Hirschsprung's disease overview: Difference between revisions
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{{Hirschsprung's disease}} | {{Hirschsprung's disease}} | ||
{{CMG}};{{AE}}{{AY}} | {{CMG}};{{AE}}{{AY}} | ||
==Overview== | ==Overview== | ||
Hirschsprung's disease involves an enlargement of the [[colon (anatomy)|colon]], caused by [[bowel obstruction]] resulting from an [[ganglion|aganglionic]] section of [[bowel]] (the normal [[Enteric nervous system|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. | Hirschsprung's disease involves an enlargement of the [[colon (anatomy)|colon]], caused by [[bowel obstruction]] resulting from an [[ganglion|aganglionic]] section of [[bowel]] (the normal [[Enteric nervous system|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== | ==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. | 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. | ||
==Classification== | |||
==Pathophysiology== | ==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. <ref>Worman and Ganiats 1995, Am Fam Physician 51, 487-494 [http://www.ncbi.nlm.nih.gov/pubmed/7840044]</ref> | Hirschsprung’s disease is a [[congenital]] disorder of the colon in which certain nerve cells, known as ganglion cells, are absent, causing chronic constipation. <ref>Worman and Ganiats 1995, Am Fam Physician 51, 487-494 [http://www.ncbi.nlm.nih.gov/pubmed/7840044]</ref> | ||
==Causes== | |||
==Differentiating Hirschsprung's dieases from other diseases== | |||
==Epidemiology and Demographics== | |||
==Risk Factors== | |||
==Natural History, Complications, and Prognosis== | |||
==Screening== | |||
==Diagnosis== | ==Diagnosis== | ||
===History and Symptoms=== | |||
===Physical Examination=== | |||
===Laboratory Findings=== | |||
===Xray=== | |||
===CT Scan=== | |||
===MRI=== | |||
===Other Imaging Findings=== | ===Other Imaging Findings=== | ||
A [[barium enema]] is the mainstay of diagnosis of Hirschsprung’s disease. | A [[barium enema]] is the mainstay of diagnosis of Hirschsprung’s disease. | ||
===Other Diagnostic Studies=== | ===Other Diagnostic Studies=== | ||
A rectal [[biopsy]] showing the lack of ganglion cells is the only certain method of diagnosis. | A rectal [[biopsy]] showing the lack of ganglion cells is the only certain method of diagnosis. | ||
==Treatment== | ==Treatment== | ||
===Medical Therapy=== | |||
===Surgery=== | ===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). | 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). | ||
===Prevention=== | |||
==References== | ==References== | ||
{{Reflist|2}} | {{Reflist|2}} | ||
{{WH}}{{WS}} | |||
Revision as of 21:49, 15 June 2017
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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.
Classification
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]
Causes
Differentiating Hirschsprung's dieases from other diseases
Epidemiology and Demographics
Risk Factors
Natural History, Complications, and Prognosis
Screening
Diagnosis
History and Symptoms
Physical Examination
Laboratory Findings
Xray
CT Scan
MRI
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
Medical Therapy
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).