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PURPOSE To evaluate the subfoveal choroidal thickness (SFCT) and vascular architecture in the fellow eyes of patients with circumscribed choroidal hemangioma (CCH). METHODS In this retrospective observational study, patients were selected from outpatient ophthalmology clinics at the Memorial Sloan Kettering Cancer Center and Vitreous Retina Macula Consultants of New York. Subfoveal choroidal thickness was measured using enhanced depth imaging spectral domain optical coherence tomography from the outer portion of Bruch membrane to the choroidal-scleral interface. Choroidal vascular architecture was qualitatively examined. The main outcome measure was SFCT in fellow eyes of patients with CCH, which was compared with an age- and gender-matched control group. RESULTS Thirty-one fellow eyes (15 right eyes and 16 left eyes) of patients with CCH (23 males and 8 females) were examined. The fellow eye had a mean SFCT of 361.2 ± 99.9 μm compared with 252.0 ± 77.6 μm in the control group (P < 0.0001). Vascular architecture was disorganized in 13 (42%) fellow eyes and 1 (3%) control eye (P < 0.0001), with no apparent gradient of vessel sizes or discrete choroidal layers. The normal association between older age and a thinner choroid existed in control eyes but not in fellow eyes. Hemangioma thickness measured by ultrasound and the presence of subfoveal fluid in the CCH eye did not correlate with the fellow-eye SFCT. CONCLUSION In patients with CCH, fellow eyes had thicker SFCT when compared with age- and gender-matched control eyes. Choroidal architecture was often irregular, without segmented vascular layers. These findings suggest that inherent choroidal changes may exist in patients with CCH.

Purpose To investigate the natural history and genetic associations of drusenoid pigment epithelial detachment (DPED) associated with age-related macular degeneration (AMD). Design Retrospective analysis of a prospective cohort study. Participants Of the 4203 Age-Related Eye Disease Study 2 (AREDS2) participants, 391 eyes (325 participants) had DPED without late AMD at the time of DPED detection. Genetic analyses included 120 white AREDS2 participants and 145 Age-Related Eye Disease Study (AREDS) participants with DPED. Methods Baseline and annual stereoscopic fundus photographs were graded centrally to detect DPED, a well-defined yellow elevated mound of confluent drusen ≥433 μm in diameter, and to evaluate progression rates to late AMD: geographic atrophy (GA) and neovascular (NV)-AMD. Five single nucleotide polymorphisms (CFH [rs10611670], C3 [rs2230199], CFI [rs10033900], C2/CFB [rs114254831], ARMS2 [rs10490924]) and genetic risk score (GRS) group were investigated for association with DPED development. Kaplan–Meier analyses and multivariable proportional hazard regressions were performed. Main Outcome Measures Progression rates to late AMD and decrease of ≥3 lines in visual acuity (VA) from the time of DPED detection; association of rate of DPED development with genotype. Results Mean (standard deviation [SD]) follow-up time from DPED detection was 4.7 (0.9) years. DPED was associated with increased risk of progression to late AMD (hazard ratio [HR], 2.36; 95% confidence interval [CI], 1.98–2.82; P Conclusions This study replicates the results of previous natural history studies of eyes with DPED including the high rates of progression to late AMD and vision loss (regardless of progression to late AMD). The genetic associations are consistent with genes associated with AMD progression.

Purpose To analyze the prevalence, incidence, and clinical characteristics of eyes with geographic atrophy (GA) in age-related macular degeneration (AMD), including clinical and genetic factors affecting enlargement. Design Prospective cohort study within a controlled clinical trial. Participants Age-Related Eye Disease Study 2 (AREDS2) participants, aged 50–85 years. Methods Baseline and annual stereoscopic color fundus photographs were evaluated for GA presence and area. Analyses included GA prevalence and incidence rates, Kaplan-Meier rates, mixed-model regression, and multivariable analysis of the square root of GA, area adjusted for covariates, including clinical/imaging characteristics and genotype. Main Outcome Measures (1) Presence or development of GA; (2) change in the square root of GA area over time. Results At baseline, 517 eyes (6.2%) of 411 participants (9.8%) had pre-existing GA (without neovascular AMD), with the following characteristics: 33% central, 67% noncentral; and the following configurations: 36% small, 26% solid/unifocal, 24% multifocal, 9% horseshoe/ring, and 6% indeterminate. Of the remaining 6530 eyes at risk, 1099 eyes (17.3%) of 883 participants developed incident GA without prior neovascular disease during mean follow-up of 4.4 years. The Kaplan-Meier rate of incident GA was 19% of eyes at 5 years. In eyes with incident GA, 4-year risk of subsequent neovascular AMD was 29%. In eyes with incident noncentral GA, 4-year risk of central involvement was 57%. GA enlargement rate (following square root transformation) was similar in eyes with pre-existing GA (0.29 mm/year; 95% confidence interval 0.27–0.30) and incident GA (0.28 mm/year; 0.27–0.30). In the combined group, GA enlargement was significantly faster with noncentrality, multifocality, intermediate baseline size, and bilateral GA (P Conclusions Analyses of AREDS2 data on natural history of GA provide representative data on GA evolution and enlargement. GA enlargement, which was influenced by lesion features, was relentless, resulting in rapid central vision loss. The genetic variants associated with faster enlargement were partially distinct from those associated with risk of incident GA. These findings are relevant to further investigations of GA pathogenesis and clinical trial planning.

Purpose To describe clinical findings in patients with acute exudative polymorphous vitelliform maculopathy (AEPVM). Design Retrospective, observational, multicenter case series review. Participants Consecutive patients diagnosed with idiopathic AEPVM. Methods Review of clinical charts, multimodal imaging, electrophysiologic findings, and genetic findings in previously unpublished patients and review of the literature. Main Outcome Measures Clinical features of idiopathic AEPVM and differential diagnosis. Results Eighteen patients (age range, 21–74 years) with typical features of AEPVM, including initial localized, serous detachments followed by the development of characteristic yellow-white deposits in the vitelliform space. Over time, this hyperautofluorescent material gravitated within the larger lesions, resulting in typical curvilinear deposits characteristic of later stages. Symptoms and clinical findings lasted from weeks to several years. Some patients showed previously undescribed features such as fluorescein-negative intraretinal cystic changes, choroidal neovascularization, serous retinal elevations mimicking retinal folds, increased choroidal thickness, lack of rapid visual recovery, and recurrence years after complete resolution of initial manifestations. Conclusions Acute exudative polymorphous vitelliform maculopathy can present with a more variable natural course than previously described. Paraneoplastic retinopathy and autosomal recessive bestrophinopathy closely resemble AEPVM, necessitating medical and hereditary evaluation to exclude these clinical possibilities. This series of patients with AEPVM expands the clinical spectrum of the disorder, including demographics, clinical manifestations, imaging features, natural course, and visual prognosis.

