DX: Klippel-Trenaunay-Weber Syndrome

This is a rare case that can actually be diagnosed clinically. Young patients with swollen leg, vascular ectasia and port wine stains are to consider. Radiographic findings may vary but usually are more depicted on MRI.

The most common major imaging finding is overgrowth of subcutaneous soft tissues in the lower limbs particularly unilateral as in this case. Vascular overgrowth appears as an area with slow uptake of contrast material in the delayed phase, a finding indicative of low flow. Deep venous malformations are commonly seen in the femoral vein. Because of overgrowth of soft tissues, the affected limb is usually thicker and longer than the nonaffected limb.

Unfortunately treatment is symptomatic and conservative in most cases and to prevent thrombophlebitis, celulitis mostly.

Here is a video that I found in Youtube similar to this case:

http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001205/

http://emedicine.medscape.com/article/1084257-overview

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