Holcomb and Ashcraft’s Pediatric Surgery [7th Edition] 9780323549776

Table of contents :
Front Cover......Page 1
Holcomb and Ashcraft’s Pediatric Surgery......Page 3
Holcomb and Ashcraft’s Pediatric Surgery......Page 5
Copyright......Page 6
Contents......Page 7
Video Table of Contents......Page 10
Preface......Page 12
List of Contributors......Page 13
Dedication......Page 23
I - General......Page 25
Hypoglycemia......Page 26
CALCIUM......Page 27
Polycythemia......Page 28
JAUNDICE......Page 29
GLOMERULAR FILTRATION RATE AND EARLY RENAL FUNCTION......Page 30
NEONATAL FLUID REQUIREMENTS......Page 31
SURFACTANT......Page 32
PULSE OXIMETRY......Page 33
PULMONARY ARTERY......Page 34
CARDIOGENIC SHOCK......Page 35
Isoproterenol......Page 36
SEPTIC SHOCK......Page 37
References......Page 39
Body Composition and Nutrient Reserves......Page 42
Energy Expenditure During Illness......Page 44
PROTEIN METABOLISM AND REQUIREMENT DURING ILLNESS......Page 45
CARBOHYDRATE METABOLISM AND REQUIREMENT DURING ILLNESS......Page 47
LIPID METABOLISM AND REQUIREMENTS DURING ILLNESS......Page 48
ENTERAL NUTRITION......Page 49
PARENTERAL NUTRITION......Page 52
Conclusion......Page 54
References......Page 55
MORBIDITY/MORTALITY......Page 59
CRITERIA FOR AMBULATORY SURGERY......Page 60
Malignant Hyperthermia Susceptibility......Page 61
PREOPERATIVE FASTING GUIDELINES......Page 62
Respiratory and Airway Considerations......Page 63
Postanesthetic Apnea......Page 64
PATIENTS WITH CONGENITAL HEART DISEASE......Page 65
Preoperative Preparation and Evaluation......Page 66
Pulmonary Hypertension......Page 67
Single Ventricle Physiology......Page 68
THE DIFFICULT PEDIATRIC AIRWAY......Page 69
ANESTHETIC CONSIDERATIONS FOR SPECIFIC SURGICAL APPROACHES......Page 70
THORACOSCOPY......Page 71
Intraoperative Awareness......Page 73
Opioids......Page 74
Prescribing Discharge Analgesics......Page 75
Conclusion......Page 76
References......Page 77
Renal Function Evaluation......Page 81
GLOMERULAR FILTRATION RATE......Page 82
Renal Tubular Acidosis......Page 83
MEDICAL MANAGEMENT......Page 84
Dialysis......Page 85
Dialysis Methods......Page 86
Acute Kidney Injury in the Neonate......Page 87
CLINICAL PRESENTATION......Page 88
MANAGEMENT......Page 89
Obstructive Uropathy in the Neonate......Page 90
PATHOPHYSIOLOGY AND ETIOLOGY......Page 91
DIAGNOSIS AND IMAGING......Page 92
Interventional Procedures......Page 93
Surgical Treatment......Page 94
OUTCOMES AND COMPLICATIONS......Page 95
References......Page 96
PLATELETS......Page 100
Clinical Evaluation......Page 102
BLEEDING TIME AND THE PFA-100 ANALYZER......Page 103
FIBRINOGEN......Page 104
GENETIC TESTING......Page 105
Hemophilia A and B......Page 106
Disseminated Intravascular Coagulation......Page 107
Fibrinolytic and Thrombotic Disorders......Page 108
Sickle Cell Disease......Page 109
INTRAOPERATIVE MANAGEMENT......Page 110
Retinopathy......Page 111
References......Page 112
Clinical Applications......Page 115
CLINICAL MEASUREMENT SYSTEMS......Page 116
PERSISTENT PULMONARY HYPERTENSION OF THE NEWBORN......Page 117
CONGENITAL DIAPHRAGMATIC HERNIA......Page 118
Methods of Extracorporeal Support......Page 119
CANNULATION......Page 120
ECMO CIRCUIT......Page 122
Patient Management on ECMO......Page 123
Air Embolism......Page 125
HYPERTENSION......Page 126
FEEDING AND GROWTH SEQUELAE......Page 127
ECMO IN PREMATURE NEONATES......Page 128
ECLS IN EXTREME PREMATURITY: THE ARTIFICIAL PLACENTA......Page 129
References......Page 131
CARBON DIOXIDE ELIMINATION......Page 135
OXYGENATION......Page 137
THE MECHANICAL VENTILATOR AND ITS COMPONENTS......Page 139
Synchronized Intermittent Mandatory Ventilation......Page 140
Volume-Assured Pressure Support Ventilation......Page 141
Continuous Positive Airway Pressure......Page 142
MANAGEMENT OF THE MECHANICAL VENTILATOR......Page 143
Permissive Hypercapnia......Page 144
Using Protective Effects of Positive End-Expiratory Pressure......Page 145
Oral Hygiene and Mucus Clearance......Page 146
Weaning From Mechanical Ventilation......Page 147
HIGH-FREQUENCY VENTILATION......Page 148
INHALED NITRIC OXIDE ADMINISTRATION......Page 149
Ventilator-Associated Pneumonia......Page 150
References......Page 152
Peripheral Venous Access......Page 157
Peripherally Introduced Central Catheter......Page 158
Central Venous Catheters......Page 159
Totally Implanted Central Venous Catheters......Page 160
Venous Cutdown......Page 161
Hemodialysis Catheters......Page 162
References......Page 163
ANATOMIC BARRIERS......Page 165
Antibiotics......Page 166
WOUND CLASSIFICATION......Page 167
POSTOPERATIVE SURGICAL SITE INFECTION......Page 168
CATHETER INFECTIONS......Page 170
Necrotizing Soft Tissue Infection......Page 171
Peritonitis......Page 172
References......Page 173
Guiding Principles......Page 177
Fetal Access, Anesthesia, and Operative Setup......Page 178
Fetal Interventions......Page 179
Congenital Pulmonary Airway Malformations, Lung Lesions, and Bronchopulmonary Sequestrations......Page 181
Sacrococcygeal Teratoma......Page 182
Twin–Twin Transfusion Syndrome......Page 184
MYELOMENINGOCELE......Page 185
HYDRONEPHROSIS AND LOW URINARY TRACT OBSTRUCTION......Page 187
ABDOMINAL WALL DEFECTS......Page 188
Fetal Surgery Programs......Page 189
References......Page 190
II - Trauma......Page 195
Esophageal Foreign Bodies......Page 196
BATTERIES......Page 197
MAGNETS......Page 198
BEZOARS......Page 200
Airway Foreign Bodies......Page 201
BRONCHOSCOPY......Page 203
References......Page 204
Cat Bites......Page 205
TREATMENT......Page 206
Tetanus......Page 207
BROWN RECLUSE SPIDERS......Page 208
BLACK WIDOW SPIDER......Page 210
CROTALID (PIT VIPER)......Page 211
Clinical Features......Page 212
Prehospital Management......Page 213
In-Hospital Management......Page 214
Role of the Surgeon......Page 215
CORAL SNAKES......Page 216
References......Page 217
Pathophysiology......Page 220
Initial Management......Page 221
Fluid Resuscitation......Page 222
RESUSCITATION ENDPOINTS......Page 224
Inhalation Injury......Page 225
Assessment of Burn Depth......Page 226
ANTIMICROBIAL AGENTS......Page 227
EXCISION AND GRAFTING......Page 229
ELECTRICAL BURNS......Page 230
Psychologic Sequelae of Burn Injuries......Page 231
References......Page 232
Injury Risks......Page 235
Injury Patterns......Page 236
NECK......Page 237
SKELETON......Page 238
RESUSCITATION......Page 239
SECONDARY SURVEY......Page 241
NONACCIDENTAL TRAUMA......Page 242
AMBULANCE TRANSPORT......Page 243
References......Page 244
Rib Fractures......Page 248
Traumatic Asphyxia......Page 249
Pneumothorax–Pulmonary Lacerations......Page 250
Pulmonary Contusion......Page 251
Diaphragmatic Injuries......Page 252
Airway Injury......Page 254
Great Vessel Injuries......Page 255
Blunt Cardiac Injury......Page 256
References......Page 257
Evaluation of Blunt Abdominal Trauma......Page 260
NONOPERATIVE MANAGEMENT GUIDELINES......Page 261
OPERATIVE MANAGEMENT OF LIVER AND SPLEEN BLEEDING......Page 262
LENGTH OF STAY......Page 264
DEFINING THE NEED FOR OPERATION......Page 265
Renal Injury......Page 266
Pancreatic Injury......Page 267
SMALL BOWEL INJURIES......Page 268
RECTAL INJURY......Page 269
Blunt Diaphragmatic Injury......Page 271
Overwhelming Postsplenectomy Infection......Page 272
REIMAGING AFTER RENAL INJURY......Page 273
References......Page 274
Axonal Shearing......Page 278
PATHOPHYSIOLOGY......Page 279
Primary Brain Injury......Page 280
Secondary Brain Injury......Page 281
INITIAL EVALUATION FOR TBI......Page 282
Severe Traumatic Brain Injury: Glasgow Coma Scale 3–8......Page 283
Nonaccidental Trauma......Page 284
Neuromuscular Blockade......Page 285
Hyperosmolar Therapy......Page 286
Barbiturate Therapy......Page 287
OUTCOME AND PROGNOSIS......Page 288
References......Page 289
Pathophysiology......Page 291
Fractures of the Lower Extremity......Page 292
CERVICAL SPINE INJURIES......Page 296
THORACIC, LUMBAR, AND SACRAL FRACTURES......Page 299
Fractures of the Upper Extremity......Page 301
References......Page 305
Hydrocephalus......Page 309
Neurosurgical Devices......Page 311
Neural Tube Defects......Page 312
Craniosynostosis......Page 314
Vascular Malformations of the Brain......Page 315
Tethered Spinal Cord......Page 317
Chiari I Malformations......Page 319
PILOCYTIC ASTROCYTOMA......Page 320
EPENDYMOMA......Page 321
DIFFUSE INTRINSIC PONTINE GLIOMA......Page 322
Intracranial Infections......Page 323
References......Page 324
III - Thoracic......Page 325
HISTORY......Page 326
CLINICAL FEATURES......Page 328
CARDIAC AND PULMONARY EFFECTS OF PECTUS EXCAVATUM......Page 329
EVALUATION AND INDICATIONS FOR OPERATION......Page 330
Minimally Invasive Pectus Repair......Page 331
Open Technique......Page 334
Late Complications......Page 337
Pectus Carinatum......Page 338
INCIDENCE AND PREVALENCE......Page 339
Cartilage Resection Operations......Page 340
Noncartilage Resection Operations......Page 342
NONSURGICAL TREATMENTS......Page 344
Pressure-Controlled Bracing......Page 345
Sternal Defects......Page 347
Thoracic Insufficiency Syndrome Associated With Diffuse Skeletal Disorders......Page 348
References......Page 353
Subglottic and Tracheal Malformations......Page 356
CONGENITAL SUBGLOTTIC AND TRACHEAL STENOSIS......Page 357
VASCULAR COMPRESSION......Page 358
Tracheomalacia–Bronchomalacia......Page 360
INTRINSIC INJURIES......Page 361
TRACHEOSTOMY......Page 362
Laryngeal and Tracheal Clefts......Page 363
CONGENITAL STENOSIS......Page 365
ACQUIRED STENOSIS......Page 366
OPERATIVE TECHNIQUES......Page 367
References......Page 370
Embryology and Development of the Bronchopulmonary Tree......Page 372
CONGENITAL PULMONARY AIRWAY MALFORMATION......Page 373
BRONCHOPULMONARY SEQUESTRATION......Page 374
BRONCHOGENIC CYSTS AND BRONCHIAL ATRESIA/STENOSIS......Page 375
CONGENITAL PULMONARY AIRWAY MALFORMATION......Page 376
CONGENITAL LOBAR EMPHYSEMA, BRONCHIAL ATRESIA......Page 377
CONGENITAL PULMONARY AIRWAY MALFORMATION......Page 378
CONGENITAL LOBAR EMPHYSEMA......Page 379
BRONCHOGENIC CYSTS AND BRONCHIAL ATRESIA......Page 380
TECHNIQUE......Page 381
References......Page 384
DIAGNOSIS......Page 385
Parapneumonic Effusion......Page 386
Empyema......Page 387
Pneumatocele......Page 389
Bronchiectasis......Page 390
MANAGEMENT......Page 391
Diffuse Interstitial Disease......Page 392
Spontaneous Pneumothorax......Page 395
References......Page 398
Associated Anomalies......Page 401
DIAPHRAGM DEVELOPMENT AND CDH PATHOGENESIS......Page 402
PULMONARY VASCULAR DEVELOPMENT AND CDH-ASSOCIATED PULMONARY HYPERTENSION......Page 404
PRENATAL DIAGNOSIS......Page 405
CLINICAL PRESENTATION......Page 406
RESUSCITATION AND STABILIZATION......Page 407
MECHANICAL VENTILATION......Page 408
EXTRACORPOREAL MEMBRANE OXYGENATION......Page 409
TIMING OF OPERATION......Page 410
MINIMALLY INVASIVE APPROACH......Page 411
Nonabsorbable Synthetic Patches......Page 413
Tissue-Engineered Patches......Page 414
OUTCOMES......Page 415
PULMONARY OUTCOMES......Page 416
NEUROLOGIC OUTCOMES......Page 417
Anterior Hernias of Morgagni......Page 418
Diaphragmatic Eventration......Page 419
References......Page 420
Diagnostic Imaging......Page 427
Principles of Management......Page 428
NON-HODGKIN LYMPHOMA......Page 429
HODGKIN LYMPHOMA......Page 430
TERATOMAS......Page 431
THYMIC HYPERPLASIA......Page 433
Vascular Tumors and Anomalies......Page 434
Foregut Cysts and Duplications......Page 435
Neural Tumors......Page 437
References......Page 441
Clinical Evaluation of the Esophagus......Page 446
ESOPHAGEAL DUPLICATION CYSTS......Page 447
Achalasia......Page 448
Foreign Body Esophageal Injury......Page 449
Esophageal Perforation......Page 451
Caustic Ingestion......Page 452
Esophageal Strictures......Page 453
Esophageal Replacement......Page 454
References......Page 459
Epidemiology......Page 461
PURE ESOPHAGEAL ATRESIA WITHOUT TEF (GROSS TYPE A)......Page 462
POSTNATAL DIAGNOSIS......Page 463
PREOPERATIVE BRONCHOSCOPY......Page 465
REPAIR VIA THORACOTOMY......Page 466
Positioning......Page 468
Initial Treatment......Page 469
Postoperative Management......Page 470
RECURRENT TRACHEOESOPHAGEAL FISTULA......Page 471
DISORDERED PERISTALSIS/GASTROESOPHAGEAL REFLUX/ESOPHAGEAL CANCER......Page 472
RECENT OUTCOME STUDIES......Page 473
Laryngeal and Laryngotracheoesophageal Cleft......Page 474
References......Page 477
IV - Abdomen......Page 483
Barriers Against GERD......Page 484
Clinical Manifestations......Page 486
Diagnostic Evaluation......Page 487
LAPAROSCOPIC NISSEN FUNDOPLICATION......Page 489
GASTROSTOMY......Page 491
Outcomes......Page 492
Redo Fundoplication......Page 496
References......Page 498
DIAGNOSIS......Page 502
The Open Approach......Page 503
The Laparoscopic Operation......Page 504
COMPLICATIONS/OUTCOMES......Page 505
Peptic Ulcer Disease......Page 506
Microgastria......Page 507
Gastric Volvulus......Page 508
Foreign Bodies and Bezoar......Page 510
References......Page 511
ETIOLOGY......Page 513
CLASSIFICATION SYSTEM......Page 515
MANAGEMENT......Page 516
Stenosis......Page 519
Type III(b) Atresia......Page 520
CLINICAL MANIFESTATIONS......Page 522
DIAGNOSIS......Page 523
OPERATIVE CONSIDERATIONS......Page 524
POSTOPERATIVE CARE......Page 525
Colonic Atresia......Page 526
References......Page 528
Presentation......Page 531
Diagnosis......Page 533
OPEN APPROACH......Page 534
LAPAROSCOPIC APPROACH......Page 535
Postoperative Management......Page 537
OTHER CONGENITAL ANOMALIES......Page 538
References......Page 539
GASTROINTESTINAL PATHOPHYSIOLOGY......Page 541
PRENATAL DIAGNOSIS AND SCREENING......Page 542
SONOGRAPHIC EVALUATION......Page 543
CLINICAL PRESENTATION......Page 544
Complicated Meconium Ileus......Page 545
RADIOGRAPHIC FEATURES......Page 546
Simple Meconium Ileus......Page 547
POSTOPERATIVE MANAGEMENT......Page 548
NUTRITIONAL MANAGEMENT......Page 549
Meconium Plug Syndrome......Page 550
BILIARY TRACT DISEASE......Page 551
DISTAL INTESTINAL OBSTRUCTION SYNDROME......Page 552
FIBROSING COLONOPATHY......Page 554
References......Page 555
Pathophysiology......Page 560
Intestinal Motility and Digestion......Page 561
Tight Junctions......Page 562
Lipopolysaccharide......Page 563
NEONATAL VASCULATURE AND THE PATHOGENESIS OF NEC......Page 564
LABORATORY STUDIES......Page 565
Ultrasound......Page 566
Medical Management......Page 567
Primary Peritoneal Drainage......Page 568
INTESTINAL FAILURE......Page 570
NEURODEVELOPMENTAL OUTCOMES......Page 571
PROBIOTICS......Page 572
Conclusion......Page 573
References......Page 574
Etiology and Genetic Basis of Disease......Page 581
Clinical Presentation and Diagnosis......Page 582
Surgical Management......Page 583
SOAVE PROCEDURE......Page 585
TRANSANAL (PERINEAL) PULL-THROUGH......Page 586
LONG-SEGMENT AGANGLIONOSIS......Page 588
OBSTRUCTIVE SYMPTOMS......Page 590
Persistent or Acquired Aganglionosis or a Transition Zone Pull-Through......Page 592
FECAL SOILING......Page 594
ENTEROCOLITIS......Page 595
INTERNAL SPHINCTER ACHALASIA......Page 596
References......Page 597
RECTOURETHRAL FISTULAS......Page 601
ANORECTAL ATRESIA WITHOUT FISTULA......Page 602
RECTOVESTIBULAR FISTULAS......Page 603
CLOACAL MALFORMATION......Page 604
SACRUM AND SPINE......Page 605
Newborn Management......Page 606
SURGICAL RECONSTRUCTION......Page 608
RECTOURETHRAL FISTULAS......Page 610
RECTOBLADDERNECK FISTULAS......Page 613
Cloacas With a Common Channel Shorter Than 3 cm......Page 614
VAGINAL AUGMENTATION AND/OR REPLACEMENT......Page 617
Postoperative Management......Page 618
Functional Outcomes......Page 619
COMPLICATIONS......Page 620
References......Page 621
Anal Canal Sensation......Page 623
Bowel Motility......Page 624
True Fecal Incontinence......Page 625
Which Children Have True Fecal Incontinence......Page 626
Bowel Management: Key Steps......Page 627
Constipation in Anorectal Malformations and Hirschsprung Disease......Page 629
Failure of Medical Management......Page 632
Surgical Options......Page 633
References......Page 635
Anal Fissure......Page 637
Anal Skin Tags, Hemorrhoids, Polyps, and Other Perianal Vascular Lesions......Page 638
Rectal Prolapse......Page 639
Rectal Trauma......Page 642
References......Page 643
Physical Examination......Page 645
ULTRASONOGRAPHY......Page 646
HYDROSTATIC AND PNEUMATIC REDUCTION......Page 647
LAPAROSCOPIC APPROACH......Page 649
Recurrent Intussusception......Page 650
References......Page 651
Clinical Presentation and Diagnosis......Page 653
ESOPHAGEAL DUPLICATIONS......Page 654
THORACOABDOMINAL DUPLICATIONS......Page 655
PANCREATIC DUPLICATIONS......Page 658
SMALL BOWEL DUPLICATIONS......Page 659
COLONIC DUPLICATIONS......Page 660
Summary......Page 662
References......Page 663
OBSTRUCTION......Page 665
Diagnosis......Page 666
Treatment......Page 668
References......Page 670
PATHOLOGY......Page 671
CLINICAL PRESENTATION......Page 672
MEDICAL MANAGEMENT......Page 673
Preoperative Considerations......Page 674
Open Proctocolectomy With Ileoanal Pull-Through Procedure......Page 675
Laparoscopic Technique......Page 677
OUTCOMES......Page 678
ETIOLOGY......Page 679
CLINICAL PRESENTATION......Page 680
MEDICAL MANAGEMENT......Page 681
SURGICAL MANAGEMENT......Page 682
References......Page 684
Pathophysiology and Natural History......Page 688
Appendicitis Risk Scores......Page 689
Imaging Studies......Page 690
APPENDECTOMY......Page 692
NONOPERATIVE MANAGEMENT......Page 694
Perforated Appendicitis......Page 695
SURGICAL MANAGEMENT......Page 696
Abscess on Presentation......Page 697
References......Page 698
43 - Biliary Atresia......Page 703
Pathogenesis......Page 704
Diagnosis......Page 705
OPEN SURGICAL TECHNIQUE......Page 707
MINIMALLY INVASIVE KASAI PORTOENTEROSTOMY......Page 710
CHOLAGOGUES......Page 711
PORTAL HYPERTENSION......Page 712
Results and Prognosis......Page 713
Liver Transplantation......Page 714
References......Page 715
ETIOLOGY......Page 719
IMAGING......Page 720
Bilio-Enteric Anastomosis After Cystectomy......Page 721
Laparoscopic Approach......Page 722
Hepaticoduodenostomy......Page 723
OUTCOMES......Page 724
Clinical Presentation and Diagnostic Assessment......Page 725
FOUR-PORT TECHNIQUE......Page 726
SINGLE-SITE LAPAROSCOPIC CHOLECYSTECTOMY......Page 727
References......Page 729
Alagille Syndrome......Page 733
Fulminant Hepatic Failure......Page 734
Organ Allocation......Page 735
Donor Selection......Page 736
THE TRANSPLANT PROCEDURE......Page 738
POSTOPERATIVE COMPLICATIONS......Page 739
Vascular Thrombosis......Page 740
Biliary Complications......Page 741
Renal Insufficiency......Page 742
Infection......Page 743
OUTCOMES FOLLOWING TRANSPLANTATION......Page 744
INDICATIONS FOR TRANSPLANTATION......Page 745
Multivisceral Allograft......Page 746
Liver–Small Bowel Composite Allograft......Page 747
Immunosuppression and Allograft Rejection......Page 748
Renal Transplantation......Page 749
Nutritional Support......Page 750
PREOPERATIVE PREPARATION......Page 751
POSTOPERATIVE MANAGEMENT......Page 752
INFECTION......Page 753
IMMUNOSUPPRESSION......Page 754
DECEASED DONOR WHOLE ORGAN PANCREAS TRANSPLANTATION......Page 755
PEDIATRIC ISLET TRANSPLANTATION......Page 756
References......Page 757
Congenital Anomalies......Page 761
ACUTE PANCREATITIS......Page 762
CHRONIC PANCREATITIS......Page 766
CONGENITAL HYPERINSULINISM......Page 767
PANCREATIC ENDOCRINE TUMORS......Page 769
ADENOCARCINOMA AND PANCREATOBLASTOMA......Page 770
References......Page 772
ACCESSORY SPLEENS......Page 774
HEREDITARY SPHEROCYTOSIS......Page 775
IMMUNE THROMBOCYTOPENIC PURPURA......Page 776
OPEN SPLENECTOMY......Page 777
LAPAROSCOPIC SPLENECTOMY......Page 778
SINGLE-INCISION LAPAROSCOPIC SURGERY AND SINGLE-INCISION LAPAROENDOSCOPIC SURGERY SPLENECTOMY......Page 779
PARTIAL SPLENECTOMY......Page 780
POSTOPERATIVE HOSPITALIZATION......Page 782
POSTSPLENECTOMY SEPSIS......Page 783
References......Page 784
INCIDENCE, EMBRYOLOGY, AND ETIOLOGY......Page 787
PRENATAL MANAGEMENT AND DELIVERY......Page 788
Primary Closure......Page 789
Staged Closure......Page 790
Management of Associated Intestinal Atresia......Page 791
CLOSING GASTROSCHISIS......Page 792
POSTOPERATIVE COURSE......Page 793
LONG-TERM OUTCOMES......Page 794
RISK ASSESSMENT AND STAGING......Page 795
Staged Neonatal Closure......Page 796
Scarification Treatment......Page 797
LONG-TERM OUTCOMES......Page 799
References......Page 800
TREATMENT......Page 804
Spigelian Hernia......Page 805
References......Page 806
V - Inguinal Region and Scrotum......Page 807
Embryology......Page 808
Clinical Findings......Page 809
INCARCERATION......Page 812
OPEN REPAIR......Page 814
LAPAROSCOPIC REPAIR......Page 816
Comparison of Open Versus Laparoscopic Repairs......Page 820
DISORDERS OF SEXUAL DEVELOPMENT......Page 822
ANESTHESIA......Page 823
References......Page 824
EMBRYOLOGY......Page 829
DIAGNOSIS......Page 830
RISK OF MALIGNANCY......Page 831
Orchiopexy......Page 832
Nonpalpable Undescended Testes: Unilateral or Bilateral......Page 833
PRESENTATION AND DIAGNOSIS......Page 837
CARCINOMA IN SITU......Page 838
Yolk Sac Tumors......Page 839
Mixed Germ Cell Tumor......Page 840
References......Page 841
Testicular Torsion......Page 845
TORSION OF TESTICULAR APPENDAGES......Page 848
TESTICULAR TRAUMA......Page 849
References......Page 850
VI - UROLOGY......Page 851
Renal Dysplasia and Hypoplasia......Page 852
Renal Agenesis......Page 853
Renal Ectopia......Page 854
HORSESHOE KIDNEY......Page 856
AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE......Page 857
SIMPLE CYSTS AND CALYCEAL DIVERTICULI......Page 858
References......Page 859
CLINICAL PRESENTATION......Page 861
DIAGNOSIS......Page 862
Indications for Intervention......Page 863
Operative Techniques......Page 865
SURGICAL RESULTS AND COMPLICATIONS......Page 866
URETERAL DUPLICATION......Page 867
URETERAL TRIPLICATION......Page 868
Treatment......Page 869
Ectopic Ureter in Girls......Page 870
Bilateral Single Ectopic Ureters......Page 871
PRESENTATION AND DIAGNOSIS......Page 872
TREATMENT......Page 873
References......Page 874
DIAGNOSIS......Page 877
EPIDEMIOLOGY......Page 878
Bacterial Factors......Page 879
Current Controversies......Page 880
Acute Phase......Page 881
Vesicoureteral Reflux......Page 882
CLASSIFICATION......Page 883
NATURAL HISTORY......Page 884
Medical Management......Page 886
Surgical Management......Page 887
CONCLUSION......Page 890
References......Page 891
Childhood Incontinence......Page 894
Medications......Page 895
Treatment......Page 896
Neurogenic Bladder......Page 897
CHILDHOOD MANAGEMENT......Page 898
SURGICAL TREATMENT......Page 900
MEGALOURETHRA......Page 903
URETHRAL DIVERTICULUM OR ANTERIOR URETHRAL VALVE......Page 904
References......Page 905
Antenatal Diagnosis, Management, and Outcomes......Page 908
Radiographic Evaluation......Page 910
Initial Management......Page 911
TEMPORARY URINARY DIVERSION......Page 912
PROGNOSIS FOLLOWING INITIAL SURGICAL THERAPY......Page 914
Late Diagnosis......Page 915
Bladder Dysfunction After Initial Therapy......Page 916
References......Page 918
PATHOGENESIS......Page 921
OPERATIVE APPROACHES......Page 922
Anatomic Reconstruction......Page 923
Delayed Primary Closure......Page 924
COMPLETE PRIMARY REPAIR FOR EXSTROPHY......Page 925
Preparation and Assessment of the Anatomy......Page 926
Urethral Plate Dissection......Page 927
Approximation of Tissues (Tubularization of Neourethra, Bladder Neck Approximation, Bladder Closure)......Page 928
Preparation and Assessment of the Anatomy......Page 930
OTHER PRIMARY RECONSTRUCTIVE TECHNIQUES......Page 931
Stage II: Epispadias Repair......Page 932
Early Complications......Page 933
Sexual Function......Page 934
ASSOCIATED ANOMALIES......Page 935
CLOACAL EXSTROPHY CLOSURE TECHNIQUES......Page 936
References......Page 937
Embryology......Page 942
ETIOLOGY......Page 943
ANATOMY OF THE DEFECT......Page 944
ASSOCIATED ANOMALIES......Page 945
Treatment......Page 946
Locating the Meatus......Page 947
DISTAL VARIANTS......Page 948
MIDSHAFT VARIANTS......Page 949
Urinary Diversion......Page 951
Analgesia......Page 952
STRICTURES......Page 954
Results......Page 955
References......Page 956
Routine Circumcision at Birth......Page 959
NEWBORN CIRCUMCISION......Page 961
REVISION CIRCUMCISION......Page 962
FREEHAND CIRCUMCISION......Page 963
References......Page 964
Genetics......Page 966
Ureters......Page 967
Prostate and Accessory Sex Organs......Page 968
Gastrointestinal......Page 969
Management Principles......Page 970
URETERAL RECONSTRUCTION......Page 971
RECONSTRUCTION OF THE ABDOMINAL WALL......Page 972
Conclusion......Page 973
References......Page 974
Normal Gender and Sexual Differentiation......Page 977
46,XX DSD......Page 978
46,XY DSD......Page 979
MÜLLERIAN-INHIBITORY SUBSTANCE DEFICIENCY (HERNIA UTERINE INGUINALE)......Page 980
MIXED GONADAL DYSGENESIS......Page 981
OTHER SYNDROMES OF ABERRANT SEXUAL DIFFERENTIATION......Page 982
Evaluation of the Newborn With Ambiguous Genitalia......Page 983
CONTROVERSIES AND CONSIDERATIONS......Page 984
FEMALE GENDER ASSIGNMENT......Page 985
References......Page 988
VII - Neoplasms......Page 991
History of Pediatric Oncology......Page 992
Tumor Biology: Understanding Childhood Cancer and Treatment Principles......Page 993
Adjuvant and Neoadjuvant Chemotherapy......Page 994
Chemotherapeutic Agents......Page 995
LONG-TERM SIDE EFFECTS OF CANCER THERAPY IN CHILDREN......Page 996
BIOLOGIC RESPONSE MODIFIERS......Page 1001
TUMOR-TARGETED ANTIBODY THERAPY......Page 1002
RADIATION ONCOLOGY......Page 1003
CRYOSURGERY......Page 1004
LYMPHATIC MAPPING......Page 1005
References......Page 1006
HISTORY......Page 1010
EPIDEMIOLOGY......Page 1011
WTX......Page 1012
PATHOLOGIC PRECURSORS: NEPHROGENIC RESTS, NEPHROBLASTOMATOSIS, AND MULTICYSTIC DYSPLASTIC KIDNEYS......Page 1013
PATHOLOGY......Page 1015
STAGING......Page 1017
CLINICAL PRESENTATION AND DIAGNOSIS......Page 1018
Surgical Details......Page 1019
Intravascular Extension......Page 1020
CHEMOTHERAPY......Page 1022
PRETREATMENT PRIOR TO SURGERY......Page 1023
RADIOTHERAPY......Page 1024
General Health......Page 1025
Congestive Heart Failure......Page 1026
Mesoblastic Nephroma......Page 1027
Cystic Nephroma......Page 1028
Renal Medullary Carcinoma......Page 1029
References......Page 1030
Pathology......Page 1034
HISTOPATHOLOGIC CLASSIFICATION......Page 1035
Molecular Biology......Page 1036
SEGMENTAL CHROMOSOME ABERRATIONS......Page 1037
Ferritin......Page 1038
Standard Radiographs......Page 1039
Magnetic Resonance Imaging......Page 1040
STAGING......Page 1041
INTERMEDIATE-RISK DISEASE......Page 1042
HIGH-RISK DISEASE......Page 1043
International Neuroblastoma Risk Group Risk Stratification......Page 1044
LOCOREGIONAL DISEASE IN PATIENTS WITH METASTATIC DISEASE......Page 1046
DELAYED SURGICAL RESECTION OF LOCOREGIONAL DISEASE......Page 1047
OPERATIVE COMPLICATIONS......Page 1048
INTRASPINAL EXTENSION OF NEUROBLASTOMA......Page 1049
OPSOCLONUS–MYOCLONUS SYNDROME......Page 1050
References......Page 1051
Clinical Presentation......Page 1055
Imaging......Page 1057
Treatment......Page 1058
Epidemiology......Page 1059
Histology......Page 1060
Incidence......Page 1061
Histology......Page 1062
Clinical Presentation......Page 1063
Associated Conditions......Page 1064
Epidemiology......Page 1065
Histology......Page 1066
Clinical Presentation......Page 1067
Staging and Risk Stratification......Page 1068
Treatment......Page 1069
Outcomes......Page 1074
Clinical Presentation......Page 1075
Histology......Page 1076
Transplantation for Hepatocellular Carcinoma......Page 1077
Imaging......Page 1078
Laboratory Findings and Differential Diagnosis......Page 1079
New Therapeutic Treatment Options for Liver Tumors......Page 1080
References......Page 1083
EMBRYOLOGY AND PATHOLOGY......Page 1090
ASSOCIATED ANOMALIES......Page 1091
SACROCOCCYGEAL TERATOMA......Page 1093
Diagnosis......Page 1094
Prenatal Diagnosis......Page 1095
Prognosis......Page 1096
Mediastinal Teratomas......Page 1100
Retroperitoneal Teratomas......Page 1101
HEAD AND NECK TERATOMAS......Page 1102
Others......Page 1104
DERMOID CYSTS......Page 1105
EPIDERMAL CYSTS......Page 1107
Warts......Page 1108
Neurofibromas......Page 1109
Congenital Epulis......Page 1112
ADIPOSE TISSUE TUMORS......Page 1113
References......Page 1114
INCIDENCE AND EPIDEMIOLOGY......Page 1121
CLASSIFICATION AND HISTOLOGIC SUBTYPING......Page 1122
CLINICAL PRESENTATION......Page 1123
STAGING......Page 1124
Principles of Therapy......Page 1125
Stage, Histology, and Response-Based Therapy......Page 1126
Non-Hodgkin Lymphoma......Page 1127
CLASSIFICATION......Page 1128
By Initial Site of Disease......Page 1129
By Histologic Subtype......Page 1130
DIAGNOSIS......Page 1131
TREATMENT......Page 1132
EARLY COMPLICATIONS......Page 1133
References......Page 1134
Tumor Biology and Histology......Page 1139
Assessment......Page 1140
CLINICAL GROUP......Page 1141
RADIOTHERAPY......Page 1142
Abdominal Sites......Page 1143
Perineal and Perianal Disease......Page 1144
Genitourinary......Page 1145
Metastatic Disease and Recurrent Rhabdomyosarcoma......Page 1146
Late Effects......Page 1147
References......Page 1148
VIII - Skin and Soft Tissue
Diseases......Page 1151
PRESENTATION......Page 1152
RISK FACTORS......Page 1153
MANAGEMENT......Page 1154
PRESENTATION......Page 1155
NEUROCUTANEOUS MELANOCYTOSIS......Page 1156
CLASSIFICATION......Page 1157
ATYPICAL SPITZ NEVI/TUMORS......Page 1158
EPIDEMIOLOGY......Page 1159
PRESENTATION......Page 1160
SURGICAL TREATMENT......Page 1162
ADJUVANT TREATMENT......Page 1163
FUTURE DIRECTIONS......Page 1165
References......Page 1166
Vascular Tumors......Page 1171
Clinical Features......Page 1172
Associated Congenital Anomalies......Page 1173
Treatment......Page 1174
CONGENITAL HEMANGIOMA......Page 1176
TUFTED ANGIOMA AND KAPOSIFORM HEMANGIOENDOTHELIOMA......Page 1177
Vascular Malformations......Page 1178
CAPILLARY MALFORMATIONS......Page 1179
Imaging......Page 1180
Treatment......Page 1181
Clinical Features......Page 1182
Treatment......Page 1184
Treatment......Page 1185
KLIPPEL–TRENAUNAY SYNDROME......Page 1186
PARKES WEBER SYNDROME......Page 1188
Interdisciplinary Vascular Anomalies Center......Page 1189
References......Page 1190
THYROGLOSSAL DUCT CYST......Page 1195
REMNANTS OF EMBRYONIC BRANCHIAL APPARATUS......Page 1197
First Cleft Anomalies......Page 1199
Third and Fourth Cleft Anomalies......Page 1201
PREAURICULAR PITS, SINUSES, AND CYSTS......Page 1202
DERMOID AND EPIDERMOID CYSTS......Page 1203
TORTICOLLIS......Page 1205
ACUTE SUPPURATIVE CERVICAL LYMPHADENITIS......Page 1206
CAT-SCRATCH DISEASE......Page 1207
References......Page 1208
IX - Special Topics......Page 1211
VULVAR ABNORMALITIES......Page 1212
GENITAL BLEEDING......Page 1214
Uterovaginal Anomalies......Page 1215
Adnexal Disease......Page 1217
OVARIAN CYSTS IN ADOLESCENTS......Page 1218
IMAGING CHARACTERISTICS......Page 1219
TUMOR MARKERS......Page 1220
Adnexal Torsion......Page 1223
POSTOPERATIVE SURVEILLANCE......Page 1224
Endometriosis......Page 1225
Fertility Preservation......Page 1226
References......Page 1227
Pathophysiology......Page 1230
HYPOPLASIA AND APLASIA......Page 1231
GYNECOMASTIA......Page 1232
BREAST TRAUMA AND FAT NECROSIS......Page 1233
GALACTORRHEA......Page 1234
EVALUATION OF BREAST MASSES......Page 1235
SIMPLE CYSTS......Page 1236
Phyllodes Tumors......Page 1237
BREAST CANCER......Page 1238
References......Page 1239
Goiter and Thyroiditis......Page 1241
Graves Disease......Page 1242
Hypothyroidism......Page 1243
Thyroid Nodules......Page 1244
Thyroid Carcinoma......Page 1245
Primary Hyperparathyroidism......Page 1249
Neonatal Severe Hyperparathyroidism and Familial Hypocalciuric Hypercalcemia......Page 1250
Cortisol......Page 1251
Hypercortisolism (Cushing Syndrome)......Page 1252
Primary Hyperaldosteronism......Page 1253
Adrenocortical Carcinoma......Page 1254
ADRENAL MEDULLA......Page 1256
PRECOCIOUS PUBERTY IN BOYS......Page 1257
Carcinoid Tumors......Page 1258
References......Page 1259
BARIATRIC PROGRAMS FOR ADOLESCENTS......Page 1264
Physical Maturation......Page 1265
Surgical Options......Page 1266
PREOPERATIVE EDUCATION AND MANAGEMENT......Page 1267
GENERAL ASPECTS OF SURGICAL TECHNIQUES FOR THE MORBIDLY OBESE......Page 1268
Postoperative Management......Page 1270
Long-Term Management......Page 1272
References......Page 1273
A......Page 1277
B......Page 1280
C......Page 1282
D......Page 1286
E......Page 1287
F......Page 1289
G......Page 1290
H......Page 1292
I......Page 1294
L......Page 1296
M......Page 1298
N......Page 1300
O......Page 1302
P......Page 1303
R......Page 1306
S......Page 1307
T......Page 1310
U......Page 1312
V......Page 1314
Z......Page 1315
IBC......Page 1317

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Holcomb and Ashcraft’s Pediatric Surgery

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Holcomb and Ashcraft’s Pediatric Surgery SEVENTH EDITION

George W. Holcomb III, MD, MBA Katharine Berry Richardson Professor of Surgery Senior Vice-President The Children’s Mercy Hospital Kansas City, MO, USA

J. Patrick Murphy, MD Professor of Surgery Department of Surgery The Children’s Mercy Hospital Kansas City, MO, USA

Shawn D. St. Peter, MD

Surgeon-in-Chief Thomas Holder and Keith Ashcraft Endowed Chair Director, Pediatric Surgery Fellowship Director, Center for Prospective Trials Professor of Surgery The Children’s Mercy Hospital Kansas City, MO, USA

Associate Editor John M. Gatti, MD Professor of Surgery Chief, Section of Urology The Children’s Mercy Hospital Kansas City, MO, USA

For additional online content visit ExpertConsult.com

Edinburgh London New York Oxford Philadelphia St Louis Sydney 2020

iii

© 2020, Elsevier Inc. All rights reserved. First edition 1980 Second edition 1993 Third edition 2000 Fourth edition 2005 Fifth edition 2010 Sixth edition 2014 Seventh edition 2020 No part of this publication may be reproduced or transmitted in any form or by any means, electronic or mechanical, including photocopying, recording, or any information storage and retrieval system, without permission in writing from the publisher. Details on how to seek permission, further information about the Publisher’s permissions policies and our arrangements with organizations such as the Copyright Clearance Center and the Copyright Licensing Agency, can be found at our website: www.elsevier.com/permissions. This book and the individual contributions contained in it are protected under copyright by the Publisher (other than as may be noted herein). Notices Practitioners and researchers must always rely on their own experience and knowledge in evaluating and using any information, methods, compounds or experiments described herein. Because of rapid advances in the medical sciences, in particular, independent verification of diagnoses and drug dosages should be made. To the fullest extent of the law, no responsibility is assumed by Elsevier, authors, editors, or contributors for any injury and/or damage to persons or property as a matter of products liability, negligence or otherwise, or from any use or operation of any methods, products, instructions, or ideas contained in the material herein. Library of Congress Control Number: 2018956545 ISBN: 978-0-323-54940-0 Ebook ISBN: 978-0-323-54976-9 Inkling ISBN: 978-0-323-54977-6 Printed in China Last digit is the print number: 

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Content Strategist: Russell Gabbedy Content Development Specialist: Nani Clansey Project Manager: Julie Taylor Design: Ryan Cook Illustration Manager: Teresa McBryan Marketing Manager: Claire McKenzie

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Contents SECTION I General

16  Abdominal and Renal Trauma,  236

1 Physiology of the Newborn,  2

17 Traumatic Brain Injury,  254

DAVID M. NOTRICA

KELLY S. TIEVES and JAY RILINGER

MARIYA E. SKUBE, BRADLEY J. SEGURA, and DANIEL A. SALTZMAN

2  Nutritional Support for the Pediatric Patient,  18

18  Pediatric Orthopedic Trauma,  267 RICHARD SCHWEND

CHARLES HONG, NILESH M. MEHTA, and TOM JAKSIC

3  Anesthetic Considerations for Pediatric Surgical Conditions,  35

19  Neurosurgical Conditions,  285   

PAUL GRABB

LAURA K. DIAZ and LYNNE G. MAXWELL

4 Renal Impairment and Renovascular Hypertension,  57

URI S. ALON, NATHAN BEINS, and BRADLEY A. WARADY

5  Coagulopathies and Sickle Cell Disease,  76 NAZIA TABASSUM IQBAL, BRIAN M. WICKLUND, and GERALD M. WOODS

6 Extracorporeal Membrane Oxygenation,  91 JOSEPH T. CHURCH and GEORGE B. MYCHALISKA

7  Mechanical Ventilation in Pediatric Surgical Disease,  111

SAMIR K. GADEPALLI and RONALD B. HIRSCHL

8  Vascular Access,  133 RAVINDRA K. VEGUNTA

9 Surgical Infectious Disease,  141 RICHARD SOLA JR. and TOLULOPE OYETUNJI

10  Fetal Therapy,  153 KUOJEN TSAO, DIANA FARMER, and SHINJIRO HIROSE

SECTION III Thoracic 20  Chest Wall Deformities,  302 ROBERT E. KELLY, JR. and MARCELO MARTINEZ-FERRO

21 Management of Laryngotracheal Obstruction in Children,  332

ANDRÉ HEBRA and PATRICIO VARELA

22  Congenital Bronchopulmonary Malformations,  348

PABLO LAJE and ALAN W. FLAKE

23 Acquired Lesions of the Lung and Pleura,  361 SHAWN D. ST. PETER

24  Congenital Diaphragmatic Hernia and Eventration,  377

MATTHEW T. HARTING, LAURA E. HOLLINGER, and KEVIN P. LALLY

25  Mediastinal Tumors,  403 JUAN A. TOVAR and LEOPOLDO MARTINEZ

SECTION II Trauma

26  The Esophagus,  422

11  Ingestion of Foreign Bodies,  172

27  Esophageal Atresia and Tracheoesophageal

SOHAIL R. SHAH and DANNY C. LITTLE

12 Bites,  181 ERIC ROSENFELD, SPENCER GREENE, and BINDI NAIK-MATHURIA

13 Burns,  196 PABLO AGUAYO and DAVID JUANG

14 Early Assessment and Management of Trauma,  211 ARTHUR COOPER

15  Thoracic Trauma,  224 DEIDRE L. WYRICK and R. TODD MAXSON

CHRISTOPHER SNYDER, PAUL M. COLOMBANI, and NICOLE CHANDLER

Fistula Malformations,  437 STEVEN S. ROTHENBERG

SECTION IV Abdomen 28  Gastroesophageal Reflux,  460 GEORGE W. HOLCOMB III

29  Lesions of the Stomach,  478 JUSTIN A. SOBRINO and MARK WULKAN

30  Duodenal and Intestinal Atresia and Stenosis,  489 SARAH B. OGLE, PETER F. NICHOL, and DANIEL J. OSTLIE

v

vi

Contents

31  Malrotation,  507 PATRICK C. BONASSO II, M. SIDNEY DASSINGER III, and SAMUEL D. SMITH

32  Meconium Disease,  517 MAURICIO A. ESCOBAR JR. and MICHAEL G. CATY

33  Necrotizing Enterocolitis,  536 JEREMY G. FISHER and R. LAWRENCE MOSS

34 Hirschsprung Disease,  557 JACOB C. LANGER

35  Anorectal Atresia and Cloacal Malformations,  577

REBECCA M. RENTEA and MARC A. LEVITT

36  Fecal Incontinence and Constipation,  599 RICHARD J. WOOD and MARC A. LEVITT

37  Acquired Anorectal Disorders,  613 VERONICA F. SULLINS, MARCUS JARBOE, and CASEY M. CALKINS

38 Intussusception,  621 TIFFANY N. WRIGHT and MARY E. FALLAT

39  Alimentary Tract Duplications,  629 KATIE W. RUSSELL and GEORGE W. HOLCOMB III

40  Meckel Diverticulum,  641 CHARLES M. LEYS

41  Inflammatory Bowel Disease,  647 CRISTINE S. VELAZCO, LISA MCMAHON, and DANIEL J. OSTLIE

42  Appendicitis,  664 SHAWN D. ST. PETER and TOMAS WESTER

43  Biliary Atresia,  679 JOEL CAZARES, BENNO URE, and ATSUYUKI YAMATAKA

44  Choledochal Cyst and Gallbladder Disease,  695 NGUYEN THANH LIEM, LEO ANDREW BENEDICT, and GEORGE W. HOLCOMB III

45  Solid Organ Transplantation in Children,  709 ALEXANDER J. BONDOC, JAIMIE D. NATHAN, MARIA H. ALONSO, and GREGORY M. TIAO

46  Lesions of the Pancreas,  737 JOSEPH C. FUSCO, MARCUS M. MALEK, and GEORGE K. GITTES

47 Splenic Conditions,  750 FREDERICK J. RESCORLA and ROBERT J. VANDEWALLE

48  Congenital Abdominal Wall Defects,  763 SALEEM ISLAM

49  Umbilical and Other Abdominal Wall Hernias,  780

JOSEPH A. SUJKA and GEORGE W. HOLCOMB III

SECTION V Inguinal Region and Scrotum 50  Inguinal Hernia,  784 CHARLES L. SNYDER, MARIA ESCOLINO, and CIRO ESPOSITO

51  Undescended Testes and Testicular Tumors,  805 PAUL R. BOWLIN and ARMANDO J. LORENZO

52 The Acute Scrotum,  821 JOHN M. GATTI and JASON AXT

SECTION VI Urology 53  Developmental and Positional Anomalies of the Kidneys,  828

HSI-YANG WU and HOWARD M. SNYDER III

54 Ureteral Obstruction and Malformations,  837 JOEL F. KOENIG and DOUGLAS E. COPLEN

55  Urinary Tract Infections and Vesicoureteral Reflux,  853

W. ROBERT DEFOOR JR., EUGENE MINEVICH, and CURTIS A. SHELDON

56  Bladder and Urethra,  870 PATRICK C. CARTWRIGHT, BRENT W. SNOW, M. CHAD WALLIS, and GLEN A. LAU

57  Posterior Urethral Valves,  884 JACK S. ELDER and ELLEN SHAPIRO

58  Bladder and Cloacal Exstrophy,  897 DANA A. WEISS, DOUGLAS A. CANNING, JOSEPH G. BORER, JOHN V. KRYGER, ELIZABETH ROTH, and MICHAEL E. MITCHELL

59  Hypospadias,  918 ALONSO CARRASCO JR. and J. PATRICK MURPHY

60 Circumcision,  935 JONATHAN C. PAPIC and STEPHEN C. RAYNOR

61  Prune Belly Syndrome,  942 MARCOS PEREZ-BRAYFIELD and VANESSA ORTIZ-HERNANDEZ

62  Differences of Sexual Development,  953 JOHN M. GATTI, TAZIM DOWLUT-MCELROY, and LAUREL WILLIG

SECTION VII Neoplasms 63  Principles of Adjuvant Therapy in Childhood Cancer,  968

DANIEL VON ALLMEN

64  Renal Tumors,  986 PETER EHRLICH and ROBERT C. SHAMBERGER

Contents

65  Neuroblastoma,  1010 ANDREW M. DAVIDOFF and JED G. NUCHTERN

66  Lesions of the Liver,  1031 WALTER S. ANDREWS, BARTHOLOMEW KANE, and RICHARD J. HENDRICKSON

71 Vascular Anomalies,  1147 EILEEN M. DUGGAN and STEVEN J. FISHMAN

72 Head and Neck Sinuses and Masses,  1171 MATTHEW B. DELLINGER and JOHN H.T. WALDHAUSEN

Tumors,  1066

SECTION IX Special Topics

JEAN-MARTIN LABERGE, PRAMOD S. PULIGANDLA, and KENNETH SHAW

73  Pediatric and Adolescent Gynecology,  1188

67  Teratomas, Dermoids, and Other Soft Tissue

68  Lymphomas,  1097 KAREN B. LEWING and KEITH J. AUGUST

69  Rhabdomyosarcoma,  1115 JUAN P. GURRIA and ROSHNI DASGUPTA

JULIE STRICKLAND and S. PAIGE HERTWICK

74  Breast Diseases,  1206 DON K. NAKAYAMA

75  Endocrine Disorders and Tumors,  1217 HANNA ALEMAYEHU and JASON D. FRASER

SECTION VIII Skin and Soft Tissue Diseases 70 Nevus and Melanoma,  1128 EMMA C. HAMILTON and MARY T. AUSTIN

76  Bariatric Surgical Procedures in Adolescence,  1240

LINDEL C. DEWBERRY, MICHAEL HELMRATH, and THOMAS H. INGE

Index,  1253

vii

Video Table of Contents 6  Extracorporeal Membrane Oxygenation 6.1  Percutaneous Placement of a Double-Lumen Cannula for DLVV-ECLS in a Neonate DONALD LIU MD, JOSEPH CHURCH MD, RONALD HIRSCHL MD, GEORGE MYCHALISKA MD

9  Surgical Infectious Disease 9.1 Placement of a Wound VAC in an Infant Following Left Congenital Diaphragmatic Hernia Repair Using Mesh RICHARD SOLA, JR. MD, TOLULOPE OYETUNJI MD

10  Fetal Therapy 10.1 Fetal Endoluminal Tracheal Occlusion (FETO) - Insertion DIANA FARMER MD, HANMIN LEE MD, SHINJIRO HIROSE

10.2  Fetal Endoluminal Tracheal Obstruction – Removal at 34 Weeks DIANA FARMER MD, HANMIN LEE MD, SHINJIRO HIROSE MD

10.3  In Utero Myelomeningocele Repair DIANA FARMER MD, HANMIN LEE MD, SHINJIRO HIROSE MD

10.4  Laser Ablation Twin-Twin Transfusion Syndrome DIANA FARMER MD, HANMIN LEE MD, SHINJIRO HIROSE MD

20 Congenital Chest Wall Deformities 20.1 The Nuss Procedure DONALD NUSS MD, ROBERT KELLY JR, MD

22  Congenital Bronchopulmonary Malformations 22.1 Thoracoscopic Right Lower Lobectomy for a CCAM STEVEN S. ROTHENBERG MD

23  Acquired Lesions of the Lung and Pleura 23.1 Thoracoscopic Lung Biopsy Using the Endoscopic Stapler and Using a Loop Ligature STEVEN S. ROTHENBERG MD

23.2 Thoracoscopic Debridement and Decortication for Empyema GEORGE W. HOLCOMB III MD

23.3 Thoracoscopic Right Middle Lobectomy GEORGE W. HOLCOMB III MD

24  Congenital Diaphragmatic Hernia and Eventration

24.1

 aparoscopic Plication of the Right Hemi-Diaphragm L STEVEN S. ROTHENBERG MD

25 Mediastinal Tumors 25.1 Thoracoscopic Biopsy of an Anterior Mediastinal Mass GEORGE W. HOLCOMB III MD, DANNY LITTLE MD

viii

26 The Esophagus 26.1 Laparoscopic Esophagomyotomy GEORGE W. HOLCOMB III MD 26.2 Single Incision Laparoscopic Heller Myotomy and Dor Fundoplication for Achalasia NICOLE CHANDLER MD, PAUL COLOMBANI MD

27  Esophageal Atresia and Tracheoesophageal Fistula Malformations

27.1 Thoracoscopic Repair of a Type 3 Esophageal Atresia and Tracheoesophageal Fistula Using the JRS 3mm Sealer STEVEN S. ROTHENBERG MD, SOPHIA ABDULHAI MD

27.2 Thoracoscopic Ligation of an H-type Tracheoesophageal Fistula Using the 5mm Stapler STEVEN S. ROTHENBERG MD, SOPHIA ABDULHAI MD

28 Gastroesophageal Reflux 28.1 Laparoscopic Fundoplication: Minimal Esophageal

Dissection and Placement of Esophago-Crural Sutures GEORGE W. HOLCOMB III MD, SHAWN D. ST. PETER MD

28.2 Laparoscopic Thal Fundoplication DANIEL J. OSTLIE MD, KUOJEN TSAO MD, GEORGE W. HOLCOMB III MD 28.3 The Use of Surgisis for Hiatal Reinforcement at Re-Do Laparoscopic Fundoplication and Antroplasty GEORGE W. HOLCOMB III MD, KUOJEN TSAO MD 28.4 Laparoscopic Gastrostomy KUOJEN TSAO MD, GEORGE W. HOLCOMB III MD

29  Lesions of the Stomach 29.1 Laparoscopic Repair of Pyloric Atresia DAVID JUANG MD, GEORGE W. HOLCOMB III MD

29.2 Laparoscopic Pyloromyotomy MARK L. WULKAN MD

30  Duodenal and Intestinal Atresia and Stenosis 30.1 Laparoscopic Repair of Duodenal Atresia and Ladd’s

Procedure and Meckel’s Diverticulectomy in a Newborn Using the JRS 3mm Sealer and 5mm Stapler STEVEN S. ROTHENBERG MD

30.2 Laparoscopic Intra-Corporeal Management of a Jejunal Atresia With an Apple Peel Deformity Using a 5mm Endoscopic Stapler STEVEN S. ROTHENBERG MD

34  Hirschsprung Disease 34.1 Trans-Anal Pullthrough MARC A. LEVITT MD

35  Anorectal Atresia and Cloacal Malformations 35.1 Repair of a Male Infant With Anorectal Atresia and a Recto-Bulbar Fistula VICTORIA A. LANE MD, CARLOS RECK MD, RICHARD J. WOOD MD, MARC A. LEVITT MD

Video Table of Contents 35.2 Repair of Anorectal Atresia in a Female Without a Urinary Fistula VICTORIA A. LANE MD, CARLOS RECK MD, RICHARD J. WOOD MD, MARC A. LEVITT MD

52  The Acute Scrotum

35.3 Repair of a Short, Cloacal Channel Malformation Using Total Urogenital Mobilization VICTORIA A. LANE MD, CARLOS RECK MD, RICHARD J. WOOD MD, MARC A. LEVITT MD

55  Urinary Tract Infection and Vesicoureteral

36  Fecal Incontinence and Constipation 36.1 Appendicostomy for Antegrade Enemas for Patients with Fecal Incontinence MARC A. LEVITT MD, ALBERTO PENA MD

43  Biliary Atresia 43.1 Laparoscopic Kasai ATSUYUKI YAMATAKA MD

44  Choledochal Cyst and Gallbladder Disease 44.1 Laparoscopic Excision of a Choledochal Cyst With Hepatico-Jejunostomy NJUYEN-THANH-LIEM MD 44.2 Laparoscopic Cholecystectomy GEORGE W. HOLCOMB III MD

46  Lesions of the Pancreas

52.1 Management of a Torsed Left Testis PAUL BOWLIN MD, JASON AXT MD

Reflux

55.1 Robotic Extravesical Ureteral Reimplantation EUGENE MINEVICH MD, BOB DEFOOR MD, CURTIS A. SHELDON MD 55.2 Double Hydrodistention Implantation Technique (HIT) Method for Vesicoureteral Reflux (VUR) ANGELA M. ARLEN MD, HAL C. SCHERZ MD, ANDREW G. KIRSCH MD

57  Posterior Urethral Valves 57.1 Endoscopic Ablation of Posterior Urethral Valves JACK ELDER MD, ELLEN SHAPIRO MD

70 Nevus and Melanoma 70.1 S  entinel Lymph Node Biopsy for Cutaneous Melanoma JEFFREY E. GERSCHENWALD MD, MERRICK E. ROSS MD

73  Pediatric and Adolescent Gynecology 73.1 Laparoscopic Excision of an Ovarian Teratoma TRACY E. ITO MD, S. PAIGE HERTWECK MD

46.1 Laparoscopic Cyst – Gastrostomy DANIEL J. OSTLIE MD

73.2 L  aparoscopic Management of Ovarian Torsion TRACY E. ITO MD, S. PAIGE HERTWECK MD

46.2 Laparoscopic Distal Pancreatectomy for Trauma GEORGE GITTES MD, JOSEPH FUSCO MD, MARCUS M. MALEK MD

73.3 Laparoscopic Oophorectomy for Fertility Preservation JULIE L. STRICKLAND MD

47  Splenic Conditions 47.1 Laparoscopic Splenectomy FREDERICK J. RESCORLA, MD 47.2 Laparoscopic Resection of a Splenic Cyst KUOJEN TSAO MD, GEORGE W. HOLCOMB III MD

50  Inguinal Hernia 50.1 Laparoscopic Inguinal Hernia Repair C.K. YEUNG MD 50.2 Laparoscopic Inguinal Hernia Repair CIRO ESPOSITO MD, MARIA ESCOLINO MD

51  Undescended Testes and Testicular Tumors 51.1 T  wo- Staged Laparoscopic Orchiopexy for a Left Non-Palpable Testis GEORGE W. HOLCOMB III MD

73.4 Technique for Identifying Endometriosis in the Adolescent Population TRACY E. ITO MD, S. PAIGE HERTWECK MD 73.5 Non-Communication Uterine Horn: The Laparoscopic Approach JULIE L. STRICKLAND MD 73.6 Prepubertal EUA and Vaginoscopy JULIE STRICKLAND MD

75  Endocrine Disorders and Tumors 75.1 Laparoscopic Right Adrenalectomy GEORGE W. HOLCOMB III, MD

76 Bariatric Surgical Procedures in Adolescents 76.1 Laparoscopic Sleeve Gastrectomy LINDEL C. DEWBERRY MD, THOMAS H. INGE MD

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Preface Welcome to the Seventh Edition of this textbook, which was originally conceived and developed by Drs. Tom Holder and Keith Ashcraft. The first edition was published in 1980. We continue to hold dearly several tenets of this book: readability, an international perspective, an emphasis on general pediatric surgery and urology, and portability. In the Preface of the Second Edition, Drs. Holder and Ashcraft wrote, “Our intent is to provide a book that has a clear explanation of a subject done in a readable style.” Our readers live all over the world, and, for this reason, we have many authors who practice outside the United States. Also, urology is a significant part of an international pediatric surgeon’s practice, and we have continued to have a relatively large portion of the book devoted to pediatric urology. Finally, for ease of access to the book, it remains a single volume book and is available online. In this edition, we have a number of new authors and many returning authors. There has been significant updating of the text and illustrations. All the illustrations are in color. Also, a large number of figures are new to this edition. Finally, there are over 50 videos that accompany this book and are designed to help the reader better understand

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many of the operative techniques described in the chapters. These videos are accessible via our Expert Consult website (expertconsult.com). The code in the front of this book allows access to the online version of the book and the videos. We are very pleased to acknowledge Mrs. Barbara Juarez, Mrs. Linda Jankowski, and Mrs. Jeannette Whitney, who have been instrumental in the behind-the-scenes production of the last several editions of this book. All three will be retiring soon, and we are truly indebted to their tireless efforts. Their many contributions to our hospital and the editors will be dearly missed in the future. This book is dedicated to these three wonderful colleagues. Again, we are pleased to offer this book for your education and, hopefully, enjoyment. We look forward to interacting with many of you over the next several years. George W. Holcomb III, MD, MBA J. Patrick Murphy, MD Shawn D. St. Peter, MD John M. Gatti, MD

List of Contributors The editor(s) would like to acknowledge and offer grateful thanks for the input of all previous editions’ contributors, without whom this new edition would not have been possible.

Pablo Aguayo, MD

Assistant Professor, Department of Surgery, Children’s Mercy Hospital, The University of Missouri–Kansas City School of Medicine, Kansas City, MO, USA Chapter 13: Burns

Hanna Alemayehu, MD

Assistant Professor of Surgery, Assistant Professor of Pediatrics, Pediatric Surgeon, Children’s and Women’s Hospital, University of South Alabama, Mobile, AL USA Chapter 75: Endocrine Disorders and Tumors

Uri S. Alon, MD

Professor of Pediatrics, Division of Pediatric N ­ ephrology, Children’s Mercy Hospital, The University of ­Missouri–Kansas City School of Medicine, Kansas City, MO, USA Chapter 4: Renal Impairment and Renovascular Hypertension

Maria H. Alonso, MD

Associate Professor of Surgery and Pediatrics, Division of Pediatric General and Thoracic Surgery, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA Chapter 45: Solid Organ Transplantation in Children

Walter S. Andrews, MD

Professor of Pediatric Surgery, Surgical Director of Transplant, Department of Pediatric Surgery, Children’s Mercy Hospital, Kansas City, MO, USA Chapter 66: Lesions of the Liver

Keith J. August, MD, MS

Associate Professor of Pediatrics, Division of Pediatric Hematology/Oncology, Children’s Mercy Hospital, Kansas City, MO, USA Chapter 68: Lymphomas

Mary T. Austin, MD, MPH

Nathan Beins, MHPE, MD

Assistant Professor of Pediatrics, Division of Pediatric Nephrology, Children’s Mercy Hospital, University of Missouri–Kansas City School of Medicine, Kansas City, MO, USA Chapter 4: Renal Impairment and Renovascular ­Hypertension

Leo Andrew Osifuye Benedict, MD

Trauma, Critical Care, and Acute Care Surgeon; Director, Trauma Surgery Research, Department of Surgery, St. Luke’s Hospital of Kansas City; Clinical Assistant Professor of Surgery, The University of Missouri–Kansas City School of Medicine, Kansas City, MO, USA Chapter 44: Choledochal Cyst and Gallbladder Disease

Patrick C. Bonasso II, MD

Research Fellow, Department of Pediatric Surgery, University of Arkansas for Medical Sciences; Little Rock, AR, USA Chapter 31: Malrotation

Alexander J. Bondoc, MD

Assistant Professor of Surgery and Pediatrics, Division of Pediatric General and Thoracic Surgery, Cincinnati Children’s Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, OH, USA Chapter 45: Solid Organ Transplantation in Children

Joseph G. Borer, MD

Director, Bladder Exstrophy Program; Co-Director, Neurourology and Urodynamics; Reconstructive Urologic Surgery Chair, Boston Children’s Hospital; Associate Professor of Surgery (Urology), Harvard Medical School, Boston, MA, USA Chapter 58: Bladder and Cloacal Exstrophy

Paul R. Bowlin, MD

Associate Professor, Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA Chapter 70: Nevus and Melanoma

Assistant Professor, Section of Urology, Children’s Mercy Hospital, University of Missouri–Kansas City School of Medicine, Kansas City, MO, USA Chapter 51: Undescended Testes and Testicular Tumors, Video 52.1 Management of a Torsed Left Testis

Jason Axt

Douglas A. Canning, MD

Attending Pediatric Surgeon, Mbingo Baptist Hospital, Mbingo, Cameroon Chapter 52: The Acute Scrotum, Video 52.1 Management of a Torsed Left Testis

Professor of Urology and Surgery, Perelman School of Medicine, University of Pennsylvania; Chief, Pediatric Urology, Children’s Hospital of Philadelphia, Philadelphia, PA, USA Chapter 58: Bladder and Cloacal Exstrophy

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List of Contributors

Casey M. Calkins, MD

Professor of Surgery, Division of Pediatric Surgery, Medical College of Wisconsin, Milwaukee, WI, USA Chapter 37: Acquired Anorectal Disorders

Alonso Carrasco Jr., MD

Assistant Professor, Section of Urology, Children’s Mercy Hospital, University of Missouri–Kansas City School of Medicine, Kansas City, MO, USA Chapter 59: Hypospadias

Patrick C. Cartwright, MD

Professor and Chief, Division of Urology; Surgeon-in-Chief, Primary Children’s Hospital, University of Utah School of Medicine, Salt Lake City, UT, USA Chapter 56: Bladder and Urethra

Michael G. Caty, MD, MMM

Robert Pritzker Professor of Surgery and Chief, Section of Pediatric Surgery, Yale University School of Medicine; Surgeon-in-Chief, Yale New Haven Children’s Hospital, New Haven, CT, USA Chapter 32: Meconium Disease

Joel Cazares, MD

Assistant Professor, Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, Tokyo, Japan Chapter 43: Biliary Atresia

Nicole Chandler, MD

Pediatric Surgeon, Johns Hopkins All Children’s Hospital; Assistant Professor, Department of Surgery, Johns Hopkins School of Medicine, St. Petersburg, FL, USA Chapter 26: The Esophagus, Video 26.2 Single Incision Laparoscopic Heller Myotomy and Dor Fundoplication for Achalasia

Joseph T. Church, MD

Pediatric Surgery Fellow, C.S. Mott Children’s Hospital, Michigan Medicine, Ann Arbor, MI, USA Chapter 6: Extracorporeal Membrane Oxygenation, Video 6.1 Percutaneous Placement of a Double-Lumen Cannula for DLVV-ECLS in a Neonate

Paul M. Colombani, MD, MBA

Chair of Surgery, Johns Hopkins All Children’s Hospital, St. Petersburg, FL; Professor of Surgery, Pediatrics, and Oncology, The Johns Hopkins University School of Medicine, Baltimore, MD, USA Chapter 26: The Esophagus, Video 26.2 Single Incision Laparoscopic Heller Myotomy and DOR Fundoplication for Achalasia

Arthur Cooper, MD, MS

Professor of Surgery, Columbia University Vagelos College of Physicians and Surgeons; Director of Pediatric Surgical and Trauma Services, New York City Health Hospitals, Harlem, New York, NY, USA Chapter 14: Early Assessment and Management of Trauma

Douglas E. Coplen, MD

Associate Professor and Director of Pediatric Urology, Division of Pediatric Urology, Washington University School of Medicine and St. Louis Children’s Hospital, St. Louis, MO, USA Chapter 54: Ureteral Obstruction and Malformations

Roshni Dasgupta, MD, MPH

Associate Professor, Division of Pediatric General and Thoracic Surgery, Cincinnati Children’s Hospital Medical Center, University of Cincinnati School of Medicine, Cincinnati, OH, USA Chapter 69: Rhabdomyosarcoma

M. Sidney Dassinger III, MD

Associate Professor of Pediatric Surgery, University of Arkansas for Medical Sciences, Arkansas Children’s Hospital, Little Rock, AR, USA Chapter 31: Malrotation

Andrew M. Davidoff, MD

Chairman, Department of Surgery, St. Jude Children’s Research Hospital, Memphis, TN, USA Chapter 65: Neuroblastoma

W. Robert DeFoor Jr., MD, MPH

Professor of Surgery (Urology), Division of Pediatric Urology, Cincinnati Children’s Hospital, Cincinnati, Ohio, USA Chapter 55: Urinary Tract Infections and Vesicoureteral Reflux

Matthew B. Dellinger, MD

Clinical Research Fellow, Pediatric General and Thoracic Surgery, Seattle Children’s Hospital, Seattle, WA, USA Chapter 72: Head and Neck Sinuses and Masses

Lindel C. Dewberry, MD

Surgery Resident, Department of Surgery, University of Colorado, Aurora, CO, USA Chapter 76: Bariatric Surgical Procedures in Adolescence, Video 76.1 Laparoscopic Sleeve Gastrectomy

Laura K. Diaz, MD

Pediatric Anesthesiologist, Department of Anesthesiology and Critical Care Medicine, The Children’s Hospital of Philadelphia; Assistant Professor of Anesthesiology and Critical Care, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA Chapter 3: Anesthetic Considerations for Pediatric Surgical Conditions

Tazim Dowlut-McElroy, MD, MS

Assistant Professor, Department of Obstetrics and Gynecology, University of Missouri–Kansas City School of Medicine; Department of Surgery, Children’s Mercy Hospital, Kansas City, MO, USA Chapter 62: Differences of Sexual Development

List of Contributors

Eileen M. Duggan, MD

Pediatric Surgery Fellow, Montreal Children’s Hospital, Montreal, Quebec, Canada Chapter 71: Vascular Anomalies

Peter Ehrlich, MD, MSc

Professor of Surgery; Vice Chair Surgery, Children’s Oncology Group, Section of Pediatric Surgery, University of Michigan School of Medicine, Ann Arbor, MI, USA Chapter 64: Renal Tumors

Jack S. Elder, MD

Chief, Division of Pediatric Urology, MassGeneral Hospital for Children, Harvard Medical School, Boston, MA, USA Chapter 57: Posterior Urethral Valves, Video 57.1 Endoscopic Ablation of Posterior Urethral Valves

Mauricio A. (Tony) Escobar Jr., MD

Chief of Staff; Medical Director, Pediatric Surgery and Trauma, Department of Pediatric Surgery and Pediatric Trauma, Mary Bridge Children’s Hospital and Health Network; Clinical Associate Professor, Department of Surgery, University of Washington, Tacoma, WA, USA Chapter 32: Meconium Disease

Maria Escolino, MD

University Researcher in Pediatric Surgery, Department of Translational Medical Sciences (DISMET), Pediatric Surgery Unit “Federico II”, University of Naples School of Medicine, Naples, Italy Chapter 50: Inguinal Hernia, Video 50.2 Laparoscopic Inguinal Hernia Repair

Ciro Esposito, MD, PhD, MFAS

Professor of Pediatric Surgery, Department of Translational Medical Sciences (DISMET), Pediatric Surgery Unit “Federico II”, University of Naples School of Medicine, Naples, Italy Chapter 50: Inguinal Hernia, Video 50.2 Laparoscopic Inguinal Hernia Repair

Mary E. Fallat, MD

Hirikati S. Nagaraj Professor and Division Director of Pediatric Surgery, Hiram C. Polk Jr. Department of Surgery, University of Louisville School of Medicine, Louisville, KY, USA Chapter 38: Intussusception

Diana Farmer, MD

Distinguished Professor and Chair, Department of Surgery; Surgeon-in-Chief, UC Davis Children’s Hospital, University of California, Davis Health System, Sacramento, CA, USA Chapter 10: Fetal Therapy, Video 10.1 Fetal Endoluminal Tracheal Occlusion (FETO) - Insertion, Video 10.2 Fetal Endoluminal Tracheal Obstruction – Removal at 34 Weeks, Video 10.3 In Utero Myelomeningocele Repair, Video 10.4 Laser Ablation Twin-Twin Transfusion Syndrome

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Alan W. Flake, MD

Professor of Surgery, University of Pennsylvania Perelman School of Medicine; Ruth and Tristram C. Colket, Jr. Chair in Pediatric Surgery; Vice Chair of Research, Department of Surgery; Director, Children’s Center for Fetal Research, Children’s Hospital of Philadelphia, Philadelphia, PA, USA Chapter 22: Congenital Bronchopulmonary Malformations

Jeremy G. Fisher, MD

Assistant Professor, Department of Pediatric Surgery, Nationwide Children’s Hospital, Ohio State University, Columbus, OH, USA Chapter 33: Necrotizing Enterocolitis

Steven J. Fishman, MD

Stuart and Jane Weitzman Family Chair in Surgery, Boston Children’s Hospital; Professor of Surgery, Harvard Medical School, Boston, MA, USA Chapter 71: Vascular Anomalies

Jason D. Fraser, MD

Director, Bariatric Surgery; Director, General Surgery ­Residents; Assistant Professor, Department of Surgery, Children’s Mercy Hospital, University of Missouri–­ Kansas City School of Medicine, Kansas City, MO, USA Chapter 75: Endocrine Disorders and Tumors

Joseph Fusco, MD

Pediatric Surgery Research Fellow, Division of Pediatric General and Thoracic Surgery, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA Chapter 46: Lesions of the Pancreas, Video 46.2 Laparoscopic Distal Pancreatectomy for Trauma

Samir K. Gadepalli, MSc, MD, MBA

Assistant Professor, Pediatric Surgery and Surgical Critical Care, Section of Pediatric Surgery, Department of Surgery, C.S. Mott Children’s Hospital, University of Michigan, Ann Arbor, MI, USA Chapter 7: Mechanical Ventilation in Pediatric Surgical Disease

John M. Gatti, MD

Professor and Chief, Section of Urology, Children’s Mercy Hospital, University of Missouri–Kansas City School of Medicine, Kansas City, MO, USA Chapter 52: The Acute Scrotum, Chapter 62: Differences of Sexual Development

George K. Gittes, MD

Benjamin R. Fisher Chair, Professor of Pediatric Surgery, Chief of Pediatric General and Thoracic Surgery, Department of Surgery, University of Pittsburgh School of Medicine; Surgeon-in-Chief Emeritus, UPMC Children’s Hospital of Pittsburgh; Director, Richard King Mellon Foundation Institute for Pediatric Research; Director of Surgical Research and Scientific Co-Director of all Research, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA Chapter 46: Lesions of the Pancreas, Video 46.2 Laparoscopic Distal Pancreatectomy for Trauma

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List of Contributors

Paul A. Grabb, MD

Chief, Section of Neurosurgery, Department of Surgery, Children’s Mercy Hospital, Kansas City, MO, USA Chapter 19: Neurosurgical Conditions

Spencer Greene, MD, MS

Director of Medical Toxicology, Assistant Professor, Henry J.N. Taub Department of Emergency Medicine, Baylor College of Medicine; Consulting Toxicologist, Southeast Texas Poison Center, Houston, TX, USA Chapter 12: Bites

Juan P. Gurría, MD, MS

Shinjiro Hirose, MD

Chief, Division of Pediatric General, Thoracic, and Fetal Surgery; Vice Chair, Department of Surgery, University of California, Davis Health System, Sacramento, CA, USA Chapter 10: Fetal Therapy, Video 10.1 Fetal Endoluminal Tracheal Occlusion (FETO) - Insertion, Video 10.2 Fetal Endoluminal Tracheal Obstruction – Removal at 34 Weeks, Video 10.3 In Utero Myelomeningocele Repair, Video 10.4 Laser Ablation Twin-Twin Transfusion Syndrome

Ronald B. Hirschl, MS, MD

Clinical Fellow, Pediatric Surgery, Division of General and Thoracic Pediatric Surgery, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA Chapter 69: Rhabdomyosarcoma

Professor of Pediatric Surgery, University of Michigan, Ann Arbor, MI, USA Chapter 7: Mechanical Ventilation in Pediatric Surgical Disease, Video 6.1 Percutaneous Placement of a Double-Lumen Cannula for DLVV-ECLS in a Neonate

Emma C. Hamilton, MD

George W. Holcomb III, MD

Trauma and Acute Care Surgeon, St. Marks Hospital, Salt Lake City, UT, USA Chapter 70: Nevus and Melanoma

Matthew T. Harting, MD, MS

Assistant Professor, Department of Pediatric Surgery, McGovern Medical School at the University of Texas Health Science Center, and Children’s Memorial ­Hermann Hospital, Houston, TX, USA Chapter 24: Congenital Diaphragmatic Hernia and ­Eventration

Andre Hebra, MD

Chief Medical Officer, Professor of Surgery, Nemours Children’s Hospital, University of Central Florida College of Medicine, Orlando, FL, USA Chapter 21: Management of Laryngotracheal Obstruction in Children

Michael Helmrath, MD

Richard and Geralyn Azizkhan Professor of Surgery, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA Chapter 76: Bariatric Surgical Procedures in Adolescence

Richard J. Hendrickson, MD

Associate Professor of Surgery, University of Missouri– Kansas City; Department of Pediatric and Transplant Surgery, Children’s Mercy Hospital, Kansas City, MO, USA Chapter 66: Lesions of the Liver

S. Paige Hertweck, MD

Chief of Gynecology, Norton Children’s Hospital; Clinical Professor of Pediatrics and Obstetrics, Gynecology, and Women’s Health, University of Louisville School of Medicine, Louisville, KY, USA Chapter 73: Pediatric and Adolescent Gynecology, Video 73.1 Laparoscopic Excision of an Ovarian Teratoma, Video 73.2 Laparoscopic Management of Ovarian Torsion, Video 73.4 Technique for Identifying Endometriosis in the Adolescent Population

Katharine B. Richardson Professor of Surgery and Senior Vice-President, Children’s Mercy Hospital, Kansas City, MO, USA Chapter 28: Gastroesophageal Reflux, Chapter 39: Alimentary Tract Duplications, Chapter 44: Choledochal Cyst and Gallbladder Disease, Chapter 49: Umbilical and Other Abdominal Wall Hernias, Video 23.2 Thoracoscopic Debridement and Decortication for Empyema, Video 23.3 Thoracoscopic Right Middle Lobectomy, Video 25.1 Thoracoscopic Biopsy of an Anterior Mediastinal Mass, Video 26.1 Laparoscopic Esophagomyotomy, Video 28.1 Laparoscopic Fundoplication: Minimal Esophageal Dissection and Placement of Esophago-Crural Sutures, Video 28.2 Laparoscopic Thal Fundoplication, Video 28.3 The Use of Surgisis for Hiatal Reinforcement at Re-Do Laparoscopic Fundoplication and Antroplasty, Video 28.4 Laparoscopic Gastrostomy, Video 29.1 Laparoscopic Repair of Pyloric Atresia, Video 44.2 Laparoscopic Cholecystectomy, Video 47.2 Laparoscopic Resection of a Splenic Cyst, Video 51.1 Two- Staged Laparoscopic Orchiopexy for a Left Non-Palpable Testis, Video 75.1 Laparoscopic Right Adrenalectomy

Laura E. Hollinger, MD

Assistant Professor of Surgery and Pediatrics, Division of Pediatric Surgery, Department of Surgery, Medical University of South Carolina, Charleston, SC, USA Chapter 24: Congenital Diaphragmatic Hernia and Eventration

Charles R. Hong, MD

Surgical Research Fellow, Department of Surgery, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA Chapter 2: Nutritional Support for the Pediatric Patient

Thomas H. Inge, MD, PhD

Professor and Division Chief, Pediatric Surgery, Children’s Hospital Colorado and University of Colorado–Denver, Aurora, CO, USA Chapter 76: Bariatric Surgical Procedures in Adolescence, Video 76.1 Laparoscopic Sleeve Gastrectomy

List of Contributors

Nazia Tabassum Iqbal, MD

Pablo Laje, MD

Saleem Islam, MD, MPH

Kevin P. Lally, MD, MS

Assistant Professor of Pediatrics, Division of Hematology, Oncology, and Bone Marrow Transplant, Children’s Mercy Hospitals and Clinics, Kansas City, MO, USA Chapter 5: Coagulopathies and Sickle Cell Disease Professor and Division Chief, Pediatric Surgery, University of Florida College of Medicine, Gainesville, FL, USA Chapter 48: Congenital Abdominal Wall Defects

Tom Jaksic, MD, PhD

W. Hardy Hendren Professor of Surgery, Harvard Medical School, Boston Children’s Hospital, Boston, MA, USA Chapter 2: Nutritional Support for the Pediatric Patient

Marcus D. Jarboe, MD

Associate Clinical Professor of Surgery and Radiology, Section of Pediatric Surgery and Division of Interventional Radiology, C.S. Mott Children’s Hospital, University of Michigan, Ann Arbor, MI, USA Chapter 37: Acquired Anorectal Disorders

David Juang, MD

Associate Professor of Surgery; Director, Trauma and Surgical Critical Care; Program Director, Surgical Critical Care Fellowship, Children’s Mercy Hospital, University of Missouri–Kansas City School of Medicine, Kansas City, MO USA Chapter 13: Burns, Video 29.1 Laparoscopic Repair of Pyloric Atresia

Bartholomew John Kane, MD, PhD

Assistant Professor, Department of Surgery, Children’s Mercy Hospital, Kansas City, MO; Division of Transplant Surgery, Department of Surgery, Kansas University Medical Center, Kansas City, KS, USA Chapter 66: Lesions of the Liver

Robert E. Kelly Jr., MD

Assistant Professor of Surgery, Division of General, Thoracic, and Fetal Surgery, Children’s Hospital of Philadelphia, University of Pennsylvania, Philadelphia, PA, USA Chapter 22: Congenital Bronchopulmonary Malformations Chairman, Department of Pediatric Surgery, McGovern Medical School–UTHealth; Surgeon in Chief, Children’s Memorial Hermann Hospital, Houston, TX, USA Chapter 24: Congenital Diaphragmatic Hernia and Eventration

Jacob C. Langer, MD

Professor of Surgery, University of Toronto; Pediatric Surgeon, Hospital for Sick Children, Toronto, Ontario, Canada Chapter 34: Hirschsprung Disease

Glen A. Lau, MD

Assistant Professor of Surgery, Department of Surgery, Division of Urology, University of Utah School of Medicine, Salt Lake City, UT, USA Chapter 56: Bladder and Urethra

Marc A. Levitt, MD

Chief, Section of Colorectal and Pelvic Reconstruction Surgery, Nationwide Children’s Hospital; Professor of Surgery and Pediatrics, The Ohio State University, Columbus, OH, USA Chapter 35: Anorectal Atresia and Cloacal Malformations, Chapter 36: Fecal Incontinence and Constipation, Video 34.1 Trans Anal Pullthrough, Video 35.1 Repair of a Male Infant with anorectal Atresia and a Recto-Bulbar Fistula, Video 35.2 Repair of Anorectal Atresia in a Female without a Urinary Fistula, Video 35.3 Repair of a Short, Cloacal Channel Malformation Using Total Urogenital Mobilization, Video 36.1 Appendicostomy for Antegrade Enemas for Patients with Fecal Incontinence

Surgeon-in-Chief, Children’s Hospital of The King’s Daughters; Professor of Clinical Surgery and Pediatrics, Eastern Virginia Medical School, Norfolk, VA, USA Chapter 20: Chest Wall Deformities, Video 20.1 The Nuss Procedure

Karen B. Lewing, MD

Joel F. Koenig, MD

Charles M. Leys, MD, MSCI

Director of Urologic Education, Department of Urology, Children’s Mercy Hospital, Kansas City, MO, USA Chapter 54: Ureteral Obstruction and Malformations

John V. Kryger, MD

Professor and Chief of Pediatric Urology, Department of Urology, Medical College of Wisconsin, Children’s Hospital of Wisconsin, Milwaukee, WI, USA Chapter 58: Bladder and Cloacal Exstrophy

Jean-Martin Laberge, MD

Professor of Surgery and Pediatric Surgery, Associate Chair, Department of Pediatric Surgery, McGill University, Montréal, Quebec, Canada Chapter 67: Teratomas, Dermoids, and Other Soft Tissue Tumors

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Associate Professor of Pediatrics, Division of Hematology/ Oncology, Children’s Mercy Hospital, Kansas City, MO, USA Chapter 68: Lymphomas Associate Professor of Surgery, Division Chief of Pediatric Surgery, Department of Surgery, University of Wisconsin School of Medicine and Public Health, Madison, WI, USA Chapter 40: Meckel Diverticulum

Nguyen Thanh Liem, PhD, MD

Director, Vinmec Research Institute of Stem Cell and Gene Technology, Hanoi, Vietnam Chapter 44: Choledochal Cyst and Gallbladder Disease, Video 44.1 Laparoscopic Excision of a Choledochal Cyst with Hepatico-jejunostomy

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List of Contributors

Danny C. Little, MD

Surgeon in Chief, McLane Children’s Hospital; Associate Professor of Surgery, Department of Surgery, Texas A&M College of Medicine, Temple, TX, USA Chapter 11: Ingestion of Foreign Bodies, Video 25.1 Thoracoscopic Biopsy of an Anterior Mediastinal Mass

Armando J. Lorenzo, MD, MSc

Pediatric Urology, Hospital for Sick Children; Associate Professor, Department of Surgery, University of Toronto, Toronto, Ontario, Canada Chapter 51: Undescended Testes and Testicular Tumors

Leopoldo Martínez, MD, PhD

Associate Professor of Pediatric Surgery and Pediatrics, Department of Pediatric Surgery and Pediatrics, University Children’s Hospital La Paz, Universidad Autónoma de Madrid, Madrid, Spain Chapter 25: Mediastinal Tumors

Marcelo Martinez-Ferro, MD

Chief, Department of Surgery, Fundación Hospitalaria Children’s Hospital, Buenos Aires, Argentina Chapter 20: Chest Wall Deformities

Robert Todd Maxson, MD

Surgeon-In-Chief, Department of Pediatric Surgery, Arkansas Children’s Hospital, University of Arkansas for Medical Sciences, Little Rock, AR, USA Chapter 15: Thoracic Trauma

Lynne G. Maxwell, MD

Department of Anesthesiology and Critical Care Medicine, The Children’s Hospital of Philadelphia; Emeritus Professor, Anesthesiology and Critical Care, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA Chapter 3: Anesthetic Considerations for Pediatric Surgical Conditions

Lisa McMahon, MD

Nilesh M. Mehta, MD

Associate Professor of Anesthesia (Critical Care), Harvard Medical School; Senior Associate in Critical Care Medicine, Department of Anesthesiology, Critical Care, and Pain Medicine, Boston Children’s Hospital, Boston, MA, USA Chapter 2: Nutritional Support for the Pediatric Patient

Eugene Minevich, MD

Professor, Division of Pediatric Urology; Chair, Pediatric Urology International Education; Director, Stone Center, Cincinnati Children’s Hospital, Cincinnati, OH, USA Chapter 55: Urinary Tract Infections and Vesicoureteral Reflux, Video 55.1 Robotic Extravesical Ureteral Reimplantation

Michael E. Mitchell, MD

Professor Emeritus; Surgical Coach, Pediatric Urology, Division of Pediatric Urology, Department of Urology, Medical College of Wisconsin, Milwaukee, WI, USA Chapter 58: Bladder and Cloacal Exstrophy

R. Lawrence Moss, MD

President and Chief Executive Officer, Nemours Children’s Health System, Jacksonville, FL, USA Chapter 33: Necrotizing Enterocolitis

J. Patrick Murphy, MD

Professor of Surgery, Department of Surgery, The Children’s Mercy Hospital, Kansas City, MO, USA Chapter 59: Hypospadias

George B. Mychaliska, MD

Robert Bartlett, M.D. Collegiate Professor of Pediatric Surgery, Professor of Surgery and Obstetrics and Gynecology, Section of Pediatric Surgery, University of Michigan Medical School, Ann Arbor, MI USA Chapter 6: Extracorporeal Membrane Oxygenation, Video 6.1 Percutaneous Placement of a Double-Lumen Cannula for DLVV-ECLS in a Neonate

Pediatric Surgeon; Surgical Director, IBD Clinic; Medical Director, Chest Wall Anomalies Clinic; Section Chief of Pediatric Surgery, Phoenix Children’s Hospital; Clinical Assistant Professor of Surgery, University of Arizona College of Medicine; Clinical Assistant Professor of Surgery, Mayo Clinic, Phoenix, AZ, USA Chapter 41: Inflammatory Bowel Disease

Bindi Naik-Mathuria, MD, MPH

Marcus M. Malek, MD

Professor of Surgery, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA Chapter 74: Breast Diseases

Assistant Professor of Surgery, University of Pittsburgh School of Medicine; Director, Pediatric Surgical Oncology; Attending Pediatric Surgeon, Division of Pediatric General and Thoracic Surgery, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA Chapter 46: Lesions of the Pancreas, Video 46.2 Laparoscopic Distal Pancreatectomy for Trauma

Associate Professor of Surgery and Pediatrics, Trauma Medical Director, Texas Children’s Hospital, Department of Surgery, Division of Pediatric Surgery, Baylor College of Medicine, Houston, TX, USA Chapter 12: Bites

Don K. Nakayama, MD, MBA

Jaimie D. Nathan, MD

Associate Professor of Surgery and Pediatrics; Surgical Director, Pancreas Care Center; Surgical Director, Kidney and Intestinal Transplant Programs, Division of Pediatric General and Thoracic Surgery, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA Chapter 45: Solid Organ Transplantation in Children

List of Contributors

Peter F. Nichol, MD, PhD

Associate Professor with Tenure, Department of Surgery, Division of Pediatric Surgery, University of Wisconsin School of Medicine and Public Health, Madison, WI, USA Chapter 30: Duodenal and Intestinal Atresia and Stenosis

David M. Notrica, MD

Trauma Medical Director, Phoenix Children’s Hospital; Associate Professor of Surgery, Mayo Clinic College of Medicine; Associate Professor of Child Health, University of Arizona College of Medicine-Phoenix, Phoenix, AZ, USA Chapter 16: Abdominal and Renal Trauma

