Overview Of Hirschsprung’s Disease
Hirschsprung disease is a blockage of the large intestine. It occurs due to poor muscle movement in the bowel. It is a congenital condition, which means it is present from birth.
Commonly Associated With
Congenital megacolon
Causes Of Hirschsprung’s Disease
Muscle contractions in the gut help digested foods and liquids move through the intestine. This is called peristalsis. Nerves between the muscle layers trigger the contractions.
In Hirschsprung disease, the nerves are missing from a part of the bowel. Areas without these nerves cannot push material through. This causes a blockage. Intestinal contents build up behind the blockage. The bowel and abdomen swell as a result.
Hirschsprung disease causes about 25% of all newborn intestinal blockages. It occurs 5 times more often in males than in females. Hirschsprung disease is sometimes linked to other inherited or congenital conditions, such as Down syndrome.
Symptoms Of Hirschsprung’s Disease
Symptoms that may be present in newborns and infants include:
- Difficulty with bowel movements
- Failure to pass meconium shortly after birth
- Failure to pass a first stool within 24 to 48 hours after birth
- Infrequent but explosive stools
- Jaundice
- Poor feeding
- Poor weight gain
- Vomiting
- Watery diarrhea (in the newborn)
Symptoms in older children:
- Constipation that gradually gets worse
- Fecal impaction
- Malnutrition
- Slow growth
- Swollen belly
Exams & Tests
Milder cases may not be diagnosed until the baby is older.
During a physical exam, the health care provider may be able to feel loops of bowel in the swollen belly. A rectal exam may reveal tight muscle tone in the rectal muscles.
Tests used to help diagnose Hirschsprung disease may include:
- Abdominal x-ray
- Anal manometry (a balloon is inflated in the rectum to measure pressure in the area)
- Barium enema
- Rectal biopsy
Treatment Of Hirschsprung’s Disease
A procedure called serial rectal irrigation helps relieve pressure in (decompress) the bowel.
The abnormal section of the colon must be taken out using surgery. Most commonly, the rectum and abnormal part of the colon is removed. The healthy part of the colon is then pulled down and attached to the anus.
Sometimes this can be done in one operation. However, it is often done in two parts. A colostomy is performed first. The other part of the procedure is done later in the child’s first year of life.
