摘要：
目的 探讨新生儿卡梅现象(Kasabach-Merritt phenomenon,KMP)临床特点、诊断及治疗经验.方法 选取本院2016年1月至2017年3月新生儿重症监护病房收治的KMP新生儿临床资料,对其临床表现、影像学检查、治疗及转归进行回顾性分析.结果 共纳入KMP新生儿9例,其中男6例,女3例,起病时间生后0～14 d.病灶位于浅表部位4例,深部脏器组织5例,其中2例宫内发现肝脏占位,3例起病隐匿.临床表现为反应弱、气促或鼻腔出血等.血小板减少9例,中位血小板计数24.0×109/L.影像学检查均提示血管瘤改变,3例合并血管畸形.1例病理活检诊断卡波两样血管内皮细胞瘤,行手术切除.全身用药治疗2例,单独介入栓塞2例,全身用药联合介入栓塞4例,未治疗1例;最终行手术治疗1例.7例治愈或好转;1例治疗2月余,因血小板严重减低、存在严重弥散性血管内凝血,家长放弃治疗;1例死亡.结论 新生儿KMP临床表现多样、与病灶累及部位有关,影像学检查有助于诊断,瘤体大量出血、多脏器功能损害可危及生命,目前治疗方案不一、疗效各异,多数经治疗预后良好.%Objective To study the clinical features,diagnosis and treatment in the neonates of Kasabach-Merritt phenomenn (KMP).Method To analyze retrospectively the data collected from the 9 neonates of Kasabach-Merritt phenomenon in our neonatal intensive care unit from January 2016 to March 2017.The data of their clinical presentation,imaging findings,treatment and outcomes were analyzed.Result Among the 9 cases,there were 6 males and 3 females,with onset age of 0 ～ 14 d.The hemangiomas were located over the body surface of the neck,back,trunk and limbs in 4 cases.The others were located in the deep organs in 5 cases.2 cases were found hepatic hemangiomas by fetal ultrasound and 3 cases had symptoms of poor response,dyspnea and nasal bleeding with occult onset.All the 9 cases had thrombocytopenia,and the average platelet count was 24.0 × 109/L.The imaging findings showed the changes of hemangiomas,and among them,3 cases were associated with arteriovenous fistula.In one case,the biopsy pathology diagnosis was Kaposiform hemangioendothelioma.Among 8 cases which was treated,2 cases had systemic medication,2 cases had interventional embolization and 4 cases were treated with combined therapy (interventional embolization together with medication),including 1 case of definitive surgical treatment.Finally 7 cases improved,1 case with severe thrombocytopenia and coagulation disorders did not improve and treatment abandoned and 1 case died before treatment.Conclusion The symptomatology of KMP is various,which is related to the location of the lesion.The imaging studies are helpful to diagnosis.Massive hemorrhage and multiple organ dysfunction can be fatal.The treatments are different among cases,for most cases with proper treatment the prognosis are good.
