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To study the correlation between the visual fields (VF) and wide-field fundus autofluorescence (FAF) in patients with retinitis pigmentosa (RP).Retrospective, observational, consecutive case series.Twenty-four eyes of 12 patients diagnosed with RP were enrolled. The VFs measured by Goldmann perimetry and wide-field FAF images were compared for each eye. The relationship between the areas of hypoautofluorescence on the wide-field FAF images and scotoma on Goldmann perimetry were evaluated. The VF and FAF images in the central 60 degrees were trimmed and superimposed to calculate the percentage agreement between the hypoautofluorescence and the scotomas and between the isoautofluorescence and hyperautofluorescence and the remaining VFs.The areas of hypoautofluorescence on the FAF images were correlated significantly (R = 0.86, P.001) with the areas of the VF defects on Goldmann perimetry. The mean percentage agreement between the hypoautofluorescence and the scotomas was 91.0% ± 7.7% and that of the isoautofluorescence and hyperautofluorescence with the remaining VFs was 84.5% ± 7.4%. The areas of geographic hypoautofluorescence with or without hyperautofluorescent bands reflected the VF defects, while nummular or mottled hypoautofluorescence without VF defects was seen in 7 eyes.These results suggested that wide-field FAF imaging is useful to evaluate the remaining retinal function in patients with RP. Abnormal fundus autofluorescence precedes loss of retinal function and is helpful for monitoring disease progression.

Purpose To assess the visualization of the retinal microvasculature with intravenous fluorescein angiography (IVFA) compared to the Retinal Function Imager (RFI). Design Multicenter, retrospective, observational case series. Methods Seven normal eyes and 26 eyes with various ocular diseases were imaged with both IVFA and the RFI. The ability to assess vessel loops, vertical collateral vessels, the size of the foveal avascular zone (FAZ), and degree of vessel branching were compared between IVFA and RFI images. Results The RFI visualized a greater number of vessel loops (1.3 vs 0.4 per eye) and vertical collateral vessels (4.42 vs 0.97 per eye) than IVFA. On average, higher order of vessel branching was seen with the RFI compared to IVFA (5.2 vs 4.6). The foveal avascular zone (FAZ) was more clearly delineated using the RFI and was significantly smaller when measured on RFI (0.35 vs 0.75 mm 2 ). Conclusions RFI, a noninvasive retinal imaging instrument, revealed vessel loops, vertical collateral vessels, the area of the FAZ, and order of vessel branching in greater detail than IVFA. This instrument may be helpful in understanding dynamic retinal vascular changes in a number of common ocular diseases, as well as in normal eyes.

We measured the contrast sensitivity function in a 16-year-old boy with cystic fibrosis, before and during vitamin A supplementation. Before vitamin A supplementation, serum levels of vitamin A were abnormally low, the electroretinogram was reduced, and contrast sensitivity was abnormally low at all spatial frequencies. During vitamin A supplementation (25,000 IU/day), serum levels of vitamin A became low normal, the electroretinogram returned to normal, and the overall contrast sensitivity function improved by 94%. We propose that the contrast sensitivity function may be abnormal in patients with cystic fibrosis who have reduced retinal function secondary to vitamin A deficiency.

To examine the effects of lutein and zeaxanthin supplementation on retinal function using multifocal electroretinograms (mfERG) in patients with early age-related macular degeneration (AMD).Randomized, double-masked, placebo-controlled trial.One hundred eight subjects with early AMD were randomly assigned to receive 10 mg/d lutein (n = 27), 20 mg/d lutein (n = 27), 10 mg/d lutein plus 10 mg/d zeaxanthin (n = 27), or placebo (n = 27) for 48 weeks. Thirty-six age-matched controls without AMD were also enrolled to compare baseline data with early AMD patients. MfERG responses and macular pigment optical densities (MPODs) were recorded and analyzed at baseline and at 24 and 48 weeks.There were significant reductions in N1P1 response densities in ring 1 to ring 3 in early AMD patients compared with the controls (P.05), whereas neither N1P1 response densities in ring 4 to ring 6 nor P1 peak latencies significantly changed. After 48-week supplementation, the N1P1 response densities showed significant increases in ring 1 for the 20 mg lutein group and for the lutein and zeaxanthin group, and in ring 2 for the 20 mg lutein group. The increases in MPOD related positively to the increases in N1P1 response density in ring 1 and ring 2 for nearly all active treatment groups. N1P1 response densities in ring 3 to ring 6 or P1 peak latencies in all rings did not change significantly in any group.Early functional abnormalities of the central retina in the early AMD patients could be improved by lutein and zeaxanthin supplementation. These improvements may be potentially attributed to the elevations in MPOD.

Six patients (three men and three women, ranging in age from 19 to 65 years) with pigmented paravenous chorioretinal atrophy, a rare disorder of unknown origin, were studied for three- to 21-year periods. The diagnosis was made on the basis of the distinctive fundus appearance of bilateral, symmetrical bone corpuscular pigment accumulation exclusively along the distribution of the retinal veins. Although the fundus abnormalities can be mild or severe, retinal function tests indicated that this is a geographic and not a generalized disorder. Central visual acuities were normal. Follow-up studies showed no evidence of ophthalmoscopic or functional deterioration in five of the patients. In the sixth (Patient 6, the most severely affected), there was a mild progression of the fundus abnormality during the 21-year follow-up period.

Evaluation of 25, 50, and 100 mug of pimaricin given intravitreally to albino rabbits with Aspergillus endophthalmitis revealed that 25 mug of pimaricin, while nontoxic to the retina, was ineffective in inhibiting the fungal process. A dose of 50 mug was significantly more effective in inhibiting fungal disease but also caused significant retinal damage with loss of retinal function and iridoplegia. Dosages above this level resulted in vitreous retraction and degeneration, iridoplegia, and retinal detachment. Twenty-five micrograms of pimaricin given in three dosages spaced three days apart may be effective in inhibiting fungal endophthalmitis.

Ten patients with Stargardt's disease and 14 with fundus flavimaculatus underwent thorough ophthalmic examinations, retinal photography, and, when possible, fluorescein angiography. Retinal function was also assessed by static and kinetic perimetry, the Farnsworth-Munsell 100-hue test, electro-oculography, and electroretinography. Visual acuity and color discrimination were reduced in all patients (mean visual acuity, 20/120; mean error score, 365). On electroretinography all patients had some significant abnormality of cone function and 24 eyes had abnormal rod function (mean V max , 298.3). Electro-oculographic findings were abnormal in 24 eyes and borderline in ten others. These abnormalities were similar in both groups but more severe in fundus flavimaculatus. Stargardt's disease and fundus flavimaculatus did not co-exist in any family studied and the mean duration of disease was similar in both, indicating that Stargardt's disease did not progress to fundus flavimaculatus. Both the age of onset and duration significantly affected the severity of fundus flavimaculatus but neither had a significant effect on Stargardt's disease.