Right-Sided Congenital Diaphragmatic Hernia: Characterization, Utility of Prenatal Imaging Predictors and Outcomes.

Abstract

Purpose: Identify the most accurate prenatal predictor of survival for infants with right-sided congenital diaphragmatic hernia (R-CDH), analyze management strategies and outcomes and evaluate differences in outcomes based on surgical repair techniques.

Methods: We conducted a retrospective review of infants diagnosed with R-CDH at a single institution from 2010-2024. Prenatal ultrasound and MRI values were analyzed to predict survival as well as secondary outcomes including need for extracorporeal membrane oxygenation (ECMO), duration on mechanical ventilation, length of hospitalization (LOH), and severity of pulmonary hypertension. Outcomes between repair techniques were compared in a subgroup analysis.

Results: Thirty-two infants with R-CDH met inclusion criteria. Overall survival to discharge was 75%. Seventeen (53%) had prenatal imaging and were diagnosed prenatally. Among ultrasound and MRI prenatal predicitive indices, no thresholds to accurately distinguish survivors from non-survivors were identified. All but one infant diagnosed prenatally required ECMO. Nine (28%) underwent primary repair, nine (28%) were repaired with a synthetic patch, and 14 (44%) with a transversus abdominus muscle flap. Infants with patch repairs required reoperation for a bleeding complication more often than those repaired with a muscle flap [5 (56%) versus 1 (7%), p=0.02].

Conclusion: No prenatal indicator of disease severity accurately predicted survival. Patch and muscle flap repair are both accepted techniques to repair R-CDH defects. All but one infant diagnosed prenatally with R-CDH required ECMO, underscoring the critical need for delivery at a tertiary care center equipped with ECMO capabilities.