- Klippel-Trenaunay syndrome (KTS) is defined as a combination of capillary malformations, soft-tissue or bone hypertrophy, and varicose veins or venous malformations.
- The diagnosis of KTS can be made when any two of the three features are present.
- Most cases of KTS are sporadic; the syndrome affects males and females equally, has no racial predilection, and manifests at birth or during childhood.
- Enlargement of the extremity consists of bone elongation, circumferential soft-tissue hypertrophy, or both.
- Often manifests as leg-length discrepancy, although any limb may be affected.
 Capillary malformations
- Most common cutaneous manifestation of KTS.
- Typically, capillary malformations involve the enlarged limb, although skin changes may be seen on any part of the body.
- The lower limb is the site of malformations in approximately 95% of patients.
- If large enough, the cutaneous lesions may sequester platelets, possibly leading to Kasabach-Merritt syndrome (consumptive coagulopathy).
 Varicose veins
- Present in a majority of patients with KTS.
- Venous malformations can occur in both the superficial and deep venous systems.
- Superficial venous abnormalities range from ectasia of small veins to persistent embryologic veins and large venous malformations.
- Deep venous abnormalities include aneurysmal dilatation, aplasia, hypoplasia, duplications, and venous incompetence.
- Treatment in a majority of patients with Klippel-Trénaunay syndrome is conservative and includes application of graded compressive stockings or pneumatic compression devices to the enlarged extremity.
- Percutaneous sclerosis of localized venous malformations or superficial venous varicosites may be indicated in some patients.
- Surgical treatment may include epiphysiodesis to control leg length discrepancy, excision of soft-tissue hypertrophy, and stripping of superficial varicose veins.
 Imaging Findings
- At radiography, bone elongation contributing to leg length discrepancy, soft-tissue thickening, or calcified phleboliths may be seen.
- T2-weighted MR images show malformed venous and lymphatic lesions as areas of high signal intensity.
- MR imaging depicts deep extension of low-flow vascular malformations into muscular compartments and the pelvis and their relationship to adjacent organs as well as bone or soft-tissue hypertrophy.
 See Also
 External Links
- Susan H. Cha, Michael A. Romeo, and Janet A. Neutze. Visceral Manifestations of Klippel-Trénaunay Syndrome. RadioGraphics 2005 25: 1694-1697.
- Charles A. James, Janice W. Allison, and Milton Waner. Pediatric Case of the Day. RadioGraphics 1999 19: 1093-1096.