Jed G. Nuchtern, MD

William J. Pokorny Professor of Surgery and Pediatrics, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX, USA Chapter 65: Neuroblastoma

Sarah B. Ogle, DO, MS

General Surgery Resident, Department of Surgery, University of Arizona College of Medicine–Phoenix, Phoenix, AZ, USA Chapter 30: Duodenal and Intestinal Atresia and Stenosis

Vanessa Ortiz-Hernández, MD

Urology Resident, Urology Section, Department of Surgery, University of Puerto Rico School of Medicine, San Juan, Puerto Rico Chapter 61: Prune Belly Syndrome

Daniel J. Ostlie, MD

Surgeon-in-Chief and Chair of Surgery, Phoenix Children’s Hospital; Professor of Surgery, Mayo Clinic School of Medicine; Professor, University of Arizona College of Medicine, Phoenix, AZ, USA Chapter 30: Duodenal and Intestinal Atresia and Stenosis, Chapter 41: Inflammatory Bowel Disease, Video 28.2 Laparoscopic Thal Fundoplication, Video 46.1 Laparoscopic Cyst – Gastrostomy

Tolulope Oyetunji, MD, MPH

Assistant Professor of Surgery, University of Missouri–­ Kansas City School of Medicine; Director, Surgical ­Scholars Program, Department of Surgery, Children’s Mercy Hospital, Kansas City, MO, USA Chapter 9: Surgical Infectious Disease, Video 9.1 Placement of a Wound VAC in an Infant Following Left Congenital Diaphragmatic Hernia Repair Using Mesh

Jonathan C. Papic, MD

Attending Pediatric Surgeon, The Studer Family Children’s Hospital at Sacred Heart, Pensacola, FL, USA Chapter 60: Circumcision

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Marcos Perez-Brayfield, MD

Associate Professor in Urology, Chief of Pediatric Urology, Division of Urology, University of Puerto Rico School of Medicine, San Juan, Puerto Rico Chapter 61: Prune Belly Syndrome

Pramod S. Puligandla, MD, MSc

Professor of Paediatric Surgery, Paediatrics and Surgery; Associate Chair for Peri-Operative Services; Program Director, Paediatric Surgery; Attending Staff, Division of Paediatric General and Thoracic Surgery; Attending Staff, Division of Paediatric Critical Care Medicine, Department of Paediatric Surgery, The Montreal Children’s Hospital of the McGill University Health Centre, Montreal, Quebec, Canada Chapter 67: Teratomas, Dermoids, and Other Soft Tissue Tumors

Stephen C. Raynor, MD

Professor of Surgery, Children’s Hospital and Medical Center, University of Nebraska College of Medicine, Omaha, NE, USA Chapter 60: Circumcision

Rebecca M. Rentea, MD

Assistant Professor of Surgery; Director, Comprehensive Colorectal Center, Children’s Mercy Hospital, University of Missouri–Kansas City, Kansas City, MO, USA Chapter 35: Anorectal Atresia and Cloacal Malformations

Frederick J. Rescorla, MD

Surgeon-in-Chief, Riley Hospital for Children, Indiana University School of Medicine, Indianapolis, IN, USA Chapter 47: Splenic Conditions, Video 47.1 Laparoscopic Splenectomy

Jay Rilinger, MD

Assistant Professor, Department of Pediatrics, Division of Pediatric Critical Care, Children’s Mercy Hospital, Kansas City, MO, USA Chapter 17: Head Injury and Facial Trauma

Eric H. Rosenfeld, MD, MPH

Research Fellow, Michael E. DeBakey Department of S ­ urgery, Baylor College of Medicine and Texas ­Children’s Hospital, Houston, TX, USA Chapter 12: Bites

Elizabeth B. Roth, MD

Associate Professor of Urology, Department of U ­ rology, Division of Pediatric Urology, Medical College of ­Wisconsin, Milwaukee, Wisconsin, USA Chapter 58: Bladder and Cloacal Exstrophy

xviii

List of Contributors

Steven Rothenberg, MD

Clinical Professor of Surgery, Columbia University College of Physicians and Surgeons; Chief of Pediatric Surgery, The Rocky Mountain Hospital for Children, Denver, CO, USA Chapter 27: Esophageal Atresia and Tracheoesophageal Fistula Malformations, Video 22.1 Video 22.1 Thoracoscopic Right Lower Lobectomy for a CCAM, Video 23.1 Thoracoscopic Lung Biopsy Using the Endoscopic Stapler and Using a Loop Ligature, Video 24.1 Laparoscopic Plication of the Right Hemi-Diaphragm, Video 27.1 Thoracoscopic Repair of a Type 3 Esophageal Atresia and Tracheoesophageal Fistula using the JRS 3mm Sealer, Video 27.2 Thoracoscopic Ligation of an H-type Tracheoesophageal Fistula using the 5mm Stapler, Video 30.1 Laparoscopic Repair of Duodenal Atresia and Ladd’s Procedure and Meckel’s Diverticulectomy in a Newborn Using the JRS 3mm Sealer and 5mm Stapler, Video 30.2 Laparoscopic Intra-Corporeal Management of a Jejunal Atresia with an Apple Peel Deformity Using a 5mm Endoscopic Stapler

Katie W. Russell, MD

Assistant Professor, Pediatric Surgery, University of Utah, Primary Children’s Hospital, Salt Lake City, UT, USA Chapter 39: Alimentary Tract Duplications

Daniel A. Saltzman, MD, PhD

Professor, Surgery and Pediatrics; AS Leonard Endowed Chair in Pediatric Surgery; Chief, Division of Pediatric Surgery, University of Minnesota Masonic Children’s Hospital, Minneapolis, MN, USA Chapter 1: Physiology of the Newborn

Richard Schwend, MD

Kenneth Shaw, MD

Assistant Professor of Surgery and Pediatric Surgery, McGill University, Attending Surgeon, Montreal Children’s Hospital of the McGill University Health Center, Montreal, Quebec, Canada Chapter 67: Teratomas, Dermoids, and Other Soft Tissue Tumors

Curtis A. Sheldon, MD

Founding Director, Division of Urology, Center for Genitourinary Reconstruction Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA Chapter 55: Urinary Tract Infections and Vesicoureteral Reflux, Video 55.1 Robotic Extravesical Ureteral Reimplantation

Mariya E. Skube, MD, MPH

General Surgery Resident, Department of Surgery, University of Minnesota Medical School, Minneapolis, MN, USA Chapter 1: Physiology of the Newborn

Samuel D. Smith, MD

Boyd Family Chair in Pediatric Surgery, Chief of Pediatric Surgery, Professor of Surgery, University of Arkansas for Medical Sciences, Arkansas Children’s Hospital, Little Rock, AR, USA Chapter 31: Malrotation

Brent W. Snow, MD

Chairman of Pediatric Urology, University of Utah School of Medicine and Primary Children’s Hospital, Salt Lake City, UT, USA Chapter 56: Bladder and Urethra

Director of Orthopaedic Research, Professor of Orthopaedics and Pediatrics, Department of Orthopaedics and Musculoskeletal Sciences, Children’s Mercy Hospital, Kansas City, MO, USA Chapter 18: Pediatric Orthopedic Trauma

Charles L. Snyder, MD

Bradley J. Segura, MD, PhD

Christopher Snyder, MD, MSPH

Sohail R. Shah, MD, MSHA

Howard M. Snyder III, MD

Assistant Professor, Departments of Pediatrics and Surgery, University of Minnesota Masonic Children’s Hospital, Minneapolis, MN, USA Chapter 1: Physiology of the Newborn Assistant Professor of Surgery and Pediatrics, Division of Pediatric Surgery, Baylor College of Medicine, Texas Children’s Hospital, Houston, TX, USA Chapter 11: Trauma Ingestion of Foreign Bodies

Robert C. Shamberger, MD

Robert E. Gross Professor of Surgery, Harvard Medical School; Chief, Department of Surgery, Boston Children’s Hospital, Boston, MA, USA Chapter 64: Renal Tumors

Ellen Shapiro, MD

Professor of Urology, Department of Urology, New York University School of Medicine, New York, NY, USA Chapter 57: Posterior Urethral Valves, Video 57.1 Endoscopic Ablation of Posterior Urethral Valves

Professor of Surgery, Department of Pediatric Surgery, University of Missouri–Kansas City School of Medicine, Kansas City, MO, USA Chapter 50: Inguinal Hernia Assistant Professor of Surgery, Division of Pediatric ­Surgery, Johns Hopkins All Children’s Hospital, St. Petersburg, FL, USA Chapter 26: The Esophagus Emeritus Professor of Surgery, Perelman School of Medicine, University of Pennsylvania, Children’s Hospital of Philadelphia, Division of Pediatric Urology, Philadelphia, PA, USA Chapter 53: Developmental and Positional Anomalies of the Kidneys

Justin A. Sobrino, MD

Surgical Critical Care Fellow, Department of Surgery, Children’s Mercy Hospital, Kansas City, MO, USA Chapter 29: Lesions of the Stomach

List of Contributors

Richard Sola Jr., MD

Assistant Professor of Surgery, Trauma/Critical Care, Department of Surgery, Morehouse School of Medicine, Atlanta, GA, USA Chapter 9: Surgical Infectious Disease, Video 9.1 Placement of a Wound VAC in an Infant Following Left Congenital Diaphragmatic Hernia Repair Using Mesh

Shawn D. St. Peter, MD

Surgeon-in-Chief, Thomas Holder and Keith Ashcraft Endowed Chair; Director, Pediatric Surgery Fellowship; Director, Center for Prospective Trials; Professor of Surgery, Children’s Mercy Hospital, Kansas City, MO, USA Chapter 23: Acquired Lesions of the Lung and Pleura, Chapter 42: Appendicitis, Video 28.1 Laparoscopic Fundoplication: Minimal Esophageal Dissection and Placement of Esophago-Crural Sutures

Julie Strickland, MD, MPH

Professor of Obstetrics and Gynecology, University of Missouri–Kansas City; Chief, Division of Gynecology, Children’s Mercy Hospital, Kansas City, MO, USA Chapter 73: Pediatric and Adolescent Gynecology, Video 73.3 Laparoscopic Oophorectomy for Fertility Preservation, Video 73.5 Non-Communication Uterine Horn: The Laparoscopic Approach, Video 73.6 Prepubertal EUA and Vaginoscopy

Joseph A. Sujka, MD

Surgical Scholar, Department of Pediatric Surgery, Children’s Mercy Hospital, Kansas City, MO, USA Chapter 49: Umbilical and Other Abdominal Wall Hernias

Veronica Sullins, MD

Assistant Clinical Professor of Pediatric Surgery, Division of Pediatric Surgery, David Geffen UCLA School of Medicine, Los Angeles, CA, USA Chapter 37: Acquired Anorectal Disorders

Gregory M. Tiao, MD

Professor of Surgery and Pediatrics; Division Director, Pediatric Surgery; Surgical Director, Liver Transplantation; Frederick Ryckman Chair in Pediatric Surgery, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA Chapter 45: Solid Organ Transplantation in Children

Kelly S. Tieves, DO, MS

Medical Director, Cardiac Critical Care, Children’s Mercy Hospital; Associate Professor, Pediatrics, University of Missouri–Kansas City School of Medicine, Kansas City, MO, USA Chapter 17: Head Injury and Facial Trauma

Juan A. Tovar, MD, PhD

Emeritus Professor, Department of Pediatrics, Universidad Autonoma de Madrid; Past Surgeon in Chief, Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain Chapter 25: Mediastinal Tumors

xix

KuoJen Tsao, MD

Professor of Pediatric Surgery and Obstetrics/Gynecology and Reproductive Medicine, Department of Pediatric Surgery and Obstetrics, Gynecology, and Reproductive Medicine, McGovern Medical School at the University of Texas Health Science Center, Houston, TX, USA Chapter 10: Fetal Therapy, Video 28.2 Laparoscopic Thal Fundoplication, Video 28.3 The Use of Surgisis for Hiatal Reinforcement at Re-Do Laparoscopic Fundoplication and Antroplasty, Video 28.4 Laparoscopic Gastrostomy, Video 47.2 Laparoscopic Resection of a Splenic Cyst

Benno Ure, MD

Chair in Pediatric Surgery, Professor of General and Pediatric Surgery, Hannover Medical School, Hannover, Germany Chapter 43: Biliary Atresia

Robert J. Vandewalle, MD, MBA

Clinical Research Fellow, Department of Pediatric Surgery, Indiana University School of Medicine Indianapolis, IN, USA Chapter 47: Splenic Conditions

Patricio Varela, MD

Professor in Pediatric Surgery, University of Chile; Director, Airway and Chest Wall Unit, Calvo Mackenna Children’s Hospital, Clinica Las Condes Medical Center, Santiago, Chile Chapter 21: Management of Laryngotracheal Obstruction in Children

Ravindra K. Vegunta, MD

Clinical Professor of Child Health and Surgery, University of Arizona College of Medicine–Phoenix, Phoenix; Cardon Children’s Medical Center, Mesa, AZ, USA Chapter 8: Vascular Access

Cristine S. Velazco, MD, MS

Chief Resident, Department of Surgery, Mayo Clinic, Phoenix, AZ, USA Chapter 41: Inflammatory Bowel Disease

Daniel von Allmen, MD

Surgeon-in-Chief and Senior Vice President, Surgical Services, Cincinnati Children’s Hospital Medical Center; Professor of Surgery, University of Cincinnati College of Medicine, Cincinnati, OH, USA Chapter 63: Principles of Adjuvant Therapy in Childhood Cancer

John H.T. Waldhausen, MD

Professor of Surgery, Seattle Children’s Hospital, University of Washington School of Medicine, Seattle, WA, USA Chapter 72: Head and Neck Sinuses and Masses

M. Chad Wallis, MD

Associate Professor of Surgery, Division of Urology, University of Utah School of Medicine, Primary Children’s Hospital, Salt Lake City, UT, USA Chapter 56: Bladder and Urethra

xx

List of Contributors

Bradley A. Warady, MD

Professor of Pediatrics, University of Missouri–Kansas City School of Medicine; Director, Division of Nephrology; Director, Dialysis and Transplantation, Children’s Mercy Hospitals and Clinics, Kansas City, MO, USA Chapter 4: Renal Impairment and Renovascular Hypertension

Dana A. Weiss, MD

Attending Urologist, Assistant Professor of Urology in Surgery, Division of Urology, Children’s Hospital of Philadelphia, Philadelphia, PA, USA Chapter 58: Bladder and Cloacal Exstrophy

Tomas Wester, MD, PhD

Professor, Chief of Pediatric Surgery, Karolinska University Hospital, Karolinska Institute, Stockholm, Sweden Chapter 42: Appendicitis

Brian M. Wicklund, MDCM, MPH

Gerald M. Woods, MD

Division Director, Professor of Pediatrics, Division of Hematology/Oncology/Bone Marrow Transplant, Children’s Mercy Hospital, University of Missouri–Kansas City School of Medicine, Kansas City, MO, USA Chapter 5: Coagulopathies and Sickle Cell Disease

Tiffany N. Wright, MD

Assistant Professor, Hiram C. Polk, Jr. Department of Surgery, University of Louisville School of Medicine, Louisville, KY, USA Chapter 38: Intussusception

Hsi-Yang Wu, MD

Associate Professor of Urology, Lucile Packard Children’s Hospital, Stanford University School of Medicine, Stanford, CA, USA Chapter 53: Developmental and Positional Anomalies of the Kidneys

Associate Professor of Pediatrics, University of Missouri– Kansas City School of Medicine; Director, Coagulation Medicine Program, Division of Hematology/Oncology, Children’s Mercy Hospitals and Clinics, Kansas City, MO, USA Chapter 5: Coagulopathies and Sickle Cell Disease

Mark Wulkan, MD

Laurel Willig, MD, MS

Deidre L. Wyrick, MD

Associate Professor, Pediatric Nephrology, Department of Pediatrics, Children’s Mercy Hospital, University of Missouri–Kansas City School of Medicine, Kansas City, MO, USA Chapter 62: Differences in Sexual Development

Richard J. Wood, MD

Colorectal Director and Attending Pediatric Surgeon, Center for Colorectal and Pelvic Reconstruction, Nationwide Children’s Hospital and The Ohio State University College of Medicine, Columbus, OH, USA Chapter 36: Fecal Incontinence and Constipation, Video 35.1 Repair of a Male Infant with Anorectal Atresia and a Recto-Bulbar Fistula, Video 35.2 Repair of Anorectal Atresia in a Female without a Urinary Fistula, Video 35.3 Repair of a Short, Cloacal Channel Malformation Using Total Urogenital Mobilization

Surgeon-in-Chief, Professor of Surgery and Pediatrics, Children’s Healthcare of Atlanta, Emory University School of Medicine, Atlanta, GA, USA Chapter 29: Lesions of the Stomach, Video 29.2 Laparoscopic Pyloromyotomy Senior Pediatric Surgery Fellow, Arkansas Children’s Hospital, University of Arkansas for Medical Sciences, Little Rock, AR, USA Chapter 15: Thoracic Trauma

Atsuyuki Yamataka, MD, PhD

Professor and Head, Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, Tokyo, Japan Chapter 43: Biliary Atresia, Video 43.1 Laparoscopic Kasai

This Seventh Edition of our book is enthusiastically dedicated to three wonderful Administrative Assistants: Mrs. Linda Jankowski, Mrs. Jeannette Whitney, and Mrs. Barbara Juarez. All three have been associated with our group for several decades and have been invaluable in the production of the last four editions of the book. The Editors

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I

SECTION

General

1

1

Physiology of the Newborn MARIYA E. SKUBE, BRADLEY J. SEGURA, and DANIEL A. SALTZMAN

Of all pediatric patients, the neonate possesses the most distinctive and rapidly changing physiologic characteristics. These changes are necessary because the newborn must adapt from placental support to the extrauterine environment. There is also early organ adaptation and the physiologic demands of rapid growth and development. This chapter will emphasize the dynamic physiologic alterations of the neonate. Newborns are classified based on gestational age, weight, head circumference, and length. Preterm infants are those born before 37 weeks of gestation. Term infants are those born between 37 and 42 weeks of gestation, whereas post-term infants have a gestational age that exceeds 42 weeks. With advances in neonatal intensive care, infants born as early as 21 weeks of gestation have survived, and the medical and ethical guidelines regarding the care of these extremely premature neonates continue to evolve.1 Babies whose weight is below the 10th percentile for age are considered small-for-gestational-age (SGA). Those at or above the 90th percentile are large-forgestational-age (LGA). Babies whose weight falls between these extremes are appropriate-for-gestational-age (AGA). Further subclassified, premature infants are characterized as moderately low birth weight if they weigh between 1501 and 2500 g, very low birth weight between 1001 and 1500 g, and extremely low birth weight if less than 1000 g. SGA newborns are thought to suffer intrauterine growth retardation (IUGR) as a result of placental, maternal, or fetal abnormalities. Conditions associated with IUGR are shown in Fig. 1.1.2 SGA infants have a body weight below what is appropriate for their age, yet their body length and head circumference are age appropriate. To classify an infant as SGA, the gestational age must be estimated by the physical findings summarized in Table 1.1. Although SGA infants may weigh the same as premature infants, they have different physiologic characteristics. Due to intrauterine malnutrition, body fat levels are frequently below 1% of the total body weight. This lack of body fat increases the risk of hypothermia in SGA infants. Hypoglycemia is the most common metabolic problem for neonates and develops earlier in SGA infants due to higher metabolic activity and reduced glycogen stores. The red blood cell (RBC) volume and the total blood volume are much higher in the SGA infant compared with the preterm AGA or the non-SGA full-term infant. This rise in RBC volume frequently leads to polycythemia, with an associated rise in blood viscosity. Due to an adequate length of gestation, the SGA infant has pulmonary function approaching that of the AGA or a full-term infant. Infants born before 37 weeks of gestation, regardless of birth weight, are considered premature. The physical 2

exam of the premature infant reveals many abnormalities. Special problems with the preterm infant include the following:   

1. Weak suck reflex 2. Inadequate gastrointestinal absorption 3. Hyaline membrane disease (HMD) 4. Intraventricular hemorrhage 5. Hypothermia 6. Patent ductus arteriosus 7. Apnea 8. Hyperbilirubinemia 9. Necrotizing enterocolitis (NEC)

Specific Physiologic Problems of the Newborn GLUCOSE METABOLISM The fetus maintains a blood glucose value of 70–80% of maternal levels by facilitated diffusion across the placenta. There is a build-up of glycogen stores in the liver, skeleton, and cardiac muscles during the later stages of fetal development, but little gluconeogenesis. The newborn must depend on glycolysis until exogenous glucose is supplied. After delivery, the baby depletes his or her hepatic glycogen stores within 2–3 hours. The newborn is severely limited in his or her ability to use fat and protein as substrates to synthesize glucose. When total parenteral nutrition (TPN) is needed, the glucose infusion rate should be initiated at 4–6 mg/kg/min and advanced 1–2 mg/kg/min to a goal of 12 mg/kg/min.

Hypoglycemia Clinical signs of hypoglycemia are nonspecific and subtle. Seizure and coma are the most common manifestations of severe hypoglycemia. Neonatal hypoglycemia is generally defined as a glucose level lower than 50 mg/dL.3 Infants who are at high risk for developing hypoglycemia are those who are premature; SGA; or born to mothers with gestational diabetes, severe preeclampsia, or HELLP (hemolysis, elevated liver enzymes, low platelet count). Newborns who require surgical procedures are at particular risk of developing hypoglycemia; therefore, a 10% glucose infusion is typically started on admission to the hospital. Hypoglycemia is treated with an infusion of 1–2 mL/kg (4–8 mg/kg/ min) of 10% glucose. If an emergency operation is required, concentrations of up to 25% glucose may be used. Traditionally, central venous access has been a prerequisite for glucose infusions exceeding 12.5%. During the first 36–48 hours after a major operation, it is common to see wide variations in serum glucose levels. 

1 • Physiology of the Newborn

3

Fetal • Gender • Chromosomal abnormalities

Maternal

Placental

• Low pre-pregnancy weight • Low weight gain • Ethnicity • Infections • Toxins • Comorbidities (e.g., diabetes, hypertension)

• Vascular abnormalities (single umbilical artery, velamentous umbilical cord insertion, twin–twin transfusion) • Placenta previa • Placental abruption • Poor perfusion

Fig. 1.1  Diagram of conditions associated with deviations in intrauterine growth. (Adapted from Simmons R. Abnormalities of fetal growth. In: Gleason CA, Devaskar SU, eds. Avery’s Diseases of the Newborn. Philadelphia: Saunders; 2012. p. 51.2)

Table 1.1  Clinical Criteria for Classification of Low Birth Weight Infants Criteria

36 Weeks (Premature)

37–38 Weeks (Borderline Premature) 39 Weeks (Term)

Plantar creases Size of breast nodule Head hair Earlobe Testicular descent and scrotal changes

Rare, shallow Heel remains smooth Not palpable to 1 month 3 months to adult

85–100 85 75 70

Adapted from Rowe PC, ed. The Harriet Lane Handbook.11th ed. Chicago: Year Book Medical; 1987. p. 25.

Signs of hypocalcemia are similar to those of hypoglycemia and may include jitteriness, seizures, cyanosis, vomiting, and myocardial arrhythmias. Hypocalcemic infants have increased muscle tone, which helps differentiate infants with hypocalcemia from those with hypoglycemia. Symptomatic hypocalcemia is treated with 10% calcium gluconate administered intravenous at a dosage of 1–2 mL/kg (100–200 mg/kg) over 30 minutes while monitoring the electrocardiogram for bradycardia.3 Asymptomatic hypocalcemia is best treated with calcium gluconate in a dose of 50 mg of elemental calcium/kg/ day added to the maintenance fluid: 1 mL of 10% calcium gluconate contains 9 mg of elemental calcium. If possible, parenteral calcium should be given through a central venous line, as skin and soft tissue necrosis may occur should the peripheral IV infiltrate. 

MAGNESIUM Magnesium is actively transported across the placenta. Half of total body magnesium is in the plasma and soft tissues. Hypomagnesemia is observed with growth retardation, maternal diabetes, after exchange transfusions, and with hypoparathyroidism. Although the mechanisms by which magnesium and calcium interact are not clearly defined, they appear to be interrelated. The same infants at risk for hypocalcemia are also at risk for hypomagnesemia. Magnesium deficiency should be suspected and confirmed in an infant who has seizures that do not respond to calcium therapy. Emergent treatment consists of magnesium sulfate 25–50 mg/kg IV every 6 hours until normal levels are obtained. 

BLOOD VOLUME Total RBC volume is at its highest point at delivery. Estimations of blood volume for premature infants, term neonates, and infants are summarized in Table 1.2. By about 3 months of age, total blood volume per kilogram is nearly equal to adult levels as infants recover from their postpartum physiologic nadir. The newborn blood volume is affected by shifts of blood between the placenta and the baby before clamping the cord. Infants with delayed cord clamping (typically defined as greater than 1 minute after birth) have higher hemoglobin levels.9 A hematocrit greater than 50% suggests placental transfusion has occurred. Although this effect on hemoglobin levels does not persist, iron stores are positively impacted up to 6 months of age by delayed cord clamping.10

80

% O2 Hb saturation

Group

O2 affinity O2 release PCO2 [H+] Temperature 2,3 -DPG HgbF

O2 affinity O2 release PCO2 [H+] Temperature 2,3 -DPG

60

40

20 “P50” values

20

40

60

80

PO2 (mmHg) Fig. 1.2 The oxygen dissociation curve of normal adult blood is shown in red. The P50, the oxygen tension at 50% oxygen saturation, is approximately 27 mmHg. As the curve shifts to the right, the affinity of hemoglobin for oxygen decreases and more oxygen is released. Increases in PCO2, temperature, 2,3-DPG, and hydrogen ion concentration facilitates the unloading of O2 from arterial blood to the tissue. With a shift to the left, unloading of O2 from arterial blood into the tissues is more difficult. Causes of a shift to the left are mirror images of those that cause a shift to the right: decreases in temperature, 2,3-DPG, and hydrogen ion concentration. (Modified from Glancette V, Zipursky A. Neonatal hematology. In: Avery GB, ed. Neonatology. Philadelphia: JB Lippincott; 1986. p. 663.)

Hemoglobin At birth, nearly 80% of circulating hemoglobin is fetal (a2Aγ2F). When infant erythropoiesis resumes at about 2–3 months of age, most new hemoglobin is adult. When the oxygen level is 27 mmHg, 50% of the bound oxygen is released from adult hemoglobin (P50 = 27 mmHg). Reduction of the affinity of hemoglobin for oxygen allows more oxygen to be released into the tissues at a given oxygen level as shown in Fig. 1.2. Fetal hemoglobin has a P50 value 6–8 mmHg lower than that of adult hemoglobin. This lower P50 value allows more efficient oxygen delivery from the placenta to the fetal tissues. The fetal hemoglobin equilibrium curve is shifted to the left of the normal adult hemoglobin equilibrium curve. Fetal hemoglobin binds less avidly to 2,3-diphosphoglycerate (2,3-DPG) compared with adult hemoglobin, causing a decrease in P50.11 This is somewhat of a disadvantage to the newborn because lower peripheral oxygen levels are needed before oxygen is released from fetal hemoglobin. By 4–6 months of age in a term infant, the hemoglobin equilibrium curve gradually shifts to the right and the P50 value approximates that of a normal adult.  Polycythemia A central venous hemoglobin level greater than 22 g/dL or a hematocrit value greater than 65% during the first

1 • Physiology of the Newborn

week of life is defined as polycythemia. After the central venous hematocrit value reaches 65%, further increases result in rapid exponential increases in blood viscosity. Neonatal polycythemia occurs in infants of diabetic mothers, infants of mothers with toxemia of pregnancy, or SGA infants. Polycythemia is treated using a partial exchange of the infant’s blood with fresh whole blood or 5% albumin. This is frequently done for hematocrit values greater than 65%. 

ANEMIA Anemia present at birth is due to hemolysis, blood loss, or decreased erythrocyte production.

Hemolytic Anemia Hemolytic anemia is most often a result of placental transfer of maternal antibodies that are destroying the infant’s erythrocytes. This can be determined by the direct Coombs test. The most common severe anemia is Rh incompatibility. Hemolytic disease in the newborn produces jaundice, pallor, and hepatosplenomegaly. The most severely affected fetuses manifest hydrops. This massive edema is not strictly related to the hemoglobin level of the infant. ABO incompatibility frequently results in hyperbilirubinemia, but rarely causes anemia. Congenital infections, hemoglobinopathies (sickle cell disease), and thalassemias produce hemolytic anemia. In a severely affected infant with a positive-reacting direct Coombs test result, a cord hemoglobin level less than 10.5 g/ dL, or a cord bilirubin level greater than 4.5 mg/dL, immediate exchange transfusion is indicated. For less severely affected infants, exchange transfusion is indicated when the total indirect bilirubin level is greater than 20 mg/dL.  Hemorrhagic Anemia Significant anemia can develop from hemorrhage that occurs during placental abruption. Internal bleeding (intraventricular, subgaleal, mediastinal, intra-abdominal) in infants can also often lead to severe anemia. Usually, hemorrhage occurs acutely during delivery, with the baby occasionally requiring a transfusion. Twin– twin transfusion reactions can produce polycythemia in one baby and profound anemia in the other. Severe cases can lead to death in the donor and hydrops in the recipient.  Anemia of Prematurity Decreased RBC production frequently contributes to anemia of prematurity. Erythropoietin is not released until a gestational age of 30–34 weeks has been reached. These preterm infants have large numbers of erythropoietin-sensitive RBC progenitors. Research has focused on the role of recombinant erythropoietin (epoetin alpha) in treating anemia in preterm infants.9–11 Successful increases in hematocrit levels using epoetin may obviate the need for blood transfusions and reduce the risk of blood borne infections and reactions. Studies suggest that routine use of epoetin is probably helpful for very low birth weight infants (90%) has been increasing with the advent of multidisciplinary programs that incorporate nutritionally focused care. New therapies aimed at enhancing bowel adaptation and reducing PN dependence, such as a long-acting glucagonlike peptide-2 (GLP-2) hormonal analog (teduglutide), show promise.173,174 

Conclusion The nutritional status of children influences outcome in surgical patients. Malnutrition is associated with physiologic instability and a longer ICU stay accompanied by increased utilization of resources. The first step in implementing appropriate nutritional support is an understanding of the metabolic events that accompany critical illness and surgery. Individualized, quantitative assessments of protein, carbohydrate, lipid, electrolyte, vitamin, and micronutrient requirements are made, and the appropriate route of nutrient delivery is determined. This nutrition regimen needs to be reviewed and amended regularly during the course of illness. The goal of nutrition therapy in sick pediatric surgical patients is to augment the short-term benefits of the metabolic stress response while minimizing any long-term consequences.

2 • Nutritional Support for the Pediatric Patient

References

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149. Mamatha B, Alladi A. Early oral feeding in pediatric intestinal anastomosis. Indian J Surg. 2015;77:670–672. 150. Ekingen G, Ceran C, Guvenc BH, et al. Early enteral feeding in newborn surgical patients. Nutrition. 2005;21:142–146. 151. Tadano S, Terashima H, Fukuzawa J, et al. Early postoperative oral intake accelerates upper gastrointestinal anastomotic healing in the rat model. J Surg Res. 2011;169:202–208. 152. Mizuno K, Ueda A. Development of sucking behavior in infants who have not been fed for 2 months after birth. Pediatr Int. 2001;43:251–255. 153. Lau C, Smith EO. Interventions to improve the oral feeding performance of preterm infants. Acta Paediatr. 2012;101:e269–e274. 154. Meert KL, Daphtary KM, Metheny NA. Gastric vs small-bowel feeding in critically ill children receiving mechanical ventilation: a randomized controlled trial. Chest. 2004;126:872–878. 155. Sanchez C, Lopez-Herce J, Carrillo A, et al. Early transpyloric enteral nutrition in critically ill children. Nutrition. 2007;23:16–22. 156. Davies AR, Morrison SS, Bailey MJ, et al. A multicenter, randomized controlled trial comparing early nasojejunal with nasogastric nutrition in critical illness. Crit Care Med. 2012;40:2342–2348. 157. Abdelhadi RA, Rahe K, Lyman B. Pediatric enteral access device management. Nutr Clin Pract. 2016;31(6):748–761. 158. Mehta NM, McAleer D, Hamilton S, et  al. Challenges to optimal enteral nutrition in a multidisciplinary pediatric intensive care unit. JPEN J Parenter Enteral Nutr. 2010;34:38–45. 159. Martinez EE, Bechard LJ, Mehta NM. Nutrition algorithms and bedside nutrient delivery practices in pediatric intensive care units: an international multicenter cohort study. Nutr Clin Pract. 2014;29:360–367. 160. Canarie MF, Barry S, Carroll CL, et  al. Risk factors for delayed enteral nutrition in critically ill children. Pediatr Crit Care Med. 2015;16:e283–e289. 161. Hamilton S, McAleer DM, Ariagno K, et al. A stepwise enteral nutrition algorithm for critically ill children helps achieve nutrient delivery goals. Pediatr Crit Care Med. 2014;15:583–589. 162. Martinez EE, Pereira LM, Gura K, et al. Gastric emptying in critically ill children. JPEN J Parenter Enteral Nutr. 2017. 148607116686330. 163. Evans DC, Forbes R, Jones C, et  al. Continuous versus bolus tube feeds: does the modality affect glycemic variability, tube feeding volume, caloric intake, or insulin utilization? Int J Crit Illn Inj Sci. 2016;6:9–15.

164. Lewis K, Alqahtani Z, McIntyre L, et al. The efficacy and safety of prokinetic agents in critically ill patients receiving enteral nutrition: a systematic review and meta-analysis of randomized trials. Crit Care. 2016;20:259. 165. Tiancha H, Jiyong J, Min Y. How to promote bedside placement of the postpyloric feeding tube: a network meta-analysis of randomized controlled trials. JPEN J Parenter Enteral Nutr. 2015;39:521– 530. 166. Honeycutt TC, El Khashab M, Wardrop 3rd RM, et  al. Probiotic administration and the incidence of nosocomial infection in pediatric intensive care: a randomized placebo-controlled trial. Pediatr Crit Care Med. 2007;8:452–458. quiz 464. 167. Manzanares W, Lemieux M, Langlois PL, et al. Probiotic and synbiotic therapy in critical illness: a systematic review and meta-analysis. Crit Care. 2016;19:262. 168. Briassoulis G, Tsorva A, Zavras N, et al. Influence of an aggressive early enteral nutrition protocol on nitrogen balance in critically ill children. J Nutr Biochem. 2002;13:560. 169. Fivez T, Kerklaan D, Mesotten D, et al. Early versus late parenteral nutrition in critically ill children. N Engl J Med. 2016;374:1111– 1122. 170. Collier SB, Gura K, DeLoid D, et al. Parenteral nutrition. In: Sonneville K, Duggan C, eds. Manual of Pediatric Nutrition. 5th ed. Shelton, CT: People’s Medical Publishing House; 2014:196–248. 171. Wales PW, Allen N, Worthington P, et al. A.S.P.E.N. clinical guidelines: support of pediatric patients with intestinal failure at risk of parenteral nutrition-associated liver disease. JPEN J Parenter Enteral Nutr. 2014;38:538–557. 172. Sulkowski JP, Minneci PC. Management of short bowel syndrome. Pathophysiology. 2014;21:111–118. 173. Jeppesen PB, Gilroy R, Pertkiewicz M, et  al. Randomised placebocontrolled trial of teduglutide in reducing parenteral nutrition and/ or intravenous fluid requirements in patients with short bowel syndrome. Gut. 2011;60:902–914. 174. Carter BA, Cohran VC, Cole CR, et  al. Outcomes from a 12-week, open-label, multicenter clinical trial of teduglutide in pediatric short bowel syndrome. J Pediatr. 2017;181:102–111. e105.

3

Anesthetic Considerations for Pediatric Surgical Conditions LAURA K. DIAZ and LYNNE G. MAXWELL

Anesthetizing children is an increasingly safe undertaking. When discussing the risks and benefits of a child’s operation with his or her family, surgeons should feel confident that their anesthesiology colleagues can provide an anesthetic that facilitates the procedure while ensuring the child’s safety. Providing optimal perioperative care for children requires close collaboration between the surgeon and anesthesiologist on issues both large and small. The intent of this chapter is to inform pediatric surgeons about considerations important to anesthesiologists.

Risks of Anesthesia MORBIDITY/MORTALITY In an effort to reduce patient complications, anesthesiologists have carefully analyzed anesthetic morbidity and mortality over the past decades. Whereas anesthesia was historically considered a dangerous enterprise, serious anesthesia-related complications are now relatively rare, especially in healthy patients. Reasons for this improvement include advances in pharmacology, improved monitoring technology, increased rigor of subspecialty training, and the ability to target potential problems using an analysis strategy. Quantifying the risk of pediatric anesthesia is difficult due to the difficulty in determining whether complications are attributable to the anesthetic and, if so, to what degree. The risk of cardiac arrest for children undergoing anesthesia was estimated in the 1990s to be 1:10,000.1,2 However, these studies did not take patient comorbidity or the surgical condition into consideration. A recent prospective, multicenter study of more than 31,000 anesthetics in children from birth to 15 years of age affirmed the significance of age, medical history, and physical status (PS) as risk factors for the occurrence of perioperative severe critical events requiring immediate intervention.3 The risk of a healthy child suffering cardiac arrest during myringotomy tube placement is significantly less than the likelihood of a child with complex cardiac disease arresting during a complex cardiac repair.4 A review of cardiac arrests in anesthetized children compared 193 events from 1998–2004 to 150 events from 1994–1997.5 A reduction in medication-caused arrests from 37–18% was identified and was attributed to a decline in the use of halothane, which causes myocardial

depression, and the advent of sevoflurane utilization, which is not associated with myocardial depression. A decrease was also noted in unrecognized esophageal intubation as a cause of arrest, due in large part to the advent of end-tidal carbon dioxide (ETCO2) monitoring, pulse oximetry, and an increased awareness of the problem. Recent large single-center reports have yielded a current estimate of anesthesia-related mortality of 1:250,000 in healthy children. To put this into perspective for parents, the risk of a motor vehicle collision on the way to the hospital or surgery center is greater than the risk of death under anesthesia. However, risks of mortality and morbidity are increased in neonates and infants 3 hours and including concern about prolonged fetal exposure to anesthetics.20 This announcement elicited responses from the pediatric and pediatric anesthesia21 and obstetric communities,22 raising concern for increasing anxiety among parents in light of the fact that the safety announcement does not appear to be based on new information. In a joint statement, the American Academy of Pediatrics (AAP) and the ASA have said: “The potential risk of negative cognitive or behavioral effects of anesthetic agents remains uncertain and must be placed in the context of the known risks and benefits of both the anesthetic and the related surgical or diagnostic procedure for which the anesthetic is required. Clinicians and parents are cautioned against the possible risk of delaying needed surgical or diagnostic procedures. Until additional information is available from ongoing studies, parents and providers should carefully weigh the risk and benefit of each contemplated procedure before proceeding.”21

Table 3.1  ASA Physical Status Classification ASA Classification

Patient Status

1 2

A normal healthy patient A patient with mild systemic disease A patient with severe systemic disease A patient with severe systemic disease that is a constant threat to life A moribund patient who is not expected to survive without the operation A declared brain-dead patient whose organs are being removed for donor purposes An emergency modifier for any ASA classification when failure to immediately correct a medical condition poses risk to life or organ viability

3 4 5 6 E

After these subspecialty group responses, the FDA issued a supplemental statement in April 2017 emphasizing that “…surgeries or procedures in children younger than 3 years should not be delayed or avoided when medically necessary. Consideration should be given to delaying potentially elective surgery in young children where medically appropriate.”23 Interestingly, a panel at the 2016 PANDA symposium discussed implications of concerns regarding anesthesia neurotoxicity for surgeons and their decision-making with parents regarding timing of surgery in infants.24 Multiple surgical subspecialists (ophthalmology, general surgery, urology, plastic surgery) identified procedures that would result in morbidity if delayed beyond infancy and agreed that surgeons should partner with anesthesiologists to discuss the balance of concerns about the risk of proceeding or delaying surgery, particularly if concerns regarding potential neurotoxicity are raised by the parents. 

Preoperative Anesthesia Evaluation Patients undergoing anesthesia benefit from a thorough preanesthetic/preoperative assessment and targeted preparation to optimize any coexisting medical conditions. The ASA Physical Status (PS) score is a means of communicating the condition of the patient but is not intended to represent operative risk and serves primarily as a common means of communication among care providers (Table 3.1). Any child with an ASA PS of 3 or greater should be seen by an anesthesiologist prior to the day of surgery. This may be modified in cases of hardship due to the distance from the surgical venue or when the patient is well known to the anesthesia service and the child’s health is unchanged. Finally, outstanding and unresolved medical issues may be significant enough to warrant cancellation of the procedure for optimization of anesthesia and/or further diagnostic workup.

CRITERIA FOR AMBULATORY SURGERY Ambulatory surgery comprises 70% or more of the caseload in most pediatric centers. Multiple factors should be considered when evaluating whether a child is suitable for

3 • Anesthetic Considerations for Pediatric Surgical Conditions

Box 3.1  Essential Elements of the Preoperative Assessment (in Addition to Physical Examination) Vital signs Height/weight Heart rate Respiratory rate Blood pressure Pulse oximetry (both in room air and with supplemental O2 if applicable) Allergies Medications Cardiac murmur history Previous subspecialty encounters Past anesthetic history including any adverse perianesthetic events Emergence delirium Postoperative nausea and vomiting Difficult intubation Difficult IV access Past surgical history Family history of pseudocholinesterase deficiency or malignant hyperthermia

outpatient surgery, with some states regulating the minimum patient age allowed in an ambulatory surgical center. For example, the minimum age in Pennsylvania is 6 months. In most cases, the child should be free of severe systemic disease (ASA PS 1 or 2). Existing family and social dynamics are also important factors. Some institutions utilize a telephone screening evaluation process to determine whether a patient can have his or her full anesthesia history and physical on the day of surgery rather than being evaluated in a preoperative evaluation clinic prior to surgery.25 Although well-controlled systemic illnesses do not necessarily preclude outpatient surgery, any concerns must be addressed in advance in a cooperative fashion between the surgical and anesthesia services. If a child has a moderate degree of impairment, but the disease is stable and the surgical procedure is of minimal insult, outpatient surgery may be acceptable. 

GENERAL PRINCIPLES In addition to the physical examination, the essential elements of the preoperative assessment in all patients are listed in Box 3.1. Patients and parents may be anxious about the occurrence or recurrence of adverse perianesthetic events such as those listed, and they should be reassured that efforts will be made to prevent these events from happening.

Patient History Documentation of allergy status is an essential part of the preoperative evaluation, particularly because prophylactic antibiotics may be administered prior to the incision. Allergies to certain antibiotics (especially penicillin, ampicillin, and cephalosporins) are the most common medication allergies in children presenting for an operation. Anaphylactic allergic reactions are rare, but can be life threatening if not diagnosed and treated promptly. Latex allergy is the most

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common etiology for an anaphylactic reaction, and children with spina bifida (myelomeningocele), bladder exstrophy, or those who have undergone multiple surgical procedures are at greatest risk for such reactions.26 In 1991, the FDA recommended that all patients should be questioned about symptoms of latex allergy prior to surgery. The general consensus among the pediatric anesthesia community is that children in the high-risk groups noted above should not be exposed to latex-containing products (e.g., gloves, adhesive tape, catheters) and latex-free alternatives should be used instead. Since 1997, the FDA has mandated that all latexcontaining medical products should be labeled as such. Many pediatric hospitals have elected to remove all latexcontaining products from their supply chain because of the high risk to these identified patient populations as well as the increasing prevalence of latex allergy in health care workers.27,28 It has been well documented that prophylactic medications (steroids, H1 and H2 blockers) are ineffective in preventing anaphylaxis in susceptible patients. If anaphylaxis occurs (hypotension, urticaria or flushing, bronchospasm), the mainstays of treatment are (1) stopping the latex exposure: stopping the operation, changing to nonlatex gloves, and removing any other sources of latex; and (2) resuscitation: fluids, intravenous (IV) epinephrine (bolus and infusion), steroids, diphenhydramine, and ranitidine.29 If anaphylaxis is suspected, blood should be drawn within 4 hours of the episode for tryptase determination, which can confirm the occurrence of an anaphylactic event but not the inciting agent. Patients should be referred to an allergist for definitive testing to identify the antigen. Such testing should occur at least 4–6 weeks after the episode of anaphylaxis to allow for reconstitution of the mediators, the depletion of which could cause a false-negative skin test. In general, parents should be instructed to continue routine administration of anticonvulsant medications, cardiac medications, and pulmonary medications even while the child is fasting. Family history should be reviewed for pseudocholinesterase deficiency (prolonged paralysis after succinylcholine) or any first-degree relative who experienced malignant hyperthermia (MH). A complete review of systems is important and should focus on those areas in which abnormalities may increase the risk of adverse events in the perioperative period. 

Miscellaneous Conditions Malignant Hyperthermia Susceptibility The incidence of an MH crisis is 1:15,000 general anesthetics in children, and 50% of patients who have an MH episode have undergone a prior general anesthetic without complication. MH is an inherited disorder of skeletal muscle calcium channels, triggered in affected individuals by exposure to either inhalational anesthetic agents (e.g., isoflurane, desflurane, sevoflurane), succinylcholine, or both in combination, resulting in an elevation of intracellular calcium. The resulting MH crisis is characterized by hypermetabolism (fever, hypercarbia, acidosis), electrolyte derangement (hyperkalemia), arrhythmias, and skeletal muscle damage (elevated creatine phosphokinase [CPK]). This constellation of events may lead to death if unrecognized and/or untreated. Dantrolene, which reduces the release of calcium from muscle sarcoplasmic reticulum,

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Box 3.2  Treatment of Malignant Hyperthermia Crisis “Some Hot Dude Better Give Iced Fluids Fast”12 Stop all triggering agents, administer 100% oxygen Hyperventilate: treat Hypercarbia Dantrolene (2.5 mg/kg) immediately Bicarbonate (1 mEq/kg): treat acidosis Glucose and Insulin: treat hyperkalemia with 0.5 g/kg glucose, 0.15 units/kg insulin Iced Intravenous fluids and cooling blanket Fluid output: ensure adequate urine output: Furosemide and/or mannitol as needed Fast heart rate: be prepared to treat ventricular tachycardia

Box 3.3  Muscle Diseases Associated with Malignant Hyperthermia Central core myopathy Becker muscular dystrophy Duchenne muscular dystrophy Myotonic dystrophy King–Denborough syndrome

when given early in the course of an MH crisis, has significantly improved patient outcomes. With early and appropriate treatment, the mortality is now less than 10%. Current suggested therapy can be remembered using the mnemonic “Some Hot Dude Better GIve Iced Fluids Fast” and is summarized in Box 3.2.30 Experts are available for consultation concerning suspected MH at the 24-hour MH hotline administered by the Malignant Hyperthermia Association of the United States (MHAUS). Recommendations for treatment of an acute MH episode are available at the MHAUS website.31 It should be noted that dantrolene must be prepared at the time of use by dissolving in sterile water. It is notoriously difficult to get into solution, and the surgeon may be asked to help with this process. Recently an alternative to dantrolene, dantrium, has become available. It is more soluble at higher concentration and therefore more quickly and easily prepared, allowing administration of a lower volume of drug for effective treatment. Patients traditionally thought to be MH susceptible include those with the spectrum of muscle diseases listed in Box 3.3. However, many patients who develop MH have a normal history and physical examination. In the past, patients with mitochondrial disorders were thought to be at risk. Recent evidence suggests that the use of inhaled anesthetic agents appears safe in this population, but succinylcholine should still be avoided, as some patients may have rhabdomyolysis (elevated CPK, hyperkalemia, myoglobinuria) with hyperkalemia without having MH. Patients with myopathies of unknown origin, often presenting for diagnostic muscle biopsy, pose a unique dilemma, and anesthetics should be planned in consultation with genetic and metabolic teams if possible.32  Trisomy 21 Perioperative complications occur in 10% of patients with trisomy 21 who undergo noncardiac surgery and include

severe bradycardia, airway obstruction, difficult intubation, post-intubation croup, and bronchospasm. Patients may experience airway obstruction due to a large tongue and mid-face hypoplasia. The incidence of obstructive sleep apnea (OSA) may exceed 50% in these patients and may worsen after anesthesia and operation. Airway obstruction may persist even after adenotonsillectomy.33 Many patients with trisomy 21 have a smaller caliber trachea than children of similar age and size; therefore, a smaller endotracheal tube (ETT) may be required. Some trisomy 21 patients may have a longer segment of tracheal stenosis due to complete tracheal rings below the level of the cricoid.34 Congenital heart disease (CHD) is encountered in 40–50% of patients with trisomy 21. The most common defects are atrial and ventricular septal defects, tetralogy of Fallot, and atrioventricular (AV) canal defects. For children with a cardiac history, records from their most recent cardiology consultation and echocardiogram should be available for review at the time of preoperative evaluation. Recent clinical changes in their condition may warrant reassessment by their cardiologist prior to operation. Patients with trisomy 21 have laxity of the ligament holding the odontoid process of C2 against the posterior arch of C1, leading to atlanto-axial instability in about 15% of these patients. Cervical spine instability can potentially lead to spinal cord injury in the perianesthetic period. The need for and utility of preoperative screening for this condition is controversial. Even if the radiographic exam is normal, care should be taken perioperatively to keep the neck in as neutral a position as possible, avoiding extreme flexion, extension, or rotation, especially during tracheal intubation and patient transfer. Any patient with trisomy 21 who has neurologic symptoms such as sensory or motor changes, or loss of bladder or bowel control should undergo preoperative neurosurgical consultation to exclude cervical cord compression. 

PREOPERATIVE FASTING GUIDELINES Fasting violations are one of the most common causes for cancellation or delay of operations. Preoperative fasting is required to minimize the risk of vomiting and aspiration of particulate matter and gastric acid during anesthesia induction. Although the risk of aspiration is generally small, it is a real risk that may be associated with severe morbidity or death. Research performed at our institution has demonstrated that intake of clear liquids (i.e., liquids that print can be read through, such as clear apple juice or Pedialyte) up until 2 hours prior to the induction of anesthesia does not increase the volume or acidity of gastric contents.35 Our policy is to recommend clear liquids until 2 hours prior to the patient’s scheduled arrival time. Breast milk is allowed up to 3 hours before arrival for infants up to 12 months of age. Infant formula is allowed until 4 hours before arrival in infants 11 years of age) may be preferable. Although it is easiest to perform a urine test for human chorionic gonadotropin (hCG), if a patient cannot provide a urine sample, blood can be drawn for serum hCG testing. Institutional policy may allow the attending anesthesiologist to waive pregnancy testing at their discretion. Certain medications, particularly anticonvulsants, should be individually assessed regarding the need for preoperative blood levels. The nature of the planned operation may also require additional studies, such as coagulation screening (prothrombin time [PT], partial thromboplastin time [PTT], international normalized ratio [INR]) prior to craniotomy, tonsillectomy, or surgeries with anticipated large blood loss. 

Clinical Scenarios and High-Risk Populations UPPER RESPIRATORY TRACT INFECTION Because perioperative respiratory adverse events are the most common cause of significant adverse events in infants and children, one of the most common questions confronting an anesthesiologist is whether to cancel a procedure in a child with an upper respiratory infection (URI). It is not uncommon for some patients to spend much of their childhood catching, suffering from, or recovering from a URI, with the highest frequency occurring in children under age 6 who attend day care or preschool.36 Patients with

39

a current or recent URI undergoing general anesthesia are theoretically at increased risk for adverse perioperative respiratory complications, including laryngospasm, bronchospasm, and hypoxia, with the youngest patients (20% TBSA burn are resuscitated using the following formula: 3 mL × weight in kg × % TBSA= total mL to be given over the first 24 hours minus the preadmission volume (mL) already received. We divide the total fluid volume by 2 and administer half of the fluid over the first 8 hours from the time of the burn injury and the remaining half over the ensuing 16 hours. In the presence of known or suspected inhalation injury, our resuscitation formula will be based

on 6 mL instead of 3 mL. For children >20 kg, we use LR as our resuscitation fluid of choice and for those 20 kg, our goal UOP is >0.5 mL/kg/h. For patients 1 mL/kg/h. For UOP of >1 mL/kg/h or 6 mL/kg/h without inhalation injury or >9 mL/kg/h with cutaneous burns and inhalation injury), we reclassify these patients as difficult to resuscitate. We then institute our difficult to resuscitate algorithm (Table 13.2).

RESUSCITATION ENDPOINTS The key to successful resuscitation is based on constant reassessment of the physiologic status of the patient. Overand/or under-resuscitation is equally fraught with complications. Historically, UOP has been the primary parameter used for assessing fluid status in burn patients. Goal UOP should be 1 mL/kg/h for children less than or equal to 20 kg and 0.5 mL/kg/h for children greater than 20 kg. It is important to mention that UOP has long been used as a surrogate of perfusion in critical care, although it is several steps removed from the indirect measurement of cardiac output and has a weak relevance to actual tissue oxygenation.35 With this in mind, using UOP as a sole or major indicator of adequate tissue perfusion can lead to misinterpretation of fluid status. A global assessment including mental status, vital signs, invasive hemodynamic monitoring when indicated, and trends in laboratory values such as lactate and base deficit should be used to more accurately monitor resuscitation in large burn victims. The concept of permissive oliguria has even been proposed as an appropriate management strategy in burn patients and is one that we occasionally use at our own institution.36 Inadequate resuscitation and/or over-resuscitation can result in hypoperfusion to the zone of stasis, with subsequent deepening of the burn, as well as hypoperfusion of major organs. During the first 6–12 hours, capillary permeability is increased and fluid moves from the intravascular space to the interstitial tissues, with worsening edema. Overly aggressive fluid administration can result in significant tissue edema, tissue hypoxia, and elevated compartment pressures, possibly necessitating surgical decompression of the affected body cavity or extremity.37 Several surgeons have shown that the addition of colloid solutions such as albumin can reduce crystalloid requirements and more rapidly establish a balanced fluid intake to output ratio.38,39 However, this is an area of significant controversy in burn resuscitation. In theory, the use of colloids such as plasma, albumin, and dextran should mitigate the effect of losing plasma into the extravascular space. It has been shown that the capillary integrity seen in the first 8–24 hours after burn injury is not enough to preclude the efflux of colloids into the extravascular space, therefore not affecting the oncotic pressure of the intravascular space enough to maintain adequate intravascular volume.40 This effect is limited to approximately the first 12 hours. It has been suggested that there may be some benefit to the use of colloids in the second half of the resuscitation algorithm. At our institution, for difficult to resuscitate patients, we change half of our resuscitation fluids to albumin only after the first 8 hours. Although there are no randomized controlled studies comparing the use of colloids

Table 13.2  Difficult to Resuscitate Algorithm 1. Once you begin this algorithm, please ensure the following: a. Foley and central line if not already in place, preference for subclavian or internal jugular vein to monitor CVP (triple) b. Echo. Consider a fluid bolus if the heart appears underfilled (10 mL/kg) c. Bladder pressures q4h d. Labs as per the difficult to resuscitate guideline 2. Hemodynamic parameters: a. CVP goals: More useful as a trend as opposed to one single measurement, even from a femoral line; however, high lines are preferred. In general, we would aim for a CVP goal of 5–12 b. SVO2 goals >60%; consider near-infrared spectroscopy monitoring if cerebral/somatic locations are not burned c. Mean arterial blood pressure >55 d. Algorithm. This is to be carried out in addition to the hourly IVF titration on the Burn Resuscitation Flow Sheet. Change to ½ albumin and ½ D5LR or LR (based on weight) at previous IVF rate if you are at least 8 h post injury. With hypotension or other evidence of poor end organ perfusion. Consider → Start NE at 0.02 mcg/ epinephrine (Epi) or norepinephrine kg/min. If CVP or UOP (NE) based on: remains low, consider 1. Clinical exam findings consistent fluid bolus (10 mL/kg) or with vasodilation and/or wide pulse increasing IVF by 20% pressure OR OR → Start Epi at 0.02 mcg/ 2. Echo showing poor function kg/min and/or clinical exam consistent with vasoconstriction and cold extremities ↓ Assess BP in addition to SVO2, → If improved, continue CVP, and UOP current management ↓ If continued hypotension or other evidence of poor end organ perfusion: Titrate the Epi or NE by 0.02 mcg/kg/min up to 0.1 mcg/kg/min to obtain hemodynamic parameters above. Notify pediatric intensive care unit provider if patient requires 0.1 mcg/kg/min. ↓ Assess BP in addition to SVO2, → If improved, continue CVP, and UOP current management ↓ If continued hypotension or other evidence of poor end organ perfusion: Start vasopressin 2 milliunits/kg/min. DO NOT TITRATE. ↓ Assess BP in addition to SVO2, → If improved, continue CVP, and UOP current management ↓ If continued hypotension or other evidence of poor end organ perfusion consider: 1. Repeat echo 2. Missed injury, hemorrhage, compartment syndrome 3. CN poisoning, methemoglobinemia, drug ingestion 4. Stress dose hydrocortisone. Trial 2 mg/kg IV × 1. If clinical response seen, schedule 0.5 mg/kg IV q6h × 24 h. 5. Discuss next tier therapy with ICU burn physician BP, blood pressure; CN, cyanide; CVP, central venous pressure; IVF, intravenous fluid; LR, lactated Ringer’s; UOP, urine output. Courtesy Jenna Miller, MD and Jennifer Flint, MD, Children’s Mercy Hospital, Kansas City, MO.

13 • Burns

versus crystalloids in burn victims, a 2011 Cochrane Database Review found that albumin does not lower mortality in adults with major burns.41 A more recent metaanalysis of controlled clinical studies also failed to show a mortality benefit unless two of the studies with a high risk of bias were excluded.33 All of these studies fall short of clear evidence, but that is likely due to the heterogeneity of the population and of the studies. Other solutions, such as hypertonic saline, have been used in an effort to provide a high osmotic pressure, which is thought to keep more volume in the intravascular space. Hypertonic saline also may have anti-inflammatory effects. Hypertonic saline should be used with caution, as it causes hypernatremia, and has not been shown to improve outcomes for hypotensive trauma patients.42 Unlike adults, children do not show hemodynamic changes reflecting hypovolemia until they are significantly volume depleted. Tachycardia may be a sign of compensation for a low-volume state or stress response to injury. Signs of inadequate perfusion include lethargy and decreased capillary refill with cool, clammy extremities. Laboratory tests should be performed along with serial clinical exams to follow the response to resuscitation. Resolving acidosis, for example, may serve as an objective marker of improvement. Hyponatremia is a frequent complication in pediatric burn patients after aggressive fluid resuscitation, and correction is required to avoid severe electrolyte imbalance. 

Inhalation Injury Concomitant or isolated inhalation injury can lead to increased mortality or significant morbidity in all burn victims. For infants and children with large burns, the incidence of associated inhalation injury has been reported to be between 20% and 30%.43 One large multicenter review of pediatric burn patients diagnosed with inhalation injury demonstrated an overall mortality of 16%, with the majority of deaths resulting from sepsis and pulmonary dysfunction.44 Mortality increased to 50% if the patients required more than 1 week of mechanical ventilation. In an enclosed space, damage can occur via two mechanisms: heated air and inhaled gasses. With heated air, most of the damage occurs in the upper airway as the reflexive glottis closes and the heated air cools significantly, causing minimal to no direct damage to the lower airways but potentially causing significant damage to the upper airway. The subsequent development of erythema or ulceration of the oropharynx can then lead to worsening obstruction over the first several hours after the injury. Additionally, aggressive fluid resuscitation can lead to worsening tissue edema in the upper airway that can manifest as hoarseness, stridor, or dyspnea.45 Injury below the vocal cords occurs as a consequence of inhaled smoke. For enclosed fires, the greatest risk of immediate mortality and morbidity is carbon monoxide (CO) and hydrogen cyanide (HCN) toxicity. Smoke from burning wood generates high concentrations of CO and aldehydes while the burning of synthetic material produces HCN. Both CO and HCN produce concentration-dependent hypoxia at the cellular level, but the mechanism of each is distinct. CO has a >200 times affinity for hemoglobin than does oxygen

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and thus displaces oxygen from hemoglobin. The oxyhemoglobin dissociation curve is shifted to the left as the ability of hemoglobin to unload oxygen in the tissues is decreased. This leads to decreased perfusion of oxygenated blood to organs and cells, leading to organ and cellular damage. Prolonged exposure leads to high concentrations of CO in the blood that leads to profound hypoxia, brain damage, and brain death.46 HCN, on the other hand, also leads to tissue hypoxia but does this by disrupting mitochondrial generation of adenosine triphosphate through the binding of ferric ions in cytochrome c oxidase, which subsequently blocks aerobic cellular metabolism.47 Damaged epithelium in the lung due to activation of immune systems by inhaled smoke then releases vasoactive substances (thromboxanes A2, C3a, and C5a) that lead to hypoxia, increased airway resistance, decreased pulmonary compliance, increased alveolar epithelial permeability, and increased pulmonary vascular resistance.48 Secondary injury is due to impaired ciliary clearance of airway debris. Neutrophil infiltration occurs, macrophages are destroyed, and bacteria accumulate, leading to pneumonia. Diagnosing inhalation injury should commence with a detailed history of the events related to the burn and a careful primary and secondary exam. Patients found in enclosed buildings or spaces are at high risk of inhalation injury. Physical exam findings such as facial burns, singed hairs in the nares, eyebrows, or head, and/or carbonaceous sputum are all nonspecific signs of smoke inhalation. If CO toxicity or exposure is suspected, carboxyhemoglobin (COHb) levels can be obtained and are readily available in most major hospitals. Approximately 5% of inhalation injuries in children involve inhaling CO.45 It is important to keep in mind that a normal or near normal serum carboxyhemoglobin level does not exclude CO toxicity, particularly in patients who have been on high flow oxygen for extended periods of time prior to arriving at the hospital. Symptoms of CO toxicity with COHb levels of 15–40% include headaches, flu-like symptoms, blurred vision, nausea, vomiting, and collapse. At levels above 40%, loss of consciousness, seizures, Cheyne–Stokes respirations, and death can occur.49 HCN is a gaseous form of cyanide, and while small amounts can safely be metabolized in the liver, larger amounts inhaled through the lungs overwhelm hepatic metabolism, leading to toxic levels.50 Neurologic deficits, persistent and unexplained acidosis, and serum lactate levels >8 mmol/L are major manifestations of cyanide toxicity. Persistent hypotension, cardiac arrhythmias, persistent metabolic acidosis, persistently increased lactate, and decreased serum or mixed venous oxygen with adequate resuscitation are signs that are consistent with profound hypoxia that can be seen with HCN toxicity.45 Cyanide levels can be measured directly in the blood or indirectly with serum lactate, anion gap, and methemoglobin concentrations. Any signs of impending respiratory difficulty should be carefully monitored as respiratory collapse can occur quickly and endotracheal intubation may become difficult in the face of significant tissue edema in the oropharynx. Chest films in the acute setting are nondiagnostic of inhalation injury. Although there are radiographic imaging modalities such as CT scans and xenon lung scanning with xenon-133 isotope, the most widely used and reliable

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&

Fig. 13.5  A superficial partial-thickness burn of the lower extremity is shown. (A) The characteristic blisters with this degree of burn injury are seen. (B) After removal of the blister, the wounds are painful and blanch when pressure is applied. (C) After several days of daily dressing changes with topical antimicrobials, the burn wounds are healing nicely.

method of diagnosing extent, severity, and progression of inhalation injury continues to be fiberoptic bronchoscopy.51 Management of pediatric burn patients with suspected inhalation injury starts with the establishment of an adequate and stable airway, and assessment of the risk of CO and HCN toxicity. Due to their immature neurologic and physical development, children, particularly young children, are unable to escape from the scene of an enclosed fire and their exposure to inhaled toxins can be significant. After the airway is secured, an inhalation treatment protocol is utilized in the intensive care unit (ICU) that focuses on the clearance of secretions and control of bronchospasm. Humidified 100% high-flow oxygen should be administered to displace CO from hemoglobin. The half-life of COHb is 60 minutes when 100% FiO2 is administered compared to 5 hours on room air.45 Although there have been many study design flaws, to date there is no conclusive evidence that supports the routine use of hyperbaric oxygenation for CO poisoning.52 For treatment of HCN toxicity, hydrocobalamin has shown some efficacy. Hydrocobalamin is a cobalt compound that binds to cyanide and transforms cyanide to cyanocobalamin, which is then excreted in the urine. Current evidence, however, does not support the empiric administration of the drug.53 Sodium thiosulfate, a chemical that binds to cyanide to donate a sulfur group to form the less toxic compound thiocyanate, can also be used. Avoid using nitrites in pediatric patients. Early and aggressive pulmonary therapy consisting of chest physiotherapy, frequent suctioning, and early mobilization should be started on all patients with a confirmed diagnosis of inhalation injury. Bronchodilators and racemic epinephrine are used to treat bronchospasm. Clearance of secretions can be assisted with inhalation treatments composed of heparin and acetylcysteine. Human autopsy and animal models have shown nebulized heparin (5000– 10,000 units/3 mL of NS q5hours) to reduce tracheobronchial cast formation improves minute ventilation and decreases peak inspiratory pressures after smoke inhalation.54–56 The addition of 20% acetylcysteine (3 mL q4h) also improves the clearance of tracheobronchial secretions and minimizes bronchospasm. Pediatric and adult studies have shown this combination of medications decreases

reintubation rates and reduces mortality.57–59 Our protocol uses 5000 units of nebulized heparin every 4 hours alternating with 3 mL of 20% N-acetylcysteine every 4 hours for 7 days or until extubated, whichever comes first. 

Assessment of Burn Depth The accurate measurement of burn depth and TBSA of the burn victim is central to the management of these patients. Historically, a structural-anatomical classification into four categories of increasing depth of the injury has been utilized to characterize the depth of a burn.60 These categories are epidermal, superficial partial thickness, deep partial thickness, and full thickness. First-, second-, third- and fourthdegree are no longer a part of the professional jargon among burn care providers or in the literature. Epidermal burns, although exquisitely painful, require no special medical attention. Superficial partial-thickness burns extend into the papillary dermis and are generally characterized by blisters and blanching tissue with pressure (Fig. 13.5). They are also painful when the underlying viable tissue is exposed. In otherwise healthy patients, these wounds will reepithelialize within 7–10 days with no untoward longterm functional or cosmetic deficits. On the other hand, deep partial-thickness burns extend into the reticular dermis and involve variable amounts of damage to skin appendages such as hair follicles. In these wounds, blanching is delayed, and the surface of the wound may be white and mottled (Fig. 13.6). Also, these wounds are usually less painful. Full-thickness burns involve the entire dermis and extend into the subcutaneous tissue. These appear charred, leathery, and firm (Fig. 13.7). Patients typically are insensate in the burned regions and may not feel pressure. Blanching does not occur when pressure is applied. Full-thickness injuries should be excised and grafted early.61 Determination of burn wound depth can sometimes be difficult. Initial evaluation by an experienced surgeon as to whether an indeterminate dermal burn will heal in 3 weeks is only about 50–70% accurate.62–64 Scald injuries are particularly difficult to assess for depth and extent of injury.

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Wound Care

Fig. 13.6  A deep partial-thickness burn of the right upper extremity is seen. Note the mottled and white appearance of the extremity.

Burn surgeons prefer to divide burn wounds into one of two categories: superficial wounds that will heal without surgery and deep wounds that will require surgical intervention. For deep partial-thickness burns, the management strategy may not be entirely clear. Many partial-thickness burns can be managed nonoperatively for 10–14 days with topical therapies and dressings. Using this strategy, the goal of burn care is to provide an optimal environment for reepithelialization by providing a warm and moist environment, removal of exudate and potentially contaminated or necrotic material (eschar), and control of bacterial proliferation. Deep partial-thickness burns should be excised and grafted if the surgeon does not believe that the wounds will heal by 3 weeks. Distinguishing between superficial and deep partial-thickness burns is not always clear and can be challenging even for experienced burn surgeons. This implies a great deal of clinical expertise because one would have to make that decision between post burn day 10 and 14 at the latest. It has been shown that if a partial-thickness burn wound heals within 2 weeks, scarring is unlikely to occur, but after 3 weeks, the risk of hypertrophic scar formation is extremely high.73,74 The burn surgeon should strive to excise and graft all known deep partial-thickness burns and all deep burns within the first 24–48 hours when possible.

ANTIMICROBIAL AGENTS

Fig. 13.7  This child sustained a full-thickness scald burn to the right thigh. The periphery of the burn is a deep partial-thickness injury.

A number of techniques or tools have been described to improve accuracy. These techniques utilize the physiology of the skin and the alterations that occur with burn injury. Detection of dead cells or denatured collagen using ultrasound (US), biopsy, or vital dyes has been trialed.65–68 Appropriate US equipment is expensive; biopsies are invasive, painful, lead to scarring; and interpretation of both modalities requires an experienced pathologist and radiologist. Other technologies such as analyzing altered blood flow using fluorescein, laser Doppler imaging (LDI), and thermography have shown some promise.69–71 LDI, in particular, has been shown to increase the accuracy of burn depth assessment when compared with evaluation by experienced burn surgeons. LDI measures the extent of the superficial microvascular blood flow, which can then correlate with the depth of the burn. Studies utilizing LDI in pediatric patients suggest a high positive predictive value and negative predictive value, providing a more accurate estimation and an earlier determination of burn depth than clinical judgment alone.72 

The initial treatment of partial-thickness burns is debridement and coverage with a topical agent or dressing that has antibacterial properties and allows for separation of the burn eschar when present.75–77 Various topical antimicrobial agents have been used (Table 13.3). These agents decrease bacterial content, but they do not eradicate or prevent colonization. Silver sulfadiazine (Silvadene, Monarch Pharmaceuticals Inc., Bristol, TN) is currently the most commonly used topical antimicrobial agent for burn care around the world. It is a white, highly insoluble compound synthesized from silver nitrate and sodium sulfadiazine.78 It has a broad spectrum of efficacy including against Staphylococcus aureus, Escherichia coli, Klebsiella, Pseudomonas, and Proteus species. It also possesses an analgesic effect but does not penetrate eschar well. The most common side effect from silver sulfadiazine is leukopenia, which is caused by margination of leukocytes, and is usually transient.79 It occurs in somewhere between 5% and 15% of patients treated.80 Changing to another topical antimicrobial agent usually resolves this side effect. Silver sulfadiazine has significant side effects in children and should be used in neonates with caution, as it increases the risk of kernicterus. It has also been shown to delay reepithelialization, can form a pseudoeschar that precludes wound evaluation, is painful to clean off, and can cause severe ocular irritation if used on the face. Most centers in the United States have moved away from using silver sulfadiazine on a regular basis, and it is reserved for only select cases. Additionally, a 2013 Cochrane Review showed that silver sulfadiazine was associated with worse outcomes than biosynthetics and silicon-coated and silver dressings while hydrogel-treated burns had better healing outcomes.81

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Table 13.3  Burn Wound Dressings Dressings

Advantages

Disadvantages

ANTIMICROBIAL SALVES Silver sulfadiazine (Silvadene)

Painless; broad spectrum; rare sensitivity

Leukopenia; some Gram-negative resistance; mild inhibition of epithelialization Painful; metabolic acidosis; mild inhibition of epithelialization

Mafenide acetate (Sulfamylon) Bacitracin/Neomycin/Polymixin B Nystatin Mupirocin (Bactroban)

Broad spectrum; penetrates eschar; effective against Pseudomonas Ease of application, painless, useful on face Effective in inhibiting fungal growth; use in combination with Silvadene, Bacitracin Effective against Staphylococcus, including MRSA

Limited antimicrobial property Cannot use in combination with mafenide acetate Cost; poor eschar penetration

ANTIMICROBIAL SOAKS 0.5% Silver nitrate

Painless; broad spectrum; rare sensitivity

Povidone-iodine (Betadine)

Broad-spectrum antimicrobial

5% Mafenide acetate 0.025% Sodium hypochlorite (Dakin’s solution) 0.25% Acetic acid

Broad-spectrum antimicrobial Effective against most organisms

No eschar penetration; discolors contacted areas; electrolyte imbalance; methemoglobinemia Painful; potential systemic absorption; hypersensitivity Painful; no fungal coverage; metabolic acidosis Mildly inhibits epithelialization

Effective against most organisms

Mildly inhibits epithelialization

Broad-spectrum antimicrobial; no dressing changes

Cost

Provides wound barrier; minimizes pain; useful for outpatient burns, hands (gloves) Provides moisture barrier; minimizes pain; useful for outpatient burns; inexpensive Provides wound barrier; accelerates wound healing Complete wound closure, including dermal substitute

Exudate accumulation risks invasive wound infection; no antimicrobial property Exudate accumulation risks invasive wound infection; no antimicrobial property Exudate accumulation risks invasive wound infection; no antimicrobial property No antimicrobial property; expensive; requires training, experience

Temporary biologic dressings

Requires access to skin bank; cost

Minimizes dressing changes

Not widely used

SILVER IMPREGNATED Aquacel, Acticoat SYNTHETIC DRESSINGS Biobrane OpSite, Tegaderm Transcyte Integra, Alloderm BIOLOGIC DRESSINGS Allograft (cadaver skin), Xenograft (pig skin) Amniotic membrane

MRSA, methicillin-resistant Staphylococcus aureus.

Mafenide acetate (Sulfamylon, UDC Laboratories, Inc., Rockford, IL) was introduced as a topical burn agent in the mid-1960s. It is more effective in penetrating eschar and is frequently used in full-thickness burns. It has broad activity against most Gram-negative and Gram-positive pathogens, but unfortunately has minimal antifungal activity.82 The application of Sulfamylon can be painful, which limits its practical use in the outpatient setting. Sulfamylon is a potent carbonic anhydrase inhibitor and can therefore cause metabolic acidosis.83 This side effect can usually be avoided by limiting its use to only 20% TBSA at any given time, and rotating application sites every several hours with another topical antimicrobial agent. Silver nitrate (0.5%) was also introduced in the mid1960s. It is typically used to soak gauze dressings, thereby avoiding frequent dressing changes with the potential loss of grafts or healing cells. Silver nitrate is painless on application and has broad coverage. Unfortunately, the compound can cause hyponatremia and hypochloremia while also creating dark gray or black stains. Another important but infrequent complication is methemoglobinemia, which occurs as a result of nitrate reduction by wound bacteria followed by the systemic absorption of the toxic nitrite.

Dressings containing biologically active silver ions (Aquacel, ConvaTec Ltd., UK; Acticoat, Smith & Nephew, London, UK) hold promise for retaining the effectiveness of silver nitrate but without its side effects. Several favorable clinical trials utilizing these products have been conducted and have found them to be as effective as traditional dressings utilizing silver nitrate. The products were also noted to be less painful than traditional dressings when applied and removed, and also were associated with decreased burn wound cellulitis.84–86 Facial burns, small areas of partial-thickness burns, and healing donor sites require special mention. On superficial facial wounds, silver sulfadiazine may retard epithelialization and should not be placed on the face.87 An alternative is petroleum-based antimicrobial ointments. These include polymyxin B, bacitracin, and polysporin. Their application is painless and transparent, which allows for easier monitoring. These agents are mostly effective against Grampositive organisms. Proteolytic enzymatic agents have been utilized to debride wounds, including proteases (sutilains) elaborated from Bacillus subtilis, collagenase, and papain-urea. Collagen is a protein that is found normally in skin (∼75%

13 • Burns

of dry weight of skin), and is the dominant protein that must be lysed to allow for eschar separation. Collagenase is an exogenous enzyme that breaks down denatured collagen but does not lyse healthy, normal collagen. Collagenase Santyl ointment (Healthpoint Biotherapeutics, Fort Worth, TX) is used in many burn units for the treatment of partial-thickness burns. The use of collagenase should be reserved for partial-thickness burns with some eschar formation and should not be used on a routine basis. 

BURN WOUND DRESSINGS The concept of an “optimal environment” is derived from the work carried out by Winter in 1962.88 In young pigs, he found that partial-thickness wounds that were kept moistened with polyethylene film epithelialized twice as fast as those left exposed to air. Hinman and Maiback confirmed this observation with a series of human volunteers.89 Therefore, for >60 years, it has been felt that a burn dressing should provide an “optimal environment” while also possessing bacterial inhibition. Typically, dressings used to cover burn wounds consist of a synthetic mesh that is either impregnated with antimicrobial ointments or compounds such as silver, or are positioned on top of ointments/creams to keep them in place between dressing changes. Nonadherent dressings such as Telfa (Tyco Healthcare Group LP, Mansfield, MA), Xeroform (Tyco Healthcare Group LP, Mansfield, MA), Adaptic (Johnson & Johnson, New Brunswick, NJ), or Mepitel (Molnlycke Health Care AB, Gothenburg, Sweden) can be placed directly on the wound to help reduce both the pain associated with dressing changes and the friction associated damage to the wound or skin graft. The nonadherent dressing and antimicrobial compound serve to provide the “optimal environment” for reepithelialization. Further advancements with burn dressings have rec­ ently led to a number of synthetic products designed to adhere to wounds until epithelialization has occurred. The benefits of these dressings include less pain related to fewer dressing changes. These dressings are very effective for superficial partial-thickness wounds. Deep wounds and those with excessive drainage do not allow adherence, and therefore negate the benefits of these synthetic dressings. An example of a synthetic mesh product is Biobrane (UCL Laboratories, Rockford, IL). It is a bilaminate thin membrane composed of thin semipermeable silicone bonded to a layer of nylon mesh, which is coated with a monomolecular layer of type I collagen of porcine origin. This dressing provides a hydrophilic coating for fibrin ingrowth, which promotes wound adherence. The dressing is placed on a clean fresh superficial partial-thickness burn wound and can be secured using Steri-Strips and/or bandages. This dressing is easily removed from the wound bed as the wound epithelializes underneath it. Fluid can accumulate under the dressing and can be aspirated, if needed. However, if a foul-smelling exudate is detected, the Biobrane should be removed and an antimicrobial dressing applied. Dressings that are commonly utilized for coverage of postoperative incisions may also be used as small partialthickness burn dressings. These alternatives include Duoderm (ER Squibb & Sons, Inc., Princeton NJ), Opsite (Smith

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& Nephew, London, UK), and Tegaderm (3M Pharmaceuticals, St. Paul, MN). Despite lacking special biological factors (collagen and growth factors), these dressings provide a cheap and transparent alternative to more expensive dressings. Also, Duoderm has been found to be less expensive than Biobrane and therefore could be a first-line treatment option for intermediate-thickness burn wounds in children.90 The disfigurement resulting from large full-thickness burns has decreased with the advent of combined synthetic and biologic materials. Integra (Integra LifeSciences Corp., Plainsboro, NJ) is a dermal substitute that can be used in larger burns where there may not be enough skin to cover the wound. It has an inner layer composed of a porous matrix of bovine collagen and the glycosaminoglycan chondroitin-6-sulfate, which facilitates fibrovascular ingrowth.91 The outer layer is a polysiloxane polymer with vapor transmission characteristics similar to normal epithelium. Integra acts as a dermal replacement. It provides a matrix for the infiltration of fibroblasts, macrophages, lymphocytes, and capillaries from the wound bed, and promotes rapid neo-dermis formation. Approximately 2–3 weeks after engraftment, the outer silicone layer is removed and is replaced with a very thin epidermal split-thickness autograft (Fig. 13.8). Integra-covered wounds have less scarring but are susceptible to infection and must be monitored carefully. Its advantages were validated in a randomized study in children with large TBSA burns.92 In this study, burn wounds in children treated with Integra demonstrated significantly decreased resting energy expenditure as well as increased bone mineral content and density. Also, improved scarring was found at 24 months after burn injury. Biological dressings include xenografts from swine and allografts from cadaver donors such as Alloderm (LifeCell Corp., Branchburg, NJ). They are especially useful for coverage of large full-thickness burns. The dressings are eventually rejected by the patient’s immune system and slough. The wound beds become excellent recipient beds for subsequent autografts. Although extremely rare, the transmission of viral diseases from the allograft is a potential concern. These dressings are useful adjuncts when autografts are not available or time is needed for donor sites to heal before being used again for grafting. 

EXCISION AND GRAFTING Prompt burn excision and grafting has been shown to improve survival, decrease length of hospitalization, and reduce costs in burn patients of all ages. Children particularly have benefited from more timely and extensive operative management.91–96 Deep burns or deep partial-thickness burns that fail to heal quickly will require tangential excision in order to minimize the infectious and scarring complications associated with these wounds. Early excision was originally described by Janzekovic in 1970.97 The eschar is sequentially shaved using a dermatome, knife blade or, more recently, a Versajet (Smith & Nephew, Inc., London, UK) water dissector until a viable tissue bed is reached.98 In a prospective randomized trial, the Versajet technique was shown to produce a more precise and faster excision than hand-held dermatome escharectomy.99

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$

%

Fig. 13.8  The use of Integra for a superior cosmetic result in a child with facial burns is depicted. (A) The application of the Integra with the silicone layer in place. Approximately 2 weeks after placement, the silicone layer is removed and split-thickness skin grafts are placed. (B) The same child after skin grafting over the Integra.

After excision, coverage is ideally completed with an autograft. Split-thickness autografts (0.008–0.012 inch thickness) are harvested and utilized as a sheet (unmeshed) or meshed graft. Sheet grafts provide better long-term aesthetic outcomes but are complicated by the development of a seroma or a hematoma, and also limited coverage. Narrow meshed autografts (1:1 or 1:2) have the advantages of limiting the total surface area of donor harvest and allowing better drainage of fluid under the grafted sites. In larger burns (>20–30%), coverage may require a combination of a meshed autograft and allograft. The meshed autografts (4:1 to 6:1) can be covered with meshed allograft (2:1) overlays.100 Alternatively, grafting with sequential harvesting of split-thickness autograft from limited donor sites may be needed until the entire burn wound is covered. The use of a widely meshed graft is avoided on the face and functionally important parts of the hand. Full-thickness grafts that include both dermal and epidermal components are commonly obtained from the lower abdomen, groin, or upper arm. These grafts provide the best outcome for wound coverage with diminished contracture and a better pigment match, and should always be used on deep hand burns. 

Nonthermal Injuries CHEMICAL BURNS Cleaning products pose a risk for accidental exposure and chemical burns. The chemical agent responsible for the injury should be identified. Contacting poison control is often necessary. During the initial evaluation, all caustic material should be flushed from the skin with copious amounts of water. Chemicals are classified as either alkali or acid. Alkalis, such as lime, potassium hydroxide, sodium hydroxide, and bleach are the common agents involved in a chemical injury and often cause liquefactive necrosis and a deep burn. Acid burns are less common and cause coagulation necrosis. Formic acid injuries are rare but can result in multiple systemic organ failure with metabolic acidosis, renal failure, intravascular hemolysis, and acute respiratory distress syndrome. Hydrofluoric acid burns are

Fig. 13.9  This child suffered a full-thickness burn to the left lateral aspect of his mouth after biting into an electrical cord. These low-voltage injuries are most common in younger children.

managed with copious water irrigation and neutralization of the fluoride ion using topical 2.5% calcium gluconate gel. Without this management, free fluoride ions cause liquefaction necrosis of the affected soft tissues, including bones. Because of potential hypocalcemia, patients should be closely monitored for prolonged QT intervals or cardiac dysrhythmias. 

ELECTRICAL BURNS Of all admitted burn patients, 3–9% are injured from electrical contact.101 Electrical burns are categorized into low- and highvoltage injuries. Low-voltage (1000 V) injuries may result from power lines or lightning strikes, and are characterized by a varying degree of local burn with destruction of deep tissues.102 The electrical current enters the body and travels preferentially through the low-resistance tissues (nerves, blood vessels, and muscles). As skin has high resistance, it is mostly spared, leaving little visible evidence of injury. Primary and secondary surveys, including electrocardiography, are

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Table 13.4  Classification of SJS/TEN Disease classification

Percentage of TBSA with epidermal detachment

SJS SJS/TEN overlap TEN

30%

SJS, Stevens–Johnson syndrome; TBSA, total body surface area; TEN, toxic epidermal necrolysis.

very important. If the initial electrocardiogram is normal, further cardiac monitoring is not needed. However, any abnormal findings require continued monitoring for 48 hours and appropriate management of dysrhythmias if detected.103 The need for routine electrocardiography on patients who sustain low-voltage electrical burns is questionable and the need for routine electrocardiography is likely not warranted. Injuries to deep tissues and organs must be identified and treated. As tissue edema worsens, patients may develop compartment syndromes requiring fasciotomy to avoid limb loss. Myoglobinuria can lead to renal failure and should be treated with vigorous hydration with sodium bicarbonate and mannitol. Low-voltage injury is typically limited to superficial thermal burn and can be treated with topical wound care. 

Toxic Epidermal Necrolysis and Stevens–Johnson Syndrome Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are thought to be the same disease along a continuum of severity (Table 13.4). They generally occur as a severe reaction to a medication or, more rarely, to an infectious etiology. The incidence of severe exfoliating skin reactions is estimated at 1–7 cases per million-person years for SJS and 0.4–1.5 cases per million person-years for TEN.104 In approximately 95% of cases, TEN is triggered either by a preceding medication or an upper respiratory tract infection. A pooled analysis from the EuroSCAR study demonstrated that, in children, anti-infective sulfonamides, phenobarbital, lamotrigine, and carbamazepine demonstrated the strongest association with SJS/TEN.105 Although the exact etiology of SJS/TEN is not completely understood, it appears there is a specific drug hypersensitivity reaction that leads to a major histocompatibility class I-restricted presentation that is then followed by a proliferation of cytotoxic T-lymphocytes (CTL).104 In turn, an infiltration of skin lesions with CTLs and natural killer cells ensues. Granulysin, a cytolytic and proinflammatory molecule released by activated CTL and natural killer cells, is probably the key mediator for disseminated keratinocyte death. The clinical hallmark of SJS/TEN is mucosal involvement, with oral sites more commonly involved than the ocular, genital, or anal mucosa.106 Drug-induced TEN usually presents with fever and influenza-like symptoms 1–3 weeks after consumption of the suspected drug. In 90% of cases, this is followed 1–3 days later by mucosal lesions. Skin lesions will then form and will manifest as generalized macules with purpuric centers that then progress to large coalescing blisters and subsequent epidermal detachment. Large denuded areas ensue as the separation of the dermis progresses (Fig. 13.10).104

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Immediate discontinuation of the inciting drug and supportive care in a burn ICU has been shown to reduce mortality and improve prognosis.107 One of the largest studies showed a mortality in adult patients of 30% after transfer to a burn center within 7 days versus 50% after 7 days.108 Supportive critical care with a focus on organ function, electrolyte balance, and appropriate nutrition and wound care are the mainstays of treatment for children who develop SJS/TEN. Currently, there is no consensus on optimal wound care. Some advocate debridement while other centers advocate an antishear approach. Both methods have been shown to have equivalent rates of survival and reepithelialization.109 Studies assessing the effectiveness of adjuvant therapies including systemic corticosteroids, intravenous immunoglobulin (IVIG), or plasmapheresis have failed to show any survival benefit for these treatment modalities. The use of these adjuvant therapies should be assessed on a case-by-case basis. At our institution, we utilize an antishear approach to wound care. All of our TEN patients undergo initial care in our burn ICU. The patients undergo dressing changes on an as-needed basis with xeroform, bacitracin, and absorptive dressing. Once the xeroform adheres to the underlying tissue, it is left intact and will subsequently slough off once reepithelialization occurs. We have not seen any drug-resistant infections or drug toxicities from the frequent and large volume bacitracin use on any of our TEN patients although our experience is limited. 

Psychologic Sequelae of Burn Injuries Burn injuries, particularly large injuries, create a sudden, massive disruption in a child and in a family’s normal life. It not only threatens their immediate physical integrity, but in addition some of these children may require lifelong treatments that can range from long-term physical therapy to long-term reconstructive surgeries. These injuries can, and often do, lead to some form of psychopathology in both the burn victims and their families.110 In a recent literature review, stress disorders such as anxiety and traumatic stress in preschool and school-aged children were reported in up to 31% of children who suffered a burn injury.111 Although most of these children exhibited resilient or recovery patterns, 8% of preschool-aged children and up to 19% of school-aged children were diagnosed with chronic post-traumatic stress disorder (PTSD). In another study, the overall lifetime prevalence of anxiety disorders among patients who suffered burns >30% TBSA was 40%.110 With regard to affective disorders such as depression and mood disturbances, prevalence rates are 18% at an average of 14 years post burn and a 44% lifetime prevalence in survivors of large burns. Associated variables with affective disorders included female sex and the adolescent stage. Conversely, there was less depression among adolescents and young adults from a more positively functioning family and with more social support from friends.112 As expected, witnessing a child’s burn injury or a child’s pain during hospitalization can be a very distressing experience for parents or guardians of pediatric burn victims. It has been shown that up to 48% of mothers and 26% of fathers experience clinically relevant post-traumatic stress

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A

B

Fig. 13.10  This 10-year-old boy has a 95% TBSA epidermal detachment due to toxic epidermal necrolysis. (A) Generalized macules with purpuric centers in different stages of coalescing blisters are seen on this image. (B) Epidermal detachment is seen in the same patient.

symptoms (PTSS) within the first month post burn.113 Although these initial symptoms decline over time, up to 19% of mothers and 4% of fathers continue to experience clinically significant PTSS 18 months after the injury.114 At our institution, all patients and their caregivers who receive treatment through the inpatient and outpatient burn center complete psychosocial screening measures that assess quality of life, parental guilt, and child and parent acute stress symptoms. Patients who are 4 years old and older complete self-report questionnaires (with assistance from medical staff/caregivers as needed), while caregivers for children of all ages 0–17 years are asked to complete caregiver questionnaires. The questionnaires and screening tools are administered and completed on a tablet device through an online database. Our current screening tools include the child PTSD symptom scale, child PTSD symptom scale and trauma screen, child stress disorder checklist, child trauma screening questionnaire, children’s dermatology life quality index, short PTSD rating interview, and screening tool for early predictors of PTSD. Cumulative and individual scores that reach a predetermined threshold are emailed to our burn psychologist, and outpatient appointments are made on an as-needed basis. 

