Hirschsprung's disease - Overview

Hirschsprung's disease (HD) - Overview 

Hirschsprung's disease (HD) is disease of mostly unknown origin which causes problem in the large intestine. This condition revels itself from birth of the child and is related with the lack of nerve fibers in the affected portion of the bowel wall ( intestine wall).


New born with HD cannot have bowel movement even beyond 24 to 48 hours after birth. Often in these times rectal wash out (rectal irrigation) with normal saline using soft rubber tube and lubricant is given with the help of doctor or nurse to evacuate the rectum.





Symptoms:

Considering the severity of the disease the signs and symptoms may vary which may appear  soon after birth and also in some cases later in life.

The most common symptom is failure to pass stool beyond 48 hours after birth..

Other may  signs and symptoms may include:

  1. Swollen belly
  2. Vomiting, including vomiting a green or brown substance
  3. Constipation or gas, which might make a newborn fussy
  4. Diarrhea

As the age of the child increases signs and symptoms may be:

  1. Swollen belly
  2. Chronic constipation
  3. Gas
  4. Failure to thrive
  5. Fatigue

Etiology:

The exact reason is yet not clear. But this may be familial and in some cases genetic mutation is held responsible.

Hirschsprung's disease occurs when nerve cells in the colon don't form completely. Nerves in the colon control the muscle contractions that move food through the bowels. Without the contractions, stool stays in the large intestine.

Risk factors:

  • Previous child with Hirschsprung's disease: Hirschsprung's disease can be inherited. If you have one child who has the condition, future biological children could be at risk.
  • Male child: Hirschsprung's disease is more common in males.
  • Other inherited conditions association: Hirschsprung's disease is associated with certain inherited conditions, such as Down syndrome and other abnormalities present at birth, such as congenital heart disease.

Complications:

Single most important complication is enterocolitis which is a form of intestinal infection and this enterocolitis can be life threatening and requires immediate medical attention. 

 


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