Right Bochdalek congenital diaphragmatic hernia: a tertiary center’s experience over 13 years

BACKGROUND: Right Bochdalek congenital diaphragmatic hernia (RB-CDH) is far less common than left Bochdalek congenital diaphragmatic hernia, accounting for only 13% of cases. There are limited published data on the outcomes and survival rate of RB-CDH. We aimed at investigating the clinical characteristics and analyzing the risk factors of survival in neonates with RB-CDH treated in our center over a period of 13 years. RESULTS: Fifteen infants with RB-CDH were identified. Most of the patients were full term (74%). The mean birth weight was 2.90± 0.72 kg. The ratio of male to female was 2:1. The mean APGAR score at 1 min was 5.31±2.34, and 7.30±1.59 at 5 min. Ten patients (67%) were imaged by antenatal ultrasound. Eleven patients (73.33%) survived to go for surgical repair. The hernia sac was found in 5 patients (45%). Most hernial defects were closed in a primary fashion. The mean age at the operative repair was 8.11±9.90 days. The average NICU stay for all patients was 40.47±50.38 days. The mean follow-up period was 20.45±9.34 months. Three patients had postoperative complications. The total survival rate in neonates with RB-CDH was 9/15 (60%). Nine out of 11 (82%) neonates survived after surgical repair. Four patients (27%) died before surgical repair. Ventilation-related bilateral pneumothorax was a contributing cause of death in three patients. Birth weight was found lower in the non-survivor’s group (P < 0.05). Moreover, the degree of pulmonary hypertension was more severe among non-survivors. No statistical significance was observed between other variables and mortality. CONCLUSION: We found that low birth weight and the presence of severe PHTN were risk factors for mortality in neonates with RB-CDH. These results are in line with previous studies on prognostic factors in CDH. Ventilator-related pneumothorax appears to be a significant contributing cause of death. Long-term follow-up studies of infants born with RB-CDH are needed as small number of cases limits large-volume RB-CDH studies.