Drugowick Rayen Millanao, Da Rós Gonçalves Lorena, Barrôso Aurea Simone, Feres-Filho Eduardo Jorge, Maia Lucianne Cople
Department of Pediatric Dentistry and Orthodontics, School of Dentistry, Federal University of Rio de Janeiro, RJ, Brazil.
J Periodontol. 2007 Jun;78(6):1159-63. doi: 10.1902/jop.2007.060378.
Bardet-Biedl syndrome (BBS) is a rare, heterogeneous, autosomal recessive condition, primarily characterized by polydactyly, obesity, mental retardation, hypogonadism, retinopathy, and renal failure. Dental anomalies, regarded as secondary manifestations, include hypodontia, microdontia, short roots, and deep palate. Few reports in the literature have described the oral manifestations of BBS. This article reports a case of BBS in a boy who presented some typical oral manifestations added to a generalized gingival overgrowth, an anomaly that had not been reported previously in patients with this syndrome.
A 12-year-old white male presented with a diagnosis of BBS and chief complaint of gingival enlargement in the anterior segment of both arcades. The treatment plan included surgical removal of the overgrown gingiva followed by orthodontic therapy. The excised tissues were submitted to histologic analysis.
There was no sign of recurrence 1 year after gingivectomy. Histopathology revealed a dense connective tissue with a mild inflammatory infiltrate, irregularly arranged fiber bundles, and epithelial acanthosis, which is characteristic of gingival overgrowth.
The gingival overgrowth was treated successfully by gingivectomy. The periodontal surgery minimized the functional, social, and emotional consequences of the oral manifestation associated with the syndrome.
巴德-比德尔综合征（BBS）是一种罕见的、异质性的常染色体隐性疾病，主要特征为多指（趾）畸形、肥胖、智力发育迟缓、性腺功能减退、视网膜病变和肾衰竭。牙齿异常被视为次要表现，包括缺牙、小牙、牙根短和腭穹隆深。文献中很少有关于BBS口腔表现的报道。本文报告了1例患有BBS的男孩，除了广泛性牙龈增生外，还出现了一些典型的口腔表现，而这种异常此前在该综合征患者中尚未有过报道。
一名12岁白人男性被诊断为BBS，主要主诉为双侧牙弓前段牙龈肿大。治疗方案包括手术切除增生的牙龈，随后进行正畸治疗。切除的组织进行了组织学分析。
牙龈切除术后1年无复发迹象。组织病理学显示为致密结缔组织，伴有轻度炎症浸润、纤维束排列不规则以及上皮棘层增厚，这是牙龈增生的特征。
牙龈增生通过牙龈切除术成功治愈。牙周手术将与该综合征相关的口腔表现所带来的功能、社会和情感影响降至最低。
