Prevalence of posterior subcapsular cataracts in volunteer cytapheresis donors.

BACKGROUND: Granulocyte donors routinely receive dexamethasone orally before donation. Steroids may increase the risk of posterior subcapsular cataract (PSC) formation. STUDY DESIGN AND METHODS: We recruited 100 granulocyte donors (four or more granulocyte donations; any number of platelet [PLT] donations) and 100 age- and sex-matched PLT donors (zero to three granulocyte donations, any number of PLT donations) to examine the risk of PSC. PSC was assessed by a masked ophthalmologist and reading center lens photograph gradings or medical record documentation of PSC as the reason for cataract extraction. RESULTS: Fourteen eyes of 10 granulocyte donors and five eyes of four PLT donors had PSCs (odds ratio [OR], 2.82; 95% confidence interval [CI], 0.83-9.61; p = 0.10). Risk of PSC increased with number of granulocyte donations: compared to zero to three donations (4.0%), the risk for four to nine, 10 to 19, and 20 or more donations was 8.6% (OR, 2.25; 95% CI, 0.31-13.99; p = 0.30), 9.5% (OR, 2.53; 95% CI, 0.44-14.20; p = 0.21), and 13.0% (OR, 3.60; 95% CI, 0.48-22.81; p = 0.11), respectively (p = 0.06 for trend). CONCLUSION: We did not demonstrate a statistically significant increased risk of PSC associated with granulocyte donation. However, although this makes a large risk unlikely, we cannot rule out a small to moderate risk and there is biologic plausibility that the steroid administration associated with granulocyte donation could be associated with PSC formation. Transfusion medicine professionals should advise granulocyte apheresis donors to maintain an appropriate frequency of eye examinations.

Epidemiology and course of disease in childhood uveitis.

PURPOSE: To describe the disease characteristics and visual outcome of pediatric uveitis. DESIGN: Retrospective, longitudinal observation. PARTICIPANTS: Five hundred twenty-seven pediatric uveitis patients from the National Eye Institute, University of Illinois, Chicago, and Oregon Health Sciences University. METHODS: Retrospective chart review. MAIN OUTCOME MEASURES: Demographics, uveitis disease characteristics, complications, treatments, and visual outcomes were determined at baseline and at 1-, 3-, 5-, and 10-year time points. RESULTS: The patient population was 54% female; 62.4% white, 12.5% black, 2.7% Asian, 2.1% multiracial, and 14.61% Hispanic. Median age at diagnosis was 9.4 years. The leading diagnoses were idiopathic uveitis (28.8%), juvenile idiopathic arthritis-associated uveitis (20.9%), and pars planitis (17.1%). Insidious onset (58%) and persistent duration (75.3%) were most common. Anterior uveitis was predominant (44.6%). Complications were frequent, and cystoid macular edema (odds ratio [OR] 2.94; P = 0.006) and hypotony (OR, 4.54; P = 0.026) had the most significant visual impact. Ocular surgery was performed in 18.9% of patients. The prevalence of legal blindness was 9.23% at baseline, 6.52% at 1 year, 3.17% at 3 years, 15.15% at 5 years, and 7.69% at 10 years. Posterior uveitis and panuveitis had more severe vision loss. Hispanic ethnicity was associated with a higher prevalence of infectious uveitis and vision loss at baseline. CONCLUSIONS: The rate and spectrum of vision threatening complications of pediatric uveitis are significant. Prospective studies using standard outcome measures and including diverse populations are needed to identify children most at risk.

Clinical characterization of retinal capillary hemangioblastomas in a large population of patients with von Hippel-Lindau disease.