Multidisciplinary Care The management of severe burn injuries may represent the surgical field with the greatest integration of health professionals, and may benefit the most from the influence of truly multidisciplinary care.115 Caring for children with burns is a complex endeavor that should not be undertaken without the appropriate resources. Short-term and long-term management of all burn survivors involves a true multidisciplinary approach made up of a team of many different medical specialists readily available at one site. This team includes child life specialists, nutritionists, anesthesiologists, surgeons, pediatric intensivists, psychiatrists/psychologists, specialty trained nurses, occupational and physical therapists, social workers, and child abuse specialists. A number of pediatric subspecialists such as dermatologists, ophthalmologists, and plastic surgeons are also essential for

specific burns, and for the management of long-term reconstruction needs. Comprehensive care for burn victims also involves establishing a local and regional burn care infrastructure in order to provide burn education, outreach and prevention, and a built-in mechanism for evaluating the clinical outcomes.

References

1. Bull JP, Fisher AJ. A study of mortality in a burn unit: a revised estimate. Ann of Surg. 1954;139:269–279. 2. Periera CT, Barrow RE, Sterns AM, et al. Age-dependent differences in survival after severe burns: a unicentric review of 1,674 patients and 179 autopsies over 15 years. J Am Coll Surg. 2006;202:536–548. 3. Centers for Disease Control and Prevention, National Center for Injury Prevention and Control. Web-based Injury Statistics Query Reporting System (WISQARS) Nonfatal Injury Data; 2015. 4. Centers for Disease Control and Prevention, National Center for Injury Prevention and Control. Web-based Injury Statistics Query Reporting System (WISQARS) Fatal Injury Data; 2015. 5. Shah A, Suresh S, Thomas R, et al. Epidemiology and profile of pediatric burns in a large referral center. Clin Pediatr. 2011;50:391–395. 6. Jackson DM. The diagnosis of the depth of burning. Br J Surgery. 1953;40:588–596. 7. Bohr S, Patel SJ, Shen K, et al. Alternative erythropoetin-mediated signaling prevents secondary microvascular thrombosis and inflammation within cutaneous burns. Proc Natl Acad Sci. 2013;110:3513– 3518. 8. Vo LT, Papworth GD, Delaney PM, et al. A study of vascular response to thermal injury on hairless mice by fiberoptic confocal imaging, laser Doppler flowmetry and conventional histology. Burns. 1998;24:319–324. 9. Demling RH, LaLonde C. Early postburn lipid peroxidation: effect of ibuprofen and allopurinol. Surgery. 1990;107:85–93. 10. Nwariaku FE, Sikes PJ, Lightfoot E, et al. Effect of a bradykinin antagonist on the local inflammatory response following thermal injury. Burns. 1996;22:324–327. 11. DelBeccaro EJ, Robson MC, Heggers JP, et  al. The use of specific thromboxane inhibitors to preserve the dermal microcirculation after burning. Surgery. 1980;87:137–141. 12. Firat C, Samdancı E, Erbatur S, et  al. β-Glucan treatment prevents progressive burn ischaemia in the zone of stasis and improves burn healing: an experimental study in rats. Burns. 2013;39:105–112. 13. Shulman CI, King DR. Pediatric fluid resuscitation after thermal injury. J Craniofac Sur. 2008;19:910–912. 14. Evers LH, Bhavsar D, Mailänder P. The biology of burn injury. Exp Dermatol. 2010;19:777–783. 15. Chung DH, Evers BM, Townsend Jr CM, et  al. Role of polyamine biosynthesis during gut mucosal adaptation after burn injury. Am J Surg. 1993;165:144–149.

13 • Burns 16. Chung DH, Evers BM, Townsend Jr CM, et  al. Burn-induced transcriptional regulation of small intestinal ornithine decarboxylase. Am J Surg. 1992;163:157–163. 17. Wolf SE, Ikeda H, Matin S, et al. Cutaneous burn increases apoptosis in the gut epithelium of mice. J Am Coll Surg. 1999;188:10–16. 18. Carter EA, Gonnella A, Tompkins RG. Increased transcellular permeability of rat small intestine after thermal injury. Burns. 1992;18:117–120. 19. Myers SI, Minei JP, Casteneda A, et  al. Differential effects of acute thermal injury on rat splanchnic and renal blood flow and prostanoid release. Prostaglandins Leukot Essent Fatty Acids. 1995;53:439–444. 20. Jeschke MG, Barrow RE, Wolf SE, et al. Mortality in burned children with acute renal failure. Arch Surg. 1998;133:752–756. 21. Brusselaers N, Monstrey S, Colpaert K, et al. Outcome of acute kidney injury in severe burns: a systematic review and meta-analysis. Intensive Care Med. 2010;36:915–925. 22. Gamelli RL, He LK, Liu H. Macrophage suppression of granulocyte and macrophage growth following burn wound infection. J Trauma. 1994;37:888–892. 23. Hunt JP, Hunter CT, Brownstein MR, et al. The effector component of the cytotoxic T-lymphocyte response has a biphasic pattern after burn injury. J Surg Res. 1998;80:243–251. 24. Ravat F, Payre J, Peslages P, et al. Burn: an inflammatory process. Pathol Bio. 2011;59:e63–e72. 25. American Burn Association/American College of Surgeons. Guidelines for the operation of burn centers. J Burn Care Res. 2007;28: 134–141. http://ameriburn.org/wp-content/uploads/2017/05/ burncenterreferralcriteria.pdf. 26. Neaman KC, Andres LA, McClure AM, et al. A new method for estimation of involved BSAs for obese and normal-weight patients with burn injury. J Burn Care Res. 2011;32:421–428. 27. Armstrong JR, Willand L, Gonzalez B, et al. Quantitative analysis of estimated burn size accuracy for transfer patients. J Burn Care Res. 2017;38:e30–e35. 28. Goverman J, Bittner EA, Friedstat JS, et al. Discrepancy in initial pediatric burn estimates and its impact on fluid resuscitation. J Burn Care Res. 2015;36:574–579. 29. Sadideen H, D’Asta F, Moiemen N, et al. Does overestimation of burn size in children requiring fluid resuscitation cause any harm? J Burn Res. 2017;38:e546–e551. 30. Romanowski KS, Palmieri T. Pediatric burn resuscitation: past, present, and future. Burns Trauma. 2017;5:26–34. 31. Cope O, Moore FD. The redistribution of body water and the fluid therapy of the burned patient. Ann Surg. 1947;126:1010–1045. 32. Carvajal HG. Fluid resuscitation of pediatric burn victims: a critical appraisal. Pediatr Nephrol. 1994;8:357–366. 33. Navickis R, Greenhalgh DG, Wilkes MM. Albumin in burn shock resuscitation: a meta-analysis of controlled clinical studies. J Burn Care Res. 2016;37:e268–e278. 34. Ansermino JM, Vandebeek CA, Myers D. An allometric model to estimate fluid requirements in children following burn injury. Paediatr Anaesth. 2010;20:305–312. 35. Dries DJ, Waxman K. Adequate resuscitation of burn patients may not be measured by urine output and vital signs. Crit Care Med. 1991;19:327–329. 36. Kulkarni S, Harrington DT, Heffernan D, et al. Tolerance of oliguria improves burn resuscitation. J Burn Care Res. 2013;34:S113. 37. Du GB, Slater H, Goldfarb IW. Influences of different resuscitation regimens on acute early weight gain in extensively burned patients. Burns. 1991;17:147–150. 38. Lawrence A, Faraklas I, Watkins H, et al. Colloid administration normalizes resuscitation ratio and ameliorates ‘fluid creep’. J Burn Care Res. 2010;31:40–47. 39. Faraklas I, Cochran A, Saffle J. Review of a fluid resuscitation protocol: ‘fluid creep’ is not due to nursing error. J Burn Care Res. 2012;33:74–83. 40. Pruitt Jr BA, Mason Jr AD, Moncrief JA. Hemodynamic changes in the early postburn patient: the influence of fluid administration and of vasodilator (hydralazine). J Trauma. 1971;11:36–46. 41. Roberts I, Blackhall K, Alderson P, et al. Human albumin solution for resuscitation and volume expansion in critically ill patients. Cochrane Database Syst Rev. 2011;11:CD001208. 42. Wigginton JG, Roppolo L, Pepe PE. Advances in resuscitative trauma care. Minerva Anestesiol. 2011;77:993–1002.

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43. Jeschke MG, Herndon DN. Burns in children: standard and new treatments. Lancet. 2014;383:1168–1178. 44. Palmieri TL, Micak RP, Sheridan R, et al. Inhalation injury in children: a 10 year experience at Shriners hospitals for children. J Burn Care Res. 2009;30:206–208. 45. Soman S. Pediatric inhalation injury. Burns Trauma. 2017;5:31. 46. Sykes OT, Walker E. The neurotoxicology of carbon monoxide– historical perspective and review. Cortex. 2016;74:440–448. 47. MacLennan L, Moiemen N. Management of cyanide toxicity in patient with burns. Burns. 2015;41:18–24. 48. Traber DL, Herndon DN, Stein MD, et al. The pulmonary lesions of smoke inhalation in an ovine model. Circ Shock. 1986;18:311–323. 49. Demling RH. Smoke inhalation lung injury: an update. Eplasty. 2008;8:e27. 50. Baud FJ. Cyanide: critical issues in diagnosis and treatment. Hum Exp Toxicol. 2007;26:191–201. 51. Ching JA, Ching YH, Shivers SC, et  al. An analysis of inhalation injury diagnostic methods and patient outcomes. J Burn Care Res. 2016;37:e27–e32. 52. Buckley NA, Juurlink DN, Isbister G, et  al. Hyperbaric oxygen for carbon monoxide poisoning. Cochrane Database Syst Rev. 2011:CD002041. 53. Shepherd G, Velez LI. Role of hydrocobalamin in acute cyanide poisoning. Clin Toxicol. 2008;42:661–669. 54. Micak RP, Suman OE, Herndon DN. Respiratory management of inhalation injury. Burns. 2007;33:2–13. 55. Miller AC, Rivero A, Ziad S, et al. Influence of nebulized unfractionated heparin and N-acetylcysteine in acute lung injury after smoke inhalation injury. J Burn Care Res. 2009;30:249–256. 56. Cancio LC. Current concepts in the pathophysiology and treatment of inhalation injury. Trauma. 2005;7:19–35. 57. Saliba Jr MJ. Heparin in the treatment of burns: a review. Burns. 2001;27:349–358. 58. Desai MH, Micak R, Richardson J, et  al. Reduction in mortality in pediatric patients with inhalation injury with aerosolized heparin/Nacetylcystine [correction of acetylcysteine] therapy. J Burn Care Rehabil. 1998;19:210–212. 59. Brown M, Desai M, Traber LD, et al. Dimethylsulfoxide with heparin in the treatment of smoke inhalation injury. J Burn Care Rehabil. 1988;9:22–25. 60. Watts A, Tyler M, Perry M, et al. Burn depth and its historical measurement. Burns. 2001;27:154–160. 61. Engrav LH, Heimbach DM, Reus JL, et al. Early excision and grafting vs. nonoperative treatment of burns of indeterminate depth: a randomized prospective study. J Trauma. 1983;11:1001–1004. 62. Hlava P, Moserová J, Königová R. Validity of clinical assessment of the depth of a thermal injury. Acta Chir Plas. 1983;25:202–208. 63. Niazi ZB, Essex TJ, Papini R, et al. New laser Doppler scanner, a valuable adjunct in burn depth assessment. Burns. 1993;19:485–489. 64. Yeong EK, Mann R, Goldberg M, et  al. Improved accuracy of burn wound assessment using laser Doppler. J Trauma. 1996;40:956–961. 65. Ho-Asjoe M, Chronnell CM, Frame JD, et al. Immunohistochemical analysis of burn depth. J Burn Care Rehabil. 1999;20:207–211. 66. Moserová J, Hlava P, Malínský J. Scope for ultrasound diagnosis of the depth of thermal damage. Preliminary report. Acta Chir Plast. 1982;24:235–242. 67. Cantrell Jr JH. Can ultrasound assist an experienced surgeon in estimating burn depth? J Trauma. 1984;24:S64–S70. 68. Kaufman T, Hurwitz DJ, Heggers JP. The India ink injection technique to assess the depth of experimental burn wounds. Burns Incl Therm Inj. 1984;10:405–408. 69. Black KS, Hewitt CW, Miller DM, et al. Burn depth evaluation with fluorometry: is it really definitive? J Burn Care Rehabil. 1986;7:313– 317. 70. Pape SA, Skouras CA, Byrne PO. An audit of the use of laser Doppler imaging (LDI) in the assessment of burns of intermediate depth. Burns. 2001;27:233–239. 71. Hackett ME. The use of thermography in the assessment of depth of burn and blood supply of flaps, with preliminary reports on its use in Dupuytren’s contracture and treatment of varicose ulcers. Br J Plast Surg. 1974;27:311–317. 72. Erba P, Espinoza D, Koch N, et al. FluxEXPLORER: a new high-speed laser Doppler imaging system for the assessment of burn injuries. Skin Res Technol. 2012;18:456–461.

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73. Bombaro K, Engrav L, Carrougher G, et al. What is the prevalence of hypertrophic scarring following burns? Burns. 2003;29:299–302. 74. Desai MH, Rutan RL, Herndon DN. Conservative treatment of scald burns is superior to early excision. J Burn Care Rehabil. 1991;12:482– 484. 75. Kumar RJ, Kimble RM, Boots R, et al. Treatment of partial-thickness burns: a prospective, randomized trial using Transcyte. ANZ J Surg. 2004;74:622–626. 76. Costagliola M, Agrosì M. Second-degree burns: a comparative, multicenter, randomized trial of hyaluronic acid plus silver sulfadiazine vs. silver sulfadiazine alone. Curr Med Res Opin. 2005;21:1235– 1240. 77. Soroff HS, Sasvary DH. Collagenase ointment and polymyxin B sulfate/bacitracin spray versus silver sulfadiazine cream in partialthickness burns: a pilot study. J Burn Care Rehabil. 1994;15:13–17. 78. Fox Jr CL. Silver sulfadiazine–a new topical therapy for pseudomonas in burns. Therapy of pseudomonas infection in burns. Arch Surg. 1968;96:184–188. 79. Jarrett F, Ellerbe S, Demling R. Acute leukopenia during topical burn therapy with silver sulfadiazine. Am J Surg. 1978;135:818–819. 80. Choban PS, Marshall WJ. Leukopenia secondary to silver sulfadiazine: frequency, characteristics and clinical consequences. Am Surg. 1987;53:515–517. 81. Wasiak J, Cleland H, Campbell F, et  al. Dressings for superficial and partial thickness burns. Cochrane Database Syst Rev. 2013:CD002106. 82. Lindberg RB, Moncrief JA, Mason Jr AD. Control of experimental and clinical burn wounds sepsis by topical application of sulfamylon compounds. Ann N Y Acad Sci. 1968;150:950–960. 83. Asch MJ, White MG, Pruitt Jr BA. Acid base changes associated with topical Sulfamylon therapy: retrospective study of 100 burn patients. Ann Surg. 1970;172:946–950. 84. Fong J, Wood F, Fowler B. A silver coated dressing reduces the incidence of early burn wound cellulitis and associated costs of inpatient treatment: comparative patient care audits. Burns. 2005;31:562– 567. 85. Tredget EE, Shankowsky HA, Groeneveld A, et al. A matched-pair, randomized study evaluating the efficacy and safety of Acticoat silver-coated dressing for the treatment of burn wounds. J Burn Care Rehabil. 1998;19:531–537. 86. Varas RP, O’Keeffe T, Namias N, et  al. A prospective, randomized trial of Acticoat versus silver sulfadiazine in the treatment of partialthickness burns: which method is less painful? J Burn Care Rehabil. 2005;26:344–347. 87. Muller MJ, Hollyoak MA, Moaveni Z, et  al. Retardation of wound healing by silver sulfadiazine is reversed by Aloe vera and nystatin. Burns. 2003;29:834–836. 88. Winter GD. Formation of the scab and the rate of epithelization of superficial wounds in the skin of the young domestic pig. Nature. 1962;193:293–294. 89. Hinman CD, Maibach H. Effect of air exposure and occlusion on experimental human skin wounds. Nature. 1963;200:377–378. 90. Cassidy C, St Peter SD, Lacey S, et al. Biobrane versus duoderm for the treatment of intermediate thickness burns in children: a prospective, randomized trial. Burns. 2005;31:890–893. 91. Tompkins RG, Burke JF. Progress in burn treatment and the use of artificial skin. World J Surg. 1990;14:819–824. 92. Branski LK, Herndon DN, Pereira C, et al. Longitudinal assessment of Integra in primary burn management: a randomized pediatric clinical trial. Crit Care Med. 2007;35:2615–2623. 93. Herndon DN, Parks DH. Comparison of serial debridement and autografting and early massive excision with cadaver skin overlay in the treatment of large burns in children. J Trauma. 1986;26:149–152.

94. Herndon DN, Gore D, Cole M, et al. Determinants of mortality in pediatric patients with greater than 70% full-thickness total body surface area thermal injury treated by early total excision and grafting. J Trauma. 1987;27:208–212. 95. Muller MJ, Herndon DN. The challenge of burns. Lancet. 1994;343:216–220. 96. Bull JP, Fisher AJ. A study of mortality in a burns unit: a revised estimate. Ann Surg. 1954;139:269–274. 97. Janzekovic Z. A new concept in the early excision and immediate grafting of burns. J Trauma. 1970;10:1103–1108. 98. Klein MB, Hunter S, Heimbach DM, et  al. The Versajet water dissector: a new tool for tangential excision. J Burn Care Rehabil. 2005;26:483–487. 99. Gravante G, Delogu D, Esposito G, et al. Versajet hydrosurgery versus classic escharectomy for burn debridement: a prospective randomized trial. J Burn Care Res. 2007;28:720–724. 100. Alexander JW, MacMillan BG, Law E, et  al. Treatment of severe burns with widely meshed skin autograft and meshed skin allograft overlay. J Trauma. 1981;21:433–438. 101. Celik A, Ergun O, Ozok G. Pediatric electrical injuries: a review of 38 consecutive patients. J Pediatr Surg. 2004;39:1233–1237. 102. Laberge LC, Ballard PA, Daniel RK. Experimental electrical burns: low voltage. Ann Plast Surg. 1984;13:185–190. 103. Robson MC, Smith DJ. Care of the thermal injured victim. In: Jurkiewicz MJ, Krizek TJ, Mathes SJ, Ariyan S, eds. Plastic Surgery: Principles and Practice. St. Louis, CV: Mosby; 1990. 104. Gerull R, Nelle M, Schaible T. Toxic epidermal necrolysis and Stevens-Johnson syndrome: a review. Crit Care Med. 2011;39:1521– 1532. 105. Mockenhaupt M, Viboud C, Dunant A, et al. Stevens-Johnson syndrome and toxic epidermal necrolysis: Assessment of medication risk with emphasis on recently marketed drugs. The EuroSCAR-study. J Invest Dermatol. 2008;128:35–44. 106. Chung WH, Want CW, Dao RL. Severe cutaneous adverse drug reactions. J Dermatol. 2016;43:758–766. 107. Gerdts B, Vloemans AF, Kreis RW. Toxic epdidermal necrolysis: 15 years experience in a Dutch burns centre. J Eur Acad Dermatol Venereol. 2007;21:781–788. 108. Palmieri TL, Greenhalgh DG, Saffle JR, et al. A multicenter review of toxic epidermal necrolysis treated in US burn centers at the end of the twentieth century. J Burn Care Rehabil. 2002;23:87–96. 109. Dorafshar AH, Dickie SR, Cohn AB, et  al. Antishear therapy for toxic epidermal necrolysis: an alternative treatment approach. Plast Reconstr Surg. 2008;122:154. 110. Bakker A, Maertens KJ, Van Son MJ, et  al. Psychological consequences of pediatric burns from a child and family perspective: a review of the empirical literature. Clin Psychol Rev. 2013;33:361– 371. 111. De young AC, Kenardy JA, Cobham VE, et al. Prevalence, comorbidity and course of trauma reactions in young burn-injured children. J Child PsycholPsychiatry. 2012;53:56–63. 112. Meyer WJ, Blakeney P, Thomas CR, et  al. Prevalence of major psychiatric illness in young adults who were burned as children. Psychosom Med. 2007;69:377–382. 113. Egberts MR, van de Schoot R, Geenen R, et al. Parents’ posttraumatic stress after burns in their school-aged child: a prospective study. Health Psychol. 2017;36:419–428. 114. Bakker A, Van der Heijden PG, Van Son MJ, et  al. Course of traumatic stress reactions in couples after a burn event to their young child. Health Psychol. 2013;32:1076–1083. 115. Al-Mousawi AM, Mecott-Rivera GA, Jeschke MG, et al. Burn teams and burn centers: the importance of a comprehensive team approach to burn care. Clin Plast Surg. 2009;36:547–554.

14

Early Assessment and Management of Trauma ARTHUR COOPER

Trauma remains the leading cause of mortality and morbidity in the United States in children ages 1–14 years. In 2015, it continued to result in more death and disability than all other childhood diseases combined, as nearly 4000 pediatric patients died from trauma.1 Moreover, in 2010, the last year for which comprehensive national data are available, trauma accounted for 8% of all pediatric hospitalizations.2 Although recent data suggest that it represents a decreasing share of pediatric intensive care unit (PICU) admissions (7% in 2004 vs 13% in 1982 and 1995),3 trauma continues to constitute nearly 20% of pediatric emergency department (ED) visits4 and nearly 50% of pediatric ambulance transports.5 Death and disability from traumatic injuries are intimately related to mechanism of injury.6

Injury Epidemiology Several injury severity scales exist in practice and in the literature. The large number of injury severity scales arises from the markedly different perspectives used in the application of the scales. The Abbreviated Injury Scale (AIS), primarily an anatomical measure of injury severity, was the first widely implemented scale used in practice. Criticism of the AIS includes the inability to account for multiple injuries to the same body region and the poor correlation of the AIS with severity and survival. The Injury Severity Score (ISS), New Injury Severity Score (NISS), and Pediatric Trauma Score (PTS) are examples of scoring systems developed to overcome the issues described. Despite controversies regarding these scales, it is commonly accepted that injuries whose severity are a threat to life correspond to an ISS of 10 or higher or a PTS of 8 or lower.7 The death rate from traumatic injury in children in 2015 was 54.6/100,000.8 However, population-based data indicating that approximately 80% of lethally injured children will die before hospital admission demonstrate the need for effective injury prevention and prehospital care.9 Blunt injuries outnumber penetrating injuries in children by a ratio of 12:1, a ratio that has increased somewhat in recent years. While blunt injuries are more common, penetrating injuries are more lethal. However, despite the decline in penetrating injuries, firearm-related deaths continue as one of the top three causes of mortality in American youth. Most blunt trauma deaths in childhood are sustained unintentionally, but nearly 30% of fatal injuries are due to intentional physical assault (suicide 7.5% among children 5–14 years of age; homicide 22.5%, about half of the latter, due to physical abuse).8 Still, the leading cause of death in

children is the motor vehicle, responsible for approximately 75% of all childhood deaths, which are evenly split between those due to pedestrian trauma and those resulting from occupant injuries (Table 14.1). 

Injury Risks The lack of adequate supervision of children during play involving possible injury hazards is recognized as a major risk factor for unintentional injury in children. However, drug and alcohol use, obesity, poverty, and race also influence the frequency of injury. Toxicology screens are reportedly positive in 10–40% of injured adolescents, and obese children and adolescents appear to have more complications and require longer stays in the intensive care unit (ICU).10–13 Socioeconomic status also has been associated with increased hospitalization and mortality following major trauma, owing to a higher frequency and more lethal mechanisms of injury, as opposed to injury severity.14 Race and ethnicity affect injury risk independent of socioeconomic status, particularly among African-American children, whose rate of death from preventable injuries, head injuries, and child abuse is three to six times higher than that of white children.15–18 Improper use of restraints may contribute to the increased fatality rates observed in African-American children, who are half as likely to be restrained as white children when involved in motor vehicle crashes (MVCs) and one-third as likely to be placed in car seats during MVCs.19 Analysis of the Crash Injury Research Engineering Network (CIREN) database has yielded valuable information about the pattern of childhood injuries after MVCs: (1) child victims in frontal crashes are more likely to suffer severe spine and musculoskeletal injuries; (2) those in lateral crashes are more likely to suffer head and chest injuries; (3) those in front seats sustain more injuries to the chest, abdomen, pelvis, and axial skeleton than those in the rear seats; (4) seat belts are especially protective against pelvic and musculoskeletal injuries; (5) children involved in high-severity, lateral-impact crashes typically sustain injuries characterized by higher ISS and lower Glasgow Coma Scale (GCS) scores.20,21 Restraint devices also have been subjected to careful analysis: (1) they do not appear to protect young victims of MVCs as well as older victims; (2) car seats may not significantly affect injury outcome; (3) improper application may predispose to abdominal injuries, even in low-severity crashes; (4) the presence of abdominal wall bruising in restrained children, although not commonly observed, is frequently indicative of intra-abdominal injury.22–27  211

Incidence (%)

Blunt  Fall  Motor vehicle traffic  Struck by, against  Transport, other  Pedal cyclist, other  Pedestrian, other  Machinery Penetrating  Firearm  Cut/pierce Other

78.78 34.11 23.70 9.84 7.04 3.17 0.50 0.42 7.62 4.40 3.22 13.60

Mortality (%) 1.46 3.19 1.35 1.44 1.07 4.09 0.68 11.19 1.56

Data from the American College of Surgeons, National Trauma Data Bank. 2016 Pediatric Annual Report.

Injury Outcomes In recent years, much effort has been devoted to outcomes research in pediatric trauma with the hope that benchmarking of treatment results may lead to better care for injured children. Both historical studies and contemporary investigations indicate that children survive more frequently and recover more fully in hospitals that specialize in pediatric trauma than in other hospitals.28–44 No less important than survival outcome is functional outcome, for which numerous studies now indicate improved outcomes in hospitals that specialize in pediatric trauma care.46–48 However, these studies also suggest that whereas children may recover from injury more quickly than adults, physical function may not fully normalize. Even so, self-perceived long-term quality of life among seriously injured children may not be adversely affected, justifying an aggressive approach to their resuscitation.47 Perhaps the most important recent developments for outcomes research in pediatric trauma have been the expansion of the National Trauma Data Bank (NTDB) of the American College of Surgeons (ACS) to include children, the development of the Pediatric Trauma Quality Improvement Program (Pediatric TQIP) by the ACS, and the founding of the Pediatric Trauma Society (http://www.pediatri ctraumasociety.org). The NTDB was initially designed as a simple case repository; efforts continue to analyze cases submitted to the NTDB to provide population estimates of severe pediatric injury and develop quality benchmarks for pediatric trauma care. Preliminary data suggest that these benchmarks perform as well as existing measures.48 Similarly, Pediatric TQIP, which is available to Level I and II pediatric trauma centers verified by the ACS Committee on Trauma (COT), is now being used to develop quality benchmarks for pediatric trauma care. Finally, the Pediatric Trauma Society has provided a forum for investigators in the field of pediatric trauma to present their work to a wide audience of pediatric trauma professionals from all relevant health care disciplines. 

Injury Prevention Injuries are not accidents, but rather predictable events that respond to harm-reduction strategies similar to those

Host

Agent

Environment

Economics

By Injury Mechanism

Education

Table 14.1  Incidence and Mortality From the Major Categories of Pediatric Trauma

Engineering

Holcomb and Ashcraft’s Pediatric Surgery

Enforcement

212

Pre-event (primary prevention) Event (secondary prevention) Post-event (tertiary prevention) Fig. 14.1  The Haddon Factor Phase Matrix, as modified and refined to include a third strategic dimension, integrates all phases of injury control into a single system. (Adapted from Haddon W. Advances in the epidemiology of injuries as a basis for public policy. Public Health Rep 1980;95:411–421; Runyan CW. Using the Haddon Matrix: Introducing the third dimension. Inj Prev 1998;4:302–307.)

applied for other diseases. The Haddon Factor Phase Matrix neatly depicts these in graphic form (Fig. 14.1).49 Strategies to lessen the burden of injury are applied to the host, agent, and environment before, during, and after the traumatic event using enforcement, engineering, education, and economics as techniques to limit the adverse impact of each factor. Effective injury-prevention programs are communitybased and require extensive collaboration with civic leaders, governmental agencies, community-based organizations, and neighborhood coalitions. Programs such as the National Safe Kids Campaign (http://www.safekids.org) and the Injury Free Coalition for Kids (http://www.injuryfree.org) have proven highly successful in reducing the burden of childhood injury in many communities. 

Injury Patterns Injury mechanism is the main predictor of injury pattern. The body regions most frequently injured in major childhood trauma are the lower extremities, head and neck, and abdomen. In minor childhood injury, soft tissue and upper extremity injuries predominate. Motor vehicle versus pedestrian trauma may result in the Waddell triad of injuries to the head, torso, and lower extremity (pelvis, femur, or tibia; Fig. 14.2). Motor vehicle accidents may cause head, face, and neck injuries in unrestrained passengers. Cervical spine injuries, bowel disruption or hematoma, and Chance fractures occur in restrained passengers (Fig. 14.3). Bicycle trauma results in head injury in unhelmeted riders as well as upper extremity and upper abdominal injuries, the latter the result of contact with the handlebar (Fig. 14.4 and Table 14.2). Direct impact from a bicycle handlebar may be predictive of the need for operation.27

14 • Early Assessment and Management of Trauma

213

Fig. 14.2 The Waddell Triad of injuries to head, torso, and lower extremity is depicted. (From Foltin G, Tunik M, Cooper A, et al., editors. Teaching Resource for Instructors of Prehospital Pediatrics. NYU School of Medicine; 1998.)

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Fig. 14.4  Children riding bicycles can sustain blunt abdominal trauma after contact with handlebars or head trauma from falling off the bicycle. (From Foltin G, Tunik M, Cooper A, et al., editors. Teaching Resource for Instructors of Prehospital Pediatrics. NYU School of Medicine; 1998.)

9HUWHEUDO IUDFWXUH

Table 14.2  Common Injury Mechanisms and Corresponding Injury Patterns in Childhood Trauma /LYHUODFHUDWLRQ

Injury Mechanism Motor vehicle injury: Occupant

Injury Pattern Unrestrained Restrained

Motor vehicle injury: Pedestrian

Single Multiple

Fall from height

Fig. 14.3  The mechanism for the development of intestinal and vertebral injuries from lap belts. (From Foltin G, Tunik M, Cooper A, et al., editors. Teaching Resource for Instructors of Prehospital Pediatrics. NYU School of Medicine; 1998.)

HEAD Head injuries are potentially more dangerous in children than in adults for several reasons. First, developing neural tissue is delicate, and the softer bones of the pediatric skull allow impact forces to be transmitted directly to the underlying brain, especially at points of bony contact. Second, intracranial bleeding in infants in whom the fontanelles and sutures remain open may, on rare occasions, be severe enough to cause hypotensive shock. Third, the proportionately larger size of the head, when coupled with the injury mechanisms commonly observed in children, generally leads to head trauma with a loss of consciousness. As a consequence, the voluntary muscles of the neck lose their tone, which can lead to soft tissue obstruction in the upper airway and hypoxia. Hypoxia exacerbates and potentiates the initial traumatic injury to the brain

Low Medium High

Fall from bicycle

Unhelmeted Helmeted Handlebar

Head/neck injuries Scalp/facial lacerations Internal abdomen injuries Lower spine fractures Lower extremity fractures Head/neck injuries Internal chest/abdomen injuries Lower extremity fractures Upper extremity fractures Head/neck injuries Scalp/facial lacerations Upper extremity fractures Head/neck injuries Scalp/facial lacerations Internal chest/abdomen injuries Upper/lower extremity fractures Head/neck injuries Scalp/facial lacerations Upper extremity fractures Upper extremity fractures Internal abdomen injuries

From American College of Surgeons Committee on Trauma. Advanced Trauma Life Support® ATLS® Student Course Manual. 9th ed. Chicago: American College of Surgeons; 2012.

(secondary insults). See Chapter 17 for more information about head injuries. 

NECK Cervical spine injury is a relatively uncommon event in pediatric trauma. It affects approximately 1.5% of all seriously

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injured children and occurs at a rate of 1.8/100,000 population, which is in contrast to closed-head injury, which occurs at a rate of 185/100,000 population.50–52 The physician should also be aware of normal variants of cervical spine anatomy. The greater elasticity of the interspinous ligaments and the more horizontal apposition of the cervical vertebrae also give rise to a normal anatomic variant known as pseudosubluxation, which affects up to 40% of children younger than age 7 years. The most common finding is a short (2–3 mm) anterior displacement of C2 on C3, although anterior displacement of C3 on C4 can also occur. This pseudosubluxation is accentuated when the pediatric patient is placed in the supine position, which forces the cervical spine of the young child into mild flexion because of the forward displacement of the head by the more prominent occiput. The greater elasticity of the interspinous ligaments also is responsible for the increased distance between the dens and the anterior arch of C1 that is found in up to 20% of children. When an injury to the cervical spine does occur, it frequently occurs at C2, C1, and the occipitoatlantal junction. These injuries are above the nerve roots that give rise to diaphragmatic innervation (C4) and predispose the afflicted child to respiratory arrest as well as paralysis. The increased angular momentum produced by movement of the proportionately larger head, the greater elasticity of the interspinous ligaments, and the more horizontal apposition of the cervical vertebrae are responsible for this spectrum of injuries. Subluxation without dislocation may cause spinal cord injury without radiographic abnormalities (SCIWORA). SCIWORA accounts for up to 20% of pediatric spinal cord injuries as well as a number of prehospital deaths that were previously attributed to head trauma.53–55 

CHEST Serious intrathoracic injuries occurred in 6% of pediatric blunt trauma victims in one study.56 Lung injuries, pneumothorax and hemothorax, and rib and sternal fractures occur most frequently (Table 14.3). Injuries to the heart, diaphragm, great vessels, bronchi, and esophagus occur less frequently, but have higher mortality rates associated with them. Because blunt trauma is nearly 10 times more deadly when associated with major intrathoracic injury, thoracic injury serves as a marker of injury severity, although it is the proximate cause of death 20% liver herniation predicting more profound morbidity.75 In addition, fetal MRI is an excellent modality for morphologic and volumetric measurements of the fetal lung (total fetal lung volume [TFLV]). It is especially advantageous in patients with oligohydramnios and maternal obesity. Eight studies included in the recently published

meta-analysis showed a statistically significant difference between the mean O/E TFLV of survivors compared with nonsurvivors with CDH.71 Survival rates with O/E TFLV 35%, survival ranged from 75–89%. 

CLINICAL PRESENTATION Newborns with CDH typically present with respiratory distress. Clinical scenarios at birth range from immediate, profound respiratory distress with concomitant respiratory acidosis and hemodynamic instability, to an initial stable period with delayed respiratory distress, to an asymptomatic newborn. Initial signs associated with respiratory distress include tachypnea, chest wall retractions, grunting, cyanosis, and/or pallor. On physical examination, infants will often have a scaphoid abdomen and may have a subtle increase in thoracic diameter. The point of maximal cardiac impulse is often displaced, a physical finding with mediastinal shift. Bowel sounds may be auscultated within the thoracic cavity with a decrease in breath sounds bilaterally. Chest excursion may be reduced, suggesting a lower tidal volume. The diagnosis of CDH is typically confirmed by a chest radiograph demonstrating intestinal loops within the hemithorax, cephalad displacement of the stomach/orogastric tube, and a mediastinal shift toward the contralateral hemithorax (Fig. 24.6). The abdominal cavity may have minimal to no gas, particularly initially. Right-sided CDH can be challenging to diagnose (Fig. 24.7). Salient features, such as intestinal and gastric herniation, may not be prominent, and the herniated right lobe of the liver can be mistaken for a right diaphragmatic elevation or eventration. Occasionally, features of lung compression may be the only radiographic sign, which can cause confusion with CPAMs, pulmonary sequestrations, bronchopulmonary cysts, neurogenic cysts, or cystic teratomas. Although most infants with CDH will be diagnosed within the first 24 hours of life, as many as 20% may present outside the neonatal period.76 These patients present with mild respiratory symptoms, chronic pulmonary infections, pleural effusions, pneumonias, feeding intolerance, or gastrointestinal pathology. As CDH is invariably associated with abnormal intestinal rotation and fixation, some children can present with intestinal obstruction or volvulus.

Fig. 24.5  Fetal MR image of a left-sided CDH at 28 weeks’ gestation. A large CDH with herniation of the small bowel and stomach is found within the left hemithorax (solid arrow). There is dextroposition of the fetal heart (dotted arrow). There is no evidence of liver herniation.

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%

Fig. 24.6  (A) Anteroposterior chest radiograph in a neonate with a CDH demonstrating air-filled loops of bowel within the left chest. The heart and mediastinum are shifted to the right, and the hypoplastic left lung can be seen medially. (B) Postoperative radiograph demonstrating hyperexpansion of the right lung with shift of the mediastinum to the left. The edge of the severely hypoplastic left lung is again easily visualized (arrow).

24 • Congenital Diaphragmatic Hernia and Eventration

Occasionally, CDH may be completely asymptomatic and is discovered only incidentally. Patients who present later in life have an excellent prognosis due to milder or absent associated complications, such as pulmonary hypoplasia and CDH-PH. 

Treatment PRENATAL CARE The prenatal diagnosis of CDH continues to improve with the increased use and refinement of fetal US examination and advanced fetal MRI.77 After initial screening, an advanced US helps to determine discordant size and dates, associated anomalies (cardiovascular, neurologic, other), as well as signs of fetal compromise (i.e., hydrops fetalis). Further, an accurate LHR can estimate the probable severity, allowing informed counseling and consideration for appropriate prenatal monitoring and/or intervention. Once diagnosed, chromosomal screening via amniocentesis for karyotyping and chromosome microarray analysis is recommended.78 Optimally, the mother and fetus should be referred to a tertiary perinatal center with protocolized fetal MRI and advanced maternal fetal medicine (a fetal center), neonatal, surgical, and critical care capabilities, including HFOV, ECMO, and pulmonary hypertension therapeutic expertise.78,79 A prenatal diagnosis enables informed counseling for the mother and family including treatment options and prognosis. 

PRENATAL MEDICATIONS In animal models, the hypoplastic lungs of CDH infants are structurally and functionally immature.49 Biochemical markers for lung maturity demonstrate decreased total

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lung DNA, total lung protein, and desaturated phosphatidylcholine in addition to a deficiency of surfactant.49 Some animal models demonstrate lung maturation and improved function as a result of prenatal administration of glucocorticoids.80,81 Initial results from small patient series seemed promising in that antenatal administration of glucocorticoids suggested improved lung function.82 However, other studies failed to demonstrate any benefit for CDH-associated pulmonary hypoplasia.46 As such, prenatal steroids are not currently recommended for CDH. Other agents targeting pulmonary morphology that rely on transplacental transport for delivery include vitamin A, glucagon-like peptide-1 agonists, phosphodiesterase (PDE) inhibitors, and tyrosine kinase inhibitors.80 Vitamin A compounds are critical in normal diaphragmatic and pulmonary development, and infants with CDH have been noted to have lower vitamin A levels.83 As such, vitamin A has been applied to the rat nitrofen model and rabbit model with mixed evidence for enhanced lung maturity and decreased pulmonary vessel thickness.84,85 However, to date, no human studies have examined the benefit of prenatal vitamin A, and there is evidence that excessive vitamin A can be teratogenic in human pregnancy.80 Animal studies have also examined the potential benefits of prenatal glucagon-like peptide-1 agonists and tyrosine kinase inhibitors with varied results.86,87 In addition, there are animal models that show promise in improving pulmonary vasculature remodeling prenatally with the administration of the PDE inhibitor sildenafil.88,89 Despite promising frontiers in research with prenatal medical and pharmacologic interventions for CDH, translation of these therapies via clinical trials remains elusive. At this point, there are no indications for any prenatal pharmacotherapy in CDH. 

RESUSCITATION AND STABILIZATION

Fig. 24.7 This infant presented with respiratory distress and a ­right-sided CDH.

After confirming the diagnosis, initial postnatal therapy is targeted at resuscitation and stabilization of the infant in cardiopulmonary distress. A rapid overall assessment is important to determine hemodynamic stability and the severity of disease. In most cases, prompt endotracheal intubation (without bag mask ventilation) and initiation of conventional mechanical ventilatory support is required. A nasogastric tube should be inserted to avoid gastric and intestinal distention. Arterial and venous access is necessary for resuscitative maneuvers. Acid–base balance and oxygenation–ventilation status should be carefully monitored. Invasive monitoring is important in accurately assessing the infant’s overall perfusion and the severity of pulmonary hypertension and hypoplasia. Umbilical venous catheters may be helpful and, if possible, may be positioned in the right atrium to measure central venous pressures. In addition, an approximation of cerebral oxygenation and perfusion should be available using preductal oxygen content and/or saturation via either a right radial arterial catheter or a transcutaneous saturation probe. Targets for initial resuscitation include preductal arterial saturation (SaO2) between 80% and 90% with strictly limited positive airway pressures. In order to maintain lower peak inspiratory pressures (PIPs), a moderate level

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of hypercarbia (PaCO2, up to 70 mmHg) is accepted as long as it does not result in a profound compensatory acidosis. Occasionally, higher levels of PaCO2 are tolerated transiently as long as a pH > 7.2 is maintained. Failure to provide adequate tissue oxygenation can result in metabolic acidosis, which may exacerbate the pulmonary hypertension. PVR is increased by hypoxia and acidosis, which should be avoided or corrected. In the event of a pulmonary hypertensive crisis, including rapidly progressive hypoxia, hypercarbia, and/or severe ductal shunting, some centers use inhaled nitric oxide (iNO). However, there is mounting evidence that iNO does not reduce the need for ECMO nor improve survival in CDH.90,91 Alternative strategies including minimal environmental stimulation, fluid optimization, HFOV, milrinone, sildenafil and/or ECMO may be effective or necessary to stabilize clinical deterioration secondary to a pulmonary hypertensive crisis.92 Depending on the degree of pulmonary hypertension and associated cardiac anomalies (both assessed by echocardiography within the first 6–12 hours of life), hemodynamic stability can be difficult to achieve. Pulmonary hypertension may be exhibited by a difference in pre- and postductal SaO2. However, echocardiography can better characterize the degree of pulmonary hypertension.56 Sonographic findings of pulmonary hypertension include poor contractility of the right ventricle, flattening or bowing of the interventricular septum, enlarged right heart chambers, and tricuspid valve regurgitation (which can be used to estimate the right-ventricular systolic pressures). There may be right-toleft or bidirectional shunting across the ductus arteriosus or ASD/VSD. Almost all infants with CDH and severe pulmonary hypertension exhibit some left ventricular dysfunction and emerging evidence continues to suggest cardiac dysfunction plays a prominent role in the outcomes of patients with CDH.93 Vasopressor agents such as dopamine, dobutamine, epinephrine, and milrinone may be needed in hemodynamically unstable patients. These inotropic agents can augment left ventricular output and increase systemic pressures in order to ameliorate right-to-left ductal shunting. 

MECHANICAL VENTILATION Optimal mechanical ventilation is a critical component in the care of infants with respiratory failure secondary to CDH. However, the physiologic limits of the hypoplastic lung and the pathophysiologic, hyporesponsive pulmonary vasculature make mechanical ventilatory management a challenge. Hypoplastic lungs in CDH infants are characterized by a decreased number of airways and smaller alveolar airspaces. Also, the pulmonary vasculature exhibits decreased vascular branching as well as increased adventitia and medial wall thickness.94 This combination results in varying degrees of respiratory failure and CDH-PH. Fortunately, pulmonary and vascular development continues after birth and these pulmonary sequelae of CDH can improve.95 Because of this ongoing maturation, mechanical ventilation strategies have trended toward less aggressive approaches with the goal of maintaining oxygenation while limiting the risks of ventilator-induced lung injury (VILI) and alveolar instability, major contributors to pulmonary morbidity and mortality.92,96–98

Conventional ventilation is the optimal initial mode of mechanical ventilation among infants with CDH.92,99 The optimal specific type of conventional mechanical ventilation remains individual clinician preference, though most cases of CDH can be managed using a pressure-controlled mode. A fractional inspired oxygen (FiO2) of 1.0 is initially utilized to maintain adequate SaO2 (>80–85%). Typically, higher respiratory rates and lower peak airway pressures (18–22 cmH2O) are employed while titrating the FiO2 to a preductal SaO2 > 80–85% and a PaCO2 less than 70 mmHg (pH > 7.2). Maintaining an acceptable pH and PaCO2 are important in managing pulmonary hypertension.96 The ventilation strategy of induced respiratory alkalosis with hyperventilation to reduce ductal shunting has been abandoned.92,100 Initial conventional mechanical ventilation settings should include pressure-limited ventilation rates between 40 and 60 breaths per minute with PIP  10,000) (2) Left shift (neutrophils >75%) (1) Sum of 7 suggests appendicitis

Pain right fossa (1) Tenderness: Light (1), medium (2), strong (3) Fever (38.5) (1) Polymorphonuclear neutrophilia: 70–84% (1), >85% (2) Leukocytosis: 10.0–14.9K (1), >15K (2) CRP: 1–4.9 mg/L (1), >5 (2) Sum of 9 suggests appendicitis

CRP, C-reactive protein; WBCs, white blood cells.

+ Dist 0.628 cm

A

+ Dist 1.14 cm

B

Fig. 42.2  These ultrasound studies from two different patients depict evidence of appendicitis. On the axial view (A), an appendicolith measuring 6.3 mm in diameter is depicted. The appendix is also larger than 6 mm in diameter, which meets criteria for appendicitis. On the right (B), in this longitudinal ultrasound view, the enlarged appendix measures 11 mm in diameter.

The PAS and the Alvarado Score have been investigated the most thoroughly, and both had initially shown sensitivity, specificity, negative predictive value (NPV), and positive predictive value (PPV) over 90%.38,39 However, large validation studies have reported the sensitivity and specificity in the 70–90% range.42–44 The scoring systems are even less reliable in adolescent females, which demonstrates the necessity for investigation beyond the scoring system in these patients.45 Overall, scoring systems are not reliable enough to be used as a single diagnostic tool and are best used to separate patients into the categories of discharge, further imaging, or surgical referral. Likewise, in trying to discern perforated from nonperforated disease preoperatively, the scoring systems require the use of imaging.46 

Imaging Studies Diagnostic imaging is often necessary to balance minimizing the risk of a negative appendectomy with the risk of a missed diagnosis. Data from children’s hospitals demonstrate extremely low negative appendectomy rates when diagnostic imaging is used.27 Plain films may demonstrate a fecalith in 5–15% of patients,47 which has been associated with appendicitis in patients with abdominal pain.48 However, these studies almost never serve as the determinant for a management

decision and are not recommended unless bowel obstruction, mass, or free peritoneal air is suspected.49 Ultrasound (US) is a rapid bedside modality that requires no intravenous (IV) access and no contrast, and emits no radiation. Graded compression US is performed by placing pressure on the transducer to displace bowel loops in order to identify the appendix. The pressure is adequate if the psoas muscle and iliac vessels are identified, which ensures the range of view is posterior to the appendix. The common US signs of appendicitis include a fluid-filled, noncompressible appendix, a diameter >6 mm, an appendicolith, periappendiceal or pericecal fluid, and increased periappendiceal echogenicity caused by inflammation (Fig. 42.2).50,51 However, US is operator dependent, may not be available during off hours, and has difficulty in visualizing the appendix early in the disease process or in obese patients.52–54 In a meta-analysis of 26 manuscripts and 7448 patients, the sensitivity and specificity of US were 88% and 94%, respectively.54 When the results of multiple pooled studies were examined from 6 manuscripts in tabular format with nearly 18,000 patients, a wide range of sensitivity (44–88%) and specificity (90–97%) was found, which suggests that pooled results may not be generalizable to individual hospitals.37 An increased sensitivity and specificity utilizing US can be obtained by changing parameters of thickness of the appendix (>7 vs 6 mm), using dedicated pediatric ultrasonographers, increasingly utilizing US, and an increased duration of abdominal pain (>48 vs 150 μm in diameter) is associated with a better prognosis. The subgroup of infants with syndromic BA have worse outcomes in terms of both clearance of jaundice and survival.26,35 The latter is related to associated malformations (particularly congenital heart disease), a predisposition to developing hepatopulmonary syndrome, and possible immune compromise from functional hyposplenism. Personal experience suggests that infants with concomitant CMV infection fare less well after a portoenterostomy. The importance of surgeon experience was shown in a British survey in which patients who underwent portoenterostomy at centers treating 1 case per year had significantly worse outcomes than patients who were treated at centers performing more than 5 cases per year.158 Since 1999, the management of BA has been centralized to three centers in England and Wales that are able to offer both portoenterostomy and transplantation. In 2011 the results of this policy change were reported, and it was found that outcomes were better,159 probably due to centralization of surgical and medical resources. Recently, outcomes in infants enrolled in the prospective Childhood Liver Disease Research and Education Network who underwent portoenterostomy were reported.160 Liver anatomy, splenic malformation, presence of ascites, liver nodularity at portoenterostomy, and early postoperative clearance of jaundice were found to be significant predictors of transplant-free survival. Irrespective of age and other factors related to the timing of portoenterostomy, a significant decrease in serum bilirubin and signs of good bile excretion in the stool may be predictive of good long-term outcome.161 Due to the possibility of sudden hepatic deterioration and the constant concern for cholangitis and portal hypertension, recent reports about long-term outcomes in BA patients consistently emphasize lifelong follow-up.131,162 Growth failure and poor mean weight z-scores 3 months after hepatoportoenterostomy are associated with poor outcome.163 A BA registry inaugurated in Japan in 1989 now has input from some 3160 patients. Of these, 1236 patients have undergone liver transplantation and continue to be followed with standardized questionnaires. Twenty-year survival rates are 89% overall and 49% with native livers.164 The prognosis of BA has markedly improved owing to surgical intervention involving Kasai portoenterostomy and liver transplantation.

According to a recent report from the Japanese Biliary Atresia Registry, the age at Kasai portoenterostomy had a significant impact on long-term native liver survival.164 The best jaundice clearance rates and best longterm native liver survival rates were achieved in patients who underwent portoenterostomy as neonates. Long-term native liver survival rates were well correlated with age at portoenterostomy. Certain substances can act as prognostic factors in BA. Serum levels of IL-6, IL-1ra, insulin-like growth factor-1 (IGF-1), vascular cell adhesion molecule-1 (VCAM-1), and ICAM-1 correlate with liver dysfunction in postoperative BA patients.53,165,166 Immunohistochemically, a reduction in the expression of CD68 and ICAM-1 at the time of portoenterostomy is associated with a better prognosis.167 The presence of ductal plate malformation in the liver predicts poor bile flow after hepatoportoenterostomy.168 

Liver Transplantation The indications for liver transplantation following portoenterostomy are: (1) lack of bile drainage; (2) signs of developmental retardation or its sequelae; and (3) presence of socially unacceptable complications/side effects. A high hepatic artery resistance index measured on Doppler US is an indication for relatively urgent transplantation.169 Deterioration in hepatic status may be precipitated by adolescence or pregnancy. However, in our experience, less than 10% of patients undergoing portoenterostomy will remain jaundice free and reach adulthood with good liver function. The dramatic improvement in survival with the use of cyclosporine and tacrolimus immunosuppression after liver transplantation raises the question of transplantation becoming a more conventional form of treatment for BA. Donor supply is a problem, alleviated to some extent by reduced-size liver transplantation (split-liver grafting) and living-related liver transplantation.170 Five-year survival after liver transplantation for BA is currently 80–90%, and long-term studies of post-transplant BA patients have shown that survivors have an acceptable to good quality of life.171,172 A study summarized the largest series (n = 464) of postportoenterostomy patients who had undergone livingrelated liver transplantation.173 The outcome of livingrelated liver transplantation in adults with BA was significantly worse than in infants and children. The overall 5- and 10-year survival rates were 70% and 56% in adults versus 87% and 81% in infants and children, respectively. In contrast, there is another report that concluded that living-related liver transplantation can be performed safely after portoenterostomy in adults with long-term survival rates similar to those for pediatric patients.174 Longer immunosuppression might ultimately lead to increased morbidity, including higher rates of cancer, infection, and metabolic diseases later in life. In addition, in living-related liver transplantation, the risk to the donor is always a concern.175 The optimal timing of transplantation in postportoenterostomy patients has yet to be established. Recently, Kasahara et  al. published a summary of living-donor liver transplantation for patients with BA

43 • Biliary Atresia

in Japan.176 They reported that the 1-, 5-, 10-, 15-, and 20-year survival rates for patients and grafts undergoing living-donor liver transplantation for BA were 91.6, 91.5, 87.1, 85.4, and 84.2% and 90.5, 90.4, 84.6, 82.0, and 79.9%, respectively. According to data from the Japanese Liver Transplantation Society, there were significant differences in survival rates between patients and grafts. Multivariate analysis showed that donor body mass index, ABO incompatibility, graft type, recipient age, center experience, and transplant era were prognostic for a better overall graft survival. Primary transplantation for BA has been reported.177 Incidence varies from 0.1% (Japan), 3% (Netherlands and the United Kingdom), 4% (France), 10% (Canada and Switzerland), and 11% (Germany). Primary liver transplantation for BA has excellent results but is performed rarely. A treatment dilemma exists for the approximately one-third of infants with BA who derive no benefit from a Kasai portoenterostomy. If these patients could be identified with specific multidisciplinary protocols, they could be prepared directly for primary liver transplantation without having more traditional surgical intervention.

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49. Seidman SL, Duquesnoy RJ, Zeevi A, et al. Recognition of major histocompatibility complex antigens on cultured human biliary epithelial cells by alloreactive lymphocytes. Hepatology. 1991;13:239–246. 50. Dillon P, Belchis D, Tracy T, et al. Increased expression of intercellular adhesion molecules in biliary atresia. Am J Pathol. 1994;145:263– 267. 51. Silveira TR, Salzano FM, Donaldson PT, et al. Association between HLA and extrahepatic biliary atresia. J Pediatr Gastroenterol Nutr. 1993;16:114–117. 52. Kobayashi H, Puri P, O’Brian DS, et  al. Hepatic overexpression of MHC class II antigens and macrophage-associated antigen (CD68) in patients with biliary atresia of poor prognosis. J Pediatr Surg. 1997;32:590–593. 53. Kobayashi H, Horikoshi K, Li L, et al. Serum concentration of adhesion molecules in postoperative biliary atresia patients: relationship to disease activity and cirrhosis. J Pediatr Surg. 2001;36:1297– 1301. 54. Allison JP. CD28-B7 interactions in T-cell activation. Curr Opin Immunol. 1994;6:414–419. 55. Kobayashi H, Li Z, Yamataka A, et  al. Role of immunologic costimulatory factors in the pathogenesis of biliary atresia. J Pediatr Surg. 2003;38:892–896. 56. Bezerra JA, Tiao G, Ryckman FC, et  al. Genetic induction of proinflammatory immunity in children with biliary atresia. Lancet. 2002;23:1653–1659. 57. Mack CL, Tucker RM, Sokol RJ, et  al. Biliary atresia is associated with CD4+ Th1 cell-mediated portal tract inflammation. Pediatr Res. 2004;56:79–87. 58. Li K, Zhang X, Yang L, et al. Foxp3 promoter methylation impairs suppressive function of regulatory T cells in biliary atresia. Am J Physiol Gastrointest Liver Physiol. 2016;311: G989-G97. 59. Klemann C, Schroder A, Dreier A, et al. Interleukin 17, produced by gammadelta T cells, contributes to hepatic inflammation in a mouse model of biliary atresia and is increased in livers of patients. Gastroenterology. 2016;150:229–241.e5. 60. Lages CS, Simmons J, Maddox A, et  al. The dendritic cell-T helper 17-macrophage axis controls cholangiocyte injury and disease progression in murine and human biliary atresia. Hepatology. 2017;65: 174–188. 61. Ito T, Horisawa M, Ando H. Intrahepatic bile ducts in biliary atresia: a possible factor determining the prognosis. J Pediatr Surg. 1983;18:124–130. 62. Raweily EA, Gibson AAM, Burt AD. Abnormalities of intrahepatic bile ducts in extrahepatic biliary atresia. Histopathology. 1990;17:521–527. 63. Lilly JR, Altman RP. Hepatic portoenterostomy (the Kasai operation) for biliary atresia. Surgery. 1975;78:76–86. 64. Desmet VJ. Intrahepatic bile ducts under the lens. J Hepatol. 1987;1:545–559. 65. Sherlock S. The syndrome of disappearing intrahepatic bile ducts. Lancet. 1987;2:493–496. 66. Ohi R, Chiba T, Endo N. Morphologic studies of the liver and bile ducts in biliary atresia. Acta Paediatr Jpn. 1987;29:584–589. 67. Chiba T, Ohi R, Kamiyama T, et al. Japanese Biliary Atresia Registry: Biliary Atresia. Tokyo: Icom Assoc; 1991. 68. Altman RP, Levy J. Biliary atresia. Pediatr Ann. 1985;14:481–485. 69. Okazaki T, Kobayashi H, Yamataka A, et al. Long-term post surgical outcome of biliary atresia. J Pediatr Surg. 1998;34:312–315. 70. Balistreri WF. Neonatal cholestasis. J Pediatr. 1985;106:171–184. 71. Brough H, Houssin D. Conjugated hyperbilirubinemia in early infancy: a reassessment of liver biopsy. Hum Pathol. 1974;5:507–516. 72. Javitt NB, Keating JP, Grand RJ, et  al. Serum bile acid patterns in neonatal hepatitis and extrahepatic biliary atresia. J Pediatr. 1977;90:736–739. 73. Ukarapol N, Wongsawasdi L, Ong-Chai S, et  al. Hyaluronic acid: Additional biochemical marker in the diagnosis of biliary atresia. Pediatr Int. 2007;49:608–611. 74. Faweya AG, Akinyinka OO, Sodeinde O. Duodenal intubation and aspiration test: utility in the differential diagnosis of infantile cholestasis. J Pediatr Gastroenterol Nutr. 1991;13:290–292. 75. Azuma T, Nakamura T, Moriuchi T, et  al. Preoperative Ultrasonographic Diagnosis of Biliary Atresia with Reference to the Presence or Absence of the Extrahepatic Bile Duct. Tokyo: Japan: Paper presented at the 38th Annual Congress of the Japanese Society of Pediatric Surgeons; 2001.

76. Park WH, Choi SO, Lee HJ. The ultrasonographic ‘triangular cord’ coupled with gallbladder images in the diagnostic prediction of biliary atresia from infantile intrahepatic cholestasis. J Pediatr Surg. 1999;34:1706–1710. 77. Kotb MA, Kotb A, Sheba MF, et al. Evaluation of the triangular cord sign in the diagnosis of biliary atresia. Pediatrics. 2001;108:416– 420. 78. Tan Kendrick AP, Ooi BC, Tan CE. Biliary atresia: making the diagnosis by the gallbladder ghost triad. Pediatr Radiol. 2003;33:311– 315. 79. Farrant P, Meire HB, Mieli-Vergani G. Ultrasound features of the gallbladder in infants presenting with conjugated hyperbilirubinaemia. Br J Radiol. 2000;73:1154–1158. 80. Abramson SJ, Berdon WE, Altman RP, et al. Biliary atresia and noncardiac polysplenia syndrome: ultrasound and surgical consideration. Radiology. 1987;163:377–379. 81. Han BK, Babcock DS, Gelfand MM. Choledochal cyst with bile duct dilatation: sonographic and 99mTc-IDA cholescintigraphy. AJR Am J Roentgenol. 1981;136:1075–1079. 82. Zhou L, Shan Q, Tian W, et  al. Ultrasound for the diagnosis of biliary atresia: a meta-analysis. AJR Am J Roentgenol. 2016;206: W73–W82. 83. Ochshorn Y, Rosner G, Barel D, et al. Clinical evaluation of isolated nonvisualized fetal gallbladder. Prenat Diagn. 2007;27:699–703. 84. Boughanim M, Benachi A, Dreux S, et  al. Nonvisualization of the fetal gallbladder by second-trimester ultrasound scan: strategy of clinical management based on four examples. Prenat Diagn. 2008;28:46–48. 85. Okazaki T, Miyano G, Yamataka A, et  al. Diagnostic laparoscopyassisted cholangiography in infants with prolonged jaundice. Pediatr Surg Int. 2006;22:140–143. 86. Nwomeh BC, Caniano DA, Hogan M. Definitive exclusion of biliary atresia in infants with cholestatic jaundice: the role of percutaneous cholecysto-cholangiography. Pediatr Surg Int. 2007;23:845–849. 87. Matsui A. Screening for biliary atresia. Pediatr Surg Int. 2017; 33(12):1305–1313. 88. Harpavat S, Garcia-Prats JA, Shneider BL. Newborn bilirubin screening for biliary atresia. N Engl J Med. 2016;375:605–606. 89. Lin JS, Chen SC, Lu CL, et al. Reduction of the ages at diagnosis and operation of biliary atresia in Taiwan: a 15-year population-based cohort study. World J Gastroenterol. 2015;21:13080–13086. 90. Serinet MO, Wildhaber BE, Broue P, et  al. Impact of age at Kasai operation on its results in late childhood and adolescence: a rational basis for biliary atresia screening. Pediatrics. 2009;123:1280–1286. 91. Yamataka A, Lane GJ, Cazares J. Laparoscopic surgery for biliary atresia and choledochal cyst. Semin Pediatr Surg. 2012;21:201–210. 92. Yamataka A, Kobayashi H, Shimotakahara A, et al. Recommendations for preventing complications related to Roux-en-Y hepaticojejunostomy performed during excision of choledochal cyst in children. J Pediatr Surg. 2003;38:1830–1832. 93. Nio M, Ohi R. Biliary atresia. Semin Pediatr Surg. 2000;9:177–186. 94. Davenport M. Surgery for biliary atresia. In: Spitz L, Coran AG, eds. Operative Pediatric Surgery. New York: Hodder Arnold; 2006:661– 672. 95. Miyano T, Fujimoto T, Ohya T, et al. Current concept of the treatment of biliary atresia. World J Surg. 1993;17:332–336. 96. Kobayashi H, Yamataka A, Urao M, et  al. Innovative modification of the hepatic portoenterostomy. Our experience of treating biliary atresia. J Pediatr Surg. 2006;41:19–22. 97. Miyano T, Ohya T, Kimura K, et  al. Current state of the treatment of congenital biliary atresia (in Japanese). J Jpn Surg Soc. 1989;90:1343–1347. 98. Kobayashi H, Horikoshi K, Yamataka A, et al. Alpha-glutathione-Stransferase as a new sensitive marker of hepatocellular damage in biliary atresia. Pediatr Surg Int. 2000;16:302–305. 99. Kobayashi H, Horikoshi K, Yamataka A, et  al. Hyaluronic acid: a specific prognostic indicator of hepatic damage in biliary atresia. J Pediatr Surg. 1999;34:1791–1794. 100. Miyano T, Suruga K, Tsuchiya H, et al. A histopathological study of the remnant of extrahepatic bile duct in so-called uncorrectable biliary atresia. J Pediatr Surg. 1977;12:19–25. 101. Nakamura H, Koga H, Wada M, et al. Reappraising the portoenterostomy procedure according to sound physiological/anatomic principles enhances postoperative jaundice clearance in biliary atresia. Pediatr Surg Int. 2012;28:205–209.

43 • Biliary Atresia 102. Nio M, Wada M, Sasaki H, Kazama T, et al. Technical standardization of Kasai portoenterostomy for biliary atresia. J Pediatr Surg. 2016;51:2105–2108. 103. Kimura K, Tsugawa C, Matsumoto T, et al. The surgical management of the unusual forms of biliary atresia. J Pediatr Surg. 1979;14:653– 660. 104. Nio M, Sano N, Ishii T, et al. Long-term outcome in type I biliary atresia. J Pediatr Surg. 2006;41:1973–1975. 105. Takahashi Y, Matsuura T, Saeki I, et al. Excellent long-term outcome of hepaticojejunostomy for biliary atresia with a hilar cyst. J Pediatr Surg. 2009;44:231–2315. 106. Esteves E, Clemente Neto E, et al. Laparoscopic Kasai portoenterostomy for biliary atresia. Pediatr Surg Int. 2002;18:737–740. 107. Wong KK, Chung PH, Chan KL, et al. Should open Kasai portoenterostomy be performed for biliary atresia in the era of laparoscopy? Pediatr Surg Int. 2008;24:931–933. 108. Kuebler JF, Kos M, Jesch NK, et al. Carbon dioxide suppresses macrophage superoxide anion production independent of extracellular pH and mitochondrial activity. J Pediatr Surg. 2007;42:244–248. 109. Mogilner JG, Bitterman H, Hayari L, et al. Effect of elevated intraabdominal pressure and hyperoxia on portal vein blood flow, hepatocyte proliferation and apoptosis in a rat model. Eur J Pediatr Surg. 2008;18:380–386. 110. Ure BM, Kueblaer JF, Schukfeh N, et  al. Survival with the native liver after laparoscopic versus conventional Kasai portoenterostomy in infants with biliary atresia. A prospective trial. Ann Surg. 2011;253:826–830. 111. Von Sochaczewski OC, Petersen C, Ure BM, et al. Laparoscopic versus conventional Kasai portoenterostomy does not facilitate subsequent liver transplantation in infants with biliary atresia. J Laparoendosc Adv Surg Tech. 2012;22:408–411. 112. Hussain MH, Alizai N, Patel B. Outcomes of laparoscopic Kasai portoenterostomy for biliary atresia: a systematic review. J Pediatr Surg. 2017;52:264–267. 113. Chan KWE, Lee KH, Mou JWC, et  al. The outcome of laparoscopic portoenterostomy for biliary atresia in children. Pediatr Surg Int. 2011;27:671–674. 114. Koga H, Miyano G, Takahashi T, et  al. Laparoscopic portoenterostomy for uncorrectable biliary atresia using Kasai’s original technique. J Laparoendosc Adv Surg Tech A. 2011;21:291–294. 115. Yamataka A, Lane GJ, Cazares J. Laparoscopic surgery for biliary atresia and choledochal cyst. Semi Pediatr Surg. 2012;21:201–210. 116. Davenport M, Yamataka A. Surgery for biliary atresia. In: Spitz L, Goran AG, eds. Operative Pediatric Surgery. 7th ed. Florida: CRC Press; 2013:655–666. 117. Cazares J, Koga H, Murakami H, et  al. Laparoscopic portoenterostomy for biliary atresia: single-center experience and review of literatures. Pediatr Surg Int. 2017;33:1341–1354. 118. Freitas L, Gauthier F, Valayer J. Second operation for repair of biliary atresia. J Pediatr Surg. 1987;22:857–860. 119. Ibrahim M, Ohi R, Chiba T, et al. Indication and Results of Reoperation for Biliary Atresia. Tokyo: Icom Association; 1991. 120. Bondoc AJ, Taylor JA, Alonso MH, et  al. The beneficial impact of revision of Kasai portoenterostomy for biliary atresia. Ann Surg. 2012;255:570–576. 121. Nakamura H, Kawano T, Yoshizawa K, et al. Long-term follow-up for anicteric survival with native liver after redo Kasai: a first report. J Pediatr Surg. 2016;51:2109–2112. 122. Sumida W, Uchida H, Tanaka Y, et al. Review of redo-Kasai portoenterostomy for biliary atresia in the transition to the liver transplantation era. Nagoya J Med Sci. 2017;79:415–420. 123. Muraji T, Higashimoto Y. The improved outlook for biliary atresia with corticosteroid therapy. J Pediatr Surg. 1997;32:1103–1107. 124. Karrer FM, Lilly JR. Corticosteroid therapy in biliary atresia. J Pediatr Surg. 1985;20:693–695. 125. Bezerra JA, Spino C, Magee JC, et al. Use of corticosteroids after hepatoportoenterostomy for bile drainage in infants with biliary atresia: the START randomized clinical trial. JAMA. 2014;311:1750– 1759. 126. Tyraskis A, Davenport M. Steroids after the Kasai procedure for biliary atresia: the effect of age at Kasai portoenterostomy. Pediatr Surg Int. 2016;32:193–200. 127. Davenport M, Stringer MD, Tizzard SA, et al. Randomized, doubleblind, placebo-controlled trial of corticosteroids after Kasai portoenterostomy for biliary atresia. Hepatology. 2007;46:1821–1827.

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128. Davenport M, Parsons C, Tizzard S, Hadzic N. Steroids in biliary atresia: single surgeon, single centre, prospective study. J Hepatol. 2013;59:1054–1058. 129. Japanese Biliary Atresia S, Nio M, Muraji T. Multicenter randomized trial of postoperative corticosteroid therapy for biliary atresia. Pediatr Surg Int. 2013;29:1091–1095. 130. Nakamura H, Koga H, Wada M, et al. Reappraising the portoenterostomy procedure according to sound physiological/anatomic principles enhances postoperative jaundice clearance in biliary atresia. Pediatr Surg Int. 2012;28:205–209. 131. Shinkai M, Ohhama Y, Take H, et al. Long-term outcome of children with biliary atresia who were not transplanted after the Kasai operation: >20-year experience at a children’s hospital. J Pediatr Gastroenterol Nutr. 2009;48:443–450. 132. Ochi T, Nakamura H, Wada M, et al. Liver transplantation for deterioration in native liver function after portoenterostomy for biliary atresia in Japan: short- versus long-term survivors. J Pediatr Surg. 2018;53(2):277–280. 133. Suruga K, Miyano T, Kimura K, et al. Reoperation in the treatment of biliary atresia. J Pediatr Surg. 1982;17:1–6. 134. Sawaguchi S, Akiyama Y, Saeki M, et al. The Treatment of Congenital Biliary Atresia with Special Reference to Hepatic Portoenteroanastomosis. Tokyo: Paper presented at the fifth annual meeting of the Pacific Association of Pediatric Surgeons; 1972. 135. Nakajo T, Hashizume K, Saeki M, et al. Intussusception-type antireflux valve in Roux-en-Y loop to prevent ascending cholangitis after hepatic portojejunostomy. J Pediatr Surg. 1990;25:311–314. 136. Tanaka K, Shirahase I, Utsunomiya H, et  al. A valved hepatic portoduodenal intestinal conduit for biliary atresia. Ann Surg. 1990;213:230–235. 137. Endo M, Katsumata K, Yokoyama J, et al. Extended dissection of the porta hepatis and creation of an intussuscepted ileocolic conduit for biliary atresia. J Pediatr Surg. 1983;12:784–793. 138. Bu LN, Chen HL, Chang CJ, et al. Prophylactic oral antibiotics in prevention of recurrent cholangitis after the Kasai portoenterostomy. J Pediatr Surg. 2003;38:590–593. 139. Lykavieris P, Chardot C, Sokhn M, et  al. Outcome in adulthood of biliary atresia: a study of 63 patients who survived for over 20 years with their native liver. Hepatology. 2005;41:366–371. 140. Altman RP, Chandra R, Lilly JR. Ongoing cirrhosis after successful portoenterostomy with biliary atresia. J Pediatr Surg. 1975;10:685– 691. 141. Tan EL, Davenport M, Driver M, et al. Does the morphology of the extrahepatic biliary remnants in biliary atresia influence survival? A review of 205 cases. J Pediatr Surg. 1994;29:1459–1464. 142. Karrer FM, Wallace BJ, Estrada AE. Late complications of biliary atresia: Hepatopulmonary syndrome and portopulmonary hypertension. Pediatr Surg Int. 2017;33:1335–1340. 143. Bu LN, Chen HL, Ni YH, et al. Multiple intrahepatic biliary cysts in children with biliary atresia. J Pediatr Surg. 2002;37:1183–1187. 144. Hol L, van den Bos IC, Hussain SM, et al. Hepatocellular carcinoma complicating biliary atresia after Kasai portoenterostomy. Eur J Gastroenterol Hepatol. 2008;20:227–231. 145. Andrews WS, Pau CM, Chase HP, et  al. Fat soluble vitamin deficiency in biliary atresia. J Pediatr Surg. 1981;16:284–290. 146. Greene HL, Helinek GL, Moran R, et al. A diagnostic approach to prolonged obstructive jaundice by 24-hour collection of duodenal fluid. J Pediatr Surg. 1979;95:412–414. 147. Barkin RM, Lilly JR. Biliary atresia and the Kasai operation: continuing care. J Pediatr Surg. 1980;96:1015–1019. 148. Raffensperger JG. A long-term follow-up of three patients with biliary atresia. J Pediatr Surg. 1991;26:176–177. 149. Agarwal GS, Saxena A, Bhatnagar V. The development of intrapulmonary arteriovenous shunts as a poor prognostic factor following surgery for biliary atresia. Trop Gastroenterol. 2009;30: 110–112. 150. Kuroda T, Saeki M, Morikawa N, et al. Biliary atresia and pregnancy: puberty may be an important point for predicting the outcome. J Pediatr Surg. 2005;40:1852–1855. 151. Kasai M, Mochizuki I, Ohkohchi N, et  al. Surgical limitation for biliary atresia: indication for liver transplantation. J Pediatr Surg. 1989;24:851–854. 152. Davenport M, Kerkar N, Mieli-Vergani G, et  al. Biliary atresia: the King’s College Hospital experience (1974–1995). J Pediatr Surg. 1997;32:479–485.

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153. Chardot C, Carton M, Spire-Bendelac N, et al. Is the Kasai operation still indicated in children older than 3 months diagnosed with biliary atresia? J Pediatr Surg. 2001;138:224–228. 154. Nio M, Sasaki H, Wada M, et al. Impact of age at Kasai operation on short- and long-term outcomes of type III biliary atresia at a single institution. J Pediatr Surg. 2010;45:2361–2363. 155. Wong KKY, Chung PHY, Chan IHY, et al. Performing Kasai portoenterostomy beyond 60 days of life is not necessarily associated with a worse outcome. J Pediatr Gastroenterol Nutr. 2010;51:631–634. 156. Kuroda T, Saeki M, Morikawa N, et al. Management of adult biliary atresia patients: should hard work and pregnancy be discouraged? J Pediatr Surg. 2007;42:2106–2109. 157. Chandra RS, Altman RP. Ductal remnants in extrahepatic biliary atresia: a histopathologic study with clinical correlation. J Pediatr Surg. 1978;93:196–200. 158. McClement JW, Howard ER, Mowat AP. Results of surgical treatment for extrahepatic biliary atresia in the United Kingdom, 1980– 1982. BMJ. 1985;290:345–347. 159. Davenport M, Ong E, Sharif K, et al. Biliary atresia in England and Wales: Results of centralization and new benchmark. J Pediatr Surg. 2011;46:1689–1694. 160. Superina RS, Magee JC, Brandt ML, et  al. The anatomic pattern of biliary atresia identified at time of Kasai hepatoportoenterostomy and early postoperative clearance of jaundice are significant predictors of transplant-free survival. Ann Surg. 2011;254:577–585. 161. Ohhama Y, Shinkai M, Fujita S, et al. Early prediction of long-term survival and the timing of liver transplantation after the Kasai operation. J Pediatr Surg. 2000;35:1031–1034. 162. Kumagi T, Drenth JPH, Guttman O, et al. Biliary atresia and survival into adulthood without transplantation: a collaborative multicenter clinic review. Liver Int. https://doi.org/10.1111/j.1478-32312011.02668.x. 163. DeRusso PA, Ye W, Shepherd R, et al. Growth failure and outcomes in infants with biliary atresia: a report from the Biliary Atresia Research Consortium. Hepatology. 2007;46:1632–1638. 164. Nio M. Japanese biliary atresia registry. Pediatr Surg Int. 2017;33: 1319–1325. 165. Phavichitr N, Theamboonlers A, Poovorawan Y. Insulin-like growth factor-1 (IGF-1) in children with postoperative biliary atresia: a cross-sectional study. Asian Pac J Allergy Immunol. 2008;26:57–61.

166. Kobayashi H, Yamataka A, Lane GJ, et al. Levels of circulating antiinflammatory cytokine interleukin-1 receptor antagonist and proinflammatory cytokines at different stages of biliary atresia. J Pediatr Surg. 2002;37:1038–1041. 167. Davenport M, Gonde C, Redkar R, et  al. Immunohistochemistry of the liver and biliary tree in extrahepatic biliary atresia. J Pediatr Surg. 2001;36:1017–1025. 168. Shimadera S, Iwai N, Deguchi E, et  al. Significance of ductal plate malformation in the postoperative clinical course of biliary atresia. J Pediatr Surg. 2008;43:304–307. 169. Broide E, Farrant P, Reid F, et al. Hepatic artery resistance index can predict early death in children with biliary atresia. Liver Transpl Surg. 1997;3:604–610. 170. Tanaka K, Uemoto S, Tokunaga Y, et  al. Surgical techniques and innovations in living related liver transplantation. Ann Surg. 1993;217:82–91. 171. Howard ER, MacClean G, Nio M, et al. Biliary atresia: survival patterns after portoenterostomy and comparison of a Japanese with a UK cohort of long-term survivors. J Pediatr Surg. 2001;36:892– 897. 172. Bucuvalas JC, Britto M, Krug S, et  al. Health-related quality of life in pediatric liver transplant recipients: a single-center study. Liver Transpl. 2003;9:62–71. 173. Uchida Y, Kasahara M, Egawa H, et al. Long-term outcome of adultto-adult living donor liver transplantation for post-Kasai biliary atresia. Am J Transp. 2006;6:2443–2448. 174. Kyoden Y, Tamura S, Sugawara Y, et  al. Outcome of living donor liver transplantation for post-Kasai biliary atresia in adults. Liver Transpl. 2008;14:186–192. 175. Trotter JF, Adam R, Lo CM, et  al. Documented deaths of hepatic lobe donors for living donor liver transplantation. Liver Transpl. 2006;12:1485–1488. 176. Kasahara M, Umeshita K, Sakamoto S, et al. Liver transplantation for biliary atresia: a systematic review. Pediatr Surg Int. 2017;33:1289– 1295. 177. Superina R. Biliary atresia and liver transplantation: results and thoughts for primary liver transplantation in select patients. Pediatr Surg Int. 2017;33:1297–1304.

44

Choledochal Cyst and Gallbladder Disease NGUYEN THANH LIEM, LEO ANDREW BENEDICT, and GEORGE W. HOLCOMB III

Choledochal Cyst A choledochal cyst is a congenital dilatation of the biliary tract. The dilatation can be found along any portion of the biliary tract. However, the most common site is the choledochus. The diameter of the bile duct varies according to the child’s age.1–3 The normal diameter of the common bile duct (CBD) is seen in Table 44.1.3 Any diameter of the bile duct greater than the upper limit for age should be considered abnormal.

CLASSIFICATION Different classifications have been proposed for choledochal cyst (CC).4–8 However, the Todani classification has been the most widely accepted (Fig. 44.1).5 According to this classification, CCs are classified into five types: Type I □  Ia: Cystic dilatation of the CBD □  Ib: Fusiform dilatation of the CBD □  Type II: Diverticulum of the CBD □  Type III: Choledochocele (dilatation of the terminal CBD within the duodenal wall) □  Type IV □  IVa: Multiple cysts of the extrahepatic and intrahepatic ducts □  IVb: Multiple extrahepatic duct cysts □  Type V: Intrahepatic duct cyst (single or multiple, as in Caroli disease). □ 

  

Forme fruste is a special variant of CC characterized by pancreaticobiliary malunion, but little or no dilatation of the extrahepatic bile duct.8–12 Children present with symptoms similar to those in patients with a CC. Excision of the extrahepatic bile duct is recommended in these children because of the likely eventual development of cancer due to chronic pancreaticobiliary reflux. Type I CCs predominate. Together with type IVa cysts, they account for more than 90% of cases. Caroli disease is characterized by segmental saccular dilatation of the intrahepatic bile ducts. It may affect the liver diffusely or be localized to one lobe.13–15 

ETIOLOGY There are many theories to explain the development of a CC. However, none of these can explain the formation of the five different types of CC. CCs seem to be either congenital

or acquired. Congenital cysts develop during fetal life.16 These appear to develop as a result of a prenatal structural defect in the bile duct. Shimotake et al. found that the total number of ganglion cells within the wall of these CCs is significantly lower than in control specimens.17 Also, smooth muscle fibers are more abundant in the cystic type than in the fusiform type.18 CCs, which develop later in life, are considered “acquired.”16 The theory of the long common biliopancreatic channel proposed by Babbitt has been widely accepted to explain the formation of this type.19 Normally, the terminal CBD and pancreatic duct unite to form a short common channel, which is well surrounded by the Oddi sphincter. This normal anatomic arrangement prevents the reflux of pancreatic fluid into the bile duct. If this common channel is long and part of it is not surrounded by the normal sphincter, pancreatic secretions can reflux into the biliary tree (Fig. 44.2). Proteolytic enzymes from the pancreatic fluid are activated and can cause epithelial and mural damage that leads to mural weakness and dilatation of the choledochus. This theory is supported by the fact that high concentrations of activated pancreatic amylase and/or lipase have been found in patients with CC and long pancreaticobiliary channels.16,20–22 In an experimental study, CC was produced by creating a choledochopancreatic endto-side ductal anastomosis.23 In addition, a high incidence of a common channel has been detected in CC patients.24 Obstruction at the level of the pancreaticobiliary junction may be an associated causal factor in choledochal dilatation. An experimental model for the study of cystic dilatation of the extrahepatic biliary system has been produced by ligation of the distal end of the CBD in the newborn lamb.25 High biliary pressure in patients with CC have also been recorded during operative correction.26 In adults, an anomalous union of the pancreaticobiliary duct is defined when the common pancreaticobiliary channel is longer than 15 mm2,27 or when its extraduodenal portion is more than 6 mm.28 In a study of 264 infants and children undergoing endoscopic retrograde cholangiopancreatography (ERCP), the maximal length of the common channel was found to be 2.7 mm among children 3 years or younger, 4 mm among children 4–9 years old, and 5 mm between 10 and 15 years old.29 A genetic basis for CC is suspected because its female predominance and different prevalence among countries in Asia and Europe versus United States. Using “trio-based” exome-sequencing of 31 patients with CC, Wong et  al. recently reported 13 genes that were recurrently mutated at different sites.30  695

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Table 44.1  Mean Common Bile Duct Diameter and Range According to Patient Age Age (Years)

Range (mm)

Mean (mm)

≤4 4–6 6–8 8–10 10–12 12–4

2–4 2–4 2–6 2–6 3–6 3–7

2.6 3.2 3.8 3.9 4.0 4.9

Choledochus

Adapted from Witcombe JB, Cremin BJ. The width of the common bile duct in childhood. Pediatr Radiol 1978;7:147–149.

Pancreatic duct Common biliopancreatic channel

Type Ia

Type Ib

Fig. 44.2  This contrast study depicts a long common biliopancreatic channel that allows reflux of pancreatic secretions into the biliary tree. A long common biliopancreatic channel is thought to be the etiology of an acquired choledochal cyst.

may be found in the CC, along with a dilated intrahepatic bile duct and a common biliopancreatic channel. Liver histology varies from normal to cirrhosis, depending on the patient’s age and degree of cholangitis.  Type II

Type III

CLINICAL FEATURES

Type IVa

Type IVb

Type V Fig. 44.1  These diagrams depict the five classifications for choledochal cyst according to Todani. (From Todani T, Watanabe Y, Narusue M, et al. Congenital bile duct cysts: classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. Am J Surg 1977;134:263–269.)

PATHOLOGY An inflammatory reaction within the CC is noted in most cases. It is minimal in infants and gradually becomes more significant as the patient gets older. The degree of mucosal ulcerations and pericystic inflammation becomes more severe after repeated bouts of cholangitis. Stones or debris

Females are affected more often than males. In our series of 400 cases, the female-to-male ratio was 3.2:1.31 Clinical presentations differ according to the age of onset and the type of cyst. An abdominal mass or jaundice is a common finding in an infant with CC, whereas abdominal pain is more often seen in older children.16,31–33 The cystic form usually presents with an abdominal mass, whereas the fusiform type is usually found in patients presenting with abdominal pain. Choledochal cysts diagnosed antenatally are more likely cystic in nature.16 Clinical manifestations among our 400 cases included abdominal pain (88%), vomiting (46%), fever (28%), icterus (25%), discolored stool (12%), abdominal tumor (7%), and the classic triad (2%).31 Complications such as perforation and hemobilia are rare.34,35 However, pancreatitis is common.16,32 Malignant change is a late complication, mostly seen in adults.36–39 

IMAGING Ultrasonography (US) is the initial imaging method of choice (Fig. 44.3A). Contour and position of the CC, the status of the proximal ducts, vascular anatomy, and hepatic echotexture can be evaluated on US. ERCP allows excellent definition of the cyst as well as the entire anatomy, including the pancreatobiliary junction. However, this investigation is invasive and has complications such as pancreatitis, perforation of the duodenal or biliary tracts, hemorrhage, and sepsis.40,41

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Fig. 44.3  (A) Ultrasound is the initial imaging method of choice for identifying a choledochal cyst. The cyst is identified as well as the portal vein (pv) lying posterior to it. (B) MRCP is highly accurate in the detection and classification of the cyst. On this MRCP image, note the fusiform choledochal cyst as well as the pancreatic duct (dotted arrow) and long common channel (solid arrow). The gallbladder is marked with an asterisk.

Magnetic resonance cholangiopancreatography (MRCP) is highly accurate in the detection and classification of the cyst (see Fig. 44.3B). The overall detection rate of MRCP for a CC is very high (96–100%) and should be considered a first-choice imaging technique for evaluation.42–44 Intraoperative cholangiography is indicated when the anatomic detail of the biliary tract cannot be demonstrated by MRCP or ERCP (Fig. 44.4). Contrast-enhanced computed tomography (CT) may be indicated in some patients with pancreatitis or if an associated tumor is suspected. 

SURGICAL TECHNIQUES General Principles Cystoduodenostomy and cystojejunostomy have been abandoned due to cholangitis, stone formation, and malignant degeneration.6,45–47 External drainage is indicated for a perforated cyst in patients whose condition is too unstable to perform cystectomy and a bilio-enteric anastomosis.34,35 Cyst excision and a bilio-enteric anastomosis is the preferred approach for most patients. The cyst should be excised at the level of the common biliopancreatic channel orifice at its distal end and approximately 5 mm from the confluence of the right and left hepatic ducts at the proximal end. Postoperative malignancy in a residual cyst on either the hepatic duct side or from the distal part has been reported.48 A review from the English language and Japanese literature of 23 patients with carcinomas of the bile duct developing after CC excision found that malignancy developed in the intrapancreatic remnant of the bile duct or CC in 6 patients, in the remnant of the CC at the hepatic side in 3 patients, in the hepatic duct at or near the anastomosis in 8 patients, and in the intrahepatic duct in 6 patients.48 Abdominal pain and pancreatitis due to leaving a remnant of the cyst in the pancreatic head also have been described.49 Operative correction can be performed safely in all age groups.30,50,51  Preoperative Preparation Biliary infection should be treated before operation. A prolonged prothrombin time secondary to cholestasis should

Fig. 44.4  In this patient, neither an MRCP nor ERCP was helpful preoperatively. Thus, an intraoperative cholangiogram was performed to identify the anatomic detail within the biliary tract. The distended gallbladder is marked with an asterisk. The enlarged choledochal cyst is seen, and the pancreatic duct is identified with a solid arrow.

be corrected with intravenous vitamin K. Drugs for elimination of ascaris are given in areas where ascaris is prevalent. 

Bilio-Enteric Anastomosis After Cystectomy Many surgeons use hepaticojejunostomy,52–57 whereas others prefer hepaticoduodenostomy.58–62 Fat malabsorption and duodenal ulcer are the main concerns with a hepaticojejunostomy.63 In addition, the operative time is longer in comparison with hepaticoduodenostomy. Complications after Roux-en-Y hepaticojejunostomy, such as a twist of the Roux limb, intestinal obstruction, and duodenal ulcers, have been reported.64–67 On the other hand, cholangitis and gastritis due to bilious reflux are the main concerns with hepaticoduodenostomy.68 However, the operative time is shorter in comparison with hepaticojejunostomy. A hepaticoduodenostomy is considered more physiologic

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Fig. 44.5 Older patients are placed in the lithotomy position, and smaller patients are moved to the end of the bed. It is helpful for the surgeon to stand either between the patient’s legs (in older patients) or at the end of the operating table (in younger patients) for laparoscopic choledochal cyst repair.

because the bile drains directly into the duodenum. This anastomosis is performed above the transverse colon mesentery, which may help prevent intestinal obstruction from adhesions. 

Laparoscopic Approach Endotracheal intubation and general anesthesia are standard. Epidural analgesia can provide excellent postoperative pain relief. Broad-spectrum intravenous antibiotics are best given at induction of anesthesia and continued for 5 days postoperatively. A nasogastric tube, rectal tube, and urinary catheter are used to decompress the stomach, colon, and bladder. The patient is placed in a 30° lithotomy position (Fig. 44.5). The surgeon stands or sits at the lower end of the operating table between the patient’s legs. The first laparoscopic operation for CC was reported in 1995.69 This approach quickly became popular and has become the routine approach in many centers.70–83 The laparoscopic approach is preferred for most types of CC: I, II, and IV. Relative contraindications are in patients with perforation, previous biliohepatic surgery, or especially newborns with damaged hepatic functions. A 10-mm cannula is inserted through the umbilicus for the telescope. Three additional 5- or 3-mm ports are introduced for the working instruments: one in the right flank, another in the left flank, and one in the left hypochondrium (Fig. 44.6). Carbon dioxide pneumoperitoneum is maintained at a pressure of 8–12 mmHg. The liver is secured to the abdominal wall with a suture placed around the round ligament (Fig. 44.7A). The cystic artery and the cystic duct are identified, clipped, and divided. A second traction suture is placed at the junction of distal cystic duct and gallbladder fundus to elevate the liver and expose the hepatic hilum (see Fig. 44.7B). The appearance of the cyst, liver, and spleen is noted. Intraoperative cholangiography via the gallbladder can be performed if the anatomy has not been clearly defined preoperatively. With a large cyst, bile can be aspirated through a catheter, which reduces the cyst size to facilitate the pericystic dissection.

Fig. 44.6  This operative photograph depicts placement of the ports for laparoscopic repair of a choledochal cyst. A 10-mm cannula (1) is introduced through the umbilicus for the telescope. Three additional 5- or 3-mm ports are then used for the working instruments (2, 3, 4). Note that the liver has been elevated anteriorly with a suture placed around the round ligament and exteriorized in the epigastric region (arrow).

