Surgical management for treatment-resistant cases of inflammatory exudative retinal detachment: Mission impossible?

Purpose: To report the outcome of surgical intervention for inflammatory, exudative retinal detachment (ERD). Methods: A retrospective analysis of eyes with ERD that underwent vitrectomy. Results: Twelve eyes (10 patients) with ERD, non?responsive to medical therapy, underwent vitrectomy. The mean age was 35.7 ± 17.7 years. Five eyes (42%) had Vogt–Koyanagi–Harada disease, three (25%) had presumed tuberculosis (TB), two (17%) pars planitis, and one (8%) had sympathetic ophthalmia. The mean time of vitrectomy was 6.76 ± 4.1 months after onset. Six (50%) eyes had a recurrence, two settled with medical treatment, and four underwent re?surgery. The mean follow?up was 2.7 years. At the last visit, 10 (83.3%) eyes had attached retina; the best?corrected visual acuity (BCVA) had reduced to 1.6 ± 0.7 logarithms of the minimum angle of resolution (logMAR) from 1.3 ± 0.7 at baseline. Conclusion: Vitrectomy in ERD can act as an adjuvant to conventional medical therapy and help maintain structural integrity. Early vitrectomy may help preserve visual function.

Efficacy of scleral buckling for retinal detachment secondary to familial exudative vitreoretinopathy / 中华实验眼科杂志

Objective:To evaluate the efficacy of scleral buckling in the treatment of retinal detachment (RD) secondary to familial exudative vitreoretinopathy (FEVR).Methods:An observational case series study was conducted.A total of 37 patients (42 eyes) of RD secondary to FEVR who were treated with scleral buckling in Beijing Tongren Hospital from July 2010 to March 2021 were enrolled.There were 30 males (35 eyes) and 7 females (7 eyes), with an average age of (15.21±5.42) years old.Scleral buckling under general anesthesia was performed in all patients.There were 22 eyes with rhegmatogenous RD (RRD), of which 21 eyes were treated with local external compression combined with cerclage, and 1 eye was treated with radial spinal compression.There were 13 eyes with tractive RD (TRD), of which 12 eyes were treated with local external compression combined with cerclage and subretinal fluid drainage, and 1 eye was treated with scleral buckling combined with vitrectomy.There were 7 eyes with RRD combined with TRD, of which 4 eyes were treated with local external compression combined with cerclage and subretinal fluid drainage, and 3 eyes were treated with scleral buckling combined with vitrectomy.The average follow-up time was (30.61±10.50) months.The main outcomes were best corrected visual acuity (BCVA) of the operated eye converted to the logarithm of the minimum angle of resolution, retinal reattachment rate, and incidence of complications.The study adhered to the Declaration of Helsinki and was approved by the Ethics Committee of Beijing Tongren Hospital, Capital Medical University (No.TRECKY2018-056-GZ[2022]-07). Written informed consent was obtained from each subject or their guardians before entering the cohort.Results:The average BCVA was 0.83±0.50 at last follow-up after surgery which was better than 1.10±0.39 before surgery, and the difference was statistically significant ( t=6.639, P<0.001). There were 39 eyes with retinal reattachment and 3 eyes without retinal reattachment.The reattachment rate was 95.45%(21/22) in RRD, 84.62%(11/13) in TRD, and 100%(7/7) in RRD combined with TRD.No serious complication occurred in any patients during postoperative follow-up. Conclusions:On the premise of optimized surgical strategy based on the indications of RD secondary to FEVR, scleral buckling has a high retinal reattachment rate in the treatment of RD secondary to FEVR.

Causality between type 2 diabetes mellitus and age-related macular degeneration: a Mendelian randomization study / 国际眼科杂志(Guoji Yanke Zazhi)

AIM: To analyze the causality between type 2 diabetes mellitus(T2DM)and age-related macular degeneration(ARMD)based on two-sample Mendelian randomization(MR).METHODS: T2DM and ARMD samples were extracted from the FinnGen database. Inverse variance weighted(IVW)was used as the main analysis method, MR-Egger and weighted median(WM)as supplementary methods to analyze the potential relationship between them. In addition, Cochran Q test and MR-Egger intercept were also used to analyze the sensitivity, and the P-value was used as the index of research results.RESULTS: IVW showed that T2DM was associated with the incidence of exudative ARMD(OR=1.14, 95%CI 1.01～1.28, P=0.021), but it was not significantly associated with the incidence of atrophic ARMD(OR=0.96, 95%CI 0.86～1.07, P=0.554). The results of sensitivity analysis confirmed that there was no heterogeneity and pleiotropy in this study, and the results were reliable.CONCLUSION: There is a causal relationship between T2DM and exudative ARMD. Considering the high rate of blindness caused by ARMD, it is of great significance to recognize and control the risk factors of ARMD to reduce its prevalence rate and early diagnosis and treatment.

