Accuracy of Repetitive Ocular Vestibular-Evoked Myogenic Potentials to Diagnose Myasthenia Gravis in Patients With Ptosis or Diplopia.

BACKGROUND AND OBJECTIVES: We developed repetitive ocular vestibular-evoked myogenic potentials (roVEMP) as an electrophysiologic test that allows us to elicit the characteristic decrement of extraocular muscles in patients with ocular myasthenia gravis (OMG). Case-control studies demonstrated that roVEMP reliably differentiates patients with OMG from healthy controls. We now aimed to evaluate the diagnostic accuracy of roVEMP for OMG diagnosis in patients with ptosis and/or diplopia. METHODS: In this blinded prospective diagnostic accuracy trial, we compared roVEMP in 89 consecutive patients presenting with ptosis and/or diplopia suspicious of OMG with a multimodal diagnostic approach, including clinical examination, antibodies, edrophonium testing, repetitive nerve stimulation of accessory and facial nerves, and single-fiber EMG (SFEMG). We calculated the roVEMP decrement as the ratio between the mean of the first 2 responses compared with the mean of the sixth-ninth responses in the train and used cutoff of >9% (unilateral decrement) in a 30 Hz stimulation paradigm. RESULTS: Following a complete diagnostic work-up, 39 patients (44%) were diagnosed with ocular MG, while 50 patients (56%) had various other neuro-ophthalmologic conditions, but not MG (non-MG). roVEMP yielded 88.2% sensitivity, 30.2% specificity, 50% positive predictive value (PPV), and 76.5% negative predictive value (NPV). For comparison, SFEMG resulted in 75% sensitivity, 56% specificity, 55.1% PPV, and 75.7% NPV. All other diagnostic tests (except for the ice pack test) also yielded significantly higher positive results in patients with MG compared with non-MG. DISCUSSION: The study revealed a high sensitivity of 88.2% for roVEMP in OMG, but specificity and PPV were too low to allow for the OMG diagnosis as a single test. Thus, differentiating ocular MG from other neuro-ophthalmologic conditions remains challenging, and the highest diagnostic accuracy is still obtained by a multimodal approach. In this study, roVEMP can complement the diagnostic armamentarium for the diagnosis of MG. CLASSIFICATION OF EVIDENCE: This study provides Class I evidence that in patients with diplopia and ptosis, roVEMP alone does not accurately distinguish MG from non-MG disorders. TRIAL REGISTRATION INFORMATION: ClinicalTrials.gov: NCT03049956.

Long-term outcomes of fornix reconstruction and cicatricial entropion repair in ocular mucous membrane pemphigoid and drug-related secondary pemphigoid.

OBJECTIVE: To investigate the long-term outcomes of fornix reconstruction and cicatricial entropion repair in patients with ocular mucous membrane pemphigoid (MMP) and secondary MMP. METHODS: Retrospective chart review of patients with MMP undergoing either fornix reconstruction (with amniotic membrane or buccal mucosal graft) or Wies cicatricial entropion repair between January 1, 2000, and September 1, 2020. Patients had a positive mucosal biopsy and (or) clinical features of MMP or secondary MMP. The primary outcome was overall success of fornix reconstruction based on fornix depth maintenance at latest follow-up. Secondary outcomes included resolution of trichiasis, visual acuity, and improvement of subjective symptoms. RESULTS: Eight patients (10 eyes) with a diagnosis of MMP (3 males and 5 females; median age, 71 years) and 4 patients (4 eyes) with a diagnosis of secondary MMP (2 females and 2 male; median age, 87 years) were enrolled. Mean follow-up was 22.7 months (range, 0.3-87.5 months) for MMP patients and 15.4 months (range, 3.0-43.9 months) for secondary MMP patients. For MMP eyes, 30.0% underwent fornix reconstruction, 60.0% underwent entropion repair, and 10.0% received both. Re-formation of symblepharon and loss of fornix depth occurred in all MMP eyes at an average of 6.4 ± 7.0 months postoperatively, and trichiasis recurred in all patients at the last follow-up visit. In secondary MMP patients, 75.0% of the eyes showed recurrence of symblepharon, and 66.7% re-formed trichiasis. Both MMP and secondary MMP patients had short-term symptom improvements. CONCLUSIONS: Fornix reconstruction and cicatricial entropion repair in our cohort of MMP and secondary MMP patients resulted in short-term symptomatic improvement, but recurrence was seen, on average, at 6 months postoperatively.

