SUBFOVEAL CHOROIDAL THICKNESS AND VASCULAR ARCHITECTURE IN FELLOW EYES OF PATIENTS WITH CIRCUMSCRIBED CHOROIDAL HEMANGIOMA.

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.

A Case of Idiopathic Bilateral Hemolacria in an 11-Year-Old Girl.

An 11-year-old healthy girl was noted to have intermittent, spontaneous, and bilateral hemolacria of 14 months' duration. Examination and workup failed to identify an organic or psychogenic cause. The patient was expectantly managed with eventual spontaneous resolution. This report draws attention to this unusual and often concerning clinical entity. The case report is in compliance with the Health Insurance Portability and Accountability Act.

IODINE-125 PLAQUE BRACHYTHERAPY FOR DIFFUSE CHOROIDAL HEMANGIOMA.

PURPOSE: To report 6 cases of diffuse choroidal hemangioma in children treated with iodine-125 plaque brachytherapy at a single tertiary care center. METHODS: Retrospective case series. RESULTS: Six pediatric patients diagnosed with diffuse choroidal hemangioma were included in the study. Preplaque visual acuity ranged from 20/150 to no light perception. All patients had extensive serous retinal detachment at presentation. An iodine-125 radioactive plaque was placed on the affected eye to administer a dose of 34.2-42.1 Gy to the tumor apex over a median of 4 days. Tumor regression and subretinal fluid resolution were observed in all eyes within 17 months of treatment. Visual acuity improved in two patients. Radiation-induced cataract and subretinal fibrosis were documented in one case, and one patient developed radiation retinopathy. No patients developed neovascular glaucoma within the follow-up time of 12-65 months. CONCLUSION: Iodine-125 plaque radiotherapy is an effective option for diffuse choroidal hemangioma, although there is a risk for radiation-induced complications.

Clinical Outcomes in Vitrectomized versus Non-vitrectomized Eyes in Patients with Primary Vitreoretinal Lymphoma.

AIM: To evaluate if there is a difference in the clinical course of primary vitreoretinal lymphoma (PVRL) in vitrectomized versus non-vitrectomized eyes. METHODS: Observational multicenter retrospective case series of patients diagnosed with PVRL between 2007 and 2019, at three tertiary centers. The main outcomes were relapse rates, inflammatory parameters, and best-corrected visual acuities (BCVA). Statistical methods used were an adjusted generalized estimating equation model, and a proportional Cox model. RESULTS: Eighty patients (150 eyes) were followed for a median of 1.7 years. At presentation, there were no clinical differences between the groups. The relapse rate was 0.091/eye-year (EY) for vitrectomized eyes and 0.087/EY for non-vitrectomized eyes (p = .35). Vitrectomized eyes had better BCVA than non-vitrectomized eyes (p < .001). CONCLUSIONS: Vitrectomy had no effect on the relapse rate in eyes with PVRL. However, the decrease in vitreous cell and debris led to vitrectomized eyes having better visual acuity than non-vitrectomized eyes.

Intravitreal dexamethasone insert in diabetic macular edema super-refractory to anti-vascular endothelial growth factor therapy.

BACKGROUND: Some patients with diabetic macular edema (DME) fail to completely respond to anti-vascular endothelial growth factor (VEGF) therapy. These patients have a high treatment burden in the absence of significant improvement. We investigate the role of intravitreal dexamethasone insert (IDI) in eyes with super-refractory DME. METHODS: A non-randomized interventional study was performed among eyes with super-refractory DME refractory to anti-VEGF therapy. Eyes were treated with IDI after failing clinical response to anti-VEGF, with a minimum of 15 prior. Failure to respond was defined as failure of vision to improve at least one line on Snellen Acuity chart, central subfield thickness (CST) greater than 320 µm, or failure of CST to improve by 10% or more. Eyes with glaucoma or prior uncontrolled steroid-responsive ocular hypertension were excluded. Patient outcomes were analyzed at weeks 6, 12, 24, and year 1. RESULTS: Six eyes of four patients were identified. All patients had failed aflibercept. The mean number of prior anti-VEGF injections was 34.5. Eyes received an average of 2.92 dexamethasone injections per person-year (PY) and required breakthrough anti-VEGF injection at 1.95/PY. Mean pre-treatment visual acuity was 0.475 LogMAR, improving to 0.342 at week 6, and 0.375 at 1 year. Mean CST pre-injection was 386.5 mm, improving to 315 mm at 1 year. No glaucoma developed. CONCLUSIONS: Intravitreal dexamethasone insert appears effective in eyes with super-refractory DME. IDI resulted in excellent anatomic improvement on SD-OCT as well as modest visual improvement. Injection burden was reduced in those who may otherwise receive years of monthly treatments.

