Per rectal endoscopic myotomy (PREM) for the treatment of adult Hirschsprung's disease - First human case (with video).

Per rectal endoscopic myotomy (PREM) for the treatment of adult Hirschsprung's disease - First human case (with video).

Dig Endosc. 2016 Jun 15;

Authors: Bapaye A, Wagholikar G, Jog S, Kothurkar A, Purandare S, Dubale N, Pujari R, Mahadik M, Vyas V, Bapaye J

Abstract
Hirschsprung's disease (HD) is a congenital disorder characterized by absence of intrinsic ganglion cells in submucosal (SM) and myenteric plexuses of the hindgut; and presents with constipation, intestinal obstruction and / or megacolon. HD commonly involves the rectosigmoid region (short segment HD) although shorter and longer variants of the disease are described. Standard treatment involves pull-through surgery for short segment HD or posterior anorectal myotomy (PARM) in selected ultra-short segment candidates. Third space endoscopy has evolved during the last few years. Peroral endoscopic myotomy (POEM) and peroral pyloromyotomy (G-POEM or POEP) are described for treatment of achalasia cardia and refractory gastroparesis respectively. Using the same philosophy of muscle / sphincter disruption for spastic bowel segments, per-rectal endoscopic myotomy (PREM) could be considered as a treatment option for short segment HD. A 24-years male patient presented with refractory constipation since childhood and habituated to high dose laxative combinations. Diagnosis was confirmed as adult short segment HD by barium enema, colonoscopic deep suction mucosal biopsies and anorectal manometry (HRM). Histopathology confirmed aganglionosis in the distal 15 cm. By implementing principles of third space endoscopy, per-rectal endoscopic myotomy (PREM) 20 cm in length was successfully performed. At twenty-four weeks follow up; patient reports significant relief of constipation and associated symptoms. Sigmoidoscopy, HRM and barium enema confirm improved rectal distensibility and reduced rectal pressures. This case report describes the first human experience of per-rectal endoscopic myotomy (PREM) for successful treatment of adult short segment HD.

PMID: 27307403 [PubMed - as supplied by publisher]

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