伴有多种眼部表现的帕里-龙贝格综合征：一例报告
Parry Romberg syndrome with a wide range of ocular manifestations: a case report.
作者信息
Fea Antonio Maria, Aragno Vittoria, Briamonte Cristina, Franzone Mauro, Putignano Davide, Grignolo Federico Maria
机构信息
Department of Clinical Sciences, Ophthalmology Institute, University of Turin, Via Juvarra 19, 10100, Turin, Italy.
出版信息
BMC Ophthalmol. 2015 Sep 5;15:119. doi: 10.1186/s12886-015-0093-0.
BACKGROUND
Parry-Romberg syndrome (PRS) is a rare disorder characterized by unilateral facial atrophy affecting the skin, subcutaneous tissue, muscles, and sometimes extending to the osteocartilaginous structures. Ocular involvement is relatively rare.
CASE PRESENTATION
We present a case of a 23-year-old female caucasian patient with Parry Romberg syndrome and extensive ocular involvement: enophthalmos, uveitis, iris atrophy. Ultrasound biomicroscopy (UBM) demonstrated hypotrophy of the ciliary body. The ciliary body atrophy has been previously reported just once and can be an explanation for the hypotony, frequently present in these patients.
CONCLUSIONS
Parry Romberg syndrome is a rare multidisciplinary disease. Our case presents a full spectrum of ocular manifestations. The pathogenesis of hypotonia is discussed.
背景
帕里-龙贝格综合征（PRS）是一种罕见疾病，其特征为单侧面部萎缩，累及皮肤、皮下组织、肌肉，有时还会延伸至骨软骨结构。眼部受累相对少见。
病例报告
我们报告一例23岁的白种女性患者，患有帕里-龙贝格综合征且伴有广泛眼部受累：眼球内陷、葡萄膜炎、虹膜萎缩。超声生物显微镜检查（UBM）显示睫状体萎缩。此前仅报道过一次睫状体萎缩，这可能是这些患者中经常出现的低眼压的一个原因。
结论
帕里-龙贝格综合征是一种罕见的多学科疾病。我们的病例呈现了完整的眼部表现谱。文中讨论了低眼压的发病机制。
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