Добавил:
Sekretar
kiopkiopkiop18@yandex.ru
t.me/Prokururor I Вовсе не секретарь, но почту проверяю
Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз:
Предмет:
Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_697_Библиотеки_им_академика_М_И_Перельмана.pdf
xx
11.3 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 165
11.4 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 166
11.4.1 Colostomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 166
11.4.2 Main Repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 166
11.4.3 Laparotomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 168
11.4.4 Laparoscopy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 178
11.5 Special Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 179
11.5.1 Dealing with Inadequate Colostomies
(Too Distal) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 179
11.6 Functional Results . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 179
11.6.1 Fecal Control . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 179
11.6.2 Urinary Control . . . . . . . . . . . . . . . . . . . . . . . . . . . 180
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 181
12 Imperforate Anus Without Fistula in Males and Females . . . . 183
12.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 183
12.2 Anatomic Characteristics . . . . . . . . . . . . . . . . . . . . . . . . . . . 184
12.3 Main Repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 185
12.4 Function and Results . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 187
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 187
13 Minimally Invasive Approach to Anorectal Malformations . . . 189
13.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 189
13.2 Males . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 192
13.3 Females . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 194
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 196
Contents
14 Rectal Atresia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 201
14.1 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 202
14.2 Surgical Repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 202
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 204
15 Rectovestibular Fistula . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 205
15.1 Defi nition/Frequency . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 205
15.2 Associated Defects . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 207
15.2.1 Sacral . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 208
15.2.2 Spinal . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 208
15.2.3 Urologic . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 208
15.2.4 Gynecologic . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 208
15.2.5 Gastrointestinal . . . . . . . . . . . . . . . . . . . . . . . . . . . 209
15.2.6 Tethered Cord . . . . . . . . . . . . . . . . . . . . . . . . . . . . 209
15.2.7 Cardiovascular . . . . . . . . . . . . . . . . . . . . . . . . . . . 209
15.3 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 211
15.4 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 212
15.4.1 Colostomy or No Colostomy . . . . . . . . . . . . . . . . 212
15.5 Main Repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 213
15.6 Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 219
15.7 Functional Results . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 219
15.8 Reoperations in Patients with Vestibular Fistula . . . . . . . . 220
15.9 Surgical Technique . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 222
15.10 Rectovestibular Fistula with Normal Anus . . . . . . . . . . . . 223
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 223
Contents
xxi
16 Cloaca, Posterior Cloaca and Absent Penis Spectrum . . . . . . . 225
16.1 Cloaca . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 225
16.1.1 Defi nition and Management . . . . . . . . . . . . . . . . . . . . 225
16.1.2 Urologic Concerns . . . . . . . . . . . . . . . . . . . . . . . . . . . 260
16.1.3 Gynecologic Concerns . . . . . . . . . . . . . . . . . . . . . . . . 261
16.1.4 Reoperations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 262
16.1.5 Transpubic Approach . . . . . . . . . . . . . . . . . . . . . . . . . 268
16.2 Posterior Cloaca and Absent Penis Spectrum . . . . . . . . . . . . 270
16.2.1 Surgical Repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 275
16.2.2 Surgical Repair of the 2-Perineal-Orifi ce
Variant of the Posterior Cloacal Spectrum . . . . . . . . 276
16.2.3 Posterior Cloaca and Absent Penis . . . . . . . . . . . . . . 276
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 279
17 Cloacal Exstrophy and Covered Cloacal Exstrophy . . . . . . . . . 285
17.1 Neonatal Approach. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 289
17.2 Pull-Through or “Permanent Stoma” . . . . . . . . . . . . . . . . . . . 291
17.3 Covered Cloacal Exstrophy . . . . . . . . . . . . . . . . . . . . . . . . . . 293
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 295
18 General Principles for the Postoperative Management
of Patients with Anorectal Malformations . . . . . . . . . . . . . . . . . 299
18.1 General Care. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 299
18.2 Local Care . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 300
18.3 Anal Dilatations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 300
18.4 Avoiding Constipation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 303
