血管内栓塞与激素联合治疗婴幼儿卡梅现象的疗效研究

The therapic effect of the combination of endovascular embolization and hormone in Kasabach- Merritt phenomenon

目的探讨血管内栓塞与临床激素联合治疗婴幼儿卡梅现象的疗效，并分析其优势。方法回顾性分析2010年5月至2012年6月6例卡梅现象患儿，入院后临床先予大剂量激素冲击治疗，7～10d后在全身麻醉下，采用Seldinger技术进行选择性血管内栓塞治疗，术后临床继续行激素治疗2周，观察血小板计数并进行疗效评价。肿瘤面积缩小未达50％者，对残余部分给予局部硬化剂治疗3—4个疗程。局部治疗在透视监视下完成，采用多点穿刺，回抽见血后缓慢注入四联药物，注射局部张力增高停止注射。结果6例患儿造影表现为肿瘤血管范围分布广泛、不规则，经1～2周综合治疗后其中4例肿瘤全部消退，随访0．5—1．0年无复发；2例肿瘤缩小未达50％，对残余部分予局部硬化剂治疗3—4个疗程后肿瘤完全消退。血小板持续稳定在正常水平。患儿精神状况改善，出血倾向缓解，皮下瘀血、出血点消退，局部软组织肿胀缓解。无严重不良反应及并发症。结论血管内栓塞与临床激素联合应用可以对卡梅现象起到良好的疗效。

Objective To explore the therapic effect of the combination of endovascular embolization and clinical hormone in treatment of Kasabach-Merritt phenomenon （ KMP）, and analyze the advantages. Methods Six cases with KMP from May 2010 to June 2012 were retrospectively analyzed. All the patients underwent large dose hormone shock therapy after admission for 7--10 d. Subsequently,selective endovascular emholization was performed using Seldinger technique under general anesthesia. Then, the hormone therapy was continued for 2 weeks after embolization. The platelet count and the effect were recorded. If the area of the tumor reduction is less than 50% ,3---4 courses of local hardening treatment was conducted for the residual treMor. The local treatment used muhipoint puncturing of the tumor and injection drugs under X-ray fluoroscopic monitoring until the tension of local vascular increased. Results On DSA, the lesions of all the 6 cases showed rich blood supply with a large number of hybrid distribution of tumor blood vessels. The lesions disappeared in 4 cases after 1--2 weeks combination therapy and no recurrence for 0. 5--1.0 year follow-up. Two cases whose tumor reduced less than 50% after combination therapy received local hardening treatment, and faded after 3---4 courses. The number of platelet for all patients kept normal and the spirit of the patients showed great improvement, the bleeding tendency and local soft tissue swelling was in remission. Subcutaneous blood stasis and petechiae disappeared. There were no serious adverse reaction and complications. Conclusion Combination therapy with endovascular embolization and clinical hormone for Kasabach-Merritt phenomenon has a good curative effect.