Research progresses on pathogenic genes and related signal pathways of familial exudative vitreoretinopathy / 中华眼底病杂志

Familial exudative vitreoretinopathy (FEVR) is a serious hereditary retinal vascular disease. The clinical manifestations vary, and the severity of the patients' condition is different. In severe cases, it may lead to bilateral blindness. The pathogenic mechanism of FEVR is also complex. At present, more than ten classical and candidate pathogenic genes have been found: NDP, FZD4, LRP5, TSPAN12, CTNNB1, KIF11, ZNF408, RCBTB1, LRP6, CTNNA1, CTNND1, JAG1, ATOH7, DLG1, DOCK6, ARHGP31 and EVR3 region. These pathogenic genes are involved in Wnt/β-catenin signaling pathway, norrin/β-catenin pathway and Notch pathway. They regulate and affect the development of retinal blood vessels, hyaloid vascular system regression, endothelial cell connections, and blood retinal barrier homeostasis, ultimately leading to the occurrence and development of FEVR disease.

The macular structure and blood flow in familial exudative vitreoretinopathy with inner retinal layer persistence / 中华眼底病杂志

Objective:To compared the changes of macular microvascular architecture in early stage familial exudative vitreoretinopathy (FEVR) patients with inner retinal layer (IRL) persistence and without IRL persistence.Methods:A retrospective clinical study. From 2017 to 2022, 94 patients with stage 1 FEVR with or without IRL residue and 45 age- and sex-matched healthy volunteers with 45 eyes (normal control group) who were confirmed by ophthalmology examination in Hangzhou Hospital of Optometry Affiliated to Wenzhou Medical University and Zhejiang Provincial People's Hospital were included in the study. According to whether there was IRL residue, the patients were divided into IRL group and non-IRL group, with 22 patients (22 eyes) and 72 patients (72 eyes), respectively. Best corrected visual acuity (BCVA) and optical coherence tomography angiography (OCTA) were performed in all eyes. Superficial vessel density (SCP) and deep vessel density (DCP) of whole image, fovea and parafovea, the area and perimeter of fovea avascular area (FAZ), A-circularity index (AI, perimeter/standard circle perimeter with equal area) and vessel density around the 300 μm width of the FAZ (FD), central macular thickness (CMT) on macular 3 mm × 3 mm scan on OCTA were measured.Results:SCP and DCP of whole image ( F=10.774, 4.583) and parafovea ( F=10.433, 3.912), CMT ( F=171.940) in IRL group and non-IRL group on macular 3 mm × 3 mm scan on OCTA were significantly lower than that in normal persons ( P<0.05). There were significant differences among three groups of the area of FAZ ( F=4.315), AI ( F=3.413), FD-300 ( F=13.592) ( P<0.05). BCVA were worst in IRL group ( P<0.05). Conclusions:Blood flow density decreased in macular area of FEVR patients. CMT is significantly thicker than normal population. The FAZ area of the foveal IRL residual eyes is small and irregular, with worse BCVA and lower macular blood density.

Progress in clinical features and treatment of perifoveal exudative vascular anomalous complex / 中华眼底病杂志

Perifoveal exudative vascular anomalous complex (PEVAC) is a very rare macular vascular disease characterized by an isolated large aneurysmal lesion in the fovea, with accompanied by small retinal hemorrhage and exudation. The main clinical symptoms of the patients are various degree of impaired vision. Clinically, it is often confused with type 1 macular telangiectasia and type 3 macular neovascularization. A thorough understanding of the clinical features of PEVAC is particularly important for its differential diagnosis. Due to the unclear pathogenesis of PEVAC, there is no specific treatment for the cause of disease. Most scholars use intravitreal injection against vascular endothelial growth factor drugs for treatment, but can not improve patients' visual acuity. At present, many attempts have been made to eliminate abnormal exudation of the lesion, maintain visual function and achieve a good prognosis by simple or combined laser photocoagulation. At present, it is still necessary to explore the pathogenesis of PEVAC, improve the understanding of the disease, and find a better treatment plan.

