Klippel and Trenaunayʼs Syndrome

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Abstract
Since 1945, 786 patients with Klippel and Trenaunay''s syndrome were operated on by the authors. Elongation of the impaired limb was invariably found while edema was present in 84%, varicose in veins in 36% and flat angiomata in 32%. Venography and surgical exploration demonstrated malformation of the deep veins involving the popliteal vein in 51%, superficial femoral vein, 16%; both popliteal and superficial femoral veins; 29%; iliac veins, 3%; and lower vena cava 1%. Good clinical results were achieved following the surgical release of these deep veins in the lower limb. During childhood, when the difference in limb length is noteworthy, ligature of the popliteal vein of the shorter limb induces a compensating elongation. Klippel and Trenaunay''s syndrome may be associated with lymphatic malformations, including lymphedema and malformation of the lymph vessels. Knowledge of the pathophysiology of these malformations of the deep veins enables a better undestanding of the clinical manifestations of the condition, and the improved treatment of the serious vesical or rectal hemorrhage which occurs in 1% of these patients.