Long-term Bowel function and pediatric health-related quality of life after transanal rectal mucosectomy and partial internal anal sphincterectomy pull-through for Hirschsprung Disease.

ABSTRACT

Objective: The aim of this study was to define controlled outcomes for bowel function and quality of life (QoL) after transanal rectal mucosectomy and partial internal anal sphincterectomy pull-through (TRM-PIAS, A modified Swenson procedure) for Hirschsprung disease (HD). Background: We have previously shown that a novel modification of transanal rectal mucosectomy and partial internal anal sphincterectomy (TRM-PIAS, A modified procedure) for Hirschsprung disease have the advantage of low postoperative Hirschsprung associated enterocolitis. The controlled long-term follow-up studies evaluating Bowel Function Score (BFS) and Pediatric Quality of Life Inventory (PedsQoL, age <18 years) remain unclear. Methods: Between Jan 2006 and Jan 2016, 243 Patients underwent TRM-PIAS older than 4 years were included, while experienced redo surgery because of complication were excluded. Patients were compared with age- and gender-matched 244 healthy children each randomly selected from the 405 general population. The enrollee was investigated for questionnaires on BFS and PedsQoL. Results: One hundred and ninety-nine (81.9%) patients' representatives for the entire study population responded. The mean age of patients was 84.4 months (48-214 months). Compared with controls, patients reported impairment of hold back defecation, fecal soiling, and the urge to defecate (P < 0.05), and no significantly different in fecal accidents, constipation and social problems. With advancing age, the total BFS of HD patients improved, with a tendency close to the normal level beyond 10 years old. But, after grouped according to presence or absence of HAEC, the non-HAEC group experienced more dramatic improvement with age increasing. Conclusions: Compared with matched peers, significant impairment of fecal control prevails after TRM-PIAS in HD patients, but bowel function improve with age and recovery faster than conventional procedure. It should be emphasized that post-enterocolitis is a high-risk factor for delayed recovery.