Multimodal imaging features of perifoveal exudative vascular anomalous complex / 中华眼底病杂志

Objective:To observe the multimodal imaging features and explore the treatment of parafoveal exudative vascular anomaly complex (PEVAC).Methods:A retrospective study. Six patients (6 eyes) with PEVAC diagnosed in Tianjin Eye Hospital were included in this study from July 2018 to December 2021. All patients were female with monocular disease. The age was (61.1±9.3) years. All patients showed a sudden painless decline in monocular vision with metamorphopsia. All patients underwent best corrected visual acuity (BCVA), color fundus photography, fundus fluorescein angiography (FFA), optical coherence tomography (OCT) and OCT angiography (OCTA). Indocyanine green angiography (ICGA) was performed in 4 eyes. In 6 eyes, 3 eyes were treated with intravitreal injection of anti-vascular endothelial growth factor drug; 5 eyes were treated with micropulse laser photocoagulation and/or local thermal laser photocoagulation; 1 eye was treated with photodynamic therapy. Five patients were followed up for (9.2±7.4) months, and 1 patient was lost. At follow-up, the same equipment and methods were used as at the initial diagnosis. The clinical manifestations, multimodal image features and treatment response were observed.Results:Baseline BCVA of affected eyes were ranged from 0.1 to 0.5. PEVAC was isolated in 6 eyes, and the fundus showed isolated hemangioma-like leision, accompanied by small bleeding and hard exudation. There were 2 isolated hemangiomatous lesions adjacent to each other in 2 eyes. In the early stage of FFA, punctate high fluorescence lesions near the macular fovea were seen, and the leakage was enhanced in the late stage. There was no leakage in the early stage of ICGA, or slight leakage with late scouring. OCT showed an oval lesion with high reflection wall and uneven low reflection. The central macular thickness (CMT) was (431±76) μm. OCTA showed blood flow signals in PEVAC, 2 eyes in the superficial capillary plexus (SCP), and it was also observed in the deep capillary plexus (DCP), but the intensity of blood flow signal was slightly weaker than that in the SCP. The blood flow signal was visible only in DCP in 2 eyes. SCP and DCP showed similar intensity of blood flow signals in 2 eyes. After treatment, the bleeding was absorbed basically in 4 eyes, the hard exudation partially subsided, the CMT decreased, the intercortical cystic cavity of the fovea nerve decreased, the hemangiomatous lesions narrowed, and BCVA increased. In 1 eye, the macular sac was reduced and partially absorbed by hard exudation, which was later relapsed due to blood pressure fluctuation.Conclusions:The majority of PEVAC patients had monocular onset. The fundus is characterized by solitary or structure with strong reflex walls, with or without retinal cysts, hard exudates, and subretinal fluid, and visible blood flow signals inside.

Clinical features and treatment of glaucoma secondary to familial exudative vitreoretinopathy / 中华眼底病杂志

Objective:To investigate the etiology, clinical features and treatment of familial exudative vitreoretinopathy (FEVR) secondary glaucoma.Methods:A retrospective clinical study. From January 1, 2016 to January 1, 2022, 15 patients (17 eyes) were diagnosed with FEVR secondary glaucoma in Beijing Tongren Hospital, Capital Medical University were included in the study. All patients underwent systematic ophthalmological evaluation. According to the patient's age, visual acuity, intraocular pressure, anterior segment, vitreous body and retina condition, the choice of translimbal lensectomy combined with vitrectomy, goniectomy, cyclophotocoagulation, intravitreal injection of anti-vascular endothelial growth factor (VEGF) treatment were chosen. The follow-up time was 3 to 37 months. The clinical characteristics of the affected eye, and the changes of intraocular pressure, anterior chamber depth and complications after surgery were observed.Results:Among the 15 patients, there were 11 males with 13 eyes, and 4 females with 4 eyes. Age was 6.14±7.37 years old. FEVR stages 2B, 3B, 4A, 4B, 5A, and 5B were 1, 1, 5, 6, 3, and 1 eye, respectively. The intraocular pressure of the affected eye was 42.74±9.06 mm Hg (1 mm Hg=0.133 kPa). All eyes had shallow anterior chamber and angle closure, anterior or posterior iris adhesions, lens opacity, retinal detachment, iris neovascularization in 4 eyes, and vitreous hemorrhage in 2 eyes. Sixteen eyes were treated with translimbal lensectomy combined with vitrectomy and goniotomy, of which 8 eyes were treated with anti-VEGF treatment; 1 eye was treated with cyclophotocoagulation combined with anti-VEGF treatment. After operation, the intraocular pressure of 16 eyes returned to normal range, and the depth of anterior chamber of 16 eyes returned to normal, and no obvious complications occurred.Conclusions:The main etiology of secondary glaucoma in FEVR is the structural and functional abnormalities of the anterior chamber and angle, which are found in the 2B and above stages of FEVR. The lensectomy and vitrectomy via limbal approach can effectively control the intraocular pressure and restore the anterior chamber, with no serious complications.

