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帕里-龙贝格综合征的多学科治疗及眼科检查结果

Interdisciplinary treatment and ophthalmological findings in Parry-Romberg syndrome.

作者信息

Dawczynski Jens, Thorwarth Michael, Koenigsdoerffer Ekkehart, Schultze-Mosgau Stefan

机构信息

Department of Ophthalmology, University Hospital Jena, Jena, Germany.

出版信息

J Craniofac Surg. 2006 Nov;17(6):1175-6. doi: 10.1097/01.scs.0000236440.20592.be.

Abstract

Parry-Romberg syndrome is a rare pathologic process, characterized by progressive hemifacial atrophy. A case of Parry-Romberg syndrome with ocular involvement is reported. A 27-year-old male patient with Parry-Romberg syndrome was interdisciplinary investigated and treated. For reconstruction of hemifacial soft tissues a free vascular parascapular graft was performed. Opthalmological findings included an evident enophthalmos and an eyelid lag with keratopathy and epiphora. Furthermore pupillary disturbances and endothelial precipitates were detectable. Complete fundus examination showed a unilateral optic disc swelling, central vitreous opacities and peripheral pigmentary disturbances. In the presented case of Parry-Romberg syndrome a rare association to ophthalmological involvement could be found. Beside the enophthalmos and eyelid alterations, also a panuveitis with papillitis should be treated by steroid therapy.

摘要

帕里-龙贝格综合征是一种罕见的病理过程,其特征为进行性半侧面部萎缩。本文报告一例伴有眼部受累的帕里-龙贝格综合征病例。对一名患有帕里-龙贝格综合征的27岁男性患者进行了多学科检查和治疗。为重建半侧面部软组织,进行了游离血管肩胛旁皮瓣移植术。眼科检查结果包括明显的眼球内陷、眼睑滞后伴角膜病变和溢泪。此外,还可检测到瞳孔紊乱和内皮沉着物。完整的眼底检查显示单侧视盘肿胀、中央玻璃体混浊和周边色素紊乱。在所呈现的帕里-龙贝格综合征病例中,发现了与眼科受累的罕见关联。除眼球内陷和眼睑改变外,伴有视乳头炎的全葡萄膜炎也应采用类固醇疗法治疗。

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