Re: "Hard Stomach" or hirchsprung's disease

Posted By Suzy on January 15, 1999 at 19:32:59:

In Reply to: Re: "Hard Stomach" or hirchsprung's disease posted by HFHSM.D.-ym on December 29, 1998 at 12:02:44:






I'm trying to find information of hirchsprung's disease and can't find any.  I've also heard it called hard stomach.  I'm also trying to get information on infantile scolaosis.  Can anyone give me direction?  
Dear Peggy,
Congenital megacolon (megarectum) is a term used to describe the large intestine in patients with Hirschprung's disease. The dilated colon results from functional obstruction because of the congenital absence of the intramural neural plexuses (nerves). This is referred to as "aganglionosis". This results in a narrowed segment of  the affected area. The narrowing occurs because the aganglionic segment fails to relax. Therefore, dilatation of the colon occurs proximal to the aganglionic segment. The diagnosis is established by full thickness or suction  biopsy of the rectum. The biopsy specimens show the absence of ganglion (nerve) cells in the myenteric and submucosal plexuses (these are groups of nerves seen in gastrointestinal tissue). Another test that is available to suggest the diagnosis of Hirschprung's disease is anorectal manometry. In this test, the pressures of the internal and external anal sphincter are measured upon inflation and deflation of a rectal balloon. In Hirschprung's disease the internal anal sphincter does not relax with rectal distention.
This clinical condition usually becomes apparent shortly after birth when the infant passes very little meconium and the abdomen is distended. In older patients, severe constipation and recurrent fecal impactions are more common. The defect occurs in approximately one of each 5000 live births and is familial. It is much more common in males (male to female ratio 5:1). There is an association with Down's syndrome and a variety of other anomalies. Once the diagnosis is established, definitive surgical cure is the treatment of choice. Long-term results are good in the majority of patients. I hope you find this information helpful.
This response is being provided for general informational purposes only and should not be considered medical advice or consultation. Always check with your personal physician when you have a question pertaining to your health.
If you would like to be seen at our institution please call 1-800-653-6568, our Referring Physicians' Office and make an appointment to see Dr, Muszkat our expert in Gastroenterology.
HFHSM.D.-ym
*Keywords: Hirschprung's disease
Peggy, my friend suffers this disease, there is a wonderful website for this "gastroedge.org(?) or com.  Good luck