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

Abstract

'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 single-or 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.

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

“…Sigmoidoscopyguided serial deep muco-submucosal biopsies were obtained every 4-5 cm using the cap-assisted endoscopic mucosal resection technique from the dilated segment up to 2 cm above the dentate line ( ▶ Fig. 2) [10,12]. The presence of ganglia in proximal biopsies, transition-zone hypoganglionosis, and absent ganglia distally are classical signs of HSCR [13][14][15][16].…”

“…Sigmoidoscopy guided serial deep muco-submucosal biopsies were obtained every 4 to 5cm using cap assisted endoscopic mucosal resection (EMR-C) technique from dilated segment up to 2cm above dentate line ( Figure 2). [10,12] Presence of ganglia in proximal biopsies, transition zone hypoganglionosis and absent ganglia distally is classical of HSCR. [13][14][15][16] Aganglionic segment was thus measured and optimum myotomy length was tailored [9,10] High-definition gastroscope (GIF-HQ190, Olympus) with a distal transparent attachment (Olympus) was used.…”

“…Third-space endoscopy permits performance of longer or tailor-made myotomy, as is evident from peroral endoscopic myotomy (POEM) for achalasia cardia and G-POEM for gastroparesis. Based on this philosophy, per-rectal endoscopic myotomy (PREM) was conceptualized, successfully performed, and reported by the authors' group in the form of two case reports and one conference abstract on adults followed by pediatric and infantile SS-HSCR patients [9][10][11]. The current case series reports a single-center experience of PREM for SS-HSCR patients with medium-term to long-term follow-up.…”

“…Based on this philosophy, per rectal endoscopic myotomy (PREM) was conceptualized, successfully performed and reported by the authors' group as two case reports and one conference abstract of adult followed by pediatric and infantile SS-HSCR patients. [9][10][11] The current case series reports a single-center experience of PREM for SS-HSCR patients with medium to long-term follow up.…”

“An Odyssey of Third Space Endoscopy”—from Entry into the Tunnel to Entry into the Peritoneal Cavity

“…Although exciting, only 2 case reports are available. 7,34 The lack of enthusiasm may be due to the risk of injuring the external anal sphincter leading to incontinence.…”