Peripapillary Retinal Nerve Fiber Layer Thicknesses Did Not Change in Long-term Hydroxychloroquine Users

PURPOSE: To evaluate changes in the peripapillary retinal nerve fiber layer (RNFL) thicknesses using spectral-domain optical coherence tomography (SD-OCT) in hydroxychloroquine (HCQ) users. METHODS: The medical records of HCQ users were retrospectively reviewed. In these HCQ users, an automated perimetry, fundus autofluorescence photography, and SD-OCT with peripapillary RNFL thickness measurements were performed. The peripapillary RNFL thicknesses were compared between the HCQ users and the control groups. The relationships between the RNFL thicknesses and the duration or cumulative dosage of HCQ use were analyzed. RESULTS: This study included 77 HCQ users and 20 normal controls. The mean duration of HCQ usage was 63.6 ± 38.4 months, and the cumulative dose of HCQ was 528.1 ± 3.44 g. Six patients developed HCQ retinopathy. Global and six sectoral RNFL thicknesses of the HCQ users did not significantly decrease compared to those of the normal controls. No significant correlation was found between the RNFL thickness and the duration of use or cumulative dose. The eyes of those with HCQ retinopathy had temporal peripapillary RNFL thicknesses significantly greater than that of normal controls. CONCLUSIONS: The peripapillary RNFL thicknesses did not change in the HCQ users and did not correlate with the duration of HCQ use or cumulative doses of HCQ. RNFL thickness is not a useful biomarker for the early detection of HCQ retinal toxicity.

Lamina Cribrosa Changes after Laser In Situ Keratomileusis in Myopic Eyes

PURPOSE: To determine deep optic nerve head structure changes after transient intraocular pressure elevation during laser in situ keratomileusis (LASIK) for myopia. METHODS: Enhanced depth imaging-optical coherence tomography was performed in each myopic eye that underwent LASIK surgery. Enhanced depth imaging-optical coherence tomography images were created at postoperative 1 day, 1 week, 2 weeks, and 1 month. Lamina cribrosa (LC) thickness, LC depth and prelaminar thickness at the superior, middle and inferior portions of the optic nerve head were measured by two investigators. RESULTS: Forty eyes in 40 patients were included in the present study. During follow-up, there were no significant differences in prelaminar thickness or LC depth. The LC demonstrated increased thickness at postoperative 1 day at all three locations (superior, middle, and inferior) (p < 0.001, p < 0.001, p < 0.001, respectively). However, no significant changes were observed at postoperative 1 week, 2 weeks, and 1 month. CONCLUSIONS: The LC thickness could increase at 1 day after LASIK surgery. However, the thickness will gradually return to baseline morphology. Temporary intraocular pressure increase during LASIK does not appear to induce irreversible LC thickness changes.

PITX2-related Axenfeld-Rieger Syndrome with a Novel Pathogenic Variant (c.475_476delCT)

No abstract available.

Clinical Characteristics of Juvenile-onset Open Angle Glaucoma

PURPOSE: To demonstrate the clinical characteristics of juvenile-onset open angle glaucoma (JOAG) and to evaluate the prognostic factors for visual field (VF) progression in eyes with JOAG. METHODS: The medical records of 125 eyes of 72 patients with JOAG were analyzed retrospectively. At least four reliable VF tests were required to determine the VF progression, and the progression was defined using the modified Anderson criteria. Comparisons in clinical manifestations among groups were performed using independent t-test, and generalized estimating equations were also conducted. RESULTS: The mean follow-up duration was 94.4 ± 50.5 months. Patients with JOAG showed a male preponderance (64 %), myopia (−4.99 ± 4.01 diopters) and a severe elevation of intraocular pressure (35.6 ± 10.8 mmHg). Forty-two JOAG patients (58 %) had complained of symptoms associated with vision and pain; however, one-third presented with no definite symptoms. Fifty-seven patients were diagnosed with JOAG in both eyes, and they were significantly older (p = 0.039) and had a greater family history (p = 0.035) than patients with unilateral JOAG. The progression group exhibited a significantly higher intraocular pressure at the last visit (p = 0.023) than the non-progression group. CONCLUSIONS: Because patients with considerable JOAG had no definite symptoms, periodic eye examinations are needed. To prevent the VF's progression, JOAG patients may require more careful management of intraocular pressure.

