Research: Congenital diaphragmatic hernia in a middle-income country: Persistent high lethality during a 12-year period

PLoS One

. 2023 Feb 10;18(2):e0281723.

 doi: 10.1371/journal.pone.0281723. eCollection 2023.

Congenital diaphragmatic hernia in a middle-income country: Persistent high lethality during a 12-year period

Ana Sílvia Scavacini Marinonio 1Milton Harumi Miyoshi 1Daniela Testoni Costa-Nobre 1Adriana Sanudo 1Kelsy Catherina Nema Areco 1Mandira Daripa Kawakami 1Rita de Cassia Xavier Balda 1Tulio Konstantyner 1Paulo Bandiera-Paiva 1Rosa Maria Vieira de Freitas 2Lilian Cristina Correia Morais 2Mônica La Porte Teixeira 2Bernadette Cunha Waldvogel 2Carlos Roberto Veiga Kiffer 1Maria Fernanda Branco de Almeida 1Ruth Guinsburg 1

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Background: In high- and middle-income countries, mortality associated to congenital diaphragmatic hernia (CDH) is high and variable. In Brazil, data is scarce regarding the prevalence, mortality, and lethality of CDH. This study aimed to analyze, in São Paulo state of Brazil, the temporal trends of prevalence, neonatal mortality and lethality of CDH and identify the time to CDH-associated neonatal death.

Methods: Population-based study of all live births with gestational age ≥ 22 weeks, birthweight ≥400g, from mothers residing in São Paulo State, Brazil, during 2004-2015. CDH definition and its subgroups classification were based on ICD-10 codes reported in the death and/or live birth certificates. CDH-associated neonatal death was defined as death up to 27 days after birth of infants with CDH. CDH prevalence, neonatal mortality and lethality were calculated and their annual percent change (APC) with 95% confidence intervals (95%CI) was analyzed by Prais-Winsten. Kaplan-Meier estimator identified the time after birth that CDH-associated neonatal death occurred.

Results: CDH prevalence was 1.67 per 10,000 live births, with a significant increase throughout the period (APC 2.55; 95%CI 1.30 to 3.83). CDH neonatal mortality also increased over the time (APC 2.09; 95%CI 0.27 to 3.94), while the lethality was 78.78% and remained stationary. For isolated CDH, CDH associated to non-chromosomal anomalies and CDH associated to chromosomal anomalies the lethality was, respectively, 72.25%, 91.06% and 97.96%, during the study period. For CDH as a whole and for all subgroups, 50% of deaths occurred within the first day after birth.

Conclusions: During a 12-year period in São Paulo State, Brazil, CDH prevalence and neonatal mortality showed a significant increase, while lethality remained stable, yet very high, compared to rates reported in high income countries.

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