Cavernous lymphangioma of the spleen in a patient with Klippel-Trenaunay-Weber syndrome.

Abstract

A 31-year-old woman presented with hypertrophy of the left upper extremity and thrombocytopenia. Physical examination revealed splenomegaly, and laboratory investigation revealed thrombocytopenia, elevation of cross-linked fibrin degradation products (XDP), and thrombin-antithrombin 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 following splenectomy. Post-operatively, the platelet count increased, and hemostatic parameters normalized. Cavernous lymphangioma is a rare complication of Klippel-Trenaunay-Weber syndrome. Splenectomy proved to be an effective therapy for both cavernous lymphangioma and consumptive coagulopathy in Klippel-Trenaunay-Weber syndrome.

• Authors: M Yamazaki, Y Kawamura, T Ohka, S Katada, K Morita, M Nakagawa, E Kubo, A Kawashima, H Shimizu, K Nobata
• Journal: Internal medicine (Tokyo, Japan) (Intern Med) Vol. 33 Issue 9 Pg. 574-7 (Sep 1994) ISSN: 0918-2918 [Print] Japan
• PMID: 8000113 (Publication Type: Case Reports, Journal Article)

Topics

• Adult
• Disseminated Intravascular Coagulation (etiology, surgery)
• Female
• Humans
• Klippel-Trenaunay-Weber Syndrome (complications)
• Lymphangioma (etiology, surgery)
• Splenectomy
• Splenic Neoplasms (etiology, surgery)