parents and other family members must be provided with information about ‘normal’ grieving and should be linked to appropriate resources. This can take the form of written information packs that parents can take away and which they may choose to read at a later time.35,36 Referral information should be readily available for support groups with particular expertise relating to the death of a child such as SIDS & Kids,37 SANDS Australia,38 Compassionate Friends,39 and other relevant organisations. The extent of involvement of support by ministers of religion will depend on the wishes of and the religious commitment of the family. Ideally there should be a protocol that facilitates ready access to this material. Practical assistance with arrangements at the time of the child’s death, including organising family support and funeral and financial assistance, should be offered to families as appropriate, while being sensitive to the social and cultural environment of the family.

Cultural implications Many cultures have specific rituals and practices concerning death. It is critical to listen to the family members and be guided as much as possible by their requests. Some of these rituals may require modification when the death of a child has been referred to the coroner’s office. Sensitivity is essential. It is difficult to make broad statements about the cultural practices related to death and dying in indigenous (Aboriginal and Torres Strait Islander) communities, because across Australia there are different practices and rituals. Examples of the kinds of cultural practices and rituals to be aware of include the following: • When a child is dying many families will want any extended family present to be in attendance. • During the grieving process pre- and post-death, loud crying/wailing may need to occur as part of the community’s customs. Privacy in these circumstances is desirable. • Senior female or male figures may wish to take a lead role in mourning

rituals after death has occurred. This can include ceremonial cleansing (washing the child’s body), dressing and handling. • Funeral arrangements may have to be organised by a specific extended family member or a senior member of the community. • ‘House smoking’ (burning leaves in order to prevent the spirit from ‘rising up’) may need to occur relatively quickly after death has occurred. This can mean that the family will want to return to their community quickly. • Many communities forbid the use of the deceased person’s name after death (for up to 1 year). Nicknames or aliases may be used after death has occurred. A family member with the same name as the deceased may use his/her second name during the mourning period. • After a year, there may be some ritual (i.e. tombstone opening) associated with the end of the mourning process, which requires community members to return home even if in hospital themselves. The Maori culture of New Zealand traditionally has family members present with the body from the time of death through interment. This maintains the harmony of the child, assisting the decedent to join his/her ancestors. Family members will want to be part of the ‘laying out’ of the body, washing, dressing, etc. Other practices reflecting different cultural belief systems that may need to be considered include parents needing to remain with the body 24 hours after the death, caring of the body by staff of the same gender as the deceased child, laying the body to face a certain direction (Mecca), special roles for specific religious/spiritual leaders and the burning of incense/candles. When working with families from different cultures following the death of a child, it is important to be guided by custom, ritual, experience and the family’s cultural environment.

Legal issues Each country, state and territory will have subtle variations as to the legal requirements for the documentation and handling of the body of a deceased. An up-to-date protocol should be available to ensure that proper procedures are followed. A life extinct form will need to be completed by one of the attending ED

medical staff. However, it will also be necessary to decide whether or not a death certificate can be completed. If the patient was known to the hospital and the death was expected, the child’s usual physician may be prepared to sign a death certificate. This physician may also discuss with the parents the option of performing a hospital-based autopsy. Usually the death of a child in the ED is not anticipated and hence becomes a coroner’s case. For a coroner’s case, only a life extinct form can be completed, laying out of the body will be restricted to spot cleaning, the local police must be notified, and parents must not be left unsupervised with the body. It is desirable for the family to formally identify the child’s body in the presence of the police. Otherwise identification will have to be performed later and probably at the morgue, a process likely to increase family distress. All medical notes, investigations, observation sheets, etc., should be provided to the police when they depart with the child’s body for the morgue. Full and accurate documentation of all events in the patient’s hospital chart is essential. This should include the date and time of death, the observations that specify that the child is clinically deceased, any relevant history surrounding the circumstances of the child’s death and any relevant conversations held with the parents or family members. There are potentially legal consequences following any death, and the forensic issues need to be considered. For example, child abuse remains an important cause of deaths in infancy.

Organ and tissue donation and collection Organ donation (e.g. heart, lungs, liver, kidneys) requires intact cardiorespiratory function but brain death. Because of the preconditions required by Transplant Acts before brain death can be declared, organ donation discussions are commonly deferred until admission to the intensive care unit.40 Tissue donation (e.g. corneas, heart valves, skin, bone ligaments and tendons) can occur from cadavers, and hence theoretically this issue could arise for children who die in the ED. However, deaths of children in the ED are usually coroner’s cases. For parents faced with the extreme distress of the sudden death of a child and the need for coroner’s case status, it may be potentially too distressing to parents for ED staff to raise the further issue of tissue donation in this setting. This can come a little later at the Forensic Pathology Institute level, when parents have had a little time to regain some degree of composure and hence may be better able to give informed consent. Sometimes donation can be

perceived by families and providers alike as a way to salvage some meaning from an acute, unanticipated, and tragic loss.7 On the rare occasion when the issue of tissue donation is spontaneously brought up by parents in the ED setting, contact with the transplant coordinator can be initiated if there are no potential medical contraindications to tissue donation. Consent by the coroner must be obtained prior to tissue removal. When children die suddenly and unexpectedly there may be merit in considering collecting perimortem samples in order to obtain as much information as possible. This might include urine and blood for metabolic profiling, genetics screening and other possible investigations such as liver of other tissues samples that may contribute to the understanding of cause of death. This will depend on location and is more likely to be valuable in a major centre where appropriate pathology facilities are immediately available.

Debriefing and support for emergency department staff Much of what is written about the family grief reactions applies equally to the ED staff, and due consideration of staff reactions is very important. A healthy approach is to factor the reality of day-to-day exposure of grief and loss into the culture of a busy ED. There is a paucity of literature on the reactions of staff and grief management among ED staff members. Truong et al.41 described the ability of ED providers to normalise the abnormal events as a protective mechanism as ‘routinisation of disaster’.41 Identifying abnormal psychological symptomatology in ED staff (flashbacks, sleep disturbance, bad dreams, absenteeism, detachment, intensified emotions, etc.) and making ongoing psychological counselling available to affected staff are clearly important. Such symptomatology may occur as a result of either a single exposure or cumulative exposures to traumatic situations. It is important for senior ED staff to promote the concept of self-care, to guarantee confidentiality to staff experiencing problems, and to ensure staff are made aware of counselling options available to them should they experience problems.42 Performing an operational debriefing of the resuscitative process with a view to clarifying events for attending staff and identifying areas for potential improvement is essential. Given the fact that it is often challenging to find a time to gather those who wish to participate in a busy ED environment, there are

suggestions that even a simple acknowledgement at the bedside after the death of the patient may be beneficial to staff, given the healing potential of such a closing ritual.41,43 The same cannot be said for psychological debriefing sessions. It has become a popular and widespread practice to conduct single session psychological counselling for personnel attending traumatic critical incidents. ED staff in attendance at an unsuccessful resuscitation fit into this situation. A recent Cochrane Review concluded that single session psychological debriefings have not only failed to reduce the incidence of post-traumatic stress disorder but actually increased the risk of developing it.44 In addition, there was no evidence of reduction in general psychological disturbance, depression or anxiety. This is an area where more research is required.

Collaboration with paediatric palliative care services Studies in children with known life span–limiting conditions report that between 3% and 20% of deaths in that population will occur in the ED.45,46 For many children receiving palliative care, advance care plans are in place, and it can be very helpful for ED staff to have an understanding, in advance, of the hopes, concerns and wishes that the child and family may have expressed.7

The concept of a good death Data from the National Center for Health Statistics for the most recent year completed (2009) in the United States showed that fewer than 2% (48,000) of deaths occur in the population of children younger than 18 years.47 This statistic is strikingly different from a century ago, when 30% of all deaths were in children younger than 5 years. On one side, these data reflect progress in child health but also underscore that child death, unlike parental or spousal death, is no longer an expected part of life.7 The Institute of Medicine report on childhood death provides the following definitions for good and bad deaths: A decent or good death is one that is: free from avoidable distress and suffering for patients, families, and caregivers; in general accord with patients’ and families’ wishes; and reasonably consistent with clinical,

cultural, and ethical standards. A bad death, in turn, is characterized by needless suffering, dishonoring of patient or family wishes or values, and a sense among participants or observers that norms of decency have been offended.48 Aspects of what might constitute a ‘good death’ in the ED are caring for the survivors of the child’s death in a way that affirms their trust and allowing them to understand the events leading up to the death of the child and to say goodbye to their child in whatever way is meaningful to them.7

Conclusion The death of a child has the most profound effect on parents, family and friends. It can also have a profound effect on staff involved in the resuscitation process. It requires the sensitivity and strength of clinical staff to help relatives through this difficult time and to assist in the initiation of a healthy grieving process. A thoughtful and sensitive approach is likely to have profound and positive longterm implications for all those impacted upon by the death of a child.

Controversies 1. There remains some controversy about actively encouraging parents to be in the resuscitation room. 2. There is little evidence to support the widespread practice of mandatory single-session psychological counselling of distressed staff who attended the child. 3. Raising the issue of tissue donation with parents in the emergency department setting is difficult.

