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Hirschsprungs Disease


Hirschsprungs disease is an uncommon condition in boys. It is caused by a lack of ganglion cells in the end of the colon and rectum. The word bowel can refer to the large and small intestines. It usually occurs in children. Some children with It can't have bowel movements at all. The stool creates a blockage in the intestine. This causes constipation , which is the classic symptom of this disorder. Hirschsprung's disease occurs when some of the nerve cells that are normally present in the wall of the intestine do not form properly during fetal development. Many children with Hirschsprungs disease have a delay in passing meconium , or they develop chronic constipation later in the first month of life. This can cause an intestinal obstruction , which may then lead to abdominal distention, vomiting, poor feeding, and poor weight gain. Children who are suspected of having Hirschsprungs disease should be examined by their Pediatrician, who do a rectal exam, which should show normal tone and an absence of stool. When the finger is removed after the rectal exam, there is usually a large amount of loose stool that is explosively forced out. Normal intestinal motility depends on a coordinated contraction wave that pushes the nutrients down stream from mouth to anus.

Most parents feel frightened when they learn that their child has a serious disease. Patients with Hirschsprung's disease lack normal motility in the segment of bowel that has the Hirschsprung's. This results in a clinical picture that resembles either bowel obstruction, particularly in a newborn baby, or chronic constipation in older children. Some cases of Hirschsprung's disease can be related to a geneticcause. There is an increased chance that a couple will have a child with Hirschsprung's disease if one of the parents has the condition, and the chance is higher if it is the mother who has the condition. Sometimes the ganglion cells are missing from the whole large intestine and even parts of the small intestine before it. When the diseased section reaches to or includes the small intestine, it is called long-segment disease. When the diseased section includes only part of the large intestine, it is called short-segment disease.

Causes of Hirschsprungs Disease

Common causes of Hirschsprung Disease

  • Rare disorders.
  • Nerve cells grow down.
  • Down syndrome.
  • Anus.

Symptoms of Hirschsprungs Disease

Common symptoms of Hirschsprungs Disease

  • Constipation.
  • Swollen abdomen.
  • Vomiting green bile.
  • Fever.
  • Loss of appetite.
  • Delayed growth.
  • Diarrhea.
  • Anemia.

Treatment of Hirschsprungs Disease

Common Treatment of Hirschsprungs Disease

  • Pull-through Surgery is called a pull-through operation. There are three common ways to do a pull-through, and they are called the Swenson, the Soave, and the Duhamel procedures. Each is done a little differently, but all involve taking out the part of the intestine that doesn't work and connecting the healthy part that's left to the anus. After pull-through surgery, the child has a working intestine.
  • Most babies are more comfortable after having an ostomy because they can pass gas more easily and aren't constipated anymore.
  • In a small percentage of patients with short segment involvement of the colon, the entire procedure can be done through the anus, without entering the abdomen. When surgery is performed with this approach, recovery time can be even shorter.
  • The doctor leaves part of the large intestine and connects that to the stoma, the surgery is called a colostomy.

 

 

 

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Disclaimer :- The information contained in this web site is for educational purposes only and is not intended or implied to be a substitute for professional medical advice. It is not a substitute for professional medical advice. For specific medical advice, diagnoses, and treatment, please consult your doctor.