title: Sleep Architecture Linked to Airway Obstruction and Intracranial Hypertension in Children with Syndromic Craniosynostosis.
abstract: BACKGROUND
  Children with syndromic craniosynostosis often have obstructive sleep apnea and intracranial hypertension. The authors aimed to evaluate (1) sleep architecture, and determine whether this is influenced by the presence of obstructive sleep apnea and/or intracranial hypertension; and (2) the effect of treatment on sleep architecture.
  
  
  METHODS
  This study included patients with syndromic craniosynostosis treated at a national referral center, undergoing screening for obstructive sleep apnea and intracranial hypertension. Obstructive sleep apnea was identified by polysomnography, and categorized into no, mild, moderate, or severe. Intracranial hypertension was identified by the presence of papilledema on funduscopy, supplemented by optical coherence tomography and/or intracranial pressure monitoring. Regarding sleep architecture, sleep was divided into rapid eye movement or non-rapid eye movement sleep; respiratory effort-related arousals and sleep efficiency were scored.
  
  
  RESULTS
  The authors included 39 patients (median age, 5.9 years): 19 with neither obstructive sleep apnea nor intracranial hypertension, 11 with obstructive sleep apnea (four moderate/severe), six with intracranial hypertension, and three with obstructive sleep apnea and intracranial hypertension. Patients with syndromic craniosynostosis, independent of the presence of mild obstructive sleep apnea and/or intracranial hypertension, have normal sleep architecture compared with age-matched controls. Patients with moderate/severe obstructive sleep apnea have a higher respiratory effort-related arousal index (p < 0.01), lower sleep efficiency (p = 0.01), and less rapid eye movement sleep (p = 0.04). An improvement in sleep architecture was observed following monobloc surgery (n = 5; rapid eye movement sleep, 5.3 percent; p = 0.04).
  
  
  CONCLUSIONS
  Children with syndromic craniosynostosis have in principle normal sleep architecture. However, moderate/severe obstructive sleep apnea does lead to disturbed sleep architecture, which fits within a framework of a unifying theory for obstructive sleep apnea, intracranial hypertension, and sleep.
  
