Klippel-Trenaunay综合征的诊断和治疗

Klippel-Trenaunay syndrome: Diagnosis and management

Klippel-Trenaunay综合征(Klippel-Trenaunay syndrome,KTS)是少见的先天性周围血管疾患,具有典型的三联征:毛细血管畸形(葡萄酒色斑)、肢体过度生长、非典型性外侧浅静脉曲张。这种复杂的血管病变是一种混合性血管畸形,有突出的毛细血管、静脉和淋巴管,而没有显著的动-静脉分流。最常累及下肢。影像学检查需要确定静脉病变的范围及深静脉的回流情况。压迫治疗是保守治疗的主要方法。手术包括浅静脉的剥脱、激光治疗等,须根据病人病情指定多学科参与的治疗方案。

Klippel-Trenaunay syndrome （KTS） is a rare, complex malformation characterized by the elinical triad of capillary malformations （port wine stain）, soft tissue and bone hypertrophy or hypotrophy of usually one lower limb occasionally and atypical lateral varicosity mostly. KTS is a mixed vascular malformation with predominant capillary, venous and lymphatic components, without significant arteriovenousshunting.Imaging before vascular interventions must confirm venous anatomy and deep venous drainage. Management is largely conservative. Compression is the hallmark of conservative management, laser can be used totreat port wine stains.Techniques for ablation of superficial veins and malformations are individualized. A muhidisciplinary approach to management of KTS is warranted.