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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 [1]. Standard treatment includes singleor multi-stage pull-through surgery [2]. We have previously reported per-rectal endoscopic myotomy (PREM) for adult Hirschsprung’s disease [3]. 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) [4, 5]. 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. 1). 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. 2). 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. 1 Barium enema in an 8-year-old boy showing the characteristic signs of Hirschsprung’s disease. ▶ Fig. 2 Biopsy taken from the rectum at 8 cm (magnification×40), stained immunohistochemically with S100, showing a nerve bundle and the absence of ganglion cells.

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