Klippel–trenaunay syndrome: A case report with radiological review

Abstract

Klippel–Trenaunay Syndrome is a congenital disorder with a rare incidence of 3-5/1,00,000. It is characterized by a triad of capillary malformation (hemangioma or port-wine stain), venous varicosities, and bony or, soft-tissue hypertrophy. The malformation in the capillary is usually limited to a single extremity though multiple extremities can be involved. This disease is subject to significant morbidities such as bleeding, deep vein thrombosis, and embolic complications. Our case study was a young male of 6 years suffering since birth, from a painful port-wine stain on the lateral aspect of the right thigh, enlarged right femur, and soft tissues. Vascular Doppler ultrasound, skeletal X-ray, and magnetic resonance imaging of the limb indicated a Klippel–Trenaunay disease. This article describes a case study of a child presenting with Klippel–Trenaunay including a review of the syndrome and treatment recommendations.

Keywords

• Angio-osteohypertrophy
• hemangiectasia hypertrophicans and nevus verucosus hypertrophicans
• Klippel–Trenaunay Syndrome
• nevus varicosus osteohypertrophicus syndrome