Optical coherence tomography and fundus autofluorescence imaging study of chorioretinal atrophy involving the macula in Alagille syndrome

Shinji Makino, Yuko Ohkubo, Hironobu TampoDepartment of Ophthalmology, Jichi Medical University, Shimotsuke, Tochigi, JapanAbstract: We report the first case in the literature of chorioretinal atrophy involving the macula in an 11-year-old girl with Alagille syndrome, as examined by optical coherenc...

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Autores principales:	Makino S, Ohkubo Y, Tampo H
Formato:	article
Lenguaje:	EN
Publicado:	Dove Medical Press 2012
Materias:	Ophthalmology RE1-994
Acceso en línea:	https://doaj.org/article/c4b44befc6c54ece9b8873d0af19dd32
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spelling	oai:doaj.org-article:c4b44befc6c54ece9b8873d0af19dd322021-12-02T01:36:27ZOptical coherence tomography and fundus autofluorescence imaging study of chorioretinal atrophy involving the macula in Alagille syndrome1177-54671177-5483https://doaj.org/article/c4b44befc6c54ece9b8873d0af19dd322012-09-01T00:00:00Zhttp://www.dovepress.com/optical-coherence-tomography-and-fundus-autofluorescence-imaging-study-a10923https://doaj.org/toc/1177-5467https://doaj.org/toc/1177-5483Shinji Makino, Yuko Ohkubo, Hironobu TampoDepartment of Ophthalmology, Jichi Medical University, Shimotsuke, Tochigi, JapanAbstract: We report the first case in the literature of chorioretinal atrophy involving the macula in an 11-year-old girl with Alagille syndrome, as examined by optical coherence tomography, and fundus autofluorescence imaging. Funduscopy revealed diffuse choroidal hypopigmentation with increased visibility of the choroidal vessels and symmetric, well circumscribed macular discoloration. Anomalous oblique configuration of the optic disc and peripapillary tortuous vessels were also detected. Optical coherence tomography demonstrated decreased retinal thickness, especially the outer retinal layer, and the photoreceptor inner segment/outer segment junctions were irregular and discontinued, corresponding to macular discoloration. Fundus autofluorescence imaging clearly defined hypofluorescent areas in the peripapillary regions that extended along the macula and had a sleep mask appearance. We suggest that transient hypovitaminosis due to Alagille syndrome early in life might contribute to the retinal degeneration seen in this case.Keywords: Alagille syndrome, chorioretinal atrophy, optical coherence tomography, fundus autofluorescence imaging, hypovitaminosisMakino SOhkubo YTampo HDove Medical PressarticleOphthalmologyRE1-994ENClinical Ophthalmology, Vol 2012, Iss default, Pp 1445-1448 (2012)
institution	DOAJ
collection	DOAJ
language	EN
topic	Ophthalmology RE1-994
spellingShingle	Ophthalmology RE1-994 Makino S Ohkubo Y Tampo H Optical coherence tomography and fundus autofluorescence imaging study of chorioretinal atrophy involving the macula in Alagille syndrome
description	Shinji Makino, Yuko Ohkubo, Hironobu TampoDepartment of Ophthalmology, Jichi Medical University, Shimotsuke, Tochigi, JapanAbstract: We report the first case in the literature of chorioretinal atrophy involving the macula in an 11-year-old girl with Alagille syndrome, as examined by optical coherence tomography, and fundus autofluorescence imaging. Funduscopy revealed diffuse choroidal hypopigmentation with increased visibility of the choroidal vessels and symmetric, well circumscribed macular discoloration. Anomalous oblique configuration of the optic disc and peripapillary tortuous vessels were also detected. Optical coherence tomography demonstrated decreased retinal thickness, especially the outer retinal layer, and the photoreceptor inner segment/outer segment junctions were irregular and discontinued, corresponding to macular discoloration. Fundus autofluorescence imaging clearly defined hypofluorescent areas in the peripapillary regions that extended along the macula and had a sleep mask appearance. We suggest that transient hypovitaminosis due to Alagille syndrome early in life might contribute to the retinal degeneration seen in this case.Keywords: Alagille syndrome, chorioretinal atrophy, optical coherence tomography, fundus autofluorescence imaging, hypovitaminosis
format	article
author	Makino S Ohkubo Y Tampo H
author_facet	Makino S Ohkubo Y Tampo H
author_sort	Makino S
title	Optical coherence tomography and fundus autofluorescence imaging study of chorioretinal atrophy involving the macula in Alagille syndrome
title_short	Optical coherence tomography and fundus autofluorescence imaging study of chorioretinal atrophy involving the macula in Alagille syndrome
title_full	Optical coherence tomography and fundus autofluorescence imaging study of chorioretinal atrophy involving the macula in Alagille syndrome
title_fullStr	Optical coherence tomography and fundus autofluorescence imaging study of chorioretinal atrophy involving the macula in Alagille syndrome
title_full_unstemmed	Optical coherence tomography and fundus autofluorescence imaging study of chorioretinal atrophy involving the macula in Alagille syndrome
title_sort	optical coherence tomography and fundus autofluorescence imaging study of chorioretinal atrophy involving the macula in alagille syndrome
publisher	Dove Medical Press
publishDate	2012
url	https://doaj.org/article/c4b44befc6c54ece9b8873d0af19dd32
work_keys_str_mv	AT makinos opticalcoherencetomographyandfundusautofluorescenceimagingstudyofchorioretinalatrophyinvolvingthemaculainalagillesyndrome AT ohkuboy opticalcoherencetomographyandfundusautofluorescenceimagingstudyofchorioretinalatrophyinvolvingthemaculainalagillesyndrome AT tampoh opticalcoherencetomographyandfundusautofluorescenceimagingstudyofchorioretinalatrophyinvolvingthemaculainalagillesyndrome
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Optical coherence tomography and fundus autofluorescence imaging study of chorioretinal atrophy involving the macula in Alagille syndrome

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