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Purpose To determine whether ultra-widefield (UWF) retinal imaging changes the staging or management of sickle cell retinopathy compared with clinical examination. Methods Prospective, observational study including patients with sickle cell disease. All patients underwent dilated fundus examination by a fellowship-trained retina specialist, as well as UWF fundus photography (FF) and fluorescein angiography (FA). Sickle retinopathy stage and treatment recommendation per eye were determined after clinical examination, UWF-FF, and UWF-FA, respectively, and differences in retinopathy stage and treatment recommendation were compared. Results A total of 70 eyes from 35 patients (17 women, 48.6%), mean age 30.4 years, were included. Sickle genotypes included 26 patients with sickle SS (74.3%), 7 SC (20.0%), and 2 β(+)thalassemia (5.7%). Based on examination, most eyes (42/70; 60.0%) had no visible retinopathy. Based on UWF-FF, about half of the eyes were found to be Goldberg Stage 2 or above (36/70; 51.4%). Based on UWF-FA, nearly all eyes were Goldberg Stage 2 or above (63/70; 90%). However, clinical examination reliably detected neovascularization, and in no case did the addition of UWF imaging change management relative to examination alone. Conclusion Ultra-widefield imaging detects a higher stage of sickle cell retinopathy compared with clinical examination alone, but these differences may not be clinically significant.

BACKGROUND: Diabetic retinopathy (DR) is a serious complication that can progress to sight-threatening disease. The prevalence of DR in youth with diabetes has been reported to be 3.8% to 20%. OBJECTIVE: We aimed to evaluate the prevalence of DR among youth with diabetes at a large ophthalmologic referral center. Secondary goals were to determine the risk factors for DR and severity of disease. METHODS: Retrospective chart review of 343 patients with diabetes, 8% had a higher risk of DR (P = .049). In this cohort, none of the patients with an HbA1c

Purpose Retinal hemorrhages are observed frequently in patients with leukemia. However, little is known about the impact and natural history of these hemorrhages. The purpose of this study was to describe leukemic retinal hemorrhages using multimodal imaging and to monitor their evolution longitudinally. Design Retrospective case series. Participants A total of 11 eyes of 7 symptomatic leukemic patients with posterior segment hemorrhages. Methods Single-center study performed at the Johns Hopkins Hospital. Symptomatic leukemic patients with posterior segment hemorrhages underwent serial dilated fundus examinations. The hemorrhages were documented longitudinally with color fundus photographs and spectral-domain (SD) OCT. Main Outcomes Measures Microanatomic locations of leukemic retinal hemorrhages and their impact on vision and evolution over time. Results A total of 7 patients (71.4% men; 57.1% white, 28.6% black, and 14.3% Hispanic) were included, with 11 eyes showing posterior segment hemorrhages. The median age at presentation was 49.8 years. All patients had intraretinal hemorrhages; these involved the vitreous and sub–internal limiting membrane (ILM) space in 1 and 3 patients, respectively. The median total follow-up duration was 4.0 months. At the final follow-up visits, 4 of 6 patients showed complete resolution of hemorrhages on examination and color fundus photographs. The final SD-OCT images of all patients did not show any retinal thinning, disruption of the ellipsoid zone, disorganization of the retinal layers, intraretinal fluid, or subretinal fluid. Conclusions Symptomatic leukemic retinal hemorrhages are associated with anemia and thrombocytopenia. These hemorrhages, including visually significant central sub-ILM hemorrhages, tend to be self-limiting and resolve within a few months with treatment of the underlying disease.

We report a case of peripheral retinal neovascularization and vitreous hemorrhage in the setting of HIV retinopathy that can serve to extend the clinical spectrum of this condition. A 53-year-old African-American woman with AIDS was referred for decreased vision in the left eye and was found to have peripheral retinal neovascularization and vitreous hemorrhage. She had a workup that was negative for etiologies of retinal ischemia. Peripheral laser photocoagulation was used to treat areas of nonperfusion. To our knowledge, this is the first reported case of peripheral retinal neovascularization and vitreous hemorrhage in the setting of HIV retinopathy, and it can serve to extend the clinical spectrum of this condition.

A 67-year-old man developed jaw claudication followed by loss of vision in the left eye caused by anterior ischemic optic neuropathy (AION). An erythrocyte sedimentation rate was normal, but C-reactive protein was slightly elevated. Although the patient had no evidence of a right optic neuropathy, magnetic resonance imaging (MRI) revealed bilateral optic nerve sheath enhancement. A temporal artery biopsy was consistent with active giant cell arteritis (GCA). Our case demonstrates that bilateral optic nerve sheath enhancement on MRI can be seen in the setting of unilateral AION. This unique combination of clinical and imaging findings has not been reported previously and extends the clinical spectrum of presentation of GCA.

