Hirschsprung's disease causes
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Ahmed Younes M.B.B.CH [2]
Overview
Hirschsprung's disease is caused by failure of migration of myentric and submucosal nerve plexuses craniocaudally into the distal part of the bowel.
Causes
- Normally, myenteric and submucosal nerve plexuses regulate motility by allowing relaxation. In the absence of their migration, there’s over contracting which prevents passage of stool.
- Absence of myenteric and submucosal plexuses causes over contraction of the smooth muscles of the rectum causing functional obstruction and chronic constipation.
- Absence of these cells in the distal colon is believed to be due to a genetic mutation causing failure of migration of these cells craniocaudally.
- Defective differentiation of neuroblasts into neural crest cells is another suggested hypothesis explaining the disease.