Peripheral Retinal Nonperfusion Research Articles

Nonperfusion of the Far Peripheral Retina in Highly Myopic Stickler Syndrome.

Purpose: To describe the clinical course of 3 patients with Stickler syndrome found on fluorescein angiography (FA) to have nonperfusion of the peripheral retina. Methods: Three patients with confirmed Stickler syndrome were examined under anesthesia. Genetic testing and FA were performed. Results: Each patient had characteristic ocular findings of Stickler syndrome, including high myopia with vitreoretinal degeneration. FA was performed on each patient and showed 360 degrees of nonperfusion of the retinal periphery in all eyes, with mild leakage in Case 3. Conclusions: The current series presents evidence of peripheral retinal nonperfusion in 3 consecutive patients with Stickler syndrome. Based on these findings, the authors recommend adopting FA as a standard imaging modality and using laser photocoagulation to treat the areas of retinal nonperfusion for all patients with Stickler syndrome.

PARACENTRAL ACUTE MIDDLE MACULOPATHY ASSOCIATED WITH SEVERE PLASMODIUM FALCIPARUM MALARIA.

To report a case of bilateral paracentral acute middle maculopathy (PAMM) lesions on spectral domain-optical coherence tomography (SD-OCT) secondary to severe Plasmodium falciparum malaria. Retrospective case report. SD-OCT, ultra-widefield fluorescein angiography, and OCT-angiography were performed and analysed. A 54-year-old healthy man presented with acute vision loss in both eyes few days after being diagnosed with severe Plasmodium falciparum malaria. Ophthalmoscopic examination was unremarkable but near-infrared reflectance imaging showed patchy hyporeflective areas located at the terminal tips of the venous branches. Corresponding SD-OCT demonstrated alternating bands of hyperreflectivity involving the inner nuclear layer and consistent with skip PAMM lesions. OCT-angiography illustrated corresponding flow signal loss at the level of the deep capillary plexus. Ultra-widefield fluorescein angiography showed peripheral retinal vein staining and capillary non-perfusion. PAMM may be an OCT manifestation of malaria retinopathy associated with severe Plasmodium falciparum infection.

Optic nerve hypoplasia and bilateral persistent fetal vasculature due to TUBA1A tubulinopathy.

To describe a case of TUBA1A-associated optic nerve hypoplasia and persistent fetal vasculature. Observational case report. A female, full term infant was found to have a Dandy-Walker malformation with cerebellar and brainstem hypoplasia, ventriculomegaly, and lissencephaly. Her ophthalmic exam was notable for persistent fetal vasculature, optic nerve hypoplasia, vitreous hemorrhage, and peripheral retinal non-perfusion. Subsequent genetic testing revealed a TUBA1A genetic variant. Persistent fetal vasculature, peripheral retinal vascular abnormalities, and optic nerve hypoplasia may be associated with TUBA1A variants. These patients should be carefully evaluated with dilated retinal exam and fluorescein angiography to detect retinal perfusion abnormalities requiring treatment.

Macular Perfusion Deficits on OCT Angiography Correlate with Nonperfusion on Ultrawide-field Fluorescein Angiography in Diabetic Retinopathy

