A population-based, complete follow-up of 146 consecutive patients after transanal mucosectomy for Hirschsprung disease

Abstract

Objective: The objective of the study is to define the population-based bowel functional outcomes and enterocolitis following transanal endorectal pull-through (TEPT) in patients with Hirschsprung disease (HD) treated at our institution between 1986 and 2011.

Methods: 146 consecutive patients who had undergone primary surgical treatment for HD were included. The median follow-up time was 15 (3-33) years. The clinical details and prevalence of enterocolitis were evaluated in all patients, and bowel function in patients >3 years of age with functional intestino-anal continuity.

Results: No patients were lost to follow-up. Overall survival was 98%. The level of disease was rectosigmoid in 83%, long segment in 7%, total colonic in 4%, and extending up to the small bowel in 6%. 29% had an associated syndrome. 22% had a preoperative stoma. Operations included TEPT (89%), proctocolectomy with ileoanal anastomosis in 9%, and 3% had a permanent endostomy owing to small intestinal aganglionosis. One patient underwent intestinal transplantation. At the latest follow-up, 42% had occasional soiling, 12% had frequent soiling and 46% had no soiling. Constipation occurred in 9%. An associated syndrome was the only predictor for soiling or constipation (OR 4.3, 95% CI 1.5-12). 44% developed recurrent postoperative enterocolitis, which was predicted by extended aganglionosis (OR 6.9, 95% CI 2.4-20) and syndromatic disease (OR 2.4, 95% CI 1.2-5.0).

Conclusion: The major functional sequelae following TEPT were recurrent enterocolitis and fecal soiling, which was mostly occasional. An associated syndrome was a predictor of a reduced bowel functional outcome, and alongside extended aganglionosis were significant risk factors for recurrent postoperative enterocolitis.

Keywords: Continence; Enterocolitis; Hirschsprung disease; Long-term outcome; Transanal surgery.