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KMID : 0355620220480030178
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Journal of Korean Association of Oral and Maxillofacial Surgeons
2022 Volume.48 No. 3 p.178 ~ p.181
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Temporomandibular joint ankylosis in Williams syndrome patient: an insight on the function of elastin in temporomandibular joint disorder
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Woo Jae-Man
Lee Choi-Ryang
Choi Jin-Young
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Abstract
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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.
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KEYWORD
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Williams-Beuren syndrome, Elastin, Temporomandibular joint, Ankylosis, Arthroplasty
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