To evaluate the correlation between nonperfusion parameters on OCT angiography (OCTA) and ultrawide-field fluorescein angiography (UWF-FA) in subjects with diabetes mellitus (DM). Prospective, cross-sectional study. Subjects with DM and a wide range of diabetic retinopathy (DR) severity seen at a tertiary referral center. We used averaged 3× 3 mm OCTA scans to measure geometric perfusion deficit (GPD), vessel density, and vessel length density in the full retina, superficial capillary plexuses (SCPs), and deep capillary plexuses (DCPs). Nonperfusion was manually delineated on UWF-FA to quantify central, peripheral, and total retinal nonperfusion (mm2 and % area). Correlation between OCTA parameters and UWF-FA nonperfusion, and accuracy of these OCTA and UWF-FA parameters in detecting clinically referable eyes, using receiver operating characteristic (ROC) curve analysis, sensitivity, specificity, and area under the ROC curve (AUC). The study included 67 eyes (12 eyes with no signs of DR, 8 mild, 22 moderate, 14 severe nonproliferative DR, and 11 treatment-naive proliferative DR). There was a fair-to-moderate correlation between either central or total retinal nonperfusion on UWF-FA (mm2) and GPD in the SCP (r= 0.482 and r= 0.464, respectively) and DCP (r= 0.470 and r= 0.456, respectively). Receiver operating characteristic analysis showed the DCP GPD significantly superior to other OCTA parameters at the DCP with the largest overall AUC on OCTA for distinguishing referable DR (0.905). Furthermore, the GPD parameter had the largest AUC in each respective capillary layer compared with other parameters. Overall, the total UWF-FA nonperfusion area showed a comparable AUC (0.907) and performed significantly better than peripheral nonperfusion (P= 0.041). Comparing the AUC values between GPD and UWF-FA nonperfusion parameters showed no significant difference in discerning referable DR. Nonperfusion as quantified on OCTA (3× 3 mm) correlated with UWF-FA parameters and both were comparable in detecting referable DR. These macular OCTA metrics, particularly DCP GPD, have the potential for gauging the overall ischemic status of the retina, with an important clinical role in identifying eyes with clinically referable DR. Proprietary or commercial disclosure may be found after the references.

Peripheral Retinal Nonperfusion in Pediatric Patients with Optic Disc Hypoplasia

This study aims to report the association of optic nerve hypoplasia (ONH), peripheral retinal nonperfusion, and secondary complications in pediatric patients. Retrospective case series. The study was conducted between January 2015 and January 2022 at the Bascom Palmer Eye Institute. Inclusion criteria were clinical diagnosis of optic disc hypoplasia, age <18 years, and a fluorescein angiography (FA) of acceptable quality. Seven patients (11 eyes) met inclusion criteria. Average age at presentation was 3.5 years (range 1 month-8 years) and the mean follow-up time was 34.28 months (range 2-87 months). Four patients (57.14%) showed bilateral optic disc hypoplasia. All eyes exhibited peripheral retina nonperfusion on FA, in which mild severity was found in 7 eyes (63.63%), moderate in 2 eyes (18.18%), severe in 1 eye (9.09%) and extreme in 1 eye (9.09%). Eight eyes (72.72%) showed evidence of 360 degrees of retinal nonperfusion. Two patients (18.18%) were diagnosed with concurrent retinal detachment that were deemed inoperable at the time of diagnosis. All cases were observed without intervention. None of the patients were observed to have complications during follow-up. Among pediatric patients with ONH, there appears to be a high rate of concurrent retinal nonperfusion. In these cases, FA is a helpful tool to detect peripheral nonperfusion. Retinal findings are subtle in some cases and may not be detectable in children with suboptimal imaging performed without examination under anesthesia.

Neovascular Glaucoma from Ocular Ischemic Syndrome Treated with Serial Monthly Intravitreal Bevacizumab and Panretinal Photocoagulation: A Case Report

