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Epidemiological profile of 58 patients with Klippel-Trenaunay-Weber syndrome followed at Ambulatório da Santa Casa de São Paulo

BACKGROUND: Klippel-Trenaunay-Weber syndrome is a rare disorder about which there are few articles available (most of them are sporadic case reports related to complications). OBJECTIVE: To assess the epidemiological profile of patients with Klippel-Trenaunay-Weber syndrome. METHODS: Medical records of 58 patients followed at the lymphatic disease and angiodysplasia outpatient clinic for the discipline of Vascular Surgery at the School of Medical Sciences of Santa Casa de São Paulo were reviewed. RESULTS: The disorder affected males and females equally (30 males and 28 females). The mean age of patients under treatment was 12.8 years. In most cases, the syndrome had been diagnosed in childhood, with Port wine stain as the first sign noticed by the family at birth or during the first year of life. The most frequently reported symptom was debilitating pain usually associated with symptoms of venous stasis. The CEAP classification was C0 and C1 for younger patients and C4-C6 for most older patients. Arteriovenous fistulas were diagnosed in 8.5% of cases. Positive family history was reported by only 6.8% of patients. CONCLUSION: Klippel-Trenaunay and Parkes Weber syndromes are different presentations of a same disorder and they can be studied as one, Klippel-Trenaunay-Weber syndrome. Childhood is the optimal moment to identify affected patients and to reduce progression of venous insufficiency and bony and soft tissue hypertrophy. Compression should be indicated for all affected patients to reduce progression of peripheral venous disease.

Angiodysplasias; Klippel-Trenaunay-Weber syndrome; vascular; syndrome


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