UNASSIGNED: In this study, we report a patient who presented with both chronic myelocytic leukemia (CML) and Susac syndrome (SS).
UNASSIGNED: A 45-year-old male diagnosed with CML in the blast phase sought consultation due to a deterioration in vision in his right eye. He also had hearing loss and severe migraneous headaches. Best corrected visual acuity was light perception and 20/20 in the right and left eyes, respectively. The slit lamp examination and intraocular pressure were within normal ranges for both eyes. Upon dilated fundoscopy, organized vitreous hemorrhage was observed in the right eye, while the left eye exhibited extensive sclerotic vessels with retinal neovascularization in the periphery. Ultrasound of the right eye showed tractional retinal detachment. Optical coherence tomography of the left retina showed thinning of the retina in temporal macula. Fluorescein angiography revealed a substantial nonperfused region in the peripheral left retina, accompanied by arterioarterial and arteriovenous collaterals, along with microaneurysms. MRI showed scattered foci of hyperintensity within the supratentorial white matter, mostly subcortical on T2-weighted and fluid-attenuated inversion-recovery. The patient received a diagnosis of SS and was subsequently referred to the neurology service for further assessment and potential treatment.
UNASSIGNED: SS may manifest as a presentation of CML. It is advisable to conduct investigations for SS in CML patients experiencing neurological, ophthalmological, or otological symptoms.

OBJECTIVE: Central and branch retinal artery occlusions are disabling ischemic strokes of the retina for which established acute treatments are lacking. This is the first published report of the use of intravenous tenecteplase (TNK) for retinal artery occlusion, in which we describe the clinical course of four patients with central retinal artery occlusion (CRAO) and one patient with branch retinal artery occlusion (BRAO).
METHODS: Patients were retrospectively recruited to the study from two stroke centers. Clinical course was determined from review of electronic medical records. The primary outcomes of interest were short and long term complications as well as visual acuity at presentation and at any subsequent follow up.
RESULTS: There were no hemorrhagic complications. None of the four patients with CRAO experienced functional visual recovery (defined as improvement to 20/100 or better). The patient with BRAO had functional visual recovery.
CONCLUSIONS: Intravenous TNK may be a safe and reasonable treatment for CRAO and BRAO.

Eales disease is an idiopathic retinal vasculitis that mainly affects the periphery of the retina. The disease commonly manifests as peripheral retinal perivasculitis, peripheral retinal capillary nonperfusion, neovascularization, and recurrent vitreous hemorrhage. Here, we present the case of a 36-year-old male who was diagnosed with Eales disease after presenting with sudden onset flashes of light, reduced visual acuity, and a black spot in his left eye. Upon examination, his left eye exhibited a superior non-foveal branch retinal artery occlusion (BRAO) with a sludged blood column, an old extramacular branch retinal vein occlusion (BRVO) with hemorrhage, and vascular sheathing. Initial laboratory investigations, including antibody testing for causes of retinal ischemia and stroke workup, were negative. Later, the patient presented with a BRAO in the right eye and a cerebral infarction shortly thereafter, further complicating his clinical picture. A diagnosis of Eales disease was made based on the evolution of retinal findings showing peripheral non-perfusion, vascular sheathing, collateral formation, neovascularization with leakage, absence of additional BRAOs following repair of his patent foramen ovale, and lack of other explanatory conditions. The initiation of systemic corticosteroids resulted in the improvement and stabilization of his vision. This case highlights the challenges in diagnosing Eales disease, underscoring the importance of timely identification for the appropriate management and prevention of vision loss.

A rarely described condition known as branch retinal artery occlusion (BRAO) with concurrent obstruction of the central retinal vein (CRVO) is characterized by diffuse retinal hemorrhages, dilated and tortuous retinal veins, macular and disc edema, cotton wool spots, and a generalized delay in arteriovenous transit on fluorescein angiography, together with a retinal whitening in the area of the affected retinal arterial branch. Although BRAO and CRVO may share underlying systemic risk factors, the pathogenesis of combined BRAO + CRVO is still unknown. We present a BRAO + CRVO case report concerning a 63-year-old white male who came to our observation complaining of sudden vision loss in his right eye. An increased risk for thrombotic event was revealed in this case, and the patient improved only with systemic anticoagulant therapy and in the absence of ocular therapy. We also explain all the clinical findings that are detectable using different diagnostic devices and analyze the scientific literature for other, similar clinical cases.

