Survival and causes of death in adults with spina bifida in Sweden: a population-based case-control study

Authors

  • Melinda Rocchi Department of Clinical Sciences Lund, Orthopedics, Lund University, Lund, Sweden
  • Johan Jarl Health Economics, Department of Clinical Sciences Malmö, Lund University, Lund, Sweden
  • Annika Lundkvist Josenby Department of Health Sciences, Lund University, Lund, Sweden; Children’s Hospital, Skåne University Hospital, Lund, Sweden
  • Ann I. Alriksson-Schmidt Department of Clinical Sciences Lund, Orthopedics, Lund University, Lund, Sweden

DOI:

https://doi.org/10.2340/jrm.v55.18244

Keywords:

spina bifida, adults, transition to adult care, mortality, survival

Abstract

Objective: To analyse survival rates and causes of death in adults with spina bifida in Sweden compared with a matched control group.

Design and methods: This population-based study included 11,900 adults born between 1950 and 1997. Three national Swedish registers were used to identify individuals with a diagnosis of spina bifida and a matched control group without spina bifida in the period 1990–2015. International Classification of Diseases codes were used to identify causes of death. Survival analysis was conducted and causes of death in the 2 groups were compared.

Results: There was a lower probability of survival for people with spina bifida in all age groups (p < 0.001) compared with the control group. The most prevalent causes of death in people with spina bifida were congenital, respiratory, nervous, cardiovascular, genitourinary, and injuries. People with spina bifida had a higher probability of dying from congenital (p < 0.001), respiratory (p = 0.002), genitourinary (p < 0.002), and nervous-related (p < 0.001) and lower probability of injury-related deaths (p < 0.001).

Conclusion: Adults with spina bifida in Sweden have a lower survival rate compared with the general population, with the frequency of certain causes of death differing between the two groups. In order to reduce excess premature mortality, prevention and careful management of potentially fatal conditions are essential throughout a patient’s lifespan.

Downloads

Download data is not yet available.

Author Biographies

Melinda Rocchi, Department of Clinical Sciences Lund, Orthopedics, Lund University, Lund, Sweden

Department of Clinical Sciences Lund

Johan Jarl, Health Economics, Department of Clinical Sciences Malmö, Lund University, Lund, Sweden

Health Economics, Department of Clinical Sciences Malmö

References

Mitchell LE, Adzick NS, Melchionne J, Pasquariello PS, Sutton LN, White-head AS. Spina bifida. Lancet 2004; 364: 1885-1895.

https://doi.org/10.1016/S0140-6736(04)17445-X DOI: https://doi.org/10.1016/S0140-6736(04)17445-X

McComb JG. A practical clinical classification of spinal neural tube de-fects. Childs Nerv Syst 2015; 31: 1641-1657.

https://doi.org/10.1007/s00381-015-2845-9 DOI: https://doi.org/10.1007/s00381-015-2845-9

Hydrocephalus and Shunts - Spina Bifida Association. (n.d.). [accessed May 21, 2021]. Available from: https://www.spinabifidaassociation.org/resource/hydrocephalus-and-shunts/

Inversetti A, Van der Veeken L, Thompson D, Jansen K, Van Calenbergh F, Joyeux L, et al. Neurodevelopmental outcome of children with spi-na bifida aperta repaired prenatally vs postnatally: systematic review and meta-analysis. Ultrasound Obstet Gynecol 2019; 53: 293-301.

https://doi.org/10.1002/uog.20188 DOI: https://doi.org/10.1002/uog.20188

Oakeshott P, Hunt G, Reid F. Expectation of life and unexpected death in open spina bifida: a 40-year complete, non-selective, longitudinal cohort study. Dev Med Child Neurol 2009; 52: 749-53.

https://doi.org/10.1111/j.1469-8749.2009.03543.x DOI: https://doi.org/10.1111/j.1469-8749.2009.03543.x

Liptak GS, Robinson LM, Davidson PW, Dziorny A, Lavalley R, Flaherty MG, et al. Life course health and healthcare utilization among adults with spina bifida. Dev Med Child Neurol 2016; 58(7): 714-720.

https://doi.org/10.1111/dmcn.12952 DOI: https://doi.org/10.1111/dmcn.12952

Dicianno BE, Wilson R. Hospitalizations of adults with spina bifida and congenital spinal cord anomalies. Arch Phys Med Rehabil 2010 Apr; 91: 529-535.

