[The radiological and clinical features of Gardner's syndrome (author's transl)].
Gardner's syndrome, completely expressed, consists of a trio of familial polyposis of the colon, osteomas and mesenchymal tumours of the skin. Inheritence is autosomal dominant. In many patients with familial polyposis of the colon, only mesenchymal skin tumours or osteomas can be demonstrated. It is therefore possible that Gardner's syndrome and familial polyposis represent two extremities of a single disease which is characterised by marked variability in the expressivity of the gene. Gardner's syndrome has been considered a rare condition occurring in only about 8% of patients with familial polyposis. Amongst the 20 patients with colonic polyposis from eleven families, mesenchymal and/or osseous lesions were found in seventeen (85%). Osteomas of the mandible were shown particularly frequently by orthopantomography. Since polyposis of the colon tends to remain symptomless for many years, the finding of osteomas in the facial skeleton, or recurrent skin tumours in young patients, should lead to further investigation.
D000293:Adolescent; D000328:Adult; D003937:Diagnosis, Differential; D005260:Female; D005736:Gardner Syndrome / Q000000981:diagnostic imaging* / Q000235:genetics; D006801:Humans; D007417:Intestinal Polyps / Q000000981:diagnostic imaging; D008297:Male; D008339:Mandibular Neoplasms / Q000000981:diagnostic imaging; D008875:Middle Aged; D010016:Osteoma / Q000000981:diagnostic imaging; D010375:Pedigree; D011859:Radiography; D012004:Rectal Neoplasms / Q000000981:diagnostic imaging; D012888:Skull Neoplasms / Q000000981:diagnostic imaging
Röntgendiagnostik und Klinik des Gardner-Syndroms.