Congenital venous valvular aplasia of the lower extremities

A 10-year-old girl had bilateral, symmetrical swelling of the lower extremities, which had been present since the age of 1 year. Noninvasive vascular laboratory measurements of the ambulatory venous pressure, venous recovery time, and maximum venous outflow revealed profound bilateral lower extremity venous valvular incompetence. Duplex imaging of the veins of the lower extremities demonstrated no evidence of thrombosis, and no venous valves could be imaged. On phlebography, the patient was found to have no venous valves in the superficial and deep systems of the leg. We conclude that congenital absence of the venous valves of the lower extremities is almost certainly underdiagnosed and that the vascular laboratory can accurately and easily differentiate between lymphedema and venous valvular imcompetence. Such differentiation may have therapeutic implications.