Agarwal Arpit, Rosenkranz Eliot, Yasin Salih, Swaminathan Sethuraman
a Division of Pediatric Cardiology , Jackson Memorial Hospital, University of Miami Miller School of Medicine , Miami , FL , USA.
b Division of Pediatric Cardiothoracic Surgery , Jackson Memorial Hospital, University of Miami Miller School of Medicine , Miami , FL , USA.
J Matern Fetal Neonatal Med. 2018 Apr;31(8):1099-1103. doi: 10.1080/14767058.2017.1306851. Epub 2017 Apr 2.
Large mediastinal teratomas in the fetus are rare and can present with direct compression of intrathoracic structures as well as pericardial and pleural effusions. Mediastinal fetal teratoma may be detected as a mass in the chest during a routine prenatal ultrasound. Because of the pressure on mediastinal structures it may result in non-immune fetal hydrops (NIFH) and polyhydramnios. The development of hydrops may lead to fetal demise. Timely obstetric and/or surgical intervention is important to improve survival in this patient population. Case review: We report a case of a large mediastinal teratoma in a fetus who presented with a large pericardial effusion at 28 weeks gestation. The fetus developed NIFH at 31 weeks gestation. The fetus was successfully managed with an ex utero intrapartum therapy (EXIT) procedure which involved pericardiocentesis and surgical resection of the large teratoma. Histological examination revealed an encapsulated immature teratoma.
Mediastinal teratomas are usually detected by routine second- and third-trimester ultrasound. Large teratomas have been described to present with airway compromise and NIFH. Some cases have been managed by postnatal surgery and rare cases by EXIT procedure.
This is the first reported case of a fetal mediastinal teratoma and severe pericardial effusion who developed NIFH, in whom EXIT procedure was successfully employed to simultaneously drain the effusion and resect the tumor.
胎儿巨大纵隔畸胎瘤罕见,可导致胸内结构直接受压以及心包和胸腔积液。纵隔胎儿畸胎瘤可在常规产前超声检查时被发现为胸部肿块。由于对纵隔结构的压迫,可能导致非免疫性胎儿水肿(NIFH)和羊水过多。水肿的发展可能导致胎儿死亡。及时的产科和/或手术干预对于提高该患者群体的生存率很重要。病例回顾:我们报告一例胎儿巨大纵隔畸胎瘤病例,该胎儿在妊娠28周时出现大量心包积液。胎儿在妊娠31周时发展为NIFH。通过产时宫外治疗(EXIT)程序成功处理了该胎儿,该程序包括心包穿刺术和巨大畸胎瘤的手术切除。组织学检查显示为包膜完整的未成熟畸胎瘤。
纵隔畸胎瘤通常通过常规孕中期和孕晚期超声检查发现。已描述巨大畸胎瘤可导致气道受压和NIFH。一些病例通过出生后手术处理,罕见病例通过EXIT程序处理。
这是首例报道的胎儿纵隔畸胎瘤合并严重心包积液并发展为NIFH的病例,成功采用EXIT程序同时引流积液并切除肿瘤。