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1.  Characteristics of intraretinal deposits in acute central serous chorioretinopathy 
Purpose
To describe the temporal and spatial characteristics of intraretinal deposits in patients with acute central serous chorioretinopathy (CSC) using spectral domain optical coherence tomography (OCT).
Materials and methods
We retrospectively reviewed the medical records of all patients that presented with acute CSC to Weill Cornell Medical College from January 2012 to May 2013. Acute CSC was defined as a diagnosis of CSC within 4 months of the onset of symptoms. Only one eye per patient was included in the study. Each patient was imaged with spectral domain OCT at the initial office visit. The decision to reimage these patients was made by the treating physician.
Results
A total of 25 patients (25 eyes; 17 men and eight nonpregnant women) were included in this review. Seven of 25 patients (28%) demonstrated intraretinal deposits within the outer plexiform layer during the initial OCT, with deposits appearing as early as the same day as the onset of symptoms. A total of 25 of 25 patients (100%) demonstrated intraretinal deposits in the outer nuclear layer upon initial (76%) or follow-up OCT, as early as 2 days after the onset of symptoms. A total of 24 of 25 patients (96%) demonstrated deposits in the external limiting membrane upon a follow-up OCT, as early as 7 days from symptoms appearing. A total of 24 of 25 patients (96%) developed intraretinal deposits in the inner segment/outer segment layer upon follow-up OCT, appearing as early as 14 days after symptom onset. At the time of resolution of subretinal fluid, 20 of 25 patients (80%) demonstrated intraretinal deposits.
Conclusion
Intraretinal deposits are present in the outer retinal layers in patients with acute CSC, with the deposits appearing progressively deeper within the retina as the condition evolves. Upon resolution of subretinal fluid, the deposits slowly resolve.
doi:10.2147/OPTH.S48894
PMCID: PMC3979800
acute central serous chorioretinopathy; intraretinal deposits; spectral domain OCT
2.  Spectrum of Ocular Manifestations in CLN2-Associated Batten (Jansky-Bielschowsky) Disease Correlate with Advancing Age and Deteriorating Neurological Function 
PLoS ONE  2013;8(8):e73128.
Background
Late infantile neuronal ceroid lipofuscinosis (LINCL), one form of Batten’s disease is a progressive neurodegenerative disorder resulting from a CLN2 gene mutation. The spectrum of ophthalmic manifestations of LINCL and the relationship with neurological function has not been previously described.
Methods
Patients underwent ophthalmic evaluations, including anterior segment and dilated exams, optical coherence tomography, fluorescein and indocyanine green angiography. Patients were also assessed with the LINCL Neurological Severity Scale. Ophthalmic findings were categorized into one of five severity scores, and the association of the extent of ocular disease with neurological function was assessed.
Results
Fifty eyes of 25 patients were included. The mean age at the time of exam was 4.9 years (range 2.5 to 8.1). The mean ophthalmic severity score was 2.6 (range 1 to 5). The mean neurological severity score was 6.1 (range 2 to 11). Significantly more severe ophthalmic manifestations were observed among older patients (p<0.005) and patients with more severe neurological findings (p<0.03). A direct correlation was found between the Ophthalmic Severity Scale and the Weill Cornell Neurological Scale (p<0.002). A direct association was also found between age and the ophthalmic manifestations (p<0.0002), with older children having more severe ophthalmic manifestations.
Conclusions
Ophthalmic manifestations of LINCL correlate closely with the degree of neurological function and the age of the patient. The newly established LINCL Ophthalmic Scale may serve as an objective marker of LINCL severity and disease progression, and may be valuable in the evaluation of novel therapeutic strategies for LINCL, including gene therapy.
doi:10.1371/journal.pone.0073128
PMCID: PMC3756041  PMID: 24015292
3.  Transconjunctival 25-gauge pars plana vitrectomy and internal limiting membrane peeling for chronic macular edema 
Background
The purpose of this study was to investigate the visual and anatomic outcomes in patients with chronic macular edema who underwent 25-gauge pars plana vitrectomy with internal limiting membrane peeling.
