Klippel-Trenaunay syndrome

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[edit] Discussion

  • 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.

[edit] Hypertrophy

  • 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.

[edit] 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).

[edit] 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-TrĂ©naunay 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.

[edit] 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.

[edit] Images

[edit] See Also

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