Metastatic neuroendocrine tumor masquerading as orbital cysticercosis.

Orbital metastases secondary to neuroendocrine tumors are exceedingly rare. We present a unique case of a 30-year-old female initially presenting with fever, chills, periorbital swelling, and painful proptosis. CT orbits revealed two ovoid-shaped ring-enhancing lesions in the right lateral and superior rectus muscles and clear sinuses, atypical for infectious post-septal cellulitis. Further work-up included serologic analysis, auto-immune panel, and MRI. Further imaging showed pseudocystic orbital lesions mimicking orbital cysticercosis. Additionally, given the bilateral nature of the lesions and patient's country of origin, this parasitic process was highly suspected. A course of albendazole and steroids led to resolution of symptoms. With a presentation at age 30, this is by far the youngest case reported in literature to date.

Quantitative Optical Coherence Tomography Angiography in Patients with Moyamoya Vasculopathy: A Pilot Study.

Moyamoya (MM) disease is a chronic cerebrovascular disease that can lead to progressive stenosis of the terminal portions of the internal carotid arteries and their proximal branches. We sought to investigate and quantify retinal vascular changes in patients with MM vasculopathy (MMV) using optical coherence tomography angiography (OCTA) compared to healthy controls. Our findings reveal retinal microvascular changes in patients with MMV and highlights the potential of OCTA imaging for the detection of subclinical retinal pathology.

INVESTIGATING MICROANGIOPATHY USING SWEPT-SOURCE OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY IN PATIENTS WITH SUSAC SYNDROME.

PURPOSE: To determine whether optical coherence tomography angiography is of diagnostic utility for Susac syndrome (SuS) by quantifying microvascular retinal changes. METHODS: We enrolled 18 eyes of 9 healthy controls and 18 eyes of 9 patients with chronic SuS (12 had previous branch retinal artery occlusions and 6 were clinically unaffected). Images of the fovea were taken using an optical coherence tomography angiography system. Analysis included vessel density, fractal dimension, vessel diameter, and measurements of the foveal avascular zone (area, eccentricity, acircularity index, and axis ratio) in deep and superficial retinal layers. RESULTS: Skeleton density and inner ring vessel density were significantly lower in patients with SuS (skeleton density: Susac 0.11 ± 0.01 vs. controls 0.12 ± 0.01, P = 0.027. VD: SuS 0.39 ± 0.04 vs. controls 0.42 ± 0.02, P = 0.041). Eccentricity and axis ratio were significantly higher in patients with SuS (EC: Susac 0.61 ± 0.11, controls 0.51 ± 0.10, P = 0.003; axis ratio: Susac 1.57 ± 0.28, controls 1.39 ± 0.11, P = 0.005). SuS eyes (affected and unaffected) had poorer outcomes of the remaining vascular parameters compared with controls (P > 0.05). CONCLUSION: Optical coherence tomography angiography identified chronic microvascular changes in the eyes of patients with chronic SuS. Even clinically unaffected SuS eyes showed poorer vascular parameters. Although further research is needed, this noninvasive imaging modality seems to have the potential to serve as a valuable additive diagnostic tool.

Surgical Technique to Retrieve a Migrated Lester Jones Lacrimal Tube.

