Hirschsprung's disease overview

Revision as of 19:03, 15 June 2017 by Ahmed Younes (talk | contribs)
Jump to navigation Jump to search


Hirschsprung's disease Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Hirschsprung's Disease from other Diseases

Epidemiology and Demographics

Risk factors

Screening

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Laboratory findings

Electrocardiogram

X-ray

CT

MRI

Echocardiography or Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary prevention

Secondary prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Hirschsprung's disease overview On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Hirschsprung's disease overview

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Hirschsprung's disease overview

CDC on Hirschsprung's disease overview

Hirschsprung's disease overview in the news

Blogs on Hirschsprung's disease overview

Directions to Hospitals Treating Hirschsprung's disease

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).

References

  1. Worman and Ganiats 1995, Am Fam Physician 51, 487-494 [1]