Methods
This study was a retrospective chart review of 24 eyes from 21 patients who underwent 25-gauge pars plana vitrectomy and indocyanine green-assisted internal limiting membrane peeling for chronic macular edema. Preoperative and postoperative spectral-domain optical coherence tomography (OCT) was examined for macular thickness and macular volume. Outcomes and variables were analyzed using the two-tailed t-test and Spearman’s rank correlation coefficient.
Results
Twenty-four eyes from 11 men and 10 women of mean age 69 (range 55–84) years were included. Four patients (17%) had chronic macular edema from uveitis, four (17%) from retinal vein occlusion, and 16 (67%) from diabetes. Mean visual acuity was 20/103 preoperatively and 20/87 postoperatively (P = 0.55). Sixty-three percent of the eyes had improved vision (47% better than 20/40), 21% maintained the same vision, and 17% had worse vision. Forty-seven percent of improved eyes and 30% of total eyes gained more than two lines of visual acuity (range −9 to +7 lines). Mean macular thickness was 455 μm preoperatively and 396 μm postoperatively (P = 0.29). Mean macular volume was 7.9 mm3 preoperatively and 7.5 mm3 postoperatively (P = 0.51). The strongest predictor of postoperative visual acuity was initial visual acuity (r = 0.673, P = 0.0003).
Conclusion
Even though a majority of patients had improved vision and decreased macular thickening after 25-gauge pars plana vitrectomy with internal limiting membrane peeling for chronic macular edema of various etiologies, the difference in visual acuity or macular thickening did not reach statistical significance.
doi:10.2147/OPTH.S33391
PMCID: PMC3402127  PMID: 22848140
chronic macular edema; diabetes mellitus; internal limiting membrane peeling; 25-gauge vitrectomy; uveitis; vein occlusion
4.  Ultra-widefield fluorescein angiography of white without pressure 
Purpose
To describe ultra-widefield fluorescein angiography (UWFA) findings in eyes with white without pressure (WWOP) and in eyes without any obvious peripheral chorioretinal disease, and to determine if a difference exists between these two groups.
Methods
A retrospective review of 379 eyes undergoing diagnostic UWFA using the Optos 200Tx imaging system. Eyes were excluded if the quality of the color photograph or UWFA prevented reliable evaluation. Eyes were also excluded if there was any evidence of peripheral retinal or choroidal disease, which was thought to have an effect on UWFA (eg, peripheral background diabetic or hypertensive retinopathy, vein occlusion, or any other peripheral vascular disorder). Eyes were determined to have WWOP, based on a dilated fundus examination and color fundus photography that contained areas of peripheral retinal whitening consistent with the diagnosis. UWFA was evaluated by trained masked graders, and determined to have or not have peripheral vascular leakage and/or staining.
Results
Of the 379 eyes evaluated, 45 eyes were included in the study. Twelve eyes were determined to have peripheral WWOP; 33 eyes did not have WWOP on examination or color fundus photography. Three common UWFA peripheral patterns were visualized. Eyes with and without WWOP were grouped into one of three patterns. The majority of eyes without WWOP demonstrated UWFA pattern one (69.7%), while those in the WWOP group demonstrated pattern three (50%). The distribution of UWFA patterns is statistically different between those with and without WWOP (P = 0.002). In eyes without WWOP, in patients with no documented systemic microvascular disease (diabetes, hypertension), 71.4% of eyes had UWFA pattern one while 14.3% had both patterns two and three.
Conclusion
This study is one of the first to specifically evaluate peripheral vascular leakage/staining in eyes with WWOP as well as in eyes without any obvious peripheral chorioretinal disease. We demonstrate that a significant portion of WWOP eyes exhibit peripheral findings on UWFA (pattern one) compared to eyes without WWOP. Importantly, even in eyes that are apparently unremarkable in the periphery on exam and color photography, UWFA can still show peripheral vascular abnormalities. These results warrant further investigation.
doi:10.2147/OPTH.S43450
PMCID: PMC3667996  PMID: 23737658
ocular imaging; ptos imaging; retina periphery
5.  Persistent Suppression of Ocular Neovascularization with Intravitreal Administration of AAVrh.10 Coding for Bevacizumab 
Human Gene Therapy  2011;22(12):1525-1535.
