Biometry Characteristics in Adults and Children With Marfan Syndrome: From the Marfan Eye Consortium of Chicago.

PURPOSE: To report on the biometric findings of adults and children with Marfan syndrome (MFS) recruited from 2 annual National Marfan Foundation conferences (2012 and 2015). DESIGN: Cross-sectional study. METHODS: Subjects diagnosed with MFS by Ghent 2 nosology were included for analysis. Subjects were divided into "adults" (≥16 years of age) and "children" (5-15 years of age). Biometric data included values for refractive error, axial length (AL), corneal curvature, anterior chamber depth, lens thickness, and central corneal thickness. RESULTS: Of the 117 subjects evaluated, 74 (35 adults, 32 children, and 7 children <5 years of age) had a definite diagnosis of MFS and were included in the study. The AL was longer (25.25 ± 0.32 mm vs 24.24 ± 0.33 mm, P = .03) and the lens was thicker (3.94 ± 0.09 mm vs 3.62 ± 0.10 mm, P = .03) in adults. Both groups had flat corneas (average keratometry [Kmed] of 41.59 ± 0.35 diopters [D] in adults vs 40.89 ± 0.36 D in children, P = .17). A negative correlation was found between AL and Kmed (-0.33, P < .001). The corneas of patients with MFS with ectopia lentis (EL) were significantly flatter and with higher degree of corneal astigmatism compared to patients without EL (Kmed of 40.68 ± 0.31 D vs 41.75 ± 0.28 D, P < .01 and corneal astigmatism of 1.68 ± 0.16 D vs 1.13 ± 0.14 D, P = .01). CONCLUSIONS: Children with established MFS have flat corneas at least to the same degree as adults. Corneas of patients with MFS with EL are flatter and have a higher degree of corneal astigmatism. We strongly suggest that corneal parameters should be measured if MFS is suspected, especially in children that may not yet have developed EL.

Sarcoidosis in the national veteran population: association of ocular inflammation and mortality.

PURPOSE: To describe the distribution of ocular sarcoidosis in the veteran population and to determine the association between ocular disease and all-cause mortality. DESIGN: Retrospective review. METHODS: The Veterans Health Administration National Patient Care Database information on medical diagnoses, date of diagnosis, age, race, gender, and Veterans Administration medical center station number for site-specific calculations for fiscal years 2010 through 2012 was collected. Mortality data were obtained from the Beneficiary Identification Records Locator Subsystem. The patient cohort was identified with a primary diagnosis of sarcoidosis using International Classification of Disease, ninth edition, code of 135 in outpatient treatment files for the study period. The sarcoidosis patients were divided into those with uveitis or orbital inflammation (defined as ocular inflammation for this study) and those without uveitis or orbital inflammation. Survival analysis was performed using the Cox proportional hazard method. MAIN OUTCOME MEASURE: Association between ocular inflammation and 1-year mortality. RESULTS: Of 15 130 subjects with sarcoidosis, 3364 (22.2%) were evaluated in an eye clinic within a Veterans Administration Medical Center. Most patients were diagnosed with anterior uveitis (n = 1013; 80.7% of ocular inflammation), and the least common diagnosis was orbital granuloma (n = 28; 2.2% of ocular inflammation). Male gender was protective to the development of uveitis (estimate, 0.76; 95% confidence interval, 0.65-0.88; P = 0.0005). The overall 1-year all-cause mortality for all patients with a diagnosis of sarcoidosis was 2.0%. Ocular inflammation was associated with a decrease in 1-year all-cause mortality (simple model: hazard ratio, 0.36; P = 0.0015; complex model: hazard ratio, 0.35; P = 0.013). CONCLUSIONS: Veterans with ocular inflammation had significantly lower 1-year all-cause mortality than those without documented ocular inflammation. The reason for this finding remains to be established.

A lack of delayed intraocular pressure elevation in patients treated with intravitreal injection of bevacizumab and ranibizumab.

PURPOSE: The purpose of this study was to report the rate of intraocular pressure (IOP) elevation after intravitreal injections of anti-vascular endothelial growth factor agents for exudative age-related macular degeneration. METHODS: Retrospective chart review of all patients receiving intravitreal ranibizumab and/or bevacizumab injections for exudative age-related macular degeneration from November 2005 to June 2010. Delayed ocular hypertension (OHT) was defined as either an IOP ≥22 mmHg on 2 consecutive visits (with an increase from baseline >6 mmHg) or an IOP >26 mmHg on a single visit with a concomitant initiation or augmentation of IOP-lowering treatment. Noninjected fellow eyes served as controls. Incidence of delayed OHT was analyzed using survival analyses, with risk assessed by Cox proportional hazards regression models. Eyes with glaucoma were evaluated separately. RESULTS: Three hundred and two treated eyes and 226 control eyes met inclusion criteria. In eyes with exudative age-related macular degeneration without glaucoma, 3 of 270 injected eyes (0.51% incidence per eye-year) developed delayed OHT compared with 4 of 195 control eyes (1.00% incidence per eye-year), a difference that was not statistically significant (hazard ratio = 0.48; 95% confidence interval: 0.11-2.23). In eyes with exudative age-related macular degeneration and glaucoma, 2 of 32 injected eyes developed delayed OHT (3.1% incidence per eye-year) compared with 3 of 31 control eyes (5.7% incidence per eye-year), a difference that was not statistically significant (hazard ratio = 0.59; 95% confidence interval: 0.10-3.60). CONCLUSION: The incidence of delayed OHT after intravitreal anti-vascular endothelial growth factor injections was low and did not differ between injected and control eyes, including eyes with glaucoma. These results argue against a significant risk of IOP elevation because of repeated anti-vascular endothelial growth factor therapy.