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Persistent bowel dysfunction after surgery for Hirschsprung's disease: A neuropathological perspective.

Sanne J Verkuijl1, Florian Friedmacher1, Patrick N Harter2

  • 1Department of Pediatric Surgery, University Hospital Frankfurt, Goethe University Frankfurt, Frankfurt 60590, Germany.

World Journal of Gastrointestinal Surgery
|September 13, 2021
PubMed
Summary

Hirschsprung's disease (HD) involves missing nerves in the gut. Even after surgery, many patients experience ongoing bowel issues due to abnormalities in the remaining bowel nerves.

Keywords:
AganglionosisConstipationGanglionicHirschsprung diseaseIncontinenceProximal

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Area of Science:

  • Gastroenterology
  • Pediatric Surgery
  • Developmental Biology

Background:

  • Hirschsprung's disease (HD) is a congenital condition affecting the gastrointestinal tract due to aganglionosis.
  • Surgical resection of the aganglionic segment is standard treatment, but persistent constipation and fecal incontinence affect many patients.

Purpose of the Study:

  • To investigate structural abnormalities in the ganglionic bowel proximal to the aganglionosis in Hirschsprung's disease.
  • To explore the potential role of these abnormalities in persistent bowel dysfunction after surgery.

Main Methods:

  • Review of existing evidence on neuropathological abnormalities in the ganglionic bowel of HD patients and animal models.
  • Analysis of histopathological findings, neurotransmitter expression, enteric pacemaker cells, smooth muscle cells, and extracellular matrix.

Main Results:

  • Evidence suggests structural abnormalities in the ganglionic bowel, including neural cell pathology, imbalanced neurotransmitter expression, abnormal pacemaker cells, smooth muscle changes, and extracellular matrix alterations.
  • These findings indicate a potential contribution to persistent bowel dysfunction in HD patients post-surgery.

Conclusions:

  • Understanding these unrecognized neuropathological abnormalities is crucial for improving patient follow-up and treatment.
  • Further integration of clinical and neuropathological data may lead to personalized treatment strategies for Hirschsprung's disease.