A Case of Klippel-Trenaunay-Weber Syndrome with Congenital Heart Anomaly / 대한피부과학회지

ABSTRACT

A 17 year old female with Klippel-Trenaunay-Weber Syndrome is presented. Of particular interest was presence of congenital heart anomaly. The nevus flarnmeus invoIved her entire right leg and foot with hypertrophy, elongation and partial ulecration. Elongation of the right leg Iead cornpensatory vertebral scoliosis (Fig.1,2). The cutaneous temperature of affected side was higher than the other side, and. was larger on somatometry and osteometry (Table 1). On auscultation, grade III systolic blowing murmur was heard on left upper sternal border with second heart sound splitting. Electrocardiograrn revealed complete right bundle branch block (Fig. 3). Chest X-ray revealed cardiomegaly, increase 3 hilar shadaws and rnild pulmonary congestion (Fig. 4). Above mentioned signs suggests strongly secundum type of atrial septal defect. Femoral angiography didnt show arteriovenous malformation (Fig. 5). Authors experienced this rare variant of Klippel-Trenaunzy-Weber Syndrome acco- mapanying with congenital heart anamaly, and report this with review of literatures.