Areas Of Nonperfusion Research Articles

Major clinical findings of cellular therapy for intravitreal use in ischemic retinopathy and macular degeneration: a systematic review

Introduction: In the scenario of eye diseases, diabetic retinopathy and retinal vein occlusion are the two most common ischemic retinopathies in the world. Ischemia is caused by retinal vascular diseases due to decreased blood perfusion and the appearance of areas of retinal non-perfusion. Also, age-related macular degeneration (AMD) is the most common cause of irreversible vision loss in people over 65 years of age in industrialized countries. By 2020, around 200 million people will be affected by AMD worldwide. Objective: the present systematic review study aimed to highlight the main clinical findings of the treatment of ischemic retinopathy and age-related macular degeneration through cell therapy with bone marrow stem cells. Methods: The rules of the Systematic Review-PRISMA Platform were followed. The search was carried out from March 2022 to June 2022 in Scopus, PubMed, Science Direct, Scielo, and Google Scholar databases. The quality of the studies was based on the GRADE instrument. The risk of bias was analyzed according to the Cochrane instrument. Results and Conclusion: It was found 235 articles involving retinitis pigmentosa, macular degeneration, and bone marrow stem cell therapy. A total of 51 were fully evaluated and 28 studies were included and developed in a systematic review in the results field. The symmetrical Funnel Plot does not suggest a risk of bias between the small sample size studies. It was concluded that intravitreal injection of bone marrow-derived stem cells in a patient with retinal vascular occlusion sequelae demonstrated that the procedure is feasible and safe to be performed in humans as there were no signs of infection, inflammation, or development of intraocular tumor formation. Also, neurotrophic effects correlate with vasculature preservation, suggesting that bone marrow-derived stem cells can be used in the treatment of diseases such as retinal degenerations and vasculopathy that currently lack effective treatment. The authors concluded that stem cells can protect retinal cells from degeneration and also suggested that they were able to replace some types of lost retinal neurons.

Evaluation of the retinal and choroidal microvasculature changes in cases of sarcoid and tuberculosis-associated posterior uveitis using OCT angiography

PurposeUsing optical coherence tomography angiography (OCTA) to evaluate retinal microvascular changes in sarcoid and tuberculous (TB) posterior uveitis.MethodsCross-sectional observational study includes 30 eyes. FFA and OCTA images were acquired. OCTA images were analyzed for areas of capillary hypo-perfusion, disorganization of the superficial and deep capillary plexuses (SCP and DCP) and intraretinal cystoid spaces and for measuring the size of the foveal avascular zone and vessel density (VD) in the SCP and DCP.ResultsA total of 11 eyes were associated with TB and 19 with sarcoidosis. By OCTA, 100% had areas of capillary non-perfusion, 36.7% choroidal voids, 30% disorganization of the SCP and DCP and 26.6% intraretinal cystoid spaces. The VD of the DCP was significantly lower in the TB group. On comparing OCTA and FFA, parafoveal ischemia was detected more frequently on OCTA and macular edema more frequently on FFA (P = < 0.001). The BCVA was not significantly correlated with the VD of the SCP or DCP.ConclusionOCTA can be used in detection of early microvascular changes, segmenting retinal layers and localizing abnormalities. The presence of these changes may aid in the diagnosis of TB and sarcoid uveitis, for prognosis, follow-up and may be the only choice when FFA is contraindicated.

Primary Occlusive Panvasculitis with Segmental Periarterial Plaques.

To describe a new clinical entity of bilateral occlusive panvasculitis with segmental periarterial plaques and its clinical course in two patients. Retrospective chart review. Two patients with no medical history of any systemic inflammatory diseases presented with bilateral segmental periarterial plaques (Kyrieleis plaques), cotton wool spots and microaneurysms. Segmental leakage, staining and vascular occlusions involved the arterioles, venules and capillaries. Leakage from the superficial capillary plexus in some areas bordering deep capillary plexus non-perfusion were observed. Both had recurrent episodes of vascular occlusions, normal brain MRI and audiology tests. Complete work-up including serology for infections, inflammatory markers and antibody titers were unremarkable. They were started on corticosteroids and immunosuppressant and there were no further vascular occlusions. Both developed neovascularization adjacent to areas of capillary non-perfusion. The second patient also developed vitreomacular traction and cystoid macular edema. He required intravitreal anti-VEGF injection, sector laser photocoagulation and underwent a vitrectomy with membrane peeling. At last visit, visual acuity was 20/30 in both eyes for the first patient, 20/20 in the right and 20/40 in the left eye for the second patient, 12 and 6.5 years after initial presentation respectively. Both patients presented with findings of bilateral panvasculitis with prominent segmental periarterial plaques and had repeated episodes of vascular occlusions before corticosteroid and immunosuppressants treatment, after which no additional occlusions were observed. We propose the constellation of findings constitute a novel clinical entity, occlusive panvasculitis with segmental periarterial plaques.

