Table 2.
Mortality rates and Kaplan-Meier Product-Limit estimates with 95% confidence intervals by age at death and registry type for infants born with omphalocele in 18 countries from 23 birth defects surveillance systems, 2000-2012
| Kaplan Meier Mortality Estimates for Age at Death3 | |||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|
| Country - registry | Surveillance period |
Number Livebirths |
< 1 day | 1-6 days | 7-27 days | 0-27 days | 28 - 364 days |
1-year | 1 - 4 years |
≥5 years | 5-year (95% CI)3 |
| Hospital-based registries | |||||||||||
| Argentina – RENAC1 | 2009-2012 | 97 | 43.3% | - | 43.3% | - | 43.3% | - | - | 43.3% (33.4, 53.2) | |
| Colombia – Bogotá1 | 2000-2012 | 50 | 20.0% | 0.0% | - | 20.0% | - | 20.0% | - | - | 20.0% (8.9, 31.1) |
| Colombia – Cali1 | 2011-2012 | 3 | 0.0% | 0.0% | - | 0.0% | - | 0.0% | - | - | 0.0% (0.0, 0.0) |
| South America – ECLAMC1 | 2000-2012 | 639 | 23.2% | 8.0% | 9.2%5 | 40.4% | 1.1%5 | 41.5% | - | - | 41.5% (37.7, 45.3) |
| Spain - ECEMC | 2000-2012 | 14 | 0.0% | 7.1% | - | 7.1% | - | 7.1% | - | - | 7.1% (0.0, 20.6)4 |
| Mexico – RYVEMCE1 | 2000-2012 | 49 | 18.4% | 4.1% | - | 22.5% | - | 22.5% | - | - | 22.5% (10.8, 34.1) |
| Iran - TROCA | 2004-2012 | 33 | 0.0% | 0.0% | - | 0.0% | - | 0.0% | - | - | 0.0% (0.0, 0.0) |
| Israel – SMC2 | 2000-2012 | 15 | 0.0% | 0.0% | 0.0% | 0.0% | 0.0% | 0.0% | 0.0% | 0.0% | 0.0% (0.0, 0.0) |
| Total | 2000-2012 | 900 | 18.9% | 11.5% | 9.3% | 39.7% | 1.1% | 40.8% | 0.0% | 0.0% | 40.8% (37.4, 44.3) |
| Population-based registries | |||||||||||
| Czech Republic | 2000-2012 | 168 | 2.4% | 3.0% | 3.0% | 8.3% | 4.8% | 13.1% | 1.8% | 1.2% | 16.1% (10.5, 21.6) |
| France - Paris | 2000-2012 | 51 | 5.9% | 2.0% | 0.0% | 7.8% | - | 7.8% | - | - | 7.8% (0.5, 15.2) |
| Germany - Saxony Anhalt | 2000-2012 | 22 | 0.0% | 0.0% | 4.5% | 4.5% | 0.0% | 4.5% | - | - | 4.5% (0.0, 13.2)4 |
| Italy - Lombardy | 2003-2012 | 10 | 0.0% | 0.0% | 0.0% | 0.0% | 10.0% | 10.0% | 0.0% | 0.0% | 10.0% (0.0, 28.6)4 |
| Italy - Tuscany | 2000-2012 | 24 | 8.3% | 8.3% | 8.3% | 25.0% | 0.0% | 25.0% | 0.0% | 0.0% | 25.0% (7.7, 42.3) |
| Malta – MCAR1 | 2000-2012 | 12 | 16.7% | 0.0% | 16.6% | 33.3% | 16.7% | 50.0% | - | - | 50.0% (37.3, 62.7) |
| Netherlands - North | 2000-2012 | 29 | 3.4% | 13.8% | 6.9% | 24.2% | 3.4% | 27.6% | - | - | 27.6% (11.3, 43.9) |
| Slovak Republic | 2001-2012 | 51 | 0.0% | 13.7% | 2.0% | 15.7% | - | 15.7% | - | - | 15.7% (5.7, 25.7) |
| Sweden | 2000-2012 | 149 | 1.3% | 6.0% | 2.0% | 9.3% | 7.4% | 16.7% | 0.7% | 0.0% | 17.4% (11.4, 23.5) |
| UK - Wales | 2000-2012 | 72 | 5.6% | 12.5% | 0.0% | 18.1% | 9.7% | 27.8% | 1.4% | 0.0% | 29.2% (18.7, 39.7) |
| Ukraine - OMNI-Net | 2000-2012 | 48 | 6.3% | 6.2% | 6.2% | 18.7% | 2.1% | 20.8% | - | - | 20.8% (9.3, 32.3) |
| USA - Arkansas | 2000–2012 | 100 | 17.0% | 12.0% | 0.0% | 29.0% | 9.0% | 38.0% | 3.0% | 0.0% | 41.0% (31.4, 50.6) |
| USA - Atlanta | 2000-2008 | 71 | 9.9% | 11.3% | 4.2% | 25.4% | 4.2% | 29.6% | 1.4% | 0.0% | 31.0% (20.2, 41.7) |
| USA - Texas | 2000-2012 | 782 | 11.0% | 7.9% | 4.5% | 23.4% | 6.4% | 29.8% | 1.2% | 0.1% | 31.1% (27.8, 34.3) |
| USA - Utah | 2000-2012 | 126 | 15.9% | 5.6% | 4.0% | 25.4% | 6.3% | 31.7% | 1.6% | - | 33.3% (25.1, 41.6) |
| Total | 2000-2012 | 1,715 | 8.7% | 7.5% | 3.6% | 19.9% | 6.4% | 26.3% | 1.3% | 0.3% | 27.8% (25.7, 30.0) |
| All registries | 2000-2012 | 2,615 | 12.2% | 8.7% | 5.0% | 25.9% | 4.8% | 30.7% | 1.2% | 0.2% | 32.1% (30.2, 34.0) |
| ETOPFA not allowed or registered versus ETOPFA allowed | |||||||||||
| Not allowed/registered | 2000-2012 | 865 | 19.8% | 12.0% | 9.3% | 41.1% | 1.3% | 42.4% | 0.0% | 0.0% | 42.4% (38.9, 45.9) |
| Allowed | 2000-2012 | 1,750 | 8.5% | 7.4% | 3.6% | 19.5% | 6.3% | 25.8% | 1.3% | 0.2% | 27.3% (25.2, 29.5) |
ETOPFA (elective termination of pregnancy for fetal anomalies) not allowed during (part of) surveillance period
data on live born children with congenital omphalocele from one hospital
cumulative percent mortality was calculated using a modified Kaplan-Meier Product-Limit method to account for censoring and differential length of follow-up
Lower limit confidence intervals fitted to zero
incomplete follow-up, but deaths reported
ECEMC= Spanish Collaborative Study of Congenital Malformations; ECLAMC=Latin American Collaborative Study of Congenital Malformations; GP=General Practitioner; MCAR=Malta Congenital Anomalies Registry; OMNI-Net=Ukraine Birth Defects Prevention Program; RENAC=National Network of Congenital Anomalies of Argentina; RYVEMCE= Mexican Registry and Epidemiological Surveillance of Congenital Malformations; TROCA=Tabriz Registry of Congenital Anomalies; SMC=Soroka Medical Center; UK=United Kingdom; USA=United States of America