婴幼儿丛状血管瘤24例  

Clinical analysis of tufted angiomas in infants

作　　者：雷红召[1] 张巧荣[1] 马玉春[1] 孙斌[1] 张伟[1] 龚毓宾 董长宪[1] 

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

出　　处：《中华实用儿科临床杂志》2014年第20期1583-1586,共4页Chinese Journal of Applied Clinical Pediatrics

基　　金：河南省科技攻关项目（112102310235）;河南省医学科技攻关项目（201204074）

摘　　要：目的 探讨婴幼儿丛状血管瘤（TA）的临床特点、疾病进程,及并发Kasabach-Merritt现象（KMP）的治疗方法.方法 收集、分析2009年1月至2013年3月收治的24例婴幼儿TA患儿的临床资料和随访资料.男10例,女14例;就诊年龄18 d～2岁,中位年龄为7.5个月.根据病变情况选择临床观察及手术治疗等.随访1.2 ～5.4年,平均3.6年.随访观察患儿病情变化.结果 临床常见症状或体征包括暗红或紫红色边界不清的体表皮损、血小板减少、疼痛或功能受限以及多汗、多毛等.其临床进程主要有3种类型：病变完全或部分自发性消退2例（8.3％）、持续存在9例（37.5％）、合并KMP 13例（54.2％）.TA发病至并发KMP平均间隔时间45.2 d（0d～4个月）.KMP发病最初症状或体征表现为迅速增大的瘤体（8例）、瘤体明显变厚张力增大（3例）、呼吸道窘迫表现为呼吸急促、三凹征明显（2例）.13例合并KMP均采用手术治疗,手术完全切除者10例,术后血小板计数1～3d升至正常,血红蛋白及凝血功能1～2周逐渐恢复正常;大部切除者3例,血小板计数术后均出现反复,但明显高于术前,多维持在60×109/L以上,经术后给予药物治疗,其中2例3～6个月后瘤体逐渐消失,血小板计数恢复正常,1例术后因多器官功能衰竭死亡.结论 积极主动干预治疗主要适用于影响外观或容貌、已经或可能并发KMP或其他功能异常.对年龄较小无严重并发症的TA,早期可以随访观察,定期监测其血小板计数,以尽早发现KMP.对合并KMP的TA,明确诊断后尽早手术治疗,可明显缩短治疗时间,降低药物不良反应,治疗效果良好,降低患儿病死率.Objective To describe and examine the clinical characteristics and spectrum of tufted angiomas （TA） in infants so as to explore the therapeutic approaches to Kasabach-Merritt phenomenon （KMP）.Methods The clinical and follow-up data of 24 patients with TA were retrospectively analyzed between Jan.2009 and Mar.2013.The median age of the patients was 7.5 months（ranged from 18 days to 2 years）,including l0 male and 14 female.Surgical excision and observation were chosen according to the lesion and conditions of the patients.The mean follow-up period was 3.6 years （1.2 to 5.4 years）.The changes in the patients＇ s condition were established by evaluating platelet counts,and the size of lesion.Results Common clinical features included dusky red or violaceous infiltrating cutaneous lesion,thrombocytopenia,pain or decreased function and hyperhidrosis or hypertrichosis.The following 3 clinical patterns of TA progress were described：spontaneous complete or partial regression （n =2,8.3%）,TA without complications and persistence over the years（n =9,37.5%）,and TA complicated by KMP（n =13,54.2%）.The average interval of development of KMP for delayed cases was 45.2 days（ranged 0 d to 4 months）.Each of the 13 patients who developed KMP subsequent to initial presentation was symptomatic at the time KMP was detected（enlarged lesion,n =8;increased lesion firmness with change in cutaneous stain,n =3 ;and respiratory distress,n =2）.All of 13 patients were cured by surgery.Complete surgical resections were performed on 10 cases.The thrombocyte count was back to normal within 1-3 days post operation,and hemoglobin and blood coagulation function gradually returned to normal within 1 to 2 weeks.Other 3 cases received major resection surgery.The number of platelets in the patients were unstable,but significantly higher than that of preoperational stage.The platelet count remained above 60 × 109/L.The residue lesions in 2 cases disappeared gradually after the operation and medication were given withi

关 键 词：婴幼儿 丛状血管瘤 血小板减少 Kasabach-Merritt现象 治疗 

分 类 号：R732.2[医药卫生—肿瘤]