Rheumatologie: Aktuelle Forschung
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Multidisciplinary Approach to Treatment of Severe Klippel-Tranaunay-Weber Syndrome

Renuka Gupta

Klippel-Trenaunay-Weber Syndrome (KTWS) is an extremely rare congenital vascular disorder occurring in less than 1 in 10,000 live birth . KTWS consists of a triad of port-wine stain, varicosities or venous malformations, and bony and soі tissue hypertrophy. Approximately 63% of patients have all three features of the syndrome. Deep venous abnormalities, brain abnormalities (including AV malformation, aneurysm and atrophy), and hemangiomas of visceral organs including the spleen and bladder are associated with KTWS. Pulmonary thromboembolism and hypercoagulability are other rare complications . Нis can present as a consumptive coagulopathy leading to thrombocytopenia and bleeding, known as “Kasabach-Merritt Syndrome” .