A randomised prospective study of outpatient haemodilution for central retinal vein obstruction.

PURPOSE: Central retinal vein obstruction (CRVO) has significant visual morbidity. We prospectively evaluated an outpatient haemodilution (HD) regimen for CRVO. METHODS: We recruited 59 patients with CRVO of less than three months' duration and visual acuity (VA) worse than or equal to 6/9.5. Thirty patients underwent HD (packed cell volume of <0.35, 12 weeks); there were 29 controls and follow-up was for six months. RESULTS: Incidence rates for VA improvement (P = 0.708) and rubeosis iridis (P = 0.619) between the two groups were not different. The incidence rate of VA deterioration was 5.315 times higher with HD (P = 0.035, Cox Proportional analysis). CONCLUSION: This data does not support the previous studies on haemodilution.

The venous closing pressure in central retinal vein obstruction.

OBJECTIVE: To assess the rate of change in the central retinal venous closing pressure in central retinal vein obstruction over time, and its relationship to visual acuity improvement and the development of rubeosis iridis. METHODS: Fifty patients presenting with central retinal vein obstruction of less than three months' duration, between the ages of 40 and 80 years, were reviewed prospectively. The central retinal venous closing pressure was measured by digital ocular compression. Patients were discharged from the study after the six-month visit. RESULTS: All patients had elevated venous closing pressure at presentation, whereas at six months only 24 patients had persistent elevation. Of 16 patients with lowering of the venous closing pressure within four months of onset of central retinal vein obstruction, 11 (69%) had two or more lines of visual acuity improvement. Only two of 10 patients (20%) developing lowering of the venous closing pressure thereafter had visual improvement. No patient developed rubeosis iridis after the venous closing pressure lowered. CONCLUSION: The central retinal venous closing pressure is raised in central retinal vein obstruction to about central retinal arterial diastolic pressure, and is its pathognomonic sign. This sign is easily elicited via digital pressure on the eyelid, and has prognostic significance for visual acuity improvement and the development of rubeosis iridis.

Indocyanine green angiography in Stargardt's flavimaculatus.

PURPOSE: We studied the indocyanine green videoangiographic characteristics of eyes in patients with Stargardt's flavimaculatus and fluorescein angiographic evidence of a dark choroid. METHODS: Affected individuals underwent ophthalmic examination and fluorescein angiographic examination. Indocyanine green videoangiography was performed on eight patients with classic Stargardt's flavimaculatus. Two additional asymptomatic patients with mild manifestations of Stargardt's flavimaculatus, both of whom were related to one patient with the classic phenotype, were also examined with indocyanine green videoangiography. RESULTS: Choroidal detail was evident in all patients examined with indocyanine green videoangiography, and varying degrees of choroidal vascular closure were documented in the maculas of eight patients. Retinal pigment epithelial flecks were found to block indocyanine green videoangiographic fluoresence progressively. Late indocyanine green videoangiographic imaes frequently showed retinal pigment epithelial involvement in areas of retina thought to be uninvolved clinically and by fluorescein angiography. Peripapillary crescents of hypofluorescence, which in some patients were not noted clinically or by fluorescein angiography, were observed in all ten patients examined with indocyanine green videoangiography. In one asymptomatic patient, retinal pigment epithelial flecks could be identified only with indocyanine green videoangiography. CONCLUSIONS: Indocyanine green videoangiography in conjunction with fluorescein angiography can be a valuable tool in the recognition and further understanding of Stargardt's flavimaculatus.

Ocular findings associated with a 3 base pair deletion in the peripherin-RDS gene in autosomal dominant retinitis pigmentosa.

Affected members of a family with autosomal dominant retinitis pigmentosa were found to have a 3 base pair deletion at codon 118 or 119 of the retinal degeneration slow gene. This mutation causes the loss of a highly conserved cysteine residue in the predicted third transmembrane domain of peripherin-rds, a photo-receptor specific structural glycoprotein localised to both rod and cone outer segment disc membranes. Four of these individuals underwent detailed clinical, psychophysical, and electroretinographic testing in order to characterise their photoreceptor dysfunction. Nyctalopia was reported early in the second decade by all patients. Global rod and cone dysfunction was recorded by the third decade with severe reduction of both photopic and scotopic function by age 30 years. This retinal degeneration slow gene mutation may lead to the primary loss of both rod and cone photo-receptor function.

Macular dystrophy associated with mutations at codon 172 in the human retinal degeneration slow gene.

BACKGROUND: Recently, mutations in the retinal degeneration slow (rds) gene which codes for peripherin-rds have been implicated as a cause of autosomal dominant retinitis pigmentosa. Because this gene is expressed in both rods and cones, mutations in the rds gene might be expected to cause degeneration affecting either the scotopic or photopic systems. Mutations at codon 172 of the rds gene have been identified in three families with autosomal dominantly inherited, progressive macular dystrophy. METHODS: Affected individuals underwent ophthalmic examination, scotopic perimetry, dark adaptometry, measurement of color-contrast sensitivity, and electroretinography to characterize the photoreceptor dysfunction. RESULTS: In all but one affected member, symptoms of progressive central visual loss developed in the third or fourth decade of life accompanied by central scotoma and well-demarcated atrophy of the retinal pigment epithelium and choriocapillaris of the macula. In general, cone and rod thresholds were elevated, and color-contrast sensitivity was absent in the central visual field. Peripherally, the scotopic sensitivities were normal, as was the recovery from bleach. Cone electroretinograms were diminished in amplitude, and delayed in all affected adults except one. Rod electroretinograms were normal or near normal in amplitude, and had normal implicit times. Affected asymptomatic children had macular changes, abnormal color-contrast sensitivity, and reduced pattern and cone electroretinograms. CONCLUSION: These results indicate that mutations in the rds gene can be expressed as a macular dystrophy, with evidence of primary cone dysfunction and preservation of peripheral rod function.