The association between drusen extent and foveolar choroidal blood flow in age-related macular degeneration.

PURPOSE: To investigate the relationship between drusen extent and foveolar choroidal blood flow in nonexudative age-related macular degeneration. METHODS: Total drusen area, average druse area, and total drusen number were determined using a computer program developed to quantify the extent of manually outlined drusen from fundus photographs of 157 patients (239 eyes) with nonexudative age-related macular degeneration. Laser Doppler flowmetry was used to assess relative choroidal blood velocity (ChBVel), volume (ChB Vol), and flow (ChBF low) in the center of the fovea. RESULTS: We found a significant inverse relationship between total drusen area and ChB Vol or ChB Flow. For every 1-mm2 increase in total drusen area, ChB Vol decreased by 0.0061 arbitrary units (P = 0.03) and ChBF low decreased by 0.23 arbitrary units (P = 0.049). Average druse area was also significantly inversely related to ChB Vol and ChBF low. For every 0.01-mm2 increase in average druse area, the ChB Vol decreased by 0.0149 arbitrary units (P = 0.001) and the ChB Flow decreased by 0.4951 arbitrary units (P = 0.003). Adjustment for age weakened the significance, although it remained strong for average druse area versus ChB Flow (P = 0.017) and ChB Vol (P = 0.004). The computer-aided quantification of drusen used in this study showed high intra- and intergrader agreement. CONCLUSION: In patients with nonexudative age-related macular degeneration, there is an association between increased drusen extent and decreased ChB Vol and ChB Flow. This suggests the presence of ischemia and possibly the reason why patients with high-risk drusen are prone to advanced disease.

Risk of relapse in primary acute anterior uveitis.

PURPOSE: To evaluate the risk of and risk factors for a second episode (relapse) among patients with remitted primary anterior uveitis. DESIGN: Retrospective cohort study. PARTICIPANTS: Patients with primary anterior uveitis presenting to 1 of 4 academic ocular inflammation subspecialty practices achieving remission of the primary episode within 90 days of initial uveitis diagnosis. METHODS: Data were obtained by standardized chart review. MAIN OUTCOME MEASURES: Time to relapse of anterior uveitis and risk factors for relapse. RESULTS: We included 102 patients with a first episode of anterior uveitis who were seen within 90 days of first-ever uveitis onset and followed for 165 person-years after achieving remission of the initial episode. Most patients were female (60%) and white (78%). Forty patients had a recurrence of anterior uveitis. The incidence of relapse was 24% per person-year (95% confidence interval [CI], 17%-33%). At 1.5 years after remission, 61% (95% CI, 48%-71%) were still in remission. Younger adults had significantly higher relapse risk than middle-aged adults (hazard ratio [18- to 35-year-old persons vs. 35- to 55-year-old persons], 2.7; 95% CI, 1.3-6.0). CONCLUSIONS: Our results suggest that many patients with remitted primary anterior uveitis presenting for tertiary uveitis care will relapse. Age in the young adult range was associated with higher risk of relapse. Given the high relapse risk, management of patients with primary anterior uveitis should include an explicit plan for detecting and managing relapses. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found after the references.

Chronic myelogenous leukemia and retinopathy treated with imatinib.

PURPOSE: To describe a case of a rapid clinical response to treatment of leukemic retinopathy and optic nerve infiltration with cytoreductive therapy, including imatinib mesylate (Gleevec), without radiation therapy. METHODS: Observational case report. RESULTS: A 13-year-old boy presented with blurry vision and a visual acuity of 20/20 in each eye. On examination, he had infiltrative retinopathy and optic nerve elevation bilaterally. Further testing revealed chronic myelogenous leukemia with severe leukocytosis. The results of the bone marrow biopsy showed the Philadelphia chromosome, t(9;22), which indicated he might respond to imatinib. He was treated with leukopheresis, followed by systemic imatinib and hydroxyurea, and his leukemic retinopathy and optic neuropathy quickly improved. CONCLUSION: This case demonstrates an excellent clinical response to systemic therapy, including imatinib, without urgent radiation. It is possible, in select cases, to consider treatment with systemic cytoreductive therapy in patients with infiltrative optic neuropathy and retinopathy with the Philadelphia chromosome.