Acknowledgements The authors gratefully acknowledge the assistance of the Indigenous Liaison Service, Herston Hospitals Complex, Brisbane. The contributions of Paul Tait, Roger Barkin and Pat Clements as authors in previous edition of the textbook are hereby acknowledged.

References 1. ACEP. Death of a child in the emergency department: a joint statement by the American Academy of Pediatrics and the American College of Emergency Physicians. Ann Emerg Med. 2002;40:409–410. 2. Olsen J.C, Buenefe M.L, Falco W.E. Death in the emergency department. Ann Emerg Med. 1998;31:758–765. 3. Seecharan G.A, Andersen E.M, Norris K, Toce S.S. Parents’ assessment of quality of care and grief following a child’s death. Arch Pediatr Adolesc Med. 2004;158:515–520. 4. McCabe M.E, Hunt E.A, Serwent J.R. Pediatric residents’ clinical and educational experiences with end-of-life care. Pediatrics. 2008;121(4):e731–e737. 5. Levetown M, American Academy of Pediatrics Committee on Bioethics, . Communicating with Children and families: from everyday interactions to skill in conveying distressing information. Pediatrics. 2008;121(5):e1441–e1460. 6. Overly F.L, Sudikoff S.N, Duffy S, Anderson A, Kobayashi L. Three scenarios to teach difficult discussions in pediatric emergency medicine: sudden infant death, child abuse with domestic violence, and medication error. Simul Healthc. 2009;4(2):114–130. 7. O’Malley P, Barata I, Snow S, American Academy of Pediatrics Committee on Pediatric Emergency Medicine, , American College of Emergency Physicians Pediatric Emergency Medicine Committee, , Emergency Nurses Association Pediatric Committee, . Death of a child in the emergency department. Pediatrics. 2014;134(1):e313–e330. 8. Doyle C.J, Post H, Burney R.E, et al. Family participation during resuscitation: an option. Ann Emerg Med. 1987;16(6):673–675. 9. Wright B. Sudden death: intervention skills for the caring professions. New York: Churchill Livingstone; 1996. 10. Mangurten J, Scott S.H, Guzzetta C.E, et al. Effects of family presence during resuscitation and invasive procedures in a pediatric emergency department. J Emerg Nurs. 2006;32(3):225–233. 11. Dudley N, Hansen K, Furnival R, Donalson A, Van Wagenen K, Scaife E. The effect of family presence on the efficiency of pediatric trauma resuscitations. Ann Emerg Med. 2008;53(6):777 e3– 784.e3.

12. Tsai E. Should family members be present during cardiopulmonary resuscitation? N Engl J Med. 2002;346:1019–1021. 13. Scott J.L, Sanford S.M, Strong L, Gable K. Survivor notification of sudden death in the emergency department. Acad Emerg Med. 1995;2:408–409. 14. Stewart A.E. Complicated bereavement and post-traumatic stress disorder following fatal car crashes: recommendations for death notification practice. Death Stud. 1999;23:289–321. 15. Leash R.M. Death notification: practical guidelines for health care professionals. Crit Care Nurs Q. 1996;19:21–34. 16. Schmidt T.A, Norton R.L, Tolle S.W. Sudden death in the ED: educating residents to compassionately inform families. J Emerg Med. 1992;10:643–647. 17. Bagatell R, Meyer R, Derron S, et al. When children die: a seminar series for paediatric residents. Pediatrics. 2002;110:348–353. 18. Swisher L.A, Nieman L.Z, Nilsen G.J, Spivey W.H. Death notification in the emergency department: a survey of residents and attending physician. Ann Emerg Med. 1993;22:1319–1323. 19. Rutkowski A. Death notification in the emergency department. Ann Emerg Med. 2002;40:521–523. 20. Jones W.H, Buttery M. Sudden death. Survivors perceptions of their emergency department experience. J Emerg Nurs. 1981;1:7. 21. Raphael R. The anatomy of bereavement: A handbook for the caring professions. Hutchinson: London; 1984. 22. Kübler-Ross E. On Death and Dying. London: Tavistock; 1970. 23. Freud S. Mourning and melancholia. Standard Edition XIV. London: Hogarth Press; 1917. 24. Bowlby J. Attachment and loss. Vol. 1. Attachment. London: Hogarth Press; 1969. 25. Parkes C.M. Bereavement: studies of grief in adult life. London: Tavistock; 1972. 26. Glick I.O, Weiss R.S, Parkes C.M. The first year of bereavement. New York: John Wiley & Sons; 1974. 27. Attig T. The importance of conceiving of grief as an active process. Death Stud. 1994;15(4):585–647. 28. Worden J.W. Grief counselling and grief therapy: a handbook for the mental health professional. London: Routledge; 1983.

29. Neimeyer R.A, Neimeyer G.J. Advances in personal construct psychology. Science & Technology Books. New York: Jai Press; 1997. 30. Dubin W.R, Sarnoff J.R. Sudden unexpected death: Intervention with the survivors. Ann Emerg Med. 1986;15:54–57. 31. Murray J. Loss as a universal concept: a review of literature to identify common aspects of loss in diverse situations. J Loss Trauma. 2001;6:219–241. 32. Murray J. Children, adolescents and loss. Loss and Grief Unit. Brisbane: University of Queensland; 2002. 33. Murray J. Understanding loss in the lives of children and adolescents: a contribution to the promotion of well being among the young. Aust J Guid Counsel. 2000;10(1):95–109. 34. Heiney S, Hasan L, Price K. Developing and implementing a bereavement program for a children’s hospital. J Pediatr Nurs. 1993;876:385–391. 35. Johnson L, Rincon C, Gober C, Rexin D. The development of a comprehensive bereavement program to assist families experiencing paediatric loss. J Pediatr Nurs. 1993;8:3. 36. Murray J. An ache in their hearts. Brisbane: University of Queensland Press; 1993. 37. SIDS and Kids, www.sidsandkids.org 38. SANDS Australia, www.sands.org.au 39. Compassionate Friends, www.thecompassionatefriends.org.au 40. Rivers E.P, Buse S.M, Bivins B.A, et al. Organ and tissue procurement in the acute care setting: principles and practice, part 1. Ann Emerg Med. 1990;19:78–85. 41. Truog R.D, Christ G, Browning D.M, Meyer E.C. Sudden traumatic death in children: “we did everything, but your child didn’t survive”. JAMA. 2006;295(22):2646–2654. 42. Everly G.S, Flannery R.B, Mitchell J.T. Critical incident stress management: a review of the literature. Aggr Violent Behav. 1999;5(1):23–40. 43. Treadway K. The code. N Engl J Med. 2007;357(13):1273–1275. 44. Rose S, Bisson J, Wessely S. Psychological debriefing for preventing post traumatic stress disorder (PTSD). Cochrane Database Syst Rev. 2002;2 CD000560. 45.

Feudtner C, Christakis D.A, Zimmerman F.J, Muldoon J.H, Neff J.M, Koepsell T.D. C of deaths occurring in children’s hospitals: implications for supportive care services. Pediatrics. 2002;109(5):887–893. 46. Leuthner S.R, Boldt A.M, Kirby R.S. Where infants die: examination of place of death and hospice/home health care options in the state of Wisconsin. J Palliat Med. 2004;7(2):269–277. 47. Kochanek K.D, Kirmeyer S.E, Martin J.A, Strobino D.M, Guyer B. Annual summary of vital statistics: 2009. Pediatrics. 2012;129(2):338–348. 48. Institute of Medicine, . Board on Health Sciences Policy. In: Field M.J, Behrman R.E, eds. When Children Die: Improving Palliative and End-of-life Care for Children and Their Families. Washington, DC: National Academies Press; 2003.

SECTION 20

Analgesia and Sedation OUTLINE 20.1. Analgesia 20.2. Paediatric procedural sedation within the emergency department

20.1

Analgesia Adrian Mark Bonsall

ESSENTIALS 1 Acute pain is one of the most common emergency department presenting problems. 2 Pain-rating scales suitable to the age and development of the child are useful in establishing a child’s level of pain and assessing the adequacy of analgesia. 3 Adopt a multimodal (non-pharmacological, pharmacological) and multidisciplinary (medical staff, play therapists, parents) approach to pain management. 4 Tailor interventions to the individual child. 5 Become familiar with dose, administration and potential complications of a range of analgesics. 6 Combining drugs without detailed knowledge and training risks serious adverse outcomes.

