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Genetic and Rare Diseases Information Center (GARD)

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Attenuated familial adenomatous polyposis


Other Names for this Disease
  • AAPC
  • AFAP
  • Attenuated adenomatous polyposis coli
  • Attenuated familial polyposis coli
  • Attenuated FAP
More Names
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Your Question

What is the recommended follow up or management of AFAP after the colon has been removed? The small intestine is connected to the rectum. I have had polyps in the rectum and also in the stomach since the colon was removed. How often do I need to do follow up tests to make sure nothing is growing in there?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

How might attenuated familial adenomatous polyposis be treated?

Management of AFAP is generally based on continued surveillance to detect if and when the formation of polyps occurs.[1] Screening by colonoscopy and endoscopy has been recommended for affected individuals starting at the age of 20 to 25 years. Individuals with polyps may undergo polypectomy (removal of polyps), followed by continued yearly surveillance. Individuals with too many adenomas to remove during endoscopy, or those who cannot undergo surveillance, may consider a prophylactic colectomy (removal of all or part of the colon).[2] About one third of individuals with AFAP have few enough colon polyps that surveillance with periodic polypectomy is sufficient.[3] Because individuals with AFAP can also develop duodenal adenomas and other cancers, upper endoscopy is typically recommended starting at age 20 to 30 years and then every one to three years, depending on the number of polyps. There is currently no consensus on screening for tumors that occur outside of the colon, so it has been suggested that affected individuals are managed as if they have classic FAP.[2] A number of drugs such as celecoxib and sulindac reportedly have been successful at reducing the number and the size of polyps in affected individuals, but these drugs generally help to prevent further complications and are not considered sufficient treatment.[1]

Additional and more detailed information about the treatment and management of FAP, including AFAP, is available on eMedicine's Web site and can be viewed by clicking here.
Last updated: 12/16/2011

What is the recommended follow-up for attenuated familial adenomatous polyposis after the colon has been removed?

Although there is sufficient literature about post-surgery recommendations for individuals with FAP, there is limited available literature specific to AFAP; many experts have suggested that individuals with AFAP adhere to the screening guidelines for FAP. For individuals known to have APC-related polyposis conditions, recommendations have included:
  • If total colectomy with ileo-anal pull-through was performed: routine endoscopic surveillance of the ileal pouch every two years
  • If subtotal colectomy (removal of part of the colon) was performed: surveillance of the remaining rectum every six to 12 months, depending on the number of polyps that develop. Cancer may still occur in the remaining rectum, but the risk is much lower.
  • Annual physical examination with palpation of the thyroid, with consideration of follow-up ultrasound examination and fine-needle aspiration if thyroid nodules are present[3]
Extra colonic manifestations of FAP generally require affected individuals to follow a lifelong surveillance program for the diagnosis and treatment of upper adenomas or desmoids.[4]

Additional and more detailed information about follow-up recommendations for individuals with FAP or AFAP, including post-surgery recommendations, is available at the following links:

Last updated: 12/18/2011

References