Lymphatic malformation is a common component of Klippel-Trenaunay syndrome.
OBJECTIVE
Few previous studies have focused on the involvement of the lymphatic system in Klippel-Trenaunay syndrome (KTS), although some evidence suggests that lymphatic abnormalities are associated with the disease. The aim of the present study was to investigate the involvement of the lymphatic system in KTS.


METHODS
Magnetic resonance lymphangiography (MRL) with the use of gadobenate dimeglumine as the contrast was performed on 32 patients with KTS involving the extremities to evaluate lymphatic vessels, lymph nodes, and veins.


RESULTS
Thirty-one of 32 patients exhibited lymphatic vessel and/or lymph node anomalies, including hyperplasia (11/31), hypoplasia or aplasia (20/31) of lymphatic vessels, and lymphedema (31/31) of the affected limbs. Twenty-two patients showed asymmetry of the inguinal nodes exhibiting either the absence, or an increase or a decrease in number and size of the inguinal nodes. Venous dysplasia was found in 31 patients in superficial and/or deep veins. The results showed a high concomitance of malformations of the lymphatic system and veins in the affected limbs of patients with KTS.


CONCLUSIONS
Lymphatic system abnormalities as examined with MRL are commonly associated with KTS and are likely to play a significant role in the disorder.
Liu
Ning-Fei
NF
Department of Plastic and Reconstructive Surgery, Shanghai 9th People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China. liuningfei@126.com
Lu
Qing
Q
Yan
Zhi-Xin
ZX
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003287:Contrast Media; D005260:Female; D005682:Gadolinium; D006801:Humans; D007715:Klippel-Trenaunay-Weber Syndrome / Q000150:complications* / Q000473:pathology; D044148:Lymphatic Abnormalities / Q000150:complications / Q000175:diagnosis*; D008208:Lymphatic System / Q000473:pathology; D008209:Lymphedema / Q000150:complications; D008279:Magnetic Resonance Imaging; D008297:Male; D008536:Meglumine / Q000031:analogs & derivatives; D009942:Organometallic Compounds; D055815:Young Adult
D003287:Contrast Media; D009942:Organometallic Compounds; C064572:gadobenic acid; D008536:Meglumine; D005682:Gadolinium