Purpose: To illustrate the rate of endogenous endophthalmitis associated with fungemia and evaluate the importance of screening in a public city hospital. Methods: A retrospective review was performed on all inpatient ophthalmology consults for fungemia from 2010 to 2015. Clinical histories, ocular examinations, and microbial cultures were reviewed. Results: Of 95 patients (mean age 51.6 years, 75% male) with fungemia, 9/95 (9.5%) demonstrated intraocular involvement. Of these nine patients, two were unable to participate in the ophthalmic exam due to intubation, while the remaining seven reported no changes in their vision. Two patients had their antifungal medications adjusted to optimize intraocular penetration and one patient progressed to develop vitreous involvement but died before further escalation of care occurred. Conclusion: All involved individuals in this study were either non-communicative or without visual complaints. This suggests that routine screening should still be recommended, especially in a public hospital setting.

Purpose To define the phenotypic characteristics of the bullous variant of central serous chorioretinopathy (CSC) using multimethod imaging. Design Retrospective, observational case series. Participants Twenty-one eyes of 14 patients with bullous retinal detachment resulting from CSC (bullous CSC group) and 122 eyes of 84 patients with chronic CSC without bullous retinal detachment (nonbullous CSC group). Methods We performed a retrospective review of clinical and multimethod imaging data of patients who sought treatment from the authors with bullous retinal detachment resulting from CSC between January 2010 and November 2015. Multimethod imaging comprised color photography, fluorescein angiography, fundus autofluorescence, and high-resolution optical coherence tomography. Consecutive cases of chronic CSC without bullous retinal detachment, seen during the same period, comprised a comparative group. Main Outcome Measures Qualitative and quantitative characteristics of the choroid, retinal pigment epithelium, and retina were compared between the 2 groups. Results Mean age of the bullous CSC group was 53.8 years. There was no difference in age, visual acuity, corticosteroid use, or the proportion of white patients and men between the 2 groups (all P > 0.132). Peripheral nonperfusion occurred only in eyes with bullous retinal detachment (38% of cases). Retinal pigment epithelial tears were seen in 95% of eyes in the bullous group and none of the eyes in the nonbullous CSC group. The bullous CSC group demonstrated a greater number of pigment epithelial detachments (PEDs) and more eyes demonstrated PEDs with internal hyperreflectivity (both P P = 0.993). More eyes in the bullous CSC group demonstrated hyperreflectivity around large choroidal vessels and at the level of the choriocapillaris on OCT ( P P Conclusions Bullous retinal detachment is a rare manifestation of chronic CSC and is characterized by a unique constellation of phenotypic and multimethod imaging features.

Purpose To report a retinal pigment epithelium (RPE) tumor with exudative maculopathy, originating from an atypical RPE lesion presumed to represent congenital hypertrophy of the RPE or RPE hyperplasia. Methods Multimodal imaging including fundus autofluorescence, optical coherence tomography, fluorescein angiography, and optical coherence tomography angiography. Results A 76-year-old West African man noted visual acuity reduction to count fingers in the right eye and 20/400 in the left eye. Features of chronic glaucoma were noted. In addition, there was a fairly well-circumscribed darkly pigmented RPE lesion in the paramacular region in the right eye, measuring 4 mm in diameter and flat and consistent with atypical congenital hypertrophy of the RPE or RPE hyperplasia. On the posterior margin of this mass was an RPE tumor, presumed to represent RPE adenoma, producing exudative maculopathy and cystoid macular edema. Multimodal imaging was used to distinguish the RPE tumor from macular neovascularization. A similar atypical congenital hypertrophy of the RPE without retinopathy measuring 3.5mm in diameter was noted in the temporal macular region in the left eye. After six monthly doses of intravitreal bevacizumab (1.25 mg/0.05 mL) in the right eye, the maculopathy resolved and the RPE mass showed partial involution with visual acuity return to baseline 20/200. Conclusion Congenital hypertrophy of the RPE and RPE hyperplasia can produce RPE adenoma with related exudative maculopathy. In this case, the maculopathy responded to bevacizumab.

PURPOSE To report multimodal imaging in a novel case of angioid streaks in a patient with Turner syndrome with 10-year follow-up. METHODS Case report of a patient with Turner syndrome and angioid streaks followed at Bellevue Hospital Eye Clinic from 2007 to 2017. Fundus photography, fluorescein angiography, and optical coherence tomography angiography were obtained. RESULTS Angioid streaks with choroidal neovascularization were noted in this patient with Turner syndrome without other systemic conditions previously correlated with angioid streaks. CONCLUSION We report a case of angioid streaks with choroidal neovascularization in a patient with Turner syndrome. We demonstrate that angioid streaks, previously associated with pseudoxanthoma elasticum, Ehlers-Danlos syndrome, Paget disease of bone, and hemoglobinopathies, may also be associated with Turner syndrome, and may continue to develop choroidal neovascularization, suggesting the need for careful ophthalmic examination in these patients.

Purpose To report on 5-year multimodal imaging of ocular findings in a patient with thiamine-responsive megaloblastic anemia. Methods Observational case report. Results A 20-year-old-man with a history of thiamine-responsive megaloblastic anemia demonstrated a symmetric bull's eye maculopathy. Spectral domain optical coherence tomography revealed disruption of the parafoveal ellipsoid zone, fundus autofluorescence demonstrated foveal hypoautofluorescence, and full-field electroretinogram testing revealed a decreased photopic and scotopic response consistent with cone-rod dystrophy. His best-corrected visual acuity remained stable over 5 years at 20/50 in the right eye and 20/40 in the left eye, and visual field testing remained stable over time. Conclusion Ocular manifestations in thiamine-responsive megaloblastic anemia are uncommon and variable. In this case, multimodal imaging and electroretinogram findings are consistent with cone-rod degeneration. The patient is taking daily thiamine supplementation, and visual acuity, funduscopic examination, spectral domain optical coherence tomography, and autofluorescence remained stable over a 5-year period.

To describe a case of unilateral Vogt-Koyanagi-Harada (VKH) disease and associated multimodal imaging.Retrospective case report.A 50-year-old Hispanic male presented with three days of painless decreased vision in his left eye, headache, and decreased hearing. His visual acuity was 20/20 in the right eye and counting fingers in the left eye. Examination of his right eye was unremarkable. Funduscopic examination of his left eye revealed multiple serous retinal detachments. Fluorescein angiography demonstrated late multifocal pinpoint hyperfluorescence in his left eye and a diagnosis of VKH disease was made. He was treated with oral prednisone. Serial re-examination demonstrated resolution of the serous retinal detachments and a taper of his oral prednisone was initiated with improvement of his visual acuity to 20/25.Our patient had imaging and a clinical course that was consistent with VKH disease. This unilateral presentation may represent a clinical variant of VKH disease.