The duodenum is retracted downward using a dissector inserted through the left lower port. The mid-portion of the cyst is dissected circumferentially. Separation of the cyst from the hepatic artery and portal vein is meticulously performed until a dissector can be passed through the space between the posterior cyst wall and the portal vein going from left to right. The cyst is then divided at this site. The inferior part of the cyst is separated from the pancreatic tissue down to the common biliopancreatic duct using a 3-mm dissector for cautery and dissection. Protein plugs or calculi within the cyst and common channel are washed out and removed. The inferior part of the cyst is opened longitudinally and inspected to identify the orifice of the common biliopancreatic channel. A small catheter is then inserted into the common channel. Irrigation with normal saline via this catheter is performed to eliminate protein plugs until clear fluid returns and the catheter can be passed down into the duodenum (Fig. 44.8A). A cystoscope can be used to measure the length of the common channel and remove protein plugs in it if its diameter permits.84,85 The distal CC is then clipped and divided at the level of the orifice of the common channel (see Fig. 44.8B). The cephalad portion of the cyst is further dissected to the common hepatic duct. The cyst is initially divided at the level of the cystic duct. After identifying the orifice of the right and left hepatic ducts, the rest of the cyst is removed, leaving a stump approximately 5 mm from the bifurcation of the hepatic ducts. Irrigation with normal saline through a small catheter inserted into the right and then into the left hepatic duct is performed to wash out protein plugs or calculi until the effluent is clear. If the cyst is intensely inflamed with extensive pericystic adhesions, the cyst is opened by a transverse incision on its anterior wall. The dissection of the cyst wall from the portal vein is then carefully performed while viewing the cyst from inside and outside. After dividing the mid-portion of the cyst, the upper and lower parts of the cyst are removed as previously described. 

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Fig. 44.7  (A) Suture has been placed through the round ligament and will be exteriorized in the epigastrium in order to help elevate the liver for exposure of the choledochal cyst. (B) A second traction suture has been positioned at the junction of the distal cystic duct and gallbladder fundus to further elevate the liver anteriorly and expose the hepatic hilum.

A

B

Fig. 44.8  (A) After opening the inferior part of the cyst to identify the orifice of the common biliopancreatic channel, a small catheter is inserted into the common channel for irrigation and elimination of protein plugs. (B) After the common channel has been irrigated, the distal choledochal cyst is being ligated with an endoscopic clip and will subsequently be divided at the level of the orifice of the common channel

Hepaticojejunostomy The ligament of Treitz is identified. A 5-0 silk suture is placed 10 cm distal to the ligament of Treitz in the newborn, 20 cm in infants, and 30 cm in children. A second suture (5-0 PDS, Ethicon, Inc., Somerville, NJ) is placed 2 cm below the first suture to mark the jejunal limb, which will be anastomosed to the hepatic duct. The jejunal segment containing the two sutures is grasped with a locking instrument. The previously made transumbilical vertical incision is extended 1.0 cm cephalad. The jejunum is then exteriorized, and the jejunojejunostomy is performed extracorporeally. The jejunum is then returned into the abdominal cavity. The extended umbilical incision is closed, and the laparoscopic instruments are reinserted. The Roux limb is passed through a window in the transverse mesocolon to the porta hepatis. The jejunum is opened longitudinally on its antimesenteric border a few millimeters from the end of the Roux limb to avoid the creation of a significant blind pouch as the child grows. The hepaticojejunostomy is fashioned using two running sutures of

5-0 PDS (interrupted sutures are used when the diameter of the common hepatic duct is 4 mm), edema, and pericholecystic fluid, a nuclear medicine study can help determine the presence of acute cholecystitis. In patients with acute cholecystitis, the radioactive analogs are excreted in the liver, but do not pass into the gallbladder due to obstruction of the cystic duct. In addition, MRCP can be a useful imaging modality for suspected pathology of the gallbladder. It is a noninvasive test and can evaluate pancreaticobiliary anatomy for evidence of a stricture, obstruction, or injuries related to trauma.123,124 However,

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the biggest limitation for using MRCP is the need for sedation or general anesthesia. A second diagnostic adjunct for cholelithiasis is the use of endoscopic US, which can help identify stones not seen on transabdominal US.125 

Jaundice, pancreatitis Ultrasound: dilated CBD, CBD stones

Suspected choledocholithiasis

ERCP

SPECIAL CONSIDERATIONS There are four special considerations when evaluating children with gallbladder disease. The first is the child with sickle cell disease. The most important principle for improved operative outcomes in children with sickle cell anemia relies on adequate hydration and transfusion to an acceptable hemoglobin level of 10 mg/dL.126 A recent retrospective review reported that elective cholecystectomy for cholelithiasis in patients with sickle cell disease decreases morbidity when compared with children undergoing an emergent operation.127 This result is most likely secondary to optimization of hydration prior to the elective cholecystectomy. The second circumstance arises in the patient with HS who is undergoing splenectomy. A right upper quadrant US should be performed to evaluate for cholelithiasis prior to splenectomy as it is relatively straightforward to remove the gallbladder at the same time if gallstones are noted. However, in a study of 17 patients with spherocytosis, but not cholelithiasis, none of these patients developed cholelithiasis with a mean follow-up of 15 years.128 Thus, it is probably not necessary to prophylactically remove the gallbladder in patients with HS undergoing splenectomy. Another area of increasing debate is the routine use of an intraoperative cholangiogram during a laparoscopic cholecystectomy. There is little evidence for its routine use. However, if there is a concern about the anatomy or a concern about the presence of a common duct stone, surgeons should consider performing this study. A fourth situation involves the child or adolescent who presents with known or suspected choledocholithiasis. A recent report noted that approximately 11% of children undergoing cholecystectomy were found to have a CBD stone.129 Associated signs include jaundice, dark urine, and acholic stools. In adults, this situation is most commonly handled by ERCP with sphincterotomy and stone extraction either before or after the laparoscopic cholecystectomy. However, in children, many pediatric gastroenterologists are not trained in this technique and many children’s hospitals require the help of a gastroenterologist for adults. One approach in children with suspected choledocholithiasis is to perform the ERCP and sphincterotomy before performing the laparoscopic cholecystectomy (Fig. 44.9).130–132 In addition, CBD exploration can be a safe approach for choledocholithiasis in situations in which there is limited availability of ERCP.129 This can be performed laparoscopically by experienced surgeons and with an open approach for those who are less experienced. 

ACALCULOUS CHOLECYSTITIS Acute acalculous cholecystitis is defined as inflammation of the gallbladder without the presence of gallstones.118 This entity can result from bile stasis, ischemia, or both.133 Risk factors include TPN, prolonged fasting, volume depletion, multiple transfusions, and sepsis. Symptoms are similar to those of biliary colic; however, the right upper quadrant pain can be more severe. The diagnosis is made

Confirmed choledocholithiasis

No stones

Stones cleared

Laparoscopic cholecystectomy

Stones not cleared

Open/laparoscopic CBD exploration

Fig. 44.9  This algorithm shows one approach for managing children with suspected choledocholithiasis. With this approach, an ERCP is performed prior to the laparoscopic cholecystectomy in a child with suspected choledocholithiasis. If stones are identified and the ERCP and sphincterotomy are successful, the surgeon can proceed with the laparoscopic cholecystectomy soon thereafter. However, if the ERCP and sphincterotomy are not successful, the surgeon will know at the time of the laparoscopic cholecystectomy whether choledochal exploration is also needed.

with an US that reveals gallbladder wall thickness (>4 mm), edema, and pericholecystic fluid without the presence of gallstones. 

LAPAROSCOPIC CHOLECYSTECTOMY The revolution in minimally invasive surgery began with the laparoscopic approach for cholecystectomy.134–139 The standard four-port technique has been modified, and now surgeons can perform this procedure through a single umbilical incision. A recent prospective randomized trial enrolled 60 children to either a single-incision laparoscopic cholecystectomy or the standard four-port technique.140 The results from that trial showed that children who underwent a singleincision laparoscopic cholecystectomy had longer mean operative times when compared with the standard fourport technique as well as greater surgical difficulty (Table 44.2). Furthermore, patients undergoing a single-incision laparoscopic cholecystectomy described having more pain, requiring higher doses of postoperative analgesia, as well as accruing more significant hospital charges (see Table 44.2). Regardless of whether the patient is undergoing the fourport technique or a single-incision approach, the patient is placed supine on the operating table and two video monitors are positioned at the head of the table. An orogastric tube is inserted for decompression of the stomach. For the singleincision approach, some surgeons prefer to stand between the patient’s legs, whereas the surgeon usually stands on the patient’s left side for the four-port technique. For both approaches, the patient is prepped and draped widely. 

FOUR-PORT TECHNIQUE Four small incisions are generally used for the traditional laparoscopic cholecystectomy. A 10-mm port is introduced

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Table 44.2  Outcome Data Between Patients Randomized to Single-Incision or Four-Port Laparoscopic Cholecystectomy Outcome Variable

Single Incision (n = 30)

Four Port (n = 30)

P Value

Operative time (minutes) Difficulty rating (1–5) Total analgesic doses Postoperative length of stay (hours) Hospital charges ($)

68.6 ± 22.1 2.7 ± 1.0 16.4 ± 17.8 22.7 ± 6.2 29.7K ± 27.3K

56.1 ± 22.1 1.9 ± 0.8 10.1 ± 4.3 22.2 ± 6.8 20.6K ± 6.9K

0.03 0.005 0.06 0.44 0.08

From Ostlie DJ, Adibe OO, Juang D, et al. Single incision versus 4-port laparoscopic cholecystectomy: a prospective randomized trial. J Pediatr Surg 2013;48(1):209–214.

Fig. 44.10  The traditional laparoscopic cholecystectomy technique utilizes four ports. The umbilical cannula is 10 mm (as seen here) or 5 mm depending on the size of the telescope. A 5-mm cannula is inserted in the epigastrium, which becomes the main operating site for the surgeon. Two instruments can often be placed through stab incisions on the patient’s right side, one in the mid-abdomen and one in the lower abdomen. These two lateral instruments are not exchanged during the operation, so the stab incision technique often works well. Also, in small patients, as depicted in this photograph, 3-mm instruments can be used on the patient’s right side.

in the umbilicus, and a 10-mm telescope is then inserted. (Although the optics are satisfactory with a 5-mm telescope, it is helpful to have a 10-mm port in the umbilicus to extract the gallbladder, especially if it is inflamed, so there is no real benefit to using a 5-mm umbilical port and telescope.) A 5-mm cannula is inserted in the epigastrium to the right of the patient’s midline, which becomes the main operating site for the surgeon. Two instruments are then placed on the patient’s right side, one in the right mid-abdomen and one in the right lower abdomen (Fig. 44.10). A stab incision technique is often possible for these two right lateral instruments as they are not exchanged during the operation. Also, 3-mm instruments can be utilized in younger patients at these two sites as well. The patient is then rotated into reverse Trendelenburg and to the patient’s left. The gallbladder is grasped using the right lower abdominal instrument and rotated cephalad over the liver to expose the triangle of Calot. The surgeon then utilizes the right upper abdominal instrument and the epigastric instrument to perform the procedure. Initial attention is directed toward lysing adhesions to the infundibulum. Blunt dissection follows to identify the cystic duct and cystic artery. At this point, lateral

retraction of the infundibulum is essential to orient the cystic duct at 90° to the CBD to help prevent misidentification of these two structures. It is important to visualize the critical view of safety to correctly identify the anatomy. This critical view is bounded by the CBD medially, the cystic duct inferiorly, the gallbladder laterally, and the liver superiorly (Fig. 44.11A).141–143 After the cystic duct and common duct have been correctly identified, two options exist. A cholangiogram can be performed if the anatomy is unclear or if there is suspicion of common duct stones. If the anatomy is clear and there is no suspicion of choledocholithiasis, it is reasonable to ligate the cystic duct with endoscopic clips and then divide it (see Fig. 44.11B). Similarly, the cystic artery is ligated and divided (see Fig. 44.11C). Once these two structures have been ligated and divided, the gallbladder is then detached from the liver using retrograde dissection with cautery (see Fig. 44.11D). The hook cautery, spatula cautery, or Maryland dissecting instrument connected to cautery can be used for this purpose. Prior to complete detachment of the gallbladder from the liver, the area of dissection should be inspected to ensure hemostasis, and then the gallbladder is completely detached. If there is little to no inflammation, the gallbladder can usually be exteriorized through the umbilical incision without using an endoscopic bag. However, for inflamed gallbladders, it is best to remove the specimen using a bag. 

SINGLE-SITE LAPAROSCOPIC CHOLECYSTECTOMY For single-site umbilical laparoscopic cholecystectomy, it is necessary to use an umbilical incision of approximately 2 cm in length. In the United States a premanufactured port is often utilized. The two main devices used are the SILS Port (Covidien, Norwalk, CT) and the TriPort (Olympus America, Center Valley, PA). The SILS Port is a foam port with three working channels. The fourth instrument can usually be placed along the left side of the port (Fig. 44.12A). Although the TriPort is designed for three instruments, a fourth 3-mm instrument can be inserted through one of the insufflation channels (see Fig. 44.12B). It is helpful to have a long telescope so that the telescope holder is standing out of the way of the operating surgeon. Outside the United States, many surgeons place a single port in the umbilicus with instruments inserted through the fascia surrounding the umbilicus. Sometimes, low-profile, 5-mm individual ports are utilized. Regardless of the technique and orientation of the instruments through the umbilicus, the principles of the procedure are the same as for the

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A

B

C

D

Fig. 44.11  These four figures depict the salient points for a laparoscopic cholecystectomy. (A) The gallbladder infundibulum is retracted laterally to orient the cystic duct (solid arrow) in relation to the common duct (asterisk). Note the critical view of safety is identified. In this view, the liver is seen through the opened space bounded by the cystic duct inferiorly, gallbladder laterally, and the liver superiorly. (B) The cystic duct has been ligated with endoscopic clips. Two clips are placed on the medial aspect of the duct, which will remain following duct division. (C) The cystic duct has been divided, and the cystic artery (dotted arrow) is visualized. (D) Following ligation and division of the cystic artery, the gallbladder is being dissected away from its liver bed using the hook cautery.

A

B

Fig. 44.12  In the United States, a premanufactured port is often utilized for a single-site umbilical laparoscopic cholecystectomy. The two main devices used are the SILS Port (Covidien, Norwalk, CT) seen on the left and the TriPort (Olympus America, Center Valley, PA) on the right. In (A) there are three working channels in this SILS Port. A fourth instrument (solid arrow) can be inserted along the left side of the port. (B) The TriPort is designed for three instruments. However, a fourth 3-mm instrument (dotted arrow) can be inserted through one of the two insufflation channels.

traditional four-port laparoscopic cholecystectomy. Lateral retraction of the infundibulum is important for visualization of the triangle of Calot and critical view of safety. The cystic duct and cystic artery are similarly ligated and divided as with the four-port technique. One difference between the two approaches is that it is best to irrigate and suction all the fluid prior to exteriorizing the gallbladder as gallbladder removal entails removing the premanufactured port (if

utilized). It can often be difficult to reinsert these ports, so it is best to irrigate and suction prior to extracting the gallbladder. After removing the gallbladder and umbilical port, the umbilical fascia is closed with either interrupted or continuous 0-absorbable suture. The skin is approximated with interrupted 5-0 plain sutures. The main advantage of the single-incision approach appears to be cosmesis, but this advantage is marginal in most patients (Fig. 44.13). 

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Fig. 44.13  These two patients both underwent a laparoscopic cholecystectomy. (A) The patient underwent a laparoscopic cholecystectomy using four ports. (B) The patient underwent a single-site umbilical laparoscopic cholecystectomy. To date, the main advantage of the single-site umbilical laparoscopic surgery approach appears to be cosmesis, but this advantage is marginal in most patients.

CHILDREN’S MERCY HOSPITAL EXPERIENCE The evolving demands of our current health care system for enhanced efficiency and safety, while at the same time decreasing the duration of hospitalization, has led us to develop an institutional protocol for same-day discharge following laparoscopic cholecystectomy. Reports of same-day discharge following laparoscopic cholecystectomy in adults began in the early 1990s. In 2013, we implemented our clinical initiative for same-day discharge.144 Following our initial experience with same-day discharge following laparoscopic cholecystectomy, we performed a prospective observational study to evaluate the protocol’s safety and efficacy.145 Patients with symptomatic cholelithiasis or biliary dyskinesia were prospectively followed from July 2014 to July 2015, labeled period 2. These patients were then compared with children undergoing a laparoscopic cholecystectomy during the initial phase of the same-day discharge protocol from January 2013 to July 2014, labeled period 1. Patients with clinical evidence of cholecystitis, gallstone pancreatitis, or choledocholithiasis or those who underwent a cholecystectomy as part of another procedure were excluded from the study. Patients underwent either a standard four-port or single-incision laparoscopic cholecystectomy. The incision sites were infiltrated with 0.25% bupivacaine hydrochloride, and ketorolac was used intraoperatively for additional pain control. After the procedure, the patients were sent to the postanesthesia care unit and then transferred to a short-stay recovery unit for 4–6 hours before being discharged home. From January 2013 to July 2015, 191 laparoscopic cholecystectomies were performed, with 116 procedures occurring in the first period and 75 in the second period. In the first period, 47% (n = 54) were discharged the same day compared with 78% (n = 59) in the second period (P < 0.001). In addition, the mean hospital length of stay in the second period was 9 hours compared with 18.7 hours in the first period (P < 0.0001). Our study found that same-day discharge following laparoscopic cholecystectomy was safe and feasible. Also, there was a similar readmission rate between the two groups.

Our group at Children’s Mercy Hospital has continued to see an increasing incidence of cholecystectomy for biliary dyskinesia in children. We have reported our experience with long-term follow-up in this patient population. With a median follow-up of 3.7 years, 61% of patients reported resolution of their biliary dyskinesia symptoms following laparoscopic cholecystectomy using a telephone survey.117 Ejection fraction, body mass index, chronic cholecystitis, and pain reproduction with CCK administration were not predictors of persistent symptoms at either short- or longterm follow-up. Interestingly, the majority of patients with continuing symptoms did not regret cholecystectomy.

References











1. Hernanz-Schulman M, Ambrosino MM, Freeman PC, et  al. Common bile duct in children: sonographic dimensions. Radiology. 1995;195:193–195. 2. Kim HJ, Kim MH, Lee SK, et  al. Normal structure, variations, and anomalies of the pancreaticobiliary ducts of Koreans: a nationwide cooperative prospective study. Gastrointest Endosc. 2002;55: 889–896. 3. Witcombe JB, Cremin BJ. The width of the common bile duct in childhood. Pediatr Radiol. 1978;7:147–149. 4. Alonso-Lej F, Rever Jr WB, Pessagno DJ. Congenital choledochal cyst, with a report of 2, and an analysis of 94, cases. Int Abstr Surg. 1959;108:1–30. 5. Todani T, Watanabe Y, Narusue M, et al. Congenital bile duct cysts: classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. Am J Surg. 1977;134:263–269. 6. Nguyen XT, Hoang GC, Nguyen TL, et  al. Surgical treatment of congenital cystic dilation of the biliary tract. Acta Chir Scand. 1986;152:669–674. 7. Nguyen TL, Valayer J. Congenital dilatation of the common bile duct in children. Study of a series of 52 cases. Presse Med. 1994;23:1565– 1568. Dilatation congenitale de la voie biliaire principale chez l’enfant. Etude d’une serie de 52 cas. 8. Miyano T, Yamataka A, Li L. Congenital biliary dilatation. Semin Pediatr Surg. 2000;9:187–195. 9. Lilly JR, Stellin GP, Karrer FM. Forme fruste choledochal cyst. J Pediatr Surg. 1985;20:449–451. 10. Miyano T, Ando K, Yamataka A, et al. Pancreaticobiliary maljunction associated with nondilatation or minimal dilatation of the common bile duct in children: diagnosis and treatment. Eur J Pediatr Surg. 1996;6:334–337. 11. Shimotakahara A, Yamataka A, Kobayashi H, et  al. Forme fruste choledochal cyst: long-term follow-up with special reference to surgical technique. J Pediatr Surg. 2003;38:1833–1836.

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12. Thomas S, Sen S, Zachariah N, et  al. Choledochal cyst sans cyst--experience with six “forme fruste” cases. Pediatr Surg Int. 2002;18:247–251. 13. Caroli JSR, Kossakowski J, et al. La dilatation polykystique congénitale des voies biliaires intrahépatiques: essai de classification. Sem Hop Paris. 1958:128–135. 14. Madjov R, Chervenkov P, Madjova V, et  al. Caroli’s disease. Report of 5 cases and review of literature. Hepato-gastroenterology. 2005;52:606–609. 15. Kassahun WT, Kahn T, Wittekind C, et  al. Caroli’s disease: liver resection and liver transplantation. Experience in 33 patients. Surgery. 2005;138:888–898. 16. Davenport M, Stringer MD, Howard ER. Biliary amylase and congenital choledochal dilatation. JJ Pediatr Surg. 1995;30:474– 477. 17. Shimotake T, Iwai N, Yanagihara J, et  al. Innervation patterns in congenital biliary dilatation. Eur J Pediatr Surg. 1995;5:265–270. 18. Imazu M, Ono S, Kimura O, et al. Histological investigations into the difference between cystic and fusiform types of congenital biliary dilatation. Eur J Pediatr Surg. 2003;13:16–20. 19. Babbitt DP. Congenital choledochal cysts: new etiological concept based on anomalous relationships of the common bile duct and pancreatic bulb. Ann Radiol. 1969;12:231–240. 20. Jeong IH, Jung YS, Kim H, et  al. Amylase level in extrahepatic bile duct in adult patients with choledochal cyst plus anomalous pancreatico-biliary ductal union. World J Gastroenterol. 2005;11: 1965–1970. 21. Ochiai K, Kaneko K, Kitagawa M, et  al. Activated pancreatic enzyme and pancreatic stone protein (PSP/reg) in bile of patients with pancreaticobiliary maljunction/ choledochal cysts. Dig Dis Sci. 2004;49:1953–1956. 22. Jung SM, Seo JM, Lee SK. The relationship between biliary amylase and the clinical features of choledochal cysts in pediatric patients. World J Surg. 2012;36:2098–2101. 23. Yamashiro Y, Miyano T, Suruga K, et al. Experimental study of the pathogenesis of choledochal cyst and pancreatitis, with special reference to the role of bile acids and pancreatic enzymes in the anomalous choledocho-pancreatico ductal junction. J Pediatr Gastroenterol Nutr. 1984;3:721–727. 24. Komi N, Tamura T, Miyoshi Y, et al. Nationwide survey of cases of choledochal cyst. Analysis of coexistent anomalies, complications and surgical treatment in 645 cases. Surg Gastroenterol. 1984;3:69– 73. 25. Spitz L. Experimental production of cystic dilatation of the common bile duct in neonatal lambs. J Pediatr Surg. 1977;12:39–42. 26. Davenport M, Basu R. Under pressure: choledochal malformation manometry. J Pediatr Surg. 2005;40:331–335. 27. Kimura K, Ohto M, Ono T, et al. Congenital cystic dilatation of the common bile duct: relationship to anomalous pancreaticobiliary ductal union. AJR Am J Roentgenol. 1977;128:571–577. 28. Ono J, Sakoda K, Akita H. Surgical aspect of cystic dilatation of the bile duct. An anomalous junction of the pancreaticobiliary tract in adults. Ann Surg. 1982;195:203–208. 29. Guelrud M, Morera C, Rodriguez M, et  al. Normal and anomalous pancreaticobiliary union in children and adolescents. Gastrointest Endosc. 1999;50:189–193. 30. Wong JK, Campbell D, Ngo ND, et al. Genetic study of congenital bileduct dilatation identifies de novo and inherited variants in functionally related genes. BMC Med Genomics. 2016;9:75. 31. Liem NT, Pham HD, Dung le A, et  al. Early and intermediate outcomes of laparoscopic surgery for choledochal cysts with 400 patients. J Laparoendosc Adv Surg Tech A. 2012;22:599–603. 32. Lai HS, Duh YC, Chen WJ, et al. Manifestations and surgical treatment of choledochal cyst in different age group patients. J Formos Med Assoc. 1997;96:242–246. 33. Okada A, Nakamura T, Higaki J, et al. Congenital dilatation of the bile duct in 100 instances and its relationship with anomalous junction. Surg Gynecol Obstet. 1990;171:291–298. 34. Ando K, Miyano T, Kohno S, et al. Spontaneous perforation of choledochal cyst: a study of 13 cases. Eur J Pediatr Surg. 1998;8:23–25. 35. Ahmed I, Sharma A, Gupta A, et al. Management of rupture of choledochal cyst. Indian J Gastroenterol. 2011;30:94–96. 36. Voyles CR, Smadja C, Shands WC, et al. Carcinoma in choledochal cysts. Age-related incidence. Arch Surg. 1983;118:986–988.

37. Imazu M, Iwai N, Tokiwa K, et al. Factors of biliary carcinogenesis in choledochal cysts. Eur J Pediatr Surg. 2001;11:24–27. 38. Kimura K, Ohto M, Saisho H, et  al. Association of gallbladder carcinoma and anomalous pancreaticobiliary ductal union. Gastroenterology. 1985;89:1258–1265. 39. Todani T, Watanabe Y, Toki A, et  al. Carcinoma related to choledochal cysts with internal drainage operation. Surg Gynecol Obstet. 1987;164:61–64. 40. Jang JY, Yoon CH, Kim KM. Endoscopic retrograde cholangiopancreatography in pancreatic and biliary tract disease in Korean children. World J Gastroenterol. 2010;16:490–495. 41. Otto AK, Neal MD, Slivka AN, et al. An appraisal of endoscopic retrograde cholangiopancreatography (ERCP) for pancreaticobiliary disease in children: our institutional experience in 231 cases. Surg Endosc. 2011;25:2536–2540. 42. Park DH, Kim MH, Lee SK, et al. Can MRCP replace the diagnostic role of ERCP for patients with choledochal cysts? Gastrointest Endosc. 2005;62:360–366. 43. Huang CT, Lee HC, Chen WT, et  al. Usefulness of magnetic resonance cholangiopancreatography in pancreatobiliary abnormalities in pediatric patients. Pediatr Neonatol. 2011;52:332–336. 44. Irie H, Honda H, Jimi M, et  al. Value of MR cholangiopancreatography in evaluating choledochal cysts. AJR Am J Roentgenol. 1998;171:1381–1385. 45. Saing H, Tam PKH, Lee JMH, et  al. Surgical management of choledochal cysts: a review of 60 cases. J Pediatr Surg. 1985;20:443– 448. 46. Shi LB, Peng SY, Meng XK, et al. Diagnosis and treatment of congenital choledochal cyst: 20 years’ experience in China. World J Gastroenterol. 2011;7:732–747. 47. Fu M, Wang YX, Zhang JZ. Evolution in the treatment of choledochal cyst. J Pediatr Surg. 2000;335:1344–1347. 48. Watanabe Y, Toki A, Todani T. Bile duct cancer developed after cyst excision for choledochal cyst. J Hepatobiliary Pancreat Surg. 1999;6:207–212. 49. Koshinaga T, Hoshino M, Inoue M, et  al. Pancreatitis complicated with dilated choledochal remnant after congenital cyst excision. Pediatr Surg Int. 2005;21:936–938. 50. Burnweit CA, Birken GA, Heiss K. The management of choledochal cysts in the newborn. Pediatr Surg Int. 1996;11:130–133. 51. Howell CG, Templeton JM, Weiner S, et  al. Antenatal diagnosis and early surgery for choledochal cysts. J Pediatr Surg. 1983;18: 387–393. 52. Ohi R, Yaota S, Kamiyama T, et al. Surgical treatment of congenital dilatation of the bile duct with special reference to late complications after total cyst excision operation. J Pediatr Surg. 1990;25:613– 617. 53. Miyano T, Yamataka A, Kato Y, et  al. Hepaticoenterostomy after excision of choledochal cyst in children: a 30-year experience with 180 cases. J Pediatr Surg. 1996;31:1417–1421. 54. Edil BH, Cameron JL, Reddy S, et al. Choledochal cyst disease in children and adults: a 30-year single-institution experience. J Am Coll Surg. 2008;206:1000–1005. 55. She W, Chung HY, Lan LCL, et al. Management of choledochal cyst: 30 years of experience and results in a single center. J Pediatr Surg. 2009;44:2307–2311. 56. Stringer MD. Wide hilar hepaticojejunostomy: the optimum method of reconstruction after choledochal cyst excision. Pediatr Surg Int. 2007;23:529–532. 57. Ono S, Fumino S, Shimadera S, et  al. Long-term outcomes after hepaticojejunostomy for choledochal cyst: a 10-27 year follow-up. J Pediatr Surg. 2010;45:376–378. 58. Todani T, Watanabe Y, Mizuguchi T, et al. Hepaticoduodenostomy at the hepatic hilum after excision of choledochal cyst. Am J Surg. 1981;142:584–587. 59. Oweida SW, Ricketts RR. Hepatico-jejuno-duodenostomy reconstruction following excision of choledochal cysts in children. Am Surg. 1989;55:2–6. 60. Cosentino CM, Luck SR, Raffensperger JG, et  al. Choledochal duct cyst resection with physiologic reconstruction. Surgery. 1992;112: 740–747. 61. Santore MT, Behar BJ, Blinman TA, et  al. Hepaticoduodenostomy vs. hepaticojejunostomy for reconstruction after resection of choledochal cyst. J Pediatr Surg. 2011;46:209–213.

44 • Choledochal Cyst and Gallbladder Disease 62. Yeung F, Chung PH, Wong KK, et al. Biliary-enteric reconstruction with hepaticoduodenostomy following laparoscopic excision of choledochal cyst is associated with better postoperative outcomes: a single-centre experience. Pediatr Surg Int. 2015;31:149– 153. 63. Bismuth H, Franco D, Corlette MB, et  al. Long term results of Roux-en-Y hepaticojejunostomy. Surg Gynecol Obstet. 1978;146: 161–167. 64. Martino A, Noviello C, Cobellis G, et al. Delayed upper gastrointestinal bleeding after laparoscopic treatment of form fruste choledochal cyst. J Laparoendosc Adv Surg Tech A. 2009;19:457–459. 65. Malhotra RS, Jain A, Prabhu RY, et al. Ischemic stricture of Rouxen-Y intestinal loop and recurrent cholangitis. Indian J Gastroenterol. 2005;24:76–77. 66. Houben CH, Chan M, Cheung G, et al. A hepaticojejunostomy: Technical errors with “twists and turns”. Pediatr Surg Int. 2006;22:841– 844. 67. Shimotakahara A, Yamataka A, Yanai T, et al. Roux-en Y hepaticojejunostomy or hepaticoduodenostomy for biliary reconstruction during the surgical treatment of choledochal cyst: which is better. Pediatr Surg Int. 2005;21:5–7. 68. Takada K, Hamada Y, Watanabe K, et  al. Duodenal gastric reflux following biliary reconstruction after excision of choledochal cyst. Pediatr Surg Int. 2005;21:1–4. 69. Farello GA, Cerofolini A, Rebonato M, et al. Congenital choledochal cyst: video-guided laparoscopic treatment. Surg Laparosc Endosc. 1995;5:354–358. 70. Tanaka M, Shimizu S, Mizumoto K, et al. Laparoscopically assisted resection of choledochal cyst and Roux-en-Y reconstruction. Surg Endosc. 2001;15:545–552. 71. Tan HL, Shankar KR, Ford WD. Laparoscopic resection of type I choledochal cyst. Surg Endosc. 2003;17:1495. 72. Li L, Feng W, Jing-Bo F, et al. Laparoscopic-assisted total cyst excision of choledochal cyst and Roux-en Y hepatoenterostomy. J Pediatr Surg. 2004;39:1663–1666. 73. Lee H, Hirose S, Bratton B, et al. Initial experience with complex laparoscopic biliary surgery in children: biliary atresia and choledochal cyst. J Pediatr Surg. 2004;39:804–807. 74. Jang JY, Kim SW, Han HS, et  al. Totally laparoscopic management of choledochal cyst using a four-hole method. Surg Endosc. 2006;20:1762–1765. 75. Laje P, Questa H, Bailez M. Laparoscopic leak-free technique for the treatment of choledochal cyst. J Laparoendosc Adv Surg Tech A. 2007;17:519–521. 76. Aspelund G, Ling SC, Ng V, et al. A role for laparoscopic approach in the treatment of biliary atresia and choledochal cysts. J Pediatr Surg. 2007;42:869–873. 77. Hong L, Wu Y, Yan Z, et  al. Laparoscopic surgery for choledochal cyst in children: a case review of 31 patients. Eur J Pediatr Surg. 2008;18:67–71. 78. Liem NT, Dung LA, Son TN. Laparoscopic complete cyst excision and hepaticoduodenostomy for choledochal cyst: early results in 74 cases. J Laparoendos Adv Surg Tech. 2009;19:s87–s90. 79. Nguyen Thanh L, Hien PD, Dung le A, et  al. Laparoscopic repair for choledochal cyst: lessons learned from 190 cases. J Pediatr Surg. 2010;45:540–544. 80. Chokshi NK, Guner YS, Aranda A, et al. Laparoscopic choledochal cyst excision: lessons learned in our experience. J Laparoendosc Adv Surg Tech A. 2009;19:87–91. 81. Lee KH, Tam YH, Yeung CK, et  al. Laparoscopic excision of choledochal cyst in children: an intermediate-term report. Pediatr Surg Int. 2009;25:355–360. 82. Qiao G, Li L, Li S, et al. Laparoscopic cyst excision and Roux-Y hepaticojejunostomy for children with choledochal cysts in China: a multicenter study. Surg Endos. 2015;29:140–144. 83. Diao M, Li L, Zhang JS, et  al. Laparoscopic-assisted clearance of protein plugs in the common channel in children with choledochal cysts. J Pediatr Surg. 2010;45:2099–2102. 84. Miyano G, Koga H, Shimotakahara A, et al. Intralaparoscopic endoscopy: its value during laparoscopic repair of choledochal cyst. Pediatr Surg Int. 2011;27:463–466. 85. Koga H, Okawada M, Doi T, et al. Refining the intraoperative measurement of the distal intrapancreatic part of a choledochal cyst during laparoscopic repair allows near total excision. Pediatr Surg Int. 2015;31:991–994.

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86. Urushihara N, Fukumoto K, Nouso H, et  al. Hepatic ductoplasty and hepaticojejunostomy to treat narrow common hepatic duct during laparoscopic surgery for choledochal cyst. Pediatr Surg Int. 2015;31:983–986. 87. Li L, Liu SL, Hou WY, et al. Laparoscopic correction of biliary duct stenosis in choledochal cyst. J Pediatr Surg. 2008;43:644–646. 88. Diao M, Li L, Li Q, et al. Challenges and strategies for single-incision laparoscopic Roux-en-Y hepaticojejunostomy in managing giant choledochal cysts. Int J Surg. 2014;12:412–417. 89. Son TN, Liem NT, Hoan VX. Transumbilical laparoendoscopic single-site surgery with conventional instruments for choledochal cyst in children: early results of 86 cases. J Laparoendosc Adv Surg Tech A. 2014;24:907–910. 90. Tang Y, Li F, He G. Comparison of single-incision and conventional laparoscopic cyst excision and Roux-en-Y hepaticojejunostomy for children with choledochal cysts. Indian J Surg. 2016;78:259–264. 91. Woo R, Le D, Albanese CT, et al. Robot-assisted laparoscopic resection of a type I choledochal cyst in a child. J Laparoendosc Adv Surg Tech A. 2006;16:179–183. 92. Meehan JJ, Elliott S, Sandler A. The robotic approach to complex hepatobiliary anomalies in children: preliminary report. J Pediatr Surg. 2007;42:2110–2114. 93. Alizai NK, Dawrant MJ, Najmaldin AS. Robot-assisted resection of choledochal cysts and hepaticojejunostomy in children. Pediatr Surg Int. 2014;30:291–294. 94. Naitoh T, Morikawa T, Tanaka N, et al. Early experience of robotic surgery for type I congenital dilatation of the bile duct. J Robot Surg. 2015;9:143–148. 95. Kim NY, Chang EY, Hong YJ, et al. Retrospective assessment of the validity of robotic surgery in comparison to open surgery for pediatric choledochal cyst. Yonsei Med J. 2015;56:737–743. 96. Liem NT, Pham HD, Vu HM. Is the laparoscopic operation as safe as open operation for choledochal cyst in children? J Laparoendosc Adv Surg Tech A. 2011;21:367–370. 97. Diao M, Li L, Cheng W. Laparoscopic versus open Roux-en-Y hepatojejunostomy for children with choledochal cysts: intermediate-term follow-up results. Surg Endosc. 2011;25:1567–1573. 98. Shen HJ, Xu M, Zhu HY, et al. Laparoscopic versus open surgery in children with choledochal cysts: a meta-analysis. Pediatr Surg Intl. 2015;31:529–534. 99. Yamataka A, Ohshiro K, Okada Y, et  al. Complications after cyst excision with hepaticoenterostomy for choledochal cysts and their surgical management in children versus adults. J Pediatr Surg. 1997;32:1097–1102. 100. Sheng Q, Lv Z, Xu W, et  al. Reoperation after cyst excision with hepaticojejunostomy for choledochal cysts: our experience in 18 cases. Med Sci Monit. 2017;23:1371–1377. 101. Kim E, Kang MH, Lee J, et al. Two cases of plug or stone in remnant intrapancreatic choledochal cysts treated with endoscopic retrograde cholangiopancreatography. Clin Endosc. 2017;50(5):504–507. 102. Xia HT, Yang T, Liang B, et  al. Treatment and outcomes of adults with remnant intrapancreatic choledochal cysts. Surgery. 2016;159:418–425. 103. Martin RF, Biber BP, Bosco JJ, et al. Symptomatic choledochoceles in adults. Endoscopic retrograde cholangiopancreatography recognition and management. Arch Surg. 1992;127:536–538. 104. Dohmoto M, Kamiya T, Hunerbein M, et  al. Endoscopic treatment of a choledochocele in a 2-year-old child. Surg Endosc. 1996;10: 1016–1018. 105. Liem NT. Laparoscopic surgery for choledochal cysts. J Hepatobiliary Pancreat Sci. 2013;20:487–491. 106. Holcomb Jr GW, O’Neill Jr JA, Holcomb III GW. Cholecystitis, cholelithiasis and common duct stenosis in children and adolescents. Ann Surg. 1980;191:626–635. 107. Walker SK, Maki AC, Cannon RM, et  al. Etiology and incidence of pediatric gallbladder disease. Surgery. 2013;154:927–933. 108. Khoo AK, Cartwright R, Berry S, et  al. Cholecystectomy in English children: evidence of an epidemic (1997-2012). J Pediatr Surg. 2014;49:284–288. 109. Lacher M, Yannam GR, Muensterer OJ, et  al. Laparoscopic cholecystectomy for biliary dyskinesia in children: frequency increasing. J Pediatr Surg. 2013;48:1716–1721. 110. Friesen CA, Neilan N, Daniel JF, et al. Mast cell activation and clinical outcome in pediatric cholelithiasis and biliary dyskinesia. BMC Res Notes. 2011;4:322.

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111. Rau B, Friesen CA, Daniel JF, et  al. Gallbladder wall inflammatory cells in pediatric patients with biliary dyskinesia and cholelithiasis: a pilot study. J Pediatr Surg. 2006;41:1545–1548. 112. Srinath AI, Youk AO, Bielefeldt K. Biliary dyskinesia and symptomatic gallstone disease in children: two sides of the same coin? Dig Dis Sci. 2014;59:1307–1315. 113. Misra S. Is biliary scintigraphy a reliable diagnostic tool for biliary dyskinesia in children? J Clin Gastroenterol. 2011;45:814–817. 114. Hofeldt M, Richmond B, Huffman K, et al. Laparoscopic cholecystectomy for treatment of biliary dyskinesia is safe and effective in the pediatric population. Am Surg. 2008;74:1069–1072. 115. Lai SW, Rothenberg SS, Kay SM, et  al. Outcomes of laparoscopic cholecystectomy for biliary dyskinesia in children. J Laparoendosc Adv Surg Tech A. 2017;27:845–850. 116. Carney DE, Kokoska ER, Grosfeld JL, et  al. Predictors of successful outcome after cholecystectomy of biliary dyskinesia. J Pediatr Surg. 2004;39:813–816. 117. Knott EM, Fike FB, Gasior AC, et  al. Multi-institutional analysis of long-term symptom resolution after cholecystectomy for biliary dyskinesia in children. Pediatr Surg Int. 2013;29:1243–1247. 118. Rothstein DH, Harmon CM. Gallbladder disease in children. Semin Pediatr Surg. 2016;25:225–231. 119. Hadigan C, Fishman SJ, Connolly LP, et al. Stimulation with fatty meal (Lipomul) to assess gallbladder emptying in children with chronic acalculous cholecystitis. J Pediatr Gastroenterol Nutr. 2003;37:178–182. 120. Tuna Kirsaclioglu C, Cuhaci Cakir B, Bayram G, et al. Risk factors, complications and outcome of cholelithiasis in children: a retrospective, single-centre review. J Paediatr Child Health. 2016;52:944–949. 121. Cooperberg PL, Burhenne HJ. Real-time ultrasonography. Diagnostic technique of choice in calculous gallbladder disease. N Engl J Med. 1980;302:1277–1279. 122. Tsai J, Sulkowski JP, Cooper JN, et al. Sensitivity and predictive value of ultrasound in pediatric cholecystitis. J Surg Res. 2013;184:378–382. 123. Delaney L, Applegate KE, Karmazyn B, et al. MR cholangiopancreatography in children: feasibility, safety, and initial experience. Pediatr Radiol. 2008;38:64–75. 124. Testoni PA, Mariani A, Curioni S, et al. MRCP-secretin test-guided management of idiopathic recurrent pancreatitis: long-term outcomes. Gastrointest Endosc. 2008;67:1028–1034. 125. Neff LP, Mishra G, Fortunato JE, et  al. Microlithiasis, endoscopic ultrasound, and children: not just little gallstones in little adults. J Pediatr Surg. 2011;46:462–466. 126. Rhodes MM, Bates DG, Andrews T, et al. Abdominal pain in children with sickle cell disease. J Clin Gastroenterol. 2014;48:99–105. 127. Goodwin EF, Partain PI, Lebensburger JD, et al. Elective cholecystectomy reduces morbidity of cholelithiasis in pediatric sickle cell disease. Pediatr Blood Cancer. 2017;64:113–120. 128. Sandler A, Winkel G, Kimura K, et al. The role of prophylactic cholecystectomy during splenectomy in children with hereditary spherocytosis. J Pediatr Surg. 1999;34:1077–1078.

129. Hill SJ, Wulkan ML, Parker PM, et al. Management of the pediatric patient with choledocholithiasis in an era of advanced minimally invasive techniques. J Laparoendosc Adv Surg Tech A. 2014;24: 38–42. 130. Mah D, Wales P, Njere I, et al. Management of suspected common bile duct stones in children: role of selective intraoperative cholangiogram and endoscopic retrograde cholangiopancreatography. J Pediatr Surg. 2004;39:808–812. 131. Newman KD, Powell DM, Holcomb 3rd GW. The management of choledocholithiasis in children in the era of laparoscopic cholecystectomy. J Pediatr Surg. 1997;32:1116–1119. 132. Zargar SA, Javid G, Khan BA, et  al. Endoscopic sphincterotomy in the management of bile duct stones in children. Am J Gastroenterol. 2003;98:586–589. 133. McChesney JA, Northup PG, Bickston SJ. Acute acalculous cholecystitis associated with systemic sepsis and visceral arterial hypoperfusion: a case series and review of pathophysiology. Dig Dis Sci. 2003;48:1960–1967. 134. Reynolds Jr W. The first laparoscopic cholecystectomy. JSLS. 2001;5:89–94. 135. Dubois F, Berthelot G, Levard H. Cholecystectomy by coelioscopy. Presse Med. 1989;18:980–982. 136. Reddick EJ, Olsen DO. Laparoscopic laser cholecystectomy. a comparison with mini-lap cholecystectomy. Surg Endosc. 1989;3: 131–13. 137. McKernan JB. Laparoscopic cholecystectomy. Am Surg. 1991; 57:309–312. 138. Holcomb III GW, Sharp KW, Olsen DO. Laparoscopic cholecystectomy in the pediatric patient. J Pediatr Surg. 1991;26:1186– 1190. 139. Holcomb III GW. Laparoscopic cholecystectomy. Semin Pediatr Surg. 1993;2:159–167. 140. Ostlie DJ, Juang OO, Iqbal CW, et al. Single incision versus standard 4-port laparoscopic cholecystectomy: a prospective randomized trial. J Pediatr Surg. 2013;48:209–214. 141. Strasberg SM, Hertl M, Soper NJ. An analysis of the problem of biliary injury during laparoscopic cholecystectomy. J Am Coll Surg. 1995;180:101–125. 142. Vettoretto N, Saronni C, Harbi A, et al. Critical view of safety during laparoscopic cholecystectomy. JSLS. 2011;15:322–325. 143. Strasberg SM, Brunt LM. Rationale and use of the critical view of safety in laparoscopic cholecystectomy. J Am Coll Surg. 2010;211:132–138. 144. Dalton BG, Gonzalez KW, Knott EM, et  al. Same day discharge after laparoscopic cholecystectomy in children. J Surg Res. 2015;195:418–421. 145. Gould JL, Poola AS, St Peter SD, et al. Same day discharge protocol implementation trends in laparoscopic cholecystectomy in pediatric patients. J Pediatr Surg. 2016;51:1936–1938.

45

Solid Organ Transplantation in Children ALEXANDER J. BONDOC, JAIMIE D. NATHAN, MARIA H. ALONSO, and GREGORY M. TIAO

A retrospective review of the United Network for Organ Sharing Database (UNOS) from 1987–2012 recently estimated that over 2 million life-years have been saved by solid organ transplant, including both adult and pediatric patients.1 The ability to successfully perform solid organ transplantation in children has led to a remarkable improvement in survival and quality of life. In this chapter, each of the abdominal solid organ transplant procedures will be discussed, including the indications, operative procedure, and postoperative complications relevant to the practicing pediatric surgeon.

Liver Transplantation Few subspecialties have undergone the dramatic improvements in survival that have occurred in pediatric liver transplantation (LT). In the early 1980s, survival rates of 30% limited the enthusiasm for this costly, work-intensive endeavor. The introduction of more effective immunosuppression along with refinements in the operative and postoperative management of infants and children has led to survival rates greater than 90%. Challenges remain, including the need for donor organs suitable for pediatric recipients of all ages and sizes, the optimization of the pretransplant patient physiology to increase peri-transplant survival, and the improvement in long-term quality of life (QOL).

INDICATIONS FOR TRANSPLANTATION The most common clinical presentations prompting transplant evaluation in children can be classified as (1) primary liver disease with the expected outcome of hepatic failure, (2) chronic liver disease with significant morbidity or known mortality, (3) hepatic-based metabolic disease, (4) fulminant hepatic failure, and (5) hepatic neoplasms such as a malignancy (i.e., hepatoblastoma, where the tumor is not resectable by conventional means) or a vascular malformation in which extensive arteriovenous shunting causes irreversible heart failure. Table 45.1 reviews the major diagnoses that lead to LT. These disease entities define the bimodal age distribution of pediatric transplant recipients. Infants and children with biliary atresia (BA) and, occasionally, rapidly progressive hepatic failure secondary to metabolic abnormalities, such as neonatal tyrosinemia, hemochromatosis, and hepatic tumors, are the patients who require transplantation early

in life. Patients with metabolic disturbances, fulminant hepatic failure, and cirrhosis present as older children and adolescents requiring LT.

Biliary Atresia Children with extrahepatic BA constitute at least 50% of the pediatric LT population. Successful biliary drainage achieving an anicteric state following the Kasai portoenterostomy is the most important factor affecting preservation of liver function and long-term survival and precludes transplantation in infancy and early childhood.2 Primary transplantation without portoenterostomy is not recommended in patients with BA unless the initial presentation is at an age greater than 120 days and a liver biopsy shows advanced cirrhosis.3,4 Patients with progressive disease following a Kasai procedure should be offered timely orthotopic liver transplantation (OLT). The sequential use of portoenterostomy and LT optimizes overall survival and organ use.4 Patients with extrahepatic BA who are seen for transplantation form several cohorts. Infants with a failed Kasai have recurrent cholangitis, ascites, rapidly progressive portal hypertension, malnutrition, and progressive hepatic synthetic failure. This cohort requires OLT within the first 2 years of life. Children with the successful establishment of biliary drainage have improved survival with their native liver, but successful drainage does not preclude the development of cirrhosis and portal hypertension leading to hypersplenism, variceal hemorrhage, ascites, and occasionally hepatopulmonary syndrome. These patients require LT later in childhood. Patients with mild hepatocellular enzyme and bilirubin elevation and mild portal hypertension can be observed with ongoing medical therapy. Currently, long-term follow-up studies indicate that virtually all patients with BA will ultimately require OLT at some point in their life.5  Alagille Syndrome Alagille syndrome (angiohepatic dysplasia) is an autosomal dominant genetic disorder that manifests as bile duct paucity that leads to progressive cholestasis, pruritus, xanthomas, malnutrition, and growth failure. Liver failure occurs at varying time points, with criteria for LT being the typical manifestations of chronic liver disease—complications of portal hypertension and growth failure. QOL issues such as intractable pruritus, hypercholesterolemia, and intractable bone disease are criteria for consideration for LT.6 Preoperative evaluation must include assessment for congenital cardiac disease and renal insufficiency, both of which are 709

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Table 45.1  Indications for Liver Transplantation at Cincinnati Children’s, 1986–2017 Diagnosis

No. of Patients Percentage (%)

Alagille syndrome Autoimmune hepatitis Biliary atresia Cholestatic liver disease Cirrhosis Cystic fibrosis Fulminant liver failure Hemangioendothelioma Hepatoblastoma/tumor Metabolic disease—glycogen storage disease Metabolic disease—other Metabolic disease—primary hyperoxaluria Metabolic disease—tyrosinemia Metabolic disease—urea cycle defects Metabolic disease—Wilson disease Metabolic disease—α1-antitrypsin deficiency Other Other tumor Progressive familial intrahepatic cholestasis Primary liver malignancy—other malignancy Primary sclerosing cholangitis TPN/Short gut Total—primary transplant Retransplant Total Liver Transplants

20 3 232 12 20 4 80 3 47 5

3.6 0.5 41.3 2.1 3.6 0.7 14.2 0.5 8.4 0.9

20 4

3.6 0.7

7 4

1.2 0.7

4

0.7

36

6.4

21 2 9

3.7 0.4 1.6

8

1.4

16 5 562 68 630

2.8 0.9 100

associated with this syndrome. Hepatocellular carcinoma (HCC) has also been reported in Alagille disease.7 Experience using external biliary diversion or internal ileal bypass accompanied by ursodeoxycholic acid therapy has demonstrated a significant decrease in both pruritus and complications of hypercholesterolemia.8 A recent report summarizing the biliary diversion experience from a multi-institutional consortium indicates that bypass relieves pruritus.9 New treatment strategies have been developed addressing pruritus and hold promise in mitigating the need for LT for this QOL issue.10 

Metabolic Disease An important indication for LT in older children is hepaticbased metabolic disease. In these patients, LT is not only lifesaving but also accomplishes phenotypic and functional cure. A review of these diseases and their mode of presentation is given in Box 45.1 and Table 45.2. Hepatic replacement to correct the metabolic defect should be considered before other organ systems are affected and before complications develop that would preclude transplantation, such as in patients with tyrosinemia, in whom there is a high risk of HCC.11 Although results of transplantation are excellent in the metabolic disease subgroup, replacement of the entire liver in order to correct single enzyme deficiencies is an inefficient but presently necessary procedure. Ongoing research centers around hepatocyte transplantation and gene therapy but to date has not demonstrated durable efficacy.12 Patients with primarily extrahepatic manifestations of their disease, such as cystic fibrosis, are occasionally helped

Box 45.1  Indications for Transplantation for Metabolic Disease in Children Wilson disease α1-Antitrypsin deficiency Crigler–Najjar syndrome (type I) Tyrosinemia Cystic fibrosis Glycogen storage disease type IV Branched-chain amino acid catabolism disorders Hemophilia A Protoporphyria Homozygous hypercholesterolemia Urea cycle enzyme deficiencies Primary hyperoxaluria Iron storage disease Reprinted from Balistreri WF, Ohi R, Todani T, et al. Hepatobiliary, Pancreatic and Splenic Disease in Children: Medical and Surgical Management. Amsterdam: Elsevier Science; 1997. p. 395–399.

by LT, although their prognosis is most often determined by their primary illness.13 

Fulminant Hepatic Failure Patients with fulminant hepatic failure without recognized antecedent liver disease are a challenging cohort of patients. Rapid clinical deterioration frequently makes establishment of a defined cause impossible before there is an urgent need for transplantation. In a multi-institutional manner, the pediatric acute liver failure (ALF) consortium established in 2003 has improved the diagnostic approaches and has better characterized the disease process.14,15 Acute liver failure of undefined etiology followed by drug toxicity and toxin exposure are the most common causes of ALF. Previously unrecognized metabolic disease also must be considered. An immune-based defect has been recognized as a cause of fulminant liver failure.16 This population needs to be identified as these children may require a combination of bone marrow and LT to achieve long-term survival. When acceptable clinical and metabolic stability make liver biopsy safe, diagnostic information allowing directed treatment of the primary liver disease is helpful. The presence of ongoing coagulopathy often dictates the need for an open approach to biopsy. The prognosis of patients with fulminant liver failure is difficult to predict, and neurologic outcome is potentially suboptimal. Use of intracranial pressure (ICP) monitoring in patients with progressive encephalopathy has allowed early recognition and treatment for increased ICP. Monitoring should be considered for patients with advancing grade III encephalopathy and in all patients with grade IV encephalopathy. Intracranial monitoring is continued intraoperatively and for 24–48 hours after OLT, because significant increases in ICP can develop during these periods. Failure to maintain a cerebral perfusion pressure greater than 50 mmHg and an ICP less than 20 mmHg has been associated with very poor neurologic outcomes.17 Efforts to identify and perform LT in children before this deterioration occurs are of utmost importance. ICP monitoring has risks, and intracranial bleeding can occur.18 When candidates are identified before they develop irreversible neurologic abnormalities, the results of transplantation are dramatic. 

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711

Table 45.2  Classification of Inherited Metabolic Disorders According to Clinical Modes of Presentation Cirrhosis

Liver Tumor

α1-Antitrypsin deficiency Wilson disease Hemochromatosis Byler disease Cystic fibrosis Tyrosinemia GSD type IV FHD EPP

Tyrosinemia GSD type I Galactosemia FHD Hemochromatosis α1-Antitrypsin deficiency

Life-Threatening Progressive Liver Disease

Failure of Secondary Organ, Normal Liver

Urea cycle defect Protein C deficiency Crigler–Najjar syndrome type 1 Niemann–Pick disease Hemochromatosis Tyrosinemia BCAA

Type 1 hyperoxalosis Hypercholesterolemia

Reprinted from Balistreri WF, Ohi R, Todani T, et al. Hepatobiliary, Pancreatic and Splenic Disease in Children: Medical and Surgical Management. Amsterdam: Elsevier Science; 1997. p. 395–399. BCAA, branched-chain amino acid catabolism disorders; EPP, erythropoietic protoporphyria; FHD, fumaryl hydrolase deficiency; GSD, glycogen storage disease.

Liver Tumors Transplantation for hepatoblastoma is recommended for individuals who, after the administration of several cycles of chemotherapy, have a neoplasm confined to the liver that is unresectable.19–21 Children who had prior isolated metastasis that disappeared while undergoing preoperative chemotherapy are also transplant candidates.22 A favorable response to pretransplant chemotherapy suggests a more favorable longterm outcome.23 In the recently completed Children’s Oncology Group (COG) trial AHEP 0731, early referral for transplant evaluation was evaluated for children who presented with large lesions that appeared unresectable and was found to be an effective strategy that appears to have improved survival.20 Transplantation for HCC is complicated by less successful chemotherapy options and frequent extrahepatic involvement. The reported 2-year survival rates are only 20–30%.24 Most deaths are due to recurrent HCC within the allograft or to extrahepatic tumor involvement. When primary HCC is discovered incidentally within a cirrhotic explant, the overall prognosis is unaffected by the tumor.25 Recent attention has focused on whether adult HCC Milan criteria for transplantation apply to the pediatric HCC population.26 In the soon-to-open Pediatric Hepatic International Tumor Trial, there are two treatment arms for the pediatric patient with HCC with transplantation as a part of the treatment strategy. From this study, it is hoped that a better understanding of pediatric HCC will be established and better treatment strategies will result. Vascular tumors represent a group of patients with diffuse pathology who can benefit from transplantation. Children with progressive, intractable congestive heart failure, even when caused by non-neoplastic arteriovenous malformations or diffuse hemangiomas, offer a unique opportunity for complete removal of the vascular malformation and correction of congestive heart failure.27 Transplantation in these instances in our experience offers significantly better long-term survival compared with embolization or hepatic artery occlusion, which can precipitate sudden and widespread hepatic necrosis. Pretransplant biopsy is essential in large or complex lesions to exclude angiosarcoma. 

CONTRAINDICATIONS Contraindications to LT include extrahepatic unresectable malignancy, malignancy metastatic to the liver, progressive

terminal nonhepatic disease, uncontrolled systemic sepsis, and irreversible neurologic injury. Relative contraindications to LT that need to be individually evaluated include advanced or partially treated systemic infection, advanced hepatic encephalopathy (grade IV), severe psychosocial difficulties, portal venous thrombosis extending throughout the mesenteric venous system, and serology positive for human immunodeficiency virus. 

DONOR CONSIDERATIONS Donor Options The single factor that continues to limit the availability of LT is the supply of donor organs. In the United States, the number of patients awaiting LT is typically between 14,000 and 15,000 patients per year.28 Typically, there are 8–10,000 deceased donors.29 As a consequence, the waiting time to transplant for all age groups is significant with persistent waiting list mortality.28 Young children and infants continue to have the highest waiting list mortality (Fig. 45.1). The limited supply of available donor organs has driven the advancement of many innovative liver transplant surgical procedures. The development of reduced-size LT allowed significant expansion of the donor pool for infants and small children. This not only has improved the availability of donor organs but has also allowed access to donors with improved stability and organ function. Evolution of these operative techniques has resulted in the development of both split-LT and live donor (LD) transplantation. In the hands of experienced transplant teams, these procedures all have success similar to that with whole organ transplantation.30  Organ Allocation In 1998 the “final rule” established by the Health Resources and Service Administration mandated the formation of a system for candidate stratification based on a continuous severity score reflecting 90-day waiting list mortality (i.e., outcome).31 The system for pediatric patients, the Pediatric End-Stage Liver Disease (PELD) score, was created using an analysis of the prospective registry of children listed for transplantation by the consortium Studies of Pediatric Liver Transplantation (SPLIT).32 The parameters selected included total bilirubin, international normalized ratio (INR), albumin, age

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Deaths per 100 waitlist years

50 40 2 standard deviations below mean, score = 1; growth 35) (Fig. 45.2).33 

Donor Selection Assessment of donor organ suitability is undertaken by evaluating clinical information, static biochemical tests, and dynamic tests of hepatocellular function. Static biochemical tests identify preexisting functional abnormalities or organ trauma, but do not serve as good benchmarks to differentiate among acceptable and poor donor allografts. Donor liver biopsy is helpful in questionable cases to identify preexisting liver disease or donor liver steatosis. The shortage of donor organs has led to expanded efforts to use individuals of advanced age and marginal stability, termed extended criteria donors (ECDs).34 The donor risk index (DRI) is used as a guide that quantifies relative risk of graft failure.35,36 In the future, organ allocation may be based on maximal life-years gained, an approach being utilized in kidney allocation.37 Anatomic replacement of the native liver in the orthotopic position requires selection or surgical preparation of the donor liver to fill but not exceed available space in the recipient. When using full-sized allografts, a donor weight range from 50–125% of the recipient weight is usually appropriate, taking into consideration body habitus and

factors that would increase the abdominal size in the recipient, such as ascites and hepatosplenomegaly. The right lobe graft, using segments 5–8, and the right trisegmentectomy graft, using segments 4–8, can be accommodated when the weight difference is no greater than 2:1 between the donor and recipient (D/R). The thickness of the right lobe makes this allograft of limited usefulness in small recipients. The left lobe, using segments 1–4, is applicable with a D/R ratio from 2.5:1–5:1, and a left lateral segment (segments 2 and 3) can be used with up to a 10:1 D/R weight difference. Although whole organ allografts are preferred, technical variant grafts are commonly employed. Preoperative preparation of variant liver allografts is based on the anatomy of the hepatic vasculature and bile ducts. In the past, reducedsize grafts were common, but because of the donor shortage, split-liver transplantation has become widespread using either an ex situ or in situ approach. The result is two transplantable grafts. The ex situ split procedure divides the right lobe allograft (segments 5–8) from the left lateral segment (LLS) allograft (segments 2 and 3) after the whole donor organ has been procured. The successful experience with in situ division of the LD LLS is a basis for the in situ split procedure. Two variations of the procedure are utilized depending on the needs of the recipients, a right-left lobe split or a right trisegmentectomy–LLS split. For the right trisegmentectomy–LLS split, the LLS is prepared similar to a living-related donor graft. The viability of segment 4 can be examined at the time of the division and is usually incorporated with the right lobe graft to increase the cellular mass of the allograft. For a leftright lobe graft, the parenchymal resection follows the anatomic lobar plane through the gallbladder fossa to the vena cava. A crush and tie technique is preferred to achieve good closure of the vascular and biliary structures. The bile duct, portal vein, and hepatic artery are divided at the right or left confluence. The vena cava is left incorporated with the allograft in both right and left lobe preparation. Vena caval reduction by posterior caval wall resection and closure is occasionally necessary. Resection of the inferior protruding portion of the caudate lobe is necessary during left lobe preparation to reduce the likelihood of arterial angulation, which can result in arterial thrombosis. This also facilitates shortening of the inferior vena cava to fit in a small recipient. With LLS allografts, the parenchymal dissection follows the right margin of the falciform ligament with preservation of the left hilar structures. Direct implantation of the left

45 • Solid Organ Transplantation in Children

713

Patient survival (%)

100 95 90 85 80

0

1

2

3

4

5

6

7

8

9

10

11

12

PELD –11 to 6 PELD 7 to 16 PELD 16 to 28 PELD >28 Status 1

Months post OLTx Fig. 45.2  Pediatric end-stage liver disease score predictive of survival after transplantation. (Redrawn from Barshes NR, Lee TC, Udell IW, et al. The PELD model as a predictor of survival benefit and of post transplant survival in pediatric liver transplant recipients. Liver Transpl 2006;12:475–480.)

800

Transplants

600 Deceased donor Living donor All

400 200 0 2004

2006

2008

2010 Year

2012

2014

2016

Fig. 45.3  Pediatric liver transplant rates by donor type. (From OPTN/SRTR 2016 Annual Data report: Liver.)

hepatic vein into the combined orifice of the right and middle/left hepatic veins in the recipient vena cava is preferred. Further reduction of the LLS graft to a monosegmental graft may be necessary in very small recipients. Resection of the distal LLS is technically easier than an anatomic segment II/III division. Because this procedure adds considerably to the donor procurement time, and the necessary skill of the donor team, it is more demanding and occasionally difficult to successfully orchestrate. This technique is, however, despite these considerations, the preferred method for splitliver donor preparation. The benefits of split-liver transplantation are best achieved when ideal donors are selected. Strict restrictions on age, vasopressor administration, predonation hepatic function, and limited donor hospitalization have been used to select optimal donor candidates. When these donors are selected, the results from both in situ and ex situ techniques are similar, with both techniques now having patient survival for both allografts of 90–93% and graft survival rates of 86–89%.38 The use of LDs has become an integral component of most pediatric transplant programs armamentarium with excellent donor safety profiles (Fig. 45.3).39,40 One of the critical elements of LD transplantation is the proper selection of a donor, usually a parent or relative. Careful attention to proper LD consent is important. Parental concerns to help their ill child make true informed consent a challenge. A dedicated donor advocate not directly associated

with the transplant team should assist with this process. Independent medical assessment of the donor is essential. UNOS has established clear criteria for this process.41 After a satisfactory medical and psychological examination by a physician not directly involved with the transplant program, computed tomography (CT) scanning is used to measure the volume of the potential donor segment to ensure it will meet the metabolic needs, but not exceed the space available in the recipient. CT angiography is undertaken to assess the hepatic arterial anatomy, thereby excluding potential donors with multiple arteries to segments 2 and 3 and facilitating minimal hilar vascular dissection at the time of LT. In most pediatric cases, the LLS donated from an adult is used as the graft. In situ dissection of the LLS, preserving the donor vascular integrity until the parenchymal division is completed, is undertaken. At the time of harvest, the left hepatic vein is divided from the vena cava and the left branch of the portal vein and hepatic artery are removed with the allograft. Vascular continuity of the hepatic arterial branches to segment 4 is maintained, if possible. Increased experience has been gained using the right lobe as an LD allograft for larger recipients such as adolescents and adults.42 This more extensive operation has proven to be a challenge to the donor and recipient alike, with complication and mortality rates significantly exceeding those of left lateral segmentectomy. The number of right lobe LD recipients now greatly exceeds the number of children

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receiving LD grafts.28 However, several publicized donor deaths have in the United States tempered the enthusiasm and growth of right lobe donation. The selection of a donor segment with an appropriate parenchymal mass for adequate function is critical to success. However, the minimal mass necessary for recovery is not yet established. Any calculation must take into account loss of function following preservation damage, acute rejection, and technical problems. Estimates of the ratio of donor graft to recipient body weight (GRWR) may prove to be a more accurate predictor of adequate graft volume.43 When the GRWR is less than 0.7%, overall allograft and patient survival suffers. In extreme cases in which small-for-size grafts are used, excessive portal flow can lead to hemorrhagic necrosis of the graft. Large-for-size allografts (GRWR >5.0%) have a less deleterious effect.44 A review of these donor anatomic options is shown in Figure 45.4. 

exposure and meticulous attention to the delivery of all normal childhood immunizations, particularly the live-virus vaccines, are imperative before LT, if time allows. Additionally, patients receive a one-time inoculation with pneumococcal vaccine and appropriate administration of hepatitis B vaccine. Preoperative assessment of cardiopulmonary reserve and hepatic vascular anatomy is also important. 

THE TRANSPLANT PROCEDURE The transplant procedure is carried out through a bilateral subcostal incision with midline extension. Meticulous ligation of portosystemic collaterals and vascularized adhesions is necessary to avoid slow but relentless hemorrhage. Dissection of the hepatic hilum, with division of the hepatic artery and portal vein above their bifurcation, allows maximal recipient vessel length to be achieved. The bile duct, when present, is divided high in the hilum to preserve the length and vasculature of the distal duct in case it is needed for primary reconstruction in older recipients. Preservation of the Roux-en-Y limb in BA patients who have undergone Kasai portoenterostomy simplifies later biliary reconstruction. Complete mobilization of the liver, with dissection of the suprahepatic vena cava to the diaphragm and the

PREOPERATIVE PREPARATION Efforts to correct abnormalities noted during candidate evaluation decrease both the operative risk and postoperative complications. Complications of portal hypertension and malnutrition are treated. Assessment of prior viral

IVC

IVC

2

7 8

4

BD

6 5 HA

3

BD

PV

PV

HA

Left lobe

Right lobe

LHV

2

IVC

2 2

7

7

8

PV

HA

Left lateral segment

1

8

3

4

LHV

3

4 6

3

BD

IVC

6

5

5

2 3

BD

In situ split liver

PV

PV

HA

Living donor IVC

Ex vivo split liver

LHV

7 8 6 5

2 BD HA PV

4

3

BD PV

HA

Discard Fig. 45.4  Anatomic donor options available through surgical reduction. The numbers correlate to the segmental hepatic anatomy as defined by Couinaud. BD, bile duct; HA, hepatic artery; IVC, inferior vena cava; LHV, left hepatic vein; PV, portal vein.

45 • Solid Organ Transplantation in Children

infrahepatic vena cava to the renal veins, completes the hepatectomy. In children with serious vascular instability who cannot tolerate caval occlusion, or when an LLS graft is being used, “piggy-back” implantation is necessary. In this procedure, the recipient vena cava is left intact and partial caval occlusion allows end-to-side implantation of a combined donor hepatic vein patch. Access to the infrarenal aorta to implant the celiac axis of the donor liver or iliac artery vascular conduits, provided by mobilizing the right colon and duodenum, is our preference for arterial reconstruction in complex allograft recipients. Control of hemorrhage is essential during the recipient hepatectomy, requiring meticulous technique. Coagulation factor assays (V, VII, VIII, fibrinogen, platelets, prothrombin time, partial thromboplastin time) allow specific blood product supplementation to improve clotting function. Use of venovenous bypass is reserved for recipients >25 kg who demonstrate hemodynamic instability at the time of venous interruption. In standard LT, the suprahepatic vena cava is prepared by suture ligation of any large phrenic orifices and creating one caval lumen from the confluence of the inferior vena cava and hepatic vein orifices. The donor liver is implanted using conventional vascular techniques and monofilament suture for the vascular anastomosis. In small recipients, interrupted suture techniques, monofilament dissolving suture material, and a “growth factor” knot has been used to allow for vessel growth. When LLS grafts are used, the left hepatic vein orifice is anastomosed directly to the anterolateral surface of the infradiaphragmatic inferior vena cava using the combined right middle hepatic vein orifices. The LLS allograft is later fixed when necessary to the undersurface of the diaphragm to prevent torsion and venous obstruction of this anastomosis. Similar fixation is not necessary with right or left lobe allografts or whole organ transplants. Before completing the vena caval anastomosis, the hyperkalemic preservation solution is flushed from the graft using 500–1000 mL of hypothermic normokalemic intravenous (IV) solutions. In reduced-size allografts and small recipients in whom we prefer direct aortic vascular inflow reconstruction, the hepatic arterial anastomosis is completed before the portal vein anastomosis to improve visibility of the infrarenal aorta without placing traction on the portal vein anastomosis. We prefer to complete all anastomoses using vascular isolation before organ reperfusion, although some transplant teams reperfuse after the venous reconstruction is complete. Before reestablishing circulation to the allograft, anesthetic adjustments must be made to address the large volume of blood needed to refill the liver, as well as hypothermic solutions released upon reperfusion. Inotropic support using dopamine (5–10 mcg/kg/min) also may be started. Calcium and sodium bicarbonate are administered to combat the effects of hyperkalemia from any remaining preservation solution or from systemic acidosis due to aortic and vena caval occlusion. Sufficient blood volume expansion, administered as packed red blood cells to raise the central venous pressure (CVP) to 12–15 cmH2O and the hematocrit to 40%, minimizes the development of hypotension on unclamping and prevents dilutional anemia. Cooperative

715

communication between the surgical and anesthesia teams facilitates a smooth sequential reestablishment of vena caval, portal venous, and then arterial recirculation to the allograft. Biliary tract reconstruction in patients with BA or in those weighing less than 25 kg is achieved through an end-to-side choledochojejunostomy using interrupted dissolving monofilament sutures. A multifenestrated Silastic internal biliary stent is placed before completing the anastomosis (Fig. 45.5). In most cases, the prior Roux-en-Y limb can be used, with a 30- to 35-cm length being preferred. Primary bile duct reconstruction without stenting is used in older patients with whole organ allografts. When closing the abdomen, increased intra-abdominal pressure should be avoided. In many cases, the abdominal fascia is not closed and mobilized skin flaps and running monofilament skin closure are used. Formal musculofascial abdominal closure can be completed within 5–7 days after the transplant procedure. 

IMMUNOSUPPRESSIVE MANAGEMENT Most centers use an immunosuppressive protocol based on the administration of multiple complementary medications. All use corticosteroids, and most use tacrolimus. Additional antimetabolites (azathioprine, mycophenolate) are added when more intensive treatment is needed. The use of polyclonal or monoclonal induction therapy has become less frequent as the combination is extremely potent and increases the risk of immunosuppressive complications (see later). A sample immunosuppression protocol is given in Table 45.3. 

POSTOPERATIVE COMPLICATIONS Most postoperative complications manifest with increasing hepatocellular enzyme levels, cholestasis, and on occasion fever, lethargy, and anorexia. Therapy directed at the specific causes of the allograft dysfunction is essential. Empiric therapy for presumed complications is fraught with misdiagnoses, morbidity, and mortality. A flow diagram outlining this evaluation is shown in Figure 45.6.

Multifenestrated internal stent

Roux-en-Y

Roux-en-Y

Fig. 45.5  Bile duct reconstruction is shown using the common hepatic duct in whole organ transplants (left) and segmental hepatic ducts into a Roux-en-Y intestinal limb for reduced-sized liver transplants (right). An internal multifenestrated stent is used in both situations. (From Ryckman F. Liver transplantation. In: Ziegler MM, Azizkhan RG, Weber T, editors. Operative Pediatric Surgery. New York: McGraw-Hill; 2003. p. 1275.)

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Primary Nonfunction Primary nonfunction (PNF) of the hepatic allograft implies the absence of metabolic and synthetic activity following LT. Complete nonfunction requires immediate retransplantation before irreversible coagulopathy and cerebral edema occur. Lesser degrees of allograft dysfunction occur more frequently and are associated with several donor, recipient, and operative factors (Box 45.3). The status of the donor liver contributes significantly to the potential for Table 45.3  Immunosuppression Protocol Utilized for Liver Transplantation Day/Week

Methylprednisolone Tacrolimus (mg/kg/day) (mg/kg/day)

Intraoperative Day 1 Day 2 Day 3 Day 4 Day 5 Day 6 Day 7 Week 2

15 10 8 6 4 3 2 1 0.9

Week 3 Week 4 Week 5 Week 6 Week 7 Week 8 Week 9 Week 10 Week 11 Week 12 >1 year

0.8 0.7 0.6 0.5 0.4 0.3 0.2 0.1 0.1 D/C

0 0.3 0.3 0.3 0.3 0.3 0.3 0.3 Adjust as needed

Tacrolimus Target Level

12–18

PNF. Ischemic injury secondary to anemia, hypotension, hypoxia, or trauma is often difficult to ascertain in the history of multisystem trauma victims. Donor liver steatosis has been recognized as a factor contributing to severe dysfunction or nonfunction in the donor liver.45,46 Macrovesicular steatosis on donor liver biopsy is somewhat more common in adult than pediatric donors and, when severe, is recognized grossly by the enlarged yellow, greasy consistency of the donor liver. The risk of PNF increases as the degree of fatty infiltration increases.45 Histologic findings are classified as mild if less than 30% of the hepatocytes have fatty infiltration, moderate if 30–60% are involved, and severe if more than 60% of the hepatocytes have fatty infiltration. The use of ABO-incompatible donors has been controversial. Allograft and patient survival rates in adult recipients have not been comparable to those achieved using ABOidentical or compatible donors.47 However, pediatric recipients of ABO-incompatible allografts have achieved survival rates equivalent to those using ABO-compatible and ABOidentical donors with either cadaveric donors (CDs) or LDs.47,48 

Vascular Thrombosis Hepatic artery thrombosis (HAT) occurs in children three to four times more frequently than in adults and occurs most often within the first 30 days following transplantation.49,50 Factors influencing the development of HAT are listed in Box 45.4. HAT presents with a variable clinical picture that may include fulminant allograft failure, biliary disruption or obstruction, or systemic sepsis. Doppler ultrasound (US) has been accurate and is used as the primary screening modality to assess vascular flow following transplantation or whenever complications arise. Acute HAT with allograft failure most often requires immediate retransplantation.

12–18

8–14

6–12 3–7

D/C, discontinue.

Hepatic dysfunction Ultrasound with Doppler Vascular Angiogram or Operative exploration

Thrombectomy Anticoagulate

Hepatocellular

Biliary

Liver biopsy Infection

Rejection Steroid recycle Rescue medication OKT-3 ATG Mycophenolate

Leak Drug reaction Preservation Recurrent injury

Obstruction

Computed tomography +/– Percutaneous transhepatic cholangiography or Endoscopic retrograde cholangiopancreatography Transhepatic stent or Operative repair

Retransplant Fig. 45.6  Schematic flow diagram for management of postoperative liver allograft dysfunction. ATG, antithymocyte globulin; OKT-3, monoclonal antibody.

45 • Solid Organ Transplantation in Children

Successful thrombectomy and allograft salvage is possible if reconstruction is undertaken before allograft necrosis.50 Biliary complications are particularly common following HAT. Ischemic injury to the biliary tree or anastomosis can result in intraparenchymal biloma formation or cholestasis. The development of septicemia or multifocal abscesses in sites of ischemic necrosis secondary to Gram-negative enteric bacteria, Enterococcus, anaerobic bacteria, or fungal infection also can occur. Antibiotic therapy directed toward these organisms, along with operative or percutaneous drainage, is indicated when specific abscesses are identified. Drainage and biliary stenting may control bile leakage and infection until retransplantation is undertaken. Late HAT can be asymptomatic or present with slowly progressive bile duct stenosis. Rarely, allograft necrosis occurs. Arterial collaterals from the Roux-en-Y limb can provide a source of revascularization through hilar collaterals. These collateral channels develop during the first postoperative months, often making late thrombosis a clinically

Box 45.3  Factors Related to Primary Nonfunction Donor Factors Preexisting disease or injury to donor, anemia, hypoxia, hypotension before organ harvest Donor organ steatosis (>60% macrovesicular fat)  Transplant Factors Prolonged cold ischemic storage (>8–12 hours) Prolonged warm ischemic time at implantation Complex vascular anastomosis requiring surgical revision Significant size discrepancy between donor and recipient  Recipient Factors Postreperfusion hypotension Vascular thrombosis Immunologic factors ABO incompatible, positive cross-match.

Box 45.4  Factors Affecting Vascular Thrombosis Donor/Recipient Age/Weight Allograft Type Whole organ > reduced size Living donor ≥ reduced size  Anastomotic Anatomy Primary hepatic artery > direct aortic artery  Allograft Edema–Increased Vascular Resistance Ischemic injury secondary to prolonged preservation; prolonged implantation Rejection Fluid overload  Recipient Hypotension and/or Hypercoagulability Administration of coagulation factors, fresh frozen plasma Procoagulant factor deficiencies

717

silent event. Conversely, disruption of this collateral supply during operative reconstruction of the central bile ducts in patients who later develop HAT can precipitate hepatic ischemia and parenchymal necrosis. When HAT is asymptomatic, careful observation alone is reasonable. Prevention of HAT requires meticulous arterial reconstruction at the time of transplantation. Anatomic reconstruction is preferred in whole organ allografts; direct implantation of the celiac axis into the infrarenal aorta is recommended for all reduced-size liver allografts. All complex vascular reconstructions of the donor hepatic artery should be undertaken ex  vivo whenever possible using microsurgical techniques before transplantation. When vascular grafts are required, they should also be implanted onto the infrarenal aorta.51 Systemic anticoagulation is not routinely used by our group, but aspirin (20–81 mg/day depending on patient weight) is administered to all children for 100 days. Portal vein thrombosis (PVT) is uncommon in whole organ allografts unless prior portosystemic shunting has altered the flow within the splanchnic vascular bed or unless severe portal vein stenosis in the recipient has impaired flow to the allograft. Preexisting PVT in the recipient can be overcome by thrombectomy, portal vein replacement, or extra-anatomic venous bypass. In BA recipients, portal vein hypoplasia is best corrected by anastomosis of the donor portal vein to the confluence of the recipient’s splenic and superior mesenteric veins. When there is inadequate portal vein length on the donor organ, iliac vein interposition grafts are used. Early PVT following LT requires immediate anastomotic revision and thrombectomy. Discrepancies in venous size imposed by reduced-size allografts can be modified to allow anastomotic construction.52 Deficiencies of anticoagulant proteins, such as proteins C and S, and antithrombin III deficiency in the recipient also must be excluded as a contributing cause for vascular thrombosis.53 Failure to recognize PVT can lead to either allograft demise or, on a more chronic basis, to significant portal hypertension, leading to varices with resulting gastrointestinal (GI) bleeding. 

Biliary Complications Complications related to biliary tract reconstruction occur in approximately 10% of pediatric LT recipients. Their spectrum and treatment is determined by the status of the hepatic artery and the type of allograft used. Although whole and reduced-size allografts have an equivalent risk of biliary tract complications, the spectrum of complications differs.54 Primary bile duct reconstruction is the preferred biliary tract reconstruction in adults, but it is less commonly used in children. It has the advantage of preserving the sphincter of Oddi, decreasing the incidence of enteric reflux and subsequent cholangitis, and not requiring an intestinal anastomosis. Experience using primary choledocho-choledochostomy without a T-tube has been favorable.55 Late complications following any type of primary ductal reconstruction include anastomotic stricture, biliary sludge formation, and recurrent cholangitis. Endoscopic dilation and internal stenting of anastomotic strictures has been successful in early postoperative cases. Roux-en-Y choledochojejunostomy is the preferred treatment for recurrent stenosis or postoperative leak.

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Roux-en-Y choledochojejunostomy is the reconstruction of choice in small children and is required in all BA recipients. Recurrent cholangitis, a theoretical risk, suggests an anastomotic narrowing, an intrahepatic biliary stricture, or a small bowel obstruction within the Roux limb or distal to the Roux-en-Y anastomosis. In the absence of these complications, cholangitis is uncommon. Reconstruction of the bile ducts in patients with reducedsize allografts is more challenging. Division of the bile duct in close proximity to the cut-surface margin of the allograft, with careful preservation of the biliary duct collateral circulation, decreases but does not eliminate a ductal stricture secondary to ischemia. In our early experience, in 14% of patients with left lobe reduced-size allografts, a short segmental stricture developed requiring anastomotic revision (Fig. 45.7).56 Operative revision of the biliary anastomosis and reimplantation of the bile ducts into the Roux-en-Y is necessary. Percutaneous transhepatic cholangiography is essential to define the intrahepatic ductal anatomy before operative revision, and temporary catheter decompression of the obstructed bile ducts promotes treatment of cholangitis and allows elective reconstruction. Operative reconstruction is accompanied by transhepatic passage of exteriorized multifenestrated biliary ductal stents, which remain in place until reconstructive success is documented. Late stenosis is unlikely. Dissection away from the vasobiliary sheath in the donor has significantly decreased the incidence of this complication. Biliary complications have been seen with an increased frequency following LD transplant in children. The LLS 2 and 3 bile ducts are frequently separate at the plane of parenchymal division. The need for individual drainage of these small biliary ducts makes the development of late anastomotic stenosis more frequent. Individual segmental strictures may not lead to jaundice in the recipient, but rather are identified by elevated γ-glutamyl transferase (GGT) or through US surveillance. Reoperation after ductal dilatation from the stricture allows for easier reconstruction due to the increased caliber of the segmental bile duct. 

Fig. 45.7  Segmental bile duct stricture at the junction of the left lateral and left medial segmental bile ducts in a left-lobe reduced-size allograft. Solid arrow, bile duct stricture; open arrow, Roux-en-Y loop and bile duct anastomosis. (From Ryckman FC. Liver transplantation in children. In: Suchy FJ, editor. Liver Disease in Children. St. Louis: CV Mosby; 1994. p. 941.)

Acute Cellular Rejection Allograft rejection is characterized by the histologic triad of endothelialitis, portal triad lymphocyte infiltration with bile duct injury, and hepatic parenchymal cell damage.57 Allograft biopsy is essential to establish the diagnosis before treatment. The rapidity of the rejection process and its response to therapy dictates the intensity and duration of antirejection treatment. Acute rejection occurs in approximately two-thirds of patients following LT. The primary treatment is a short course of high-dose steroids. Bolus doses administered over several days with a rapid taper to baseline therapy is successful in 80% of cases.58 In our experience, when refractory or recurrent rejection occurs, antilymphocyte therapy using thymoglobulin is successful in nearly all patients.  Chronic Rejection Chronic rejection occurs in 5–10% of transplanted patients. Its incidence appears to be decreasing in all transplant groups, perhaps related to better overall immunosuppressive strategies. There is some suggestion that the use of tacrolimus-based immunosuppression is a key element in this apparent decrease.59 Risk factors for its development are many, and no factor predicts the outcome of treatment. The chronic rejection rate is significantly lower in recipients of LD grafts compared with cadaveric grafts.59 In addition, the number of acute rejection episodes, transplantation for autoimmune disease, occurrence of post-transplant lymphoproliferative disease (PTLD), and cytomegalovirus (CMV) infection are all significant risk factors for chronic rejection. The primary clinical manifestation is a progressive increase in biliary ductal enzymes (alkaline phosphatase, GGT) and progressive cholestasis. Chronic rejection can be initially asymptomatic or may follow unsuccessful treatment for acute rejection. It can occur within weeks of transplantation or much later. Recent studies have focused on donor-specific antibodymediated abnormalities that may be the pathophysiologic basis for chronic rejection.60 The immunologic nature of this process is emphasized by the primary target role of the biliary and vascular endothelium, the only tissues in the liver that express class II antigen. Other interdependent cofactors such as CMV infection, human leukocyte antigen (HLA) mismatching, positive B-cell cross-matching, and differing racial demographics of the donor to recipient have all failed to show consistent correlation with the development of chronic rejection.  Renal Insufficiency The long-term success of LT has been related to the effective immunosuppression with calcineurin inhibitors (CNIs) such as cyclosporine and tacrolimus. However, nephrotoxicity associated with their long-term use has become a major problem that can affect up to 70% of all nonrenal transplant recipients. Renal insufficiency can manifest in many ways following LT and CNI administration. When it occurs during the initial post-transplant weeks, it is most often related to transiently excessive CNI blood levels and is reversible with appropriate dose correction. Impaired glomerular filtration rate (GFR) seen in pediatric recipients with stable graft function represents a more serious problem. Up to 20% may have a drop in their GFR

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to below 50 mL/min/1.73 m2, and 5% may progress to endstage renal disease (ESRD). Adult studies have shown a progressive increase in chronic renal failure from 0.9% at year 1 to 8.6% at year 13 after OLT.61 Similarly, ESRD rose from 1.6% at year 1 to 9.5% at year 13 after LT, yielding a total incidence of renal dysfunction of 18%. The presence of an elevated serum creatinine pre-LT at 1-year post-transplant and the presence of hepatorenal syndrome prior to transplant were all identified risk factors.61,62 Cyclosporine and tacrolimus appear to be similar in risk. In a review from our program, in children who were more than 3 years post-LT, we found that 32% had a GFR less than 70 mL/min/1.73 m2.63 The factors primarily related to lower GFR were the presence of an elevated creatinine at 1 year after LT and the length of time following transplantation. Our data supported the concept of a continued decline in renal function following LT.64 Considering the expected long survival for children undergoing LT, the possibility of progressive asymptomatic renal insufficiency leading to severe kidney disease poses a significant challenge. Efforts to reverse ongoing renal insufficiency using protocols that include instituting non-nephrotoxic agents, such as mycophenolate mofetil (MMF) while decreasing the CNI dose, have shown limited success in improving GFR while protecting against the risks of acute rejection at the time of immunosuppressive drug conversion.65 Sirolimus also has been integrated into treatment strategies to preserve renal function.66 Whether these efforts will prevent the later development of renal insufficiency is yet unknown.67 Efforts to completely eliminate CNI use have been complicated by acute or ductopenic rejection. Protocolized immunosuppressive regimens, including ours, emphasize sequential reduction in goal serum CNI levels to allow for concurrent dose reduction.68 By doing so, transplant programs attempt to decrease the incidence of CNI-induced nephropathy, but it is likely not possible to eliminate this complication.69,70 Once established, chronic renal failure does not appear to resolve with CNI dose adjustment. Although CNI toxicity is now well appreciated, the association of both hepatic and renal disease in many metabolic diseases of childhood may also contribute to the GFR abnormalities seen after LT. 

Infection Infectious complications are the most common source of morbidity and mortality following LT. Multiple organism infection is common, as are concurrent infections by different agents. Bacterial infections occur in the immediate post-transplant period and are most often caused by Gram-negative enteric organisms, Enterococcus, or Staphylococcus species. Intraabdominal abscesses or infected collections of serum along the cut surface of the reduced-size allograft are best addressed with extraperitoneal, abdominal, or percutaneous drainage. Intrahepatic abscesses suggest hepatic artery stenosis or thrombosis, and treatment is directed toward the vascular status of the allograft and associated bile duct abnormalities. Sepsis originating at sites of invasive monitoring lines can be minimized by replacing or removing all intraoperative lines soon after transplantation. Antibacterial prophylaxis is discontinued as soon as possible to prevent the development of resistant organisms.