18.5 Toilet Training . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 305
19 Postoperative Evaluation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 307
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 309
20 Bowel Management for the Treatment
of Fecal Incontinence . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 311
20.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 311
20.2 Goals of the Bowel Management Program . . . . . . . . . . . . 314
20.3 Evaluation of the Patient for Bowel Management . . . . . . . 315
20.4 Individualization of the Management . . . . . . . . . . . . . . . . 316
20.5 Laxative Trial . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 320
20.6 About Our Program . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 321
20.7 Content of the Enema . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 323
20.8 Rationale to Change the Type of Enema . . . . . . . . . . . . . . 325
20.9 Bowel Management for the Treatment of Severe
Diaper Rash . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 329
20.10 Bowel Management Through a Stoma . . . . . . . . . . . . . . . . 330
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 331
21 Operations for the Administration of Antegrade Enemas . . . . 333
21.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 333
21.2 Our Preferred Technique . . . . . . . . . . . . . . . . . . . . . . . . . . . . 334
21.3 Surgical Technique: Continent Appendicostomy . . . . . . . . . . 336
21.4 Continent Neo-appendicostomy . . . . . . . . . . . . . . . . . . . . . . . 339
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 345
xxii
22 Reoperations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 349
22.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 349
22.2 Reoperations to Improve Bowel Control . . . . . . . . . . . . . . . . 350
22.3 Reoperations Performed After Failed Attempted
Repair (Catastrophes) Males . . . . . . . . . . . . . . . . . . . . . . . . . 356
22.4 Reoperations for Postoperative Recto- urinary Fistula . . . . . . 357
22.4.1 Recurrent Fistula (17 Cases) . . . . . . . . . . . . . . . . . . . 357
22.4.2 Persistent Rectourethral Fistula (24 Cases). . . . . . . . 359
22.4.3 Acquired Fistula (9 Cases) . . . . . . . . . . . . . . . . . . . . 360
22.5 Posterior Urethral Diverticulum (32 Cases) . . . . . . . . . . . . . . 361
22.6 Acquired Rectal Atresia or Stenosis (83 Cases) . . . . . . . . . . 361
22.7 Presacral Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 362
22.8 Reoperations in Female Patients . . . . . . . . . . . . . . . . . . . . . . 362
22.9 Prolapse . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 363
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 366
23 Urologic Problems in Anorectal Malformations . . . . . . . . . . . . 371
23.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 371
23.2 Neonatal Approach. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 372
23.3 The Importance of the Colostomy Type from
the Urologic Point of View. . . . . . . . . . . . . . . . . . . . . . . . . 373
23.4 Most Common Urologic Abnormalities in Male
Patients with Anorectal Malformations . . . . . . . . . . . . . . . 373
23.4.1 Absent Kidney . . . . . . . . . . . . . . . . . . . . . . . . . . . 373
23.4.2 Urethral Problems . . . . . . . . . . . . . . . . . . . . . . . . . 376
23.5 Bifi d Scrotum . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 377
23.6 Hypospadias . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 380
23.7 Ectopic Ureters in Males . . . . . . . . . . . . . . . . . . . . . . . . . . 382
23.8 Ectopic Ureters in Females . . . . . . . . . . . . . . . . . . . . . . . . 382
23.9 Ectopic Vas Deferens . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 384
23.10 Ectopic Verumontanum . . . . . . . . . . . . . . . . . . . . . . . . . . . 385
23.11 Megalourethra . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 386
23.12 Ureterovesical and Ureteropelvic Obstruction . . . . . . . . . . 386
23.13 Neurogenic Bladder . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 386
23.14 Postoperative Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . 389
23.15 Posterior Urethral Diverticulum . . . . . . . . . . . . . . . . . . . . . 390
23.16 Sexual Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 391
23.17 Tethered Cord . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 391
23.18 The Ultimate Concern, Kidney Function . . . . . . . . . . . . . . 392
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 392
Contents
24 Hirschsprung’s Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 397
24.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 397
24.2 Historical Review . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 398
24.3 Incidence, Inheritance, and Associated Anomalies . . . . . . 399
24.4 Pathogenesis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 400
24.5 Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 403
24.6 Clinical Manifestations and Differential Diagnosis . . . . . . 403
24.7 Histologic Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 405
24.8 Differential Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . 406
Contents
xxiii
24.9 Early Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 407
24.10 Surgical Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 407
24.10.1 The Authors’ Approach . . . . . . . . . . . . . . . . . . . 408
24.10.2 Other Surgical Techniques for the
Treatment of Hirschsprung’s Disease . . . . . . . . . 417
24.11 Total Colonic Aganglionosis . . . . . . . . . . . . . . . . . . . . . . . 422