Abstract
Vascular endothelial growth factor (VEGF) plays an important role in the pathogenesis of neovascular age-related macular degeneration and diabetic retinopathy. Bevacizumab, an anti-VEGF monoclonal antibody, is efficacious for these disorders, but requires monthly intravitreal administration, with associated discomfort, cost, and adverse event risk. We hypothesized that a single intravitreal administration of adeno-associated virus (AAV) vector expressing bevacizumab would result in persistent eye expression of bevacizumab and suppress VEGF-induced retinal neovascularization. We constructed an AAV rhesus serotype rh.10 vector to deliver bevacizumab (AAVrh.10BevMab) and assessed its ability to suppress neovascularization in transgenic mice overexpressing human VEGF165 in photoreceptors. Intravitreal AAVrh.10BevMab directed long-term bevacizumab expression in the retinal pigmented epithelium. Treated homozygous mice had reduced levels of neovascularization, with 90±4% reduction 168 days following treatment. Thus, a single administration of AAVrh.10BevMab provides long-term suppression of neovascularization without the costs and risks associated with the multiple administrations required for the current conventional bevacizumab monoclonal drug delivery.
Mao and colleagues demonstrate that a single injection of an adeno-associated viral vector based on rhesus serotype 10 into the vitreous is capable of expressing an anti-vascular endothelial growth factor antibody in the eye and suppressing retinal neovascularization.
doi:10.1089/hum.2011.090
PMCID: PMC3278820  PMID: 21801028
6.  Boston type I keratoprosthesis-donor cornea interface evaluated by high-definition spectral-domain anterior segment optical coherence tomography 
Background
The purpose of this study was to assess whether the resolution offered by two different, recently commercially available high-resolution, spectral-domain anterior segment optical coherence tomography (AS-OCT) instruments allows for detailed anatomic characterization of the critical device-donor cornea interface in eyes implanted with the Boston type I permanent keratoprosthesis.
Methods
Eighteen eyes of 17 patients implanted with the Boston type I keratoprosthesis were included in this retrospective case series. All eyes were quantitatively evaluated using the Cirrus HD-OCT while a subset (five eyes) was also qualitatively imaged using the Spectralis Anterior Segment Module. Images from these instruments were analyzed for evidence of epithelial migration onto the anterior surface of the keratoprosthesis front plate, and presence of a vertical gap between the posterior surface of the front plate and the underlying carrier donor corneal tissue. Quantitative data was obtained utilizing the caliper function on the Cirrus HD-OCT.
Results
The mean duration between AS-OCT imaging and keratoprosthesis placement was 29 months. As assessed by the Cirrus HD-OCT, 83% of eyes exhibited epithelial migration over the edge of the front plate. Fifty-six percent of the keratoprosthesis devices displayed good apposition of the device with the carrier corneal donor tissue. When a vertical gap was present (44% of eyes), the mean gap was 40 (range 8–104) microns. The Spectralis Anterior Segment Module also displayed sufficient resolution to allow for similar characterization of the device-donor cornea interface.
Conclusion
Spectral-domain AS-OCT permits high resolution imaging of the keratoprosthesis device-donor cornea interface. Both the Cirrus HD-OCT and the Spectralis Anterior Segment module allowed for visualization of epithelial coverage of the device-donor cornea interface, as well as identification of physical gaps. These imaging modalities, by yielding information in regard to integration of the keratoprosthesis with surrounding corneal tissue, may help identify those at risk for keratoprosthesis-related complications, such as extrusion and endophthalmitis, and hence guide clinical management.
doi:10.2147/OPTH.S34787
PMCID: PMC3437949  PMID: 22969280
keratoprosthesis; anterior segment optical coherence tomography; epithelialization
7.  Peripheral retinal ischaemia, as evaluated by ultra-widefield fluorescein angiography, is associated with diabetic macular oedema 
Purpose
To determine the relationship between retinal ischaemia and the presence of macular oedema (DMO) in patients with diabetic retinopathy (DR) using ultra-widefield fluorescein angiography (UWFA) imaging.