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Fungal sepsis represents a significant potential problem in the early post-transplant period. Aggressive protocols for pretransplant prophylaxis are based on the concept that fungal infections originate from organisms colonizing the recipient’s GI tract. Fungal infection most often occurs in patients requiring multiple operative procedures and those who have had multiple antibiotic courses. Development of fungemia or urosepsis requires retinal, cardiac, and renal investigation, and antifungal therapy should be promptly initiated. Severe fungal infection has a mortality rate greater than 80%, making early treatment essential. All patients undergoing LT should receive antifungal prophylaxis with fluconazole. The majority of early and severe viral infections are caused by viruses of the Herpesviridae family, including Epstein–Barr virus (EBV), CMV, and herpes simplex virus (HSV). CMV transmission dynamics are well studied and serve as a prototype for herpesvirus transmission in the transplant population. The likelihood that CMV infection will develop is influenced by the preoperative CMV status of the donor and recipient.71 Seronegative recipients receiving seropositive donor organs are at greatest risk, with seropositive donor-to-recipient combinations at the next greatest risk. Use of various immune-based prophylactic protocols including intravenous immunoglobulin G (IVIG) or hyperimmune anti-CMV IgG coupled with acyclovir or ganciclovir/valganciclovir, have all achieved success in decreasing the incidence of symptomatic CMV infection, although seroconversion in seronegative recipients from seropositive donor organs inevitably occurs. EBV infection occurring in the perioperative period represents a significant risk to the pediatric recipient.72 It has a varying presentation including a mononucleosis-like syndrome, hepatitis-simulating rejection, extranodal lymphoproliferative infiltration with bowel perforation, peritonsillar or lymph node enlargement, and encephalopathy. In small children, its primary portal of entry is often the tonsils, making asymptomatic tonsillar hypertrophy a common initial presentation. EBV infection can occur as a primary infection or following reactivation of a past infection. When serologic evidence of active infection exists, an immediate reduction in immunosuppression is indicated. It has become clear that continuous surveillance is necessary as the presentation is often nonspecific and the prognosis is related to early diagnosis. Screening using quantitative polymerase chain reaction (PCR) testing to determine the EBV blood viral load appears to be the best current predictor of risk. However, viral loads have been identified in asymptomatic patients and patients recovering from PTLD, limiting the specificity of this approach. The balance between viral load measured by quantitative PCR and specific cellular immune response, perhaps mediated by CD8 T-cells specific to EBV, may explain this lack of specificity to viral load alone. PTLD, a potentially fatal abnormal proliferation of B-lymphocytes, can occur in any situation in which immunosuppression is used. The importance of PTLD in pediatric LT is a result of the intensity of the immunosuppression required, its lifetime duration, and the absence of prior exposure to EBV infection in 60–80% of pediatric recipients. PTLD is the most common tumor in children following transplantation, representing 50% of all tumors compared with 15% in adults.73 About 80% of cases occur

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within the first 2 years following transplantation. Multiple studies analyzing immunosuppressive therapy and the development of PTLD have shown a progressive increase in its incidence with the increase in total immunosuppressive load, EBV-naïve recipients, and intensity of active viral load. No single immunosuppressive agent has been directly related to PTLD, although high-dose cyclosporine, tacrolimus, polyclonal antilymphocyte sera (MALG, ALG), and monoclonal antibodies (OKT-3) have all been implicated. Unfortunately, prolonged treatment with anti–Tcell agents and the increased duration, intensity, and total immunosuppressive load are the origin of the immunity that creates the background for neoplasia.73 The second pathogenic feature influencing PTLD appears to be EBV infection. Primary or reactivation infections usually precede the recognition of PTLD. Active EBV infection, whether primary or reactivation, involves B-cell proliferation. A simultaneous increase in cytotoxic T-cell activity is the normal host’s mechanism for preventing EBV dissemination. Loss of this natural protection as a result of the administration of T-cell inhibitory immunotherapy allows polyclonal B-cell proliferation to progress following EBV viral replication and release. These EBV proliferating cells express specific viral antigens that represent possible targets for the immune system, thereby explaining the welldescribed regression of PTLD after immunosuppressive tapering. With time, transformation of a small population of cells results in a malignant monoclonal aggressive B-cell lymphoma.73 Most tumors seen in children are large cell lymphomas, 80% being of B-cell origin. Extranodal involvement, uncommon in primary lymphomas, is seen in 70% of PTLD cases. Extranodal sites include liver, 25%; lung, 21%; central nervous system, 21%; intestine, 19%; kidney, 18%; and spleen, 13%.74 Allograft involvement is common and can mimic rejection. T-cell and B-cell immunohistochemical markers of the infiltrating lymphocyte population define the B-cell infiltrate and assist in establishing an early diagnosis. Treatment of PTLD is stratified according to the immunologic cell typing and clinical presentation. Documented PTLD requires an immediate decrease or discontinuation of immunosuppression and institution of anti-EBV therapy. We prefer to use IV ganciclovir for initial antiviral therapy owing to the high incidence of concurrent CMV infection. The development of newer antiviral alternatives such as valganciclovir may offer better long-term options in the future.75 Patients with polyclonal B-cell proliferation frequently show regression with this treatment. If tumor cells express B-cell marker CD 20 on histology, the anti-CD 20 monoclonal antibody rituximab can be administered weekly. Although it is associated in many cases with significant reduction in tumor mass, patients have frequently experienced reversible neutropenia requiring granulocyte colony-stimulating factor (GCSF) and hypogammaglobulinemia requiring supplementation.76 Acute liver rejection has frequently been seen during rituximab treatment. Patients with aggressive monoclonal malignancies have poor survival even with immunosuppressive reduction, acyclovir, and conventional chemotherapy or radiation therapy. These additional treatment modalities often precipitate the development of fatal systemic infection. Efforts to reconstitute the EBV-specific cellular immunity using

partially HLA-matched EBV-specific cytotoxic T-cells may offer improved treatment outcome for advanced cases.77 The future development of anti-EBV vaccine may decrease the present significant risks of this unique complication of pediatric transplantation.78 When treatment is successful, careful follow-up to identify recurrent disease or delayed central nervous system involvement is essential. 

RETRANSPLANTATION The majority of retransplantation procedures in infants and children are done as a result of acute allograft demise caused by HAT or PNF. Acute rejection, chronic rejection, and biliary complications are more uncommon causes.79 Many of these complications are associated with concurrent sepsis, which further complicates reoperation and compromises success. Survival following transplantation is directly related to prompt identification of appropriate patients and acquisition of a suitable organ. When retransplantation is promptly undertaken for early graft failure, patient survival rate, in our experience, is good (80%).79 However, when retransplantation is undertaken for chronic allograft failure, often complicated by multiple organ system insufficiency, the survival rate is lower. 

OUTCOMES FOLLOWING TRANSPLANTATION Although complications following LT are frequent, the overall results are rewarding. Improvements in organ preservation, operative management, immunosuppression, and treatment of postoperative complications have all contributed to the excellent survival rate that is currently seen. Factors influencing the survival of children undergoing transplantation are detailed in Box 45.5. Most successful transplant programs have overall 1-year survival rates of greater than 90%, with greatly decreased risk thereafter (Fig. 45.8). The significant success now achieved following LT cannot overshadow the need for improved management of post-transplant consequences of immunosuppression. The most significant factors contributing to long-term failure of the allograft or patient death in our and other programs is consequences of immunosuppressive medications: late infection, PTLD, and chronic rejection. Our ability to overcome these challenges will determine the lifelong success of LT for our youngest recipients. The overriding objective of LT in children is complete rehabilitation with improved QOL. Factors contributing to successful achievement of this goal include an improved nutritional status with appropriate growth and development, as well as enhanced motor and cognitive skills that allow social reintegration. 

Box 45.5  Factors Affecting Transplant Survival Medical status at orthotopic liver transplantation Primary diagnosis Age and size Comorbid conditions Encephalopathy Infection Multiple organ dysfunction

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% Survival

80 60 40 Whole SRG All

20 0

0

1

2

3

4

5

6

7

8

9

10

Years post-transplant Fig. 45.8  Ten-year patient and allograft survival subdivided by whole and surgically reduced grafts (SRG). (Data from Cincinnati Children’s Hospital Medical Center, Liver Care Center, Cincinnati, OH.)

Intestinal Transplantation Intestinal failure (IF) can be a significant problem in the pediatric population, but the exact incidence in children is unclear because the diagnosis encompasses a diverse group of conditions and overall disease incidence is low.80 Generally, IF is the inability of the intestine to function due to inadequate anatomic length or lack of absorptive function requiring total parenteral nutrition (TPN) as the first-line therapy in children who have a loss of enteral autonomy. The North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) proposed that IF be defined as the need for TPN for longer than 60 days due to intestinal disease, dysfunction, or resection.81 Sequelae of long-term TPN, such as cholestatic liver disease, also known as intestinal failure–associated liver disease (IFALD), venous thromboses, and central line– associated blood stream infection (CLABSI), may preclude its continued use. When such complications become life threatening, and the bowel length is too short for enteral alimentation, the alternative becomes intestinal transplantation. According to a current review of the Organ Procurement and Transplantation Network (OPTN) website, approximately 60% of the patients who are currently on the national intestinal transplantation waiting list are children, with the majority of active patients being between 1 and 10 years of age. Advances in immunosuppressive regimens, the technical aspects of the transplant operation, and the surveillance and treatment of transplant-related complications have continued to improve the outcome of patients who have required intestinal transplantation.82 As a result, the role of intestinal transplantation has become an essential part of the armamentarium in the management of patients with IF. Since the initial approval of federal reimbursement for intestinal transplantation by the Centers for Medicare and Medicaid Services in October 2000, continued success with intestinal transplantation in children has shifted focus from short-term patient survival to optimizing long-term allograft function and patient survival.83

INTESTINAL REHABILITATION TPN is the standard treatment for patients who experience IF. Intestinal rehabilitation should be aggressively pursued

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because intestinal adaptation can result in eventual enteral autonomy, which has been shown to result in significantly improved transplant-free survival at 5 years.84 A formalized, multidisciplinary intestinal rehabilitation (IR) team utilizes a combination of TPN, gradual reintroduction of enteral feeds, intestinal antimotility agents, and treatment of small bowel bacterial overgrowth, in association with hepatoprotective lipid minimization strategies and ethanol lock usage to reduce the incidence of catheter-related blood stream infections to achieve enteral autonomy in many patients.81 Autologous intestinal reconstruction procedures, such as the serial transverse enteroplasty (STEP) and longitudinal intestinal lengthening and tailoring, may be beneficial in selected patients. In general, survival and complete return of GI function may be predicted when the postresection length of the intestine exceeds 5% of normal for gestational age if the ileocecal valve is present or is greater than 10% of normal if the ileocecal valve is absent.85 Data from an international, multicenter registry of patients who underwent STEP demonstrated that both pre- and post-STEP bowel length was associated with transplant-free survival for IF patients, but, interestingly, the presence of the ileocecal valve did not.86 Because of the success treating IF patients cared for by IR teams, intestinal transplant rates in the United States have decreased since 2008.87 Finally, Groen et  al. used a discrete-event model based on retrospectively collected data to conclude that IR improves pediatric patient survival with concomitant cost savings.88 However, due to the heterogeneity of data and affected patients, variety of programspecific management algorithms, and overall length of follow-up, the need for IF and IR registries remains paramount to make definitive conclusions about IR. 

INDICATIONS FOR TRANSPLANTATION The causes of IF can be divided into three broad categories: short bowel syndrome (SBS), intestinal dysmotility syndromes, and absorptive disorders. SBS, usually caused by the loss of intestinal length due to an intra-abdominal catastrophe or in the setting of a congenital GI disorder, is the most common cause in children. Disease processes necessitating an operation that result in SBS most commonly include necrotizing enterocolitis and gastroschisis, followed by intestinal atresia in newborns.89 Other indications, specifically in older children, include Crohn disease, traumatic injury to the main intestinal blood supply, insidious tumors that involve the mesenteric root or that involve multiple intra-abdominal viscera, and complete portomesenteric thromboses.90,91 Midgut volvulus, another frequent cause of SBS, can occur at any age, although the majority of cases occur in infants. Intestinal dysmotility syndromes include total intestinal aganglionosis and the constellation of disorders known as chronic idiopathic intestinal pseudo-obstruction. Absorptive disorders lead to intractable diarrhea due to impaired enterocyte absorption and include congenital epithelial mucosal diseases, such as microvillus inclusion disease, tufting enteropathy, and autoimmune enteritis. Although these latter disorders are rare, affected children face lifelong difficulty with GI absorptive function and require TPN for long-term survival.

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The current Medicare-approved indications for intestinal transplantation are shown in Box 45.6.83 Progressive IFALD results in significant mortality. Unless the TPN can be stopped, liver failure is a strong indication for combined liver/intestinal or multivisceral transplantation. Intuitively, more advanced IFALD correlates with higher mortality rates, but a recent review of the Pediatric Intestinal Failure Consortium (PIFCon) demonstrated that a direct bilirubin level of >4 mg/dL conferred a fourfold increase in mortality.92 The remaining criteria of limited central venous access, multiple CLABSI, and frequent episodes of severe dehydration reflect complications from chronic TPN use and, in the absence of significant concurrent liver dysfunction, are indications for isolated intestinal transplantation. In a recent review, Soltys et al. summarized the current role of intestinal transplant in patients with IF in light of reported successes with IR programs.93 They and other authors advocate a personalized, patient-specific approach that weighs the ongoing risk of IR versus the previously mentioned complications related to parenteral nutrition.94 This group found that patient and graft survival after intestinal transplantation were improved if recipients undergo transplantation prior to the onset of growth impairment and secondary organ damage, especially liver disease. Despite the lack of consensus with regard to optimal timing of intestinal transplantation, it has become clear that early and timely patient referral for pretransplant evaluation is critical to ensuring the best opportunity for longterm survival.95 The contraindications to intestinal transplantation are similar to those for any other solid organ transplantation. The presence of severe cardiopulmonary dysfunction, an active nonresectable malignancy, severe neurologic disabilities, or life-threatening extraintestinal illness or infection precludes intestinal transplantation. 

OPERATIVE CONSIDERATIONS The three types of intestinal allografts include multivisceral, liver-small bowel composite, and isolated small bowel. The type of intestinal allograft is tailored to physiologic and anatomic requirements of the individual patient. The biggest limitation to intestinal transplantation in the pediatric

Box 45.6  Medicare-Approved Indications for Intestinal Transplantation 1. Impending or overt liver failure due to TPN-induced liver injury. Liver failure defined as increased serum bilirubin or liver enzyme levels (or both), splenomegaly, thrombocytopenia, gastroesophageal varices, coagulopathy, stomal bleeding, hepatic fibrosis, or cirrhosis 2. Thrombosis of two or more central veins (subclavian, jugular, or femoral) 3. The development of two or more episodes of systemic sepsis secondary to central line-associated blood stream infection that requires hospitalization, a single episode of line-related fungemia, or septic shock and/or acute respiratory distress syndrome 4. Frequent episodes of severe dehydration despite intravenous fluid supplementation in addition to TPN TPN, total parenteral nutrition.

population is the need for size-matched grafts. Patients with IF generally have limited abdominal domain (due in part to a lack of intestine volume), which necessitates nearidentical-size donors or, preferably, donors smaller than the recipient. Advances in the use of reduced-size liver allografts have been applied to the liver–small bowel composite allograft as a means of increasing the flexibility of donorto-recipient size matching. However, nearly 50% of patients on the intestinal transplant waiting list die before undergoing transplantation owing to the lack of appropriate donors, and the highest risk subgroup are children younger than 1 year of age.96 To address this problem, some centers have engaged in LD intestinal transplant as well as combined LD intestinal transplant and LT in children with reasonable results.97 In the past, it was thought that recipients who were CMV-negative should not receive intestinal grafts from CMV-positive donors because of a high incidence of severe, potentially life-threatening CMV infection after transplantation. However, current antiviral therapies as well as specific immunomodulating, inductive therapies can reduce the risk of CMV infection in this high-risk group.98 In an early series, a higher risk of infectious complications was reported in patients receiving allografts that included donor colon.99 More recently, however, morbidity and mortality rates in recipients of colon-inclusive allografts have not been found to be higher.100 Children with total intestinal aganglionosis and chronic idiopathic intestinal pseudo-obstruction most commonly receive colon-inclusive allografts. An ileostomy is created in all recipients so that surveillance endoscopy and biopsy of the small bowel mucosa can be performed to monitor the allograft for rejection. If not already present, a gastrostomy is placed at the time of transplantation for gastric decompression and for access to the upper GI tract. Additionally, jejunal access is often essential for initiating postoperative feeds. This can be achieved via nasoenteric tube or gastrojejunostomy (GJ) tube. However, in our experience, GJ tubes can cause perforations of the intestinal allograft, leading to significant morbidity postoperatively, and therefore must be used cautiously. A particular challenge of intestinal transplantation in the pediatric recipient is closure of the abdominal incision due to pretransplant loss of domain and size discrepancy between allograft and recipient intra-abdominal space. A variety of techniques have been utilized for abdominal wall closure, including staged abdominal closure, acellular dermal matrix patches, nonvascularized rectus sheath fascia allografts, and abdominal wall transplantation.101 

THE TRANSPLANT PROCEDURE Multivisceral Allograft Multivisceral transplantation consists of transplantation of the stomach, pancreaticoduodenal complex, and intestine, either with (multivisceral allograft) or without (modified multivisceral allograft) the liver. In the pediatric population, the primary indications for multivisceral transplantation are the intestinal dysmotility syndromes. On occasion, a giant desmoid tumor of the mesentery that extensively infiltrates the mesentery may require this form of transplantation. Exenteration of the native intra-abdominal viscera is followed by transplantation of the multivisceral graft using arterial inflow through the donor celiac and superior

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mesenteric arteries (SMAs). Venous outflow from the multivisceral allograft occurs via the transplanted liver placed in the standard orthotopic position. For the modified multivisceral allograft, portal venous return is via an anastomosis to the recipient’s native portal vein. GI continuity is completed via a gastrogastric anastomosis proximally and an ileocolic anastomosis distal to the ileostomy. 

Fig. 45.9 Schematic diagram of liver/intestine composite allograft. (From Abu-Elmagd K, Reyes J, Todo S, et al. Clinical intestinal transplantation: new perspectives and immunologic considerations. J Am Coll Surg 1998;186:512–527.)

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Liver–Small Bowel Composite Allograft A liver–small bowel composite allograft is a modification of the multivisceral allograft in which the stomach is removed during procurement. This form of transplantation is indicated in patients with IF and impending or overt TPN-induced liver failure (Fig. 45.9). The recipient’s liver and remaining small intestine are removed, and the native stomach, duodenum, pancreas, and spleen are left intact. A portocaval shunt from the native portal vein to the inferior vena cava is necessary to provide venous outflow from the recipient’s foregut organs (Fig. 45.10). As in the multivisceral allograft, the donor celiac artery and SMA are the source of arterial inflow to the transplanted organs. The donor portal vein and biliary tree remain intact, having not undergone dissection during procurement. As a result, no portal vein or bile duct reconstruction is needed. The pancreas is also left intact to protect the peri-biliary ductal vessels and to prevent the possibility of pancreatic leak from a divided surface. Venous outflow from the transplanted organs is once again provided by the donor liver placed in a standard orthotopic position. If the liver is too large, an ex vivo hepatic lobectomy can be performed, usually removing the right lobe of the liver (Fig. 45.11). GI continuity from the patient’s native stomach and duodenum to the newly transplanted small bowel is achieved by anastomosis of the native duodenum to the donor jejunum. If the recipient has any colon remaining, a donor ileum to recipient colon anastomosis is created distal to the ileostomy. 

Portocaval shunt

Residual recipient foregut

Composite graft with residual recipient foregut

Liver / pancreas / duodenum / small bowel composite graft Fig. 45.10  Schematic diagram of liver/intestine composite allograft with native portocaval shunt. (© Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, 2008.)

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Fig. 45.11  Schematic diagram of reduced-size liver/intestine composite allograft. (From Reyes J, Mazariegos GV, Bond GMD, et al. Pediatric intestinal transplantation: historical notes, principles and controversies. Pediatr Transplant 2002;6:193–207.)

Isolated Small Bowel Allograft Transplantation of the small intestine alone entails procurement of only the jejunum and ileum. During procurement, the SMA and vein are divided just below the third portion of the duodenum at the root of the mesentery, generating an allograft of jejunum and ileum (Fig. 45.12). This type of transplant is indicated in patients with IF, but without liver dysfunction. Arterial inflow is provided by anastomosis of the SMA to the recipient’s aorta. Venous drainage of the transplanted intestines is into either the inferior vena cava or the native SMA. Initially it was thought that venous drainage into the native portal circulation was beneficial to the liver, but there is likely minimal benefit. Therefore, currently, the most common approach to venous reconstruction is an end-to-side anastomosis of the donor superior mesenteric vein to the native inferior vena cava. GI continuity is restored by anastomosis of the recipient’s native proximal bowel to the transplanted jejunum. Once again, if residual colon is present, a donor ileum to native colon anastomosis is created downstream from the ileostomy. 

POSTOPERATIVE COMPLICATIONS Although the outcomes of intestinal transplantation continue to improve, postoperative complications are not uncommon. A breakdown of intestinal integrity either at sites of anastomosis or in areas of mucosal injury from ischemic reperfusion injury often necessitate reexploration. Bowel perforation also can occur following surveillance endoscopy and biopsy. Patients with a significant amount of peritoneal contamination after intestinal perforation may require serial operative explorations to clear foci of intra-abdominal infection. Postoperative bleeding is frequent, especially in patients who undergo transplantation with liver–small bowel composite allografts, because preexisting portal hypertension results in varices throughout the abdominal cavity. Chylous leaks are also frequent because lymphatic drainage may be disrupted during both the procurement and the transplant procedure. Most chylous leaks can be managed conservatively. However, a high percentage of patients who undergo intestinal transplantation will require reexploration at some point in the postoperative period.

SMV, SMA

Fig. 45.12  Schematic diagram of isolated small intestinal transplant. SMA, superior mesenteric artery; SMV, superior mesenteric vein. (Adapted from Abu-Elmagd K, Fung J, Bueno J, et al. Logistics and technique for procurement of intestinal, pancreatic, and hepatic grafts from the same donor. Ann Surg 2000; 232:680–687.)

Immunosuppression and Allograft Rejection The most significant recent advances in the management of intestinal transplant recipients have occurred in the development of immunosuppression regimens. Acute rejection occurs in approximately 35–55% of pediatric recipients within the first year after transplantation.87 Overwhelming rejection used to be one of the most frequent causes of allograft loss during the early transplant experience due, in part, to the limited number of immunosuppressive agents available. Prior to the development of tacrolimus as a maintenance immunosuppression approach, high-dose immunosuppression was necessary to prevent rejection. Infections, PTLD, and adverse effects related to high-dose immunosuppression were frequent causes of a poor outcome. The goal of current immunosuppressive regimens is to use just enough immunosuppression to prevent rejection, but not so much that infections and PTLD occur. In an effort

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to achieve tolerance, many centers utilize a lymphocytedepleting strategy, such as thymoglobulin or alemtuzumab, to induce early elimination of graft-specific T-lymphocytes. The management of immunosuppression in patients who undergo intestinal transplantation remains the most challenging aspect of the postoperative care. Surveillance endoscopy and biopsy is initiated within 5 days of transplantation to evaluate for acute rejection and is performed twice per week for the first month. Significant progress in the definition of the histologic characteristics of acute small bowel rejection has been achieved.102 Interestingly, one center’s experience revealed that the endoscopic appearance of the allograft mucosa was poorly predictive of histologic rejection.103 Thus, adjunctive, noninvasive assays have been developed to monitor for both acute cellular and antibody-mediated rejection.104,105 If acute rejection is diagnosed, a short course of highdose corticosteroids is administered. If rejection is severe or persists despite high-dose corticosteroid administration, antilymphocyte antibody agents (thymoglobulin or alemtuzumab) are used. Chronic rejection remains the most common cause of late allograft dysfunction and failure. 

Infection Owing in large part to the high level of immunosuppression needed after intestinal transplantation, bacterial and fungal infections are common. Patients with IF are frequently colonized with antibiotic-resistant bacteria due to recurrent infections while on TPN. As mentioned previously, because intestinal perforation is common, peritonitis is a frequent complication often requiring repeat abdominal explorations to completely clear foci of infection. CMV, EBV, adenovirus, and calicivirus are the most frequent viral pathogens found in the postoperative period, and many can masquerade as acute rejection. PTLD remains a significant problem because most infants are EBV-negative at the time of transplantation. Surveillance for CMV and EBV using PCR, followed by aggressive treatment when detected, has diminished the impact of these dangerous pathogens on patient outcome. All patients are maintained on prophylactic antiviral agents after intestinal transplantation.  Graft-Versus-Host Disease Graft-versus-host disease (GVHD) occurs in approximately 5–10% of intestinal transplant recipients, most commonly in the youngest pediatric recipients. Its high incidence is related to the large burden of donor lymphocytes that are cotransplanted with the allograft. Acute GVHD characteristically affects the skin, native liver, and native GI tract and carries a high mortality. Monitoring of donor-derived T-cell chimerism is being utilized to identify patients at risk for GVHD. Management strategies for GVHD after intestinal transplantation include high-dose corticosteroids and lymphocyte depletion with alemtuzumab. 

OUTCOME FOLLOWING TRANSPLANTATION Early outcomes of patients who underwent intestinal transplantation was poor, with few long-term survivors. With advances in operative techniques, immunosuppression strategies, allograft surveillance, and monitoring and

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treatment of infections and other transplant-related complications, significant improvements in patient survival have occurred in the past 15 years. Currently, according to the OPTN database, approximately 80% of pediatric recipients survive 1 year and 65% survive 5 years after transplantation.106 Short-term allograft survival has likewise increased significantly. Patient survival for solitary intestinal transplant is higher compared with composite intestine-liver transplant, likely due to preoperative status of patients requiring concurrent liver for IFALD. Despite improvements in short-term outcomes, the long-term outcomes have remained largely unchanged over the past decade. Current impediments to long-term allograft survival are primarily related to sepsis and rejection, particularly in isolated small bowel transplantation.107 Further advances in surveillance and immunosuppressive strategies are needed to optimize long-term allograft function and survival. 

Renal Transplantation Acute kidney injury (AKI) results in either decreased GFR or an increase in relative or absolute serum creatinine as well as a decrease in expected daily urine output.108 The interplay of hemodynamic instability that leads to poor perfusion or hypoxia resulting in acute tubular necrosis (ATN), with risk factors such as perinatal events, sepsis, and nephrotoxic medications determines the severity of AKI.109 Although AKI is not an indication for renal transplantation, a meta-analysis suggests it is a risk factor for mild chronic kidney disease (CKD) and mortality.110 CKD is uncommon in infants as the estimated incidence of ESRD is 7–9 per million total population of infants younger than 2–4 years of age.111,112 Congenital anomalies of the kidney and urinary tract (CAKUT), including renal aplasia/dysplasia and obstructive and complex urologic malformations, as well as focal segmental glomerulosclerosis (FSGS), are the most common causes of ESRD in children younger than 5 years of age (Table 45.4). Antenatal renal failure caused by dysplastic kidneys or obstructive malformations and the resultant oligohydramnios or anhydramnios often leads to fetal demise or pulmonary insufficiency incompatible with postnatal survival. With the evolution of fetal diagnosis and in utero therapy such as vesicoamniotic shunting, pulmonary insufficiency may be attenuated, creating a population of pulmonary survivors with perinatal renal insufficiency.113 Our institution and others have advanced research protocols centered around percutaneous amnioinfusion in the setting of renal dysplasia for similar reasons. Currently, however, such approaches can only be characterized as experimental and require further study.114 FSGS and various glomerulonephritidies, as well as recurrent pyelonephritis, are the common causes of ESRD in older children. Additionally, as the number of patients who have undergone renal transplantation in childhood has increased, chronic rejection following renal transplantation has recently become a cause of ESRD. Additionally, pediatric recipients of nonkidney, solid organ transplants are also at higher risk for ESRD due to acute and chronic kidney injury from nephrotoxins including standard immunosuppressants such as CNI.115

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Table 45.4  Primary Diagnoses in Children Requiring Renal Transplantation Primary Diagnosis

N

%

Aplasia/hypoplasia/dysplasia kidney Obstructive uropathy Focal segmental glomerulosclerosis Reflux nephropathy Chronic glomerulonephritis Polycystic disease Medullary cystic disease Congenital nephrotic syndrome Hemolytic uremic syndrome Prune belly Familial nephritis Cystinosis Idiopathic crescentic glomerulonephritis Membranoproliferative glomerulonephritis—type I Pyelonephritis/interstitial nephritis Systemic lupus erythematosus Renal infarct Berger (immunoglobulin A) nephritis Henoch–Schönlein nephritis Membranoproliferative glomerulonephritis—type II Wegener granulomatosis Wilms tumor Oxalosis Drash syndrome Membranous nephropathy Other systemic immunologic disease Sickle cell nephropathy Diabetic glomerulonephritis Other Unknown

1769 1713 1308 576 344 339 305 289 288 279 247 225 195 191 189 172 144 135 115 87 71 59 58 57 51 34 16 11 1223 692

15.8 15.3 11.7 5.1 3.1 3.0 2.7 2.6 2.6 2.5 2.2 2.0 1.7 1.7 1.7 1.5 1.3 1.2 1.0 0.8 0.6 0.5 0.5 0.5 0.5 0.3 0.1 0.1 10.9 6.2

Data from the North American Pediatric Renal Transplant Cooperative Study 2014 Annual Report.

PRETRANSPLANT MANAGEMENT Due to the advances in surgical technique, immunosuppressive regimens, and medical management, renal transplant is the gold-standard renal replacement therapy (RRT) for both children and adults. Therefore, pretransplant management is critical for infants and children with ESRD because a number of longitudinal, population-based studies found that mortality rates in these patients are 30 times higher than in age-matched children.116 Children with ESRD beginning in infancy or early childhood experience significant complications from growth retardation, renal osteodystrophy, and neuropsychiatric developmental delay. And like their adult counterparts, these patients often suffer significant morbidity from cardiovascular disease and infectious complications. Recent advances in dialysis regimens, nutritional supplementation, management of hypertension, and recombinant human erythropoietin and growth hormone have all significantly improved the pretransplant management and ultimately the overall survival of these patients.117

Dialysis RRT is indicated when complications of ESRD occur despite optimal medical management, specifically hyperkalemia, volume overload, acidosis, intractable hypertension, and uremic symptoms such as vomiting and fatigue. In older children, lethargy and poor school performance can signal the need for more aggressive treatment. In addition, dialysis may be necessary to facilitate the administration of

adequate protein as part of an extensive nutritional resuscitation plan. When dialysis is undertaken, the use of peritoneal dialysis (PD) is preferred for the following reasons: it avoids the multiple blood transfusions associated with hemodialysis; it allows a gradual correction of electrolyte abnormalities, preventing cerebral disequilibrium syndrome in small infants; it allows for easier control of osteodystrophy; it optimizes nutrition; and it is less labor and resource intensive for clinicians and families. Intuitively, these factors should be magnified in infants requiring maintenance dialysis. However, one study demonstrated that there is no difference in survival or likelihood of transplantation for either modality.118 Surgeons must consider a number of options when placing a PD catheter that may affect patency and success for PD including approach (open vs laparoscopic), type of catheter (single-cuff vs double cuff, swan neck vs straight catheter body, and linear vs coiled tip), adjunct procedures such as omentectomy, and exit direction of the catheter (cranially vs caudally). A number of studies and meta-analyses have been published on this subject, but limited information in pediatric patients seems to favor a linear tip catheter.119 There remain conflicting data with regard to the other aforementioned considerations. It is our practice to utilize a precurved catheter with its fascial entry site on one side of the midline and exit site on the contralateral side. The exit site is always caudally oriented and covered with a silver-impregnated, antimicrobial dressing (Fig. 45.13). We perform an omentectomy as well. Hemodialysis can be used when the peritoneal cavity is nonfunctional due to prior surgery or multiple peritoneal infections. However, the construction and maintenance of adequate long-term vascular access sites in small infants and children can be difficult. Although the National Kidney Foundation’s clinical practice guidelines advocate for the creation of arteriovenous fistulae (AVFs) in pediatric patients who weigh >20 kg and are unlikely to receive a transplant in 1 year, use of central venous catheters rather than AVFs is our preferred mode for temporary hemodialysis access in infants and small children, although infection and vascular thromboses complicate this therapy. Access via the internal jugular veins is preferred over subclavian routes to avoid obstruction of the venous outflow from the upper extremity, which could compromise future AVF creation. Primary and secondary patency for pediatric AVFs can be excellent, but primary patency rates improve with increasing age.120 Our institution and others have policies of avoiding peripheral venous access being placed above the wrist as well as peripherally inserted central catheters (PICCs).121 

Nutritional Support Vigorous nutritional support of children with ESRD is crucial to prevent growth impairment, as the most intense period of a child’s growth occurs during the first 2 years of life. Growth disturbance is multifactorial, including anorexia that leads to protein and calorie insufficiency, renal osteodystrophy, aluminum toxicity, uremic acidosis, impaired somatomedin activity, and growth hormone and insulin resistance.122

45 • Solid Organ Transplantation in Children

A

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B

Fig. 45.13  Preferred orientation of a peritoneal dialysis (PD) catheter (A). Our practice is to use precurved, double-cuffed PD catheters when possible. The “X” indicates the location of the Dacron cuff where it enters the abdomen. The cuff is sutured to the posterior rectus sheath using a fine, nonabsorbable monofilament suture placed in a purse-string fashion. The catheter is tunneled subcutaneously in a gentle curve to exit the skin contralateral to the entrance site in a caudal direction. Note the exit site is small and well approximated to the catheter, thus preventing peri-catheter leakage. Dressing of the peritoneal dialysis catheter site (B). In our institution, the PD catheter exit site is typically covered with a chlorhexidine-impregnated protective disk. The site is then sealed with a transparent dressing.

In a recent annual report from the North American Pediatric Renal Transplant Cooperative Study (NAPRTCS), the mean weight at the time of renal transplantation has increased from 1.6–1.09 standard deviation (SD) below the appropriate age-adjusted and gender-adjusted mean for normal children.123 This deficit was greater (2.2 SD) in children younger than 5 years of age. Transplantation afforded an approximately 0.85 SD increase in growth over the first post-transplant year. Children 6 years of age and older showed significantly less improvement in their height and weight deficit 6 years after transplantation. These limitations to “catch-up” growth emphasize the need for early transplantation in young ESRD patients. Normalization of growth rarely occurs with hemodialysis or PD. 

PREOPERATIVE PREPARATION In preparation for renal transplantation, an extensive evaluation of the urinary tract and immunologic status of the patient is important. Because urinary tract abnormalities as a whole are the most common cause of ESRD in infants and children, pretransplant investigation of the urinary tract for sites of obstruction, the presence of ureteral reflux, and the functional state and capacity of the urinary bladder is important.124 This investigation is best accomplished by obtaining an US or intravenous pyelogram evaluating the upper urinary tract and a voiding cystourethrogram (VCUG) to assess the bladder and reflux. Any concerns about bladder function or anatomy requires urodynamics and cystoscopy. In patients with long-standing oliguric ESRD, the bladder may be very small. In the absence of obstructive or neuromuscular pathology, enlargement of the bladder with normal urinary production is expected. Any operative correction of urethral obstruction or augmentation of bladder size should be performed well in advance of transplantation. Preoperative sterilization of the urinary tract and the development of unobstructed urinary outflow should be the ultimate goals of evaluation and reconstruction. Although complex anomalies of the urogenital tract often require many operative procedures to augment, reconstruct, or

create an acceptable lower urinary tract, most children can undergo successful reconstruction with continent urinary reservoirs without the need for intestinal conduits.125 Immunologic assessment includes tissue typing and panel reactive antibody (PRA) analysis. Patients should be monitored periodically for the development of a positive cross-match to their potential LD or a positive cytotoxic antibody to a panel of random donors. Induction and posttransplant immunosuppression plans are usually modified for populations known to be of higher immunologic risk with traditionally poorer outcomes: African-American patients, high peak PRA, previous transplant history, and degree of HLA mismatch. In addition, reactivity to CMV, EBV, HSV, and hepatitis also should be performed. Childhood immunizations should be current, and immunization against hepatitis B virus is important. Any immunizations with live-virus vaccines should be given well in advance of transplantation because their use is contraindicated in the early post-transplant period. Selection of the appropriate donor source for transplantation is a decision for the transplant team and family to consider together. A related immediate family member has the advantage of a low incidence of postoperative ATN, improved histologic matching, and extended organ function. In addition, any operative procedures required for preparation of the recipient, as well as the transplant procedure, can be scheduled around the needs of the patient, simplifying the preoperative care and potentially avoiding the complications of dialysis. Parents form the majority of donors. The 2014 NAPRTCS report indicates that 79% of children receive an LD kidney from a parent. Thorough evaluation of the potential donor to exclude intrinsic renal anomalies, vascular anomalies, and systemic illness is important. Deceased donor (DD) kidneys are currently used for 50% of renal transplants. The unpredictability of donor organ availability and the need to establish a negative antibody cross-match for DD transplantation make surgical planning impossible. The size of a potential allograft, DD or related LD, is also important. Kidneys from adult donors can be transplanted into infants as small as 5 kg with good success.126

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DD organs from pediatric donors 5 years of age or older also result in an excellent survival rate. However, a recent analysis of the Scientific Registry of Transplant Recipients (SRTR) database demonstrated progressive decrease in 1-year graft survival and increased rates of acute rejection when transplanting kidney(s) from very small pediatric donors (24 hours) and the presence of ATN with delayed graft function also increase this risk. Immediate post-transplant Doppler US is helpful in confirming suitable allograft blood flow following abdominal closure, especially when large allografts are implanted into small recipients. Adequate hydration is important to maintain perfusion. Anticoagulation has not been used in most series. Urinary leak, most often at the neocystostomy site, presents with oliguria and persisting uremia. US or nuclear imaging can be useful for identifying an extravesical fluid collection. Operative repair is necessary to prevent urinoma formation and its potential infectious complications. Urinary collections should be differentiated from lymphoceles at the transplant site. Lymphoceles that do not resolve are best opened into the peritoneal cavity via laparoscopy. 

METABOLIC SYNDROME AND CARDIOVASCULAR DISEASE External iliac vein

Renal artery Renal vein Ureter A Inferior vena cava

Aorta

Renal artery Renal vein

Up to 25–70% of pediatric renal transplant recipients develop metabolic syndrome.133 Although the manifestations of this syndrome, including obesity, dyslipidemia, hypertension, and hyperglycemia, may be heterogeneous, all can contribute to the development of significant cardiovascular disease, which is one of the primary causes of death in children and adolescents after renal transplant.134 Immediately following transplantation, as many as 80% of all patients require antihypertensive therapy. Perhaps even more startling is the estimate that 50–70% of patients remain on therapy up to 5 years post-transplant, with onethird remaining hypertensive.135 Careful attention to the pretransplant control of hypertension and dietary management improves post-transplant management. Additionally, the incidence of metabolic syndrome decreases as dose reduction of CNIs and corticosteroids occurs, especially in the first year post-transplant. Although hypertension in any patient population is a risk factor for cardiovascular disease, a recent study has linked elevated ambulatory blood pressure with left ventricular hypertrophy. A subset analysis demonstrated an association between use of antihypertensives and worsened allograft function.136 Other studies have confirmed the association of post-transplant hypertension and graft loss.137

INFECTION B Fig. 45.14 Schematic diagram showing the transplant arterial and venous anastomosis to the (A) iliac vessels or the (B) aorta and vena cava. Ureteral implantation using the Lich extravesical ureteroneocystostomy is preferred.

Much like liver and intestinal transplantation, the highest risks for infectious complications arise within the first 6 months post-transplant. During this time, immunosu­ ppression is intense and susceptibility to life-threatening infection is increased. Additionally, the youngest recipients (90%, although sometimes a second dilation or endoscopic incision have been needed to reach this final success rate.94–97 In a recent study, onset VUR occurred in 27% of ureters, one-third of which spontaneously resolved on follow-up VCUG.98 This approach may be particularly useful in older children because of the marked decrease in morbidity associated with a completely endoscopic approach. If failure or clinically significant reflux develops, standard open or laparoscopic or robotic techniques can be used to perform a tailored or tapered reimplant. 

ECTOPIC URETER An ectopic ureter is defined as one that does not drain on the trigone, but enters at the bladder neck or more caudally. Embryologically, this results from a cranial insertion of the ureteral bud on the mesonephric duct that allows distal migration with the mesonephric duct as it is absorbed into the urogenital sinus.99 The incidence of ureteral ectopia is approximately 1 in 2000.61 Eighty percent of ectopic ureters are reported in association with a duplicated renal system. As clinical problems are more common in girls with ectopia, only 15% of ectopic ureters have been reported in boys.61,99 Ectopia is bilateral in 20% of patients.61 Single ectopic ureters are rare but are more common in boys.100

Ectopic Ureter in Girls The fundamental difference between ureteral ectopia in boys and girls arises from the ureteral insertion distal to the continence mechanism in girls (Fig. 54.14). Approximately one-third of ureters open at the level of the bladder neck, one-third are in the vestibule around the urethral opening, and the remainder empty into the vagina, uterus, or cervix. All of these insertions are along the course of the mesonephric duct remnant (Gartner’s duct). Fifty percent of affected girls initially have continuous urinary incontinence despite what appears to be a normal voiding pattern.61,99 If the system is markedly hydronephrotic

54 • Ureteral Obstruction and Malformations

4

5 1

1

6 5

2 1

3

2

4 3

A

6

7 B

Fig. 54.14  (A) Ureteral ectopia in a boy. Possible sites are above the external sphincter (1–3), or into the seminal vesicle (4, 5), or anorectal (6). (B) Ureteral ectopia in a girl may be located at the bladder neck (1), or beyond the continence mechanism in the urethra (2), or on the perineum (3). Uterine or vaginal insertion (4–6) may also cause incontinence. Anorectal insertion (7) can also occur.

and functions poorly, urine leakage may occur only in the upright position and may be confused with stress incontinence. Persistent foul-smelling vaginal discharge can suggest an ectopic ureter. When the ectopic ureter is present in the urethra or the bladder neck, both obstruction and reflux are frequently found. The diagnosis of an ectopic ureter can be straightforward or difficult. When the distal end exits in the vagina, cervix, or uterus, the kidney may not visualize on US if the renal moiety is small and atrophic, and not associated with hydronephrosis. Often significant hydronephrosis is found in the upper pole of a duplicated system, and the US image may show a dilated ectopic ureter behind the bladder. However, the upper pole may also be a small remnant (Fig. 54.15). A dimercaptosuccinic acid (DMSA) scan is a good test for localizing a small ectopic kidney when an orthotopic kidney is not identified on standard imaging and when there is a high suspicion of an ectopic ureter in the vagina, cervix, or uterus. As long as there is some dilation, MRU may be the most precise method for making this diagnosis.101 A VCUG should also be obtained to exclude occult reflux.102 The diagnosis is confirmed with physical examination, panendoscopy, and retrograde pyelography. Filling the bladder with dyes that stain the urine (via catheter or at the time of cystoscopy) may have a role as urine in the bladder will be colored, but the urine flowing from an ectopic ureter is seen as clear leakage. Meticulous examination of the area around the urethral meatus and vagina will often reveal an asymmetry or bead of fluid coming from an opening that can be probed and injected in retrograde fashion (see Fig. 54.15). Vaginoscopy with attention to the superior lateral aspect of the vagina may also reveal a large ectopic orifice. 

Ectopic Ureter in Boys The most common sites of ectopic ureteral insertion in boys are the posterior urethra (40–50%) and the seminal vesicle (20–60%), depending on the age at presentation.103 Symptoms in boys may not occur until after the onset of sexual activity and include prostatitis, seminal vesiculitis, or an infected seminal vesical cyst causing painful bowel movements. The genital insertion accounts for the common presentation with epididymitis. He may have post-void dribbling

847

secondary to pooling of urine in the prostatic urethra, but incontinence is never as pronounced as in a female. Ectopic ureters in the boy are more commonly obstructed and hydronephrotic, so US is often more useful. If the ectopic insertion site is outside the urethra, it is rarely identified on endoscopy. 

Management Operative treatment is dependent on the associated renal parenchyma.104,105 Single-system ectopic ureters that enter the genital system usually have poor renal function, and nephroureterectomy is appropriate. Simple laparoscopic ligation of the ectopic ureter associated with a nonfunctioning kidney has also been described with a reduced operative time compared with nephrectomy, and with good short-term results.106 Long-term results regarding issues such as hypertension are still pending, and one should be cautious of adopting this approach for chronically infected systems. When the ectopic ureter is associated with duplication, the function in the upper pole is usually poor, and an open or laparoscopic partial nephroureterectomy has been the most common approach. The distal ureter can be left open assuming a lack of reflux has previously been documented. Also, a ureteroureterostomy can be performed proximally or distally as well. The dilated upper pole is diverted into the normal caliber lower pole system. There are potential concerns regarding the size discrepancy of the ureters and injury to the recipient ureter, but one large series showed excellent success with a very low complication rate.104 This approach avoids potential injury to the lower pole of the kidney and can be performed laparoscopically or through a small inguinal incision.107,108 The obstruction, dilation, and incontinence usually resolve following ureteroureterostomy. Even if the upper pole is poorly functioning, it should not cause significant long-term problems. A common sheath ureteral reimplantation can be performed with tailoring of the ureter from the upper pole, but the increased morbidity and complication rate associated with ureteral tapering limit the utility of this approach. Robotic and laparoscopic approaches have also been described for reimplantation and upper tract reconstruction.109–111 The distal ureteral stump rarely causes a problem in genital ectopia. However, if urethral or bladder neck insertion of the ectopic ureter and reflux into the ureter is identified preoperatively, excision of the distal stump is important, but can be tedious.105 If the plane of dissection is kept immediately adjacent to the ureter behind the bladder, the bladder neck and sphincter should not be damaged. A transvesical approach can also be used and aids in exposure of the urethral insertion. In a postpubertal girl, access to the urethra can be performed transvaginally as well.  Bilateral Single Ectopic Ureters This is a rare finding in which the altered ureteral embryologic development is associated with failure of normal bladder neck development.112 Genital and anal anomalies are commonly present. In girls, the ureter inserts into the distal urethra. They are usually initially seen with infection or are noted to have continuous urinary leakage. The bladder is usually poorly developed because it has never stored urine. Boys have somewhat larger bladders because some urine will have entered it. However, because the bladder neck is not formed normally, they also have some degree of urinary incontinence.

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A

B

Fig. 54.15  This 3-year-old girl had continuous urinary incontinence despite a normal voiding pattern. The renal ultrasound image was essentially normal on both sides with no evidence of hydronephrosis or an echogenic upper pole. (A) A ureteral catheter has been inserted into an ectopic left ureter. (B) The retrograde ureterogram shows a very small upper left ureter and a small cystic calyx (arrow) in the left upper pole medial to the lower-pole collecting system that was opacified through an orifice on the trigone. The patient was continent after a laparoscopic left upper pole partial nephroureterectomy.

The child who is incontinent with bilateral single ectopic ureters presents a major reconstructive challenge that may require ureteral reimplantation, bladder neck reconstruction, and bladder augmentation if the bladder capacity is insufficient. 

Ureteroceles Ureteroceles are cystic dilatations of the terminal, intravesical ureter that usually have a stenotic orifice.61 In children, ureteroceles are most commonly associated with the upper pole of a duplex system (80%) with an ectopic orifice (60%) in the urethra. In adults, they are usually part of a completely intravesical single system. Ureteroceles occur four to seven times more frequently in girls and are more common in whites. Bilateral ureteroceles are found in 10% of affected patients. A single embryologic theory does not explain all ureteroceles. Historically, a persistent Chwalla membrane at the junction of the Wolffian duct and urogenital sinus was theorized to cause a ureterocele.113 It is more likely that a ureterocele is the result of an abnormal induction of the trigone and distal ureter by many of the genes and growth factors that are important in renal and ureteral growth and development. Gross inspection of the intravesical portion of ureteroceles shows deficiencies in the trigonal musculature of patients with ureteroceles that are not present in in ectopic ureters without ureterocele formation. This theory results in a pseudodiverticulum (ureterocele eversion) and reflux into laterally displaced, poorly supported ureters. This is further supported by the clinical observation of multicystic dysplasia and the absence of hydronephrosis in association with a ureterocele.114 The classification of ureteroceles can be confusing. The current recommended nomenclature classifies ureteroceles as either intravesical (entirely within the bladder) or ectopic (some portion is situated permanently at the bladder neck or in the urethra).115

PRESENTATION AND DIAGNOSIS Most ureteroceles are identified prenatally although 10–15% still present postnatally due to infection.116–118 The obstructed renal unit may be palpable, but most have

Fig. 54.16  This 2-week-old baby presented with sepsis and was found to have this prolapsing ectopic ureterocele. The ureterocele was aspirated with return of purulent debris and underwent prompt decompression. Recovery was uneventful.

no clinically apparent abnormality. Bladder outlet obstruction is rare because most ureteroceles decompress during micturition, but the most common cause of urethral obstruction in girls is prolapse of a ureterocele (Fig. 54.16). Abdominal US reveals a well-defined cystic intravesical mass that is located within the posterior bladder wall (Fig. 54.17). This can be followed to a dilated ureter in the pelvis and to upper-pole hydroureteronephrosis in a duplicated system. The thickness and echogenicity of the renal parenchyma are often consistent with dysplasia and poor function. A VCUG typically shows ipsilateral lower pole or contralateral reflux.119 During cystoscopy, the bladder should be examined when it is full and also completely empty because compressible ureteroceles may not be evident in a full bladder or may appear as a bladder diverticulum. The dilated lower end of an ectopic ureter or megaureter may elevate the trigone,

54 • Ureteral Obstruction and Malformations

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* A

B

Fig. 54.17  (A) Ultrasound image of the bladder demonstrates a ureterocele (asterisk). (B) The ureterocele appears as a nonopacified filling defect (arrow) at the base of the bladder on the cystogram.

*

A

B

Fig. 54.18  (A) This intravesical ureterocele (asterisk) was found at cystoscopy. (B) The ureterocele was punctured (black arrow) and decompressed using a 3 French electrode (white arrow).

creating the cystoscopic, radiographic, and US appearance of a ureterocele, a so-called pseudoureterocele.120 

TREATMENT The goals for ureterocele management include control of infection, preservation of renal function, protection of normal ipsilateral and contralateral units, and continence. There is a subset of ureteroceles associated with multicystic dysplasia, no hydroureter, and no reflux. The multicystic moiety usually involutes, and the ureterocele rarely causes symptoms and can be observed.114 Up to 10–15% of prenatally identified ureteroceles have these clinical findings. Neonates given suppressive antibiotics rarely develop a febrile UTI.117,118 If significant hydroureteronephrosis is found, it is assumed that there is significant urinary tract obstruction and antibiotics should be initiated. The usual treatment of duplex ectopic ureteroceles has been upper-pole heminephrectomy through a separate flank incision, ureterocele excision, and ipsilateral lowerpole ureteral reimplant via a lower incision. The bladderlevel operation may require repair of a sizable defect in the bladder base and tapering or plication of the lower ureter. The distal extent of the ureterocele can often be dissected through the bladder neck. Incomplete excision can result in an obstructing urethral flap. Also, resection of the entire ureterocele risks damaging the continence mechanisms of the bladder neck. Experienced surgeons report excellent results with low rates of reoperation (50%). Even when US shows little renal parenchyma in the upper pole of a duplex system, incision can be performed. The decompressed system may not require further treatment if iatrogenic upper-pole reflux does not develop. In older children, incision is the preferred option when functioning renal parenchyma is found, the ureterocele is intravesical, or the kidney is drained by a single system. Single-system ureteroceles are more commonly seen in older children and adults, and are associated with better function and less hydronephrosis than is found in duplex kidneys. Most often, they are incidental findings that do not require treatment. Antenatally detected single-system ureteroceles may not show significant obstruction on a furosemide washout renal scan. Clinically, these behave like nonobstructed megaureters and can be safely followed with preventive antibiotics. If treatment is required, endoscopic incision is effective most of the time.

References







1. Dicke JM, Blanco VM, Yan Y, et al. The type and frequency of fetal renal disorders and management of renal pelvis dilatation. J Ultrasound Med. 2006;25:973–977. 2. Lee RS, Cendron M, Kinnamon DD, et  al. Antenatal hydronephrosis as a predictor of postnatal outcome: a meta-analysis. Pediatrics. 2006;118:586–593. 3. Hubert KC, Palmer JS. Current diagnosis and management of fetal genitourinary abnormalities. Urol Clin North Am. 2007;34:89–101. 4. Capello SA, Kogan BA, Giorgi LJ, et al. Prenatal ultrasound has led to earlier detection and repair of ureteropelvic junction obstruction. J Urol. 2005;174:1425–1428. 5. Carr M, El-Ghoneimi A. Anomalies and surgery of the ureteropelvic junction in children. In: Wein A, Kavoussi L, Novick A, eds. CampbellWalsh Urol. 9th ed. Philadelphia: WB Saunders; 2007:3359–3382. 6. Ruano-Gil D, Coca-Payeras A, Tejedo-Mateu A. Obstruction and normal recanalization of the ureter in the human embryo. Its relation to congenital ureteric obstruction. Eur Urol. 1975;1:287–293. 7. Abrams HJ, Buchbinder MI, Sutton AP. Benign ureteral lesions: rare causes of hydronephrosis in children. Urology. 1977;9:517–520. 8. Stephens FD. Ureterovascular hydronephrosis and the “aberrant” renal vessels. J Urol. 1982;128:984–987. 9. Koff SA, Hayden LJ, Cirulli C, et al. Pathophysiology of ureteropelvic junction obstruction: experimental and clinical observations. J Urol. 1986;136:336–338. 10. Starr NT, Maizels M, Chou P, et al. Microanatomy and morphometry of the hydronephrotic “obstructed” renal pelvis in asymptomatic infants. J Urol. 1992;148:519–524. 11. Hanna MK, Jeffs RD, Sturgess JM, et al. Ureteral structure and ultrastructure. Part II. Congenital ureteropelvic junction obstruction and primary obstructive megaureter. J Urol. 1976;116:725–730. 12. Djurhuus JC, Nerstrom B, Gyrd-Hansen N, et al. Experimental hydronephrosis. An electrophysiologic investigation before and after release of obstruction. Acta Chir Scand Suppl. 1976;472:17–28.

13. Wang Y, Puri P, Hassan J, et al. Abnormal innervation and altered nerve growth factor messenger ribonucleic acid expression in ureteropelvic junction obstruction. J Urol. 1995;154:679–683. 14. Hollowell JG, Altman HG, Snyder HM, et al. Coexisting ureteropelvic junction obstruction and vesicoureteral reflux: diagnostic and therapeutic implications. J Urol. 1989;142:490–501. 15. Coplen DE, Austin PF, Yan Y, et al. Correlation of prenatal and postnatal ultrasound findings with the incidence of vesicoureteral reflux in children with fetal renal pelvic dilatation. J Urol. 2008;180:1631– 1634. 16. Nguyen HT, Herndon CDA, Cooper C, et  al. The Society for Fetal Urology consensus statement on the evaluation and management of antenatal hydronephrosis. J Pediatr Urol. 2010;6:212–231. 17. Nguyen HT, Benson CB, Bromley B, et al. Multidisciplinary consensus on the classification of prenatal and postnatal urinary tract dilation (UTD classification system). J Pediatr Urol. 2014;10:982–998. 18. Islek A, Güven AG, Koyun M, et  al. Probability of urinary tract infection in infants with ureteropelvic junction obstruction: is antibacterial prophylaxis really needed? Pediatr Nephrol. 2011;26:1837– 1841. 19. Zee RS, Herndon CDA, Cooper CS, et  al. Time to resolution: a prospective evaluation from the society for fetal urology hydronephrosis registry. J Pediatr Urol. 2017;13:316.e1–316.e5. 20. Heyman S, Duckett JW. The extraction factor: an estimate of single kidney function in children during routine radionuclide renography with 99m-technetium diethylenetriaminepentaacetic acid. J Urol. 1988;140:780–783. 21. Chung S, Majd M, Rushton HG, et  al. Diuretic renography in the evaluation of neonatal hydronephrosis: is it reliable? J Urol. 1993;150:765–768. 22. Conway JJ. “Well-tempered” diuresis renography: its historical development, physiological and technical pitfalls, and standardized technique protocol. Semin Nucl Med. 1992;22:74–84. 23. Grattan-Smith JD, Little SB, Jones RA. MR urography evaluation of obstructive uropathy. Pediatr Radiol. 2008;38:49–69. 24. Chua ME, Ming JM, Farhat WA. Magnetic resonance urography in the pediatric population: a clinical perspective. Pediatr Radiol. 2016;46:791–795. 25. Veenboer PW, de Jong TPVM. Antegrade pressure measurement as a diagnostic tool in modern pediatric urology. World J Urol. 2011;29:737–741. 26. Rushton HG, Salem Y, Belman AB, et al. Pediatric pyeloplasty: is routine retrograde pyelography necessary? J Urol. 1994;152:604–606. 27. Ulman I, Jayanthi VR, Koff S. The long-term followup of newborns with severe unilateral hydronephrosis initially treated nonoperatively. J Urol. 2000;164:1101–1105. 28. Ross SS, Kardos S, Krill A, et  al. Observation of infants with SFU Grades 3-4 hydronephrosis: worsening drainage with serial diuresis renography indicates surgical intervention and helps prevent loss of renal function. J Pediatr Urol. 2011;7:266–271. 29. Arora S, Yadav P, Kumar M, et al. Predictors for the need of surgery in antenatally detected hydronephrosis due to UPJ obstruction--A prospective multivariate analysis. J Pediatr Urol. 2015;11:248.e1– 248.e5. 30. Kelley JC, White JT, Goetz JT, et al. Sonographic renal parenchymal measurements for the evaluation and management of ureteropelvic junction obstruction in children. Front Pediatr. 2016;4:42. 31. Palmer LS, Maizels M, Cartwright PC, et  al. Surgery versus observation for managing obstructive grade 3 to 4 unilateral hydronephrosis: a report from the Society for Fetal Urology. J Urol. 1998;159:222–228. 32. Yiee J, Wilcox D. Management of fetal hydronephrosis. Pediatr Nephrol. 2008;23:347–353. 33. Ulman I, Jayanthi VR, Koff SA. The long-term followup of newborns with severe unilateral hydronephrosis initially treated nonoperatively. J Urol. 2000;164:1101–1105. 34. Cartwright PC, Duckett JW, Keating MA, et  al. Managing apparent ureteropelvic junction obstruction in the newborn. J Urol. 1992;148:1224–1228. 35. Atar A, Oktar T, Kucukgergin C, et al. The roles of serum and urinary carbohydrate antigen 19-9 in the management of patients with antenatal hydronephrosis. J Pediatr Urol. 2015;11:133.e1–133.e5. 36. Gawłowska-Marciniak A, Niedzielski JK. Evaluation of TGF-β1, CCL5/RANTES and sFas/Apo-1 urine concentration in children with ureteropelvic junction obstruction. Arch Med Sci. 2013;9:888–894.

54 • Ureteral Obstruction and Malformations 37. Lee RS. Biomarkers for pediatric urological disease. Curr Opin Urol. 2009;19:397–401. 38. Foley FEB. A new plastic operation for stricture at the uretero-pelvic junction. Report of 20 operations. J Urol 2002. 1937;167:1075– 1096. 39. Culp OS, DeWeerd JH. A pelvic flap operation for certain types of ureteropelvic obstruction; preliminary report. Proc Staff Meet Mayo Clin. 1951;26:483–488. 40. Austin PF, Cain MP, Rink RC. Nephrostomy tube drainage with pyeloplasty: is it necessarily a bad choice? J Urol. 2000;163:1528– 1530. 41. Casale P, Mucksavage P, Resnick M, et  al. Robotic ureterocalicostomy in the pediatric population. J Urol. 2008;180:2643–2648. 42. Tanaka ST, Grantham J, Thomas JC, et al. A comparison of open vs. Laparoscopic pediatric pyeloplasty using the pediatric health information system database-do benefits of laparoscopic approach recede at younger ages. J Urol. 2008;180:1479–1485. 43. Duckett JW, Gibbons MD, Cromie WJ. An anterior extraperitoneal muscle-splitting approach for pediatric renal surgery. J Urol. 1980;123:79–80. 44. Orland SM, Snyder HM, Duckett JW. The dorsal lumbotomy incision in pediatric urological surgery. J Urol. 1987;138:963–966. 45. Lindgren BW, Hagerty J, Meyer T, et al. Robot-assisted laparoscopic reoperative repair for failed pyeloplasty in children: a safe and highly effective treatment option. J Urol. 2012;188:932–937. 46. Davis TD, Burns AS, Corbett ST, et  al. Reoperative robotic pyeloplasty in children. J Pediatr Urol. 2016;12:394.e1–394.e7. 47. Nakada SY, Johnson M. Ureteropelvic junction obstruction. Retrograde endopyelotomy. Urol Clin North Am. 2000;27:677–684. 48. Streem SB. Percutaneous endopyelotomy. Urol Clin North A. 2000;27:685–693. 49. Kim EH, Tanagho YS, Traxel EJ, et al. Endopyelotomy for pediatric ureteropelvic junction obstruction: a review of our 25-year experience. J Urol. 2012;188:1628–1633. 50. Peters CA, Schlussel RN, Retik AB. Pediatric laparoscopic dismembered pyeloplasty. J Urol. 1995;153:1962–1965. 51. Tan HL. Laparoscopic Anderson-Hynes dismembered pyeloplasty in children. J Urol. 1999;162:1045–1048. 52. Cascio S, Tien A, Chee W, et  al. Laparoscopic dismembered pyeloplasty in children younger than 2 years. J Urol. 2007;177:335–338. 53. Minnillo BJ, Cruz JAS, Sayao RH, et  al. Long-term experience and outcomes of robotic assisted laparoscopic pyeloplasty in children and young adults. J Urol. 2011;185:1455–1460. 54. Lam PN, Wong C, Mulholland TL, et al. Pediatric laparoscopic pyeloplasty: 4-year experience. J Endourol. 2007;21:1467–1472. 55. Silay MS, Spinoit AF, Undre S, et  al. Global minimally invasive pyeloplasty study in children: results from the pediatric urology expert group of the european association of urology young academic urologists working party. J Pediatr Urol. 2016;12:229.e1– 229.e7. 56. Ossandon F, Androulakakis P, Ransley PG. Surgical problems in pelvioureteral junction obstruction of the lower moiety in incomplete duplex systems. J Urol. 1981;125:871–872. 57. van den Hoek J, de Jong A, Scheepe J, et  al. Prolonged follow-up after paediatric pyeloplasty: are repeat scans necessary? BJU Int. 2007;100:1150–1152. 58. Cost NG, Prieto JC, Wilcox DT. Screening ultrasound in follow-up after pediatric pyeloplasty. Urology. 2010;76:175–179. 59. Rickard M, Braga LH, Oliveria JP, et  al. Percent improvement in renal pelvis antero-posterior diameter (PI-APD): prospective validation and further exploration of cut-off values that predict success after pediatric pyeloplasty supporting safe monitoring with ultrasound alone. J Pediatr Urol. 2016;12:228.e1–228.e6. 60. Mackie GG, Awang H, Stephens FD. The ureteric orifice: the embryologic key to radiologic status of duplex kidneys. J Pediatr Surg. 1975;10:473–481. 61. Schlussel R, Retik AB. Chapter 58, Ectopic ureter, ureterocele, and other anomalies of the ureter. In: Weizer A, Kavoussi LR, Novick A, eds. Campbell-Walsh Urol. 9th ed. Philadelphia: WB Saunders; 2009:3383–3387. 62. Klauber GT, Reid EC. Inverted Y reduplication of the ureter. J Urol. 1972;107:362–364. 63. Meyer R. Normal and abnormal development of the ureter in the human embryo; A mechanistic consideration. Anat Rec. 1946;96:355–371.

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64. Fehrenbaker LG, Kelalis PP, Stickler GB. Vesicoureteral reflux and ureteral duplication in children. J Urol. 1972;107:862–864. 65. Barrett DM, Malek RS, Kelalis PP. Problems and solutions in surgical treatment of 100 consecutive ureteral duplications in children. J Urol. 1975;114:126–130. 66. Shelfo SW, Keller MS, Weiss RM. Ipsilateral pyeloureterostomy for managing lower pole reflux with associated ureteropelvic junction obstruction in duplex systems. J Urol. 1997;157:1420– 1422. 67. Kohri K, Nagai N, Kaneko S, et al. Bilateral trifid ureters associated with fused kidney, ureterovesical stenosis, left cryptorchidism and angioma of the bladder. J Urol. 1978;120:249–250. 68. Zaontz MR, Maizels M. Type I ureteral triplication: an extension of the Weigert-Meyer law. J Urol. 1985;134:949–950. 69. Finkel LI, Watts FB, Corbett DP. Ureteral triplication with a ureterocele. Pediatr Radiol. 1983;13:346–348. 70. Soderdahl DW, Shiraki IW, Schamber DT. Bilateral ureteral quadruplication. J Urol. 1976;116:255–256. 71. Considine J. Retrocaval ureter. A review of the literature with a report on two new cases followed for fifteen years and two years respectively. Br J Urol. 1966;38:412–423. 72. Hollinshead W. Anatomy for Surgeons. 3rd ed. Philadelphia: Harper and Row; 1982. 73. Zhang XD, Hou SK, Zhu JH, et al. Diagnosis and treatment of retrocaval ureter. Eur Urol. 1990;18:207–210. 74. Castillo OA, Cabrera W, Aleman E, et al. Pieloplastia laparoscópica: técnica y resultados en 80 procedimientos consecutivos. Actas Urológicas Españolas. 2014;38:103–108. 75. Smith KM, Shrivastava D, Ravish IR, et  al. Robot-assisted laparoscopic ureteroureterostomy for proximal ureteral obstructions in children. J Pediatr Urol. 2009;5:475–479. 76. Hellström M, Hjälmås K, Jacobsson B, et al. Normal ureteral diameter in infancy and childhood. Acta Radiol Diagn (Stockh). 1985;26:433– 439. 77. Khoury AE, Bagli DJ. Reflux and megaureter. In: Kavoussi LR, Novick A, Partin A, eds. Campbell-Walsh Urol. 9th ed. Philadelphia: WB Saunders; 2007:3423–3481. 78. Leibowitz S, Bodian M. A study of the vesical ganglia in children and the relationship to the megaureter megacystis syndrome and Hirschsprung’s disease. J Clin Pathol. 1963;16:342–350. 79. Tanagho EA. Embryologic basis for lower ureteral anomalies: a hypothesis. Urology. 1976;7:451–464. 80. Boyd SD, Raz S, Ehrlich RM. Diabetes insipidus and nonobstructive dilation of urinary tract. Urology. 1980;16:266–269. 81. Kass EJ, Silver TM, Konnak JW, et  al. The urographic findings in acute pyelonephritis: non-obstructive hydronephrosis. J Urol. 1976;116:544–546. 82. Mollard P, Foray P, De Godoy JL, et  al. Management of primary obstructive megaureter without reflux in neonates. Eur Urol. 1993;24:505–510. 83. Cozzi F, Madonna L, Maggi E, et al. Management of primary megaureter in infancy. J Pediatr Surg. 1993;28:1031–1033. 84. Keating MA, Escala J, Snyder HM, et al. Changing concepts in management of primary obstructive megaureter. J Urol. 1989;142:636– 640. 85. Baskin LS, Zderic SA, Snyder HM, et al. Primary dilated megaureter: long-term followup. J Urol. 1994;152:618–621. 86. Hendren WH. Operative repair of megaureter in children. J Urol. 1969;101:491–507. 87. Hendren WH. Commentary: surgery of megaureter. In: Whitehead D, Leiter E, eds. Curr Oper Urol. Philadelphia: Harper and Row; 1984:473–482. 88. Fretz PC, Austin JC, Cooper CS, et  al. Long-term outcome analysis of Starr plication for primary obstructive megaureters. J Urol. 2004;172:703–705. 89. Perdzyński W, Kaliciński ZH. Long-term results after megaureter folding in children. J Pediatr Surg. 1996;31:1211–1217. 90. McLorie GA, Jayanthi VR, Kinahan TJ, et al. A modified extravesical technique for megaureter repair. Br J Urol. 1994;74:715–719. 91. Peters CA, Mandell J, Lebowitz RL, et  al. Congenital obstructed megaureters in early infancy: diagnosis and treatment. J Urol. 1989;142:641–645. 92. Lee S, Akbal C, Kaefer M. Refluxing ureteral reimplant as temporary treatment of obstructive megaureter in neonate and infant. J Urol. 2005;173:1357–1360.

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93. Kaefer M, Misseri R, Frank E, et  al. Refluxing ureteral reimplantation: a logical method for managing neonatal UVJ obstruction. J Pediatr Urol. 2014;10:824–830. 94. Christman MS, Kasturi S, Lambert SM, et  al. Endoscopic management and the role of double stenting for primary obstructive megaureters. J Urol. 2012;187:1018–1022. 95. García-Aparicio L, Rodo J, Krauel L, et  al. High pressure balloon dilation of the ureterovesical junction -- First line approach to treat primary obstructive megaureter? J Urol. 2012;187:1834– 1838. 96. Capozza N, Torino G, Nappo S, et al. Primary obstructive megaureter in infants: our experience with endoscopic balloon dilation and cutting balloon ureterotomy. J Endourol. 2015;29:1–5. 97. Bujons A, Saldaña L, Caffaratti J, et al. Can endoscopic balloon dilation for primary obstructive megaureter be effective in a long-term follow-up? J Pediatr Urol. 2015;11:37.e1–37.e6. 98. García-Aparicio L, Blázquez-Gómez E, de Haro I, et  al. Postoperative vesicoureteral reflux after high-pressure balloon dilation of the ureterovesical junction in primary obstructive megaureter. Incidence, management and predisposing factors. World J Urol. 2015;33:2103–2106. 99. Schulman CC. [Ectopic implantations of the ureter]. Acta Urol Belg. 1972;40:201–478. 100. Johnston JH, Davenport TJ. The single ectopic ureter. Br J Urol. 1969;41:428–433. 101. Kreissl MC, Lorenz R, Ohnheiser G, et al. Dystopic dysplastic kidney with ectopic ureter: improved localization by fusion of MR urography and (99m)Tc-DMSA SPECT datasets. Pediatr Radiol. 2008;38:241– 244. 102. Wyly JB, Lebowitz RL. Refluxing urethral ectopic ureters: recognition by the cyclic voiding cystourethrogram. AJR Am J Roentgenol. 1984;142:1263–1267. 103. Berrocal T, López-Pereira P, Arjonilla A, et al. Anomalies of the distal ureter, bladder, and urethra in children: embryologic, radiologic, and pathologic features. Radiographics. 2002;22:1139–1164. 104. Lashley DB, McAleer IM, Kaplan GW. Ipsilateral ureteroureterostomy for the treatment of vesicoureteral reflux or obstruction associated with complete ureteral duplication. J Urol. 2001;165:552–554. 105. Plaire JC, Pope JC, Kropp BP, et al. Management of ectopic ureters: experience with the upper tract approach. J Urol. 1997;158:1245– 1247. 106. Romao RLP, Figueroa V, Pippi Salle JL, et al. Laparoscopic ureteral ligation (clipping): a novel, simple procedure for pediatric urinary incontinence due to ectopic ureters associated with non-functioning upper pole renal moieties. J Pediatr Urol. 2014;10:1089–1094. 107. Prieto J, Ziada A, Baker L, et  al. Ureteroureterostomy via inguinal incision for ectopic ureters and ureteroceles without ipsilateral lower pole reflux. J Urol. 2009;181:1844–1850. 108. Storm DW, Modi A, Jayanthi VR. Laparoscopic ipsilateral ureteroureterostomy in the management of ureteral ectopia in infants and children. J Pediatr Urol. 2011;7:529–533. 109. McLeod DJ, Alpert SA, Ural Z, et  al. Ureteroureterostomy irrespective of ureteral size or upper pole function: a single center experience. J Pediatr Urol. 2014;10:616–619. 110. Bansal D, Cost NG, Bean CM, et  al. Infant robot-assisted laparoscopic upper urinary tract reconstructive surgery. J Pediatr Urol. 2014;10:869–874.

111. Arlen AM, Broderick KM, Travers C, et  al. Outcomes of complex robot-assisted extravesical ureteral reimplantation in the pediatric population. J Pediatr Urol. 2016;12:169.e1–169.e6. 112. Noseworthy J, Persky L. Spectrum of bilateral ureteral ectopia. Urology. 1982;19:489–494. 113. Chwalla R. The process of formation of cystic dilations of the vesical end of the ureter and of diverticula at the ureteral ostium. Urol Cutan Rev. 1927;31:499. 114. Coplen DE, Austin PF. Outcome analysis of prenatally detected ureteroceles associated with multicystic dysplasia. J Urol. 2004;172:1637–1639. 115. Glassberg KI, Braren V, Duckett JW, et  al. Suggested terminology for duplex systems, ectopic ureters and ureteroceles. J Urol. 1984;132:1153–1154. 116. Caldamone AA, Snyder HM, Duckett JW. Ureteroceles in children: follow-up of management with upper tract approach. J Urol. 1984;131:1130–1132. 117. Cooper CS, Passerini-Glazel G, Hutcheson JC, et al. Long-term followup of endoscopic incision of ureteroceles: intravesical versus extravesical. J Urol. 2000;164:1097–1100. 118. Husmann DA, Ewalt DH, Glenski WJ, et  al. Ureterocele associated with ureteral duplication and a nonfunctioning upper pole segment: management by partial nephroureterectomy alone. J Urol. 1995;154:723–726. 119. en S, Beasley SW, Ahmed S, et al. Renal function and vesicoureteric reflux in children with ureteroceles. Pediatr Surg Int. 1992;7:192– 194. 120. Sumfest JM, Burns MW, Mitchell ME. Pseudoureterocele: potential for misdiagnosis of an ectopic ureter as a ureterocele. Br J Urol. 1995;75:401–405. 121. Coplen DE, Barthold JS. Controversies in the management of ectopic ureteroceles. Urology. 2000;56:665–668. 122. Scherz HC, Kaplan GW, Packer MG, et al. Ectopic ureteroceles: surgical management with preservation of continence -- Review of 60 cases. J Urol. 1989;142:538–541. 123. Shekarriz B, Upadhyay J, Fleming P, et  al. Long-term outcome based on the initial surgical approach to ureterocele. J Urol. 1999;162:1072–1076. 124. Rickwood AM, Reiner I, Jones M, et  al. Current management of duplex-system ureteroceles: experience with 41 patients. Br J Urol. 1992;70:196–200. 125. Tank ES. Experience with endoscopic incision and open unroofing of ureteroceles. J Urol. 1986;136:241–242. 126. Hagg MJ, Mourachov PV, Snyder HM, et al. The modern endoscopic approach to ureterocele. J Urol. 2000;163:940–943. 127. Husmann D, Strand B, Ewalt D, et  al. Management of ectopic ureterocele associated with renal duplication: a comparison of partial nephrectomy and endoscopic decompression. J Urol. 1999;162:1406–1409. 128. Monfort G, Guys JM, Coquet M, et al. Surgical management of duplex ureteroceles. J Pediatr Surg. 1992;27:634–638. 129. Snyder HM, Johnston JH. Orthotopic ureteroceles in children. J Urol. 1978;119:543–546. 130. Haddad J, Meenakshi-Sundaram B, Rademaker N, et al. “Watering can” ureterocele puncture technique leads to decreased rates of de novo vesicoureteral reflux and subsequent surgery with durable results. Urology. 2017:1–5.

55

Urinary Tract Infections and Vesicoureteral Reflux W. ROBERT DEFOOR JR., EUGENE MINEVICH, and CURTIS A. SHELDON

Urinary Tract Infections Urinary tract infections (UTIs) are a common and significant source of morbidity in children. By age 7, approximately 8% of girls and 2% of boys will have had at least one UTI.1,2 Children who have had at least one UTI are at risk for having another one.3 The long-term sequelae include renal scarring, hypertension, chronic renal insufficiency, and pregnancy-related complications. Predisposing risk factors for UTIs include renal and bladder structural abnormalities as well as functional bladder and bowel dysfunction.4 UTIs in children are a significant health burden and have been estimated to result in at least 13,000 hospital admissions, with inpatient costs exceeding $180 million per year in the United States.5

DIAGNOSIS Localized clinical signs and symptoms are important clues in the diagnosis of a UTI and depend on the age of the child. Constellations of findings can be more useful than individual ones in identifying affected children.6 For example, neonates rarely present with symptoms specific to the urinary tract. Nonspecific symptoms of lethargy, irritability, temperature instability, anorexia, emesis, or jaundice predominate. Bacteremia is common with neonatal UTIs, and a urine culture is an important aspect in the evaluation of neonatal sepsis.7 Confirmation of a UTI by microscopic examination and a quantitative culture of a properly collected specimen is important. Older infants may present with fever, nonspecific abdominal discomfort, emesis, diarrhea, and poor weight gain including failure to thrive. Malodorous or cloudy urine may be reported by the parents, but this is not always accurate as an isolated symptom to rule in/out a diagnosis of UTI.8 Older children frequently present with dysuria and urinary frequency, urgency, and enuresis. Table 55.1 outlines the incidence of UTI symptoms as a function of age.9,10 As the symptoms can sometimes be nonspecific, it is important that care providers have a high index of suspicion in ill-appearing children. An unexplained high fever in an infant or toddler should prompt the clinician to obtain a urine sample. Analysis of a properly collected urine sample is the cornerstone in the diagnosis of UTI.11 Errors in diagnosis most commonly result from failure to confirm a clinically suspected UTI by culture, or by reliance on a specimen that has been inadequately collected or mishandled. Specimens may be obtained by bag collection, clean catch, urethral catheterization, and suprapubic aspiration. Although invasive,

urethral catheterization or suprapubic aspiration offer the lowest risk of false-positive results.12 The results of a bag specimen or clean-catch specimen in a non-toilet-trained child are helpful to exclude a UTI if negative.13 Bag specimens can be useful in an infant with a history of UTIs or structural abnormalities in whom a fever is present, but the suspicion for a UTI is otherwise low. Positive findings should be confirmed using a catheter or aspiration specimen unless the clinical presentation and laboratory findings are unequivocal. The accuracy of positive findings from a bag specimen in an infant has been estimated at 7.5%,14 whereas those from a midstream clean-catch specimen varies with age: 42% 105 colony-forming units per milliliter of a single bacterial species. The accuracy of such a positive finding on culture is estimated at 80% (single specimen) and 96% (confirmed by second culture).16 Table 55.2 outlines the probability of infection as a function of colony count and the methods of collection that are used in children.17 However, one must avoid applying these criteria too Table 55.1  Presenting Symptoms in 200 Children With Urinary Tract Infection as a Function of Age Age Symptom Failure to thrive, poor feeding Jaundice Screaming, irritability Foul-smelling, cloudy urine Diarrhea Vomiting Fever Convulsions Hematuria Frequency, dysuria Enuresis Abdominal pain Loin pain Male-to-female ratio

0–1 Months

1–24 Months

2–5 Years

5–12 Years

53%

36%

7%

0

44% 0 0 18% 24% 11% 2% 0 0 0 0 0 1:2

0 13% 9% 16% 29% 38% 7% 7% 4% 0 0 0 1:13

0 7% 13% 0 16% 57% 9% 16% 34% 27% 23% 0 1:10

0 0 0 0 3% 50% 5% 6% 41% 29% 44% 12% 1:10

From Smellie JM, Hodson CJ. Edwards D, et al. Clinical and radiological features of urinary tract infection in childhood. BMJ. 1964;2:1222; Bickerton MW. Duckett JW. Urinary tract infections in pediatric patients. AUA Update Service, Lesson 26. 1985;4:4.

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Table 55.2  Criteria for Diagnosis of Urinary Tract Infections Method of Collection Suprapubic aspiration

Catheterization

Clean voided (male) Clean voided (female)

Colony Count (Pure Culture)

Probability of Infection

Gram-negative bacilli: any number Gram-positive cocci: a few thousand >105 104–105 103–104 105 Three specimens >105 Two specimens >105 One specimen >105

>99% >99% 95% Likely Suggestive Unlikely Likely 95% 90% 80%

Modified from Hellerstein S. Recurrent urinary tract infection in children. Pediatr Infect Dis. 1982;1:275.

strictly. The colony count varies as a function of hydration (dilution) and urinary frequency (bacterial multiplication time). One study of six untreated children with proven bacteriuria found colony counts varied from 103 to 108 over a 24-hour period.18 Although it is traditionally felt to be the most accurate laboratory test, an immediate diagnosis is not possible from a urine culture. Thus, the initial treatment is generally guided by the urinalysis. Microscopic evaluation of a urine specimen should be performed immediately on collection. This practice minimizes misleading ex  vivo bacterial multiplication and deterioration of cellular elements. The identification of bacteria in an unspun urine specimen is very suggestive of significant bacteriuria.18 Pyuria (>10 leukocytes/mm3) is suggestive19 but can also be seen in vaginitis, dehydration, calculi, trauma, chemical irritation, gastroenteritis, and viral immunization. Urinary Gram stain has been found to be reliable in detecting UTIs in young infants.20 A popular and indirect measurement of bacteriuria employs nitrite and leukocyte esterase analysis. Nitrate, normally present in urine, is converted to nitrite in the presence of bacteria. A positive nitrite reaction is indicative of bacteria, with a specificity and positive predictive value approaching 100%.21 The nitrate-to-nitrite reaction requires a relatively long incubation period. Thus, urinary frequency and hydration may produce a false-negative result. Inadequate dietary nitrate and infection caused by nitrite-negative organisms can also cause false-negative reactions.22 The combination of nitrite and leukocyte esterase is more sensitive and specific than either alone.23 Overall, the combination of dipstick analysis and microscopic examination for bacteria has a sensitivity and negative predictive value approaching 100%.21 

CLASSIFICATION Classification of UTIs helps to determine the need for hospital admission and parenteral antibiotic therapy as opposed to outpatient oral antibiotic therapy. An attempt is made to distinguish between upper tract (pyelonephritis) and lower tract infections (cystitis). Fever, flank pain and/or

A

B

Fig. 55.1  Technetium-99m dimercaptosuccinic acid (DMSA) scan. (A) The magnified view of the left kidney, seen by using a pinhole collimator, demonstrates defects in both poles that extend deep into the renal parenchyma (arrows), suggestive of acute pyelonephritis. (B) The right kidney has an upper pole defect (arrow) that may represent either acute or chronic pyelonephritis. (Courtesy Michael J. Gelfand, MD.)

tenderness, and leukocytosis suggest pyelonephritis and require antibiotics to minimize the risk of renal injury. Laboratory studies designed to distinguish a lower tract from an upper tract UTI include antibody-coated bacteria assay, β2-microglobulin excretion, antibodies to Tamm– Horsfall protein, and urinary lactic dehydrogenase assay and procalcitonin.24,25 These tests are not sufficiently reliable for routine clinical use and may not be universally available. Newer biomarkers including neutrophil gelatinase associated lipocalin (NGAL) have been found to be useful in identifying acute pyelonephritis in children with febrile UTIs.26 Direct culture by ureteral catheterization or percutaneous puncture is reliable, although cumbersome, and represents an option in complicated clinical situations. A quite useful study for localizing infection to the kidney is a radioisotope renal cortical scan (e.g., technetium-99m dimercaptosuccinic acid [DMSA]) during the initial presentation of the patient with a documented infection (Fig. 55.1). Unfortunately, there have been difficulties obtaining this agent in the United States in recent years. Technetium-99m mercaptoacetyltriglycine (MAG3) is an alternative radioisotope that can be used to assess renal function and drainage patterns. Another important consideration regarding classification is the distinction between re-infection and persistent infection. Re-infection with a new organism is very common. Persistent UTI with the same organism, although less common, is important, as it implies either an ineffective antimicrobial therapy or a structural abnormality, such as a urinary tract calculus or ureteral obstruction. 

EPIDEMIOLOGY Figure 55.2 outlines the age- and gender-related incidence of UTIs. At all ages, except for the neonatal period, the incidence of UTI is greater in females than in males. In both males and females, the incidence increases with advanced age. Although the male has one early peak in the newborn period, the female has two peaks, one at 3–6 years and the other at the onset of sexual activity. The actual incidence of infection as a function of age and gender is difficult to determine from the literature. Table 55.3 summarizes the available data.17 

55 • Urinary Tract Infections and Vesicoureteral Reflux

10.0

% incidence

8.0 Married, nonpregnant

6.0 4.0

Unmarried

2.0

Females Males

Nuns

0 0

10

20

30

40

50

60

70

80

Age in years Fig. 55.2  The age and gender distribution of urinary tract infection incidence. (From Devine CJ, Stecker JF. Urology in Practice. Boston: Little, Brown; 1978. p. 444.)

Table 55.3  Incidence of Urinary Tract Infection as a Function of Age, Gender, and Presence of Symptoms Symptomatic (%) Age Newborn Preschool School age

Male

Asymptomatic (%)

Female Male 0.15

0.7

Female 1.0–1.4a

2.8

0.2 0.03

0.8 1.0–2.0

a2.4–3.4%

in premature infants. Data compiled from multiple sources by Hellerstein S. Recurrent urinary tract infections in children. Pediatr Infect Dis. 1982;1:271.

855

cycle.31 High intravesical pressure may also potentiate infection in children. In the absence of an elevated residual urine, uninhibited bladder contractions are associated with an increased risk of recurrent UTI, which may be lessened by anticholinergic therapy.32 Dysfunctional elimination syndrome with abnormal voiding habits and constipation can affect the development of UTI as well.33 The acidic pH of urine, as well as its osmolality, further discourages bacterial growth.34 The uroepithelial cells of healthy individuals suppress bacterial growth and are capable of killing bacteria. The uroepithelial cells secrete a mucopolysaccharide substance that, on coating the surface of the uroepithelium, provides an additional barrier to uroepithelial adherence.31 Glycosaminoglycans are continuously shed and thus function to entrap and eliminate bacteria. Abnormalities at the ureterovesical junction (UVJ) and altered ureteral peristalsis may allow vesicoureteral reflux (VUR), which potentiates but is not always necessary for upper tract invasion. Distortion of the pyramids allows renal parenchymal invasion, which results in irreversible renal injury. The anatomy of the renal papillae helps prevent intrarenal reflux (IRR) (Fig. 55.4).10 Structural abnormalities that potentiate infection include phimosis, obstructive uropathy at any level (e.g., ureteropelvic [UPJ] and UVJ obstructions, posterior urethral valves [PUV]), VUR, bladder diverticula, urinary calculi or foreign bodies, and the renal papillary anatomy. 

Bacterial Factors Several bacterial factors may potentiate a UTI and are outlined in Box 55.1.10 O antigens are lipopolysaccharides that are part of the cell wall. They are thought to be responsible

PATHOPHYSIOLOGY POTENTIATING

PROTECTIVE

Urolithiasis Intrarenal reflux 1. Compound papillae 2. Acquired

Unobstructed urine transport Unidirectional urine flow

Operative antimicrobial urothelial activity Regular, complete bladder emptying Normal perineal resistance

Vesicoureteral reflux Ascent of infection

Host Factors The establishment of clinical infection and its consequent injury to the urinary tract results from a complex interplay between host resistance and bacterial virulence. Generally, UTI-causing organisms originate from the feces of their host. Conceptually, four levels of defense are identifiable: periurethral, bladder, ureterovesical junction, and renal papillae.10 These concepts are illustrated in Figure 55.3. Bacteria generally possess an ability to adhere to vaginal mucosal cells in order to readily establish infection.27 The resultant periurethral colonization then allows replication and migration, which ultimately lead to transurethral invasion to the bladder. Healthy girls have low bacterial colonizations of the periurethral region. Girls prone to UTIs experience greater colonization, especially prior to a new episode of UTI. Moreover, the cultured organism from the introital region belongs to the same strain as that from the urine during the UTI that ensues. Periurethral bacterial colonization is correspondingly low in UTI patients after resolution of recurrent UTIs.28 A similar mechanism may apply to bacterial adherence in the prepuce of males.29 This may explain why 92% of male infants 20 mg/dL.105 These infants benefit from parathyroidectomy with transplantation of one gland and were traditionally managed this way, but newer literature supports the use of potent intravenous bisphosphonates.105,106 This entity must be differentiated from transient neonatal hyperparathyroidism that occurs secondary to maternal hypocalcemia. Familial hypocalciuric hypercalcemia is an inherited autosomal dominant disorder caused by a heterozygous mutation in the Ca2+-sensing receptor gene, resulting in a relatively asymptomatic form of neonatal severe hyperparathyroidism.105,107 It differs from primary hypoparathyroidism in that the PTH value is normal but urinary excretion of calcium is low. Patients are usually asymptomatic with an elevated serum calcium level. This disorder can usually be managed medically. 

75 • Endocrine Disorders and Tumors

Secondary Hyperparathyroidism Secondary hyperparathyroidism occurs in children with renal insufficiency resulting in reduced renal vitamin D activation and therefore decreased GI absorption of calcium. It can also be seen in children with malabsorption. Hypocalcemia is worsened due to a reduced renal excretion of phosphate and the resulting excess serum phosphate binding to calcium, which decreases active calcium levels in the serum. The resulting hypocalcemia triggers PTH production, which in turn chronically overstimulates the parathyroid glands and causes four-gland hyperplasia.105 Medical treatment focuses on decreasing intestinal phosphorus absorption, and patients typically respond well. In rare cases, severe renal osteodystrophy can result in skeletal fractures and metastatic calcifications. These markedly severe cases of secondary hyperparathyroidism may benefit from total parathyroidectomy with autotransplantation.94  Tertiary Hyperparathyroidism Tertiary hyperparathyroidism occurs when the parathyroid glands are exposed to prolonged stimulation from long-term hypocalcemia and begin autonomous PTH production, even after the inciting stimulus has been removed. After the calcium levels rise to normal or higher, the glands continue autonomous PTH production because they have lost their response to the negative feedback loop. This can be seen in chronic renal failure patients and a very small percentage of secondary hyperparathyroidism patients who undergo renal transplantation.108 As tertiary hyperparathyroidism is commonly due to hyperplasia of all four glands, these children should be offered total parathyroidectomy with autotransplantation. 

Adrenal Glands EMBRYOLOGY The adrenal glands are composed of the cortex and medulla. Embryologically the cortex is composed of both permanent cortex and fetal cortex. The primordial adrenal cortex arises from mesoderm as a groove in the coelom between the base of the mesentery medially and the mesonephros and undifferentiated gonad laterally.109 This explains the association of ectopic adrenal tissue with testes (see Fig. 50.29) or ovaries, and sometimes an infrarenal location. The adrenal cortex becomes visible between weeks 4 and 6 of gestation after initial proliferation. Subsequently it undergoes differentiation into the inner fetal zone and the outer permanent zone between weeks 8 and 10 of gestation.110 The fetal cortex, whose function is unknown, makes the fetal adrenal gland four times the size of the fetal kidney at the fourth month of gestation. At birth, the adrenal gland remains proportionally large. However, the fetal cortex decreases in size within hours of birth and is completely gone by the first year of life. The permanent cortex is arranged into three separate zones: the zona glomerulosa, zona fasciculata, and zona reticularis.111 The zona glomerulosa gives rise to the narrowed zona fasciculata and reticularis of the adult cortex. The zona reticularis does not complete development into the adult form until late childhood.112

1227

The adrenal medulla is an ectodermal derivative and develops from the migrating neural crest cells that also form the sympathetic trunk, the sympathetic plexuses, and the paraganglia. In primitive vertebrates, the cortex and medulla remain independent and are fused only in mammals. In humans, they are fused but remain distinct, with the ectodermal tissue enclosed by mesodermal tissue.109 Anomalous locations of adrenal glands can be seen due to the migratory nature of the cortex and medulla during embryogenesis. The gland may be in its normal location, but under the capsule of the kidney (adrenal–renal heterotopia) or capsule of the liver (adrenal–hepatic heterotopia). Alternatively, extra-adrenal tissue (adrenal rest) can be found anywhere in the abdomen, but typically is located along the anatomic derivatives of the urogenital ridge for the adrenal cortex (even on autopsy the ovaries or testes), and along the dorsal root ganglia for the medullary tissue. In an autopsy study, 16% of the patients had complete accessory adrenal glands, and another 16% had accessory adrenal glands that lacked a medullary component.113 

PHYSIOLOGY The adrenal cortex produces three major hormones: aldosterone, cortisol, and androgens. The zona glomerulosa lacks the enzyme 17α-hydroxylase, which is necessary to produce the precursors to cortisol and androgens, and therefore only produces aldosterone. Cortisol, androgens, and small amounts of estrogens are produced by the zona fasciculata and zona reticularis. These areas lack the enzymes necessary to produce the precursors to aldosterone.

