Advertisement

Surgery Today

, Volume 27, Issue 8, pp 735–740 | Cite as

Klippel-Trénaunay Syndrome: Clinical features, complications, and management

  • Mussaad M. S. Al-Salman
Original Articles

Abstract

Klippel-Trénaunay Syndrome (KTS) is rare and not well recognized among most physicians, which has lead to inappropriate management and delayed referral in many cases. Presented herein is the largest series ever reported from a single center in Saudi Arabia, comprised of 18 patients seen between 1990 and 1996, whose clinical features, complications, and management are discussed. All 18 patients had large angiomatous navei, hypertrophy of the soft tissue with bone overgrowth in the lower limbs, and extensive lower limb varicosities, which extended to the buttocks and lower abdomen in 2 patients. There were 4 patients who had undergone at least one operation for lower limb varicose veins prior to referral. The diagnostic workup and treatment for KTS is discussed in detail. It is essential that physicians understand how this complex syndrome presents, and the most appropriate means of investigating and managing it, to avoid unnecessary surgery being performed prior to referral to a specialist center.

Key Words

Klippel-Trénaunay syndrome varicosities soft tissue overgrowth bone overgrowth 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    Servelle M (1985) Klippel and Trenaunay sydrome. 788 operated cases. Ann Surg 201:365–373PubMedGoogle Scholar
  2. 2.
    Lindenauer SM (1965) The Klippel-Trenaunay Syndrome: varicosity, hypertrophy, and hemangioma with no arteriovenous fistula. Ann Surg 162:203–213Google Scholar
  3. 3.
    Baskerville PA, Ackroyd JS, Thomas ML, Browse NL (1935) The Klippel-Trenaunay syndrome: Clinical, radiological, and hemodynamic features and management. Br J Surg 72:232–236Google Scholar
  4. 4.
    Gorenstein A, Katz S, Schiller M (1988) Congenital angiodysplasia of the superficial venous system of the lower extremities in children. Ann Surg 207:312–318Google Scholar
  5. 5.
    Vollmar J, Voss E (1979) Vena marginalis lateralis persistens: die vergessene Vene der Angiologen. Vasa 8:192–202PubMedGoogle Scholar
  6. 6.
    Klippel M, Trénaunay P (1990) Du naevus variquex ostéohypertrophique. Arch Gen Med (Paris) 3:614–672Google Scholar
  7. 7.
    Young AE (1978) Congenital mixed vascular deformities of the limbs and their associated lesions. Birth Defects 14:289–296PubMedGoogle Scholar
  8. 8.
    Baskerville PA, Ackroyel JS, Brouse NL (1985) The etiology of the Klippel-Trenaunay syndrome. Ann Surg 202:624–627PubMedGoogle Scholar
  9. 9.
    Szilagyi DE, Smith RF, Elliott JP, Hageman JH (1976) Congenital arteriovenous anomalies of the limb. Arch Surg 111:423–429PubMedGoogle Scholar
  10. 10.
    Aelvoet GE, Jorens PG, Roelen LM (1992) Genetic aspects of the Klippel Trenaunay syndrome. Br J Dermatol 126:603–607PubMedGoogle Scholar
  11. 11.
    Gloviczki P, Hollier LH, Telander RL, Kaufman B, Bianco AJ, Stickler GB (1983) Surgical implications of Klippel-Trenaunay syndrome. Ann Surg 197:353–362PubMedGoogle Scholar
  12. 12.
    Gloviczki P, Stenson AW, Stickler GB, Johnson CM, Toomey BJ, Meland NB, Rooke TW, Cherry KJ (1991) Klippel-Tranaunay syndrome. The risk and benefit of vascular intervention. Surgery 110:469–479PubMedGoogle Scholar
  13. 13.
    Telander RL, Kaufman BH, Glovicski P, Stickler GB, Hollier LH (1984) Prognosis and management of lesions of the trunk in children with Klippel-Trenaunay syndrome. J Pediatric Surg 19:417–422Google Scholar
  14. 14.
    Taheri SA, Williams J, Boman L, Pisanos (1989) Superficial femoral vein transposition in Klippel Trenaunay syndrome. J Pediatr Surg 24:494–496PubMedGoogle Scholar
  15. 15.
    Folkman J, Klagsburn M (1987) Angiogenic factors. Science 235:442–447PubMedGoogle Scholar

Copyright information

© Springer-Verlag 1997

Authors and Affiliations

  • Mussaad M. S. Al-Salman
    • 1
  1. 1.College of MedicineKing Saud UniversityRiyadhSaudi Arabia

Personalised recommendations