KIippel- Trenaunay症候群--臨床及放射線攝影之評估

KUPPEL-TRENAUNAY SYNDROlVIE-RADIOLOGICAL AND CLINICAL ASPECTS

龐海麟(Hei-Lin Pang) ； 蕭海松(Hai-Sung Hsio) ； 張國華(Kuo-Hwa Chang)

《中華放射線醫學雜誌》 13卷2期 (1988/06) Pp. 187-191

摘要

Klippel-Trenaunay症候群為一罕見的先天性血管疾病。其臨床上表徵為下肢靜脈曲張（varicosevein），骨骼及軟組織的肥厚（hypertrophy）或葡萄酒色皮膚血管瘤（cutaneous hemangioma）。三軍總醫院和空軍總醫院自民國70年9月至76年8月，共有的15例Klippel-Trenaunay症候群之病例，男性13例，女性2例，年齡最小為12歲，最大35歲，平均24歲，所有病例皆發現下肢靜脈曲張；合併有靜脈曲張，骨骼及軟組織肥厚和皮膚血管瘤有7例；合併有靜脈曲張和皮膚血管瘤的有6例。此15例皆是接受常規X光攝影，深層靜脈攝影（deep venography），或股動脈攝影（femoral arteriography）檢查。於常規X光攝影檢查發現骨皮質與軟組織肥厚的有7例。靜脈攝影檢查顯示15例均有下肢深層靜脈系統之不正常，其中14例為深層靜脈的缺失（abscent），另1例為纖維索壓迫淺股靜脈（superficial femoral vein）。7例接受股動脈攝影檢查，僅3例發現下肢血管瘤，但無動靜脈瘻管（arteriovenous fistula）發生，Klippel-Trenaunay症候群除了臨床表徵外，須藉著靜脈攝影和動脈攝影來證實。

關鍵字

血管瘤(Hemangfoma) ； 靜脈攝影(Venography)

並列摘要

The Klippel-Trenaunay syndrome is a congenital vascular anomaly consisting of the triad of soft-tissue and bony hypertrophy of the extremities, port-wine cutaneous hemangioma, and superficial varicosities. Since September 1981 to August 1987, 15 cases of Klippel -Trenaunay syndrome was diagnosed by conventional radiography, venography and arteriography of the involved extremities. Conventional radiography indicated bony and soft-tissue hypertrophy in 7 cases. Venography was performed and found superficial varicose vein in all cases, abscent of deep venous system in 14 cases. There is one case with a fibrous cord compressing the deep venous channels. In 7 of 15 cases received femeral arteriography, hemangioma was found in 3 cases, and no case of arteriovenous fistula was found. We concluded that a triad of varicose vein, bony and soft-tissue hypertrophy, and cutaneous hemangioma is diagnostic in Klippel-Trenaunay syndrome. Conventional radiography, venography and arteriography could be recommended as procedures in diagnosis.