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.
作者
季易
崔杰
陈建兵
邹继军
陈海妮
韩涛
沈卫民
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)
出处
《中华小儿外科杂志》
CSCD
2017年第1期28-31,共4页
Chinese Journal of Pediatric Surgery