Depth and Area of Retinal Nerve Fiber Layer Damage and Visual Field Correlation Analysis

PURPOSE: To evaluate the relationship between the structural damage as assessed by time-domain optical coherence tomography (OCT) and functional changes in glaucoma. METHODS: In total, 190 patients with normal tension glaucoma or primary open angle glaucoma were included in this study. The thickness of retinal nerve fiber layer (RNFL) around the optic disc and the area of RNFL defect were determined using OCT scans. The relationships between the RNFL thickness or area of the defect and visual field (VF) indices were assessed using the Lowess function, regression analysis and partial Spearman correlation. The differences between these associations depending on the stage of VF damage were further analyzed. Age, optic disc size, refraction, central corneal thickness and the presence of systemic disease were corrected for in order to exclude confounding factors. RESULTS: A logarithmic scale of RNFL thickness showed a negative linear relationship with VF indices. The area of the RNFL defect showed a weak correlation with the pattern of standard deviation, whereas the remnant RNFL thickness was moderately correlated with the pattern of standard deviation (partial Spearman correlation coefficient, 0.39, -0.47, respectively; p < 0.0001). Many outliers were detected in the Lowess-plotted graphs. Multiplication of the area and the inverted RNFL thickness showed a moderately correlated logarithmic relationship with the VF indices (partial Spearman correlation coefficient, 0.46; 95% confidence interval, 0.34 to 0.57; p < 0.0001). In the severe stage of VF damage, correlation between the area of the RNFL defect and mean deviation was significantly greater than in other stages (partial Spearman correlation coefficient, -0.66; p = 0.02). CONCLUSIONS: The thickness of the RNFL had a negative logarithmic correlation with the VF indices and was more relevant to the VF indices than the area of the RNFL defect, as measured by OCT.

Novel c.300_301delinsT Mutation in PITX2 in a Korean Family with Axenfeld-Rieger Syndrome

Axenfeld-Rieger syndrome (ARS) is characterized by anomalies of the anterior segment of the eye and systemic abnormalities. Mutations in the FOXC1 and PITX2 genes are underlying causes of ARS, but there has been few reports on genetically confirmed ARS in Korea. We identified a novel PITX2 mutation (c.300_301delinsT) in 2 Korean patients from a family with ARS. We expand the spectrum of PITX2 mutations and, to the best of our knowledge, this is the first confirmed family of PITX2-related ARS in Korea.

Assessment of the Vision-Specific Quality of Life Using Binocular Esterman Visual Field in Glaucoma Patients

PURPOSE: To investigate the correlation of binocular visual field (VF) with vision-specific quality of life in glaucoma patients. METHODS: Sixty patients who were diagnosed as open-angle glaucoma were recruited for the present study. The patients had at least moderate VF defect in 1 eye. VF testing was performed using the unilateral Humphrey Field Analyzer (HFA) and binocular Esterman VF test which was divided into 6 clusters: upper and lower center 10', upper and lower center 30', and upper and lower periphery. The 25-item National Eye Institute Visual Function Questionnaire (VFQ) was used to evaluate patients' vision-specific quality of life. We analyzed the correlation between the efficiency score of each cluster from binocular Esterman VF test, mean deviation of HFA, and the scores of VFQ (Spearman correlation). RESULTS: The correlation between the composition score of VFQ and total score of binocular Esterman visual field test was significant. The highest correlation was observed in the lower periphery cluster (all p 0.05). CONCLUSIONS: Binocular Esterman VF test is an easy and effective way to evaluate the vision-specific quality of life in glaucoma patients.