https://doi.org/10.1016/j.apmr.2009.11.023 DOI: https://doi.org/10.1016/j.apmr.2009.11.023

Simeonsson RJ, McMillen JS, Huntington GS. Secondary conditions in children with disabilities: spina bifida as a case example. Ment Retard Dev Disabil Res Rev 2002; 8: 198-205.

https://doi.org/10.1002/mrdd.10038 DOI: https://doi.org/10.1002/mrdd.10038

Kaufman BA, Terbrock A, Winters N, Ito J, Klosterman A, Park TS. Dis-banding a multidisciplinary clinic: effects on the health care of mye-lomeningocele patients. Pediatr Neurosurg 1994; 21: 36-44.

https://doi.org/10.1159/000120812 DOI: https://doi.org/10.1159/000120812

McDonnell GV, McCann JP. Why do adults with spina bifida and hydro-cephalus die? A clinic-based study. Eur J Pediatr Surg 2000; 10 Suppl 1: 31-32.

https://doi.org/10.1055/s-2008-1072411 DOI: https://doi.org/10.1055/s-2008-1072411

Singhal B, Mathew KM. Factors affecting mortality and morbidity in adult spina bifida. Eur J Pediatr Surg 1999; 9 Suppl 1: 31-32.

https://doi.org/10.1055/s-2008-1072310 DOI: https://doi.org/10.1055/s-2008-1072310

Peyronnet B, Gao F, Brochard C, Oger E, Scailteux LM, Balusson F, et al. Urologic disorders are still the leading cause of in-hospital death in patients with spina bifida. Urology 2020; 137: 200-204.

https://doi.org/10.1016/j.urology.2019.11.006 DOI: https://doi.org/10.1016/j.urology.2019.11.006

Dicianno BE, Sherman A, Roehmer C, Zigler CK. Co-morbidities associat-ed with early mortality in adults with spina bifida. Am J Phys Med Re-habil 2018; 97: 861-865.

https://doi.org/10.1097/PHM.0000000000000964 DOI: https://doi.org/10.1097/PHM.0000000000000964

Cai B, McDermott S, Wang Y, Royer JA, Mann JR, Hardin JW, et al. Skin ulcers and mortality among adolescents and young adults with spina bifida in South Carolina during 2000-2010. J Child Neurol 2016; 31: 370-377.

https://doi.org/10.1177/0883073815596611 DOI: https://doi.org/10.1177/0883073815596611

Kancherla V, Druschel CM, Oakley GP Jr. Population-based study to de-termine mortality in spina bifida: New York State Congenital Malfor-mations Registry, 1983 to 2006. Birth Defects Res A Clin Mol Teratol 2014; 100: 563-575.

https://doi.org/10.1002/bdra.23259 DOI: https://doi.org/10.1002/bdra.23259

Davis BE, Daley CM, Shurtleff DB, Duguay S, Seidel K, Loeser JD, et al. Long-term survival of individuals with myelomeningocele. Pediatr Neurosurg 2005; 41: 186-191.

https://doi.org/10.1159/000086559 DOI: https://doi.org/10.1159/000086559

Hollung SJ, Vik T, Wiik R, Bakken IJ, Andersen GL. Completeness and correctness of cerebral palsy diagnoses in two health registers: im-plications for estimating prevalence. Dev Med Child Neurol 2017; 59: 402-406.

https://doi.org/10.1111/dmcn.13341 DOI: https://doi.org/10.1111/dmcn.13341

Brooke HL, Talbäck M, Hörnblad J, Johansson LA, Ludvigsson JF, Druid H, et al The Swedish cause of death register. Eur J Epidemiol 2017; 32: 765-773.

https://doi.org/10.1007/s10654-017-0316-1 DOI: https://doi.org/10.1007/s10654-017-0316-1

European Shortlist for Causes of Death. (n.d.). Joinup. [Date accessed: 20/05/2021] Available from: https://joinup.ec.europa.eu/collection/eu-semantic-interoperability-catalogue/solution/european-shortlist-causes-death

Published

2023-11-27

How to Cite

Rocchi, M., Jarl, J., Lundkvist Josenby, A. ., & Alriksson-Schmidt, A. I. (2023). Survival and causes of death in adults with spina bifida in Sweden: a population-based case-control study. Journal of Rehabilitation Medicine, 55, jrm18244. https://doi.org/10.2340/jrm.v55.18244

Issue

Section

Articles

Categories