  
  METHODS
  Risk, II.
authors.affiliation: Rotterdam, The Netherlands; London, United Kingdom; and Boston, Mass.|From the Dutch Craniofacial Center; the Departments of Plastic and Reconstructive Surgery and Hand Surgery, Clinical Neurophysiology, and Neurosurgery, and the Pediatric Intensive Care Unit, Erasmus Medical Center-Sophia Children's Hospital, University Medical Center Rotterdam; the Department of Neurosurgery, Great Ormond Street Hospital for Children; 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.affiliation: Rotterdam, The Netherlands; London, United Kingdom; and Boston, Mass.|From the Dutch Craniofacial Center; the Departments of Plastic and Reconstructive Surgery and Hand Surgery, Clinical Neurophysiology, and Neurosurgery, and the Pediatric Intensive Care Unit, Erasmus Medical Center-Sophia Children's Hospital, University Medical Center Rotterdam; the Department of Neurosurgery, Great Ormond Street Hospital for Children; and the Departments of Neurology and Anaesthesia (Pediatrics), Harvard Medical School and Boston Children's Hospital.
authors.forename: Irene M J
authors.initials: IMJ
authors.lastname: Mathijssen
authors.affiliation: Rotterdam, The Netherlands; London, United Kingdom; and Boston, Mass.|From the Dutch Craniofacial Center; the Departments of Plastic and Reconstructive Surgery and Hand Surgery, Clinical Neurophysiology, and Neurosurgery, and the Pediatric Intensive Care Unit, Erasmus Medical Center-Sophia Children's Hospital, University Medical Center Rotterdam; the Department of Neurosurgery, Great Ormond Street Hospital for Children; and the Departments of Neurology and Anaesthesia (Pediatrics), Harvard Medical School and Boston Children's Hospital.
authors.forename: Hansje H
authors.initials: HH
authors.lastname: Bredero-Boelhouwer
authors.affiliation: Rotterdam, The Netherlands; London, United Kingdom; and Boston, Mass.|From the Dutch Craniofacial Center; the Departments of Plastic and Reconstructive Surgery and Hand Surgery, Clinical Neurophysiology, and Neurosurgery, and the Pediatric Intensive Care Unit, Erasmus Medical Center-Sophia Children's Hospital, University Medical Center Rotterdam; the Department of Neurosurgery, Great Ormond Street Hospital for Children; and the Departments of Neurology and Anaesthesia (Pediatrics), Harvard Medical School and Boston Children's Hospital.
authors.forename: Perumpillichira J
authors.initials: PJ
authors.lastname: Cherian
authors.affiliation: Rotterdam, The Netherlands; London, United Kingdom; and Boston, Mass.|From the Dutch Craniofacial Center; the Departments of Plastic and Reconstructive Surgery and Hand Surgery, Clinical Neurophysiology, and Neurosurgery, and the Pediatric Intensive Care Unit, Erasmus Medical Center-Sophia Children's Hospital, University Medical Center Rotterdam; the Department of Neurosurgery, Great Ormond Street Hospital for Children; and the Departments of Neurology and Anaesthesia (Pediatrics), Harvard Medical School and Boston Children's Hospital.
authors.forename: Linda J A
authors.initials: LJA
authors.lastname: Corel
authors.affiliation: Rotterdam, The Netherlands; London, United Kingdom; and Boston, Mass.|From the Dutch Craniofacial Center; the Departments of Plastic and Reconstructive Surgery and Hand Surgery, Clinical Neurophysiology, and Neurosurgery, and the Pediatric Intensive Care Unit, Erasmus Medical Center-Sophia Children's Hospital, University Medical Center Rotterdam; the Department of Neurosurgery, Great Ormond Street Hospital for Children; and the Departments of Neurology and Anaesthesia (Pediatrics), Harvard Medical School and Boston Children's Hospital.
authors.forename: Marie-Lise
authors.initials: ML
authors.lastname: van Veelen
authors.affiliation: Rotterdam, The Netherlands; London, United Kingdom; and Boston, Mass.|From the Dutch Craniofacial Center; the Departments of Plastic and Reconstructive Surgery and Hand Surgery, Clinical Neurophysiology, and Neurosurgery, and the Pediatric Intensive Care Unit, Erasmus Medical Center-Sophia Children's Hospital, University Medical Center Rotterdam; the Department of Neurosurgery, Great Ormond Street Hospital for Children; and the Departments of Neurology and Anaesthesia (Pediatrics), Harvard Medical School and Boston Children's Hospital.
authors.forename: Richard D
authors.initials: RD
authors.lastname: Hayward
authors.affiliation: Rotterdam, The Netherlands; London, United Kingdom; and Boston, Mass.|From the Dutch Craniofacial Center; the Departments of Plastic and Reconstructive Surgery and Hand Surgery, Clinical Neurophysiology, and Neurosurgery, and the Pediatric Intensive Care Unit, Erasmus Medical Center-Sophia Children's Hospital, University Medical Center Rotterdam; the Department of Neurosurgery, Great Ormond Street Hospital for Children; and the Departments of Neurology and Anaesthesia (Pediatrics), Harvard Medical School and Boston Children's Hospital.
authors.forename: Robert C
authors.initials: RC
authors.lastname: Tasker
authors.affiliation: Rotterdam, The Netherlands; London, United Kingdom; and Boston, Mass.|From the Dutch Craniofacial Center; the Departments of Plastic and Reconstructive Surgery and Hand Surgery, Clinical Neurophysiology, and Neurosurgery, and the Pediatric Intensive Care Unit, Erasmus Medical Center-Sophia Children's Hospital, University Medical Center Rotterdam; the Department of Neurosurgery, Great Ormond Street Hospital for Children; and the Departments of Neurology and Anaesthesia (Pediatrics), Harvard Medical School and Boston Children's Hospital.
authors.forename: Koen F M
authors.initials: KFM
authors.lastname: Joosten
mesh_terms: D000293:Adolescent; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D003398:Craniosynostoses / Q000150:complications* / Q000503:physiopathology / Q000601:surgery; D005260:Female; D006801:Humans; D007223:Infant; D019586:Intracranial Hypertension / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology*; D008297:Male; D017286:Polysomnography; D011446:Prospective Studies; D012720:Severity of Illness Index; D012890:Sleep / Q000502:physiology*; D020181:Sleep Apnea, Obstructive / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology*; D013577:Syndrome; D016896:Treatment Outcome