Klippel Trenaunay Syndrome associated with popliteal vein aplasia: Doppler Ultrasonography, CT Angiography and MRI Angiography Findings of a Rare Case

Öz

Klippel Trenaunay Syndrome is a rare congenital anomaly characterized by capillary malformations,  soft tissue or bony hypertrophy and superficial varicose veins. 29-year-old male presented with pain and swelling at lower limb. Physical examination revealed multipl varicose veins at right lower limb, gigantisim of right lower limb and cutaneus hiperpigmentation. He was diagnosed with Klippel Trenaunay Syndrome and Doppler ultrasonography, CT angiography and MRI angiography was made to evaluate deep venous system. Symptomatic treatment is preferred because of popliteal venous aplasia. CT angiography and MRI angiography are required to evaluate  deep venous system in those who is diagnosed with Klippel Trenaunay Syndrome. Aplastic deep vein is absolute contraindication of for surgical therapy.

Anahtar Kelimeler

• Klippel Trenaunay Syndrome; varicose veins

Klippel Trenaunay Sendromu ile birlikte görülen popliteal ven aplazisi: Nadir Bir Olgunun Doppler US, BT Anjiografi ve MR Anjiografi Bulguları

Öz

Klippel Trenaunay Sendromu kapiller malformasyonlar, yumuşak doku veya kemik hipertrofisi ve superfisiyel variköz venler ile karakterize nadir görülen konjenital anomalidir. 29 yaşında erkek hasta alt ekstremitede ağrı, şişlik şikayeti ile başvurdu. Fizik muayenede sağ alt ekstremitede yaygın variköz venler, ekstremitede uzunluk ve kalınlık artışı, ciltte hiperpigmente alanlar saptandı. Klippel Trenaunay Sendromu tanısı konuldu ve derin venöz sistemi değerlendirmek üzere Doppler US, BT anjiografi, MR anjiografi tetkikleri yapıldı. Popliteal veni aplazik olan hastada semptomatik tedavi yöntemleri tercih edildi. Klippel Trenaunay Sendromu tanılı hastalarda BT ya da MR anjiografi derin venöz sistemi değerlendirmede önemlidir. Derin venöz sistemde aplazi varlığı cerrahi tedavi için kesin kontraendikasyondur.

Anahtar Kelimeler

• Klippel Trenaunay Sendromu; varikoz venler

Kaynakça

1. - Naik RS. Klippel-trenaunay syndrome. Indian J Surg. 2007;69(4):158-9.
2. - Zea MI, Hanif M, Habib M, Ansari A. Klippel-Trenaunay Syndrome: a case report with brief review of literature. J Dermatol Case Rep. 2009;3(4):56-9.
3. - Nitecki S, Bass A. Ultrasound-guided foam sclerotherapy in patients with Klippel-Trenaunay syndrome. Isr Med Assoc J. 2007;9(2):72-5.
4. - Abdul-Rahman NR, Mohammad KF, Ibrahim S. Gigantism of the lower limb in Klippel-Trenaunay syndrome: anatomy of the lateral marginal vein. Singapore Med J. 2009;50(6):223-5.
5. - 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(3):744-9.
6. - Samo S, Sherid M, Husein H, Sulaiman S, Yungbluth M, Vainder JA. Klippel-Trenaunay Syndrome Causing Life-Threatening GI Bleeding: A Case Report and Review of the Literature. Case Rep Gastrointest Med. 2013;2013:813653.
7. - Usta S, Günday M. [Klippel Trenaunay Syndrome ]. Türk Göğüs Kalp Damar Cerrahisi Dergisi 2013;21(1):179-182
8. - Baskerville PA, Ackroyd JS, Browse NL. The etiology of the Klippel-Trenaunay syndrome. Ann Surg. 1985;202(5):624–7

9. - Namba K, Nemoto S. Parkes weber syndrome and spinal arteriovenous malformations.
10. AJNR Am J Neuroradiol. 2013;34(9):110-2.
11. - Browse NL, Burnand KG, Thomas ML. The Klippel- Tre´naunay syndrome. In: Browse NL, Burnand KG, Thomas ML, editors. Diseases of the veins: pathology, diagnosis and treatment. London: Edward Arnold; 1988. P. 609-25.
12. - Redondo P, Bastarrika G, Aguado L, Martínez-Cuesta A, Sierra A, Cabrera J, et al. Foot or hand malformations related to deep venous system anomalies of the lower limb in Klippel-Trénaunay syndrome. J Am Acad Dermatol 2009;61 (4):621-8.
13. - Kim YW, Lee BB, Cho JH, Do YS, Kim DI, Kim ES. Haemodynamic and clinical assessment of lateral marginal vein excision in patients with a predominantly venous malformation of the lower extremity. Eur J Vasc Endovasc Surg. 2007;33 (1):122–127.
14. - Noel AA, Gloviczki P, Cherry KJ Jr, Rooke TW, Stanson AW, Driscoll DJ. Surgical treatment of venous malformations in Klippel-trénaunay syndrome. J Vasc Surg 2000;32 (5):840-7.
15. - Eifert S, Villavicencio JL, Kao TC, Taute BM, Rich NM. Prevalence of deep venous anomalies in congenital vascular malformations of venous predominance. J Vasc Surg 2000;31(3):462-71.