Introduction Analgesia is the relief of the perception of pain without sedation. Pain is a more difficult concept to define precisely or to measure objectively. The International Association for the Study of Pain (IASP) defines pain as an unpleasant sensory and emotional experience, associated with actual or potential tissue damage, or described in terms of such damage.1 It is the significant emotional dimension that creates considerable variability in how a painful

stimulus is experienced and thus how the interactions of physiological, psychological, developmental and situational factors can modify behaviour in both the short and long term. Acute pain in children is one of the most common reasons for presentation to the emergency department (ED).2 In addition to the underlying injury or illness, subsequent medical procedures may also engender pain which is often associated with increased anxiety, avoidance behaviour, systemic symptoms and parental distress. Treating pain may not only relieve acute suffering but also decrease ongoing anxiety and negative memories, facilitate medical investigations and aid cooperation with other non-painful procedures and treatments. The use of analgesia in procedural sedation is discussed in more detail in Chapter 20.2. Pain may be classified in a number of ways, e.g. by severity, cause or pathophysiology.3 A simple classification is shown in Box 20.1.1. Most commonly in the paediatric ED setting it is procedural and acute pain which need to be addressed. Children with painful conditions can be difficult to assess, and their pain is often still underestimated and undertreated. Children often receive less analgesia than adults, and the administration of analgesia varies by age, with the youngest patients at the highest risk of receiving inadequate analgesia.4,5 Box 20.1.1 Pain classification Procedural

Transient while stimulus is applied but before significant tissue damage occurs

Acute

Significant local tissue damage with acute inflammation but normal innervation

Intractable (chronic)

Continuing in the absence of acute inflammation and normal healing

Neuropathic

Associated with peripheral, central or autonomic nerve damage

Children’s pain can be underestimated because assessment requires tools that account for the wide range of children’s developmental stages. Pain is often undermedicated because of fears of oversedation, respiratory depression, addiction and unfamiliarity with use of sedative and analgesic agents in children.5 ED staff should be proficient in the assessment and safe management of pain in children. Early and appropriate analgesia may be best achieved by using a systematic approach with well-developed pain management educational programmes, specific pain assessment and management policies, and benchmarked standards for time-to-analgesia within the ED.

Assessment of pain Assessment of pain should be individualised, continuous, measured and documented. Pain assessment and measurement tools have been developed that are suitable for children of different ages and developmental stages. Accurate assessment requires a detailed pain history and consideration of the complexity of the child’s pain perception and the influence of situational, psychological and developmental factors. Four useful means of recognising pain in children are outlined in Box 20.1.2. Box 20.1.2 Recognition of pain in children 1. The child’s self-report of pain 2. Behavioural changes, e.g. crying, guarding, facial grimacing 3. Physiological changes, e.g. pallor, tachycardia, and tachypnoea 4. Pathophysiological process, e.g. fracture or burn Because of its subjective nature, pain is best assessed using the child’s selfreport. Observational assessment scoring may be useful when the child is too young or self-report is not possible, e.g. children with cognitive impairment. Pain ratings provided by parents or regular carers may also be used.6 However, whilst there is good correlation between the child’s and the parent’s assessment of pain intensity, parents tend to underscore more severe pain being experienced by their children.7 Physiological measures (e.g. heart rate and respiratory rate) may be useful in pain assessment in non-verbal or sedated children but may be confounded by stress reactions. For example, the child who is febrile or an infant who is hungry may give inappropriately high scores. Specific pain assessment tools employing behavioural and vital sign observations have been developed for neonates (e.g. CRIES) and non-verbal (e.g. FLACC) or cognitively impaired children (e.g. r-FLACC, NCCPC-R or COMFORT tools).8–12 Pain scales for older children able to self-report, such as the Faces rating scale, which uses facial expressions depicting increasing pain or simple numerical or analogue scales, are commonly employed. Some of these age-dependent pain rating scales are outlined in Table 20.1.1.

Management

Optimal analgesia is achieved by the combination of both non-pharmacological and pharmacological strategies with regular pain assessments. When using drugs there should also be careful consideration of the most appropriate route and dosage and close monitoring for adverse effects. Pain management strategies should be individualised for the child’s level of pain and the anticipated discomfort of any procedure to be undertaken. Choice of agent will also depend on local resources, familiarity with doses, duration of action, adverse effects and contraindications.

Non-pharmacological methods There are many non-pharmacological techniques that can be used to mitigate pain and distress in the ED and complement the use of pharmacological methods.13–17 Some of these are listed in Box 20.1.3. Providing a non-threatening, friendly environment, which might include wall decorations of animals or familiar cartoon/TV characters, can help reduce anxiety. Positioning of the child can be important. For example, babies may be more settled when swaddled; conversely, constraint in toddlers can be distressing, so allow a child to take up the most comfortable position and provide pillows to support an injured limb. Other physical techniques include slings, splints or other immobilisation methods and application of ice or cold/heat packs. Gentle, unhurried and confident handling can often minimise distress and pain. Older children may respond to age-appropriate explanations and providing realistic expectations on the management of their pain. It is helpful to gain a rapport and to be honest when reassuring. Claims of the child feeling no pain should be avoided as they are often soon disproved and risk loss of confidence or cooperation from the child and parents. Parents play an important role as a familiar comforter in unfamiliar surroundings. They can be included in many distraction techniques. However, overt parental anxiety can fuel a child’s discomfort. Play therapists are a good resource if available.18 They are trained in many distraction strategies and can also be very useful in preparing a child for a painful procedure. Specific distraction strategies, which can be provided by staff or parents, may include toys, bubbles, guided imagery, music, TV and DVD/video players, hand-held game consoles, and smart phones or tablets. In infants, breast-feeding or the use of a pacifier may be comforting. Other

methods, more commonly used in the chronic setting, include reinforcement of coping behaviours, hypnosis, biofeedback, muscle relaxation and deep-breathing techniques.

Pharmacological methods The choice of agent is dependent on acuity, severity and source of the pain, route availability, expected duration and patient factors such as age or genetic variation. Some of the more commonly used agents discussed below have doses detailed in Table 20.1.2.

Oral analgesic agents Apart from sucrose, these agents may also be administered via a naso- or orogastric tube and a gastrostomy tube if in a suitable liquid or crushed tablet form.

Oral paracetamol and NSAIDs Paracetamol is usually the first line of therapy for mild to moderate painful conditions. It can also be given rectally and intravenously (IV). The rectal route is contraindicated in severely immunocompromised children. IV paracetamol is available when oral and rectal dosing is contraindicated and is dosed on lean body weight. Paracetamol may be synergistic with other analgesics.19 It is generally safe; however, hepatotoxicity is possible in overdose or extended use. Non-steroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen, are usually second-line therapy and have excellent analgesic and anti-inflammatory properties, which may enhance the effects of paracetamol. NSAIDs should be avoided if there is GI ulceration, coagulopathy or active bleeding, severe asthma, renal disease and in some orthopaedic conditions where there is a high risk that bone healing may be compromised. Both drugs should be used with caution where there is significant dehydration or liver dysfunction. In addition aspirin is not recommended as an analgesic for children due to the reported association with Reye syndrome in the context of some viral infections.20

Oral sucrose Oral sucrose (24–25%) has been shown to be a simple, safe and effective means

of providing analgesia for neonates and young infants for short painful events (e.g. heel prick, venepuncture, lumbar puncture).21–23 It stimulates endogenous opioid and non-opioid pathways in the brain and thus may be less effective if the baby is already on an opioid. It may be administered via oral syringe or on a pacifier approximately 2 minutes prior to the painful event.

Codeine and oxycodone Oral codeine has traditionally been used for moderate to severe pain and can be found in combination formulations with paracetamol; however, it is a prodrug with little inherent pharmacological activity and must first be metabolised by the liver into morphine. There is substantial genetic variability in the activity of the cytochrome P450 hepatic enzyme (CYP2D6) responsible for this conversion. Non-metabolisers will have no analgesic benefit, while ultrarapid metabolisers run the risk of significant respiratory suppression especially if there is any obstructive sleep apnoea.24 Oxycodone is a semisynthetic opioid that does not require metabolism to be active. Evidence from recent work shows that oral oxycodone produces greater pain relief compared with codeine and also has a better side effect profile with less itching, less nausea, and fewer allergic reactions.25 The majority is processed in the liver by CYP3A4 to less active compounds, but a small proportion is also metabolised by CYP2D6 to the active oxymorphone which means ultrarapid metabolisers still may be at some risk. A slow release formulation of oxycodone may be used for background analgesia in more chronic pain. Table 20.1.1 Pain scoring CRIES pain rating scale8 The CRIES scale can be used in neonates and young infants up to a few months of age. It was originally designed for post-operative pain assessment; however, it can be employed for other procedures where baseline vital signs are known. Each of the 5 categories is scored from 0 to 2, and the scores are added to get a total score from 0 to 10.

FLACC scale The FLACC scale is a behavioural scale for scoring pain in children between the ages of 2 months and 7 years or in

persons unable to communicate. Each of the 5 categories is scored from 0 to 2, and the scores are added to get a total score from 0 to 10.

Faces rating scales (FRS) These scales can be used with young children (as young as 4 years of age). They also work well for older children and adolescents, including those who speak a different language. Ask the patient to choose the face that best describes how he/she feels. The far left face indicates ‘no hurt’, and the far right face indicates ‘hurts worst’.

The Faces Pain Scale – Revised (FPS-R) can be downloaded (including instructions in multiple languages) from The International Association for the Study of Pain (IASP) website at www.iasp-pain.org/Education/Content.aspx? ItemNumber=1519&navItemNumber=577.

Numerical rating score (NRS) This tool may be used for children over the age of 6–8 years. Instruct the patient to rate his/her pain intensity on a scale of 0 (‘no pain’) to 10 (‘the worst pain imaginable’).

BP, blood pressure; HR, heart rate; Sat, saturation.