24.12 Ultrashort-Segment Hirschsprung’s Disease . . . . . . . . . . . 425
24.13 Problems, Complication, and Sequela Secondary
to Operations for Hirschsprung’s Disease . . . . . . . . . . . . . 425
24.13.1 Preventable Complications (Catastrophes) . . . . . . 425
24.13.2 Non-preventable Complications . . . . . . . . . . . . . . 429
24.13.3 Partially Preventable Complications . . . . . . . . . . . 430
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 430
25 Idiopathic Constipation and Other Motility Disorders . . . . . . . 435
25.1 Defi nition and Terminology . . . . . . . . . . . . . . . . . . . . . . . . 435
25.2 Incidence, Social Impact, and Relevance . . . . . . . . . . . . . . 435
25.3 Etiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 435
25.3.1 Ultrashort Segment Hirschsprung’s Disease . . . . 436
25.3.2 Rectal Manometry . . . . . . . . . . . . . . . . . . . . . . . . 437
25.3.3 Doubts and Questions About the Anatomy
of the Internal Sphincter . . . . . . . . . . . . . . . . . . . . 437
25.3.4 Questions About Myectomy Technique . . . . . . . . 438
25.3.5 Botulinum Toxin Injection . . . . . . . . . . . . . . . . . . 438
25.4 Pathogenesis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 440
25.5 Natural History and Clinical Manifestations . . . . . . . . . . . 442
25.6 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 443
25.6.1 Colonic Transit Time . . . . . . . . . . . . . . . . . . . . . . 445
25.6.2 The Evaluation of Severity: Search
for Objective “Instruments” . . . . . . . . . . . . . . . . . 446
25.7 Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 447
25.7.1 Fecal Disimpaction Protocol . . . . . . . . . . . . . . . . 448
25.7.2 Determination of Laxative Requirements . . . . . . . 449
25.7.3 Electric Stimulation . . . . . . . . . . . . . . . . . . . . . . . 450
25.8 Surgical Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 450
25.8.1 Operations to Administer Antegrade Enemas
(ACE Procedures) . . . . . . . . . . . . . . . . . . . . . . . . . 450
25.8.2 Colonic Resection . . . . . . . . . . . . . . . . . . . . . . . . . 451
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 453
26 Posterior Sagittal Approach for the Treatment
of Other Conditions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 457
26.1 The Kraske Operation . . . . . . . . . . . . . . . . . . . . . . . . . . . . 457
26.2 Urogenital Sinus with Normal Rectum . . . . . . . . . . . . . . . 458
26.3 Urogenital Sinus with Normal Rectum
and Adrenal Hyperplasia . . . . . . . . . . . . . . . . . . . . . . . . . . 467
26.4 Acquired Urethral Atresia . . . . . . . . . . . . . . . . . . . . . . . . . 467
26.5 Acquired Rectourethral Fistula . . . . . . . . . . . . . . . . . . . . . 471
26.6 Giant Seminal Vesicle . . . . . . . . . . . . . . . . . . . . . . . . . . . . 472
26.7 Urethral Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 473
xxiv
26.8 Acquired Rectovaginal Fistula . . . . . . . . . . . . . . . . . . . . . . 473
26.9 Rectal Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 475
26.10 Presacral Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 479
26.11 Surgical Technique . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 480
26.12 Posterior Sagittal Approach, Its Application in Cases
with Hirschsprung’s Disease . . . . . . . . . . . . . . . . . . . . . . . 481
26.13 Vaginal Atresia with Normal Rectum . . . . . . . . . . . . . . . . 482
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 484
27 Miscellaneous Conditions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 487
27.1 Part I: Perianal Abscess and Fistula . . . . . . . . . . . . . . . . . . 487
27.1.1 Defi nition . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 487
27.1.2 Etiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 488
27.1.3 Natural History . . . . . . . . . . . . . . . . . . . . . . . . . . . 488
27.1.4 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 489
27.1.5 Fistulotomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 489
27.2 Part II: Perianal Fistula and Rectovestibular Fistula
with Normal Anus in Females . . . . . . . . . . . . . . . . . . . . . . 490
27.2.1 Surgical Treatment . . . . . . . . . . . . . . . . . . . . . . . . 490
27.3 Part III: Other Conditions . . . . . . . . . . . . . . . . . . . . . . . . . . 493
27.3.1 Anal Fissure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 493
27.3.2 Hemorrhoids . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 493
27.3.3 Idiopathic Rectal Prolapse . . . . . . . . . . . . . . . . . . 493
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 496
Contents
History of the Treatment of Anorectal Malformations
1
1.1 Introduction
So the conservative who resists change is as valuable as the radical who proposes it. It is good that
new ideas should be heard, for the sake of the few
that can be used; but it is also good that new ideas
should be compelled to go through the mill of
objection, opposition, and contumely; this is the
trial heat which innovations must survive before
being allowed to enter the human race. It is good
that the old should resist the young, and that the
young should prod the old; out of this tension, as
out of strife of the sexes and the classes, comes a
creative tensile strength, a stimulated development, a secret and basic unity and movement of the
whole. By Will and Ariel Durant [
The history of the surgical treatment of anorectal malformations is a representative sample
of the history and evolution of medicine.