OBJECTIVE: To report the epidemiology and ocular phenotype of retinal capillary hemangioblastomas associated with von Hippel-Lindau (VHL) disease in a large cohort of patients and to correlate patient and ocular characteristics to visual morbidity in this population. DESIGN: Cross-sectional study. PARTICIPANTS: In 220 unrelated pedigrees, 335 patients affected with VHL disease and retinal capillary hemangioblastomas (RCHs) in at least 1 eye. METHODS: Demographics of the patient population were recorded and the ocular phenotype of each patient was obtained with a comprehensive ocular examination. MAIN OUTCOME MEASURES: The patient population was characterized and the ocular phenotype described in relationship to tumor location, number, and extent of retinal involvement. Correlations between patient demographics, ocular phenotype, and visual function were analyzed. RESULTS: We detected RCHs unilaterally in 42.1% and bilaterally in 57.9% of patients. No correlation was detected between the age, gender, or laterality of involvement. Of involved eyes, 86.6% had tumors that could be individually visualized; of these, tumors were commonly found in the peripheral retina (84.7%) only, and less commonly in the juxtapapillary area (15.3%). The tumor count in the periphery averaged 2.5+/-1.8 per eye, with 25.2% of eyes having >1 quadrant of retinal involvement. Of involved eyes, 13.4% were enucleated or prephthsical; approximately 1 in 5 patients had > or =1 eyes so affected. Severe visual impairment (visual acuity < or =20/160) in affected eyes were more likely to be associated with increasing age, the presence of juxtapapillary lesions, and an increasing number and extent of peripheral lesions. CONCLUSIONS: This large cohort of VHL patients with RCHs has enabled a systematic and quantitative characterization of the demographics, ocular features, and visual function in VHL disease. Clinical correlations between the visual morbidity and ocular features of the disease were also performed, producing measures that can help clinicians to estimate visual prognoses better based on the ocular phenotype of the disease.

The HtrA1 promoter polymorphism, smoking, and age-related macular degeneration in multiple case-control samples.

OBJECTIVE: To assess the association and combined effect on the risk of age-related macular degeneration (AMD) by the HtrA1 and complement factor H (CFH) polymorphisms, smoking, and serum cholesterol. DESIGN: Clinic-based and population-based case control study. PARTICIPANTS: A total of 805 AMD cases and 921 controls from The Eye Clinic of National Eye Institute, Age-Related Eye Diseases Study, Blue Mountain Eye Study Cohort, and Minnesota Lions Eye Bank. METHODS: DNA samples were genotyped for polymorphisms of rs11200638 in HtrA1 promoter and rs380390 in CFH. HtrA1 protein in ocular tissue was measured. Interactions of the HtrA1 risk allele with the CFH risk variant, smoking status, and cholesterol were assessed. MAIN OUTCOME MEASURES: AMD was evaluated by retinal specialists, and AMD subtypes (geographic atrophy and neovascularization) were determined. RESULTS: Strong associations of the HtrA1 risk allele (A) with AMD were present in all sample sets. A similar magnitude of association was observed for central geographic atrophy and neovascular AMD. The combination of the HtrA1 and CFH risk alleles increased AMD susceptibility, as did the combination of the HtrA1 risk allele with smoking. No combined effect of HtrA1 risk allele and cholesterol level was found. Enhanced expression of HtrA1 protein was detected in retina with AMD. CONCLUSIONS: Findings from multiple samples support an AMD genetic variant harbored within HtrA1. The risk of advanced AMD increased when the presence of risk alleles from HtrA1 was combined with either CFH risk alleles or history of smoking.

Behçet's disease: comparing 3 decades of treatment response at the National Eye Institute.

BACKGROUND: The goal of the present study was to analyze differences in response to the treatment of ocular Behçet's disease (BD) in the 1960s, 1980s, and 1990s. METHODS: Medical records of 120 patients with uveitis due to BD followed at the National Eye Institute, National Institutes of Health, from 1962 to 2004, were reviewed. RESULTS: The patients were categorized into 3 groups according to the time of follow-up: the first group was followed from 1962 until 1972, the second group from 1983 until 1992, and the third group from 1992 through 2004. Snellen visual acuity was converted to logMAR values. The range of values for inflammation was 0.5 (trace), 1 (mild), 2 (moderate), and 3 (severe). There were 45 patients (89 affected eyes) in the 1960s group, 26 patients (52 eyes) in the 1980s group, and 49 patients (94 eyes) in the most recent group. Statistical analysis showed that the mean logMAR score decreased with each decade. Mean visual acuity in the 1990s group was significantly better than in the previous decades (p < 0.001 for the 1960s group and p = 0.019 for the 1980s). The mean inflammation score was significantly higher in the 1960s than in the subsequent decades (p < 0.001 both for the 1980s and for the 1990s). INTERPRETATION: BD is a severe, blinding disorder. There was a definitive trend toward improvement in clinical outcome from the 1960s to 1990s. We attribute this trend to the introduction of newer, more potent corticosteroid-sparing agents and targeted therapy.