Effect of Heat Shock Protein 72 Expression on Etoposide-induced Cell Death of Rat Retinal Ganglion Cells

PURPOSE: To assess whether the expression of heat shock protein 72 (Hsp72) protects rat retinal ganglion cells (RGC-5) from apoptotic cell death. METHODS: Hsp72 expression in RGC-5 cells transduced with replication-deficient recombinant adenovirus was analyzed by Western blot analysis and immunofluorescence. The effect of Hsp72 expression on etoposide-induced apoptotic cell death was examined by microscopic analysis and confirmed by cell proliferation assay. RESULTS: Western blot analysis and immunofluorescence clearly showed adenovirus-mediated Hsp72 expression in RGC-5 cells. Treatment with etoposide resulted in the death of a proportion of the cells by apoptosis. However, this apoptotic cell death was significantly reduced in cells expressing Hsp72, with the reduction in cell death correlating to the level of Hsp72 expression. CONCLUSIONS: Over-expression of Hsp72 alone is sufficient to rescue neuronal cells from apoptotic cell death, suggesting that fine-tuning its expression may be an effective neuroprotective approach in retinal degenerative disease.

Comparative Study of Encapsulated Blebs Following Ahmed Glaucoma Valve Implantation and Trabeculectomy with Mitomycin-C

PURPOSE: To compare the histopathologic and morphologic findings of encapsulated blebs following Ahmed glaucoma valve implantation and primary standard trabeculectomy with mitomycin-C. METHODS: We reviewed the records of patients with otherwise uncontrollable glaucoma who had undergone Ahmed glaucoma valve implantation or trabeculectomy with mitomycin-C. Five eyes that underwent Ahmed valve implantation and three eyes that underwent trabeculectomy needed surgical revision of the initial surgery due to encapsulated bleb development with total loss of function. The surgically removed encapsulated blebs were analyzed macroscopically and microscopically. RESULTS: Removal of the encapsulated bleb was performed at a mean follow-up time of 26.6 +/- 19.4 weeks in the Ahmed valve implantation group and 12.0 +/- 11.4 weeks in the trabeculectomy group. The fibrotic wall of the encapsulated blebs had an overall thickness of 2.48 +/- 0.42 mm in the Ahmed valve implantation group and 1.62 +/- 0.37 mm in the trabeculectomy group. Macroscopically, the coconut flesh-like smooth surface was split into two layers, and the wall of the capsule was thicker in the Ahmed valve implantation group than in the trabeculectomy group. Histopathologically, the fibrotic capsule was composed of an inner fibrodegenerative layer and an outer fibrovascular layer, and there were no histopathological differences between the two groups. CONCLUSIONS: The fibrotic capsule wall was thicker in the Ahmed valve group, but there were no differences in histological findings between the two groups.

Optic Disc Atrophy in Patient with Posner-Schlossman Syndrome

A 32-year-old man with blurred vision in the right eye and headache presented with anterior uveitis, an intraocular pressure (IOP) of 60 mmHg, an open angle, no visual field defects, and normal optic nerve. He had a history of five previous similar attacks. In each of the previous instances, his anterior uveitis and high IOP were controlled with antiglaucoma medications and topical steroids. However, at the fifth attack, his optic disc was pale and a superior paracentral visual field defect was shown. Brain magnetic resonance image studies were normal. This case represents that a recurrent Posner-Schlossman syndrome (PSS)-induced optic disc atrophy likely due to ocular ischemia caused by a recurrent, high IOP. Although PSS is a self-limiting syndrome, we should manage high IOP and prevent ischemia of the optic nerve head by treating with ocular antihypertensive medications.

