title: Guidelines for the surveillance and management of familial adenomatous polyposis (FAP): a world wide survey among 41 registries.
abstract: OBJECTIVE
  FAP is an autosomal dominant disease characterized by numerous adenomas in the colorectum. The majority of patients also develop adenomas in the duodenum. The purpose of the study was to evaluate the management and screening protocols implemented in FAP families at various polyposis registries in the world.
  
  
  METHODS
  A questionnaire was mailed to the members of The Leeds Castle Polyposis Group requesting information on: diagnostic evaluation of polyposis, recommendations for screening and surgical management of the colon, surveillance of the upper gastrointestinal tract and the management of duodenal polyposis.
  
  
  RESULTS
  Almost all members agreed that a newly diagnosed patient should be referred to a polyposis registry and to a clinical genetics centre for genetic counselling and DNA testing. If the mutation has been detected in the family, DNA testing should be offered to first-degree relatives of patients from age 10-12 years onwards. The surveillance protocol generally advised includes sigmoidoscopy from age 10-12 years at 2-year intervals until age 40 years. There was no agreement on the preferred surgical treatment of colonic polyposis. Almost all members advised follow up after colonic surgery. Surveillance of the duodenum was recommended by most members; this would start from age 30 years. There was no agreement on the preferred surgical treatment of duodenal polyposis, or on the indication for operation.
  
  
  CONCLUSIONS
  This survey provides insight into the guidelines used at various polyposis registries for the surveillance and management of FAP patients, and this insight may contribute to the appropriate management of these patients.
authors.affiliation: The Netherlands Foundation for the Detection of Hereditary Tumours, Leiden, The Netherlands The Danish Polyposis Register, Hvidovre, Denmark.
authors.lastname: Vasen
authors.lastname: Bülow