Total colonic aganglionosis: management and long-term outcomes at a referral centre
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In brief
In brief
This 22-year retrospective study examines management of total colonic aganglionosis in an Indian referral center, highlighting diagnostic challenges including incongruent contrast enemas and need for multiple stoma revisions. Despite resource constraints, long-term bowel function outcomes were comparable to developed nations.
- Contrast enema is unreliable for TCA diagnosis in LMIC settings—71% were incongruent with final histological findings.
- Multiple interim surgeries are common before definitive repair—45% required stoma relevelling, median 8 procedures per patient.
- Long-term outcomes in LMIC can match developed nations—73% achieved favorable bowel function at mean 10-year follow-up.
- TCA presentation varies beyond neonatal obstruction—36% presented with chronic constipation rather than acute symptoms.
- Prolonged stoma duration is typical—median 13 months before definitive pull-through surgery at 19.5 months of age.
Written by the GCMD Library team from the article.
Abstract
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.