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 is based on the extent of colon involvement. The disease can be classified as rectosigmoid, long segment, or 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.