Primary Lymphedema with Concomitant Vascular and Lymphatic Lesions

Primary extremity lymphedema is a debilitating disease with a reported incidence of 1-3 in every 10,000 births, and it may present with concomitant vascular lesions or retroperitoneal lymphangiomatosis with chylous ascites. The diagnosis of primary extremity lymphedema includes history, physical examination, lymphoscintigraphy, and indocyanine green lymphography. Doppler ultrasound and computed tomography angiography are the key evaluation tools for concomitant vascular lesions. Magnetic resonance imaging and single photon emission computed tomography are required for the diagnosis of retroperitoneal lymphangiomatosis. Microsurgical procedures, including lymphovenous anastomosis and vascularized lymph node transfer, have been shown to provide functional improvements in primary extremity lymphedema. Most patients with primary extremity lymphedema require vascularized lymph node transfer due to severe and prolonged symptoms or total obstruction on lymphoscintigraphy. Vascular stenting is indicated and effective for proximal venous or arterial occlusion, which is recommended to be performed 6 months before lymphedema microsurgery for primary extremity lymphedema. Chylovenous bypass is effective for solving the problem of retroperitoneal lymphangiomatosis and can be performed at the time of lymphedema microsurgery if diagnosis is confirmed along with primary extremity lymphedema.