Congenital aplasia of the deep veins of lower extremities in children: The role of ascending functional phlebography

Eighty-two children with vascular diseases underwent phlebography. Congenital aplasia of the deep venous system of the lower extremities was diagnosed in 10 children. With ascending functional phlebography, three anatomic variations with different degrees of venous dysfunction were defined: (a) aplasia of the femoral vein with collateral drainage into the common femoral vein, (b) aplasia of the femoral vein with drainage into the internal iliac vein, and (c) total aplasia with drainage into the axillary vein. The phlebographic findings correlated with the symptoms. Patients with type a aplasia had no clinical and radiologic signs of venous insufficiency. Patients with types b and c aplasia had severe venous insufficiency that necessitated supportive treatment. Palliative surgical procedures such as hemorrhoidectomy, clitoridectomy, and excision of bleeding vascular nevi were performed. Ligation or excision of the dilated superficial veins (usually the main channels for venous drainage from the extremity) is absolutely contraindicated. Ascending functional phlebography is therefore essential for correct diagnosis and planning of future therapy.