PURPOSE: To describe a technique for retrieving an inferomedially migrated Jones lacrimal tube. METHODS: The distal end of the migrated Jones tube is visualized directly via the nose. The tip of an endoilluminator (23 Gauge Constellation Vision Systems) is bent to form a 45° to 60° angle using a mosquito forceps and inserted into the distal opening of the tube. The endoilluminator is used to push the tube upward toward the original medial canthal opening and limit downward displacement as the caruncular area is surgically explored. The light transilluminating the tube facilitates its visualization and retrieval in the caruncular area. A new tube may be threaded through the same passage, or if the ostium is traumatized, through a new passage. RESULTS: This technique is time-efficient and has proven to be safe and successful in subsequent patients. CONCLUSIONS: The described technique aids in retrieving an inferomedially migrated Jones tube with minimal trauma to the surrounding structures.

Diagnosing Myasthenia Gravis With Repetitive Ocular Vestibular Evoked Myogenic Potentials.

Timely and accurate diagnosis of myasthenia gravis, particularly in patients with fluctuating, isolated ocular involvement, remains challenging. Serological antibody testing and repetitive nerve stimulation of peripheral muscles usually have low sensitivity in these patients. Edrophonium testing may cause adverse events, single-fiber electromyography (SFEMG) is time-consuming and both tests are often unavailable outside specialized institutions. Repetitive ocular vestibular evoked myogenic potential (roVEMP) stimulation has recently been introduced to facilitate the diagnosis of myasthenia gravis. Similar to repetitive nerve stimulation, roVEMPs detect muscle decrements with the benefit of being non-invasive and allowing for direct measurement of the extraocular muscles. This review summarizes the clinical evidence of the diagnostic value of roVEMP for myasthenia. Prospective clinical trials have demonstrated high sensitivity and specificity. RoVEMPs are of particular interest in challenging myasthenia subgroups with isolated ocular involvement, negative serology, and/or negative conventional electrophysiological results. Optimal roVEMP repetition rates of 20-30 Hz have been identified. This promising novel diagnostic tool merits further attention and investigation to establish its value as a clinical test for myasthenia.

Repetitive ocular vestibular evoked myogenic potentials in myasthenia gravis.

OBJECTIVE: To validate the repetitive ocular vestibular evoked myogenic potentials (RoVEMP) test for diagnostic use in myasthenia gravis (MG) and to investigate its value in diagnostically challenging subgroups. METHODS: The RoVEMP test was performed in 92 patients with MG, 22 healthy controls, 33 patients with a neuromuscular disease other than MG (neuromuscular controls), 4 patients with Lambert-Eaton myasthenic syndrome, and 2 patients with congenital myasthenic syndrome. RESULTS: Mean decrement was significantly higher in patients with MG (28.4% ± 32.2) than in healthy controls (3.2% ± 13.9; p < 0.001) or neuromuscular controls (3.8% ± 26.9; p < 0.001). With neuromuscular controls as reference, a cutoff of ≥14.3% resulted in a sensitivity of 67% and a specificity of 82%. The sensitivity of the RoVEMP test was 80% in ocular MG and 63% in generalized MG. The RoVEMP test was positive in 6 of 7 patients with seronegative MG (SNMG) with isolated ocular weakness. Of 10 patients with SNMG with negative repetitive nerve stimulation (RNS) results, 73% had an abnormal RoVEMP test. The magnitude of decrement was correlated with the time since the last intake of pyridostigmine (B = 5.40; p = 0.019). CONCLUSIONS: The RoVEMP test is a new neurophysiologic test that, in contrast to RNS and single-fiber EMG, is able to measure neuromuscular transmission of extraocular muscles, which are the most affected muscles in MG. Especially in diagnostically challenging patients with negative antibody tests, negative RNS results, and isolated ocular muscle weakness, the RoVEMP test has a clear added value in supporting the diagnosis of MG. CLASSIFICATION OF EVIDENCE: This study provides Class III evidence that RoVEMP distinguishes MG from other neuromuscular diseases.