Advanced OCT Analysis of Biopsy-proven Vitreoretinal Lymphoma.

PURPOSE: Although diagnosing vitreoretinal lymphoma (VRL) can be challenging, early detection is critical for visual prognosis. We analyzed the spectrum of optical coherence tomography (OCT) findings in patients with biopsy-proven VRL and correlate these features with clinical parameters. DESIGN: This retrospective cross-sectional study was a multicenter chart review from 13 retina, uveitis, and ocular oncology clinics worldwide from 2008 to 2019. We included patients with a diagnosis of biopsy-proven VRL imaged with OCT at presentation. Ocular information, systemic information, and multimodal retinal imaging findings were collected and studied. The main outcome measure was the characteristics of VRL on OCT. RESULTS: A total of 182 eyes of 115 patients (63 women, mean age 65 years) were included in this study. The disease was bilateral in 81 patients (70%), and mean baseline visual acuity was 0.2 ± 0.89 logMAR (Snellen equivalent, 20/32). At baseline, 38 patients (33%) presented with isolated ocular involvement, 54 (45%) with associated central nervous system involvement, and 11 (10%) with other systemic lymphomatous involvement; an additional 12 patients (10%) presented with central nervous system and other systemic involvement. On OCT, tumor infiltration was identified in various retinal layers, including lesions in the subretinal pigment epithelium compartment (91% of eyes), the subretinal compartment (43% of eyes), and the intraretinal compartment (7% of eyes). OCT analysis of eyes with VRL identified 3 main regions of retinal infiltration. Subretinal pigment epithelium location, with or without subretinal infiltration, was the most common pattern of involvement and isolated intraretinal infiltration was the least.

Fundus albipunctatus photoreceptor microstructure revealed using adaptive optics scanning light ophthalmoscopy.

PURPOSE: Fundus albipunctatus is an inherited cause of congenital stationary night blindness. The objective of this report is to describe structural changes occurring in a macular phenotype of a novel RDH5 mutation producing fundus albipunctatus using high-resolution in vivo imaging. A 62-year-old male with longstanding night blindness underwent imaging and genetic evaluation. High-resolution images of the photoreceptor mosaic were compared to those of a healthy subject. Results of a comprehensive ophthalmic evaluation and genetic testing with imaging including fundus photography, spectral-domain optical coherence tomography (OCT), fluorescein angiography (FA), OCT angiography (OCT-A), and adaptive optics scanning light ophthalmoscopy (AOSLO) are described. OBSERVATIONS: The patient presented with visual acuity of 20/25 in both eyes and longstanding poor dark adaptation. Anterior segment examination was unremarkable. Fundoscopy revealed well circumscribed bilateral perifoveal mottling and atrophy in both eyes. Discrete white-yellow flecks were present beyond the vascular arcades extending to the far periphery. Genetic testing revealed a novel compound heterozygous RDH5 mutation (c.388C > T, p.Gln130*; c.665T > C, p.Leu222Pro). OCT demonstrated perifoveal photoreceptor and outer retinal irregularities, which corresponded to a window defect with late staining on FA. OCT-A demonstrated normal retinal vasculature with patchy areas of non-perfusion in the choriocapillaris. Macular abnormalities in both eyes were imaged using AOSLO to assess cone and rod photoreceptor architecture. While clinical features are consistent with a primary rod disorder, confocal AOSLO showed a paucity of normal cones with a small spared central island in both eyes. Rods appeared larger and more irregular throughout the macula. Non-confocal split detection AOSLO imaging revealed the presence of cone inner segments in dark regions of confocal imaging, indicating some degree of photoreceptor preservation. CONCLUSIONS AND IMPORTANCE: The AOSLO imaging of this particular macular phenotype of fundus albipunctatus demonstrates some of the structural photoreceptor abnormalities that occur in this condition, adding insight to the variable presentation of RDH5 retinopathy. The presence of preserved inner segment architecture suggests the possibility that gene therapy could play a future role in treating this condition.

Intra-Arterial Tissue Plasminogen Activator for Central Retinal Artery Occlusion.