Genotype-phenotype correlation in von Hippel-Lindau disease with retinal angiomatosis.

OBJECTIVES: To characterize the germline mutations found in a large population of persons having von Hippel-Lindau (VHL) disease mutations with the clinical characteristics of associated retinal capillary hemangioblastomas (RCHs), to measure the prevalence of RCHs among patients with VHL disease generally and specifically for each genotype category, to establish genotype-phenotype correlations between genotype category and phenotypic features of ocular VHL disease, and to establish genotype-phenotype correlations between genotype category and visual function. METHODS: Cross-sectional and molecular genetic study. Of 890 patients with VHL disease, 335 had ocular involvement in the form of RCHs. Statistical analysis was used to correlate the structure of the mutated VHL protein with the ocular phenotype. RESULTS: Three genotype categories (amino acid substitutions, protein-truncating mutations, and complete deletions of VHL protein) were defined in all patients. The prevalence of RCHs was lowest (14.5%) among patients with complete deletions; the overall prevalence of retinal angiomatosis was 37.2%. Genotype category had no correlation with the unilaterality or bilaterality of ocular disease or with the number or extent of peripheral RCHs. The prevalence of RCHs at the juxtapapillary location was lower among patients with protein-truncating mutations compared with those with amino acid substitutions. Complete deletions were associated with the highest mean visual acuity compared with the other 2 genotype categories. CONCLUSION: Patients with complete deletions of VHL protein have the lowest prevalence of ocular disease and the most favorable visual outcome. CLINICAL RELEVANCE: The VHL mutation genotype may be used to predict the prevalence and outcome of ocular VHL disease and to guide ophthalmic follow-up.

Detection of the bcl-2 t(14;18) translocation and proto-oncogene expression in primary intraocular lymphoma.

PURPOSE: Primary intraocular lymphoma (PIOL) is a diffuse large B cell lymphoma that initially infiltrates the retina, vitreous, or optic nerve head, with or without central nervous system involvement. This study examined the expression of the bcl-2 t(14;18) translocation, the bcl-10 gene, and high expression of bcl-6 mRNA in PIOL cells. METHODS: Microdissection and PCR analysis were used to examine vitreous specimens in patients with PIOL for the presence of bcl-2 t(14;18) translocations, the bcl-10 gene, and expression of bcl-6 mRNA. A medical record review was also conducted to determine whether the bcl-2 t(14;18) translocation correlated with prognosis. RESULTS: Forty of 72 (55%) PIOL patients expressed the bcl-2 t(14;18) translocation at the major breakpoint region. Fifteen of 68 (22%) patients expressed the translocation at the minor cluster region. The bcl-10 gene was detected in 6 of 26 (23%) patients, whereas 4 of 4 (100%) PIOL patients expressed higher levels of bcl-6 mRNA compared with inflammatory lymphocytes. An analysis of clinical outcome in 23 PIOL patients revealed no significant association between bcl-2 t(14;18) translocations and survival or relapse. However, patients with the translocation were significantly younger. CONCLUSIONS: PIOL has unique molecular patterns of bcl-2, bcl-10, and bcl-6 when compared with other systemic lymphomas. This study lays the foundation for future studies aimed at exploring the genotypic classification of PIOL based on the quantitative molecular framework of gene expression profiling, with the goal of providing useful adjuncts to the pathologic diagnosis of this complex disease.

Visual outcomes in children with juvenile idiopathic arthritis-associated uveitis.

