title: Risk factors of sudden death in young adult patients with myelomeningocele.
abstract: OBJECTIVE
  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.
authors.affiliation: Department of Neurosurgery, Children’s Hospital, Boston, Massachusetts 02115, USA.
authors.forename: Sarah C
authors.initials: SC
authors.lastname: Jernigan
authors.forename: Jay G
authors.initials: JG
authors.lastname: Berry
authors.forename: Dionne A
authors.initials: DA
authors.lastname: Graham
authors.forename: Stuart B
authors.initials: SB
authors.lastname: Bauer
authors.forename: Lawrence I
authors.initials: LI
authors.lastname: Karlin
authors.forename: Nedda M
authors.initials: NM
authors.lastname: Hobbs
authors.forename: R Michael
authors.initials: RM
authors.lastname: Scott
authors.forename: Benjamin C
authors.initials: BC
authors.lastname: Warf
mesh_terms: D000328:Adult; D000367:Age Factors; D000704:Analysis of Variance; D015992:Body Mass Index; D015331:Cohort Studies; D003645:Death, Sudden / Q000453:epidemiology* / Q000209:etiology*; D004827:Epilepsy / Q000150:complications / Q000453:epidemiology; D005260:Female; D006331:Heart Diseases / Q000150:complications / Q000453:epidemiology; D006801:Humans; D007674:Kidney Diseases / Q000150:complications / Q000453:epidemiology; D008279:Magnetic Resonance Imaging; D008297:Male; D008591:Meningomyelocele / Q000401:mortality* / Q000473:pathology; D008636:Mesencephalon / Q000473:pathology; D012140:Respiratory Tract Diseases / Q000150:complications / Q000453:epidemiology; D012249:Rhombencephalon / Q000002:abnormalities; D012307:Risk Factors; D012600:Scoliosis / Q000150:complications / Q000453:epidemiology; D012737:Sex Factors; D020182:Sleep Apnea, Central / Q000150:complications / Q000453:epidemiology; D016896:Treatment Outcome; D017287:Ventriculoperitoneal Shunt; D055815:Young Adult