Hirschsprung's disease classification
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ahmed Younes M.B.B.CH [2]
Overview
Hirschsprung's disease classification, based on the extent of colon involvement, include: rectosigmoid, long segment, and ultrashort segment disease.
Classification
Hirschsprung's disease may be classified based on the extent of colon involvement into the following:
Rectosigmoid (short segment) disease:
- It is the most common form, seen in 75-80% of infants diagnosed with Hirschsprung's disease.
Long segment Hirschsprung's disease:
- It is also known as total colonic aganglionosis.
- The disease has ileal involvement up to 50 cm proximal to the ileocecal junction.
- It is uncommon and accounts for 5-7% of cases diagnosed with Hirschsprung's disease.
Ultrashort segment Hirschsprung's disease:
- Only the internal anal sphincter is affected.
- It is also called as internal anal sphincter achalasia.