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.

Birth Defects Epidemiology and Surveillance Branch Texas Department of State Health Services Austin Texas USA

CARIS The Congenital Anomaly Register for Wales Singleton Hospital Swansea Wales UK

Department of Basic Sciences of Health School of Health Pontificia Universidad Javeriana Cali Colombia

Department of Epidemiology Arkansas Center for Birth Defects Research and Prevention and Arkansas Reproductive Health Monitoring System University of Arkansas for Medical Sciences Little Rock Arkansas USA

Department of Epidemiology Emory University Rollins School of Public Health Atlanta Georgia USA

Department of Genetics RYVEMCE Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán México City Mexico

Department of Genetics University of Groningen University Medical Center Groningen EUROCAT Northern Netherlands Groningen The Netherlands

Department of Medical Genetics Thomayer University Hospital Prague Czech Republic

Department of Neonatology Soroka Medical Center Beer Sheva Israel

Division of Medical Genetics Department of Pediatrics University of Utah School of Medicine Salt Lake City Utah USA

ECLAMC Centro de Educación Médica e Investigaciones Clínicas Buenos Aires Argentina

Genetics Department Hospital Universitario Dr José E González Universidad Autonóma de Nuevo León San Nicolás de los Garza Mexico

Health Services Management Research Centre Tabriz University of Medical Sciences Tabriz Iran

Human Genetics Institute Pontificia Universidad Javeriana Bogotá Colombia

Institute of Clinical Physiology National Research Council and Fondazione Toscana Gabriele Monasterio Tuscany Registry of Congenital Defects Pisa Italy

Instituto Académico Pedagógico de Ciencias Humanas Universidad Nacional de Villa María Córdoba Argentina

International Center on Birth Defects International Clearinghouse for Birth Defects Surveillance and Research Rome Italy

Lombardy Congenital Anomalies Registry Cancer Registry Unit Fondazione IRCCS Istituto Nazionale Tumori Milan Italy

Malta Congenital Anomalies Registry Directorate for Health Information and Research Tal Pietà Malta

Medical Faculty Malformation Monitoring Centre Saxony Anhalt Otto von Guericke University Magdeburg Germany

Metro Atlanta Congenital Defects Program Division of Birth Defects and Infant Disorders National Center on Birth Defects and Developmental Disabilities CDC Atlanta Georgia USA

National Board of Health and Welfare Stockholm Sweden

National Network of Congenital Anomalies of Argentina National Ministry of Health and Social Development Buenos Aires Argentina

Omni Net for Children International Charitable Fund Rivne Ukraine

Regional Register Congenital Malformation Maule Health Service Maule Chile

Slovak Teratologic Information Centre Slovak Medical University Bratislava Slovak Republic

Université de Paris CRESS Obstetrical Perinatal and Pediatric Epidemiology Research Team INSERM INRA Paris France

Utah Birth Defect Network Bureau of Children with Special Health Care Needs Division of Family Health and Preparedness Utah Department of Health Salt Lake City Utah USA

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