Letter to the editor re initial experience with per-rectal endoscopic myotomy for Hirschsprungʼs disease

We read with great interest the article by Bapaye et al. who described per-rectal endoscopic myotomy as a treatment for Hirschsprungʼs disease (HSCR) [1]. We congratulate the authors on their novel technique but have concerns regarding patient selection, outcome assessment, and the long-term results reported in this study.

In this series, nine patients underwent this procedure at a median age of 7.5 years. In the modern era, HSCR should be surgically treated during infancy. It is extremely unfavorable to undergo a late operation because of the poor functional outcome [2]. The authors have not provided comprehensive clinical details, without which, the diagnosis of HSCR could be in doubt.

Although myotomy could relieve the high sphincter pressure, as in the case of esophageal achalasia, the pathology in HSCR is entirely different owing to the absence of enteric ganglion cells. We therefore question whether the rectocolonic dysmotility could really be improved after myotomy.

We are particularly concerned about the long-term outcomes reported by the authors. The median follow-up period was only 17 months and we do not agree that this can be considered as “medium- and long-term outcomes” as stated in the title. In most medium- and long-term studies, the follow-up period should be at least 5 years [3]. Moreover, the outcome measurements were oversimplified. The authors assessed the outcomes by reporting the frequency of bowel motions and usage of laxatives, but a more robust assessment should have been done using one of the international validated bowel function scores [4]. Lastly, the incidence of Hirschsprungʼs disease-associated enterocolitis, one of the most important complications, was not mentioned in the entire study [5].

We applaud the authors for their attempt to report a novel procedure, yet we have concerns and reservations regarding the applicability of this procedure for HSCR.

Publication History

Article published online:
21 April 2022

• References

• 1 Bapaye A, Dashatwar P, Biradar V. et al. Initial experience with per-rectal endoscopic myotomy for Hirschsprungʼs disease: medium and long term outcomes of the first case series of a novel third-space endoscopy procedure. Endoscopy 2021; 53: 1256-1260
• 2 Onishi S, Kaji T, Nakame K. et al. Optimal timing of definitive surgery for Hirschsprung’s disease to achieve better long-term bowel function. Surg Today 2022; 52: 92-97
• 3 Neuvonen MI, Kyrklund K, Rintala RJ. et al. Bowel function and quality of life after transanal endorectal pull-through for Hirschsprung disease: controlled outcomes up to adulthood. Ann Surg 2017; 265: 622-629
• 4 Nah SA, Ong CCP, Saffari SE. et al. Anorectal malformation & Hirschsprungʼs disease: A cross-sectional comparison of quality of life and bowel function to healthy controls. J Pediatr Surg 2018; 53: 1550-1554
• 5 Tam PKH, Chung PHY, St Peter SD. et al. Paediatric Surgery: Advances in paediatric gastroenterology. Lancet 2017; 390: 1072-1082