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- 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.
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- 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%.
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- 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.
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- 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.
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- It is not known at the present time
what the risk factors are, other than possessing the gene
that causes the disease.
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- 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.
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- 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.
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- There is no specific way to prevent
this disease.
- Cancers associated with this disease can be detected early through routine doctor visits.
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