Purpose To describe a case of open-angle neovascular glaucoma (NVG) secondary to ocular ischemic syndrome (OIS) treated with a planned series of 6 monthly anti-VEGF injections with interspersed panretinal photocoagulation (PRP) sessions. We term this treatment protocol the Salvaging Conventional Outflow Pathway in Neovascular Glaucoma (SCOPING) Protocol, and this is our (MQ and DS) standard of care for all NVG patients presenting with partially or completely open angles. Case A 66-year-old man's right eye had a visual acuity of 20/50, intraocular pressure (IOP) of 42 mmHg on 0 IOP-lowering medications, and neovascularization of the iris and angle with no peripheral anterior synechiae. Fundoscopy revealed midperipheral dot-blot hemorrhages without diabetic retinopathy or vein occlusion. Fluorescein angiography revealed peripheral retinal nonperfusion in both eyes. The patient was diagnosed with open-angle NVG secondary to OIS and treated with 6 serial monthly anti-VEGF injections interspersed with 4 PRP sessions, after which his anterior segment neovascularization regressed and IOP normalized on 0 medications. Ten weeks after the last injection, the anterior segment neovascularization and elevated IOP recurred, so he underwent 4 more monthly anti-VEGF injections and 4 PRP sessions, after which his anterior segment neovascularization regressed and his IOP normalized on 0 medications. However, 6 weeks after the last injection, the anterior segment neovascularization and elevated IOP again recurred, so he was resumed on a third course of lifetime monthly anti-VEGF injections, which may be continued in perpetuity. Conclusion The patient's NVG was quiescent while under the protection of serial anti-VEGF injections with interspersed PRP; however, the disease recurred each time injections were stopped. Therefore, in patients with open-angle NVG secondary to OIS, serial monthly anti-VEGF injections may be necessary combined with PRP to suppress underlying neovascular drive and regress anterior segment neovascularization, maintain physiologic IOP, and prevent synechial angle closure.

IRVAN syndrome: A retrospective review of 9 cases from Tunisia

To describe clinical features, relevant imaging findings, disease course, and response to treatment in 9 patients (18 eyes) with idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome. Retrospective review of the charts of nine patients (18 eyes) diagnosed with IRVAN syndrome at Fattouma Bourguiba University Hospital, Monastir, Tunisia, from January 1, 2011 to January 1, 2022. Nine patients were included with bilateral involvement in all cases. Mean initial best-corrected visual acuity (VA) was 20/32 (range, 20/1600-20/20). Clinical findings at presentation included vitreous cells (10 eyes, 55.6%), peripapillary exudates (12 eyes, 66.7%), partial or complete macular star (11 eyes, 61.1%), and vascular sheathing (11 eyes, 61.1%). Fluorescein angiography showed arteriolar aneurysms (18 eyes, 100%), areas of peripheral capillary non-perfusion (16 eyes, 88.9%), and retinal neovascularization (6 eyes, 33.3%). Optical coherence tomography showed macular edema in 5 eyes (27.8%). Optical coherence tomography angiography of the optic disc demonstrated papillary aneurysms in 4 eyes of 2 patients. Indocyanine green angiography showed retinal arteriolar aneurysmal dilatations in 4 eyes of 2 patients. Ten eyes (55.6%) had stage 2 disease, 6 eyes (33.3%) had stage 3, and 2 eyes (11.1%) had stage 1. Treatment modalities included peripheral photocoagulation (16 eyes, 88.9%), intravitreal bevacizumab (4 eyes, 22.2%), and intravitreal triamcinolone acetonide (1 eye, 5.6%). Mean final best-corrected VA was 20/32 (range, 20/600-20/20). Ocular complications included vitreous hemorrhage in 3 eyes (16.7%), branch retinal artery occlusion in 2 eyes (11.1%) and submacular fibrosis in 3 eyes (16.7%). IRVAN syndrome should be highly suspected in patients with peripapillary exudates associated with vascular sheathing and vitreous cells. Multimodal imaging confirms the diagnosis by showing retinal macroaneurysms. Early treatment of macular edema and/or peripheral retinal non-perfusion is mandatory to improve prognosis.