PURPOSE: To analyze visual outcomes in children affected by juvenile idiopathic arthritis (JIA)-associated uveitis. DESIGN: Retrospective interventional case series. PARTICIPANTS: Eighty-nine children with JIA-associated uveitis. METHODS: Charts of children with JIA-associated uveitis were reviewed. MAIN OUTCOME MEASURE: Change in patients' visual acuities (VAs). RESULTS: Of 269 children with uveitic syndromes referred, 89 (33%) had JIA-associated uveitis. The process was bilateral in 76 children. Seventy-three patients were female, and 84% of patients were Caucasian. Mean age of onset of uveitis was 5.7 years. Mean follow-up was 2.96 years. Antinuclear antibody positivity was detected in 56 patients, 44 of them female. Patients with JIA-associated uveitis developed numerous complications in the course of their disease: of 165 affected eyes, 105 (64%) developed cataracts, 33 (20%) developed increased intraocular pressure, and 76 (46%) developed band keratopathy; posterior synechiae were present in 96 (58%). Of 89 children, 73% were treated with immunomodulators, 40% were treated with nonsteroidal antiinflammatory agents alone or in combination with immunomodulators, and 21% were treated with topical and/or systemic steroids. Of 65 children who required immunomodulation, only one chemotherapeutic agent was used in 30, two agents in 21, and > or =3 in 14. Visual acuities of 65 children (122 eyes) were documented and compared at standard intervals. By mixed-models linear regression, improvement in VA of 0.03 logarithm of the minimum angle of resolution units per year was not found to be statistically significant (standard error, 0.02, P = 0.089). CONCLUSIONS: Juvenile idiopathic arthritis-associated uveitis is a sight-threatening disease. However, much of the children's vision can be preserved if patients are treated appropriately.

Hypergammaglobulinemia and corneal opacities in patients with human T-cell lymphotrophic virus type-1.

PURPOSE: To investigate the relationship between serum immunoglobulin levels and corneal opacities in a cohort of patients with human T-cell lymphotrophic virus type-1 (HTLV-1). DESIGN: Retrospective case series. METHODS: Complete ophthalmologic examination was performed on 44 patients with HTLV-1 infection (25 patients with adult T-cell leukemia/lymphoma [ATL], 18 patients with HTLV-1 that was associated myelopathy/tropical spastic paraparesis [HAM/TSP], and one patient who was asymptomatic). Corneal opacities were described by shape, size, color, and location. Serum immunoglobulin (Ig) levels (IgG, IgM, and IgA) were measured by nephelometry. RESULTS: Corneal opacities were identified in 15 of 25 patients (60%) with ATL and five of 18 patients (28%) with HAM/TSP. The prevalence of corneal opacities was associated statistically with elevated IgG level (P = .023) in patients with ATL, but not in patients with HAM/TSP (P > .99). CONCLUSION: Although the mechanism remains unclear, hypergammaglobulinemia is associated with the development of the corneal opacities in patients of African descent with ATL.

Associations of mortality and diabetes complications in patients with type 1 and type 2 diabetes: early treatment diabetic retinopathy study report no. 27.

OBJECTIVE: Diabetes is a leading cause of morbidity and mortality. The purpose of this study is to assess the associations between diabetes complications and mortality in the Early Treatment Diabetic Retinopathy Study (ETDRS). RESEARCH DESIGN AND METHODS: We examined demographic, clinical, and laboratory characteristics of the 3,711 subjects enrolled in the ETDRS, a randomized controlled clinical trial designed to evaluate the role of laser photocoagulation and aspirin therapy for diabetic retinopathy. The outcome assessed was all-cause mortality. Multivariable Cox proportional hazards regression was used to assess associations between diabetes complications and mortality for type 1 and type 2 diabetes separately. RESULTS: The 5-year estimates of all-cause mortality were 5.5 and 18.9% for patients with type 1 and type 2 diabetes, respectively. In patients with type 1 diabetes, amputation (hazard ratio [HR] 5.08 [95% CI 2.06-12.54]) and poor visual acuity (1.74 [1.10-2.75]) remained significantly associated with mortality, after adjusting for other diabetes complications and baseline characteristics. In patients with type 2 diabetes, macrovascular disease and worsening levels of nephropathy, neuropathy, retinopathy, and visual acuity are associated with progressively increasing risks of mortality, after controlling for other baseline risk factors. CONCLUSIONS: Amputation is the strongest predictor for mortality in patients with type 1 diabetes. All complications independently predict mortality in patients with type 2 diabetes. There is an increased risk for mortality as the degree of each complication worsens. Additional studies are needed to investigate the effectiveness of tertiary prevention to decrease mortality in these patients.

Serum inflammatory markers in diabetic retinopathy.

