Brief Description of the Purpose of the StudyKlippel-Trenaunay-Weber Syndrome (KTWS) is a rare congenital disease. The clinical diagnostic criteria consists of the classic triad: capillary malformations in the skin, bone or soft tissue hypertrophy and vascular malformations. Complex vascular anomalies may occur (venous, arterial, lymphatic) as well as arteriovenous fistulas (AVFs), a frequent cause of pain. The purpose of this study is to report clinical and sonographic findings encountered in KTWS.MethodsBetween June 2009 and November 2012, 80 patients (ages ranging from 5 months to 45 years, 61% women) with a clinical diagnosis of KTWS were examined. Clinical evaluation and usual Ultrasound (US) Doppler techniques were used with an exam protocol developed for KTWS.Main ResultsMain clinical findings: port-wine skin patches(80%);lesions on lower limb(78%); on upper limb(18%); bilateral(10%), on all limbs(5%); increase in the length of the limb (50%); increase of limb volume (72%). Main sonographic findings: deep venous malformations (49%); lateral marginal vein (35%); anomalous arterial branches (96%); AVFs (88%); venous aneurisms (23%, among which 35% with AVFS); thrombosis (15%) and lymphatic cysts (19%).Importance of the ConclusionsWhen performed sistematically, the US-Doppler provides morphologic and hemodynamic evaluation of complex vascular malformations, especially of AVFs, indispensable in the treatment of KTWS. Brief Description of the Purpose of the StudyKlippel-Trenaunay-Weber Syndrome (KTWS) is a rare congenital disease. The clinical diagnostic criteria consists of the classic triad: capillary malformations in the skin, bone or soft tissue hypertrophy and vascular malformations. Complex vascular anomalies may occur (venous, arterial, lymphatic) as well as arteriovenous fistulas (AVFs), a frequent cause of pain. The purpose of this study is to report clinical and sonographic findings encountered in KTWS. Klippel-Trenaunay-Weber Syndrome (KTWS) is a rare congenital disease. The clinical diagnostic criteria consists of the classic triad: capillary malformations in the skin, bone or soft tissue hypertrophy and vascular malformations. Complex vascular anomalies may occur (venous, arterial, lymphatic) as well as arteriovenous fistulas (AVFs), a frequent cause of pain. The purpose of this study is to report clinical and sonographic findings encountered in KTWS. MethodsBetween June 2009 and November 2012, 80 patients (ages ranging from 5 months to 45 years, 61% women) with a clinical diagnosis of KTWS were examined. Clinical evaluation and usual Ultrasound (US) Doppler techniques were used with an exam protocol developed for KTWS. Between June 2009 and November 2012, 80 patients (ages ranging from 5 months to 45 years, 61% women) with a clinical diagnosis of KTWS were examined. Clinical evaluation and usual Ultrasound (US) Doppler techniques were used with an exam protocol developed for KTWS. Main ResultsMain clinical findings: port-wine skin patches(80%);lesions on lower limb(78%); on upper limb(18%); bilateral(10%), on all limbs(5%); increase in the length of the limb (50%); increase of limb volume (72%). Main sonographic findings: deep venous malformations (49%); lateral marginal vein (35%); anomalous arterial branches (96%); AVFs (88%); venous aneurisms (23%, among which 35% with AVFS); thrombosis (15%) and lymphatic cysts (19%). Main clinical findings: port-wine skin patches(80%);lesions on lower limb(78%); on upper limb(18%); bilateral(10%), on all limbs(5%); increase in the length of the limb (50%); increase of limb volume (72%). Main sonographic findings: deep venous malformations (49%); lateral marginal vein (35%); anomalous arterial branches (96%); AVFs (88%); venous aneurisms (23%, among which 35% with AVFS); thrombosis (15%) and lymphatic cysts (19%). Importance of the ConclusionsWhen performed sistematically, the US-Doppler provides morphologic and hemodynamic evaluation of complex vascular malformations, especially of AVFs, indispensable in the treatment of KTWS. When performed sistematically, the US-Doppler provides morphologic and hemodynamic evaluation of complex vascular malformations, especially of AVFs, indispensable in the treatment of KTWS.