Quantitative analysis of radial peripapillary capillary plexuses in patients with clinically unilateral pseudoexfoliation syndrome.

PURPOSE: The aim of this study was to image the radial peripapillary capillary vessel densities (RPCvds) of the affected eyes and fellow unaffected eyes of individuals with unilateral pseudoexfoliation syndrome (PES) using optical coherence tomography angiography (OCTA) and to compare the RPCvds with those of normal age-matched individuals. METHODS: The eyes were divided into three groups: the pseudoexfoliative material (PXM)-positive eyes of patients with clinically unilateral PES (study eyes), the fellow eyes of the PXM-positive patients (fellow eyes), and the eyes of healthy patients (control eyes). Those patients with glaucomatous findings, including peripapillary hemorrhaging, cupping, notching, focal thinning of the neuroretinal rim, or intraocular pressure readings greater than 21 mmHg, were excluded from the study. The RPCvd (%), peripapillary retinal nerve fiber layer (RNFL) thickness (µm), cup/disc area ratio, rim area (mm2), disc area (mm2), and cup volume (mm3) were automatically calculated via OCTA. RESULTS: This cross-sectional comparative prospective study included 128 eyes of 88 patients: 40 PXM-positive eyes, 40 fellow eyes, and 48 control eyes. The RPCvds and RNFL thicknesses in the peripapillary region were significantly lower in the study eyes than in the fellow eyes and the control eyes (p = 0.011 and p = 0.011, p = 0.009 and p = 0.004, respectively). There were no significant differences between the fellow eyes and the control eyes with regard to the RPCvd and RNFL values in any region (p > 0.05 for all). CONCLUSION: Lower RPCvds could provoke capillary deficiency and deterioration of the perfusion of the optic nerve head in patients with PES.

Premature Expression of Pseudoexfoliation Syndrome With Presenile Cataract in a 28-Year-Old Lady.

PURPOSE: We report a case of premature expression of pseudoexfoliation syndrome with presenile cataract in a 28-year-old lady with primary developmental glaucoma who had undergone glaucoma filtration surgery 26 years ago. METHODS/RESULTS: We report a case of a 28-year-old Indian lady with progressive diminution of vision associated with photophobia in the left eye for 5 years and loss of vision in the right eye since childhood. She underwent glaucoma filtration surgery in the left eye at the age of 2 and was on 2 topical glaucoma medications when she presented to us. Refractive error was -17.00 D with -3.50 D @ 90-degree cylinder in the left eye. The right eye was phthisical. Left eye showed superior diffuse bleb, enlarged but clear cornea with superior Haab's striae, deep and quiet anterior chamber and patent surgical iridectomy at 1 o'clock position. There was diffuse iris atrophy with pseudoexfoliation at the pupillary ruff and over the anterior lens capsule. Lens showed grade 2 nuclear cataract. Intraocular pressure in the left eye was 23 mm Hg. Fundus examination showed 0.9 cupping with an inferior notch and diffuse pallor of the optic disc. Axial length of left eye was 31.44 mm. On the basis of these findings, she was diagnosed with primary developmental glaucoma and high myopia, status after glaucoma filtration surgery with presenile cataract and pseudoexfoliation in the left eye. The topical antiglaucoma medications were augmented. After 1 month, intraocular pressure in the left eye was reduced to 14 mm Hg. She was advised to continue topical glaucoma medications and regular follow-up. CONCLUSIONS: The present case is the first to describe the unusual presentation of pseudoexfoliation in a young individual along with presenile cataract. Simultaneous occurrence of pseudoexfoliation with cataract could be due to previous intraocular surgery, iris trauma, possible low-grade inflammation, and high myopia in a predisposed eye. The clinician should be aware that although a rare condition, pseudoexfoliation can occur in the young and may be associated with presenile cataract.

LOXL1 gene analysis in Turkish patients with exfoliation glaucoma.

The purpose of this study is to evaluate whole lysyl oxidase like 1 (LOXL1) gene by sequence analysis in Turkish patients with exfoliation glaucoma (XFG). A total of 48 (35 male, 13 female) patients with XFG were enrolled. Besides routine ophthalmological examination, peripapillary retinal nerve fibre layer (RNFL) analysis with optic coherence tomography was performed. Blood samples of 2 ml with EDTA were obtained and sent to Medical Genetics Department, Molecular Genetics Laboratory for LOXL1 polymorphism (PCR and agarose gel imaging) analysis. The role of the detected changes on disease severity was evaluated. No LOXL1 gene mutations in any of the patients were detected. Three types of single-nucleotide polymorphisms (SNPs) including R141L(rs1048661), A320A(rs41435250), and F184F were detected in 17 (35.3 %) patients. When compared, SNP-positive patients had thinner RNFL than SNP-negative patients (64.5 ± 17.6 and 66.1 ± 20.4 µ, respectively), and SNP-positive patients had higher cupping/disc ratio than SNP-negative patients (0.76 ± 0.2 and 0.70 ± 0.3, respectively). However, both values were not statistically significant (p = 0.966 and p = 0.539, respectively). When compared, R141L-positive patients had significantly thinner cornea thickness (516.11 ± 30.3 µ) than R141L-negative patients (556.69 ± 27.2 µ) (p = 0.004). There was not any statistical significant difference in the means of age, gender, BCVA, MD, PSD, IOP, number of hypotensive agents, and percent of glaucoma surgery (p > 0.05). In this study group of Turkish population, no LOXL1 mutations were found. No associations between the defined SNPs (A320A, R141L and F184F) and the severity of the disease were detected.

