Abstract

Introduction Vein of Galen Malformations (VOGM) are rare vascular conditions resulting from anomalous vascular shunts between primitive choroidal vessels and the Markowski Prosencephalic Vein (MPV). They comprise only 1% of all intracranial vascular malformations with a high mortality rate up to 76.7% if untreated. In this case report, we aim to bring awareness of this condition as a cause of progressive visual loss with the objective of making it relevant in our medical community. Methods We discuss the case of a truck driver who progressively and slowly lost his vision as a consequence of a VOGM. Results In this case report we present a 49‐year‐old man with a past medical history of hypertension and renal insufficiency who initially presented to his ophthalmologist for progressive vision loss accompanied by headaches. Bilateral papilledema was seen on examination as well as near complete vision loss on the right eye and 20/40 visual acuity on the left eye. He was referred to neurosurgery for suspicion of elevated intracranial pressure. CT angiogram demonstrated multiple areas of fistualization to the VOGM, including branches from the meningeal hypophyseal divisions, bilateral distal PICA divisions, and the thalamic striate divisions. A multisession endovascular treatment with multiple embolizations was pursued. Initially, the patient underwent a transarterial embolization of the left middle meningeal artery (MMA) and left occipital artery (OA) followed by embolization of the 4 branches of the right MMA. Then, he underwent embolization of 8 large feeding branches and three weeks later, transvenous coiling of the MPV was done. Post‐embolization angiograms showed progressive decrease in the size of the malformation, the degree of the shunt and, and in cortical venous reflux with near normalization of the capillary and venous phases timing in the left and right hemispheres. Although, the patient did not recover from prior visual deficits, his symptoms remained stable. Conclusions It is important to keep this unusual cause of progressive vision loss within the differential diagnosis. The earlier this diagnosis is made, the quicker patients can receive treatment preventing long term deficits, morbidity and mortality.

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