Digestive System Disorders: Hirschsprung's Disease
Hirschsprung's Disease
What is Hirschsprung's Disease?
Hirschsprung's Disease, also known as aganglionic megacolon or congenital megacolon, is a disease of the large intestine that usually occurs in children. It is an abnormality that develops before the child is born where the nerve cells in the large intestine stop growing to the end of the digestive tract. In a normal large intestine, nerve cells known as ganglion cells, are located at the last part of the small intestine to the end of the large intestine. Ganglion cells are responsible for making the muscles in the large intestine push waste to the anus where it leaves the body. With these important cells missing, a person with Hirschsprung's suffers constipation while other people with the disease are unable to have a bowel movement at all. The stool creates a blockage in the intestine and if not treated, can fill the large intestine and cause infection, a bursting of the colon and even death. Even though the cause is unknown, it is known that the disease is not caused by the mother's habits and that it can sometimes be heredity. Hirschsprung's Disease is also seen in association with some chromosome abnormalities, such as Down's Syndrome and other rare disorders. It can be either a short segment disease, meaning only part of the colon, or a long segment disease, meaning the diseased section has reached to, or has included, the small intestine.
What Are The Symptoms I Should Look For In My Child?
The symptoms of Hirschsprung's Disease occur mostly in infants in the first 6 weeks of life or they can occur later in infancy or childhood depending on the extent of the disease. Symptoms may vary within individuals; however, the most common symptoms are:
* Not being able to pass stool in the first 48 hours of life
* Vomiting
* Bloating of the abdomen
* Constipation
* Fever
* Poor weight gain
* Loss of appetite
* Passing small, watery stools
* Malabsorbtion
These symptoms may also resemble other medical conditions that need prompt attention and therefore it is advised that you take your child to see their pediatrician for an adequate diagnosis.
What Kind Of Tests Do Doctors Use To Diagnose Hirschsprung's Disease?
The physician will examine the child and obtain a medical history then other tests may be done. These test may include an abdominal x-ray, a barium enema, an anal manometry and a biopsy of the large intestine or rectum.
* Abdominal x-rays show a picture of the bowel and the amount of stool in the bowel.
* Barium enemas are procedures in which barium is given into a rectum as an enema. Barium is a metallic, chemical, chalky, liquid used to coat the inside of organs so that they will show up on an x-ray. After the enema is given, an x-ray is taken to show abnormalities such as blockages, dialated intestine above the blockages or narrowed areas.
* An anal manometry is a balloon study in which a balloon type device is inserted into the rectum. This device is used to enlarge the anus to test the nerve reflexes which would be missing in Hirschsprung's Disease.
* Biopsies of the large intestine and rectum, sometimes performed under sedation, are tests that take samples of cells from the large intestine or rectum. These samples are put under a microscope for further examination.
What Is The Treatment For Hirschsprung's Disease?
Treatment for Hirschsprung's Disease depends on the medical history, overall health, and the extent of the problem as well as the child's age. Other factors include the child's tolerances for certain procedures, medications or therapies as well as expectations for the course of the disorder. Surgery is the only treatment for Hirschsprung's Disease where the abnormal section of the bowel is removed; such as the case with short segent disease. This procedure, called a Bowel Reconstruction, is done in two stages. The first stage consists of creating a colostomy. A colostomy brings a portion of the large intestine through the abdominal wall to carry feces out of the body. The bowel contents are therefore discharged into a waste bag. After the child is healthy enough, the second stage of the procedure is performed to close the colostomy, remove the diseased portion of bowel, and to connect the healthy bowel to the anus. Colostomies in this case can be temporary or permanent; depending on how much the bowel is affected. If it is a long segment disease, then an ileostomy is performed. The entire large intestine is removed and a portion of the small intestine is brought out through the abdominal wall to carry feces outside the body. Like a colostomy, the waste of an ileostomy is also discharged into a specially made waste bag.
The prognosis after treatment is very good in children with Hirschsprung's Disease. With the diseased portion gone, they go on to lead normal healthy and happy lives.
Technorati Tags: Hirschsprung's Diseasetis,
Digestive System Disorders
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What is Hirschsprung's Disease?
