Hirschsprung: definition, symptoms, causes, treatment • hello healthy

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Definition

What is Hirschsprung?

Hirschsprung or hisprung is a congenital disorder in newborns that makes the colon has trouble passing feces. This condition is also called congenital aganglionic megacolon.

This condition appears from birth. This is because the nerve cells in the baby's large intestine, called ganglion cells, do not develop properly while the baby is still in the womb.

This abnormality in babies is usually seen within the first two months after birth. In milder cases, this condition may only be detected when it has entered childhood.

How common is this condition?

Hirschsprung or hisprung is a rare condition. Quoting from the U.S National Library of Medicine, it is estimated that 1 in 5,000 newborns experience hyssprung.

Hirschsprung can be treated by reducing risk factors. Talk to your doctor for more information.

Signs & Symptoms

What are the signs and symptoms of Hirschsprung?

The signs and symptoms of Hirschsprung's disease vary in the severity of the condition. Usually the signs appear after delivery, but sometimes they don't show up until later in life.

Generally, the most obvious sign of Hirschsprung is that the baby cannot defecate within 48 hours of being born. Even though normally, babies will pass meconium or their first feces at birth.

In addition, other symptoms of hisprung experienced in newborns are:

• swollen stomach and bloating in babies,
• the child vomits green or brown,
• constipation or difficulty having a bowel movement,
• gas in the stomach which can cause the baby to be fussy,
• babies and children have fever,
• difficulty in urinating,
• failing to excrete meconium after birth,
• frequency of bowel movements infrequently,
• jaundice,
• difficulty breastfeeding, and
  
• bad weight gain.

Babies with Hirschsprung can also experience diarrhea and enterocolitis or a life-threatening intestinal infection.

There are some children who only show symptoms of Hirschprung when they get older. The signs include:

• stomach swelling and bloating,
• constipation that gets worse,
• a stomach full of gas,
• delayed growth or failure to thrive in a child,
• fatigue,
• fecal impaction,
• malnutrition, and
  
• difficult to gain weight.

There may be signs and symptoms not listed above. If you have concerns about a certain symptom your little one is experiencing, consult your doctor.

When should I see a doctor?

Hirschsprung or hisprung is a congenital defect condition since newborns. If you see a baby having the symptoms above or other questions, consult a doctor immediately.

Hirschsprung's disease can become serious if left untreated, it is important to seek help as soon as possible.

Cause

What causes Hirschsprung?

Normally, as long as the baby develops in the womb, nerve cells are formed. These cells belong to the digestive system, which are formed from the esophagus, leading to the stomach, and ending in the anus.

A typical baby will have about 500 million types of nerve cells that form from the esophagus to the anus.

The number of nerve cells performs many functions, one of which is moving or flowing food from one part of the digestive system to another.

However, the process of forming these nerve cells is different in babies who have Hirschsprung or Hisprung.

The growth of nerve cells in babies with Hirschsprung stops at the end of the large intestine or just before the rectum and anus.

This is why newborns who have Hirschsprung's disease usually can't have a bowel movement after birth.

In some other babies, nerve cells can also be lost or stop growing in any part of the digestive system. The stopping of the growth of these nerve cells makes the feces that should come out stop at a certain point.

This makes stool stuck and difficult to pass so that it accumulates in the digestive system. As a result, the baby's intestine becomes blocked, making the stomach swollen and bloated.

According to Bostons's Children's Hospital, the cause of aganglion cells in babies with Hirschsrpung or hisprung is not certain.

However, the cause of Hirschsrpung or hisprung is thought to be due to genetic factors passed down from parent to child or there is a family history of it.

So, if one parent has a Hirschsprung or Hisprung condition, the chances of a baby being born with the same abnormality will certainly be higher.

For example, if one child in a family has Hirschsprung or Hisprung's condition, the siblings have a 3-12% chance of developing the condition.

Risk factors

What increases the risk of getting Hirschsprung?

There are several factors that increase the risk of a baby developing Hirschsprung. When there are siblings who have Hirschsprung or Hisprung, their chances of having this condition will be greater.

In addition, hisprung is usually more common in male babies than female babies.

This condition is also associated with birth defects, such as Down syndrome or congenital heart disease. It can be said that babies born with defects are more prone to experiencing hisprung.

Diagnosis & Treatment

The information provided is not a substitute for medical advice. ALWAYS consult your doctor.

How to diagnose this condition?

