RETINAL PIGMENT EPITHELIUM UNDULATIONS IN ACUTE STAGE OF VOGT-KOYANAGI-HARADA DISEASE: Biomarker for Functional Outcomes After High-Dose Steroid Therapy.

PURPOSE: To determine the clinical significance of retinal pigment epithelium (RPE) undulations in the acute stage of Vogt-Koyanagi-Harada disease. METHODS: Retinal pigment epithelium undulations were detected and classified into 3 grades: Grade 1, slight; Grade 2, moderate; and Grade 3, severe undulations, in the enhanced depth imaging optical coherence tomographic images. The relationship between the clinical characteristics and the presence of RPE undulations was investigated. RESULTS: Among the 61 eyes of 31 patients with Vogt-Koyanagi-Harada disease, 40 eyes had some degree of RPE undulations (Grade 1 = 12, Grade 2 = 15, and Grade 3 = 13). The patients with RPE undulations in both eyes were significantly older at the onset (P = 0.0002). The eyes with RPE undulations were more likely to develop posterior recurrences (P = 0.032) and have worse vision at 12 months (P = 0.043). Multiple regression analysis revealed that RPE undulations were an independent predictor of posterior recurrences (P = 0.009) and poor visual outcomes (P = 0.035). CONCLUSION: Retinal pigment epithelium undulations detected by enhanced depth imaging optical coherence tomographic are relatively frequent occurrences at the acute stage of Vogt-Koyanagi-Harada, and their presence is a predictor of posterior recurrences and poor visual outcomes after high-dose steroid therapy.

Choroidal thickness and visual acuity in highly myopic eyes.

PURPOSE: To examine predictive factors for visual acuity in highly myopic eyes. METHODS: Consecutive patients with high myopia (≥6 diopters [D]) with no other pathology such as lacquer cracks in the fovea, choroidal neovascularization, or myopic macular schisis, were evaluated. The study was performed in 2 retina centers, one in the United States and the other in Japan. Enhanced depth imaging optical coherence tomography was obtained, and the central foveal, outer retinal hyporeflective layer and inner segment to retinal pigment epithelium aggregate, and the subfoveal choroidal thicknesses were measured. Correlations were calculated among the measured variables and visual acuity. Generalized estimating equation models were used to identify predictors of visual acuity. RESULTS: The New York cohort was composed of 35 eyes of 25 patients who had a mean age of 57 years (standard deviation, ±18.1 years) and a mean refractive error of -10.9 D (±3.6 D). The Japanese cohort was composed of 110 eyes of 61 patients who had a mean age of 46.8 years (±14.7 years) and a mean refractive error of -9.2 D (±3.1 D) and a mean axial length of 27 mm (±1.4 mm). The mean subfoveal choroidal thickness was 113.3 µm (±53.9 µm) in the New York group and 172.9 µm (±72.8 µm) in the Japanese group. In each group, the subfoveal choroidal thickness showed a significant inverse correlation with age and myopic refractive spherical equivalent. The subfoveal choroidal thickness was inversely correlated with logarithm of the minimum angle of resolution visual acuity (P = 0.041, New York group; P = 0.001, Japan group). The only significant predictor in the pooled data for logarithm of the minimum angle of resolution visual acuity was subfoveal choroidal thickness (P ≤ 0.001). Clinic location was not a significant predictor. CONCLUSION: Choroidal thickness in high myopia is inversely correlated with increasing age and myopic refractive error and is an important predictor of visual acuity. Given that myopia is increasing worldwide, these findings may have epidemiologic significance.

Subfoveal choroidal thickness after treatment of Vogt-Koyanagi-Harada disease.

PURPOSE: To evaluate the subfoveal choroidal thickness in Vogt-Koyanagi-Harada (VKH) disease using enhanced depth imaging optical coherence tomography. METHODS: Retrospective observational study. Subfoveal choroidal thickness was measured using enhanced depth imaging optical coherence tomography, in which the optical coherence tomography instrument was placed close enough to the eye to obtain an inverted image, which was averaged for 100 scans. All patients were diagnosed as having the ocular findings of VKH disease with or without extraocular disorders. The patients were followed during their initial treatment with corticosteroids. RESULTS: All 8 patients (16 eyes) with acute phase VKH disease presented with thickening of the choroid. The serous retinal detachment disappeared in 1 month after corticosteroid treatment. The mean choroidal thickness in 16 eyes decreased from 805 ± 173 µm at the first visit to 524 ± 151 µm at 3 days (P < 0.001) and 341 ± 70 µm by 2 weeks (P < 0.001). CONCLUSION: Patients with active VKH disease have markedly thickened choroids, possibly related not only to inflammatory infiltration but also to increased exudation. Both the choroidal thickness and the exudative retinal detachment decreased quickly with corticosteroid treatment. Enhanced depth imaging optical coherence tomography can be used to evaluate the choroidal involvement in VKH disease in the acute stages and may prove useful in the diagnosis and management of this disease noninvasively.

