Recurrent cerebral infarction in Klippel-Trenaunay–Weber syndrome

Abstract

KTWS is characterised by capillary hemangioma, varicose veins, nd bone and soft tissue hypertrophy. We present a 53-year-old oman with sporadic congenital Klippel-Trenaunay–Weber synrome (KTWS) with involving asymmetrically the four limbs (i.e. one and soft tissue hypertrophy predominant in the right-sided imbs, and varicose veins in both lower limbs), the trunk (large capllary hemagioma on the left-sided trunk, Fig. 1), and the head. In bsence of cardiovascular risk factors, she had a history of transient schemic attack (aphasia) at the age of 9 years, ischemic stroke at he age of 20 years with right-sided hemiparesis lasting for days ithout leaving permanent deficit, and another ischemic stroke at he age of 29 years leaving a permanent right-sided hemiplegia. o underlying cause for these cerebral infarctions was identified at hat time. Age 53, she presented with acute vertigo, nausea, dysarthria, and ait ataxia. MRI revealed two chronic ischemic left middle cerebral rtery infarctions, and a recent ischemic right superior cerebellar rtery infarction. CT angiography showed an aspect of fibromusular dysplasia in both extracranial internal carotid arteries (in bsence of pseudoaneurysm, aneurysm, atheromatous plaque, or igns of acute dissection), and several intraand extracranial enous malformations (Fig. 1). Duplex ultrasound and CT angiogaphy showed no renal artery abnormalities. Carotid angiography