Optical Coherence Tomography Angiography in Central Retinal Vein Occlusion: Macular Changes and Their Correlation with Peripheral Nonperfusion at Ultra-Widefield Fluorescein Angiography

Introduction: The aim of this study was to investigate the correlation between ischemic index (ISI) measured on ultra-widefield (UWF) fluorescein angiography (FA) images and macular parameters obtained by optical coherence tomography angiography (OCT-A) in eyes affected by central retinal vein occlusion (CRVO). Methods: Retrospective study of data from 12 eyes affected by treatment-naïve CRVO. All patients underwent a comprehensive ocular examination including structural OCT, OCT-A, and UWF FA. Variables analyzed included best corrected visual acuity (BCVA) measured with the ETDRS chart; foveal avascular zone (FAZ) area at full-thickness OCT-A angiogram; perfusion density (PD) in the superficial (SCP) and deep capillary plexus (DCP); ISI; and central macular thickness (CMT). Results: ISI showed a significant positive correlation with FAZ area (r = 0.63, p = 0.019) and a significant negative correlation with PD in the SCP (r = −0.62, p = 0.022), PD in the DCP (r = −0.66, p = 0.011), and BCVA (r = −0.75, p = 0.002). FAZ area also negatively correlated to PD in the SCP (r = −0.75, p = 0.002) and DCP (r = −0.64, p = 0.016). BCVA positively correlated to PD in the SCP (r = 0.67, p = 0.009) and DCP (r = 0.68, p = 0.008), while a negative correlation was found with FAZ area (r = −0.65, p = 0.013) and CMT (r = −0.70, p = 0.006). Discussion/Conclusion: OCT-A macular parameters (namely, FAZ area and PD of SCP and DCP) significantly correlated with ISI, a quantitative way to assess peripheral retinal nonperfusion on UWF FA. Macular OCT-A analysis may help in assessing the need for additional UWF FA testing in eyes affected by CRVO.

Multimodal imaging in IRVAN syndrome presenting with Branch Retinal Artery Occlusion.

To describe multimodal imaging findings in a patient with idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome presenting with branch retinal artery occlusion (BRAO). A 33-year-old woman presented with acute BRAO in the right eye. A diagnosis of underlying IRVAN syndrome was made based on the presence of arteriolar aneurysms on the optic disc and along major arterioles and faint retinal hard exudates in both eyes. Eight months later, best-corrected visual acuity was 20/25 in the right eye and 20/20 in the left eye. The hard exudates had increased, and there were extensive areas of peripheral retinal capillary nonperfusion without new vessels. Optical coherence tomography (OCT) showed a localized retinal thinning corresponding to the prior BRAO. Fundus autofluorescence showed nodulo-linear periarterial hypoautofluorescence. OCT angiography (OCTA) showed localized ischemic changes, mainly involving the deep capillary plexus, corresponding to the area of resolved BRAO. It also clearly delineated the optic disc aneurysms. The patient received bilateral scatter laser photocoagulation directed to areas of peripheral capillary nonperfusion. Over a 6-month follow-up period, visual acuity remained unchanged, and there was no evidence of disease progression. Multimodal imaging, including fundus autofluorescence, OCT, and OCTA can provide additional valuable information in the evaluation of IRVAN syndrome complicated with BRAO.

The prognostic value of peripheral retinal nonperfusion in diabetic retinopathy using ultra-widefield fluorescein angiography.

