Crossing birth and mortality data as a clue for prevalence of congenital diaphragmatic hernia in Sao Paulo State: A cross sectional study

Background Congenital diaphragmatic hernia (CDH) is a severe embryological defect that causes pulmonary hypoplasia and hypertension. The prevalence and mortality rate of CDH varies around the world and little information is available about CDH in Latin America. Our aim was to estimate the general prevalence, mortality rate, prevalence of associated anomalies and features related to the outcomes of CDH in newborns from Sao Paulo state, Brazil. Methods Population-based cross-sectional study based on data gathered from the Live Births Information System (SINASC) and the Mortality Information System (SIM) of children born in Sao Paulo state between January 1st, 2006, and December 31st, 2017. Findings From 7,311,074 total survival discharges between 2006 and 2017, 1,155 were CDH-related, resulting in a prevalence rate of 1:6329 (95%CI = 1/6715 - 1/5984) and a mortality rate of 63 center dot 72% (95%CI = 60.95 - 66.50), 510 presented complex associated anomalies (44 center dot 15%). Maternal data showed higher prevalence among older mothers (older than 35 years old: 2 center dot 13 per 10,000) and, also, women with more years of schooling (higher than 12 years: 1 center dot 99 per 10,000). Presence of associated anomalies (95%CI = 5.69-11.10), 1-min Apgar (95%CI = 1.44-2.95), maternal schooling (95%CI = 1.06-2.43) and birth weight (95%CI = 1.04-2.26) were the most significant features associated with mortality. Interpretation There was 1 CDH case for every 6329 newborns in Sao Paulo and the mortality rate among those cases was 63 center dot 72% - a high rate compared to other countries. Copyright (c) 2022 The Author(s). Published by Elsevier Ltd. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/)