TABLE 3.
Case fatality rates and birth prevalence rates for congenital hydrocephalus by registry characteristics, International Clearinghouse for Birth Defects Surveillance and Research (ICBDSR), 2000–2014
| Registry characteristic | Case fatality ratea | Total birth prevalence rateb | ||
|---|---|---|---|---|
| Rate per 100 liveborn cases | (95% CI) | Rate/10,000 births | (95% CI) | |
| ETOPFA policyc | ||||
| No | 8.52 | (7.63–9.49) | 9.49 | (9.21–9.78) |
| Yes | 4.22 | (3.73–4.76) | 6.00 | (5.87–6.14) |
| Region | ||||
| South America | 8.74 | (7.81–9.76) | 10.54 | (10.22–10.87) |
| Europe | 5.07 | (4.12–6.17) | 5.04 | (4.89–5.21) |
| Asia | 8.19 | (5.25–12.19) | 12.45 | (11.43–13.54) |
| North America | 3.61 | (3.07–4.23) | 6.39 | (6.21–6.58) |
| System | ||||
| Hospital based | 8.08 | (7.26–8.96) | 8.50 | (8.28–8.73) |
| Population based | 4.14 | (3.63–4.70) | 6.00 | (5.87–6.12) |
| ICBDSR 2000–2014 | 5.86 | (5.39–6.35) | 6.82 | (6.70–6.93) |
Abbreviations: CI, confidence interval using Poisson distribution; ETOPFA, elective termination of pregnancy for fetal anomalies; ICBDSR, International Clearinghouse for Birth Defects Surveillance and Research.
Congenital hydrocephalus case fatality rate per 100 liveborn cases (CFR) was calculated as the total number of liveborn congenital hydrocephalus cases who died before postnatal day 7 (early neonatal death) divided by the total number of live births with congenital hydrocephalus.
Birth prevalence rate per 10,000 births (BPR/10,000) was calculated as the total number of CH cases (live births + stillbirths + ETOPFA for congenital hydrocephalus when allowed) divided by the total number of births (live births + stillbirths) in a specified period.
ETOPFA policy, elective termination of pregnancy for fetal anomalies allowed in the country where the registry is located.