Department of Oral and Maxillofacial Surgery, University Magna Graecia, Catanzaro, Italy.
Oral Surg Oral Med Oral Pathol Oral Radiol. 2013 Mar;115(3):e1-6. doi: 10.1016/j.oooo.2011.10.020. Epub 2012 Jun 1.
Gardner syndrome (GS) is an autosomal dominant genetic disorder with almost complete penetrance (80%) and variable expression. GS is a variant of familial adenomatous polyposis and characterized by extracolonic manifestations including osteomas and soft tissue tumors (desmoid tumors, epidermoid cysts). We describe clinical and surgical approaches in a family in which the genetic disorder was diagnosed in 3 generations.
The studied family underwent clinical history and instrumental and genomic studies. Two members of this family, affected with GS, underwent surgery for skeletal osteomas.
The patients that we treated with clinical-instrumental monitoring for a period of 5 years had no major disturbances of the stomatognathic system and no clinical signs of pathology of the gastrointestinal tract, eyes, or endocrine systems.
The orofacial complex disorders are exclusively functional and esthetic, concerning primarily the stomatognathic system. We had no cases of malignant transformation of osteomatosis lesions. Clinical sequelae are manly facial eumorphy and occlusion problems of the temporomandibular joint.
Gardner 综合征（GS）是一种常染色体显性遗传疾病，几乎完全外显（80%），表现具有变异性。GS 是家族性腺瘤性息肉病的一种变体，其特征为结肠外表现，包括骨瘤和软组织肿瘤（硬纤维瘤、表皮样囊肿）。我们描述了一个家族中 3 代人遗传疾病的临床和手术方法。
对该家族进行了临床病史、仪器和基因组研究。该家族的 2 名 GS 患者接受了骨骼骨瘤手术。
我们对这 2 名患者进行了 5 年的临床仪器监测，他们的口腔颌面部系统没有出现重大紊乱，胃肠道、眼睛或内分泌系统也没有出现明显的病理迹象。
口腔颌面部复杂疾病是功能和美观方面的问题，主要涉及口腔颌面部系统。我们没有发生骨瘤病变恶变的病例。临床后果主要是面部正常形态和颞下颌关节咬合问题。
