Cavernous Lymphangioma of the Spleen in a Patient with Klippel-Trenaunay-Weber Syndrome.

Abstract

Masahide Yamazaki1^, Youichi Kawamura2^Takio Ohka2), Shoichi Katada3\ Katsuya Morita3M asaaki Nakagawa3), Eiko Kubo4), Aio Kawashima4), Hiroshi Shimizu5), Koji Nobata5), Shigeho Rikimaru5^Syugen Rin6), Hidesaku Asakura1^and Tamotsu Matsuda1Â 31-year-old womanpresented 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 lymphangiomawas diagnosed by ultrasonography and angiography, and was confirmed by pathology following splenectomy. Post-operatively, the platelet count increased, and hemostatic parameters normalized. Cavernous lymphangiomais a rare complication of KlippelTrenaunay-Weber syndrome. Splenectomy proved to be an effective therapy for both cavernous lymphangioma and consumptive coagulopathy in Klippel-Trenaunay-Weber syndrome. (Internal Medicine 33: 574-577, 1994)

Repetitive cerebral bleeding in an adult with Klippel-Trénaunay Syndrome

“…Peripheral bleeding can, however, be caused without apparent AVM [15][16][17][18]. Reduced factor XIII, as seen in this case, has been described with KTS [19].…”

“…Reduced factor XIII, as seen in this case, has been described with KTS [19]. Changes in coagulation are usually related to thrombocytopenia and low plasma fibrinogen caused by continuous consumption of platelets and fibrinogen in the altered vessels [3,4,17,19]. Disseminated intravascular coagulation is therefore an important complication, and haemorrhage remains a fatal risk in KTS patients.…”

Extensive cervical-mediastinal cystic lymphatic malformation treated with sclerotherapy in a child with Klippel-Trenaunay syndrome

“…Although abdominal or limb location of a cystic lymphatic malformation has been reported in KTS, to the best of our knowledge, mediastinal location of any type of lymphatic malformation has not been previously reported in patients with KTS [7,8].…”

Selected Case From the Arkadi M. Rywlin International Pathology Slide Series

“…9,23 The differential diagnosis by imaging includes parasitic cysts (mainly hydatid disease), nonparasitic cysts, and other benign and malignant splenic vascular proliferations. By US, lymphangiomatosis appear as hypoechoic masses with internal septations.…”