Long-term functional outcomes and multidisciplinary management after Duhamel pull-through for total colonic aganglionosis- 20-year experience in a tertiary surgical centre

Abstract

Purpose Management of patients with total colonic aganglionosis (TCA) is challenging for paediatric surgeons. The purpose of this study was to review our institution’s 20-year experience regarding long-term outcomes in these patients. Methods Retrospective review was conducted for patients diagnosed with TCA. Data was collected on demographics, clinical presentation, complications, need for additional surgery and long-term effects on bowel function. Results Out of 202 patients with Hirschsprung’s disease (HSCR), 13 were diagnosed with TCA (6.4 %). Clinical presentation was variable - 11 presented in neonatal period and 2 in infancy. Ileorectal Duhamel pull-through was performed in all patients. Median follow up was 13 years. 11 are toilet trained, of whom 5 are fully continent. 6 continue to have problems with bowel continence or constipation. 1 developed recurrent episode of Hirschsprung’s associated enterocolitis (HAEC). 2 patients had stoma re-established. Patients experiencing difficulties in bowel function are jointly managed by a multidisciplinary team consisting of surgeons, gastroenterologists, paediatric psychologists, and clinical nurse specialists. Conclusions TCA can be associated with significant long-term morbidity. Nearly half of the patients have ongoing problems with bowel continence requiring a permanent stoma in some. Diligent follow-up coupled with multidisciplinary team input has helped manage these patients in our institution.