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
Based on the extent of colon involvement Hirschsprung's disease can be classified into rectosigmoid disease, long segment disease and ultrashort segment disease.
Classification
Based on the extent of colon involvement Hirschsprung's disease can be classified into the following:
Rectosigmoid (short) form:
- It is the most common form and is seen in 80 to 75% of infants diagnosed with Hirschsprung's disease.
Long segment colonic Hirschsprung disease:
- 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 to 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.