Anti-VEGF reduces inflammatory features in macular edema secondary to retinal vein occlusion.

To investigate the anti-inflammatory effect of intravitreal injection of anti-vascular endothelial growth factor (anti-VEGF) in patients with macular edema secondary to retinal vein occlusion (RVO-ME). Twenty-eight eyes from twenty-eight treatment-naïve patients (14 males and 14 females) with RVO-ME were included in this retrospective study. The retinal vein occlusion (RVO) was comprised of both central retinal vein occlusion (CRVO, n=14) and branch retinal vein occlusion (BRVO, n=14). Intravitreal injection of anti-VEGF reagents were administered monthly for three consecutive months, in which 18 patients were injected with ranibizumab and 10 patients were injected with conbercept. All eyes were imaged with optical coherence tomography angiography (OCTA) at baseline and 1wk after monthly intravitreal anti-VEGF injection. The visual acuity (VA), central macular thickness (CMT), the number of hyperreflective foci (HRF) recognized as an inflammatory sign in OCT images, and non-perfusion area (NPA), were compared before and after anti-VEGF treatments. The mean interval between baseline and follow-up was 29.4±0.79 (range, 27-48)d. Compared with the baseline, the VA improved (logMAR 1.5±0.1 vs 0.8±0.1, P<0.05) and CMT decreased (460±34.0 µm vs 268.8±12.0 µm, P<0.05), significantly, after anti-VEGF treatment. The number of HRF was decreased significantly (76.5±4.8 vs 47.8±4.3, P<0.05) after anti-VEGF treatment. Anti-VEGF therapy is effective in treating RVO-ME. The mechanisms for the decreased HRF and the reduction of NPA by anti-VEGF therapy merits further exploration.

Association of Ultra-Widefield Fluorescein Angiography–Identified Retinal Nonperfusion and the Risk of Diabetic Retinopathy Worsening Over Time

Presence of predominantly peripheral diabetic retinopathy (DR) lesions on ultra-widefield fluorescein angiography (UWF-FA) was associated with greater risk of DR worsening or treatment over 4 years. Whether baseline retinal nonperfusion assessment is additionally predictive of DR disease worsening is unclear. To assess whether the extent and location of retinal nonperfusion identified on UWF-FA are associated with worsening in Diabetic Retinopathy Severity Scale (DRSS) score or DR treatment over time. This cohort study was a prospective, multicenter, longitudinal observational study with data for 508 eyes with nonproliferative DR and gradable nonperfusion on UWF-FA at baseline. All images were graded at a centralized reading center; 200° ultra-widefield (UWF) color images were graded for DR at baseline and annually for 4 years. Baseline 200° UWF-FA images were graded for nonperfused area, nonperfusion index (NPI), and presence of predominantly peripheral lesions on UWF-FA (FA PPL). Treatment of DR or diabetic macular edema was at investigator discretion. Association of baseline UWF-FA nonperfusion extent with disease worsening, defined as either 2 or more steps of DRSS worsening within Early Treatment Diabetic Retinopathy Study fields on UWF-color images or receipt of DR treatment. After adjusting for baseline DRSS, the risk of disease worsening over 4 years was higher in eyes with greater overall NPI (hazard ratio [HR] for 0.1-unit increase, 1.11; 95% CI, 1.02-1.21; P = .02) and NPI within the posterior pole (HR for 0.1-unit increase, 1.35; 95% CI, 1.17-1.56; P < .001) and midperiphery (HR for 0.1-unit increase, 1.08; 95% CI, 1.00-1.16; P = .04). In a multivariable analysis adjusting for baseline DRSS score and baseline systemic risk factors, greater NPI (HR, 1.11; 95% CI, 1.02-1.22; P = .02) and presence of FA PPL (HR, 1.89; 95% CI, 1.35-2.65; P < .001) remained associated with disease worsening. This 4-year longitudinal study has demonstrated that both greater baseline retinal nonperfusion and FA PPL on UWF-FA are associated with higher risk of disease worsening, even after adjusting for baseline DRSS score and known systemic risk. These associations between disease worsening and retinal nonperfusion and FA PPL support the increased use of UWF-FA to complement color fundus photography in future efforts for DR prognosis, clinical care, and research.

