Initial experience with per-rectal endoscopic myotomy for Hirschsprung’s disease: medium and long term outcomes of the first case series of a novel third-space endoscopy procedure
Amol Bapaye, Parag Dashatwar, Vishnu Biradar, Shital Biradar, Rajendra Pujari
Abstract
Abstract Introduction Hirschsprung’s disease (HSCR) is congenital aganglionosis affecting the hindgut and presents with constipation. Surgical pull-through is the current standard treatment but causes morbidity. Per-rectal endoscopic myotomy is a novel third-space endoscopy technique for treating short-segment (SS)-HSCR. Methods Retrospective study of SS-HSCR patients diagnosed on history, contrast enema, rectal biopsies, and anorectal manometry, and treated by PREM. The aganglionic segment was mapped before PREM was performed using third-space endoscopy principles. Stool frequency and laxative usage before and after PREM were compared. Results Nine patients (age 7.5 [± 5.2] years; 7 male) underwent PREM during a 4-year period. Mean aganglionic segment length was 6.3 cm, mean procedure time 96.1 minutes, and mean length of hospital stay 2.5 days. Median follow-up was 17 months (range 9–58 months). Stool frequency was 1/4.4 days before vs. 1/1.2 days after PREM (P = 0.0004). Mean laxative usage was 5.4 units of laxative (UL) before vs. 0.4 UL after PREM (P = 0.0002). No laxatives were used by 6/9 patients after PREM. The single adverse effect seen (anal stenosis) was treated with dilatation. Conclusions PREM is a safe and effective minimally invasive procedure to treat SS-HSCR and results in long-term response.