Klippel-Trenaunay综合征临床实践指南

Clinical practice guidelines for Klippel-Trenaunay syndrome(translation version)

Klippel-Trenaunay综合征(Klippel-Trenaunay syndrome,KTS)是一种罕见的先天性脉管疾病,包括3大特征:葡萄酒色斑、软组织和骨的异常过度生长、静脉/淋巴管畸形。此病病因及发病机制目前不明。KTS患者的症状和严重程度因人而异,治疗的目的在于减轻症状,减少并发症发生的风险。本文回顾KTS的临床表现及诊断研究的结果,并提供治疗建议,以帮助更好地处理患者。

Klippel-Trenaunay syndrome（KTS） is a rare congenital vascular disorder with three clinical features： portwine stain, abnormal overgrowth of soft tissues and bones, and venous/lymphatic malformations. The etiology and pathogenesis remain unknown. Symptoms and severity of KTS vary greatly among each individual. The goals of treatment are to alleviate symptoms and minimize the risk of complications. This article reviewed the clinical presentation and diagnostic studies, and provided management guidelines to help care for patients with this syndrome.