UNASSIGNED: Retinal artery occlusion (RAO) is an ophthalmic emergency that can lead to poor visual outcomes and is associated with an increased risk of stroke and cardiovascular events. Wide-field swept-source OCT-A (WF SS-OCTA) can provide quick and non-invasive angiographic information with a wide field of view. Here, we looked for associations between OCT-A vascular imaging metrics and vision in RAO patients.
UNASSIGNED: Patients with diagnoses of central (CRAO) or branched retinal artery occlusion (BRAO) were included. 6mm × 6mm Angio and 15mm × 15mm AngioPlex Montage OCT-A images were obtained for both eyes in each patient using Zeiss Plex Elite 9000 WF SS-OCTA device. Each 6mm × 6mm image was divided into nine Early Treatment Diabetic Retinopathy Study (ETDRS) subfields. Non-perfusion area (NPA) was manually measured using 15mm × 15mm images. A linear regression model was utilized to identify correlation between imaging metrics and vision. P-values less than 0.05 were considered as statistically significant.
UNASSIGNED: Twenty-five subjects were included. For RAO eyes, there was a statistically significant inverse correlation between retinal thickness as well as superficial capillary plexus (SCP) vessel density (VD) and vision. An inverse correlation was found between deep capillary plexus (DCP) VD and vision without statistical significance. There was a positive correlation between choroidal thickness as well as choroidal volume and vision without statistical significance. No significant correlation was found between the metrics and vision in contralateral eyes. For NPA and vision, no significant correlation was identified.
UNASSIGNED: This is the first study to investigate the utility of WF SS-OCTA in RAO and to demonstrate correlations between retinal vascular imaging metrics and visual outcomes. The results of this study provide a basis to understand the structural changes involved in vision in RAO and may guide management of RAO and prevention of cerebral stroke and cardiovascular accidents.

UNASSIGNED: To report a novel case of central retinal artery occlusion in a 44-year-old male caused by emboli from a non-traumatic maxillary artery pseudoaneurysm.
UNASSIGNED: A 44-year-old male with history of hypertension presented to clinic with painless vision loss in his left eye. He was found to have a central retinal artery occlusion. Ocular massage and intraocular pressure lowering agents were administered and the patient was transferred to the emergency department for cerebrovascular work-up. Remarkably, the patient had rapid symptom improvement from no light perception to 20/70 after ocular massage and IOP agents. Neuroimaging studies discovered a maxillary artery pseudoaneurysm with anastomotic branches to the internal carotid artery via the foramen rotundum and Vidian artery. Endovascular embolization was performed to prevent further thromboembolic event.
UNASSIGNED: We believe this to be the first reported case of retinal artery occlusion caused by a maxillary artery pseudoaneurysm. This case demonstrates that visual deficits can be the presenting symptom of a non-traumatic maxillary artery pseudoaneurysm.

Susac\'s syndrome is a rare microangiopathy affecting small vessels of the retina, inner ear and brain. It is characterised by a triad of symptoms: encephalopathy, visual defects, and sensorineural hearing loss. The disease is probably caused by an autoimmune process. Diagnosis is based on the typical symptoms, brain MRI, and, most importantly, fluorescein angiography. It is important to distinguish between Susac\'s syndrome and multiple sclerosis or migraine with aura, because misdiagnosis leads to the wrong treatment. To date, no detailed guidelines for the treatment of Susac\'s syndrome have been developed. Immunosuppression seems to be effective. It must be remembered that early and aggressive treatment is crucial, and that delays in diagnosis, and as a result in treatment implementation, worsen the prognosis.

