Cutting-edge Regenerative Therapy for Hirschsprung Disease and Its Allied Disorders

Overview

Journal Surg Today

Specialty General Surgery

Date 2023 Sep 5

PMID 37668735

Authors

Koichiro Yoshimaru

Toshiharu Matsuura

Yasuyuki Uchida

Soichiro Sonoda

Shohei Maeda

Keisuke Kajihara

Yuki Kawano

Takeshi Shirai

Yukihiro Toriigahara

Alvin Santoso Kalim

Xiu-Ying Zhang

Yoshiaki Takahashi

Naonori Kawakubo

Kouji Nagata

Haruyoshi Yamaza

Takayoshi Yamaza

Tomoaki Taguchi

Tatsuro Tajiri

Affiliations

Soon will be listed here.

Abstract

Hirschsprung disease (HSCR) and its associated disorders (AD-HSCR) often result in severe hypoperistalsis caused by enteric neuropathy, mesenchymopathy, and myopathy. Notably, HSCR involving the small intestine, isolated hypoganglionosis, chronic idiopathic intestinal pseudo-obstruction, and megacystis-microcolon-intestinal hypoperistalsis syndrome carry a poor prognosis. Ultimately, small-bowel transplantation (SBTx) is necessary for refractory cases, but it is highly invasive and outcomes are less than optimal, despite advances in surgical techniques and management. Thus, regenerative therapy has come to light as a potential form of treatment involving regeneration of the enteric nervous system, mesenchyme, and smooth muscle in affected areas. We review the cutting-edge regenerative therapeutic approaches for managing HSCR and AD-HSCR, including the use of enteric nervous system progenitor cells, embryonic stem cells, induced pluripotent stem cells, and mesenchymal stem cells as cell sources, the recipient intestine's microenvironment, and transplantation methods. Perspectives on the future of these treatments are also discussed.

Citing Articles

Causes and consequences: development and pathophysiology of Hirschsprung disease.

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Robotic intersphincteric resection for low rectal cancer: a cumulative sum analysis for the learning curve.

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PMID: 38717597 DOI: 10.1007/s00595-024-02841-x.

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