Hirschsprung's Disease, also known as aganglionic megacolon or congenital megacolon, is a disease of the large intestine that usually occurs in children. It is an abnormality that develops before the child is born where the nerve cells in the large intestine stop growing to the end of the digestive tract. In a normal large intestine, nerve cells known as ganglion cells, are located at the last part of the small intestine to the end of the large intestine. Ganglion cells are responsible for making the muscles in the large intestine push waste to the anus where it leaves the body. With these important cells missing, a person with Hirschsprung's suffers constipation while other people with the disease are unable to have a bowel movement at all. The stool creates a blockage in the intestine and if not treated, can fill the large intestine and cause infection, a bursting of the colon and even death. Even though the cause is unknown, it is known that the disease is not caused by the mother's habits and that it can sometimes be heredity. Hirschsprung's Disease is also seen in association with some chromosome abnormalities, such as Down's Syndrome and other rare disorders. It can be either a short segment disease, meaning only part of the colon, or a long segment disease, meaning the diseased section has reached to, or has included, the small intestine.
What Are The Symptoms I Should Look For In My Child?
The symptoms of Hirschsprung's Disease occur mostly in infants in the first 6 weeks of life or they can occur later in infancy or childhood depending on the extent of the disease. Symptoms may vary within individuals; however, the most common symptoms are:
* Not being able to pass stool in the first 48 hours of life
* Vomiting
* Bloating of the abdomen
* Constipation
* Fever
* Poor weight gain
* Loss of appetite
* Passing small, watery stools
* Malabsorbtion
These symptoms may also resemble other medical conditions that need prompt attention and therefore it is advised that you take your child to see their pediatrician for an adequate diagnosis.
What Kind Of Tests Do Doctors Use To Diagnose Hirschsprung's Disease?
The physician will examine the child and obtain a medical history then other tests may be done. These test may include an abdominal x-ray, a barium enema, an anal manometry and a biopsy of the large intestine or rectum.
* Abdominal x-rays show a picture of the bowel and the amount of stool in the bowel.
* Barium enemas are procedures in which barium is given into a rectum as an enema. Barium is a metallic, chemical, chalky, liquid used to coat the inside of organs so that they will show up on an x-ray. After the enema is given, an x-ray is taken to show abnormalities such as blockages, dialated intestine above the blockages or narrowed areas.
* An anal manometry is a balloon study in which a balloon type device is inserted into the rectum. This device is used to enlarge the anus to test the nerve reflexes which would be missing in Hirschsprung's Disease.
* Biopsies of the large intestine and rectum, sometimes performed under sedation, are tests that take samples of cells from the large intestine or rectum. These samples are put under a microscope for further examination.
What Is The Treatment For Hirschsprung's Disease?
Treatment for Hirschsprung's Disease depends on the medical history, overall health, and the extent of the problem as well as the child's age. Other factors include the child's tolerances for certain procedures, medications or therapies as well as expectations for the course of the disorder. Surgery is the only treatment for Hirschsprung's Disease where the abnormal section of the bowel is removed; such as the case with short segent disease. This procedure, called a Bowel Reconstruction, is done in two stages. The first stage consists of creating a colostomy. A colostomy brings a portion of the large intestine through the abdominal wall to carry feces out of the body. The bowel contents are therefore discharged into a waste bag. After the child is healthy enough, the second stage of the procedure is performed to close the colostomy, remove the diseased portion of bowel, and to connect the healthy bowel to the anus. Colostomies in this case can be temporary or permanent; depending on how much the bowel is affected. If it is a long segment disease, then an ileostomy is performed. The entire large intestine is removed and a portion of the small intestine is brought out through the abdominal wall to carry feces outside the body. Like a colostomy, the waste of an ileostomy is also discharged into a specially made waste bag.
The prognosis after treatment is very good in children with Hirschsprung's Disease. With the diseased portion gone, they go on to lead normal healthy and happy lives.
Technorati Tags: Hirschsprung's Diseasetis,
Digestive System Disorders
IceRocket Tags: Hirschsprung's Disease, Digestive System Disorders
posted by Tim at
11:31 AM
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