Klippel-Trenaunay syndrome in a patient presenting with venous stasis ulcer

Kevser Tural; Zeynep Bilgi; Ceyhun Coşkun

doi:10.28982/josam.415187

Authors

Kevser Tural
Zeynep Bilgi
Ceyhun Coşkun

DOI:

https://doi.org/10.28982/josam.415187

Keywords:

Klippel-Trenaunay syndrome, Port-wine stain, Venous ulcer, Limb hypertrophy

Abstract

Klippel-Trenaunay sendromu kapiller malformasyon, yumuşak doku ve kemik hipertrofisi ve atipik varikoziteler ile karakterize edilen konjenital bir kapiler venöz vasküler malformasyondur. Klippel-Trenaunay sendromu genellikle alt ekstremiteyi etkiler; ancak kollar, gövde, baş, boyun, abdominal ve pelvik organlar ve santral sinir sistemini de etkileyebilir. Derin venöz sistem anomalileri, bu vakaların tedavisinin yönlendirilmesinde önemlidir. Derin ven hipoplazisi olan olgularda marjinal ven rezeke edilebilir çünkü rezeksiyondan sonra hipoplastik derin venler kendiliğinden dilate olurlar. Derin venöz aplazisi olan olgularda ise embriyonal ven bacağın ana drenaj veni olabilir ve rezeksiyonu olası değildir. Bu yazıda, sağ bacak çapında artış, port-wine lekesi görünümü ve venöz ülseri bulunan, Klippel-Trenaunay sendromu olan 20 yaşında erkek olgu sunulmuştur.

Downloads

Download data is not yet available.

References

Klippel M, Trenaunay P. Du naevus variquex osteohypertrophique. Arch Genet Med Paris. 1900;3:11-72.

Weber FP. Angioma formation in connection with hypertrophy of limbs and hemi-hypertrophy. Brit J Derm. 1907;19:231-5.

Berry SA, Peterson C, Mize W, Bloom K, Zachary C, Blasco P, et al. Klippel-Trenaunay syndrome. Am J Med Genet. 1998;79:319-26.

Al-Salman MM. Klippel-Trenaunay syndrome: clinical features, complications, and management. Surg Today. 1997;27:735-40.

Ghahremani GG, Kangarloo H, Volberg F, Meyers MA. Diffuse cavernous hemangioma of the colon in the Klippel-Trenaunay syndrome. Radiology. 1976;118:673-8.

Meier S. Klippel-Trenaunay syndrome. Advances in Neonatal Care. 2009;9:120-4.

Whelan AJ, Watson MS, Porter FD, Steiner RD. Klippel-Trenaunay-Weber syndrome associated with a 5:11 balanced translocation. Am J Med Genet. 1995;59:492-4.

Aelvoet GE, Jorens PG, Roelen LM. Genetic aspect of the Klippel-Trenaunay syndrome. Br J Dermatol. 1992;126:603-7.

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.

Servelle M. Klippel and Trenaunay’s syndrome.768 operated cases. Ann Surg. 1985;201(3):365-73.

Capraro PA, Fisher J, Hammond DC, Grossman JA. Klippel-Trenaunay syndrome. Plastic Reconstr Surg. 2002;109:2052-62.

Gloviczki P, Driscoll DJ. Klippel-Trenaunay syndrome: current management. Phlebology. 2007; 22:291-8.

Weber FP. Hemangiectatic Hypertrophy of Limbs-Congenital Phlebacteriectasis and So-called Congenital Varicose Veins. Brit J Child Dis. 1918;15:13-7.

Lindenauer SM. The Klippel Trenaunay syndrome: varicosity, hypertrophy and haemangioma with no arteriovenous fistula. Ann Surg. 1965;162:303-14.

Baskerville PA, Ackroyd JS, Lea Thomas M, Browse NL. The Klippel-Trenaunay syndrome: clinical, radiological and haemodynamic features and management. Br J Surg. 1985;72: 232-6.

Baskerville PA, Ackroyd JS, Browse NL. The etiology of the Klippel-Trenaunay syndrome. Ann Surg. 1985;202:624-7.

Browse NL, Burnand KG, Thomas ML. The Klippel-Trenaunay syndrome.In: Browse NL, Burnand KG, Thomas ML, editors. Diseases of the veins: pathology, diagnosis and treatment. London:Edward Arnold; 1988. p. 609-25.

Samuel M, Spitz L. Klippel-Trenaunay syndrome: clinical features, complications and management in children. Br J Surg. 1995;82:757-61.

Gloviczki P, Stanson AW, Stickler GB, Johnson CM, Toomey BJ, Meland NB et al. Klippel Trenaunay syndrome: the risks and benefits of vascular interventions. Surgery. 1991;110:469-79.

Gorenstein A, Katz S, Schiller M. Congenital angiodysplasia of the superficial venous system of the lower extremities in children. Ann Surg. 1988;207:312-8.

Mattassi R, Vaghi M. Management of the marginal vein: current issues. Phlebology. 2007;22:283-6.

Harrison CC, Holdstock JM, Price BA, Whiteley MS. Endovenous radiofrequency ablation and combined foam sclerotherapy treatment of multiple refluxing perforator veins in a Klippel-Trenaunay syndrome patient. Phlebology. 2014 Dec;29(10):698-700.

McGrory BJ, Amadio PC. Klippel-Trenaunay syndrome: orthopaedic considerations. Orthop Rev. 1993;22:41-50.