LONG-TERM MORBIDITY IN TOTAL COLONIC AGANGLIONOSIS

Background/Purpose: No surgical treatment for total colonic agangliono sis (TCA) clearly has been proven superior. To identify clinical crite ria associated with long-term functional outcome, a 28-year retrospect ive institutional review of this entity from 1969 through 1996 inclusi ve was undertaken. Methods: Total colonic aganglionosis (TCA), defined here as aganglionosis extending from the anus to at least the ileocec al valve but no further than 50 cm proximal to the ileocecal valve, wa s identified in 29 infants and children. Appropriate leveling ileostom y was performed in 28 of 29 patients, and definitive surgical reconstr uction was performed in 26 of 29. Three groups were identified based o n the definitive surgical repair performed: group 1, construction lack ing or incorporat ing a short ganglionic-aganglionic common channel (m odified Soave or modified Duhamel, n = 8); group II, construction of a n extended common channel (Martin-Duhamel, Martin-Soave; n = 6); and g roup III, all others including an intermediate-length common channel ( n = 13). Results: Functional outcome at extended follow-up (mean, 6.6 +/- 5.6 years; range, 0.7 to 23) was determined based on survival, lon g-term ostomy requirements, growth, major complications, continence, a nd enterocolitis and bowel movement frequency. Although long-term func tional outcome was deemed satisfactory in six of seven patients in gro up I, function was satisfactory in none of six group II patients. Grou p III results were intermediate (satisfactory in 6 of 13). Conclusions : Acceptable long-term outcome was most frequent in TCA patients whose definitive repair did not incorporate an extended ganglionic-aganglio nic common channel. The use of extensive lengths of aganglionic bowel to maximize fluid absorption is frequently met with substantial morbid ity. J Pediatr Surg 33:961-966. Copyright (C) 1998 by W.B. Saunders Co mpany.