Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Meta-Analysis
. 2022 Jul 15;114(12):631-644.
doi: 10.1002/bdr2.2045. Epub 2022 May 28.

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

Juan Antonio Gili  1   2 , Jorge Santiago López-Camelo  1 , Wendy N Nembhard  3 , Marian Bakker  4 , Hermien E K de Walle  4 , Erin B Stallings  5 , Vijaya Kancherla  6 ; ECEMC Peripheral Group  7 ; Paolo Contiero  8 , Saeed Dastgiri  9 , Marcia L Feldkamp  10 , Amy Nance  11 , Miriam Gatt  12 , Laura Martínez  13 , María Aurora Canessa  14 , Boris Groisman  15 , Paula Hurtado-Villa  16 , Karin Källén  17 , Danielle Landau  18 , Nathalie Lelong  19 , Margery Morgan  20 , Jazmín Arteaga-Vázquez  21 , Anna Pierini  22 , Anke Rissmann  23 , Antonin Sipek  24 , Elena Szabova  25 , Wladimir Wertelecki  26 , Ignacio Zarante  27 , Mark A Canfield  28 , Pierpaolo Mastroiacovo  29
Affiliations
Meta-Analysis

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

Juan Antonio Gili et al. Birth Defects Res. .

Abstract

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.

Keywords: ETOPFA; birth defects; case fatality rate; congenital hydrocephalus; early neonatal deaths; population surveillance; prevalence; trends.

PubMed Disclaimer

Conflict of interest statement

CONFLICT OF INTERESTS

The authors declare that they have no competing interests in publishing this manuscript. The authors received no funding for this analysis.

Figures

FIGURE 1
FIGURE 1
Forest plot of congenital hydrocephalus case fatality rate by (a) elective termination of pregnancy for fetal anomalies (ETOPFA), (b) region and (c) registry type, International Clearinghouse for Birth Defects Surveillance and Research (ICBDSR), 2000–2014. ES (95% CI), case fatality rate per 100 liveborn cases. ETOPFA NO, elective termination of pregnancy for fetal anomaly policy not allowed in the country where the registry is located. ETOPFA YES, elective termination of pregnancy for fetal anomaly policy allowed in the country where the registry is located
FIGURE 2
FIGURE 2
Forest plot of congenital hydrocephalus birth prevalence rate per 10,000 births by (a) elective termination of pregnancy for fetal anomalies (ETOPFA), (b) region and (c) registry type, International Clearinghouse for Birth Defects Surveillance and Research (ICBDSR), 2000–2014. ES (95% CI), case fatality rate per 100 liveborn cases. ETOPFA NO, elective termination of pregnancy for fetal anomaly policy not allowed in the country where the registry is located. ETOPFA YES, elective termination of pregnancy for fetal anomaly policy allowed in the country where the registry is located
See this image and copyright information in PMC

References

    1. Alijahan R, Mirzarahimi M, Ahmadi Hadi P, & Hazrati S (2013). Prevalence of congenital abnormalities and its related risk factors in Ardabil, Iran, 2011. The Iranian Journal of Obstetrics, Gynecology and Infertility, 16(54), 16–25. 10.22038/ijogi.2013.1086 - DOI
    1. Bakker MK, Kancherla V, Canfield MA, Bermejo-Sanchez E, Cragan JD, Dastgiri S, … Mastroiacovo P (2019). Analysis of mortality among neonates and children with spina bifida: An international registry-based study, 2001–2012. Paediatric and Perinatal Epidemiology, 33(6), 436–448. 10.1111/ppe.12589 - DOI - PMC - PubMed
    1. Best KE, Rankin J, Dolk H, Loane M, Haeusler M, Nelen V, … Khoshnood B (2020). Multilevel analyses of related public health indicators: The European Surveillance of Congenital Anomalies (EUROCAT) Public Health Indicators. Paediatric and Perinatal Epidemiology, 34(2), 122–129. 10.1111/ppe.12655 - DOI - PMC - PubMed
    1. Bradburn M, Deeks J, & Altman DG (1998). metan—An alternative meta-analysis command. Stata Techicall Bulletin, 44, 4–15.
    1. Daliri S, Safarpour H, Bazyar J, Sayehmiri K, Karimi A, & Anvary R (2019). The relationship between some neonatal and maternal factors during pregnancy with the prevalence of congenital malformations in Iran: A systematic review and meta-analysis. The Journal of Maternal-Fetal & Neonatal Medicine: The Official Journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians, 32(21), 3666–3674. 10.1080/14767058.2018.1465917 - DOI - PubMed

Publication types