Pigmented Paravenous Retinochoroidal Atrophy
Title: Pigmented Paravenous Retinochoroidal Atrophy
Author: Adam Jorgensen, MD
Date: 08/13/2014
Keywords/Main Subjects: Retinal degenerations, Retinitis Pigmentosa, Rod-cone dystrophy
Diagnosis/Differential Diagnosis: Pigmented Paravenous Retinochoroidal Atrophy
Brief Description of Case: A 26 year-old female presented to the retina clinic with complaints of progressive worsening of vision in both eyes over the past 12 years. She described “tunnel vision” in the right eye and poor night vision. Her medical, ocular, and family histories were unremarkable.
- This color fundus photo of the right eye shows slight optic disc pallor, attenuated vessels, and perivascular pigment clumping in a bone spicule configuration with surrounding hypopigmentation and atrophy. The left eye had asimilar appearance.
- This autofluorescence image of the left eye shows hypoautofluorescence corresponding with areas of perivascular pigment clumping and chorioretinal atrophy.
- This OCT image of the right eye shows atrophy of the outer retinal layers and retinal thinning.
Summary of the Case: Pigmented Paravenous Retinochoroidal Atropy (PPRCA) is a rod/cone dystrophy with some similar presenting features and exam findings as Retinitis Pigmentosa. The disease is usually bilateral and symmetric, and usually sporadic though some cases of familial occurrence have been reported. The cause is unknown and there is a wide spectrum of symptoms and ophthalmoscopic findings, ranging from mild to severe. Exam findings which may differentiate PPRCA from RP are: accumulation of pigment and atrophy distributed along the retinal veins, usually normal-appearing optic discs, and usually normal-appearing vessels. Fluorescein and ICG angiography and electrophysiologic testing can further aid in diagnosis. PPRCA is usually very slowly or non-progressive.
References:
- Huang, H.-B. & Zhang, Y.-X. Pigmented paravenous retinochoroidal atrophy (Review). Exp Ther Med 7, 1439–1445 (2014).
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