Analysis of factors affecting the clinical features of familial exudative vitreoretinopathy / 中华眼底病杂志

Objective:To observe and investigate the related factors that might affect clinical features of familial exudative vitreoretinopaty (FEVR) patients.Methods:A retrospective chart study. From January 2012 and December 2021, 42 patients with 84 eyes with a diagnosis of FEVR from Department of Ophthalmology, Peking University People's Hospital were included in the study. The patients came from 42 separate families. There were 31 males and 11 females, with an average age of first diagnosis was 16.6±33.7 months. There were 21 patients referred from other hospitals for the fundus disease found in eye screening after birth, 21 patients were first seen in our hospital. There were 4 and 38 premature and full-term infants, respectively. Two patients with a positive family history of FEVR. All patients are FEVR stages 1-5. The wide-angle digital pediatric retinal imaging system after general anesthesia for fluorescein fundus angiography (FFA) examination were performed for patients aged <5 years. If patients ≥ 5 years old, routine FFA examination was performed. Sixty-eight first-degree relatives from 28 families undergo routine fundus examinations and FFA examination. Genetic examination was performed for 26 families, including 26 probands and 57 first-degree relatives. Genetic examination were performed on gene the coreceptor of low density lipoprotein receptor-associated protein 5 ( LRP5), Wnt receptor coiled protein 4 ( FZD4), Norrie disease ( NDP), tetraporin 12 ( TSPAN12), catenin β1 ( CTNNB1) genes known to be involved in FEVR. The clinical features and the genotype of FEVR were observed in relation to the clinical phenotype. Results:Among the 42 patients, 13 patients were first observed by strabismus and nystagmus, with the median age of 12 months. Eight patients were complained non-chasing or vision-related symptoms. Among the 84 eyes, FEVR stage 1 or 2, 3 or 4, and 5 were 50 (59.5%, 50/84), 31 (36.9%, 31/84), and 3 (3.6%, 3/84) eyes, respectively. Among the 23 patients who were > 3 months at first diagnosis, 16 patients had at least one eye severer than stage 3 (69.6%, 16/23). Of the 68 first-degree relatives, 22 (32.4%, 22/68) had FEVR-like changes. Among the 26 families that underwent genetic detection, 13 families (50%, 13/26) of 16 variants of FEVR-related genes were detected, of which 10 mutations of LRP5 gene were the most common. There were 10 families with single gene mutations, including 6, 2 and 2 families of LRP5, FZD4 and CTNNB1 genes, respectively. One family of LRP5 gene mutations were compound heterozygous mutations, 1 family with LRP5 gene mutaition combined with NDP gene mutation, and 1 family with LRP5 and TSPAN12 gene mutation. Among the proband with FEVR pathogenic genes, 6 cases with similiar stage of both eyes, and 7 cases with inconsistent disease stages, and there was no obvious correlation between gene mutations and clinical phenotypes. Conclusion:In addition to the age of first diagnosis, no exact factors affecting the clinical manifestations of FEVR are found, and the association between clinical phenotypic and genetic heterogeneity still needs to be further explored.

Clinical presentation and treatment outcomes of an algorithmic approach to uveal effusion syndrome

