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]

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.

Ultra short segment Hirschsprung's disease:

• Only the internal anal sphincter is affected.
• It is also called as internal anal sphincter achalasia.