Samimi Mahtab, Lorette Gérard
Université François-Rabelais de Tours, BP 3223, 37032 Tours cedex 1, France.
Presse Med. 2010 Apr;39(4):487-94. doi: 10.1016/j.lpm.2009.10.016. Epub 2010 Feb 26.
Klippel-Trenaunay syndrome (KTS) is a rare complex vascular congenital malformation. The characteristic triad is an association of a cutaneous capillary angioma of a limb, venous malformations, and hypertrophy of soft tissue and/or bone. Diagnosis is essentially clinical. Work-up of the lesion may involve noninvasive imaging: Doppler ultrasound, standard radiography, or magnetic resonance imaging (MRI). The presence of arteriovenous malformations is sought by clinical examination or ultrasound: they rule out a diagnosis of KTS. Management is multidisciplinary and involves especially venous control and orthopedic management of unequal limb lengths.
克-特综合征(KTS)是一种罕见的复杂血管先天性畸形。其特征三联征为肢体皮肤毛细血管瘤、静脉畸形以及软组织和/或骨骼肥大。诊断主要依靠临床。对病变的检查可能包括无创成像:多普勒超声、标准X线摄影或磁共振成像(MRI)。通过临床检查或超声查找动静脉畸形:存在动静脉畸形可排除KTS诊断。治疗是多学科的,尤其涉及静脉控制和对肢体不等长的骨科处理。