Repetitive ocular vestibular evoked myogenic potential stimulation for the diagnosis of myasthenia gravis: Optimization of stimulation parameters.

OBJECTIVE: To determine the most effective stimulation parameters for the diagnosis of ocular myasthenia gravis (MG) using repetitive ocular vestibular evoked myogenic potentials (oVEMP) for quantification of the extraocular muscle response decrement. METHODS: Repetitive bone-conducted oVEMPs were elicited in 18 MG patients and 20 healthy subjects. We compared four different stimulus repetition rates (20 Hz, 30 Hz, 40 Hz, 50 Hz) and 100 Hz continuous stimulation, as well as recordings from the inferior oblique muscles and the lateral rectus muscles to determine the most sensitive and specific oVEMP parameters for decrement detection. RESULTS: Repetitive stimulation at all tested repetition rates with recordings from inferior oblique muscles allowed for effective differentiation between MG patients and healthy subjects. Among all repetition rates, 30 Hz showed a trend towards superiority, with a sensitivity of 71% and a specificity of 94% (area under the curve (AUC) 0.88) when using the smaller decrement of the two eyes and -10% as cutoff. Considering the larger decrement for analysis (-9% as cutoff), sensitivity increased to 82%, but specificity decreased to 78% (AUC 0.81). CONCLUSIONS: Our study demonstrates, that repetitive oVEMP stimulation elicits a robust decrement in the inferior oblique muscles of MG patients at repetition rates between 20 Hz and 50 Hz, with a probable optimum at 30 Hz. SIGNIFICANCE: Repetitive inferior oblique oVEMP stimulation with optimal stimulus parameters facilitates early and accurate diagnosis of ocular MG.

IMPACT OF LONG-TERM INTRAVITREAL ANTI-VASCULAR ENDOTHELIAL GROWTH FACTOR ON PREEXISTING MICROSTRUCTURAL ALTERATIONS IN DIABETIC MACULAR EDEMA.

PURPOSE: Evaluation of the influence of long-term intravitreal anti-vascular endothelial growth factor treatment on preexisting retinal microstructural alterations in patients with diabetic macular edema. METHODS: Eyes with diabetic macular edema and a history of ≥ 20 intravitreal anti-vascular endothelial growth factor (aflibercept and/or ranibizumab) injections were included in this retrospective study. Primary outcome was the extent of disorganization of retinal inner layers, alterations at the outer plexiform layer/Henle fiber layer junction, disruption of external limiting membrane/ellipsoid zone, disruption of retinal pigment epithelium/Bruch complex, and retinal atrophy at baseline versus after ≥ 20 intravitreal injections as visualized by spectral-domain optical coherence tomography images. RESULTS: Of 383 eyes screened, 37 eyes were included in the current study. With the exception of outer plexiform layer/Henle fiber layer junction restoration, no significant changes regarding microstructural alterations between baseline and end of study were encountered after long-term anti-vascular endothelial growth factor (disorganization of retinal inner layers P = 0.381, outer plexiform layer/Henle fiber layer junction P = 0.001, external limiting membrane/ellipsoid zone P = 0.524, retinal pigment epithelium/Bruch complex P = 0.122, retinal atrophy P = 0.317). Best-corrected visual acuity significantly increased over the course of the study, corresponding to central retinal thickness and intraretinal fluid reduction (all P < 0.0001). The extent of microstructural alterations was negatively correlated with best-corrected visual acuity (P < 0.05). CONCLUSION: Apart from outer plexiform layer/Henle fiber layer junction layer restoration, no effect on preexisting retinal alterations was encountered after long-term intravitreal injections. Thus, intravitreal ranibizumab or aflibercept did not have a major effect (neither positive nor negative) on microstructural alterations.

Angio-OCT de la zona avascular foveal en ojos con oclusión venosa de la retina.

