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

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.

Racial and ethnic differences in infant survival for hydrocephaly—Texas, 1999–2017

“…In this study, unadjusted results showed infants with chromosomal or syndromic hydrocephaly had approximately five‐times higher hazard of death, compared with infants born with isolated hydrocephaly. This finding is consistent with those from an international study concerning early neonatal hydrocephaly mortality that reported case fatality rates approximately 2.7 times higher among infants with syndromic versus nonsyndromic hydrocephaly (Gili et al, 2022). A previous Texas study also reported 88%, 63%, and 25% survival for infants with hydrocephaly with zero, one, and two co‐occurring “life‐threatening” defects, respectively, but did not focus on chromosomal or syndromic defects specifically (Nembhard et al, 2001).…”

“…Therefore, this study focused on congenital hydrocephaly without spina bifida (hereafter referred to as hydrocephaly, except where otherwise indicated). If left untreated, hydrocephaly leads to chronic morbidity and can be fatal (Gili et al, 2022; Isaacs et al, 2018). Prevalence of hydrocephaly differs worldwide (Isaacs et al, 2018).…”

“…The study showed an overall 25‐year survival of 83% among infants with hydrocephaly (Wang et al, 2011). Studies also report that infant survival is influenced by maternal and infant‐associated risk factors, such as maternal race and ethnicity, preterm birth, low birth weight, and maternal age (Ely & Driscoll, 2019; Gili et al, 2022; Ratnasiri et al, 2020; Shi et al, 2004), including improvements in care of preterm infants in more recent years (Glinianaia et al, 2020). However, a fuller understanding of mortality risk factors is needed using current information on survival estimates.…”

“…Prevalence of hydrocephaly differs worldwide (Isaacs et al, 2018). Total birth prevalence of hydrocephaly in North America is estimated to be approximately 5–6 cases/10,000 live births (Gili et al, 2022; Isaacs et al, 2018). In Texas, prevalence of hydrocephaly appears to be higher than national levels and is estimated to be 8 cases/10,000 live births (Texas Birth Defects Registry, n.d.).…”

“…A limited number of published population‐based studies are available regarding hydrocephaly mortality or survival. In a recent international study, 6% of neonates with hydrocephaly died within 7 days, and infants with syndromic hydrocephaly had approximately a two times higher risk for death (Gili et al, 2022). However, the study did not assess mortality beyond 7 days.…”