Klippel-Trenaunay综合征的序贯综合治疗和文献回顾

目的 观察不同治疗方法对Klippel-Trenaunay综合征治疗的疗效.方法 回顾分析了1990年至2016年我院收治的48例Klippel-Trenaunay综合征患儿,男26例,女22例,年龄2个月～6岁.所有病变均位于下肢,均为单侧下肢,左侧19例,右侧29例.其中12例患儿进行了手术切除部分畸形静脉和增大的软组织;16例患儿进行了静脉血管内射频消融加注射聚桂醇治疗(每个月治疗1次,重复治疗3～6次);20例患儿选择综合序贯治疗,早期穿着弹力裤,随诊6个月后,予以静脉血管内注射聚桂醇加射频消融,每个月1次,重复治疗3～6次,随诊6个月,如疾病仍有发展,就采用手术切除部分畸形静脉和增大的软组织1次或血管栓塞1次,在整个治疗周期内,持续穿着弹力裤.结果 48例患儿术后随访6年,12例选择手术治疗的患儿,对整个下肢的改善不明显.16例只选择单纯的静脉血管内注射聚桂醇治疗的患儿,患肢周径未及明显变化,无继续增大者.20例患儿选择序贯治疗.20例患儿患肢周径较治疗前缩小平均3.8cm,无继续增大,肌力正常,活动较正常肢体未及明显异常.结论 Klippel-Trenaunay综合征治疗不能采用单一的治疗方法,早期保守的物理治疗是防止疾病发展的关键,控制好了以后的治疗效果会加倍.而综合序贯治疗是一种安全可靠的治疗方法.效果较为理想,对Klippel-Trenaunay综合征治疗急需规范.

Treatment of Klippel-Trenaunay syndrome with comprehensive and sequential regimens and a literature review

Objective To explore the effieacies of different treatment for Klippel-Trenaunay syndrome.Methods From 1990 to 2016,a total of 48 cases of Klippel-Trenaunay syndrome were recruited.The therapeutic regimens included excision of partial soft tissue (n =12),3-6 monthly injections of lauromacrogol and comprehensive and sequential treatments (n =20).After wearing elastic pants for 6 months,lauromacrogol was injected and radiofrequency ablation once monthly for 3-6 times.During a follow-up period of 6 months,if disease worsened,excision of partial vascular malformation and expanding soft tissue or vascular embolization therapy was applied.Within the entire treatment cycle,all children wore elastic pants.Results During a follow-up period of 6 years,operative children showed no obvious improvements in lower extremities.Injection resulted in no marked change of extremity perimeter.And there was no further enlargement.For sequential treatment,there was a mean shrinkage of 3.8 cm in extremity perimeter.No further expansion was seen.Muscular tone and activity were normal.Conclusions Klippel-Trenaunay syndrome should be standardized and treated comprehensively.Early conservative physical treatment is essential for preventing deterioration.And sequential treatment is both safe and efficacious.