Objetivo: El objetivo del estudio comprendía visualizar y cuantificar las alteraciones patológicas de la zona avascular foveal (ZAF) mediante angio-OCT en ojos con oclusión venosa de la retina (OVR) en comparación con el ojo contralateral sano. Procedimientos: La angio-OCT se llevó a cabo mediante el sistema Avanti® RTVue 100 XR (Optovue Inc., Fremont, Calif., EE. UU.). Los bordes de la capa vascular superficial (CVS) se definieron como 3 µm por debajo de la membrana limitante interna y 15 µm por debajo de la capa plexiforme interna y, para la capa vascular profunda (CVP), como 15 y 70 µm por debajo de la membrana limitante interna y de la capa plexiforme interna, respectivamente. La longitud de la ZAF horizontal, vertical y máxima de la CVS y la CVP en cada ojo se midió de forma manual. Además, se midió el ángulo entre el diámetro máximo de la ZAF y el plano papilomacular. Resultados: La angio-OCT representó los defectos dentro de la vasculatura en el área perifoveal en ojos con oclusión de rama venosa de la retina (ORVR; n = 11) y con oclusión de la vena central de la retina (OVCR; n = 8). Esto resultó en un crecimiento del diámetro máximo de la ZAF en ojos con OVR (n = 19) en comparación con el ojo contralateral (n = 19; 921 ± 213 frente a 724 ± 145 µm; p = 0,008). Además, se observó una correlación significativa entre la mejor agudeza visual corregida (MAVC) y el diámetro máximo de la ZAF en la CVP (ρ de Spearman = -0,423, p < 0,01). Por último, en los ojos con OVR, el ángulo entre el plano papilomacular y el diámetro máximo de la ZAF se dio tan solo en el 21,05% (CVS) y en el 15,79% (CVP) de los casos a 0 ± 15 ó 90 ± 15°, respectivamente. En ojos sanos, estos ángulos (que supuestamente representan una configuración de la ZAF regular) fueron más prevalentes (CVS 68,42 frente a 21,05%, p = 0,003; CVP 73,68 frente a 15,79%, p < 0,001). Conclusiones: La angio-OCT muestra alteraciones morfológicas de la ZAF en ojos con OVCR y ORVR. La correlación del diámetro máximo de la ZAF con la MAVC indica que estas alteraciones resultan funcionalmente relevantes.

Comparison of Progression Rate of Retinal Pigment Epithelium Loss in Patients with Neovascular Age-Related Macular Degeneration Treated with Ranibizumab and Aflibercept.

Purpose. Retinal pigment epithelium (RPE) loss in neovascular age-related macular degeneration (nAMD) seem to have a linear progression but might be influenced by the treatment. The purpose of the study is the comparison of RPE loss over three years in patients treated with intravitreal ranibizumab to patients who were switched to aflibercept. Methods. A retrospective analysis with 96 eyes switched to aflibercept was conducted. The progression rate of RPE loss was evaluated in patients who showed atrophy one year prior to switch (n = 17) or on switch date (n = 19). The RPE loss was evaluated by spectral domain optical coherence tomography (SD-OCT). Further, 22 eyes from patients treated with ranibizumab were compared. Results. The median yearly progression of RPE loss after square root transformation showed no significant difference in the year prior to switch compared to the year after switch (p = 0.854). In patients who received only ranibizumab, the median yearly progression of RPE loss was 0.15 mm/y, for aflibercept patients, 0.13 mm/y. This difference was not statistically significant (p = 0.172). Conclusions. There seems to be a linear progression rate of RPE loss in patients treated with ranibizumab as well as in patients with aflibercept. No significant increase of progression rate was found after switch to aflibercept.

LOW ENDOPHTHALMITIS RATES AFTER INTRAVITREAL ANTI-VASCULAR ENDOTHELIAL GROWTH FACTOR INJECTIONS IN AN OPERATION ROOM: A Retrospective Multicenter Study.

