药物联合手术治疗Kasabach-Merritt综合征  

Management of Kasabaeh-Merritt syndrome by drug therapy and surgery

作　　者：雷红召[1] 黄静[1] 孟小分[1] 马玉春[1] 孙斌[1] 乔军波[1] 董长宪[1] 

机构地区：[1]河南省人民医院血管瘤外科,河南省血管瘤(畸形)诊疗中心,郑州450003

出　　处：《中华整形外科杂志》2013年第2期104-108,共5页Chinese Journal of Plastic Surgery

基　　金：河南省科技攻关项目（112102310235）

摘　　要：目的总结和探讨Kasabach-Merritt综合征（KMS）的临床特点和治疗方法，评价药物联合手术治疗的效果。方法回顾性分析2004至2010年接受药物联合手术治疗的59例Kasabach-Merritt综合征患儿的临床和随访资料，男33例，女26例，男女比例为1．269：1；年龄7d至2，5岁，平均2．9个月。根据病变部位的不同分别采用不同的手术治疗方法，其中颌面部（28例）行颈外动脉结扎置管尿素和激素灌注治疗，躯干四肢（31例）采用手术切除方法。随访6．5个月至7．3年，平均2．8年。统计分析血小板计数变化和瘤体消退情况，随访观察瘤体有无复发，患儿生长发育及肢体功能等。结果59例患儿，痊愈58例，死亡1例。其中因瘤体破溃出血急诊手术4例，择期手术55例。术后血小板计数、血红蛋白及凝血功能1～2周逐渐升至正常，术后1周患儿精神状况改善，食欲增强，体重增加，哭闹不安情绪缓解，出血情况消退。颈外动脉结扎置管联合药物灌注治疗后颌面部瘤体逐渐缩小消失，瘤体6～12个月消失18例，13～24个月10例。长期随访观察瘤体无复发，体重身高、免疫力等同正常儿童发育，功能活动良好。结论药物联合手术治疗KMS明显缩短治疗时间，降低药物不良反应，有良好治疗效果，降低患儿死亡率。Objective To summarize the characters of Kasabach-Merritt syndrome （KMS） and to evaluate the therapeutic effect of drug therapy combined with surgery. Methods From 2004 to 2010, 59 cases with KMS, who underwent drug therapy and surgery, were retrospectively studied. The average age of the patients, including 33 male and 26 female （male/female, 1. 269/1 ） , was 2.9 months （range, 7 days- 2.5 years）. 28 cases with maxillofaeial lesions were treated with the ligation of external carotid artery and injection of carbonyldiamide and methylprednisolone. 31 cases with lesions at trunks and extremities were treated by excision of lesions. All the patients were followed up for 2.8 years （ range, 6.5 months -7.3 years）. Therapeutic outcomes were assessed by evaluating platelet counts, size of lesion, function of trunk and limb. Results 58 cases were cured except for one dead case. Emergency operation was given in 4 cases, and selective operation was performed in other cases （55 cases）. The thrombocyte count,hemoglobin and blood coagulation function returned to normal within 1-2 weeks. The mental condition, appetite, body weight, sleeping were greatly improved one week after treatment. The size of the lesions decreased gradually after the management of ligation of external carotid artery including 18 cases within 6-12 months and 10 cases within 13-24 months. Long term follow-up studies indicated that there was no recurrent case, and the weight, height, immunity of the patients with good function activities were in keeping with the normal counterparts, Conclusions The drug combined with surgery therapy is a very reliable management with high curative rate, short disease period and minimum side-effect.

关 键 词：KASABACH-MERRITT综合征 药用制剂 血小板减少 

分 类 号：R739.5[医药卫生—肿瘤]