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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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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
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author Fea, Antonio Maria
Aragno, Vittoria
Briamonte, Cristina
Franzone, Mauro
Putignano, Davide
Grignolo, Federico Maria
author_facet Fea, Antonio Maria
Aragno, Vittoria
Briamonte, Cristina
Franzone, Mauro
Putignano, Davide
Grignolo, Federico Maria
author_sort Fea, Antonio Maria
collection PubMed
description 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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spelling pubmed-45609312015-09-06 Parry Romberg syndrome with a wide range of ocular manifestations: a case report Fea, Antonio Maria Aragno, Vittoria Briamonte, Cristina Franzone, Mauro Putignano, Davide Grignolo, Federico Maria BMC Ophthalmol 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-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. BioMed Central 2015-09-05 /pmc/articles/PMC4560931/ /pubmed/26340917 http://dx.doi.org/10.1186/s12886-015-0093-0 Text en © Fea et al. 2015 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Case Report
Fea, Antonio Maria
Aragno, Vittoria
Briamonte, Cristina
Franzone, Mauro
Putignano, Davide
Grignolo, Federico Maria
Parry Romberg syndrome with a wide range of ocular manifestations: a case report
title Parry Romberg syndrome with a wide range of ocular manifestations: a case report
title_full Parry Romberg syndrome with a wide range of ocular manifestations: a case report
title_fullStr Parry Romberg syndrome with a wide range of ocular manifestations: a case report
title_full_unstemmed Parry Romberg syndrome with a wide range of ocular manifestations: a case report
title_short Parry Romberg syndrome with a wide range of ocular manifestations: a case report
title_sort parry romberg syndrome with a wide range of ocular manifestations: a case report
topic Case Report
url 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
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