PURPOSE: To evaluate the association of serum factors with the severity of diabetic retinopathy and to assess their presence in retinal tissue obtained at autopsy. METHODS: The following serum factors of 93 subjects were examined at the National Eye Institute (NEI) clinical center: the chemokines regulated on activation, normal T-cell expressed and presumably secreted (RANTES)/CCL5, epithelial neutrophil activator (ENA)-78/CXCL5, interferon-induced protein (IP)-10/CXCL10, stromal cell-derived factor (SDF)-1alpha/CXCLl2, monocyte chemoattractant protein (MCP)-1/CCL2, macrophage inflammatory protein (MIP)-1alpha/CCL3, interleukin (IL)-8/CXCL8; the cytokine IL-6; the cell adhesion molecules intercellular adhesion molecule (ICAM-1/CD54) and vascular cell adhesion molecule (VCAM/CD106); and the growth factor vascular endothelial growth factor (VEGF). Logistic regression was performed to assess the association of these factors with age, sex, severity of retinopathy, hemoglobin A(1C), total cholesterol, creatinine, duration of diabetes, and presence of macular edema. The outcome assessed was severity of retinopathy. Frozen sections of two donor eyes obtained at autopsy from a donor with documented severe nonproliferative diabetic retinopathy and diabetic macular edema and of a normal nondiabetic eye were processed by immunoperoxidase staining with primary antibodies against RANTES, MCP-1, ICAM-1, and LFA-1alpha/CD11a. RESULTS: The levels of RANTES and SDF-1alpha were significantly elevated in patients with at least severe nonproliferative diabetic retinopathy compared with those with less severe diabetic retinopathy (P < 0.001 and 0.007, respectively). Positive immunostaining was observed in the inner retina for MCP-1 and RANTES of the patient with diabetes. Staining was strongly positive throughout the diabetic retina for ICAM-1. Normal retinal tissues showed little reactivity. CONCLUSIONS: Serum chemokines were significantly elevated in patients with at least severe nonproliferative diabetic retinopathy compared with those who had less severe retinopathy. Elevated levels of the chemokines and cell adhesion molecules were also identified in eyes of a donor with ischemic diabetic retinopathy. These findings provide evidence to support the role of inflammation in the pathogenesis of diabetic retinopathy.

Central visual function and the NEI-VFQ-25 near and distance activities subscale scores in people with type 1 and 2 diabetes.

PURPOSE: To investigate relationships between clinical measures of central visual function and NEI-VFQ-25 Near and Distance Activities subscales in patients with diabetic retinopathy. DESIGN: Clinic-based, cross-sectional, observational study. METHODS: The NEI-VFQ-25 was administered to 170 people with type 1 or 2 diabetes before an ocular examination that included visual acuity, contrast sensitivity, and central visual fields. Multiple linear regression and exact multiple logistic regression were used to assess the relationship between poor acuity (<69 letters), poor contrast sensitivity (<1.5 log units), and abnormal visual fields (mean deviation < or = -5dB) and NEI-VFQ-25 subscale scores. RESULTS: Final multivariable linear models explained a beta = 4.7 letter difference (P < or = .001) for each 25-point Near Activities subscale score difference. Similar effects were observed for the Distance Activities subscale, although the magnitudes of regression and partial correlation coefficients were lower (beta = 3.3 letters, P < or = .01). Final logistic regression models on abnormal clinical categories of central visual function demonstrated relationships only with the Near Activities subscale. For a 1-point change in Near Activities subscale score, the odds of obtaining a poor score for visual acuity, central visual fields, and contrast sensitivity changed by 0.08 (P < or = .001), 0.07 (P < or = .05), and 0.12 (P < or = .001), respectively. CONCLUSIONS: NEI-VFQ-25 Near and Distance Activities subscales demonstrate utility as measures of central visual function in persons with type 1 or 2 diabetes. Low scores on the NEI-VFQ-25 may reflect poor central visual fields and contrast sensitivity in addition to poor visual acuity.

Use of an arginine-restricted diet to slow progression of visual loss in patients with gyrate atrophy.

