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Variant Hirschsprung's disease.

Prem Puri1, Udo Rolle

  • 1Children's Research Centre, Our Lady's Hospital for Sick Children, Crumlin, Dublin 12, Ireland. prem.puri@ucd.ie

Seminars in Pediatric Surgery
|January 22, 2005
PubMed
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Histological techniques can diagnose conditions mimicking Hirschsprung disease (HD) by identifying intestinal neuronal dysplasia (IND) and internal sphincter achalasia. Careful consideration of surgical intervention is crucial for these rare smooth muscle disorders.

Area of Science:

  • Gastroenterology
  • Histopathology
  • Pediatric Surgery

Background:

  • Conditions clinically resembling Hirschsprung disease (HD) may present despite ganglion cells on rectal biopsies.
  • Accurate diagnosis requires specialized histological techniques and adequate tissue samples.

Purpose of the Study:

  • To differentiate conditions mimicking HD, such as intestinal neuronal dysplasia (IND) and internal sphincter achalasia.
  • To highlight diagnostic methods and treatment considerations for these rare gastrointestinal motility disorders.

Main Methods:

  • Utilizing adequate suction rectal biopsies and various histological techniques.
  • Employing anorectal manometry for diagnosing internal sphincter achalasia.
  • Histological examination for nitrergic nerve depletion in internal sphincter achalasia.

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Main Results:

  • Intestinal neuronal dysplasia (IND) is a distinct clinical entity provable by histology, involving abnormalities in submucosal, myenteric plexuses, and muscle innervation.
  • Internal sphincter achalasia shows nitrergic nerve depletion and can be diagnosed via manometry.
  • Most surgical explorations for these smooth muscle disorders have not been beneficial.

Conclusions:

  • Accurate histological analysis is key to diagnosing conditions that mimic HD.
  • Internal sphincter achalasia is treatable with myectomy, while other smooth muscle disorders often have fatal outcomes.
  • Surgical intervention should be individualized and approached with caution due to limited efficacy in many cases.