FLORID ORAL MANIFESTATIONS IN AN ATYPICAL FAMILIAL ADENOMATOUS POLYPOSIS FAMILY WITH LATE PRESENTATION OF COLORECTAL POLYPS

Early identification and prophylactic surgery are essential in prevent ing development of malignancy in colorectal polyps in familial adenoma tous polyposis (FAP). Previous studies indicate a 100% cumulative risk of developing colonic polyps in individuals with FAP by the age of 34 yr. However, we have previously reported late-onset and non-penetranc e of polyposis in four families. We describe here in detail one of the se families with florid oral manifestations detectable on panoramic ra diography of the jaws, which were instrumental in the diagnosis of FAP in the index patient and for ascertainment of her family for screenin g. The pathological adenomatous polyposis coli (APC) gene mutation in this family was shown to segregate with dento-osseous changes. Panoram ic jaw radiography is a common examination carried out prior to dental or oral surgical procedures. This case illustrates the importance of appropriate investigations, including family history and colonoscopy, even in relatively older patients with radiological findings such as t hose described here and in members of their families at risk of FAP.