Introduction
Hirschsprung’s disease (HSCR) is congenital aganglionosis affecting hindgut and presents with constipation. Surgical pull-through is current standard but has morbidity. Per-rectal endoscopic myotomy (PREM) is a novel third space endoscopy technique for treating SS-HSCR.
Methods
Retrospective series of SS-HSCR patients diagnosed on history, contrast enema, rectal biopsies, anorectal manometry and treated by PREM. Aganglionic segment mapped pre-PREM. PREM performed using third space endoscopy principles. Objective – to compare stool frequency and unit laxative (UL) usage pre and post-PREM.
Results
N = 9; duration 4-years. Mean age – 7.5 (± 5.2) years; 7 males. Mean aganglionic segment length – 6.3 (± 4.4) cm. Mean procedure time – 96.1 (±37.9) minutes. Mean LOS – 2.5 (±0.7) days. Median follow-up –17 months (9 – 58). Stool frequency – pre – 1/4.4 (±1.5) vs. post – 1/1.2 (±0.4) days (p = 0.0004). Mean UL usage – pre – 5.4 (±4.9) vs. post – 0.4 (±0.7) units (p = 0.0002). No laxatives in 6 (66%). Single AE (anal stenosis) – dilatation.
Conclusions
PREM is a safe and effective minimally invasive procedure for SS-HSCR and provides long-term response.
Per-rectal endoscopic myotomy (PREM) for pediatric Hirschsprung’s disease
