Congenital Diaphragmatic Hernia with Pulmonary Sequestration- Incidence and Outcomes

Background: Bronchopulmonary sequestration (BPS) has long been identified in patients with congenital diaphragmatic hernia (CDH), however the reported incidence in the literature varies widely and is not based on large series. Our objective was to identify the incidence and outcomes among infants with these concomitant entities.

Methods: Version 4 of the Congenital Diaphragmatic Hernia Study Group (CDSHG) had questions specifically to ask about BPS. The data were prospectively collected between 2015-2020. The registry was queried for patients with CDH and associated BPS (CDH+BPS). Clinical characteristics and outcomes for CDH+BPS patients were compared to patients without BPS. Univariate and multiple regression analyses were performed using Stata/IC.

Results: Out of 2120 total patients, 74 had a pulmonary sequestration (3.5%). The majority of these patients had type C defects (33/74, 46.5%). The amount of CDH+BPS with high-risk (CDHSG type C and D) defects was significantly greater than those without BPS (69.0% vs 49.8%, respectively; p = 0.001). The number of patients with CDH+BPS needing ECMO was significantly higher than other patients (35/74, 47.3%). Overall mortality was 31.1% (23/74). When corrected for hernia size, cardiac and chromosomal anomalies, the need for ECMO (OR 2.2, p = 0.004) and mortality (OR 2.1, p = 0.013) was significantly greater in CDH+BPS.

Conclusions: This is the largest series to look at the incidence of BPS in children with CDH. Overall, they are associated with larger defect sizes, a greater need for ECMO and higher mortality than those patients without BPS. The timing and method of management of the sequestrations requires further investigation.