Repeatability of Intraocular Pressure Patterns in Glaucomatous Patients

PURPOSE: To investigate the repeatability of 24-hour intraocular pressure (IOP) patterns in glaucomatous patients. METHODS: The author reviewed bilateral IOP data of 18 glaucoma patients who were admitted for 2 days. IOP data were collected in both eyes every 2 hours using Goldmann applanation tonometry during a 48-hour admission except at 02:00 o'clock (13 patients) and 04:00 o'clock (5 patients). The intraclass correlation coefficient was used to analyze the agreement of IOP patterns between the first and the second 24 hours. RESULTS: IOP reached its peak between 04:00 o'clock and 06:00 o'clock in both eyes and on both days. On evaluation, the IOP data excluding the results of 02:00 o'clock and 04:00 o'clock, agreement of IOP values of 18 patients was good to excellent at 06:00, 18:00, 20:00, and 24:00 o'clock in both eyes. Agreement of IOP values at 08:00 and 12:00 o'clock was good in the right eye and the left eye, respectively. The agreement of IOP values of 13 patients who underwent IOP assessment at 04:00 o'clock and not at 02:00 o'clock, was good to excellent only at 04:00 and 06:00 o'clock in both eyes. Otherwise, agreement was poor or not significant except at 18:00 and 22:00 o'clock which showed good agreement in the right eye and the left eye, respectively. CONCLUSIONS: Presumable, a circadian IOP pattern exists, because in the present study, IOP reached its peak in the early morning on both days. However, to stipulate IOP patterns were repeatable appears to be difficult since there were numerous time points with poor agreement.

Tissue Incarceration after Ahmed Valve Implantation

PURPOSE: To report cases of intraocular pressure that was normalized, after removing the capsule that had incarcerated into the valve after Ahmed valve implantation. CASE SUMMARY: A 34-month-old boy visited our clinic for elevated intraocular pressure of the right eye. The patient had received Ahmed valve implantation 1 year earlier. At the first ophthalmological examination in our hospital, intraocular pressure of the right eye measured by Perkins tonometer was 28 mm Hg using Cosopt(R) and Iopidine(R), and 26 mm Hg after additional digital massage; the authors decided to remove the capsule with surgery. The fibrotic tissue was removed after finding the tissue incarcerated into the valve during the operation. One day after the surgery, without using any glaucoma medication, intraocular pressure of the right eye measured by Perkins tonometer was 15 mm Hg. Three months after surgery, using topical intraocular pressure lowering agents, intraocular pressure of the right eye was 18 mm Hg. Because the intraocular pressure of other patients after Ahmed valve implantation was not regulated well, surgery was performed, and the fibrotic tissue was removed after finding the tissue incarcerated in the valve during the operation.

Risk Factors for Rapid Visual Field Progression in Normal-Tension Glaucoma

PURPOSE: To evaluate risk factors of rapid visual field progression in normal-tension glaucoma. METHODS: The authors of the present study reviewed the medical records of patients with normal-tension glaucoma who were treated between 2001 and 2011. The rapid progression group was composed of patients who showed a visual field index progression rate of -1.0%/yr using a Humphrey visual field analyzer (HFA). Age, sex, diabetes, hypertension, migraine history, family history of glaucoma, refractive error, central corneal thickness, disc hemorrhage, beta-zone parapapillary atrophy, baseline mean deviation, pattern standard deviation, initial IOP, mean follow-up IOP, peak IOP, IOP fluctuation, and number of eye drops between the 2 groups were compared. RESULTS: Among the 52 patients with normal-tension glaucoma, 26 were classified into the rapid progression group and 26 were classified into the slow progression group. The mean visual field index progression rate was -3.92%/yr and -0.42%/yr, respectively. In the univariable model, migraine history, disc hemorrhage, beta-zone parapapillary atrophy, and number of eye drops were associated with rapid visual field progression. According to multivariate analysis, disc hemorrhage and number of eye drops were statistically significant risk factors. CONCLUSIONS: Disc hemorrhage and number of eye drops were associated with rapid visual field progression in normal tension glaucoma patients.

The Relations Between Progression of Peripapillary Chorioretinal Atrophy and Progression of Normal Tension Glaucoma

PURPOSE: To evaluate the relations between progression of glaucoma in visual field and peripapillary area change in normal tension glaucoma (NTG). METHODS: We respectively evaluated 66 patients (66 eyes) with normal tension glaucoma; these patients were classified as progressive glaucoma group and non-progressive glaucoma group by visual field test. Interobserver and intraobserver agreement was evaluated for qualitative assessment. Zone beta change was measured by Image J (National Institute of Health, Bethesda, USA) by two methods. One method used paired t-test and the other method used CV (correlation of variation) to define significant progression of zone beta. RESULTS: 46 patients (46 eyes) were classified as progressive glaucoma and 20 patients (20 eyes) were classified as non-progressive glaucoma. There was no statistically significant different change of zone beta between two groups and there were no changes of zone beta over coefficient of variation (CV). CONCLUSIONS: The measurement of change of zone beta seems to be of no use in recognition of progression in normal tension glaucoma.

