Per-rectal endoscopic myotomy (PREM) for pediatric Hirschsprung’s disease

Abstract

Per-rectal endoscopic myotomy (PREM) for pediatric Hirschsprung's disease Hirschsprung's disease is a congenital disorder characterized by the absence of intrinsic ganglion cells in the myenteric plexuses of the hindgut. The majority of patients present during infancy or childhood with constipation, intestinal obstruction, or megacolon Standard treatment includes single-or multi-stage pull-through surgery We have previously reported per-rectal endoscopic myotomy (PREM) for adult Hirschsprung's disease The rationale of this is to disrupt the spastic bowel segments, like those in achalasia or pylorospasm are disrupted by peroral endoscopic myotomy (POEM) or pyloromyotomy (G-POEM) This report describes the technique and outcome of PREM in a pediatric patient with Hirschsprung's disease. An 8-year-old boy had had refractory constipation since the neonatal period that was partially relieved by high-dose laxatives. Barium enema demonstrated a spastic rectum and dilated sigmoid colon, which were classical of Hirschsprung's disease ( Fig. Colonoscopy showed a spastic non-distensible empty rectum and dilated sigmoid colon with stool residue. Deep mucosal biopsies demonstrated ganglion cells at 8 cm but these were absent distally ( Fig. Manometry demonstrated absent rectoanal inhibitory reflex (RAIR). Given our previous experience, we obtained consent for PREM from the patient's parents. PREM was performed Fig.