Pigmented paravenous retinochoroidal atrophy: A literature review supported by a unique case and insight

Abstract

Pigmented paravenous retinochoroidal atrophy (PPRCA) is a rare disorder of unknown origin characterised by bone corpuscle pigmentation accumulation along the distribution of the retinal veins. In

Pigmented paravenous retinochoroidal atrophy (Review)

“…The earliest case report indicated an inflammatory cause since the affected individual had tubercular spondylitis (1), while another early case report described a child with congenital syphilis (32). Following these early studies, a number of inflammatory PPRCA cases were reported with a variety of inflammatory causes, including Behçet’s disease (33), measles (34), rubeola (35), uveitis (11,36) and other unknown causes of inflammation (4,37). However, these cases that developed due to various inflammatory agents or other definite reasons, particularly inflammatory retinal vein vasculopathy resulting from various causes, are not real PPRCA but should be considered as a pseudo PPRCA.…”

“…Certain patients who complained of poor dark adaptation, poor night vision or a blind eye did not suffer from PPRCA but instead had retinitis pigmentosa (RP) or pseudo PPRCA, as aforementioned (4,19,20,26,30,37). Particular observations in PPRCA have also been reported, including a shadow in front of the eye, diminished peripheral vision, mild pain and photopsia, involuntary closure of the upper eyelid, headaches and observing halos around lights (4,15,39). However, whether these observations are associated with the disease entity remains unknown.…”

“…Visual field changes may be normal. Depending on the topography of pigmentation and atrophy, the visual field may be manifested as a ring scotoma (20), a geographic scotoma, a quadrant defect (6), paracentral scotomas (4,17,20,26), concentric constriction (3,17,22,26,30,31,39), an enlarged blind spot (11,35) or scattered scotomas (41) corresponding to the atrophic paravenous areas. Color vision is usually normal, although mild non-specific dyschromatopsia or an acquired color vision defect has been observed in specific cases (13,26,30,31).…”

“…In the majority of cases, anterior segment and vitreous examination reveals no significant features (4). As for the pseudo PPRCA cases secondary to inflammation or other causes, slitlamp examination of the anterior segment may reveal keratic precipitates (11), cells in the anterior chamber, iridic synechiae, cataracts (11), floaters in the anterior vitreous, marked cells and snowball opacities in vitreous (37) or dense vitreous opacity (11).…”

“…However, Yanagi et al (17) considered the disease to be stationary in younger patients and slowly progressive in older subjects. Although it has been argued that specific cases of PPRCA are progressive, these patients may have pseudo PPRCA (3,4,74,75). There is no specific treatment for RPE or choriocapillaris atrophy.…”