The enteric nervous system consists of the myenteric plexus (Auerbach — between the muscle layers) and the submucosal plexus (Meissner — in the submucosa), forming an intrinsic nervous system of approximately 500 million neurons that controls gut motility, secretion, and absorption independently of the central nervous system.
Hirschsprung's disease results from failure of neural crest cell migration into the distal bowel, leaving an aganglionic segment devoid of myenteric and submucosal plexus neurons. Diagnosis requires full-thickness rectal biopsy demonstrating absence of ganglion cells. The transition zone between ganglionic and aganglionic bowel is identified intraoperatively. Achalasia results from myenteric plexus destruction at the lower oesophageal sphincter.
Failure of enteric plexus neural crest migration producing an aganglionic colonic segment with functional obstruction in neonates, diagnosed by rectal biopsy and treated by pull-through surgery excising the aganglionic segment.
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