Aldosterone Extracellular fluid volume, sodium, and potassium balance are regulated by aldosterone, which in turn is released in response to the renin–angiotensin system. The juxtaglomerular cells in the kidney secrete renin as a response to decreased pressure in the renal afferent arterioles and decreased plasma concentration detected by the macula densa. Renin converts angiotensinogen into angiotensin I in the liver, which is subsequently converted to angiotensin II by the angiotensin converting enzyme in the lung. Angiotensin II is a potent vasoconstrictor and causes release of aldosterone by directly stimulating the zona glomerulosa. The renal tubular reabsorption of sodium in exchange for potassium and hydrogen is then upregulated by aldosterone, resulting in increased renal fluid resorption and expanded intravascular volume.  Cortisol Cortisol-releasing factor (CRF) is released by the hypothalamus and is responsible for cortisol regulation. CRF stimulates pituitary release of adrenocorticotropic hormone (ACTH). Cortisol has far-reaching physiologic effects including stimulation of hepatic gluconeogenesis, inhibition of protein synthesis, increased protein catabolism, and lipolysis of adipose tissue. Negative effects of cortisol include loss of collagen, decreased fibroblast activity resulting in inhibition of wound healing, and induction of a negative calcium balance leading to osteoporosis. 

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Box 75.3  Differential Diagnosis of an Adrenal Mass

Box 75.4  Etiology of Cushing Syndrome: Exogenous Corticosteroid Administration

Functioning tumors Adrenal adenoma Adrenocortical carcinoma Pheochromocytoma Nonfunctioning tumors Neuroblastoma Adrenal cyst Hemangioma Leiomyoma Leiomyosarcoma Non-Hodgkin lymphoma Malignant melanoma Metastatic disease to the adrenal gland Squamous cell carcinoma of the lung Hepatocellular carcinoma Breast cancer Traumatic adrenal hemorrhage Neonatal child abuse

ACTH-dependent causes Cushing disease (pituitary adenoma) Ectopic ACTH production Small cell bronchogenic carcinoma Carcinoid tumors Pancreatic islet cell carcinoma Thymoma Medullary thyroid carcinoma Pheochromocytoma ACTH-independent causes Adrenal adenoma Adrenocortical carcinoma Adrenal hyperplasia Administration of ACTH

Androgens Adrenal androgens include dehydroepiandrosterone (DHEA) and DHEA sulfate (DHEA-S). After systemic release by the adrenal gland, these hormones are converted to the biologically active forms testosterone and dihydrotestosterone. Adrenal androgens account for 90% of CAH. 

ADRENAL MASSES The differential diagnosis of adrenal masses is listed in Box 75.3. More than 90% of adrenal masses in children are neuroblastomas. However, the incidental finding of an adrenal mass in children who have undergone crosssectional imaging for other conditions is of unknown significance. Adrenal masses are detected in fewer than 1% of patients younger than age 30 years at autopsy,114 and this increases to 7% in patients older than 70 years. The finding of an incidentally discovered adrenal mass should prompt hormone evaluation, including a 1 mg dexamethasone suppression test, aldosterone levels, and measurement of plasma free metanephrines.114 All functioning adrenal cortical tumors and pheochromocytomas should undergo resection. In children, most surgeons will resect adrenal tumors regardless of size, especially with laparoscopic adrenalectomy becoming more common (Fig. 75.10). However, there is no clear evidence to support this management over conservative therapy, especially in lesions smaller than 3 cm.115–117 

ADRENAL CORTEX Hypercortisolism (Cushing Syndrome) Hypercortisolism, or Cushing syndrome, results from systemic glucocorticoid excess. It can be caused by ACTH secreting pituitary adenomas, hormonally active adrenal tumors including carcinoma and adenoma, ectopic ACTH syndrome, nodular adrenal hyperplasia, and ACTHproducing tumors (Box 75.4). Additionally, iatrogenic Cushing syndrome is the most common cause of hypercortisolism in adults and children, and is due to exogenous administration of supraphysiologic amounts of ACTH or glucocorticoids over a prolonged time period. On the other hand, Cushing disease is caused by a pituitary adenoma, which is the second most common cause of Cushing syndrome in children. Tumors that can produce ACTH are rare in children and include pulmonary neoplasms, neuroblastomas, pancreatic islet cell carcinomas, thymomas, carcinoids, MTCs, and pheochromocytomas. In children, ectopic ACTH is most commonly due to a bronchial carcinoid, with presenting ACTH levels usually 10–100 times higher than in Cushing disease. Such significantly elevated levels of ACTH result in hypokalemic alkalosis. Hypercortisolism can also be due to ACTH-independent multinodular adrenal hyperplasia, which is characterized by hypersecretion of both cortisol and adrenal androgens.118 Hypercortisolism is more common in children than previously recognized.119 The most common cause of Cushing syndrome in children younger than 7 years is an adrenal tumor (Fig. 75.11). In those older than 7 years, adrenal hyperplasia secondary to hypersecretion of pituitary ACTH is most common. The clinical features of Cushing syndrome can take 5 years or longer to develop. Thus, the classic Cushingoid appearance may not be seen in children. Weight gain and growth failure are the most common and the most reliable findings.120 Therefore, any obese child who stops growing should be evaluated for Cushing syndrome, which consists of laboratory screening followed by localization if the labs suggests a source.118,121 Laboratory evaluation involves measuring the plasma cortisol to coincide with the diurnal variation: at 8:00 a.m. (normal levels, 6 cm tumors and invasive tumors. They also recommend open resection for paragangliomas except for small, noninvasive tumors in surgically favorable locations.168 Although the data in children are currently limited to small series, laparoscopic adrenalectomy and resection of paragangliomas appear equally safe and effective in experienced hands, and is becoming the standard approach (Fig. 75.13).117,129,130,171,172 Up to 40% of pediatric paragangliomas are familial, with the majority (75%) having detectable germline mutations.173–176 This finding has prompted some authors to recommend routine genetic analysis in children with paragangliomas. Germline mutations in the following genes have been associated with pheochromocytoma: VHL (von Hippel–Lindau syndrome), NF1 (von Recklinghausen neurofibromatosis type 1), RET (MEN 2A or 2B), and SDHD and SDHB (familial paragangliomas associated with gene mutations of the mitochondrial succinate dehydrogenase family).163,177 Pheochromocytomas are more frequently associated with MEN 2 syndromes in children than in adults. In children, they are more likely to be bilateral and benign. Controversy exists over management of patients with bilateral pheochromocytoma. Bilateral adrenalectomy is associated with significant morbidity secondary to corticosteroid replacement as well as significant complications of medication noncompliance, such as Addisonian crises.165 Cortical-sparing adrenalectomies are recommended by the Endocrine Society CPG for patients with bilateral tumors and those at high risk for developing a metachronous contralateral lesion.168,178 131I-MIBG scanning and chemotherapy may be effective for patients with malignant disease. Current evidence

75 • Endocrine Disorders and Tumors

A

1233

B

Fig. 75.13  A teenage boy was found to have marked hypertension and a pheochromocytoma after a metabolic evaluation. He underwent laparoscopic left adrenalectomy and tolerated the procedure nicely. (A) The pheochromocytoma (asterisk) is seen lying on the cephalad portion of the left kidney. The tumor was removed uneventfully. (B) On the cut section of the adrenal gland, the tumor can be seen to be nicely demarcated from the normal adrenal gland.

supports high initial doses of 131I-MIBG for all patients with metastatic lesions who have positive diagnostic 131I-MIBG scans. Survival is 4.7 years after treatment when tumors respond symptomatically or hormonally to treatment.179 Stable disease related to tumor volume and partial hormonal response was achieved in 40–50% of patients in a contemporary systematic review and meta-analysis, although this response may also be related in part to the natural history of the disease.180 Chemotherapy seems to have an additive effect with 131I-MIBG scanning to improve survival in these patients,181 and has been found to have a partial response in terms of tumor volume and catecholamine excess.182 Data relating to children are limited. The European Society of Endocrinology recommends lifelong annual follow-up with assaying plasma or urinary metanephrines for all children with paragangliomas.183 

Precocious Puberty Precocious puberty is defined as the development of secondary sexual characteristics before age 9 years in boys. In girls, the development of breasts (thelarche) before age 7.5 years, the development of pubic hair (pubarche) before age 8.5 years, or the onset of menses (menarche) before age 9.5 years is considered precocious. However, there is significant controversy regarding these definitions in girls.184–188 Central precocious puberty results from early pulsatile gonadotropin-releasing hormone (GnRH) release. In most cases it is idiopathic, but it can result from hyothalamic tumors. Peripheral precocious puberty results from adrenal or gonadal tumors or exogenous exposure to sex steroids, and can lead to central precocious puberty (central GnRH release) as well.189,190 Recent literature has shown autonomous gonadal activation can be caused by specific somatic or germline mutations both in central and peripheral precocious puberty.191

PRECOCIOUS PUBERTY IN GIRLS True precocious puberty, resulting from premature activation of the hypothalamic–pituitary axis, is idiopathic in

most girls and is rare (1:5000–10,000) in American girls. Neurogenic disorders such as hydrocephalus, cerebral palsy, trauma, irradiation, chronic inflammatory disorders, or tumors, such as hypothalamic hamartomas or pineal tumors, can cause true precocious puberty. The mechanism is related to interference with inhibitory signals to the hypothalamus, or the production of excitatory signals. Patients with McCune–Albright syndrome have the classic triad of precocious puberty, café-au-lait nevi with irregular “coast of Maine” borders, and polyostotic fibrous dysplasia. In these patients, precocious puberty is caused by autonomously functioning ovarian follicular cysts. Acromegaly, Cushing syndrome, and hyperthyroidism have also been associated with this syndrome.192 Incomplete precocious puberty is seen with the development of isolated signs of secondary development such as thelarche or pubarche. Prepubertal vaginal bleeding is usually caused by a foreign body, sexual abuse, or tumors of the genital tract and requires further investigation because isolated prepubertal menses are rare. Incomplete precocious puberty can be a normal variant of development or can be due to the production of hormones from neuroendocrine, adrenal, ovarian, or exogenous sources. In Van Wyk– Grumbach syndrome, which is a combination of hypothyroidism, precocious puberty, and ovarian cysts, growth is inhibited rather than stimulated.193,194 

PRECOCIOUS PUBERTY IN BOYS In boys, true precocious puberty is more often neurogenic than in girls, with 50–70% being neurogenic and the remainder idiopathic.190 A hamartoma of the tuber cinereum is the most common neurogenic tumor that causes male precocious puberty. These are ectopic hypothalamic lesions connected to the posterior hypothalamus. This is a high-risk location for resection, and these tumors are nonprogressive in nature. Consequently, they are generally treated with GnRH agonists. Other disorders causing precocious puberty in boys are gliomas of the optic nerve or hypothalamus, astrocytomas, choriocarcinomas, meningiomas, rhabdomyosarcomas, neurofibrosarcomas,

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nonlymphocytic leukemia, ependymomas, neurofibromatosis type 1, and germinomas.195 True precocious puberty can also be caused by other space-occupying lesions or causes of increased intracranial pressure such as head trauma, suprasellar cysts, granulomas, brain irradiation, and hydrocephalus. Congenital adrenal hyperplasia, caused by lack of the 21-hydroxylase or the 11-hydroxylase enzymes, result in excess androgen production causing virilization. In untreated patients, ACTH stimulation can cause enlargement of embryologic adrenal rests found in the testes, and increased secretion of androgens. Interstitial cell tumors of the testes may also be responsible for increased androgen production. Finally, exogenous administration of androgens or human chorionic gonadotropin (for treatment of undescended testes) can cause precocious puberty.190 

EVALUATION Evaluation of precocious puberty begins with a thorough history and physical examination including height, weight, bone age, and growth curve evaluation. Determining pathology versus extreme variants of normal depends on correlating the bone age and height age. If the bone age and the height age correlate closely, it is likely the presenting symptom is a variant of normal growth. A repeat evaluation in 6 months is important. Further workup must be obtained, however, if the bone age is abnormally accelerated relative to the height age. Documentation of tanner stage, size, and shape of the testes and evaluation for gynecomastia in boys should be performed. In girls, Tanner stage should also be documented as well along with careful examination for breast development, especially in obese girls, to avoid overestimating the breast size. Evaluation of the testes in boys is critical because, in true precocious puberty, the testes generally enlarge symmetrically, whereas asymmetric or nodular enlargement is noted with Leydig cell tumors or adrenal rests.190 All patients should have skin examinations evaluating for facial acne, oily skin, or café-au-lait spots. Laboratory workup includes evaluation of serum estradiol, testosterone, and DHEA levels. Adrenal tumors typically have significantly elevated DHEA levels. A GnRH test will help differentiate complete or incomplete precocious puberty. Patients with true precocious puberty respond to GnRH with a typical pubertal pattern.196 Alternatively, a sleep-related increase in plasma luteinizing hormone (LH) levels can be diagnostic but is more cumbersome to obtain. Increased basal or stimulated LH secretion is the biochemical basis of diagnosis for some practitioners.186 In patients with feminizing or masculinizing features, US is useful to locate abdominal, pelvic, or testicular masses. MRI is recommended in patients with true precocious puberty to evaluate for intracranial lesions. 

TREATMENT In general, tumors causing precocious puberty should be removed if they are surgically accessible. There are a number of agents used to medically treat this problem. When puberty starts before the age of 6 years in girls, treatment is recommended to preserve height potential. Other criteria for

treatment include significant bone age advancement (the bone age is more advanced than what would be expected for the patient’s chronologic age), decreased predicted height prognosis, and for psychosocial and behavioral reasons.186 True (gonadotropin-dependent) precocious puberty treatment is with long-acting GnRH agonists including deslorelin, buserelin, nafarelin, leuprolide, and triptorelin.197 Incomplete precocious puberty is treated with medroxyprogesterone, ketoconazole, testolactone, and androgen antagonists. The progression of secondary sexual characteristics and menstruation can be halted by medroxyprogesterone. Ketoconazole inhibits the synthesis of testosterone by blocking the conversion of 17-hydroxyprogesterone to androstenedione, and testolactone prevents conversion of androgens to estrogens by competitively inhibiting the aromatase enzyme responsible for that conversion. Therapy is stopped based on bone age, chronologic age, height, and rate of growth.186 

Carcinoid Tumors Carcinoid tumors comprise 120% of the 95th percentile) have been used to refer to increasing grades of excess weight in children.8 Whereas more than 32% of adults in the United States are obese, 18% of children and adolescents are obese—a prevalence that has more than tripled in the last two decades.1 Currently, approximately 9% of adolescents meet the definition of severe obesity,21 which is problematic particularly because metabolic and health risks mount with increasing severity of obesity.22–24 In addition, longitudinal analysis of data from our study data shows that essentially all adolescents and most children with a BMI in the severely obese range will continue to be obese as adults.22 In addition, in a recent study, it was found that surgical treatment was associated with a 50% reduction in obesity-related mortality risk.25 Thus, bariatric surgery is considered a reasonable option for weight control and longterm health improvement in severely obese adolescents.  1240

National Institutes of Health (NIH) guidelines suggest that it is reasonable to consider weight loss surgery for adults with a BMI of 35 kg/m2 or greater in the presence of severe obesity related comorbidities or 40 kg/m2 or greater with or without comorbidities.35 Similarly, in adolescents with a BMI ≥35 kg/m2 and major comorbid conditions such as type 2 diabetes mellitus (DM), obstructive sleep apnea (OSA), severe nonalcoholic steatohepatitis, or symptomatic pseudotumor cerebri, surgery may be an appropriate initial treatment option. Surgery is also considered a reasonable first treatment option for those adolescents with a BMI of 40 kg/m2 or greater with other weight related comorbidities or risk factors that are responsive to weight loss (e.g., hypertension, mild OSA, glucose intolerance, obesity-related renal dysfunction, or dyslipidemia), functional impairment, or quality of life (QOL) impairment.36 Figure 76.1 outlines a suggested algorithm for management. 

BARIATRIC PROGRAMS FOR ADOLESCENTS For highly motivated adolescents who meet patient selection criteria (Box 76.2) following unsuccessful prior attempts at weight loss, bariatric surgery should be considered a treatment option. Youth being considered for bariatric surgical procedures should be referred to a specialized center with a multidisciplinary bariatric team with pediatric expertise. Such a team is equipped to manage the sometimes difficult patient selection decisions and can provide appropriate follow-up and management of the

76 • Bariatric Surgical Procedures in Adolescence

unique challenges posed by the severely obese adolescent. Guidelines have been established by the American College of Surgeons that define such multidisciplinary bariatric teams, which include expertise in obesity evaluation

Box 76.1  Selected Comorbidities of Adolescent Obesity Complication of Pediatric Obesity

Psychosocial

Poor self-esteem Depression Eating disorders Discrimination and prejudice Quality of life Sexual abuse Pseudotumor cerebri Sleep apnea, asthma, and exercise intolerance Dyslipidemia Hypertension Coagulopathy Chronic inflammation Endothelial dysfunction Gallstones Nonalcoholic fatty liver disease Glomerulosclerosis Type 2 diabetes mellitus Insulin resistance Polycystic ovary syndrome Slipped capital femoral epiphysis Blount’s disease Forearm fractures Flat feet

Neurologic Pulmonary Cardiovascular

Gastrointestinal Renal Endocrine Musculoskeletal

and management, psychology, nutrition, physical activity, and bariatric surgical treatment.37 Depending on the individual needs of the adolescent, additional expertise in developmental pediatrics, adolescent medicine, endocrinology, pulmonology, gastroenterology, cardiology, orthopedics, social work, and ethics should be readily available. In programs dedicated to adolescent bariatric care, the patient review process is similar to that used in the multidisciplinary oncology and transplant programs.38,39 This review by a panel of experts from various disciplines results in specific treatment recommendations for individual patients, including appropriateness and timing of possible operative intervention based on patient understanding, compliance, family dynamics, and psychosocial support. 

FACTORS INFLUENCING TIMING OF SURGERY Physical Maturation The timing for operative treatment of severely obese adolescents remains controversial and depends on the compelling health needs of the patient. However, certain physiologic factors should be considered when planning the operative treatment. There is a theoretical concern about the impact of significant caloric restriction on attainment of their genetically predetermined goal adult stature. Physiologic maturation is generally complete by the time of sexual maturation, Tanner stage 3 or 4.40 Skeletal maturation (adult stature) is normally attained by the age of 13–14 years in girls and 15–16 years in boys.41,42 Overweight children generally experience accelerated onset of puberty. As a result, they are likely to be taller and have advanced bone age compared with age-matched lean children.

Adolescent unsuccessful with prior weight management

BMI ≥ 35

No Continue behavioral approaches

BMI ≥ 40

Yes No

No

Yes

Severe comorbidity*?

No

Severe or less severe comorbidity*? Yes

No

Continue behavioral approaches

Yes

Good surgical candidate**?

*See Box 76.2 **See Box 76.3

1241

No

Yes Consider specific bariatric surgical options

Fig. 76.1  Algorithm for management of the severely obese adolescent. BMI, Body mass index.

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Holcomb and Ashcraft’s Pediatric Surgery

Box 76.2  Clinical Indications for Adolescent Bariatric Surgery DOCUMENTED OUTCOME AFTER ADOLESCENT BARIATRIC SURGERY (REFERENCES) Serious Comorbidities □ Type 2 diabetes mellitus □ Obstructive sleep apnea □ Pseudotumor cerebri □ Severe nonalcoholic steatohepatitis (with NASH [nonalcoholic steatohepatitis] activity score [NAS] ≥4 or with presence of fibrosis) Less Serious Comorbidities □ Pre-diabetes (HbA1c 5.7–6.4%) □ Hypertension □ Dyslipidemias □ Fatty liver disease (any) □ Significant impairment in activities of daily living □ Stress urinary incontinence □ Gastroesophageal reflux disease □ Weight-related arthropathies that impair physical activity □ Weight-related quality-of life-impairment

If uncertainty exists about whether adult stature has been attained, skeletal maturation (bone age) can be objectively assessed with a radiograph of the hand and wrist.43 If an individual has attained >95% of skeletal maturation, little concern exists that an intervention will significantly impair completion of linear growth.44 Importantly, there is no evidence to suggest that commonly used bariatric procedures in preadolescent youth impairs attainment of adult stature. Indeed, to the contrary, data exist demonstrating that linear growth does occur in preadolescent youth following vertical sleeve gastrectomy (VSG).45 

Psychological Maturation Adolescent psychological development also influences the ability to participate in surgical decision-making and postoperative dietary compliance. At any given age, adolescents are at varying stages of cognitive, psychosocial, and physiologic maturity. The more mature adolescent who can reason and think abstractly is able to consider the consequences of taking or not taking nutritional supplements or of adhering to prescribed medical and nutritional regimens that are necessary for lifelong success (e.g., maintenance of weight loss and prevention of avoidable nutritional complications) after bariatric operations.46 The use of weight loss surgery in individuals with cognitive impairment remains controversial. There are data suggesting weight loss surgery is safe and effective in these individuals.45,47 However, long-term outcomes, in regard to adverse events and weight loss durability, remain to be determined. Before any decision for surgical treatment is made, all candidates should undergo a comprehensive psychological evaluation. Goals of this evaluation are (1) to determine the level of cognitive and psychosocial development, primarily to judge the extent to which the adolescent is capable of participating in the decision to

(12, 96, 98) (47, 99–101) (88, 102) (103, 104) (12, 47, 105) (12, 13, 96) (12, 47) (104) (106) (107) (108–110)

proceed with the intervention; (2) to identify past and present psychiatric, emotional, behavioral, or eating disorders; (3) to define potential support for, or barriers to, regimen compliance and family readiness for the required lifestyle changes (particularly if one or both parents are obese); (4) to assess reasoning and problemsolving ability; (5) to assess whether reasonable outcome expectations exist; (6) to assess family unit stability and identify psychological stressors or conflicts within the family; (7) to determine whether the adolescent is autonomously motivated to consider bariatric surgical treatment or whether coercion is present; and (8) to assess weight-related QOL status. A complete psychological assessment is helpful for team decision-making. With this type of comprehensive assessment, the team is generally able to reach agreement about whether obstacles exist that need focused attention prior to proceeding to surgery (Box 76.3). 

Surgical Options In 1991, the NIH Bariatric Consensus Development Conference established parameters that led to a more uniform use of bariatric surgical procedures for adults. At that time, insufficient data existed to make recommendations about bariatric surgical treatment for patients younger than 18 years. In 2006, the NIH funded a landmark study of adolescent bariatric outcomes to allow investigators to document beneficial effects of surgery in youth, and to objectively describe the risks of surgery when used in adolescence. The multicenter research consortium called Teen-Longitudinal Assessment of Bariatric Surgery now includes surgical and nonsurgical investigators at the Children’s Hospital of Colorado, Cincinnati Children’s Hospital, Texas Children’s Hospital, Children’s Hospital of Alabama, Nationwide Children’s Hospital, and the University of Pittsburgh. In 2016

76 • Bariatric Surgical Procedures in Adolescence

Box 76.3  Favorable Attributes of Adolescent Bariatric Candidate □ □ □ □ □ □ □ □ □ □ □ □

 atient is motivated and has good insight. P Patient has realistic expectations. Family support and commitment is present. Patient is compliant with health care commitments. Family and patient understand that long-term lifestyle changes are needed. Agrees to long-term follow-up. Decisional capacity is present. Well documented and at least temporarily successful weight loss attempts. No major psychiatric disorders that may complicate postoperative regiment adherence. No major conduct/behavioral problems. No substance abuse in preceding year. No plans for pregnancy in upcoming 2 years

the consortium was awarded a third cycle of federal funding to enable follow-up of the entire cohort to at least 10 years postoperatively, to examine the durability of the beneficial effects of operative therapy, and to assess possible late untoward effects. Of the many procedures that have been advocated for weight loss, the operations that have been used most frequently are shown in Figure 76.2 and include laparoscopic roux-en-Y gastric bypass (RYGB), laparoscopic adjustable gastric banding (AGB), and laparoscopic VSG. With RYGB and VSG, there is evidence of numerous mechanisms contributing to significant weight loss such as increased postprandial peptide YY concentrations promoting satiety.48 Weight loss following AGB is more modest compared with RYGB and VSG, and it never achieved U.S. Food and Drug Administration approval for use in adolescents contributing to the low number of procedures done with this technique. The VSG is currently the most commonly used procedure in adults and adolescents (Figs. 76.3 and 76.4). In this procedure, most of the fundus and body of the stomach are removed to create a tube-like stomach based on the poorly distensible lesser curve. It is a less complex procedure than RYGB and an appealing first option when considering weight loss surgery because it produces similar weight loss to RYGB with fewer operative risks.12,49–51 In addition, VSG has predictably fewer adverse effects on micronutrient absorption, a finding confirmed with measurement of blood vitamin levels.12 RYGB consists of both a small gastric pouch and a malabsorptive component (Figs. 76.5–76.7). The proximal gastric pouch is separated from the remaining stomach remnant and attached to a roux limb of intestine with a length of 75–150 cm. Moreover, it also offers an additional negative reinforcement of “dumping syndrome” in some patients, providing excellent weight loss in adolescents.12 The partial biliopancreatic bypass with duodenal switch is a primarily malabsorptive procedure that results in good weight loss for adults with the highest classes of obesity (generally >60 BMI), but at the expense of higher risks of operative complications and postoperative nutritional risks. This duodenal switch procedure is generally not recommended in

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adolescents due to the increased risk of complications and the lack of data about safety in this younger age group. 

Perioperative and Surgical Management PREOPERATIVE EDUCATION AND MANAGEMENT The multidisciplinary preoperative evaluation that leads to the decision to offer surgical treatment is followed by considerable patient and family preoperative education. It is important that this process is organized and not rushed because patients must comprehend a great deal of information about anatomic and physiologic changes that occur following the operation that impact the success and risks of the short- and long-term complications. Detailed information about the options for various surgical procedure(s), nursing care, dietary strategies, physical activity, and behavioral approaches to support adherence to the postoperative regimen is provided. Patients may also benefit from discussion with others who have undergone operative management. In the weeks before the operation, a final outpatient visit for anesthesiology consultation, final informed permission (consent) discussion, and final review of the postoperative regimen is scheduled. At the conclusion of this visit, some programs require the patient to take a written test, which is scored and reviewed with the patient as further documentation of his or her level of understanding of the procedure and known and potential adverse and beneficial consequences. During the evaluation of a potential surgical patient, studies include serum chemistry and liver profile, lipid profile, complete blood count, hemoglobin A1C, fasting blood glucose, and Helicobacter pylori antigen fecal titers (if indicated). Micronutrient screening is performed including 25-hydroxyvitamin D, vitamin A, and iron studies. If these are low, patients are given vitamin D and iron supplementation in addition to a multivitamin.52 An electrocardiogram is obtained to screen for cardiac problems and dysrhythmias. For instance, prolonged QT syndrome can exist in morbidly obese adolescent patients and may be previously unrecognized. Because unrecognized sleep disorders are relatively prevalent in the severely obese, a complete sleep history is sought, including a history of snoring, irregular breathing, and increased daytime somnolence. A history suggestive of sleep apnea should prompt formal polysomnography. If the fasting glucose is elevated, patients should undergo a 2-hour glucose tolerance test to determine whether more significant abnormalities of carbohydrate metabolism exist, including type 2 DM. On the day before operation, the patient is limited to clear liquids. Preoperative medications include low-molecularweight heparin (40 mg injected subcutaneously and continued daily postoperatively), and a second-generation cephalosporin antibiotic is administered within 1 hour of operation. Sequential compression boots also are used intraoperatively and postoperatively. Most patients are candidates for laparoscopic procedures, while some of the heavier and centrally obese patients may be challenging laparoscopic cases, particularly in the early portion of a surgeon’s learning curve. 

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A

C

B

D

100 cm

Fig. 76.2  Operative procedures for weight loss that are performed laparoscopically. (A) Adjustable gastric band (AGB). (B) Vertical sleeve gastrectomy (VSG). (C) Roux-en-Y gastric bypass (RYGB). (D) Duodenal switch operation.

GENERAL ASPECTS OF SURGICAL TECHNIQUES FOR THE MORBIDLY OBESE In general, open laparotomy should be avoided in morbidly obese individuals due to difficulty with adequate visualization and the higher risk of wound complications. For initial abdominal access, we have found that a laparoscopically guided technique using a bladeless, optical 12-mm trocar to be safe and efficient. Alternatively, some surgeons prefer a blind puncture using the Veress needle in the left upper quadrant. To access the gastroesophageal junction for bariatric procedures, a wide variety of surgical instruments

have been manufactured with the morbidly obese patient in mind. It should be noted, however, that the majority of laparoscopic procedures in morbidly obese individuals can be efficiently and safely accomplished using standard adult 5-mm instrumentation. The left lobe of the liver can be retracted and elevated with a locking clamp placed in a port at the xyphoid and clamped to the right crus, or the Nathanson retractor (Cook Surgical, Bloomington, IN) can be used to expose the stomach. The details and nuances of the procedures used for adolescents can be obtained from a variety of other excellent bariatric texts. 

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76 • Bariatric Surgical Procedures in Adolescence

Small triangular portion left behind

Gastric tube

Greater omentum

Tape

6 cm

Fig. 76.4  The completed sleeve gastrectomy is seen. Note that a small triangle of stomach is left near the gastroesophageal junction to help prevent any inadvertent resection or encroachment on the esophagus.

A

Ultrasonic scalpel

Grasper

Stomach

Gore peristrip

Staples

Biliopancreatic limb

Duodenum Right colon

Jejunum Stapler

B

Stapler

Fig. 76.3  (A) For the VSG, the greater omental attachments and the short gastric vessels are taken down along the entire extent of the greater curvature of the stomach using the ultrasonic scalpel. This dissection is started 6 cm from the end of the pylorus. (B) The endoscopic stapler, first using a green load and then a blue load, is used to ligate and divide the stomach. A 34 French bougie is inserted along the lesser curve of the stomach to act as a guide for resection.

Roux limb

Fig. 76.5  For the RYGB, a jejunostomy is created with a linear stapler connecting a 50-cm biliopancreatic limb and 100–150 cm roux limb as shown. The mesenteric defect is then closed to prevent herniation of bowel through this space.

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Oesophagus

Gastrojejunostomy

EEA anvil New stomach pouch Gastric pouch

EEA stapler

Roux limb Stomach remnant Bypassed portion of stomach A

EEA anastomosis

Stapler

B Fig. 76.6  (A) A 30-mL gastric pouch is created along the lesser curve of the stomach just beyond the gastroesophageal junction using a linear cutting stapler with the anvil of the 25-mm EEA stapler in place. The Roux limb is brought anterior to the colon and gastric remnant. The end of the Roux limb is then opened and the EEA stapler is introduced, extended, and then mated with the anvil as shown to perform the gastrojejunostomy. (B) The open end of the roux limb is then stapled closed, thus completing the gastrojejunostomy.

Postoperative Management Postoperatively, the patients are typically placed in a monitored, nonintensive care unit setting, and maintenance fluids are administered at a rate of 75 mL/h. Early warning signs of complications include fever, tachycardia, tachypnea, increasing oxygen requirement, oliguria, hiccoughs, regurgitation, left shoulder pain, worsening abdominal pain, a feeling of anxiety, or acute alteration in mental status. These signs warrant aggressive attention and appropriate investigation because they may signal a postoperative complication such as a gastrointestinal leak, bleeding, pulmonary embolus, bowel obstruction, or acute dilation and

Jejunojejunostomy

Fig. 76.7  The Roux-en-Y gastric bypass consists of both a restrictive pouch size and a mildly malabsorptive component (the bypass of the stomach and duodenum). The gastric pouch is based on the lesser curve and is created using a 34 French orogastric tube as a guide. A 75–150 cm Roux limb is used in most patients.

impending rupture of the bypassed gastric remnant. The reported rate of major complications varies between 5% and 8%.12,53 During a surgeon’s initial experience, we recommend obtaining a water-soluble upper gastrointestinal contrast study on postoperative day 1 (Fig. 76.8). After passage of the contrast without a leak is documented, patients are begun on clear liquids and subsequently advanced to a high-protein liquid diet. Bariatric surgical treatment reduces appetite, and intake is markedly reduced as well. Due to reduced intake of food items rich in essential fatty acids, vitamins, and other specific nutrients, the diet must be prescribed by a qualified dietitian. Nutritional and metabolic consequences of bariatric surgical procedures have been well delineated in adults.54–65 To avoid nutritional complications, patients must adhere to guidelines for diet and vitamin/mineral supplementation. Gastric bypass essentially results in a surgically enforced very low-calorie, low-carbohydrate dietary intake, thus requiring attention to an adequate (1 g/kg ideal weight) daily protein intake to minimize lean mass loss during the rapid weight-loss phase. Impaired absorption of iron, folate, calcium, vitamin D, and vitamin B12 can occur after gastric bypass.57,66,67 Some obese adolescents have vitamin deficiency prior to operative intervention,68,69 and even with postoperative supplementation, severe deficiencies may occur. Adolescents also may be at particular risk for thiamine deficiency.70 B12 deficiency is of specific concern, as it may lead to irreversible myeloneuropathy

76 • Bariatric Surgical Procedures in Adolescence

A

1247

B

Fig. 76.8  This anteroposterior projection of contrast study (A) obtained on postoperative day 1 shows an anatomically correct, narrow lumen of the stomach after sleeve gastrectomy. The operative photograph (B) was taken the day before in the same patient and demonstrates a technically correct remnant stomach after sleeve gastrectomy. Note the straightness of staple line without torsion or irregularity.

if left untreated.71 In addition, poor postoperative compliance among adolescents who have undergone bariatric operations has been reported with the main reasons for noncompliance cited as difficulty swallowing or forgetting to take the medication.72,73 Because certain micronutrient deficiencies, such as folate and calcium, have established ramifications for the patient and potential offspring, both warrant special consideration. Folate is a water-soluble B vitamin that is essential for growth, cell differentiation and embryonic morphogenesis, gene regulation, repair, and host defense.74 Adequate maternal periconceptional folic acid consumption during critical periods of organ formation early in the first trimester may reduce the likelihood of fetal malformations including neural tube defects and perinatal complications such as low birth weight, prematurity, and placental abruption and infarction. These facts are particularly relevant because the majority of adolescents seeking bariatric surgical treatment are females, many of whom will want to be mothers in the future. Thus, physicians caring for adolescents who undergo bariatric surgical procedures must stress the importance of daily folate and other B-complex vitamin intake. Moreover, patients should be monitored for serum vitamin levels, particularly when uncertainty about compliance exists.74 There are no studies examining outcomes of pregnancy after bariatric surgical procedures in the adolescent population. However, unplanned teen pregnancy is a legitimate concern following massive weight loss.75,76 There may be an increased risk of unplanned pregnancy in adolescents undergoing bariatric surgical procedures.76 We recommend that all females and caregivers be informed about increased fertility (a physiologic change due to increased insulin sensitivity and resumption of ovulation) and the likelihood for increased risk-taking following surgical weight loss. These patients should be counseled to avoid pregnancy during the 2-year period following operation and should be offered reliable contraception. The modern intrauterine devices

are effective and safe and may be the preferred method of contraception. Additionally, the already elevated risk for venous thromboembolism in this patient population is increased with the use of oral contraception. The intrauterine device can even be placed at the time of the bariatric procedure.77 Adolescence is a period of rapid skeletal mineral accretion and is, therefore, a window of opportunity to influence lifelong bone health, both positively and negatively. It has been demonstrated that although the obese adolescent has higher than average bone mineral density/content, they may well have less than normal bone mineral density and content for their weight.78 This may translate into a greater risk for fractures. Furthermore, impaired accretion of bone mineral content in adolescence increases the risk for osteoporosis and results in a twofold greater risk of fracture in later life.79,80 Given the impaired absorption of both vitamin D and calcium after bariatric surgical procedures and the large individual variation in bone accretion, it is essential to closely monitor the bone mineral density of adolescents undergoing bariatric surgical treatment. Behavioral strategies can and should be used to encourage compliance with postoperative vitamin and mineral intake, which should positively influence nutritional outcomes after adolescent bariatric surgical procedures.80 Adolescent compliance is specifically enhanced by (1) visual aids, (2) focus on immediate benefit from treatment, (3) participation in self-management, (4) self-monitoring, and (5) reinforcement (Box 76.4).81,82 With the alterations in eating patterns required after bariatric surgical procedures, repetitive reinforcement is needed to facilitate the formation of lifelong health-promoting habits. The adolescent bariatric surgical program should build on the best practices of other adolescent disease-management programs. Success will be based on the premise that sustained weight control for the adolescent requires ongoing behavioral intervention, structured family involvement, and continued support.80,81,83,84 

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Long-Term Management Postoperative follow-up visits after bariatric operations in adolescence are essential to monitor weight loss and screen for potential complications of the procedure. We recommend seeing RYGB and VSG patients at 2 weeks, 6 weeks, 3 months, and then every third month for the first year, and additional visits, as necessary. Dietary advancement is a methodical process of introducing new items of gradually increasing complexity over 6 months toward the goal of a well-balanced, small portion (∼1 cup per meal) diet, which includes the daily intake of 0.5–1 g of protein/kg of ideal

Box 76.4  Strategies to Improve Postoperative Compliance □ □

□

□

□

 ietary regimen rehearsal preoperatively enables problem D identification and solving before the surgical intervention. Use of actual measuring cups, a food scale, and photographs of specific food items that are recommended enhances the adolescent’s ability to follow through with plans. Provide the adolescent with a diet diary and exercise diary with form pages for them to fill out, and practice this preoperatively. Provide a list of acceptable food items for every phase of their postoperative recovery (first week, second through fourth week, second through third month, etc.) including the caloric density and protein, carbohydrate, and fat content of the items to encourage label reading. Provide a detailed listing of micronutritional supplements needed postoperatively, which includes the reason why the supplement is needed and the potential consequences of not taking it.

A

weight. Dysphagia warrants a contrast study to evaluate for a complication such as seen in Figure 76.9. Nonsteroidal anti-inflammatory medications should be avoided to reduce the risk of intestinal ulceration and bleeding after gastric bypass. A proton pump inhibitor is prescribed for 6 months in VSG patients. Routine preventive postoperative vitamin and mineral supplementation typically consists of pediatric hard chewable multivitamins, a calcium/vitamin D supplement, a B12 supplement, and an iron supplement for menstruating females. Because of the severity of potential thiamine deficiency, an additional B-complex or B1 vitamin, beyond what is contained in multivitamin preparations, should be given for the first 6 months.85 We routinely reemphasize five basic “rules” with each patient encounter: (1) eat protein first, (2) drink 64–96 ounces of water or sugar-free liquids daily, (3) no snacking between meals, (4) exercise 30–60 minutes per day, and (5) always remember vitamins and minerals. Iron studies and 25-hydroxyvitamin D are obtained postoperatively at 3 months, then every 3–6 months until 1 year, and then yearly. Serum albumin, total protein, alkaline phosphatase, parathyroid hormone, complete blood count, and representative B-complex vitamin levels (e.g., B1, methylmalonic acid level [for B12], folate) are obtained at postoperative 6 and 12 months, and then yearly. Vitamin A levels are obtained postoperatively at 6 and 12 months, and then yearly for RYGB patients.52 Consideration should be given to bone density assessment with dual energy X-ray absorptiometry analysis (DEXA) at even years postoperatively (e.g., year 2, 4, 6, etc.). DEXA allows not only for the measurement of bone mineral density changes but also for the change in fat and lean body mass. 

B

Fig. 76.9  This anteroposterior projection of a contrast study (A) was obtained at postoperative month 3 after sleeve gastrectomy in a patient with dysphagia and heartburn, and shows a proximal pouch (arrow) just beyond the gastroesophageal junction. On the right, the oblique view (B) in the same patient demonstrates the posterior gastric “diverticulum” (arrow), which, at reoperation, was found to be a portion of posterior fundus left in place at the time of the sleeve gastrectomy. At reoperation, this diverticulum was resected, entirely correcting the anatomic defect with resolution of the symptoms.

76 • Bariatric Surgical Procedures in Adolescence

Outcomes in Adolescents

Short- and intermediate-term results of RYGB have been retrospectively reviewed in small series of adolescents with generally satisfactory results in regard to clinically important decreases in weight and BMI.14,72,86–95 The largest prospective, longitudinal study of adolescent bariatric surgery demonstrated that durable weight loss can be achieved.12 This study included 242 patients and demonstrated >25% weight reduction in most participants at 3 years of follow-up. Weight regain to baseline occurred in only 4% of the VSG group and 2% with RYGB at 3 years follow-up. In longer-term follow-up studies, patients with lowest baseline BMI achieved more normal weights than in those with higher baseline BMI, suggesting earlier intervention may be beneficial.13,96,97 Also, operative management resulted in significant long-term improvement and resolution of obesity related comorbidities. The most substantial results were seen in insulin resistance, cardiovascular health (dyslipidemia and hypertension), renal function, and type 2 DM.12,13 96 







Summary

The obesity epidemic in this country has generated a population of adolescents with the premature onset of adult obesity-related disease. Current data is clear that bariatric surgical procedures can achieve significant weight loss. However, it is not yet understood if weight loss or comorbidity resolution after an adolescent bariatric surgical procedure is sustainable over the adolescent’s lifetime, and longer-term risks must still be better defined. Currently we can only apply rational principles of adolescent medicine and evidence from adult bariatric studies to guide the application of these procedures to a group of young patients who have the serious and progressive medical and psychological comorbidity of severe obesity. Given the immediacy of some of the medical and psychosocial complications, the impaired QOL, and the added health care costs of adolescent obesity, it is logical that adolescent bariatric surgical programs be developed to meet these needs. Adolescent bariatric surgical programs should have expertise that enables them to assess and meet the unique medical, cognitive, physiologic, and psychosocial needs of the adolescent. The operations should be performed in centers committed to clinical research and capable of long-term, detailed follow-up and data collection.



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76 • Bariatric Surgical Procedures in Adolescence 88. Sugerman HJ, Sugerman EL, DeMaria EJ, et  al. Bariatric surgery for severely obese adolescents. JGastrointestSurg. 2003;7:102– 108. 89. Inge TH, Garcia VF, Daniels SR, et  al. A multidisciplinary approach to the adolescent bariatric surgical patient. JPediatrSurg. 2004;39:442–447. 90. Anderson AE, Soper RT, Scott DH. Gastric bypass for morbid obesity in children and adolescents. JPediatrSurg. 1980;15:876–881. 91. Breaux CW. Obesity surgery in children. ObesSurg. 1995;5:279– 284. 92. Collins J, Mattar S, Qureshi F, et al. Initial outcomes of laparoscopic Roux-en-Y gastric bypass in morbidly obese adolescents. Surg Obes Relat Dis. 2007;3:147–152. 93. Lawson ML, Kirk S, Mitchell T, et  al. One-year outcomes of Rouxen-Y gastric bypass for morbidly obese adolescents: a multicenter study from the Pediatric Bariatric Study Group. J Pediatr Surg. 2006;41:137–143. 94. Barnett SJ, Stanley C, Hanlon M, et al. Long-term follow-up and the role of surgery in adolescents with morbid obesity. Surg Obes Relat Dis. 2005;1:394–398. 95. Treadwell JR, Sun F, Schoelles K. Systematic review and metaanalysis of bariatric surgery for pediatric obesity. Ann Surg. 2008;248:763–776. 96. Inge TH, Jenkins TM, Xanthakos SA, et  al. Long-term outcomes of bariatric surgery in adolescents with severe obesity (FABS5+): a prospective follow-up analysis. Lancet Diabetes Endocrinol. 2017;5:165–173. 97. Inge TH, Jenkins TM, Zeller M, et  al. Baseline BMI is a strong predictor of nadir BMI after adolescent gastric bypass. J Pediatr. 2010;156:103–108. e1. 98. Inge TH, Miyano G, Bean J, et  al. Reversal of type 2 diabetes mellitus and improvements in cardiovascular risk factorsafter surgical weight loss in adolescents. Pediatrics. 2009;123:214–222. 99. Amin R, Simakajornboon N, Szczesniak R, et al. Early improvement in obstructive sleep apnea and increase in orexin levels after bariatric surgery in adolescents and young adults. Surg Obes Relat Dis. 2017;13:95–100.

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Index A

AAST duodenal injury grades, 244, 245t Abbreviated Injury Scale (ALS), 211 ABCDE criteria, for melanoma, 1136– 1137, 1137t “Abdominal kidney”, 831 Abdominal pain, ureteropelvic junction obstruction, 837 Abdominal pressure, barriers to gastroesophageal reflux, 462 Abdominal radiography, intussusception diagnosis, 622, 622f Abdominal teratomas, 1077–1078 gastric, 1078 retroperitoneal, 1077, 1078f Abdominal trauma, 236–253 blunt aortic, 247 complications of, 248–249 evaluation of, 236–237, 237f, 237t intestinal, 244–247 resuscitation of, 236 follow-up care for, 249–250 injury patterns of, 214 laparoscopy in, 247f, 248 liver injury, management of, 237–242, 239f spleen injury, management of, 237–242, 239f Abdominal wall, prune belly syndrome, 942f, 945 reconstruction, 948, 948f–950f Abernathy syndrome, 1038 Ablation, for Graves disease, 1219 Abscess appendicitis, 673–674, 674f breast, 1210 Absorbable biosynthetic patches, for congenital diaphragmatic hernia, 390, 390f Acalculous cholecystitis, 702 ACC. see American College of Cardiology Accessory sex organs, prune belly syndrome, 944–945, 945f Accessory spleen, 750–751, 752f laparoscopic splenectomy complications, 758 Accidental decannulation, ECMO, 101 Acetaminophen (paracetamol), 51 Acetic acid, of burn wound care, 204t Acetyl-coenzyme A (CoA), 24 Achalasia, 424–425, 424f–425f Acid burns, 206 Acidic injury, 428 Acidosis, in congenital diaphragmatic hernia, 388

Acinar cell carcinoma, 747 Acinus, in lung development, 380 Acquired aganglionosis, 568 Acquired anorectal disorders, 613–620 anal fissure, 613–614 anal skin tags, hemorrhoids, polyps, and other perianal vascular lesions, 614–615, 615f–616f fistula-in-ano, 613, 614f–615f perianal and perirectal abscess, 613, 613f–614f rectal prolapse, 615–618, 616f–617f rectal trauma, 618–619, 618f Acquired asphyxiating thoracic dystrophy, 324 Acquired choledochal cyst, 695, 696f Acquired melanocytic nevi, 1128–1131 management of, 1130–1131 presentation of, 1128–1129 risk factors for, 1129–1130, 1130b Acquired subglottic stenosis, 334, 335f Acquired tracheal stenosis, 334, 335f tracheal repair in, 342–343, 344f ACTH. see Adrenocorticotropic hormone Acticoat, 204t Activated partial thromboplastin time (aPTT), hemostasis evaluation of, 78 Activity restriction, in abdominal and renal trauma, 249 Acute kidney injury (AKI), 60–63 hyperkalemia, 61b medical management of, 60–63 dialysis, 61–63, 63f metabolic acidosis, 61 in neonates, 63–66, 64b clinical presentation of, 64–65 diagnostic evaluation of, 65 intrinsic, 64 management, 65–66 oliguric variety, 64 postrenal, 64 prerenal, 64 pathophysiology of, 60 Acute Physiology and Chronic Health Evaluation (APACHE II), 120–121 Acute scrotum, 821–826 differential diagnoses of, 821b intermittent testicular pain, 824 Acute subdural hemorrhage, 257 Acute suppurative cervical lymphadenitis, 1182–1183, 1182f Acute suppurative thyroiditis, 1218 ACV. see Assist-control ventilation Acyclovir, 720 Adalimumab, 658

Adenocarcinoma Barrett’s esophagitis and, 463 ductal, 747 pancreas, 747 Adenoma, adrenal, 1229 Adenomatosis, 1040 Adenotonsillectomy, sickle cell disease and, 87–88 ADH. see Antidiuretic hormone Adipose tissue tumors, 1089–1090, 1089f–1090f Adjustable gastric band (AGB), 1243, 1244f Adjuvant therapy, cancer chemotherapy, 970–971 Adnexal disease, 1193–1195 etiology of, 1195b Adolescents bariatric programs for, 1240–1241, 1242b ovarian cysts in, 1194–1195 Adoptive immunotherapy, 978 ADPKD. see Autosomal dominant polycystic kidney disease Adrenal adenoma, primary hyperaldosteronism and, 1229 Adrenal glands, 1227–1233 adrenal masses in, 1228, 1228b, 1229f anomalous locations of, 1227 cortex, 1228–1232 adrenocortical carcinoma, 1230– 1232, 1231f hypercortisolism, 1228–1229, 1228b, 1230f primary hyperaldosteronism, 1229–1230 embryology of, 1227 medulla of, 1232–1233 pheochromocytoma, 1232, 1233f physiology of, 1227–1228 Adrenalectomies, cortical-sparing, 1232 Adrenal-hepatic heterotopia, 1227 Adrenal-renal heterotopia, 1227 αdrenergic receptor, 11–12 α-Adrenergic receptor, 11–12 β-Adrenergic receptor, 11–12, 870 Adrenocortical carcinoma, 1230–1232, 1231f Adrenocorticotropic hormone (ACTH), 1227 in congenital adrenal hyperplasia, 954–955 Advanced neuromonitoring, for traumatic brain injury, 264

Note: Page numbers followed by “f” indicate figures, “t” indicate tables, “b” indicate boxes. 1253

1254

Index

Advanced Trauma Life Support (ATLS), 258–259 AEF. see Aortoesophageal fistula AGA. see Appropriate-for-gestational-age AGA (appropriate-for-gestational-age), 2 Aganglionosis acquired, 568 long-segment, 564–566, 567f–568f persistent, 568 AGB. see Adjustable gastric band Age appendicitis, 665 in pectus excavatum, 307, 307f AHA. see American Heart Association Air embolism ECMO, 101–102 in pneumothorax-pulmonary lacerations, 226–227 Air leak, in pneumothorax-pulmonary lacerations, 226 Airway anatomy of, 224 injury of, 230–231, 230f Airway injuries, 337–339 endotracheal intubation in, 338, 339t extrinsic, 338, 338f intrinsic, 337–338 tracheostomy in, 338–339, 339f, 339t Airway pressure release ventilation (ARPV), 119, 119f AKI. see Acute kidney injury Alagille syndrome (angiohepatic dysplasia), 709–710 ALCL. see Anaplastic large cell lymphoma Aldosterone, 1227 Aldrate score, modified, 52t Alimentary tract duplication, 629–640, 638f–639f associated conditions, 629 classification, 630–638 clinical presentation, 629–630, 629f–631f, 632t colonic duplication, 632t, 636–638, 637f diagnosis, 629–630 duodenal duplication, 632t, 634, 634f embryology, 629 esophageal duplication, 630–631, 632t, 633f gastric duplication, 483, 483f, 632t, 634f ileal duplication, 632t imaging, 630, 631f incidence, 629 jejunal duplication, 632t pancreatic duplication, 634 rectal duplication, 632t, 638, 638f small bowel duplication, 635–636, 635f–636f thoracoabdominal duplication, 631–634, 632t, 633f ALK inhibition, in neuroblastoma management, 1027 ALK oncogene, in neuroblastoma, 1010, 1014, 1027 Alkali ingestions, 428 Alkaline phosphatase, in biliary tract rhabdomyosarcoma, 1055

Alkylating agents, cancer chemotherapy, 973t–974t Allergy anesthesia preoperative evaluation in, 37 in pectus repair, 313 Alloderm, 204t Allografts of burn wound care, 204t full-sized allografts, 712 isolated small bowel allograft, 724, 724f rejection, intestinal transplantation, 724–725 variant liver, 712 All-trans-retinoic acid, as anticancer drugs, 973t–974t ALS. see Abbreviated Injury Scale Alveolar rhabdomyosarcoma, 1116, 1116f Alveolar stage, in lung development, 380 Ambulance transport, trauma, 219 Ambulatory surgery, as anesthesia preoperative evaluation, 36–37 American College of Cardiology (ACC), 44 American Heart Association (AHA), 44 American Society of Anesthesiologists (ASA), 35 Amifostine, adverse effects, 972 Amino acids glucose production, 21 protein metabolism and, 21 supplementation, 549 Aminosalicylates, 657 5-Aminosalicylic acid (5-ASA), 650 Amnioreduction, for twin-twin transfusion syndrome, 160 Amniotic membrane, of burn wound care, 204t Amoxicillin posterior urethral valves, 887–888 for urinary tract infections, 858t Amylase levels, acute pancreatitis, 739 Anal canal sensation, 599–600, 600f Anal fissure, 613–614 Anal skin tags, hemorrhoids, polyps, and other perianal vascular lesions, 614–615, 615f–616f Anal strictures, 596 Analgesia acute pancreatitis management, 740–741 discharge, 51–52 hypospadias, 928–930 for pectus excavatum, 308–309 regional, 51 for traumatic brain injury, 261 Analysis, urinary tract infection, 853 Anaplasia, in Wilms tumor, 991–992, 992f Anaplastic large cell lymphoma (ALCL), 1103 Anastomotic leaks, in esophageal atresia, 447 Anastomotic stricture, in esophageal atresia, 447 Anatomic barriers, infections, 141–142 Anatomic reconstruction, bladder exstrophy, 899–900

Anderson-Hynes dismembered technique, 842, 843f Androgens, 1228 deficit, in 46,XY, 955–956 Anemia hemolytic, 5 hemorrhagic, 5 in hepatoblastoma, 1043–1044 in neonates, 5 of prematurity, 5 Anesthesia anterior mediastinal mass of, 41, 42f bladder exstrophy, 900 for congenital heart disease, 41–43 preoperative preparation, 42–43 difficult pediatric airway in, 45, 45t, 46f discharge criteria of, 52, 52t, 52b for former preterm infants, 39–40 for inguinal hernia management, 799–800 intraoperative management of, 46–49 considerations for specific surgical approaches, 46–47 monitoring, 46 thoracoscopy, 47–49, 48f vascular access, 46 morbidity/mortality, 35–36 postanesthesia care of, 49–52 common problems, 49 intraoperative awareness, 49–50 pain management, 50–52 postoperative nausea and vomiting, 49 respiratory complications, 49 preoperative evaluation of, 36–39 ambulatory surgery, 36–37 congenital heart disease, 41–43 endocarditis prophylaxis, 43, 43b fasting guidelines, 38–39 general principles, 37–38, 37b laboratory testing, 39 patient history, 37 trisomy 21, 38 renal transplantation, 728 risk of, 35–36 for upper respiratory tract infection, 39 Anesthetic considerations, for pediatric surgical conditions, 35–56 Angioembolization, in liver and spleen injury, 242, 242f Angiogenesis, dysregulation of, 1148 Angiographic embolization, 1161–1162 Angiohepatic dysplasia. see Alagille syndrome Angioma, tufted, 1153–1154 Angiomyolipomas, 1005 Angiopoietin 1 (Ang-1), 1155 Angiotensin II, 1227 Angle of His, 461 ANGPT1, 1037 ANGPT2, 1037 Ankle fractures, 271 Ann Arbor system, for non-Hodgkin and Hodgkin lymphoma, 406, 1101t Annular pancreas, 737–738 in duodenal atresia/stenosis, 491 Anoplasty, 583, 583f

Index

Anorectal atresia without fistula as female defects, 580 as male defects, 578–579 Anorectal defects, 577–598 associated, 581–582 classification of, 577b female defects, 579–580 anorectal atresia without fistula. see Anorectal atresia without fistula cloacal malformation. see Cloacal malformations rectoperineal fistula. see Rectoperineal fistula rectovestibular fistula. see Rectovestibular fistula vaginal augmentation/replacement, 593–594 vaginal switch maneuver, 593, 593f functional outcomes of, 595–596, 596f constipation, 596 fecal incontinence, 596 historical aspects of, 577 male defects, 577–579 anorectal atresia without fistula. see Anorectal atresia without fistula rectal atresia/stenosis. see Rectal atresia; Rectal stenosis rectobladderneck fistula. see Rectobladderneck fistula rectoperineal fistula. see Rectoperineal fistula rectourethral fistula. see Rectourethral fistula newborn management of, 582–585 algorithm, 582–583, 582f, 584f anoplasty, 583, 583f antibiotics, 583 colostomy, 583, 585f descending colostomy, 584, 585f divided descending colostomy, 584, 585f intravenous fluids, 583 nasogastric decompression, 583 protective colostomy, 583 radiology, 583 rectoperineal fistula, 583, 583f surgical reconstruction, 584–585 vestibular fistula, 580f, 583 postoperative management of, 594–595, 595t result evaluation, 596–597 complications of, 596–597 sacral defects and, 581–582, 581f spinal defects and, 581–582 Anorectal malformations, 599 children with, 602–603 constipation in, 605–608, 606f–607f predictions of prognosis of, 602t prognostic signs of, 602t Anorectal manometry, for functional constipation, 609 Antegrade pyelography, 65 Anterior cricoid split procedure, 344, 345f Anterior hernias of Morgagni, 394–395 Anterior iliac osteotomies, 901 Anterior mediastinal mass, 41, 42f Anterior urethra, prune belly syndrome, 944

Anterior urethral valve, 880–881, 880f Anteroposterior radiographs abdominal, in malrotation diagnosis, 509, 509f for cervical spine injuries, 274 Anthracyclines, adverse effects, 972 Antibiotics, 142–143 appendicitis management, 668 biliary atresia, 687 in esophageal perforation, 427–428 for necrotizing enterocolitis, 543–544 prevention, 548 for neonatal anorectal defects, 583 perforated appendicitis, 671–672 pharmacokinetics of, 143 prophylaxis, 857 for myelomeningocele, 874 surgical infection prevention of, 144 for ureteropelvic junction obstruction, in children, 838–839 ulcerative colitis management, 650 surgery, 651 for ureterocele, 849 for urinary tract infections, 857–858 Antibodies, 13 Anticonvulsant, for traumatic brain injury, 263 Antidiuretic hormone (ADH) inappropriate response, 7b nocturnal enuresis, 871 urine volumes of, 58 Antigen-presenting cells (APC), 680–681 Anti-inflammatory management, 650 Antimetabolites, as anticancer drugs, 973t–974t Antimicrobial agents, 203–205, 204t Antineutrophil cytoplasmic antibodies, 647 Antithrombotic therapy, 43 Antivenom, 185 Antral web, 484, 484f Anus, dilatation, post-colostomy closure, 594, 595t Aortoesophageal fistula (AEF), in foreign body esophageal injury, 426–427 Aortopexy, in tracheomalacia, 448 APC. see Antigen-presenting cells APC gene, 1042 Aplasia, breast disease and, 1207–1208 Apnea, postanesthetic, 40, 40f Apoptosis, in secondary brain injury, 258 Appendectomy carcinoid tumors and, 1234 complicated appendicitis, 670 complications, 672 interval, 672 laparoscopic, 668–669 in malrotation management, 511– 512, 513f technique, 668–669 uncomplicated appendicitis, 670 wound infection, 672 Appendicitis, 664–678 age, 665 clinical features, 665 clinical pathways, 668

1255

Appendicitis (Continued) cystic fibrosis and, 530 differential diagnosis, 668, 668b imaging studies, 666–667 MRI, 667 US, 666, 666f incidence, 664 laboratory studies, 665 nonperforated, 668–671, 669f–670f pathophysiology and natural history, 664, 664f perforated, 671–673, 671f risk scores, 665–666, 666t surgical management, 672–673, 672f–673f treatment, 668 Appendicitis Inflammatory Response (AIR) Score, 665 Appropriate-for-gestational-age (AGA), 2 aPTT. see Activated partial thromboplastin time Aquacel, 204t Arginine, 549 Argyle (Dover) tracheostomy tube, 339t ARPKD. see Autosomal recessive polycystic kidney disease ARPV. see Airway pressure release ventilation Arsenic, as anticancer drugs, 973t–974t Arterial blood gas analysis, of burns, 197 neonatal pulmonary monitoring, 9 Arterial cannulation, ECMO, 96 Arterial catheter, 138 Arterial catheterization, for infantile hemangioma, 1152 Arterial embolization, in focal nodular hyperplasia management, 1039 Arterial oxygen tension (PaO2), 9 Arteriovenous fistula (AVF), in brain, 293 Arteriovenous malformations (AVMs), 1161–1162 in brain, 291–292, 292f clinical features of, 1161, 1161f, 1161t imaging of, 1161, 1161f treatment of, 1161–1162 Artificial urinary sphincter, 878 ASA. see American Society of Anesthesiologists Ascites, acute pancreatitis, 741 ASCT. see Autologous stem cell transplant Ask-Upmark kidney, 828–829 Asphyxia, traumatic, 225–226, 226f Asphyxiating thoracic dystrophy (Jeune syndrome), 302, 324, 327f Asplenia, 750 Assist-control ventilation (ACV), 117, 117t Asthma, in congenital diaphragmatic hernia, 393 Astrocytoma, pilocytic, 296–297, 296f ATLS. see Advanced Trauma Life Support ATOMAC guideline, for nonoperative management, of liver and spleen injury, 237–238 ATP-sensitive potassium channel, 744 Atretic encephaloceles, 288 Atrophy, breast disease and, 1208

1256

Index

ATRX, 1014 Atypical malrotation, 514 Autologous stem cell transplant (ASCT), 1102 Autologous tissue patches, for congenital diaphragmatic hernia, 390 Autosomal dominant polycystic kidney disease (ADPKD), 833–834 Autosomal inheritance, jejunoileal atresia/stenosis and, 495 Autosomal recessive polycystic kidney disease (ARPKD), 833, 833f AVF. see Arteriovenous fistula AVMs. see Arteriovenous malformations Avulsion fractures, 272–273 Axonal shearing, 254 Azathioprine, 715 Azotemia, prerenal, 60 Azygos vein, 422

B

B7-1, 680–681 B7-2, T-cell activation, 680–681 Bacillus postoperative infections, 146 Bacitracin, 204t, 773 Baclofen pump, 287 Bacterial infections, 719 Bacterial laryngotracheitis, 337 Bacterial tracheitis, 337 BAL. see Bronchoalveolar lavage Balanitis, 935 Balanitis xerotica obliterans, 935 Balloon dilation in achalasia, 424 and stenting, for megaureter, 846 Bannayan-Riley-Ruvalcaba (BRRS), 1165 Bar removal, in pectus excavatum, 314, 314f Barbiturate therapy, for traumatic brain injury, 263–264 Bariatric surgery, 1240–1252 for adolescents, 1240–1241, 1242b guidelines, 1240–1242 long-term management, 1248, 1248f morbidly obese, 1244 options, 1242–1243 adjustable gastric band, 1243, 1244f laparoscopic vertical sleeve gastrectomy, 1243, 1245f Roux-en-Y gastric bypass, 1243, 1245f–1246f outcomes, 1249 patient selection, 1240, 1241f, 1241b postoperative management, 1246– 1247, 1247f compliance, 1247, 1248b preoperative education, 1243 preoperative management, 1243– 1244 timing of, 1241–1242 physical maturation, 1241–1242 psychological maturation, 1242, 1243b Barium esophagography, 422 in achalasia, 424 in vascular compression, 335 Barium swallow, in esophageal replacement, 433f

Barrett’s esophagitis, 463 in esophageal atresia, 448 BAT. see Brown adipose tissue Batteries, ingestion, 173–174, 175f–176f Battle’s sign, 256 B-cell deficiencies, 142 immunity, 142 Beckwith-Wiedemann syndrome (BWS) adrenal carcinoma and, 1230 hepatoblastoma, 1041 omphalocele and, 771 pancreatic adenocarcinoma/ pancreatoblastoma, 746–747 Benjamin-Lindholm laryngoscopes, 338 Betamethasone, 157 Bezoars, 176–177, 177f, 486, 486f BF. see Bladder exstrophy Bianchi procedure, of Hirschsprung disease, 565–566 Bicarbonate, 60–61 Bidirectional cavopulmonary anastomosis, 45 Bifid scrotum, 922f Bifid sternum, 302, 323, 325f Bilateral coronal synostosis, 290–291 Bilateral iliac osteotomies, 901 Bilateral lumbar hernia, 782 Bilateral renal agenesis, 830 Bilateral single ectopic ureters, 847–848 Bilateral Wilms tumor, 1001, 1001f Bile acids, 681–682 Bile flow cessation, biliary atresia complication, 687 Bilevel control of positive airway pressure (BiPAP), 119 Biliary atresia, 679–694 classification, 679, 679f complications bile flow cessation, 687 cholangitis, 688 hepatic malignancies, 689 hepatopulmonary syndrome, 689 intrahepatic cysts, 689, 689f portal hypertension, 688, 688f diagnosis, 681–683, 682b bile acids levels, 681–682 duodenal aspiration, 681–682 hepatobiliary scintigraphy, 681–682 jaundice, 681 laparoscopy-assisted cholangiography, 683 lipoprotein-X, 681–682 neonatal hepatitis, 681–682 US, 682–683, 682f–683f genetics, 680 in hepatocellular carcinoma, 1051 histopathology, 681, 681f historical aspects, 679 incidence, 680 liver transplantation, 709 operative management, 683–687 hepaticojejunostomy, 683–686 Kasai hepatic portoenterostomy, 679 liver transplantation, 679–680, 690–691 minimally invasive Kasai portoenterostomy, 686–687

Biliary atresia (Continued) modified Kasai original portoenterostomy, 684f, 686 open surgery technique, 683–686, 684f–685f prognosis, 689–690 re-do hepatic portoenterostomy, 687 results, 689–690 pathogenesis, 680–681 postoperative management, 687–688 screening, 683 Biliary tract dyskinesia, 701 liver transplantation complications, 717–718, 718f neoductules, 681, 681f reconstruction after liver transplantation, 715, 715f rhabdomyosarcoma, 1119–1120 Biliary tract disease, cystic fibrosis and, 527–528 Biliary tract rhabdomyosarcoma, 1055–1056, 1056f Bilio-enteric anastomosis after cystectomy, 697–698 Bilirubin, 1055 Biloma, in liver injury, 249 Biobrane, 204t Biofeedback, for bladder instability, 872 Biologic response modifiers, cancer chemotherapy, 977 Biopsy gonadal, 960 in Hirschsprung disease diagnosis, 558–559 for interstitial disease, 369 lymph node, 995 percutaneous, 41 for rhabdomyosarcoma, 1117, 1117f in vulvar abnormalities, 1188–1189 BiPAP. see Bilevel control of positive airway pressure “Bird’s beak” sign, 424, 424f Birth weight, ECMO, 93 Bites, 181–183, 182t mammals, 181–182 wound management, 183b Black widow spider (Latrodectus mactans), 186 Bladder, 870–883 augmentation, 877f, 878 autoaugmentation, 876–878, 877f catheter placement, 65 exstrophy. see Bladder exstrophy fullness, 870 instability, in daytime incontinence, 872–873 prune belly syndrome, 943, 943f rhabdomyosarcoma, 1121 underactive, 873 Bladder exstrophy (BF), 897–911 diagnosis of, 897 incidence of, 897 long-term outcomes, 910 quality of life, 910 sexual function, 910–911 natural history of, 898 operative approaches of, 898–900

Index

Bladder exstrophy (BF) (Continued) anatomic reconstruction, 899–900 complete primary repair, 901–902. see also Modern staged repair of exstrophy. other primary reconstructive techniques, 907–908 outcomes, 909–911 preoperative care, 900 urinary diversion, 899, 899t operative considerations in, 900–901 adjunctive aspects of repair, 901 delayed primary closure, 900, 900t immobilization, 901, 902f osteotomies, 901 pathogenesis of, 897–898 prenatal diagnosis of, 897 reconstruction principles in, 898 treatment of, 898 Bladder neck fascial sling, 878 incompetent, 873 reconstruction, 909 Bladder outlet obstruction, 859 Bladder plate, cloacal exstrophy, 911, 911f Bleeding hypospadias, 930 intrathoracic, in hemothorax, 227 liver and spleen delayed, 241 operative management of, 238–240, 241f time, 79–80 Bleomycin, as anticancer drugs, 973t–974t Blind-loop syndrome, 493 Blood-brain barrier, in secondary brain injury, 257–258 Blood supply of esophagus, 422 mesenchymal hamartoma and, 1035 Blood volume, neonates, 4–5, 4t Blue rubber bleb nevus syndrome, 1160f Blunt abdominal trauma aortic, 247 evaluation of, 236–237, 237f, 237t computed tomography in, 236, 237f–238f, 237t contrast-enhanced US in, 237, 239f focused assessment with sonography for trauma, 237, 238f shock index in, 236 ultrasound in, 237 intestinal, 244–247 colonic injury in, 245 duodenal injuries in, 244, 245f, 245t gastric injuries in, 244 rectal injury in, 245–247, 246f small bowel injuries in, 244–245, 246f resuscitation of, 236 Blunt cardiac injury, 232–233 Blunt diaphragmatic injury, 247, 247f Blunt intestinal injury, 244–247 colonic injury in, 245 duodenal injuries in, 244, 245f, 245t

Blunt intestinal injury (Continued) gastric injuries in, 244 rectal injury in, 245–247, 246f small bowel injuries in, 244–245, 246f BMI. see Body mass index “Bochdalek” location, in congenital diaphragmatic hernia, 377 Body composition, neonates, 18–20 Body fat, neonates, 2 Body fluid regulation, 57 Body mass index (BMI), 1240 Bone marrow of intraosseous access, 137 in neuroblastoma, 1017 Bone morphogenic protein, 437 Bortezomib, 1102 Boston Children’s Hospital Vascular Anomalies Center, 1035 Botulinum toxin (Botox) in achalasia, 424 neurogenic bladder, 875 Bowel management, 599, 603–605, 604f–606f Bowel motility, 600–601, 600f–601f Bowman’s capsule, 828 Boys, precocious puberty in, 1233–1234 BPD. see Bronchopulmonary dysplasia Brachytherapy, local tumor control, 980 Bracing, in pectus excavatum, 304 BRAF mutations, nevi and, 1130 Brain, vascular malformations of, 291–293, 292f–293f Brain tumors, 296–299 choroid plexus tumors in, 298, 298f craniopharyngiomas in, 298 diffuse intrinsic pontine glioma in, 298–299, 299f ependymoma in, 297, 297f germ cell neoplasms in, 298 medulloblastoma in, 297, 297f pilocytic astrocytoma in, 296–297, 296f Breast anatomy of, 1206 development of, 1206 physiology of, 1206 Breast cancer, 1214–1215 Breast diseases, 1206–1216 development/growth disorders, 1207–1209 aplasia, 1207–1208 gynecomastia, 1208–1209, 1209f, 1209t hypertrophy, 1208 hypoplasia, 1207–1208 neonatal hypertrophy, 1207, 1207f polythelia, 1207, 1207f premature thelarche, 1208, 1208t inflammatory lesions, 1209–1210 abscesses, 1210 fat necrosis, 1209–1210 mastitis, 1210, 1210f trauma, 1209–1210 masses. see Breast masses nipple discharge, 1210–1211 galactorrhea, 1210–1211 mastalgia, 1210 non-milky, 1211, 1211f pathophysiology of, 1206

1257

Breast masses, 1211–1215 benign disease, 1212 evaluation of, 1211–1212, 1211b fibroepithelial tumors, 1213–1214 breast cancer, 1214–1215 fibroadenomas, 1213, 1213f phyllodes tumors, 1212f, 1213– 1214 prepubertal, 1212 simple cysts, 1212–1213 Breath magnitude, mechanical ventilation of, 115 Brentuximab, 1102 Bronchial atresia, 351–352 postnatal management of, 356–357 prenatal and perinatal management of, 353–354 Bronchial carcinoid, 1235 Bronchial stenosis, 351–352 Bronchiectasis, 366–368 etiology of, 366t management of, 367–368 presentation and diagnosis of, 367, 367f Bronchoalveolar lavage (BAL), 369 Bronchogenic cysts, 351–352, 412, 413f postnatal management of, 356–357 Bronchopulmonary dysplasia (BPD), 39 Bronchopulmonary malformations, congenital, 348–360 classification of, 349–352, 349b perinatal management of, 352–354 postnatal management of, 354–355 prenatal diagnosis of, 349–352 prenatal management of, 352–354 thoracoscopic lobectomy for, 357–359, 358f–359f Bronchopulmonary sequestration, 157–158, 350–351, 351f extralobar, 350–351, 351f intralobar, 350 postnatal management of, 355, 356b, 357f prenatal and perinatal management of, 353 Bronchopulmonary tree, embryology and development of, 348, 348f Bronchoscopy for airway foreign bodies, 179–180, 179f in esophageal atresia, 440 flexible, 179 preoperative, 441–442, 441f Broviac catheter, 135–136, 135f Brown adipose tissue (BAT), 6 Brown recluse spiders (Loxoceles), 184–186, 185f BRRS. see Bannayan-Riley-Ruvalcaba Burkitt lymphoma, 405, 1103, 1105f classification of, 1104 treatment of, 1108–1109 Burns, 196–210 arterial blood gas analysis, 197 assessment of depth, 202–203, 202f–203f carboxyhemoglobin, 197 chemical, 206 depth assessment, 202–203

1258

Index

Burns (Continued) electrical, 206–207, 206f escharotomy, 197 excision and grafting of, 205–206 fluid resuscitation of, 198–201, 199t–200t colloid solutions, 200–201 dextrose-containing solutions, 198 hypertonic saline, 200–201 Parkland formula, 198 Shriners-Galveston formula, 198 tachycardia, 201 TBSA-based formulas, 198 urine output, 197 inhalation injury of, 201–202 initial management of, 197–198, 199f escharotomy, 197, 198f mechanisms of, 201 multidisciplinary care for, 208 pathophysiology of, 196 psychologic sequelae of, 207–208 resuscitation endpoints of, 200–201 signs and symptoms of, 197 superficial second-degree, 205 systemic response to, 196 thermal, in intrinsic injuries, 337–338 third-degree, 204 wound care of, 203–206 antimicrobial agents, 203–205 wound dressings, 205, 206f zone of coagulation, 196 zone of hyperemia, 196 zone of stasis, 196 zones of burn injury, 196, 197f Burst fractures, 276–277 Busulfan, as anticancer drugs, 973t–974t Button batteries, 426 BWS. see Beckwith-Wiedemann syndrome

C

C1 vertebral trauma, 213–214 C2 vertebral trauma, 214 C3 vertebral trauma, 213–214 C4 vertebral trauma, 213–214 CA. see Colonic atresia CAB. see Circulation, airway, and breathing CAH. see Congenital adrenal hyperplasia Calcifying epithelioma of Malherbe, 1085, 1085f Calcineurin inhibitors, 718 Calcitonin gene-related peptide (CGRP), 784, 805 Calcium hypocalcemia. see Hypocalcemia neonates, 3–4 overload. see Hypercalcemia Calcium channel blockers, in achalasia, 424 Calcium gluconate, 4 Calorimetry, indirect, 20 Calretinin, 559, 561f Calyceal diverticuli, 834–835 Canalicular stage, in lung development, 380

Cancer biology, 969–970 cytogenetics, 970 incidence of, 969 pathology, 969 protection by circumcision, 937 survival statistics for, 969, 969t Cancer chemotherapy, 967–985 adjuvant therapy, 970–971 agents used, 971–972 biologic targeted therapy, 977–979 adoptive immunotherapy, 978 biologic response modifiers, 977 signal transduction inhibitors, 977, 978f tumor-targeted antibody therapy, 978–979 clinical trials, 970 dose intensity, 971 duration, 968 history of, 968 neoadjuvant therapy, 970–971 regimens, 968 supportive care, 972–977 toxicity, 972–977 Cancer therapy adjunctive techniques, 980–982 chemoembolization, 981 local tumor control, 979–980 brachytherapy, 980 intensity-modulated radiation therapy, 980 intraoperative radiation, 980 proton therapy, 980 radiation oncology, 979–980 long-term side effects of, 972–977 lymphatic mapping, 981–982 radiofrequency ablation, 981 Cannulation, ECMO, 96–97, 96f–98f Capillary-lymphatico-venous malformation (CLVM), 1162–1163 Capillary malformations, 1155, 1155f Carbohydrate(s) metabolism, 23–24 requirements in, 23–24 stress response of, 18 Carbon dioxide anesthetic intraoperative management of, 47 tension, 10 Carbon monoxide (CO), inhalation injury of, 201 Carboplatin, as anticancer drugs, 973t–974t Carboxyhemoglobin, burns, 197 Carcinoid tumors, 1234–1235, 1235f Carcinoma, ductal, 1214 Carcinoma in situ, testicular neoplasms, 814–815 Cardiac anomalies, prune belly syndrome, 945 Cardiac contusion, 232–233 Cardiac effects, of pectus excavatum, 305–306, 305t Cardiac injuries, 232–233 Cardiac output (CO), 10 in congenital heart disease, 44 Cardiogenic shock, in neonates, 11–13

Cardiopulmonary failure, ECMO, 91–92 Cardiotoxicity, cancer therapy complications, 972–976 Cardiovascular support, for traumatic brain injury, 261 Cardiovascular system, anomalies of, congenital diaphragmatic hernia and, 377–378 Cardioverter defibrillators, implantable, 44 Carmustine, as anticancer drugs, 973t–974t Caroli disease, surgical technique, 700 Cartilaginous rings, trachea, 333 Cat bites, 181 Catecholamine metabolites, in neuroblastoma, 1015 Catheter drainage, for parapneumonic effusion, 363 surgical infections of, 146–147 Cat-scratch disease, 1183–1184 Caustic ingestion, in esophagus, 428– 429, 428t, 429f Cavernous malformations (CM), in brain, 292–293, 293f CBAVD. see Congenital bilateral absence of the vas deferens CBD. see Common bile duct CBF. see Cerebral blood flow CCAMs. see Congenital cystic adenomatoid malformations CD5, 405 CD7, 405 CD19, 405 CD20, 405 CD22, 405 CD28, 680–681 CD40, 680–681 Cefotaxime, 671–672 Centers for Medicare and Medicaid Services (CMS), 146 Central lymph neck dissection, for thyroid cancer, 1222 Central nervous system, anomalies of, congenital diaphragmatic hernia and, 377–378 Central venous catheters (CVCs), 133, 135–136 alternative routes for, 138, 138f Broviac catheter, 135f Doppler ultrasound, 138 Hickman catheter, 135–136 neonatal pulmonary monitoring, 10 Seldinger technique, 135, 135f surgical infections of, 146–147 totally implanted, 136, 137f ultrasound of, 136f upper body vein, 135–136 Cephalexin posterior urethral valves, 887–888 urinary tract infection, 858t Cerebral autoregulation, 256 Cerebral blood flow (CBF), 255 Cerebral microdialysis, 264 Cerebral perfusion pressure (CPP), 255 intracranial pressure monitoring and, 262

Index

Cerebrospinal fluid (CSF), 255, 285 rhinorrhea, 256 Certolizumab, 658 Cervical collar, in cervical spine injuries, 274 Cervical lymphadenitis, acute suppurative, 1182–1183, 1182f Cervical myelomeningoceles, 288–289 Cervical spine injuries, 272–275 avulsion fractures in, 272–273 history in, 273–274 immobilization in, 273, 274f–275f mechanisms of, 272–273 MRI in, 272–273, 273f radiographic evaluation for, 274 spinal cord injury without radiographic abnormality in, 272, 275 Cervical spine instability, of trisomy 21, 38 Cervical teratomas, 1078–1080, 1079f Cesarean delivery, for gastroschisis, 764 CEUS. see Contrast-enhanced ultrasound CF. see Cystic fibrosis CFC1, 680 CFTR (cystic fibrosis transmembrane conductance regulator) gene, 737–738 chronic pancreatitis genetics, 742 in cystic fibrosis genetics, 517 mutation analysis, 523 CGRP. see Calcitonin gene-related peptide Chalasia, 462 CHAOS. see Congenital high airway obstruction syndrome CHARGE, 438 CHD. see Congenital heart disease CHD7, 437 Chemical burns, 206 Chemoembolization, 981, 1054 Chemotherapy for hepatoblastoma treatment, 1048, 1048f for Hodgkin lymphoma, 1101–1102 for teratomas, 408–409 for undifferentiated embryonal sarcoma of the liver (UESL) treatment, 1055 for Wilms tumor, 998–999, 999t Chest drain, in esophageal atresia, 443–444 Chest film, in diaphragmatic injuries, 228 Chest malleability, in pectus excavatum, 304 Chest pain, in esophageal perforation, 427 Chest radiograph in anterior hernias of Morgagni, 394, 395f in bronchiectasis, 367, 367f in congenital diaphragmatic hernia, 382, 382f in foregut cysts, 412 in Hodgkin lymphoma, 1099–1100 in parapneumonic effusion, 362 Chest reconfiguration, in pectus excavatum, 304 Chest trauma, injury patterns of, 214, 214t Chest wall after CDH repair, 387 anatomy of, 224

Chest wall deformities, 301–331, 302t congenital, 302 pectus carinatum in, 314–323, 315f, 324f pectus excavatum in, 302–314, 303f, 303b Poland syndrome in, 323 sternal defects in, 323–324, 325f–326f thoracic insufficiency syndrome, associated with diffuse skeletal disorders, 324–328, 327f–328f Chest X-ray in great vessel injury, 231 in rib fractures, 224 Chiari I malformations, 295, 295f–296f Chiari II malformation, 289 Childhood, ovarian cysts in, 1194, 1195f Childhood Interstitial Lung Disease Pathology Co-operative Group, 369–370 Children’s Hospital of Philadelphia, 352–353, 354f, 354t Children’s Oncology Group (COG) in hepatoblastoma staging, 1045, 1048t neuroblastoma group risk stratification, 1019t staging system, 814, 815t tumor studies, in Wilms tumor, 999 in Wilms tumor staging, 993, 993t Cholagogues, biliary atresia, 687–688 Cholangiography biliary atresia, 683–684, 684f gallbladder disease, 702 intraoperative, 697, 697f percutaneous transhepatic, 1056 Cholangitis, 688 Cholecystectomy cholelithiasis in cystic fibrosis, 528 sickle cell disease and, 87 Cholecystitis, acalculous, 702 Choledochal cyst, 695–708 acquired, 695, 696f classification, 695, 696f clinical features, 696 congenital, 695 etiology, 695 imaging, 696–697, 697f male:female ratio, 696 pathology, 696 surgical technique, 697–700 bilio-enteric anastomosis after cystectomy, 697–698 cystoduodenostomy, 697 cystojejunostomy, 697 hepaticoduodenostomy, 697–700 hepaticojejunostomy, 697–699 intraoperative complications, 700 laparoscopic approach, 698, 698f–699f open operation, 700 outcomes, 700–701 postoperative care, 700 preoperative preparation, 697 Roux-en-Y hepaticojejunostomy, 697–698 Todani classification, 695, 696t Choledochocele, surgical technique, 700

1259

Choledocholithiasis gallbladder disease, 702 management, 702f Cholelithiasis, 701–702 cystic fibrosis and, 528 sickle cell disease and, 87 Cholestasis, meconium ileus and, 525–526 Cholestatic jaundice, 527 Chordee, 918, 918b hypospadias complications, 931 Choriocarcinoma, 1198t Choroid plexus tumors, 298, 298f Chromosomal abnormalities, in hepatoblastoma, 1042 Chromosome, in neuroblastoma, 1013–1014 Chromosome 1 deletion, 1013–1014 Chromosome 11 deletions, 1014 Chronic autoimmune thyroiditis, 1217–1218 Chronic idiopathic intestinal pseudoabsorption, intestinal transplantation indication, 721 Chronic interstitial lung disease, 368– 369, 371t Chronic lymphadenitis, 1183 Chronic lymphocytic (Hashimoto) thyroiditis, 1217–1218 Chronic pain, as inguinal hernia complications, 800 Chronic rejection, liver transplantation, 718 Chronic subdural hematomas, 257 Chylothorax, 227, 368, 369f–370f Chylous leaks, 724 Ciprofloxacin, 654–655 Circulation, airway, and breathing (CAB), 258 Circumcision, 935–941 cancer protection, 937 complications, 940, 940f medical indications, 935 prevalence of, 936f routine at birth, 935–937 sexually transmitted diseases, 936 surgical technique, 937–940 freehand circumcision, 939–940, 939f newborn, 937, 938f revision circumcision, 938, 939f urinary tract infections, 936 CIREN. see Crash Injury Research Engineering Network Cisplatin adverse effects, 972 for cancer chemotherapy, 973t–974t in hepatoblastoma treatment, 1046 13-cis-Retinoic acid, as anticancer drugs, 973t–974t Cladribine, as anticancer drugs, 973t–974t Classic laryngotracheoplasty, 344, 345f Clavicle fractures, 277 Clean intermittent catheterization, for neurogenic bladder, 873 Cleft sternum, 323 Clinical trials, cancer chemotherapy, 970

1260

Index

Clitoris, 1190 CLND. see Completion lymph node dissection Cloacal exstrophy, 897, 911–913 associated conditions, 911–912 bladder plate, 911, 911f closure techniques, 912–913, 913f gastrointestinal reconstruction, 913 gender of rearing, 913 mortality, 911 perioperative management, 912 Cloacal malformations, 577–598 with common channel longer than 3 cm, 593 with common channel shorter than 3 cm, 590–591, 591f–593f as female defects, 580, 581f repair of, 590–593 Clofarabine, as anticancer drugs, 973t–974t Clonidine, 51 Clostridium difficile infection enterocolitis, 571 surgical infections of, 146 Closure, of umbilical hernia management, 780–781, 781f Clothesline injury, 338, 338f CLOVES syndrome, 1164, 1164f CLVM. see Capillary-lymphatico-venous malformation CM. see Cavernous malformations CMV. see Controlled mechanical ventilation CO. see Cardiac output CO2 laser, in tracheal repair, 341–342 Coagulation cascade, 77f fibrinolysis, 78 Coagulopathies, 76–90 Codeine, 52 COG. see Children’s Oncology Group Coins, in foreign body esophageal injury, 426 Colectomy Crohn disease management, 659 pouch-anal reconstruction and, 660 segmental and anastomosis, 659 total, 651 Collagen, 76–78 Collar of Helvetius, 460 Colloid solutions, fluid resuscitation in burns, 200–201 Colon duplication, 632t, 636–638, 637f interposition, in esophageal replacement, 433f nondilated, 603f Colonic atresia (CA), 502, 502f associated anomalies, 502 clinical features of, 502, 503f colostomy for, 502 diagnosis of, 502 incidence of, 502 Colonic enema, 529 Colonic hypermotility, children with, 603 Colonic hypomotility, children with, 603 Colonic injury, 245 Colonoscopy, for fibrosing colonopathy, 530

Colony-stimulating factors (CSFs), 14–15 Colorectal carcinoma, ulcerative colitis, 648 Colostomy descending, 584, 585f divided descending, 584, 585f for neonatal anorectal defects, 583, 585f protective. see Protective colostomy upper sigmoid (descending), 584, 585f Combined vascular malformations, 1162–1165 Common bile duct (CBD), 696t Compartment syndrome, 278 of crotalid snakebites, 188 Complete primary repair of exstrophy (CPRE), 901–902 in boy, 902–906, 902f–906f in girl, 906–907, 906f–908f Completion lymph node dissection (CLND) for melanoma, 1138–1139 for Spitz nevi, 1135 Complex injuries, in orthopedic trauma, 268, 268t, 269f Compliance, of mechanical ventilation, 112, 113f Composite polypropylene, for congenital diaphragmatic hernia, 389 Compression fractures, in spinal injuries, 275–276 Computed tomographic enterography, Crohn disease, 657 Computed tomographic pancreatography, acute pancreatitis diagnosis, 741 Computed tomography (CT) in acute pancreatitis diagnosis, 739–740 in airway injury, 230, 230f in alimentary tract duplication, 630, 631f in anterior mediastinal mass, 41, 42f in appendicitis, 667, 667f in arteriovenous malformation, 1161 in blunt abdominal trauma, 236, 237f–238f, 237t in bronchiectasis, 367, 367f in cervical spine injuries, 274 in clavicle fracture, 277, 277f concerns over, 667 in diaphragmatic injuries, 228 in esophageal atresia, 440–441 in esophageal perforation, 427 in focal nodular hyperplasia, 1038, 1038f in foregut cysts, 412 in great vessel injury, 231 in hemothorax, 227 in hepatoblastoma, 1044 in hepatocellular adenoma, 1040 in hepatocellular carcinoma, 1052 in Hodgkin lymphoma, 407 in hydrocephalus, 286, 286f in infantile hepatic hemangiomas, 1033, 1033f in insulinomas, 745–746 in intussusception diagnosis, 623, 624f in liver transplantation donor selection, 713

Computed tomography (CT) (Continued) in lower extremity fractures, 268–270 in lung abscess, 365 in mediastinal tumors, 404 in mesenchymal hamartoma, 1036 in neuroblastoma diagnosis, 1016 in parapneumonic effusion, 362, 363f in pectus excavatum, 306–307, 306f in pheochromocytoma, 1232 in pneumothorax-pulmonary lacerations, 226 in pulmonary contusion, 227–228 in remnants of embryonic branchial apparatus, 1175 in secondary brain injury, 258 in skull fractures, 256 in spontaneous pneumothorax, 372 in teenage malrotation, 514, 514f in testicular neoplasms, 807 in ulcerative colitis, 649, 649f in vascular compression, 335–336 in Wilms tumor diagnosis, 994 Computed tomography-guided drainage, in lung abscess, 365 Concussion, 255 Condylomata acuminata, 1084–1085 Congenital abdominal wall defects, 763–779 Congenital adrenal hyperplasia (CAH), 1234 causes of, 954 diagnosis of, 955 incidence of, 954 treatment of, 955 virilization in, 954–955 Congenital bilateral absence of the vas deferens (CBAVD), 517–518, 518f Congenital bronchopulmonary malformations, 348–360 classification of, 349–352, 349b perinatal management of, 352–354 postnatal management of, 354–355, 356f prenatal diagnosis of, 349–352 prenatal management of, 352–354 thoracoscopic lobectomy for, 357–359, 358f–359f Congenital choledochal cyst, 695 Congenital chylothorax, 368 Congenital cystic adenomatoid malformations (CCAMs), 157 microcystic, 157 resection, 157 Congenital diaphragmatic hernia, 155–156, 377–402 associated anomalies with, 377–378 associated pulmonary hypertension, 380–381 “Bochdalek” location in, 377 clinical presentation of, 382–383, 382f–383f congenital pulmonary airway malformation and, 349–350 cost of, 378 diagnosis of, 381–383 prenatal, 381–382, 381f–382f ECMO, 94–95 embryology of, 378–381

