[Klippel-Trenaunay syndrome with complications during pregnancy]

In Klippel-Trenaunay syndrome there are congenital vascular malformations, usually involving the limbs. It is characterized by extreme varicose veins, bone and soft tissue hypertrophy and pigmentary skin changes in the affected limbs. We report a 29-year-old pregnant woman with this syndrome. The combination of the physiologic changes which occur in the vascular system and in blood coagulation during pregnancy, and the malformations of this syndrome is very unusual. During the patient's pregnancy there was significant worsening of the typical complications. There was extreme dilatation of varicose veins in the left leg, from the ankle up to the posterior aspect of the buttock. The involved area was swollen and edematous, and there were signs of superficial thrombophlebitis. Because we were unable to rule out deep vein thrombosis in the affected limb, prophylactic anticoagulant treatment was given during the 3rd trimester and into the early puerperium. Only then did the complications begin to resolve, but return to the prepregnant state was incomplete.