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Paediatric Pathology Online

Pathology of Hirschsprung's Disease

Dr Sampurna Roy MD 





Hirschsprung's disease is characterized by aganglionosis of the enteric neural plexuses and failure of migration of fetal neuroblasts from the cephalic neural crest down the alimentary tract is the most likely embryonal basis for this condition.

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The rectum is most commonly involved and classification depends on the length of the ganglionic segment:

- Short segment disease involves rectum and sigmoid.

- In long segment disease aganglionosis extends more proximally than the sigmoid colon.

- The whole colon may be involved and, rarely the entire intestinal tract is aganglionic.

Use of rectal suction mucosal biopsies has now replaced full thickness biopsies.

The diagnosis depends predominantly on clinical presentation, barium studies and rectal biopsy.

Absence of ganglion cells, often accompanied by thickened nerve fascicles, is required for histological diagnosis.

The acetylcholinesterase reaction has substantially facilitated evaluation of suction biopsies and the technique can be carried out fairly rapidly.

Ischemic enterocolitis is a serious complication of untreated Hirschsprung's disease and carries significant morbidity and mortality.

Further reading:

Experience of acetylcholinesterase histochemistry application in the diagnosis of chronic constipation in children.

Duhamel operation vs neonatal transanal endorectal pull-through procedure for Hirschsprung disease: which are the changes for pathologists?

Enzyme histochemistry of classical and ultrashort Hirschsprung's disease .

Conventional histological diagnostics in coloproctology].

At what age is a suction rectal biopsy less likely to provide adequate tissue for identification of ganglion cells?

Evolution of the technique in the transanal pull-through for Hirschsprung's disease: effect on outcome.

A new rapid acetylcholinesterase staining kit for diagnosing Hirschsprung's disease.

Hirschsprung's disease in a young adult: report of a case and review of the literature.

Hirschsprung's disease: diagnosis and management.

Clinicopathological features in 102 cases of Hirschsprung disease.

Rectal biopsy: what is the optimal procedure?

Altered distribution of interstitial cells of Cajal in Hirschsprung disease.

Colonic atresia and Hirschsprung's disease: importance of histologic examination of the distal bowel.



Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)






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