Index

Congenital diaphragmatic hernia (Continued) epidemiology of, 377 fetal interventions of, 155–156, 156f genetics of, 377 incidence of, 377 lung development in, 380 “Morgagni” hernias in, 377, 394–395, 395f operative repair for, 386–394 approach in, 387 diaphragmatic replacements in, 389–391, 389f fetal therapy in, 391 minimally invasive approach in, 387–388, 388f open approach in, 387–388, 387f timing of, 386–387 outcomes in, 391 follow-up guidelines for, 392, 392t–393t gastrointestinal, 394 musculoskeletal, 394 neurologic, 393–394 pulmonary, 392–393 risk stratification and, 392 volume-based, 392 pathogenesis of, diaphragm development and, 378–380, 378f–379f prognostic criteria of, 155 pulmonary hypoplasia in, 380 pulmonary vascular development and, 380–381 treatment of, 383–386 extracorporeal membrane oxygenation, 385–386 mechanical ventilation, 384–385 prenatal care, 383 prenatal medications, 383 pulmonary vasodilators, 385 resuscitation, 383–384 stabilization, 383–384 surfactant, 385 Congenital epulis, 1088–1089, 1089f Congenital esophageal stenosis, 423, 423f Congenital heart disease (CHD) anterior mediastinal mass of, 41–43 cyanosis, 44 implantable cardioverter defibrillators of, 44 pacemakers, 44 pulmonary vascular hypertension, 43–44 single ventricle physiology, 44–45 special issues in patients with, 43–45 trisomy 21, 38 Congenital hemangioma, 1152, 1152f Congenital high airway obstruction syndrome (CHAOS), 349–350, 352 Congenital hyperinsulinism, 743–744 diffuse, 744 focal, 744 genetics, 744 hypoglycemia, 744 management, 744 stabilization, 744 type differential, 744, 745f

Congenital lobar emphysema, 351, 352f postnatal management of, 355–356 prenatal and perinatal management of, 353–354 Congenital melanocytic nevi, 1131–1133 classification of, 1131, 1131f, 1132t genetic mutations in, 1132 giant, 1131 management of, 1133, 1135 melanoma and, 1136 neurocutaneous melanocytosis, 1132–1133 presentation of, 1131–1132, 1132f, 1134f Congenital nevus-like nevi, 1132 Congenital pulmonary airway malformation (CPAM), 157–158, 349–350, 350f, 350t, 353f–354f classification of, 349f disappearing, 352–353 prenatal and perinatal management of, 352–353 serial imaging in, 352 US in, 349–350, 350t Congenital scoliosis with multiple vertebral anomalies and fused or absent ribs (jumbled spine), 324 Congenital subglottic stenosis, 333, 333f Congenital tracheal stenosis, 333, 333f tracheal repair in, 341–342, 341f–343f Congenital urethral fistula, 880 Congestive heart failure, in Wilms tumor, 1002–1003 Conn syndrome, 1229 Connective tissue disorder, 304 Constipation, 599–612 in anorectal malformations and Hirschsprung disease, 605–608, 606f–607f functional, 608–611 treatment, 608 children with, 603 postoperative anorectal defect repair, 596 Constriction bands, crotalid snakebite management of, 189 Continence after CPRE, 909 intellectual and psychological capacity to develop, 601 mechanism of, 599–601 Continent catheterizable stoma, 878, 879f Continuous incontinence, 873 Continuous positive airway pressure (CPAP), 118–119 Continuous renal replacement therapy (CRRT) acute kidney injury management of, 62 types of, 63 Continuous urinary incontinence, 846–847 Continuous venovenous hemodiafiltration (CVVHDF), 63 Continuous venovenous hemodialysis (CVVHD), 63 Continuous venovenous hemofiltration (CVVH), 63 Contralateral evaluation, in inguinal hernia management, 791–792

1261

Contrast enema in meconium ileus, 522–523, 522f water-soluble, 558 Contrast-enhanced ultrasound (CEUS), in blunt abdominal trauma, 237, 239f Contrast radiographs, in laryngotracheoesophageal cleft, 451 Controlled mechanical ventilation (CMV), 116, 117t Coral snake bites, 192–193 Corpus spongiosum, 918 Cortical-sparing adrenalectomies, 1232 Corticosteroids biliary atresia, 687 immune thrombocytopenic purpura management, 752–753 for non-Hodgkin lymphoma, 1109 post-liver transplantation, 715 Cortisol, 1227 Cortisol-releasing factor (CRF), 1227 Cosmesis, hypospadias, 924 Cowden syndrome, 1165 CPAM. see Congenital pulmonary airway malformation CPAP. see Continuous positive airway pressure CPP. see Cerebral perfusion pressure CPRE. see Complete primary repair of exstrophy Cranial imaging, in hydrocephalus, 286 Cranial suspensory ligament (CSL), 805 Craniofacial teratomas, 1080 Craniopharyngiomas, 298 Craniosynostosis, 290–291, 291f Crash Injury Research Engineering Network (CIREN), 211 C-reactive protein (CRP), appendicitis, 665 Creatinine, 59 CRF. see Cortisol-releasing factor Crohn disease, 655–660 clinical presentation, 656, 656t diagnosis, 657 etiology, 655–656 historical aspects, 655 medical management, 657–658 pathologic findings, 656, 656f surgical management, 657f–660f, 658–659 outcomes, 660 Cross-fused renal ectopia, 833 Crotalid snakebites, 187–192, 187f–190f, 189t clinical features of, 188–189 disposition and follow-up care, 192 in-hospital management of, 190–191 pathophysiology of, 188 prehospital management of, 189–190 antivenom, 185 constriction bands, 189 Crotalidae polyvalent immune fab ovine, 191 cryotherapy, 190 hypotension, 188 pressure immobilization, 189 serum sickness, 191 tourniquet, 189 role of surgeon, 191–192, 192f

1262

Index

Crotalidae polyvalent immune fab ovine, crotalid snakebite management of, 191 Croup, 337 post-intubation, 49 Crouzon’s syndrome, 290–291 CRRT. see Continuous renal replacement therapy Cryotherapy, of crotalid snakebite management, 190 Cryptorchidism, gastroschisis and, 770 CSF. see Cerebrospinal fluid CSFs. see Colony-stimulating factors CSL. see Cranial suspensory ligament CT. see Computed tomography CTNNB1, 988, 988t Cullen sign, 739, 739f Curcumin, 1035 Currarino-Silverman syndrome, 310, 312f Cushing syndrome, 1228–1229, 1230f etiology of, 1228, 1228b Cushingoid facies, infantile hemangioma and, 1150–1151 Cutaneous vesicostomy, 888, 889f Cutis marmorata telangiectatica congenita, 1156 CVCs. see Central venous catheters CVVH. see Continuous venovenous hemofiltration CVVHD. see Continuous venovenous hemodialysis CVVHDF. see Continuous venovenous hemodiafiltration Cyanosis, congenital heart disease, 44 Cyclophosphamide, as anticancer drugs, 973t–974t Cyclosporine, 718 Cystatin C, renal function evaluation of, 58 Cysteine in hepatoblastoma, 1042 protein quality of, 23 Cystic Cowper’s gland ducts, 881 Cystic fibrosis (CF), 517–520, 738 appendicitis and, 530 clinical presentation of, 520 complications of, 527–531 biliary tract disease, 527–528 gastroesophageal reflux disease, 527 diagnostic testing of, 523 fibrosing colonopathy and, 530–531 gastrointestinal pathophysiology of, 517–518 genetics of, 517 histologic findings consistent with, 519, 519f incidence of, 517 inguinal hernia and, 785 intussusception and, 530 prenatal diagnosis of, 518–519, 520f US, 518–519 prevalence of, 518 screening of, 518–519 sonographic evaluation of, 519–520, 521f Cystic nephroma, 834, 1005f

Cystic partially differentiated nephroblastoma, 834 Cystoduodenostomy, 697 Cystojejunostomy, 697 Cystoscopy in ureterocele, 847 in Wilms tumor, 995 Cysts dermoid, 1081–1083, 1083f, 1179– 1181, 1180f epidermal, 1083–1084 epidermoid, 1179–1181 esophageal duplication, 423 of foregut, 411–413, 413f–414f intrahepatic cysts, 689, 689f mediastinal, 412 neuroenteric, 411–412, 413f non-neoplastic pancreatic, 746 ovarian. see Ovarian cysts pillar, 1084 preauricular, 1178–1179, 1179f retention pancreatic cysts, 746 sebaceous, 1084 of thymus, 409–410 trichilemmal, 1084 Cytarabine adverse effects, 972 for cancer chemotherapy, 973t–974t Cytokines cancer chemotherapy, 971, 977 immune response of, 142 for septic shock, 13–14 tight junctions and, 538–539 Cytomegalovirus (CMV) infections biliary atresia, 679 chronic liver transplant rejection, 718 post-liver transplantation, 720 preoperative renal transplantation, 727 Cytotoxic edema, 257–258

D

Dacarbazine, as anticancer drugs, 973t–974t Dactinomycin, as anticancer drugs, 973t–974t Dakin’s solution (sodium hypochlorite), 204t Dasatanib, as anticancer drugs, 973t–974t Daunomycin, as anticancer drugs, 973t–974t DAX1, 953 Daytime incontinence, 870, 872–873 DBS. see Deep brain stimulators de Quervain thyroiditis, 1218 Dead space (Vd), 111–112 Debulking, for Klippel-Trenaunay syndrome, 1163 Decannulation, accidental, 101 Deceased donor (DD) kidneys, 727–728 Decompression for posterior urethral valves, 887 of sacral nerve roots, 277 Decompressive craniectomy, in traumatic brain injury, 263 Deep brain stimulators (DBS), 287 Dehydroepiandrosterone (DHEA), 1228

Dehydroepiandrosterone sulfate (DHEA-S), 1228 Delayed gastric emptying (DGE), gastroesophageal reflux vs., 464 Delayed primary closure, bladder exstrophy, 900, 900t Delayed staged closure, of omphalocele, 773 Dermis, 196 anatomy of, 1128, 1128f–1129f Dermoid cysts, 1081–1083, 1083f, 1179–1181, 1180f Descending colostomy, 584, 585f divided, 584, 585f Desmoid tumors, 1089 17,20-Desmolase, 956 Desmopressin, 872 Desmosis coli, 573 Destot sign, 268–270 Detrusor hyperactivity, neurogenic bladder, 873 Developmental venous anomalies (DVA), 293 Devitalized skin flaps, 930 Dexamethasone, 342–343 as anticancer drugs, 973t–974t suppression test, 1228–1229 Dextrose-containing solutions, fluid resuscitation, 198 DGE. see Delayed gastric emptying DHA. see Docosahexanoic acid DHEA. see Dehydroepiandrosterone DHPLN. see Diffuse hyperplastic perilobar nephroblastomatosis Diagnostic esophagoscopy, 422 Diagnostic laparoscopy, for undescended testes, 809 Dialysis acute kidney injury management of, 61–63, 63f characteristics of, 62t post-renal transplantation, 726, 727f Diaphragm development of, 378–380, 378f replacements in, 389–391, 389f Diaphragmatic eventration, 395–396, 396f Diaphragmatic injuries, 228, 228f–229f Diazoxide, 744 DIC. see Disseminated intravascular coagulation Diet, for chylothorax, 368 Dietary modifications, in gastroesophageal reflux management, 463 Diet-induced thermogenesis (DIT), 20 Differences of sexual development (DSD), 953–966 classification of, 954 incidence of, 954 Klinefelter syndrome, 958 Leydig cell abnormalities, 957 Mayer-Rokitansky-Küster-Hauser syndrome, 959 mixed gonadal dysgenesis, 957–958, 958f Müllerian-inhibitory substance deficiency (hernia uterine inguinale), 956–957, 957f

Index

Differences of sexual development (DSD) (Continued) newborn evaluation in, 959–960, 959f–960f, 959t ovotesticular DSD (true hermaphrodite), 957 partial androgen insensitivity syndrome, 956 pure gonadal dysgenesis, 958 reconstructive genital surgical procedures for, 960–963 considerations in, 960–961 controversies in, 960–961 female gender assignment, 961– 963, 961f–963f male gender assignment, 961 vanishing testis syndrome, 958 46,XX (female pseudohermaphrodite), 953t, 954–955, 955f 46,XX testicular DSD (XX sex reversal), 959 46,XY (male pseudohermaphrodite), 955–956 Diffuse hepatic hemangioma, 1153 Diffuse hyperplastic perilobar nephroblastomatosis (DHPLN), 990–991, 991f Diffuse interstitial disease, 368–371, 371f, 371t Diffuse intrinsic pontine glioma, 298– 299, 299f Diffuse large B-cell lymphoma (DLBCL), 1103 Diffuse skeletal disorders, thoracic insufficiency syndrome associated with, 324–328, 327f–328f Digestion, in intestinal barrier, 537–538 DIOS. see Distal intestinal obstruction syndrome Direct Coombs test, neonatal hemolytic anemia, 5 Direct inguinal hernia, 798 Direct puncture sclerotherapy, 1162 Discharge analgesia, 51–52 Dismembered pyeloplasty, 841, 841f Disordered peristalsis, in esophageal atresia, 448–449 Disorders of sexual dysfunction (DSD), hypospadias, 919 Disseminated intravascular coagulation (DIC), 83–84 activated partial thromboplastin time, 84 clinical evaluation of, 78–79 laboratory evaluation of, 79 prothrombin time, 84 tests for, 81 Distal forearm fractures, 279 Distal humerus, fractures of, 277–278 Salter Harris type I, 279 supracondylar, 277–278, 278f Distal intestinal obstruction syndrome (DIOS), 525, 528–530, 528f–529f Distributive shock, 13 DIT. see Diet-induced thermogenesis Diuretic(s), mechanical ventilation, 123 Diuretic isotopic renogram, 839, 840f

Diuretic renography for megaureter, 845 neonatal acute renal injury and, 65 Diversion with sigmoid colostomy, 651 Diverticulum, hypospadias, 931, 931f Divided descending colostomy, 584, 585f DLBCL. see Diffuse large B-cell lymphoma DNA analysis, for non-Hodgkin lymphoma, 1107–1108 DNA content, 1013 DNA index, in neuroblastoma, 1013 DO2 (oxygen delivery), 114 Dobutamine, 13 as vasoactive medications in newborn, 12t Docosahexanoic acid (DHA), 24 Docotaxel, as anticancer drugs, 973t–974t Dog bites, 181, 182f Donor risk index (DRI), liver transplantation, 712 Dopamine, as vasoactive medications in newborn, 12t, 13 Doppler ultrasound, of central venous catheters, 138 Dose intensity, cancer chemotherapy, 971 Double-lumen single cannula venovenous (DLVV) bypass, 95 Dover tracheostomy tube, 339t Doxorubicin adverse effects, 972 for cancer chemotherapy, 973t–974t in hepatoblastoma treatment, 1048 in Wilms tumor chemotherapy, 999 Drainage, for parapneumonic effusion, 363 Dressings, hypospadias, 928 DRI. see Donor risk index Drugs, associated with gynecomastia, 1209t DSD. see Differences of sexual development; Disorders of sexual dysfunction Ductal adenocarcinoma, pancreas, 747 Ductal carcinoma, 1214 Duhamel procedure, 562, 563f, 568 Duodenal atresia/stenosis, 489–506 classification system of, 491, 491f–492f diagnosis of, 492, 492f–493f etiology of, 489–491, 490f–491f management of, 492–495 duodenoduodenoscopy, 493, 493f duodenojejunostomy, 493 electrolytes, 492–493 fluid balance, 492–493 gastrojejunostomy, 493 laparoscopy, 493–494, 494f postoperative mortality, 494–495 pathology of, 492, 492f Duodenal injuries, 244, 245f, 245t Duodenoduodenoscopy, 493, 493f Duodenojejunoplasty, tapering, 501 Duodenojejunostomy, 493 Duodenum aspiration, biliary atresia diagnosis, 681–682 duplication, 632t, 634, 634f

1263

Duplication cysts, esophageal, 423 DVA. see Developmental venous anomalies Dysgerminoma, 1198t Dysphagia in esophageal perforation, 427 recurrent of, 424 Dysphonia, 178 Dyspnea, in vascular compression, 335

E

eBPS. see Extralobar bronchopulmonary sequestration ECCO2R. see Extracorporeal carbon dioxide removal ECDs. see Extended criteria donors Echocardiography, in esophageal atresia, 440 ECLS. see Extracorporeal life support ECMO. see Extracorporeal membrane oxygenation Ectopia, renal, 830–832 Ectopic pancreatic rests, 737 Ectopic ureter, 846–848 bilateral single, 847–848 in boys, 847 in girls, 846–848, 847f–848f incidence of, 846 management of, 847 Edema cytotoxic, 257–258 postextubation subglottic, 49 subglottic, 49 EELV. see End-expiratory lung volume EGF. see Epidermal growth factor Ehlers-Danlos syndrome, 304 Elbow, fractures of, 277 Electrical burns, 206–207, 206f Electrocautery, 1197 Electrolytes anesthesia preoperative evaluation of, 39 for duodenal atresia/stenosis management, 492–493 in neonates, 6–7, 6f of parenteral nutrition, 28–29 regulation, 57 Embolization angiographic, 1161–1162 in infantile hepatic hemangiomas management, 1035 Embryonal carcinoma, 1198t Embryonal rhabdomyosarcoma, 1115– 1116, 1116f Embryonic branchial apparatus remnants, 1173–1178, 1173f–1174f complete fistula, 1174–1175, 1176f excision of, 1173f first cleft anomalies, 1175–1177, 1176f incision and drainage of, 1175 physical examination of, 1175 resection of, 1175, 1177 second cleft anomalies, 1177, 1177f third and fourth cleft anomalies, 1177–1178, 1178f

1264

Index

Embryonic stage, in lung development, 380 Emergency laminectomy, 414–415 Emesis, postoperative, 481 Emotional stress, ulcerative colitis, 648 Emotional support, trauma, 218 Emphysema, subcutaneous, 427 Empyema, 361 classification scheme for, 362t diagnosis of, 361–362, 363f epidemiology of, 361 management of, 363–365, 364f pathogenesis of, 361, 362f pneumonia and, 361 Encephaloceles, 290 End-expiratory lung volume (EELV), 112, 113f Endocarditis prophylaxis, 43, 43b Endocrine disorders and tumors, 1217– 1239 Endodermal sinus tumor, 1198t Endometrioid carcinoma, 1198t Endometriosis, 1201–1202, 1201t, 1202f Endopyelotomy, 842, 842f Endoscopic retrograde cholangiopancreatography (ERCP) acute pancreatitis diagnosis, 738–739 choledochal cyst, 695 chronic pancreatitis, 743 in liver and spleen injury, 242, 242f Endoscopy, 422–423 in caustic ingestion, 428 in Crohn disease, 657 in foreign body esophageal injury, 426 in foreign body ingestion, 172–173 in gastroesophageal reflux diagnosis, 464 in ulcerative colitis, 649 Endothelial cells, hemostasis, 76–78 Endotoxin, 13 Endotracheal intubation in airway injuries, 338, 339t for esophageal atresia, 441 trauma resuscitation of, 215–216 End-stage liver disease (ESLD), 527 End-stage renal disease (ESRD) posterior urethral valves, 894 renal insufficiency in liver transplantation, 718–719 renal transplantation, 725 End-tidal carbon dioxide neonatal pulmonary monitoring, 10 Enema in simple meconium ileus management, 523 water-soluble contrast, 558 Energy expenditure, during illness, 20–21 Energy requirements, neonates, 18 Enteral nutrition, 25–28, 27f acute pancreatitis management, 740 Enterocolitis, Hirschsprung disease and, 571–572, 571t, 572f Enterostomy, Mikulicz double-barreled, 524, 525f Enuresis, 870 Enuretic alarms, 871

Environmental factors of hypertrophic pyloric stenosis, 478 hypospadias, 919 EPE. see Extended portoenterostomy Ependymoma, 297, 297f Epidermal cysts, 1083–1084 Epidermal growth factor (EGF), in intestinal barrier, 540 Epidermis, 196 anatomy of, 1128, 1128f–1129f Epidermoid cysts, 1179–1181 Epididymitis, testicular torsion vs., 825 Epidural analgesia, for pectus excavatum, 308–309 Epigastric hernia, 781, 782f Epiglottis, 333 Epiglottitis, 337, 337t Epignathus, 1080, 1080f Epilepsy, medically refractory, vagal nerve stimulators, 287 Epinephrine, as vasoactive medications in newborn, 12, 12t Epiphysis, in long bone, 268 Epispadias, 897 repair, bladder exstrophy, 908–909 Epstein-Barr virus (EBV) infections Hodgkin lymphoma and, 1098 post-liver transplantation, 719 preoperative renal transplantation, 727 ERCP. see Endoscopic retrograde cholangiopancreatography Erect abdominal radiography, 529, 529f Erlotirab, as anticancer drugs, 973t–974t Erosive esophagitis, 460 Erythema nodosum, 648, 648f Erythrocyte sedimentation rate (ESR), Hodgkin lymphoma and, 1100 Erythropoietin, 44 Escharotomy, 197, 198f ESLD. see End-stage liver disease Esophageal atresia, 437–458 associated anomalies of, 438 classification of, 438–439, 438f complications of, 447–450 anastomotic leaks in, 447 anastomotic stricture in, 447 disordered peristalsis in, 448–449 esophageal cancer in, 448–449 gastroesophageal reflux in, 448–449 recent outcome studies in, 449–450 recurrent tracheoesophageal fistula in, 447–448, 447f–448f respiratory morbidity in, 449 thoracotomy-related morbidity in, 449 tracheomalacia in, 448 vocal cord dysfunction in, 449 diagnosis of, 439–441 antenatal, 439 postnatal, 439–441, 439f–440f with distal fistula, 438, 441 positioning for, 444–445, 444f–445f preoperative bronchoscopy for, 441–442, 441f repair via thoracotomy for, 442– 444, 442f–443f thoracoscopic repair for, 444–445

Esophageal atresia (Continued) embryology of, 437 epidemiology of, 437–438 H-type fistula without, 439 management of, 441 preoperative, 441 operative repair for, 441–447 with proximal and distal fistula, 439 with proximal fistula, 439 without distal fistula, 445–447 esophageal reconstruction for, 446, 446b, 447f initial treatment for, 445–446 postoperative management for, 446–447 without TEF, 438 Esophageal cancer, 448–449 Esophageal injury, 233 Esophageal replacement, 430–433, 432f, 434f barium swallow in, 433f colon interposition, 433f complications of, 433 gastric transposition in, 430 gastric tube in, 430, 431f, 433f jejunal interposition graft in, 430 techniques in, 434t Esophagitis, erosive, 460 Esophagomyotomy, in achalasia, 424–425 Esophagus, 422–436 achalasia in, 424–425, 424f–425f anatomy of, 422 barriers to gastroesophageal reflux, 460 blood supply of, 422 caustic ingestion in, 428–429, 428t, 429f clearance, 462 clinical evaluation of, 422–423 congenital anomalies of, 423 contractions, 462 development of, 422 duplication, 630–631, 632t, 633f embryology of, 422 foreign body esophageal injury in, 425–427, 426f foreign body ingestion, 172–173, 172f–175f length of, 422, 461 muscularis mucosa in, 422 muscularis propria in, 422 perforation in, 427–428, 427f physiology of, 422 strictures in, 429–430, 429f–430f strictures management, 465 submucosa in, 422 wall, 422 ESR. see Erythrocyte sedimentation rate ESRD. see End-stage renal disease Estradiol, 1234 Estrogen, in gynecomastia, 1208 Etoposide, as anticancer drugs, 973t–974t Ex utero intrapartum treatment (EXIT), 353–354

Index

Excision for dermoid and epidermoid cysts, 1180–1181 of imperforate hymen, 1191 for pilocytic astrocytoma, 296–297 for thyroglossal duct cyst, 1172, 1173f Excisional biopsy, Hodgkin lymphoma and, 1100 EXIT. see Ex utero intrapartum treatment Exocrine tumors, pancreas, 746 Expandable metal stents, 343 Extended criteria donors (ECDs), 712 Extended laparoscopic esophagomyotomy, in achalasia, 424 Extended portoenterostomy (EPE), 683, 684f External genitalia, in sexual differentiation, 954, 954f Extracorporeal carbon dioxide removal (ECCO2R), 113 Extracorporeal cardiopulmonary resuscitation, 95 Extracorporeal life support (ECLS), 122 Extracorporeal membrane oxygenation (ECMO), 10–11, 91–110 advances and areas of research, 104–105 artificial placenta, 105, 106f ECMO II, 104 in premature neonates, 104–105 antibiotics and, 143 cannulation, 96–97, 96f–98f circuit, 98–99, 99f–100f clinical applications of, 91–92 cardiopulmonary failure, 91–92 complications of, 101–103 accidental decannulation, 101 air embolism, 101–102 coagulation abnormalities, 102 decannulation, 101 hemorrhagic complications, 102 hypertension, 102–103 mechanical complications, 101–102 membrane failure, 101 neurologic complications, 102 patent ductus arteriosus, 102 renal failure, 102 in congenital diaphragmatic hernia, 385–386 for congenital pulmonary airway malformation, 355 extracorporeal cardiopulmonary resuscitation, 95 history of, 91 indications for, 92–93 classic contraindications and possible treatment expansion, 93 clinical measurement systems, 92–93 congenital diaphragmatic hernia, 94–95 reversible cardiopulmonary disorders, 92 long-term sequelae, 103–104 neurodevelopment, 104 respiratory, 104 methods of, 95–99

Extracorporeal membrane oxygenation (ECMO) (Continued) operative procedures during, 101 outcomes of, 103, 103f, 104t patient management on, 99–101, 100f, 100t weaning, 101 patient selection criteria of, 92, 92b birth weight, 93 bleeding complications, 93 gestational age, 93 intracranial hemorrhage, 93 second course of, 95 unique indications for, 93–95 Extraintestinal symptoms Crohn disease, 657 ulcerative colitis, 648, 648b Extralobar bronchopulmonary sequestration (eBPS), 350–351, 351f postnatal management of, 355 Extrarenal Wilms tumor, 998 Extravaginal torsion, testicular, 821, 822f Extrinsic injuries, airway, 338, 338f

F

F508del mutation, 517 Facial hemihypoplasia, 1182 Facial trauma, 254–266 Factor IX, 78 Factor VII hemostasis, 76 recombinant activated, 85 Factor VIIa, hemostasis, 76 Factor VIII, 78 Factor X, 78 Factor XIII deficiency, 81 Familial adenomatous polyposis, 1221–1222 Familial form of multiple intestinal atresia (FMIA), 498 Familial hypocalciuric hypercalcemia, 1226 Family history anesthesia preoperative evaluation and, 37 nocturnal enuresis, 871 Fascial anomalies, cloacal exstrophy with, 912 Fasciotomy, 191–192 FAST. see Focused assessment with sonography for trauma Fasting guidelines, preoperative, of anesthesia, 38–39 Fat necrosis, 1209–1210 Fatty acids, 24 synthesis, 25f FC. see Functional constipation Fecal incontinence, 599–612 anal canal sensation in, 599–600, 600f bowel motility in, 600–601, 600f–601f intellectual and psychological capacity in, 601 mechanism of, 599–601 postoperative anorectal defect repair, 596 true, 601–603, 601f in children, 602–603 voluntary muscle structures in, 599

1265

Fecal soiling, in Hirschsprung disease, 570–571, 570f–571f, 570b Feeding strategies, for necrotizing enterocolitis prevention, 549 advancement, 549 initiation and trophic feeds, timing of, 549 Female gender assignment, 961–963, 961f–963f Female pseudohermaphrodite, 953t, 954–955, 955f Femoral hernia, 798, 798f Femur neck, fractures of, 268–270, 270f shaft fractures, 270, 270f Fentanyl, 50–51 Ferritin, in neuroblastoma, 1014 Fertility hypospadias, 931 posterior urethral valves, 894 postoperative bariatric surgery, 1247 preservation, 1202–1203 undescended testes, 807 Fetal hemoglobin, 4 oxygen dissociation curve, 4f Fetal surgery programs, 165–166, 166t Fetal therapy, 153–170 abdominal wall defects of, 164–165 access, 154–155 anesthesia of, 154–155 anomalies amenable to fetal surgery of, 155–165 for congenital diaphragmatic hernia, 391 fetal surgery programs, 165–166, 166t future work of, 166 gene therapy of, 165, 165b guiding principles of, 153–154, 154t hydronephrosis of, 163–164 indications of, 166 neoplasms, 157–159 low urinary tract obstruction of, 163–164 morbidity, 153 myelomeningocele, 161–163 operative setup of, 154–155 stem cells for, 165, 165b twin gestation abnormalities in, 160–161 monochorionic twins, 161 Fetal urine electrolytes, 164t Fetiform teratomas, 1066–1067, 1068f α-fetoprotein (AFP) in gastroschisis diagnosis, 764 in hepatoblastoma, 1044, 1050 in infantile hepatic hemangiomas, 1032–1033 in omphalocele, 771 in testicular neoplasms, 813 Fever appendicitis, 665 in esophageal perforation, 427 FGF-2. see Fibroblast growth factor-2 Fiberoptic bronchoscopy, inhalation injury of, 201–202 Fibrin formation, 78, 84 Fibrinogen, 80–81 Fibrinolysis, 78

1266

Index

Fibrinolytics, for empyema, 364 Fibroadenomas, 1213, 1213f Fibro-adipose vascular anomaly, 1165, 1165f Fibroblast growth factor-2 (FGF-2), 1035–1036, 1155 Fibrosarcoma, 1088, 1088f Fibrosing colonopathy, cystic fibrosis and, 530–531 Fibrous tissue tumors, 1088–1089 congenital epulis, 1088–1089, 1089f desmoid tumors, 1089 fibrosarcoma, 1088, 1088f keloid, 1089 myofibromas, 1088 myofibromatosis, 1088 nodular fasciitis, 1088 Fibula, nonphyseal fractures of, 271 Field triage, 215 Fine-needle aspiration (FNA) for breast masses, 1212 in mediastinal tumors, 404 FIP. see Focal intestinal perforation FISH. see Fluorescence in situ hybridization Fistula hypospadias, 930 rectobladderneck. see Rectobladderneck fistula rectoperineal. see Rectoperineal fistula rectourethral. see rectourethral fistula rectovestibular. see Rectovestibular fistula remnant of embryonic branchial apparatus and, 1174–1175, 1176f urethrovaginal, 597 vestibular. see Vestibular fistula Fistula-in-ano, 613, 614f–615f Fixation, malrotation and, 507 Flail chest, 225 Flame burns, 196 Flexible bronchoscopy, 179 Flexible endoscopy, 422–423 complications of, 423 Flexion/distraction injuries, 275–276 Flow rate, mechanical ventilation of, 115 Flow-volume loops, anterior mediastinal mass, 41 Fludarabine phosphate, as anticancer drugs, 973t–974t Fluid balance in duodenal atresia/stenosis management, 492–493 fluid overload, 61 neonatal acute kidney injury management of, 65 in neonates, 6–7, 6f water requirements, 58t Fluid overload, 61 Fluorescence in situ hybridization (FISH) MYCN, 1013 for non-Hodgkin lymphoma, 1107– 1108 Fluorodeoxyglucose-positron emission tomography (18FDG-PET) for Hodgkin lymphoma, 1101 in mediastinal tumors, 404

Fluoroscopy, anterior mediastinal mass, 41 5-Fluorouracil as anticancer drugs, 973t–974t in hepatoblastoma treatment, 1048 FMIA. see Familial form of multiple intestinal atresia FNA. see Fine-needle aspiration FNH. see Focal nodular hyperplasia Focal congenital hyperinsulinism, 744 Focal hepatic hemangioma, 1153 Focal intestinal perforation (FIP), 543 Focal nodular hyperplasia (FNH), 1037–1039 histology of, 1038, 1039f imaging of, 1038, 1038f incidence of, 1037–1038 treatment of, 1039 Focal segmental glomerulosclerosis (FSGS), 725 Focused assessment with sonography for trauma (FAST), in blunt abdominal trauma, 237, 238f Folate, 1247 Follicle-stimulating hormone (FSH), 806–807 Follicular thyroid cancer, 1223 Fontan procedure (total cavopulmonary anastomosis), 45 Food aspiration, 178 Food impaction, 177 Food products, stool consistency and, 603t Foot enlargement, Klippel-Trenaunay syndrome and, 1163–1164 Forearm fractures, 279, 280f Foregut cysts, duplications and, 411– 413, 413f–414f Foregut duplication, pancreatic cysts, 746 Foreign bodies, airways, bronchoscopy of, 179–180, 179f Foreign body ingestion, 171–180 airways, 177–180, 178f–179f, 178t food aspiration, 178 management of, 179 symptoms of, 172 batteries, 173–174, 175f–176f esophagus, 172–173, 172f–175f gastrointestinal system, 173, 175f incidence of, 172 magnets, 174–176, 176f–177f sharp, 176 stomach lesions, 486 Former preterm infants, anesthesia, 39–40 Formic acid injuries, 206 Four-port technique, gallbladder disease, 702–703, 703f–704f Fowler-Stephens orchiopexy, 811 FOXO, 1115 Fraction of inspired oxygen (FtO2), 113 Fractional excretion of sodium, 59 of substances, 59–60 Fractional Na excretion (FE Na), 7b Fracture-dislocations, of spine, 276–277

Fractures femur, 268–270, 270f of lower extremity, 268–271, 270f–272f open, 268, 268t remodeling of, 268 Salter Harris classification of, 268, 268f of upper extremity, 277–280, 277f–280f Frantz tumor, 747, 748f Free radical, in secondary brain injury, 257–258 Freehand circumcision, 939–940, 939f FSGS. see Focal segmental glomerulosclerosis FSH. see Follicle-stimulating hormone Full-sized allografts, liver transplantation donor selection, 712 Fulminant hepatic failure, 710 Functional constipation (FC), 608–611 failure of medical management, 608–609 goals of treatment, 608–611 indications for further testing, 609 surgical options, 609–611, 611t Functional megacolon, 570 Fundoplication, 465 redo, 472–474, 472f–473f Fungal infections, 719 Furosemide, 61 Fushi-Tarzu factor-1 (FTZ-F1), in sexual differentiation, 953

G

Galactorrhea, 1210–1211 Gallbladder disease, 695–708 biliary atresia, 681, 681f biliary dyskinesia, 701 cholangiogram, 702 choledocholithiasis, 702 cholelithiasis, 701–702 post-splenectomy, 702 sickle cell disease, 702 surgical technique four-port technique, 702–703, 703f–704f laparoscopic cholecystectomy, 702 single-site laparoscopic cholecystectomy, 703–704, 704f–705f studies, 703t, 705, 705t Ganciclovir, 720 Ganglion cells, in Hirschsprung disease, 557, 559, 560f Gas exchange, during high-frequency ventilation, 124 Gas flow, mechanical ventilation of, 115 Gastric acid hypersecretion, 526 in intestinal barrier, 537 Gastric injuries, 244 Gastric teratomas, 1078 Gastric transposition, in esophageal replacement, 430 Gastric tube, in esophageal replacement, 430, 431f, 433f Gastrinomas, 746 diagnosis, 746

Index

Gastroesophageal reflux (GER), 459–477 barrier against, 460–462 abdominal pressure, 462 angle of His, 461 collar of Helvetius, 460 esophageal clearance, 462 esophageal contractions, 462 esophageal length, 461 lower esophageal sphincter, 460 phrenoesophageal membrane, 460 saliva, 462 clinical manifestations of, 462–463, 462t delayed gastric emptying vs., 464 diagnostic evaluation of, 463–464 endoscopy, 464 manometry, 464 multichannel intraluminal impedance, 464 pH probe monitoring, 464 in esophageal atresia, 448–449 in former preterm infants, 39 medical management of, 463 dietary modifications, 463 non-pharmacologic therapies, 463 operative management of, 465–468, 465f fundoplication, 465 gastrostomy, 467–468, 468f–469f laparoscopic Nissen fundoplication, 465–467, 466f–467f, 467t outcomes, 468–472, 470t–471t postoperative care, 468 redo fundoplication, 472–474, 472f–473f pathophysiology of, 460, 461t congenital anomalies, 460 erosive esophagitis, 460 hiatal hernia, 460, 461f Gastroesophageal reflux disease (GERD), 460 in congenital diaphragmatic hernia, 394 cystic fibrosis and, 527 omphalocele and, 775 Gastrointestinal system, congenital diaphragmatic hernia in, 394 Gastrointestinal tract atresia, gastroschisis, 763–765, 764t–765t, 767–768 cloacal exstrophy, 911 failure, necrotizing enterocolitis and, 546–547 of foreign body ingestion, 173, 175f immunologic defenses of, 539 innate and adaptive immunity, 539 passive immunity, 539 obstruction, Meckel’s diverticulum, 641–642, 643f perforation, intestinal transplantation complications, 724 prophylaxis, ECMO, 99–100 prune belly syndrome, 945–946 reconstruction, cloacal exstrophy, 913 strictures, necrotizing enterocolitis and, 547, 547f surgical infection prevention of, 141–142 venous malformations of, 1160

Gastrojejunostomy, 493 Gastroschisis, 763–765, 763f closing, 768–769, 769f–770f diagnosis of, 764 embryology of, 763–764 etiology of, 763–764 fetal therapy for, 164 incidence of, 763–764 long-term outcomes of, 770–771 neonatal resuscitation for, 765, 765f, 765t omphalocele vs., 764t postnatal management of, 765–771 prenatal management and delivery of, 764–765 presentation of, 764 risk stratification of, 765, 766f surgical management of, 765 associated intestinal atresia, 765t, 767–768 postoperative course of, 769–770 primary closure of, 765–766, 767f staged closure of, 766–767, 768f–769f Gastrostomy, 467–468, 468f–469f Gaucher disease, 753 GCS. see Glasgow Coma Scale G-CSF. see Granulocyte colonystimulating factor GDNF. see Glial cell line-derived neurotrophic factor Gemcitabine, as anticancer drugs, 973t–974t Gender-assignment team, 960 Gender of rearing, cloacal exstrophy, 913 Gender ratio, choledochal cyst, 696 Gene therapy, 165, 165b General anesthesia, fetal therapy access, 154 Genetics biliary atresia, 680 Crohn disease, 656 hypospadias, 919 prune belly syndrome, 942 ulcerative colitis, 647 Genital bleeding, 1190–1191, 1190f, 1190b introital masses, 1190–1191 penetrating injuries, 1190 urethral prolapse, 1190, 1190f Genital herpes, circumcision, 936 Genitourinary tumors, rhabdomyosarcoma, 1121–1122, 1121f–1122f GERD. see Gastroesophageal reflux disease Germ cell neoplasms, 298 Germ cell tumors, 407–409, 815–817 teratomas as, 1066 Gestational age ECMO, 93 for posterior urethral valve, 884 GFR. see Glomerular filtration rate GGT. see γ-glutamyltransferase Girls, precocious puberty in, 1233 Glans shape, hypospadias, 924 Glanular urethra, embryology, 918

1267

Glasgow Coma Scale (GCS), 211, 215t in traumatic brain injury, 259, 259t, 260f Glial cell line-derived neurotrophic factor (GDNF), 557 Glomerular filtration rate (GFR) in neonates, 6–7 renal function of, 58–59, 59t Glucocorticoids for congenital diaphragmatic hernia, 383 Crohn disease management, 657 Glucokinase (GCK) mutations, 744 Glucose importance of, 23 metabolism, neonates, 2–4 GLUT-1 congenital hemangioma and, 1152 infantile hemangioma and, 1148 Glutamate, 258 Glutamine for necrotizing enterocolitis prevention, 549 protein quality of, 23 γ-glutamyltransferase (GGT), 1055 Glycemia, omphalocele, 771 Glycogen storage disease (GSD), 745 type 1, 1038 type 1A, 1040 Glypican 3 (GPC3), 1044 GM-CSF. see Granulocyte-macrophage colony-stimulating factors Goiter, 1217–1218 differential diagnosis of, 1218b Gomco circumcision, 937, 938f Gonadoblastoma, 1198t Gonads, biopsies, 960 Gore-Tex®, for congenital diaphragmatic hernia, 389 Gorham-Stout disease, 1158, 1159f Gotitinib, as anticancer drugs, 973t–974t GPC3. see Glypican 3 Graft to recipient body weight ratio (GRWR), 714 Graft-versus-host disease (GVHD), intestinal transplantation, 725 Granulocyte colony-stimulating factor (G-CSF), cancer chemotherapy, 971 Granulocyte-macrophage colonystimulating factors (GM-CSF), 13–14 Granulomas, Crohn disease, 656 Granulosa cell tumor, 1198t Graves disease, 1218–1219, 1218f, 1219t Grey Turner sign, 739 Growth cancer therapy complications, 972 of Crohn disease, 656 factors, cancer biology, 972 of mediastinal lymphomas, 405–406 of necrotizing enterocolitis, 547 sequelae, ECMO, 103–104 GRWR. see Graft to recipient body weight ratio GSD. see Glycogen storage disease Gubernaculum, 805 Gustilo-Anderson system, 268, 268t GVHD. see Graft-versus-host disease

1268

Index

Gynecology, pediatric and adolescent, 1187–1205 genital examination of, 1188–1191, 1189f normal anatomy of, 1188, 1188t puberty, 1188 Gynecomastia, 1208–1209, 1209f associated drugs, 1209t breast masses, 1211

H

Haddon Factor Phase Matrix, 212, 212f HAEC. see Hirschsprung-associated enterocolitis Haemophilus influenzae infection overwhelming postsplenectomy infection, 759 thyroglossal duct cyst and, 1173 Halo nevus, 1130, 1130f Halo vest immobilization, in cervical spine injuries, 274–275, 275f Hand fractures, 280 Hashimoto thyroiditis, 1217–1218 HAT. see Hepatic artery thrombosis HB-EGF. see Heparin-binding epidermal growth factor HCA. see Hepatocellular adenoma HCC. see Hepatocellular carcinoma HD. see Hirschsprung disease Head and neck sinuses, 1171–1186 inflammatory lesions of, 1182–1184 acute suppurative cervical lymphadenitis, 1182–1183, 1182f cat-scratch disease, 1183–1184 chronic lymphadenitis, 1183 mycobacterial lymphadenitis, 1183 lesions of embryonic origin in, 1171– 1182 dermoid and epidermoid cysts, 1179–1181 preauricular pits, sinuses, and cysts, 1178–1179, 1179f torticollis, 1181–1182, 1181f Head and neck tumors, rhabdomyosarcoma, 1119, 1120f Head injury, 254–266. see also Traumatic brain injury. initial evaluation for, 258–264 management of, 258–264 outcome of, 264 pathophysiology of, 255–258, 255f Head teratomas. see Teratomas; head and neck Head trauma, injury patterns of, 213 Health Resources and Service Administration (HRSA), liver transplantation, 711–712 Hearing impairment, in congenital diaphragmatic hernia, 394 Heat loss, in neonates, 6 Hedgehog congenital pancreatic anomalies, 737 in esophageal atresia, 437 Hedgehog/Notch complex, 557 Heidelberg Pouchitis Activity Score, 654–655

Helicobacter pylori infection bariatric surgery, 1243 in peptic ulcer disease, 482–483 Heller myotomy, in achalasia, 424 Hemangioendothelioma, Kaposiform, 1153–1154 Hemangioma, congenital, 1152, 1152f Hemicolectomy, right, 1234–1235 Heminephrectomy, upper-pole, 849 Hemodialysis acute kidney injury management of, 62 catheters, 138, 139f characteristics of, 62t post-renal transplantation, 726 Hemodynamic support, in hepatocellular adenoma management, 1041 Hemoglobin in liver and spleen injury, 241 in neonates, 4 Hemoglobin S disease, 85 Hemolytic anemia, neonates, 5 Hemophilia A, 82–83 clotting factor administration in, 83 viral infections of, 82 Hemophilia B, 82–83 viral infections of, 82 Hemorrhage in gastroesophageal reflux, 463 Meckel’s diverticulum, 641 sentinel, in vascular compression, 335–336 subarachnoid, 257, 257f subdural, 256–257, 257f Hemorrhagic anemia, neonates, 5 Hemostasis, 76 clinical evaluation of, 78–79, 79b clotting factor dosing of, 83 coagulation cascade of, 77f endothelial cells of, 76–78 fibrinogen, 80–81 genetic testing of, 81 inhibitor screening tests for, 81 laboratory evaluation of, 79–81, 80f activated partial thromboplastin time, 78 bleeding time, 79–80 flow cytometry, 81 PFA-100 analyzer, 79–80 platelet count, 79 platelet function studies, 81 prothrombin time, 80 specific factor assays, 81 thromboelastograph, 81 neonatal, 83 partial thromboplastin time, 80 platelets, 76–78, 77f Hemothorax, 224, 227, 227f in pectus repair, 313 Henoch-Schönlein purpura, testicular torsion vs., 825 Heparin, 100 Heparin-binding epidermal growth factor (HB-EGF), 540 of necrotizing enterocolitis, 549 Hepatic artery pseudoaneurysm, in liver injury, 249 Hepatic artery thrombosis (HAT), 716–717, 717b

Hepatic cirrhosis, 1051 Hepatic duct transection, 700 Hepatic failure, fulminant, 710 Hepatic hemangioma, 1153, 1153f differential diagnosis, 1153 diffuse, 1153 focal, 1153 multifocal, 1153 Hepatic portal vein absence, 1038 hypertension, 688, 688f thrombosis, 759 trauma, 700 Hepatic tumors, 1031–1065, 1031t–1032t, 1032f benign, 1031–1041 biliary atresia complications, 689 liver transplantation, 711 malignant, 1041–1056 new therapeutic treatment options for, 1056–1058, 1057f–1058f Hepaticoduodenostomy, choledochal cyst, 697–700 Hepaticojejunostomy biliary atresia, 686 choledochal cyst, 697–699 Hepatitis, 727 Hepatobiliary scintigraphy, 681–682 Hepatoblastoma, 1041–1050 associated conditions with, 1042b biology of, 1042 clinical presentation of, 1043–1044 cytogenetics of, 1042 epidemiology of, 1041–1042 histology of, 1042–1043, 1043f, 1043t imaging of, 1044, 1044f–1045f management, liver transplantation, 711 risk stratification of, 1044–1045, 1047t staging of, 1044–1045, 1046f, 1046t, 1048t treatment of, 1045–1050, 1048f outcomes of, 1050–1051 transplantation, 1050–1051 Hepatocellular adenoma (HCA), 1039– 1041, 1039t associated conditions, 1040 clinical presentation of, 1039–1040 histology of, 1041 imaging of, 1040, 1040f incidence of, 1039 treatment of, 1041 Hepatocellular carcinoma (HCC), 1051–1054 cirrhosis and, 1051 clinical presentation of, 1051–1052 conditions associated with, 1051b fibrolamellar variant, 1052 histology of, 1052–1053, 1052f–1053f imaging of, 1052, 1052f laboratory studies in, 1051–1052 liver transplantation, 711 predisposing factors for, 1051 transplantation for, 1053–1054, 1053t treatment of, 1053

Index

Hepatopulmonary syndrome, 689 Hereditary hemorrhagic telangiectasia (HHT), 1156 Hereditary spherocytosis (HS) accessory spleen, 750–751 cholelithiasis, 701 splenectomy, 751–752 Hermaphroditism, true, 957 Hernia uterine inguinale, 956–957, 957f Hernias epigastric, 781, 782f femoral, 798, 798f hiatal, 460, 461f incisional, 482 inguinal, 783–804 lumbar, 782 malrotation and, 507 Morgagni, 377, 394–395, 395f Spigelian, 781–782 umbilical. see Umbilical hernias Herpes simplex virus (HSV) infection, 727 Heterochromia of the iris, 414 Heterotaxy syndrome (HS), 515 HFJV. see High-frequency jet ventilation HHT. see Hereditary hemorrhagic telangiectasia Hiatal hernia, 460, 461f Hickman catheter, 135–136 High-frequency jet ventilation (HFJV), 124–125, 125f High-frequency oscillation, ECMO, 94 High-frequency vibration, ECMO, 94 Hinman syndrome, 873, 874f Hirschsprung disease (HD), 557–576, 599 -associated inflammatory bowel disease, 572 associated syndromes, 557, 558b clinical presentation of, 558–559 constipation in, 605–608, 606f–607f diagnosis of, 558–559, 558f–561f etiology of, 557 future directions of, 573 genetic basis of, 557 incidence of, 557 long-term outcomes of, 566–572 acquired aganglionosis, 568 enterocolitis, 571–572, 571t, 572f fecal soiling, 570–571, 570f–571f, 570b functional megacolon, 570 internal sphincter achalasia, 559f, 570 mechanical obstruction, 568, 569f motility disorder, 570 obstructive symptoms, 566–570, 568b, 569f persistent aganglionosis, 568 postoperative management of, 566 preoperative preparation of, 559 spectrum of, 557 surgical management of, 559–566 Bianchi procedure, 565–566 Duhamel procedure, 562, 563f, 568 Kimura procedure, 565, 568f laparoscopic pull-through, 562, 563f–566f long-segment aganglionosis, 564–566, 567f–568f

Hirschsprung disease (HD) (Continued) Martin procedure, 565, 568f serial transverse enteroplasty procedure, 565–566 Soave procedure, 561–562, 562f Swenson procedure, 561, 562f transanal pull-through, 562–564, 567f variant, 572–573 Hirschsprung-associated enterocolitis (HAEC), 559 Histidine, protein quality of, 23 HIV infection, 142 circumcision, 936 HL. see Hodgkin lymphoma HLA, biliary atresia, 680 HLA-W27, ulcerative colitis, 647 HNF4A, 744 Hodgkin lymphoma (HL), 406–407, 408f, 1097–1103 classification of, 1098–1099 clinical presentation of, 1099–1100, 1099t, 1100f complications of, 1103 diagnosis of, 1100, 1100b epidemiology of, 1097–1098 histologic subtyping of, 1098–1099, 1098f incidence of, 1097–1098, 1098f laboratory examination in, 1100 long-term sequelae, 1103 Reed-Sternberg (RS) cell in, 1097 staging of, 1100–1101, 1101f, 1101t subtypes of, 1099 treatment of, 1101–1102 chemotherapy in, 1102 principles of, 1101–1102 radiation therapy in, 1102 results of, 1103 second malignancies in, 1103 stage, histology, and response-based therapy in, 1102 Holliday-Segar method, fluid requirements, 28–29, 30t Hormonal treatment, for undescended testes, 808 Hormones, in sexual differentiation, 953, 953t Horner syndrome, 414, 415f Horseshoe kidney, 832–833, 832f surgery for, 998 Hospital preparedness, trauma, 220 Host defense, of surgical infections, 141 Host factors in cancer, 972 urinary tract infection, 855, 855f–857f HPS. see Hypertrophic pyloric stenosis HRSA. see Health Resources and Service Administration HS. see Hereditary spherocytosis; Heterotaxy syndrome H-type fistula, 450, 450f–451f Human bites, 182 Human chorionic gonadotropin (hCG) hypospadias, 919–920 for testicular neoplasms, 808f Human milk, for necrotizing enterocolitis prevention, 549

1269

Human papillomavirus (HPV) infection, 936 Humerus distal Salter Harris type I fractures in, 279 supracondylar fracture in, 277–278, 278f fractures of, 277–278 lateral condyle of, fractures of, 279, 279f Humoral and cell-mediated immunity, 142 Hydration, chronic pancreatitis management, 743 Hydrocele, inguinal hernia and, 788 Hydrocephalus, 285–287, 286f anesthetic intraoperative management of, 47 associated diseases, inguinal hernia, 785 Hydrocolpos, 580, 581f Hydrofluoric acid burns, 206 Hydromorphone, 51 Hydronephrosis, 163–164 Hydrops, 157 Hydrostatic reduction, 623–625, 624f Hydroxyacyl-coenzyme A dehydrogenase (HADH) mutations, 744 3β-Hydroxylase deficiency, 955 11β-Hydroxylase deficiency, 955 21-Hydroxylase deficiency, 954–955 17β-Hydroxysteroid oxidoreductase, 956 Hymen, 1188 imperforate, 1191, 1192f Hyoid bone, 1171 Hyperaldosteronism, primary, 1229– 1230 Hyperamylasemia, 739 Hyperbaric oxygen (HBO) therapy, inhalation injury of, 202 Hyperbilirubinemia, 5 Hypercalcemia differential diagnosis of, 1225, 1225b familial hypocalciuric, 1226 Hypercapnia in congenital diaphragmatic hernia, 388 permissive, 120–121 Hyperchloremic metabolic acidosis, 67 Hyperchromasia, 991–992, 992f Hypercortisolism, 1228–1229, 1230f etiology of, 1228, 1228b Hyperglycemia, in neonates, 3 Hyperinsulinism diffuse congenital, 744 focal congenital, 744 Hyperkalemia acute kidney injury, 61 obstructive uropathy, 67 Hypermotility, colonic, children with, 603 Hyperosmolar therapy, for traumatic brain injury, 262–263, 263t Hyperparathyroidism, 1225–1227 neonatal severe, 1226 primary, 1225–1226, 1225f–1226f secondary, 1227 tertiary, 1227

1270

Index

Hyperphosphatemia, 65 Hypertension autosomal dominant polycystic kidney disease and, 833 ECMO, 102–103 medically refractory intracranial, in traumatic brain injury, 263 renal transplantation complications, 729 in renal trauma, 249 renovascular. see Renovascular hypertension systemic, 66 Hypertonic saline fluid resuscitation in burns, 200–201 for traumatic brain injury, 262–263, 263t Hypertrophic pyloric stenosis (HPS), 478–482 diagnosis of, 478, 479f etiology of, 478 treatment of, 479–481 complications, 481–482 laparoscopic operation, 480–481, 481f open approach, 479–480, 480f outcomes, 481–482, 482f postoperative care, 481 pyloromyotomy, 479 Hypertrophy, breast disease and, 1207– 1208, 1207f Hyperuricemia, 1109 Hyperventilation, for traumatic brain injury, 264 Hypocalcemia, neonatal acute kidney injury management of, 66 Hypochloremic hypokalemic metabolic alkalosis, 479 Hypoganglionosis, 572 Hypoglycemia congenital hyperinsulinism, 744 neonates, 2 Hypomagnesemia, 4 Hypomotility, colonic, children with, 603 Hyponatremia, urine volumes, 61 Hypoplasia breast diseases and, 1207–1208 renal, 828–829, 829f Hypospadias, 918–934 anatomy of defect, 920–921, 920f–922f associated anomalies, 921–922 classification of, 918b clinical aspects of, 919–922 complications of, 930–931 bleeding, 930 devitalized skin flaps, 930 diverticulum, 931, 931f infection, 930 persistent chordee, 931 recurrent multiple, 931 retrusive meatus, 931 sexual function, 931 strictures, 930–931 urethrocutaneous fistula, 930 definition of, 918 embryology of, 918–919 etiology of, 919–920

Hypospadias (Continued) historical perspective of, 919 incidence of, 919 operative approaches for, 924–927, 925f distal variants, 924–925, 925f–926f midshaft variants, 925–926, 927f proximal variants, 927, 928f–929f results, 931–932 technical perspectives in, 927–930 analgesia, 928–930 dressings, 928 instruments, 927 optical magnification, 927–930 sutures, 927 urinary diversion, 927–928, 929f–930f treatment of, 922–924 age at repair, 923 cosmesis, 924 glans shape, 924 meatus location, 923–924 objectives, 923–924 straightening, 923, 923f urethral construction, 924 Hypotension of crotalid snakebite management, 188 intraoperative, 433 systolic, 217 in traumatic brain injury, 258 Hypothermia intraoperative, 143 selective, 100–101 therapeutic, for traumatic brain injury, 264 Hypothyroidism, 1219–1220 Hodgkin lymphoma and, 1103 infantile hepatic hemangiomas, 1032 Hypovolemic shock in neonates, 11 trauma resuscitation, 216–217 Hypoxia, in traumatic brain injury, 258 Hysterotomy, open surgery, 156

I

IBD. see Inflammatory bowel disease iBPS. see Intralobar bronchopulmonary sequestration IC. see Indeterminate colitis ICAM-1 (intercellular adhesion molecule-1), 680 ICH. see Intracranial hemorrhage ICP. see Intracranial pressure Idarubicin, as anticancer drugs, 973t–974t IDH-1, 1164 IDH-2, 1164 Idiopathic scrotal edema, testicular torsion vs., 825 Ifosfamide, as anticancer drugs, 973t–974t IHH. see Infantile hepatic hemangioma ILD. see Interstitial lung disease Ileal duplication, 632t Ileostomy, 722 permanent, 659

Imaging for acute pancreatitis diagnosis, 739 for diaphragmatic injuries, 228, 229f for Klippel-Trenaunay syndrome, 1163 for neural tumors, 414 for non-Hodgkin lymphoma, 406 for parapneumonic effusion, 362 for sexual differentiation disorders, 959 for undescended testes, 812–813 for urinary tract infection, 857 for Wilms tumor diagnosis, 994 Imatinib mosytate, as anticancer drugs, 973t–974t 131I-MIBG scanning, for pheochromocytoma, 1232 Imipramine, 871–872 Immature teratoma, 1198t Immobilization bladder exstrophy, 901, 902f in cervical spine injuries, 273, 274f–275f Immune system, 142 Immune thrombocytopenic purpura (ITP) accessory spleen, 750–751 management of, 84 corticosteroids, 752–753 intravenous immunoglobulin, 752–753 rituximab, 753 romiplostim, 753 splenectomy, 752 Immunodeficiencies, 142 melanoma and, 1136 Immunoglobulins, 14 Immunologic assessment, preoperative renal transplantation, 727 Immunosuppression intestinal transplantation, 721 complications, 724–725 liver transplantation, 715, 716t renal transplantation complications, 730–731 ulcerative colitis management, 650 Immunotherapy adoptive, 978 in renal cell carcinoma, 1003 Imperforate anus without fistula, as male defects, surgical repair of, 590 Imperforate hymen, 1191, 1191f–1192f Implantable cardioverter defibrillators, 44 IMV. see Intermittent mandatory ventilation Inappropriate antidiuretic hormone response, 7b Incarceration, inguinal hernia and, 788–790, 789f–790f Incision and drainage, 1175 Incontinence childhood, 870–871 continuous, 873 fecal, 599–612 anal canal sensation in, 599–600, 600f bowel motility in, 600–601, 600f–601f in children, true, 602–603 intellectual and psychological capacity in, 601

Index

Incontinence (Continued) mechanism of, 599–601 true, 601–603, 601f voluntary muscle structures in, 599 stress, 871 IND. see Intestinal neuronal dysplasia Indeterminate colitis (IC), 660 Infantile hemangioma, 1148–1152, 1149f associated congenital anomalies in, 1149–1150, 1150f clinical features of, 1148–1149, 1149f differential diagnosis of, 1149 imaging of, 1150 incidence of, 1148 local complications in, 1149 other manifestations of, 1150, 1150f pathophysiology of, 1148 periorbital, 1149, 1149f proliferative phase, 1148 treatment of, 1150–1152 Infantile hepatic hemangioma (IHH), 1031–1035 clinical presentation of, 1031–1033, 1032t histology of, 1034 imaging of, 1033–1034, 1033f incidence of, 1031 treatment of, 1034–1035, 1034t Infections biliary atresia, 680 in ECMO, 103 in hepatocellular carcinoma, 1051 Hodgkin lymphoma and, 1098 hypospadias, 921–922, 930 liver transplantation, 719–720 non-Hodgkin lymphoma and, 1109– 1110 pathogenesis of, 141 requiring surgery for, 147–149 necrotizing soft tissue infection, 147–148, 148f peritonitis, 148–149 sepsis, 148 thyroglossal duct cyst and, 1173 ulcerative colitis, 649 Inferior thyroid artery, 422 Inflammation choledochal cyst, 696 Meckel’s diverticulum, 642 Inflammatory bowel disease (IBD), 647–663 Inflammatory obstructions, 337, 337t Infliximab, 658 Infrequent voider, daytime incontinence, 873 Inguinal hernia, 783–804 associated diseases, 785, 785b, 786f classifications of, 785, 786f–787f clinical findings of, 785–790, 787f hydrocele, 788, 788f incarceration, 788–790, 789f–790f complications of, 800 chronic pain, 800 recurrence, 800 configurations of, 785f direct, 798 embryology of, 784–785

Inguinal hernia (Continued) epidemiology of, 784 history of, 784 hypospadias, 921–922 incidence of, 784 in infants, 796, 796f operative technique of, 790–796 anesthesia, 799–800 contralateral evaluation, 791–792 laparoscopic repair, 792–798, 792f–795f, 797t open repair, 790–792, 791f–792f, 797t timing of, 790 other, 799, 799f risk factors of, 785 sexual development, differences of, 798–799, 798f Inguinal pain, 787 Inhalation injury, of burns, 201–202 Inhibitor screening tests, hemostasis evaluation, 81 Injury Free Coalition for Kids, 212 Injury Severity Score (ISS), 211 Inoculum size, surgical infections of, 141 INPC. see International Neuroblastoma Pathology Classification INRG. see International Neuroblastoma Risk Group INSS. see International Neuroblastoma Staging System Insulin-like factor 3 (INSL3), 805 Insulin-like growth factor (IGF)-2, 1230 Insulinomas, 745 INSURE. see INtubate; SURfactant; Extubate Integra, 204t, 205 Intensity-modulated radiation therapy, 980 Intensive care, in vascular compression, 335–336 Intercellular adhesion molecule-1 (ICAM1), 680 Interdisciplinary Vascular Anomalies Center, 1165 Interferon, recombinant, 1151 Interferon-γ(IFN-γ), 537 Interleukin-1 (IL-1) septic shock, 13–14 Interleukin-6 (IL-6), 764 Interleukin-8 (IL-8), 764 Interleukin-11 (IL-11), 971 Interleukins, 977 Intermittent mandatory ventilation (IMV), 116, 117t, 118f Intermittent testicular pain, 824 Internal sphincter achalasia, 572–573 Hirschsprung disease and, 559f, 570 International Neuroblastoma Pathology Classification (INPC), 1011, 1013t International Neuroblastoma Risk Group (INRG), 1017–1020 International Neuroblastoma Staging System (INSS), 1017–1020, 1018t Interstitial lung disease (ILD), 368–369, 371t Intestinal atresia and stenosis, 489–506

1271

Intestinal barrier, 537–539 digestion in, 537–538 gastric acid in, 537 inflammation and injury, molecular mechanisms of, 539–540 epidermal growth factor, 540 lipopolysaccharides, 539 nitric oxide, 540 platelet-activating factor, 540 intestinal motility and, 537–538 mucous layer of, 538 tight junctions of, 538–539 Intestinal dysmotility syndrome, 721 Intestinal injury, blunt, 244–247 colonic injury in, 245 duodenal injuries in, 244, 245f, 245t gastric injuries in, 244 rectal injury in, 245–247, 246f small bowel injuries in, 244–245, 246f Intestinal motility gastroschisis postoperative care and, 769 intestinal barrier and, 537–538 Intestinal neuronal dysplasia (IND), 570, 572 Intestinal transplantation, 721–725 contraindications, 722 for gastroschisis, 765 immunosuppression, 721 indications, 721–722, 722b operative considerations, 722 outcome, 725 postoperative complications, 724–725 allograft rejection, 724–725 graft-versus-host disease, 725 immunosuppression, 724–725 infection, 725 procedure, 722–724 isolated small bowel allograft, 724, 724f liver-small bowel composite allograft, 723, 723f–724f multivisceral allograft, 722–723 rehabilitation, 721 total parenteral nutrition, 721 Intra-abdominal adhesions, in malrotation management, 513 Intracardiac teratomas, 1077 Intracranial hemorrhage (ICH), ECMO, 93 Intracranial infections, 299–300, 299f Intracranial pressure (ICP) cerebral blood flow and, 255 monitoring, for traumatic brain injury, 262 volume relationship, 255, 255f Intrafascial overgrowth, KlippelTrenaunay syndrome and, 1163 Intrahepatic bile duct development, 680 Intrahepatic cysts, 689, 689f Intralesional corticosteroids, for infantile hemangioma, 1151 Intralesional sclerotherapy, for venous malformations, 1160 Intralobar bronchopulmonary sequestration (iBPS), 350 postnatal management of, 355

1272

Index

Intraoperative awareness, postanesthesia care, 49–50 Intraoperative cholangiography, 697, 697f Intraoperative complications, choledochal cyst, 700 Intraoperative hypotension, 433 Intraoperative radiation therapy, 980 Intraosseous (IO) access, 137 Intraparenchymal monitors, in intracranial pressure monitoring, 262 Intraperitoneal teratomas, 1077 Intrarenal reflux, 859 Intraspinal extension, of neuroblastoma, 1025–1026 Intrathecal baclofen pump, 287 Intrathoracic bleeding, in hemothorax, 227 Intrauterine growth retardation (IUGR), 2, 3f Intravaginal torsion, testicular, 821 Intravascular extension, in Wilms tumor, 993f, 996–997 Intravenous fluids appendicitis management, 668 for neonatal anorectal defect, 583 ulcerative colitis management, 650 Intravenous immunoglobulins (IVIGs) immune thrombocytopenic purpura, 752–753 septic shock therapy, 14 Intraventricular devices, in intracranial pressure monitoring, 262 Intrinsic acute kidney injury, neonates, 64 Intrinsic injuries, airway, 337–338 Introital masses genital bleeding and, 1190–1191 vagina, 1190–1191, 1191f INtubate, SURfactant, Extubate (INSURE), 9 Intussusception, 621–628 clinical presentation, 621 cystic fibrosis and, 530 diagnosis, 622–623 abdominal radiography, 622 CT, 623, 624f MRI, 623 US, 622–623, 623f historical aspects, 621 incidence, 621 nonoperative management, 623–625 operative management, 625–626 laparoscopic approach, 625, 625f–626f open approach, 626, 627f pathophysiology, 621 physical examination, 621–622, 622f postoperative, 627 primary, 621 prolapse, 622 recurrent, 626–627 secondary, 621 Invasive monitoring, 383 Inverse ratio ventilation (IRV), 119 Iodine-131 (131I), for Graves disease, 1219 Iodothyronine deiodinase, 1032

IORT. see Intraoperative radiation therapy Ipilimumab, for melanoma, 1139–1140 IRF4, nevi and, 1130 Irinotecan as anticancer drugs, 973t–974t in hepatoblastoma treatment, 1050 Irritability, in gastroesophageal reflux, 462 IRV. see Inverse ratio ventilation Isolated frequency syndrome, 873 Isolated lambdoidal synostosis, 291 Isolated small bowel allograft, 724, 724f Isoproterenol for neonatal cardiogenic shock, 12 as vasoactive medications in newborn, 12t ISS. see Injury Severity Score ITP. see Immune thrombocytopenic purpura IUGR. see Intrauterine growth retardation IVIGs. see Intravenous immunoglobulins

J

Jagged-1 gene, 680 Jarcho-Levin syndrome, 324, 327, 327f Jaundice biliary atresia diagnosis, 681 cholestatic, 527 in neonates, 5, 5t Jejunal duplication, 632t Jejunal interposition graft, in esophageal replacement, 430 Jejunoileal atresia/stenosis, 495–502 autosomal inheritance, 495 clinical manifestations of, 498–499 diagnosis of, 499–500, 499f differential diagnosis of, 500 etiology of, 495, 495f Grosfeld classification system, 495, 496f management of, 500 operative considerations of, 500–501 pathology of, 495–498 atresia type I, 496, 496f–497f atresia type II, 496, 496f atresia type IIIa, 496, 496f–497f atresia type IIIb, 496–498, 496f–497f atresia type IV, 496f, 498 stenosis, 495–496, 496f pathophysiology of, 498 surgical considerations of, 499f, 500 postoperative care, 501–502 prognostic factors, 501 Jeune syndrome, 302, 327, 327f. see also Asphyxiating thoracic dystrophy. Jumbled spine, 324, 327 Juvenile granulosa cell tumor, 1198t Juvenile hypertrophy, breast masses, 1211 Juvenile papillomatosis, breast masses and, 1212

K

K antigens, 856 Kaposiform hemangioendothelioma, 411, 1153–1154, 1154f Kasabach-Merritt phenomenon (KMP), 411, 1153

Kasai hepatic portoenterostomy, 679 modified, 684f, 686 Kelly operation, bladder exstrophy, 908 Keloid, 1089 Keratin-8 gene, 680 Keratin-18 gene, 680 Kernicterus, neonatal jaundice, 5 Ketone bodies, 24 Ketorolac, 51 Kidneys Ask-Upmark, 828–829 cystic tumors, 833–835 developmental and positional anomalies of, 827–836 embryology, 828, 828f fusion defects, 832–833 lumbar, 831 multicystic dysplastic, 834 oligomeganephronic, 828–829 prune belly syndrome, 944 supernumerary, 830 Kimura procedure, for Hirschsprung disease, 565, 568f Klinefelter syndrome, 958 Klippel-Feil anomalies, 1181 Klippel-Trenaunay syndrome (KTS), 1162–1164, 1162f–1163f KMP. see Kasabach-Merritt phenomenon Knee-chest position, in genital examination, 1188 Knee injuries, 271 Kocher maneuver, in malrotation management, 511 Kropp’s procedure, for neurogenic bladder, 878 KTP. see Potassium titanyl phosphate KTS. see Klippel-Trenaunay syndrome Kyphoscoliosis, 324, 327

L

La Roque maneuver, 789–790 Labial adhesions, 1189 Laboratory studies anesthesia preoperative evaluation and, 39 appendicitis, 665 Hodgkin lymphoma and, 1100 of necrotizing enterocolitis, 541–542 renal function evaluation and, 65 Lactate dehydrogenase (LDH) in hepatocellular carcinoma, 1051– 1052 in neuroblastoma, 1014 in parapneumonic effusion, 361 Lactose intolerance, 501–502 Laminectomy, emergency, 414–415 Laminotomy, 414–415 Langerhans cell histiocytosis (LCH), 288, 417 Laparoscopic appendectomy, 668–669 Laparoscopic approach anesthetic intraoperative management of, 46–47 choledochal cyst, 698, 698f–699f Crohn disease management, 657f, 658 in duodenal atresia/stenosis management, 493–494, 494f

Index

Laparoscopic approach (Continued) in gastroesophageal reflux management, 471–472 intussusception management, 625, 625f–626f in malrotation management, 511–513, 512f–513f Meckel’s diverticulum management, 645, 645f in ovarian cysts management, 1197, 1200f ulcerative colitis surgery, 653–654, 654f–655f Laparoscopic cholecystectomy gallbladder disease, 702 sickle cell disease and, 87 Laparoscopic Nissen fundoplication, in gastroesophageal reflux management, 463, 465–467, 466f–467f, 467t Laparoscopic operation, of hypertrophic pyloric stenosis management, 480–481, 481f Laparoscopic pull-through, of Hirschsprung disease, 562, 563f–566f Laparoscopic pyeloplasty, ureteropelvic junction obstruction, 842 Laparoscopic rectopexy, 616–618 Laparoscopic repair, of inguinal hernia, 792–796, 792f–795f Laparoscopic splenectomy, 754–755, 754f–757f complications, 758–759 accessory spleen, 758 open splenectomy conversion, 758 operative time, 758 postoperative hospitalization, 758–759 splenomegaly, 758 Laparoscopic transabdominal adrenalectomy, 1232 Laparoscopic vertical sleeve gastrectomy (LSG), 1243, 1245f Laparoscopy, in abdominal trauma, 247f, 248 Laparoscopy-assisted cholangiography, biliary atresia diagnosis, 683 Laparotomy diagnostic, for undescended testes, 809 Hodgkin lymphoma and, 1101 for necrotizing enterocolitis, 544, 544f for penetrating injuries, 219 Lapatinib, as anticancer drugs, 973t–974t Laryngeal cleft, 450–452 Laryngeal nerve injury, parathyroid glands and, 1222, 1222f Laryngeal webs, 334, 334f Laryngoscopes, in endotracheal intubation, 338 Laryngospasm, postanesthesia care, 49 Laryngotracheal obstruction, 332–347, 332t airway injuries in, 337–339 anatomy of, 332, 332f embryology of, 332

Laryngotracheal obstruction (Continued) inflammatory obstructions in, 337, 337t laryngeal clefts in, 339–341, 340f subglottic malformation in, 332–336 tracheal agenesis in, 346, 346f tracheal clefts in, 339–341, 340f tracheal malformation in, 332–336 tracheal repair in, 341–346 tracheomalacia-bronchomalacia in, 336–337 vascular compression in, 334–336, 335f–337f barium esophagogram, 335 CT, 335–336 dyspnea, 335 intensive care, 335–336 MRI, 335–336 pulmonary vascular sling, 334–335 Sengstaken-Blakemore tube, 335–336 sentinel hemorrhage, 335–336 stridor, 335 US in, 335–336 Laryngotracheobronchitis, characteristics of, 337t Laryngotracheoesophageal cleft, 450–452 classification systems for, 452f, 452t contrast radiographs in, 451 incidence of, 450 tracheobronchoscopy in, 451, 453f Laryngotracheoplasty, 344, 345f Larynx, 333 clefts in, 339–341, 340f Laser Doppler imaging, burn depth, 202–203 Laser therapy for infantile hemangioma, 1151 for twin-twin transfusion syndrome, 160 Lateral pancreatojejunostomy (modified Puestow procedure), 743, 743f Latex allergy, 37 Latissimus dorsi muscle, 390 Latrodectus mactans (black widow spider), 186 LCH. see Langerhans cell histiocytosis LDH. see Lactate dehydrogenase LDHL. see Lymphocyte-depleted Hodgkin lymphoma Left lateral segment (LLS) allografts, liver transplantation, 712 Left ventricular dysfunction, 384 LES. see Lower esophageal sphincter Leukocyte esterase, 854 Levonorgestrel, 1202 Leydig cells, 805 abnormalities, 957 LH. see Luteinizing hormone LHR. see Lung-to-head ratio Li Fraumeni syndrome, 1115 Ligament of Treitz, 509 Light criteria, 361 LILT. see Longitudinal intestinal lengthening and tailoring Limb hypertrophy, Klippel-Trenaunay syndrome and, 1163

1273

Linoleic acid, 24 Linolenic acid, 24 Lipases, 739 Lipid metabolism, 24–25, 29f requirements, 24–25, 29f stress response of, 18 Lipopolysaccharides (LPS), 539 Lipoprotein-X, 681–682 Liposarcoma, 417 Liver anatomy, 1032f lesions of, 1031–1065. see also Hepatic tumors. Liver function testing, 648–649 Liver injury, 237–242, 239f angioembolization in, 242, 242f bleeding delayed, 241 operative management of, 238–240, 241f complications of, 249 endoscopic retrograde cholangiopancreaticography in, 242, 242f hemoglobin in, 241 length of stay in, 240–241 nonoperative failure of, 238, 240t guidelines for, 237–238, 240f operation, defining the need for, 241–242 reimaging after, 249 transfusion in, 241 Liver transplantation, 709–720 biliary atresia, 679–680, 690–691 contraindications, 711 for cystic fibrosis, 527–528 donor considerations, 711–714 donor options, 711, 712f donor selection, 712–714, 714f organ allocations, 712b, 713f in hepatoblastoma treatment, 1050– 1051 in hepatocellular carcinoma, 1053– 1054, 1053t immunosuppressive management, 715, 716t indications, 709–711, 710t Alagille syndrome, 709–710 biliary atresia, 709 fulminant hepatic failure, 710 liver tumors, 711 metabolic disease, 710, 710b, 711t in infantile hepatic hemangiomas management, 1035 operative procedure, 714–715 biliary tract reconstruction, 715, 715f piggy-back implantation, 715 orthotopic, 1050 outcomes, 720, 720b, 721f postoperative complications, 715–720, 716f acute cellular rejection, 718 biliary complications, 717–718, 718f chronic rejection, 718

1274

Index

Liver transplantation (Continued) infections, 719–720 primary nonfunction, 716, 717b renal insufficiency, 718–719 vascular thrombosis, 716–717, 717b preoperative preparation, 714 retransplantation, 720 Liver-small bowel composite allograft, 723, 723f–724f Lobectomy for congenital pulmonary airway malformation, 352 for right middle lobe syndrome, 371 Lobular capillary hemangiomas, 1084, 1084f Local anesthesia, 41 for hypertrophic pyloric stenosis management, 480–481 Lomustine, as anticancer drugs, 973t–974t Longitudinal intestinal lengthening and tailoring (LILT), 721 Longitudinal tracheoesophageal folds, 437 Long-segment aganglionosis, 564–566, 567f–568f Loperamide, 501–502 Loss of heterogeneity, in Wilms tumor (WT), 989, 989f Loss of heterozygosity, rhabdomyosarcoma and, 1115 Lower esophageal sphincter (LES), 422 barriers to gastroesophageal reflux, 460 dilation of, 424 Lower extremity, fractures of, 268–271, 270f–272f Lower urinary tract obstruction (LUTO) of fetal therapy, 163–164 posterior urethral valves, 884 Loxoscelism, 184, 184f differential diagnosis of, 185b incidence of, 184 laboratory findings of, 185b LPS. see Lipopolysaccharides LRHL. see Lymphocyte-rich Hodgkin lymphoma LSG. see Laparoscopic vertical sleeve gastrectomy Lumbar drain, for traumatic brain injury, 264 Lumbar fractures, in spinal injuries, 275–277, 276f Lumbar hernia, 782 “Lumbar kidney”, 831 Lund and Browder chart, 197–198, 198f Lung(s) abscess, 365, 365f–366f acquired lesions of, 361–376 biopsy, for interstitial disease, 369 development of, 380 maturation of, 8 Lung lesions, 157–158 Lung-to-head ratio (LHR) congenital diaphragmatic hernia, 155 in congenital diaphragmatic hernia, 381 ECMO, 94–95

Luteinizing hormone (LH) in sexual differentiation, 954 undescended testes, 806–807 LUTO. see Lower urinary tract obstruction Lymph nodes biopsy, 995 rhabdomyosarcoma, 1117 Lymphatic malformations, 1156–1158 clinical features of, 1156, 1157f Gorham-Stout disease, 1158, 1159f imaging, 1156, 1157f lymphedema, 1158, 1159f treatment of, 1157–1158 Lymphatic systems, embryology and development of, 1155 Lymphedema, 1158, 1159f Lymphedema distichiasis syndrome, 1158 Lymphoblastic lymphoma, 1103 prognostic risk factors in, 1108 Lymphocyte-depleted Hodgkin lymphoma (LDHL), 1099 Lymphocyte-rich Hodgkin lymphoma (LRHL), 1099 Lymphoid hyperplasia, appendicitis, 664 Lymphomas, 405–407, 1097–1114 classification of, 1097 undifferentiated, 405 Lymphoscintigraphy, for melanoma, 1139, 1139f

M

Macrogynecomastia, 1209 Macrophage migration inhibitory factor, 680 Mafenide acetate, 204, 204t Maffucci syndrome, 1164 Magnesium, hypomagnesemia, 4 Magnetic resonance angiography (MRA), of arteriovenous malformation, 1161, 1161f Magnetic resonance cholangiopancreatography (MRCP) acute pancreatitis diagnosis, 740, 740f choledochal cyst, 697, 697f chronic pancreatitis, 743 Magnetic resonance imaging (MRI) of appendicitis, 667 of cervical spine injuries, 272–273, 273f of congenital diaphragmatic hernia, 382, 382f for congenital diaphragmatic hernia surgery prognosis, 155 of esophageal atresia, 439 of focal nodular hyperplasia, 1038 of foregut cysts, 412 of genital examination, 1188 of hepatoblastoma, 1044 of hepatocellular adenoma, 1040 of hepatocellular carcinoma, 1052, 1052f of hydrocephalus, 286 of infantile hemangioma, 1150 of infantile hepatic hemangiomas, 1033 for intussusception diagnosis, 623 of mediastinal tumors, 404

Magnetic resonance imaging (MRI) (Continued) of medulloblastoma, 297, 297f of mesenchymal hamartoma, 1036 for neuroblastoma diagnosis, 1016, 1016f of pectus excavatum, 307 of pheochromocytoma, 1232 of remnants of embryonic branchial apparatus, 1175 of renal agenesis, 830 of teenage malrotation, 514 of ulcerative colitis, 649 of vascular compression, 335–336 of venous malformation, 1160 Magnetic resonance urography for posterior urethral valves, 887 for ureteropelvic junction obstruction, in children, 839, 841f Magnets, as foreign body ingestion, 174–176, 176f–177f MAGPI. see Meatal advancement and glansplasty Major bile duct injury, in liver injury, 249 Male gender assignment, 961 Male pseudohermaphrodite, 955–956 Malignant epithelial tumors, 1085 Malignant hyperthermia associated muscle disease, 38b susceptibility, anesthesia preoperative evaluation and, 37–38 treatment of, 38b Malignant thymic tumors, 410 Malleability, of chest, in pectus excavatum, 304 Malrotation, 507–516 associated anomalies with, 508t atypical, 514 congenital anomalies and, 514 diagnosis of, 509–510, 509f embryology, 507, 508f heterotaxy syndrome and, 515 incidence of, 507–508 incomplete rotation, 507, 508f management of, 510–513 laparoscopic approach, 511–513, 512f–513f open approach, 510–511, 510f–511f, 510b postoperative, 513 preoperative, 510 midgut volvulus and, 507 nonrotation, 507, 508f in older patient, 514, 514f–515f presentation of, 507–509 reversed rotation, 507 special considerations of, 514–515 superior mesenteric artery and, 507 Management of myelomeningocele study (MOMS), 162, 162t Mannitol, for traumatic brain injury, 262–263, 263t Manometry, 422, 464 Manual detorsion, 823 Marfan syndrome, 304 Marlex®, for congenital diaphragmatic hernia, 389

Index

Marsupialization in mesenchymal hamartoma management, 1037 splenic cysts, 751, 752f Martin procedure, 565, 568f Mastalgia, 1210 Mastitis, 1210, 1210f Maternal hysterectomy with resection, 162f Mayer-Rokitansky syndrome, 830, 831f Mayer-Rokitansky-Küster-Hauser syndrome, 959 uterovaginal anomalies and, 1193, 1193f–1194f McCune-Albright syndrome, 1233 Meatal advancement and glansplasty (MAGPI), 924–925, 925f–926f Meatal stenosis, 879 Meatus location, hypospadias, 923–924 Mechanical complications, ECMO, 101–102 Mechanical obstruction, of Hirschsprung disease outcomes, 568, 569f Mechanical ventilation adjunctive maneuvers of, 123 adjuncts to, 124–126 in airway injury, 230 carbon dioxide elimination of, 111– 113 compliance, 112, 113f dead space (Vd), 111–112 end-expiratory lung volume, 112, 113f positive end-expiratory pressure, 112 tidal volume, 116f total lung capacity, 112 volume/pressure relationship, 112, 112f components of, 115–116, 115f for congenital diaphragmatic hernia, 384–385 high-frequency ventilation of, 124– 125, 125f intermittent mandatory ventilation of, 116, 117t, 118f management of, 119–120 modes of, 116–119, 117t airway pressure release ventilation, 119, 119f assist-control ventilation, 117, 117t bilevel control of positive airway pressure, 119 continuous positive airway pressure, 118–119 controlled mechanical ventilation, 116, 117t inverse ratio ventilation, 119 neurally adjusted ventilatory assist/ noninvasive ventilation with neurally adjusted ventilatory assist, 118 pressure support ventilation, 117, 117t proportional assist ventilation, 117t, 118 synchronized intermittent mandatory ventilation, 116–117, 117t, 118f

Mechanical ventilation (Continued) volume support ventilation, 117, 117t volume-assured pressure support ventilation, 117, 117t nitric oxide administration of, 125– 126, 125f nonconventional modes to, 124–126 oxygenation of, 113–115, 114f fraction of inspired oxygen (FtO2), 113 oxygen delivery, 114 partial pressure of oxygen in arterial blood, 113 in pediatric surgical disease, 111–132 physiology of gas exchange during, 111–124 respiratory failure of, 120–124 early mobilization, 122 oral hygiene and mucus clearance, 122–123 permissive hypercapnia, 120–121 positive end-expiratory pressure, 121–122, 121f, 121b prone positioning, 122 ventilator-induced lung failure prevention, 120, 121f surfactant replacement therapy of, 126, 126f ventilator-associated pneumonia of, 126–128, 127b weaning from, 123–124, 124b Mechlorethamine, as anticancer drugs, 973t–974t Meckel diverticulum, 641–646 clinical presentation, 641–642, 643f bleeding, 641 inflammation, 642 intestinal obstruction, 641–642, 643f diagnosis, 642–644 differential diagnosis, 643–644 epidemiology, 641, 642f historical aspects, 641, 642f male:female ratio, 641 treatment, 644–645, 644f–645f Meconium disease, 517–535. see also Cystic fibrosis. distal intestinal obstruction syndrome, 525, 528–530, 528f–529f meconium plug syndrome, 526–527, 526f outlook of, 531 Meconium ileus (MI), 517, 518f. see also Cystic fibrosis. cholestasis and, 525–526 clinical presentation of, 520 complicated, 521–526 operative management of, 524 complications of, 527–531 gastric acid hypersecretion and, 526 nutritional management of, 525–526 pancreatic insufficiency and, 517–518 postoperative management of, 524–525 prognosis of, 526 pulmonary management of, 526 radiographic features of, 522–523, 522f contrast enema, 522–523, 522f

1275

Meconium ileus (MI) (Continued) simple, 521, 521f–522f nonoperative management of, 523 operative management of, 523–524, 524f–525f Meconium plug syndrome (MPS), 526–527, 526f Medial end, of clavicle, injuries of, 277 Median sternotomy, for teratomas, 408–409 Mediastinal cysts, 412 Mediastinal disease, Hodgkin lymphoma and, 1099–1100 Mediastinal mass, anterior, 41, 42f Mediastinal teratomas, 408, 1070t, 1076–1077, 1076f Mediastinal tumors, 403–421 clinical features of, 403 diagnostic imaging in, 403–404 location of, 404t management of, principles of, 404– 405, 405f primary, classification of, 404t rare, 416f–417f, 417 Mediastinum, 403 Medically refractory epilepsy, vagal nerve stimulators, 287 Medically refractory intracranial hypertension, in traumatic brain injury, 263 Medullary thyroid cancer (MTC), 1221, 1223, 1223f–1224f Medulloblastoma, 297, 297f Megacolon, functional, 570 Megalourethra, 879–880, 880f Megarectosigmoid colon, 600, 600f Megaureter, 845–846, 846f classification of, 845, 845b diuretic renography for, 845 primary obstructive, 845 treatment of, 845–846 US, 845–846, 845f Meige disease, 1158 Melanocytes, 1129f Melanocytic nevi, 1128 acquired, 1128–1131 clinical and histologic features of, 1129t congenital, 1131–1133 Melanocytosis, neurocutaneous, 1132–1133 Melanoma, 1127–1146 adjuvant treatment for, 1139–1141 epidemiology of, 1135–1136 outcomes of, 1138 presentation of, 1136–1137, 1137f prevention of, 1141, 1141t primary site of, 1137 risk factors for, 1136 surgical treatment of, 1138–1139, 1138t, 1139f–1140f Melphalan, as anticancer drugs, 973t–974t Membrane failure, ECMO, 101–102 Meningoceles, 288 Mercaptopurine, as anticancer drugs, 973t–974t Mesalamine, 650

1276

Index

Mesenchymal hamartoma, 1035–1037 clinical presentation of, 1036 epidemiology of, 1035–1036 histology of, 1036–1037, 1036f–1037f imaging of, 1036 incidence of, 1035 treatment of, 1037 Mesenteric angiography, Meckel’s diverticulum diagnosis, 644 Mesenteroaxial gastric volvulus, 484 Mesoblastic nephroma, 1003–1004, 1004f Mesoderm, tumors of, 1088 Mesonephros, 828 Metabolic acidosis in acute kidney injury, 61 neonatal acute kidney injury management of, 65 Metabolism liver transplantation, 710, 710b, 711t stress, 18 Metaiodobenzylguanidine imaging, in neuroblastoma diagnosis, 1017, 1017f Metaphysis, of long bone, 268 Metastatic disease, rhabdomyosarcoma and, 1122–1123, 1122f–1124f Methicillin-resistant Staphylococcus aureus (MRSA), 1210 Methimazole (MTH), 1219 Methotrexate adverse effects, 972 for cancer chemotherapy, 973t–974t Methylprednisolone, 186 Metronidazole Crohn disease management, 657–658 perforated appendicitis, 671–672 postoperative pouchitis, 654–655 tetanus management of, 183 MI. see Meconium ileus MIBG therapy, in neuroblastoma management, 1027 Microbiome, necrotizing enterocolitis and, 541 Microcystic (solid) congenital cystic adenomatoid malformations, 157 Microgastria, 483–484, 484f Midgut volvulus, malrotation and, 507 MIF. see Müllerian inhibitory factor MII. see Multichannel intraluminal impedance Mikulicz double-barreled enterostomy, 524, 525f Milan criteria, in hepatocellular carcinoma liver transplantation, 1053–1054 Miller laryngoscope, 338 Milrinone for neonatal cardiogenic shock, 13 as vasoactive medications in newborn, 12t Milroy disease, 1158 Mineral supplements, 1248 Minimally invasive approach, for congenital diaphragmatic hernia, 387–388, 388f Minimally invasive Kasai portoenterostomy, 686–687