Atypical Adams-Oliver syndrome with typical ocular signs of familial exudative vitreoretinopathy

AIM: To report an atypical Adams-Oliver syndrome (AOS) family with typical ocular signs of familial exudative vitreoretinopathy (FEVR). METHODS: A patient with visible avascular area and obvious non-perfusion zone in the peripheral retina with systemic signs of AOS was reported. Familial and personal characteristics were collected for the patient and his sister. Gene sequencing and ophthalmic examinations including fluorescein angiography were all performed for the whole family. RESULTS: Two novel mutations of DOCK6 (c.1396C&gt;T and c.4796G&gt;A) were identified in the proband and his family, and two compound heterozygous mutations were revealed in the proband and his sister. The patient and his sister showed physical deformities and mental abnormalities while FEVR mimicking retinal disorder can also be defined. No remarkable ocular or systemic abnormality can be observed for their parents. Peripheral retinal non-perfusion area, obvious abnormal vascularization or even retinal fold were observed in the proband and his sister, while only small avascular zone was identified for their parents. CONCLUSION: This is the first genetic authenticated AOS case mimicked as FEVR with genetic sequencing of a family. For the patients with ocular phenotype of FEVR, further examination should be performed if the systemic or mental abnormalities exist.

Microvascular Changes in the Cystic Lesion of Branch Retinal Vein Occlusion Imaged by Swept-Source Optical Coherence Tomography Angiography

Introduction: This study aimed to describe the quantitative features of the microvasculature in the cystic lesions of branch retinal vein occlusion (BRVO). Methods: A total of 43 eyes with BRVO, treated with anti-vascular endothelial growth factor therapy, were analyzed. Using wide-field swept-source optical coherence tomography angiography (OCTA), en face OCT images were obtained by depth-integrated reflectivity of the retina, and vascular density (VD), vascular length (VL), vascular lacunarity, and fractal dimension (FD) were evaluated in a 12 × 12-mm area of retinal nonperfusion. Results: The mean area of affected lesions was 38.7 ± 19.8 mm², and cystic lesions were 8.5 ± 10.1 mm². VD, VL, and FD were significantly decreased in the cystic lesions compared to other affected lesions in the same eyes (p = 0.0010, p = 0.0001, and p = 0.0003, respectively) and in all eyes (p = 0.0281, p = 0.0050, and p < 0.0001, respectively). VD in cystic lesions within the vascular arcade (25 eyes) correlated with best-corrected visual acuity on OCTA (r = −0.433, and p = 0.0492). Conclusions: Vascular structure in the cystic lesions was unpreserved compared to the other lesions in BRVO. These findings may help in understanding the pathophysiology of retinal edema in BRVO.

RETINAL NONPERFUSION IN THE EARLY TREATMENT DIABETIC RETINOPATHY STUDY SEVEN FIELDS COMPARED WITH WIDEFIELD FLUORESCEIN ANGIOGRAPHY

