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Hirschsprung's disease and idiopathic megacolon in adults and adolescents

P R Barnes, J E Lennard-Jones, P R Hawley

    Gut
    |May 1, 1986
    PubMed

    Insights

    This study reviews 94 patients diagnosed with Hirschsprung's disease or idiopathic megacolon after age 10. It highlights diagnostic and treatment challenges in later-diagnosed pediatric megacolon cases.

    Area of Science:

    • Pediatric Gastroenterology
    • Colorectal Surgery
    • Clinical Diagnosis

    Background:

    • Distinguishing Hirschsprung's disease from idiopathic megacolon in children has historically relied on clinical, radiological, and histological findings.
    • Both Hirschsprung's disease and idiopathic megacolon are now understood to be heterogeneous conditions with varied underlying pathologies.

    Purpose of the Study:

    • To describe the clinical experience with 94 patients diagnosed with Hirschsprung's disease or idiopathic megacolon after the age of 10.
    • To illustrate the diagnostic and treatment challenges associated with later-onset pediatric megacolon.

    Main Methods:

    • Retrospective review of clinical data from 94 patients diagnosed with megacolon after age 10 over a 15-year period.
    • Analysis of clinical presentation, diagnostic methods, and treatment outcomes for Hirschsprung's disease and idiopathic megacolon.

    Main Results:

    • Patients with Hirschsprung's disease can present with various myenteric plexus abnormalities beyond complete aganglionosis.
    • Idiopathic megacolon may represent a heterogeneous group, with potential clinical differences between childhood-onset and later-onset cases.

    Conclusions:

    • Later diagnosis of Hirschsprung's disease and idiopathic megacolon presents unique challenges in pediatric care.
    • Clinical grounds suggest that idiopathic megacolon presenting in childhood may differ from cases developing symptoms later in life.

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