Purpose: To describe the clinical features and treatment outcomes in spontaneous uveal effusion syndrome (UES). Methods: A 10?year retrospective chart review of UES patients from a tertiary eye center was carried out. Optical coherence tomography (OCT), fundus fluorescein angiography, and ultrasound biomicroscopy (UBM) scans were performed. UES was managed based on presenting best?corrected visual acuity (BCVA), symptoms, and fundus findings. Patients with secondary causes of uveal effusion were excluded. Results: Twenty?five eyes of 16 patients were included. Of the 16 patients, 14 (88%) were male and 9 (56%) had bilateral disease. Fifteen of 25 affected eyes had nanophthalmos (axial length (AL) <20.5 mm) and 6 had hyperopia with AL >20.5 mm. The presenting mean distance BCVA was 0.74 ± 0.64 logMAR (mean Snellen: 20/100). Eleven eyes had exudative retinal detachment, and 4 also had exudative choroidal detachment (CD). Choroidal thickness (CT) was increased in 11 eyes on B?scan ultrasonography, and the mean CT was 1.74 ± 0.38 mm. Sub?retinal fluid (SRF) and retinal folds were the most common OCT findings. UBM findings included shallow angles, peripheral CD, and supra?ciliary effusion. A combination of local and systemic corticosteroids was used to successfully treat 12 eyes, 6 needed surgery, and 7 were observed. Partial sclerectomy with anterior chamber maintainer?assisted SRF drainage was the favored surgery. The median period of follow?up was 6.5 months (0.1–76 months), and the mean distance BCVA at the last follow?up was 0.58 ± 0.42 logMAR (mean Snellen: 20/80). Conclusion: UES can be suitably managed both medically and surgically based on clinical presentation

Medical Thoracoscopy: Diagnostic Role in the Management of Undiagnosed Pleural Effusions and its Complications

Introduction: Cases of undiagnosed exudative pleural effusions are common in clinical practice and pose a diagnostic challenge for pulmonologists. Medical thoracoscopy allows both direct visualizations of pleural space for diagnostic evaluation and chemical pleurodesis for therapeutic purposes. Objectives: This study investigated the diagnostic role of medical thoracoscopy in the cases of undiagnosed exudative pleural effusions and complications of thoracoscopic pleural biopsy. Patients and methods: Between December 2016 and August 2019, 195 patients of undiagnosed exudative pleural effusions underwent medical thoracoscopy in our institute. Pleural biopsies were taken and sent for histopathological and microbiological examination. Results: The diagnostic yield of medical thoracoscopy in this study was 89.7%. Definite diagnosis was achieved in 175 out of 195 patients of the study population and only 20 (10.3%) patients were failed to be diagnosed by medical thoracoscopy. Histopathological results of thoracoscopic pleural biopsy among the study population revealed tubercular pleuritis in 31.79% (62 patients), metastatic adenocarcinoma in 23.07% (45 patients), malignant mesothelioma in 18.46% (36 patients), parapneumonic effusions in 6.66% (13 patients), metastatic squamous cell carcinoma in 5.64% (11 patients), small cell carcinoma in 3.07% (6 patients), malignant lymphoma in 0.51% (1 patient), and rheumatoid pleuritis in 0.51% (1 patient). Only 19.4% (38 patients) had minor complications like pain, minor bleeding, subcutaneous emphysema, and re-expansion pulmonary edema. Conclusion: Thoracoscopy is a safe, well-tolerated procedure with minimal risk allowing the accurate diagnosis of undiagnosed pleural effusion. Besides determining the underlying cause, it also provides unique therapeutic approaches like pleurodesis to patients with malignant pleural effusions.

Viruela símica y faringitis exudativa en Argentina / Monkeypox and exudative pharyngitis in Argentina

Resumen Desde mayo de 2022 se han notificado casos de viruela símica en países no endémicos con características diferentes a la descripción tradicional de la enfermedad, predominio en población de hombres que tienen sexo con hombres, posible transmisión sexual y ausencia de mortalidad a la fecha. Reporta mos el que sería el primero diagnosticado en Argentina sin nexo de viaje a países con circulación viral. Se trata de un hombre joven, bisexual, en profilaxis antirretroviral de pre-exposición al HIV, cuya forma de presentación fue faringitis exudativa sin otra etiología documentada y adenopatías que evolucionaron a la necrosis, presencia de lesiones asincrónicas características en cara, cuello, abdomen, extremidades y genitales. El diagnóstico fue por detección de ADN viral por reacción en cadena de la polimerasa en lesiones de piel, exudado de fauces y semen. El compromiso faríngeo con presencia de exudado luego del descarte de otras infecciones, podría ser una rareza para la forma epidémica de la enfermedad. El hallazgo del virus en semen ya reportado en otras publica ciones no es suficiente aún para confirmar la vía sexual como forma de trasmisión. En conclusión, nuestro caso alerta sobre otras posibles formas de presentación de la viruela símica epidémica y la necesidad de aumentar el nivel de sospecha para su detección precoz, como estrategia para evitar la diseminación y proteger a grupos vulnerables. Se requiere aún más información para determinar la transmisión sexual de esta enfermedad.

