Hirschsprung's disease classification: Difference between revisions
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==Overview== | ==Overview== | ||
Hirschsprung's disease classification | Hirschsprung's disease classification is based on the extent of [[Colon (anatomy)|colon]] involvement. The disease can be classified as rectosigmoid, long segment, or ultrashort segment disease. | ||
==Classification== | ==Classification== | ||
Hirschsprung's disease may be classified based on the extent of [[colon]] involvement into the following: | Hirschsprung's disease may be classified based on the extent of [[colon]] involvement into the following:<ref name="pmid20301612">{{cite journal |vauthors=Pagon RA, Adam MP, Ardinger HH, Wallace SE, Amemiya A, Bean LJH, Bird TD, Ledbetter N, Mefford HC, Smith RJH, Stephens K, Parisi MA |title= |journal= |volume= |issue= |pages= |year= |pmid=20301612 |doi= |url=}}</ref> | ||
===Rectosigmoid (short segment) disease:=== | ===Rectosigmoid (short segment) disease:=== | ||
*It is the most common form | *It is the most common form seen in 75-80% of infants diagnosed with Hirschsprung's disease. | ||
===Long segment Hirschsprung's disease:=== | ===Long segment Hirschsprung's disease:=== | ||
*It is also known as total colonic [[aganglionosis]]. | *It is also known as total colonic [[aganglionosis]]. | ||
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===Ultrashort segment Hirschsprung's disease:=== | ===Ultrashort segment Hirschsprung's disease:=== | ||
*Only the [[internal anal sphincter]] is affected. | *Only the [[internal anal sphincter]] is affected. | ||
*It is also called as [[ | *It is also called as [[internal anal sphincter]] [[achalasia]]. | ||
==References== | ==References== | ||
{{ | {{Reflist|2}} | ||
Latest revision as of 15:09, 21 August 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 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:[1]
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.