Hirschsprung's disease overview
|
Hirschsprung's disease Microchapters |
|
Diagnosis |
|---|
|
Treatment |
|
Case Studies |
|
Hirschsprung's disease overview On the Web |
|
American Roentgen Ray Society Images of Hirschsprung's disease overview |
|
Risk calculators and risk factors for Hirschsprung's disease overview |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2];Associate Editor(s)-in-Chief: Ahmed Younes M.B.B.CH [3]
Overview
Hirschsprung's disease involves an enlargement of the colon, caused by bowel obstruction resulting from an aganglionic section of bowel (the normal enteric nerves are absent) that starts at the anus and progresses upwards. The length of bowel that is affected varies but seldom stretches for more than a foot or so.
Historical Perspective
This disease is named for Harald Hirschsprung, the Danish physician who first described the disease in 1886, describing two infants who had died with swollen bellies. The autopsies showed identical pictures with a pronounced dilatation and hypertrophy of the colon as the dominant features.
Pathophysiology
Hirschsprung’s disease is a congenital disorder of the colon in which certain nerve cells, known as ganglion cells, are absent, causing chronic constipation. [1]
Diagnosis
Other Imaging Findings
A barium enema is the mainstay of diagnosis of Hirschsprung’s disease.
Other Diagnostic Studies
A rectal biopsy showing the lack of ganglion cells is the only certain method of diagnosis.
Treatment
Surgery
The usual treatment is pull-through surgery where the portion of the colon that does have nerve cells is pulled through and sewn over the part that lacks nerve cells (National Digestive Diseases Information Clearinghouse).