Klippel-Trenaunay Syndrome



Klippel-Trenaunay syndrome is a rare sporadic complex congenital abnormality characterized clinically by the triad of capillary malformations (port-wine stain), soft tissue and bone hypertrophy or hypotrophy of one lower limb, and lateral varicosity with or without deep vein anomalies. It is a mixed vascular malformation with predominant capillary, venous, and lymphatic components [1]. It is often mistakenly identified as AV malformation. This chapter deals with the following aspects of this disease:


Vascular Malformation Varicose Vein Venous Malformation Superficial Vein Capillary Hemangioma 


  1. 1.
    Gloviczki P, Driscoll DJ. Klippel Trenaunay syndrome, current management. Phlebology. 2007;22:291–8.PubMedCrossRefGoogle Scholar
  2. 2.
    Klippel M, Trenaunay P. Du naevus variqueux osteohypertrophique. Arch Gen Med. 1900;185:641–72.Google Scholar
  3. 3.
    Weber FP. Angioma-formation in connection with hypertrophy of limbs and hemi-hypertrophy. Br J Dermatol. 1907;19:231–5.Google Scholar
  4. 4.
    Konrad EA, Mesiter P, Weber-Syndrome FP. Definition und differential diagnostische Abgrenzung. Der Pathologe, Heidelberg. 1981;2:103–5.Google Scholar
  5. 5.
    Lindenauer SM. The Klippel-Trenaunay-Weber syndrome: varicosity, hypertrophy and hemangioma with no arteriovenous fistula. Ann Surg. 1965;162:303–14.PubMedCrossRefPubMedCentralGoogle Scholar
  6. 6.
    Lee B-B. Venous embryology: the key to understanding anomalous venous conditions. Phlebolymphology. 2012;19(4):170–81.Google Scholar
  7. 7.
    Dodd H, Cockett FB. Pathology and surgery of veins of the lower limbs. 2nd ed. Edinburgh/London: Churchill Livingstone; 1976. p. 161.Google Scholar
  8. 8.
    Wang Q, Timur AA, Szafranski P, Sadgephour A, Jurecic V, Cowell J, Baldini A, Driscoll DJ. Identification and molecular characterization of de novo translocation t(8;14)(q22.3;q13) associated with a vascular and tissue overgrowth syndrome. Cytogenet Cell Genet. 2001;95(3–4):183–8.PubMedCrossRefPubMedCentralGoogle Scholar
  9. 9.
    Hu Y, Li L, Seidelmann SB, Timur AA, Shen PH, Driscoll DJ, Wang QK. Identification of Association of Common AGGF1Variants with Susceptibility for Klippel-Trenaunay Syndrome Using the Structure Association Program. Ann Hum Genet. 2008;72(Pt 5):636–43.PubMedCrossRefPubMedCentralGoogle Scholar
  10. 10.
    Lee A, Driscoll D, Gloviczki P, Clay R, Shaughnessy W, Stans A. Evaluation and management of pain in patients with Klippel-Trenaunay syndrome: a review. Pediatrics. 2005;115:744–9.PubMedCrossRefGoogle Scholar
  11. 11.
    Lee BB, Laredo J, Lee TS, Huh S, Neville R. Terminology and classification of congenital vascular malformations. Phlebology. 2007;22:249–52.PubMedCrossRefGoogle Scholar
  12. 12.
    Yamaki T, Konoeda H, Fugisawa D, Ogino K, Osada A, Hamahata A, Noazaki M, Sakurai H. Prevalence of various congenital vascular malformations in patients with Klippel Trenaunay syndrome. J Vasc Surg. 2013;1(2):187–93.Google Scholar
  13. 13.
    Cherry KJ, Gloviczki P, Stanson AW. Persistent sciatic vein: diagnosis and treatment of a rare condition. J Vasc Surg. 1996;23:490–7.PubMedCrossRefGoogle Scholar
  14. 14.
    Liu NF, Lu Q, Yan ZX. Lymphatic malformation is a common component of Klippel-Trenaunay syndrome. J Vasc Surg. 2010;52(6):1557–63.PubMedCrossRefGoogle Scholar
  15. 15.
    Jacob AG, Driscoll DJ, Shaughnessy WJ, Stanson AW, Clay RP, Gloviczki P. Klippel-Trenaunay syndrome: spectrum and management. Mayo Clin Proc. 1998;73:28–36.PubMedCrossRefGoogle Scholar
  16. 16.
    Akcali C, Inaloz S, Kirtak N, Ozkur A, Inaloz S. A case of Klippel-Trenaunay syndrome involving only upper limbs. G Ital Dermatol Venereol. 2008;143(4):267–9.PubMedGoogle Scholar
  17. 17.
    Funayama E, Sasaki S, Oyama A, Furukawa H, Hayashi T, Yamamoto Y. How do the type and location of a vascular malformation influence growth in Klippel-Trénaunay syndrome? Plast Reconstr Surg. 2011;127(1):340–6.PubMedCrossRefGoogle Scholar
  18. 18.
    Bastarrika G, Redondo P, Sierra A, et al. New techniques for the evaluation and therapeutic planning of patients with Klippel-Trenaunay syndrome. J Am Acad Dermatol. 2007;56:242–9.PubMedCrossRefGoogle Scholar
  19. 19.
    Li X, Tian J. Multidetector row computed tomography arteriography in the preoperative assessment of patients with Klippel-Trénaunay syndrome. J Am Acad Dermatol. 2009;60(2):345–6.PubMedCrossRefGoogle Scholar
  20. 20.
    McDonagh B, Sorenson S, Cohen A, Eaton T, Huntley DE, La Baer S, Campbell K, Guptan RC. Management of venous malformations in Klippel–Trenaunay syndrome with ultrasound-guided foam sclerotherapy. Phlebology. 2005;20(2):63–81.CrossRefGoogle Scholar
  21. 21.
    Huang Y, Jiang M, Li W, Lu X, Huang X, Lu M. Endovenous laser treatment combined with a surgical strategy for treatment of venous insufficiency in lower extremity: a report of 208 cases. J Vasc Surg. 2005;42(3):494–501.PubMedCrossRefGoogle Scholar

Copyright information

© Springer India 2015

Authors and Affiliations

  1. 1.General SurgeryAmrita Institute of Medical Sciences, Amrita LaneKochiIndia

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