Hirschsprung Disease

Excerpt

Hirschsprung disease (HD) is a congenital disorder defined by the absence of ganglion cells (GC) at the Meissner's plexus of the submucosa and Auerbach's plexus of the muscularis in the terminal rectum that extends in a variable distance proximally. Its prevalence varies from 1 to 1.63 per 10,000 births.

Once considered deadly, surgical treatment has reduced the disease mortality to 3% in developed countries. HD most often affects the newborn. It is responsible for non-specific symptomatology, including chronic constipation and intestinal obstruction. Its diagnosis relies upon the histopathological examination of rectal biopsies.