Klippel-Trénaunay-Weber syndrome

Case contributed by Dr Michael P Hartung

Presentation

Multiple scans for disease evaluation.

Patient Data

Age: 30 years
Gender: Male

CT abdomen/pelvis

CT

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

MRI

Contrast enhanced and T2-weighted fat-suppressed imaging of the pelvis demonstrating multiple venous and lymphatic malformations. 

MRA

MRI

MRA TRICKS demonstrating sequential filling of multiple right lower extremity venous malformations. 

MR right knee

MRI

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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