Kasabach-Merritt综合征血管造影特征分析及介入栓塞价值探讨

The angiographic characteristics and transcatheter arterial embolization therapy of Kasabach-Merritt syndrome

目的 分析Kasabach-Merritt综合征（KMS）血管造影特征并探讨其介入栓塞价值.方法 回顾性分析2010年6月至2016年6月间在我科被诊断为KMS患儿的临床资料,其中36例接受过介入栓塞治疗,介入术后4周评估其治疗效果,若仍未达治愈标准则可再次行介入栓塞治疗.总结KMS患儿接受介入栓塞治疗次数及每次介入术后血小板变化情况.对比分析行介入治疗的KMS患儿与52例普通婴幼儿血管瘤血管造影结果.结果 KMS患儿血管造影通常表现为肿瘤样染色、形态不规整、染色不均、边界不清、染色灶包绕正常动脉主干,供血动脉自正常动脉主干发出,数量众多且非常纤细,其直径与肿瘤大小不成比例;普通婴幼儿血管瘤造影也表现为肿瘤样染色,但通常为类圆形或椭圆形、边界清晰、染色均匀、分布于正常动脉主干的一侧,供血动脉自正常动脉主干发出,通常为1~4支供血动脉,其直径与肿瘤大小呈比例.KMS患儿接受经导管动脉栓塞术2.0~4.0次/例,共107.0次,平均（2.9±0.7）次/例.KMS患儿11.0次介入术后血小板仍持续下降,96.0次介入术后血小板稳步上升;治疗后4.0~13.0 d,平均（7.1±2.4）d血小板全部上升至≥100×109/L,此后血小板随病情反复波动.复发时间为术后12.0~69.0 d,平均（45.9±21.8）d.随访6个月至2年,36例患儿术后最终联合糖皮质激素、长春新碱或西罗莫司等其他综合治疗,8例治愈,28例好转.结论 KMS患儿血管造影特征不同于普通婴幼儿血管瘤,供血动脉众多、纤细,超选择动脉栓塞难度大,介入栓塞治疗起效迅速,但效果难以长效维持.建议联合其他药物进行综合治疗.

Objective To analyze the angiographic characteristics of Kasabach-Merritt syndrome （KMS） and study the value of transcatheter arterial embolization （TAE） in KMS therapy. Methods The clinical data of 36 infants with KMS treated between June 2010 and June 2016 in our hospital were reviewed retrospectively. All infants performed angiography and TAE therapy. These angiographic characteristics were analyzed by comparing with infantile hemangioma（IH）.The times of TAE and the level of platelet after TAE therapy were observed. Results The angiographic characteristics of KMS were as follows： The capillary blush of KMS were ill defined with no-uniform distribution. There were a lot of very fine feeding arteries. The diameter of feeding artery was disproportionate to the volume of tumor blush because the feeding arteries were too small or fine. Normal artery was usually embedded in tumor blush. However, the angiographic characteristics of IH were different as follows：the capillary blush of IH were well defined with uniform distribution.There were about 1-4 feeding arteries.The diameter of feeding artery was proportional to the volume of tumor blush. Normal artery was not embedded in tumor blush. Tumor blush was usually located beside the normal artery. In 36 patients, 107 embolization treatments were performed. The platelet declined for 11 times and increased for 96 times after TAE therapy. The platelet count for these 96 cases increased to ≥100×109/L at（7.1 ± 2.4）days following therapy. However, the platelet level fluctuated thereafter and the average relapse time was（45.9 ± 21.8）days. All cases were followed-up ranging from 6 months to 2 years and finally received other therapies.Eight cases were cured and the other 28 cases were improved. Conclusions The angiographic characteristics of KMS are different from common infantile hemangioma. It is difficult to embolize the feeding arteries of KMS because there are a lot of very fine feeding arteries.TAE may rapidly improve the level of platelets while the long term effect was poor.It might be better to combine TAE with other medical therapies to treat KMS.