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

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-y...

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Detalles Bibliográficos
Autores principales: Fea, Antonio Maria, Aragno, Vittoria, Briamonte, Cristina, Franzone, Mauro, Putignano, Davide, Grignolo, Federico Maria
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4560931/
https://www.ncbi.nlm.nih.gov/pubmed/26340917
http://dx.doi.org/10.1186/s12886-015-0093-0
Descripción
Sumario: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.