Management of congenital vascular malformations of the limbs

Abstract

Congenital vascular malformations (CVMs) of the limbs are rare lesions of uncertain etiology with structural disorders of the vascular system, nondegenerative and noninflammatory in origin, which occur during embryologic development. They affect one or more systems, commonly coexist with other malformations, and create a polymorphy, and their classification and radical cure are questionable.

Surgical treatment, where indicated, presupposes morphological and hemodynamic evaluation of each case, knowledge of the pathophysiology, and highly specialized surgical ability.

In this series of 31 patients with CVMs of the limbs, 12 arteriovenous (av) fistulae, 8 Klippel-Trenaunay syndromes, 1 Kasabach-Merritt syndrome, and 10 pure venous angiomata were studied. Ten of the 12 av fistulae were managed operatively, and the other CVMs were managed mainly conservatively.

Congenital av fistulae require surgical therapy because of their serious systemic and local complications. Surgical options include division and ligation of the fistula, skeletonization of the feeding artery, or resection of the fistula and ligation of the feeding vessels. In the other groups of CVMs nonoperative management offers the best results and consists of elastic support of the limb, appropriate profession selection, body weight control, orthopedic shoes, avoidance of oral contraceptives in women, and rarely, conservative operations on the veins of compression sclerotherapy.

In 6 of the 10 limbs with av fistulae operated on the results remain good at mean follow-up of 7.2 years, but 4 of the remaining recurred (rate 40%), and in 2 of the recurrences reoperation has been planned for further reduction of the shunt. In the CVMs other than av fistulae, the condition of the patients treated with conservative measures only has stabilized, whereas in the ones who had surgery, compression sclerotherapy, or laser therapy the results were unsatisfactory.