In previous landmark studies on central retinal vein occlusion, retinal nonperfusion assessments were obtained using 7-field (7F) angiography. The widespread current use of widefield imaging allows better visualization of the peripheral retina and more comprehensive estimation of the total area of nonperfusion. The relationship between nonperfusion measurement of 7F and widefield angiography (WFA) in central retinal vein occlusion has not been studied. We aim to identify the correlation of retinal nonperfusion measured within the 7F and on WFA in eyes with central retinal vein occlusion. Retinal nonperfusion in participants with central retinal vein occlusion was determined using a 7F Early Treatment Diabetic Retinopathy Study template and the concentric rings method. A total of 153 eyes were included. Pearson correlation test showed a near-perfect positive, linear correlation between the nonperfusion found in the 7F and total retinal nonperfusion on WFA (0.985 95% CI [0.793, 0.999]) The regression line equation for nonperfusion on 7F and WFA was y = 37 + 3.2x. Eyes with 0 disk areas (DA), >0 DA to 10 DA and >10 DA of nonperfusion on 7-fields had on average 23 DA 95% CI (19.20, 27.06), 45 DA 95% CI (35.75, 55.18), and 115 DA 95% CI (88.89, 142.05) on widefield respectively. There is a positive and linear relationship between nonperfusion measured by 7F and WFA in central retinal vein occlusion with more than 3-times the amount of nonperfusion identified on WFA. Despite <10 DA no areas of nonperfusion on 7F, there is typically at least 35 DA of nonperfusion on WFA whereas eyes with >10 DA of nonperfusion on 7F had at least 88 DA on WFA.

Progress of Imaging in Diabetic Retinopathy-From the Past to the Present.

Advancement of imaging technology in retinal diseases provides us more precise understanding and new insights into the diseases’ pathologies. Diabetic retinopathy (DR) is one of the leading causes of sight-threatening retinal diseases worldwide. Colour fundus photography and fluorescein angiography have long been golden standard methods in detecting retinal vascular pathology in this disease. One of the major advancements is macular observation given by optical coherence tomography (OCT). OCT dramatically improves the diagnostic quality in macular edema in DR. The technology of OCT is also applied to angiography (OCT angiograph: OCTA), which enables retinal vascular imaging without venous dye injection. Similar to OCTA, in terms of their low invasiveness, single blue color SLO image could be an alternative method in detecting non-perfused areas. Conventional optical photography has been gradually replaced to scanning laser ophthalmoscopy (SLO), which also make it possible to produce spectacular ultra-widefield (UWF) images. Since retinal vascular changes of DR are found in the whole retina up to periphery, it would be one of the best targets in UWF imaging. Additionally, evolvement of artificial intelligence (AI) has been applied to automated diagnosis of DR, and AI-based DR management is one of the major topics in this field. This review is trying to look back on the progress of imaging of DR comprehensively from the past to the present.

OCT-Angiography in Detecting Preclinical Diabetic Retinopathy

The introduction of OCT-angiography was a milestone in studying the early stages of diabetic retinopathy (DR). The latest findings show significant changes in foveal avascular zone (FAZ) parameters (FAZ area, FAZ perimeter, acirculatory index, axis ratio, FAZ angle) in diabetes mellitus (DM) patients with no ophthalmoscopic signs of DR. Many research groups evaluate vessel density (VD) in superficial and deep plexuses in these patients using different methods of image postprocessing, different qualitative and quantitative parameters. Nonperfusion areas (NA) are very important findings at the preclinical stage of DR, which can be detected in superficial and deep layers. With OCT-A it is possible not only to reveal them but also to calculate their area. Vascular tortuosity (VT) was described earlier using fundus photography. Nowadays VT can be assessed at the microcirculation level in OCT-A scans. Microaneurysms are the earliest clinical sign of DR. They can be missed in fundus photos, but easily detected with OCT-A. The aim of the current review is to analyze the latest OCT-A findings at the preclinical stage of DR and to discuss the future diagnostic value of OCT-A in DR.

Diabetic retinopathy with extensively large area of capillary non-perfusion: characteristics and treatment outcomes

