title: [Extracolonic manifestations of familial adenomatous polyposis: incidence and impact on the disease outcome].
abstract: BACKGROUND
  Familial adenomatous polyposis is a hereditary disease with autossomic and dominant features, frequently associated to many extracolonic manifestations.
  
  
  OBJECTIVE
  To report extracolonic manifestations incidence and to analyze its impact on the disease's outcome.
  
  
  METHODS
  Revision of patient charts treated from 1977 to 2001, relating associated extracolonic manifestations and its complications.
  
  
  RESULTS
  Among 59 familial adenomatous polyposis patients, 23 (38,9%) presented some extracolonic manifestations at diagnosis or during follow-up. There were registered 37 different extracolonic manifestations (1.6 per patient). The most common manifestations were osteomas and congenital hypertrophy of the retinal pigment epithelium, diagnosed in 25% and 20% of the investigated patients, respectively. Other extracolonic manifestations were represented by upper digestive adenomas, epidermoid cysts, desmoid tumor (seven each), gastric cancer (three) and tireoid cancer (two). Desmoid-associated complications were reported in six patients, being intestinal obstruction in four and hidronephrosis in two; two deaths (28,5%) were registered.
  
  
  CONCLUSIONS
  Extracolonic manifestations incidence is high (40%) and may affect disease's outcome and patient's quality of life. For these reasons, the research, prevention and adequate treatment of extracolonic manifestations turn into vital importance in familial adenomatous polyposis patients.
authors.affiliation: Departamento de Gastroenterologia da Faculdade de Medicina da Universidade de São paulo, Brasil. fgmcampos@terra.com.br
authors.forename: Fábio Guilherme
authors.initials: FG
authors.lastname: Campos
authors.forename: Angelita
authors.initials: A
authors.lastname: Habr-Gama
authors.forename: Desidério Roberto
authors.initials: DR
authors.lastname: Kiss
authors.forename: Fábio César
authors.initials: FC
authors.lastname: Atuí
authors.forename: Fábio
authors.initials: F
authors.lastname: Katayama
authors.forename: Joaquim
authors.initials: J
authors.lastname: Gama-Rodrigues
mesh_terms: D011125:Adenomatous Polyposis Coli / Q000150:complications* / Q000601:surgery; D000293:Adolescent; D000328:Adult; D000368:Aged; D001938:Brazil; D002648:Child; D005260:Female; D005736:Gardner Syndrome / Q000150:complications / Q000601:surgery; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies