Pediatric surgery international · 2024
Total colonic aganglionosis: management and long-term outcomes at a referral centre.
СтатьяНазвание и аннотация: Перевод в очереди
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Hegde Shalini Gajanan, Raju Rajkiran Srinivas, Shubha Attibele Mahadevaiah, Mahadevappa Kiran, Kumar Prasanna, Deb Mainak, Mohanty Suravi, Das Kanishka
Аннотация
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.
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.