Hirschsprung's Disease Information

What is Hirschsprung's Disease ?

Hirschsprung's is the congenital absence of parasympathetic innervation of the distal intestine. The colon proximal to the aganglionic segment, in an effort to overcome the partial obstruction, becomes distended and its wall markedly thickened because of muscle hypertrophy. Occurs 1 in 1000-1500 live births with a 4:1 male predominance. 96% are TAGA. 4% prematures.

The parasympathetic ganglion cell network located between the circular and longitudinal muscle layers is referred to as Auerbach's plexus, whereas Meissner's plexus is the submucosal layer of ganglion cells just beneath the muscularis mucosa. In Hirschsprung's disease, ganglion cells are absent from all layers. That aganglionic segment usually involves the terminal intestine, i.e. the rectum or rectosigmoid. The aganglionic segment may, however, include the entire large bowel and even small bowel.

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