We emphasize the relatively favourable visual prognosis in patients suffering from inherited retinal dystrophies complicated with choroidal neovascularization. Therapeutic approaches other than laser treatment could be attempted in these patients.
The electro-oculogram (EOG) was studied in 156 normal patients, 103 patients with Best's disease, and 52 patients with dominant cystoid macular dystrophy (DCMD). Statistical analysis was performed by comparing the distribution of Lp/Dt ratios of the groups. Strength of association between Lp/Dt ratio and age was studied with correlation and regression analysis. In normal patients and in those with Best's disease, there was no significant correlation between age and Lp/Dt ratio. Obviously, the gene defect in Best's disease causes an altered light-sensitive slow oscillation that remains stable throughout life. In DCMD patients, there was a significant negative correlation between age and Lp/Dt ratio for the total sample and for the female subgroup. Likely, the gene defect in DCMD interferes with capillary permeability, that becomes susceptible to changes of the female hormones.
Digital indocyanine green videoangiography (ICGV) was done in 34 eyes of 24 patients with aging macular degeneration (AMD), including drusen, either alone (6 eyes) or in association with other AMD changes (9 eyes), geographic atrophy of the retinal pigment epithelium (2 eyes), well-defined choroidal neovascularization (CNV; 3 eyes), occult CNV (12 eyes) and recurrent CNV (11 eyes). Of the 11 eyes with soft drusen, 10 showed abnormal fluorescence in the late ICGV picture. ICGV of the 4 eyes with hard drusen showed no abnormality. The geographic atrophy of the retinal pigment epithelium and choriocapillaris remained hypofluorescent with sharply demarcated boundaries throughout the study. ICGV confirmed the presence of CNV in all 3 eyes with well-defined CNV and in 11 of the 12 eyes with occult CNV. Additionally, all but 1 eye with primary occult CNV and 6 of the 8 eyes with recurrent occult CNV could be reclassified in this study as having well-defined CNV. Overall, ICGV yielded additional information in 17 of the 20 eyes with primary and recurrent occult CNV. Its clinical importance for the evaluation of early stages of AMD has still to be confirmed by future investigations. ICGV is recommended as a diagnostic examination in eyes with CNV poorly defined by fluorescein angiography.
We report the clinical history of 2 patients affected with reticular dystrophy of the retinal pigment epithelium and central choroidal neovascularization. With time, spontaneous reduction of the subretinal fluid associated with consequent improvement of the visual acuity has been noted in our first case. The second patient showed a stable fibrotic subfoveal choroidal neovascularization. Conventional fluorescein angiography and indocyanine green videoangiography findings are illustrated. The differential diagnosis between other reticular pigmented lesions often associated with choroidal neovascularization is discussed.
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