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Variant Hirschsprung's disease

P Puri1

  • 1Children's Research Centre, Our Lady's Hospital for Sick Children, Dublin, Ireland.

Journal of Pediatric Surgery
|February 1, 1997
PubMed
Summary
This summary is machine-generated.

Variant Hirschsprung's disease, presenting with ganglia cells, can be diagnosed using advanced histological techniques. Intestinal neuronal dysplasia and internal sphincter achalasia are distinct entities requiring specific diagnostic and treatment approaches.

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Area of Science:

  • Pediatric Surgery
  • Gastroenterology
  • Histopathology

Background:

  • Many conditions mimic Hirschsprung's disease despite ganglia cells on rectal biopsy.
  • Delineating these variant conditions requires advanced diagnostic methods.

Purpose of the Study:

  • To investigate and classify functional bowel disorders that present similarly to Hirschsprung's disease but have ganglia cells present.
  • To establish diagnostic criteria and treatment implications for these variant conditions.

Main Methods:

  • Analysis of 66 full-thickness rectal biopsies and surgical specimens from patients with Hirschsprung's-like symptoms.
  • Utilized histochemical, immunohistochemical, silver staining, and electron microscopy.

Main Results:

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  • Diagnosed intestinal neuronal dysplasia (23 patients), hypoganglionosis (6), immature ganglia (4), absent argyrophil plexus (3), internal sphincter achalasia (15), and smooth muscle disorders (15).
  • Intestinal neuronal dysplasia involves plexuses, muscle innervation, and the internal sphincter.
  • Internal sphincter achalasia shows nitrergic nerve depletion and responds to myectomy.
  • Smooth muscle disorders have a poor prognosis.

Conclusions:

  • Adequate biopsies and diverse histological techniques are crucial for diagnosing Hirschsprung's variants.
  • Intestinal neuronal dysplasia is a distinct entity with complex innervation defects.
  • Internal sphincter achalasia is identifiable and treatable.
  • Smooth muscle disorders require careful consideration regarding surgical intervention due to poor outcomes.