BackgroundCongenital diaphragmatic hernia (CDH) can be associated with genetic or structural anomalies with poor prognosis. However, intrapericardial CDH is a very rare phenotype with good outcome.Imaging Findings or Procedure DetailsA 26-year-old primiparous woman was referred for fetal anomalies. Under ultrasound examination, a fetus was diagnosed as intrapericardial CDH with massive pericardial effusion and liver herniation at 22 gestational weeks. Genetic amniocentesis was performed with normal karyotype (46, XY). Serial follow-up images were examined during her gestation. Cesarean section was performed at 37 weeks after premature rupture of membrane without labor progression. A male baby was delivered, weighted as 2,950 gm, with immediate endotracheal intubation and transferred to NICU. Surgery was arranged and a huge retrosternal defect of diaphragm (7 x 5 cm) with left lobe liver herniation into pericardial space was found. Reposition of the liver with closure of the defect was done. Two days after surgery, the baby was removed his ventilator successfully and experienced a very good outcome till his 4-year-old birthday.ConclusionIntrapericardial CDH is different from those common forms of CDHs which usually having poor perinatal outcome if happened early in gestation with poor pulmonary development. We will present serial ultrasound images and indicate how to predict fetal outcome. BackgroundCongenital diaphragmatic hernia (CDH) can be associated with genetic or structural anomalies with poor prognosis. However, intrapericardial CDH is a very rare phenotype with good outcome. Congenital diaphragmatic hernia (CDH) can be associated with genetic or structural anomalies with poor prognosis. However, intrapericardial CDH is a very rare phenotype with good outcome. Imaging Findings or Procedure DetailsA 26-year-old primiparous woman was referred for fetal anomalies. Under ultrasound examination, a fetus was diagnosed as intrapericardial CDH with massive pericardial effusion and liver herniation at 22 gestational weeks. Genetic amniocentesis was performed with normal karyotype (46, XY). Serial follow-up images were examined during her gestation. Cesarean section was performed at 37 weeks after premature rupture of membrane without labor progression. A male baby was delivered, weighted as 2,950 gm, with immediate endotracheal intubation and transferred to NICU. Surgery was arranged and a huge retrosternal defect of diaphragm (7 x 5 cm) with left lobe liver herniation into pericardial space was found. Reposition of the liver with closure of the defect was done. Two days after surgery, the baby was removed his ventilator successfully and experienced a very good outcome till his 4-year-old birthday. A 26-year-old primiparous woman was referred for fetal anomalies. Under ultrasound examination, a fetus was diagnosed as intrapericardial CDH with massive pericardial effusion and liver herniation at 22 gestational weeks. Genetic amniocentesis was performed with normal karyotype (46, XY). Serial follow-up images were examined during her gestation. Cesarean section was performed at 37 weeks after premature rupture of membrane without labor progression. A male baby was delivered, weighted as 2,950 gm, with immediate endotracheal intubation and transferred to NICU. Surgery was arranged and a huge retrosternal defect of diaphragm (7 x 5 cm) with left lobe liver herniation into pericardial space was found. Reposition of the liver with closure of the defect was done. Two days after surgery, the baby was removed his ventilator successfully and experienced a very good outcome till his 4-year-old birthday. ConclusionIntrapericardial CDH is different from those common forms of CDHs which usually having poor perinatal outcome if happened early in gestation with poor pulmonary development. We will present serial ultrasound images and indicate how to predict fetal outcome. Intrapericardial CDH is different from those common forms of CDHs which usually having poor perinatal outcome if happened early in gestation with poor pulmonary development. We will present serial ultrasound images and indicate how to predict fetal outcome.