Sleep Architecture Linked to Airway Obstruction and Intracranial Hypertension in Children with Syndromic Craniosynostosis

Spruijt, Bart; Mathijssen, Irene M.J.; Bredero-Boelhouwer, Hansje; Cherian, Perumpillichira J.; Corel, L. J. A.; Veelen, Marie-Lise van; Hayward, Richard; Tasker, Robert C.; Joosten, Koen

doi:10.1097/prs.0000000000002741

Cited by 16 publications

(16 citation statements)

References 34 publications

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“…Although moderate to severe OSA has been shown to confer increased risk of ICH development in craniosynostosis, it has not resulted in cortical thickness changes in this study. The mechanism by which OSA is thought to contribute to ICH is through hypercapnia leading to altered cerebral hemodynamics .…”

Section: Discussionmentioning

confidence: 62%

Intracranial hypertension and cortical thickness in syndromic craniosynostosis

Wilson

Ottelander

Goederen

et al. 2020

Develop Med Child Neuro

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Aim To evaluate the impact of risk factors for intracranial hypertension (ICH) on cerebral cortex thickness in syndromic craniosynostosis. Method ICH risk factors including papilloedema, hydrocephalus, obstructive sleep apnea (OSA), cerebellar tonsillar position, occipitofrontal circumference (OFC) curve deflection, age, and sex were collected from the records of patients with syndromic craniosynostosis (Apert, Crouzon, Pfeiffer, Muenke, Saethre‐Chotzen syndromes) and imaging. Magnetic resonance images were analysed and exported for statistical analysis. A linear mixed model was developed to determine correlations with cerebral cortex thickness changes. Results In total, 171 scans from 107 patients (83 males, 88 females [including repeated scans], mean age 8y 10mo, range 1y 1mo–34y, SD 5y 9mo) were evaluated. Mean cortical thickness in this cohort was 2.78mm (SD 0.17). Previous findings of papilloedema (p=0.036) and of hydrocephalus (p=0.007) were independently associated with cortical thinning. Cortical thickness did not vary significantly by sex (p=0.534), syndrome (p=0.896), OSA (p=0.464), OFC (p=0.375), or tonsillar position (p=0.682). Interpretation Detection of papilloedema or hydrocephalus in syndromic craniosynostosis is associated with significant changes in cortical thickness, supporting the need for preventative rather than reactive treatment strategies. What this paper adds Papilloedema is associated with thinning of the cerebral cortex in syndromic craniosynostosis, independently of hydrocephalus.

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Section: Discussionmentioning

confidence: 62%

Intracranial hypertension and cortical thickness in syndromic craniosynostosis

Wilson

Ottelander

Goederen

et al. 2020

Develop Med Child Neuro

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“…OSA may result in hypercapnia and subsequent vasodilatation of the cerebral vasculature which contributes to ICHT [32]. As a result of relaxation of the pharyngeal musculature, OSA occurs mainly in rapid eye movement sleep, when the intracranial pressure already rises due to increased blood flow [33]. OSA and intracranial pressure are interrelated and treatment of ICHT should go hand in hand.…”

Section: Article Highlightsmentioning

confidence: 99%

“…This is a multi-parametric test over night that captures electro-encephalography, heart rate, oxygen saturation, respiration movements, and intravenous CO 2 examination. PSG studies have shown a higher respiratory effort-related arousal index, lower sleep efficiency, and less rapid eye movement sleep in patients with syndromic craniosynostosis with moderate or severe OSA [33]. For follow-up, ambulant polygraphy may be considered [34].…”

Section: Article Highlightsmentioning

confidence: 99%

“…It may vary from tracheostomy, treatment of choanal atresia, nasal corticosteroids, adenotonsillectomy, noninvasive ventilation, palatal split, and midface advancement [21,31]. The disturbed sleep architecture in patients with Apert syndrome was shown to normalize after monobloc surgery [33]. If a cleft palate is present, timing of surgical closure should be critically considered in case of OSA.…”

Section: Osamentioning

confidence: 99%

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Apert syndrome: the Paris and Rotterdam philosophy

Driessen

vanVeelen-Vincent

Joosten

et al. 2017

Expert Opinion on Orphan Drugs

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Introduction: Apert syndrome is a rare type of syndromic craniosynostosis. Patients have an explicit phenotype with craniofacial dysmorphologies and severe symmetrical syndactyly of the hands and feet. This review includes background information about the syndrome and several aspects of the treatment. Areas covered: The cause of Apert syndrome is found in unique mutations in the Fibroblast Growth Factors Receptor (FGFR) 2 gene in 99%. It results in cranial suture fusion, craniofacial dysmorphologies and severe symmetrical syndactyly of the hands and feet. Patients with Apert syndrome are at risk for mental retardation, mobility impairment and intracranial hypertension (ICHT). This is the result of a complex interaction between (1) abnormal skull growth, (2) ventriculomegaly, (3) venous outflow obstruction and (4) obstructive sleep apnea (OSA). Mental retardation is mainly determined by the FGFR2 mutation and treatment is directed at protecting the intrinsic potential of neurocognition. Expert Opinion: To prevent ICHT, we prefer an occipital expansion in the first year of life. Screening on ICHT and its underlying causes is necessary at least until the age of ten by means of skull circumference measurements, fundoscopy, optical coherence tomography, MRI and polysomnography. Multicentre studies on long-term outcome are required to validate the rationale of different clinical protocols. ARTICLE HISTORY

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“…We have previously shown that children with sCS with no OSA or ICH have normal sleep pattern, which was determined by the presence of normal total sleep time (TST), normal number of arousals, and normal sleep architecture using electroencephalography (EEG) derived hypnograms (8). In contrast, sCS children with moderate-to-severe OSA had higher arousal index, higher respiratory effort-related arousal (RERA) index, lower sleep efficiency, less rapid-eye movement (REM) sleep, and more non-REM stage 1 (N1) sleep.…”

Section: Introductionmentioning

confidence: 99%