Pigmented paravenous retinochoroidal atrophy: Evidence of progression to macular involvement in a family with a 42-year history

Abstract

Sir,Pigmented paravenous retinochoroidal atrophy:evidence of progression to macular involvement in a family with a 42-year history

Pigmented paravenous retinochoroidal atrophy (Review)

“…Visual loss is usually mild and nyctalopia is not common. 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).…”

“…In specific cases, central visual acuity may be mildly reduced. Severely reduced vision has also been observed as a result of rare changes, including anisometropia amblyopia (19), cataracts (11), glaucoma (26,40), nystagmus (19,20), vitreous opacity (37) and macular involvement (detail vide infra ). However, these changes may be observations specific to individuals rather than associations within the phenotype.…”

“…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).…”

“…Macular changes include cystoid macular edema (37), star-shaped exudate (35), macular wrinkling (31), epiretinal membrane (6), macular pigmentary stippling (35), pigmentary macular degeneration (15,26), macular depigmentation (17), lamellar macular holes (42), macular dysplasia (43), macular RPE atrophy (17), central areolar macular atrophy (19), excavated macula (19) and macular coloboma (14,30,44). However, whether these macular observations are associated with the phenotype of the disease entity or distinct findings remains unknown.…”

“…Other ocular problems have been reported as complications to PPRCA, including angle closure glaucoma (16,26,40), nystagmus (19,20), hypermetropia (13,19,40), hyperopic astigmatism (31), anisometropia (13,19,24), amblyopia (19,24), convergent squint (26), esotropia (19,40) and exotropia (6). However, these problems may only be particular observations and not associations within the phenotype.…”