3:54 PM Abstract No. 162 Persistent embryonic and orthotopic venous anatomy in Klippel-Trenaunay syndrome

Abstract

Persistent embryonic veins (marginal and sciatic veins) and occasional orthotopic veins may cause phlebectasia in patients with Klippel-Trenaunay syndrome (KTS). These incompetent veins increase the risk of thromboembolism. It is essential to delineate the venous anatomy of the lower extremity prior to attempting closure of the anomalous veins. The purpose of this study was to evaluate the drainage pattern of persistent embryonic veins as well as abnormal dilation of orthotopic veins. Imaging studies of patients with KTS who underwent contrast venography in our vascular anomalies center in the last 16 years were reviewed. Ninety-three lower extremities in 89 patients (4 months – 33 years old, median 11 years old, 42 female) were included. Four patients underwent bilateral lower extremity venography. Eighty-five of 93 lower extremities (91%) showed marginal veins, 54 lower extremities (58%) showed sciatic veins, and 8 lower extremities (9%) showed no embryonic vein. Forty-seven lower extremities (55%) showed single draining veins and 38 lower extremities (45%) showed more than two draining veins of the marginal veins. The common draining veins of the marginal veins were sciatic (64%), deep femoral (41%), gluteal (31%), and great saphenous veins (16%). The draining veins of the sciatic veins were internal iliac (83%) and deep femoral veins (17%). The abnormally dilated orthotopic veins were inferior vena cava (48%), common iliac (44%), internal iliac (43%), small saphenous (42%), popliteal (22%), and external iliac veins (14%). Most of the patients with KTS showed persistent embryonic veins, and a substantial number of the patients showed abnormally dilated orthotopic veins. The common draining veins of the marginal veins were sciatic, deep femoral, gluteal, and great saphenous veins. The draining veins of the sciatic veins were internal iliac and deep femoral veins.Tabled 1Draining Veins of Marginal Veins∗A total of 38 lower extremities showed single draining veins, and 47 lower extremities showed multiple draining veins of marginal veins. Sciatic veins, 54/85 (64%); deep femoral veins, 35/85 (41%); gluteal veins, 26/85 (31%); and great saphenous veins, 14/85 (16%).Draining veinsNumber (% of 85)Complete sciatic vein24 (28.2%)Upper sciatic vein21 (24.7%)Lower sciatic vein9 (10.6%)Common femoral vein4 (4.7%)Deep femoral vein35 (41.2%)Femoral vein4 (4.7%)Superior gluteal vein16 (18.8%)Inferior gluteal vein10 (11.8%)Great saphenous vein14 (16.4%)Popliteal vein1 (1.2%)∗ A total of 38 lower extremities showed single draining veins, and 47 lower extremities showed multiple draining veins of marginal veins. Sciatic veins, 54/85 (64%); deep femoral veins, 35/85 (41%); gluteal veins, 26/85 (31%); and great saphenous veins, 14/85 (16%). Open table in a new tab