Methods
A retrospective review of 122 eyes of 70 treatment-naïve diabetic patients who underwent diagnostic UWFA using the Optos 200Tx imaging system. Two independent, masked graders quantified the area of retinal ischaemia. Based on clinical examination and optical coherence tomography (OCT), each patient was given a binary classification as either having DMO or no DMO. McNemar's test (with Yates' correction as indicated) and a two-sample test of proportions were used to determine the relationship between DMO and ischaemia for binary and proportional data, respectively. Linear and logistic models were constructed using generalised estimating equations to test relationships between independent variables, covariates and outcomes while controlling for inter-eye correlation, age, gender, haemoglobin A1c, mean arterial pressure and dependence on insulin.
Results
Seventy-six eyes (62%) exhibited areas of retinal ischaemia. There was a significant direct correlation between DMO and peripheral retinal ischaemia as seen on UWFA (p<0.001). In addition, patients with retinal ischaemia had 3.75 times increased odds of having DMO compared with those without retinal ischaemia (CI 1.26 to 11.13, p<0.02).
Conclusion
Retinal ischaemia is significantly correlated with DMO in treatment-naïve patients with DR. UWFA is a useful tool for detecting peripheral retinal ischaemia, which may have direct implications in the diagnosis, follow-up and treatment such as targeted peripheral photocoagulation.
doi:10.1136/bjophthalmol-2011-300774
PMCID: PMC3329634  PMID: 22423055
Ultra-wide field imaging; fluorescein angiography; diabetes; diabetic retinopathy; diabetic macular oedema; retina; epidemiology; retina
8.  Surface epithelialization of the type I Boston keratoprosthesis front plate: immunohistochemical and high-definition optical coherence tomography characterization 
Background
The aim of this work is to characterize a transparent tissue layer partially covering the anterior surface of the type I Boston permanent keratoprosthesis front plate in four patients.
Methods
The tissue over the front plate was easily scrolled back as a single transparent layer using a sponge. In two cases, histopathologic analysis was undertaken and immunofluorescent staining with a cytokeratin 3-specific antibody was performed. The relationship of the tissue to the keratoprosthesis device was further characterized using spectral domain high-definition optical coherence tomography (HD-OCT).
Results
Histopathologic analysis revealed the tissue to be non-keratinized squamous epithelium. No goblet cells were seen, suggesting the cells were of corneal, and not conjunctival, epithelial origin. Immunofluorescent staining of all cells was positive for cytokeratin 3, a protein strongly associated with corneal epithelium. The tissue was easily discerned by HD-OCT and was of substantial thickness near the external junction between the keratoprosthesis device and the carrier corneal tissue. In three cases, visual acuity was unaffected by the presence or absence of this tissue. In one case, a prominent tissue margin temporarily obscured the visual axis and reduced visual acuity; this resolved with mechanical central debridement and has not recurred.
Conclusions
The transparent tissue layer covering the anterior surface of the type I Boston keratoprosthesis front plate was found to represent non-keratinized squamous epithelium, most likely of corneal epithelial origin. This potentially represents a further step in bio-integration of the keratoprosthesis device. In particular, epithelial coverage of the critical junction between the device and the carrier corneal tissue might serve an important barrier function and further reduce the incidence of infection and extrusion of the type I Boston permanent keratoprosthesis.
doi:10.1007/s00417-012-1960-5
PMCID: PMC3404271  PMID: 22371021
Keratoprosthesis; Corneal epithelium; Bio-integration
9.  Optical coherence tomography predicts visual outcome in macula-involving rhegmatogenous retinal detachment 
Purpose
Visual recovery after rhegmatogenous retinal detachment (RRD) repair depends upon various anatomical factors. We investigated spectral-domain optical coherence tomography (SD-OCT) abnormalities, pre- and postoperatively, in patients with nontraumatic RRD and correlated these findings with visual outcome.
Methods
The medical records of all patients presenting to Weill Cornell Medical College with nontraumatic macula-involving RRD from August 2010 to September 2011 were retrospectively reviewed in this single-center, consecutive case series. All patients underwent pre- and postoperative visual acuity (VA) testing, slit-lamp biomicroscopy, and dilated fundus examination. Spectral domain optical coherence tomography was obtained preoperatively in twelve patients and postoperatively in ten patients.
