Journal of Cardiovascular Imaging

Korean Society of Echocardiography (한국심초음파학회)
권호 표지
DOI QR코드

DOI QR Code

A CASE OF KLIPPEL-TRENAUNAY SYNDROME WITH ACUTE SUBMASSIVE PULMONARY THROMBOEMBOLISM TREATED WITH THROMBOLYTIC THERAPY

  • Chu, Seong-Taek (Department of Internal Medicine, National Medical Center) ;
  • Han, Yung-Hee (Department of Internal Medicine, National Medical Center) ;
  • Koh, Jung-A (Department of Internal Medicine, National Medical Center) ;
  • Kim, Seon-Jae (Department of Internal Medicine, National Medical Center) ;
  • Lee, Hak-Cheol (Department of Internal Medicine, National Medical Center) ;
  • Kim, Si-Eun (Department of Internal Medicine, National Medical Center) ;
  • Shin, Yong-Chul (Cardiovascular Center, National Medical Center) ;
  • Sir, Jung Ju (Department of Internal Medicine, National Medical Center) ;
  • Choi, Seung Min (Department of Internal Medicine, National Medical Center) ;
  • Joo, Shin Bae (Department of Internal Medicine, National Medical Center)
  • Received : 2015.06.08
  • Accepted : 2015.11.18
  • Published : 2015.12.27
⟨ Previous Next ⟩

Abstract

Klippel-Trenaunay syndrome is a rare congenital mesodermal abnormality characterized by varicose veins, cutaneous hemangiomas, soft tissue and bony hypertrophy of limb. Potential complications such as deep venous thrombosis and pulmonary thromboembolism have not been reported in Korea to date. We demonstrate the case of a 48-year-old woman with Klippel-Trenaunay syndrome with extensive varicose veins on right lower limb, hypertrophy of left big toe and basilar artery tip aneurysm, complicated with acute submassive pulmonary thromboembolism treated successfully with intravenous thrombolytic therapy.

