Center for Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development, Tokyo, Japan.
Department of Pediatric Surgery, National Center for Child Health and Development, Tokyo, Japan.
J Obstet Gynaecol Res. 2022 Dec;48(12):3308-3313. doi: 10.1111/jog.15436. Epub 2022 Sep 27.
We report a very rare case of large fetal mediastinal cystic lymphatic malformation (cLM), formerly called lymphangioma, that was managed with prenatal serial thoracocentesis and postnatal sclerotherapy. A fetal pericardial effusion-like lesion gradually increased the size until it occupied more than half of the thoracic cavity. Thoracocentesis was performed three times, namely at 31 and 35 weeks of gestation, and also just before the delivery for decompression. The characteristic shape changes of the lesion were observed after thoracocentesis. A boy delivered by planned cesarian section at term required respiratory support after birth. Postnatal magnetic resonance imaging (MRI), which showed a large cystic lesion with septa and hemorrhage without solid components, led to the diagnosis of cLM. The fluid from the cyst continued to be drained. Neonatal sclerotherapy using OK-432 was effective and the infant recovered well. Fetal thoracocentesis may be useful for the diagnosis and management of a large mediastinal cLM.
我们报告了一例非常罕见的胎儿纵隔大囊性淋巴管畸形（cLM）病例，该病以前称为淋巴管瘤，采用产前系列胸腔穿刺术和产后硬化治疗进行治疗。胎儿心包积液样病变逐渐增大，直至占据胸腔的一半以上。胸腔穿刺术进行了三次，分别在 31 周和 35 周妊娠时进行，并且也在分娩前进行了减压。胸腔穿刺术后观察到病变的特征形状变化。一名足月通过计划性剖宫产分娩的男孩出生后需要呼吸支持。出生后磁共振成像（MRI）显示一个大的囊性病变，有分隔和出血，但无实性成分，提示为 cLM。囊肿中的液体继续排出。使用 OK-432 的新生儿硬化治疗有效，婴儿恢复良好。胎儿胸腔穿刺术对于诊断和治疗大的纵隔 cLM 可能是有用的。