Tramadol Tramadol is a synthetic analogue of codeine with high oral bioavailability. It compares favourably with oral NSAIDs for pain and may be used if opioids are contraindicated, ineffective or causing side effects.26 It is a weak central μ-opioid

agonist (30% of effect) and also inhibits noradrenaline (norepinephrine) and serotonin reuptake (70% of effect). Thus it should be used with caution with other opioids and avoided if the patient has been on serotonin re-uptake inhibitors, tricyclic antidepressants, major tranquillisers, fentanyl, pethidine or monoamine oxidase inhibitors as seizures or a serotonin syndrome can result. The most common side effects are nausea, vomiting and dizziness, however it has less sedative, respiratory depressive and pruritic effects than morphine. It can also be given by slow infusion IV over 15–20 minutes to reduce the incidence of nausea and vomiting. A ceiling effect limits tramadol usefulness to moderate pain. Box 20.1.3 Supportive and distractive techniques

Environment • Calm friendly non-clinical atmosphere • Toys, mobiles, pictures and videos

Psychological • Parental presence • Age-appropriate communication • Clear confident instructions

Cognitive–behavioural • Distraction techniques • Hypnosis and biofeedback • Art/stories • Music/video/TV • Interactive computer games • Guided imagery • Muscle relaxation and deep-breathing techniques • Reinforcement of coping behaviours

Physical • Massage/rubbing • Comfort swaddling (infants) • Heat/cold techniques • Immobilisation and elevation (injured part)

Breast-feeding • Comforter (favourite blanket/soft toy)

Oral morphine Morphine is an effective analgesic and widely used in parenteral formulations. However, it has a low oral bioavailability (30–40%), and with the advent of other synthetic opioids with higher non-parenteral route bioavailabilities and better side effect profiles, oral morphine is not often used in acute pain management in the ED.

Intranasal or inhaled options Methoxyflurane Methoxyflurane is a halogenated ether. Its rapid action, portability and ease of administration mean that it is most useful in the acute, pre-hospital setting, when alternatives are limited or impractical.27 It is particularly effective for trauma pain but may also be used for brief painful procedures such as wound and burns dressings. A recent review has shown that the inhalational agent methoxyflurane is also safe and effective in the ED setting.28 The commonly used ‘Penthrox’ inhaler is now available with an activated charcoal scavenging chamber to reduce environmental contamination.

Intranasal fentanyl Intranasal fentanyl using a mucosal atomiser device provides safe and effective analgesia equivalent to parenteral morphine in children as young as 1 year of age.29–32 It offers quick onset and is less invasive, and its duration of action,

although only 30–60 minutes, allows time for topical anaesthetic application prior to IV cannulation for ongoing analgesia. It is particularly useful for fractures or burns dressings, but its utilisation is spreading into other areas.

Nitrous oxide Nitrous oxide mixed with oxygen has a potent analgesic action with rapid onset and offset. It is an excellent analgesic sedative for relatively brief painful procedures such as gaining rapid IV access, injecting local anaesthetics (LAs), performing a nerve block, laceration suturing or splinting a fractured limb.33–35 Entonox® (50% nitrous oxide, 50% oxygen) is usually delivered via a demand-valve system. This limits its use in younger or uncooperative children. Machines that deliver variable concentration (30–70%) nitrous oxide via a continuous flow system allow the use of this agent down to age 1 year, where it has been shown to be safe when embedded in a comprehensive sedation programme.35 Nitrous oxide alone is effective in achieving moderate levels of procedural sedation for a high percentage of children with painful conditions but may require the use of adjunctive analgesics for very painful procedures.35,36 Oxygen should be administered for 3–5 minutes after cessation of nitrous oxide to prevent diffusion hypoxia. Nitrous oxide is contraindicated in conditions involving closed air spaces (e.g. pneumothorax, bowel obstruction). The most common adverse effect is vomiting, and a fasting time of at least 2 hours is advised. Prolonged exposure has been associated with alterations in vitamin B12 and folate metabolism, megaloblastic bone marrow changes and subacute combined degeneration of the cord.

Parenteral analgesics Although these may be any systemic analgesics given by a route other than the gastrointestinal tract, the common parenteral routes are IV, intramuscular (IM) and subcutaneous (SC). The IV route is often favoured for analgesia because it generally has faster onset and better efficacy than the IM and SC routes which can have variable absorption depending on perfusion. However, the IV route does require the delay and discomfort of cannulation, but that may be preferred to multiple IM injections if the need for parenteral pain relief is ongoing. Continuous IV (or occasionally SC) infusion may be used for ongoing analgesia requirements.

Patient-controlled analgesia (PCA) and nurse-controlled analgesia (NCA) If a patient needs regular strong analgesia (most commonly opioids), a special IV pump may be set up to deliver bolus doses within pre-set dose and frequency limits to allow older, competent children to self-manage pain relief by pressing a button.37 In children not able to use a PCA machine, NCA may be used by nurses with close monitoring and serial assessments.38

Parenteral opioid analgesics Opioid analgesics are the mainstay of treatment for severe pain. Opioids are ideally administered IV in a dilute solution and titrated against response. Important side effects include respiratory depression, drowsiness, bradycardia, constipation, nausea and vomiting, and, in some, histamine release. Common choices would include morphine and fentanyl. The latter is a potent rapid-acting analgesic with a shorter duration of action. As fentanyl is metabolised in the liver to inactive metabolites, it is preferred to morphine (which is mainly excreted in the urine) in kidney impairment. Ex-premature infants and young infants (under 3 months) may require opioid dose reduction as they are at increased risk of apnoea and other side effects because they metabolise opioids more slowly.39 In addition there is concern that early prolonged exposure to opioids may affect neurodevelopment of a number of brain systems.40

Ketamine Ketamine is a phencyclidine derivative with NMDA antagonist and opioid receptor agonist properties. It is an analgesic dissociative anaesthetic that is an increasingly popular choice for procedural sedation as its action produces a trance-like state of sedation, amnesia, analgesia and some motion control with few side effects. This is discussed in greater detail in Chapter 20.2. Subdissociative doses of ketamine are not yet commonly used in ED for analgesia alone as the evidence base for use in children is limited. However, there are some studies that suggest equivalent efficacy to opioids.41 Multiple routes of administration are available. IV and IM routes are commonly used, but there is growing evidence that intranasal ketamine may be comparable to intranasal fentanyl for pain associated with limb injuries.42 The oral route is another route that is available but not currently employed regularly in the paediatric ED.

Local anaesthetic agents The expanded use of LAs has revolutionised the management of simple lacerations in the ED and has also greatly improved conditions for IV cannulation and lumbar puncture. These agents provide a non-invasive means of producing local anaesthesia and can be applied at triage to facilitate timely management in the ED.43 In addition LA use in regional anaesthesia can provide timely pain relief and reduce the need for general anaesthesia in managing some fractures. However, clinicians must be mindful of the risk of overdose. LA toxicity may be heralded by dizziness, peri-oral tingling, metallic taste, altered mental status, arrhythmias and seizures and result in refractive hypotension and cardiac arrest. Methaemoglobinaemia can also complicate benzocaine, prilocaine and lidocaine administration.44 Table 20.1.2

GI, gastrointestinal; LA, local anaesthetic; IM, intramuscular; IN, intranasal; IV, intravenous; PO, per orem; PR, per rectum.

Topical anaesthetic agents EMLA® (a eutectic mixture of two LAs: 2.5% lidocaine [lignocaine] and 2.5% prilocaine) is a well-established topical anaesthetic for use on intact skin prior to venepuncture, intravenous cannulation or lumbar puncture. Its use in the ED is, however, limited, due to its long onset to peak effect (at least 1 hour) and its vasoconstrictive effect, which may make cannulation more difficult. EMLA® has a theoretical risk of methaemoglobinaemia and is not recommended in infants less than 3 months of age.45 Amethocaine, also known as tetracaine, can also be used for similar skinpuncturing procedures (e.g. Ametop® or AnGel® both contain 4% amethocaine).