PURPOSE: To evaluate the rate of presumed endophthalmitis (EO) after intravitreal anti-vascular endothelial growth factor (anti-VEGF) injections in three European hospitals performed in an operation room (OR) under sterile conditions. METHODS: A retrospective multicenter study between 2003 and 2016 at three European sites, City Hospital Triemli Zurich, Switzerland (CHT), Zealand University Hospital Roskilde, Denmark (ZUH) and University Clinic Bern, Switzerland (UCB). Intravitreal injection (IVI) database of each department was reviewed. All anti-vascular endothelial growth factor injections were performed using a standardized sterile technique in an operation room. Injection protocols were similar between the three sites. No preinjection antibiotics were given. Postoperative antibiotics varied among sites. RESULTS: A total of 134,701 intravitreal injections were performed at the 3 sites between 2003 and 2016. Ten cases of presumed endophthalmitis were documented: 4 in 50,721 at CHT (95% CI: 0.0071-0.0087%), 2 in 44,666 at ZUH (95% CI: 0.0039-0.0051%), and 4 in 39,314 at UCB (95% CI: 0.0092-0.011%). This results in one case in 13,470 intravitreal injections and a combined incidence of 0.0074% per injection (95% CI: 0.0070-0.0078%). Positive cultures were found in 4 out of 10 presumed endophthalmitis cases. CONCLUSION: The standardized sterile technique in an operation room with laminar airflow showed very low rates of endophthalmitis at three European sites.

Aflibercept in branch retinal vein occlusion as second line therapy: clinical outcome 12 months after changing treatment from bevacizumab/ranibizumab-a pilot study.

PURPOSE: To evaluate the effect of aflibercept (as second line therapy) on the clinical outcome in patients with chronic macular edema secondary to branch retinal vein occlusion (BRVO) insufficiently responding to prior treatment with bevacizumab and/or ranibizumab. METHODS: Ten eyes of ten patients (n = 10) with chronic macular edema secondary to BRVO were included in a retrospective analysis. These patients received aflibercept after an insufficient response to treatment with ranibizumab and/- or bevacizumab. All intravitreal injections were administered according to a "treat and extend" regimen. Insufficient response was defined as the necessity of injection intervals of 6 weeks or less. The primary outcome of the study was the change in mean injection interval from baseline (prior switching to aflibercept) to month 12 after conversion to aflibercept. Secondary outcomes included the change in best corrected visual acuity (BCVA), central retinal thickness (CRT), central retinal volume (CRV) and intraocular pressure (IOP). RESULTS: All patients completed 12 months follow-up. In total, patients received a mean of 15.5 injections of ranibizumab and/or bevacizumab over a mean period of 23.1 months prior to switching to aflibercept. The primary endpoint indicated a significant increase in the injection interval from 5.0 weeks at baseline to 8.3 weeks at month 12 (p = 0.002). Secondary outcomes showed favorable results. Mean BCVA increased from 72.7 letters at baseline to 77.9 letters at month 12 after treatment initiation with aflibercept (+5.2 letters, p = 0.375). Correspondingly, CRT values decreased by 61.7 µm (p = 0.344) and the mean CRV (6 mm diameter) by 0.86 mm3 (p = 0.021) from baseline to 1 year after treatment initiation with aflibercept. During the treatment period with aflibercept no significant changes in intraocular pressure were registered (p = 0.238). CONCLUSIONS: Changing treatment to aflibercept in patients with chronic macular edema secondary to BRVO showed a statistically significant extension of the retreatment interval as well as beneficial anatomic changes in our study group. Our data do not allow a definite conclusion since the study was not controlled.

Optical Coherence Tomography Angiography of the Foveal Avascular Zone in Retinal Vein Occlusion.