Abstract Since May 2022, monkeypox cases have been reported in non-endemic countries with different charac teristics from the traditional description of the disease, predominantly in men who have sex with men, with possible sexual transmission and with no documentation of mortality to date. We report what would be the first patient diag nosed in Argentina with no travel nexus to countries with viral circulation. Young, bisexual man, on antiretroviral HIV pre-exposure prophylaxis, whose presentation was exudative pharyngitis with no other documented aetiology and lymphadenopathy that progressed to necrosis, presence of characteristic asynchronous lesions on the face, neck, abdomen, extremities, and genitals, in a total of no more than 25. Polymerase-chain-reaction assay of samples from skin lesions, fauces exudate and semen were positive for monkeypox. Pharyngeal involvement with the presence of exudate after ruling out other infections could be a rarity for the epidemic form of the disease. The finding of the virus in semen, already reported in other publications, is still not enough to confirm the sexual route as a form of transmission. In conclusion: Our case warns about other possible forms of presentation of epidemic monkeypox and the need to increase the level of suspicion for its early detection as a strategy to prevent dissemination and protect vulnerable groups. Even more information is required to determine the sexual transmission of this disease.

Management and surgical outcomes of pediatric retinal detachment associated with familial exudative vitreoretinopathy – Our experience at a tertiary care ophthalmic center in North India

Purpose: To report the clinical profile, management, and long?term anatomical and visual acuity (VA) outcomes of pediatric macula?off rhegmatogenous retinal detachment (RRD) secondary to familial exudative vitreoretinopathy (FEVR). Methods: This was a prospective, interventional study of 14 eyes of 13 children aged ?18 years with macula?off FEVR?RRD. The primary outcomes were anatomical reattachment and VA changes. Results: The mean (±SD) age of the study population was 12.14 (±3.23) years (range 6–18 years) with a male preponderance (M:F – 10:3). Of the 14 eyes, 10 underwent vitrectomy with silicone oil injection, while four underwent scleral buckling surgery. Significant improvement in VA was noted at a mean (±SD) follow?up duration of 3.32 (±1.34) years, with the mean (±SD) LogMAR VA improving from 1.42 (±0.48) (Snellen equivalent 2/60; range from 6/36 to counting finger close to face [CFCF]) to 0.6 (±0.31) (Snellen equivalent 6/24; range 6/9–6/36) (P < 0.00001) at the final visit. Successful anatomical reattachment was achieved in 13/14 eyes (92.85%). Screening of the other eye and family members was performed for FEVR and treated with laser photocoagulation when deemed necessary (7/10 contralateral eye; 12/20 siblings; 0/24 parents). Conclusion: To conclude, RRD may arise in eyes with FEVR at a young age and with a male predilection in Indian population. Timely surgical intervention by scleral buckling procedure or vitrectomy, based on the patient profile, can achieve excellent anatomical and VA outcomes. Careful clinical and angiographic screening of the other eye and family members is vital.

Exudative retinal detachment as an initial presentation of retinopathy of prematurity: Clinical profile and outcomes of a rare presentation

Purpose: To describe the clinical profile and treatment outcomes of infants with retinopathy of prematurity (ROP) presenting with isolated exudative retinal detachment (ERD). Methods: Retrospective interventional case series. Preterm infants diagnosed with ROP with ERD at presentation were included. All demographic details, clinical findings, and treatment given were documented. The anatomical outcome was categorized as good, fair, and poor. Refractive outcome was classified into mild, moderate, and severe according to spherical equivalent at the last visit. Results: Fifteen eyes (8 patients) were included. Mean gestational age was 31.3 weeks, and birth weight was 1462.6 g. All eyes presented with aggressive ROP. Patches of retinal edema in avascular retina were seen in all eyes. A total of 86.6% of eyes had vascular sclerosis while 86.6% of eyes had subretinal exudates. The anatomical outcome was good in all eyes. In addition, 40% of eyes had a mild refractive error. Conclusion: Exudative retinal detachment in ROP is rare. The use of unregulated oxygen can be a contributory factor. Vascular sclerosis is consistent with hyperoxia?induced retinopathy models. Retinal edema and subretinal exudates indicate disrupted inner and outer blood?retinal barrier. Treatment outcomes are good when diagnosed and treated in time.

