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Gardner's Syndrome

more about Gardner's Syndrome

  • Gardner's Syndrome is an inherited condition that leads to the formation of multiple polyps throughout the intestinal tract.  There is no specific treatment for this disease other than surgery to prevent the development of cancer.  Fortunately, Gardner's Syndrome is quite rare.
  • Polyps are growths that develop within the intestines.  They are quite common and many people will have a few in the colon, especially as they get older.  People with this disease, however, have many polyps (sometimes hundreds to thousands) and the polyps develop at an earlier age, usually by age 16.  They may occur throughout their stomach, small intestine, and large intestine, and they have a high potential for becoming cancerous.  There is no specific treatment other than close follow-up and surgery to prevent cancer.  Any complications that may arise will need to be treated accordingly.

  • There are no specific symptoms.
  • If the polyps become very large, they may cause intestinal bleeding, Intussusception, or Intestinal Blockage.  Otherwise, they may not cause any symptoms.
  • People with this disease also have a higher risk of developing dermoid tumors, abnormalities of the retina, skin tumors, and bone tumors.
  • The intestinal polyps usually appear by the early to mid teenage years.  By age 35, almost all with this disease will have polyps.
  • Polyps in the colon will become cancerous in almost everyone with this disease by age 50.
  • Polyps are found in the stomach in over half of those affected with this disease. However, stomach polyps usually do not become cancerous.
  • Polyps are also found in the small intestine in over 90% of those with this disease, and tend to become cancerous in up to 10 to 15%.

  • This is an inherited disease (autosomal dominant inheritance). A gene known as the APC (adenomatous polyposis coli) is present in over 80% of those with this disease.  It is believed that this genetic abnormality leads to the development of the numerous polyps.  However, it is still not known what exactly causes the genetic abnormality.

  • Usually determined when multiple polyps are found during an endoscopy being done for other reasons.
  • The polyps may also be found during surgery for some unrelated problem, or because of a complication of the disease.
  • Polyps usually develop at a young age, often as early as the early teens.  By 35, almost all individuals with this disease will have polyps.
  • This condition has a very strong pattern of inheritance.  Close relatives of anyone with this disease should be tested for the presence of abnormal polyps.  Though there is no treatment or cure at this time, close monitoring by a doctor may help prevent some of the complications. 
  • Once a person is found to have the genetic abnormalities associated with this disease, he/she needs to be followed extremely closely.  That person will need to have a Sigmoidoscopy starting at age 10, and repeat Sigmoidoscopy every 1 to 2 years.
  • Upper endoscopy should also be done (in people with the abnormal gene) to look for polyps in the stomach and small intestine.  This needs to be started at the same time as the Sigmoidoscopy, and repeated every 1 to 3 years.

  • It is not known at the present time what the risk factors are, other than possessing the gene that causes the disease.

  • There is no specific treatment for this disease.
  • People with this disease have a very high risk of developing cancer.  In fact, almost all with this disease will develop cancer by the time they turn 50.
  • Therefore, those with this disease need to be checked and screened very carefully and very often for any abnormalities.
  • Also, all persons with this condition will need to have the entire colon removed.  This is a preventive measure done to prevent cancer.  Depending on the type of surgery, sigmoidoscopies will be needed afterward to make sure no cancer develops in remaining parts of the intestine.  This will be determined by one's primary care doctor and the surgeon who performed the surgery.
  • Periodic upper endoscopy is used to make sure that cancer does not develop in the stomach or intestine, and will need to be continued even after the colon is removed.

  • If the polyps become very large, they can cause Intestinal Blockage, Intussusception, or bleeding.
  • People with this disease are at a very high risk of developing cancer.

  • Seek medical attention as soon as possible.
  • Once the diagnosis is made, other family members should also be told to see their doctors and seek genetic testing and counseling.

  • There is no specific way to prevent this disease.
  • Cancers associated with this disease can be detected early through routine doctor visits.

more about Gardner's Syndrome

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