Temporomandibular joint ankylosis in Williams syndrome patient: an insight on the function of elastin in temporomandibular joint disorder

• 발행기관: 대한구강악안면외과학회
• 간행물 정보: 『대한구강악안면외과학회지』대한구강악안면외과학회지 제48권 제3호, 178~181쪽, 전체 4쪽
• 주제분류: 의약학 > 내과학
• 파일형태: PDF
• 발행일자: 2022.06.30

영문 초록

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.

목차

I. Introduction

II. Case Report

III. Discussion

ORCID

Authors’ Contributions

Conflict of Interest

References

키워드

Williams-Beuren syndrome Elastin Temporomandibular joint Ankylosis Arthroplasty