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

OBJECTIVES

The 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.

METHODS

A 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.

RESULTS

Twenty-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.

CONCLUSION

The 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.