Gardner综合征诊疗现状及进展

The diagnosis and treatment progress of Gardner syndrome

Gardner综合征是口腔颌面部疾病中比较罕见的一类疾病,以多发结直肠道息肉、多发性骨瘤、皮肤和软组织肿瘤三联征为特征。其早期治疗有更好的预后,因此早期诊断非常重要。Gardner综合征早期病变一般在口腔颌面部表现出来,因此,口腔医生在本病的早期诊断中扮演了重要角色。本文总结了Gardner综合征与基因突变相关的病因研究,并且归纳了Gardner综合征以口腔颌面部畸形、多发胃肠道息肉以及硬纤维瘤为特点的临床表现及其相关治疗的进展,综述如下。

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.