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

Abstract

Introduction Hirschsprung’s disease (HSCR) is congenital aganglionosis affecting the hindgut and presents with constipation. Surgical pull-through is the current standard treatment but causes morbidity. Per-rectal endoscopic myotomy is a novel third-space endoscopy technique for treating short-segment (SS)-HSCR.

Methods Retrospective study of SS-HSCR patients diagnosed on history, contrast enema, rectal biopsies, and anorectal manometry, and treated by PREM. The aganglionic segment was mapped before PREM was performed using third-space endoscopy principles. Stool frequency and laxative usage before and after PREM were compared.

Results Nine patients (age 7.5 [± 5.2] years; 7 male) underwent PREM during a 4-year period. Mean aganglionic segment length was 6.3 cm, mean procedure time 96.1 minutes, and mean length of hospital stay 2.5 days. Median follow-up was 17 months (range 9–58 months). Stool frequency was 1/4.4 days before vs. 1/1.2 days after PREM (P = 0.0004). Mean laxative usage was 5.4 units of laxative (UL) before vs. 0.4 UL after PREM (P = 0.0002). No laxatives were used by 6/9 patients after PREM. The single adverse effect seen (anal stenosis) was treated with dilatation.

Conclusions PREM is a safe and effective minimally invasive procedure to treat SS-HSCR and results in long-term response.

Publication History

Received: 03 October 2020

Accepted after revision: 08 December 2020

Accepted Manuscript online:
08 December 2020

Article published online:
02 March 2021

• References

• 1 Wyllie R. Motility disorders and Hirschsprung disease. In: Kliegman R, Behrman R, Jenson H, Stanton B, eds. Nelson textbook of pediatrics. 18th edn. Philadelphia, PA: Saunders Elsevier 2008: 1565-1567
• 2 Ikeda K, Goto S. Diagnosis and treatment of Hirschsprung’s disease in Japan. An analysis of 1628 patients. Ann Surg 1984; 199: 400-405
• 3 Bai Y, Chen H, Hao J. et al. Long term outcome and quality of life after Swenson procedure for Hirschsprung’s disease. J Pediatr Surg 2002; 37: 639-642
• 4 Pini PratoA, Gentilino V, Giunta C. et al. Hirschsprung’s disease: 13 years’ experience in 112 patients from a single institution. Pediatr Surg Int 2008; 24: 175-182
• 5 Rentea RM, Snyder CL. Early and late complications following pull-through operation for Hirschsprung’s disease. In: Puri P, ed. Hirschsprung’s disease and allied disorders. 4th edn. Springer International 2019: 383-401
• 6 Lynn HB, van Heerden JA. Rectal myectomy in Hirschsprung disease: a decade of experience. Arch Surg 1975; 110: 991-994
• 7 Sawin R, Hatch E, Schaller R. et al. Limited surgery for lower-segment Hirschsprung’s disease. Arch Surg 1994; 129: 920-924
• 8 Thomas Jr. CG, Bream CA, DeConnick P. Posterior sphincterotomy and rectal myotomy in the management of Hirschsprung’s disease. Ann Surg 1970; 171: 796-810
• 9 Bapaye A, Wagholikar G, Jog S. et al. Per rectal endoscopic myotomy for the treatment of adult Hirschsprung’s disease: first human case (with video). Dig Endosc 2016; 28: 680-684
• 10 Bapaye A, Bharadwaj T, Mahadik M. et al. Per-rectal endoscopic myotomy (PREM) for pediatric Hirschsprung’s disease. Endoscopy 2018; 50: E644-E645
• 11 Bapaye A, Mahadik M, Kumar Korrapati S. et al. Per rectal endoscopic myotomy (PREM) for infantile Hirschsprung’s disease. Endoscopy 2018; 50: OP209V
• 12 Nabi Z, Chavan R, Shava U. et al. A novel endoscopic technique to obtain rectal biopsy specimens in children with suspected Hirschsprung’s disease. VideoGIE 2018; 3: 157-158
• 13 Knowles CH, De Giorgio R, Kapur RP. et al. The London classification of gastrointestinal neuromuscular pathology: report on behalf of the Gastro 2009 International Working Group. Gut 2010; 59: 882-887
• 14 Subramanian H, Badhe BA, Toi PC. et al. Morphometric profile of large intestinal neuronal plexuses in normal perinatal autopsies and Hirschsprung disease. Neurogastroenterol Motil 2017; 29: e12939
• 15 Kapur RP. Intestinal motor disorders. In: Russo P, Ruchelli E, Piccoli D. Pathology of pediatric gastrointestinal and liver disease. 2nd edn. Berlin, Heidelberg: Springer; 2014: 255-270
• 16 Qualman SJ, Jaffe R, Bove KE. et al. Diagnosis of Hirschsprung disease using the rectal biopsy: multi-institutional survey. Pediatr Dev Pathol 1999; 2: 588-596
• 17 Cotton PB, Eisen GM, Aabakken L. et al. A lexicon for endoscopic adverse events: report of an ASGE workshop. Gastrointest Endosc 2010; 71: 446-454
• 18 Wang L, Cai Q, Fan C. et al. Mo1628 A new potential method per anus endoscopic myotomy for treatment of internal anal sphincter achalasia. Gastrointest Endosc 2013; 77: AB451
• 19 Bandres DJ, Prada CE, Soto JR. et al. Tu1690 Per rectal endoscopic myotomy for Hirshprung’s disease in rescue of unsuccessful previous surgical myomectomy. Gastroenterology 2019; 156: S1501