This study aims to describe the clinical profile of severe vaso-occlusive retinal disorders in patients with systemic lupus erythematosus (SLE) and it is a retrospective case series. The clinical characteristics of three patients with SLE with vascular occlusions in four eyes were described. Branch retinal artery occlusion (BRAO) was present in all three patients with combined non-ischemic central retinal vein occlusion (NICRVO) in one patient and evolving ischemic CRVO in another patient. Additional branch retinal artery insufficiency was observed in the other eye of a patient with BRAO. Antinuclear antibody (ANA) titer was elevated in all patients. One patient had a positive lupus anticoagulant with elevated activated partial thromboplastin time (aPTT), and concurrent homocysteinemia was present in another patient. Intravitreal anti-vascular endothelial growth factor (ranibizumab) injection was administered to two eyes. Intravenous methyl prednisolone (IVMP) injection along with oral azathioprine was used in all patients with the need for anticoagulation in two patients along with SLE treatment. Vision in two eyes did not improve to the functional level despite aggressive therapy. Visually blinding severe vaso-occlusive retinopathy in the form of BRAO with or without CRVO can manifest in patients with SLE. Undetected antiphospholipid syndrome and homocysteinemia may be associated risk factors for such ophthalmic complications.

OBJECTIVE: To review clinical experience, efficacy, and safety of transluminal Nd:YAG laser embolectomy/embolysis (TYE) for retinal artery occlusion.
METHODS:  Electronic databases were searched for all published clinical studies and case-reports reporting on TYE in central (CRAO) or branch (BRAO) retinal artery occlusion. Individual patient data was evaluated in a weighted pooled analysis.
RESULTS: Sixty-one cases were reported, 47 with BRAO and 14 with CRAO. Visual acuity (VA) at onset averaged 20/252 (1.1 LogMAR) and improved following the procedure to 20/47 (0.37 LogMAR) at first follow-up (avg. 6 days, P < 0.001) and to 20/30 (0.18 LogMAR) at last reported follow-up (avg. 1.1 years, P = 0.02). Patients with worse VA (<20/200) improved further (12 vs. three lines, P < 0.001). VA was not improved when using higher pulse energies (≥ 2.4 mJ) which were associated with more vitrectomies. In a weighted analysis vitreous/sub-retinal hemorrhage was estimated to occur in 54% of cases and required vitrectomy in 18% of cases.
CONCLUSIONS: TYE was followed by significant visual improvement in the vast majority of cases, including CRAO, and was frequently associated with vitreous hemorrhage. Patients with poor visual acuity appear to benefit further and higher pulse energies may be detrimental. Lack of randomization and intrinsic biases prevent any definite conclusions regarding the benefits and further research is warranted.

BACKGROUND: Ocular vascular occlusion (OVO), first diagnosed during or immediately after giving birth, often reflects superposition of the physiologic thrombophilia of pregnancy on previously undiagnosed underlying familial or acquired thrombophilia associated with spontaneous abortion, eclampsia, or maternal thrombosis.
OBJECTIVE: We describe OVO, first diagnosed during pregnancy or immediately postpartum, in three young females (ages 32, 35, 40) associated with previously undiagnosed familial thrombophilia.
RESULTS: Branch retinal artery occlusion (BRAO) occurred at 9 and 13 weeks gestation in two females, aged 32 and 35. Central retinal vein occlusion occurred immediately postpartum in a 40-year-old. One of the two females with BRAO subsequently developed eclampsia, and one had a history of unexplained first trimester spontaneous abortion. All three females were found to have previously unexplained familial thrombophilia. The two females with BRAO had low first trimester free protein S 42 (41%), lower normal limit (50%), and one of these two had high factor VIII (165%, upper normal limit 150%). The woman with central retinal vein occlusion had high factor XI (169%, upper normal limit 150%). Enoxaparin (40-60 mg/day) was started and continued throughout pregnancy in both females with BRAO to prevent maternal-placental thrombosis, and of these two females, one had an uncomplicated pregnancy course and term delivery, and the second was at gestational week 22 without complications at the time of this manuscript. There were no further OVO events in the two females treated with enoxaparin or in the untreated patient with postpartum eclampsia.
CONCLUSIONS: OVO during pregnancy may be a marker for familial or acquired thrombophilia, which confers increased thrombotic risk to the mother and pregnancy, associated with spontaneous abortion or eclampsia. OVO during pregnancy, particularly when coupled with antecedent adverse pregnancy outcomes, should prompt urgent thrombophilia evaluation and institution of thromboprophylaxis to prevent adverse maternal and fetal-placental thrombotic events.