Hirschsprung disease and Paediatric Intestinal Pseudo-obstruction

Atchariya Chanpong; Osvaldo Borrelli; Nikhil Thapar

doi:10.1016/j.bpg.2021.101765

Hirschsprung disease and Paediatric Intestinal Pseudo-obstruction

Best Pract Res Clin Gastroenterol. 2022 Feb-Mar:56-57:101765. doi: 10.1016/j.bpg.2021.101765. Epub 2021 Sep 18.

Authors

Atchariya Chanpong¹, Osvaldo Borrelli², Nikhil Thapar³

Affiliations

¹ Neurogastroenterology & Motility Unit, Gastroenterology Department, Great Ormond Street Hospital for Children, London, WC1N 3JH, United Kingdom; Division of Gastroenterology and Hepatology, Department of Pediatrics, Faculty of Medicine, Prince of Songkla University, Songkhla, 90110, Thailand; Stem Cells and Regenerative Medicine, UCL Great Ormond Street Institute of Child Health, London, WC1N 1EH, United Kingdom.
² Neurogastroenterology & Motility Unit, Gastroenterology Department, Great Ormond Street Hospital for Children, London, WC1N 3JH, United Kingdom.
³ Stem Cells and Regenerative Medicine, UCL Great Ormond Street Institute of Child Health, London, WC1N 1EH, United Kingdom; Gastroenterology, Hepatology and Liver Transplant, Queensland Children's Hospital, Brisbane, Queensland, 4101, Australia. Electronic address: Nikhil.Thapar@health.qld.gov.au.

PMID: 35331399
DOI: 10.1016/j.bpg.2021.101765

Abstract

Hirschsprung disease (HSCR) and Paediatric Intestinal Pseudo-obstruction (PIPO) comprise two of the most recognized and severe disorders of gastrointestinal (GI) motility. HSCR is a developmental disorder of the enteric nervous system invariably affecting the large intestine, whereas the majority of PIPO conditions represent congenital disorders of one or more components of the neuromusculature and more diffusely affect the GI tract. Histopathology is deemed the gold standard for the diagnosis of HSCR and, arguably, of PIPO, but, other diagnostic modalities such as manometric and genetic studies have seen recent advances that may increase their utility. Especially for PIPO, management is multidisciplinary and best performed in specialist referral centres. Surgery remains the only viable treatment for HSCR and appears essential to optimize and sustain feeding and viability of intestinal function in PIPO patients. Novel therapies such as neural stem cell transplants show promise for the future.

Keywords: Congenital; Constipation; Dysmotility; Gastrointestinal motility; Intestinal pseudo-obstruction; Paediatric.

Publication types

Review

MeSH terms

Child
Enteric Nervous System* / pathology
Gastrointestinal Motility / physiology
Hirschsprung Disease* / complications
Hirschsprung Disease* / diagnosis
Hirschsprung Disease* / therapy
Humans
Intestinal Pseudo-Obstruction* / diagnosis
Intestinal Pseudo-Obstruction* / etiology
Intestinal Pseudo-Obstruction* / therapy