OBJECTIVE: To quantify the effect of long-term reduction of plasma ornithine levels through adherence to an arginine-restricted diet on visual function in patients of all ages with gyrate atrophy of the retina and choroid. METHODS: A long-term observational study was conducted on 27 patients with gyrate atrophy, 17 of whom elected to comply with the arginine-restricted diet and 10 who were unable to comply. The mean rates of change in the electroretinogram combined response, electroretinogram flicker response, and kinetic and static perimetry were determined. RESULTS: After mean follow-up of 13.9 years for the patients on the diet and 14.1 years for those not on the diet, the mean rates of change for the diet group compared with those of the no-diet group were statistically significantly slower for all outcome measures (age-adjusted P<.05) except for static perimetry (P =.06). CONCLUSIONS: Adhering to an arginine-restricted diet so as to lower the plasma ornithine level below an average of 5.29 to 6.61 mg/dL (400-500 micromol/L) will slow the loss of function as measured by sequential electroretinography and visual field examinations.

Dry eye signs and symptoms in women with premature ovarian failure.

OBJECTIVE: To examine whether women with premature ovarian failure (POF) have abnormal findings in ocular surface or tear parameters and whether they report symptoms of ocular discomfort compared with age-matched controls. METHODS: Sixty-five patients with POF and 36 age-matched healthy controls were examined for signs and symptoms of dry eye. The Ocular Surface Disease Index questionnaire and the 25-item National Eye Institute Visual Function Questionnaire (NEI-VFQ 25) were administered to the participants. Assessments of ocular surface damage (Oxford and van Bijsterveld scores of vital dye staining) and tear status (Schirmer tests 1 [without anesthesia] and 2 [with anesthesia] and tear breakup time) were performed. RESULTS: Women with POF scored significantly worse than controls on all ocular surface damage parameters: Oxford score (3.2 vs 1.7; P =.001), conjunctival lissamine green (2.1 vs 1.3; P =.02), corneal fluorescein staining (1.2 vs 0.4; P =.005), and van Bijsterveld score (2.1 vs 1.3; P =.02). Further, the proportion of patients with POF meeting the dry eye diagnostic criterion of a van Bijsterveld score greater than or equal to 4 was significantly greater among women with POF than among controls (20% vs 3%; P =.02). The POF group also tended to have worse scores than controls on self-reported symptoms, as measured by the overall Ocular Surface Disease Index (12.5 vs 2.1; P<.001) and the overall NEI-VFQ (94 vs 98; P =.001) after adjustment for age and race. Schirmer test scores and tear breakup time did not differ. CONCLUSIONS: Women with POF were more likely to exhibit ocular surface damage and symptoms of dry eye than age-matched controls. They were not, however, more likely to have reduced tear production. To our knowledge, this association between ocular surface disease and POF has not been previously reported. These data provide further evidence of the multifaceted role of sex hormones in the health and disease of the ocular surface.

Infantile cataract in the collaborative perinatal project: prevalence and risk factors.

OBJECTIVES: To estimate the prevalence of 4 categories of infantile cataract in subjects surviving the neonatal period in a US cohort, and to investigate risk factors for isolated infantile cataract. DESIGN: Prospective study of 55 908 pregnancies enrolled in the Collaborative Perinatal Project from 1959 to 1965 at 12 university medical centers. METHODS: We gathered data on demographic, lifestyle, and prenatal and perinatal obstetrical and postnatal factors using a standardized protocol. Pediatricians and neurologists examined infants at birth, 4 months, 1 year, and 7 years. We used exact logistic regression methods to compare putative risk factors in infants with isolated cataract with those in infants with no history of cataract. OUTCOME MEASURES: Infantile cataract as diagnosed using a standardized dilated ophthalmic examination. RESULTS: Infantile cataract occurred in 13.6 per 10 000 infants (95% confidence interval [CI], 10.7-17.1). Isolated infantile cataract occurred 3.8 times as often among infants born at weights at or below 2500 g than among those born at or above 2500 g (95% CI, 1.5-8.6; P<.001), after controlling for a set of covariates; we observed similar results for bilateral isolated cataract (odds ratio = 4.4; 95% CI, 1.2-13.9). No risk factor identified in bivariate analyses was independently associated with the odds of developing isolated unilateral infantile cataract. CONCLUSIONS: Infantile cataract is a rare disorder occurring during childhood. Prevalence estimates reported here are within the limits of those from large-cohort studies in economically developed nations. Infants born at weights at or below 2500 g have a 3- to 4-fold increased odds of developing infantile cataract.

Comparison of the NEI-VFQ and OSDI questionnaires in patients with Sjögren's syndrome-related dry eye.

