Moyamoya Syndrome in a Patient With Klippel-Trenaunay Syndrome

Moyamoya syndrome consists of internal carotid artery stenosis with development of collateral vasculature responsible for ischemic events and cerebral hemorrhage. Moyamoya vasculopathy is commonly treated with external carotid artery to internal carotid artery bypass, either through direct or indirect anastomosis. Klippel-Trenaunay Syndrome (KTS) is a tissue hyper-proliferation disorder known to have a significant angio-dysplastic component to the pathology. No other instances of a patient with both KTS and Moyamoya syndrome are presently reported in the literature. We present a patient who had been diagnosed with KTS as a child who was found to have Moyamoya vasculopathy after experiencing frequent cerebral ischemic events. He underwent a left direct superficial temporal artery to middle cerebral artery bypass with subsequent significant improvement of his stroke symptoms. This case report demonstrates an association between KTS and Moyamoya syndrome with a possible shared pathophysiology. Patients with KTS may benefit from screening for cerebral ischemic events and monitoring for development of Moyamoya syndrome.