Total colonic aganglionosis: management and long-term outcomes at a referral centre

ObjectivesThe management of total colonic aganglionosis (TCA) in low-middle income countries (LMIC) is challenging. We aimed to analyze the profile, management, and outcome of patients with TCA at an Indian referral centre.MethodsA retrospective review of demography, presentation, investigations, and treatment of patients with TCA at a single centre over 22 years with a standardized protocol is detailed. Long-term outcome was assessed with qualitative evaluation and bowel function score.ResultsTwenty-two patients were reviewed. Although neonatal intestinal obstruction was common, 36% had chronic constipation. Delayed meconium history was evident in 54% and a classical microcolon in 28%. 71% of contrast enemas were incongruent with the final histological diagnosis. The median time on a levelled stoma was 13 months. 45.5% had 15 interim surgical interventions, including 8 relevelling (36%) before the definitive surgery at a median age of 19.5 months. At a mean follow-up age of 10.3 years, eleven had a bowel function score of >= 17 and five others had a favorable qualitative outcome.ConclusionThe diagnosis, histological levelling and management of TCA continues to be challenging in LMIC. The morbidity and long-term outcomes with the described protocol were comparable to studies from developed nations.