Purpose To investigate the posterior segment in cases of clinically evident intraocular inflammation for punctate reflections consistent with that expected to arise from inflammatory cells. Methods Patients with ocular inflammatory diseases imaged with spectral-domain optical coherence tomography (SD-OCT) were retrospectively reviewed. Results There were 7 patients with mean age of 66.7 years, and the diagnosis was toxoplasmosis in 5 eyes, multiple evanescent white dot syndrome in 1 eye, and posttraumatic outer retinitis in 1 eye. At baseline, the SD-OCT showed vitreous cells as numerous punctate spots in the vitreous in all seven eyes. The SD-OCT also showed similar-sized hyperreflective dots in the retina in all seven eyes. During follow-up, reduced vitreous cellular infiltration was correlated with a decrease in the number of the punctate spots visible by SD-OCT. Conclusion Eyes with intraocular inflammation had SD-OCT images of the vitreous containing punctate spots of a size consistent with that expected from inflammatory cells. Similarly sized punctate spots were seen within the retina in regions of retinitis. Additional characterization of the optical section should permit stereological estimations of actual cell counts per unit volume.

Evaluation of: You IC, Im SK, Lee SH, Yoon KC. Photodynamic therapy with verteporfin combined with subconjunctival injection of bevacizumab for corneal neovascularization. Cornea 30(1), 30–33 (2011).Corneal neovascularization occurs following infectious, ischemic, inflammatory and other corneal diseases, as well as corneal injuries. It has a detrimental effect on visual acuity and also presents a significant risk for the failure of corneal grafting. To date, the therapeutic modalities are limited and address either the underlying inflammatory and infectious processes or selectively ablate those vessels using photocoagulation or diathermy. More recent strategies include pharmacological inhibition of various steps of angiogenesis and selective photochemical vascular occlusion with photodynamic therapy. The article under evaluation presents a prospective, interventional case series treating corneal neovascularization with a combination of photodynamic therapy and subconjunctival injection of bevacizumab (Ava...

Purpose The purpose of this study was to report two unusual cases of macular telangiectasia type 2 with pronounced asymmetric clinical presentations. Patients and methods Two patients. Observational case study. Results Two patients presented with acute complaints of decreased vision and metamorphopsia in one eye. Each was found to have unilateral, juxtafoveal, subretinal neovascularization and minimal or no clinical evidence of macular telangiectasia type 2 in the fellow eye. In one patient, clinical and angiographic findings of macular telangiectasia type 2 were observed in the previously unaffected eye 13 years after the initial presentation. Conclusion Macular telangiectasia type 2 is a bilateral condition, but in rare cases, significant asymmetry between the two eyes concerning disease severity can occur, potentially delaying diagnosis and management.

The purpose of this study was to investigate if monthly intravitreal ranibizumab decreases risk of macular hemorrhages in patients with choroidal neovascularization secondary to age-related macular degeneration.Incidences of macular hemorrhages in the control and ranibizumab groups from three, multicenter, randomized, clinical trials (MARINA, ANCHOR, and PIER) were compared. Two time intervals (Months 0-3 and 5-17) were evaluated to account for transition from monthly to quarterly injections in PIER. Time interval after Month 17 was excluded because of crossover from control to active treatment in all trials.Months 0-3: All trials showed higher incidence rates of hemorrhages in control compared with ranibizumab groups (ANCHOR: photodynamic therapy [27.3%], 0.3 mg [8.0%], 0.5 mg [8.6%]; MARINA: sham [18.6%], 0.3 mg [8.8%], 0.5 mg [8.8%]; and PIER: sham [16.1%], 0.3 mg [3.4%], 0.5 mg [3.3%]). In ANCHOR and MARINA, data of Months 5-17 showed higher incidence rates in control compared with monthly ranibizumab groups (ANCHOR: photodynamic therapy [47.8%], 0.3 mg [12.5%], 0.5 mg [12.3%]; and MARINA: sham [38.0%], 0.3 mg [13.2%], 0.5 mg [13.0%]), but this was not seen for quarterly ranibizumab groups in PIER (sham [22.4%], 0.3 mg [23.7%], 0.5 mg [28.3%]).Treatment with monthly intravitreal ranibizumab was associated with reduced risk of new macular hemorrhages when compared with photodynamic therapy (ANCHOR) or sham (MARINA and PIER). There was no difference between PIER quarterly ranibizumab-treated and sham patients.

Purpose To report a case of bullous retinal detachment complicated by peripheral retinal ischemia and neovascularization in a patient with chronic central serous chorioretinopathy (CSC). Results Focal laser photocoagulation to the active retinal pigment epithelial leaks in the posterior pole resulted in resolution of the bullous detachment and regression of the retinal neovascularization. Conclusions Patients with chronic CSC and a large, dependent serous detachment with peripheral retinal ischemia and neovascularization may be managed by focal treatment to active pigment epithelial leaks alone. This could spare patients of the adverse effects associated with widespread laser treatment and reduce the risk of visual loss due to vitreous hemorrhage and/or more severe complications resulting from progressive retinal ischemia.

Photodynamic therapy with verteporfin was the first approved pharmacological treatment for choroidal neovascularization in patients with age-related macular degeneration, pathologic myopia and ocular histoplasmosis syndrome. With the introduction of anti-VEGF therapy, verteporfin photodynamic therapy is no longer the first line of treatment for neovascular age-related macular degeneration. Ongoing clinical trials will determine the future role of PDT alone or as part of a more individualized combination therapy.

To compare noninvasive autofluorescence (AF) photography with conventional fundus photography and fluorescein angiography (FA) in the detection of basal laminar drusen (BLD).A retrospective case review of 20 patients with BLD studied with AF and conventional imaging was performed. Three selected patients with different degrees of BLD are presented. AF imaging employed an excitation filter at 580 nm and a barrier filter at 695 nm with acquisition by a Topcon 50X fundus camera. Corresponding detail regions in each image were enlarged for comparison. The AF detail image was registered by a projective transformation in Matlab (Mathworks 7.0, Natick, MA) with the color photograph/red free photograph (RF) and/or FA image detail for exact superimposition in Photoshop and lesion comparison.Each visible drusen in the color or RF photograph corresponded when superimposed to a focal hypoautofluorescent lesion in the AF image. However, similar to the starry sky pattern in FA, the AF lesions significantly outnumbered the clinically evident drusen. When BLD lesions were not advanced enough to show the classic starry sky fluorescein hyperfluorescence, the BLD were detectable with AF.In our case series, AF imaging demonstrated a higher level of sensitivity when compared with conventional fundus photography and is less invasive than FA for the detection of BLD. Fundus AF, therefore, is valuable for diagnosing and following BLD, particularly since these patients are at risk for development of pseudovitelliform detachment which may simulate CNV.

Photodynamic therapy (PDT) has become a well-established treatment for vascular forms of age-related macular degeneration (AMD). The implementation of evidence-based medicine principles into the treatment regimen of AMD seems to be immensly important, since AMD continues to be the most frequent cause of blindness among patients older than 65 years in industrialized countries. Numerous randomized prospective studies demonstrated high levels of evidence for the efficacy of various treatment approaches such as laser photocoagulation, PDT, subretinal surgery or novel anti-angiogenic drugs [Arch Ophthalmol 2006;124:597–599]. The high evidence shown by these studies supported the rationale to use PDT also in additional, less frequent, vasoproliferative diseases. Although these ‘case series’ and ‘individual case control studies’ have a low level of evidence, they give us important information for treatment decisions in these rare conditions. The goal of this survey is to review the current literature regarding PDT in vasoproliferative and exudative ocular diseases outside AMD. Many studies modified the treatment parameters of PDT to address the specific pathology of the underlying disease. Table 1 summarizes the diseases and treatment parameters that are described in this part 2, the entire table of this review is included in part 1 (www.karger.com/doi/10.1159/ 000101922).

