胎儿卡波西样血管内皮瘤并发卡梅综合征的产前特征:1例报告及文献复习  

Prenatal features of fetal Kaposiform hemangioendothelioma with Kasabah-Meritt syndrome:a case report and literature review

作　　者：符忠蓬 王珏 任芸芸 FU Zhong-peng;WANG Jue;REN Yun-yun(Department of Ultrasound,Obstetrics and Gynecology Hospital,Fudan University,Shanghai 200011,China;Department of Obstetrics,Obstetrics and Gynecology Hospital,Fudan University,Shanghai 200011,China)

机构地区：[1]复旦大学附属妇产科医院超声科,上海200011 [2]复旦大学附属妇产科医院产科,上海200011

出　　处：《复旦学报（医学版）》2024年第5期868-872,共5页Fudan University Journal of Medical Sciences

摘　　要：探讨胎儿卡波西样血管内皮瘤(Kaposiform hemangioendothelioma,KHE)并发卡梅综合征(KasabachMerritt syndrome,KMS)的围产期特点。本文报道1名36岁女性孕38周产检时,超声偶然发现胎儿背部皮下肿块,其他产前检查结果无异常。肿块呈中低回声,直径约为6~7 cm,多普勒显示有条状血流信号。胎儿生长测量与胎龄相符,羊水量正常。经剖宫产娩出1名活产男婴,该新生儿左背皮下存在一处巨大血管瘤,实验室检查显示该患儿存在血小板减少、凝血功能障碍,被诊断为KMS,予以静注大剂量甲强龙,口服西罗莫司,手术切除以及输血等治疗。组织病理学和免疫组化结果证实为簇状血管瘤和KHE。术后随访半年无残留复发。To investigate the perinatal features of fetal Kaposiform hemangioendothelioma(KHE)complicated with Kasabach-Merritt syndrome(KMS),we reported an incidental ultrasound finding of a subcutaneous mass on the back of the foetus in a 36-year-old woman at 38 weeks of pregnancy,with no other abnormal prenatal findings.The dorsal mass was hypoechoic,approximately 6-7 cm in diameter,and Doppler showed a striated blood flow signal.Fetal growth measurements were consistent with gestational age,and the amniotic fluid volume was normal.A live-born boy was delivered by caesarean section.The neonate had a large subcutaneous haemangioma on the left dorsum with thrombocytopenia and coagulopathy.He was diagnosed with KMS and was treated with intravenous high dose pulse methylprednisolone,sirolimus,surgical resection and blood transfusion.Histopathological and immunohistochemical findings confirmed tufted hemangioma and KHE.There was no residual recurrence on postoperative follow-up of 6 months.

关 键 词：妊娠 卡波西样血管内皮瘤(KHE) 卡梅综合征(KMS) 胎儿水肿 胎儿贫血 

分 类 号：R249[医药卫生—中医临床基础]