Pigmented paravenous chorioretinal atrophy

Definition:

Pigmented paravenous chorioretinal atrophy (PPCRA) is a rare retinal disorder characterized by the presence of bilaterally symmetrical chorioretinal atrophy, with accumulation of bone corpuscle pigmentation along the distribution of the retinal veins. PPCA is bilaterally symmetric, although unilateral cases have been reported. Most cases are sporadic and may represent an acquired response pattern to an infectious or inflammatory disease. There may be an autosomal dominant modes of inheritance caused by a mutation in the CRB1 gene or an X-linked inheritance mode.

References:

[1]. A T Murray, et al. Pigmented paravenous retinochoroidal atrophy: a literature review supported by a unique case and insight. Eye (Lond). 2000 Oct;14 Pt 5:711-6. [Content Brief]

[2]. Gareth J McKay, et al. Pigmented paravenous chorioretinal atrophy is associated with a mutation within the crumbs homolog 1 (CRB1) gene. Invest Ophthalmol Vis Sci. 2005 Jan;46(1):322-8. [Content Brief]

[3]. Sergio E Hernández-Da Mota, et al. Bilateral pigmented paravenous chorioretinal atrophy: a case report. Case Rep Ophthalmol. 2011 May;2(2):228-31. [Content Brief]

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