title: Algorithm for the Management of Intracranial Hypertension in Children with Syndromic Craniosynostosis.
abstract: BACKGROUND
  The purpose of this study was to examine the relationship of head growth, obstructive sleep apnea, and intracranial hypertension in patients with syndromic or complex craniosynostosis, and to evaluate the authors' standardized treatment protocol for the management of intracranial hypertension in these patients.
  
  
  METHODS
  The authors conducted a prospective observational cohort study of patients with syndromic craniosynostosis at a national referral center, treated according to a standardized protocol. Measurements included occipitofrontal head circumference, with growth arrest defined as downward deflection in occipitofrontal head circumference trajectory greater than or equal to a 0.5 SD fall from baseline over 2 years, or lack of change in occipitofrontal head circumference growth curve; sleep studies, with results dichotomized into no/mild versus moderate/severe obstructive sleep apnea; and funduscopy to indicate papilledema, supplemented by optical coherence tomography and/or intracranial pressure monitoring to identify intracranial hypertension.
  
  
  RESULTS
  The authors included 62 patients, of whom 21 (33.9 percent) had intracranial hypertension, 39 (62.9 percent) had obstructive sleep apnea, and 20 (32.3 percent) had occipitofrontal head circumference growth arrest during the study. Age at which intracranial hypertension first occurred was 2.0 years (range, 0.4 to 6.0 years). Preoperatively, 13 patients (21.0 percent) had intracranial hypertension, which was associated only with moderate/severe obstructive sleep apnea (p = 0.012). In the first year after surgery, intracranial hypertension was particularly related to occipitofrontal head circumference growth arrest (p = 0.006). Beyond 1 year after surgery, intracranial hypertension was associated with a combination of occipitofrontal head circumference growth arrest (p < 0.001) and moderate/severe obstructive sleep apnea (p = 0.007).
  
  
  CONCLUSIONS
  Children with syndromic craniosynostosis are at risk of intracranial hypertension. The major determinant of this after vault expansion is impaired head growth, which may occur at varying ages. The presence of moderate/severe obstructive sleep apnea also significantly increases the risk of intracranial hypertension.
  
  
  METHODS
  Risk, III.
authors.affiliation: Rotterdam, The Netherlands; and Boston, Mass. From the Dutch Craniofacial Center; the Pediatric Intensive Care Unit; and the Departments of Biostatistics, Ophthalmology, Otorhinolaryngology, Oral and Maxillofacial Surgery, and Neurosurgery, Sophia Children's Hospital-Erasmus University Medical Center; and the Departments of Neurology and Anaesthesia (Pediatrics), Harvard Medical School and Boston Children's Hospital.
authors.forename: Bart
authors.initials: B
authors.lastname: Spruijt
authors.forename: Koen F M
authors.initials: KFM
authors.lastname: Joosten
authors.forename: Caroline
authors.initials: C
authors.lastname: Driessen
authors.forename: Dimitris
authors.initials: D
authors.lastname: Rizopoulos
authors.forename: Nicole C
authors.initials: NC
authors.lastname: Naus
authors.forename: Marc P
authors.initials: MP
authors.lastname: van der Schroeff
authors.forename: Eppo B
authors.initials: EB
authors.lastname: Wolvius
authors.forename: Marie-Lise C
authors.initials: MC
authors.lastname: van Veelen
authors.forename: Robert C
authors.initials: RC
authors.lastname: Tasker
authors.forename: Irene M J
authors.initials: IMJ
authors.lastname: Mathijssen
mesh_terms: D017677:Age Distribution; D000465:Algorithms; D002508:Cephalometry / Q000379:methods; D002675:Child, Preschool; D015897:Comorbidity; D003398:Craniosynostoses / Q000175:diagnosis / Q000453:epidemiology* / Q000601:surgery*; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D019586:Intracranial Hypertension / Q000175:diagnosis / Q000453:epidemiology* / Q000601:surgery*; D008297:Male; D008991:Monitoring, Physiologic / Q000379:methods; D017286:Polysomnography / Q000379:methods; D011379:Prognosis; D011446:Prospective Studies; D018570:Risk Assessment; D012720:Severity of Illness Index; D017678:Sex Distribution; D012891:Sleep Apnea Syndromes / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy; D041623:Tomography, Optical Coherence / Q000379:methods; D016896:Treatment Outcome