Advertisement

Klippel-Trenaunay Syndrome

  • Magdiel Trinidad-Hernandez
  • Peter Gloviczki
Chapter

Case Report

A 38-year-old woman with the diagnosis of Klippel-Trenaunay Syndrome (KTS) presents with severe pain over venous malformations on the left thigh and severe hyperhidrosis of the left leg and foot. She has a history of pulmonary embolism at age 17. Otherwise, she is healthy and compliant with the use of compression garments. The diagnosis of KTS was made soon after birth because of the port wine stains, slightly larger and longer leg and lateral varicose veins of her left leg. Physical examination shows a port wine nevus on the left buttock with varicose veins and soft tissue hypertrophy along the anterolateral aspect of the left leg and thigh. These areas are tender to palpation and leg elevation eases their appearance. Hyperhidrosis behind the knee and dorsum of the foot and in the interdigital spaces is obvious. Limb length discrepancy is 1 cm. The remainder of the exam is unremarkable.

Question 1

Which of the following is not a characteristic finding in patients with KTS?

References

  1. 1.
    Bergan J, Cheng V. Foam sclerotherapy of venous malformations. Phlebology. 2007;22(6):299–302.CrossRefPubMedGoogle Scholar
  2. 2.
    Servelle M, Bastin R, Loygue J, et al. Hematuria and rectal bleeding in the child with Klippel and Trenaunay syndrome. Ann Surg. 1976;183(4):418–28.CrossRefPubMedPubMedCentralGoogle Scholar
  3. 3.
    Servelle M. Klippel and Trenaunay’s syndrome. 768 operated cases. Ann Surg. 1985;201(3):365–73.CrossRefPubMedPubMedCentralGoogle Scholar
  4. 4.
    Cherry KJ, Gloviczki P, Stanson AW. Persistent sciatic vein: diagnosis and treatment of a rare condition. J Vasc Surg. 1996;23(3):490–7.CrossRefPubMedGoogle Scholar
  5. 5.
    Gloviczki P, Driscoll J. Klippel-Trenaunay syndrome: current management. Phlebology. 2007;22(6):291–8.CrossRefPubMedGoogle Scholar
  6. 6.
    Delis KT, Gloviczki P, Wennberg PW, Rooke TW, Driscoll DJ. Hemodynamic impairment, venous segmental disease, and clinical severity scoring in limbs with Klippel-Trenaunay syndrome. J Vasc Surg. 2007;45(3):561–7.CrossRefPubMedGoogle Scholar
  7. 7.
  8. 8.
    Noel AA, Gloviczki P, Cherry KJ, Rooke TW, Stanson AW, Driscoll DJ. Surgical treatment of venous malformations in Klippel-Trenaunay syndrome. J Vasc Surg. 2000;32:840–7.CrossRefPubMedGoogle Scholar
  9. 9.
    Burrows PE, Mason KP. Percutaneous treatment of low flow vascular malformations. J Vasc Interv Radiol. 2004;15:431–45.CrossRefPubMedGoogle Scholar
  10. 10.
    Cabrera J, Cabrera J Jr, Garcia-Olmedo MA, Redondo P. Treatment of venous malformations with sclerosant in microfoam form. Arch Dermatol. 2003;139(11):1409–16.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Division of Vascular and Endovascular Surgery, Gonda Vascular CenterMayo ClinicRochesterUSA

Personalised recommendations