Syndrome de Klippel-Trenaunay et déficit en antithrombine III

Klippel-Trenaunay syndrome (KTS) is a rare phakomatosis characterized by cutaneous hemangiomata, venous varicosities and bone and soft tissue hypertrophy also of the affected limb. Central nervous system involvement is rare, arising from a malformation or from coagulation disorders. We report the case of a patient presenting a KTS with stroke. The biological assessment revealed antithrombin III deficiency. Although rare, antithrombin III deficiency should be kept in mind in KTS patients with neurological involvement.