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Radiological Aspect of Klippel-Trénaunay Syndrome: A Case Series With Review of Literature

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Abstract

Klippel-Trénaunay syndrome (KTS) is a rare angio-osteo-hypertrophic syndrome characterized by vascular malformations, soft tissue and/or bone hypertrophy, and varicose veins. For the purpose of describing the imaging findings and elucidating the role of medical imaging in the diagnosis and assessment of patient with KTS, we have reviewed the imaging data of 14 KTS patients. The imaging features on different imaging modalities were analyzed. Unilateral lower limb involvement was evident in 71% of cases (n=10) and bilateral but asymmetric lower limb involvement in the remaining 29% of cases (n=4). The most commonly depicted imaging features were varicosities in 93% (n=13), muscle hypertrophy in 79% (n=11) and venous anomalies in 64% (n=9). Other less common imaging findings included lymphedema in 29% (n=4), arterial malformations 29% (n=4), soft tissue hemangiomas 21% (n=3), pelvic and thigh phleboliths 21% (n=3), venous aneurysms 21% (n=3), bone abnormalities 14% (n=2) and lymphadenopathy 14% (n=2). A severe unilateral lower limb deformity resulting in contractures and muscle atrophy of the whole limb was depicted in 1 case. The pathognomonic marginal vein of Servelle was identified in 2 cases. AV shunt was highly suspected in 4 cases and was confirmed by DSA in 1 case, making Klippel-Trénaunay-Weber syndrome a more apt diagnosis. Associated ipsilateral duplicated renal artery was found in 1 case. We have concluded that medical imaging is the cornerstone in the diagnosis and assessment of severity and complications, follow-up and differentiation of KTS from other similar conditions. Different imaging modalities play complementary roles in the evaluation of KTS patients.

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Authors and Affiliations

  1. Department of Radiology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China

    Osamah Alwalid, Joyman Makamure, Qi-guang Cheng, Wen-jun Wu & Ping Han

  2. Department of Vascular Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China

    Chao Yang

  3. Department of Dermatology and Venereology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China

    Elham Samran

  4. Department of Interventional Radiology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China

    Hui-min Liang

Authors
  1. Osamah Alwalid
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  2. Joyman Makamure
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  3. Qi-guang Cheng
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  4. Wen-jun Wu
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  5. Chao Yang
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  6. Elham Samran
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  7. Ping Han
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  8. Hui-min Liang
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Correspondence to Ping Han or Hui-min Liang.

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Alwalid, O., Makamure, J., Cheng, Qg. et al. Radiological Aspect of Klippel-Trénaunay Syndrome: A Case Series With Review of Literature. CURR MED SCI 38, 925–931 (2018). https://doi.org/10.1007/s11596-018-1964-4

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  • DOI: https://doi.org/10.1007/s11596-018-1964-4

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