Unilateral pigmented paravenous retinochoroidal atrophy with retinitis pigmentosa in the contralateral eye: A case report

Purpose: We describe a sporadic case of unilateral pigmented paravenous retinochoroidal atrophy (PPRCA) with retinitis pigmentosa (RP) in the contralateral eye. Observations: a 24-year-old female aware of the narrowing of visual field was examined at our hospital. Funduscopic examination revealed le...

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Main Authors: Shuichiro Aoki, Tatsuya Inoue, Mari Kusakabe, Masaya Fukushima, Kohdai Kitamoto, Asako Ogawa, Motoshi Yamamoto, Ryo Obata
Format: Article
Language:English
Published: Elsevier 2017-12-01
Series:American Journal of Ophthalmology Case Reports
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S2451993617300555
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author Shuichiro Aoki
Tatsuya Inoue
Mari Kusakabe
Masaya Fukushima
Kohdai Kitamoto
Asako Ogawa
Motoshi Yamamoto
Ryo Obata
author_facet Shuichiro Aoki
Tatsuya Inoue
Mari Kusakabe
Masaya Fukushima
Kohdai Kitamoto
Asako Ogawa
Motoshi Yamamoto
Ryo Obata
author_sort Shuichiro Aoki
collection DOAJ
description Purpose: We describe a sporadic case of unilateral pigmented paravenous retinochoroidal atrophy (PPRCA) with retinitis pigmentosa (RP) in the contralateral eye. Observations: a 24-year-old female aware of the narrowing of visual field was examined at our hospital. Funduscopic examination revealed left eye showing retinochroidal atrophy along the retinal veins with pigment accumulation while right eye showing peripheral diffuse retinal pigmented epithelium atrophy with bone spicule pigmentation. Fundus autofluorescence, electroretinogram, visual field test and optic coherent tomography were also performed and obtained results were compatible with funduscopic observation. Conclusions and importance: Simultaneous manifestation of PPRCA and RP observed in this case is rare and supports a shared genetic basis between the two diseases. Further genetic investigations are needed to elucidate the etiology and to properly manage PPRCA.
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spelling doaj.art-2c5e141ce71c4713b36ecb196d13bf6c2022-12-22T01:58:37ZengElsevierAmerican Journal of Ophthalmology Case Reports2451-99362017-12-018C141710.1016/j.ajoc.2017.08.003Unilateral pigmented paravenous retinochoroidal atrophy with retinitis pigmentosa in the contralateral eye: A case reportShuichiro AokiTatsuya InoueMari KusakabeMasaya FukushimaKohdai KitamotoAsako OgawaMotoshi YamamotoRyo ObataPurpose: We describe a sporadic case of unilateral pigmented paravenous retinochoroidal atrophy (PPRCA) with retinitis pigmentosa (RP) in the contralateral eye. Observations: a 24-year-old female aware of the narrowing of visual field was examined at our hospital. Funduscopic examination revealed left eye showing retinochroidal atrophy along the retinal veins with pigment accumulation while right eye showing peripheral diffuse retinal pigmented epithelium atrophy with bone spicule pigmentation. Fundus autofluorescence, electroretinogram, visual field test and optic coherent tomography were also performed and obtained results were compatible with funduscopic observation. Conclusions and importance: Simultaneous manifestation of PPRCA and RP observed in this case is rare and supports a shared genetic basis between the two diseases. Further genetic investigations are needed to elucidate the etiology and to properly manage PPRCA.http://www.sciencedirect.com/science/article/pii/S2451993617300555Pigmented paravenous retinochoroidal atrophy (PPRCA)Retinitis pigmentosa (RP)
spellingShingle Shuichiro Aoki
Tatsuya Inoue
Mari Kusakabe
Masaya Fukushima
Kohdai Kitamoto
Asako Ogawa
Motoshi Yamamoto
Ryo Obata
Unilateral pigmented paravenous retinochoroidal atrophy with retinitis pigmentosa in the contralateral eye: A case report
American Journal of Ophthalmology Case Reports
Pigmented paravenous retinochoroidal atrophy (PPRCA)
Retinitis pigmentosa (RP)
title Unilateral pigmented paravenous retinochoroidal atrophy with retinitis pigmentosa in the contralateral eye: A case report
title_full Unilateral pigmented paravenous retinochoroidal atrophy with retinitis pigmentosa in the contralateral eye: A case report
title_fullStr Unilateral pigmented paravenous retinochoroidal atrophy with retinitis pigmentosa in the contralateral eye: A case report
title_full_unstemmed Unilateral pigmented paravenous retinochoroidal atrophy with retinitis pigmentosa in the contralateral eye: A case report
title_short Unilateral pigmented paravenous retinochoroidal atrophy with retinitis pigmentosa in the contralateral eye: A case report
title_sort unilateral pigmented paravenous retinochoroidal atrophy with retinitis pigmentosa in the contralateral eye a case report
topic Pigmented paravenous retinochoroidal atrophy (PPRCA)
Retinitis pigmentosa (RP)
url http://www.sciencedirect.com/science/article/pii/S2451993617300555
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