Journal of The Korean Ophthalmological Society (대한안과학회지)

The Korean Ophthalmological Society (대한안과학회)
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A Case of Unilateral Focal Pigmented Paravenous Retinochoroidal Atrophy

단안에 국소적으로 발현된 색소정맥옆망막맥락막 위축 1예

  • Received : 2018.09.13
  • Accepted : 2018.11.30
  • Published : 2018.12.15
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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.

목적: 단안에 국소적으로 발생한 색소정맥옆망막맥락막 위축(pigmented paravenous retinochoroidal atrophy) 1예를 경험하여 이를 보고하고자 한다. 증례요약: 46세 여자 환자가 건강검진상에서 발견된 우안 망막 이상 소견으로 내원하였다. 나안시력은 양안 모두 1.0이었다. 세극등현미경으로 시행한 전안부 검사상 특이 이상 소견은 관찰되지 않았다. 안저검사상 우안의 상측 망막정맥을 따라 국소적으로 분포된 뼈 조각 모양의 색소침착이 동반된 망막맥락막위축 소견이 관찰되었다. 안저자가형광상 우안의 망막맥락막위축의 경계에서 다소 과형광을 보이면서 병변의 대부분은 저형광 소견을 보였다. 빛간섭단층촬영상 해당 병변은 망막색소상피층의 위축 및 뭉침이 혼재되어 있었고, 맥락막층에서 맥락막모세혈관층이 얇아진 소견을 보였다. 형광안저촬영상 병변 내 창문비침 및 형광차단이 혼재되어 관찰되었으며 이는 망막색소상피층의 위축 및 뭉침 부위와 일치하였다. 결론: 단안에 국소적으로 발생한 색소정맥옆망막맥락막 위축 1예를 경험하여 이를 국내 최초로 보고하는 바이다.

Keywords

References

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