Ning-Fei Liu, MD, PhD, Qing Lu, MD, Zhi-Xin Yan, MD 

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Presentation transcript:

Lymphatic malformation is a common component of Klippel-Trenaunay syndrome  Ning-Fei Liu, MD, PhD, Qing Lu, MD, Zhi-Xin Yan, MD  Journal of Vascular Surgery  Volume 52, Issue 6, Pages 1557-1563 (December 2010) DOI: 10.1016/j.jvs.2010.06.166 Copyright © 2010 Society for Vascular Surgery Terms and Conditions

Fig 1 (a) A 12-year-old boy with enlarged left lower extremity and skin port wine stain. (b) Coronal T2-weighted MR image of left leg shows numerous dilated intramuscular blood vasculature (arrows). (c) MR lymphangiogram shows a few enhanced lymphatic vessels (large arrow) in the distal part and a varicose vein (arrow head) in the lateral part of the left lower leg. Note enhanced lymphatic vessels in the contralateral asymptomatic limb (small arrows). (d) Axial section of T2-weighted MR image shows edema fluid (high signal intensity) dispersing within significantly hypertrophic subcutis of the affected lower limb. Journal of Vascular Surgery 2010 52, 1557-1563DOI: (10.1016/j.jvs.2010.06.166) Copyright © 2010 Society for Vascular Surgery Terms and Conditions

Fig 2 (a) A 27-year-old woman with extensive skin port-wine staining and enlarged left lower extremity. (b) No lymph node was identifiable in the inguinal region of the left side in contrast with clearly visualized lymph nodes (arrow) on the right side on coronal T2-weighted MR images. (c) Fine lymphatic vessels (arrows) found in the distal region of lower leg on MR lymphangiogram. (d) Axial section of T2-weighted MR image shows hypertrophy of subcutaneous tissue and edema fluid accumulated in the subcutis of the left lower extremity. Journal of Vascular Surgery 2010 52, 1557-1563DOI: (10.1016/j.jvs.2010.06.166) Copyright © 2010 Society for Vascular Surgery Terms and Conditions

Fig 3 (a) A 34-year-old man with chief clinical features of KTS, including skin port wine stain and hypertrophy of the left lower limb. (b) Axial section of T2-weighted MR image shows hypertrophy of subcutaneous tissue and edema (high-signal intensity) in the whole subcutaneous layer. (c) No inguinal lymph nodes were visualized on the left side in contrast with lymph nodes (arrowheads) visualized on the contralateral side on coronal T2-weighted MR image. (d) Composition image of MR lymphangiogram shows numerous hyperplastic lymphatic vessels (arrowheads) and enlarged deep vein (arrow). Journal of Vascular Surgery 2010 52, 1557-1563DOI: (10.1016/j.jvs.2010.06.166) Copyright © 2010 Society for Vascular Surgery Terms and Conditions

Fig 4 (a) A 26-year-old woman with enlarged and shortened left lower extremity. (b) Composition image of MR lymphangiogram shows numerous significantly enlarged lymphatic channels with cystic dilatation (arrows). (c) MR lymphangiogram shows enlargement of inguinal lymph nodes on the left side with partial (in cortical region) contrast enhancement (arrow). (d) Coronal T2-weighted MR image shows macrocystic lymphatic malformations (arrow) in the popliteal fossa of the left lower extremity. (e) Axial section of T2-weighted MR image shows thickness and edema of the subcutis (high signal intensity) of the left lower limb. Note intramuscular accumulation of edema fluid (arrow). Journal of Vascular Surgery 2010 52, 1557-1563DOI: (10.1016/j.jvs.2010.06.166) Copyright © 2010 Society for Vascular Surgery Terms and Conditions

Fig 5 (a) A 15-year-old girl with enlarged and longer right lower extremity. (b) MR lymphangiogram shows the poorly contrast-enhanced inguinal lymph nodes (arrowhead) with increased size and number on the right side. (c) Composition image of MR lymphangiogram shows hyperplastic lymphatic vessels (large arrows) and enlarged superficial veins (small arrows) of the right lower extremity. (d) Coronal T2-weighted MR image shows increased intramuscular blood vessels (arrows) of right lower limb. (e) Axial section of T2-weighted MR image shows hypertrophy and edema of subcutaneous tissue. Note more edema fluid accumulated superficially encircling the deep fascia of the right lower extremity. Journal of Vascular Surgery 2010 52, 1557-1563DOI: (10.1016/j.jvs.2010.06.166) Copyright © 2010 Society for Vascular Surgery Terms and Conditions