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A Journal on Surgery

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Chirurgia 2010 August;23(4):103-6


language: English

The clinical manifestations and outcomes in patients with Klippel-Trenaunay Syndrome. A single center study

Jung S-M. 1, Lee K-B. 1, Yun W-S. 1, Park U-J. 1, Roh Y-N. 1, Kim Y-W. 1, Do Y-S. 2, Park K-B. 2, Park H-S. 2, Dong-Ik K. 1

1 Division of Vascular Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea 2 Department of Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea


Aim. Klippel-Trenaunay syndrome (KTS) is a rare congenital malformation that is characterized by a triad of varicose veins, cutaneous capillary malformations and hypertrophy of bone and soft tissue. However, appropriate treatment methods for KTS are still unknown. We report the clinical manifestations and outcomes of the patients who received treatment for KTS.
Methods. We performed a retrospective review of 1929 patients who visited the Congenital Vascular Malformation Clinic in Samsung Medical Center between January 1995 and December 2006. Among these patients, 87 patients (47 males and 40 females) matched the diagnostic criteria for KTS and they were analyzed for their clinical manifestations and outcomes.
Results. The mean duration of follow up was 35.4 months and the most common site was the lower extremity. Sclerotherapy and surgical interventions were done in 20 and 34 patients, respectively and both in 14 patients. Forty-four patients received conservative treatment such as an elastic stocking and bandage. We reviewed 26 patients in more detail, and we divided into 3 groups (improved, no change, and aggravated). Clinical improvement was observed in some these 26 patients who received active treatments.
Conclusion. Active treatment with multidisciplinary treatment planning, when indicated, can improve the symptoms in patients with KTS.

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