To investigate the prognostic value of peripheral retinal nonperfusion in patients with diabetic retinopathy using ultra-widefield fluorescein angiography (UWFA). A cross-sectional study included 78 treatment-naïve eyes with nonproliferative and proliferative diabetic retinopathy (NPDR and PDR). Eyes were divided into three groups: mild/moderate NPDR (n = 31), severe NPDR (n = 31), and PDR (n = 16). Three nonperfusion variables were calculated reflecting the proportion of nonperfused to visible retina based on initial UWFA: central nonperfusion (CNP) index, peripheral nonperfusion (PNP) index, and PNP ratio. The relationships between these indices and central subfield thickness (CST) and spectacle-corrected visual acuity (SCVA) were evaluated. CNP and PNP indices were significantly higher in the PDR group vs. mild/moderate NPDR group (p = 0.007 and 0.008, respectively) but not in the PDR group vs. severe NPDR group (p = 0.149 and p = 0.535, respectively). A significant linear correlation was found between the CNP and PNP indices in both severe NPDR and PDR groups (R2 = 0.141, p = 0.041, and R2 = 0.311, p = 0.025, respectively). Nonperfusion predominance was not statistically correlated with the presence of macular edema (p = 0.058) or disorganization of retinal inner layers (p = 1). In the severe NPDR group, there was a moderately positive correlation between CNP index and CST (rs = 0.496, p = 0.019) and no correlation between CNP index and SCVA when controlling for CST (p = 0.160). In the PDR group, a strong negative correlation between PNP ratio and CST was found (rs = -0.659, p = 0.014), but no correlation was observed between CNP index, CST, and SCVA. In the PDR group, a positive correlation was found between PNP index, PNP ratio, and SCVA (rs = 0.549, p = 0.027, and rs = 0.626, p = 0.010, respectively), even after controlling for CST (rs = 0.599, p = 0.040). Higher amounts of retinal nonperfusion are seen in patients with more severe retinopathy. Increased CNP is associated with macular thickening and subsequent vision loss. Having predominantly PNP was independently associated with worse VA, regardless of macular thickness. Further studies are needed to investigate the role of PNP in vision loss in diabetic retinopathy.

Management of florid diabetic retinopathy

Background: Florid diabetic retinopathy (FDR) is a severe form of proliferative diabetic retinopathy associated with high risk of blindness. Aim: The aim of this study is to evaluate the efficacy and safety of prompt intravitreal injection of bevacizumab followed by complete pan-retinal photocoagulation (PRP) for treating eyes with FDR. Setting and Design: Prospective, interventional case series. Patients and Methods: Patients with FDR were treated initially with intravitreal injections of bevacizumab 1.25 mg. One week after the injection, patients were sent for wide field fluorescein angiography (WFA), and PRP was performed in two sessions, 2 weeks apart. Patients were examined every 2–4 weeks to determine the status of new vessels (NVs). Additional laser burns were applied in cases with recurrence of NVs. Statistical Analysis: Numbers and percentages were calculated and reported. Results: Eleven patients (18 eyes) with FDR were included. All included patients had poorly controlled diabetes mellitus with mean glycated hemoglobin of 10.6%. One week after the injection, all eyes showed clinical regression of NVs. WFA showed extensive areas of peripheral retinal capillary nonperfusion. Eight weeks after the injection, three eyes (16.7%) showed recurrent increase in NVs, and additional laser therapy was performed for these eyes with special attention to ablating the areas of peripheral capillary non-perfusion. After 16 weeks from the primary treatment, 15 eyes (83.3%) had complete neovascular regression on clinical and angiographic assessment. Pars planavitrectomy was required for three eyes (16.7%) with tractional retinal detachment (2 eyes) or macular epi-retinal membranes (one eye). Conclusions: According to this study, most cases with FDR stabilized after prompt bevacizumab injection followed by complete PRP with special attention for ablating the peripheral capillary nonperfusion areas, guided by WFA images.

Ranibizumab Pro Re nata versus Dexamethasone in the Management of Ischemic Retinal Vein Occlusion: Post-hoc Analysis from the COMRADE Trials

