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Gardner syndrome is a rare disease in oral and maxillofacial diseases which is characterized by intestinal polyposis, multiple osteomas, skin and soft tissue tumors. Early treatment has a better prognosis; therefore the early diagnosis of Gardner syndrome is very important. Maxillofacial pathology always appears to precede the other pathologies, so the importance of early detection of the syndrome by dentists is also emphasized. This review summarizes the etiology of Gardner syndrome-related mutations and the clinical manifestations of Gardner syndrome characterized by oral and maxillofacial deformities, multiple gastrointestinal polyps, and desmoid tumour, and their associated treatment.
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