Management of Acute Central Retinal Artery Occlusion, a "Retinal Stroke": An Institutional Series and Literature Review.

OBJECTIVES: Acute central retinal artery occlusion (CRAO) is an ophthalmologic emergency that often results in permanent vision loss. Over 25% are associated with acute cerebral ischemia. In the absence of existing Level I treatment options, this study aims to examine institutional practice patterns and review the literature to develop a formalized approach to the treatment of CRAO in the era of ischemic stroke protocols. MATERIALS AND METHODS: This is a retrospective review of institutional practices in the workup and treatment of patients diagnosed with acute non-arteritic (NA) CRAO at a single center from January 2017 to August 2020. RESULTS: Of 91 patients managed for acute NA-CRAO, 62.6% were male and average age was 66.4 years. Only 20.9% of patients presented within 4 h of symptom onset. 12.1% of patients had evidence of acute stroke on MRI, and 27.5% had ipsilateral internal carotid artery stenosis >50%. Half (52.7%) did not receive any acute treatment for CRAO, excluding antiplatelet/anticoagulation. 48.5% of patients undergoing acute medical treatment had improved visual acuity compared to 29.4% without treatment (p=0.14). CONCLUSIONS: There is a lack of clear protocol for the management of NA-CRAO. While not reaching statistical significance, our experience mirrors the literature with patients undergoing medical treatment demonstrating improved visual acuity over those without treatment. Given the presence of acute ischemic stroke, carotid disease, and/or stroke risk factors in over 25% of patients with CRAO, multidisciplinary involvement and modern stroke algorithms should be considered for this disease.

A comprehensive review on the development of sporadic cerebral arteriovenous malformations: from Padget to next-generation sequencing.

Cerebral arteriovenous malformations (AVMs) are a leading cause of intracerebral hemorrhage in both children and young adults. With the continued advancement of science and technology, the understanding of the pathophysiology behind the development of these lesions has evolved. From early theory published by Harvey Cushing and Percival Bailey in 1928, Tumors Arising from the Blood-vessels of the Brain: Angiomatous Malformations and Hemangioblastoma, which regarded AVMs as tumors arising from blood vessels, to the meticulous artistry of Dorcas Padget's embryological cataloguing of the cerebral vasculature in 1948, to the proliferative capillaropathy theory of Yasargil in 1987, to Ramey's 2014 hierarchical model of vascular development, there have been multiple hypotheses of congenital, developmental, and genetic two-hit theories in the pathogenesis of AVMs. Most recent evidence implicates somatic KRAS mutations in the cerebral endothelium, producing an important understanding of the pathogenesis of this disease, which is critical to the development of targeted therapeutics. The authors present the historical progression of their understanding of AVM pathogenesis. They focus on the foundation laid by early pioneers, discussing embryological anatomy and vasculogenesis, the prominent theories of AVM development that have emerged over time, and culminate in an overview of the most current understanding of the pathogenesis of these complex vascular lesions and the clinical implications of our scientific progress.

Unexpected occlusion of the contralateral transverse sinus after stenting for idiopathic intracranial hypertension.

A woman in her thirties with intracranial hypertension underwent stenting of the right transverse-sigmoid (TS) junction with resolution of the pressure gradient. Due to persisting symptoms at follow-up, she underwent a repeat study showing a patent right TS stent but the non-dominant left transverse sinus, patent on initial studies, was now completely occluded. According to the positive feedback loop hypothesis, stenting of the right transverse sinus should have resulted in a reduction in intracranial pressures (confirmed by post-stenting lumbar puncture), but also an increase in left transverse sinus diameter, opposed to the occlusion seen on venography. This unexpected finding can be explained by the positive feedback loop hypothesis if a revision is made accounting for intramural venous pressures as an opposing force of venous sinus stenosis, as venous outflow obstruction in the dominant venous sinus pathway provided an increased intramural venous pressure in the non-dominant sinus facilitating patency against extramural pressures.