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Fibromuscular dysplasia of arteries in Hirschsprung's disease

T Taguchi, K Tanaka, K Ikeda

    Gastroenterology
    |May 1, 1985
    PubMed

    Insights

    Hirschsprung's disease in children can be linked to abnormal intestinal arteries. These arteries show changes similar to fibromuscular dysplasia, suggesting a new condition called "adventitial fibromuscular dysplasia."

    Area of Science:

    • Gastroenterology
    • Pediatric Surgery
    • Vascular Pathology

    Background:

    • Hirschsprung's disease is a congenital condition affecting the large intestine.
    • Abnormalities in intestinal vasculature have been implicated in its pathogenesis.
    • Previous studies suggest a link between vascular issues and neural crest cell migration.

    Purpose of the Study:

    • To investigate the arterial histology in children diagnosed with Hirschsprung's disease.
    • To identify potential vascular pathologies contributing to the disease.
    • To propose a new pathological entity based on observed arterial changes.

    Main Methods:

    • Histological examination of intestinal tissue from 25 children with Hirschsprung's disease.
    • Analysis of arterial morphology, focusing on the adventitia and smooth muscle layers.
    • Comparison of findings with known vascular conditions like fibromuscular dysplasia.

    Main Results:

    • Abnormal arteries were identified in 8 out of 25 children (32%).
    • Histological features included smooth muscle cell proliferation in a thickened adventitia, resembling fibromuscular dysplasia.
    • These arterial abnormalities were exclusively found in the transitional zone of the intestine.

    Conclusions:

    • A new pathological entity, "adventitial fibromuscular dysplasia," is proposed.
    • The findings support the hypothesis that embryonic intestinal ischemia, due to abnormal arteries, may disrupt ganglion cell migration in Hirschsprung's disease.

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