CAJ | 학술논문

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.
목적 탐토영유인총상혈관류(TA)적림상특점、질병진정,급병발Kasabach-Merritt현상(KMP)적치료방법.방법 수집、분석2009년1월지2013년3월수치적24례영유인TA환인적림상자료화수방자료.남10례,녀14례;취진년령18 d～2세,중위년령위7.5개월.근거병변정황선택림상관찰급수술치료등.수방1.2 ～5.4년,평균3.6년.수방관찰환인병정변화.결과 림상상견증상혹체정포괄암홍혹자홍색변계불청적체표피손、혈소판감소、동통혹공능수한이급다한、다모등.기림상진정주요유3충류형:병변완전혹부분자발성소퇴2례(8.3％)、지속존재9례(37.5％)、합병KMP 13례(54.2％).TA발병지병발KMP평균간격시간45.2 d(0d～4개월).KMP발병최초증상혹체정표현위신속증대적류체(8례)、류체명현변후장력증대(3례)、호흡도군박표현위호흡급촉、삼요정명현(2례).13례합병KMP균채용수술치료,수술완전절제자10례,술후혈소판계수1～3d승지정상,혈홍단백급응혈공능1～2주축점회복정상;대부절제자3례,혈소판계수술후균출현반복,단명현고우술전,다유지재60×109/L이상,경술후급여약물치료,기중2례3～6개월후류체축점소실,혈소판계수회복정상,1례술후인다기관공능쇠갈사망.결론 적겁주동간예치료주요괄용우영향외관혹용모、이경혹가능병발KMP혹기타공능이상.대년령교소무엄중병발증적TA,조기가이수방관찰,정기감측기혈소판계수,이진조발현KMP.대합병KMP적TA,명학진단후진조수술치료,가명현축단치료시간,강저약물불량반응,치료효과량호,강저환인병사솔.
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 within 3-6 months.And the other case died of multiple organ failure post-operation.Conclusions Surgical intervention can be applied to TA that severely makes damage to children's appearance or looks or may be complicated with KMP or functional abnormality.A closely monitored policy seems appropriate for the early small tumor without severe complications.And it is necessary to monitor the number of the platelet regularly and find the KMP by as early as possible.TA associated with KMP is vitol to infants.And surgical therapy after clear diagnosis should be done as early as possible.The surgical therapy is a reliable management with higher curative rate,short disease period and minimal side-effect.