ABSTRACTPurpose: To compare ischemia-related clinical outcomes in patients treated with either ranibizumab pro re nata (PRN) or single dexamethasone implant in the Branch Retinal Vein Occlusion (COMRADE-B) or Central Retinal Vein Occlusion (COMRADE-C) trials.Methods: A post-hoc analysis of the Phase IIIb, 6-month, multicenter, double-masked, randomized, COMRADE-B and COMRADE-C trials. Change over 6 months in retinal ischemia status (central avascular [CA] zone and peripheral nonperfusion [PNP]), mean best-corrected visual acuity (BCVA), the development of shunt vessels and neovascularization, and frequency of laser therapy were assessed in retinal vein occlusion (RVO) patients treated with either ranibizumab 0.5 mg PRN or single dexamethasone 0.7 mg implant, as per European labels, in the COMRADE-B (N = 244; ranibizumab, 126, dexamethasone, 118) or COMRADE-C (N = 243; ranibizumab, 124, dexamethasone, 119) trials. BCVA progression in ischemic vs. non-ischemic patients based on the ischemia assessment at month 6 was carried out.Results: Visual acuity (VA) gains from baseline to month 6 were higher with ranibizumab than with dexamethasone in both patients with central ischemia and those with peripheral retinal nonperfusion, independent of the type of RVO (branch or central). The presence of CA and PNP had a significant impact on VA gain over 6 months in CRVO patients (p < .0001), while there was no significant impact in BRVO. Ranibizumab was associated with less new ischemia than dexamethasone. Central RVO patients treated with dexamethasone received more laser treatments over the 6 months than those treated with ranibizumab, while there was no difference in the frequency of laser therapy between the branch RVO treatment groups.Conclusions: VA gain over six months in ranibizumab-treated RVO patients is not affected by ischemia, and is associated with less development of new ischemia during the first 6 months of treatment and equal or fewer laser treatments than dexamethasone implant.

Retinal Vascular Abnormalities in Phakomatosis Pigmentovascularis

To describe the spectrum of retinal vascular abnormalities in patients with phakomatosis pigmentovascularis (PPV). Multicenter, retrospective, noncomparative, consecutive case series. Eligible patients underwent detailed retinal examination including indirect ophthalmoscopy. Ultra-widefield fundus imaging, including color fundus photography and angiography, was performed using standardized protocols, and findings were recorded and reviewed and analyzed. Three patients with a clinical diagnosis of PPV are presented. Evaluation of all patients (n = 6 eyes of 3 patients) with widefield fluorescein angiography showed several retinal vascular abnormalities, including peripheral retinal nonperfusion (n = 3 eyes), peripheral vascular leakage (n = 3 eyes), aberrant retinal vessels (n = 1 eyes), vascular tortuosity (n = 1 eyes), and disruption of the foveal avascular zone including fovea plana (n = 3 eyes). In addition, 2 eyes demonstrated peripheral retinal vascular straightening and leakage similar to the features of familial exudative vitreoretinopathy. One of the patients was a carrier of a somatic GNA11 R183C pathogenic variant that has been associated with PPV. Fluorescein angiography, especially with widefield capability, reveals numerous retinal vascular abnormalities in patients with PPV. Considering the association of GNA11 pathogenic variants with PPV and allied disorders, these observations may suggest a role of guanine-binding proteins (G-proteins) in retinal vascular development. Supplemental material available at www.ophthalmologyretina.org.

Retinal haemangioblastoma associated with peripheral non-perfusion: widefield fluorescein angiography analysis of 41 cases

AimsTo investigate the association of peripheral retinal non-perfusion with retinal haemangioblastoma.MethodsMedical and widefield fluorescein angiography records of patients diagnosed with retinal haemangioblastoma from 1990 to 2018 were reviewed for patient...

Retinal Nonperfusion Characteristics on Ultra-Widefield Angiography in Eyes With Severe Nonproliferative Diabetic Retinopathy and Proliferative Diabetic Retinopathy

