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Purpose: To present a 65-year-old patient with macular hole (MH) and submacular hemorrhage (SMH) secondary to a ruptured retinal arterial macroaneurysm (RAM) which was successfully treated with a novel surgical technique.Patient: A 65-year-old woman presented with a 1-week history of sudden-onset visual loss in her right eye. Her best-corrected visual acuity (BCVA) was hand motion in the right eye. Her fundus examination revealed intraretinal and massive subretinal hemorrhage with macular involvement. An MH with an approximate diameter of 600 microns was also noted. The patient was treated by a standard pars plana vitrectomy (PPV) and internal limiting membrane (ILM) peeling, followed by 10 µg recombinant tissue plasminogen activator injection into the submacular space via the MH with a 25-gauge vitrectomy probe. BCVA of the patient increased to 20/320 and 20/60, one and four weeks after surgery, respectively. Optical coherence tomography (OCT) images confirmed the complete anatomical closure of the MH and the restoration of the outer retinal layers such as the external limiting membrane and the ellipsoid zone.Conclusion: This case report expands our knowledge about the management of MH in combination with SMH after a ruptured RAM. We suggest the use of a vitrectomy probe and MH for subretinal recombinant tissue plasminogen activator (rtPA) injection.

Objective: To report a case of neurofibromatosis-1 (NF-1) with retinal vasoproliferative tumor (RVPT) and macular edema and exudation that was successfully treated with intravitreal bevacizumab (IVB).Method: A retrospective case report of patient with neurofibromatosis, retinal vasoproliferative tumor and macular edema who received three monthly intravitreal injections of bevacizumab. Optical coherence tomography (OCT) and fluorescein angiography (FAG) before and three months after treatment were done. Results: Macular edema and exudation of the right eye was effectively resolved with IVB injection and vascularity of RVPT significantly decreased after treatment with IVB. Conclusion: Macular edema and exudation secondary to RVPT in patients with NF-1 could be successfully treated with IVB.

Purpose: Gyrate atrophy of the choroid and retina (GACR) is a rare congenital disorder and mutations in the ornithine aminotransferase (OAT) gene has been specified as the underlying cause. Patients show a high level of ornithine in body fluids which may be controlled by low protein diets. Pyridoxine (vitamin B6) supplementation may also be effective, however, most patients appear to be nonresponsive to this modality of treatment. Case Report: Here, we report a characterized case of a vitamin B6-responsive GACR who had a splicing mutation in the OAT gene. The GACR diagnosis was confirmed through the clinical features, imaging, biochemical findings, and whole-exome sequencing (WES) results. WES data revealed the splicing mutation in intron 4 of the OAT gene (NM_001322967: c.425-1G>A). Conclusion: Our knowledge about the diagnosis and treatment of GACR can be improved by identifying novel mutations in the OAT gene and accurate follow-up of the patients to determine how they respond to treatment. [ABSTRACT FROM AUTHOR]

Diabetic retinopathy (DR) is one of the most complications of diabetes. It is a progressive disease leading to significant vision loss in the patients. Abnormal capillary nonperfusion (CNP) regions are one of the important characteristics of DR increasing with its progression. Therefore, automatic segmentation and quantification of abnormal CNP regions can be helpful to monitor the patient's treatment process. We propose an automatic method for segmentation of abnormal CNP regions on the superficial and deep capillary plexuses of optical coherence tomography angiography (OCTA) images using the marker-controlled watershed algorithm. The proposed method has three main steps. In the first step, original images are enhanced using the vesselness filter and then foreground and background marker images are computed. In the second step, abnormal CNP region candidates are segmented using the marker-controlled watershed algorithm, and in the third step, the candidates are modeled using an undirected weighted graph and finally, by applying merging and removing procedures correct abnormal CNP regions are identified. The proposed method was evaluated on a dataset with 36 normal and diabetic subjects using the ground truth obtained by two observers. The results show the proposed method outperformed some of the state-of-the-art methods on the superficial and deep capillary plexuses according to the most important metrics. [ABSTRACT FROM AUTHOR]

Purpose: To provide the clinical recommendations for the administration of intravitreal anti-vascular endothelial growth factor (VEGF) drugs especially bavacizumab for ocular vascular diseases including diabetic macular edema, neovascular age-related macular degeneration, myopic choroidal neovascularization, retinal vein occlusion and central serous chorioretinopathy. Methods: Twenty clinical questions were developed by the guideline technical committee. Relevant websites and databases were searched to find out the pertinent clinical practice guidelines to answer the questions. The technical committee provided possible answers (scenarios) according to the available evidences for each question. All scenarios along with their levels of evidence and the supported articles were sent to the experts for external review. If the experts did not agree on any of the scenarios for one particular clinical question, the technical committee reviewed all scenarios and their pertinent evidences and made the necessary decision. After that, the experts were asked to score them again. All confirmed scenarios were gathered as the final recommendations. Results: All the experts agreed on at least one of the scenarios. The technical committee extracted the agreed scenario for each clinical question as the final recommendation. Finally, 56 recommendations were developed for the procedure of intravitreal anti-VEGF injection and their applications in the management of ocular vascular diseases. Conclusion: The implementation of this guideline can standardize the management of the common ocular vascular diseases by intravitreal injection of anti-VEGF agents. It can lead to better policy-making and evidence-based clinical decision by ophthalmologists and optimal evidence based eye care for patients. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the effect of placental growth factor (PlGF) gene knockdown in a murine model of laser-induced choroidal neovascularization. Methods: Choroidal neovascularization was induced in the left eyes of 11 mice by infrared laser. Small interfering RNA (siRNA, 20 picomoles/10 μl) corresponding to PlGF mRNA was administered intravitreally by Hamilton syringe in all subjects. One month later, fluorescein angiography and histolologic examination were performed. Results: No leakage was apparent in the 11 eyes treated with siRNA cognate to PlGF. The results of histological evaluation were consistent with angiographic findings showing absence of choroidal neovascularization. Conclusion: Knockdown of the PlGF gene can inhibit the growth of laser-induced choroidal neovascularization in mice. [ABSTRACT FROM AUTHOR]