It has a quicker onset of action (30–45 minutes) than EMLA, and its vasodilating effect may facilitate cannulation. Side effects reported include itching and erythema. Amethocaine has been shown to be superior to EMLA® in terms of lessening pain associated with IV cannulation and is more effective than EMLA® when application time is less than 60 minutes.46 There is also an ophthalmic formulation (0.5–1%) for eye use. Topical wound anaesthetics, e.g. Laceraine® (ALA: adrenaline [epinephrine] 1:1000, lidocaine 4%, amethocaine 0.5%), permit wound management with minimal to no discomfort.47,48 It should be placed on a small wad of gauze/cotton wool inside the wound and covered with a watertight dressing for 20–30 minutes to provide sufficient anaesthesia for suturing 75–90% of scalp and facial lacerations and 40–60% of extremity wounds.48,49 Its vasoconstrictive effect is also useful prior to application of cyanoacrylate tissue adhesive. Cocaine-containing topical wound anaesthetics (e.g. TAC – tetracaine, adrenaline, cocaine), though effective, are more expensive, require secure storage and potentially risk the serious systemic side effects that have been reported with cocaine-containing preparations.50–52 Topical preparations for mucous membranes are also available. For example, xylocaine viscous, a formulation of lidocaine, has been used to encourage fluid intake when applied to painful lesions in the oropharynx, though recently this efficacy has been challenged.53 Similarly pain from oral cavity ulcers or teething may respond to teething gels. Some of these contain choline salicylate, which has led to salicylate toxicity or Reye-like syndrome in several cases of excessive usage.54–57

Local anaesthetic infiltration The pain associated with wound infiltration using an injectable LA can be lessened by buffering (e.g. add 1 mL 8.4% sodium bicarbonate to 9 mL 1% lidocaine), warming the solution to body temperature, using fine-bore needles and injecting slowly through the wound edges rather than through intact skin.58–62 In addition, use of other analgesia, e.g. nitrous oxide or distraction techniques, during the process of injection often mitigates the pain of instillation. Adrenaline can be added to prolong the local effect of the anaesthetic and reduce bleeding. Traditionally adrenaline is not used where there are end arteries, such as the tips of the digits, pinnae, nose and penis. Although more recent evidence suggests 1:100,000 adrenaline appears to be safer than first thought, it may be prudent to avoid it in these situations in the ED until more evidence in children is

available.63–65 It is important to consider safe dosage regimes of local anaesthetics, with and without adrenaline, and include any use of topical anaesthetic in the total dose.

Regional anaesthetic techniques Regional nerve blocks may be used for either pain relief (e.g. femoral nerve or fascia iliaca blocks for femur fracture) or to facilitate foreign body removal, suturing, fracture or dislocation reduction (e.g. auricular, digital or metacarpal blocks).45,66 Nerve blocks have traditionally been performed using ‘blind’ techniques based on anatomical landmarks with or without a nerve stimulator. The increasing availability and expertise of ultrasound in the ED has the potential to achieve equivalent or better success rates with fewer adverse events.67

Femoral blocks Femoral blocks have been shown to be effective and reduce the need for opioid analgesia with femoral shaft fractures.68,69 There are a number of methods available for femoral blocks. Femoral nerve block (FNB) with ultrasound guidance or fascia iliaca compartment block (FICB) with or without ultrasound guidance is probably the best and safest. Use of a nerve stimulator or ‘blind’ approach for an FNB is less ideal as there is potential for painful muscle contraction and inadvertent intravascular or intraneural injection. The FICB is technically easier and, because it is performed away from the femoral vessels, can be safely performed without ultrasound guidance. In addition to the femoral nerve, it also blocks the lateral femoral cutaneous nerve and the obturator nerve more reliably than the FNB.70 It is a field block that relies on volume to maximise its effect. With the maximum dose limitation, the concentration of the LA may have to be reduced in order to maintain an effective volume. Outlines for these femoral block procedures are presented in Box 20.1.4 and suggested LA doses given in Table 20.1.2.

Bier block This is an example of IV regional anaesthesia. It is most commonly used for cooperative older children with distal forearm fractures that require closed reduction in the ED. If appropriate local anaesthetics and LA doses are chosen,

Bier blocks are safe, effective and avoid the need for sedation or a general anaesthetic in these patients.71–73 In order to be carried out safely, the procedure requires at least two trained doctors, a nurse, a blood pressure tourniquet capable of maintaining a pressure of at least 200 mmHg, availability of resuscitation equipment and full non-invasive monitoring. It is contraindicated if the patient has an open fracture, compromised circulation or infection of the limb, pathologic hypertension, or a bleeding disorder. A brief outline for the procedure is presented in Box 20.1.5 and suggested LA doses given in Table 20.1.2.

Chronic pain The usual focus in the ED is acute pain, but patients with chronic pain will also present because of their chronic condition, complications of their analgesics or an unrelated painful issue. Chronic pain may be treated in a number of ways to provide ongoing background analgesia with provision for breakthrough pain. The non-pharmacological techniques and analgesics used can be some of those described above, but drugs may be time-released formulations or delivered in different ways, e.g. fentanyl transdermal patches. Examples of other drugs used include clonidine, amitriptyline, tapentadol, gabapentin and pregabalin. Advice from a chronic pain specialist can be invaluable with these patients who often have pain management plans tailored to the individual. Box 20.1.4 Femoral nerve blocks

General • Ensure the patient has no contraindications – allergy to local anaesthesia (LA), local infection or bleeding disorder. • Examine and document pre-block neurological function of limb. • Use 0.5 mL 1% lidocaine to anaesthetise the injection site. • Identify inguinal ligament as a line between the ipsilateral anterior superior iliac spine and pubic tubercule. • Use short-bevelled ‘blunt’ needles for block injection attached to a 20 mL syringe. • Use bupivacaine 0.25–0.5% or ropivacaine 0.5% without adrenaline for

the block (see Table 20.1.2 for doses). • When correctly positioned: aspirate, inject slowly and re-aspirate every 3– 5 mL to check not in a vessel. • Should be able to inject without significant resistance, and should not elicit paraesthesia or significant pain. • Block may take 20–30 minutes to become effective. It can last 4–8 hours depending on the LA used.

Fascia iliaca compartment block • Injection point is ∼1 cm caudal to the junction of the lateral and middle thirds of inguinal ligament. • Insert block needle perpendicularly to skin, then angle cranially and advance slowly until 2 distinct ‘pops’ are felt as the needle traverses the fascia lata and then the fascia iliaca. • Apply gentle pressure just caudal to injection site to encourage LA to flow cranially. • If resistance is felt, may be in muscle; retract needle slightly. If still have resistance, pull back to skin and re-advance. • If an ultrasound is available it can also be used to confirm correct placement.

Femoral nerve block with ultrasound guidance • Palpate the femoral pulse ∼1 cm caudal to the inguinal ligament. • Using a transverse orientation of the ultrasound probe, identify and centre femoral vessels and nerve on ultrasound probe display. The femoral nerve is lateral to the pulsating artery, which itself is just lateral to the compressible vein. • In-plane approach of block needle from lateral end of probe. • Position needle tip just lateral to nerve. • When injecting should see LA flood around femoral nerve.

Discharge analgesia Lack of appropriate or inadequate dosing of discharge analgesia is an ongoing problem. Pain experienced at home after acute injury or procedure in the ED can place considerable extra burden on family physicians for pain-related issues. It is essential to include adequate discharge analgesia in ED pain management guidelines. Ibuprofen was found to be preferable to paracetamol and codeine for outpatient management for children with uncomplicated arm fractures.74 Box 20.1.5 Upper limb Bier block • Place a cannula distal to the fracture. • Insert a second cannula in the contralateral arm in case additional analgesia to tolerate the tourniquet is required or in the rare event of local anaesthetic (LA) toxicity. • Ensure a radiographer is immediately available, and prepare materials for applying a plaster back slab. • Elevate the affected arm for 1–2 minutes with compression of the brachial artery. • Inflate an upper arm blood pressure cuff to ∼200 mmHg, and maintain the pressure for the duration of the procedure. • Inject LA through the ipsilateral cannula (see Table 20.1.2 for doses) and the cannula removed. • Once anaesthesia is achieved (usually 5–10 minutes), reduce the fracture, apply a back slab and have a portable X-ray taken to confirm adequate reduction. • The cuff may only be deflated slowly in stages after a minimum of 20–25 minutes. • Observe for LA toxicity.

Controversies and future directions 1. The employment in the emergency department (ED) of drugs and techniques previously the province of anaesthetists and pain specialists

has been controversial in the past, e.g. ketamine. However, improvements in training programmes and experience have led to increasing acceptance of their routine use. 2. Use of quality-improvement processes and high-quality research will boost safety and efficient use of these analgesics and engender other novel agents and non-pharmacological techniques to improve the experience of children presenting to the ED with painful conditions. 3. Alternative routes of drug administration have already had a great impact on paediatric analgesia practices. The delivery of established drugs by innovative, less-invasive methods is an exciting area for future research. 4. Consensus-based recommendations for standardising the terminology used for reporting adverse will help to create a uniform reporting mechanism for future studies in this area.

References 1. Merskey H, Bogduk N. IASP task force on taxonomy. Classification of chronic pain. In: Merskey H, Bogduk N, eds. Part III: Pain Terms, a Current List with Definitions and Notes on Usage. Seattle: IASP Press; 1994:209–214. 2. Maurice S.C, O’Donnell J.J, Beattie T.F. Emergency analgesia in the pediatric population. Part 1, Current practice and perspectives. J Emerg Med. 2002;19:4–7. 3. WHO. Guidelines on the Pharmacological Treatment of Persisting Pain in Children with Medical Illnesses. Geneva: World Health Organization; 2012:1 Classification of pain in children. https://www.ncbi.nlm.nih.gov/books/NBK138356/. 4. American Academy of Pediatrics. Committee on Psychosocial Aspects of Child and Family Health; Task Force on Pain in Infants, Children and Adolescents. The assessment and management of acute pain in infants, children and adolescents. Pediatrics. 2001;108:793–797. 5. Zempsky W.T, Cravero J.P. Committee on Pediatric Emergency Medicine, and Section on Anesthesiology and Pain Medicine. Relief of pain and anxiety in pediatric patients in emergency medical systems. Pediatrics. 2004;114:1348–1356.

