Clinical and radiological findings in Gardner's syndrome: a case report and follow-up study.

Abstract

Gardner's syndrome is characterized by colorectal adenomas, multiple osteomas, especially of the skull, and various soft-tissue tumours. The disease is inherited as an autosomal dominant disorder and all untreated patients will develop colorectal adenocarcinomas. Since the clinical and radiological stigmas in the maxillofacial area, such as exosteal and endosteal osteomas, skin cysts, atypical skin pigmentation and abnormal dental findings or radiopaque lesions can precede the often symptomless adenomas for many years, dentists, maxillofacial surgeons and radiologists should be familiar with the manifestations of this disease. A case is reported to illustrate the dentist's role in the diagnosis of Gardner's syndrome. In a follow-up study of 11 patients with colorectal adenomas, the typical triad of features of Gardner's syndrome was found in eight. In six patients, bony changes were demonstrated by panoramic radiography. It is proposed that radiography of the jaws may serve as a valuable tool for the early detection of carriers of Gardner's syndrome.

Osteomas exofíticos múltiplos de ossos craniofaciais não associados à Síndrome de Gardner: relato de caso

“…Thus, it is important to have precise diagnosis, differentiating exophytic osteomas, osteochondromas, organized subperiosteal hematoma, mature ossifying fibroma that causes expansion of cortical lamina and periosteal osteosarcoma or chondrosarcoma 5 . Gardner's syndrome is a hereditary dominant autosomal disorder with estimated prevalence of 1 in 14,000 13 . It consists of multiple polyposis of large intestine, bone osteomas, including long bones, head and mandible, multiple epidermal or cutaneous sebaceous cysts, particularly on the scalp and dorsal region, with occasional frequency of dermoid tumors and included permanent and supranumeric teeth 14 .…”

“…Most patients with Gardner's syndrome do not show complete clinical picture of the disease: the term binary for Gardner's syndrome has been applied to subjects that have 2 or 3 of the aspects traditionally described 13,15 . Bone lesions in Gardner's syndrome are not real neoplasms.…”

Skin and teeth

“…Auch Karies tritt vermehrt auf. In manchen Fällen können Röntgenaufnahmen des Ober-und Unterkiefers hilfreich zur frühzeitigen Diagnosestellung des Gardner-Syndroms beitragen [25].…”

Paranasal Sinus Osteomas and Gardner's Syndrome

“…6 Multiple osteomas of the skull and jaw are often found in at-risk relatives, commonly before the appearance of colonic polyps. 10 Benign soft tissue tumors associated with multiple polyposis usually appear during the first and second decades. Epidermoid cysts, fibromas, and dermoids of Gardner's syndrome commonly involve the skin.…”