Abstract
BackgroundParry-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 presentationWe 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.ConclusionsParry Romberg syndrome is a rare multidisciplinary disease. Our case presents a full spectrum of ocular manifestations. The pathogenesis of hypotonia is discussed.
Highlights
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
Our case presents a full spectrum of ocular manifestations
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 [1,2,3,4]
Summary
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 [1,2,3,4]. We present a case of PRS with enophthalmos, uveitis, ciliary body hypotrophy and phacodonesis. Case presentation 23 years old lady with 16 years history of Parry Romberg syndrome was referred to our clinic. No other cases of PRS were reported in the family.
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