Canabarro Antonio, Galheigo Tania Maria, Galheigo de Oliveira e Silva Diogo, Tinoco Eduardo Muniz Barretto
School of Dentistry, Rio de Janeiro State University, RJ, Brazil.
Quintessence Int. 2008 Apr;39(4):307-11.
Proteus syndrome is an extremely rare congenital disorder with progressive asymmetric overgrowth of multiple tissues. The etiology is unknown to date. The aim of this article was to describe the clinical and radiographic periodontal findings and other oral manifestations in a patient with Proteus syndrome. A 13-year-old Caucasian Brazilian girl with Proteus syndrome was evaluated by means of clinical examination and full-mouth intraoral radiographic analysis. The patient showed skeletal malformations; gigantism; facial phenotype; and overgrowth of multiple tissues, including disproportionate overgrowth of skull, left hand, and lower limbs. Evaluation of the plantar surface of the left foot showed cerebriform connective nevus. The hands also showed reduced range of motion. Intraoral examination revealed enamel hypoplasia of permanent teeth restricted to the left side of the mouth, generalized gingival overgrowth, abundant dental plaque, and malpositioned teeth. Neither periodontal pockets nor alveolar bone destruction were detected. In conclusion, oral manifestations of Proteus syndrome may include gingival overgrowth and malposition of teeth, as well as unilateral enamel hypoplasia, as shown in this case report.
变形综合征是一种极其罕见的先天性疾病,表现为多个组织进行性不对称过度生长。其病因至今不明。本文旨在描述一名变形综合征患者的临床和影像学牙周表现及其他口腔表现。对一名患有变形综合征的13岁巴西白人女孩进行了临床检查和全口口腔内影像学分析。该患者表现出骨骼畸形、巨人症、面部表型以及多个组织的过度生长,包括颅骨、左手和下肢的不成比例过度生长。对左脚足底表面的评估显示有脑回状结缔组织痣。双手的活动范围也减小。口腔检查发现恒牙釉质发育不全仅限于口腔左侧,牙龈普遍增生,牙菌斑丰富,牙齿排列不齐。未检测到牙周袋或牙槽骨破坏。总之,如本病例报告所示,变形综合征的口腔表现可能包括牙龈增生、牙齿排列不齐以及单侧釉质发育不全。