Hypervirulent Klebsiella pneumoniae infection in a woman with a CDH1 gene mutation.

The CDH1 gene, which encodes E-cadherin, may be associated with cancer when mutated, but the significance of mutations in the context of infection is unknown. In this report, we describe a case of disseminated hypervirulent Klebsiella pneumoniae infection in a 49 year old Caucasian woman with a documented CDH1 mutation.

Choroidal nonperfusion on optical coherence tomography angiography in a case of unilateral posterior segment ocular sarcoidosis misdiagnosed as MEWDS.

PURPOSE: To report a case of presumed ocular sarcoidosis initially presenting with features of multiple evanescent white dot syndrome (MEWDS) with atypical optical coherence tomography angiography (OCTA) findings. OBSERVATIONS: A 23 year-old woman presented with a unilateral central scotoma, photophobia, and decreased visual acuity after a viral illness. Examination of the right eye revealed multiple round white macular spots and stippled granularity at the fovea. Multimodal imaging with fluorescein angiography (FA), indocyanine green angiography (ICG), fundus autofluorescence (FAF), and optical coherence tomography (OCT) was consistent with a diagnosis of MEWDS. However, OCTA demonstrated choriocapillaris (CC) flow deficits, which is not typical for MEWDS. The clinical course was initially consistent with MEWDS, with spontaneous recovery of symptoms over ensuing months. The patient presented five months later with floaters and a central scotoma. Examination showed panuveitis, and systemic evaluation revealed an elevated angiotensin converting enzyme (ACE) and hilar lymphadenopathy on chest x-ray consistent with presumed sarcoidosis. CONCLUSIONS AND IMPORTANCE: A case of MEWDS atypically demonstrated CC flow deficits on OCTA and subsequently presented as uveitis secondary to presumed sarcoidosis. Atypical features in MEWDS may be a sign of another disorder masquerading early on as MEWDS and ought to prompt further investigation.

Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis Syndrome Presenting With Branch Retinal Artery Occlusion.

Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) is a rare syndrome affecting the retinal and optic disc vasculature. Diffuse retinal ischemia, macular edema, and neovascularization may lead to bilateral vision loss. The authors report a case of a 36-year-old woman presenting with branch retinal artery occlusion (BRAO) in her right eye who was subsequently diagnosed with IRVAN syndrome. She was treated with panretinal photocoagulation for peripheral retinal ischemia and pars plana vitrectomy for vitreous hemorrhage. She later developed a BRAO in her left eye. This case demonstrates that BRAO may be a presenting feature of IRVAN syndrome. [Ophthalmic Surg Lasers Imaging Retina. 2017;48:948-951.].

Distinguishing Diabetic Macular Edema From Capillary Nonperfusion Using Optical Coherence Tomography Angiography.

BACKGROUND AND OBJECTIVE: To describe the appearance of diabetic macular edema (DME) using optical coherence tomography angiography (OCTA) and distinguish it from capillary nonperfusion. PATIENTS AND METHODS: Patients with DME were recruited for OCTA imaging. Eyes with confounding retinal diseases were excluded. Using 3 mm × 3 mm OCT angiograms segmented into the superficial and deep inner retinal vascular plexuses, two graders described the appearance of DME and confirmed the diagnosis with structural OCT and fluorescein angiography. RESULTS: DME was evaluated in 17 eyes of 12 patients. The cystoid spaces in DME appeared completely devoid of flow on the OCT angiograms and were oblong in shape with smooth borders that did not follow the distribution of surrounding capillaries, whereas areas of capillary nonperfusion were a greyer hue and had irregular borders. CONCLUSIONS: The cystoid spaces in DME can be differentiated from capillary nonperfusion using OCTA. OCTA may help to guide treatment decisions in the future.

CHARACTERIZING THE EFFECT OF ANTI-VASCULAR ENDOTHELIAL GROWTH FACTOR THERAPY ON TREATMENT-NAIVE CHOROIDAL NEOVASCULARIZATION USING OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY.

PURPOSE: To use optical coherence tomography angiography (OCTA) to characterize the effects of anti-VEGF injections on treatment-naive choroidal neovascularization (CNV). METHODS: From August 2014 to May 2015, treatment-naive eyes with CNV were scanned using a prototype OCTA system on a commercially available SD-OCT device (Optovue Inc, Fremont, CA). Optical coherence tomography angiography scans were obtained before anti-VEGF injection and at follow-up visits. The CNV area and greatest linear dimension (GLD) were measured along with the maximum retinal pigment epithelial detachment (RPED) height. Changes in subretinal and/or intraretinal fluid were also assessed. RESULTS: Six eyes of six patients with treatment-naive CNV were included. Diagnoses included neovascular age-related macular degeneration, idiopathic polypoidal choroidal vasculopathy, CNV secondary to central serous chorioretinopathy and multifocal choroiditis, and macular telangiectasia Type 2 with subretinal neovascularization. After treatment, all patients with fluid on OCT initially showed a decrease in the amount of fluid. Five of six patients demonstrated decreases in CNV GLD and area with an average reduction of 23.6% and 29.8% respectively. CONCLUSION: Both CNV greatest linear dimension and area measured using OCTA decreased after anti-VEGF treatment in most patients. Optical coherence tomography angiography may be a useful tool for monitoring and quantifying the response of CNV to treatment.

