CAVERNOUS LYMPHANGIOMA OF THE SPLEEN IN A PATIENT WITH KLIPPEL-TRENAUNAY-WEBER-SYNDROME

A 31-year-old woman presented with hypertrophy of the left upper extre mity and thrombocytopenia. Physical examination revealed splenomegaly, and laboratory investigation revealed thrombocytopenia, elevation of cross-linked fibrin degradation products (XDP), and thrombin-antithrom bin III complex (TAT). A diagnosis of Klippel-Trenaunay-Weber (K-T-W) syndrome was established by the dermatologic findings and angiography of the extremities. A splenic cavernous lymphangioma was diagnosed by ultrasonography and angiography, and was confirmed by pathology follow ing splenectomy. Post-operatively, the platelet count increased, and h emostatic parameters normalized. Cavernous lymphangioma is a rare comp lication of Klippel-Trenaunay-Weber syndrome. Splenectomy proved to be an effective therapy for both cavernous lymphangioma and consumptive coagulopathy in Klippel-Trenaunay-Weber syndrome.