A Young Man With Visual Field Loss, Decreased Hearing, and Confusion

A 27-year-old man noted imbalance and staggering when walking. Vertigo, nausea, vomiting, and mild fever developed. This was presumed to be due to an inner ear infection, and antibiotics were prescribed. He began experiencing intermittent left face and arm numbness, bilateral hearing loss and tinnitus. Audiography indicated low-frequency hearing loss in both ears, left worse than right. He reported headaches and neck stiffness, and his family noticed that he was moody, easily aggravated, and confused, with slow mentation. Magnetic resonance imaging showed patchy, nodular, leptomeningeal enhancement involving both cerebral hemispheres and the posterior fossa, with scattered hyperintense T2 signal changes of the internal capsule and prominent abnormal signal changes in the corpus callosum. Cerebrospinal fluid analysis was remarkable for a markedly increased protein concentration and white blood cells. Eye examination showed 20/20 vision in both eyes with a superior visual field defect in the right eye. Retinal whitening was noted in the vascular distribution of the inferotemporal arcade. Intravenous fluorescein angiography showed delayed filling in this region consistent with a branch retinal artery occlusion and scattered areas of arteriolar wall hyperfluorescence. A diagnosis of Susac syndrome was made on the basis of the branch retinal artery occlusion, magnetic resonance imaging findings, and hearing deficit. Intravenous methylprednisolone was given, followed oral prednisone, which resulted in substantial improvement in headaches and cognition. Cyclophosphamide was also started at the same time as intravenous methylprednisolone. A new visual field defect developed due to a branch retinal artery occlusion in the left eye, which prompted initiation of intravenous immunoglobulin and transition from cyclophosphamide to rituximab. He had no recurrent branch retinal artery occlusions or other relapses of his underlying Susac syndrome on this treatment regimen. Susac syndrome was initially described as a microangiopathy of the brain and retina. It is an idiopathic autoimmune disorder that primarily affects the brain, eye, and inner ear.

Complications of Intra-Arterial tPA for Iatrogenic Branch Retinal Artery Occlusion: A Case Report through Multimodal Imaging and Literature Review

Background and Objectives: To document, through multimodal imaging, the post-procedural clinical course and visual outcome of a patient who received intra-arterial tissue plasminogen activator (tPA) for acute iatrogenic branch retinal artery occlusion (BRAO), and to review the literature and guidelines regarding the use of tPA for retinal arterial occlusions. Methods: A 28-year-old female patient who sustained an iatrogenic BRAO and subsequently received intra-arterial tPA was followed through her post-interventional course of 3 months with serial exams and multimodal imaging, including color fundus photography, visual field testing, spectral domain optical coherence tomography (SD-OCT), and OCT angiography (OCT-A). Results: A patient with history of left internal cerebral artery (ICA) aneurysm and baseline visual acuity (VA) of 20/20 developed an acutely symptomatic BRAO after undergoing a neuroendovascular procedure and was acutely treated with tPA through the left ophthalmic artery. At two weeks follow-up, a central posterior pole hemorrhage was noted although VA was preserved. A superior altitudinal defect was shown on automated perimetry. VA dropped to 20/50 at 7 weeks follow-up and hyperreflective material deep to the attachment between the posterior hyaloid and the internal limiting membrane (ILM) consistent with hemorrhage was noted on SD-OCT. At 11 weeks follow-up, VA returned to 20/20, SD-OCT revealed a membrane bridging the foveal depression, OCT-A showed decreased vascularity in the inferior macula, and the visual field defect was stable by automated perimetry. Conclusions: Intraocular hemorrhage is a possible complication of intra-arterial tPA administration for BRAO, and a careful analysis of risks, benefits, and goals of this procedure must be considered by both provider and patient before such intervention.

Central and Branch Retinal Artery Occlusion—Do They Harbor the Same Risk of Further Ischemic Events?