PURPOSE: The aim of the study was to visualize and to quantify pathological foveal avascular zone (FAZ) alterations through optical coherence tomography angiography (OCT-A) in eyes with retinal vein occlusion (RVO) in comparison to the unaffected fellow eyes. PROCEDURES: OCT-A was conducted with the Avanti® RTVue 100 XR system (Optovue Inc., Fremont, Calif., USA). The borders of the superficial vascular layer (SVL) were defined as 3 µm below the internal limiting membrane and 15 µm below the inner plexiform layer, and for the deep vascular layer (DVL) as 15 and 70 µm below the inner plexiform layer, respectively. The length of the horizontal, vertical and maximum FAZ was manually measured for the SVL and DVL in each eye. Additionally, the angle between the maximum FAZ diameter and the papillomacular plane was measured. RESULTS: OCT-A depicted defects within the perifoveal vasculature in eyes with branch retinal vein occlusion (BRVO; n = 11) and central retinal vein occlusion (CRVO; n = 8). These resulted in an enlargement of the maximum FAZ diameter in eyes with RVO (n = 19) in comparison to the healthy fellow eyes (n = 19; 921 ± 213 vs. 724 ± 145 µm; p = 0.008). Furthermore, a significant correlation was found between best-corrected visual acuity (BCVA) and the maximum FAZ diameter in the DVL (Spearman's x03C1; = -0.423, p < 0.01). Lastly, in the eyes with RVO, the angle between the papillomacular plane and the maximum FAZ diameter was only in 21.05% (SVL) and 15.79% (DVL) of the cases at 0 ± 15 or 90 ± 15°, respectively. In healthy eyes, these angles (which putatively represent a regular FAZ configuration) were more prevalent (SVL 68.42 vs. 21.05%, p = 0.003; DVL 73.68 vs. 15.79%, p < 0.001). CONCLUSION: OCT-A shows morphological alterations of the FAZ in eyes with CRVO and BRVO. The correlation of the maximum FAZ diameter with BCVA suggests that these alterations are functionally relevant.

Optical coherence tomography angiography of the foveal avascular zone in diabetic retinopathy.

PURPOSE: To analyze foveal avascular zone (FAZ) dimensions and symmetry in patients with diabetic retinopathy (DR) compared to healthy controls using optical coherence tomography angiography (OCT angiography). METHODS: OCT angiography was performed via an Avanti® RTVue 100 XR OCT system (Optovue, Inc., Fremont, CA, USA) in patients with diabetes mellitus (DM) and healthy adults. A frame centered on the fovea was used for FAZ measurements. The borders of the superficial vascular layer were defined as 3 µm below the internal limiting membrane (ILM) and 15 µm below the inner plexiform layer (IPL), and for the deep vascular layer as15 µm and 70 µm below the IPL, respectively. Angles of maximum FAZ diameter were measured in all eyes by two graders. RESULTS: In healthy eyes (N = 25), the FAZ surrounding vascular arcades were intact, showing a vertical or horizontal oval symmetrical formation with a maximum diameter usually on the horizontal or vertical axis. Diabetic eyes (N = 29) presented with disintegrity of the vascular arcades, resulting in an enlarged FAZ. In the superficial layer, the mean horizontal FAZ diameter was significantly larger in the DR group (753 µm ±272 µm) than in the control group (573 µm ±177 µm, p = 0.029). The difference was even more pronounced in the deep layer, with a mean value of 659 µm ±194 µm in the control group and 1009 µm ±342 µm in the DR group (p = 0.001). Furthermore, in the superficial layer, the angle of the maximum FAZ diameter was 0° (±15°) or 90° (±15°) in 72.0 % of healthy eyes. In eyes with DR, the angle was 0° (±15°) or 90° (±15°) in only 6.9 % of cases, due to the irregular configuration of the FAZ. CONCLUSIONS: OCT angiography is capable of imaging retinal vasculature without dye injection. Our data suggest that it can detect disintegrity of the vascular arcades surrounding the FAZ, thus differentiating DM from healthy eyes. Vascular abnormalities were more pronounced in the deep vascular layer.