The pediatrician will perform a diagnosis of Hirschsprung or hisprung and an examination by asking about the frequency and habits of bowel movements of the child or baby

One or more tests that doctors can recommend to diagnose Hirschsprung or hisprung disease are as follows.

1. Abdominal x-ray examination using a contrast dye

The process of this X-ray or x-ray examination is done with barium or other contrast dye inserted into the intestine through a special tube inserted in the rectum. The barium fills and coats the lining of the intestine, resulting in a clear silhouette of the colon and rectum.

This X-ray or x-ray examination helps distinguish the part of the contrast between the normal intestine and the intestine without nerves that are swollen due to Hirschsprung or Hisprung.

2. Control the muscles around the rectum

Usually, doctors will perform manometric tests on older children and adults by inflating a balloon inside the rectum.

Under normal conditions, the muscles around the rectum should relax during the test. If what happens is better, this is the sign that the child has Hirschsprung or hisprung.

3. Take a sample of the colon tissue

Tissue sampling at the diagnosis of Hirschsprung or Hisprung is done for biopsy testing.

A biopsy sample can be taken through a suction device and then examined under a microscope to see the presence or absence of nerve cells related to Hirschsprung or hisprung.

What are the treatments for Hirschsprung?

Some are treatment options to treat babies who are exposed to hives.

1. Surgery cuts part of the intestine

This operation is performed by cutting or removing parts of the large intestine that do not have nerve cells. Next, the part of the large intestine that is normal or has nerve cells is pulled and connected to the child's anus.

Surgery to treat Hirschsprung or hisprung is usually done using the laparoscopic method, using a device with a small camera that is inserted into the child's digestive system.

2. Ostomy surgery

An ostomy to treat Hirschsprung or hisprung is a surgery that can be done in two stages.

First, the abnormal part of the colon is removed and the healthy upper colon is threaded through the hole the doctor made in the child's stomach.

The stool then leaves the body through an opening into a sac at the end of the intestine that protrudes through the opening in the stomach (stoma). This will allow the lower part of the colon to recover.

In the second stage of ostomy surgery to treat Hirschsprung or hisprung, the normal part of the intestine is then connected to the anus to close the stoma.

The ostomy procedure to treat Hirschsprung or hisprung includes:

• Ileostomy: The doctor removes the entire colon and connects the small intestine to the stoma. Stool leaves the body through the stoma into the sac.
• Colostomy: The doctor leaves part of the large intestine intact and attaches it to the stoma. Stool leaves the body through the end of the large intestine.

After ostomy surgery as a Hirschsprung or hisprung treatment, most children can have normal bowel movements.

Even so, there are some children who experience diarrhea at first. Teach toilet training or how to use the toilet to urinate and defecate may take longer.

This is because the child needs to learn to coordinate the muscles for defecating. In the long term, there is a chance that your child will experience continuing constipation, swelling of the stomach, or leaky stools as a result of medical procedures.

Children are still at risk of developing intestinal infections (enterocolitis) after ostomy surgery to treat Hirschsprung or hisprung, especially in the first year. Therefore, it is important that you pay attention to the various symptoms that appear in children. Immediately contact your doctor if your little one experiences symptoms of enterocolitis such as:

• rectum bleeding,
• diarrhea,
• fever,
• swollen stomach, and
  
• gag.

Do not delay to have it checked if these symptoms have appeared.

Home Remedies

What are some lifestyle changes or home remedies that can be done to treat Hirschsprung?

Here are the lifestyle and home remedies that can help you deal with Hirschsprung.

1. Provide high-fiber foods

If the child is already eating solid food, don't forget to provide high-fiber foods. Offer whole grains, fruits and vegetables and limit white bread and other low-fiber foods.

This is because a sudden increase in high fiber foods can worsen constipation at first, give high fiber foods slowly.

Meanwhile, if the child has not eaten solid food, ask the doctor for formula milk that can relieve constipation. Some babies may need a temporary tube to make it easier to eat.

2. Drink more

Ask the child to drink more water. This is because when part or all of the child's colon is removed, the child may have difficulty absorbing enough water.

Drinking more water can help your child stay hydrated, which will help relieve constipation.

3. Increase physical activity

If the child is old enough, invite him to increase physical activity in order to launch a bowel movement. Physical activities that can be done in children with Hirschsprung can include sports, playing, and others.

4. Giving a laxative from a doctor

The doctor may give a laxative to help smooth bowel movements in children with Hirschsprung.

However, this only applies if the child's condition does not improve despite increasing fiber intake, drinking water, and doing physical activity.

If you have any questions, consult your doctor for the best solution to your problem.