Vascular comorbidity in patients with low-tension glaucoma.

PURPOSE: To report vascular comorbidities, arterial hypertension (HT), ischemic heart disease (IHD), and diabetes mellitus (DM) in patients with low-tension glaucoma (LTG) with maximum intraocular pressure (IOP) of 18 mm Hg or less. Uniform criteria for glaucoma and the comorbidities were applied. METHODS: We reviewed records of 519 consecutive patients to whom the Finnish National Social Insurance Institution (FSII) had granted cost-free medication for the treatment of glaucoma. The FSII operates national health insurance, which is compulsory for all Finnish citizens. There were 344 patients with primary open-angle glaucoma (POAG) and 155 with exfoliative glaucoma (EG). Twenty cases were discarded for having other types of glaucoma. In the POAG group, there were 38 patients, with a median IOP of 16 mm Hg (range 12-18). We were masked to the systemic comorbidities when the registry provided us data on those to whom FSII had also granted cost-free medication for HT, IHD, or DM according to the uniform national criteria. RESULTS: None of the patients with LTG had exfoliation syndrome. There was a female predominance, 81%, compared to 68% in high-tension POAG. Hypertension had been diagnosed in 34%, which is the same as in high-tension POAG. A total of 24% had IHD, which is the same as in the Finnish population registry. Diabetes mellitus was present in only 5%. In all groups, patients with LTG with systemic comorbidity were markedly older than those without. CONCLUSIONS: In patients with LTG with median IOP 16 mm Hg (range 12-18), glaucomatous optic disc cupping and glaucomatous visual field defects probably developed independently of the systemic vascular comorbidity. However, the diagnostic criteria for HT, IHD, and DM used in the current study were based on the severity of stages set in the FSII system.

Delayed intraocular pressure elevation after pupillary dilation in exfoliation syndrome.

PURPOSE: To determine the effect of pupillary dilation on intraocular pressure (IOP) in normotensive patients with exfoliation syndrome (XFS). METHODS: Patients with XFS were enrolled in this prospective trial. All eyes were untreated, had no previous laser or operative surgery and were normotensive with full visual fields and open angles. IOP was measured before dilation and hourly for four consecutive hours after dilation with tropicamide 1% and phenylephrine 2.5% eyedrops. RESULTS: Twenty-five eyes of 19 White patients (nine male, 10 female) with XFS were enrolled. Twelve eyes (48%) had a rise in IOP of ≥ 4 mmHg above the pre-dilation baseline IOP and four (16%) had a rise of ≥ 9 mmHg (9-28 mmHg). Post-dilation gonioscopy confirmed the presence of an open anterior chamber angle in all eyes. The maximum IOP was reached 3 hr post-dilation in three eyes and after 2 hr in the remaining eyes. The four eyes with marked IOP rise exhibited an elevation of between 1 and 7 mmHg at 1 hr. Extensive pigment release was noticed in all eyes that had a rise in IOP. CONCLUSION: Patients with XFS are at risk of developing delayed post-dilation IOP rises. Awareness of this phenomenon is particularly important in patients with advanced cupping and/or severe visual field loss who may not be able to tolerate a marked elevation of IOP. An early, mild rise in IOP at 1 hr may serve as a warning sign for a more severe, delayed response. Eyes with XFS should be monitored carefully after dilation, especially those with marked pigment release.

Frequency of systemic vascular diseases in patients with primary open-angle glaucoma and exfoliation glaucoma.

PURPOSE: Abnormal fibrils can be identified by electron microscopy in the heart, lung, liver, kidney, cerebral meninges and other tissues of patients with exfoliation syndrome (ES). However, a clinical association of ES with arterial hypertension (HT), ischaemic heart disease (IHD), cerebrovascular accidents and aneurysm of the abdominal aorta is debated. We conducted a national registry-based survey to further assess the first two of these associations. METHODS: We reviewed the records of 519 consecutive patients to whom the Social Insurance Institution of Finland had granted free medication for glaucoma according to national common criteria. The glaucoma was classified either as primary open-angle glaucoma (POAG) or exfoliation glaucoma (EG), masked to any systemic diseases; 20 patients with other types of glaucoma were excluded from the survey. Masked to the type of glaucoma, the registry provided data on free medication similarly granted for HT, IHD and diabetes mellitus (DM), a known modifier of risk for cardiovascular disease. Data were analysed by logistic regression, modelling age, gender and DM as confounders. RESULTS: The control group of 344 patients with POAG was comparable as regards gender with the study group of 155 patients with EG, but patients with POAG were both younger (mean 69 versus 73 years; P < 0.0001) and had DM twice as often (10% versus 5%; P = 0.05) compared to those with EG. Adjusting for age, gender and presence of DM, no difference in frequency of HT [odds ratio (OR) 0.80 for presence of EG; 95% confidence interval (CI) 0.52-1.23, P = 0.31] or IHD (OR 0.86 for presence of EG; 95% CI 0.49-1.13, P = 0.66) was detected between the two groups. CONCLUSION: In this population-based registry survey, no difference in frequency of HT or IHD was noted between patients with POAG and EG who had been granted free medication for these chronic diseases according to national common criteria. The frequency of DM was lower among patients with EG, in line with several previous reports.

