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

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

• Document type: Journal article
• Source: PLoS One; v. 18, n. 2, p. 12-pg., 2023-02-10.

Abstract

BackgroundIn 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 Sao Paulo state of Brazil, the temporal trends of prevalence, neonatal mortality and lethality of CDH and identify the time to CDH-associated neonatal death. MethodsPopulation-based study of all live births with gestational age >= 22 weeks, birthweight >= 400g, from mothers residing in Sao 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. ResultsCDH 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. ConclusionsDuring a 12-year period in Sao 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. (AU)

• FAPESP's process: 17/03748-7 - Secular trends, spatial evolution, and maternal & neonatal conditions associated to early and late neonatal deaths caused by respiratory distress, perinatal asphyxia, infections, and congenital anomalies, in São Paulo State, Brazil, between 2002 and 2015
• Grantee: Ruth Guinsburg
• Support Opportunities: Research Projects - Thematic Grants