Fundus autofluorescence and visual acuity in central serous chorioretinopathy.

PURPOSE: To investigate the fundus autofluorescence (FAF) abnormalities in central serous chorioretinopathy (CSC) and evaluate potential correlations with visual acuity. DESIGN: Retrospective, observational case series. PARTICIPANTS: Four hundred seventy-five eyes of 238 patients with CSC. METHODS: Consecutive patients with CSC underwent FAF imaging, as well as routine ophthalmologic examinations. Confluent hypoautofluorescence was defined as a region of absent autofluorescence greater than one fourth of a disk diameter. Granular hypoautofluorescence was defined if there was a grainy or coarse region of decreased fluorescence as compared with normal surrounding areas greater than one fourth of a disc diameter in size. A descending tract was a downward leading swathe of decreased autofluorescence originating from the posterior pole to extend below the inferior arcade. MAIN OUTCOME MEASURES: The pattern and frequency of FAF abnormalities and their correlations with corrected visual acuity. RESULTS: The mean age of the subjects was 57.1 years (standard deviation, 13.3), and 181 (76.1%) were male. Confluent and granular hypoautofluorescence was detected in the macula of 54 (11.4%) and 300 (63.2%) of 475 eyes, respectively. Descending tracts from the macula were observed in 43 (9.1%) eyes and from the optic disc in 43 (9.1%) eyes. Multiple regression analysis revealed that confluent hypoautofluorescence of the macula, granular hypoautofluorescence of the macula, and increasing age all were independent predictors of decreased visual acuity. CONCLUSIONS: The FAF abnormalities in CSC show multiple distinct patterns and seem to provide functional information.

Vitreous levels of vascular endothelial growth factor in eyes with anterior hyaloidal fibrovascular proliferation.

PURPOSE: To determine the intravitreal levels of vascular endothelial growth factor (VEGF) in eyes with anterior hyaloidal fibrovascular proliferation (AHFVP). METHODS: Three eyes of three patients who underwent vitrectomy for proliferative diabetic retinopathy (PDR) and subsequently developed an AHFVP (AHFVP group) were studied. We measured the level of VEGF in vitreous samples collected at the primary and following operations by enzyme-linked immunosorbent assay. The vitreous levels of VEGF in 25 eyes of 22 patients with PDR were also studied as controls (PDR group). RESULTS: The averaged VEGF level in the samples collected at the primary surgery was 1.98 ± 2.23 ng/mL in the PDR group, and it was 9.07, 1.94, and 8.07 ng/mL in the AHFVP cases. After the primary surgery, the VEGF level rose up to 49.50, 15.60, and 50.60 ng/mL at the subsequent surgeries for respective cases of the AHFVP group. These levels of VEGF were more than five times higher than the baseline at the primary surgery. CONCLUSION: The subsequent increase of the VEGF level after the primary surgery in eyes with an AHFVP suggests that the vitreous levels of VEGF are associated with the development of the AHFVP although only three eyes were studied.

Monozygotic twins with polypoidal choroidal vasuculopathy.

PURPOSE: To present the first findings in the set of monozygotic twins with polypoidal choroidopathy (PCV). METHODS: Sixty two-year old monozygotic twin sisters were studied. The concordances and discordances of the clinical features of the twins were determined. Genomic DNA was extracted and genotyped for three established PCV risk-associated single nucleotide polymorphisms, viz CFH I62V, CFH Y402H, and ARMS A69S. RESULTS: Both patients had hemorrhagic pigment epithelial detachments with orange lesions beneath the retinal pigment epithelium. Indocyanine green angiography showed pathognomonic choroidal vascular networks with polypoidal structures uniocularly in one twin and binocularly in the other twin. Both twins were treated with photodynamic therapy, retinal photocoagulation, and anti-vascular endothelial growth factor therapy, but both showed limited response to all the treatments, with recurrent exudative lesions with enlarged vascular network, and poor visual outcome. Genetic analyses showed that both sisters had homozygous risk alleles for ARMS2 A69S, and one risk allele each of CFH I62V and CFH Y402H. CONCLUSIONS: We present the first findings in a set of monozygotic twins with typical PCV under long-term observation. The concordances in disease progression and response to treatment between the twins indicate that these genetic factors most likely played important roles in determining the clinical manifestations.

