Hirschsprung Disease

What Is Hirschsprung Disease?

Hirschsprung's Disease is a pathological condition present during birth in which there is a problem with the colon which makes it difficult for the baby to pass stools. Hirschsprung's Disease is caused due to missing nerve cells in some of the muscles of the colon of the newborn baby. A new born baby with Hirschsprung's Disease will not pass stool on the first day of life itself. In some case, this disease is so mild that it is not detected till late in childhood. This disease is basically treated with a surgical procedure which bypasses the affected part of the colon or a procedure in which the diseased part of the colon is removed.

Hirschsprung Disease

What Are The Causes Of Hirschsprung Disease?

The root cause of Hirschsprung's Disease is not entirely clear. It is opined by some researchers that it is a congenital defect and is caused due to mutations in certain genes. This disease occurs when certain nerve cells of the muscles of the colon are missing which affects normal contraction of the muscles which is required for smooth movement of the food through the bowels. Under normal circumstances, as the baby grows in the womb there is development of nerve cells within layers of muscles in the colon. In Hirschsprung's Disease, these nerve cells are missing or underdeveloped hence causing Hirschsprung's Disease.

What Are The Symptoms Of Hirschsprung Disease?

The symptoms of Hirschsprung's Disease can be observed immediately after birth but in some cases symptoms may not be observed until late in childhood. Some of the symptoms of Hirschsprung's Disease are:

  • Swelling in the area around the belly
  • Vomiting with a greenish-brown vomitus
  • Constipation
  • Flatulence
  • Diarrhea
  • Failure to gain weight
  • Severe fatigue.

How Is Hirschsprung Disease Diagnosed?

Some methods to diagnose Hirschsprung's Disease are:

Abdominal X-ray using Contrast Dye: In this test, a contrast dye is placed in the rectum through a tube which then fills and forms a coating along the lining of the colon. An x-ray is then taken which can clearly show the normal part of the colon and the part of the colon which has the missing nerves.

Measurement of Muscle Control Around Rectum: This is done through a manometry test. In this test, the physician inserts a balloon inside the rectum which is then inflated. Under normal circumstances, when the balloon is inflated the muscles should contract as a result. If this does not happen then it is a clear signal of Hirschsprung's Disease.

Colon Biopsy: This is done to identify if there is any abnormalities in the colon and this is the best way to identify Hirschsprung's Disease.

What Are Treatments For Hirschsprung Disease?

The treatment options for Hirschsprung's Disease are illustrated below:

Surgery For Hirschsprung's Disease: A surgical procedure is done in which the diseased area in the colon is either bypassed completely or removed from the body and the normal colon is pulled from inside and attached to the anus. This is called a Pull-Through Procedure.

The second type of surgery is the ostomy procedure in which the diseased portion of the colon is removed and the healthy portion is attached to an ostomy in the abdomen. The stool is then passed through this ostomy into a bag which is attached at the end of the intestine. There are two types of ostomies

  • Ileostomy
  • Colostomy.

How Is The Quality Of Life Of A Child With Hirschsprung's Disease Postsurgery?

The affect on the quality of life of a child with Hirschsprung's Disease is illustrated below. This is dependent on the type of the surgery the child undergoes:

Ostomy Procedure: Usually, children feel a lot better postprocedure as they are able to pass stool much easily. In case of adolescents, their quality of life also improves but they need to get adjusted to living with the ostomy bag. They are instructed on how to take care of the abdominal opening which is also called stoma and also how to change the ostomy bag. Once these things are able to be taken care of then the children lead normal lives, although there might be a level of anxiety about being different from their peers.

Pull-through Procedure: Majority of children postprocedure are able to pass stool normally. They may have diarrhea for a short period of time but this tends to resolve with time. Some infants may have diaper rash which can be treated with creams. With the passage of time, the quality of stool normalizes and it becomes more solid and bowel movements are more regular and the frequency becomes less. In some cases, children may have bowel incontinence but once they learn how to use the anal muscles adequately such accidents also resolve.

Complications Postsurgery In Children With Hirschsprung Disease

Some children suffering from Hirschsprung disease can end up having an infection of the intestines postprocedure called enterocolitis. Some of the symptoms for this infection are:

  • Fever
  • Abdominal swelling
  • Vomiting
  • Diarrhea
  • Rectal bleeding
  • Lethargy.

This infection is treated in a hospital setting utilizing intravenous fluids and antibiotics. The large intestine is also cleaned thoroughly at regular intervals until the stool is removed completely. In some cases, advent of an infection may indicate the beginning of a more serious problem and further surgery may need to be conducted in order to correct the problem and prevent further infections.

Home Remedies For Hirschsprung Disease

Some of the home remedies suggested for Hirschsprung's Disease are:

  • The child needs to be fed food which is high in fiber so that stool is formed easily. Some foods rich in fiber are whole grains, or whole wheat bread. Fruits and vegetables should also be given in adequate amounts. Fatty foods should be avoided.
  • Try and increase the intake of fluids, which helps the child stay hydrated when the colon is in the healing phase as one of its function is to absorb water on the final stages of digestion.
  • The child should be encouraged to increase physical activity and do aerobics if possible.
  • Use of some laxatives might help ease up constipation and promote smooth bowel movements.

Written, Edited or Reviewed By:


Last Modified On: May 30, 2015

Pain Assist Inc.

Pramod Kerkar
  Note: Information provided is not a substitute for physician, hospital or any form of medical care. Examination and Investigation is necessary for correct diagnosis.

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