Centuries ago, medicine was related to religion
and mysticism; the treatment of the different diseases and surgical conditions was performed by
witches, barbers, or those who showed some
“wisdom” in the community. It took many centuries for medicine and surgery to become scientifi c disciplines. Even in current days, the practice
of medicine and surgery has a great element
of art.
Because of its nature, an anorectal malformation is a particular defect that has been well
known for many centuries. The explanation is
very obvious; one does not have to be a doctor to
make the diagnosis of an absent anal opening.
That is perhaps one of the explanations for the
1 ]
existence of illustrations in history books, going
back hundreds of years in different cultures and
civilizations, related to the treatment of surgical
conditions of the anus.
One’s goal in the study of history should not
be to try to memorize names and dates, but rather
to take advantage of the unique opportunity to
look back and have a wide perspective of the evolution of our knowledge. Contemplation of the
historical facts, hopefully without prejudices,
allows us to recognize patterns of human behavior. Some of those patterns are creative and positive and should be imitated, and some others are
to be abandoned. It allows us to see repetitive
behaviors that disclose our limitations as human
beings as well as the creativity when dealing with
unknown facts. One can learn, for instance, that
some of the “new discoveries” are not really new.
Other times, an old concept is brought back, but
with a different vision, and even when it is not
essentially new, represents an advantage when
compared to previous procedures. The dilemma
of those who study history is always the commitment to “the truth.” We are limited by the literature that is available that may or may not be
absolutely truthful.
Finally, we, the authors of this book, must
confess that we are biased when describing the
history of the surgical treatment of anorectal
malformations. We are biased and impressed by
the fact that the real, intrinsic anatomy of the
anorectal malformations was really not known
until 1980. Looking into the many historical
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_1, © Springer International Publishing Switzerland 2015
1
2
1 History of the Treatment of Anorectal Malformations
publications that we reviewed, one can fi nd diagrams that only show the imagination of the
authors and the medical illustrators, but not the
real anatomy. Those diagrams were followed by
interpretations and erroneous conclusions about
what should and should not be done in the treatment of these malformations. There are very few
photographs showing the real anatomy, for
instance, of the connection between the gastrointestinal tract and the urogenital tract. Some of
the few real pictures of the intrinsic anatomy of
these defects prior to 1980 belong to the publications of Dr. Douglas Stephens [ 2 ]. Yet, they are
not representative of the whole spectrum of anorectal malformations.
The retrospective analysis of the history of
anorectal malformations shows a very common
human tendency to classify biological phenomena into types, groups, and categories. It is understandable that this is usually done for the specifi c
purpose of communicating among ourselves and
comparing our results. Yet, Mother Nature continues producing biological phenomena following a pattern of a spectrum without paying much
attention to our classifi cations. Anorectal malformations are not an exception. In other words,
anorectal malformations do not occur in artifi cially created groups, traditionally described as
“high,” “intermediate,” and “low.” They occur as
most biological phenomena, following a spectrum type of pattern. Over time and with careful
analyses of presentation and results, it has
become more and more clear that there are no
“nevers” and no “always” when describing the
variety of anorectal malformations.
1.2 The Early Times
The fi rst reference of an anorectal malformation
was found in Babylon, about 650 years B.C. It
was written in stone, “When a woman gives birth
to a baby with a closed anus the entire Earth will
suffer from disease” [ 3 ].
Geracao and Aristotle wrote a book on the
Generation of Animals; there, they described a
cow that was born without an anus and defecated
through the urethra [ 4 ].