Fundus autofluorescence and optical coherence tomographic findings in acute zonal occult outer retinopathy.

PURPOSE: The purpose of this study was to investigate the fundus autofluorescence and optical coherence tomography findings in eyes with acute zonal occult outer retinopathy (AZOOR). METHODS: A retrospective observational case series of the fundus autofluorescence and spectral domain optical coherence tomography in a series of patients with AZOOR. RESULTS: There were 19 eyes of 11 patients (10 women), who had a mean age of 49.1 +/- 13.9 years. Fundus autofluorescence abnormalities were seen in 17 of the 19 eyes, were more common in the peripapillary area, and were smaller in extent than the optical coherence tomography abnormalities. Nine eyes showed progression of hypoautofluorescence area during the mean follow-up of 69.7 months. The mean thickness of the photoreceptor layer at fovea was 177 microm in eyes with AZOOR, which was significantly thinner than controls (193 microm, P = 0.049). Abnormal retinal laminations were found in 12 eyes and were located over areas of loss of the photoreceptors. The subfoveal choroidal thickness was 243 microm, which is normal. CONCLUSION: Fundus autofluorescence abnormalities in AZOOR showed distinct patterns of retinal pigment epithelial involvement, which may be progressive. Thinning of photoreceptor cell layer with loss of the outer segments and abnormal inner retinal lamination in the context of a normal choroid are commonly found in AZOOR.

High-resolution optical coherence tomography findings in optic pit maculopathy.

PURPOSE: The purpose of this study was to characterize retinal manifestations of optic pit maculopathy using high-resolution optical coherence tomography. METHODS: Consecutive patients with optic pit maculopathy, diagnosed by their typical appearance by ophthalmoscopy, were imaged by color fundus photography and optical coherence tomography. The location and characteristics of any fluid within and under the retina were determined. RESULTS: The mean age of the 16 patients (7 women) was 35.9 years (standard deviation: 18.5 years). The visual acuity ranged from 20/20 to 20/1000 (median, 20/200). Retinal detachment was found in 11 eyes (69%), intraretinal fluid in the outer nuclear layer in 15 eyes (94%), in the inner nuclear layer in 13 eyes (81%), in the ganglion cell layer in 7 eyes (44%), and in the subinternal limiting membrane space in 2 eyes (13%). An outer layer hole was identified in only 3 of 11 eyes (27%) with retinal detachment. CONCLUSION: Fluid from the optic pit can go directly to the subinternal limiting membrane space, ganglion cell layer, inner nuclear layer, outer nuclear layer, or the subretinal space, although the outer nuclear layer is most commonly affected. An outer layer hole appears not to be common in optic pit maculopathy.

Prevalence and significance of subretinal drusenoid deposits (reticular pseudodrusen) in age-related macular degeneration.

PURPOSE: To determine the prevalence and significance of subretinal drusenoid deposits (reticular pseudodrusen) among patients with age-related macular degeneration (AMD). DESIGN: A prospective study with a nested case-control study of consecutive patients with AMD seen in a referral retinal practice. PARTICIPANTS: There were 153 patients with AMD, 131 of whom had > or =1 eye with late AMD, which was defined as either central geographic atrophy or choroidal neovascularization. The control group consisted of 101 patients who did not have AMD as their primary diagnosis, central serous chorioretinopathy, high myopia, retinal detachment, or laser treatment in the macular area. METHODS: The presence of subretinal drusenoid deposits was determined by 2 methods, using the blue channel of color fundus photograph and the spectral domain optical coherence tomography (SD-OCT) sections. Soft drusen were determined from color fundus photographs and confirmed by SD-OCT. MAIN OUTCOME MEASURES: Prevalence of ocular risk factors and subretinal drusenoid deposits in eyes with AMD and their association with late AMD. RESULTS: There were 153 patients who had any form of AMD, with a mean age of 80.3 years. Subretinal drusenoid deposits were diagnosed in the case group in 13 (8.7%) of right and 18 (12.0%) of left eyes using the blue channel of the color photograph and in 58 (38.4%) of right and 54 (35.8%) of left eyes using SD-OCT. Soft drusen and subretinal drusenoid deposits detected by SD-OCT were found to be independently correlated with late AMD (soft drusen odds ratio = 16.66 [P<0.001]; subretinal drusenoid deposits as detected by OCT odds ratio = 2.64 [P = 0.034]). In the control group, subretinal drusenoid deposits were diagnosed in 6 (6.5%) of right and 6 (6.3%) of left eyes using SD-OCT. CONCLUSIONS: Both soft drusen and subretinal drusenoid deposits occur in patients with AMD and both are significantly associated with late AMD. These findings suggest that detection and classification of drusen and consequently assignment of risk should be based on a methodology that includes SD-OCT.

