Abstract 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.

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

FEA, Antonio Maria;ARAGNO, VITTORIA;BRIAMONTE, Cristina;PUTIGNANO, DAVIDE;GRIGNOLO, Federico
2015

Abstract

Abstract 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.
BMC OPHTHALMOLOGY
15
1
119
124
http://www.biomedcentral.com/bmcophthalmol/
Atrophy; Ciliary Body; Enophthalmos; Facial Hemiatrophy; Female; Humans; Imaging, Three-Dimensional; Iris; Microscopy, Acoustic; Tomography, X-Ray Computed; Uveitis; Young Adult; Ophthalmology
Fea, Antonio Maria; Aragno, Vittoria; Briamonte, Cristina; Franzone, Mauro; Putignano, Davide; Grignolo, Federico
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/2318/1564999
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