Soranus de Ephesus was considered the father
of obstetrics in ancient Rome. He wrote the book
On the Care Of the Newborn . In that book, one
can read that he instructed the women in charge
of delivering babies how to trim off their fi ngernail of the little fi nger, to dilate the anus of those
babies who did not pass meconium after birth [
5 ].
Paul of Aegina (625–690) made the fi rst description of an operation for imperforate anus: “If possible, the membrane that covers the anus must be
divided with the fi nger. If this is not successful,
then an incision must be done.” To avoid or to prevent the scarring or stricture of the new anus, he
recommended a form of bougienage consisting of
the local application of wine and balsam [ 6 ].
Perhaps the fi rst illustration describing an
anorectal procedure in pediatrics was found in a
book entitled Cerrahiyei Ilhaniye , written in
1465 by Dr. Sharaphedin in Turkey [ 7 ].
In 1606, Guilhelmus Fabricius Hildanus
described a case of a recto-bladder fi stula. For
that case, many doctors were consulted; they all
saw meconium coming out of the urethra, and
nobody wanted to do anything. The baby died on
the 17th day of life [ 8 ].
Littre, in 1710, proposed (but did not perform)
the opening of a colostomy in cases of anorectal
malformation [ 9 ].
Frederik Ruysch (1683–1731) was immortalized in a famous painting showing the autopsy of
a baby. He described the spontaneous rupture of
an anal membrane after 5 days of life. The baby
died soon thereafter [
10 ].
The practice of a perineal incision followed by
dilatations, in babies born with “imperforate
anus,” was a method of choice until the later part
of the nineteenth century. During that time, there
were many anecdotal descriptions of babies with
anorectal malformations that were treated that
way, but the overwhelming majority of them died
[ 11 ]. Some surgeons disagreed with the way of
treating those patients, such as Dr. Bigelow,
Professor of Surgery at the Massachusetts
General Hospital in Boston 1857 [ 12 ]. He men-
tioned, “Based on the analysis of the results of
those procedures, I believe that considering the
state of the art in surgery for those anorectal
defects, it is better to let those babies die.”
1.2 The Early Times
3
In 1753, M. Louis from Paris described the
case of a little girl who had an orifi ce that was
considered a cloacal malformation [ 13 ]. She
was menstruating through the anus! That patient
got married and told her secret to her husband.
He convinced her to have sex with him, and she
became pregnant. The lady had a “normal”
delivery and was described as producing a
“minor laceration” of the anal sphincter. The
presentation of that case was considered in the
Parisian courts, and it was decided by theologists and modernists that Dr. M. Louis somehow
had acted in an illegal manner. The father of the
baby was called, M. Louis was fi nally declared
innocent, and the court allowed M. Louis to
publish the case.
In 1771, Bertin [ 14 ] described a case of a baby
that was passing feces through the urethra. He
was convinced that the baby would die unless he
had an operation. He approached the patient
through the perineum and could not fi nd the rectum. The baby died and Bertin concluded that the
operation of choice for that particular case should
have been a cystostomy.
In 1787, Benjamin Bell (1749–1806) from
Edinburgh [ 15 ] described two successful opera-
tions in which the rectum was found to be located
“high” in the pelvis. The procedure that he
described consisted in the introduction of a sharp
instrument in a blind fashion at the location
where the anus was supposed to be located. This
procedure was followed frequently by complications that included bladder perforation and opening of the cul-de-sac of Douglas, and in some
cases, the rectum was never found. In his book
entitled A System of Surgery , Bell described dif-
ferent types of anorectal malformations including
“anal agenesis,” “anorectal agenesis,” “vesical
fi stula,” and “vaginal fi stula.” Benjamin Bell was
probably the fi rst one to emphasize the need and
importance of decreasing the pain during these
procedures that were generally done using
homeopathic techniques.
It was Antoine Dubois, in 1783, who apparently performed the fi rst inguinal colostomy on
the left side in a 1-day-old baby with imperforate
anus. The patient died 10 days later [
16 ]. In 1793,
Duret, following the suggestion of Littre in 1710,
was probably the fi rst one to perform an inguinal
colostomy in the sigmoid colon in a baby boy
with imperforate anus; a week later, the patient
was still alive [
17 ].
In 1832, almost 100 years later, Martin
decided to follow the suggestion of Bertin and to
perform a cystostomy in a patient who was passing stool through the urethra. Unfortunately, the
patient died [ 18 ].
Roux de Brignoles, in 1834, suggested that the
fi bers of the sphincter mechanism should be
meticulously preserved during the perineal dissection [ 19 ].
