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Hirschsprung's disease

Hirschsprung's disease

Hirschsprung's disease is a disorder of the large intestine that causes feces or faeces to get stuck in the intestine. This congenital disease, which is classified as rare, can cause babies to be unable to defecate (BAB) from birth.

Nerves in the large intestine function to control bowel movements. Under normal conditions, this movement of the large intestine pushes the stool out. However, in Hirschsprung 's disease , the nerves in the large intestine don't form properly. As a result, feces accumulate in the large intestine.

Hirschsprung's disease can generally be known from birth. However, in mild conditions, this disease is only detected after the child is older.

Causes of Hirschsprung's Disease

It is not yet known why the large intestine nerves in Hirschsprung's disease are not fully formed. However, this condition is thought to be related to several factors, namely:

  • Male gender
  • Have a family with Hirschsprung's disease
  • Suffering from other hereditary diseases, such as Down syndrome or congenital heart disease .

Symptoms of Hirschsprung's Disease

Symptoms of Hirschsprung's disease depend on the severity. Generally, symptoms can be detected since the baby is born, that is, the baby does not defecate (BAB) within 48 hours after birth.

Apart from babies not having bowel movements, other symptoms of Hirschsprung's disease in newborns are:

  • Vomit is brown or green
  • Growing belly
  • Fussy
  • Fever
  • Watery and foul-smelling diarrhea

In mild Hirschsprung's disease, new symptoms appear when the child is older. Symptoms include:

  • Easily tired
  • Stomach bloated and looks distended
  • Long-term (chronic) constipation
  • Loss of appetite
  • Not gaining weight
  • Developmental disorders

When to see a doctor

Immediately contact the doctor if your baby does not defecate within 48 hours after birth. Meanwhile, for older children, check with the pediatrician if the symptoms mentioned above appear.

Patients who are on medication or have undergone surgery to treat Hirschsprung's disease also need to undergo periodic examinations according to the doctor's recommendations, so that their condition can continue to be monitored.

Diagnosis of Hirschsprung's disease

The pediatrician will ask about the symptoms experienced by the child, then carry out a physical examination, including a digital rectal examination. If the patient is suspected of having Hirschsprung's disease, the doctor may perform additional tests, such as:

  • X
    -rays X- rays are taken to see possible blockages in the large intestine.
  • Anorectal manometry
    In this procedure, the doctor will use a special tool in the form of a balloon and a pressure sensor to check bowel function. This test is done on older children.
  • Biopsy
    The doctor will take samples of colon tissue which will then be examined under a microscope.

Treatment of Hirschsprung's Disease

Treatment of Hirschsprung's disease is a surgical procedure that is adapted to the child's condition. These methods are:

1. Bowel withdrawal procedure ( pull-through surgery )

In this procedure, the doctor removes the non-neuronal part of the large intestine, then pulls and connects the healthy intestine directly to the rectum or anus.

2. Ostomy

Ostomy is performed in children whose condition is unstable or born prematurely. In this procedure, the doctor cuts out the problematic part of the intestine, then directs the healthy intestine into a hole ( stoma ) made in the stomach. This hole serves as a way to get rid of feces.

Next, the doctor will attach a special bag to the stoma as a stool storage. If the patient's condition is stable and the colon is recovering, the doctor will close the hole in the stomach and connect the healthy intestine to the anus.

After surgery, the patient needs to stay in the hospital for a few days. During the treatment period, the intestine will generally recover gradually and can function normally again.

At the beginning of the recovery period, the child will feel pain when defecating. In addition, he can also experience constipation . To overcome this, you can take the following steps:

  • Giving children to drink sufficient amounts of water, to make the stool softer while meeting the body's fluid needs
  • Give children high-fiber foods, such as fruit and vegetables, when they can digest them
  • Consult a doctor about the right type of food to treat constipation in children who cannot digest food
  • Invite children to play, because body movements can help smooth the digestive system and increase bowel movements
  • Give laxatives by first consulting a doctor

Hirschsprung's disease complications

Hirschsprung's disease that is not treated can cause complications, such as:

  • Malnutrition and dehydration
  • Infection of the intestines ( enterocolitis ), which can be life threatening
  • Intestine rupture

After undergoing surgery, children are also at risk of having uncontrolled bowel movements ( fecal incontinence ).

Prevention of Hirschsprung's Disease

Hirschsprung's disease is difficult to prevent, because the cause is not known with certainty. However, if you have a family who suffers from this condition, you are advised to consult with your doctor before planning a pregnancy.

Last updated: March 10, 2022
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