Retinopatía del prematuro con presentación atípica / Retinopathy of prematurity with atypical presentation

La retinopatía de la prematuridad es una enfermedad dinámica vasoproliferativa de la retina inmadura postnatal que afecta a los bebés prematuros. Cuando aparecen signos de atipicidad en su diagnóstico o evolución deben descartarse otras entidades vasculares de la retina, que generalmente tienen un trasfondo genético y semejan o coexisten con dicha entidad. Se presenta un caso con características de Retinopatía del prematuro y algunos signos de atipicidad. Se describe su manejo y evolución, así como una breve descripción de las entidades que conforman el diagnóstico diferencial(AU)

Retinopathy of prematurity is a dynamic vasoproliferative disease of the immature postnatal retina that affects premature babies. When signs of atypicality appear in its diagnosis or evolution, other vascular entities of the retina must be ruled out, which generally have a genetic background and resemble or coexist with said entity. We present a case with characteristics of Retinopathy of prematurity and some signs of atypicality. Its management and evolution are described, as well as a brief description of the entities that make up the differential diagnosis(AU)

Advances in the study of perifoveal exudative vascular anomalous complex / 中华眼底病杂志

Perifoveal exudative vascular anomalous complex (PEVAC) are class of diseases characterized by isolated aneurysmal lesions of the perifovea with hemorrhage and hard exudates, which are found in recent years. Its pathogenesis and pathological process are not yet fully elucidated. The current consensus through multimodal imaging studies is that PEVAC is an idiopathic perifoveal retinal vascular abnormality with cystoid macular edema (CME) and hard exudate. It is not easily distinguished clinically from retinal microaneurysm due to diabetic retinopathy or retinal vein occlusion, aneurysmal telangiectasia, and type 3 macular neovascularization (stage Ⅰ). PEVAC is a partially self-healing property, and some aneurysms and CME may resolve on their own during follow-up period. Laser photocoagulation is an effective measure for the treatment of PEVAC. In the future, with the rapid development of imaging technology, the progress of research on pathogenesis and treatment strategies and the updating of theories are expected to provide more effective treatments for PEVAC.

Parafoveal exudative vascular anomalous complex and its characteristics of multi-modal imaging in 11 cases / 国际眼科杂志(Guoji Yanke Zazhi)

AIM: To observe the imaging characteristics of multi-modal imaging in perifoveal exudative vascular anomalous complex(PEVAC).METHODS: A retrospective case analysis. There were 11 patients(11 eyes)diagnosed as PEVAC by multi-modal imaging in our hospital included in this study from February 2021 to February 2022. All patients underwent the examinations of best corrected visual acuity(BCVA), fundus photography, fluorescein fundus angiography(FFA), spectral-domain optical coherence tomography(SD-OCT)and optical coherence tomography angiography(OCTA).RESULTS: On fundus examination, six out of eleven eyes presented as a unilateral and isolated lesion with diameter ≥150μm, five out of eleven eyes presented as multiple lesions(ranging from 2 to 4)and the diameter of each lesion &#x003C;150μm, seven eyes with retinal edema and/or hard exudation. FFA revealed an oval-like hyper-fluorescent lesion of parafoveal in the late phase with enhancement but no leakage. Late peripheral telangiectasia leakage is seen with oedema. OCTA examination revealed a parafoveal small mass hyper-reflex showed aneurysmal dilation in the superficial and deep capillary, and capillary rarefaction was observed around the lesion. B-scan showed strong blood flow signal inside. Anti-vascular endothelial growth factor(VEGF)therapy was performed in 4 patients with macular edema. However, there was no notable improvement after treatment.CONCLUSION: Multi-modal imaging can help confirm the diagnosis and differential diagnosis of PEVAC, there is a poorly response to anti-VEGF therapy.

Perifoveal exudative vascular anomalous complex / 国际眼科杂志(Guoji Yanke Zazhi)

Parafoveal exudative vascular anomalous complex(PEVAC)refers to a large, isolated aneurysmal lesion in the superficial or deep capillary plexus of the parafoveal retina demonstrated by optical coherence tomography(OCT)imaging and has been identified as a new entity. Patients are almost middle-aged and elder people, not accompanied by retinal vascular diseases, but can develop with myopic or age-related macular degeneration(ARMD). It has been defined as PEVAC-resembling lesions when associated with retinal vascular diseases, such as diabetic retinopathy(DR)or retinal vein occlusion. It is non-exudative in the early stage, but later intraretinal exudation may induce metamorphopsia or visual decline. In some cases, the lesion was unchanged or spontaneously resolved during long-term follow-up.Differentiation from type 3 retinal neovascularization(retinal angiomatous proliferation, RAP)and Type I MacTel may be required. The anti-vascular endothelial growth factor(VEGF)therapy is ineffective and micropulse laser treatment could achieve improvement in a few cases. It is speculated that the lesion is associated with degeneration of retinal microvascular pericytes.

