Hirschsprung’s disease is due to:
Correct Answer: Failure of migration of neural crest cells from cranial to caudal direction
Description: The cause of Hirschsprung's disease remains incompletely understood, although current thinking suggests that the disease results from a defect in the migration of neural crest cells, which are the embryonic precursors of the intestinal ganglion cell. Under normal conditions, the neural crest cells migrate into the intestine from cephalad to caudad.
The process is completed by the twelfth week of gestation, but the migration from the midtransverse colon to anus takes 4 weeks. During this latter period, the fetus is most vulnerable to defects in the migration of neural crest cells. This may explain why most cases of aganglionosis involve the rectum and rectosigmoid.
The length of the aganglionic segment of bowel is therefore determined by the most distal region that the migrating neural crest cells reach. In rare instances, total colonic aganglionosis may occur.
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