Hirschsprung's disease - Symptoms, causes and treatment

Hirschsprung's disease is disturbance in the large intestinecause feces or feces trapped in the intestines. Disease congenital which is quite rare can resulting in the baby not defecate (CHAPTER) since birth.

Hirschsprung's disease is caused by a disorder of the nerves that control bowel movements. This causes the large intestine to not be able to push feces out, so that it accumulates in the large intestine and the baby cannot defecate.

Although generally it can be known since the newborn, Hirschsprung's disease symptoms can also only appear after the child is older, if the abnormality is mild.

Causes of Hirschsprung's Disease

Hirschsprung's disease occurs when the nerves in the large intestine do not form properly. This nerve controls the movement of the large intestine. Therefore, if the nerves of the colon are not formed properly, the large intestine cannot push feces out. As a result, feces will accumulate in the large intestine.

The exact cause of this nerve problem is not known. However, there are several conditions that are thought to increase the risk of incomplete formation of the nerves of the colon, including:

  • Male gender.
  • Has a sibling who has Hirschsprung's disease.
  • Have parents, especially mothers, who have had Hirschsprung's disease.
  • Suffering from other inherited diseases, such as Down syndrome and congenital heart disease.

Symptoms of Hirschsprung's Disease

Hirschsprung's disease has different symptoms, depending on the severity. Symptoms generally can be detected since the newborn, where the baby does not defecate (BAB) within 48 hours after birth.

In addition to the baby not defecating, below are other symptoms of Hirschsprung's disease in newborns:

  • Vomiting with brown or green fluid
  • Distended stomach
  • Fussy

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

  • Easy to feel tired
  • Stomach is bloated and looks distended
  • Constipation that occurs in the long term (chronic)
  • Loss of appetite
  • Not gaining weight
  • Disrupted growth and development

When to go to the doctor

  • Immediately contact your pediatrician or pediatrician, gastro-hepatologist, if your baby does not defecate within 48 hours after birth. As for older children, immediately consult a pediatrician if the symptoms mentioned above appear.
  • Patients who are on medication or have undergone surgery to treat Hirschsprung's disease also need to have regular checkups as recommended by doctors, so that their condition can be continuously monitored.
  • If after surgery, the stitches bleed again or there are signs of infection such as fever, swollen stitches, or discharge of pus, go back to the doctor immediately.

Hirschsprung's Disease Diagnosis

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

  • X-Ray Photon

    X-rays are done to see the condition of the colon more clearly. Previously, a special dye made from barium would be inserted into the intestine through a tube that was inserted from the rectum.

  • Test measuring intestinal muscle strength

    In this procedure, the doctor will use a special instrument in the form of a balloon and a pressure sensor to check bowel function.

  • Biopsy

    The doctor will take a sample of colon tissue, which will then be examined under a microscope.

Hirschsprung's Disease Treatment

Hirschsprung's disease is a serious condition that requires immediate surgery, either laparoscopic or open surgery. Patients whose condition is stable usually only require one operation, namely bowel retraction surgery.

If the patient's condition is unstable, or when the patient is a premature baby, has a low birth weight, or is sick, an ostomy is usually required, to reduce the risk of complications.

bowel withdrawal procedurepull-through surgery)

In this procedure, the doctor will remove the inner portion of the large intestine that is not supplied with nerves, then withdraw and attach the healthy intestine directly to the rectum or anus.

Ostomy procedure

This procedure is carried out in 2 stages. The first stage is cutting the part of the patient's intestine that is problematic. After the bowel is cut, the doctor will direct the healthy intestine into the new opening (stoma) created in the abdomen. The hole is a substitute for the anus to dispose of feces. This procedure is also known as a colostomy.

Next, the doctor will attach a special bag to the stoma. The bag will hold the feces. When it is full, the contents of the bag can be thrown away.

After the patient's condition is stable and the colon has started to recover, the second stage of the ostomy procedure can be performed. This second stage is done to close the hole in the stomach and connect the healthy intestine to the rectum or anus.

After undergoing the surgical procedure, the patient will be hospitalized for several days, while being given an intravenous drip and given pain medication until his condition improves. During the treatment period, the intestine will gradually recover until it can function normally again.

Early in the recovery period, older children may feel pain when they have a bowel movement. While younger children, will be fussy when defecating. In addition, patients can also experience constipation. In dealing with constipation, patients need to:

  • Get enough water intake

    Adequate water intake is useful for making stools softer, while meeting the body's fluid needs.

  • Eat foods that are high in fiber

    Give fruit and vegetables to children who can already digest them. If not, ask your pediatrician, what foods can be given to children to treat constipation.

  • Invite to play

    Movement of the body can help smooth the digestive system and improve bowel movements.

  • Taking laxatives according to doctor's instructions

    The benefits and risks of using laxatives need to be discussed with your pediatrician first.

Complications of PHirschsprung disease

Children with Hirschsprung's disease are at high risk of developing intestinal infections.enterocolitis), which can be life threatening. Not only from the disease, surgery to treat this disease can also cause complications. Complications that can occur after a patient undergoes surgery include:

  • The appearance of a small hole or tear in the intestine
  • Fecal incontinence
  • Malnutrition and dehydration
  • Megacolon