Results
Twelve patients (12 eyes) were included in the final analysis. Preoperative optical coherence tomography revealed that the inner segment/outer segment (IS/OS) junction was disrupted in 10/12 eyes (83%), the external limiting membrane (ELM) was disrupted in 9/12 (75%) eyes, cystoid macular edema (CME) was present in 10/12 (83%) eyes, an epiretinal membrane (ERM) was present in 2/12 eyes (17%) and outer retinal corrugation was present in 7/12 (58%) eyes. In postoperative imaging of 10 eyes, the IS/OS junction was disrupted in 4/10 (40%), the ELM was disrupted in 3/10 (30%) eyes, CME was present in 2/10 (20%), and an ERM in 1/10 (10%). All retinas were attached postoperatively. Outer retinal corrugation was the most predictive of worse preoperative (P = 0.0016) and 1-month postoperative visual acuity (P = 0.05).
Conclusion
Preoperative SD-OCT demonstrating outer retinal corrugation in macula involving RRD predicts poor visual acuity outcome in nontraumatic RRD. Such findings may have implications for the urgency for these eyes to undergo surgical repair.
doi:10.2147/OPTH.S28173
PMCID: PMC3261694  PMID: 22275812
macula-involving; optical coherence tomography; outer retinal corrugation; retinal detachment
10.  Surgical management and ultrastructural study of choroidal neovascularization in punctate inner choroidopathy after bevacizumab 
Purpose
This study aims to describe surgical management results and the pathologic features of choroidal neovascularization (CNV) secondary to punctate inner choroidopathy (PIC) following anti-vascular endothelial growth factor treatment.
Design
This study is a case report on the surgical management and ultrastructural study of choroidal neovascularization.
Methods
Clinicopathologic and ultrastructural report of CNV membranes excised from both eyes of one patient was presented.
Results
The right eye responded to bevacizumab, and recurrence 17 months later did not; the left eye never responded. Excision of the active CNVs was performed 3 months after the last injection. In the right eye, there was no recurrence 23 months after surgery. In the left eye, CNV recurred after 6 months, with no response to bevacizumab. Electron microscopy revealed subretinal neovascular tissue and, additionally, Bruch's membrane and inner choroid in the left. In the right eye, lumens of many neovascular channels were occluded by microfibrils and pericytes were infrequent. In the left eye, patent CNV units with pericytes were present. There were scattered macrophages but no lymphocytes in either membrane. An inner focal choroidal lymphocytic infiltrate was discovered.
Conclusions
Submacular surgery did not cause complications following treatment with bevacizumab. Mostly nonfunctional capillaries in the right membrane failed to display pericytes. The left membrane, which was unresponsive to bevacizumab, displayed well-formed neovascular units consistently exhibiting pericytes. A focus of inner choroidal lymphocytic infiltration was found in the left eye despite the absence of overt clinical intraocular inflammation. This is the first pathological study employing human tissue that points to pericytes as a potential critical therapeutic target with the aggravating influence of inner choroidal chronic inflammation in PIC.
doi:10.1007/s12348-011-0050-x
PMCID: PMC3302998  PMID: 22120962
Bevacizumab; Choroidal neovascularization; Electron microscopy; Light microscopy; Punctate inner choroidopathy; Submacular surgery
11.  Surgical management and ultrastructural study of choroidal neovascularization in punctate inner choroidopathy after bevacizumab 
Purpose
This study aims to describe surgical management results and the pathologic features of choroidal neovascularization (CNV) secondary to punctate inner choroidopathy (PIC) following anti-vascular endothelial growth factor treatment.
Design
This study is a case report on the surgical management and ultrastructural study of choroidal neovascularization.
Methods
Clinicopathologic and ultrastructural report of CNV membranes excised from both eyes of one patient was presented.
