Concurrent Bronchopulmonary Sequestration in Congenital Diaphragmatic Hernia: Is Frequency Increasing or Are We Better at Diagnosis?

OBJECTIVE

To assess the incidence and clinical outcomes of neonates with congenital diaphragmatic hernia with patients with concurrent bronchopulmonary sequestration (CDH + BPS).

STUDY

DESIGN

In this retrospective cohort study, we compared BPS diagnoses in patients with CDH across 4 epochs (2002-2008, 2009-2015, 2016-2019, 2020-2023) to assess incidence and outcomes of neonates with CDH + BPS. Because of marked epoch differences in key outcomes, we only compared extracorporeal membrane oxygenation use and survival for CDH + BPS and CDH alone across the final 2 epochs using multinominal regression analysis.

RESULTS

Among 383 neonates with CDH, we concurrently diagnosed 15 (3.9%) with BPS. Consistent with increasing fetal CDH diagnoses (56% in 2002-2008 vs 74% in 2020-2023; P < .05) and fetal magnetic resonance imaging (0% in 2002-2008 vs 92% in 2020-2023; P < .001), CDH + BPS rates increased from 1.9% across the first 3 epochs to 13.2% in the 2020-2023 epoch (P < .001). There were no differences in other anomalies, repair rates, thoracic liver, or defect size. From 2016 onward, all 10 neonates diagnosed with CDH + BPS survived without extracorporeal membrane oxygenation. CDH + BPS neonates had larger defects and increased rates of nonprimary repair. After we controlled for defect size, neonates with CDH + BPS had greater rates of vasodilator therapy for postoperative pulmonary hypertension (56% vs 23%; P < .05). However, there were no differences in home discharge on pulmonary vasodilator medications or oxygen.

CONCLUSION

Incidence of CDH + BPS diagnoses increased over time with an increased need of inpatient vasodilatory therapy. Additional studies with enhanced imaging analyses and intraoperative assessment are needed to better define the incidence and potential impact of concurrent BPS on CDH morbidities and outcomes.