Amussat, a prominent young surgeon, also in
1835, in Paris, described the case of a 2-day-old
girl who was not passing meconium. He operated
on the patient on the dining room table of the
patient’s house, assisted by his collaborators. He
found the blind rectum, and he is considered the
fi rst surgeon who decided to suture the wall of the
rectum to the skin edges, which could be considered the fi rst anoplasty. After 28 days, the baby
was doing very well, without complications [ 20 ].
It was also Amussat who classifi ed the anorectal
malformations into fi ve types: type 1, anal stenosis; type 2, anal membrane; type three, a blind
rectum at a variable distance from the anal skin;
type 4, a blind but also very “defi cient” rectum;
and type 5, the rectum communicated with other
organs, such as the bladder, urethra, or vagina.
He recommended dilatation for type 1, incision
and excision of the membrane followed by dilatations in type 2, and suture of the rectum to the
skin in type 3. In types 4 and 5, he recommended
mobilization of the posterior part of the rectum
and pulling it down to the perineum. In cases in
which it was diffi cult to fi nd the rectum through
the perineal incision, he recommended making
the incision larger and to totally or partially
remove the coccyx.
In 1844, Stromeyer [
21 ] suggested that in
cases in which the rectum could not be found
through the perineal dissection, the peritoneal
cavity should be opened through the perineum,
and the surgeon should look for the blind rectum with a fi nger. That idea was practiced in
1872 by Leiserink, and he described a “good
result” [ 22 ].
4
1 History of the Treatment of Anorectal Malformations
In 1860, Bodenhamer [ 23 ] proposed a classifi ca-
tion dividing these malformations into four types:
Type 1: Incomplete rupture of the “inner mem-
brane” or anal stenosis
Type 2: Imperforate anus due to a persistence of
the “anal membrane”
Type 3: Imperforate anus with blind rectum sepa-
rated from the “anal membrane”
Type 4: The presence of a blind rectum separated
from the anal canal
In 1866, Chassaignac [ 24 ] decided to follow
the idea suggested by Martin de Lyon of opening
a colostomy in order to introduce some sort of
guide through the intestinal lumen of the colostomy, to facilitate fi nding of the blind rectal end.
The perineum was then opened where the surgeon
could feel the bulging of the guide. Chassaignac
operated on a 7-month-old baby who had a previous colostomy and was able to create an opening
in a “satisfactory” manner using that technique.
Delens, in 1874 [ 25 ], described a case in
which he achieved good exposure in the perineum
area by removing or mobilizing back the coccyx
without resecting it. The next year, Polaillon
described splitting of the coccyx in the midline,
obtaining better exposure to be able to dissect the
rectum in a deeper area [ 26 ].
In 1880, Neil McLeod was the fi rst to suggest a
combined abdominoperineal approach. He chose
to start the operation through the perineum, and if
the rectum was not found, to open the abdomen
through a midline incision. With a fi nger, as a
guide from inside the abdomen, the perineal incision should be created to reach the peritoneal cavity and the rectum pulled through [
27 ].
In 1887 Vincent of Lyons performed a parasacral incision instead of a mid-sacral one. This
was described by Maitre [ 28 ].
In 1894, Paul Delageniere suggested performing a lateral laparotomy to fi nd the rectum and to
reach the perineum through the abdominal cavity,
using his fi nger as a guide and then pulling
through the rectum [ 29 ].
In 1897, Rudolph Matas [ 30 ], a brilliant sur-
geon in New Orleans, mentioned that cutting,
dividing, or destroying the sacrum had a negative
effect because it damaged the muscle insertions
as well as the innervation and blood supply of the
pelvic structures. He suggested entering the pelvis through the third sacral foramen. He supported the idea of opening a colostomy. He also
believed that the rectal ampulla could move down
spontaneously; therefore, he proposed to open a
colostomy and wait. In 1897, Matas wrote 22
conclusions related to the management of anorectal malformations. Some of which are still
valid:
1. “The most common types of anorectal malformations can be repaired through a perineal approach.” Interestingly, this conclusion
is quite accurate.
2. “There are no external signs to determine the
internal anatomic malformations.” This conclusion is partially valid since now we know
that we can learn a lot just by careful inspection of the perineum.
3. “One should not depend on the introduction of
guides through the vagina or the urinary tract to
determine the presence or absence of intestine.
