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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.

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.


  1. Huang, H.-B. & Zhang, Y.-X. Pigmented paravenous retinochoroidal atrophy (Review). Exp Ther Med 7, 1439–1445 (2014).

Copyright:  Adam Jorgensen, ©2015. Please see terms of use page for more information.