Acht Jahre Gardner-Syndrom in einer Familie

Abstract

In all affected members of a family with Gardner's syndrome the complete triad of skin tumours, osteomas and polyps of the colon has developed since 1968 when examination revealed an apparently bisymptomatic variant of Gardner's syndrome. In this family bony changes consisting of circumscript osteomas, diffuse sclerosing zones within certain bones, and ridge-like widening of the corticalis were noted, having developed during puberty but remaining constant thereafter. Similarly, no significant new skin tumours appeared after puberty. Most colonic polyps however, occurred in adolescence or thereafter, with malignant transformation after a variable interval of several years. The necessity of long-term observation of the colon, also in patients presenting with a bi- or monosymptomatic form, is stressed. Coloscopy has proven to be more useful diagnostically than radiological methods.