Bone and dental abnormalities as first signs of familial Gardner’s syndrome in a Chinese family: a literature review and a case report

¹ M.S., D.D.S., Attending doctor, Department of Oral and Maxillofacial Surgery, First Affiliated Hospital, College of Medicine, Zhejiang University. 79# Qingchun Road, Hangzhou 310003, People’s Republic of China
² B.D.S., Resident, Department of Oral and Maxillofacial Surgery, National Dental Centre Singapore. Second Hospital Avenue, 168938, Singapore
³ M.D., Ph.D., Professor, Department of Oral and Maxillofacial Surgery, First Affiliated Hospital, College of Medicine, Zhejiang University. 79# Qingchun Road, Hangzhou 310003, People’s Republic of China
⁴ M.D., Ph.D., Professor and Chairman, Department of Oral and Maxillofacial Surgery, First Affiliated Hospital, College of Medicine, Zhejiang University. 79# Qingchun Road, Hangzhou 310003, People’s Republic of China
⁵ M.S., D.D.S., Attending doctor, Department of Oral and Maxillofacial Surgery, First Affiliated Hospital, College of Medicine, Zhejiang University. 79# Qingchun Road, Hangzhou 310003, People’s Republic of China

^* Corresponding author: Yi Lin 1507155@zju.edu.cn

Abstract

Gardner’s syndrome (GS) is an autosomal dominant disease characterized by the presence of familial adenomatous polyposis (FAP) as well as extraintestinal manifestations such as osteomas, dental anomalies, epidermoid cysts and ocular abnormalities. These intestinal polyps carry a 100% risk of malignant change, so early diagnosis is crucial. As craniofacial osteomas and dental anomalies of GS usually precede gastrointestinal symptoms, otolaryngologists, oral surgeons and dentists play an important role in the diagnosis of GS. GS is extensively reported in literature in the Caucasian race but not in the Mongoloid race. We report a case of a 22-year-old patient with a manifestation of three features of GS - multiple osteomas, soft tissue tumors and dental anomalies in the craniofacial region, with no intestinal polyps at the time of reporting. A family pedigree with our patient as the proband was constructed and revealed 3 consecutive generations in his lineage with GS.