Prenatal diagnosis of ectopic intrathoracic kidney with right congenital diaphragmatic hernia manifesting as fetal mesocardia

Abstract

Although an ectopic intrathoracic kidney can be complicated by congenital diaphragmatic hernia (CDH), prenatal diagnosis of this condition is rare. We report a rare case of prenatally diagnosed ectopic intrathoracic kidney with CDH presenting as fetal mesocardia. A 32-year-old gravida 1, para 0 showed fetal mesocardia on ultrasonographic screening performed at 18 weeks’ gestation. No additional ultrasonographic anomalies were identified at the time. Detailed examination using magnetic resonance imaging at 30 weeks’ gestation showed that the fetal right kidney and a part of the liver were intrathoracic in location, and we diagnosed right CDH. She underwent cesarean section at 37 weeks’ gestation and delivered a female infant weighing 2,812 g with Apgar scores of 5-7. The infant was intubated immediately after birth, and mechanical ventilation was initiated. Although mild pulmonary hypertension was observed, her cardiorespiratory status stabilized smoothly. Computed tomography confirmed the diagnosis of right CDH, and we performed surgical repair on day 2 of birth. The posterior segment of the hepatic lobe and the right kidney were herniated through a right diaphragmatic defect and were covered by a hernia sac. The right kidney was located retroperitoneally and could not be replaced under the diaphragm. We diagnosed right CDH and concomitant ectopic intrathoracic kidney. We performed plication of the hernia sac with the transversus abdominis and Gerota’s fascia. In this case, fetal mesocardia led to the prenatal diagnosis. Our case suggests that CDH can cause an ipsilateral mediastinal shift by complication with ectopic intrathoracic kidney.