A case report of a patient with Pfeiffer syndrome, an FGRF 2 mutation (Trp290Cys) and unique ocular anterior segment findings.

PURPOSE: To report a case of a child with Pfeiffer syndrome, unique ocular anterior segment findings and a mutation in FGFR2 (Trp290Cys). METHODS: Case Report. RESULTS: We describe a patient with Pfeiffer syndrome with a unique constellation of ocular anterior segment anomalies including microcornea, limbal scleralization, corectopia and glaucoma. Genomic DNA extraction was heterozygous for a G to T mutation at nucleotide 870 of the fibroblast growth factor receptor 2 gene (FGFR2) which changes tryptophan (TGG) to cysteine (TGT) at amino acid position 290 (Trp290Cys). CONCLUSION: This case supports the association between Pfeiffer syndrome and severe ocular anterior segment anomalies, including glaucoma, and underscores the possible role that FGFR2 has in development of the anterior segment of the eye.

The rate of retinal vessel dilation in severe retinopathy of prematurity requiring treatment.

PURPOSE: To assess whether the rate of change of retinal vessel diameter can help identify infants at the highest risk for severe retinopathy of prematurity (ROP). DESIGN: Thirty-five infants at risk for ROP were included in this prospective, longitudinal study. METHODS: Images were obtained using the NIDEK NM200D noncontact camera (NIDEK Inc, Aichi, Japan) at the time of ROP examinations in the intensive care unit. Vessel diameters were measured from digital fundus photographs of right eyes in a masked fashion using VesselMap image analysis software (IM-EDOS GmbH, Weimar, Germany). The rate of change of vessel diameter was calculated based on the linear regression slope and was compared between eyes in which type 1 ROP requiring treatment developed and in controls without ROP or with ROP less severe than type 1. RESULTS: Multivariate analysis showed that the group of eyes in which type 1 ROP developed had a greater increase in diameter over time in the inferior temporal veins (P = .01), superior temporal veins (P < .0001), mean temporal veins (P < .0001), superior temporal arteries (P = .02), and mean temporal arteries (P = .004). The area under receiver operator characteristic curve for venous diameter change was 0.96 for the superior temporal vein, 0.86 for the inferior temporal vein, and 0.96 for the mean temporal vein. CONCLUSIONS: On average, the rate of retinal vessel change was greater in eyes with type 1 ROP requiring treatment than in control eyes. The rate of venous diameter change had the best discriminative ability to differentiate between the 2 groups.

Effect of sildenafil citrate (Viagra) on retinal blood vessel diameter.

PURPOSE: The present investigation was conducted to evaluate the effect of sildenafil citrate (Viagra, Pfizer Inc., New York, NY) on retinal blood vessel diameter. DESIGN: Double-blind randomized, placebo-controlled, cross-over study. METHODS: Fifteen healthy male volunteers (mean age 39 years) received 100-mg doses of sildenafil or matching placebo on 2 separate days. Monochromatic fundus photography was obtained in one eye, and brachial artery blood pressure and intraocular pressure were measured at baseline, 1 hour, and 5 hours after dosing. The diameters of two major temporal veins and one artery were measured in a masked fashion from digitized photographic negatives. RESULTS: In comparison with placebo, no statistically significant change in average venous diameter was observed for the superior (analysis of variance [ANOVA], P =.97), inferior retinal temporal vein (ANOVA, P =.73), or the retinal temporal artery (ANOVA, P =.89) after sildenafil treatment. In comparison to placebo, there was no significant difference in the percentage change from baseline in venous or arterial diameter at 1 or 5 hours after sildenafil. The power to detect a 6.5% change in retinal vascular diameter following sildenafil was approximately 80% (P =.05). CONCLUSIONS: These data suggest that at the maximum therapeutic dose used clinically (100 mg), sildenafil does not have a significant effect on retinal vascular caliber.