Research progress on pathogenic genes of familial exudative vitreoretinopathy / 国际眼科杂志(Guoji Yanke Zazhi)

@#Familial exudative vitreoretinopathy(FEVR)is a severe clinically and genetically heterogeneous retinal disease which characterized by abnormal development of the peripheral retinal vessels. FEVR presents many clinical phenotypes, the main and typical feature is retinal folds. There are various inheritance modes with high genetic heterogeneity of FEVR including autosomal recessive, X-recessive, autosomal dominant recessive, and other scattered inheritance modes. So far, nine FEVR pathogenic genes have been reported: NDP, FZD4, LRP5, CTNNA1, TSPAN12, ZNF408, KIF11, CTNNB1, and JAG1 genes. These genes are mainly involved in signaling pathways such as Wnt, Notch, and Norrin-β-catenin. This article reviews the above nine FEVR pathogenic genes and their signaling pathways.

Clinical characteristics and multimodal imaging of perifoveal exudative vascular anomalous complex / 中华眼底病杂志

Objective:To investigate the clinical features and multimodal imaging features of eyes with perifoveal exudative vascular anomalous complex (PEVAC).Methods:A retrospective case study. From February 2014 to November 2020, 7 eyes of 7 patients with PEVAC diagnosed by ophthalmology examination in Department of Ophthalmologyof Peking University People's Hospital were included in this study. There were 6 males and 1 female. The age was 60.1±9.1 years. All were monocular. The chief complaints of visual deformation and vision loss were 3 and 1 cases, respectively. All patients underwent best corrected visual acuity (BCVA), fundus color photography, optical coherence tomography (OCT), fundus fluorescein angiography (FFA). BCVA examination was performed using the standard logarithmic visual acuity chart, which was converted to logarithm of the minimum angle of resolution (logMAR) visual acuity. OCT angiography (OCTA) and indocyanine green angiography (ICGA) were performed in 4 and 2 eyes, respectively. Three eyes were treated with intravitreal injection of anti-vascular endothelial growth factor (VEGF) combined with local laser photocoagulation. Two eyes were treated with laser photocoagulation alone. The follow-up time was 16.7±19.1 months. During follow-up, relevant examinations were performed with the same equipment and methods as at the initial diagnosis. The multimodal imaging characteristics and treatment response of the affected eyes were observed.Results:The baseline logMAR BCVA was 0.33±0.19 (0.20-0.80). All eyes showed isolated hemangiomatous lesions in the macular fovea with rigid retinal exudation, and 2 adjacent isolated hemangiomatous lesions were observed in 1 eye. FFA and ICGA examination showed that all eyes with macular hemangiomatous lesions showed clear boundary and strong fluorescence in the early stage. No other retinal or choroidal vascular abnormalities were observed. On OCT examination, circular lumen-like structures with strong reflective wall near the fovea were observed in the macular region of all eyes, accompanied by intraretinal cystic lumen. The macular central retinal thickness (CMT) was 326±125 (207-479) μm. In the four eyes examined by OCTA, blood flow signals were observed in the circular lumenoid structures with strong reflective walls adjacent to the fovea. Blood flow signals were observed in the superficial capillary layer (SCP) and deep capillary layer (DCP) of the retina in 3 eyes. SCP showed blood flow signal in 1 eye. In 4 eyes treated with intravitreal injection of anti-VEGF drugs, there was no significant improvement in the intraretinal capsule space after treatment. Subretinal fluid absorption, retinal cystoid edema persisted, and rigid exudation decreased in 1 eye. CMT decreased and BCVA increased in 5 eyes treated with laser photocoagulation or laser photocoagulation alone. At last follow-up, logMAR BCVA was 0.16±0.06 (0.10-0.20) and CMT was 212±34 (154-252) μm. Compared with baseline, the difference of BCVA was statistically significant ( t=2.661, P=0.037). Conclusions:The fundus of PEVAC patients is characterized by solitary or multiple solitary hemangiomatous lesions in the macular fovea. Round lumenoid structures with strong reflective walls, with or without intraretinal cystic lumen, rigid exudate, and subretinal fluid, in which blood flow signals can be seen in OCT.