by Kimberley Allen R.N.
Hirshchsprungs disease is a disorder of the colon that developes during fetal development. It occurs because certain nerve cells
called nueral crest cells that control muscle movement in the large intestine do not fully grow during fetal development. Early in fetal development these nerve cells begin to grow in the babies intestine. They grow starting at the top and gradually moving down the intestine to the rectum. In Hirschsprungs disease the nerve cells stop moving or growing down the intestine leaving a portion without nerve cells to create muscle function. It is not caused by something that the mother does.
Hirschsprungs disease is an inherited disease that is considered rare occuring approximately 17 in 100,000 births. It has been found to occur more commonly in males than females and is more prevelant in whites than non-whites.
Though the reason why the nerve cells stop growing is unknown there are certaian risk factors that are known to increase the risk for developing Hirschsprungs disease. Since Hirschsprungs disease is an inherited condition if one sibling has it the risk that other siblings will also have it increasess. There are certaian other inherited conditions that increase the risk of Hirschprungs disease like Down syndrome and congenital heart defects. Approximately 9% of children with Hirschsprungs disease also have Down syndrome. There has also been indications that it may be associated with a condition known as multiple endocrine neoplasia type IIB.
The symptoms of Hirschsprungs disease varies depending on how much of the colon is affected and how severly is is affected. In some cases Hirschsprungs disease is suspected when a newborn doesn’t pass their first bowel movement known as muconium with in the first 48hours. Newborns also frequently vomit the green liquid known as bile after eating. Their abdomens become bloated from constipation and gas making them fussy. If the newborn has a milder form of Hirschsprungs
disease the symptoms may not manifest until the child is older. When questioned it is usually discovered that the child has had problems with constipation since birth. Most will usually have anemia as well due to blood lost in stool.
The standard treatment for Hirschsprungs disease is surgery. The surgery involves removing the portion of the colon that is damaged and pulling the undamaged portion down to the rectum and securing it, this
is called a “pull-through” There are 3 types of pull-through methods, the swenson, the soave, and the duhamel proceedures. They are all similar and your Dr will discuss which one is appropriate for your child. Most of the time the surgery is a one step proceedure that can be done soon after diagnosis. However, in children that have been very ill a temporary colostomy may be needed to help the child get healthy before having the pull-through surgery. Some Drs prefer to do a colostomy on all children prior to pull-through surgery to insure the colon has had time to heal and become healthy. Colostomies can be
very intimidating especially in children, it’s important to research and voice your concerns when talking with your Dr to ensure the best outcome for your child.
Kimberly Allen is a registered nurse with an AND in nursing. She has worked in ACF, LCF and psychiatric facilities, although she spent most of her career as a home health expert. She is now a regular contributor to HealthAndFitnessTalk.com, dispensing advice and knowledge about medical issues and questions. You can reach her with any comments or questions at firstname.lastname@example.org.