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

VernacularTitle: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
Author: Zhong-Peng FU ¹ ; Jue WANG ; Yun-Yun REN
Author Information

1. 复旦大学附属妇产科医院超声科上海 200011
Keywords: pregnancy; Kaposiform hemangioendothelioma(KHE); Kasabach-Merritt syndrome(KMS); hydrops fetalis; fetal anemia
From: Fudan University Journal of Medical Sciences 2024;51(5):868-872
CountryChina
Language:Chinese
Abstract: 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.