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All to Know about Hirschsprung's disease

What

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Hirschsprung's disease is a sporadic disorder that makes poo stuck in the intestines. The condition mostly affects young children.
Ordinarily, the bowel squeezes and relaxes in a continuous manner to move poo along; this is a process controlled by the nervous system.
In Hirschsprung's disease, the nerves located at the end of the bowel and have the task of controlling and regulating the movements, are missing, which results in accumulation of poo and whereby forming a blockage.
If the condition is not detected and treated early, severe constipation and probably serious bowel infection (enterocolitis) could come up as the result.
However, the condition is typically detected early after birth and very soon gets treated with surgery.

Symptoms

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Considering the severity of Hirschsprung disease, various symptoms could be expected. Typically those with severe cases indicate the symptoms in their very first days of life. However, the moderate cases may not be detected until some days afterward.
Newborns may have symptoms including:
•    diarrhea
•    lack of ability to pass stool in the first or second day of life
•    enflamed tummy, bloating, and gas
•    nausea, and sometimes vomiting a green and brown substances
The most important symptom for detection of Hirschsprung disease is the newborn inability to poop within the first two days of his life. This could be very valuable in diagnosing the condition.
Those children with less severe cases of Hirschsprung condition sometimes may be ignored till later childhood, or even adolescence. Naturally the signs in these cases are more moderate. However, they may last longer. Symptoms may be as follows:
•    stomach inflammation
•    difficult weight gaining
•    constipation
•    nausea
•    gas
Since Hirschsprung disease may affect the child's ability to absorb nutrients, grow and development can be hindered.

Causes

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Although the causes of Hirschsprung's disease are not clear, it sometimes happens in relatives and could be related to a genetic mutation.
Hirschsprung's disease happens when the nerve cells located in the colon are not completely formed. Muscle contractions that leads to bowls movements are controlled by nerves in the colon. When there is no contraction, stool cannot be passed; so that, it remains in the large intestine.

Diagnosis


Meticulous physical examination should be performed by medical practitioner then the findings are assessed based on physical findings and the severity of the condition. Some diagnostic studies are required as well to establishing the proper diagnosis. They may include:
•    Abdominal X-ray. The bowel blockage is indicated through this test. Abdominal X-ray merely functions for physician who suspect the diagnosis; it does not give a definitive diagnosis.
•    Contrast enema. In order to examine colon for abnormalities, this procedure is performed. During this test a contrast agent is sent into the rectum to cover the inside of organs so that they will show up on an X-ray. Contrast enema is the best radiologic test for establishing the diagnosis.

An X-ray of the stomach will indicate a narrowed colon, obstacles and dilated intestine above the obstacle.
•    Rectal biopsy. Hirschsprung disease is diagnosed through rectal biopsy. During this procedure a sample of the rectum cells is taken and then, using a microscope, it’ll be scrutinized. Having Hirschsprung disease is diagnosed based on the absence of ganglion cells and the presence of nonmyelinated nerves in the biopsy segment.

A suction rectal biopsy in infants could be performed at the bedside because there is no sensory nerves at the site of biopsy, the procedure is not painful. If a suction biopsy is inadequate, surgical biopsy will be carried out under general anesthesia.
•    Anorectal manometry. This test indicates whether normal reflexes involving the rectum and the anus exist or not. Anorectal manometry is only used in older children and could be performed at the bedside.

Complications

Those children with Hirschsprung's disease are suitable to sever intestinal infection known as enterocolitis.
Enterocolitis may be put the child’s life at risk. It can be treated in the hospital through colon cleaning and taking antibiotics.

Treatment

Surgery

The condition is treated through surgery. During the surgery, the surgeon takes out the portion of the colon that contains the nerve cells that are malfunctioning. Then, they he reattaches the rectum to the healthy part of the colon. So that, the child would be able to pass the stool normally. In the moderate cases, the procedure could be carried out in one single operation. However, most of the time a colostomy is also required which is a separate surgical procedure.

Colostomy

For a severe case of Hirschsprung disease, after removing the abnormal section, a colostomy is employed to give the lower section of the colon time to heal. Through this procedure, a tiny hole (known as stoma) is made in their belly and the upper part of the colon is attached to it. This way the stool will leave through the stoma and enter into a collection bag, which should be changed many times a day.
Considering the length of child’s taken away intestine, the stoma can be permanent. However, in often the stoma could be surgically closed, and the colon could be surgically joined to the rectum.

After surgery

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If constipated after surgery, plenty of water should be consumed so that dehydration would be avoided. If the child is old enough be on a full-solid diet, he should consume those types of foods that are high in fiber, like whole grains and vegetables. Since consuming a lot of fiber at once could actually worsen constipation, they should be add to the diet slowly. Under the doctor’s supervision, the child could also take laxatives.

 

 

 


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