PURPOSE: To investigate the benefit of early intra-arterial tissue plasminogen activator (IAT) for treatment of central retinal artery occlusion (CRAO). PATIENTS AND METHODS: Fifteen eyes of 15 patients presenting with acute CRAO were included in this retrospective consecutive interventional case series. Patients were excluded if treatment with IAT was not initiated within 12 hours. The diagnosis was confirmed by an ophthalmologist. IAT was performed via a transfemoral arterial approach. Tissue plasminogen activator (tPA) was infused into the ophthalmic artery in aliquots up to 3mg to a maximum of 22mg. Paracentesis was done at the ophthalmologist's discretion. The primary outcome measure was visual acuity after three weeks. Adverse events were recorded during treatment and follow-up visits. RESULTS: After treatment with IAT, there was a statistically significant improvement in visual acuity, with a mean change of -0.76 (SD 0.91; range -2.4 to 0.85) logMAR (p=0.006). Vision improved by 3 or more lines in 53%, and of these, the mean Snellen visual acuity improvement was >6 lines. Notably, 4 patients (27%) improved from CF or worse to 20/80 or better. The mean dose of tPA used was 17mg and the mean time to treatment was 8.83 hours (range: 5.5 to 12 hours). There were no statistically significant differences based on time to treatment, dose of tPA, or use of a paracentesis. No major adverse events were recorded. CONCLUSION: IAT was safe and showed significant visual improvement in this small uncontrolled study. Larger studies and efforts to decrease time to treatment should be initiated to optimize outcomes.

Optical Coherence Tomography Characteristics of the Choroid Underlying Congenital Hypertrophy of the Retinal Pigment Epithelium.

PURPOSE: This study evaluates and characterizes the choroid underlying congenital hypertrophy of the retinal pigment epithelium (CHRPE). METHODS: Retrospective observational study of CHRPE at least 2 mm in diameter. Choroidal vascular architecture was qualitatively examined. Choroidal thickness was measured by 2 independent observers using enhanced depth imaging spectral domain optical coherence tomography. RESULTS: Forty-six eyes of 46 patients with CHRPE were included. Thirty-two lesions had imaging sufficient for analysis. Haller's layer was healthy in 18 (56%), thin in 13 (41%), and absent in 1 (2%). Sattler's layer was atrophic in 30 (94%), and choriocapillaris was atrophic in 31 (97%). CHRPE with thinned Haller's layer had significantly larger diameter. The mean sub-CHRPE choroidal thickness was 82.4 ± 7.9 µm, compared to a thickness of 148.4 ± 9.6 µm in the normal adjacent choroid (p < 0.0001). Mean retinal thickness overlying the CHRPE was 77.3 ± 4.3 µm, compared to a retinal thickness of 137.8 ± 2.9 µm overlying the normal adjacent choroid (p < 0.0001). Sub-CHRPE choroidal thickness was a mean of 56.2 ± 3.1% of the adjacent normal choroidal thickness. CONCLUSION: The underlying choroid CHRPE is thinner than the adjacent normal choroid. All layers of the choroid can be thin with a preference of the inner Sattler's and choriocapillaris layers.

Rapidly progressive streptococcus dysgalactiae corneal ulceration associated with erlotinib use in stage IV lung cancer.

PURPOSE: To present a unique case of streptococcus dysgalactiae keratitis with progression to corneal perforation and endophthalmitis, in the setting of epidermal growth factor receptor inhibitor (erlotinib) therapy for advanced non-small cell lung cancer. OBSERVATIONS: An 89-year-old female with non-small cell lung cancer on erlotinib presented with corneal perforation due to infectious keratitis. Microbial cultures grew streptococcus dysgalactiae, a virulent pathogen known to affect immunocompromised patients that has not been previously described to cause infectious keratitis. Despite aggressive medical intervention, the clinical course was complicated by rapid progression to no light perception visual acuity in the setting of endophthalmitis with orbital cellulitis, necessitating evisceration. CONCLUSIONS AND IMPORTANCE: Epidermal growth factor receptor inhibitor therapy can result in significant ocular complications including dry eyes, epithelial keratopathy, non-healing abrasions, infectious keratitis, and rarely, corneal melting and perforation. These side effects can predispose patients to aggressive infections with rare organisms, highlighting the importance of understanding the ocular side effects of systemic chemotherapeutic agents.

A Convergence of Ophthalmic and Life-threatening Emergencies: Acute Angle Closure Glaucoma and Subarachnoid Hemorrhage.

PURPOSE: To report a unique case of acute angle closure glaucoma in the setting of a subarachnoid hemorrhage due to a ruptured cerebral aneurysm. MATERIALS AND METHODS: Observational case report and review of the literature. RESULTS: A 75-year-old woman presented with blurry vision, nausea, vomiting, and left eye pain. She was found to have a complete third nerve palsy, with ptosis, exotropia, hypotropia, and a fixed mydriasis with resultant acute angle closure glaucoma. Pilocarpine was initiated, and neuroimaging revealed a subarachnoid hemorrhage from a ruptured posterior communicating artery aneurysm. The aneurysm was successfully coiled, and outpatient laser iridotomies were subsequently performed. Only 4 prior cases of acute angle closure glaucoma in the setting of a third nerve palsy have been reported in the literature. To our knowledge, this case is the first report of angle closure glaucoma in the setting of a subarachnoid hemorrhage. CONCLUSIONS: This case of a complete third nerve palsy in the setting of a subarachnoid hemorrhage leading to acute angle closure highlights the importance of ruling out this life-threatening diagnosis when neurological signs of increased intracranial pressure and cranial nerve palsies are present.

