What is Hırschsprung’s Disease? What Are the Symptoms of Hırschsprung?

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Hirschsprung’s disease is a disease that involves the nervous system of the intestine, but it is a disease that occurs when the lower rectum is not sufficient to remove stool from the anus, and over time it causes the formation of an abnormally large or enlarged large intestine.

This disease should be considered mostly in infants or children who cannot poop without auxiliary stimuli during their infancy and later periods, and who have a delay in removing the poop called meconium within the first 24 hours after birth.

Hirschsprung’s disease is also known as congenital enlargement of the large intestine.

Hirschsprung disease, a rare disease in premature infants, accounts for 33% of large bowel obstruction in newborns.


What Are the Symptoms of Hirschsprung's Disease? How Is Hirschsprung Disease Diagnosed?

Rectal biopsy is the most effective and best method that can be used in the diagnosis of an abnormally sized large intestine in a newborn baby after birth.

After a rectal examination, it is observed that the newborn baby defecates explosively, so to speak. In some cases, it has been observed that the baby even vomits feces.

In addition to all these hirschsprung symptoms, weight loss and water loss are also observed. Many newborns may even show signs of constipation and diarrhea.

  • One of the first signs in newborn babies should be noted that the first defecation is problematic (failure to pass Mechanium stool). Some children cannot go out at all and are only relieved by enemas.
  • Swelling in the abdomen
  • vomiting
  • Inability to defecate
  • Biliary vomiting in newborn babies
  • Abdominal swelling
  • Inability to extract meconium
  • Radiology-verified bowel obstruction
  • Bad-smelling and sometimes bloody diarrhea
  • Growth problems
  • Unexplained fever

All of the above-mentioned symptoms can start with high fever and result in serious consequences in a short time with hypovolemic shock, septicemia, intestinal perforation.

hirschspprung disease, which is mostly a disease of healthy term infants, can be seen with 5% down syndrome, 5% of genitourinary system anomalies, rarely cardiac anomalies, digestive system atresia, diseases of neuroectodermal origin, CNS anomalies.

As the most typical symptom, defecations in the form of bursting or gushing can be shown. Depending on the symptoms, necessary examinations are made with radiological techniques, and then the definitive diagnosis is made by rectal biopsy.

What Are the Symptoms of Hirschsprung's Disease in Newborns?

  • Green or brown vomit
  • Abdominal swelling
  • Diarrhea (usually bloody diarrhea)
  • Chronic constipation
An early symptom in some newborns is the failure of the first bowel movement within 48 hours after birth.

What Causes Hirschsprung's Disease?

During the early development of the baby placed in the mother’s womb, hirschsprung’s disease occurs because the growth of nerve cells stops towards the end of the intestine.

In the normal process, the growth of nerve cells in the relevant region starts at the beginning of the intestine and continues to grow towards the end. However, in the course of hirschsprung’s disease, it was determined that the growth of these cells could not reach the child’s intestine.

In the opinion of scientists, existing genetic defects increase the probability of a child having Hirschsprung disease.

In addition, there is no test that is capable of diagnosing this disease while the baby is in the mother’s womb, that is, during pregnancy. Studies of scientists are still continuing in order to obtain data on this subject.

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How Is Hirschsprung's Disease Treated?

Hirschsprung’s disease is a life-threatening disease and requires surgical treatment. After the surgery for Hirschsprung’s disease, the quality of life of children who undergo this surgery increases and they feel better.

The symptoms of the side effects brought by the disease are reduced and a visible recovery process is obtained. As in many diseases, Hirschsprung disease affects patients in infancy and childhood to a greater extent than other individuals.

Since it is a life-threatening disease, it is a disease that can even result in death if the necessary treatments are not applied. A rectal biopsy is performed when diagnosing Hirschsprung’s disease.

The diagnosis of the disease is made after a rectal biopsy. After the diagnosis is confirmed, the aganglionic part is removed with a single-stage transanal endorectal pull-through operation, and the ganglion-containing intestine is connected to the anus.

During treatment of Hirschsprung’s disease, a pediatric surgeon will perform a transition procedure or an ostomy surgery. During both procedures, the surgeon may remove all or part of the colon, called a colectomy.

In case of possible complications or in delayed cases, a temporary colostomy may be required at the first stage of the 3-stage surgical treatment. Depending on this, closed or open surgical methods may be required.

Even if there is a patient who has successfully completed every stage of the surgery, he or she should be followed up with a maximum of 3 year intervals. During this process, diet and toilet training should be specially planned and followed.

Most cases with Hirschsprung’s disease require surgery to treat. The intestinal part, which has shrunk due to its size outside the normal, is removed with the help of surgical intervention and the upper intestine is sutured to the lower rectum. Surgical interventions generally proceed in this way.

In the treatment of Hirschsprung’s disease, an exit is made into the inner part of the abdomen so that the stool can be filled into a disposable bag. Although this is only a temporary measure, this opening continues throughout the 12 to 18 months of the child’s life. After the completion of this process, the opening is closed with another operation.

It is very important to fully meet the energy and nutrients needed in infants and children, especially in the growth and development period, during the treatment applied, since babies and children may become tired during this process. For this reason, enteral and parental nutritional support should be used if deemed necessary by the doctor.

Withdrawal in the Treatment of Hirschsprung's Disease

During the operation of Hirschsprung’s disease, the surgeon, who is responsible for the operation and following the process, removes the missing part of the nerve cells of the large intestine and connects the healthy part to the anus.

Ostomy Surgery in the Treatment of Hirschsprung's Disease

In the ostomy surgery performed in the treatment of Hirschsprung’s disease, after removing a part of the intestine, it is the process of directing the stool to be removed normally from the body and to maintain its normal movement.

In other words, ostomy means passing a part of the disturbed intestine through the abdominal wall. In this way, feces leave the body from outside the anus, not from inside the anus.

The opening in the abdomen through which stool is expelled is called a stoma. After these procedures, the surgeon in charge of the operation ties an ostomy layer to the outside of the opening for the accumulation of waste from the intestine.

Ostomy surgery is mostly a temporary measure until you are ready for the extraction procedure. Although ostomy is a temporary treatment method, after the feces in the diseased part are thrown out of the body, extraction surgery is performed and then the treatment is terminated.

In general, the individual who is treated after most surgical operations can lead a healthy and normal life. In some individuals, it may be necessary to continue their treatment for a while because they do not show full recovery.

After ostomy surgery performed in the treatment of Hirschsprung’s disease, it is possible to experience problems such as constipation, diarrhea or urinary incontinence in children. If you or your child shows such symptoms, you should contact a health care provider as soon as possible.

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