BackgroundCapillary non-perfusion is an important characteristic for diabetic retinopathy (DR) indicating microvascular damage and ischemia. Data on the description and treatment outcomes of DR with large area of non-perfusion are lacking to date. We aim to describe the characteristics and treatment outcomes in a series of patients with DR who presented extensively large area of capillary non-perfusion (LACNP).MethodsFundus fluorescein angiograms from medical charts in patients diagnosed with DR between Jan 2017 and Dec 2019 were retrospectively reviewed. Clinical data in eyes with LACNP including imaging and laboratory findings at the first presentation were analyzed. The LACNP was defined as over 70% area of capillary non-perfusion throughout the whole image retina. The mean follow-up duration was 12.4 ± 16.7 months. Follow-up data including extensive pan-retinal photocoagulation and surgical intervention and treatment outcomes were evaluated.ResultsA total of 43 eyes in 24 patients with LACNP were included, accounting for 3.3% of DR populations in the same period. The overall percentage of non-perfusion area was 79.1 ± 8.1%. All patients received proper control of diabetes and hypertension, and extensive pan-retinal laser photocoagulation. During the follow-up periods, 20 eyes (46.5%) developed severe neovascular complications, of which 15 eyes (34.9%) underwent vitrectomy and/or anti-glaucoma surgeries. Conservative therapies including glycemic control and supplemental laser photocoagulation were conducted in 23 eyes (53.5%) without neovascular complications. In the final follow-up, best corrected visual acuity improved or maintained stable in 19 eyes (44.2%) while deteriorated in 24 eyes (55.8%).ConclusionsThe presence of LACNP is the hallmark of advanced DR and often indicates a poor visual outcome, although aggressive treatments may slow DR progression and maintain central vision for some time.

Deep Learning Models for Segmenting Non-perfusion Area of Color Fundus Photographs in Patients With Branch Retinal Vein Occlusion.

PurposeTo develop artificial intelligence (AI)-based deep learning (DL) models for automatically detecting the ischemia type and the non-perfusion area (NPA) from color fundus photographs (CFPs) of patients with branch retinal vein occlusion (BRVO).MethodsThis was a retrospective analysis of 274 CFPs from patients diagnosed with BRVO. All DL models were trained using a deep convolutional neural network (CNN) based on 45 degree CFPs covering the fovea and the optic disk. We first trained a DL algorithm to identify BRVO patients with or without the necessity of retinal photocoagulation from 219 CFPs and validated the algorithm on 55 CFPs. Next, we trained another DL algorithm to segment NPA from 104 CFPs and validated it on 29 CFPs, in which the NPA was manually delineated by 3 experienced ophthalmologists according to fundus fluorescein angiography. Both DL models have been cross-validated 5-fold. The recall, precision, accuracy, and area under the curve (AUC) were used to evaluate the DL models in comparison with three types of independent ophthalmologists of different seniority.ResultsIn the first DL model, the recall, precision, accuracy, and area under the curve (AUC) were 0.75 ± 0.08, 0.80 ± 0.07, 0.79 ± 0.02, and 0.82 ± 0.03, respectively, for predicting the necessity of laser photocoagulation for BRVO CFPs. The second DL model was able to segment NPA in CFPs of BRVO with an AUC of 0.96 ± 0.02. The recall, precision, and accuracy for segmenting NPA was 0.74 ± 0.05, 0.87 ± 0.02, and 0.89 ± 0.02, respectively. The performance of the second DL model was nearly comparable with the senior doctors and significantly better than the residents.ConclusionThese results indicate that the DL models can directly identify and segment retinal NPA from the CFPs of patients with BRVO, which can further guide laser photocoagulation. Further research is needed to identify NPA of the peripheral retina in BRVO, or other diseases, such as diabetic retinopathy.

Оценка эффективности комбинированной терапии постокклюзионных изменений сетчатки

Purpose. To evaluate the volume of therapeutic measures necessary for the relief of macular edema in patients with Branch Retinal Vein Occlusion (BRVO) depending on the initial macular ischemia. Material and methods. 142 patients with macular edema on the background of BRVO were included in the study. All patients received intravitreal injection of ranibizumab in 1+PRN regimen, laser coagulation if necessary. All patients underwent examination, including visometry, electroretinography, optical coherence tomography (OCT) and OCT angiography. The number of injections and laser manipulations over the entire follow up period was taken into account. The comparative analysis of the obtained results was carried out in 3 groups formed depending on the area of the initial macular ischemia. The significance level was p&lt;0.0125. Results. It was found that in group 1 patients with an initial area of macular ischemia from 0.3 to 1.5 mm² , 1.5±0.7 injections of ranibizumab were required to resorb macular edema and increase visual acuity by more than 2 times. In group 2 patients with an area of macular ischemia from 1.5 to 1.8 mm² , an average of 7.2±2.2 injections of ranibizumab were required to relieve macular edema, in 62% of cases, sectoral laser coagulation, in 15% – panretinal laser coagulation of the retina, while visual acuity improved unreliably (p&gt;0.05). In group 3 patients with an initial area of macular ischemia more than 1.8 mm² , despite an average of 3.6±1.4 injections of ranibizumab and in 58% of cases of panretinal laser coagulation, visual acuity did not significantly change (p&gt;0.05), and the area of capillary nonperfusion increased in the perifoveal zone and on the periphery of the retina. Conclusion. The volume of therapeutic measures in patients with macular edema on the background of BRVO and the possibility of improving visual functions depends not only on the degree of ischemia of the peripheral retina, but also on the initial area of ischemia of the macular zone according to OCT angiography, the amplitude of the b-wave ERG and oscillatory potentials, which can be considered as markers that allow predicting the effectiveness of combined therapy of post-occlusive retinal changes. Key words: central retinal vein branch occlusion, retinal ischemia, macular ischemia, anti-VEGF therapy, OCT, OCT angiography.

