Survival of infants and children born with severe microcephaly, Texas, 1999-2015.
UCD
congenital anomalies
microcephalus
microcephaly
survival
trisomy
underlying cause of death
Journal
Birth defects research
ISSN: 2472-1727
Titre abrégé: Birth Defects Res
Pays: United States
ID NLM: 101701004
Informations de publication
Date de publication:
01 Jan 2023
01 Jan 2023
Historique:
revised:
08
09
2022
received:
24
06
2022
accepted:
21
09
2022
pubmed:
9
11
2022
medline:
25
1
2023
entrez:
8
11
2022
Statut:
ppublish
Résumé
Severe microcephaly is a brain reduction defect where the delivery head circumference is <3rd percentile for gestational age and sex with subsequent lifelong morbidities. Our objective was to evaluate survival among 2,704 Texas infants with severe microcephaly delivered 1999-2015. Infants with severe microcephaly from the Texas Birth Defects Registry were linked to death certificates and the national death index. Survival estimates, hazard ratios (HR) and confidence intervals (CI) were calculated using the Kaplan-Meier method and Cox proportional hazards models stratified by presence versus absence of co-occurring defects. We identified 496 deaths by age 4 years; most (42.9%) occurred in the neonatal period, and another 39.9% died by 1 year of age. Overall infant survival was 84.8%. Lowest infant survival subgroups included those with chromosomal/syndromic conditions (66.1%), very preterm deliveries (63.9%), or co-occurring critical congenital heart defects (44.0%). Among infants with severe microcephaly and a chromosomal/syndromic co-occurring defect, the risk of death was nearly three-fold higher among those with: proportionate microcephaly (i.e., small baby overall), relative to non-proportionate (HR = 2.84, 95% CI = 2.17-3.71); low-birthweight relative to normal (HR = 2.72, 95% CI = 1.92-3.85); critical congenital heart defects (CCHD) relative to no CCHD (HR = 2.90, 95% CI = 2.20-3.80). Trisomies were a leading underlying cause of death (27.5%). Overall, infants with severe microcephaly had high 4-year survival rates which varied by the presence of co-occurring defects. Infants with co-occurring chromosomal/syndromic anomalies have a higher risk of death by age one than those without any co-occurring birth defects.
Sections du résumé
BACKGROUND
BACKGROUND
Severe microcephaly is a brain reduction defect where the delivery head circumference is <3rd percentile for gestational age and sex with subsequent lifelong morbidities. Our objective was to evaluate survival among 2,704 Texas infants with severe microcephaly delivered 1999-2015.
METHODS
METHODS
Infants with severe microcephaly from the Texas Birth Defects Registry were linked to death certificates and the national death index. Survival estimates, hazard ratios (HR) and confidence intervals (CI) were calculated using the Kaplan-Meier method and Cox proportional hazards models stratified by presence versus absence of co-occurring defects.
RESULTS
RESULTS
We identified 496 deaths by age 4 years; most (42.9%) occurred in the neonatal period, and another 39.9% died by 1 year of age. Overall infant survival was 84.8%. Lowest infant survival subgroups included those with chromosomal/syndromic conditions (66.1%), very preterm deliveries (63.9%), or co-occurring critical congenital heart defects (44.0%). Among infants with severe microcephaly and a chromosomal/syndromic co-occurring defect, the risk of death was nearly three-fold higher among those with: proportionate microcephaly (i.e., small baby overall), relative to non-proportionate (HR = 2.84, 95% CI = 2.17-3.71); low-birthweight relative to normal (HR = 2.72, 95% CI = 1.92-3.85); critical congenital heart defects (CCHD) relative to no CCHD (HR = 2.90, 95% CI = 2.20-3.80). Trisomies were a leading underlying cause of death (27.5%).
CONCLUSIONS
CONCLUSIONS
Overall, infants with severe microcephaly had high 4-year survival rates which varied by the presence of co-occurring defects. Infants with co-occurring chromosomal/syndromic anomalies have a higher risk of death by age one than those without any co-occurring birth defects.
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
26-42Subventions
Organisme : Maternal and Child Health block grants title V
Informations de copyright
© 2022 Wiley Periodicals LLC.
