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KMID : 0363219750130030223
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Korean Journal of Dermatology
1975 Volume.13 No. 3 p.223 ~ p.229
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A Case of Klippel-Trenaunay-Weber Syndrome with Congenital Heart Anomaly
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金蘭姬/Kim, Nan Hee
金秀男/兪泰演/Kim, Soo Nam/Yoo, Tae Yun
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Abstract
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A 17 year old female with Klippel-Trenaunay-Weber Syndrome is presented. Of particular interest was presence of congenital heart anomaly.
The nevus flammeus involved her entire right leg and foot with hypertrophy, elongation and partial ulecration. Elongation of the right leg lead compensatory 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 1Q systolic blowing murmur was heard on left upper sternal border with second heart sound splitting.
Electrocardiogram revealed complete right bundle branch block (Fig. 3).
Chest X-ray revealed cardiomegaly, increased hilar shadows and mild pulmonary congestion (Fig. 4).
Above mentioned signs suggests strongly secundum type of atrial septal defect. Femoral angiography didn´t show arteriovenous malformation (Fig. 5).
Authors experienced this rare variant of Klippel-Trenaunay-Weber Syndrome accomapanying with congenital heart anomaly, and report this with review of literatures.
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