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Also known as aganglionic megacolon (AY-gang-lee-on-ic MEG-a-colon). The nerve supply that allows the normally contracted muscles of the tube of the gut to relax forms first to supply the mouth end of the fetus and migrates progressively tailward to the anus. If the relaxation nerve supply does not properly form, the anus and perhaps the lower rectum will be unable to relax and open to allow stool passage - defecation. This is exactly what happens in the condition commonly known as Hirschsprung disease. It is a birth defect involving lack of the proper nerve supply to the very lowest end of the colon, and is characterised by chronic severe constipation. In so-called short segment Hirschsprung disease, the problem may affect only a very short region of the lower rectum, and remain diagnosed only as severe constipation for years.
Hirschsprung disease is diagnosed definitively by a minor rectal biopsy, but may be strongly suggested by a barium enema xray study. Rectal exam should be done on all infants with chronic or severe constipation, and an xray study or perhaps rectal biopsy should be considered for these children.
This condition affects about 1 in 4,000 children. Boys are four times as likely to have Hirschsprung disease as girls. While most cases of Hirschsprung disease involve the lower colon only, there may be total colon involvement in as many as 10 per cent of cases. Total colon involvement is a much more serious condition.
Treatment is always surgical. The affected section of colon must be removed, and the remaining intestine is spliced to the anal area.