J Korean Ophthalmol Soc > Volume 59(12); 2018 > Article
Journal of the Korean Ophthalmological Society 2018;59(12):1190-1194.
DOI: https://doi.org/10.3341/jkos.2018.59.12.1190    Published online December 15, 2018.
A Case of Unilateral Focal Pigmented Paravenous Retinochoroidal Atrophy.
Hyo Song Park, Jong Yun Yang, Hyun Ju Park
1Siloam Eye Hospital, Seoul, Korea. hjpeye@siloam.co.kr
2The Institute of Vision Research, Department of Ophthalmology, Yonsei University College of Medicine, Seoul, Korea.
단안에 국소적으로 발현된 색소정맥옆망막맥락막 위축 1예
실로암안과병원1, 연세대학교 의과대학 안과학교실 시과학연구소2
Correspondence:  Hyun Ju Park,
Email: hjpeye@siloam.co.kr
Received: 13 September 2018   • Revised: 23 October 2018   • Accepted: 30 November 2018
We report a case of unilateral, focal, pigmented paravenous retinochoroidal atrophy (PPRCA). CASE SUMMARY: A 46-year-old female visited our clinic in complaint of a vague problem with her right eye identified during a general medical examination. The visual acuity (without correction) of both eyes was 1.0. Slit-lamp examination of both eyes revealed no specific signs. Fundus examination of the right eye revealed focal, bony-spicule-shaped retinochoroidal atrophy with pigmentation along the course of the superior retinal vein. A fundus autofluorescence examination revealed principally hypofluorescence with some hyperfluorescence at the margin of the atrophic retinochoroidal lesion. Optical coherence tomography revealed mixed clumping and atrophy of the retinal pigment epithelium (RPE) layer and thinning of the choriocapillaris layer. Fluorescence angiography revealed a window defect and blockage at the site of the lesion (the fluorescent material did not enter the lesion). The site of the window defect was in correlation with the atrophic RPE region. The site of the blockage at lesion also matched with the site of the regional pigment clumping. No definite leakage was observed. CONCLUSIONS: To the best of our knowledge, this is the first case of unilateral focal PPRCA reported from Korea.
Key Words: Pigmented paravenous retinochoroidal atrophy;Retinal pigment epithelium

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