Risk factors of sudden death in young adult patients with myelomeningocele: Clinical article

Sarah C. Jernigan, Jay G. Berry, Dionne A. Graham, Stuart B. Bauer, Lawrence I. Karlin, Nedd A.M. Hobbs, R. Michael Scott, Benjamin C. Warf

Research output: Contribution to journalArticlepeer-review

14 Scopus citations


Object. Although survival for patients with myelomeningocele has dramatically improved in recent decades, the occasional occurrence of sudden, unexplained death in young adult patients with myelomeningocele has been noted by the authors. This study was undertaken to determine risk factors for sudden death in this population. Methods. The authors performed a retrospective chart review of patients born between 1978 and 1990 who received care at Children's Hospital Boston. The relationship between sudden death and patient demographics, presence of CSF shunt and history of shunt revisions, midbrain length as a marker for severity of hindbrain malformation, seizures, pulmonary and ventilatory dysfunction, body mass index, scoliosis, renal dysfunction, and cardiac disease was evaluated using the t-test, Fisher exact test, and logistic regression analysis. Results. The age range for 106 patients in the study cohort was 19-30 years, with 58 (54.7%) women and 48 (45.3%) men. Six patients, all of whom were young women, experienced sudden death. In multivariate analysis, female sex, sleep apnea, and midbrain elongation ≥ 15 mm on MR imaging remained significantly associated with a higher risk of sudden death. These risk factors were cumulative, and female patients with sleep apnea and midbrain length ≥ 15 mm had the greatest risk (adjusted risk ratio 24.0, 95% CI 7.3-79.0; p < 0.05). No other comorbidities were found to significantly increase the risk of sudden death. Conclusions. Young adult women with myelomeningocele are at significantly increased risk of sudden death in the setting of midbrain elongation and sleep apnea. Further investigation is needed to determine the benefit of routine screening to identify at-risk patients for closer cardiopulmonary monitoring and treatment.

Original languageEnglish (US)
Pages (from-to)149-155
Number of pages7
JournalJournal of Neurosurgery: Pediatrics
Issue number2
StatePublished - Feb 2012


  • Chiari malformation
  • Myelomeningocele
  • Risk factor
  • Sleep apnea
  • Spina bifida
  • Spine
  • Sudden death

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology


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