卡梅现象患儿围手术期治疗体会  

Perioperative managements of infant patients with Kasabach-Merritt phenomenon

作　　者：郭晓楠[1] 董长宪[1] 龚毓宾 张红宇[1] 张源方 王笑林[1] Guo Xiaonan;Dong Changxian;Gong Yubin;Zhang Hongyu;Zhang Yuanfang;Wang Xiaolin(Department of Hemangioma&Vascular Malformation,Henan Provincial People’s Hospital,Zhengzhou 450003,China)

机构地区：[1]河南省人民医院血管瘤科,郑州450003

出　　处：《中华整形外科杂志》2021年第9期1036-1040,共5页Chinese Journal of Plastic Surgery

摘　　要：目的探讨更加安全、有效、规范的卡梅现象患儿围手术期治疗方法。方法回顾性分析2017年1月至2019年9月在河南省人民医院血管瘤科进行手术治疗的卡梅现象患儿临床资料。纳入标准:(1)诊断为卡梅现象,以位于躯干或肢体的巨大血管瘤、重度血小板减少和消耗性凝血功能障碍为特征;(2)2017年1月至2019年9月在该中心接受手术治疗;(3)年龄≤1岁。术前予以甲泼尼龙琥珀酸钠2 mg/kg,敏感患者应用至术前,不敏感患者于术前1 d单次大剂量血小板输注,纠正凝血功能;采用气管插管静脉复合麻醉方式,配合深静脉置管,动脉穿刺置管并持续有创血压监测,维持血流动力学稳定;行瘤体根治性切除术,必要时配合皮瓣成形术或原位植皮术;术后常规带管入ICU,呼吸循环恢复后酌情拔除气管插管,ICU留观过夜,评估稳定后转出;术后监测血小板动态变化,加强营养支持,病变位于四肢的患者(原位植皮者除外),于术后第3天开始进行被动康复训练;随访6~36个月,行血常规、凝血功能、彩色多普勒超声检查,必要时行MRI检查。肢体病变患儿,复诊时检查邻近关节活动度、肌力情况。结果共纳入55例卡梅现象患儿。术前1 h末梢血检测显示54例血小板>100×10^(9)/L,1例>80×10^(9)/L,血红蛋白均纠正至10 g/L以上;手术时间48~135 min,平均87 min;无术中、术后死亡病例;术后血小板恢复至正常水平历时4~36 h,平均8.4 h;手术标本病理检查结果均为卡波西样血管内皮瘤;住院时间9~30 d,平均16.7 d;手术切口延迟愈合3例,瘢痕挛缩致活动障碍1例,瘢痕增生3例;随访周期内无死亡病例,血小板均稳定在正常范围。结论手术治疗合并卡梅现象的血管肿瘤,疗效确切、起效迅速、疗程短、费用低。积极的术前准备、有效的凝血功能纠正措施、完善的麻醉及监护方式、稳定的血流动力学保障、精细的手术操作及术后早期进行的�Objective To investigate more safe,effective and standard perioperative managements of infant patients with Kasabach-Merritt phenomenon(KMP).Methods We made a retrospective analysis on the clinical data of KMP infant patients,who received surgical intervention in our department between January 2017 and September 2019.Inclusion criteria:(1)diagnosed as KMP that characterized by a large hemangioma(located in trunk or limb),profound thrombocytopenia and consumptive coagulopathy;(2)received surgical treatment in our center during January 2017 and September 2019;(3)age≤1 year.Before surgical treatment,all the patients were given glucocorticoid and continued to the operation day in the sensitive group.The insensitive group received single large dose of platelet(PLT)transfusion 1 day before surgery,for the purpose of correcting thrombocytopenia and coagulopathy.Endotracheal intubation and intravenous anesthesia,combined with deep vein catheterization,arterial puncture catheterization and continuous invasive blood pressure monitoring were used to maintain hemodynamic stability.Radical resection of the tumor,combined with flap plasty or in situ skin grafting was carried out when necessary;after the operation,the endotracheal tube was routinely taken to ICU,and the endotracheal tube was removed as appropriate after the recovery of respiratory and circulation.The patient was kept overnight in ICU,and patient was transferred out after evaluation of stability.The dynamic changes of platelet were monitored and nutritional support was strengthened.Patients with lesions in limbs(except those with in situ skin grafting)were given passive rehabilitation training on the third day after surgery.The patients were followed up for 6-36 months.Routine blood examination,coagulation function,color Doppler ultrasonography and MRI were performed when necessary.The range of motion and muscle strength of adjacent joints were examined during the follow-up visit.Results A total of 55 infant patients with KMP were included in this study.Periph

关 键 词：血管肿瘤 卡梅现象 围手术期 外科治疗 卡波西样血管内皮瘤 丛状血管瘤 

分 类 号：R732.2[医药卫生—肿瘤]