Coincidence of ischemic stroke and recurrent brain haemorrhage in a patient with Klippel-Trenaunay Syndrome.

Cerebrovascular manifestations in Klippel-Trenaunay Syndrome (KTS) have been reported but are extremely rare. Case reports describe brain embolism in KTS-associated coagulopathy as well as bleeding due to arteriovenous malformations. We describe a 45-year-old patient with KTS and both acute ischemic stroke and repeated cerebral haemorrhage. The underlying aetiology of both events remained undetermined despite extensive diagnostic work-up, including coagulation tests and dynamic MR angiography. It is most likely that both a pathological coagulation and increased vessel fragility comparable to amyloid angiopathy were responsible for the combined brain lesions in this patient. We conclude that KTS is a very rare but relevant aetiology of cerebral ischemia and that anticoagulation treatment in these cases should be carefully considered as the risk of cerebral haemorrhage is probably elevated.