Ocular photodynamic therapy (PDT) was introduced as a novel treatment for neovascular forms of age-related macular degeneration and choroidal neovascularization (CNV) secondary to pathologic myopia in the mid/end 1990s. The current treatment recommendations are based on the results of two large, prospective, multicenter, randomized clinical trials (Treatment of Age-Related Macular Degeneration with Photodynamic Therapy and Verteporfin in Photodynamic Therapy Studies) and thousands of patients have been treated worldwide over the last years. Meanwhile, PDT has been performed in several other ocular pathologies with some remarkable results, however, with most reports being case reports and small case series without statistical significance. These extended applications include CNV secondary to choroiditis and retinochoroiditis, angioid streaks, central serous chorioretinopathy, retinal angiomatous proliferation, parafoveal telangiectasia or CNV associated with macular dystrophy and idiopathic CNV, as well as diseases without CNV, such as choroidal hemangioma, retinal hamartoma, choroidal melanoma, chronic central serous chorioretinopathy, angiomatous lesions secondary to systemic diseases, rubeosis iridis or neovascular glaucoma. To date, with the introduction of anti-VEGF therapy, the role of PDT will certainly change. However, it is reasonable to believe that it will maintain an important role in combination therapy due to its unique properties of selective vascular targeting. Therefore, it is essential for the ophthalmologist to be familiar with the extended applications and their modifications of treatment parameters. This review will summarize the standard and experimental applications of PDT based on our own results and the literature.

Objective To evaluate the potential benefit and risks of photodynamic therapy (PDT) in the treatment of papillary capillary hemangioma. Design Prospective, noncomparative, interventional case series. Participants Five patients with solitary capillary hemangioma on the temporal portion of the optic nerve presenting with exudative decompensation and decrease in visual acuity (VA). Methods All eyes received a standardized PDT treatment with 6 mg/kg body surface area verteporfin and application of 100 J/cm 2 light at 692 nm. One to three PDT courses were performed until resolution of exudation was achieved. A continuous follow-up was provided with documentation 1 week before and at 4 to 6 weeks, 3 months, and 12 months after the last treatment application. Main outcome measures Functional parameters included best-refracted VA (Early Treatment Diabetic Retinopathy Study), and central scanning laser ophthalmoscope (SLO) scotometry and peripheral (automated perimetry) visual fields; anatomic parameters were presence of retinal edema or serous detachment (ophthalmoscopy) and tumor size (ultrasonography). Results Pretreatment VA levels ranged from 20/40 to 20/800; posttreatment levels ranged from 20/64 to 20/2000. Tumor regression with resolution of macular exudate and serous retinal detachment was obtained in all eyes. A decline in VA of 1, 3, and 10 lines, respectively, was documented in three patients. Complications included transient decompensation of vascular permeability, occlusion of retinal vessels, and ischemia of the optic nerve. Conclusions PDT is successful in reducing tumor size and exudative activity. Vaso-occlusive effects at the level of the retina and optic nerve compromise the functional benefit. Parameters proven safe in choroidal neovascularization may be inappropriate in retinal capillary lesions of the optic nerve.

Purpose: To investigate the association of the rs2301995 haplotype-tagging single nucleotide polymorphism (htSNP) in the elastin gene (ELN) with polypoidal choroidal vasculopathy (PCV) in European-American patients.Methods: Association analysis of allele and genotype frequencies, determined by TaqMan assays, was performed for the rs2301995 haplotype-tagging single nucleotide polymorphism (htSNP) in the ELN locus in fifty-six patients with PCV, 368 patients with advanced age-related macular degeneration (AMD) and 368 age- and ethnically-matched unaffected controls.Results: The ELN rs2301995 SNP was not statistically significantly associated with the PCV phenotype (P = 0.9). The frequency of the minor allele of the rs2301995 SNP was practically identical in the PCV, AMD and control groups (6.3% vs. 5.4% vs. 7.1%).Conclusion: The PCV phenotype in European-American patients is not associated with rs2301995 SNP in the ELN locus.

Purpose To report a case of atypical unilateral maculopathy associated with acute exudative polymorphous vitelliform maculopathy-like yellowish deposits. Methods Observational case report of one patient. Results A 52-year-old man presented with reduced vision in the left eye. Findings resembling acute exudative polymorphous vitelliform maculopathy were noted with ophthalmoscopy, fluorescein angiography, and optical coherence tomography. Funduscopic examination revealed an exudative macular detachment with yellowish subretinal deposits inferior to the fovea. On fluorescein angiography, the perifoveal lesions were minimally hyperfluorescent, with no abnormal fluorescence in the central macula. The subretinal deposits were found to be hyperautofluorescent on fundus autofluorescence imaging. Optical coherence tomography confirmed a serous detachment of the retina with intraretinal cystic spaces. The right eye did not show any abnormalities except for an epiretinal membrane. Conclusion We describe a case of atypical unilateral maculopathy associated with acute exudative polymorphous vitelliform maculopathy-like yellowish deposits.

PURPOSE: To report the long-term follow-up of a case of enhanced S-cone syndrome (ESCS). METHODS: Retrospective chart review. RESULTS: The patient was misdiagnosed with atypical retinitis pigmentosa at 17 years of age. Twenty-seven years of follow-up showed slow deterioration but relative preservation of vision. The most striking clinical feature was the formation of a ring of heavy round pigment clumping around the vascular arcades. Electroretinogram was reported as extinguished in advanced stages of the condition. Genetic testing revealed the most common mutation of the NR2E3 gene reported in the Goldmann-Favre syndrome/ESCS entity. CONCLUSION: Visual acuity can be relatively preserved over the course of ESCS. In advanced stages, genetic testing can be a valuable diagnostic tool.

Purpose The purpose of this article was to report a case of Stage 3 Takayasu retinopathy with marked venous staining. Methods Case report with funduscopic, fluorescein angiography, computed tomography angiography, and magnetic resonance imaging angiography correlations. Results A 25-year-old woman presented with Stage 3 Takayasu retinopathy with striking venous staining while maintaining visual acuity of 20/30 because of arterial collateralization to the internal carotid arteries. Conclusion Patients with Takayasu arteritis should be evaluated promptly for Takayasu retinopathy even with good vision because stenosis of major vessels off the aorta is associated with worse visual prognosis.

