EP09.108: Intrathoracic kidney position in fetuses with congenital diaphragmatic hernia: prenatal diagnosis and postnatal management

To present cases of prenatal diagnosis and postnatal management of intrathoracic kidney (ITK) position in fetuses with congenital diaphragmatic hernia (CDH). We reviewed our experience with prenatal diagnosis and postnatal management of ITK in fetuses with CDH at the Fetal Medicine Center of Rabin Medical Center from December 2018 to January 2024. During the study period, 86 fetuses with CDH were followed and delivered at our medical centre. Among them, two fetuses had ITK: one with moderate left CDH and another with severe right CDH. In addition, one fetus with severe right CDH exhibited a high kidney position. All fetuses were male and had normal chromosomal microarray examinations. In one of them, whole exome sequencing analyses were performed postnatally and revealed cardiac-urogenital syndrome. The mean gestational age at CDH diagnosis was 22 + 3 weeks. Abnormal kidney position was noted during CDH diagnosis in one case and during prenatal follow-up visits in two other cases. Fetuses with severe CDH underwent prenatal fetoscopic endoluminal tracheal occlusion followed by planned fetoscopy 6 weeks later for occlusion reversal. Prenatal diagnoses were confirmed during the neonatal surgery on day 2 after delivery in all three cases. The kidneys show no functional damage after surgical correction. All neonates were discharged home with normal renal function. The intrathoracic kidney may be present in both left and right CDH, of varying severity. Whole exome sequencing may be beneficial. Kidney repositioning is feasible and with a good urological outcome.