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Related Experiment Videos

Adult Hirschsprung's disease.

N L Crocker1, J M Messmer

  • 1Medical College of Virginia, Richmond.

Clinical Radiology
|October 1, 1991
PubMed
Summary
This summary is machine-generated.

Adult Hirschsprung's disease, characterized by chronic constipation, is diagnosed via barium enema and confirmed by biopsy. Early suspicion in patients with constipation and specific radiographic findings is crucial for timely diagnosis.

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Area of Science:

  • Gastroenterology and Colorectal Surgery
  • Pediatric Surgery (in adult presentation)

Background:

  • Hirschsprung's disease is typically a congenital condition affecting newborns, but adult cases present unique diagnostic challenges.
  • Chronic constipation is the hallmark symptom in nearly all adult patients diagnosed with Hirschsprung's disease.

Observation:

  • Barium enema reveals a narrowed segment with proximal dilation in 83% of adult Hirschsprung's disease cases.
  • A significant minority (14%) of patients present with a dilated colon without a discernible narrowed segment.
  • Anal manometry can aid diagnosis by demonstrating the absence of the normal anorectal inhibitory reflex.

Findings:

  • Definitive diagnosis requires histological confirmation: the absence of ganglion cells in the colonic biopsy.
  • While total colonic aganglionosis is exceptionally rare in adults, it has been reported.

Related Experiment Videos

  • The study reviews two new cases and 47 previously reported adult cases of Hirschsprung's disease.
  • Implications:

    • Increased clinical suspicion for adult Hirschsprung's disease is warranted in patients with chronic constipation and suggestive imaging.
    • Accurate diagnosis through imaging and biopsy is essential for appropriate management and improved patient outcomes.
    • Understanding the varied presentations of adult Hirschsprung's disease aids in earlier detection and intervention.