Purpose To describe the clinical and imaging findings in 3 patients with maculopathy secondary to handheld laser exposure. Design Retrospective, observational case series. Methods We evaluated the multimodal imaging including fundus autofluorescence and spectral-domain optical coherence tomography (OCT) for 3 patients with histories of exposure to handheld lasers. Results An 18-year-old woman with a history of repetitive self-inflicted handheld laser exposure was found to have bilateral outer retinal streaks in the macula and the superior peripheral retina on both ophthalmoscopy and multimodal imaging. Initial spectral-domain OCT revealed vertical hyper-reflective bands at the level of the outer retina corresponding to the streaks. An 11-year-old boy who played with a green laser developed a yellow foveal lesion and outer retinal streaks in the superior macula. Spectral-domain OCT showed vertical hyper-reflective bands in the outer retina corresponding to the streaks. A 14-year-old boy developed bilateral focal foveal lesions and ellipsoid loss on spectral-domain OCT following peer-inflicted laser injury. Conclusions In a series of 3 patients, outer retinal streaks were associated with self-inflicted handheld laser injury. In contrast, accidental and peer-inflicted laser injuries were found to result in focal foveal lesions.

In a prospective, double-blind study, 19 patients with a classical subfoveal choroidal neovascularisation due to AMD were followed-up over a period of 2 years. Every 3 months a standardised visual acuity test, a contrast sensitivity test and fluorescein-angiography were performed. Overall, visual acuity dropped from 20/125 initially down to 20/250 after 2 years. Contrast sensitivity decreased from 23 down to 19 recognised letters. The functionally superior group 1 (n = 7) with a visual acuity of 20/65 at baseline, improved up to 20/50 after 2 years, while group 2 (n = 12) with a baseline visual acuity of 20/125 showed a decrease down to 20/400 after 24 months. Contrast sensitivity stabilised or improved in 10 patients from 22 to 25 letters, while in 9 patients the score dropped from 23 down to 8 recognised letters. The results underline the unfavourable functional prognosis of classical subfoveal lesions in AMD. However, stabilisation is possible depending on baseline visual acuity which is critical whenever results of nonplacebo-controlled studies have to be assessed. Moreover these results suggest that the urgency of a therapeutical intervention might be overestimated. As treatment, a fairly gentle, primarily atraumatic, CNV-selective strategy should be chosen, so that the self-limiting potential of this disease can be utilised in addition to the therapeutic effect.

Keywords: Photomedicine group Reference LPAS-ARTICLE-2001-009 Record created on 2007-07-20, modified on 2016-08-08

Objective This study was aimed at localizing and quantifying retinal defects in patients with branch retinal vein occlusion by means of scanning laser perimetry and analyzing the mechanism involved in the functional recovery after laser therapy. Design Prospective nonrandomized clinical trial with concurrent comparison group. Participants Fifty-eight patients with isolated branch retinal vein occlusion. Thirty-nine eyes received laser treatment; 19 eyes were observed without treatment. Intervention Argon laser photocoagulation was performed according to the Branch Vein Occlusion Study recommendations. Main outcome measures Retinal functional deficits were evaluated with scanning laser perimetry and fluorescein angiography first at baseline, at 3-month follow-up visits and 3 months after laser treatment. Results After laser treatment, the scotoma encroached on foveal fixation in 31% of eyes, remained stable in 36%, and regressed from the foveal avascular zone in 33%. Improvement in vision was correlated with increasing scotoma distance from fixation. Total scotoma size increased in 50% of eyes after treatment. Depth of scotoma and degree of angiographic leakage showed no direct correlation. Conclusions Stabilization and increase in visual acuity after laser treatment did not correlate with an overall decrease in scotoma size. Improved central visual function seen in 25% of treated eyes appeared to be due to withdrawal of scotoma from the fovea.

Purpose: Photodynamic therapy (PDT) has been shown to provide immediate occlusion of choroidal neovascularization (CNV), followed by recurrent leakage after single PDT in the majority of the cases after 3 months. Indocyanine green angiography (ICG-A) was used to evaluate completeness of CNV occlusion, effects on physiological choroid and patterns of CNV recurrence. Methods: ICG-A was performed using a confocal laser scanning ophthalmoscope (HRA) before PDT at 1 week, 4 and 12 weeks following PDT. Twenty patients with single and 10 patients with repeated PDT treatments with administration of benzoporphyrin derivative and radiant exposures between 50 and 150 J/cm2 were evaluated. Results: Before PDT well-defined CNV was detectable during early ICG-A in all lesions. Depending on the number of treatments, CNV was absent in early phase ICG-A in 46–83 %. CNV reappeared at week 4 in many and at 12 weeks in 77 (66 %) of the cases. The treated area regularly showed hypofluorescence, which persisted until week 12. The intensity of choroidal hypofluorescence showed wide interindividual variability. Recurrence may originate from persistent feeder vessels. Conclusion: With ICG-A we demonstrated that PDT induces hypofluorescence of CNV and choroid possibly due to perfusion changes or blockade phenomena. Recurrence may be due to reperfusion of the preexisting CNV or regrowth from feeder vessels.

Purpose: Branch retinal vein occlusion (BRVO) induces variable functional deficits depending on the grade of vascular occlusion and its localisation. Theses deficiences are not easily defined by visual acuity measurements. However, microperimetry offers topical mapping of retinal function, allowing precise documentation of the intensity and dimension of retinal functional loss in BRVO.

Purpose The presence or absence of functional changes associated with solitary, congenital, hypopigmented lesions of the retinal pigment epithelium (RPE) have been a matter of controversy. This case report describes retinal and functional findings in a young patient with such a lesion. Methods A 10-year-old Hispanic female with a solitary congential hypopigmented spot of the RPE was examined using fundus photography, fluorescein angiography, autofluorescence imaging (AF) and optical coherence tomography (OCT). Functional analyses were performed using the Humphrey 24 - 2 visual field, Goldmann perimetry and the multifocal ERG (mfERG). Results A small visual field defect was demonstrated on both Goldmann perimetry (I/ 2e test object) and on Humphrey 24 - 2 visual field testing (significant at the 0.5 % level for pattern deviation). The multifocal ERG response amplitudes were decreased in the corresponding area and increased in implicit time. Autofluorescence imaging showed an absence of fluorescence corresponding to the area of the lesion. OCT findings were indicative of a small amount of subretinal fluid or schisis-like changes overlying the RPE anomaly. Conclusion The results indicate that solitary, albinotic spots of the RPE can be associated with visual field defects and outer retinal deficits; these may be related to impaired RPE function and/or chronic exudative changes.

