Woo Jaeman, Lee Choi-Ryang, Choi Jin-Young
Department of Oral and Maxillofacial Surgery, Jeju National University College of Medicine, Jeju, Korea.
Department of Oral and Maxillofacial Surgery, Seoul National University Dental Hospital, Seoul, Korea.
J Korean Assoc Oral Maxillofac Surg. 2022 Jun 30;48(3):178-181. doi: 10.5125/jkaoms.2022.48.3.178.
Williams-Beuren syndrome (WS) is a rare genetic disorder that results from microdeletion at chromosome 7, which harbors the elastin gene. Clinical findings include arteriopathy, aortic stenosis, hypertension, and laxities and contractures in different joints throughout the body. While many components of the temporomandibular joint (TMJ) normally contain elastin, there are few reports on TMJ manifestations of WS. This study reports a TMJ ankylosis case in a WS patient and shares insight on a possible link between development of TMJ ankylosis and elastin deficiency in WS patients. A WS patient presented with bilateral TMJ ankylosis and was successfully treated with TMJ gap arthroplasty. Hypermobility of TMJ and lack of elastin in retrodiscal tissue can induce anterior disc displacement without reduction. Due to lack of elastin, which has a significant role in the compensatory and reparatory mechanism of TMJ, WS patients might be prone to TMJ ankylosis.
威廉姆斯-贝伦综合征(WS)是一种罕见的遗传性疾病,由7号染色体上的微缺失引起,该染色体包含弹性蛋白基因。临床症状包括动脉病变、主动脉狭窄、高血压以及全身不同关节的松弛和挛缩。虽然颞下颌关节(TMJ)的许多组成部分通常含有弹性蛋白,但关于WS患者TMJ表现的报道很少。本研究报告了1例WS患者的TMJ强直病例,并就TMJ强直的发生与WS患者弹性蛋白缺乏之间的可能联系进行了深入探讨。1例WS患者出现双侧TMJ强直,并通过TMJ间隙关节成形术成功治疗。TMJ活动过度和盘后组织中缺乏弹性蛋白可导致关节盘前移位且无法复位。由于弹性蛋白在TMJ的代偿和修复机制中起重要作用,缺乏弹性蛋白可能使WS患者易患TMJ强直。