Mutton fat-like subretinal precipitates associated with Vogt-Koyanagi-Harada disease.

PURPOSE: To report a case of Vogt-Koyanagi-Harada (VKH) disease presenting mutton fat like subretinal precipitates. DESIGN: Observational case report. METHODS: A 52-year-old Japanese woman developed bilateral uvetis with serous retinal detachment and mutton fat like subretinal precipitates. RESULTS: According to Opthalmologic, auditory, and systemic examination, we diagnosed the patient with VKH disease. Inflamation was controlled by three time steroid therapy and subretinal fluid and precipitates decreased and disappeared. CONCLUSIONS: Subretinal granulomatous lesion may present a manifestation of VKH and mean long standing subretinal fluid and inflammation.

Frequency of glaucoma in central serous chorioretinopathy: a case-control study.

PURPOSE: The purpose of this study was to investigate the frequency of glaucoma and related conditions in patients with central serous chorioretinopathy (CSC), a condition associated with increased choroidal thickness. METHODS: A case-control study was conducted. Consecutive patients with CSC examined from January 1, 2006, through July 31, 2008, were compared with a sex- and age-matched control group from the same referral practice seen during the same period for the frequency of glaucoma and related conditions. The charts of the cases and the control subjects were reviewed for diagnosis of glaucoma. RESULTS: In 287 patients with CSC, the mean age was 56.8 years and 207 (72.1%) were men. In the control group of 235, the mean age was 59.5 years and 168 (71.5%) were men. Glaucoma was found in 10 of 287 patients (3.4%) with CSC and in 20 of 235 control subjects (8.5%, P = 0.014, odds ratio = 0.39, 95% confidence interval = 0.16-0.89). The mean intraocular pressures were similar in the two groups. However, the number of patients diagnosed with ocular hypertension (0.3% versus 3.4%, P = 0.0076) and the number of patients using pressure-lowering eye drops (3.8% versus 13.2%, P

Enhanced depth imaging optical coherence tomography of the choroid in central serous chorioretinopathy.

PURPOSE: The purpose of the study was to evaluate the choroidal thickness in patients with central serous chorioretinopathy, a disease attributed to increased choroidal vascular hyperpermeability. METHODS: Patients with central serous chorioretinopathy underwent enhanced depth imaging spectral-domain optical coherence tomography, which was obtained by positioning a spectral-domain optical coherence tomography device close enough to the eye to acquire an inverted image. Seven sections, each comprising 100 averaged scans, were obtained within a 5 degrees x 30 degrees rectangle to encompass the macula. The subfoveal choroidal thickness was measured from the outer border of the retinal pigment epithelium to the inner scleral border. RESULTS: The mean age of subjects undergoing enhanced depth imaging spectral-domain optical coherence tomography was 59.3 years (standard deviation, 15.8 years). Seventeen of 19 patients (89.5%) were men, and 12 (63.2%) patients had bilateral clinical disease. The choroidal thickness measured in 28 eligible eyes of the 19 patients was 505 microm (standard deviation, 124 microm), which was significantly greater than the choroidal thickness in normal eyes (P < or = 0.001). CONCLUSION: Enhanced depth imaging spectral-domain optical coherence tomography demonstrated a very thick choroid in patients with central serous chorioretinopathy. This finding provides additional evidence that central serous chorioretinopathy may be caused by increased hydrostatic pressure in the choroid.

Enhanced depth imaging optical coherence tomography of the choroid in highly myopic eyes.

PURPOSE: To measure macular choroidal thickness (CT) in highly myopic eyes using enhanced depth imaging optical coherence tomography (OCT). DESIGN: Retrospective, observational case series. METHODS: Enhanced depth imaging OCT images were obtained in highly myopic eyes (> or =6 diopters [D]). Images of CT were obtained by positioning a spectral-domain OCT device close enough to the eye to acquire an inverted image. CT was measured from the outer border of the retinal pigment epithelium to the inner scleral border at 1000-mum intervals of a horizontal section from 3 mm temporal to the fovea to 3 mm nasal to the fovea. Statistical analysis was performed to evaluate CT at each location and to correlate CT with age and refractive error. RESULTS: The mean age of the 31 patients (55 eyes) was 59.7 years (+/- 17.6 years; range, 24 to 90 years), and the mean refractive error was -11.9 D (+/- 3.7 D). The mean subfoveal CT was 93.2 microm (+/- 62.5 microm) and was correlated negatively with age (P = .006), refractive error (P < .001), and history of choroidal neovascularization (P = .013). Regression analysis suggested that subfoveal CT decreased by 12.7 mum for each decade of life and by 8.7 microm for each D of myopia. CONCLUSIONS: The choroid in highly myopic eyes is very thin and undergoes further thinning with increasing age and degree of myopia. Abnormalities of the choroid may play a role in the pathogenesis of myopic degeneration.

