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

Sanne J. Verkuijl*, Florian Friedmacher, Patrick N. Harter, Udo Rolle, Paul M. A. Broens

*Corresponding author for this work

    Research output: Contribution to journalReview articlepeer-review

    17 Downloads (Pure)

    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.

    Original languageEnglish
    Pages (from-to)822-833
    Number of pages12
    JournalWorld journal of gastrointestinal surgery
    Volume13
    Issue number8
    DOIs
    Publication statusPublished - 27-Aug-2021

    Keywords

    • Hirschsprung disease
    • Aganglionosis
    • Proximal
    • Ganglionic
    • Constipation
    • Incontinence
    • QUALITY-OF-LIFE
    • INTESTINAL NEURONAL DYSPLASIA
    • ENDORECTAL PULL-THROUGH
    • LONG-TERM OUTCOMES
    • KIT-POSITIVE CELLS
    • REACHED 18 YEARS
    • INTERSTITIAL-CELLS
    • EXTRACELLULAR-MATRIX
    • NEUROTRANSMITTER EXPRESSION
    • ABNORMAL DISTRIBUTION

    Fingerprint

    Dive into the research topics of 'Persistent bowel dysfunction after surgery for Hirschsprung's disease: A neuropathological perspective'. Together they form a unique fingerprint.

    Cite this