Pigmented paravenous retino-choroidal atrophy. Helicoid retino-choriodal atrophy.

Abstract

The occurrence of bilateral patchy retino-choroidal atrophy in relation to the retinal venous system has been described as "retino-choroiditis radiata" (Brown, I937; HsinHsiang, I948), "chorio-retinitis striata" (Kraffel, I955), and "pigmented paravenous choroidal atrophy" (Weve, I957). The reported examples of this curious affection of the fundus all came to light as incidental findings on routine ophthalmoscopy. We wish to record a further example of this condition which was uncovered in the same way.Case report A boy aged 13 years was referred when an abnormality of the retina in each eye was noted during examination for a refractive problem. A satisfactory standard of visual acuity was achieved and normal pupillary responses were noted. Ophthalmoscopy revealed the presence of patchy areas of retinal pigment atrophy in relation to the retinal veins, superimposed on a poorly pigmented fundus background. The atrophic areas did not extend closer to the optic disc or macula than three disc diameters. Pigment clumps were present on the vein walls, sufficient in places to obscure the blood column. The choroid in the base of the atrophic areas was also atrophic (Fig. i, opposite). An area of peripapillary atrophy was present at the upper edge of the right optic disc.Some constriction of the visual field was found on kinetic perimetry, and a marked loss of retinal sensitivity was found commencing at 300 from fixation on quantitative perimetry. Colour discrimination examination by the Farnsworth Munsell One Hundred Hue Test, while revealing no recognizable hue discrimination loss pattern, showed a gross error score at the upper limit of normality for a patient of this age. Serum vitamin A and carotene concentrations were within normal limits (Vitamin A 66 ,tg./Ioo ml.; carotene 40 Ftg./Ioo ml.). Electro-oculography gave a borderline result with light maximum/dark minimum ratios of i65 per cent. in both eyes and electro-retinography also gave a normal result although the 5-minute dark-adapted b-wave amplitude was reduced to 250 [tV. Repeat electro-oculography 4 months later gave a normal result with a light maximum/dark minimum ratio of 20o per cent.The patient had been a healthy child and, apart from a measles infection at the age of 3 years, there was no significant past history. There was no familial ocular disease and the fundi ofthe parents and other siblings were normal.Fluorescein angiography revealed the presence of large geographical areas of partial atrophy, and scattered smaller areas ofcomplete atrophy of the retinal pigment epithelium. The areas of complete atrophy lay in close relation to the retinal venous system and exposed the underlying choroidal blood vessels. Some delay occurred in the filling of the retinal veins which suggested that there was a "capillary free" zone corresponding to the areas of complete atrophy. Normal "choroidal show"

Pigmented paravenous retinochoroidal atrophy (Review)

“…Fluorescein studies in young patients with this entity revealed RPE atrophy with preservation of the choroidal vasculature underlying the atrophic RPE (10). In patients with a more advanced stage of the disease, choroidal atrophy has been observed in the involved areas (15).…”

ERG and EOG in progressive paravenous retinochoroidal atrophy

“…Patients with analogous fundus changes were described by several other authors (HsinHsiang, 1948;Archer, Krill and Ernest, 1972;Chisholm and Dudgeon, 1973;Francois and De Roucke, 1974;Pearlman et al, 1975Pearlman et al, , 1978and Skalka 1979). Noble and Carr (1983) reported the longest follow-up study of 21 years.…”

Bilateral macular coloboma and pigmented paravenous retinochoroidal atrophy.

“…[12][13][14][15] Though some parts of the retina in this patient showed chorioretinal atrophy the marked perivascular pigment aggregations and chorioretinal atrophy were similar to that of the disease.…”