Purpose: To report a case of idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome associated with positive perinuclear antineutrophil cytoplasmic antibody (P-ANCA). Case Report: A 51-year-old man presented with loss of vision in his right eye since many years ago and blurred vision in his left eye over the past year. Ophthalmologic examination revealed optic atrophy and old vascular sheathing in the right eye and blurred disc margin, macular exudation, flame shaped hemorrhages, retinal vascular sheathing and multiple aneurysms at arterial bifurcation sites in the left eye, findings compatible with IRVAN syndrome. On systemic workup, the only notable finding was P-ANCA positivity. Conclusion: IRVAN syndrome may be a retinal component of P-ANCA associated vasculitis. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the demographic and clinical features of childhood pars planitis, and to determine the therapeutic and visual outcomes of the disease. Methods: Medical records of pediatric patients (less than 16 years of age at diagnosis) with pars planitis and at least 6 months of follow-up who were referred to Labbafinejad Medical Center, Tehran, Iran over a 22 year period were reviewed. Results: Overall, 117 eyes of 61 patients including 51 (83.6%) male subjects were included. Mean age at the time of diagnosis was 7.8±3.2 (range, 3-16) years. Mean best corrected visual acuity (BCVA) was 0.88±0.76 logMAR at presentation which improved to 0.39±0.51 logMAR at final visit (P<0.001). Endotheliitis was present in 23 (19.6%) eyes and was significantly more prevalent in subjects younger than 9 years (P=0.025). Cataract formation (41.9%) and cystoid macular edema (19.7%) were the most prevalent complications. Univariate regression analysis showed that better baseline visual acuity (OR=0.38, 95%CI 0.21-0.70, P=0.002), age older than 5 years at disease onset (OR=0.36, 95%CI 0.14-0.9, P=0.029), absence of endotheliitis (OR=0.39, 95%CI 0.15-0.99, P=0.047) and female gender (OR=3.77, 95%CI 1.03-13.93, P=0.046) were significantly associated with final BCVA of 20/40 or better. Conclusion: Childhood pars planitis was much more common among male subjects. Endotheliitis may be a sign of inflammation spillover and is more prevalent in younger patients. Visual prognosis is favorable in most patients with appropriate treatment. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the results of intravitreal expansile gas injection, with or without recombinant tissue plasminogen activator (rtPA), followed by intravitreal bevacizumab injection for treatment of submacular hemorrhage (SMH) secondary to neovascular age-related macular degeneration (AMD). Methods: In this interventional case series, 5 eyes of 5 patients with SMH secondary to choroidal neovascularization (CNV) due to neovascular AMD were treated with 0.3 cc intravitreal SF6 (and 50 μg of rtPA in two eyes), followed by face-down positioning; 24 hours later, 1.25 mg of bevacizumab was injected intravitreally. Main outcome measures included displacement of SMH and best corrected visual acuity (BCVA). Results: Mean patient age was 75.6±9.2 (range, 60-83) years, mean duration of symptoms was 6.4±3.2 (range, 3-10) days, and mean number of bevacizumab injections was 1.8 (range, 1-3). Mean preoperative BCVA was 1.28±0.27 logMAR which improved significantly to 0.57±0.33 logMAR at 12 months (P=0.042). SMH displacement occurred in all eyes, and visual acuity improved and remained stable during the follow-up period of 12 months. Conclusion: Intravitreal expansile gas injection, with or without rtPA, followed by intravitreal bevacizumab injection, seems to be an effective modality for SMH displacement and treatment of the underlying CNV in neovascular AMD. [ABSTRACT FROM AUTHOR]

Purpose: To investigate the retinal vascular characteristics among patients with different types of inherited retinal dystrophies (IRDs)., Methods: This comparative cross-sectional study was conducted on 59 genetically confirmed cases of IRD including 37 patients with retinitis pigmentosa (RP) (74 eyes), 13 patients with Stargardt disease (STGD) (26 eyes), and 9 patients with cone-rod dystrophy (CRD) (18 eyes). Both eyes of 50 age- and sex-matched healthy individuals were investigated as controls. All participants underwent optical coherence tomography angiography to investigate the vascular densities (VDs) of superficial and deep capillary plexus (SCP and DCP) as well as foveal avascular zone area., Results: In RP, significantly lower VD in whole image ( P = 0.001 for DCP), fovea ( P = 0.038 for SCP), parafovea ( P < 0.001 for SCP and DCP), and perifovea ( P < 0.001 for SCP and DCP) was observed compared to controls. In STGD, VD of parafovea ( P = 0.012 for SCP and P = 0.001 for DCP) and fovea ( P = 0.016 for DCP) was significantly lower than controls. In CRD, the VD of parafovea ( P = 0.025 for DCP) was significantly lower than controls. Whole image density was significantly lower in RP compared to STGD ( P < 0.001 for SCP) and CRD ( P = 0.037 for SCP). VD in parafovea ( P = 0.005 for SCP) and perifovea ( P < 0.001 for SCP and DCP) regions was significantly lower in RP compared with STGD. Also, foveal VD in STGD was significantly lower than RP ( P = 0.023 for DCP)., Conclusion: Our study demonstrated lower VDs in three different IRDs including RP, STGD, and CRD compared to healthy controls. Changes were more dominant in RP patients., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Journal of Current Ophthalmology.)