Importance New funduscopic findings in patients with enhanced S-cone syndrome (ESCS) may help clinicians in diagnosing this rare autosomal recessive retinal dystrophy. Objective To expand the clinical spectrum of ESCS due to mutations in the NR2E3 gene. Design Retrospective, noncomparative case series of 31 patients examined between 1983 and 2012. Setting Academic and private ophthalmology practices specialized in retinal dystrophies. Participants A cohort of patients diagnosed with ESCS and harboring known NR2E3 mutations. Intervention Patients had ophthalmic examinations including visual function testing that led to the original diagnosis. Main Outcomes and Measures New fundus features captured with imaging modalities. Results New clinical observations in ESCS include (1) torpedo-like, deep atrophic lesions with a small hyperpigmented rim, variably sized and predominantly located along the arcades; (2) circumferential fibrotic scars in the posterior pole with a spared center and large fibrotic scars around the optic nerve head; and (3) yellow dots in areas of relatively normal-appearing retina. Conclusions and Relevance Enhanced S-cone syndrome has more pleiotropy than previously appreciated. While the nummular type of pigmentation at the level of the retinal pigment epithelium and cystoid or schisis-like maculopathy with typical functional findings remain classic hallmarks of the disease, changes such as circumferential fibrosis of the macula or peripapillary area and “torpedo-like” lesions along the vascular arcades may also direct the clinical diagnosis and focus on screening the NR2E3 gene for a molecular diagnosis.

This study investigated whether the presence of vitreomacular interface abnormalities (VMIAs) affects the improvement in visual acuity and edema of patients with diabetic macular edema (DME) who received three anti-vascular endothelial growth factor (VEGF) injections. Fifteen eyes of 11 patients with clinically significant macular edema were retrospectively divided into either the control group (only DME) or the experimental group (DME and VMIA) based on optical coherence tomography images. We defined VMIA patterns as epiretinal membrane and/or anomalous vitreomacular adhesion. Changes in central macular thickness (CMT), total macular volume (TMV), and best-corrected visual acuity (BCVA) from the baseline to post third injection were compared between the two groups. After the third injection, the decreases in CMT and TMV were not statistically different between the two groups. The improvement in BCVA was larger in the control group (0.1742 ± 0.0508 logMAR) than in the experimental group (0.0766 ± 0.0562 logMAR; p < 0.01). Our study showed that after the third anti-VEGF injection, the BCVA of patients with both DME and VMIAs improved significantly less than that of patients with only DME. Our results suggest that VMIAs may play a crucial role in reducing the therapeutic effects of anti-VEGF agents.

Purpose:To report one year outcomes of focal Navigated Retina Laser Therapy (NAVILAS) for diabetic macular edema (DME).Methods:Retrospective cohort series of 7 diabetic patients treated with NAVILAS focal laser. Statistical analysis included descriptive and continuous variables (Best-corrected logMAR Visual Acuity and time-domain optical coherence tomography (OCT) parameters) which were compared using a non-parametric procedure, the Friedman tests for repeated measures. A p-value of less than 0.05 was considered to denote statistical significance.Results:diabetic patients (4 male; 3 female) with an average age of 60.8 years (range 48-85 years) were included. All treated eyes were phakic; patients had an average hemoglobin A1C of 9.1 (range 7.8-11.7) at baseline and 8.0 (range 7.4-8.4) at 12 months. Six of the 7 patients had intravitreal bevacizumab injections prior to focal laser treatment with 1 patient having had more than 1 prior injection (total 3). At 12 months, median logMAR improved from 0.695 (± interquartile range 0.574) to 0.477 (± 0.573, p Conclusions:Focal NAVILAS showed to be safe and effective in treating DME with improvement in visual acuity and macular edema on OCT over 12 months in this case series. In clinical practice, combined treatment with focal laser including NAVILAS and anti-vascular endothelial growth factor may provide long-term improvement in DME.

Purpose To evaluate the short-term outcomes after intravitreal aflibercept (Eylea; Regeneron Pharmaceuticals, Inc.) injection in a general clinic population treated for neovascular age-related macular degeneration (AMD). Methods This is aretrospective chart review of consecutive patients who received intravitreal aflibercept injection for AMD. The main outcome measures were mean visual acuity (VA), central macular thickness, presence of subretinal fluid, cystic changes and pigment epithelial detachment on optical coherence tomography (OCT). Results Sixty-three eyes of 52 patients (mean age 81.2, range 59-98years) were evaluated. Mean baseline visual acuity was 0.79 LogMar (STDEV 0.43)at baseline and 0.66 at follow-up(STDEV 0.48) (P = 0.43). Mean follow-up was 1 to 4 month (average 1.7). Mean baseline central macular thickness was 352 microns and improved to 257 microns at 3 months (P < 0.001) in patients who had at least 3 months of follow up. 39 patients (75%) were treated with monthly injections, the remaining patients were treated based on a “treat and extend” protocol. Treatment was well tolerated with no adverse events reported. Conclusion Short-term results of aflibercept therapy showed encouraging results with improvement in OCT parameters. Patients who underwent antiVEGF treatment with other medications prior the use of aflibercept may stabilize but not further improve after short-term treatment with aflibercept. Further studies are needed to assess the effect of aflibercept in patients who are not treatment naive.

Purpose Fundus autofluorescence (AF) has become a standard imaging test for evaluating retinal disorders such as age-related macular degeneration, hereditary, and inflammatory conditions. Little attention has been given to the influence of technical specifications of individual imaging devices and their potential influence on the studies obtained. Methods AF was recorded using a Heidelberg confocal scanning laser ophthalmoscope (cSLO) with 488 nm laser exciter (488 nm-AF), and a conventional Topcon fundus camera with halogen lamp exciter and 580 nm band-pass filter (580 nm-AF). Images of patients with various macular conditions were obtained and compared. Results The autofluorescent signal is largely believed to derive from lipofuscein in the retinal pigment epithelium (RPE) and to represent a correlate of cellular metabolic activity. However, while images of geographic atrophy appeared to be rather comparable, significant differences were noted in patients with central serous chorioretinopathy, rip of the RPE and cystoids macular edema. It has been hypothesized that macular pigments (MPs) and more precisely changes of MPs density account in part for the differences, as MPs block 488nm-AF more intensely than 580nm-AF. Conclusion Fundus autofluorescence images obtained with different imaging systems may not be interchangeable depending on the exciter and band-pass filters used. Caution has to be taken when comparing diagnostic or therapeutic findings on AF from imaging devices with different specifications

Purpose: To describe the retinal imaging findings in the index patient with Heimler syndrome (OMIM #234580).Design: Non-interventional case report.Methods: A 29-year-old woman with Heimler syndrome developed bilateral vision loss. Fluorescein angiography (FA), fundus autofluorescence (FAF), spectral domain optical coherence tomography (SD-OCT) and electroretinography (ERG) were performed to assess the retinal anatomy and function.Results: FA showed mottling of the retinal pigment epithelium (RPE) in the posterior pole and periphery of the retina. FAF revealed hyper and hypoautofluorescent dots corresponding to the RPE mottling observed on FA. SD-OCT documented loss of the inner/outer segments boundary, and RPE thinning. ERG testing excluded generalized rod-cone dysfunction.Conclusion: We report an adult-onset macular dystrophy in one of the previously reported patients with Heimler syndrome and hypothesize that this syndrome is probably an expression of a ciliopathy.