A case of herpetic epithelial keratitis after triamcinolone acetonide subtenon injection.

BACKGROUND: We report a case of herpes simplex virus (HSV) epithelial keratitis that developed after a subtenon triamcinolone acetonide (TA) injection. METHOD: A 78-year-old woman with a branch retinal vein occlusion and diffuse macular edema in her left eye received a subtenon injection of 20 mg of TA. RESULTS: Six days after the injection, the patient presented with a foreign body sensation, tearing, redness, and photophobia in the same eye. Slit-lamp examination revealed multiple corneal dendriform ulcers. She was diagnosed with HSV epithelial keratitis and treated with topical acyclovir ointment. The epithelial lesions healed after 1 week of therapy. CONCLUSION: HSV epithelial keratitis can be a possible complication of a TA subtenon injection.

Idiopathic choroidal neovascularization as an early manifestation of inflammatory chorioretinal diseases.

PURPOSE: We describe four patients with idiopathic choroidal neovascularization (ICNV) who developed inflammatory chorioretinal diseases in the ipsilateral or contralateral eye. METHODS: The medical records of 58 eyes of 58 patients with an initial diagnosis of ICNV (age range, 19-49 years; mean, 34.9 years) were reviewed. RESULTS: Of the 58 patients, 4 women (7.0%) with moderate to high myopia (age range, 17-39 years) developed inflammatory chorioretinal diseases. In Cases 1 and 2, white dots were transiently seen in the deep retina of the contralateral eye, consistent with the clinical features of multiple evanescent white dot syndrome (MEWDS). In Case 3, vitritis and multifocal white spots emerged in the ipsilateral eye. These white spots spread throughout the ocular fundus and progressed to punched-out chorioretinal scars, which led to a diagnosis of multifocal choroiditis (MFC). In Case 4, an enlarged blind spot and a few chorioretinal scars around the optic nerve head developed without vitritis in the ipsilateral eye, suggesting a diagnosis of punctate inner choroidopathy (PIC). CONCLUSIONS: These findings suggest that ICNV can be an early manifestation of inflammatory chorioretinal diseases, including MEWDS, MFC, and PIC.

Closure of a macular hole associated with a retinal arterial aneurysm without surgical intervention.

PURPOSE: To report a case in which a macular hole (MH) associated with a ruptured retinal arteriolar aneurysm (RAM) closed without surgical intervention. PATIENT AND RESULTS: A 65-year-old woman presented complaining of sudden visual loss due to vitreous hemorrhage in the left eye. Correctable visual acuity in the left eye was 2/100 at the initial visit. Three weeks later, the vitreous hemorrhage had resolved, revealing an MH, serous retinal detachment, and subretinal hemorrhage associated with a ruptured RAM. Laser photocoagulation was applied to the RAM to promote absorption of the subretinal fluid. Two months after onset, the MH closed with resolution of the exudative retinal lesions and recovery of vision to 20/60. CONCLUSIONS: The present case indicates that closure of an MH can take place within a couple of months without surgical intervention. This suggests that observation without surgical intervention may be advocated when an MH is associated with a ruptured RAM and massive subretinal hemorrhage spares the macula.

Unilateral cone dysfunction as a manifestation of acute zonal occult outer retinopathy.

PURPOSE: To report a patient with unilateral cone dysfunction (UCD) who later developed acute zonal occult outer retinopathy (AZOOR) in the contralateral eye. METHODS: A 19-year-old Japanese woman was referred complaining of decreased vision and photopsia of the left eye. Static perimetry, full-field electroretinography (ERG), and multifocal ERGs (mfERGs) were performed to evaluate her visual functions. She returned 9 months later with visual field defect and photopsia in the right eye. RESULTS: The static visual field of the left eye demonstrated a scotoma that extended from the physiologic blind spot to the center of the visual field. The cone full-field ERGs were extinguished with preservation of rod function. The mfERGs were reduced throughout the posterior pole of the left eye. The patient was diagnosed with UCD. She revisited us 9 months later complaining of visual symptoms in the right eye and was found to have an arcuate scotoma in the upper visual field corresponding to decreased mfERGs. These findings were consistent with clinical signs of AZOOR. CONCLUSION: These findings indicate that UCD is one of the clinical manifestations of AZOOR.