Klippel—Trenaunay综合征的序贯综合治疗和文献回顾  

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

作　　者：季易[1] 崔杰[1] 陈建兵[1] 邹继军[1] 陈海妮[1] 韩涛[1] 沈卫民[1] Ji Yi Cui Jie Chen J ianbin Zou Jijun Chen Haini Han Tao Shen Weimin(Department of Plastic Surgery, Municipal Children＇s Hospital, Nanjing Medical University, Nanjing 210008, China)

机构地区：[1]南京医科大学附属儿童医院整形科,210008

出　　处：《中华小儿外科杂志》2017年第1期28-31,共4页Chinese Journal of Pediatric Surgery

摘　　要：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。Objective To explore the efficacies 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.

关 键 词：KLIPPEL-TRENAUNAY综合征 消融 聚桂醇 

分 类 号：R726.5[医药卫生—儿科]