Mechanisms Underlying Trabecular Meshwork Cell Death Caused by Mutant Myocilin Expression

PURPOSE: To determine whether the expression of mutant myocilin can lead to death of human trabecular meshwork (HTM) cells and to determine whether the mechanism by which this occurs is apoptosis. METHODS: HTM cells were transduced with a recombinant adenovirus expressing human mutant (Q368X) myocilin. The apoptotic death of HTM cells caused by expression of mutant myocilin was examined using a cell proliferation assay, flow cytometry, Western blot analysis, and immunocytochemistry. RESULTS: It appeared that the expression of mutant myocilin itself was not sufficient to cause HTM cell death. Furthermore, the expression of mutant myocilin did not lead to apoptosis of HTM cells although it did elicit a protein unfolding response. CONCLUSIONS: Our data suggest that the mechanism of myocilin glaucoma is not apoptotic death of HTM cells caused by mutant myocilin expression, and that the actual mechanism remains unknown.

Comparison of Dietary Patterns Between Glaucoma Patients and Normal Control Subjects

PURPOSE: To compare the dietary patterns between normal or high tension glaucoma patients and a normal control population. METHODS: A survey using the self-assessment food-frequency questionnaire was given to normal tension glaucoma (NTG) or high tension glaucoma (HTG) patients who visited a glaucoma clinic between June 2008 and September 2008. Healthy Korean subjects who had received routine health examinations at Samsung Medical Center between October 2007 and September 2008 were evaluated with the same questionnaire. Statistical analysis of variable factors including fatty fish, seaweeds, soy products and greasy meats, was performed to compare between groups. RESULTS: Sixty-two of NTG patients, 25 of HTG patients and 29,376 normal subjects were included in the present study. The mean age of each group was 51.3 +/- 13.4, 51.8 +/- 17.4 and 50.5 +/- 10.4 years, respectively. No significant difference in dietary pattern was found between the subjects with glaucoma and the normal controls (p > 0.2 in all factors). The dietary pattern of NTG and HTG patients showed no statistically significant difference in all factors (p > 0.2). CONCLUSIONS: There was no significant difference in a dietary pattern between glaucoma patients and normal subjects. In terms of glaucoma, no possible beneficial or harmful food was found.

The Effect of Myopic Optical Defocus on the Humphrey Matrix 30-2 Test

PURPOSE: To investigate the effect of myopic optical defocus on Humphrey Matrix 30-2 test. METHODS: Twenty-nine myopic eyes of 29 patients underwent 2 consecutive tests with the Humphrey Matrix 30-2 threshold program. The first and second tests were performed with and without correction for myopia. Differences between the mean deviation (delta MD) and the pattern standard deviation (delta PSD) were calculated, and a correlation between the spherical equivalent (SE) and delta MD and delta PSD was investigated. The influence of optical defocus according to the visual field (central and peripheral) and the severity of glaucomatous visual field damage (area with total deviation plot of 'P<0.1%' or 'P<0.5%' and the other area) were also evaluated. RESULTS: The correlation between SE and delta MD was significant (p=0.02, r=0.62). However, the correlation between SE and delta PSD was not significant (p=0.81, r=0.15). The differences in the mean total deviation printout value of the central and peripheral visual field groups between the two tests were 5.85+/-4.26dB and 5.66+/-3.56dB, respectively (P=0.86). The differences in the mean total deviation printout value of severely damaged and less damaged areas between the two tests were 3.16+/-3.39dB and 6.62+/-4.62dB, respectively (P<0.01). CONCLUSIONS: Myopic optical defocus has a significant effect on the results of the Humphrey Matrix 30-2 test. There is no difference in the effect of myopia between the central and peripheral visual fields, and the effect is decreased in severely damaged visual fields.