Inflammatory cytokines and retinal nonperfusion area in quiescent proliferative diabetic retinopathy.

We sought to investigate the association between inflammatory cytokine levels and retinal capillary nonperfusion area in eyes with quiescent proliferative diabetic retinopathy (PDR). Samples of aqueous humor were collected from 67 eyes (n = 42 patients) with treatment-naïve PDR. Levels of interleukin (IL)-10, IL-1β, IL-6, IL-8, monocyte chemoattractant protein 1 (MCP-1), and tumor necrosis factor-α (TNF-α) were obtained using multiplex bead assay. Areas of capillary nonperfusion at the posterior pole and peripheral retina were measured via ultra-widefield fluorescein angiography and correlated with cytokine levels. The levels of IL-10, IL-6, IL-8, MCP-1, and TNF-α were positively correlated with the nonperfusion area of the peripheral retina (r = 0.298, 0.401, 0.265, 0.435, and 0.393; all P ≤ 0.030). There were positive correlations between IL and 10, IL-6, IL-8, MCP-1, and TNF-α (all R ≥ 0.247; all P ≤ 0.043). IL-1β did not show a significant correlation with the nonperfusion area (P = 0.972 for posterior pole and 0.392 for periphery) but was positively correlated with TNF-α (r = 0.334; P = 0.006). An increased level of inflammation was observed in PDR eyes with larger nonperfusion areas, which suggests inflammation as a possible target for suppressing PDR progression associated with nonperfusion.

Correlation between ultrawide-field fluorescence contrast results and white blood cell indexes in diabetic retinopathy

BackgroundDiabetic retinopathy (DR) is one of the most common microvascular complications of diabetes. DR involves a state of systemic inflammation, and chronic inflammation can promote microvascular and macrovascular diseases in diabetic patients and accelerate disease progression. Ultrawide-field FFA (UWFA) systems are increasingly being used to examine a wider retina. The aim of this study was to explore the correlation between the different manifestations of retinopathy under UWFA and the systemic indicators of white blood cells in patients with diabetic retinopathy.MethodsThis retrospective study included the hospitalized DR patients in the Department of Ophthalmology and Endocrinology of the Affiliated Hospital 2 of Nantong University between January 2016 and March 2019. This study examined the correlations between the UWFA examination results and glycated hemoglobin (HbA1c), routine blood tests,and the neutrophil-to-lymphocyte ratio of patients with clinically diagnosed DR during hospitalization.ResultsA total of 115 patients with DR (53 females and 62 males) were included (199 eyes: 102 right eyes and 97 left eyes). UWFA revealed that most eyes (77.4%) had grade 4 microvascular leakage, 52.8% had grade 0 capillary non-perfusion area, 59.3% had grade 0 neovascularization, and 92.0% had grade 0 fibrous proliferative membranes. Microvascular leakage was correlated with the NLR (r = 0.186, P = 0.027). Capillary non-perfusion area was correlated with the monocyte ratio (r = 0.144, P = 0.042) and the eosinophil ratio (r = 0.123, P = 0.044). Neovascularization was correlated to the monocyte ratio (r = 0.324, P = 0.018). Finally, the fibrous proliferative membrane was correlated to the monocyte ratio (r = 0.418, P = 0.002). Only the eosinophil ratio was independently associated with proliferative DR (odds ratio = 1.25, 95% confidence interval: 1.04–1.51, P = 0.018).ConclusionThe results of UWFA imaging in patients with DR are correlated with white blood cell population indexes. The eosinophil ratio was independently associated with proliferative DR.