Results
The right eye responded to bevacizumab, and recurrence 17 months later did not; the left eye never responded. Excision of the active CNVs was performed 3 months after the last injection. In the right eye, there was no recurrence 23 months after surgery. In the left eye, CNV recurred after 6 months, with no response to bevacizumab. Electron microscopy revealed subretinal neovascular tissue and, additionally, Bruch's membrane and inner choroid in the left. In the right eye, lumens of many neovascular channels were occluded by microfibrils and pericytes were infrequent. In the left eye, patent CNV units with pericytes were present. There were scattered macrophages but no lymphocytes in either membrane. An inner focal choroidal lymphocytic infiltrate was discovered.
Conclusions
Submacular surgery did not cause complications following treatment with bevacizumab. Mostly nonfunctional capillaries in the right membrane failed to display pericytes. The left membrane, which was unresponsive to bevacizumab, displayed well-formed neovascular units consistently exhibiting pericytes. A focus of inner choroidal lymphocytic infiltration was found in the left eye despite the absence of overt clinical intraocular inflammation. This is the first pathological study employing human tissue that points to pericytes as a potential critical therapeutic target with the aggravating influence of inner choroidal chronic inflammation in PIC.
doi:10.1007/s12348-011-0050-x
PMCID: PMC3302998  PMID: 22120962
Bevacizumab; Choroidal neovascularization; Electron microscopy; Light microscopy; Punctate inner choroidopathy; Submacular surgery
12.  Retinal nerve fiber layer evaluation in multiple sclerosis with spectral domain optical coherence tomography 
Purpose:
Histopathologic studies have reported retinal nerve fiber layer (RNFL) thinning in various neurodegenerative diseases. Attempts to quantify this loss in vivo have relied on time-domain optical coherence tomography (TDOCT), which has low resolution and requires substantial interpolation of data for volume measurements. We hypothesized that the significantly higher resolution of spectral-domain optical coherence tomography (SDOCT) would better detect RNFL changes in patients with multiple sclerosis, and that RNFL thickness differences between eyes with and without optic neuritis might be identified more accurately.
Methods:
In this retrospective case series, patients with multiple sclerosis were recruited from the Judith Jaffe Multiple Sclerosis Center at Weill Cornell Medical College in New York. Patients with a recent clinical diagnosis of optic neuritis (less than three months) were excluded. Eyes with a history of glaucoma, optic neuropathy (other than multiple sclerosis-related optic neuritis), age-related macular degeneration, or other relevant retinal and/or optic nerve disease were excluded. Both eyes of each patient were imaged with the Heidelberg Spectralis® HRA + OCT. RNFL and macular thickness were measured for each eye using the Heidelberg OCT software. These measurements were compared with validated published normal values, and were modeled as linear functions of duration of disease. The odds of an optic neuritis diagnosis as a function of RNFL and macular thickness were calculated.
Results:
Ninety-four eyes were prospectively evaluated using OCT. Ages of patients ranged from 26 to 69 years, with an average age of 39 years. Peripapillary RNFL thinning was demonstrated in multiple sclerosis patients; mean RNFL thickness was 88.5 μm for individuals with multiple sclerosis compared with a reported normal value of 97 μm (P < 0.001). Eyes with a history of optic neuritis had more thinning compared with those without optic neuritis (83.0 μm versus 90.5 μm, respectively, P = 0.02). No significant differences were observed in macular thickness measurements between eyes with and without optic neuritis, nor were macular thickness measurements significantly different from normal values. As a function of multiple sclerosis duration and controlling for age, RNFL thickness was decreased in patients with a duration of multiple sclerosis greater than five years compared with those with a duration less than or equal to one year (P = 0.008).
Conclusions:
Patients with a history of multiple sclerosis had RNFL thinning that was detectable on SDOCT. Decreasing RNFL thickness in eyes with optic neuritis was found, and the odds of having optic neuritis were increased significantly with decreasing RNFL thickness. Average RNFL thinning with increasing duration of disease was an excellent predictor of a reported history of optic neuritis. SDOCT retinal imaging may represent a high-resolution, objective, noninvasive, and easily quantifiable in vivo biomarker of the presence of optic neuritis and severity of multiple sclerosis.
PMCID: PMC2946989  PMID: 20922034
multiple sclerosis; spectral-domain optical coherence tomography; optical coherence tomography; nerve fiber layer; nerve fiber layer thickness; optic neuritis

Results 1-12 (12)