Intra-individual, Inter-ocular Comparison In Normal Tension Glaucoma with Unilateral Field Loss

PURPOSE: To investigate whether there are differences in ocular risk factors reportedly associated with the development of normal tension glaucoma (NTG) between glaucomatous eyes and normal visual field (VF) eyes of monocular NTG patients. METHODS: Thirty-six patients who had NTG in one eye and normal RNFL and VF in the fellow eye were included in this hospital-based cross-sectional retrospective survey. We analyzed the difference between glaucomatous eyes and normal VF eyes according to an intra-individual, inter-ocular comparison of ocular parameters, including intraocular pressures (IOP), central corneal thickness (CCT), optic disc size, myopia, optic disc hemorrhage, and zone beta of peripapillary atrophy. Measurements of optic disc size and area and the angular and radial extent of zone beta were obtained using a Heidelberg Retina Tomograph. We also evaluated the correlation of each parameter with mean deviation (MD) and pattern standard deviation (PSD). RESULTS: There were no inter-ocular differences of IOP, CCT, optic disc size, or myopia (n=36). Optic disc hemorrhage was found in only three glaucomatous eyes. The area of zone beta was greater in glaucomatous eyes than in normal VF eyes (p=0.003). No parameters were correlated with MD or PSD. CONCLUSIONS: Except for optic disc hemorrhage and zone beta area, all other parameters showed no inter-ocular differences. The asymmetry of disc hemorrhage and zone beta area in monocular NTG patients implies that optic disc ischemia might play a role in the pathogenesis of NTG.

The Effect of Age and Gender on the Intraocular Pressure in Koreans: A Cross-sectional Study

PURPOSE: To assess the effect of age and gender on intraocular pressure (IOP) in a large Korean population. METHODS: This cross-sectional study included 102,218 healthy Koreans who were aged between 20 and 79 and had no preexisting ocular conditions that could affect the IOP. All the subjects had undergone a physical check up between 1996 and 2005, and their medical records were reviewed retrospectively. Anthropometric measurements, blood tests, noncontact tonometry, and fundus examination were performed on all participants. Subjects were grouped according to decade of age. For all subjects and each age group, age and systemic variables were analyzed by multiple regression analysis on their relationship with IOP. RESULTS: A significant trend of decreasing IOP was observed in the 40s, 50s, and 60s in men, while a significant trend of increasing IOP was found in the 50s, 60s, and 70s in women. Multiple regression analysis revealed different IOP trends with age between age groups in both men and women. In general, the IOP had a significant positive correlation with systolic blood pressure, body-mass index (BMI), hematocrit, and serum cholesterol, especially with BMI in men and hematocrit in women. CONCLUSIONS: In a multiple regression analysis, the IOP trend in each age group was quite different from each other in a large Korean population, and it was suggested that women may have a steeper increasing slope (or less steep decreasing slope) of IOP with age than men. Further investigations with longitudinal study would be required to clarify the age- and gender-related physiologic changes of IOP.

Intraviteal Bevacizumab (Avastin(R)) Injection for the Treatment of Early-Stage Neovascular Glaucoma

PURPOSE: This case is conducted to assess the short term safety and efficacy of intravitreal bevacizumab injection in patient with early stage of the neovascular glaucoma (NVG) without peripheral anterior synechiae. CASE SUMMARY: A 66 year old patient with a history of proliferative diabetic retinopathy presented with neovascularization of the iris and the angle and high intraocular pressure of 30 mmHg. The patient received a single injection of bevacizumab (1.25 mg /0.05 mg) intravitreally. The visual acuity (VA), intraocular pressure (IOP), regression of the iris and the angle neovascularization were measured up to the twenty ninth week after injection. Regression of the iris and angle neovascularization were confirmed from the second week after injection. The visual acuity had continued stable and the IOP had been controlled from 30 mmHg to 20 mmHg from fifth week without the need for topical antiglaucoma medications until the twenty ninth week. CONCLUSIONS: Bevacizumab may be an effective medication for the treatment of neovascular glaucoma. Bevacizumab seems to be a useful adjunct or an advantageous treatment option to panretinal photocoagulation in the treatment of neovascular glaucoma.