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Video of Issue Per-rectal endoscopic myotomy for ultrashort-segment Hirschsprung’s disease in adult patients
Harshal Surendra Mandavdhare1orcid, Arvind Sekar2orcid, Ritesh Acharya1orcid, Rajani Kant Kumar3orcid

DOI: https://doi.org/10.5946/ce.2025.002
Published online: May 8, 2025

1Department of Gastroenterology, Post Graduate Institute of Medical Education and Research, Chandigarh, India

2Department of Histopathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India

3Department of Anaesthesiology, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Correspondence: Harshal Surendra Mandavdhare Department of Gastroenterology, Post Graduate Institute of Medical Education and Research, ground floor, F block, Chandigarh 160012, India E-mail: hmandavdhare760@gmail.com
• Received: January 2, 2025   • Revised: January 30, 2025   • Accepted: January 31, 2025

© 2025 Korean Society of Gastrointestinal Endoscopy

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (https://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Per-rectal endoscopic myotomy (PREM), which is similar to peroral endoscopic myotomy for achalasia cardia, has been found to benefit patients with Hirschsprung’s disease (HSCR), yielding good medium-term outcomes.1-3 We present our first experience with PREM with a short-term follow-up period of 3 months.
A 19-year-old woman presented with constipation since the age of 1 year. Barium enema revealed a significantly dilated rectosigmoid with conical narrowing of the distal rectum (Fig. 1). Endoscopic mucosal resection biopsies were obtained at 5 cm, 10 cm, 15 cm, 25 cm, and 35 cm from the anal verge. Ganglion cells were present from 35 to 10 cm (Fig. 2), but the biopsy from 5 cm did not show ganglion cells, even with PHOX2B immunohistochemical staining (Fig. 3). Ultrashort-segment HSCR was thus diagnosed. All possible treatment options were explained to the patient, who provided consent for PREM (Video 1). The procedure was performed under general anesthesia with the patient in a semi-jackknife position (Fig. 4). A mucosal bleb was created along the dentate line of the posterior wall. A transverse incision was made over the bleb, and an entry was made into the submucosal space. Subsequently, a tunnel was created by submucosal dissection up to 3 cm into the dilated ganglionic segment of the rectum. This was followed by full-thickness myotomy up to the entry incision, taking care not to damage the external anal sphincter. After achieving hemostasis, the incision was closed using hemoclips. Appreciable post-procedure opening of the distal narrowing was achieved. At the 3-month follow-up, the patient passed normal stools daily without the need for laxatives. PREM is thus a minimally invasive therapeutic option for short-segment HSCR. More data, including larger sample size and a longer follow-up, are warranted to determine its true efficacy.
Video 1. Demonstration of the per-rectal endoscopic myotomy procedure for ultrashort-segment Hirschsprung’s disease.
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A video related to this article can be found online at https://doi.org/10.5946/ce.2025.002.
Fig. 1.
Barium enema image showing hugely dilated rectosigmoid with a conical narrowing at the distal rectum (arrow).
ce-2025-002f1.jpg
Fig. 2.
Endoscopic mucosal resection biopsy obtained 10 cm from the anal verge reveals the presence of ganglion cells (arrow) (hematoxylin and eosin stain, ×400).
ce-2025-002f2.jpg
Fig. 3.
(A) A biopsy from 5 cm shows no ganglion cells (hematoxylin and eosin, ×200), (B) further confirmed by PHOX2B immunostaining (×400).
ce-2025-002f3.jpg
Fig. 4.
Semi-jackknife position for per-rectal endoscopic myotomy.
ce-2025-002f4.jpg
  • 1. Kyrklund K, Sloots CE, de Blaauw I, et al. ERNICA guidelines for the management of rectosigmoid Hirschsprung's disease. Orphanet J Rare Dis 2020;15:164.ArticlePubMedPMCPDF
  • 2. Bapaye A, Dashatwar P, Biradar V, Biradar S, Pujari R. 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. Endoscopy 2021;53:1256–1260.ArticlePubMed
  • 3. Nabi Z, Reddy DN. Submucosal endoscopy: the present and future. Clin Endosc 2023;56:23–37.ArticlePubMedPMC

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      Per-rectal endoscopic myotomy for ultrashort-segment Hirschsprung’s disease in adult patients
      Image Image Image Image
      Fig. 1. Barium enema image showing hugely dilated rectosigmoid with a conical narrowing at the distal rectum (arrow).
      Fig. 2. Endoscopic mucosal resection biopsy obtained 10 cm from the anal verge reveals the presence of ganglion cells (arrow) (hematoxylin and eosin stain, ×400).
      Fig. 3. (A) A biopsy from 5 cm shows no ganglion cells (hematoxylin and eosin, ×200), (B) further confirmed by PHOX2B immunostaining (×400).
      Fig. 4. Semi-jackknife position for per-rectal endoscopic myotomy.

      Fig. 1.

      Fig. 2.

      Fig. 3.

      Fig. 4.

      Per-rectal endoscopic myotomy for ultrashort-segment Hirschsprung’s disease in adult patients
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