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Sung HM, Chung HY, Lee SJ, et al. Clinical Experience of the Klippel-Trenaunay Syndrome. Arch Plast Surg. 2015;42(5):552-8doi: 10.5999/aps.2015.42.5.552.
Sung, H. M., Chung, H. Y., Lee, S. J., Lee, J. M., Huh, S., Lee, J. W., Choi, K. Y., Yang, J. D., & Cho, B. C. (2015). Clinical Experience of the Klippel-Trenaunay Syndrome. Archives of plastic surgery, 42(5), 552-8. https://doi.org/10.5999/aps.2015.42.5.552
Sung, Hyung Min, et al. "Clinical Experience of the Klippel-Trenaunay Syndrome." Archives of plastic surgery vol. 42,5 (2015): 552-8. doi: https://doi.org/10.5999/aps.2015.42.5.552
Sung HM, Chung HY, Lee SJ, Lee JM, Huh S, Lee JW, Choi KY, Yang JD, Cho BC. Clinical Experience of the Klippel-Trenaunay Syndrome. Arch Plast Surg. 2015 Sep;42(5):552-8. doi: 10.5999/aps.2015.42.5.552. Epub 2015 Sep 15. PMID: 26430625; PMCID: PMC4579165.
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Arch Plast Surg. 2015 Sep;42(5):552-8. doi: 10.5999/aps.2015.42.5.552. Epub 2015 Sep 15.

Clinical Experience of the Klippel-Trenaunay Syndrome.

Archives of plastic surgery

Hyung Min Sung, Ho Yun Chung, Seok Jong Lee, Jong Min Lee, Seung Huh, Jeong Woo Lee, Kang Young Choi, Jung Dug Yang, Byung Chae Cho

Affiliations

  1. Department of Plastic and Reconstructive Surgery, Kyungpook National University School of Medicine, Daegu, Korea.
  2. Department of Plastic and Reconstructive Surgery, Kyungpook National University School of Medicine, Daegu, Korea. ; Kyungpook National University Hospital Vascular Anomalies Center, Daegu, Korea.
  3. Kyungpook National University Hospital Vascular Anomalies Center, Daegu, Korea. ; Department of Dermatology, Kyungpook National University School of Medicine, Daegu, Korea.
  4. Kyungpook National University Hospital Vascular Anomalies Center, Daegu, Korea. ; Department of Radiology, Kyungpook National University School of Medicine, Daegu, Korea.
  5. Kyungpook National University Hospital Vascular Anomalies Center, Daegu, Korea. ; Department of Surgery, Kyungpook National University School of Medicine, Daegu, Korea.

PMID: 26430625 PMCID: PMC4579165 DOI: 10.5999/aps.2015.42.5.552

Abstract

BACKGROUND: The Klippel-Trenaunay syndrome (KTS) is characterized by three clinical features, namely cutaneous capillary malformations, venous malformations, and soft tissue and/or bony hypertrophy of the extremities. The varied manifestations are attributed to the unpredictable clinical nature and prognosis of the syndrome. To elucidate the clinical characteristics of this disease, we reviewed a relatively large number of KTS patients who presented to our vascular anomalies center.

METHODS: We conducted a retrospective study with 19 patients who were diagnosed with KTS and treated in our vascular anomalies clinic between 2003 and 2014, and examined their demographic characteristics, their clinical features, and the treatments administered.

RESULTS: The sex distribution was balanced, with 9 (47%) males and 10 (53%) females. The mean follow-up period was 4.1 years (range, 7 months-9 years). Most of the patients received conservative treatments such as medication or physiotherapy. Compression therapies such as wearing of elastic garments/bandages were also administered, and surgical interventions were considered only when the patients became excessively symptomatic. Other treatments included laser therapy and sclerotherapy, and all the treatments were adjusted according to each case, tailored to the conditions of the individual patients.

CONCLUSIONS: KTS is an extremely rare, multifactorial disorder that induces widely varied symptoms. Because of this unique feature, plastic surgeons, when not careful, tend to attach a one-sided importance to typical symptoms such as limb hypertrophy or capillary malformation and thus overlook other symptoms and clinical features. KTS can be suspected in all infants who show capillary malformations or limb hypertrophy and require a multi-disciplinary approach for comprehensive management.

Keywords: Capillary malformations; Klippel-Trenaunay-Weber syndrome; Vascular malformations

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