Hirschsprung’s Disease (Congenital Megacolon)

Hirschsprung’s disease is a birth defect in which the nerves which regulate the rhythmic intestinal contractions are missing in a portion of the large intestine, affecting the movement of the fecal material towards the anus. This condition, which causes the symptoms usually associated with intestinal obstruction, is also known as congenital megacolon.

  • The large intestine is affected and normal contractions of the intestine are absent in the affected area.
  • Delay in passing the meconium by the newborn infant is the first indication. Abdominal distension, vomiting, and refusal of feeds, are the other symptoms which develop in the infant later on.
  • Biopsy of the rectum, and manometry to measure the rectal pressure, help diagnose this condition.
  • Surgical intervention is necessary, and it is aimed at facilitating the normal passage of fecal material through the large intestine.

The rhythmic movement of the large intestine is controlled by a several nerves in the intestinal wall which brings about the synchronized contractions which move as a wave down the digestive tract, carrying the intestinal contents along. Bowel movements are possible because of these contractions. When Hirschsprung’s disease (congenital megacolon) affects a section of the large intestine, it is unable to contract in the normal way, thus impeding the passage of the stool to the anus.

Normally, meconium, a sticky substance, dark green in color, is passed by the infants soon after birth.  Hirschsprung’s disease is suspected when the passage of this fecal material is delayed. Symptoms of intestinal obstruction such as abdominal distension, vomiting with bile in the vomit, and refusal to feed may appear later on. When only a limited area of the intestine is affected by Hirschsprung’s disease, the associated symptoms also may be mild, often resulting in the condition remaining undiagnosed till much later in childhood. Abdominal distension, passing ribbon-like stools, and the child not gaining weight normally, are also indications of Hirschsprung’s disease. Rarely, the only indication of this condition is chronic constipation.

A potentially fatal condition called toxic enterocolitis may result from Hirschsprung’s disease (congenital megacolon). Fever and abdominal distension develop suddenly, accompanied by explosive diarrhea, which may be bloody in some cases.

Biopsy of the rectum, and measuring the pressure in the rectum known as manometry, are the tests to detect Hirschsprung’s disease accurately. Another test called barium enema also may be helpful. In this test, barium as well as air is introduced into the rectum, and then x-rays are taken.

Toxic enterocolitis may develop if Hirschsprung’s disease (congenital megacolon) is not treated rapidly by removing the abnormal parts of the intestine. The unaffected part of the intestine is then attached to the rectum to facilitate the normal functioning of the digestive system. Occasionally, when an infant is severely ill, an alternate route is created for the passage of stools by a procedure called colostomy. The healthy part of the intestine is temporarily connected to an opening created on the abdomen to which a plastic bag is attached for the collection of stool. The defective, disconnected portion of the intestine remains in the abdominal cavity till the infant recovers, and becomes older and ready for further surgery. In a surgery known as the pull-through procedure, the defective portion is removed and the healthy portion of the intestine is reattached to the rectum for normal functioning. The opening in the abdomen is also closed.

Watch This Explanatory Video Of  Hirschsprung’s Disease

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Yasser Elnahas

MD, PHD, Professor Of CardioVascular Surgery
Dr. Yasser Elnahas, Is an associate Professor of Cardiovascular Surgery. Dr. Elnahas was trained as a fellow At Texas Heart Institute And Mayo Clinic Foundation.Dr. Elnahas is dedicated to educating the general public about different disease conditions and simplifying the medical knowledge in an easy to understand terminology.

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