RESUMO
OBJECTIVES: The purpose of this study is to illustrate a rare vascular malformation and to make a literature review. PATIENT: Klippel-Trenaunay's syndrome is a congenital disorder which characterized by capillary malformation, varicosities and bony or soft tissue hypertrophy. This disease is subject to significant morbidity like bleeding, deep vein thrombosis, embolic complications and in some cases enlargement of limb that may require amputation. Our case study was a young woman of twenty years suffering since birth, from a painful, heaviness and enlarged left lower limb that reached 2.5 cm. The distal hypertrophy at the big toe which the site of a botriomycoma (telengectasic granuloma). The inner side of the thigh was also the site of small port-wine stains and varicose of the great saphenous vein. Vascular Doppler ultrasound, skeletal computed tomography and angio-MRI of the limb indicated a klippel-Trenaunay disease. A surgical abstention was decided. The patient underwent only to resection of the toe's botriomycoma followed by an elastic contention associated with "a heel pad compensation" and an anti platelet therapy to prevent deep vein thrombosis and embolic complications. CONCLUSION: The syndrome of Klippel-Trenaunay is a rare vascular malformation. The basic pathology can not be corrected. The treatment's aims are to control varicose veins, to prevent complications and to preserve aesthetic and functional prognosis of the leg.
