RESUMEN

Purpose: In this study, we report a patient who presented with both chronic myelocytic leukemia (CML) and Susac syndrome (SS). Observations: 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. Conclusion and importance: 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.

RESUMEN

OBJECTIVES: 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). MATERIALS AND 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.

Asunto(s)

RESUMEN

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.

RESUMEN

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.

Asunto(s)

RESUMEN

Purpose: 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. Methods: 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. Results: 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. Conclusion: 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.

RESUMEN

Purpose: 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. Observations: 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. Conclusion and Importance: 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.

RESUMEN

Introduction: It is reported that eyes with a branch retinal artery occlusion (BRAO) had normal full-field electroretinography (ERG) but the response of the multifocal electroretinography (mfERG) was reduced in the area of the arterial occlusion. Optical coherence tomography angiography (OCTA) is a recently appeared modality that can evaluate microvascularizations in different retinal layers and in different regions of the retina. The purpose of this study was to determine the density of the microcirculation and the function of the macular area of eyes with BRAO, and to determine whether they are significantly correlated. Methods: The OCTA and mfERG findings of 7 eyes of 6 patients (3 men, 3 women) were studied. The mean age of the patients was 71.7±10.6 years. The OCTA examinations were made with volume scans of 3 × 3 and 6 × 6 mm squares centered on the fovea. The macular vessel densities (mVD) in the superficial retinal layer (SRL) and deep retinal layer (DRL) were measured for the superior and inferior halves of 3  × 3  and 6  × 6 mm diameter concentric circles. The mfERGs were recorded with targets set to stimulate the focal areas of the retina corresponding to the areas examined by OCTA. Results: The OCTA examinations showed that the mVD of the 3 mm concentric circle in the SRL was significantly lower on the affected side than on the unaffected side (P = 0.022). No such difference was observed in the DRL. The N1 amplitude of the 20.2° concentric circle and the N1-P1 amplitude of the 10.1° concentric circle of the mfERGs were significantly smaller on the affected side than on the unaffected side (P = 0.047 and 0.031). A significant positive correlation was found between the mVD of the 6 mm concentric circle in the DRL and the P1-N2 amplitude of the 20.2° concentric circle (ρ = -0.929 and p = 0.003). Discussion: These findings indicate that OCTA images may be able to show changes in the density of the retinal macular microcirculation, and the mfERGs may be able to show alterations in the function of the macular area of the eyes with BRAO. A layer-by-layer analysis of the local retinal microcirculation and function should help in determining the pathogenesis of BRAO.

RESUMEN

Animal models of retinal artery occlusion (RAO) have been widely used in many studies. However, most of these studies prefer using a central retinal artery occlusion (CRAO) which is a typical global ischemia model of the retina, due to the technical limitation of producing single vessel targeted modeling with real-time imaging. A focal ischemia model, such as branch retinal artery occlusion (BRAO), is also needed for explaining interactions, including the immunological reaction between the ischemic retina and adjacent healthy retina. Accordingly, a relevant model for clinical RAO patients has been demanded to understand the pathophysiology of the RAO disease. Herein, we establish a convenient BRAO mouse model to research the focal reaction of the retina. As a photo-thrombotic agent, Rose bengal was intravenously injected into 7 week-old transgenic mice (CX3CR1-GFP) for making embolism occlusion, which causes pathology similarly to clinical cases. In an optimized condition, a 561 nm laser (13.1 mw) was projected to a targeted vessel to induce photo-thrombosis for 27 s by custom-built retinal confocal microscopy. Compared to previous BRAO models, the procedures of thrombosis generation were naturally and minimal invasively generated with real-time retinal imaging. In addition, by utilizing the self-remission characteristics of Rose bengal thrombus, a reflow of the BRAO with immunological reactions of the CX3CR1-GFP+ inflammatory cells such as the retinal microglia and monocytes was monitored and analyzed. In this models, reperfusion began on day 3 after modeling. Simultaneously, the activation of CX3CR1-GFP+ inflammatory cells, including the increase of activation marker and morphologic change, was confirmed by immunohistochemical (IHC) staining and quantitative real-time PCR. CD86 and Nox2 were prominently expressed on day 3 after the modeling. At day 7, blood flow was almost restored in the large vessels. CX3CR1-GFP+ populations in both superficial and deep layers of the retina also increased around even in the BRAO peri-ischemic area. In summary, this study successfully establishes a reproducible BRAO modeling method with convenient capabilities of easily controllable time points and selection of a specific single vessel. It can be a useful tool to analyze the behavior of inflammatory cell after spontaneous arterial recanalization in BRAO and further investigate the pathophysiology of BRAO.