BACKGROUND: To examine the associations between vision-targeted health-related quality of life (VT-HRQ) and ocular surface parameters in patients with Sjögren's syndrome, a systemic autoimmune disease characterized by dry eye and dry mouth. METHODS: Forty-two patients fulfilling European / American diagnostic criteria for Sjögren's syndrome underwent Schirmer testing without anesthesia, ocular surface vital dye staining; and measurement of tear film breakup time (TBUT). Subjects were administered the Ocular Surface Disease Index (OSDI) and the 25-item National Eye Institute Vision Functioning Questionnaire (NEI-VFQ). Main outcome measures included ocular surface parameters, OSDI subscales describing ocular discomfort (OSDI-symptoms), vision-related function (OSDI-function), and environmental triggers, and NEI-VFQ subscales. RESULTS: Participants (aged 31-81 y; 95% female) all had moderate to severe dry eye. Associations of OSDI subscales with the ocular parameters were modest (Spearman r (rho) < 0.22) and not statistically significant. Associations of NEI-VFQ subscales with the ocular parameters reached borderline significance for the near vision subscale with TBUT (rho = 0.32, p =.05) and for the distance vision subscale with van Bijsterveld score (rho = 0.33, p =.04). The strongest associations of the two questionnaires were for: ocular pain and mental function with OSDI-symptoms (rho = 0.60 and 0.45, respectively); and general vision, ocular pain, mental function, role function, and driving with OSDI-function (rho = 0.60, 0.50, 0.61, 0.64, 0.57, and 0.67, respectively). CONCLUSIONS: Associations between conventional objective measures of dry eye and VT-HRQ were modest. The generic NEI-VFQ was similar to the disease-specific OSDI in its ability to measure the impact of Sjögren's syndrome-related dry eye on VT-HRQ.

Age-related prevalence of anterior segment complications in patients with infantile nephropathic cystinosis.

PURPOSE: As a result of successful renal transplantation, patients with nephropathic cystinosis are now living into adulthood. As these patients age, anterior segment ocular complications, other than deposition of corneal crystals, become more evident. With our experience with 172 patients followed up at the National Institutes of Health between 1976 and 2000, the prevalence of anterior segment complications in nephropathic cystinosis was determined. METHODS: A cross-sectional examination of age-specific prevalence was performed with logistic regression analysis of prevalence change with age. RESULTS: Besides the corneal crystals apparent in all age groups, superficial punctate keratopathy, filamentary keratopathy, severe peripheral corneal neovascularization, band keratopathy, and posterior synechiae with iris thickening and transillumination were noted in the older age groups. The prevalence increased with age for each complication. CONCLUSIONS: As patients with cystinosis grow older, more severe ophthalmic manifestations become evident. It remains to be seen how the prevalence of these complications will be altered by early initiation of oral and topical cysteamine therapy.

Strabismus after regional anesthesia for cataract surgery.

BACKGROUND: An adult strabismus clinic in a regional eye centre provided an opportunity to estimate the risk of postoperative diplopia from regional anesthesia after cataract surgery and to describe the management of these patients. METHODS: Retrospective cohort analysis. All cases were referred over a 22-month period (November 1997 to September 1999) for investigation of postoperative diplopia after cataract surgery. The surgical and anesthetic records were reviewed to determine the number and location of injections, and the volume and type of regional anesthesia. The evolution and management of patients' diplopia was studied prospectively. RESULTS: Twenty-one patients (12 women and 9 men aged 63 to 88 [median 76] years) were ascertained with postoperative diplopia. In 11 cases the right eye was affected, and in 10 cases, the left eye. Vertical diplopia occurred most commonly from inferior rectus restriction (16 cases). Three cases resolved without treatment, which indicated that the diplopia was transient. Four patients declined treatment, five required prism in eyeglasses only, and nine required strabismus surgery. One patient required surgery and prism in eyeglasses. Two patients required more than one strabismus operation to relieve the diplopia. For the period of data collection, we estimate the risk of nontransient postoperative diplopia from regional anesthesia as 2.6 cases per 1000 cataract procedures (95% confidence interval 1.9-4.7). INTERPRETATION: Patients should be forewarned of the risk of diplopia after cataract surgery with regional anesthesia. The treatment of the diplopia may require prism in eyeglasses or strabismus surgery or both.