Associations with retinal vascular occlusions in a diverse, urban population.

PURPOSE: Retinal vascular occlusions can lead to sudden and permanent visual impairment or blindness. Few epidemiological studies on retinal vascular occlusions have been conducted, especially on diverse populations. METHODS: This is a retrospective case-control study of all incident retinal vascular occlusions occurring during a three and one-half year study period at Montefiore Medical Center, capturing all potential cases by diagnosis codes. Patients with retinal venous occlusions (RVO) and retinal arterial occlusions (RAO) were analyzed separately and compared to age-matched control groups. RESULTS: All potential charts (n = 700) were reviewed, confirming 214 RVO and 35 RAO incident cases. In multivariable analyses, RVO was associated with type 2 diabetes mellitus (OR 2.41, p < 0.001), history of cerebrovascular accident (OR 2.14, p = 0.011), hypertension (OR 1.83, p = 0.004), glaucoma (OR 6.91, p < 0.001), black race (OR 3.72, p < 0.001), and male gender (OR 2.19 p < 0.001). RAO was significantly associated with current and former smoking combined (OR 8.95, p = 0.021) and male gender (OR 2.56, p = 0.038). CONCLUSION: Cardiovascular risk factors and glaucoma are reaffirmed as significant predictors of retinal vascular occlusions in a diverse patient population. Retinal vascular occlusions are more common in certain races and ethnicities, and further study into this may help identify high-risk individuals based on demographics.

Strabismus After Ocular Surgery.

Many types of ocular surgery can cause diplopia, including eyelid, conjunctival, cataract, refractive, glaucoma, retinal, and orbital surgery. Mechanisms include direct injury to the extraocular muscles from surgery or anesthesia, scarring of the muscle complex and/or conjunctiva, alteration of the muscle pulley system, mass effects from implants, and muscle displacement. Diplopia can also result from a loss of fusion secondary to long-standing poor vision in one eye or from a decompensation of preexisting strabismus that was not recognized preoperatively. Treatment, which typically begins with prisms and is followed by surgery when necessary, can be challenging. In this review, the incidence, mechanisms, and treatments involved in diplopia after various ocular surgeries are discussed. [J Pediatr Ophthalmol Strabismus. 2017;54(5):272-281.].

Treatment and management of scleral disorders.

Scleral inflammatory disorders, with their extensive differential diagnoses and sometimes as controversial treatment options, pose both diagnostic and clinical challenges for physicians. Targeted laboratory investigations derived from careful history taking and physical examination are crucial in the early identification of patients with associated systemic conditions. Prompt discrimination between episcleritis and scleritis is of therapeutic importance, as management, prognosis, and complications differ for these diseases. Although immunomodulatory and new biologic agents have improved the management of scleritis, continued studies are warranted to establish effective treatment guidelines across patient populations, especially in refractory cases.

Case-Control Study of Herpes Simplex Eye Disease: Bronx Epidemiology of Human Immunodeficiency Virus Eye Studies.

PURPOSE: To determine whether human immunodeficiency virus and acquired immune deficiency syndrome (HIV/AIDS), diabetes mellitus, and atopic disease are associated with herpes simplex virus (HSV) eye disease and to examine the characteristics of patients with HIV infection and HSV eye disease. DESIGN: Retrospective case-control study. METHODS: A hospital-based control group was matched to outpatient visits from June 1, 2010, through May 31, 2014, at Montefiore Medical Center (Bronx, NY). Inclusion criteria were a diagnosis of HSV eye disease during the study period and residency in the Bronx. Associations evaluated included age, sex, HIV/AIDS, diabetes mellitus, and atopic disease. RESULTS: HSV eye disease was confirmed in 70 patients, who were compared with 280 controls. Patients with ocular HSV had a greater prevalence of HIV/AIDS compared with controls (8.6% and 2.9%, respectively). Using multivariate analysis to control for age and sex, atopic dermatitis [odds ratio (OR) 3.08, 95% confidence interval (CI), 0.84-11.20] and diabetes with chronic complications (OR 2.25, 95% CI, 0.91-5.61) approached significance, whereas HIV/AIDS (OR 3.37, 95% CI, 1.09-10.40), an age less than 45 years (OR 2.89, 95% CI, 1.54-5.41), and male sex (OR 1.85, 95% CI, 1.07-3.18) were significant. In patients with HIV infection and HSV eye disease, 3 of 6 (50%) had confirmed AIDS at the time of ocular HSV diagnosis. CONCLUSIONS: Patients with HIV infection have more than a 3-fold increased risk of developing ocular HSV.