Temporomandibular joint ankylosis in Williams syndrome patient: an insight on the function of elastin in temporomandibular joint disorder
우재만, 이최량, 최진영,
소속 상세정보
우재만 ( Woo Jae-Man ) - Jeju National University College of Medicine Department of Oral and Maxillofacial Surgery
이최량 ( Lee Choi-Ryang ) - Seoul National University Dental Hospital Department of Oral and Maxillofacial Surgery
최진영 ( Choi Jin-Young ) - Seoul National University Dental Hospital Department of Oral and Maxillofacial Surgery
Abstract
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.
키워드
Williams-Beuren syndrome; Elastin; Temporomandibular joint; Ankylosis; Arthroplasty
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