title: Assessing health-related quality of life in children with spina bifida.
abstract: OBJECTIVE
  The purpose of this study is to explore various aspects of health-related quality of life (HRQOL) in children with spinal dysraphism.
  
  
  METHODS
  The authors enrolled a prospective cohort of 159 patients from the multidisciplinary spina bifida clinic. Surveys were distributed to caregivers of patients with spina bifida who were 5 years old and older. Data were collected using the Health Utilities Index Mark 3 focusing on vision, speech, hearing, dexterity, ambulation, cognition, emotions, and pain. Each participant received an overall HRQOL utility score and individual domain subscores. These were correlated with demographic and treatment variables. Analysis was done using SPSS statistics (version 21).
  
  
  RESULTS
  There were 125 patients with myelomeningocele, 25 with lipomyelomeningocele, and 9 with other dysraphisms. Among patients with myelomeningocele, 107 (86%) had CSF shunts in place, 14 (11%) had undergone Chiari malformation Type II decompression, 59 (47%) were community ambulators, and 45 (36%) were nonambulatory. Patients with myelomeningocele had significantly lower overall HRQOL scores than patients with closed spinal dysraphism. Among patients with myelomeningocele, younger patients had higher HRQOL scores. Patients with impaired bowel continence had lower overall HRQOL scores. History of a ventriculoperitoneal shunt was associated with worse HRQOL (overall score, ambulation, and cognition subscores). History of Chiari malformation Type II decompression was associated with worse overall, speech, and cognition scores. Patients who could ambulate in the community had higher overall and ambulation scores. A history of tethered cord release was correlated with lower pain subscore. No association was found between sex, race, insurance type, or bladder continence and HRQOL.
  
  
  CONCLUSIONS
  Patients with myelomeningocele have significantly lower HRQOL scores than those with other spinal dysraphisms. History of shunt treatment and Chiari decompression correlate with lower HRQOL scores.
authors.affiliation: Pediatric Neurosurgery, Department of Neurosurgery, University of Alabama at Birmingham, Alabama; and.
authors.forename: Brandon G
authors.initials: BG
authors.lastname: Rocque
authors.forename: E Ralee'
authors.initials: ER
authors.lastname: Bishop
authors.forename: Mallory A
authors.initials: MA
authors.lastname: Scogin
authors.forename: Betsy D
authors.initials: BD
authors.lastname: Hopson
authors.forename: Anastasia A
authors.initials: AA
authors.lastname: Arynchyna
authors.forename: Christina J
authors.initials: CJ
authors.lastname: Boddiford
authors.forename: Chevis N
authors.initials: CN
authors.lastname: Shannon
authors.forename: Jeffrey P
authors.initials: JP
authors.lastname: Blount
mesh_terms: D000293:Adolescent; D001139:Arnold-Chiari Malformation / Q000601:surgery*; D017028:Caregivers; D002648:Child; D002675:Child, Preschool; D005260:Female; D006304:Health Status; D006801:Humans; D007223:Infant; D008297:Male; D008591:Meningomyelocele / Q000601:surgery*; D011446:Prospective Studies; D011788:Quality of Life; D016135:Spinal Dysraphism / Q000601:surgery*; D011795:Surveys and Questionnaires; D017287:Ventriculoperitoneal Shunt / Q000009:adverse effects; D055815:Young Adult
keywords: CM-II = Chiari malformation Type II; COA = Children's of Alabama; CPC = choroid plexus cauterization; ETV = endoscopic third ventriculostomy; HRQOL = health-related QOL; HUI3 = Health Utilities Index Mark 3; QOL = quality of life; VP = ventriculoperitoneal; congenital; hydrocephalus; lipomyelomeningocele; myelomeningocele; quality of life; shunt; spina bifida