Hirschsprungs Disease
What is Hirschsprung disease in children?
Hirschsprung disease is a very rare birth defect. It has an effect on the nerve cells of the large intestine. These nerve cells regulate the muscles that transport food and waste, or stool, through the large intestine.
Babies with Hirschsprung disease are missing nerve cells in all or part of the large intestine. In most cases, only the end parts of the colon are affected. The muscles cannot move food and waste through that section of the large intestine unless these nerve cells are present. Stool can’t move forward. It stays in the large intestine.
The intestine may become partially or completely obstructed. It starts to become larger than usual. This can cause constipation, swelling, pain, and infection.
What is the cause of Hirschsprung disease in children?
Nerve cells grow along the intestines of a newborn during pregnancy. They start in the mouth and end in the anus. The nerve cells in newborns with Hirschsprung disease do not expand beyond a particular point in the large intestine. The cause of this condition is unknown.
Which children are at risk for Hirschsprung disease?
A child is more likely to get Hirschsprung disease if the problem occurs in the family. The illness is also connected to several hereditary abnormalities, such as Down syndrome.
Hirschsprung disease is more common in males than in females.
What are the signs and symptoms of Hirschsprung disease in children?
The majority of newborns with Hirschsprung disease exhibit symptoms during the first few weeks of life. In certain situations, just a small portion of the intestine is damaged. The symptoms may not appear for months or years.
The symptoms of each child may differ. Among the symptoms that infants may experience are:
- Inability to have a bowel movement during the first 48 hours of life
- Slow swelling or bloating of the belly
- Constipation that gets worse over time
- Appetite loss
- Growth that is slow or delayed
- minimal, watery, bloody stools
- Loss of energy
Hirschsprung disease symptoms might mimic those of other medical conditions. Consult your pediatrician for a diagnosis.
How is Hirschsprung disease identified in children?
Your doctor will examine the child and gather a medical history. The doctor will inquire about constipation and bowel movements. Other tests may be performed to determine if your child has Hirschsprung disease. Among these tests are:
X-ray of the abdomen -A lack of stool in the large intestine or at the anus may be detected by this test. It may also reveal whether a section of the big intestine is bulging. The bulge is produced by a blocked stool.
Barium enema-This X-ray exam checks for any abnormalities in the large intestine. Your child will be given a metallic fluid known as barium. The organs are coated with barium so that they can be viewed on an X-ray. The barium is placed in a tube and administered as an enema into your child’s rectum. An X-ray of the abdomen will reveal any constricted or blocked regions. It will also reveal whether or not the gut is bulging over a blockage.
Anorectal manometry-0This test is often administered to older children. A small tube is inserted into the rectum to assess the effectiveness of the rectal muscles. If the muscles do not relax, Hirschsprung disease may be present.
Biopsy of the rectum or large intestine-A small section of the big intestine is removed. It is examined under a microscope to see if any nerve cells are absent.
What is the prognosis of Hirschsprung’s disease?
Children with Hirschsprung disease usually need surgery since the chances of long-term survival without treatment are low. Hirschsprung disease may be treated surgically in a number of ways. Common surgical techniques include:
Pull through surgery: In this procedure, the end of the intestine that does not include nerve cells is removed fully or almost completely, and the end of the colon that does contain nerve cells is linked to the anal opening. Stool emerges through the natural anal aperture after surgery. In some circumstances, the pull through surgery may be completed in a single procedure, but if the child is very unwell or the intestine is severely dilated, it is more typical to create a temporary ostomy.
Ostomy: A link between the bowel’s interior and the skin of the abdomen. An ileostomy is a connection between the end of the small intestine and the skin, while a colostomy is a connection to the colon. A small plastic bag is attached to the opening to collect the stool.
In most situations, the ostomy is a temporary solution to allow the child to develop, settle intestinal inflammation, or decrease the colon back to normal size before undergoing pull-through surgery. As long as the ostomy allows stool to bypass the region of the bowel without nerve cells, children typically function well with an ostomy
For more information & consultation on Hirschsprung’s Disease, Get in touch with Dr. Adwait Prakash a Pediatric Surgeon in Indore. will help you out in understanding your problem and guide you through every stage of your treatment.
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