RESUMEN

Vasculitis is a heterogeneous group of disorders characterized by multifocal segmental inflammation of the small and medium vessels of the central nervous system. The predominant symptoms of cerebral vasculitis are stroke, headache, and encephalopathy. Additional symptoms include seizures, cranial nerve palsies, and myelopathy. Imaging techniques play a crucial role in identifying the diagnosis of vasculitis and demonstrating brain involvement. An 89-year-old woman with permanent atrial fibrillation developed an embolic stroke. In treatment, intravenous thrombolysis and thrombectomy with complete antegrade reperfusion of the left middle cerebral artery was used, without the clinical effectiveness. Brain MRI revealed bilateral oval lesions in medial parts of the orbits, which were initially misinterpreted as orbital tumors. Final diagnosis confirmed thickened arterial walls as orbital changes due to inflammatory arteritis. Ten days later, follow-up MRI was performed and showed complete regression of the orbital masses. Primary central nervous system vasculitis, manifesting as acute ischemic stroke, may be reversible with early systemic thrombolytic treatment.

RESUMEN

Purpose: To report a case of branch retinal artery occlusion (BRAO) followed by branch retinal vein occlusion (BRVO) and paracentral acute middle maculopathy (PAMM) in a patient with confirmed calciphylaxis. Observations: A 52-year-old female with a history of BRAO in the right eye one-year prior presented with decreased vision and a new inferotemporal scotoma. Computed tomography angiography of the head and neck demonstrated vascular calcifications at the origin of both ophthalmic arteries, which were otherwise poorly visualized. Ophthalmic examination demonstrated retinal whitening superiorly with intraretinal hemorrhages inferiorly. Optical coherence tomography (OCT) demonstrated middle retinal hyperreflectivity and a mild epiretinal membrane. Fluorescein angiography (FFA) demonstrated delayed perfusion of superior retinal arcade. On further questioning, patient was found to have a history of IgA nephropathy with end-stage renal disease, secondary hyperparathyroidism and calciphylaxis. Calciphylaxis is a systemic disease, characterized by high levels of calcium and progressive calcification of the vascular medial layer leading to ischemia. Anterior ischemic optic neuropathy (AION) and crystalline retinopathy have been reported as ocular manifestations of calciphylaxis, however, there are very few reports on ophthalmic manifestations of calciphylaxis. Conclusion and importance: Clinical manifestations of calciphylaxis are variable and a detailed clinical history is important to suspect calciphylaxis. Calciphylaxis should be considered in the differential diagnosis of BRAO, BRVO, PAMM or any ophthalmic vascular manifestation in patients with end-stage renal disease.

RESUMEN

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.

Asunto(s)

RESUMEN

PURPOSE: To report a case of ocular Gnathostomiasis presenting as branch retinal artery occlusion. METHOD: Observational case report. RESULT: A 22-year-old Asian woman presented to her ophthalmologist with redness, tearing, and decreased vision in her left eye. Examination revealed anterior uveitis and branch retinal artery occlusion associated with both intra-retinal and vitreous hemorrhage. The patient was treated with topical corticosteroids and cycloplegics. After 3 weeks, she presented in our emergency, with further decrease in vision and worsening pain in the left eye. Slit lamp examination revealed a brown colored live worm on the posterior corneal surface, anterior uveitis, multiple iris holes, and vitreous cells. Indirect ophthalmoscopy showed focal retinal hemorrhages, subretinal tracts, and vitreous hemorrhage. Surgical removal of the worm from anterior chamber was done immediately. CONCLUSION: Branched retinal artery occlusion with intraretinal and vitreous hemorrhage, panuveitis, and multiple iris holes may suggest the presence of an intraocular parasite.

Asunto(s)

RESUMEN

PURPOSE: Susac's syndrome (ScS) is a rare, potentially life-threatening auto-immune disease. Ophthalmic imaging can depict characteristic branch retinal arteriolar occlusions (BRAO) and arterial wall hyperfluorescences that form one of the three diagnostic pillars of this condition. We aim to demonstrate that ophthalmological ultrawide-field (UWF) imaging allows for a qualitative and quantitative assessment in ScS, with application in diagnostics, monitoring of treatment response, and titration of therapy. METHODS: In seven ScS patients (♀:♂ = 2:5), with a median age of 36 years, range 18 to 57 years, serial ultrawide-field fluoresceine angiography (UWF-FA) studies were performed, with adjunctive wide-field optical coherence tomography angiography analyses (WF-OCTA) in five patients. Mean follow-up was 12.5 months, range 1 to 46 months. RESULTS: In all seven patients, BRAO and arterial wall hyperfluorescences were present in UWF-FA and calculated as a quantitative score of disease activity in fluorescein angiography (DA-FA) during follow-up visits. Treatment response was accessible in follow-up imaging as partial reperfusion of retinal vessels, resolution of arterial wall hyperfluorescences and consequently, in reduction of DA-FA score. While qualitative analysis of WF-OCTA provided further information about retinal micro-perfusion, quantitative analysis did not demonstrate a vectored treatment response as it was accessible in FA. CONCLUSION: DA-FA score, as a comprehensive disease activity parameter in ScS has potential to facilitate optimal communication between subspecialties and thereby treatment success.

