Incidence and mortality trends in congenital diaphragmatic hernia in the United States

The average incidence of congenital diaphragmatic hernia (CDH) in the United States (US) is 2.6 per 10,000 live births but it varies based on the population studied, the database used, and the study period. Further, previous studies suggest that pre-discharge mortality in CDH is declining but this may not capture the 'hidden mortality' and post-discharge mortality. We examined a population-based database to evaluate the trends in the incidence (2016-2023) and CDH-related infant mortality rate (CDH-IMR) [2007-2022] in the US.

We conducted a retrospective cross-sectional analysis of the CDC WONDER database. First, we queried the 2016-2023 natality dataset derived from birth certificates for live births with CDH (ICD-10 code Q79.0). We expressed CDH incidence as per 10,000 live births. Next, we queried the linked birth/infant death dataset from 2007 to 2022 for CDH-IMR through 1 year of age. CDH-IMR was expressed per 100,000 live births. Trends were evaluated with Joinpoint regression and reported using average annual percentage change (AAPC) with 95 % confidence intervals (CI).

Among 29, 880, 509 live births between 2016 and 2023, 3,797 had CDH (1.3 per 10,000). Of these, 33.4 % were transferred within 24 h after birth. There was no significant change in the CDH incidence during the study period (AAPC 0.93 %; CI: -0.1, 2.0). The overall CDH-IMR (per 100,000) was 5.7, and it declined significantly from 6.3 in 2007 to 4.7 in 2022 (AAPC: -1.5 %; CI: -2.2, -0.8).

The CDH incidence, which was lower than previously reported and did not change from 2016 to 2023, requires validation. The downward trend in mortality needs ongoing surveillance to monitor the impact of new management strategies on mortality rates.