Minimally invasive pectus repair, for pectus excavatum, 307–309, 309f–312f Mitchell technique, 909 Mitotane, 1231–1232 Mitoxantrone, as anticancer drugs, 973t–974t Mixed cellularity Hodgkin lymphoma, 1099 Mixed germ cell tumor, 816–817, 1198t Mixed gonadal dysgenesis, 957–958, 958f MMF. see Mycophenolate mofetil Modern staged repair of exstrophy (MSRE), 908–909 bilateral ureteral reimplantation, 909 bladder closure, 908 bladder neck reconstruction, 909 epispadias repair, 908–909 Mitchell technique, 909 modified Cantwell-Ransley technique, 908–909 postoperative care, 908–909 Young-Dees-Leadbetter technique, 909 Modified Aldrate score, 52t Modified Cantwell-Ransley technique, 908–909 Modified Kasai original portoenterostomy, 684f, 686 Modified Puestow procedure (lateral pancreatojejunostomy), 743, 743f Mogen clamp, 937 MOMS. see Management of myelomeningocele study Monfort abdominoplasty, 948, 948f Monitoring acute pancreatitis management, 740 of anesthesia, 46 in hepatocellular adenoma management, 1041 invasive, 383 Monoclonal antibodies, 658 Monozygotic twins, Hodgkin lymphoma and, 1098 Monro-Kellie doctrine, 255, 255f, 258 Morbidity, in esophageal perforation, 428 “Morgagni” hernias, 377, 394–395, 395f Morphine, 50, 50t Motor vehicle accidents (MVAs) injury risk of, 212 MPS. see Meconium plug syndrome MRA. see Magnetic resonance angiography MRCP. see Magnetic resonance cholangiopancreatography MRI. see Magnetic resonance imaging MSRE. see Modern staged repair of exstrophy MTC. see Medullary thyroid cancer Mucin, 538 Mucinous carcinoma, 1198t Mucosectomy, 651 Mucous coat, of intestinal barrier, 538 Mucous membranes, as anatomic barrier, 141–142 Mucoviscidosis, 517–518 Mucus clearance, mechanical ventilation of, 122–123

Müllerian inhibitory factor (MIF), 805 testicular descent, 805 Müllerian-inhibitory substance deficiency (hernia uterine inguinale), 956– 957, 957f Multichannel intraluminal impedance (MII), 464 Multicystic dysplastic kidney, 834 in Wilms tumor, 989–991 Multifocal hepatic hemangioma, 1153 “Multilocular cyst”, 834 Multiple endocrine neoplasia type I (MEN I), insulinomas, 745 Multivisceral allograft, 722–723 Mupirocin, 204t Muscle flaps, in congenital diaphragmatic hernia, 390 Muscularis mucosa, 422 Muscularis propria, 422 Musculoskeletal problems, cancer therapy complications, 972 Musculoskeletal system congenital diaphragmatic hernia in, 377–378, 394 prune belly syndrome, 946 Mutations, 1014 MVAs. see Motor vehicle accidents MYCN in esophageal atresia, 437 in neural tumors, 414 in neuroblastoma, 1013, 1013f Mycobacterial lymphadenitis, 1183 Mycobacterium avium-intracellularescrofulaceum (MAIS) complex, 1183 Mycophenolate mofetil (MMF) post-liver transplantation, 719 renal insufficiency in liver transplantation, 719 Myelomeningocele, 161–163, 288, 289f of fetal therapy, 162f neurogenic bladder, 874 Myelosuppression, cancer chemotherapy, 972 Myer classification system, 452, 452f, 452t Myofibromas, 1088 Myofibromatosis, 1088 Myxomas, 1088

N

Nails, femoral shaft fractures and, 270, 270f Nasogastric decompression for gastroschisis, 765, 765f for neonatal anorectal defect, 583 Nasopharyngeal teratomas, 1080 NAT. see Nonaccidental trauma National Institutes of Health (NIH), bariatric surgery patient selection, 1240 National Safe Kids Campaign, 212 National Trauma Data Bank (NTDB), 212 in great vessel injuries, 231 Native asparaginase, as anticancer drugs, 973t–974t NEC. see Necrotizing enterocolitis

Index

Neck teratomas. see Teratomas; head and neck Neck trauma, injury patterns of, 213–214 Necrotizing enterocolitis (NEC), 536–556 clinical diagnosis of, 541–543, 541f laboratory studies, 541–542 radiography, 542 ultrasound, 542 differential diagnosis of, 543 epidemiology of, 536 gastroschisis and, 770 grading system of, 543 medical management of, 543–544 microbiome and, 541 modified Bell classification of, 542t mortality, regionalization of care and, 546 outcomes of, 546–548 growth, 547 intestinal failure, 546–547 intestinal strictures, 547, 547f mortality, 546 neurodevelopmental outcomes, 547–548 recurrence, 546 stoma complications, 547 pathogenesis of, neonatal vasculature, 540–541 pathophysiology of, 536–541, 537f–538f. see also Intestinal barrier. pneumatosis intestinalis, 536 radiography, 536, 537f prevention of, 548–549 amino acid supplementation, 549 antibiotics, 549 feeding strategies, 549 human milk, 549 probiotics, 548–549 surgical management of, 544–545 exploratory laparotomy, 544, 544f primary peritoneal drainage, 544–545, 545t Necrotizing fasciitis surgery, 148f Necrotizing soft tissue infection, 147– 148, 148f NECSTEPS trial, 545 Nelarabine, as anticancer drugs, 973t–974t Neomycin of burn wound care, 204t for omphalocele scarification management, 773 Neonatal Inhaled Nitric Oxide Study (NINOS) Group, 125–126 Neonatal physiology, 1–17 anemia, 5 blood volume, 4–5, 4t calcium, 3–4 cardiovascular system of, 8–9 fluid requirements, 7, 7t fluids and electrolytes, 6–7, 6f glucose metabolism of, 2–4 hemoglobin, 4 jaundice, 5, 5t magnesium, 4 pulmonary system of, 8 monitoring, 9–11

Neonatal physiology (Continued) retinopathy of prematurity, 6 shock, 11–15 cardiogenic shock, 11–13 distributive shock, 13 hypovolemic shock, 11 septic shock, 13–15 thermoregulation, 6 Neonatal Wilms tumor, 998 Neonates classification, 3t ovarian cysts in, 1194 pulmonary vascular hypertension, 91–92 Nephroblastoma, cystic partially differentiated, 834 Nephroblastomatosis, in Wilms tumor, 989–991, 991f Nephrogenic rests, in Wilms tumor, 989–991, 990f, 990t Nephroma, cystic, 834 Nephroureterectomy, upper-pole partial, 849 Nerve tissue tumors, 1085–1088 neurofibromas, 1085–1088, 1085t–1086t, 1085b, 1086f–1087f xanthomas, 1088, 1088f NET trial, of necrotizing enterocolitis management trial, 545 Neural tube defects, 288–290, 289f Neural tumors, 413–417, 414f–416f Neuroblastoma, 1010–1030 adrenal masses, 1228 bone marrow examination for, 1017 clinical presentation of, 1014–1017 diagnostic imaging for, 1015–1017 computed tomography, 1016 magnetic resonance imaging, 1016, 1016f metaiodobenzylguanidine imaging, 1017, 1017f standard radiographs, 1015 ultrasonography, 1016 differential diagnosis of, 1017 etiology of, 1010 histopathologic classification of, 1011–1012, 1013t, 1011f imaging of, 994, 994f incidence of, 1010 International Neuroblastoma Risk Group Risk Stratification, 1020– 1022, 1021t, 1021b high-risk disease, 1019–1020, 1020f intermediate-risk disease, 1018– 1019 low-risk disease, 1018 proposed risk-directed therapy, 1022 recurrent high-risk, 1022 intraspinal extension of, 1025–1026 laboratory findings of, 1014–1015 management of, 1025–1026 ALK inhibition, 1027 MIBG therapy, 1027 targeted therapy, 1027 metastases, 1014, 1015f, 1015t

1277

Neuroblastoma (Continued) molecular biology of, 1012–1014 amplification of MYCN, 1013, 1013f DNA content, 1013 mutations, 1014 segmental chromosome aberrations, 1013–1014 opsoclonus-myoclonus syndrome, 1026 pathology of, 1010–1012 screening for, 1025 sites, 1014, 1015f stage 4S, 1025, 1025f–1026f staging, 1017, 1018t surgery for, 1022–1025 complications of, 1024–1025 delayed resection, 1023 localized tumors, 1022 locoregional disease in patients with metastatic disease, 1022–1023, 1023f operative principles, 1024, 1024f recurrent disease, 1024 Neurocutaneous melanocytosis, 1132– 1133 Neurodevelopment, sequelae, ECMO, 104 Neuroenteric cyst, 411–412, 413f Neurofibromas, 1085–1088, 1085t–1086t, 1085b, 1086f–1087f Neurofibromatosis type 1 (NF1), 1085, 1085b, 1086t rhabdomyosarcoma, 1115 Neurogenic bladder, 873–879 acquired lesions, 873 childhood management for, 874–876, 875f classification of, 873 detrusor hyperactivity, 873 incompetent bladder neck, 873 myelomeningocele, 874 surgical treatment for, 876–879 artificial urinary sphincter, 878, 878f bladder augmentation, 876, 876f bladder autoaugmentation, 876– 878, 877f bladder neck fascial sling, 878 continent catheterization stoma, 879f Kropp’s procedure, 878 Pippi Salle’s procedure, 878 Young-Dees technique, 878 Neurologic system, congenital diaphragmatic hernia in, 393–394 Neurological disease, cloacal exstrophy, 912 Neuromodulation, for bladder instability, 873 Neuromonitoring, advanced, for traumatic brain injury, 264 Neuromuscular blockade, for traumatic brain injury, 261–262 Neurosurgical conditions, 285–300 brain tumors in, 296–299 Chiari I malformations in, 295, 295f–296f craniosynostosis in, 290–291, 291f hydrocephalus in, 285–287, 286f

1278

Index

Neurosurgical conditions (Continued) intracranial infections in, 299–300, 299f neural tube defects in, 288–290, 289f neurosurgical devices in, 287, 287f–288f skull masses in, 288, 288f tethered spinal cord in, 293–294, 293f–294f vascular malformations of the brain in, 291–293, 292f–293f Neurosurgical devices, 287, 287f–288f Neurotoxicity, concern for possible anesthetic related, 35–36 Neurturin, 557 Neutrophils, 14 immune response, 142 Nevi, 1127–1146 melanocytic. see Melanocytic nevi Spitz, 1133–1135 New Injury Severity Score (NISS), 211 Newborn, circumcision, 937, 938f NHL. see Non-Hodgkin lymphoma NICH. see Noninvoluting congenital hemangioma NICHD Neonatal Research Network, 545 NIH. see National Institutes of Health NISS. see New Injury Severity Score Nitrates, in achalasia, 424 Nitric oxide (NO) in intestinal barrier regeneration, 540 mechanical ventilation of, 125–126, 125f necrotizing enterocolitis pathogenesis, 541 Nitrofurantoin, 858t Nitroglycerin, 186 Nocturnal enuresis, 870–872 NOD2/CARD15, 656 NOD-2insC polymorphism, 647 Nodular fasciitis, 1088 Nodular sclerosis, 1099 Nog, 437 Nonabsorbable synthetic patches, for congenital diaphragmatic hernia, 389–390 Nonaccidental trauma (NAT), 218–219 in orthopedic trauma, 267, 267t in rib fractures, 225 in traumatic brain injury, 260–261 Nonacid reflux, 464 Nonfunctioning kidney, surgery for, 998 Non-Hodgkin lymphoma (NHL), 405– 406, 406f–407f, 1103–1110 classification of, 1104–1105 clinical presentation of, 1105–1107 by histologic subtype, 1106–1107 in immunodeficient patients, 1107 by initial site of disease, 1105–1106, 1105f–1106f complications of, 1109–1110 diagnosis of, 1107–1108, 1107b epidemiology of, 1104 incidence of, 1104 laboratory findings in, 1106 long-term sequelae, 1110 prognostic risk factors for, 1108 staging of, 1108, 1108t

Non-Hodgkin lymphoma (NHL) (Continued) treatment of, 1108–1109 results of, 1109 Noninvoluting congenital hemangioma (NICH), 1152, 1152f Non-milky nipple discharge, 1211, 1211f Non-neoplastic cysts, pancreas, 746 Nonpenetrating cranial trauma, 254 Nonproliferative disorders, breast masses and, 1212 Nonradioactive stable isotope techniques, resting energy expenditure measurement, 21 Nonsteroidal anti-inflammatory drugs (NSAIDs) postanesthesia analgesia, 51 postoperative bariatric surgery, 1248 Nonteratomatous germ cell tumors, 409 Norepinephrine, as vasoactive medications in newborn, 12t Normothermia, 143 Nosocomial infections, 146 Notch signaling, 737 NRAS mutations, congenital melanocytic nevi and, 1132 NSAIDs. see Nonsteroidal antiinflammatory drugs NTDB. see National Trauma Data Bank Nuclear medicine, Hodgkin lymphoma and, 1101 Nutrients reserves, 18–20, 19t for surgical infections, 141 Nutrition acute pancreatitis management, 740 for gastroschisis postoperative care, 769 liver transplantation outcomes, 720 of meconium ileus, 525–526 neonatal acute kidney injury management of, 66 postoperative bariatric surgery, 1246–1247 post-renal transplantation, 726–727 for traumatic brain injury, 262 ulcerative colitis management, 650 Nutritional support body composition of, 18–20, 19t macronutrient intake of, 21–25 nutrient reserves, 18–20, 19t parenterally-derived lipid solutions, 24 for pediatric patient, 18–34 protein quality of, 23 routes of, 25–30 Nystatin, 204t

O

Obesity consequences, 1240 definitions, 1240 Obstruction appendicitis, 664 uropathy, 66–67 Obstructive megaureter, primary, 845–846 Obstructive pulmonary disease, in congenital diaphragmatic hernia, 393

Obstructive sleep apnea (OSA), trisomy 21, 38 Obstructive symptoms, of Hirschsprung disease outcomes, 566–570, 568b, 569f Octreotide, 746 Oligohydramnios fetal therapy of, 164 for posterior urethral valve, 884 Omental flap, 344 Omeprazole, gastrinoma management, 746 Omitting abdominal computed tomography, seven-element criteria for, 237t Omphalocele, 771–776 embryology of, 771 etiology of, 771 gastroschisis vs., 764t incidence of, 771 long-term outcomes of, 775–776 neonatal management of, 771 neonatal resuscitation for, 771 perinatal care for, 771 postoperative course of, 775 prenatal diagnosis of, 771 prenatal management of, 771 risk assessment in, 771–772 ruptured, 775, 775f scarification management of, 773, 773f–774f staging of, 771–772 surgical management of, 772 delayed staged closure, 773 immediate primary closure, 772, 772f staged neonatal closure, 772–773, 773f OMS. see Opsoclonus-myoclonus syndrome Oncogenes, 970 Onlay island flap technique, hypospadias, 925–926, 927f Oophorectomy, 1196–1197 Open approach of hypertrophic pyloric stenosis management, 479–480, 480f in malrotation management, 510–511, 510f–511f, 510b Open fetal surgery for congenital pulmonary airway malformation, 352 congenital pulmonary airway malformation management, 155 thoracotomy, 157, 158f Open fractures, 268, 268t Open pneumothorax, 225, 225f Open repair, of inguinal hernia, 790– 792, 791f–792f Open splenectomy, 753–754 laparoscopic splenectomy complications, 758 laparoscopic splenectomy conversion, 758 Open surgery biliary atresia, 683–686, 684f–685f choledochal cyst, 700 hysterotomy, 156

Index

Open surgery (Continued) Meckel’s diverticulum management, 644 proctocolectomy with ileoanal pullthrough procedure, 651–653, 652f Open technique, pectus repair, 310–311, 312f Opioids, 50–51 OPSI. see Overwhelming postsplenectomy infection OpSite, 204t Opsoclonus-myoclonus syndrome (OMS), 1026 Oral contraceptives in focal nodular hyperplasia, 1037–1038 in hepatocellular carcinoma, 1040 Oral hygiene, mechanical ventilation of, 122–123 Orchiopexy bilateral, prune belly syndrome, 947 microvascular, 812 undescended testes, 808–809, 808f Organ donation, liver transplantation, 712b, 713f Organoaxial gastric volvulus, 484 Oropharyngeal teratomas, 1080 Orthopedic trauma, 267–284 complex injuries in, 268, 268t, 269f lower extremity, fractures of, 268–271, 270f–272f management of, pearls and pitfalls of, 280, 280b nonaccidental trauma in, 267, 267t pathophysiology of, 267–268, 268f spine injuries in, 272–277 cervical, 272–275, 273f–275f thoracic, lumbar, and sacral fractures, 275–277, 276f upper extremity, fractures of, 277– 280, 277f–280f Orthotic bracing, 323 Orthotopic liver transplantation, 1050 Ossifying renal tumor of infancy, 1005 Osteomyelitis, 86 Osteonecrosis, 268–270 Osteotomies bladder exstrophy, 901 cloacal exstrophy, 912–913 Ostomy, in complicated meconium ileus management, 524 Otolaryngologic laryngoscope, 338 Ovarian cysts in adolescents, 1194–1195 in childhood, 1194, 1195f management of, 1194 in neonate, 1194 Ovarian torsion, 1199–1201, 1200f–1201f Overhydration, in neonates, 7b Overweight, definition, 1240 Overwhelming postsplenectomy infection (OPSI), 752, 759 in splenic injury, 248–249 Ovotesticular DSD (true hermaphrodite), 957 Owl’s eye cells, Hodgkin lymphoma and, 1098, 1099f

Oxaliplatin, as anticancer drugs, 973t–974t Oxidative stress, in secondary brain injury, 258 Oxybutynin, 872 Oxygen administration, 215 Oxygen delivery (DO2), 114 Oxygen dissociation curve, fetal hemoglobin, 4f Oxygen saturation (SaO2), 9

P

Pacemakers, 44 Paclitaxel, as anticancer drugs, 973t–974t PAF. see Platelet-activating factor Pain appendicitis, 665 chronic, inguinal hernia, 800 of crotalid snakebites, 190 inguinal, 787 Paired corpora cavernosa, 918 PAIS. see Partial androgen insensitivity syndrome PALS. see Pediatric Advanced Life Support pANCA. see Perinuclear antineutrophil cytoplasmic antibody Pancreas, 737 anatomy, 737, 738f congenital anomalies, 737–738 duplication, 634 embryology, 737, 738f fistula, 741–742 functional disorders, 743–745 glycogen storage disease, 745 insufficiency, meconium ileus and, 517–518 transplantation, 731–733 decreased donor whole organ, 731–732 trauma, duct trauma, 700 tumors and cysts, 745–747 adenocarcinoma, 746–747 endocrine tumors, 745–746 exocrine tumors, 746 non-neoplastic cysts, 746 pancreatoblastoma, 746–747, 747f Pancreas divisum, 739 Pancreatectomy, subtotal, 743 Pancreatic enzymes, in meconium ileus/ cystic fibrosis management, 526 Pancreatic injury, 243–244, 243f–244f scale, 243t Pancreatic pseudocysts acute pancreatitis, 741, 741f–742f chronic pancreatitis, 743, 743f Pancreatitis, 738–743 acute, 738–742, 738f causes, 738–739 pancreas divisum, 739 trauma, 738–739, 739f diagnosis, 739 incidence, 739 management, 740 chronic, 742–743 calcifying, 742, 743f genetics, 742

1279

Pancreatitis (Continued) management, 743 non-calcifying, 742 signs and symptoms, 743 Pancreatoblastoma, 746–747, 747f Paneth cells, 540 PaO2 (arterial oxygen tension), 9 Papillary-cystic tumor, 747, 748f Papillomatosis, breast, juvenile, breast masses and, 1212 Paracetamol (acetaminophen), 51 Paraphimosis, 935 Parapneumonic effusions (PPE), 361– 365 classification scheme for, 362t diagnosis of, 361–362, 363f epidemiology of, 361 management of, 362–363, 363f pathogenesis of, 361, 362f Paratesticular tumors, rhabdomyosarcoma, 1121–1122 Parathormone, 1225 Parathyroid glands, 1225–1227 embryology of, 1225 physiology of, 1225 Parenteral nutrition, 28–30 electrolyte status of, 28–29 fluid status of, 28–29 for jejunoileal atresia/stenosis, 501 micronutrients, 26t, 28 protein, 30 Parkes Weber syndrome, 1164–1165 Parkland formula, fluid resuscitation, 198 Parsons laryngoscope, 338 PART. see Polyhydramnios affecting a recipient-like twin Partial androgen insensitivity, in 46,XY, 956 Partial androgen insensitivity syndrome (PAIS), 956 Partial ectopia cordis, 323 Partial nephroureterectomy, upper-pole, 849 Partial pressure of oxygen in arterial blood (PaO2), 113 Partial splenectomy, 752, 756–758 hereditary spherocytosis, 752, 753f Partial thromboplastin time, 80–81 Patent ductus arteriosus (PDA) in congenital diaphragmatic hernia, 378 ECMO, 102 Patent processus vaginalis (PPV), incident, 799, 799f Patient-controlled analgesia, for pectus excavatum, 308–309 Patient history, anesthesia preoperative evaluation and, 37 Patient positioning, for traumatic brain injury, 261 PAV. see Proportional assist ventilation PAX3, 1115 PAX7, 1115 PAX7-FOXO fusion, 1115 Pazopenib, as anticancer drugs, 973t–974t PBS. see Prune belly syndrome PCCO. see Pulse contour cardiac output

1280

Index

PD. see Peritoneal dialysis PDA. see Patent ductus arteriosus PECARN. see Pediatric Emergency Care Applied Research Network Pectus carinatum, 302, 314–323, 315f, 324f diagnosis of, 316 etiology of, 315 incidence of, 315–316 nonsurgical treatments for, 320–323, 320f–321f pressure-controlled bracing in, 321–322, 322f, 322t operative treatment for, 316–320 cartilage resection, 316–318, 317f noncartilage resection, 318–319, 319f prevalence of, 315–316 treatments for, classification of, 316t Pectus excavatum, 302–314, 303f, 303b cardiac effects of, 305–306 clinical features of, 304–305, 305f, 305t etiology of, 304 evaluation for, 306–307 algorithm for, 306f CT scans in, 306–307, 306f MRI in, 307 pulmonary function tests in, 307 history of, 302–304 incidence of, 304 indications for, 306–307 age parameters in, 307, 307f operative approaches for, 307–313 bar removal in, 314, 314f complications of, 313, 314f, 314t minimally invasive pectus repair in, 307–309, 309f–312f open technique in, 310–311, 312f results in, 313, 313t pulmonary effects of, 305–306 treatment options for, 307, 307f–308f Pectus posture, in pectus excavatum, 304, 305f Pediatric adjusted shock index, in blunt abdominal trauma, 236 Pediatric Advanced Life Support (PALS), 137 Pediatric Emergency Care Applied Research Network (PECARN), in computed tomography, 236, 237t Pediatric End-Stage Liver Disease (PELD) score, 711–712, 712b, 713f Pediatric Health Information System (PHIS), 392 Pediatric islet transplantation, 732–733 Pediatric orthopedic trauma, 267–284 Pediatric Trauma Score (PTS), 211, 215t PEEP. see Positive end-expiratory pressure PEG-asparaginase, as anticancer drugs, 973t–974t Pelvis, fractures of, 214 Penetrating injury, 232, 232f diaphragmatic injuries and, 228 genital bleeding and, 1190 Penetrating trauma incidence from, 212t special considerations of, 219 in traumatic brain injury, 254

Penicillin G perforated appendicitis, 671–672 tetanus management of, 183 Penis, embryology of, 918 Peptic ulcer disease, 482–483, 484f Percent predicted lung volume (PPLV), 155 Percutaneous biopsy, of anterior mediastinal mass, 41 Percutaneous Shunting in Lower Urinary Tract Obstruction (PLUTO) trial, 885 Percutaneous transhepatic cholangiography, 1056 Perianal and perirectal abscess, 613, 613f–614f Perianal disease, rhabdomyosarcoma, 1120–1121, 1121f Pericarditis, 313 Perinatal testicular torsion, 824 Perineal disease, rhabdomyosarcoma, 1120–1121, 1121f Perinuclear antineutrophil cytoplasmic antibody (pANCA), 649 Periorbital infantile hemangioma, 1149, 1149f Peripheral intravenous (PIV) cannula, 133 Peripheral venous access, 133–134 Peripherally introduced central venous catheter (PICC), 134–135, 135f Peristalsis, disordered, in esophageal atresia, 448–449 Peritoneal dialysis (PD) acute kidney injury management in, 62 characteristics of, 62t efficacy of, 62 Peritoneal drainage, of necrotizing enterocolitis, 544–545, 545t Peritonitis, 148–149 Permacol®, for congenital diaphragmatic hernia, 390 Permanent ileostomy, 659 Permissive hypercapnia, 120–121 Peroral endoscopic myotomy (POEM), in achalasia, 425 Peroxynitrite, 540 Persistent aganglionosis, of Hirschsprung disease, 568 Persistent cloaca, 593, 593f Persistent pulmonary hypertension of the neonate (PPHN), 93–94, 385 Personal watercraft injuries, 245–247 Petechiae, 79 Peutz-Jeghers syndrome, 621 PFA-100 analyzer, 79–80 pH probe monitoring, 464 PHACES, 1149–1150, 1150f Phase I clinical trials, 970 Phase II clinical trials, 970 Phase III clinical trials, 970 Pheochromocytoma, 1232, 1233f Phimosis, 935 PHIS. see Pediatric Health Information System Phosphodiesterase (PDE) inhibitors, 383 Phospholipases A2, 78 Phospholipases C, 78

Phototherapy, neonatal jaundice and, 5 PHOXB2, in neuroblastoma, 1014 Phrenoesophageal membrane, 460 Phyllodes tumors, 1212f, 1213–1214 Physical activity (PA), energy expenditure, 20 Physical examination for ectopic ureter in girls, 847 for hemostasis, 79 for inguinal hernia, 785–786 for mesenchymal hamartoma, 1036 for renal function evaluation, 65 for sexual differentiation disorders, 959 for undescended testes, 806 Physical maturity, bariatric surgery timing, 1241–1242 Physical status score, 36, 36t Physical support, trauma, 218 PICC. see Peripherally introduced central venous catheter Piggy-back implantation, liver transplantation, 715 Pillar cysts, 1084 Pilocytic astrocytoma, 296–297, 296f Pilomatrixoma, 1085, 1085f Pin site infections, 274–275 Pippi Salle’s procedure, 878 Pits, preauricular, 1178–1179 Placental markers, infantile hemangioma, 1148 Plain radiographs, in hydrocephalus, 286 Plastibell, 937, 938f Platelet(s) adhesion of, 76–78 aggregation, 78 count, of hemostasis evaluation, 79 disorder management of, 84 function studies, 81 hemostasis of, 76–78, 77f Platelet-activating factor (PAF), 540 Platelet-activating factor-acethydrolase (PAF-AH), 540 Plates, in femoral shaft fractures, 270 Pleura acquired lesions of, 361–376 air or fluid collection of, 230 Pleuroperitoneal folds (PPFs), 378–379 Pneumatic dilation, in achalasia, 424 Pneumatic reduction, intussusception management, 623–625, 624f Pneumatoceles, 365–366 Pneumatosis intestinalis, 536 Pneumococcus infection, 759 Pneumonia empyema and, 361 lung abscess and, 365 in pectus repair, 313 Pneumoperitoneum, 47b tension, 623–625 Pneumothorax, 224 open, 225, 225f in pectus repair, 313 spontaneous, 371–373, 372f–373f tension, 226, 227f Pneumothorax-pulmonary lacerations, 226–227, 227f POEM. see Peroral endoscopic myotomy Poland syndrome, 302

Index

Polycythemia of congenital heart disease, 44 in neonates, 4–5 Polyhydramnios, twin-twin transfusion syndrome, 160 Polyhydramnios affecting a recipient-like twin (PART), 161 Polymyxin B, of burn wound care, 204t Polysomnography, bariatric surgery, 1243 Polysplenia, 750 Polytetrafluoroethylene (PTFE), for congenital diaphragmatic hernia, 389 Polythelia, breast disease and, 1207, 1207f PONV. see Postoperative nausea and vomiting Port placement, in esophageal atresia, 444, 444f Portal vein thrombosis (PVT), 717 Portoenterostomy, biliary atresia, 683, 684f “Position theory”, in testicular neoplasms, 807 Positional changes, torticollis and, 1181–1182 Positioning for anterior mediastinal mass anesthesia, 41 for esophageal atresia, 444–445, 444f–445f for traumatic brain injury, 261 Positive end-expiratory pressure (PEEP), 111f respiratory failure, 121–122, 121f, 121b using protective effects of, 121–122, 121f, 121b Positron emission tomography-computed tomography (PET-CT), in Hodgkin lymphoma, 407, 407f Postanesthetic apnea, 40, 40f Posterior iliac osteotomies, 901 Posterior urethra, prune belly syndrome, 944, 945f Posterior urethral valves, 884–896 antenatal diagnosis of, 884–886 clinical presentation of, 886 embryology and anatomy of, 884 end-stage renal disease, 884, 894 fertility, 894 initial management of, 887–891 adult sexual function and fertility, 894 amoxicillin, 887–888 bladder dysfunction, 892–894 cephalexin, 887–888 decompression, 887 follow-up after, 890 late diagnosis, 891–892 primary valve ablation, 888, 888f prognosis, 890–891, 891f renal transplantation, 894 temporary urinary diversion, 888–890, 889f–890f vesicostomy closure, 892 vesicoureteral reflux, 892, 893f

Posterior urethral valves (Continued) management of, 884–886 outcomes, 884–886 pathophysiologic changes in, 893t radiographic evaluation for, 886–887, 887f sexual function, 894 types of, 884 Postextubation subglottic edema, 49 Postoperative emesis, 482 Postoperative intussusception, 627 Postoperative nausea and vomiting (PONV), 49 Postpyloric feeds, enteral nutrition, 26–27 Post-transplant lymphoproliferative disease (PTLD) chronic liver transplant rejection, 718 liver transplantation infections, 720 management, 720 Post-traumatic intracranial hemorrhage, 256 Potassium titanyl phosphate (KTP), in tracheal repair, 341–342 Pouchitis, ulcerative colitis, 654–655 Povidone-iodine for burn wound care, 204t for omphalocele scarification management, 773 PPD. see Purified protein derivative PPE. see Parapneumonic effusions PPFs. see Pleuroperitoneal folds PPHN. see Persistent pulmonary hypertension of the neonate PPIs. see Proton pump inhibitors PPLV. see Percent predicted lung volume PPV. see Patent processus vaginalis Preauricular cysts, 1178–1179, 1179f Preauricular pits, 1178–1179 Preauricular sinuses, 1178–1179 Precocious puberty, 1233–1234 in boys, 1233–1234 evaluation of, 1234 in girls, 1233 treatment of, 1234 Prednisolone, 687, 1034 as anticancer drugs, 973t–974t Prednisone, 1034, 1150–1151 as anticancer drugs, 973t–974t Pregnancy testing, 39 Wilms tumor in, 1002 Premanagement Extent of Disease (PRETEXT) Grouping System, 1044–1045, 1046f, 1046t Premature birth, in hepatoblastoma, 1042 Premature thelarche, 1208, 1208t Prentiss maneuver, 811 Preoperative bronchoscopy, 441–442, 441f Prepubertal breast masses, 1212 Prepuce, 935, 936f Prerenal acute kidney injury, neonates, 64 Prerenal azotemia, 60 Pressure dressings, 780 Pressure immobilization, crotalid snakebite management of, 189

1281

Pressure support ventilation (PSV), 117, 117t Preterm delivery, 764–765 Pretreatment re-excision, for rhabdomyosarcoma, 1119 Primary brain injury, 256–257, 256f–257f Primary closure, of gastroschisis surgery, 765–766, 767f Primary hyperaldosteronism, 1229– 1230 Primary hyperparathyroidism, 1225– 1226, 1225f–1226f Primary intussusception, 621 Primary nocturnal enuresis, 870 Primary obstructive megaureter, 845–846 Primary survey, trauma, 215, 216b Probiotics for necrotizing enterocolitis prevention, 548–549 for ulcerative colitis, 654 Procarbazine, as anticancer drugs, 973t–974t Processus vaginalis, 784 Proctocolectomy with ileoanal pullthrough procedure open surgery, 651–653, 652f with staple anastomosis, 653, 653f Prokinetic medication, for gastroschisis postoperative care, 769–770 Prone positioning, mechanical ventilation of, 122 Pronephros, 828 Prophylaxis, postexposure, 184 Proportional assist ventilation (PAV), 117t, 118 Propranolol, 1035, 1151, 1151f Propylthiouracil (PTU), 1219 Prostaglandin(s), lipid metabolism, 24 Prostaglandin E1 (PGE1), 385 Prostate gland prune belly syndrome, 944–945 rhabdomyosarcoma, 1121 Protective colostomy for female rectovestibular fistula, 579–580 for neonatal anorectal defects, 583 Protein(s) parenteral nutrition, 30 quality, 23 requirements of, 24–25, 29f Protein C, 78 Protein metabolism, 21–23, 22t modulation, 23 Protein S, 78 Proteolytic enzymatic agents, 204–205 Proteus syndrome, 1164 Prothrombin time (PT) disseminated intravascular coagulation, 84 hemostasis evaluation of, 78 Proton pump inhibitors (PPIs), gastrinoma management, 746 Proton therapy, local tumor control, 980 Proximal humerus, fractures of, 277 Proximal tibial physeal injuries, 271 PRSS1, 742

1282

Index

Prune belly syndrome (PBS), 942–952 clinical features of, 943–946 clinical presentation of, 946 embryology of, 943 extragenitourinary anomalies, 945–946 abdominal wall, 942f, 945 cardiac and pulmonary, 945 gastrointestinal, 945–946 musculoskeletal, 946 spectrum of disease, 946, 946t genetics of, 942 genitourinary anomalies, 943–945 bladder, 943, 943f kidneys, 944 prostate and accessory sex organs, 944–945, 945f testes, 945 ureter, 943–944, 943f–944f urethra, 944, 944f management principles of, 946–947, 947f postnatal evaluation of, 946 prenatal evaluation of, 946 surgical management of, 947–949 bilateral orchiopexy, 947 cutaneous vesicostomy, 947 reconstruction of abdominal wall, 948, 948f–950f reduction cystoplasty, 948 renal transplant, 949 ureteral reconstruction, 947 urethral reconstruction, 947 Pseudochylothorax, 368 Pseudoglandular stage, in lung development, 380 Pseudohermaphrodite female, 953t, 954–955, 955f male, 955–956 Pseudoinflammatory tumor, mediastinal, 417 PSV. see Pressure support ventilation Psychological issues, bariatric surgery timing, 1242, 1243b Psychological stress, nocturnal enuresis and, 871 PT. see Prothrombin time PTEN hamartoma tumor syndrome, 1165 PTLD. see Post-transplant lymphoproliferative disease PTS. see Pediatric Trauma Score Puberty, females, 1188 Pulmonary artery, 10–11 Pulmonary contusion, 227–228, 228f Pulmonary effects, of pectus excavatum, 305–306, 305t Pulmonary function tests, in pectus excavatum, 307 Pulmonary hypertension, CDHassociated, 380–381 Pulmonary hypoplasia, in congenital diaphragmatic hernia, 380 Pulmonary metastases, thyroid carcinoma and, 1222–1223 Pulmonary system congenital diaphragmatic hernia in, 392–393 prune belly syndrome, 945

Pulmonary teratomas, 1077 Pulmonary tuberculosis, 1183 Pulmonary vascular development, 380–381 Pulmonary vascular hypertension, CDHassociated, 377 Pulmonary vascular resistance (PVR) in congenital diaphragmatic hernia, 380–381 congenital heart disease anesthesia, 44 neonatal pulmonary hypertension, 93–94 Pulmonary vascular sling, in vascular compression, 334–335 Pulmonary vasodilators, for congenital diaphragmatic hernia, 385 Pulmonary wedge pressure, 10 Pulse contour cardiac output (PCCO), 10 Pulse oximetry, 9–10 Pure gonadal dysgenesis, 958 Purified protein derivative (PPD), 1183 Purpura, hemostasis evaluation, 79 PVR. see Pulmonary vascular resistance PVT. see Portal vein thrombosis Pyelography, antegrade, 65 Pyeloplasty, dismembered, 841, 841f Pyloric atresia, 482, 483f Pyloromyotomy, 479 Pyocolpos, 580 Pyogenic granulomas, 1084, 1084f

Q

Quantitative urine culture, 853–854, 854t

R

Rabies, 184 Raccoon eyes, in skull fractures, 256 Radiation exposure in computed tomography, 362 CT, 667 thyroid carcinoma and, 1221 Radiation oncology, local tumor control, 979–980 Radiation therapy for Hodgkin lymphoma, 1102 intensity-modulated, 980 intraoperative radiation, 980 Radiofrequency ablation (RFA), 981 Radiography of adrenocortical carcinoma, 1231 anteroposterior, for cervical spine injuries, 274 in anteroposterior abdominal, 509, 509f Crohn disease, 657, 657f in duodenal atresia/stenosis diagnosis, 492, 492f–493f erect abdominal radiography, 529, 529f in esophageal atresia, 440, 440f in Hirschsprung disease diagnosis, 558 in necrotizing enterocolitis, 536, 537f, 542 in neonatal anorectal defects, 583, 583f in neuroblastoma diagnosis, 1015 in Wilms tumor diagnosis, 994, 994f–995f

Radiotherapy, for Wilms tumor (WT), 1000 RAIR. see Rectoanal inhibition reflex Range-of-motion exercises, torticollis and, 1181–1182 Rapamycin, 1158 Rapid growth, of mediastinal lymphomas, 405–406 Rapidly involuting congenital hemangioma (RICH), 1152, 1152f Rapunzel syndrome, 486 RAS proto-oncogene, 1221 Rasburicase, 1109 Ravitch-type PE repairs, 327 Receptor tyrosine kinases (RTKs), 1014 Recombinant activated factor VII, 85 Recombinant interferon, 1151 Reconfiguration, chest, in pectus excavatum, 304 Rectal atresia, as male defect, 579, 579f surgical repair of, 590 Rectal duplication, 632t, 638, 638f Rectal injury, 245–247, 246f Rectal prolapse, 615–618, 616f–617f Rectal stenosis, as male defect, 579 surgical repair of, 590 Rectal trauma, 618–619, 618f Rectoanal inhibition reflex (RAIR), 600 Rectobladderneck fistula, 578, 579f surgical repair of, 589–590, 589f Rectoperineal fistula, 577, 578f female defects, 579, 580f surgical repair of, 590 male defects, 577, 578f repair of, 586 Rectourethral fistula, male defects, 577–578, 578f surgical repair of, 586–589, 586f–589f Rectovestibular fistula, 579–580, 580f surgical repair of, 590, 590f–591f Recurrent disease, intussusception, 626–627 Recurrent rhabdomyosarcoma, 1122– 1123, 1122f–1124f Recurrent tracheoesophageal fistula, in esophageal atresia, 447–448, 447f–448f Redo fundoplication, in gastroesophageal reflux management, 472–474, 472f–473f Re-do hepatic portoenterostomy, 687 Reduction cystoplasty, prune belly syndrome, 948 REE. see Resting energy expenditure Reed-Sternberg (RS) cell, 1097 Regional anesthesia, 51 Regional lymph nodes, rhabdomyosarcoma, 1121 Renal abnormalities cloacal exstrophy with, 911 omphalocele and, 771 Renal agenesis, 829–830, 830b bilateral, 830 female, 830 unilateral, 830, 830b VACTERL association, 830 Renal artery pseudoaneurysms, in renal trauma, 249

Index

Renal artery thrombosis, 243 Renal cell carcinoma, 1003, 1003f Renal dysplasia, 828–829, 829f Renal ectopia, 830–832 Renal embryology, 828, 828f Renal failure, 58 ECMO, 102 liver transplantation, 718–719 in neonates, 7b Renal function evaluation of, 57–60 cystatin C, 58 fractional excretion of substances, 59–60 glomerular filtration rate, 58–59, 59t laboratory tests, 65 physical examination, 65 urinary creatinine excretion, 57–58 urine volume, 58 neonates, 6–7 Wilms tumor in, 1002 Renal hypoplasia, 828–829, 829f Renal impairment, 57–75 Renal medullary carcinoma, 1005–1006 Renal transplantation, 725–729 graft rejection rates, 728t indications, 726t end-stage renal disease, 725 focal segmental glomerulosclerosis, 725 outcomes, 731, 731f posterior urethral valve, 894 postoperative complications, 729 hypertension, 729 immunosuppression, 730–731 infection, 729–730 postoperative management, 728–729 algorithm, 728–729, 730f preemptive transplantation, 728 preoperative preparation, 727–728 pretransplant management, 726–727 dialysis, 726, 727f nutritional support, 726–727 procedure, 728, 729f prune belly syndrome, 949 recurrence rates, 728t Renal trauma, 236–253, 242f complications of, 249 reimaging after, 249–250 Renal tubular acidosis (RTA), 59–60 type IV, 60 Renal tumors, 986–1009 classification of, 986b ossifying renal tumor of infancy, 1005 Rendu-Osler-Weber disease, 1156 Renovascular hypertension, 67–72 clinical presentation of, 67t, 68 complications of, 71–72 diagnosis of, 68–69, 69t, 70f etiology of, 67–68, 68f imaging of, 68–69, 69t, 70f interventional procedures of, 69–70 outcomes of, 71–72 pathophysiology of, 67–68, 68f surgical treatment of, 70–71 treatment of, 69–71

Resection of adrenocortical carcinoma, 1231 for arteriovenous malformation, 1162 for congenital pulmonary airway malformation, 355 of embryonic branchial apparatus remnants of, 1175, 1177 in hepatoblastoma treatment, 1045– 1046 in hepatocellular carcinoma treatment, 1053 for infantile hemangioma, 1151–1152 infantile hepatic hemangiomas management, 1035 insulinomas, 746 for lymphatic malformation, 1157, 1157f Meckel’s diverticulum management, 644–645, 644f of pheochromocytoma, 1232 with primary anastomosis, 524 in simple meconium ileus management, 524 Respiratory and airway considerations, anesthetic, 39–40 Respiratory infections, in congenital diaphragmatic hernia, 393 Respiratory monitoring, 261 Respiratory morbidity, in esophageal atresia, 449 Respiratory quotient (RQ), 20 Respiratory symptoms, of gastroesophageal reflux, 463 Respiratory syncytial virus (RSV), in congenital diaphragmatic hernia, 393 Resting energy expenditure (REE), 19 Resuscitation of blunt abdominal trauma, 236 for congenital diaphragmatic hernia, 383–384 fluid, neonatal hypovolemic shock, 11 RET proto-oncogene, 1221 Retention pancreatic cysts, 746 Retinopathy of prematurity (ROP), 6 Retrocaval ureter, 845, 845f Retrolental fibroplasia (RLF), 6 Retroperitoneal teratomas, 1077, 1078f Retrusive meatus, 931 Reversible cardiopulmonary disorders, ECMO, 92 Revision circumcision, 938, 939f RFA. see Radiofrequency ablation Rhabdomyosarcoma (RMS), 1115–1126 assessment for, 1116–1117 lymph node evaluation in, 1117 biliary tract, 1055–1056, 1056f familial syndromes associated with, 1115 groups of, 1115–1116 histology of, 1115–1116 late effects in, 1123–1124 local control management for, 1119–1122 abdominal sites, 1119–1120, 1121f in extremity, 1121 genitourinary, 1121–1122, 1121f–1122f

1283

Rhabdomyosarcoma (RMS) (Continued) in head and neck, 1119, 1120f perineal and perianal disease, 1120–1121, 1121f metastatic disease and, 1122–1123, 1122f–1124f molecular diagnosis of, 1115 outcomes for, 1123 presentation of, 1116, 1117f recurrent, 1122–1123, 1122f–1124f staging of, 1116–1118 clinical group in, 1117–1118, 1118t risk group stratification in, 1118, 1118t treatment of, 1118–1122 delayed primary excision, 1119 medical, 1118 pretreatment re-excision in, 1119 primary surgical resection in, 1119 radiotherapy in, 1118–1119 tumor biology of, 1115–1116 Rib fractures, 224–225 RICH. see Rapidly involuting congenital hemangioma Right hemicolectomy, 1234–1235 Right-left lobe split, liver transplantation, 712–713 Right lobe grafts, liver transplantation, 712 Right lower quadrant pain, appendicitis, 665 Right middle lobe syndrome, 371, 372f Right thoracoscopy, for chylothorax, 368 Right trisegmentectomy-left lateral segment split, liver transplantation, 712–713 Rigid endoscopy, 423 Rigid esophagoscopy, 423 in foreign body esophageal injury, 426 Rituximab immune thrombocytopenic purpura management, 753 PTLD management, 720 RLF. see Retrolental fibroplasia RMS. see Rhabdomyosarcoma Romiplostim, 753 ROP. see Retinopathy of prematurity Rotation, of malrotation, 507 Roux sign, 268–270 Roux-en-Y gastric bypass (RYGB), 1243, 1245f–1246f Roux-en-Y hepaticojejunostomy, 697–698 RQ. see Respiratory quotient RSV. see Respiratory syncytial virus RTA. see Renal tubular acidosis RTKs. see Receptor tyrosine kinases Ruptured omphalocele, 775, 775f Rye classification, of Hodgkin lymphoma, 1099 RYGB. see Roux-en-Y gastric bypass

S

Saccular stage, in lung development, 380 Sacral defects, 581–582, 581f Sacral fractures, in spinal injuries, 275–277, 276f Sacral nerve roots, decompression of, 277

1284

Index

Sacral nerve stimulation (SNS), for functional constipation, 610 Sacrococcygeal teratoma (SCT), 1069– 1076 diagnosis of, 1070, 1070f fetal surgery of, 158–159 maternal hysterectomy with resection, 158–159, 162f operative approach for, 1072, 1073f–1075f prenatal diagnosis of, 1071–1072, 1071f prognosis for, 1072–1076 SAH. see Subarachnoid hemorrhage Saline load test, 1229–1230 Saliva, 462 Salter Harris classification in fractures, 268, 268f in knee injuries, 271 type I fractures, of distal humerus, 279 type II fractures, angulated, 277 “SAMPLE” history, trauma secondary survey, 217 Santorini duct, 737 Santyl ointment, 204–205 SaO2 (oxygen saturation), 9 Sarcomas in mediastinal tumors, 417 soft-tissue, 980 SBS. see Short bowel syndrome Scald burns, 196 Scaphoid megalourethra, 944, 944f Scar Assessment Questionnaire Scores, 670t Scarification management, of omphalocele, 773, 773f–774f SCIWORA. see Spinal cord injury without radiographic abnormality Sclerotherapy direct puncture, 1162 intralesional, for venous malformations, 1160 Scrotum, bifid, 922f SCT. see Sacrococcygeal teratoma Seat belt injuries, 276 Sebaceous cysts, 1084 Secondary hyperparathyroidism, 1227 Secondary intussusception, 621 Secondary malignancies cancer therapy complications, 976–977 Wilms tumor in, 1002, 1002f Secondary nocturnal enuresis, 870 Secondary survey, trauma, 217–218 Secretory breast cancer, 1214 Sedation, for traumatic brain injury, 261 Segmental chromosome aberrations, 1013–1014 Segmental colectomy and anastomosis, 659 Seizures, 285 Seldinger technique, central venous catheters, 135, 135f Selective hypothermia, ECMO, 100–101 Seminoma, 817 Sengstaken-Blakemore tube, in vascular compression, 335–336

Sensorineural hearing loss (SNHL), in congenital diaphragmatic hernia, 394 Sentinel hemorrhage, in vascular compression, 335–336 Sentinel lymph node biopsy (SLNB) for atypical Spitz nevi/tumor, 1135 for melanoma, 1138, 1140f–1141f for rhabdomyosarcoma, 1117 Sepsis, surgery, 148 Septic shock, in neonates, 13–15 Serial Transverse Enteroplasty Procedure (STEP), 721 of Hirschsprung disease, 565–566 Serous carcinoma, 1198t Serratus anterior muscles, 390 Sertoli cells, 805 Sertoli-Leydig cell tumor, 1198t Serum glutamic oxaloacetic transaminase (SGOT), 1051–1052 Serum sickness, crotalid snakebite management of, 191 Sexual development, differences of, 798–799, 798f Sexual differentiation external organs, 954, 954f normal gender and, 953–954 Sexual function bladder exstrophy, 910–911 hypospadias, 931 posterior urethral valves, 894 Sexually transmitted diseases, circumcision, 936 SFU. see Society for Fetal Urology SGA. see Small-for-gestational-age SGOT. see Serum glutamic oxaloacetic transaminase Sharp, as foreign body ingestion, 176 Shiley tracheostomy tube, 339t Shock distributive, 13 index, in blunt abdominal trauma, 236 Short bowel syndrome (SBS) intestinal transplantation indications, 721 necrotizing enterocolitis, 544 S/HPFH. see Sickle cell/hereditary persistence of fetal hemoglobin Shriners-Galveston formula, 198 Sickle cell disease, 85–88 adenotonsillectomy, 87 avascular necrosis, 88 cholecystectomy, 87 cholelithiasis, 87, 702 gallbladder disease, 702 hydroxyurea, 88 intraoperative management, 86–87 management of, 86 osteomyelitis, 86 postoperative management of, 87 preoperative assessment of, 86 retinopathy, 87–88 splenectomy, 87 splenectomy indications, 753 splenic sequestration of, 87 Sickle cell hemoglobin, 85–88 Sickle cell/hereditary persistence of fetal hemoglobin (S/HPFH), 85

SIDS. see Sudden infant death syndrome Sigmoid colostomy, diversion with, 651 Signal transduction inhibitors, 968, 977 SILA. see Single-incision laparoscopic appendectomy Silastic pouch, 1025 Silastic (Dow Corning) tracheostomy tube, 339t Sildenafil, 385 Silicone rubber stents, 343 Silicone T-tubes, 346 Silk glove sign, 787 Silo, for gastroschisis surgery, 769 Silver nitrate, 204, 204t Silver sulfadiazine of burn wound care, 203, 204t for omphalocele scarification management, 773 Simple cysts, breast masses and, 1212– 1213 Simple renal cyst, 834–835 SIMV. see Synchronized intermittent mandatory ventilation Single kidney, surgery for, 998 Single-incision laparoscopic appendectomy (SILA), 668–669 Single-port appendectomy (SPA), 756, 757f Single-site laparoscopic cholecystectomy (SSULS), 703–704, 704f–705f Single ventricle physiology, 44–45 Sinuses, preauricular, 1178–1179 SIP. see Spontaneous intestinal perforation Sirolimus, as anticancer drugs, 973t–974t Sistrunk procedure, 1172 Skeleton trauma, injury patterns of, 214–215 Skin as anatomic barrier, 141–142 anatomy of, 196, 1128f Skin flaps, devitalized, 930 Skull fractures, 256 Skull masses, 288, 288f Skull shape abnormalities, 290 SLC16A mutations, 744 Sleep apnea, 1243 Sleep disorders, nocturnal enuresis and, 871 Slide tracheoplasty, 342, 342f–343f SLNB. see Sentinel lymph node biopsy SMA. see Superior mesenteric artery Small bowel duplication, 635–636, 635f–636f Small bowel injuries, 244–245, 246f Small-for-gestational-age (SGA), 2 Snake bites, 187–193 coral, 192–193 exotic, 193 species identification of, 187f SNHL. see Sensorineural hearing loss SNS. see Sacral nerve stimulation Soave procedure, 561–562, 562f Société Internationale d’Oncologie Pédiatrique (SIOP), in Wilms tumor (WT), 993, 993t, 996 Society for Fetal Urology (SFU), 838–839

Index

Society of Pediatric Oncology, in hepatoblastoma staging, 1044– 1045 Socioeconomic status, of injury risk, 211 Sodium, fractional excretion of, 59 Sodium hypochlorite. see Dakin’s solution Sodium nitroprusside, as vasoactive medications in newborn, 12t Soft tissue sarcomas, 980 Soft tissue tumors, 1084–1090, 1084t adnexal tumors, 1084–1085 epidermal tumors, 1084–1085 Solid, reamed femoral nails, in femoral shaft fractures, 270 Solid organ transplantation, 709–736 Solid pseudopapillary tumor, 747, 748f Somatostatin analogs for chylothorax, 368 congenital hyperinsulinism management, 744 Sorafenib, as anticancer drugs, 973t–974t SOX2, 437 SOX9, 953 SOX-10, 557 SPA. see Single-port appendectomy Sphincteroplasty, 743 Spider bites, 184–186, 184f black widow spider, 186 Spigelian hernia, 781–782 Spinal cord injury without radiographic abnormality (SCIWORA), 214 in cervical spine injuries, 272, 275 Spinal cord ischemia, in great vessel injuries, 231–232 Spinal defects, anorectal defects and, 581–582 Spinal pathways, bladder innervation, 870 Spine, jumbled, 324, 327 Spine injuries, 272–277 cervical, 272–275, 273f–275f lumbar fractures in, 275–277, 276f sacral fractures in, 275–277, 276f thoracic fractures in, 275–277, 276f SPINK1, 742 Spitz nevi, 1133–1135, 1134f atypical Spitz nevi/tumors, subtypes of, 1135 classification of, 1133–1134 risk stratification system for, 1135t Spleen, 750–762 anatomy, 750 abnormalities, 750–751 cysts, 751, 752f embryology, 750 gonadal fusion, 751 physiology, 750 sequestration in sickle cell disease, 87 wandering, 750, 751f Spleen injury, 237–242, 239f angioembolization in, 242, 242f bleeding delayed, 241 operative management of, 238–240, 241f complications of, 248–249

Spleen injury (Continued) endoscopic retrograde cholangiopancreaticography in, 242, 242f hemoglobin in, 241 length of stay in, 240–241 nonoperative failure of, 238, 240t guidelines for, 237–238, 240f operation, defining the need for, 241–242 reimaging after, 249 transfusion in, 241 Splenectomy, 753–755 gallbladder disease, 702 indications, 751–753 Gaucher disease, 753 hereditary spherocytosis, 751–752 immune thrombocytopenic purpura, 752–753 sickle cell disease, 753 thalassemia, 753 postoperative sepsis, 759 sickle cell disease, 87 single-port access, 756, 757f Splenic pseudoaneurysm, in splenic injury, 248, 248f Splenic pseudocysts, in splenic injury, 248 Splenic vein thrombosis, laparoscopic splenectomy complications, 759 Splenomegaly hereditary spherocytosis, 752 laparoscopic splenectomy complications, 758 Splenosis, 759 SPLIT. see Studies of Pediatric Liver Transplantation Split-thickness autografts, 206 Spondylothoracic dysplasia, 324, 327, 327f Spontaneous intestinal perforation (SIP), 543 Spontaneous pneumothorax, 371–373, 372f–373f SRS. see Stereotactic radiosurgery SRY, 953 SSULS. see Single-site laparoscopic cholecystectomy St. Jude (Murphy) staging system, for non-Hodgkin lymphoma, 1108t Stabilization, for congenital diaphragmatic hernia, 383–384 Stage 4S neuroblastoma, 1025, 1025f–1026f Staged closure of gastroschisis surgery, 766–767, 768f–769f neonatal, of omphalocele surgery, 772–773, 773f Staphylococcus aureus acute suppurative cervical lymphadenitis and, 1182 thyroglossal duct cyst and, 1173 Staphylococcus epidermidis, thyroglossal duct cyst and, 1173 Stem cells, fetal therapy of, 165, 165b

1285

Stenosis acquired subglottic, 334, 335f acquired tracheal, 334, 335f bronchial, 351–352 congenital subglottic, 333, 333f congenital tracheal, 333, 333f Stents, expandable metal, 343 STEP. see Serial Transverse Enteroplasty Procedure Stereotactic radiosurgery (SRS), 292 Sternoclavicular joint, dislocation of, 277 Sternocleidomastoid muscle, 1174–1175 Sternum bifid, 302, 323 defects in, 323–324, 325f–326f Steroidogenic factor-1 (SF-1), 953 Steroids biosynthesis, 955f for congenital pulmonary airway malformation, 352 Stoma formation, for functional constipation, 610 as necrotizing enterocolitis complications, 547 Stomach decompression of, 523 duplication, 483, 483f, 632t, 634f lesions, 478–488 antral web, 484, 484f bezoars, 486, 486f foreign bodies, 486 gastric duplication, 483, 483f gastric perforation, 482 gastric volvulus, 484–485, 485f microgastria, 483–484, 484f peptic ulcer disease, 482–483, 484f perforation, 482 volvulus, 484–485, 485f Straddle injuries, 1190 Stratifying adnexal masses, 1195–1197 imaging characteristics of, 1195– 1196, 1196f–1197f, 1196t postoperative surveillance of, 1200– 1201 size of, 1195 tumor markers of, 1196–1197, 1198f–1200f, 1198t Streptococcus pneumoniae infection, overwhelming postsplenectomy infection, 759 Streptococcus pyogenes, thyroglossal duct cyst and, 1182 Stress incontinence, 871 metabolic response to, 18, 19f Stretching exercises, torticollis and, 1181–1182 Strictures, hypospadias, 930–931 Stridor airway foreign bodies, 178 in vascular compression, 335 Studies of Pediatric Liver Transplantation (SPLIT), 711–712 Subacute subdural hemorrhage, 256–257 Subacute (de Quervain) thyroiditis, 1218 Subarachnoid hemorrhage (SAH), 257, 257f

1286

Index

Subarachnoid space, 255 Subclavian artery, 422 Subcutaneous emphysema, 427 Subdartos pouch, 809 Subdural hemorrhage, 256–257, 257f Subglottic edema, postextubation, 49 Subglottic malformation, 332–336 Subglottic stenosis acquired, 334, 335f congenital, 333, 333f Submucosa, in esophagus, 422 Subtotal pancreatectomy, chronic pancreatitis, 743 Suction, crotalid snakebite management of, 190 Sudden infant death syndrome (SIDS), 463 Sulfamylon, 204 Sulfasalazine, 650 Sunitinib, as anticancer drugs, 973t–974t “Sunsetting” eyes, 285, 286f Superficial second-degree burns, 205 Superior mesenteric artery (SMA), 507 Supernumerary kidneys, 830 Supervision, of injury risk, 211 Supine position, anesthesia, 41 Suppurative cervical lymphadenitis, acute, 1182–1183, 1182f Suppurative thyroiditis, acute, 1218 Supracondylar fracture, 277–278, 278f Surfactant, 8–9 adverse effects of, 9t for congenital diaphragmatic hernia, 385 ECMO, 94 Surfactant replacement therapy, of mechanical ventilation, 126, 126f Surgical infections intestinal transplantation complications, 725 renal transplantation complications, 729–730 Surgical infectious disease, 141–152 anatomic barriers of, 141–142 catheters, 146–147 components of, 141 host defense of, 141 immune response of, 141 nosocomial infection, 146 nutrients for, 141 postoperative, 144–146, 145f–146f prevention of, 143–144 bowel preparations, 144 patient characteristics, 143 sterile techniques, 147 surgical preparation, 143 types of, 144–149 virulence of, 141 Surgical preparation, infection prevention of, 143 Surgimend®, for congenital diaphragmatic hernia, 390 Surgisis® (SIS), for congenital diaphragmatic hernia, 390 Sweat test, in cystic fibrosis diagnosis, 523

Swelling in crotalid snakebites, 188 in secondary brain injury, 258 Swenson procedure, 561, 562f Synchronized intermittent mandatory ventilation (SIMV), 116–117, 117t, 118f Synovial tissue tumors, 1090 Synthetic mesh product, 205 Synthetic patches, nonabsorbable, for congenital diaphragmatic hernia, 389–390 Systemic hypertension, 66 Systems issues, trauma, 219

T

T cell(s), activation, 680–681 T3 (triiodothyronine), 1217 T4 (thyroxine), 1217 TACE. see Transarterial chemoembolization Tachycardia, fluid resuscitation in burns, 201 Tacrolimus, 718 Tapering duodenojejunoplasty, 501 Tardive congenital nevi, 1132 Targeted therapy, in neuroblastoma management, 1027 Tazobactam, perforated appendicitis, 671–672 TBI. see Traumatic brain injury TBSA-based formulas, 198 T-cell deficiencies, 142 99mTc-sestamibi, 1225–1226, 1226f TDF. see Testis determining factor Technetium-99m (99mTc) pertechnetate radionuclide study, 643–644, 644f Technetium-99m (99mTc) scintigraphy, 630 Technetium-99m-labeled diethylenetriamine pentaacetic acid (99mTc-DTPA), 839 Technetium-99m-labeled dimercaptosuccinic acid (99mTcDMSA), 887 TEE. see Total energy expenditure Tegaderm, 204t TEK, 1159 Telangiectasias, 1156 Temozolomide, as anticancer drugs, 973t–974t Temporary urinary diversion, 888–890, 889f–890f Temsirolimus, as anticancer drugs, 973t–974t TENS. see Transcutaneous electrical nerve stimulation Tension pneumoperitoneum, 623–625 Tension pneumothorax, 226, 227f Teratomas, 407–409, 409f–410f, 816, 1066–1081 abdominal, 1077–1078 gastric, 1078 retroperitoneal, 1077, 1078f associated anomalies with, 1067– 1069, 1068f–1069f

Teratomas (Continued) challenges with, in low-income and middle-income countries, 1081, 1081f–1082f cited theory on origin of, 1066f classification of, 1066 diagnosis of, 1069–1081 embryology of, 1066–1067, 1067f fetiform, 1066–1067, 1068f as germ cell tumors, 1066 head and neck, 1078–1081, 1079f cervical teratomas, 1078–1080, 1079f craniofacial teratomas, 1080 epignathus, 1080, 1080f nasopharyngeal teratomas, 1080 oropharyngeal teratomas, 1080 management of, 1069–1081 mediastinal, 1076–1077, 1076f pathology of, 1066–1067 sacrococcygeal, 1069–1076 by site, 1070t thoracic, 1076–1077 intracardiac teratomas, 1077 intraperitoneal teratomas, 1077 mediastinal teratomas, 1070t, 1076–1077, 1076f pulmonary teratomas, 1077 Testicular appendages, torsion of, 821–824, 825f Testicular descent, 805f Testicular microlithiasis, 815f, 817 Testicular neoplasms, 813–817, 813f, 813t carcinoma in situ, 814–815 diagnosis of, 813–814, 814f germ cell tumors, 815–817 mixed, 816–817 seminoma, 817 teratoma, 816 yolk sac tumors, 815–816, 816f nongerm cell tumors, 816f–817f, 817 presentation of, 813–814, 814f Testicular torsion, 821–824, 821f, 821t, 823f age, 822, 822f diagnosis of, 822–823 extravaginal, 821, 822f intravaginal, 821 manual detorsion, 823 perinatal, 824 score distribution and incidence of, 823t Testicular trauma, testicular torsion vs., 825–826 Testis prune belly syndrome, 945 trauma, 825–826 Testis determining factor (TDF), 953 Testosterone in 46,XY, 955–956 Leydig cells, 805 precocious puberty and, 1234 in sexual differentiation, 953 Tetanus, 183, 183t diagnosis of, 183 incidence of, 183 Tetanus immunoglobulin (TIG), 183

Index

Tethered spinal cord, 293–294, 293f–294f Tetracycline, 186 TFLV. see Total fetal lung volume Thalassemia, splenectomy indications, 753 The Abbreviated Injury Scale (ALS), 211 Thecoma-fibroma, 1198t Thelarche, premature, 1208, 1208t Therapeutic esophagoscopy, 422 Therapeutic hypothermia, for traumatic brain injury, 264 Thioguanine, as anticancer drugs, 973t–974t Third-degree burns, 204 Thoracic duct ligation, for chylothorax, 368 Thoracic ectopia cordis, 323, 325f Thoracic fractures, in spinal injuries, 275–277, 276f Thoracic insufficiency syndrome, associated with diffuse skeletal disorders, 324–328, 327f–328f “Thoracic kidney”, 831 Thoracic teratomas. see Teratomas; thoracic Thoracic trauma, 224–235 anatomy in, 224 in chest wall, 224–226 flail chest, 225 open pneumothorax, 225, 225f rib fractures, 224–225 traumatic asphyxia, 225–226, 226f epidemiology of, 224 incidence of, 224 in mediastinum, 230–233 airway injury, 230–231, 230f blunt cardiac injury, 232–233 cardiac injuries, 232–233 esophageal injury, 233 great vessel injuries, 231–232, 231f penetrating injury, 232, 232f physiology in, 224 in pleural cavity and pulmonary parenchyma, 226–228 chylothorax, 227 diaphragmatic injuries, 228, 228f–229f hemothorax, 227, 227f pneumothorax-pulmonary lacerations, 226–227, 227f pulmonary contusion, 227–228, 228f specific injuries in, 224–233, 225b Thoracoabdominal duplication, 631– 634, 632t, 633f Thoracoabdominal injury, 219 Thoracocentesis, for parapneumonic effusion, 363 Thoracoscopic lobectomy, for congenital bronchopulmonary malformations, 357–359, 358f–359f Thoracoscopic repair, for esophageal atresia, 444–445 Thoracoscopy anesthetic intraoperative management of, 47–49, 48f for foregut cysts, 412–413

Thoracoscopy (Continued) for mediastinal tumors, 404 right, for chylothorax, 368 for spontaneous pneumothorax, 372 for teratomas, 408–409 Thoracostomy for parapneumonic effusion, 363 wedge, 328 Thoracotomy, for esophageal atresia, 442–444, 442f–443f Thoracotomy-related morbidity, in esophageal atresia, 449 Thrombin disorder management of, 84 generation of, 78 Thrombocytopenias, 84 Thrombocytosis, 1042–1044 Thromboelastograph, 81 Thromboplastin, 76 Thrombotic disorders, 84–85 Thymic cysts, 410 Thymolipoma, 417 Thymus cysts of, 409–410 hyperplasia, 409–410, 411f rebound, 410 tumors of, 409–410 Thyroglossal duct, embryogenesis of, 1171 Thyroglossal duct cyst, 1171–1173, 1171f–1172f elective surgical excision of, 1172 infection of, 1173 preoperative evaluation for, 1172 recurrence of, incidence of, 1172 Thyroid cancer, 1103 Thyroid carcinoma, 1221–1225 exposure to radiation and, 1221 incidence of, 1221 management of, 1221f RAS proto-oncogene, 1221 RET proto-oncogene, 1221 Thyroid gland, 1217–1225 embryology of, 1217 neoplastic conditions of, 1220–1225 thyroid carcinoma, 1221–1225 thyroid nodules, 1220, 1220f, 1220t non-neoplastic conditions of, 1217– 1220 physiology of, 1217 Thyroid Imaging Reporting and Data System (TIRADS), 1220 Thyroid nodules, 1220, 1220f, 1220t Thyroidectomy for Graves disease, 1219, 1219t for thyroid cancer, 1222 for thyroid carcinoma, 1224–1225 Thyroiditis, 1217–1218 acute suppurative, 1218 chronic lymphocytic, 1217–1218 differential diagnosis of, 1218b subacute (de Quervain), 1218 Thyroid-stimulating hormone (TSH), 1217 Thyrotropin, 1217 Thyroxine (T4), 1217 Tibia, nonphyseal fractures of, 271 Tidal volume (Vt), 111–112 TIG. see Tetanus immunoglobulin

1287

Tight junctions, of intestinal barrier, 538–539 TIP. see Tubularized incised plate urethroplasty TIRADS. see Thyroid Imaging Reporting and Data System Tissue-engineered patches, for congenital diaphragmatic hernia, 390–391 Tissue factor (thromboplastin), 76 TLC. see Total lung capacity TNM classification, of rhabdomyosarcoma, 1118t Todani classification, choledochal cyst, 695, 696t Toilet training, 870 Tolterodine, 875 Topoisomerase inhibitors, as anticancer drugs, 973t–974t Topotecan as anticancer drugs, 973t–974t in hepatoblastoma treatment, 1050 Torsion, ovarian, 1199–1201, 1200f–1201f Torticollis, 1181–1182, 1181f Total cavopulmonary anastomosis, 45. see also Fontan procedure. Total colectomy, ulcerative colitis surgery, 651 Total energy expenditure (TEE), 20 Total fetal lung volume (TFLV), in congenital diaphragmatic hernia, 382 Total lung capacity (TLC), 112, 112t Total pancreatectomy with islet autotransplantation (TPIAT), 732 Total parenteral nutrition (TPN) for acute pancreatitis management, 740 for gastroschisis, 770 for intestinal transplantation, 721 for jejunoileal atresia/stenosis surgery, 500 for meconium ileus/cystic fibrosis management, 525 Totally implanted central venous catheters, 136, 137f Tourniquets, crotalid snakebite management of, 189 Toxic epidermal necrolysis and StevensJohnson syndrome, 207, 207t, 208f Toxicology screens, of injury risk, 211 TP53, in Wilms tumor, 988, 988t TPIAT. see Total pancreatectomy with islet autotransplantation TPN. see Total parenteral nutrition Trachea, 333 acquired stenosis, 334 agenesis in, 346, 346f anatomy of, 224 clefts in, 339–341, 340f congenital stenosis, 333 malformation of, 332–336 Tracheal occlusion, in congenital diaphragmatic hernia, 391 Tracheal repair, 341–346 for acquired stenosis, 342–343, 344f for congenital stenosis, 341–342, 341f–343f operative techniques in, 343–346, 345f–346f

1288

Index

Tracheitis, bacterial, 337 Tracheobronchial stents, 343 Tracheobronchial tree, 403 injuries to, 230 Tracheobronchoscopy in esophageal atresia, 441 in laryngotracheoesophageal cleft, 451, 453f Tracheoesophageal fistula malformations, 437–458 recurrent, in esophageal atresia, 447–448, 447f–448f Tracheomalacia, in esophageal atresia, 448 Tracheomalacia-bronchomalacia, 336–337 Tracheoplasty, slide, 342, 342f–343f Tracheostomy in airway injuries, 338–339, 339f, 339t T-shaped, 337–338 Transanal (perineal) pull-through, of Hirschsprung disease, 562–564, 567f Transarterial chemoembolization (TACE), 1050 Transcutaneous electrical nerve stimulation (TENS), 873 Transcyte, 204t Transfusion, in liver and spleen injury, 241 Transumbilical laparoscopic-assisted appendectomy (TULAA), 668–669, 670f Transverse vaginal septum, 1191–1192, 1192f–1193f TRAP. see Twin reversal arterial perfusion Trauma abdominal, 236–253 acute pancreatitis, 738–739, 739f ambulance transport, 219 breast diseases and, 1209–1210 centers, 219 colonic, 245 duodenal, 244, 245f, 245t early assessment and management of, 211–223 emergency care for, 215 primary survey, 215, 216b secondary survey, 217–218 emotional support of, 218 epidemiology of, 211 gastric, 244 hospital preparedness, 220 incidence of, 212t injury outcomes of, 212 injury patterns of, 212–215, 213f, 213t injury prevention of, 212 injury risk of, 211 liver, 237–242, 239f pancreatic, 243–244, 243f–244f physical support of, 218 portal vein, 700 prehospital care of, 215 rectal, 245–247, 246f regional pediatric system, 219–220 renal, 236–253, 242f

Trauma (Continued) resuscitation, 215–217 scores, 215t small bowel, 244–245, 246f spleen, 237–242, 239f Traumatic asphyxia, 225–226, 226f Traumatic brain injury (TBI), 254 axonal shearing in, 254 concussion in, 255 initial evaluation for, 258–264 circulation, airway, and breathing in, 258 Glasgow Coma Scale in, 259, 259t, 260f hypotension in, 258 hypoxia in, 258 nonaccidental trauma, 260–261 management of, 258–264 acute surgical, 261 advanced neuromonitoring for, 264 analgesia for, 261 anticonvulsant prophylaxis for, 263 barbiturate therapy for, 263–264 cardiovascular support for, 261 decompressive craniectomy for, 263 hyperosmolar therapy for, 262–263, 263t hyperventilation for, 264 intracranial pressure monitoring and, 262 lumbar drain for, 264 medically refractory intracranial hypertension in, 263 Monro-Kellie doctrine for, 258 neuromuscular blockade for, 261–262 nutrition for, 262 patient positioning for, 261 pediatric brain trauma guidelines in, 261 respiratory monitoring and, 261 sedation for, 261 therapeutic hypothermia for, 264 mechanisms of, 254–255 nonpenetrating cranial trauma in, 254 outcome of, 264 pathophysiology of, 255–258, 255f penetrating trauma in, 254 primary, 256–257, 256f–257f prognosis of, 264 secondary, 257–258 Trichilemmal cysts, 1084 Trichobezoars, 486 Triiodothyronine (T3), 1217 Trimethoprim-sulfamethoxazole, 858t Trisomy 18, in esophageal atresia, 437 Trisomy 21 in achalasia, 424 anesthesia preoperative evaluation and, 38 in duodenal atresia/stenosis, 489 in esophageal atresia, 437 True hermaphrodite, 957 TSH. see Thyroid-stimulating hormone T-shaped tracheostomy, 337–338 TTTS. see Twin-twin transfusion syndrome

T-tubes, 343 silicone, 346 Tube enterostomy, in simple meconium ileus management, 525f Tube thoracostomy, for parapneumonic effusion, 363 Tubular adenomas, breast masses and, 1212 Tubularized incised plate urethroplasty (TIP), 925, 926f Tufted angioma, 1153–1154 TULAA. see Transumbilical laparoscopicassisted appendectomy Tumor lysis syndrome, 1109 Tumor necrosis factor (TNF), septic shock, 13 Tumor suppressor genes, 970 Tumor volume to fetal weight ratio (TVR), 158, 159f Tumors of adipose tissue, 1089–1090, 1089f–1090f of choroid plexus, 298, 298f of fibrous tissue, 1088–1089 of mesoderm, 1088 of nerve tissue, 1085–1088 neural, 413–417, 414f–416f pancreatic exocrine, 746 of soft tissue, 1084–1090, 1084t of synovial tissue, 1090 of thymus, 409–410 Tumor-targeted antibody therapy, 978–979 Turner syndrome, 832 TVR. see Tumor volume to fetal weight ratio Twin reversal arterial perfusion (TRAP), 161 Twin-twin transfusion syndrome (TTTS), 160–161 amnioreduction for, 160 laser ablation for, 160 polyhydramnios, 160 stages of, 160, 160t

U

UAC. see Umbilical artery catheter UC. see Ulcerative colitis UDT. see Undescended testes UES. see Upper esophageal sphincter UESL. see Undifferentiated embryonal sarcoma of the liver Ulcerative colitis (UC), 647–655 clinical presentation, 648–649, 648f, 648b diagnosis, 649 epidemiology, 647 etiology, 647 historical aspects, 647 medical management, 649–650 pathology, 647–648 surgical management, 650–654, 651t elective operation, 651, 652t emergency operations, 651 laparoscopic technique, 653–654, 654f–655f

Index

Ulcerative colitis (UC) (Continued) open proctocolectomy with ileoanal pull-through procedure, 651–653, 652f outcomes, 654–655 preoperative consideration, 650– 651 Ultra-short segment Hirschsprung disease, 573 Ultrasound (US) for acute pancreatitis diagnosis, 739 for appendicitis, 666, 666f for arteriovenous malformation, 1161 for biliary atresia diagnosis, 682–683, 682f–683f for blunt abdominal trauma, 237 for central venous catheters, 136f for choledochal cyst, 696, 697f for congenital diaphragmatic hernia, 381, 381f for congenital pulmonary airway malformation, 349–350, 350t for cystic fibrosis prenatal diagnosis, 518–519 for duodenal atresia/stenosis diagnosis, 492 for ectopic ureter, 846f, 847 for esophageal atresia, 439 for fetal therapy, 153 for gastroschisis diagnosis, 764 for genital examination, 1188 for hepatoblastoma, 1044 for hepatocellular carcinoma, 1040 for hydrocephalus, 286 for hypertrophic pyloric stenosis diagnosis, 478, 479f for imperforate hymen, 1191 for infantile hemangioma, 1150 for infantile hepatic hemangiomas, 1031–1032 for inguinal hernia, 787 for insulinomas, 745–746 for intussusception diagnosis, 622– 623, 623f for jejunoileal atresia/stenosis, 498 lung-to-head ratio, 94–95 for malrotation diagnosis, 509 for Meckel’s diverticulum diagnosis, 642–644 for megaureter, 845–846, 845f for mesenchymal hamartoma, 1036 for necrotizing enterocolitis, 542 for neonatal acute renal injury, 65 for neuroblastoma diagnosis, 1016 for omphalocele, 771 for parapneumonic effusion, 362 for peripheral venous access, 133–134 for pneumothorax-pulmonary lacerations, 226 for posterior urethral valves, 884 for testicular neoplasms, 807 for testicular torsion diagnosis, 822–823 for thyroglossal duct cyst, 1171–1172 for thyroid carcinoma, 1222 for undescended testes, 807 for ureteropelvic junction obstruction, 837

Ultrasound (US) (Continued) for urinary tract infections, 856–857 for vascular compression, 335–336 for venous malformation, 1160 Umbilical and other abdominal wall hernias, 780–782 Umbilical artery catheter (UAC), 133, 134f Umbilical hernias, 780–781 anatomy of, 780, 780f incidence of, 780 treatment of, 780–781, 781f Umbilical vein and artery access, 134, 134t Umbilical venous catheter (UVC), 133 Underactive bladder, 873 Undescended testes (UDT), 805–813, 805f classification of, 806 diagnosis of, 806–807 embryology of, 805–806 fertility and, 807 hypospadias, 921–922 incidence of, 806 management and treatment for, 808–813 hormonal treatment in, 808 indication for, 808 nonpalpable undescended testes, 809–813, 811f–812f orchiopexy, 808–809, 810f palpable undescended testes, 809, 811f secondary or iatrogenic, 813 timing for, 808 nomenclature of, 806 risk of malignancy and, 807–808 Undifferentiated embryonal sarcoma of the liver (UESL), 1054–1055 clinical presentation of, 1054 histology of, 1055, 1055f imaging of, 1054, 1054f incidence of, 1054 treatment of, 1055 Undifferentiated lymphomas, 405 Unequal placental sharing, monochorionic twins, 161 Unilateral hypertrophy, breast disease and, 1208 Unilateral renal agenesis, 830, 830b United Network Organ Sharing (UNOS), liver transplantation, 713 UNOS. see United Network Organ Sharing Upper body veins, central venous catheters, 135–136 Upper esophageal sphincter (UES), 422 Upper extremity fractures, 214–215, 277–280, 277f–280f Upper-pole heminephrectomy, 849 Upper-pole partial nephroureterectomy, 849 Upper respiratory tract infection, anesthesia, 39 Upper sigmoid (descending) colostomy, 584, 585f Urease, 856 Ureter, prune belly syndrome, 943–944, 943f–944f

1289

Ureteral abnormalities, 843–846 duplication, 843–844, 844f megaureter. see Megaureter retrocaval ureter, 845, 845f Ureteral folding techniques, for megaureter, 846 Ureteral obstruction and malformations, 837–852, 837f Ureteral reconstruction, prune belly syndrome, 947 Ureteral tapering, for megaureter, 846 Ureteroceles, 848–850 classification of, 848 diagnosis of, 848–849, 848f presentation of, 848–849, 849f treatment of, 849–850, 849f Ureteropelvic junction obstruction, in children, 837–842 antibiotic prophylaxis for, 838–839 classification for, 838–839 clinical presentation of, 837, 838f diagnosis of, 838–839, 839t diuretic isotopic renogram, 839, 840f magnetic resonance urography, 839, 841f voiding cystourethrogram, 838–839 duplex kidney, 842 etiology of, 837, 838f histologic evaluation for, 837 incidence of, 837 management of, 839–842 indications for, 839–841, 840f operative techniques for, 841–842, 841f–843f surgical results and complications of, 842–843 Ureteroureterostomy, for ectopic ureters, 847 Ureterovesical junction, abnormalities of, 855, 856f Ureters development, 843 retrocaval, 845, 845f Urethra, 870–883 hypospadias, 924 prune belly syndrome, 944, 944f Urethral atresia, 880 Urethral disorders, 879–881 anterior urethral valve, 880–881, 880f congenital urethral fistula, 880 cystic Cowper’s gland ducts, 881 meatal stenosis, 879 megalourethra, 879–880 urethral atresia, 880 urethral diverticulum, 880–881 urethral duplication, 880 urethral prolapse, 879, 879f urethral strictures and stenosis, 880 Urethral diverticulum, 880–881 Urethral duplication, 880 Urethral prolapse, 879, 879f genital bleeding and, 1190, 1190f Urethral reconstruction, prune belly syndrome, 947 Urethral strictures and stenosis, 880 Urethrocutaneous fistula, hypospadias, 930

1290

Index

Urethrovaginal fistula, 597 Urgency incontinence, 871 Urinary creatinine excretion, 57–58 Urinary diversion bladder exstrophy, 899, 899t hypospadias, 927–928, 929f–930f Urinary frequency, urinary tract infection and, 853 Urinary incontinence continuous, 846–847, 873 daytime, 870 nocturnal enuresis, 870 primary, 870 secondary, 870 Urinary sphincter, artificial, 878, 878f Urinary tract infections, 853–869 circumcision, 936 classification of, 854, 854f diagnosis of, 853–854, 853t epidemiology of, 854, 855f incidence of, 854, 855t investigations for, 856–857 lower, vs. upper, 854 pathophysiology of, 855–856 bacterial factors, 855–856, 856b host factors, 855, 855f–857f treatment of, 857–858 acute phase of, 857–858 prophylactic antibiotics, 858 Urine culture, quantitative, 853–854, 854t Urine indices, 65 Urine output, 197 Urine volume, 58 Urinoma, in renal trauma, 249 Urodynamic studies for bladder instability, 872 noninvasive, 860 Urogenital system, anomalies of, congenital diaphragmatic hernia and, 377–378 Ursodeoxycholic acid, 687–688 US. see Ultrasound Uterovaginal anomalies, 1191–1193 imperforate hymen, 1191, 1191f–1192f Mayer-Rokitansky-Küster-Hause syndrome, 1193, 1193f–1194f transverse vaginal septum, 1191– 1192, 1192f–1193f uterine duplication, 1192–1193, 1193f Uterus anomalies. see Uterovaginal anomalies duplication, 1192–1193, 1193f rhabdomyosarcoma, 1122 UVC. see Umbilical venous catheter

V

VACTERL, 438 association, of renal agenesis, 830 Vacuum-assisted closure devices, for arteriovenous malformation, 1162 VAD. see Vascular access device Vagal nerve stimulators (VNS), 287 Vagina, 1188 anomalies. see Uterovaginal anomalies augmentation/replacement, 593–594

Vagina (Continued) introital masses, 1190–1191, 1191f reconstruction with rectum, 593, 594f reconstruction with small bowel, 594, 595f replacement with colon, 594, 594f rhabdomyosarcoma, 1122 transverse septum, 1191–1192, 1192f–1193f Vaginal delivery after cesarean section (VBAC), 155 Vaginitis, recurrence, 1189 Valsalva maneuver, 225–226 Valve bladder syndrome, 893t Vandotanib, as anticancer drugs, 973t–974t Vanishing testis syndrome, 958 VAP. see Ventilator-associated pneumonia VAPSV. see Volume-assured pressure support ventilation Variant liver allografts, 712 Vascular access, 133–140 anesthesia intraoperative management of, 46 complications of, 133 indications for, 133t venous cutdown of, 137–138 Vascular access device (VAD), 133 Vascular anomalies, 410–411, 1147–1170 capillary malformations, 1155, 1155f classification of, 1147, 1148t combined vascular malformations as, 1162–1165 cutis marmorata telangiectatica congenita, 1156 nomenclature of, 1147 telangiectasias, 1156 tumors, 1147–1154 Vascular disruption, in jejunoileal atresia/ stenosis, 495 Vascular endothelial growth factor (VEGF), 1155 Vascular malformations, 411, 412f of brain, 291–293, 292f–293f Vascular system, embryology and development of, 1155 Vascular thrombosis, 716–717, 717b Vascular tumors, 411, 1147–1154 liver transplantation, 711 Vasoconstriction, trauma resuscitation, 217 Vasogenic swelling, in secondary brain injury, 257–258 Vasopressin, as vasoactive medications in newborn, 12t VATS. see Video-assisted thoracoscopic surgery VBAC. see Vaginal delivery after cesarean section VCUG. see Voiding cystourethrography VEGF. see Vascular endothelial growth factor Vein of Galen aneurysm, 293 Vemurafenib, for melanoma, 1139–1140 Venoarterial (VA) cannulation, 99f Venography, for venous malformation, 1160

Venous cannulation, 96 Venous cutdown, of vascular access, 137–138 Venous malformations, 1158–1160 clinical features of, 1158–1160, 1159f imaging of, 1160 treatment of, 1160 Venous oximetry, 11 Ventilation/perfusion scans, in bronchiectasis, 367 Ventilator-associated pneumonia (VAP), 126–128, 127b Ventilator-induced lung failure, prevention of, 120, 121f Ventilatory dynamics, 47 Ventriculoperitoneal shunts (VPS), inguinal hernia and, 785 VEPTR. see Vertically expandable prosthetic titanium rib Versajet technique, 205 Vertebral anomalies, 438 Vertically expandable prosthetic titanium rib (VEPTR), 328, 328f Very low birth weight infants (VLBW), necrotizing enterocolitis in, 536 Vesicostomy closure, 892 cutaneous, 888, 889f prune belly syndrome, 947 Vesicoureteral reflux (VUR), 892 classification of, 859, 859f diagnostic evaluation of, 860 epidemiology of, 860 incidence of, 860 natural history for, 860–862, 861f pathophysiology of, 859, 859f prune belly syndrome, 943 Randomized Intervention for Children with Vesicoureteral Reflex (RIVUR), 857 treatment of, 862–866 American Urological Association (AUA) Pediatric Vesicoureteral Reflux Guidelines, 863, 863t antireflux procedures, 863, 864t medical management in, 862–863, 862b, 864f–865f surgical management in, 863–866, 866f urinary tract infection, 858–867 Vestibular fistula, in neonatal anorectal defects, 580f, 583 Video-assisted thoracoscopic surgery (VATS), 363–364 Vinblastine adverse effects, 972 for cancer chemotherapy, 973t–974t Vincristine adverse effects, 972 for cancer chemotherapy, 973t–974t in Wilms tumor chemotherapy, 999 Vinorelbine, as anticancer drugs, 973t–974t VIPomas, 745 Viral infections of hemophilia A and B, 82 post-liver transplantation, 719 Viral laryngotracheitis, 337

Index

Virilization of adrenocortical carcinoma, 1231 in congenital adrenal hyperplasia (CAH), 954–955 Virulence, 141 factors, 856 Vitamin(s), postoperative bariatric surgery, 1248 Vitamin K deficiency, 84 VLBW. see Very low birth weight infants VNS. see Vagal nerve stimulators Vocal cord dysfunction, in esophageal atresia, 449 Voiding cystourethrography (VCUG) for neonatal acute renal injury, 65 prune belly syndrome, 943f urinary tract infections, 856–857 Voiding dysfunction (VD), bladder instability and, 872 Volume-assured pressure support ventilation (VAPSV), 117, 117t Volume-controlled ventilation, 116 Volume-limited ventilation, 116 Volume/pressure relationship, mechanical ventilation, 112, 112f Volume resuscitation in ductal intestinal obstruction syndrome, 529 trauma resuscitation, 216–217 Volume support ventilation (VSV), 117, 117t Voluntary muscle structures, in fecal incontinence, 599 Volvulus, malrotation and, 513 von Willebrand disease, 78 von Willebrand factor (vWF), 76 VPS. see Ventriculoperitoneal shunts VSV. see Volume support ventilation Vulva abnormalities, 1188–1189 dermatoses, 1189, 1189f hematomas, 1190, 1191f rhabdomyosarcoma, 1122 VUR. see Vesicoureteral reflux vWF. see von Willebrand factor

W

Waddell triad of injury, 212, 213f Wandering spleen, 750, 751f Warts, 1084–1085 Water requirements, 58t Water-soluble contrast enema, 558 Wedge thoracostomy, 328 Weight loss, Crohn disease, 656

Whipple procedure, chronic pancreatitis, 743 WHO. see World Health Organization Wilms tumor (WT), 986–1003 bilateral, 1001, 1001f chemotherapy for, 998–999, 999t Children’s Oncology Group Renal tumor studies, 999 clinical presentation of, 994–995 diagnosis of, 994–995, 994f differential diagnosis of, 994 epidemiology of, 987–988 extrarenal, 998 health consequences of, 1001–1003, 1002t congestive heart failure, 1002–1003 general health, 1001–1002, 1002f pregnancy, 1002 renal function, 1002 secondary malignancies, 1002, 1002f thoracic, 1003 histology of, 991, 991f history of, 986–987 molecular genetics of, 988–989, 988t 1q gain, 989 CTNNB1, 988, 988t loss of heterogeneity, 989, 989f TP53, 988, 988t WT1, 988t, 989 WT2, 988t, 989 WTX, 988, 988t multicystic dysplastic kidneys, 989–991 neonatal, 998 nephroblastomatosis, 989–991, 991f nephrogenic rests, 989–991, 990f, 990t pathologic precursors of, 989–991 pathology of, 991–993, 991f–993f prognostic factors in, 994 radiotherapy, 1000 staging, 993, 993t studies, 987t surgery for, 995–998, 996f complications, 996 cystoscopy, 995 intravascular extension, 993f, 996–997 lymph node biopsy, 995 pretreatment prior to, 999–1000 SIOP protocols, 996 tumor extension in ureter, 998 unresectable tumors, 996, 997f very low risk Wilm’s tumor, 998 treatment of, 970, 995

1291

Wirsung duct, 737 Wis-Hipple laryngoscope, 338 Wolffian system, 828 World Health Organization (WHO), Hodgkin lymphoma classification of, 1099 Wounds classification, 143–144, 144t dehiscence, 482 infection, 481 Wrist, fractures of, 280 WT. see Wilms tumor WT1 in sexual differentiation, 953 in Wilms tumor, 988t, 989 WT2, 988t, 989 WTX, 988, 988t

X

X chromosome, 953 Xanthomas, 1088, 1088f Xenograft, of burn wound care, 204t, 205 Xenon-enhanced computed tomography, 255 Xenon lung scanning, inhalation injury of, 201–202 Xeroderma pigmentosum, melanoma and, 1136 46,XX (female pseudohermaphrodite), 953t, 954–955, 955f 46,XX testicular DSD (XX sex reversal), 959 46,XY (male pseudohermaphrodite), 955–956

Y

Y chromosome, 953 Yolk sac tumors, 815–817, 1198t Young-Dees technique, 878 Young-Dees-Leadbetter technique, 909

Z

ZES. see Zollinger-Ellison syndrome ZIC3 transcription factor, 680 Zollinger-Ellison syndrome (ZES), 746 Zone of coagulation, burns, 196 Zone of hyperemia, burns, 196 Zone of stasis, burns, 196

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