Hirschsprung Disease in Babies

Altering poopy diapers is among those undesirable tasks of parenting that most people are able to joke about. However when your baby is not able to poop, this subject is no laughing matter.

Babies who have difficulty emptying their bowels in some cases have a problem called Hirschsprung disease. Treatment for this condition usually requires surgery. Fortunately, a lot of children who have surgery are fully treated and able to pass defecation usually.

Hirschsprung disease takes place when some of the nerve cells that are generally present in the wall of the intestine do not form appropriately during fetal development.

About Hirschsprung Disease

Hirschsprung (HERSH-sproong) disease impacts the large intestine (colon) of babies, babies, and toddlers. The condition– which avoids bowel movements (stool) to pass through the intestinal tracts due to missing nerve cells in the lower part of the colon– is caused by an abnormality. Most of the time, the problems with pooping start at birth, although in milder cases symptoms may appear months or years later.

Hirschsprung disease can cause constipation, diarrhea, and vomiting and sometimes cause severe colon complications, like enterocolitis and toxic megacolon, which can be deadly. So it’s important that Hirschsprung disease be identified and treated as early as possible.

Causes of Hirschsprung Disease

The large intestinal tract relocations absorbed product through the gut by a series of contractions called peristalsis. This is controlled by nerves in between the layers of muscle tissue in the intestinal tract.

Children who have Hirschsprung disease are missing out on those nerves along part of the length of their colons. This avoids the colon from relaxing, which can cause a clog of absorbed product and make it tough to pass stool.

Hirschsprung disease can affect the entire big intestinal tract, which is called long-segment disease, or it can affect a shorter length of the colon closer to the rectum (short-segment disease). It’s more common for afferent neuron to stop developing closer to the anus, because in the womb cells develop on a pathway that starts at the top of the big intestinal tract and ends at the end, near the anus. (So, with Hirschsprung disease, nerve cells simply stop developing while on that path.).

Physicians aren’t completely sure why some children get Hirschsprung disease, but they do know it can run in families and affects boys regularly than girls. In fact, Hirschsprung disease has to do with five times more common in males than women. Children with Down syndrome and genetic heart disease also have an increased risk of Hirschsprung disease.

Hirschsprung Disease in Babies

Symptoms of Hirschsprung Disease

The symptoms of Hirschsprung disease may differ depending upon the seriousness of the condition. Children with severe cases usually will have symptoms within the first few days of life. Milder cases might not be identified until later.

Babies with Hirschsprung disease may show these signs:

  • inability to pass stool within the first or 2nd day of life.
  • swollen belly, bloating, or gas.
  • diarrhea.
  • vomiting, which may include vomiting a green or brown substance.

Inability to poop within the first Two Days of life is often the secret to discovering Hirschsprung disease in a newborn. This warning can be really important in detecting the condition.

Less severe cases of Hirschsprung disease may go undetected until later youth, or sometimes even teenage years or their adult years. The symptoms in these cases are normally milder however can be lasting, chronic conditions. Symptoms might consist of:

  • abdominal swelling.
  • constipation.
  • trouble gaining weight.
  • vomiting.
  • gas.

Due to the fact that Hirschsprung disease can affect the body’s ability to soak up nutrients, growth may be delayed in older kids who have it.

Diagnosis

To detect Hirschsprung disease, medical professionals frequently do a test called a barium enema. Barium is a dye that is put into the colon using an enema. The barium shows up better on X-rays and can help physicians get a clearer picture of the colon. (In kids with Hirschsprung disease, the intestine typically appears too narrow where the nerve cells are missing.).

In many cases, the doctor may do a rectal suction biopsy. This test, which can often be carried out in the workplace, involves utilizing a suction device to get rid of some cells from the colon’s mucous lining. This test will expose whether afferent neuron are missing and can assist make the diagnosis of Hirschsprung disease.

For older kids, physicians might use different tests, such as manometry or a surgical biopsy. Manometry is a test in which a balloon is inflated inside the anus to see if the anal muscle unwinds as an outcome. If the muscle does not unwind, the child might have Hirschsprung disease. In a surgical biopsy, the doctor removes a sample of tissue from the colon to take a look at under a microscope.

Treatment for Hirschsprung Disease

Surgery is believed to be the most effective treatment for Hirschsprung disease. This can be carried out in one step or 2, depending on the condition’s intensity. Children who are extremely sick at the time of surgery (due to an irritated colon or poor nutrition) may need to go through surgery in two steps.

The most common surgery to remedy Hirschsprung disease includes removing the section of the colon without nerves and reattaching the staying portion of the colon to the anus. Frequently, this can be done through minimally invasive (laparoscopic) surgery immediately after the condition is diagnosed.

Sometimes, the doctor might do the surgery in two actions. In the primary step, the doctor will get rid of the unhealthy portion of the colon and then carry out a procedure called an ostomy. In an ostomy, the doctor develops a little hole, or stoma, in the child’s abdomen and connects the upper, healthy part of the colon to the hole.

The two types of ostomy are:

  • Ileostomy: eliminating the entire big intestinal tract and connecting the small intestine to the stoma.
  • Colostomy: eliminating simply a part of the colon.

The child’s stool goes through the stoma into a bag that is linked to it and has to be cleared a number of times a day. This enables the lower part of the colon to heal prior to the 2nd surgery. In the second surgery, the doctor closes up the hole and connects the normal portion of the colon to the rectum.

After surgery, kids often get constipated. Laxatives can offer some relief, however consult your doctor about which would be best for your child if you decide to use them. For children old enough to eat solid foods, a high-fiber diet can alleviate and prevent constipation. Drinking lots of water is likewise crucial, and assists prevent dehydration. The big intestine assists take in water from food, so dehydration can be an issue for children who have had part of their intestine eliminated.

Kids who continue to have symptoms or develop brand-new ones after surgery (such as explosive and watery diarrhea, fever, a swollen belly, or bleeding from the rectum) must have medical attention right now. These can be signs of enterocolitis, a swelling of the intestines.

Outlook

The general outlook for children who have actually been dealt with surgically for Hirschsprung disease is excellent. After surgery, most can pass stool normally and have no long lasting complications. However, a couple of kids may continue to have symptoms, consisting of constipation and bowel control problems.

 

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