In utero repair of rectal atresia after complete resection of a sacrococcygeal teratoma

Purpose: A case of a fetus with a prenatally diagnosed sacrococcygeal terat oma that produced high-output cardiac failure, hydrops, rectal atresia, and urinary tract obstruction is presented. The unique prenatal surgical manag ement along with the embryogenesis of tumor-related rectal atresia is discu ssed. Case Report: 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 cardia c 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 u rinary diversion via bilateral flank ureterostomies, The rectum was found t o be completely atretic due to apparent encasement by the tumor. Pull-throu gh anorectoplasty was carried out concurrently. At 30 weeks' gestation, the mother developed preterm labor and a 1.8-kg was delivered by cesarean sect ion. The baby did very well for 3 days but had a cardiac arrest and died du e to an atrial perforation by a transfemoral venous catheter, Conclusions: To our knowledge this is the first report of a complete prenatal resection of a sacrococcygeal teratoma with concomitant pull-through anorectoplasty f or rectal atresia. Copyright (C) 2000 S. Karger AG, Basel.