Congenital Angiodysplasia of the Lower Limb: The Klippel—Trenaunay Syndrome and Arteriovenous Fistulae

Abstract

During a 10-year period 13 patients were admitted on suspicion of congenital arteriovenous fistulae. Two patients turned out to have a Klippel-Trenaunay syndrome without evidence of arteriovenous fistula and two patients had agenesis of the venous drainage system. Evaluation by noninvasive methods for classification of this rare condition is of the microsphere injection technique before arteriography and venous emptying measurement before venography. From our experience only the microsphere technique is recommended to evaluate the severity of the shunting.

The possibility of a partial excision of the angiodysplasia is the task of the surgeon facing the impossibility of radical operation. Therefore arteriograms should guide the surgeon in his attempt to reduce the hemodynamic consequence of shunting. Patients having a normal arteriogram should have a venogram before surgical exploration is attempted. In the series three patients turned out to have venous defects and removal of superficial varicosities would have aggravated the situation.