Hirschsprung's disease classification: Difference between revisions
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==Overview== | ==Overview== | ||
Hirschsprung's disease may be classified based on the extent of [[Colon (anatomy)|colon]] involvement into rectosigmoid disease, long segment disease, and ultrashort segment disease. | |||
==Classification== | ==Classification== | ||
Hirschsprung's disease may be classified based on the extent of colon involvement into the following: | |||
===Rectosigmoid (short) form:=== | ===Rectosigmoid (short) form:=== | ||
*It is the most common form and is seen in 80 to 75% of infants diagnosed with Hirschsprung's disease. | *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:=== | ===Long segment colonic Hirschsprung disease:=== | ||
*It is also known as total colonic aganglionosis. | *It is also known as total colonic [[aganglionosis]]. | ||
*The disease has ileal involvement up to 50 cm proximal to the [[Ileocecal|ileocecal junction]]. | *The disease has [[Ileum|ileal]] involvement up to 50 cm proximal to the [[Ileocecal|ileocecal junction]]. | ||
*It is uncommon and accounts for 5 to 7% of cases diagnosed with Hirschsprung's disease. | *It is uncommon and accounts for 5 to 7% of cases diagnosed with Hirschsprung's disease. | ||
===Ultrashort segment Hirschsprung's disease:=== | ===Ultrashort segment Hirschsprung's disease:=== | ||
Revision as of 21:53, 15 June 2017
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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 may be classified based on the extent of colon involvement into rectosigmoid disease, long segment disease, and ultrashort segment disease.
Classification
Hirschsprung's disease may be classified based on the extent of colon involvement 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:
- 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 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.