Threshold of retinal nonperfusion for the development of proliferative diabetic retinopathy (PDR) is unclear. To identify a threshold of retinal nonperfusion for the presence of retinal neovascularization and the distribution and area of retinal nonperfusion in eyes with severe nonproliferative diabetic retinopathy (NPDR), PDR, neovascularization of the optic disc (NVD), and retinal neovascularization elsewhere (NVE). This cross-sectional image analysis study was performed between September 24, 2018, and October 24, 2018, at a multicenter national study in the United Kingdom. Baseline images were obtained from 2 completed randomized clinical trials (Ranibizumab for Diabetic Macular Edema Panretinal Photocoagulation [RDP] study and Clinical Efficacy of Intravitreal Aflibercept vs Panretinal Photocoagulation for Best Corrected Visual Acuity in Patients With Proliferative Diabetic Retinopathy at 52 Weeks [CLARITY] study). The RDP study recruited eyes with severe NPDR between April 1, 2014, and December 31, 2015, and the CLARITY study recruited eyes with PDR between August 22, 2014, and November 20, 2015. Ultra-widefield angiography images of eyes with no prior panretinal photocoagulation treatment were included. The total area of retinal nonperfusion, the area of posterior pole retinal nonperfusion, and the area of peripheral retinal nonperfusion were measured. A total of 92 patients (92 eyes) were included in the study: 59 in the PDR group (mean [SD] age, 42 [15] years; 20 female [33.9%]) and 33 in the NPDR group (mean [SD] age, 63 [10] years; 3 female [9.1%]). Forty eyes had NVE and 19 had NVD with or without NVE. We identified a retinal nonperfusion threshold of 118.3 disc areas (DA) with a specificity of 84.9% (95% CI, 68.1% to 94.9%) for PDR. The median area of retinal nonperfusion was 67.8 DA (95% CI, 44.2 to 107.3 DA) in the NPDR eyes and 147.9 DA (95% CI, 127.4 to 173.5 DA) for eyes with proliferative changes, with a difference of 69.0 DA (95% CI, 42.2 to 97.7 DA; P < .001). No difference was found in the median area of posterior nonperfusion between NPDR and PDR, with a difference of 0 DA (95% CI, -6.7 to 5.2 DA; P = .56). As for peripheral nonperfusion, NPDR eyes measured 64.1 DA and PDR eyes measured 130.6 DA, with a difference of 70.8 DA (95% CI, 48.4 to 94.9 DA; P < .001). Eyes with NVD had the largest total area of retinal nonperfusion, with a difference of 65.1 DA (95% CI, 28.6 to 95.8 DA; P < .001) compared with eyes with only NVE. These findings suggest eyes with at least 107.3 DA of nonperfusion are at risk of proliferative disease, and eyes with NVD have the largest area of retinal nonperfusion.

Combined Multimodal Analysis of Peripheral Retinal and Macular Circulation in Diabetic Retinopathy (COPRA Study)

To systematically examine the relationships between the microvascular indices that are measured on OCT angiography (OCTA) and the presence and extent of peripheral nonperfusion in persons with diabetic retinopathy. A retrospective cross-sectional study of patients who had varying degrees of diabetic retinopathy. The study sample was recruited from 2 large tertiary referral retina clinics. In total, 82 eyes of 45 patients with varying degrees of diabetic retinopathy were enrolled and analyzed. Relationships between peripheral ischemia measured on fluorescein angiography (FA) and OCTA metrics, including foveal avascular zone (FAZ) and vessel density measurements. A significant decrease in mean signal index in both the superficial and deep plexus and binarized flow index in the superficial plexus were found with increasing duration of diabetes mellitus. OCT and OCTA grading showed increasing central macular thickness and prevalence of microvascular abnormalities in the superficial and deep capillary bed with worse retinopathy as measured on the Diabetic Retinopathy Severity Scale. FAZ area and major axis and minor axis length were strongly associated with diabetic retinopathy severity. On classifying eyes into tertiles of peripheral ischemia measured on FA, significant increases in various FAZ metrics, including FAZ area and minor axis length, were noted. Statistically worsening of FAZ OCTA metrics was only seen between tertiles 2 and 3, indicating a non-linear relationship. The presence of neovascularization of the disc, neovascularization elsewhere, or intraretinal microvascular abnormality was associated with a significant increase in FAZ major axis length in the superficial plexus and a significant decrease in binarized flow index in the deep plexus. OCTA metrics are indicators of the severity of peripheral retinal nonperfusion. However, the central ischemic index did not exhibit a linear relationship with peripheral capillary nonperfusion. Our findings suggest that a rise in intraocular vascular endothelial growth factor as a consequence of mild peripheral capillary nonperfusion may play a compensatory role in maintaining the central macular microcirculation. Further investigations with studies employing longitudinal design will improve our understanding of the relationship between macular microcirculation and peripheral ischemia.