6. Wilson G.A, Doyle E. Validation of three paediatric pain scores for use by parents. Anaesthesia. 1996;51:1005–1007. 7. Kelly A.M, Powell C.V, Williams A. Parent visual analogue scale ratings of children’s pain do not reliably reflect pain reported by child. Pediatr Emerg Care. 2002;18:159–162. 8. Krechel S.W, Bildner J. CRIES: a new neonatal postoperative pain measurement score. Initial testing of validity and reliability. Paediatr Anaesthesia. 1995;5:53–61. 9. Merkel S.I, Voepel-Lewis T, Shayevitz J.R, Malviya S. The FLACC: a behavioral scale for scoring postoperative pain in young children. Pediatr Nurs. 1997;23(3):293–297. 10. Malviya S, Voepel-Lewis T, Burke C, Merkel S, Tait A.R. The revised FLACC observational pain tool: improved reliability and validity for pain assessment in children with cognitive impairment. Paediatr Anaesth. 2006;16(3):258–265. 11. Breau L.M, McGrath P.J, Camfield C.S, Finley G.A. Psychometric properties of the non-communicating children’s pain checklistrevised. Pain. 2002 Sep;99(1-2):349–357. 12. van Dijk M, Peters J.W, van Deventer P, Tibboel D. The COMFORT Behavior Scale: a tool for assessing pain and sedation in infants. Am J Nurs. 2005 Jan;105(1):33–36. 13. Pillai Riddell R.R, Racine N.M, Gennis H.G, et al. Non-pharmacological management of infant and young child procedural pain. Cochrane Database Syst Rev. 2015;12 CD006275. 14. Sinha M, Christopher N.C, Fenn R, Reeves L. Evaluation of nonpharmacologic methods of pain and anxiety management for laceration repair in the pediatric emergency department. Pediatrics. 2006 Apr;117(4):1162–1168. 15. The Royal Australasian College of Physicians, P.C.H. Division. Guideline Statement: Management of Procedure-related Pain in Children and Adolescents. Sydney: The Royal Australasian College of Physicians; 2005. 16. Wente S.J.K. Nonpharmacologic pediatric pain management in emergency departments: a systematic review of the literature. J Emerg Nurs. 2013;39(2):140. 17. Koller D, Goldman R.D. Distraction techniques for children undergoing procedures: a critical review of pediatric research. J Pediatr

Nurs. 2012;27:652–681. 18. Bratton S.C, Ray D, Rhine T, Jones L. The efficacy of play therapy with children: a meta-analytic review of treatment outcomes. Prof Psychol Res Pr. 2005;36(4):376–390. 19. Miranda H.F, Puig M.M, Prieto J.C, Pinardi G. Synergism between paracetamol and nonsteroidal anti-inflammatory drugs in experimental acute pain. Pain. 2006 Mar;121(1-2):22–28. 20. Pugliese A, Beltramo T, Torre D. Reye’s and Reye’s-like syndromes. Cell Biochem Funct. 2008 Oct;26(7):741–746. 21. Stevens B, Yamada J, Ohlsson A. Sucrose for analgesia in newborn infants undergoing painful procedures. Cochrane Database Syst Rev 4:CD001069. 22. Harrison D, Beggs S, Stevens B. Sucrose for procedural pain management in infants. Pediatrics. 2012;130(5):918–925. 23. Carbajal R, Verapen S, Coudere S. Analgesic effect of breast feeding In term neonates: randomised controlled trial. Br Med J. 2003;326:13. 24. Tobias J.D, Green T.P, Coté C.J. AAP section on anesthesiology and pain medicine, AAP committee on drugs. Codeine: Time To Say “No”. Pediatrics. 2016;138(4):e20162396. 25. Charney R.L, Yan Y, Schootman M, et al. Oxycodone versus codeine for children with suspected forearm fracture: a randomized controlled trial. Pediatr Emerg Care. 2008;9:595–600. 26. Courtney M.J, Cabraal D. Tramadol vs diclofenac for post-tonsillectomy analgesia. Arch Otolaryngol Head Neck Surg. 2001;127:385–388. 27. Bendall J.C, Simpson P.M, Middleton P.M. Prehospital analgesia in New South Wales, Australia. Prehosp. Disaster Med. 2011;26:422–426. 28. Grindley J, Babl F.E. Review article: efficacy and safety of methoxyflurane analgesia in the emergency department and prehospital setting. Emerg Med Australas. 2009;2:4–11. 29. Cole J, Shepherd M, Young P. Intranasal fentanyl in 1-3-year-olds: a prospective study of the effectiveness of intranasal fentanyl as acute analgesia. Emerg Med Australas. 2009;21:395–400. 30. Borland M, Jacobs I, King B, O’Brien D. A randomized controlled trial comparing intranasal fentanyl to intravenous morphine for managing acute pain in children in the emergency department. Ann Emerg Med. 2007;49:335–340. 31. Borland M.L, Bergesio R, Pascoe E.M, et al. Intranasal fentanyl is an

equivalent analgesic to oral morphine in paediatric burns patients for dressing changes: a randomised double blind crossover study. Burns. 2005;31:831–837. 32. Borland M.L, Jacobs I, Geelhoed G. Intranasal fentanyl reduces acute pain in children in the emergency department: a safety and efficacy study. Emerg Med Australas. 2002;14:275–280. 33. Kanagasundaram S.A, Lane L.J, Cavalletto B.P, et al. Efficacy and safety of nitrous oxide in alleviating pain and anxiety during painful procedures. Arch Dis Child. 2001;84:492–495. 34. Gall O, Annequin D, Benoit G, et al. Adverse events of premixed nitrous oxide and oxygen for procedural sedation in children. Lancet. 2001;358:1514–1515. 35. Babl F.E, Oakley E, Seaman C, et al. High-concentration nitrous oxide for procedural sedation in children: adverse events and depth of sedation. Pediatrics. 2008;121:528–532. 36. Babl F.E, Oakley E, Puspitadewi A, et al. Limited analgesic efficacy of nitrous oxide for painful procedures in children. Emerg Med J. 2008;25:717–721. 37. Bender L.H, Weaver K, Edwards K. Postoperative patient-controlled analgesia in children. Pediatr Nurs. 1990;16:549–554. 38. Monitto C.L, Greenberg R.S, Kost-Byerly S, et al. The safety and efficacy of parent-/nurse-controlled analgesia in patients less than six years of age. Anesth Analg. 2000;91:573–579. 39. Simons S.H.P, Anand K.J.S. Pain control: Opioid dosing, population kinetics and side-effects. Seminars in Fetal and Neonatal Medicine. 2006 August;11(4):260–267. 40. McPherson C. Morphine exposure in preterm infants correlates with impaired cerebellar growth and poorer neurodevelopmental outcome. Evid Based Med. 2016 Dec;21(6):234. 41. Sin B, Ternas T, Motov S.M. The use of subdissociative-dose ketamine for acute pain in the emergency department. Acad Emerg Med. 2015 Mar;22(3):251–257. 42. Yeaman F, Oakley E, Meek R, Graudins A. Sub-dissociative dose intranasal ketamine for limb injury pain in children in the emergency department: a pilot study. Emerg Med Australas. 2013;25:161–167. 43. Priestley S.J, Kelly A.M, Chow L, et al. Application of topical local anesthetic at triage reduces treatment time for children with laceration: a

randomized controlled trial. Ann Emerg Med. 2003;42:34–40. 44. Guay J. Methemoglobinemia related to local anesthetics: a summary of 242 episodes. Anesth Analg. 2009 Mar;108(3):837–845. 45. Barnett P. Alternatives to sedation for painful procedures. Pediatr Emerg Care. 2009;25:415–419. 46. Lander J.A, Welman B.J, So S.S. EMLA and amethocaine for reduction of children’s pain associated with needle insertion. Cochrane Database Syst Rev. 2006;3 CD004236. 47. Dart C. Comparison of lignocaine 1% injection and adrenaline-cocaine gel for local anaesthesia in repair of lacerations. Emerg Med Australas. 1998;10:38–44. 48. Schilling C.G, Bank D.E, Borchert B.A, et al. Tetracaine, epinephrine (adrenalin), and cocaine (TAC) versus lidocaine, epinephrine and tetracaine (LET) for anesthesia of laceration in children. Ann Emerg Med. 1995;2(5):203–208. 49. Ernst A.A, Marvez E, Nick T.G, et al. Lidocaine adrenaline tetracaine gel versus tetracaine adrenaline cocaine gel for topical anesthesia in linear scalp and facial laceration in children aged 5 to 17 years. Pediatrics. 1995;95:255–258. 50. Eidelman A, Weiss J.M, Enu I.K, et al. Comparative efficacy and costs of various topical anaesthetics for repair of dermal lacerations: a systematic review of randomized, controlled trials. J Clin Anesth. 2005;17:106–116. 51. Barnett P. Cocaine toxicity following dermal application of adrenalinecocaine preparation. Pediatr Emerg Care. 1998;14:280–281. 52. Daya M.R, Burton B.T, Schleiss M.R, et al. Recurrent seizures following mucosal application of TAC. Ann Emerg Med. 1988;17:646–648. 53. Hopper S.M, McCarthy M, Tancharoen C, Lee K.J, Davidson A, Babl F.E. Topical lidocaine to improve oral intake in children with painful infectious mouth ulcers: a blinded, randomized, placebo-controlled trial. Ann Emerg Med. 2014 Mar;63(3):292–299. 54. Williams G.D, Kirk E.P, Wilson C.J, Meadows C.A, Chan B.S. Salicylate intoxication from teething gel in infancy. Med J Aust. 2011 Feb 7;194(3):146–148. 55. Oman T.K, Stewart M.C, Burns A, Lang T.F. Topical choline salicylates

