J Korean Ophthalmol Soc.  2018 Dec;59(12):1190-1194. 10.3341/jkos.2018.59.12.1190.

A Case of Unilateral Focal Pigmented Paravenous Retinochoroidal Atrophy

• Park HS
• Yang JY
• Park HJ

• KMID: 2428693
• DOI: http://doi.org/10.3341/jkos.2018.59.12.1190

Abstract

PURPOSE
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.

Keyword

Pigmented paravenous retinochoroidal atrophy; Retinal pigment epithelium

MeSH Terms

Atrophy*
Female
Fluorescein Angiography
Humans
Korea
Middle Aged
Pigmentation
Retinal Pigment Epithelium
Retinal Vein
Tomography, Optical Coherence
Visual Acuity

Figure

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  Figure 1 Wide fundus photography and fundus autofluorescence of the patient's right eye. (A) Focal bony- spicule shaped retinochoroidal atrophy with pigmentation along superior retinal vein was noted. (B) Besides some hyperfluorescence at margin of retinochoroidal atrophy, most part of the lesion showed hypofluorescence.

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  Figure 2 Optical coherence tomography of the patient's right eye. (A) Mixed signals of clumping and thinning of retinal pigment epithelium layer were noted. (B) Thinning of choriocapillaris layer was noted at the lesion site while choriocapillaris layer thickness was within normal range at locations other than the lesion site. S = superior; N = nasal; I = inferior; T = temporal.

• 

  Figure 3 Fluorescence angiography of the patient's right eye. (A) Arterial phase and (B) venous phase shows window defect with hyperfluorescence and blockage of fluorescence at the lesion. No definite leakage was noted.

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