Références
Ashwal, S., Michelson, D., Plawner, L., Dobyns, W. B., & Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. (2009). Practice parameter: Evaluation of the child with microcephaly (an evidence-based review): Report of the quality standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology, 73(11), 887-897. https://doi.org/10.1212/WNL.0b013e3181b783f7
Auger, N., Quach, C., Healy-Profitós, J., Lowe, A. M., & Arbour, L. (2018). Congenital microcephaly in Quebec: Baseline prevalence, risk factors and outcomes in a large cohort of neonates. Archives of Disease in Childhood. Fetal and Neonatal Edition, 103(2), F167-F172. https://doi.org/10.1136/archdischild-2016-311199
Benjamin, R. H., Salemi, J. L., Canfield, M. A., Nembhard, W. N., Ganduglia Cazaban, C., Tsao, K., … Agopian, A. J. (2021). Causes of neonatal and postneonatal death among infants with birth defects in Texas. Birth Defects Research, 113(9), 665-675. https://doi.org/10.1002/bdr2.1879
Centers for Disease Control and Prevention, & National Center for Health Statistics. (2020). ICD-10 Cause-of-Death Lists for Tabulating Mortality Statistics (Updated September 2020 to include WHO updates to ICD-10 for data year 2019). Instruction Manual Part 9. https://www.cdc.gov/nchs/data/dvs/Part9InstructionManual2019-508.pdf
Cragan, J. D., Isenburg, J. L., Parker, S. E., Alverson, C. J., Meyer, R. E., Stallings, E. B., … National Birth Defects Prevention Network. (2016). Population-based microcephaly surveillance in the United States, 2009 to 2013: An analysis of potential sources of variation. Birth Defects Research. Part A, Clinical and Molecular Teratology, 106(11), 972-982. https://doi.org/10.1002/bdra.23587
Falcheck, S. (2019). Microcephaly. In Merck manual consumer version. Merck & Co., Inc. Rahway, NJ, USA. Retrieved October 29, 2021, from. https://www.merckmanuals.com/home/children-s-health-issues/birth-defects-of-the-brain-and-spinal-cord/microcephaly
Glinianaia, S. V., Rankin, J., Pierini, A., Coi, A., Santoro, M., Tan, J., … Morris, J. K. (2022). Ten-year survival of children with congenital anomalies: A European cohort study. Pediatrics, e2021053793. https://doi.org/10.1542/peds.2021-053793
Hoyt, A. T., Canfield, M. A., Langlois, P. H., Waller, D. K., Agopian, A. J., Shumate, C. J., … Scheuerle, A. E. (2018). Pre-Zika descriptive epidemiology of microcephaly in Texas, 2008-2012. Birth Defects Research, 110(5), 395-405. https://doi.org/10.1002/bdr2.1164
Leviton, A., Holmes, L. B., Allred, E. N., & Vargas, J. (2002). Methodologic issues in epidemiologic studies of congenital microcephaly. Early Human Development, 69(1-2), 91-105. https://doi.org/10.1016/s0378-3782(02)00065-8
McElrath, T. F., Allred, E. N., Kuban, K., Hecht, J. L., Onderdonk, A., O'Shea, T. M., … ELGAN Study Investigators. (2010). Factors associated with small head circumference at birth among infants born before the 28th week. American Journal of Obstetrics and Gynecology, 203(2), 138.e1-138.e1388. https://doi.org/10.1016/j.ajog.2010.05.006
National Association for Public Health Statistics and Information Systems (NAPHSIS). (2021). State and Territorial Exchange of Vital Events (STEVE). Retrieved from https://www.naphsis.org/steve
National Birth Defects Prevention Network (NBDPN) (2016). NBDPN Abstractor's Instructions Final Draft of February 28, 2016, Appendix 3.1, on Congenital Microcephaly. Retrieved from https://www.nbdpn.org/docs/NBDPN_Case_Definition_-_Surveillance_Microcephaly_2016Feb28_Final_DRAFT.pdf
National Birth Defects Prevention Network (NBDPN). (2017). Appendix 3.1, Birth Defects Descriptions for NBDPN Core, Recommended, and Extended Conditions, Updated March 2017. Botto, L., Carey, J., Cassell, C., Colarusso, T., Cragan, J., Feldkamp, M., Frias, J., Lin, A., Mai, C., Olney, R., Stanton, C., Siffel, C https://www.nbdpn.org/docs/Appendix_3_1_BirthDefectsDescriptions_2017MAR24.pdf
National Center on Birth Defects and Developmental Disabilities (NCBDDD). Centers for Disease Control and Prevention. (2020). Facts about Microcephaly. Retrieved from https://www.cdc.gov/ncbddd/birthdefects/microcephaly.html
Nembhard, W. N., Waller, D. K., Sever, L. E., & Canfield, M. A. (2001). Patterns of first-year survival among infants with selected congenital anomalies in Texas, 1995-1997. Teratology, 64(5), 267-275. https://doi.org/10.1002/tera.1073
Nembhard, W. N., Salemi, J. L., Ethen, M. K., Fixler, D. E., & Canfield, M. A. (2010). Mortality among infants with birth defects: Joint effects of size at birth, gestational age, and maternal race/ethnicity. Birth Defects Research. Part A, Clinical and Molecular Teratology, 88(9), 728-736. https://doi.org/10.1002/bdra.20696
Sattolo, M. L., Arbour, L., Bilodeau-Bertrand, M., Lee, G. E., Nelson, C., & Auger, N. (2022). Association of Birth Defects with Child Mortality before age 14 years. JAMA Network Open, 5(4), e226739. https://doi.org/10.1001/jamanetworkopen.2022.6739
Qin, C., Hsia, J., & Berg, C. J. (2008). Variation between last-menstrual-period and clinical estimates of gestational age in vital records. American Journal of Epidemiology, 167(6), 646-652. https://doi.org/10.1093/aje/kwm345
von der Hagen, M., Pivarcsi, M., Liebe, J., von Bernuth, H., Didonato, N., Hennermann, J. B., … Kaindl, A. M. (2014). Diagnostic approach to microcephaly in childhood: A two-center study and review of the literature. Developmental Medicine and Child Neurology, 56(8), 732-741. https://doi.org/10.1111/dmcn.12425
World Health Organization. International Fetal and Newborn Growth Consortium for the 21st Century, Child Growth Standards for birth to 5 years. https://www.who.int/tools/child-growth-standards/standards/head-circumference-for-age. 2021. World Health Organization; ©Copyright 2009-2021, INTERGROWTH-21st. License: CC BY-NC-SA 3.0 IGO.