Abstract
Thirty-two patients with total colonic aganglionosis (TCA) were seen in 30 years, representing 10% of the total number with aganglionosis seen during that period (1954–1983). The mortality rate was 45%, but has shown a progressive decrease from 82% in the first decade to 9% in the last. The improvement is specifically related to earlier diagnosis, control of sepsis, and the use of parenteral nutrition in dealing with ileostomy losses, but appears to have been independent of the definitive surgical procedure. Follow-up study indicates some delay in achieving continence without any clear-cut relationship to the operative procedure. The 32 patients have been reviewed with particular emphasis on sex ratio, age at diagnosis, clinical features, and results of treatment.
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References
Asch MJ, Weitzman JJ, Hay DM, Brennan LO (1972) Total colonic aganglionosis. Arch Surg 105: 74–78
Bodian M, Carter CO, Ward BCH (1951) Hirschsprung's disease. Lancet 1: 302–309
Boley SJ (1984) A new operative approach to total aganglionosis of the colon. Surg Gynecol Obstet 159: 481–486
Brennen LO, Weitzman JJ, Swenson O (1967) Pitfalls in the management of Hirschsprung's disease. J Pediatr Surg 2: 1–12
Burrington JD, Wayne ER (1976) Modified Duhamel procedure for treatment of total aganglionic colon in childhood. J Pediatr Surg 11: 391–398
Careskey JM, Weber TM, Grosfeld JL (1982) Total colonic aganglionosis: analysis of 16 cases. Am J Surg 143: 160–168
Carter CO, Evans K, Hickman V (1981) Children of those treated surgically for Hirschsprung's disease. J Med Genet 18: 87–90
Cohen IT, Gadd MA (1982) Hirschsprung's disease in a kindred: a possible clue to the genetics of the disease. J Pediatr Surg 17: 632–634
Coran AG, Bjordal R, Eek S, Knutrud O (1971) The surgical management of toal colonic aganglionosis and partial intestinal aganglionosis. J Pediatr Surg 4: 531–537
Davies MRQ, Cywes S, Rode H (1981) The manometric evaluation of the rectosphincteric reflex in total colonic aganglionosis. J Pediatr Surg 16: 660–663
De Campo JF, Mayne V, Boldt DW, De Campo M (1984) Radiological findings in total aganglionosis coli. Pediatr Radiol 14: 205–209
Dickinson VA (1978) Maintenance of anal continence: a review of pelvic floor physiology. Gut 19: 1163–1174
Frech RS (1968) Aganglionosis involving the entire colon and a variable length of small bowel. Radiology 90: 247–257
Freeman NV (1971) Long segment Hirschsprung's disease. Proc Roy Soc Med 64: 30–32
Gaisie G, Kook Sang OH, Young LW (1979) Co-existent neuroblastoma with Hirschsprung's disease — another manifestation of the neurocristopathy. Pediatr Radiol 8: 161–163
Gastrin U, Josephson S (1970) Appendiceal peritonitis and megacolon in the neonatal period. Acta Chir Scand 136: 153–156
Gerald B (1965) Aganglionosis of the colon and terminal ileum. Long-term survival. Am J Radiol 95: 230–234
Hirst GDS (1979) Mechanics of Peristalsis. Br Med Bull 35: 263–268
Ikeda K, Goto S (1984) Diagnosis and treatment of Hirschsprung's disease in Japan. Ann Surg 199: 400–405
Kimura K, Nishijma E, Muraji T, Tsugawa C, Matsumoto Y (1981) A new surgical approach to extensive aganglionosis. J Pediatr Surg 16: 840–843
Kirby ML, Bockman DE (1984) Neural crest and normal development: a new perspective. Anat Rec 209: 1–6
Kleinhaus S, Boley SJ, Sheran M, Sieber WK (1979) Hirschsprung's disease: a survey of members of the surgical section of the american academy of pediatrics. J Pediatr Surg 14: 588–597
Louw JH (1971) Total colonic aganglionisis. Can J Surg 21: 397–405
Martin LW (1972) Surgical management of total colonic aganglionosis. Ann Surg 176: 343–346
Martin LW (1982) Total colonic aganglionosis: preservation and utilization of the entire colon. J Paediatr Surg 13: 635–637
McCredie J (1974) Embryonic neuropathy: a hypothesis of neural crest injury as the pathogenesis of congenital malformations. Med J Aust 1: 159–163
Moore TC, Landers DB, Lachman RS, Ament ME (1979) Hirschsprung's disease in discordant in monozygotic twins: a study of possible environmental factors in production of colonic aganglionosis. J Pediatr Surg 14: 158–161
Myers MB, Bradburn D, Vella R, Payzant A, Karlin S (1966) Total aganglionic colon in an adult: first reported case. Ann Surg 163: 97–102
Narasimharao KL, Pathak IC, Mitra SK (1983) A new approach to total colonic aganglionosis. Aust NZ J Surg 53: 557–559
Ott WR, Joppich I (1981) Late postoperative complications in Hirschsprung's disease. Kinderchir 32: 115–120
Passage E (1967) The genetics of Hirschsprung's disease. Evidence for heterogenous etiology and a study of 63 families. N Engl J Med 276: 138–143
Porter L, Weeks A (1915) Megacolon and microcolon. Am J Dis Child 9: 283–299
Shandling B (1984) Total colon aganglionosis — a new operation. J Pediatr Surg 19: 503–505
Sandegard E (1953) Hirschsprung's disease with ganglion cell aplasia of the colon and terminal ileum. Acta Chir Scand 106: 68–75
So HB, Schwartz DL, Becker JM, Daum F, Schnieder KM (1980) Endorectal “pull-through” without preliminary colostomy in neonates with Hirschsprung's disease. J Pediatr Surg 15: 470–471
Soltero-Harrington LR, Garcia-Rinaldi R, Able LW (1979) Total aganglionosis of the colon: recognition and management. J Pediatr Surg 4: 330–338
Stone WD, Thomas BM, Hendrix MD, Schuster MM (1965) Aganglionosis of the enteric colon in an adolescent. Gastroenterol 48: 636–641
Swenson O, Sherman JO, Fisher JH (1973) Diagnosis of congenital megacolon: an analysis of 501 patients. J Pediatr Surg 7: 587–594
Swenson O, Sherman JO, Fisher JH, Cohen E (1975) The treatment and post-operative complications of congenital megacolon. A 25-year follow up. Ann Surg 182: 266–273
Walker AW, Kemson RL, Ternberg JL (1966) Aganglionosis of the small intestine. J Pediatr Surg 60: 449–457
Webster W (1973) Embryogenesis of the enteric ganglion in normal mice and in mice that develop congenital aganglionic megacolon. J Embryol Exp Morphol 30: 573–585
Zuelzer WW, Wilson JOL (1948) Functional intestinal obstruction on a congenital neurological basis in infancy. Am J Dis Child 75: 46–64
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Cass, D.T., Myers, N. Total colonic aganglionosis: 30 years' experience. Pediatr Surg Int 2, 68–75 (1987). https://doi.org/10.1007/BF00174176
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DOI: https://doi.org/10.1007/BF00174176