Anomalous Scleral Insertion of Superior Oblique in Axenfeld-Rieger Syndrome

Axenfeld-Rieger syndrome (ARS) is associated with ocular and systemic anomalies. PITX2 is known to be a major controlling gene in the pathogenesis of ARS and is associated with differentiation in both the neural crest and mesoderm during eye development. A 4-year-old girl with bilateral ARS had 20 p...

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Autores principales: Park, Sang Woo, Kim, Hwang Gyun, Heo, Hwan, Park, Yeoung Geol
Formato: Texto
Lenguaje:English
Publicado: The Korean Ophthalmological Society 2009
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2655743/
https://www.ncbi.nlm.nih.gov/pubmed/19337485
http://dx.doi.org/10.3341/kjo.2009.23.1.62
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author Park, Sang Woo
Kim, Hwang Gyun
Heo, Hwan
Park, Yeoung Geol
author_facet Park, Sang Woo
Kim, Hwang Gyun
Heo, Hwan
Park, Yeoung Geol
author_sort Park, Sang Woo
collection PubMed
description Axenfeld-Rieger syndrome (ARS) is associated with ocular and systemic anomalies. PITX2 is known to be a major controlling gene in the pathogenesis of ARS and is associated with differentiation in both the neural crest and mesoderm during eye development. A 4-year-old girl with bilateral ARS had 20 prism diopters (PD) of exotropia with 30PD of A- pattern deviation, more than 20PD of dissociated vertical deviation (DVD), and severe superior oblique overaction (SOOA). During surgery we observed that the SO inserted more posteriorly than normal. We believe this finding is one of the abnormal manifestations of the development of the extraocular muscles in ARS.
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spelling pubmed-26557432009-04-01 Anomalous Scleral Insertion of Superior Oblique in Axenfeld-Rieger Syndrome Park, Sang Woo Kim, Hwang Gyun Heo, Hwan Park, Yeoung Geol Korean J Ophthalmol Case Report Axenfeld-Rieger syndrome (ARS) is associated with ocular and systemic anomalies. PITX2 is known to be a major controlling gene in the pathogenesis of ARS and is associated with differentiation in both the neural crest and mesoderm during eye development. A 4-year-old girl with bilateral ARS had 20 prism diopters (PD) of exotropia with 30PD of A- pattern deviation, more than 20PD of dissociated vertical deviation (DVD), and severe superior oblique overaction (SOOA). During surgery we observed that the SO inserted more posteriorly than normal. We believe this finding is one of the abnormal manifestations of the development of the extraocular muscles in ARS. The Korean Ophthalmological Society 2009-03 2009-03-09 /pmc/articles/PMC2655743/ /pubmed/19337485 http://dx.doi.org/10.3341/kjo.2009.23.1.62 Text en Copyright © 2009 by the Korean Ophthalmological Society http://creativecommons.org/licenses/by-nc/3.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Park, Sang Woo
Kim, Hwang Gyun
Heo, Hwan
Park, Yeoung Geol
Anomalous Scleral Insertion of Superior Oblique in Axenfeld-Rieger Syndrome
title Anomalous Scleral Insertion of Superior Oblique in Axenfeld-Rieger Syndrome
title_full Anomalous Scleral Insertion of Superior Oblique in Axenfeld-Rieger Syndrome
title_fullStr Anomalous Scleral Insertion of Superior Oblique in Axenfeld-Rieger Syndrome
title_full_unstemmed Anomalous Scleral Insertion of Superior Oblique in Axenfeld-Rieger Syndrome
title_short Anomalous Scleral Insertion of Superior Oblique in Axenfeld-Rieger Syndrome
title_sort anomalous scleral insertion of superior oblique in axenfeld-rieger syndrome
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2655743/
https://www.ncbi.nlm.nih.gov/pubmed/19337485
http://dx.doi.org/10.3341/kjo.2009.23.1.62
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