Chiba T, Albanese C T, Jennings R W, Filly R A, Farrell J A, Harrison M R
Fetal Treatment Center, University of California, San Francisco, Calif., 94143-0570, USA.
Fetal Diagn Ther. 2000 May-Jun;15(3):187-90. doi: 10.1159/000021003.
A case of a fetus with a prenatally diagnosed sacrococcygeal teratoma that produced high-output cardiac failure, hydrops, rectal atresia, and urinary tract obstruction is presented. The unique prenatal surgical management along with the embryogenesis of tumor-related rectal atresia is discussed.
A large fetal sacrococcygeal teratoma with a significant intrapelvic component was detected at routine ultrasound in a 35-year-old gravida 3 para 2. Fetal hydrops developed rapidly due to high-output cardiac failure from the vascular 'steal' by the growing tumor. The urinary tract was obstructed due to the intrapelvic tumors mass. At 27 weeks' gestation, the female fetus underwent hysterotomy, resection of the entire mass and urinary diversion via bilateral flank ureterostomies. The rectum was found to be completely atretic due to apparent encasement by the tumor. Pull-through anorectoplasty was carried out concurrently. At 30 weeks' gestation, the mother developed preterm labor and a 1.8-kg was delivered by cesarean section. The baby did very well for 3 days but had a cardiac arrest and died due to an atrial perforation by a transfemoral venous catheter.
To our knowledge this is the first report of a complete prenatal resection of a sacrococcygeal teratoma with concomitant pull-through anorectoplasty for rectal atresia.
本文报告一例产前诊断为骶尾部畸胎瘤的胎儿病例，该畸胎瘤导致高输出量心力衰竭、胎儿水肿、直肠闭锁及尿路梗阻。文中讨论了独特的产前手术管理方法以及与肿瘤相关的直肠闭锁的胚胎发生情况。
一名35岁、孕3产2的孕妇在常规超声检查时发现一个巨大的胎儿骶尾部畸胎瘤，盆腔内有较大占位。由于生长中的肿瘤造成血管“窃血”，导致胎儿迅速出现水肿及高输出量心力衰竭。盆腔内肿瘤肿块致使尿路梗阻。孕27周时，该女胎接受了子宫切开术，完整切除整个肿块，并通过双侧胁腹输尿管造口术进行尿液改道。发现直肠因明显被肿瘤包绕而完全闭锁。同时进行了拖出式肛门直肠成形术。孕30周时，母亲出现早产，通过剖宫产分娩出一名体重1.8千克的婴儿。婴儿术后3天情况良好，但因经股静脉导管导致心房穿孔，出现心脏骤停并死亡。
据我们所知，这是首例对骶尾部畸胎瘤进行完整产前切除并同时对直肠闭锁进行拖出式肛门直肠成形术的报告。
