Brucoli Matteo, Giarda Mariangela, Benech Arnaldo
Department of Maxillo-Facial Surgery Azienda Ospedaliera Maggiore della Carità University of Piemonte Orientale Amedeo Avogadro, Novara, Italy.
J Craniofac Surg. 2011 May;22(3):946-8. doi: 10.1097/SCS.0b013e31821028a6.
Gardner syndrome, a variant of familial adenomatous polyposis, is an autosomal dominant genetic disease characterized by the combined presence of multiple intestinal polyps and extraintestinal manifestations. The extraintestinal manifestations include multiple osteomas, connective tissue tumors, thyroid carcinomas, and hypertrophy of the pigmented epithelium of the retina. Osteoma is a benign neoplasm of bone tissue characterized by slow continuous growth that usually affects the long bones and cranial bones and is a major symptom for Gardner syndrome. The authors report the extraintestinal lesions affecting the maxillofacial regions in 2 male patients (father and son) with Gardner syndrome. The presurgical planning and surgical management of these lesions are described.
加德纳综合征是家族性腺瘤性息肉病的一种变体，是一种常染色体显性遗传病，其特征是同时存在多个肠道息肉和肠外表现。肠外表现包括多发性骨瘤、结缔组织肿瘤、甲状腺癌以及视网膜色素上皮肥大。骨瘤是骨组织的一种良性肿瘤，其特征是持续缓慢生长，通常影响长骨和颅骨，是加德纳综合征的主要症状。作者报告了2例患有加德纳综合征的男性患者（父子）的颌面部肠外病变。描述了这些病变的术前规划和手术处理。
