Vascular CT Appearances

Klippel-Trenaunay Syndrome (KTS) CT Findings

• Capillary malformation (“port-wine stain”)
• Venous malformation, AVM, and/or lymphatic malformation
• Atypical veins that are large and tortuous
• Hemangiomas in the muscle, spleen, and/or colon
• Limb hypertrophy
• Most commonly affects the lower limbs but can also affect the upper limbs

Other Information About Klippel-Trenaunay Syndrome (KTS)

Etiology:

• KTS is a genetic disorder usually caused by mutations

Epidemiology:

• Rare

Presentation:

• “Port-wine stain” birthmark
• Vein malformations visible through the skin
• Abnormal growth of bone and soft tissue

Prognosis:

• Symptoms of KTS can be managed
• KTS itself is not fatal but increases the risk of fatal complications