Plasma homocysteine levels and vitamin B status in patients with Pseudoexfoliation syndrome.

OBJECTIVE: To determine and compare the plasma levels of homocysteine and vitamin B (B6, B12 and folate) in patients with Pseudoexfoliation syndrome (PEXS), pseudoexfoliation glaucoma (PEXG), retinal vein occlusion with pseudoexfoliation (PEX+RVO) and in normal individuals. METHODS: The current study was conducted in the Third Eye Clinic, Ankara Numune Training and Research Hospital, Turkey, between August 2004 and February 2005. Twenty cases with PEXS (Group 1), 20 cases with PEXG (Group 2), 16 cases with PEX+RVO (central or branch retinal vein occlusion) (Group 3) and 20 normal individuals (control group) were included in the study. Those who use vitamin supplements or drugs affecting the plasma homocysteine levels were excluded from the study. RESULTS: Plasma homocysteine levels were 17.6+/-4.4 mmol/l in Group 1, 18.5+/-4.5 mmol/l in Group 2, 22.2+/-6.0 mmol/l in Group 3, and 14.0+/-3.1 mmol/l in the control group. It was highest in Group 3 (p<0.001). The ratio of hyperhomocysteinemia was calculated as 35% (Group 1), 45% (Group 2), 68.7% (Groups 3) and 15% (control). These values were statistically higher in the groups with PEXS than in the control group (p=0.009). We did not find any statistically significant difference between the groups with respect to the levels of vitamin B6 and B12 (p>0.05), but the level of folate was lowest in Group 3 (p<0.001). CONCLUSION: Hyperhomocysteinemia is a risk factor for thromboembolic vasculopathy in patients with PEXS and PEXG. Therefore, vitamin B supplementation should be considered in these patients when hyperhomocysteinemia is detected.

Clinical signs of the pigment dispersion syndrome in blacks.

BACKGROUND: The pigment dispersion syndrome (PDS) is considered rare in blacks, and minimal literature exists concerning the condition in this patient population. The diagnosis of PDS in blacks may present unique challenges because some of the typical clinical signs that are present in whites, including iris transillumination defects, posterior iris bowing, and noticeable anterior iris stromal pigment dusting, may not occur as commonly. Diagnosis can be particularly difficult when neither these signs nor significant corneal endothelial pigmentation exists. Although zonular and peripheral lens pigment has been found to be consistently present in whites with PDS, attention has not been given to this as a potentially important diagnostic sign in blacks. METHODS: From among a primary care population, we identified and studied 7 patients (13 eyes) who exhibited moderate to heavy trabecular meshwork (TM) pigmentation, as well as zonule and/or peripheral lens pigmentation. Patients were identified during routine clinical care provided by one of the authors, as well as from notification by other practitioners. All patients received complete eye examination and other signs of PDS were looked for. RESULTS: Four males and 3 females were identified, their average age being 37 years (range = 15 to 51) at the time of their initial identification. All but one patient was myopic (average approximately -2.50 D spherical equivalent). Iris transillumination defects were present in only one eye of one patient, and no eyes showed overt posterior iris bowing, although the iris contours were usually flat and the anterior chambers appeared relatively deep. Corneal endothelial pigmentation was frequently barely detectable and could not be relied on as a predictor of trabecular meshwork or lenticular pigmentation. Glaucoma, or a suspicion of glaucoma due to increased intraocular pressure (IOP) or cupping, was common among the group. Using heavy TM pigmentation as well as any degree of zonular and/or peripheral lenticular pigmentation as a criteria for the diagnosis of PDS, we calculated the prevalence of PDS among blacks in a nonreferred primary care population (> age 7) to be at least 15 cases per 10,000. CONCLUSIONS: More investigation is needed to study the clinical presentation of PDS in blacks because it may be substantially different than in whites. Zonular and peripheral lenticular pigmentation may be a particularly useful diagnostic sign of PDS in blacks, especially in those cases where other traditional signs, including iris transillumination defects, pronounced corneal endothelial pigmentation, posterior iris bowing, and visible anterior iris stromal pigment dusting, are absent. The "classic" variety of PDS may be more common among blacks than previously recognized.