Klippel-Trénaunay-Weber syndrome
Presentation
Multiple scans for disease evaluation.
Patient Data
CT abdomen/pelvis
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Splenic hemangiomatosis. Right retroperitoneal venous/lymphatic malformations with small phleoboliths. Extensive venous/lymphatic malformations in the right flank, scrotum, and imaged right lower extremity.
MR Pelvis
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Contrast enhanced and T2-weighted fat-suppressed imaging of the pelvis demonstrating multiple venous and lymphatic malformations.
MRA
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MRA TRICKS demonstrating sequential filling of multiple right lower extremity venous malformations.
MR right knee
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Multiple MRI sequences of the right knee showing hypertrophy of the lower extremity with numerous venous and lymphatic malformations.
Case Discussion
Klippel-Trénaunay-Weber syndrome is diagnosed with at least two of these three features are present: capillary malformations, soft tissue or bone hypertrophy, and varices or venous malformations. Most cases are sporadic. Most cases involve the lower limb as the site of malformations.
These patients are at risk for sequestration of platelets, resulting in Kasabach-Merritt syndrome (a consumptive coagulopathy). They can also suffer from stasis dermatitis, thrombophlebitis, cellulitis, congestive heart failure, bleeding, and thromboembolic events.
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