Keywords

References

  1. Huiras EE, Barnes CJ, Eichenfield LF, Pelech AN, Drolet BA. Pulmonary thromboembolism associated with Klippel-Trenaunay syndrome. Pediatrics 2005;116:e596-600. https://doi.org/10.1542/peds.2004-1607
  2. 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. https://doi.org/10.1016/S0025-6196(11)63615-X
  3. 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. https://doi.org/10.1002/bjs.1800720331
  4. Mazoyer E, Enjolras O, Laurian C, Houdart E, Drouet L. Coagulation abnormalities associated with extensive venous malformations of the limbs: differentiation from Kasabach-Merritt syndrome. Clin Lab Haematol 2002;24:243-51. https://doi.org/10.1046/j.1365-2257.2002.00447.x
  5. Oduber CE, van Beers EJ, Bresser P, van der Horst CM, Meijers JC, Gerdes VE. Venous thromboembolism and prothrombotic parameters in Klippel-Trenaunay syndrome. Neth J Med 2013;71:246-52.
  6. Muluk SC, Ginns LC, Semigran MJ, Kaufman JA, Gertler JP. Klippel-Trenaunay syndrome with multiple pulmonary emboli--an unusual cause of progressive pulmonary dysfunction. J Vasc Surg 1995;21:686-90. https://doi.org/10.1016/S0741-5214(95)70199-0
  7. Ndzengue A, Rafal RB, Balmir S, Rai DB, Jaffe EA. Klippel-Trenaunay syndrome: an often overlooked risk factor for venous thromboembolic disease. Int J Angiol 2012;21:233-6. https://doi.org/10.1055/s-0032-1328969
  8. Yamada T, Ohba T, Yamamoto T, Kimata N, Inami T, Munakata R, Murakami D, Maruyama M, Takano M, Ibuki C, Hata N, Seino Y, Mizuno K. A 17-year-old girl with Klippel-Weber syndrome complicated with a pulmonary thromboembolism and RV thrombus. Intern Med 2013;52:1337-40. https://doi.org/10.2169/internalmedicine.52.9149
  9. Star A, Fuller CE, Landas SK. Intracranial aneurysms in Klippel-Trenaunay/Weber syndromes: case report. Neurosurgery 2010;66:E1027-8; discussion E1028. https://doi.org/10.1227/01.NEU.0000368392.69904.BE
  10. Kim HJ, Kim SC. A case of pseudo-Kaposi’s sarcoma associated with Klippel-Trenaunay syndrome. Korean J Dermatol 2006;44:450-3.
  11. Lee YK, Kim JK, Cho KS. Bladder hemangioma associated with Klippel-Trenaunay syndrome: case report. J Korean Radiol Soc 2003;48:271-4. https://doi.org/10.3348/jkrs.2003.48.3.271
  12. Chung YW, Han DS, Paik CH, Park YK, Kim JP, Lee HL, Kim JB, Sohn JH, Hahm JS, Park HK. A case of Klippel-Trenaunay-Weber syndrome with sigmoid varices. Korean J Gastrointestinal Endosc 2003;27:21-5.
  13. Kim JY, Lee JB, Lee SC, Won YH. A case of Klippel-Trenaunay syndrome with lymphangioma circumscriptum. Korean J Dermatol 2000;38:1522-6.
  14. Jaff MR, McMurtry MS, Archer SL, Cushman M, Goldenberg N, Goldhaber SZ, Jenkins JS, Kline JA, Michaels AD, Thistlethwaite P, Vedantham S, White RJ, Zierler BK; American Heart Association Council on Cardiopulmonary, Critical Care, Perioperative and Resuscitation; American Heart Association Council on Peripheral Vascular Disease; American Heart Association Council on Arteriosclerosis, Thrombosis and Vascular Biology. Management of massive and submassive pulmonary embolism, iliofemoral deep vein thrombosis, and chronic thromboembolic pulmonary hypertension: a scientific statement from the American Heart Association. Circulation 2011;123:1788-830. https://doi.org/10.1161/CIR.0b013e318214914f
  15. Konstantinides SV, Torbicki A, Agnelli G, Danchin N, Fitzmaurice D, Galie N, Gibbs JS, Huisman MV, Humbert M, Kucher N, Lang I, Lankeit M, Lekakis J, Maack C, Mayer E, Meneveau N, Perrier A, Pruszczyk P, Rasmussen LH, Schindler TH, Svitil P, Vonk Noordegraaf A, Zamorano JL, Zompatori M; Task Force for the Diagnosis and Management of Acute Pulmonary Embolism of the European Society of Cardiology (ESC). 2014 ESC guidelines on the diagnosis and management of acute pulmonary embolism. Eur Heart J 2014;35:3033-69, 3069a-3069k. https://doi.org/10.1093/eurheartj/ehu283
  16. Meyer G, Vicaut E, Danays T, Agnelli G, Becattini C, Beyer-Westendorf J, Bluhmki E, Bouvaist H, Brenner B, Couturaud F, Dellas C, Empen K, Franca A, Galie N, Geibel A, Goldhaber SZ, Jimenez D, Kozak M, Kupatt C, Kucher N, Lang IM, Lankeit M, Meneveau N, Pacouret G, Palazzini M, Petris A, Pruszczyk P, Rugolotto M, Salvi A, Schellong S, Sebbane M, Sobkowicz B, Stefanovic BS, Thiele H, Torbicki A, Verschuren F, Konstantinides SV; PEITHO Investigators. Fibrinolysis for patients with intermediate-risk pulmonary embolism. N Engl J Med 2014;370:1402-11. https://doi.org/10.1056/NEJMoa1302097
  17. Sumner CJ, Golden JA, Hemphill JC 3rd. Should thrombolysis be contraindicated in patients with cerebral arteriovenous malformations? Crit Care Med 2002;30:2359-62. https://doi.org/10.1097/00003246-200210000-00028
  18. Edwards NJ, Kamel H, Josephson SA. The safety of intravenous thrombolysis for ischemic stroke in patients with pre-existing cerebral aneurysms: a case series and review of the literature. Stroke 2012;43:412-6. https://doi.org/10.1161/STROKEAHA.111.634147

Cited by

  1. Successful Treatment of Unilateral Klippel-Trenaunay Syndrome With Pulsed-Dye Laser in a 2-Week Old Infant vol.8, pp.2, 2015, https://doi.org/10.15171/jlms.2017.18
  2. Klippel-Trenaunay Syndrome Involving Upper Limb and Chest Wall: A Case Report and Review of the Literature vol.47, pp.2, 2015, https://doi.org/10.2478/amb-2020-0020