Purpose To investigate safety and maculat telangiectasia type 2. Methods 5 patients were followed monthly and received 3 initial doses of ranibizumab with monthly PRN treatment thereafter. All patients were evaluated by clinical examination, visual acuity, leakage on fluorescein angiography and OCT. Results Results: An average of 8 injections (3-12) over 12 months were given. Mean VA was 0.49 (LogMar) at BSL, 0.41 at wk 1, 0.42 at M1 and 0.45 at M12. Central macular thickness (CMT) measured 390, 265, 350 and 408 µm respectively. Adverse events were limited to surface irritations. Conclusion Conclusion: Short-term improvements in visual acuity and CMT, as seen after wk 1, were not sustained over 12 months.

Purpose To explore whether monthly intravitreal ranibizumab injections are associated with a lower rate of new choroidal neovascularization (CNV) in fellow eyes of patients with unilateral neovascular age-related macular degeneration. Design Retrospective data analysis of randomized, controlled clinical trials. Methods Incidence of new CNV in fellow eyes was calculated at 12 and 24 months from 2 clinical trials (the Minimally Classic/Occult Trial of the Anti-VEGF Antibody Ranibizumab in the Treatment of Neovascular Age-Related Macular Degeneration [MARINA] study and the Anti-VEGF Antibody for the Treatment of Predominantly Classic Choroidal Neovascularization in Age-Related Macular Degeneration [ANCHOR] study), based on fluorescein angiographic reading center criteria and investigator evaluation. Patients treated with monthly ranibizumab (0.3 and 0.5 mg) were compared with those receiving a sham injection (MARINA) or photodynamic therapy (ANCHOR). Results In MARINA, new CNV developed in fellow eyes in 20.3% of the 0.3-mg ranibizumab group by 12 months and in 30.4% by 24 months. The conversion rate in the 0.5-mg ranibizumab group was 21.1% and 38.0% by 12 and 24 months, respectively. In the sham group, 26.4% converted by 12 months and 36.3% converted by 24 months. In ANCHOR, fellow eyes in 15.9% of the 0.3-mg ranibizumab group converted by 12 months and fellow eyes in 23.8% converted by 24 months. The conversion rate in the 0.5-mg ranibizumab group was 24.3% and 35.1% by 12 and 24 months, respectively. In the photodynamic therapy group, 25.4% converted by 12 months and 38.8% converted by 24 months. Differences in conversion rates at 12 and 24 months between the 0.3-mg or 0.5-mg ranibizumab groups and respective controls (sham or photodynamic therapy) were not statistically significant. Conclusions Results of this study do not support the hypothesis that monthly ranibizumab injections reduce the rate of CNV development in untreated fellow eyes.

purpose : To describe a neovascular pattern associated with treatment-refractory neovascular age-related macular degeneration (AMD). Design A retrospective observational case series. Methods setting: Clinical practice. patient population: Twelve eyes of 12 patients with neovascular AMD in which a poor anatomic response to anti–vascular endothelial growth factor (VEGF) therapy was related to polypoidal choroidal vasculopathy (PCV). observation procedure: Slit-lamp biomicroscopy, optical coherence tomography, fluorescein and indocyanine green angiography. main outcome measures: Snellen visual acuity (VA), anatomic response to therapy including presence or absence of retinal edema, hemorrhage, and lipid exudates. Results New or persistent PCV was identified in a cohort of patients demonstrating increasing macular exudation despite regular intravitreal ranibizumab (Lucentis; Genentech Inc, South San Francisco, California, USA) or bevacizumab (Avastin; Genentech Inc) injections for a minimum of 6 months. Treatment with verteporfin photodynamic therapy (PDT), PDT/anti-VEGF combination therapy, or continued anti-VEGF monotherapy resulted in complete resolution of exudation in 9 of 12 patients and partial resolution of exudation in the remaining 3 patients. Conclusion Treatment-refractory neovascular AMD may harbor vascular abnormalities such as PCV. Modifications in therapeutic protocols may be indicated in order to improve visual and anatomic outcomes in this population.

To evaluate vitreomacular relations in different stages of age-related macular degeneration (AMD) without the influence of genetics and environmental factors.Retrospective, observational case series.This was a multicenter study consisting of 29 previously untreated subjects with active exudative (wet) AMD in one eye and active nonexudative (dry) AMD in the fellow eye who were compared with 10 previously untreated subjects with end-stage geographic atrophy in one eye and an end-stage fibrotic (disciform) scar in the fellow eye. All subjects were studied with ultrasonography to identify the presence of posterior vitreous detachment (PVD) and by optical coherence tomography to detect vitreomacular adhesion (VMA).The incidence of PVD in eyes with nonexudative AMD was 20 (69%) of 29, compared with 6 (21%) of 29 with active exudative AMD (P = .002). VMA was present in 11 (38%) of 29 of eyes with exudative AMD and in only 3 (10%) of 29 eyes with nonexudative AMD (P = .008). The incidence of PVD in geographic atrophy was 7 (70%) of 10, compared with 4 (40%) of 10 with disciform scar (P = .44). VMA was present in 2 (20%) of 10 eyes with disciform scars and in 0 (0%) of 10 eyes with geographic atrophy (P = .48).PVD may protect against exudative AMD, whereas VMA may promote exudative AMD. This phenomenon is not evident in end-stage disease because of an increased incidence of PVD and a decreased incidence of VMA in eyes with disciform scars. Genetic and environmental factors do not seem to influence these observations.

Purpose To compare the frequency of genes associated with age-related macular degeneration (AMD), specifically the complement factor H alleles in patients with choroidal neovascularization (CNV) secondary to pathologic myopia (CNV). Methods Patients with pathologic myopia (defined as axial length > 25.50mm or - 6.00 dpt) were examined. The presence of choroidal neovascularization was confirmed by clinical examination and fundus photography; fluorescein angiography and autofluorescence images were analyzed when available. DNA samples from all study subjects were screened for variants in the CFH genes, commonly associated with age-related macular degeneration. Results Seventy-three patients (47 female and 36 male; age 23 – 90 years; average: 59 years) were included into the study. Visual acuity ranged from 20/25 to count fingers in 1 meter. The frequency of the CFH Y402H as well as other common CFH polymorphisms in this cohort corresponded to that detected in the general population. Conclusion Factor h variants commonly associated with AMD and other forms of CNV may not play a causal role in neovascular complications of pathologic myopia.

Retinal angiomatous proliferation (RAP) is a distinct form of neovascularization in patients with age-related macular degeneration. Lacking definitive sequential histopathologic evidence of its intraretinal versus choroidal origin, the clinical observations of early stages of RAP lesions may provide clues to help further expand our understanding of this entity.Five eyes of four patients with early Stage 1 RAP were examined. Fundus photography, fluorescein and indocyanine green angiography as well as time-domain and spectral-domain optical coherence tomography were performed. Images were assessed to determine the characteristics of neovascularization in early stage RAP lesions and the response of the lesions to treatment or observation.The analysis of the selected cases suggests a choroidal origin of the neovascular complex with the early formation of a retinal choroidal anastomosis without evidence of underlying occult Type 1 neovascularization. Three eyes responded to a single treatment with intravitreal ranibizumab (0.5 mg) and 2 eyes (1 patient) resolved spontaneously without treatment.The neovascularization in RAP may originate not only from deep retinal capillaries but also from the choroid. We therefore propose the more descriptive term "Type 3 neovascularization" for this entity to emphasize the intraretinal location of the vascular complex and distinguish this type from the two types of neovascularization previously described by J. Donald Gass in his classic text.