Purpose: Retinal artery occlusion (RAO) is associated with an increased risk of cardiovascular events such as ischemic stroke and myocardial infarction, but whether different RAO subtypes such as central retinal artery occlusion (CRAO) or branch retinal artery occlusion (BRAO) carry similar risk of these events is unclear. Our aim was to determine whether the risk of cardiovascular events differs between CRAO and BRAO. Methods: This single-center, retrospective study included 131 patients hospitalized in our clinic in 2010–2020 with CRAO or BRAO confirmed by ophthalmic examination. Data on demographics, previous ischemic stroke and myocardial infarction, comorbidities, the results of echocardiographic and ultrasound carotid artery examinations and laboratory tests were assessed. Data on ischemic stroke, myocardial infarction, and all-cause mortality occurring after RAO were obtained from the Polish National Health Service, which collects data on all publicly funded hospitalizations. Using these data, Kaplan-Meier analyses and Cox proportional hazard regression were performed. Results: Ischemic stroke occurred in 9.9% of patients after RAO: 10.6% in the CRAO group and 8.1% in the BRAO group (p = 0.662). Myocardial infarction occurred in 2.3% of patients after RAO: 2.1% in the CRAO group and 2.7% in the BRAO group (p = 0.843). All-cause mortality occurred in 22.9% of patients after RAO: 25.5% in the CRAO group and 16.2% in the BRAO group (p = 0.253). The composite endpoint of ischemic stroke, myocardial infarction, and all-cause mortality after RAO occurred in 28.2% of patients: 30.9% in the CRAO group and 21.6% in the BRAO group (p = 0.338). There was no difference between CRAO and BRAO in median time to ischemic stroke (32 vs. 76.4 months; p = 0.352), all-cause mortality (35.9 vs. 36.3 months; p = 0.876) or composite endpoint (37.5 vs. 41.5 months; p = 0.912) after RAO. The Kaplan-Meier analysis showed no differences between CRAO and BRAO in ischemic stroke, myocardial infarction, all-cause mortality, or the composite endpoint; similar results were obtained in analyses of patients with and without cardiovascular events before RAO. Conclusions: The prognosis for ischemic stroke, myocardial infarction, and all-cause mortality is similar in patients with CRAO and BRAO. Ischemic strokes occur with a similar frequency before and after RAO. Myocardial infarctions are observed significantly more frequently before an episode of RAO than after. The results of our study indicate that both CRAO and BRAO require expanded diagnostics to assess the risk of recurrent cardiovascular events, especially ischemic strokes, to implement appropriate prophylaxis and reduce mortality.

Branch Retinal Artery Occlusion after Percutaneous Coronary Intervention

We report a case of branch retinal artery occlusion (BRAO) that occurred after percutaneous coronary intervention (PCI). A 59-year-old man with no other previous diseases presented visual acuity deterioration in the left eye 24 hours after PCI. Fundus examination revealed ischemia at the temporal branch of the retinal artery associated with inner layer edema. Prompt treatment was performed with ocular digital massage and paracentesis of the anterior chamber. However, at discharge, the patient had a persistent visual loss with a central scotoma that persisted at 35-day follow-up without improvement of the visual acuity. The patient did not suffer from any other systemic complications. Retinal infarction should be considered a potential complication of PCI. Patients and health care providers should be aware of any visual signs. Permanent visual disability can be prevented by immediate diagnosis and prompt intervention.

Occlusive retinal vasculopathy with macular branch retinal artery occlusion as a leading sign of atypical hemolytic uremic syndrome – a case report

Background Hemolytic Uremic Syndrome (HUS) is a rare disorder characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure, considered within the group of thrombocytic microangiopathies. Ocular complications in HUS are very rare. Here, we report an adult patient who suffered from acute onset of paracentral scotoma, caused by branch retinal artery occlusion (BRAO), as a leading symptom of atypical HUS. Case presentation A 39-year-old healthy male was lately diagnosed with essential hypertension and mild renal impairment. He complained about acute onset of central scotoma in his left eye. Fundus examination revealed marked narrowing of retinal vessels, cotton wool spots and few retinal hemorrhages in both eyes. The patient was diagnosed with bilateral ischemic retinal vasculopathy and acute macular BRAO in his left eye. Workup revealed thrombocytopenia, worsening renal failure. Renal biopsy showed signs of chronic thrombotic microangiopathy. The patient was diagnosed with atypical HUS (aHUS) and started on plasmapheresis, together with eculizumab. As his condition continued to worsen, he was put on renal replacement therapy. Due to a persistent monoclone of IgG1, the patient underwent bone marrow biopsy which revealed Monoclonal Gammopathy of renal significance, triggering a HUS and treatment was initiated accordingly. Two months after initial presentation, the patient developed neovascularization of the optic disc (NVD) in his left eye, and was treated with 3 monthly intravitreal bevacizumab injections with complete regression of the NVD. The patient suffered from myocardial infarction in the later course and was lost for follow-up. He returned 11 months after the last bevacizumab injection because of sudden loss of vision in his left eye caused by a dense vitreous hemorrhage. Biomicroscopy revealed a new NVD in his right eye. The patient underwent panretinal photocoagulation in both eyes with regression of neovascularization. Vision improved and remained 20/20 in both eyes. Conclusion We present a case report showing retinal ischemia can be linked with aHUS. As clinal diagnosis might be challenging, physicians should be aware of the rare ocular manifestations of this devastating multi-organ disease. In case of retinal ischemia, panretinal photocoagulation should be initiated soon to avoid blinding complications.