Background/purpose: No surgical treatment for total colonic aganglionosis (TCA) clearly has been proven superior. To identify clinical criteria associated with long-term functional outcome, a 28-year retrospective institutional review of this entity from 1969 through 1996 inclusive was undertaken.
Methods: Total colonic aganglionosis (TCA), defined here as aganglionosis extending from the anus to at least the ileocecal valve but no further than 50 cm proximal to the ileocecal valve, was identified in 29 infants and children. Appropriate leveling ileostomy was performed in 28 of 29 patients, and definitive surgical reconstruction was performed in 26 of 29. Three groups were identified based on the definitive surgical repair performed: group 1, construction lacking or incorporating a short ganglionic-aganglionic common channel (modified Soave or modified Duhamel, n = 8); group II, construction of an extended common channel (Martin-Duhamel, Martin-Soave; n = 6); and group 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, long-term ostomy requirements, growth, major complications, continence, and enterocolitis and bowel movement frequency. Although long-term functional outcome was deemed satisfactory in six of seven patients in group I, function was satisfactory in none of six group II patients. Group 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-aganglionic common channel. The use of extensive lengths of aganglionic bowel to maximize fluid absorption is frequently met with substantial morbidity.