Neurofibromatosis Type 1 Vasculopathy Presenting as Impending Central Retinal Artery Occlusion

Neurofibromatosis Type 1 Vasculopathy Presenting as Impending Central Retinal Artery Occlusion

Peripheral retinal nonperfusion using widefield imaging with von Hippel-Lindau disease

BackgroundTo describe a case of von Hippel-Lindau disease with peripheral retinal nonperfusion.Case presentationA 66-year-old female with known cerebellar and midbrain hemangioblastomas was evaluated for a retinal hemangioblastoma in the right eye. She underwent widefield fluorescein angiography, which showed hyperfluorescence localized to the hemangioblastoma surrounded by peripheral retinal nonperfusion in the same quadrant.ConclusionsFurther widefield imaging studies are required to determine if peripheral retinal nonperfusion is a common finding in von Hippel-Lindau disease.

Peripheral Retinal Nonperfusion in Pediatric Patients With Morning Glory Syndrome.

To report the association of morning glory syndrome (MGS) with peripheral retinal nonperfusion in pediatric patients with MGS. The authors retrospectively analyzed the records of pediatric patients with MGS using fundus fluorescein angiography. The peripheral retinal vascular architecture was recorded and graded according to the severity of peripheral retinal nonperfusion. Eighty-six eyes of 74 patients were enrolled. Seventy-three of 86 eyes (84.88%) had peripheral retinal nonperfusion, in which mild severity was found in 31 of 86 eyes (36.05%), moderate in 17 of 86 eyes (19.77%), severe in 18 of 86 eyes (20.93%), and extreme in seven of 86 eyes (8.14%). Secondary complications of nonperfusion included leakage in six of 73 eyes (8.22%), fibrovascular proliferation in two of 73 eyes (2.74%), and tractional retinal detachment in one of 73 eyes (1.34%). There is a high prevalence of peripheral retinal nonperfusion in pediatric MGS eyes, with secondary complications in some, suggesting that more attention should be paid to the peripheral retina in MGS. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:674-679.].

Symmetry of peripheral retinal nonperfusion in diabetic retinopathy by ischemic index

PurposeDiabetic retinopathy is the leading cause of blindness among working-age adults in most developed countries. It affects eyes bilaterally and is generally believed to be symmetrical, yet there are few studies evaluating the symmetry of diabetic retinopathy. The purpose of the present study was to evaluate the symmetry of the amount of peripheral retinal ischemia in patients with diabetic retinopathy. MethodsUltra-widefield fluorescein angiography images were obtained on both eyes of 54 subjects, mean age 56.4 years, from an urban eye clinic. A single, high quality image from the arteriovenous phase of the angiogram of each eye was selected for analysis. The total area of gradable fundus and area of nonperfusion seen in the arteriovenous phase of the ultra-widefield fluorescein angiogram were determined. An ischemic index (ISI) was calculated by dividing the non-perfused retinal area by the total retinal area and multiplying by 100. ResultsThe mean ISI OD was 11.27, mean ISI OS was 11.64. The mean absolute value (±SD) of ISI difference between OD and OS was 4.46±6.09. A difference in ISI of 10% or less was found in 92.6% of subjects. A statistically significant correlation was found in the ISI between right and left eyes (rs=0.80, p<0.0001) and there was no statistically significant difference in ISI between the right and left eyes (p=0.85). ConclusionAsymmetrical retinopathy in diabetic patients is uncommon and additional pathological processes should be considered in the presence of asymmetric DR.