Sorsby fundus dystrophy (SFD), originally described by Sorsby and Mason in 1949, 1 is a rare autosomal dominant retinal degeneration. To date, 8 mutations in the tissue inhibitor of metalloproteinases 3 (TIMP3) gene on chromosome 22q13 have been described in patients with SFD. 2 Herein we describe clinical features in a family of Eastern European-Jewish ancestry with a novel TIMP3 mutation.

Background Age-related macular degeneration (ARMD) is the most prevalent cause of visual loss in patients older than 60 years in the United States. Observation of drusen is the hallmark finding in the clinical evaluation of ARMD. Objectives To segment and quantify drusen found in patients with ARMD using image analysis and to compare the efficacy of image analysis segmentation with that of stereoscopic manual grading of drusen. Design Retrospective study. Setting University referral center. Patients Photographs were randomly selected from an available database of patients with known ARMD in the ongoing Columbia University Macular Genetics Study. All patients were white and older than 60 years. Interventions Twenty images from 17 patients were selected as representative of common manifestations of drusen. Image preprocessing included automated color balancing and, where necessary, manual segmentation of confounding lesions such as geographic atrophy (3 images). The operator then chose among 3 automated processing options suggested by predominant drusen type. Automated processing consisted of elimination of background variability by a mathematical model and subsequent histogram-based threshold selection. A retinal specialist using a graphic tablet while viewing stereo pairs constructed digital drusen drawings for each image. Main Outcome Measures The sensitivity and specificity of drusen segmentation using the automated method with respect to manual stereoscopic drusen drawings were calculated on a rigorous pixel-by-pixel basis. Results The median sensitivity and specificity of automated segmentation were 70% and 81%, respectively. After preprocessing and option choice, reproducibility of automated drusen segmentation was necessarily 100%. Conclusions Automated drusen segmentation can be reliably performed on digital fundus photographs and result in successful quantification of drusen in a more precise manner than is traditionally possible with manual stereoscopic grading of drusen. With only minor preprocessing requirements, this automated detection technique may dramatically improve our ability to monitor drusen in ARMD.

Background: The hallmarks of age related macular degeneration (AMD) are the subretinal deposits known as drusen. Current manual methods of drusen segmentation and quantification are laborious and subjective. The authors introduced a digital method and tested it for accuracy and reliability. Methods: Fourteen eyes with drusen were selected. The authors digitally reconstructed the macular background using normal background areas (“dots”) fitted to quadratic polynomials in two zones. The model was used to level the reflectance for the purpose of segmenting drusen by a global threshold. Measurements of drusen areas were compared with those of a semi-automated background levelling technique and manual drawings from stereo pairs. Results: Intraobserver reproducibility had standard deviations from 0.1% to 4.1%. Interobserver reproducibility yielded 95% limits of agreement of −2.7% to 6.3%. The dots method compared with manual drawings and with the semi-automated method had 95% limits of agreement of −8.3% to 2.8% and −7.1% to 4.8%, respectively. Conclusions: The dots method was reproducible and accurate with respect to validated methods. It provided less total operating time and greater precision than that of standard fundus photo grading. With implementation of commercial software, this technique for macular image analysis has potential for use in clinical research.

Purpose The aim of this study was to describe the clinical and angiographic response of squamous cell carcinoma (SCC) of the conjunctiva to treatment with photodynamic therapy (PDT). Design Interventional case series. Methods In a prospective study, three patients (62 to 86 years old) with SCC of the conjunctiva were treated with PDT. Patients received one to three treatments of verteporfin (6 mg/m 2 body surface area, intravenously). The light dose was calculated as 50 J/cm 2 . All tumors were irradiated 1 minute after injection. The mean follow-up time was 8.6 months (7 to 12 months). Main outcome measurements were clinical and angiographic response and treatment-related side effects. Results One week after treatment, angiographic occlusion of tumor vasculature and normal conjunctival vessels was observed in all patients. Tumor regression was noted in all patients 1 month after treatment. Two patients had complete regression (clinical and angiographic observation) after one or two treatments for the entire follow-up time. One tumor involved large aspects of the conjunctiva and cornea. In this case, only the treated areas showed tumor regression. PDT caused minimal temporary local irritation in two patients, and small conjunctival hemorrhages and mild transient chemosis in the three eyes directly after treatment. One patient had infusion-related back pain. Conclusion The preliminary results of this study suggest that PDT may be a valuable addition to the treatment of patients with SCC of the conjunctiva. However, longer follow-up is necessary to assess the duration and degree of tumor control.

Age-related macular degeneration (AMD) is a degenerative eye disease that can cause severe and irreversible loss of central vision. While most patients with AMD experience only a slow, moderate decrease of visual acuity as a result of atrophic changes of the retina, 10% of patients suffer from a severe and rapidly developing visual loss. According to literature and clinical experience, up to 90% of this significantly affected group present with the neovascular form of AMD (Bressler et aI.1982; Guyer et al.1986; Bressler et al.1988). This form is characterized by the development of abnormal blood vessels under the retina, which leak fluid, lipids and blood.Over time the leakage leads to fibrosis of the central retina with loss of photoreceptors and concomitant vision decline (Bressler et al.1982). In North America and Europe this is the leading cause of legal blindness in people over 65 years of age (Leibowitz et al.1980).

Purpose: Screening for detection of retinopathy of prematurity (ROP) currently is limited to indirect ophthalmoscopy, which requires considerable examiner skill and experience. We investigated whether conventional 10 MHz B-scan ultrasonography could document the clinical stages of ROP as accurately as indirect ophthalmoscopy. Methods: Thirty-four eyes of 18 neonates were examined by masked, independent observers with indirect ophthalmoscopy and digitally recorded 10-MHz B-scan ultrasonography. After pupil dilation and lid speculum placement, the retinologist recorded the stage of retinopathy with a retinal drawing. The ultrasonographer, without use of papillary mydriatics or lid speculum, determined the presence or absence of a ridge or tractional elements, if present on the ridge. Results: Ultrasound grade correlated with clinical grade ( R = .79, P < .001). However, nine eyes were overdiagnosed by one stage, and one eye, in which a peripheral detachment was mistaken for an artifact, was underdiagnosed. Conclusions: Ten-megahertz ultrasonography offers the potential of imaging and detecting the clinical stages of ROP; the use of higher ultrasound frequencies, now becoming commercially available, is likely to enhance diagnostic accuracy. Care must be taken to distinguish between artifact and true anatomical structures in noncontact ultrasound examinations. Neonates with suspected ROP could be screened with B-scan ultrasonography by neonatal personnel without pupillary dilatation or lid speculum, thus eliminating potential morbidity, and clinically significant cases of ROP then could be referred to the retinologist. J Pediatr Ophthalmol Strabismus 2004;41:345–350.