A patient with Klippel-Trénaunay-Syndrom and cerebral venous sinus thrombosis

Introduction: A 31-year old female patient with Klippel-Trénaunay-Syndrom presented to the neurology department with persisting headache for 6 weeks, without neurological deficits.

Method: MRI revealed cerebral venous sinus thrombosis in the absence of cerebral vessel malformations. Anticoagulation with enoxaparin 6000 IU BID was implemented for three months and subsequently reduced to enoxaparin 6000 once daily. 10 months into treatment, anticoagulation was changed to Edoxaban 30 mg once daily and continued for the last six years until today.

Results: Headache resolved after initiation of anticoagulation until today. Laboratory data showed no thrombophilic disorder. D-Dimer levels remain high between 1.7 und 3.1 mg/l. A factor VII deficiency (40%), not associated to liver disease or Vitamin K deficiency, was found. No bleeding symptoms were reported.

Conclusion: Cerebral venous sinus thrombosis was seen as the result of contraception with Gestagen injections every three months and the underlying Klippel-Trénaunay-Syndrom. However, no intracerebral vascular malformation was identified. In spite of the absence of thrombophilic disorders and half-therapeutic anticoagulation, D-Dimer levels remain elevated. This may be explained by the underlying vascular disease. According to the literature, Klippel-Trénaunay is associated with the risk of venous thrombosis in the affected limbs, but no case of cerebral vein thrombosis has been reported. Because of the spontaneous appearance of thrombosis and continuously elevated D-Dimer levels, it remains unclear, if anticoagulation can be discontinued.

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Artikel online veröffentlicht:
13. Februar 2025