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Revista Brasileira de Coloproctologia

On-line version ISSN 0101-9880

Abstract

FERNANDES, Gabriella Oliveira; PEREIRA JUNIOR, Jaime José; FRANCA, Marco Aurélio Viana  and  COSTA, José Hermes Gomes. Attenuated familial adenomatous polyposis. Rev bras. colo-proctol. [online]. 2007, vol.27, n.2, pp.179-184. ISSN 0101-9880.  http://dx.doi.org/10.1590/S0101-98802007000200008.

Attenuated Familial Adenomatous Polyposis (AFAP) is a heritable autosomally dominant syndrome, with later diagnosis than the classical condition of Familial Adenomatous Polyposis. Amid its main features there are : a) the presence of less than 100 polyps; b) the mild course of the disease and its later diagnosis and development of colon cancer; c)the polyps are more frequent in the right colon; d)the rectum may be relatively or even totally spared. To analyze the clinical manifestations, treatment and follow-up of 13 patients with AFAP. The mean age was 55 years, five patients had positive family history of polyposis and/or colon cancer and nine (69%) patients had already developed colonic cancer at the time of the diagnosis. Most of the patients had polyps located in the right colon. Six out of 13 patients patients had undergone surgical resection, either proctocolectomy or colectomy. The average follow-up time was 26 months. Periodically colonoscopy or retosigmoidoscopy were employed for follow-up evaluation, according to the previous surgical procedure. The diagnosis of AFAP was made later than the one of the classic form of the disease and most of the polyps were located in the right colon. Frequent follow-up with endoscopic examination as a follow-up is mandatory. Colectomy with ileo-rectal anastomosis is a very good option in the surgical management of these patients with low recurrence rate of rectal polyps.

Keywords : familiar poluposis; colonic polyps; adnome.

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