Late contralateral recurrence of retinal detachment in incontinentia pigmenti: A case report

BACKGROUNDIncontinentia pigmenti (IP) is a rare X-linked genetic disease. It mainly manifests as skin lesions and causes problems in the eyes, teeth, bones, and central nervous system. Of the various ocular manifestations, the most severe with difficult recovery is retinal detachment (RD). Here, we report an unusual case of bilateral asymmetrical RD. CASE SUMMARYWe present the case of an 11-year-old Chinese girl with IP who complained of sudden blurring of vision in the left eye. At that time, she had been blind in her right eye for 4 years. RD with traction was observed in both eyes. A massive retinal proliferative membrane, exudation, and hemorrhage were seen in the left eye. We performed vitrectomy in her left eye. Her visual acuity recovered to 20/50, and her retina had flattened within 2 d after surgery. During the 3-mo follow-up, we performed retinal laser treatment of the non-perfused retinal area in her left eye. Eventually, her visual acuity returned to 20/32, and no new retinal abnormalities developed. CONCLUSIONIn patients with IP with fundal abnormalities in one eye, it is important to focus on the rate of fundal change in the other eye. RD in its early stages can be effectively treated with timely vitrectomy and laser photocoagulation.

Wide-field Fundus Imaging and Fluorescein Angiography Findings in Various Pseudoretinoblastoma Conditions.

To report wide-field fundus imaging and fluorescein angiography findings in conditions mimicking retinoblastoma (pseudoretinoblastoma). The clinical and imaging records of 28 patients (36 eyes) imaged with RetCam 3 (Clarity Medical Systems, Inc) wide-field fundus photography and fluorescein angiography who were diagnosed as having various pseudoretinoblastoma disorders between February 2020 and August 2021 were retrospectively evaluated. Most patients were referred with suspicion of retinoblastoma. Other patients had unresolved eye conditions and received initial treatment elsewhere and they were referred later for exclusion of retinoblastoma. On RetCam fundus photography and fluorescein angiography, pseudoretinoblastoma conditions were diagnosed as Coats disease (9 eyes), congenital glaucoma (3 eyes), persistent fetal vasculature (3 eyes), familial exudative vitreoretinopathy (2 eyes), retinopathy of prematurity (2 eyes), myelinated retinal nerve fibers (2 eyes), optic nerve hypoplasia (2 eyes), oculocutaneous albinism (2 eyes), meridional fold (2 eyes), combined hamartoma of retina and retinal pigment epithelium (2 eyes), grouped congenital hypertrophy of the retinal pigment epithelium (1 eye), retinal astrocytic hamartoma (1 eye), morning glory syndrome (1 eye), optic glioma (1 eye), giant choroidal nevus (1 eye), vasculitis (1 eye), and chorioretinitis (1 eye). RetCam imaging and fluorescein angiography demonstrated telangiectasias, microaneurysms, submacular exudation/fibrosis, capillary non-perfusion, and exudation posterior to telangiectasias in Coats disease. Hypofluorescent optic discs, peripheral non-perfusion, and vascular leakage were observed in congenital glaucoma. Large areas of capillary non-perfusion, poor foveal morphology/formation, elongated ciliary processes with hyperfluorescent central core, and hyperfluorescent fibrovascular stalk at the posterior pole were hallmarks of persistent fetal vasculature. RetCam wide-field fundus imaging and fluorescein angiography play an important role in the diagnosis of conditions simulating retinoblastoma. [J Pediatr Ophthalmol Strabismus. 2023;60(1):60-74.].

