[Gardner syndrome: apropos of 3 families and 7 cases].
Gardner's syndrome, an hereditary affection of the different blastoderm layers, is rarely recognised, even though it is seen in 8-16% of cases of colorectal polyposis. The authors present 7 cases of Gardner's syndrome within 3 families: 4 female and 3 male. Six of the patients belonged to 2 families, of which several members 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, 4 patients underwent total colectomy, 2 a simple polypectomy and 1 refused all treatment or monitoring. From these patients, one of the polyps excised at the level of the rectum was found to be undergoing malignant transformation. The extra-colic manifestations of this condition precede the polyposis within the gut; they attract attention and thus permit an early diagnosis.
D000015:Abnormalities, Multiple / Q000235:genetics / Q000601:surgery; D000328:Adult; D005260:Female; D005736:Gardner Syndrome / Q000235:genetics*; D006801:Humans; D007417:Intestinal Polyps / Q000235:genetics / Q000601:surgery; D008297:Male
Syndrome de Gardner: à propos de 3 families et 7 observations.