The approval of voretigene neparvovec-rzyl by the US Food and Drug Administration (FDA), in December 2017, marked the beginning of a new era in medicine in which many inherited diseases will be essentially corrected by gene therapy. Voretigene neparvovec-rzyl, with the trade name of Luxturna, is the first gene therapy for an inherited disease. It is intended for the treatment of RPE65 mutation-associated RETINAL dystrophy, making it the second commercially available treatment for inherited RETINAL disease (IRD) after RETINAL prostheses (Argus II and Alpha IMS) which were commercialized a few years earlier. Unlike RETINAL prostheses, voretigene neparvovec-rzyl modifies the course of the disease.

Purpose: To explore the correlation between RETINAL capillary non‑ perfusion and the distribution of RETINAL neovascularization and vascular leakage (VL) in patients with proliferative diabetic retinopathy (PDR). Methods: Ultra‑ widefield angiograms of 96 eyes of 69 patients with PDR were reviewed for the proportion of non‑ perfused area to total gradable area, and for the presence of neovascularization and VL. Results: RETINAL neovascularization was distributed as such: neovascularization elsewhere (NVE), 57. 3%; neovascularization of the disc (NVD), 11. 5%; both neovascularization of the disc and elsewhere (NVED), 31. 3%. The proportion of non‑ perfused retina, so‑ called ischemic index, was greater in eyes with NVED compared to eyes with NVE only, but not when compared to NVD only. Overall, 83% of eyes had VL. The presence and the extent of VL correlated with the proportion of the ischemic index. While VL and ischemic index were more severe in the mid-periphery and far-periphery, the majority of NVE was located in the posterior pole. Conclusions: The presence of both NVD and NVE is associated with a greater ischemic index than NVE alone. Although both VL and ischemic index is significantly higher in peripheral zones, the majority of neovascularization occurs at the posterior pole.

Background: Different criteria have been proposed to determine the mortality rate of primary intracerebral hemorrhage (ICH). This study aimed to evaluate ICH and Modified New ICH scores in predicting 30-day mortality in patients with primary ICH. Methods: In this prospective cohort study, 107 patients diagnosed with primary ICH were enrolled at an interval of six months (October 2015-March 2016). They were evaluated using Modified New ICH and ICH scores. The Modified New ICH score was different from the New ICH score since the National Institute of Health Stroke Scale (NIHSS) variables were replaced by Modified Rankin Scale (MRS) in the modified score. Results: A total of 61 patients (57%) died, and 46 (43%) survived during the 30-day hospitalization. ICH 2 and Modified New ICH 3 scores predicted 30-day mortality rate in patients with the sensitivity and specificity rates of 87 and 63 percent, and 88 and 53 percent, respectively. Conclusion: The current study showed that both ICH and Modified New ICH scores were almost equally effective in determining the mortality rate in patients with primary ICH, and both criteria had acceptable value in determining the mortality rate of patients. Therefore, routine assessment of ICH and Modified New ICH scores in patients with ICH in emergency wards is recommended.

The gold standard treatment for end-stage heart failure is heart transplantation; however, the rate of transplantation remains inadequate because of the paucity of organ donation. The left ventricular assist device (LVAD) has been used as a bridge therapy before transplantation. The LVAD is being used increasingly because it reduces mortality despite the accompanying morbidities. Therefore, the anesthetic management of LVAD-related morbidities is important and requires experience and knowledge. Herein, we describe a 60-year-old male patient with an LVAD with complaints of right hemiparesis, dysphasia, and facial paralysis. We aim to present the anesthetic management of a patient with intracranial hemorrhage who underwent LVAD exchange due to thrombosis.

Background & Aim: Endovascular neurosurgery is widely accepted as a useful current modality for the management of neurovascular disorders. The aim of this retrospective study was to evaluate the causes of diagnostic and therapeutic neurovascular procedures, and the prevalence of vas cular pathologies seen in these procedures.Methods & Materials/Patients: Endovascular neurosurgery department of Firoozagar hospital is a referral center of neurovascular disorders from all of country. The activity of this center was started in February 2013 and has continued until now, as the most active center for the management of neurovascular disorders. In this study, we analyzed the causes and results of 761 neuroendovascular procedures done on 598 patients, during the 18 month period of February 2013 to September 2014 in Firoozgar Hospital in Tehran, Iran. The mean age of the patients was (44.83 ± 18.73) years and 50.3% of them were females.Results: In total, 761 diagnostic and therapeutic neuroendovascular procedures were done on 598 patients in the first 18 months’ activity of this center. The mean age of the patients was (44.83 ± 18.73) years and 50.3% of them were females. The most prevalent cause of these procedures was spontaneous intracerebral HEMORRHAGES (27.6%). After that, ruling out vascular lesions (14.2%) due to suspicious MRI, and focal neurologic defects (11.2%) were the most common causes.This study also showed that 26 out of 598patients were treated for trigeminal neuralgia, 14 patients were treated for retinoblastoma, and 14 patients were treated for radiculopathies symptoms. The vascular angiographies done on the remaining 544 patients were due to diagnosis and treatment of vascular lesions of whom, 167 patients had normal angiographies and 377 patients had neurovascular pathologies. Intracranial aneurysms, AVMs and carotid stenosis formed respectively the most common types of vascular malformat ions in our study.Conclusion: Descriptive studies are basic for the complementary research and evaluation of results in referral centers and other new centers. In the first 18 months of activity of our center, favorable results were obtained, since about 70% of diagnostic angiographies showed vascular pathologi es.

Purpose: To report a case of congenital macular macrovessels Case report: An 8-year-old girl was referred for decreased vision in her right eye. Her best corrected visual acuity (BCVA) in the right eye was 20/200 and in the left eye 20/20. Anterior segment examinations were normal in both eyes. Posterior segment examination of the right eye showed abnormal macrovessels crossing the macula and passing over the fovea. Left funduscopy finding was insignificant. Conclusion: Congenital RETINAL macrovessels (CRM) are rare anomalies. They are aberrant RETINAL vessels, usually venules, present in the posterior pole and crossing the avascular foveal region. Most of these cases are unilateral and stable with excellent visual prognosis and are detected on routine examination. Foveal cysts, HEMORRHAGES and displaced foveola may be also associated with this anomaly. These entities must be distinguished from racemose angiomas, capillary hemangiomas of the retina and even when associated with neurological symptoms, should be considered as part of the Wyburn-Masson syndrome. In children with decreased visual Acuity, it should be treated promptly to avoid amblyopia. Regular examination and careful follow-up of vascular lesion is mandatory to limit possible complications.

Though microaneurysms are the hallmark of diabetic retinopathy (DR), large aneurismal changes termed as ''macroaneurysms'' (MAs) may also occur in the course of chronic diabetic macular edema. MAs are usually accompanied by intraRETINAL hard exudates, fluid accumulation and RETINAL HEMORRHAGES. Detection of MAs is clinically important as it implies that macular edema is usually chronic and therefore can be resistant to intravitreal anti-vascular endothelial growth factor injections. Multimodal imaging consisting of fluorescein angiography (FA), indocyanine green angiography (ICGA), optical coherence tomography (OCT) or OCT-angiography (OCTA) can be performed to detect and understand the nature of MA and thereby select proper treatment modality. Herein, we report multimodal imaging features of a 64-year-old woman with insulin-dependent diabetes mellitus presented with treatment naï ve severe macular edema and a macroaneurysm at the right temporal macula. In conclusion, FA, ICGA and OCT seem to be far superior to OCTA to detect these lesions due to probable slow flow inside the MA...