Analysis of early neonatal case fatality rate among newborns with congenital hydrocephalus, a 2000–2014 multi-country registry-based study

Background: Congenital hydrocephalus (CH) comprises a heterogeneous group of birth anomalies with a wide-ranging prevalence across geographic regions and registry type. The aim of the present study was to analyze the early neonatal case fatality rate (CFR) and total birth prevalence of newborns diagnosed with CH. Methods: Data were provided by 25 registries from four continents participating in the International Clearinghouse for Birth Defects Surveillance and Research (ICBDSR) on births ascertained between 2000 and 2014. Two CH rates were calculated using a Poisson distribution: early neonatal CFR (death within 7 days) per 100 liveborn CH cases (CFR) and total birth prevalence rate (BPR) per 10,000 births (including live births and stillbirths) (BPR). Heterogeneity between registries was calculated using a meta-analysis approach with random effects. Temporal trends in CFR and BPR within registries were evaluated through Poisson regression modeling. Results: A total of 13,112 CH cases among 19,293,280 total births were analyzed. The early neonatal CFR was 5.9 per 100 liveborn cases, 95% confidence interval (CI): 5.4–6.8. The CFR among syndromic cases was 2.7 times (95% CI: 2.2–3.3) higher than among non-syndromic cases (10.4% [95% CI: 9.3–11.7] and 4.4% [95% CI: 3.7–5.2], respectively). The total BPR was 6.8 per 10,000 births (95% CI: 6.7–6.9). Stratified by elective termination of pregnancy for fetal anomalies (ETOPFA), region and system, higher CFR were observed alongside higher BPR rates. The early neonatal CFR and total BPR did not show temporal variation, with the exception of a CFR decrease in one registry. Conclusions: Findings of early neonatal CFR and total BPR were highly heterogeneous among registries participating in ICBDSR. Most registries with higher CFR also had higher BPR. Differences were attributable to type of registry (hospital-based vs. population-based), ETOPFA (allowed yes or no) and geographical regions. These findings contribute to the understanding of regional differences of CH occurrence and early neonatal deaths.Fil: Gili, Juan Antonio. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Parque Centenario. CEMIC-CONICET. Centro de Educaciones Médicas e Investigaciones Clínicas "Norberto Quirno". CEMIC-CONICET; ArgentinaFil: López Camelo, Jorge Santiago. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Parque Centenario. CEMIC-CONICET. Centro de Educaciones Médicas e Investigaciones Clínicas "Norberto Quirno". CEMIC-CONICET; ArgentinaFil: Nembhard, Wendy N.. University of Arkansas for Medical Sciences; Estados UnidosFil: Bakker, Marian. Universitair Medisch Centrum Groningen; Países BajosFil: de Walle, Hermien E. K.. Universitair Medisch Centrum Groningen; Países BajosFil: Stallings, Erin B.. No especifíca;Fil: Kancherla, Vijaya. No especifíca;Fil: Contiero, Paolo. No especifíca;Fil: Dastgiri, Saeed. Tabriz Health Services Management Research Center; IránFil: Feldkamp, Marcia L.. University of Utah; Estados UnidosFil: Nance, Amy. University of Utah; Estados UnidosFil: Gatt, Miriam. No especifíca;Fil: Martínez, Laura. Universidad Autónoma de Nuevo León; MéxicoFil: Canessa, María Aurora. No especifíca;Fil: Groisman, Boris. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorios e Institutos de Salud "Dr. Carlos G. Malbrán". Centro Nacional de Genética Médica; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; ArgentinaFil: Hurtado Villa, Paula. Pontificia Universidad Javeriana; ColombiaFil: Källén, Karin. No especifíca;Fil: Landau, Danielle. Soroka University Medical Center; IsraelFil: Lelong, Nathalie. Universite de Paris; FranciaFil: Morgan, Margery. No especifíca;Fil: Arteaga Vázquez, Jazmín. Instituto Nacional de la Nutrición Salvador Zubiran; MéxicoFil: Pierini, Anna. No especifíca;Fil: Rissmann, Anke. No especifíca;Fil: Sipek, Antonin. No especifíca;Fil: Szabova, Elena. No especifíca;Fil: Wertelecki, Wladimir. No especifíca;Fil: Zarante, Ignacio. Pontificia Universidad Javeriana; ColombiaFil: Canfield, Mark A.. University of Texas; Estados UnidosFil: Mastroiacovo, Pierpaolo. No especifíca