SPR Unknown case # 103 -- FINAL

Agustin Cardenas, MD

UCSF Benioff Children's Hospital

Peter Marcovici, MD

UCSF Benioff Children's Hospital


Publication Date: 2014-01-29

History

9 year old female with multiple episodes of bleeding, itching and scarring from different sites of her right leg. Physical exam demonstrates asymmetry between both lower extremities.

Past medical history relevant for coagulopathy, intermittent elevation of D-dimers and recent episode of deep venous thrombosis in the right leg.

Findings

Images # 1-8: coronal STIR images of both legs. There is asymmetry of both legs, being the right longer and wider than the left. Multiple serpiginous areas of hyperintensity are detected distributed throughout the subcutaneous tissue and muscle. The signal intensity of the bone is normal.

Image # 9: axial STIR image including both legs in the field-of-view. Again note the important asymmetry between both legs. The superficial veins are markedly dilated, there is cellulitis of the subcutaneous tissue as well as dilated lymphatic vessels and veins, which partially replace the muscle fibers. Of note, the vascular filling defects visualized in this image were later known to corresponded to motion artifact. At the time of diagnosis there was no evidence of venous thrombosis.

Image #10: coronal T1 of both legs, demonstrating similar findings as described in the images 1-8.

Diagnosis

Klippel Trenaunay Syndrome

Discussion

Klippel Trenaunay syndrome (KTS) is defined as a combination of the following features in an extremity: capillary malformation (“port-wine stain”), soft tissue and/or bone hypertrophy, lymphatic malformations and abnormal veins/varicosities.

Complications of this entity include thrombosis, thrombophlebitis, pulmonary embolism, cellulitis and bleeding from abnormal vessels.

Files

10 images