Asunto(s)

RESUMEN

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.

RESUMEN

BACKGROUND/PURPOSE: Susac syndrome is a rare microangiopathy of suspected autoimmune origin affecting arteries of the retina, the cochlea and the brain. The aim of the study was to give a review of the disease entity and determine the proportion of cases and their characteristics in a uveitis referral centre. PATIENTS AND METHODS: Charts of patients with the diagnosis of Susac syndrome seen in the Uveitis Clinic of the Centre for Ophthalmic Specialised Care (COS), Lausanne, Switzerland were reviewed retrospectively to determine the frequency of such cases in a uveitis referral centre. Clinical symptoms and signs, functional data, imaging signs and evolution were analysed in the 3 COS cases and one case shared with the Uveitis Clinic of the Department of Ophthalmology, University of Innsbruck, Austria. Characteristic signs were searched possibly allowing a prompt diagnosis. RESULTS: During the period from 1994 to 2019 (24 years, 2045 patients), 3 charts with the diagnosis of Susac syndrome were found (0.15%). The whole collective, including the additional case, comprised three women aged 28, 32 and 63 at presentation and one man, aged 42. None of the 3 cases that were referred were diagnosed beforehand. The characteristic item found in all 4 cases was the abrupt arterial stop or segmental interruption of arteries and increased staining of arterial wall on angiography more clearly shown on indocyanine green angiography that can potentially be proposed as a crucial diagnostic element. All 4 cases responded to dual steroidal and non-steroidal immunosuppression. Under treatment, all four patients did not show any further evolution. CONCLUSION: Susac syndrome is a multilocation arteritis of the head that can involve the eye, ear and brain often first diagnosed by the ophthalmologist. The diagnosis is rapidly reached in uveitis referral centres but seems to be missed otherwise, A helpful angiographic sign to be searched is an abrupt or segmental arterial stop and increased staining of the arterial wall more clearly seen on indocyanine green angiography. Patients often present first to the ophthalmologist who should be acting as a whistleblower to avoid severe involvement of the brain.

Asunto(s)

RESUMEN

PURPOSE: To report two cases of central retinal artery occlusion (CRAO) associated with vitreoretinal surgery. OBSERVATIONS: Two patients underwent vitreoretinal surgery and were diagnosed with CRAO on postoperative day one. Both had received retrobulbar anesthetic blocks, followed by pars plana vitrectomy in one patient and scleral buckling in the other patient. Best-corrected visual acuity at last follow-up was 20/40 and 20/400. CONCLUSIONS/IMPORTANCE: CRAO is a rare but serious adverse event after vitreoretinal surgery. The causative mechanism is not known in these patients.

RESUMEN

Branch retinal artery occlusion (BRAO) is a prevalent vascular occlusive disorder of the eye. In transient BRAO, an embolus temporarily blocks the vessel and then moves on resulting in recovery of blood circulation. In this case, we have documented migration of the embolus with recovery of perfusion of the retina and improvement of visual acuity in a patient with transient BRAO. Early diagnosis and fast action are important in the case of patients with retinal artery occlusion.

RESUMEN

AIMS: 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.

Asunto(s)

RESUMEN

PURPOSE: To report a case of Susac syndrome with ocular complications in the chronic stage of the disease. METHODS: Case report. RESULTS: A 37-year-old male patient of Indian origin presented with painless blurring of vision in the right eye. Ophthalmoscopic examination revealed vitreous hemorrhage in the right eye and NVE in both eyes well-documented angiographically. Old medical records revealed a previous attack of bilateral BRAO as a complete triad of Susac syndrome 10 years before, with an uncomplicated clinical course. Panretinal photocoagulation helped in stabilization of the condition. CONCLUSION: Ocular neovascularisation like NVE with vitreous hemorrhage can be a late manifestation of occlusive arteriolitis in Susac syndrome.

Asunto(s)