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World J Gastrointest Surg. Aug 27, 2021; 13(8): 822-833
Published online Aug 27, 2021. doi: 10.4240/wjgs.v13.i8.822
Persistent bowel dysfunction after surgery for Hirschsprung’s disease: A neuropathological perspective
Sanne J Verkuijl, Florian Friedmacher, Patrick N Harter, Udo Rolle, Paul MA Broens
Sanne J Verkuijl, Florian Friedmacher, Udo Rolle, Department of Pediatric Surgery, University Hospital Frankfurt, Goethe University Frankfurt, Frankfurt 60590, Germany
Sanne J Verkuijl, Patrick N Harter, Neurological Institute (Edinger-Institute), University Hospital Frankfurt, Goethe University Frankfurt, Frankfurt 60528, Germany
Sanne J Verkuijl, Paul MA Broens, Department of Surgery, Division of Pediatric Surgery, University of Groningen, University Medical Center Groningen, Groningen 9700 RB, Netherlands
Author contributions: Verkuijl SJ and Friedmacher F drafted the manuscript; Friedmacher F, Harter PN, Rolle U and Broens PMA critically read and revised the manuscript.
Conflict-of-interest statement: The authors declare no conflict of interest for this article.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Sanne J Verkuijl, BSc, Research Fellow, Department of Pediatric Surgery, University Hospital Frankfurt, Goethe University Frankfurt, Theodor-Stern-Kai 7, Frankfurt 60590, Germany. s.j.verkuijl@umcg.nl
Received: February 12, 2021
Peer-review started: February 12, 2021
First decision: May 4, 2021
Revised: May 12, 2021
Accepted: July 5, 2021
Article in press: July 5, 2021
Published online: August 27, 2021
Processing time: 188 Days and 22.1 Hours
Abstract

Hirschsprung’s disease (HD) is a congenital disorder, characterized by aganglionosis in the distal part of the gastrointestinal tract. Despite complete surgical resection of the aganglionic segment, both constipation and fecal incontinence persist in a considerable number of patients with limited treatment options. There is growing evidence for structural abnormalities in the ganglionic bowel proximal to the aganglionosis in both humans and animals with HD, which may play a role in persistent bowel dysfunction. These abnormalities include: (1) Histopathological abnormalities of enteric neural cells; (2) Imbalanced expression of neurotransmitters and neuroproteins; (3) Abnormal expression of enteric pacemaker cells; (4) Abnormalities of smooth muscle cells; and (5) Abnormalities within the extracellular matrix. Hence, a better understanding of these previously unrecognized neuropathological abnormalities may improve follow-up and treatment in patients with HD suffering from persistent bowel dysfunction following surgical correction. In the long term, further combination of clinical and neuropathological data will hopefully enable a translational step towards more individual treatment for HD.

Keywords: Hirschsprung disease; Aganglionosis; Proximal; Ganglionic; Constipation; Incontinence

Core Tip: Hirschsprung’s disease (HD) is a congenital disorder, characterized by aganglionosis in the distal part of the gastrointestinal tract. Despite surgical resection of the aganglionic bowel segment, bowel dysfunction persists in a considerable number of patients with limited treatment options. There is growing evidence regarding structural abnormalities in the proximal, ganglionic colon of both humans and animals with HD, which may play a role in persistent bowel dysfunction. Hence, understanding these previously unrecognized neuropathological abnormalities in the proximal, ganglionic bowel of patients with HD may improve current follow-up and treatment of persistent postoperative bowel dysfunction in certain patients.