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

Autor: Fea AM; Department of Clinical Sciences, Ophthalmology Institute, University of Turin, Via Juvarra 19, 10100, Turin, Italy. antoniomfea@gmail.com., Aragno V; Department of Clinical Sciences, Ophthalmology Institute, University of Turin, Via Juvarra 19, 10100, Turin, Italy. vittoria.aragno@hotmail.it., Briamonte C; Department of Clinical Sciences, Ophthalmology Institute, University of Turin, Via Juvarra 19, 10100, Turin, Italy. cristina.briamonte@gmail.com., Franzone M; Department of Clinical Sciences, Ophthalmology Institute, University of Turin, Via Juvarra 19, 10100, Turin, Italy. maurofranzone@virgilio.it., Putignano D; Department of Clinical Sciences, Ophthalmology Institute, University of Turin, Via Juvarra 19, 10100, Turin, Italy. edivado461@gmail.com., Grignolo FM; Department of Clinical Sciences, Ophthalmology Institute, University of Turin, Via Juvarra 19, 10100, Turin, Italy. federico.grignolo@unito.it.
Jazyk: angličtina
Zdroj: BMC ophthalmology [BMC Ophthalmol] 2015 Sep 05; Vol. 15, pp. 119. Date of Electronic Publication: 2015 Sep 05.
DOI: 10.1186/s12886-015-0093-0
Abstrakt: 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.
Databáze: MEDLINE