[Gardner syndrome. Genetic aspects of 7 cases in 3 families].
Gardner's syndrome, a hereditary affection of the bastoderm layers, is rarely recognized though it is seen in 8-16% of cases of colorectal polyposis. Seven cases (4 female and 3 male) of Gardner's syndrome in 3 families are presented. Six of the patients belonged to 2 families, several members of which suffered from familial polyposis; the seventh case was an isolated case of Gardner's syndrome without a family history of polyposis. Because of the high risk of malignancy in the presence of polyps, the treatment of Gardner's syndrome is identical to that of familial polyposis. Four patients underwent total colectomy and 2 simple polypectomy. One refused all treatment or monitoring. From these patients, one of the polyps excised from the rectum was found to be undergoing malignant transformation. The extracolic symptoms of this condition precede polyposis within the gut; they attract attention and thus permit early diagnosis.
D000328:Adult; D005260:Female; D005736:Gardner Syndrome / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery; D006801:Humans; D008297:Male; D010375:Pedigree
Syndrome de Gardner. Aspects génétiques à propos de 3 familles et 7 observations.