Parry Romberg syndrome with a wide range of ocular manifestations: a case report

被引:32
作者
Fea, Antonio Maria [1 ]
Aragno, Vittoria [1 ]
Briamonte, Cristina [1 ]
Franzone, Mauro [1 ]
Putignano, Davide [1 ]
Grignolo, Federico Maria [1 ]
机构
[1] Univ Turin, Dept Clin Sci, Inst Ophthalmol, I-10100 Turin, Italy
关键词
PROGRESSIVE HEMIFACIAL ATROPHY; SCLERODERMA EN-COUP; FACIAL HEMIATROPHY; LINEAR SCLERODERMA; DE-SABRE; UVEITIS;
D O I
10.1186/s12886-015-0093-0
中图分类号
R77 [眼科学];
学科分类号
100212 ;
摘要
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.
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页数:6
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