Optical Coherence Tomography with Angiography in the Diagnosis of Changes in the Organ of Vision at Atherosclerosis

Background. The study of the parameters of optical coherence tomography with angiography (OCTA) broadens the understanding of the state of hemodynamics for the early detection of vascular disorders of the organ of vision in atherosclerosis (AS).Purpose. To study the parameters of OCTA with changes in the organ of vision against the background of AS.Patients and methods. 26 patients (41 eyes) with changes in the organ of vision in AS. The age contingent of patients ranged from 32 to 78 years, the average age was 47.5 ± 2.0 year, of which 10 were women, 16 men. Depending on the damage to target organs, all patients were conditionally divided into 2 groups: group I (19 eyes) included patients without damage, and group II (22 eyes) consisted of patients with target organs damage.Results. The registration of OCTA in patients of group I, a decrease in the density of capillaries of the superficial vascular network was 15 % and amounted to 45.21 ± 2.62 % (p &lt; 0.05), of the deep vascular plexus by 19 %, which amounted to 45.89 ± 2, 71 % (p &lt; 0.05). In the macular region, the area of the hypoperfused retina was 1.07 ± 0.14 mm2 (p &lt; 0.05). In the region of the optic nerve disc, areas of hypoperfusion were noted in the superficial layers in 7 eyes and in the deep layers in 4 eyes. Analysis of OCTA in patients of group II revealed a sharp decrease (by 48 %) in the density of capillaries in both the superficial and deep vascular plexuses of the retina, which amounted to 33.91 ± 3.01 % (p &lt; 0.05); 33.65 ± 2.89 % (p &lt; 0.05), respectively. In the macular region, the area of the non-perfused retina was 2.19 ± 0.21 mm2 (p &lt; 0.05). In the area of the optic nerve disc, areas of nonperfusion were noted both in the surface layers and in the deep layers in 4 eyes.Conclusion. The use of OCTA allows to detect changes in hemoperfusion in all layers of the retina and optic nerve in the early stages of AS development, which will allow early diagnosis and monitoring of the disease.

Non-Perfusion Area Index for Prognostic Prediction in Diabetic Retinopathy.

Fundus fluorescent angiography is a standard examination in Japan that can directly visualize the circulatory failure in diabetic retinopathy but is not used in Western countries. In this study, we examine the relationship between the non-perfusion area in fundus fluorescent angiography and the progression of diabetic retinopathy. We evaluated 22 eyes between 22 patients who had their first fundus fluorescent angiography during a clinical episode at Keio University Hospital from January 2012 to May 2015, were diagnosed as having preproliferative diabetic retinopathy, and could be followed for at least three years. The non-perfusion area index (%) in nine segmented fundi in the initial fundus fluorescent angiography was calculated, and the progression to proliferative diabetic retinopathy over three years was evaluated. Three out of the 22 eyes (13.6%) developed proliferative diabetic retinopathy over three years. The non-perfusion area index for the initial fundus fluorescent angiography was significantly associated with progression to proliferative diabetic retinopathy. The non-perfusion area index in the posterior pole was most strongly correlated with the progression to proliferative diabetic retinopathy. Thus, the non-perfusion area index in the posterior pole among those with preproliferative diabetic retinopathy may predict the progression to proliferative diabetic retinopathy in the subsequent three years.

Baseline retinal vascular bed area on ultra-wide field fluorescein angiography correlates with the anatomical outcome of diabetic macular oedema to ranibizumab therapy: two-year analysis of the DAVE Study.

To determine the relationship between baseline retinal non-perfusion area (NPA) and retinal vascular bed area (RVBA) on ultra-wide field fluorescein angiography (UWF FA) and long-term response to intravitreal ranibizumab therapy in diabetic macular oedema (DMO). A post-hoc, 2-year observational case series. Baseline UWF FA images (Optos 200Tx) of 40 eyes from 29 patients with diabetes mellitus and treatment naïve DMO in the DAVE (NCT01552408) study were montaged and stereographically projected at the Doheny Image Reading Center to adjust for peripheral distortion. The retinal vasculature was automatically extracted to calculate RVBA. NPA was manually delineated by two masked certified graders. RVBA and NPA were computed in mm2 automatically by adjusting for peripheral distortion and then correlated with the severity of DMO. While global NPA at baseline was not correlated to retinal thickness measurements, baseline NPA in the superior retina was associated with the macular volume (MV) improvement (P = 0.022). Multivariate analysis revealed a smaller RVBA at baseline was correlated with a better MV outcome at two-year follow-up after adjusting for confounding factors (P = 0.049). Eyes with smaller baseline RVBA appear to have a better long-term anatomic outcome of DMO.