Perioperative Stroke in Patient Undergoing EC-IC Bypass for Moyamoya: A Delicate Balance

Moyamoya disease is a syndrome of progressive stenosis and occlusion of the intracranial branches of the internal carotid artery and the formation small collaterals resulting in the typical "puff of smoke" appearance on... [ view full abstract ]

Moyamoya disease is a syndrome of progressive stenosis and occlusion of the intracranial branches of the internal carotid artery and the formation small collaterals resulting in the typical "puff of smoke" appearance on angiography.  The condition can be associated with both ischemic and hemorrhagic strokes, and it is most effectively treated with direct bypass procedures, which connect a branch of the external carotid artery to a more distal branch of the internal carotid, such as the middle cerebral artery.  These surgeries, however, are associated with a number of complications including graft failure, ischemic stroke, intracranial hemorrhage, cerebral hyperperfusion syndrome, and graft problems such as thrombosis or compression.  Here, we present a case of a 62 yo F with Moyamoya disease and prior successful right-sided STA-MCA bypass and no baseline neurologic deficits, who underwent a left-sided STA-MCA bypass.  While the surgery successfully achieved a patent bypass between STA and MCA and the patient initially woke up well from anesthesia, she rapidly deteriorated after extubation and had seizure-like activity during transport to ICU.  She also developed new aphasia and facial droop, and post-operative imaging revealed multiple small infarcts in watershed territories.  This case provides an example the complex intracerebral hemodynamics in Moyamoya disease patients, and the unfortunate complications that can result from perioperative perturbations of those hemodynamics.