implicated in Reye’s syndrome. BMJ. 2008;336:1376. 56. Paynter A.S, Alexander F.W. Salicylate intoxication caused by teething ointment. Lancet. 1979;2:1132. 57. Sarll D.W, Duxbury A.J. Choline salicylates and Reye’s syndrome. Br Dent J. 1986;161:317–318. 58. Mader T.J, Playe S.J, Garb J.L. Reducing the pain of local anesthetic infiltration: warming and buffering have a synergistic effect. Ann Emerg Med. 1994;23:550–554. 59. Bartfield J.M, Gennis P, Barbera J, et al. Buffered versus plain lidocaine as a local anesthetic for simple laceration repair. Ann Emerg Med. 1990;19:1387–1390. 60. Scarfone R.J. Pain of local anesthetics: rate of administration and buffering. Ann Emerg Med. 1998;31:36–40. 61. Kelly A.M, Cohen M, Richards D. Minimizing the pain of local infiltration anesthesia for wounds by injection into the wound edges. J Emerg Med. 1994;12:593–595. 62. Davies R.J. Buffering the pain of local anaesthetics: a systematic review. Emerg Med Australas. 2003;15:81–88. 63. Krunic A.L, Wang L.C, Soltani K, Weitzul S, Taylor R.S. Digital anesthesia with epinephrine: an old myth revisited. J Am Acad Dermatol. 2004 Nov;51(5):755–759. 64. Hormozi A.K, Zendehnam H, Hosseini S.N, Rasti M, Rostami K, Hamraz H. Epineph effect in varying concentrations on the end artery organ in an animal model. J Coll Physicians Surg Pak. 2010 Feb;20(2):90–92. 65. Chowdhry S, Seidenstricker L, Cooney D.S, Hazani R, Wilhelmi B.J. Do not use epinephrine in digital blocks: myth or truth? Part II. A retrospective review of 1111 cases. Plast Reconstr Surg. 2010 Dec;126(6):2031–2034. 66. Peutrell J.M, Mather S.J. Regional Anaesthesia in Babies & Children. Oxford: Oxford University Press; 1997. 67. Rubin K, Sullivan D, Sadhasivam S. Are peripheral and neuraxial blocks with ultrasound guidance more effective and safe in children? Pediatr Anesthesia. 2009 Feb;19(2):92–96. 68. Wathen J.E, Gao D, Merritt G, Georgopoulos G, Battan F.K. A randomized controlled trial comparing a fascia iliaca compartment nerve block to a traditional systemic analgesic for femur fractures in a

pediatric emergency department. Ann Emerg Med. 2007 Aug;50(2):162–171. 69. Neubrand T.L, Roswell K, Deakyne S, Kocher K, Wathen J. Fascia iliaca compartment nerve block versus systemic pain control for acute femur fractures in the pediatric emergency department. Pediatr Emer Care. 2014;30:469–473. 70. Dalens B, Vanneuville G, Tanguy A. Comparison of the fascia iliaca compartment with “3-in-1” block in children. Anesth Analg. 1989;69:705–713. 71. Guay J. Adverse events associated with intravenous regional anesthesia (Bier block): a systematic review of complications. J Clin Anesth. 2009 Dec;21(8):585–594. 72. Mohr B. Safety and effectiveness of intravenous regional anesthesia (Bier block) for outpatient management of forearm trauma. CJEM. 2006 Jul;8(4):247–250. 73. Aarons C.E, Fernandez M.D, Willsey M, Peterson B, Key C, Fabregas J. Bier block regional anesthesia and casting for forearm fractures: safety in the pediatric emergency department setting. J Pediatr Orthop. 2014 Jan;34(1):45–49. 74. Drendel A.L, Gorelick M.H, Weisman S.J, et al. A randomized clinical trial of ibuprofen versus acetaminophen with codeine for acute pediatric arm fracture pain. Ann Emerg Med. 2009;54:553–560. 75. Ivani G, Mereto N, Lampugnani E, et al. Ropivacaine in paediatric surgery: preliminary results. Paediatr Anaesth. 1998;8:127–129.

Further reading Green S.M. Research advances in procedural sedation and analgesia. Ann Emerg Med. 2007;49:31–36. National Institute of Clinical Studies. Emergency Care Acute Pain Management Manual. Canberra: National Health and Medical Research Council; 2011. Paediatrics & Child Health Division. The Royal Australasian College of Physicians. Guideline statement: management of procedure-related pain in children and adolescents. J Paediatr Child Health. 2006;42 S1S29. Palmer G.M. Pain management in the acute care setting: update and debates. J Paediatr Child Health. 2016;52:213–220.

20.2

Paediatric procedural sedation within the emergency department David M. Krieser, Shona McIntyre, and Bindu Bali

Introduction Emergency department (ED) attendances due to acute pain are very common in children. The Paediatric Research in Emergency Departments International Collaborative (PREDICT) reported on 314,025 attendances, with abdominal pain, lacerations and forearm fractures among the ten most common ED diagnoses,1 each inherently associated with pain. Pain can be the trigger for ED presentation but may also be the consequence of necessary medical management within the ED. The development of procedural sedation (PS) parallels the development of paediatric emergency medicine as a subspecialty. As clinicians developed confidence in managing the sedated child, procedures previously performed in the operating theatre have migrated to the ED.2 ED performance of PS allows more rapid discharge, reduces costs, and increases clinician skills and job satisfaction, while making unpleasant procedures more tolerable to children and their families.3,4

The goal of procedural sedation The ultimate goal of PS is to safely achieve a level of consciousness that allows successful completion of the necessary procedure, a return to consciousness and discharge home within a reasonable time frame. However, it is not enough to safely sedate a child. A practitioner competent in the procedure that is deemed necessary must be available. If there is doubt, then ED PS may be inappropriate. Collaborative planning between the ED staff and other specialists may be required. The procedure may need to be done in an operating theatre or may still

be achieved with the ED practitioner taking responsibility for safe sedation and a relevant proceduralist undertaking the procedure.5 One clinician must remain responsible for, and focus on, safe PS, whether or not the procedure is undertaken by an ED clinician or another specialist. Although serious adverse events are rare,6–9 sedation providers should be prepared to intervene should complications occur. Cote10 and Hoffman11 demonstrated that patient selection, adherence to a prescribed process and minimisation of the number of sedating agents were important in reducing PS risks. Sedation registries have provided data accumulated over many thousands of sedation events. The Pediatric Sedation Research Consortium (www.pedsedation.org) in the United States receives data from over 30 centres that perform PS. No deaths, one cardiac arrest (requiring CPR) and one aspiration event were reported by Cravero et al.12 in a cohort who represented 30,000 PS episodes. Unplanned intervention was needed in 336 PS episodes (111.9 per 10,000 PS episodes), with bag-valve-mask ventilation (63.9 per 10,000 PS episodes) and intubation (9.7 per 10,000 PS episodes) the most invasive. The phases and the tasks involved in PS can be divided into pre-procedure, intra-procedure and post-procedure (Fig. 20.2.1). This division provides a framework to build one’s practice upon.

Pre-procedure Governance Governance structures that include guideline development, education and credentialing improve practice in PS3,13 with a reduction in adverse events.10 This is the foundation for safe PS practice and should occur before any child is sedated. In Victoria, Australia, over the last decade, the development and dissemination of a PS programme13–16 (handbook, presentations for trainers, practical train-the-trainer sessions and testing materials) have provided a structure for teaching and providing practical experience. Hospital networks and EDs all play a role in the establishment and maintenance of PS education and procedural systems.

Patient selection and risk assessment Appropriate patient selection reduces the risk of PS. Assigning risks as potential

threats to airway, breathing, circulation, neurological and other factors can assist in developing a structure to assessment (Table 20.2.1). Sedation risk is higher in younger children. Those under 12 months, and especially infants under 6 months, have an increased risk of apnoea.6,7 Airway interventions, such as bagvalve-mask ventilation and/or endotracheal intubation, if needed, may be more complicated in younger infants as well.17 Intercurrent illness such as active asthma and upper respiratory tract infection can complicate general anaesthesia and may also have an adverse effect on PS. As with any ED attendance a history of current medications and allergies is required. Physical examination must document patient weight, vital signs, an assessment of conscious state, airway evaluation (e.g. Malampati score) with assessment of cardiac and respiratory systems. A Malampati score of Class 3 or 4 suggests more difficult bag-valve-mask ventilation and risk of airway obstruction.5 The Malampati score requires a certain degree of cooperation that may not be possible in younger infants.