The use of a needle to aspirate meconium is
also dangerous because of the risk of peritoneal
contamination.” This is still true.
4. “The operation should be done as early as
possible to avoid death consecutive to the
passing of stool to the blood, peritonitis,
intestinal obstruction, absorption of toxins,
and migration of bacteria from the intestines.” Although now we are aware of many
new, sophisticated pathophysiologic mechanisms, this concept is still valid.
5. “The tolerance of the baby to the trauma is
inversely proportional to the age in days after
birth. And in addition, the baby without sepsis is as tolerant to trauma as the adult.”
Again, he was right.
6. “The ideal result in this kind of operation is
the restoration of the passage of stool, creating an anus in a normal position with bowel
control.” This, of course, is still valid.
7. “The only way to obtain this kind of result is
performing a proctoplasty as proposed by
Amussat.” Obviously, this is mostly wrong.
8. “In order to obtain the best possible results
from the functional point of view, the operator must avoid the unnecessary injury of the
sphincter mechanism, for that, the incision
1.2 The Early Times
5
must be performed strictly in the midline.”
He was right!
9. “The old method of stab of the perineum
without a proctoplasty was not justifi ed.” He
was right.
10. “The initial peritoneal exploration of the pelvis through a perineal-sacral aperture was
one of the greatest advances in the treatment
of these conditions.” Of course, that is no
longer true.
11. “The peritoneal exploration through the
perineum must be attempted systematically
when the rectum is not found through the
perineum.” This is no longer valid.
12. “Those techniques that use a sacral resection
or excision or osteoplasty to increase the
exposure and to reach the peritoneum looking for the rectum are valid.” Obviously, we
do not use that anymore.
13. “The best approach is a midline incision
through the coccyx and sacrum.” This is
mostly true.
14. “A predisposition to suffer prolapse must be
expected in cases of resection of the sacrum.”
Obviously, we do not touch the sacrum
anymore.
15. “A primary exploratory laparotomy is not
indicated as a rule.” This is true.
16. “The great majority of imperforate anus can
be treated successfully through the
perineum.” That is true.
17. “The perineal anus can be created pulling the
colon and connecting it to the perineum. But,
in cases of emergency, one can connect the
small bowel to the perineum.” Obviously, we
do not do that.
18. “The mortality from a colostomy is greater
than the anoplasty and perineal-sacral
approach.” This is obviously wrong by modern standards.
19. “Primary colostomy in the groin, as a primary procedure, is only indicated when the
baby is extremely sick. Under all of the other
circumstances, the perineal incision must be
the fi rst one.” This statement is partially true.
20. “An exploratory laparotomy is only performed after the rectum was not found
through the perineum.” This statement is
also partially true.
21. “If, for some reason, the surgeon decided to
open a colostomy fi rst, he should always
make every effort in a second procedure to
open the anus in the perineum.” This is
mostly true.
22. “The perineal-sacral anus, when it is correctly done, is almost certain to have bowel
control as time goes by.” That is, of course,
mostly not true.
In 1899 and published in 1908, Mastin demonstrated that a permanent colostomy was compatible with growth and development [ 31 ]. He
operated on a newborn baby and created a
colostomy, and when he offered the family the
opening of an anus, the family refused to have
that operation done because the patient was
doing very well and has adapted to the presence
of the stoma, playing sports and growing and
developing normally. In 1903, Mastin was
called to take care of another case. He performed a perineal midline incision. He was able
to fi nd the bowel and perform an anoplasty that
he sutured to the skin with catgut. He described
that 4 years later, the patient had bowel
control.
In 1915, Brenner [ 32 ] published an excel-
lent paper in Surgical Gynecology and
Obstetrics and described his experience with
61 cases. He described different degrees of
development of the external sphincter. He suggested that the operations to repair imperforate
anus should “last no more than 5–8 min!!” He
performed a posterior incision, and he suggested opening a colostomy if the perineal
approach was unsuccessful. He described that
in males, the rectum opens more often into the
bladder rather than the urethra, which is not
true, since now we have well- documented evidence that the connection between the rectum
and the bladder only occurs in 10 % of the
male cases. He suggested that if the patient did
not have external sphincter fi bers, bowel control must be obtained by an axial rotation of the
gut or using some muscle fi bers from the
gluteal region. Even though Brenner’s
conclusions are not valid at the present time,
his work is very signifi cant, because of the
number of cases and the meticulous description of them.