新生儿卡波西样淋巴管瘤病1例  

Kaposiform lymphangiomatosis in a newborn

作　　者：黄超 彭艳芬 吕俊健 牛会林 何秋明 钟微 Huang Chao;Peng Yanfen;Lyu Junjian;Niu Huilin;He Qiuming;Zhong Wei(Department of Neonatal Surgery,Guangzhou Women and Children's Medical Center,Guangzhou 510623,China;Department of Pathology,Guangzhou Women and Children's Medical Center,Guangzhou 510623,China)

机构地区：[1]广州市妇女儿童医疗中心新生儿外科监护室,广州510623 [2]广州市妇女儿童医疗中心病理科,广州510623

出　　处：《中华围产医学杂志》2023年第10期865-868,共4页Chinese Journal of Perinatal Medicine

摘　　要：本文报道了1例胎儿期发现、新生儿期确诊的卡波西样淋巴管瘤病(kaposiform lymphangiomatosis,KLA)病例。孕19周常规超声检查发现胎儿全身多处包块(累及颈部、胸壁、腋下)合并胸腹腔积液,孕26周+5外院行胎儿右侧胸腔积液引流术。胎龄34周+3因"宫内窘迫"剖宫产出生,新生儿出生后因呼吸窘迫需要有创呼吸机辅助通气治疗,放置胸腹腔引流管引流出大量血性积液。生后第3天出现凝血功能障碍、血小板计数降低,考虑KLA,予西罗莫司经验性治疗效果不理想,生后第7天取胸部病灶送病理学检查。生后第12天因呼吸、循环功能衰竭抢救无效死亡。死亡后1 d病理结果回报符合KLA。结合患儿临床表现及病理结果,最终诊断KLA。This article reported a case of kaposiform lymphangiomatosis(KLA)identified in the fetal stage and diagnosed at the neonatal stage.A routine ultrasound examination at 19 weeks of gestation showed multiple masses in the whole body of the fetus(involving neck,chest wall and armpit)complicated by pleural and peritoneal effusion.Shunting was performed to drain pleural effusion from the right chest in another hospital at 26+5 weeks of gestation.The patient was born at 34+3 weeks of gestation by cesarean section due to"intrauterine distress"and required invasive ventilator assisted ventilation support after birth because of respiratory distress.A large amount of hemorrhagic effusion was drained out during the shunting.Coagulation dysfunction and thrombocytopenia occurred on the 3rd day after birth and KLA was suspected.Empirical treatment with sirolimus turned out to be ineffective.Biopsy was taken on postnatal day 7.However,the patient died on the 12th day after birth due to respiratory and circulatory failure.Pathological findings obtained the day after death were consistent with the features of KLA.The diagnosis of KLA was confirmed based on the clinical manifestations and pathological results.

关 键 词：淋巴管畸形 淋巴管扩张 婴儿 新生 

分 类 号：R722.1[医药卫生—儿科]