Fasting The association between fasting time, vomiting and aspiration is not well supported by evidence, although fasting remains a powerful principle in anaesthesia. Babl18 noted vomiting in 162 of 2002 (8%) children undergoing PS with either nitrous oxide or ketamine, while Cravero et al., reporting on the pediatric sedation research consortium data, identified 142 vomiting events out of 30,037 PS (0.47%) episodes.19 Bellolio et al., as part of a meta-analysis, reported 498 vomiting events out of 7865 (6.33%) PS episodes.6 Given conflicting reports, a pragmatic sedation plan will recognise the patient’s medical, surgical and anaesthetic history (where relevant), the nature of the last meal eaten and the urgency of the procedure required.20

Consent Informed consent should include general risks associated with PS and the procedure being undertaken. In addition, the specific effects of the chosen sedation plan must be discussed. For example, if ketamine were the chosen pharmacological agent, discussion regarding vomiting, hyperlacrimation, hypersalivation, hiccups and emergence phenomena should be initiated. Preprepared information sheets for specific, and common, PS plans can be useful.

Equipment and personnel The staff involved in a PS episode must be competent in the procedure being performed and in the management of children undergoing sedation. Depending on the sedation plan, a minimum of two practitioners should be involved. One, ‘the sedationist’, is responsible for the sedation, monitoring (especially cardiac and respiratory status) and documentation of vital signs during PS.21 The other, ‘the proceduralist’, will be responsible for the procedure. If parenteral sedation is utilised it is prudent to have a third practitioner, to document events or assist with the procedure or the PS. In many EDs the supervising senior doctor must be notified of the procedure and the PS so that in the event of complications they can become involved.13,14,16 Depending on the involved practitioners, the patient and the procedure, they may not be at the bedside. On some occasions this person will, however, be undertaking the PS as well.

FIG. 20.2.1 The phases and tasks of procedural sedation. Krieser D, Kochar A. Paediatric procedural sedation within the emergency department. J of Paediatr & Child Health. 2016;52(2):197–203. Used with permission.

While intravenous (IV) access is desirable, it is not essential for safe PS.21,22 IV access is certainly not required for nitrous oxide PS. Ketamine can be given

intramuscularly (IM), but a larger dose is needed, and sedation persists for longer and is associated with a higher incidence of vomiting.23,24 If vascular access is not achieved prior to PS then the expertise and equipment to rapidly achieve access, including intraosseous needles, should be immediately available.

Rapport building Developing rapport with paediatric patients and their carers is a requirement for most clinical work in paediatric emergency medicine. In PS, good rapport may mean that less pharmacological sedation is required, as the child feels less threatened. The use of distraction throughout the PS episode with items such as books, bubbles, screens (via phones, tablet computers, or televisions) or music is helpful. Prior to the procedure, a developmentally appropriate discussion should occur with the child. Play therapy,25 guided imagery and meditation have been used to reduce pharmacological doses. Caregivers should be provided with instructions in what to say and what not to say,26 avoiding bribery and statements that suggest the procedure is ‘nearly finished’ or that ‘it doesn’t hurt’. For nitrous oxide delivery, the use of commercially available food essences (e.g. strawberry, chocolate or orange essences) makes the use of the mask more acceptable and provides some choice where the child may perceive few choices. It also provides another distraction trigger, where the PS provider can ask, ‘Can you smell the strawberries?’ or ‘I also like chocolate’.

Intra-procedure Real-time monitoring of airway, breathing and circulation are the mainstays of safe PS. Identified abnormalities should trigger appropriate responses from trained personnel. Equipment must be available for real-time monitoring of heart rate, respiratory rate, oxygen saturations and blood pressure (if parenteral drugs are utilised). Nasal cannulae that can monitor expired CO2 can be useful as a rise in expired CO2 will identify hypopnea prior to the development of hypoxia.11,20 It is not clear if this translates to safer PS27,28 and currently is not available in all EDs. Equipment to manage threats to airway, breathing and circulation must be available for all sizes of children. The necessary equipment should be prepared and drug doses calculated prior to the commencement of sedation. Sedation depth can be monitored and documented; however, variations in sedation depth can be difficult to control along the continuum of sedation. This

varies by pharmacological agent and will be discussed later in the text. Sedation can be reduced as the procedure nears conclusion. Judgment is required to balance the need for comfort for the remaining elements of the procedure with the duration of action of the pharmacological agent used. Local anaesthetic application or infiltration in laceration repair can potentially reduce the need for additional systemic PS. Nitrous oxide concentration can be weaned progressively, if possible. Rapport building prior to the commencement of the procedure may also reduce the need for additional systemic PS.25

Post-procedure No patient wants to remain in the ED longer than the minimum required period, and there are quality indicators that value shorter lengths of stay. That said, following PS, a period of observation is needed.21,29 As children emerge from pharmacological PS, vomiting may occur. Emergence dysphoria is well recognised following ketamine PS. Dysphoria can be reduced following PS by optimising the environment: dimming of lights, playing familiar music and allowing only familiar voices (i.e. family members only) while minimising medical procedures. Pharmacological treatment with a benzodiazepine, while rarely required,29 could be used in this situation. Table 20.2.1 Factors to consider in assessing risk for procedural sedation Active medical problems

Past history

Airway

Croup Foreign body Head and facial trauma

Breathing

Respiratory tract infection (e.g. pneumonia, bronchiolitis) Asthma

Sleep apnoea

Circulation

Shock (e.g. hypovolaemia, sepsis) Arrhythmia

Congenital cardiac disease (e.g. cardiac failure, pulmonary hypertension)

Neurological

Altered level of consciousness (seizure, meningitis, trauma) Space-occupying lesion

Other

Unstable psychiatric disorder

Previous airway surgery Laryngomalacia Craniofacial abnormalities Risk of vomiting (e.g. bowel obstruction, gastrooesophageal reflux)

Unstable epilepsy Neuromuscular disease History of sedation failure History of anaesthetic reaction Family history of anaesthetic reaction

Modified from Paediatric Procedural Sedation; ED Manual. Victorian Department of Health (ECIICN, Royal Children’s Hospital, VMIA) 2013 and Daud YN, Carlson DW. Pediatric sedation. Pediatr Clin North Am 2014;61(4):703–17.

A return to pre-sedation level of consciousness is not essential for discharge, but the child should be near these levels. Vital signs should be in the normal range and documented. Persistent vomiting may preclude early discharge, with a need for longer monitoring periods and/or treatment with antiemetics and fluids. The provision of written discharge instructions has been demonstrated to improve post-discharge care in a variety of situations,30 and following PS such instructions should be given to families. Information should include hospital contact phone numbers, recommended levels of activity following PS, dietary guidelines and advice on when to return to the ED. Following PS it may not be appropriate to undertake activities requiring high levels of coordination (e.g. bike riding, climbing on monkey bars) for 24 hours.

Non-pharmacological methods A balanced multidisciplinary approach using pharmacological and nonpharmacological strategies is essential to providing optimal analgesia and sedation for children. Non-pharmacological techniques can be particularly useful in pain management (whether or not medications are used as well) as they are free of side effects and may be utilised before, during and after painful procedures. The planning of procedures for children in the ED should include ageappropriate psychological interventions, such as distraction techniques. Involvement of play therapists or any additional team member to facilitate distraction techniques can prove very useful. Better procedure outcomes are reported for children and carers provided with age-appropriate distraction and the reduction of perceived pain, observed pain and situational anxiety.31–37 A child’s anxiety and cooperation are affected by age, anxiety of the parent and previous medical experiences. The child and parent should be consulted about previous experiences and consideration of distraction techniques to use. Familiarisation with some equipment may help–for example, showing the child the mask to be used prior to nitrous use or asking the child to choose a ‘flavour’ to be placed into the mask. An informative and collaborative approach with a clear plan between all parties often produces smoother procedures. Some useful non-pharmacological strategies that are usually used in

combination are outlined in Box 20.2.1.

Selection of agents by procedure and age Parental preparation to obtain informed consent and provide support to the child for the procedure is essential. Discussion with the parents should include expected effects of the medication on their child and options for escalation or cessation if the procedure does not provide adequate sedation. Nonpharmacological techniques should always be utilised in synergy. Box 20.2.1 Non-pharmacological strategies during

procedures Environment • Calm, friendly staff (verbal and non-verbal communication) and atmosphere • Parental/carer preparation and presence • Age-appropriate communication • Comforter (favourite blanket/soft toy)

Distraction techniques • Visual, e.g. TV, electronic hand-held device, projected visual images, large story book, distraction cards, virtual reality, bubbles • Oral, e.g. music, storytelling, singing children’s songs, guided imagery • Olfactory, e.g. flavour smell in mask, aromatherapy • Activity, e.g. electronic game, search and find story book, arts activity

Physical • Comfort swaddling (infants) • Position of infant on parent so unable to see procedure, e.g. arm tucked under parent’s side for IV cannulation • Massage/rubbing • Vibration or cold temperature to pain distract, e.g. Buzzy® for injections

• Oral sucrose/breast-feeding/pacifier for infants