To determine whether the degree of peripheral nonperfusion seen on ultra-widefield (UWF) fluorescein angiography (FA) correlates with measures of macular vascular flow as seen on OCT angiography (OCTA) in sickle cell retinopathy.Prospective, observational study.Patients with sickle cell disease undergoing an eye examination at an urban, tertiary medical center.All patients underwent dilated fundus examination as well as UWF FA and macular OCTA imaging on the same day. The peripheral nonperfusion seen on UWF FA was measured to calculate an ischemic index (visualized nonperfusion/total visualized retinal area × 100%), and OCTA measurements of macular vessel density were recorded. The degree of peripheral nonperfusion and vessel density were then correlated.Correlation between ischemic index as seen on UWF FA and macular vessel density on OCTA.Thirty-six eyes from 19 patients (10 women, 9 men) with a mean age of 30.8 years were included. Sickle genotypes included 14 patients with SS (73.7%), 4 with SC (21.1%), and 1 with β-thalassemia (5.2%). Average ischemic index was 4.4% for all eyes and was found to be higher in patients with sickle SC (8.0%) than in those with sickle SS (3.2%; P = 0.01). Ischemic index also was higher in those with proliferative sickle cell retinopathy (9.3%) than in those without (2.8%; P0.01). Ischemic index on UWF FA showed a statistically significant correlation (P0.05) with vessel density on OCTA in the temporal subfield of the superficial capillary plexus and in all subfields of the deep capillary plexus.Peripheral nonperfusion seen on UWF FA is greater in those with sickle SC disease and proliferative retinopathy and is correlated with macular vessel density on OCTA, especially the deep retinal plexus.

Importance Incontinentia pigmenti (IP) is a rare, X-linked dominant disease with potentially severe ocular complications that predominantly affect the peripheral retina. However, little is known about its effects on the macula. Objective To describe the structural and vascular abnormalities observed in the maculas of patients with IP and to correlate these findings with peripheral pathologies. Design, Setting, and Participants Prospective, cross-sectional study at Wilmer Eye Institute, Johns Hopkins University. Five participants with a clinical diagnosis of IP were included and underwent multimodal imaging with ultra–wide-field fluorescein angiography (FA), spectral-domain optical coherence tomography (OCT), and OCT angiography. Main Outcomes and Measures The structural and vascular abnormalities observed on spectral-domain OCT and OCT angiography and their correlation with peripheral pathologies seen on ultra–wide-field FA. Results A total of 9 eyes from 5 patients (median age, 20.5 years; range, 8.4-54.2 years) were included. Median Snellen visual acuity was 20/32 (range, 20/16 to 20/63). ultra–wide-field FA-identified retinal vascular abnormalities in all 7 eyes in which FA was obtained. These abnormalities included microaneurysms, areas of nonperfusion, and vascular anastomoses, most of which were peripheral to the standard view of 30° FA with peripheral sweeps. Structural abnormalities were observed in 6 eyes on spectral-domain OCT, including inner retinal thinning and irregularities in the outer plexiform layer. Optical coherence tomography angiography abnormalities were noted in all 9 eyes, including decreased vascular density, abnormal vascular loops, and flow loss in the superficial and deep plexuses, which corresponded to areas of retinal thinning on spectral-domain OCT. Conclusions and Relevance Although our study is limited by the small sample size, the findings suggest that multimodal imaging is useful for detecting structural and vascular abnormalities that may not be apparent on ophthalmoscopy in patients with IP. Macular pathologies, especially a decrease in vascular density on OCT angiography, are common. Further studies are needed to characterize further the association between macular and peripheral abnormalities in patients with IP.

Purpose Primary central nervous system lymphoma (PCNSL) is a rare disease. The index report describes a patient with intraocular lymphoma secondary to recurrent PCNSL and corresponding retinal findings on spectral domain optical coherence tomography (SD-OCT). Methods Case report. Results OCT changes were documented and correlated with the clinical course of intraocular lymphoma progression in the index patient. The OCT changes, manifested as hyperreflective material accumulation in the intraretinal and subretinal pigment epithelial spaces, were caused by lymphomatous infiltration. Conclusion SD-OCT can be useful in diagnosing and monitoring the progression or regression of intraocular lymphoma with retinal involvement.