Outer Retinal Changes Preceding Secondary Macular Hole Formation Years After Vitreomacular Traction Release Demonstrated on Spectral-Domain OCT.

This report describes changes documented on spectral-domain optical coherence tomography (SD-OCT) in the right eye of a 58-year-old woman who eventually developed a secondary full-thickness macular hole (FTMH). She had a history of vitreomacular traction (VMT) that had resolved spontaneously with a complete posterior vitreous detachment. Despite VMT resolution, a hyperreflective band from the internal to the external limiting membrane and evidence of photoreceptor disruption persisted on SD-OCT for 2 years, at which point an FTMH developed. Despite VMT release, the unexpected FTMH coupled with the preceding outer retinal changes suggest persistent secondary damage to Müller cells as a potential factor.

DETECTION OF MICROVASCULAR CHANGES IN EYES OF PATIENTS WITH DIABETES BUT NOT CLINICAL DIABETIC RETINOPATHY USING OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY.

PURPOSE: To evaluate the ability of optical coherence tomography angiography to detect early microvascular changes in eyes of diabetic individuals without clinical retinopathy. METHODS: Prospective observational study of 61 eyes of 39 patients with diabetes mellitus and 28 control eyes of 22 age-matched healthy subjects that received imaging using optical coherence tomography angiography between August 2014 and March 2015. Eyes with concomitant retinal, optic nerve, and vitreoretinal interface diseases and/or poor-quality images were excluded. Foveal avascular zone size and irregularity, vessel beading and tortuosity, capillary nonperfusion, and microaneurysm were evaluated. RESULTS: Foveal avascular zone size measured 0.348 mm² (0.1085-0.671) in diabetic eyes and 0.288 mm² (0.07-0.434) in control eyes (P = 0.04). Foveal avascular zone remodeling was seen more often in diabetic than control eyes (36% and 11%, respectively; P = 0.01). Capillary nonperfusion was noted in 21% of diabetic eyes and 4% of control eyes (P = 0.03). Microaneurysms and venous beading were noted in less than 10% of both diabetic and control eyes. Both diabetic and healthy control eyes demonstrated tortuous vessels in 21% and 25% of eyes, respectively. CONCLUSION: Optical coherence tomography angiography was able to image foveal microvascular changes that were not detected by clinical examination in diabetic eyes. Changes to the foveal avascular zone and capillary nonperfusion were more prevalent in diabetic eyes, whereas vessel tortuosity was observed with a similar frequency in normal and diabetic eyes. Optical coherence tomography angiography may be able to detect diabetic eyes at risk of developing retinopathy and to screen for diabetes quickly and noninvasively before the systemic diagnosis is made.

Spectral-domain optical coherence tomography angiography of choroidal neovascularization.

PURPOSE: To describe the characteristics as well as the sensitivity and specificity of detection of choroidal neovascularization (CNV) on optical coherence tomography angiography (OCTA) using spectral-domain optical coherence tomography. DESIGN: Observational, retrospective study. PARTICIPANTS: Seventy-two eyes of 61 subjects (48 eyes of 43 subjects with CNV, 24 eyes of 18 subjects without CNV). METHODS: Patients imaged using the prototype AngioVue OCTA system (Optovue, Inc, Fremont, CA) between August 2014 and October 2014 at New England Eye Center were assessed. Patients in whom CNV was identified on OCTA were evaluated to define characteristics of CNV on OCTA: size using greatest linear dimension (small, <1 mm; medium, 1-2 mm; large, >2 mm), appearance (well-circumscribed, poorly circumscribed), and presence of subretinal and intraretinal fluid. Concurrently, an overlapping second cohort of patients who underwent same-day OCTA and fluorescein angiography (FA) for suspected CNV was evaluated to estimate sensitivity and specificity of OCTA in detecting CNV using FA as ground truth. MAIN OUTCOME MEASURES: Choroidal neovascularization appearance, CNV size, and presence of subretinal and intraretinal fluid. RESULTS: In 48 eyes, CNV was visualized on OCTA. Thirty-one eyes had CNV associated with neovascular age-related macular degeneration. Size of CNV was small in 23% (7/31), medium in 42% (13/31), and large in 35% (11/31). Poorly circumscribed vessels, subretinal fluid, and intraretinal fluid each were seen in 71% (22/31). Seven eyes had CNV associated with central serous chorioretinopathy. Size of CNV was small in 71% (5/7) and large in 29% (2/7). Seventy-one percent (5/7) had well-circumscribed vessels, 86% (6/7) had subretinal fluid, and 14% (1/7) had intraretinal fluid. Thirty eyes with OCTA and same-day FA were evaluated to determine sensitivity and specificity of CNV detection on OCTA. Sensitivity was 50% (4/8) and specificity was 91% (20/22). CONCLUSIONS: Using OCTA allows the clinician to visualize CNV noninvasively and may provide a method for identifying and guiding treatment of CNV. The specificity of CNV detection on OCTA compared with FA seems to be high. Future studies with larger sample sizes are needed to elaborate better on the sensitivity and specificity of CNV detection and to illustrate clinical usefulness.