The role of the posterior fossa in developing Chiari I malformation in children with craniosynostosis syndromes

Rijken, Bianca Francisca Maria; Lequin, Maarten H.; Lijn, Fedde van der; Veelen, M.L.C. van; Rooi, Johan de; Hoogendam, Yoo Young; Niessen, Wiro J.; Mathijssen, Irene M J

doi:10.1016/j.jcms.2015.04.001

Cited by 32 publications

(26 citation statements)

References 31 publications

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“…8,39,40 Children with craniosynostosis and tonsillar herniation have a higher ratio between cerebellar and posterior fossa volume compared with age-matched controls (0.77 vs. 0.75, p ¼ 0.008). 41 In addition, in children with craniosynostosis, the surface area of the foramen magnum is smaller compared with controls contributing to the conflict between the bony structures of the skull base and the brain stem. 42 In patients with shunted hydrocephalus, a tonsillar herniation is a common feature of the "overdrainage" constellation of features.…”

Section: Overcrowding Caused By a Small Skull And/or Posterior Fossamentioning

confidence: 99%

Chiari Type 1 Deformity in Children: Pathogenetic, Clinical, Neuroimaging, and Management Aspects

et al. 2016

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Our understanding of cerebellar tonsillar herniation evolved over time and nowadays various pathomechanisms have been proposed. Causes of tonsillar herniation share a discrepancy between content (fore- and hindbrain) and container (supratentorial cranial vault, posterior fossa), may be associated with abnormalities of the craniocervical junction, and may have a developmental or acquired nature. In tonsillar herniation, the hindbrain is not malformed but deformed. Accordingly, "Chiari type 1 deformity," not "Chiari type 1 malformation" is the correct term to characterize primary tonsillar herniation. Chiari type 1 deformity is commonly seen in pediatric neurology, neuroradiology, and neurosurgery and may have various clinical presentations depending on patient age. In addition, Chiari type 1 deformity is increasingly found by neuroimaging studies as an incidental finding in asymptomatic children. An accurate and reliable selection of patients based on clinical and neuroimaging findings is paramount for the success of neurosurgical treatment. Future studies are needed to provide selection criteria with a higher sensitivity and specificity.

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Section: Overcrowding Caused By a Small Skull And/or Posterior Fossamentioning

confidence: 99%

Chiari Type 1 Deformity in Children: Pathogenetic, Clinical, Neuroimaging, and Management Aspects

et al. 2016

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“…All MRI was performed on a 1.5 T scanner (GE Healthcare, MR Signa Excite HD, Little Chalfont, UK) and the imaging protocol included a 3D T1‐weighted fast spoiled gradient‐echo magnetic resonance sequence. Imaging parameters for craniosynostosis patients were as follows: 2mm slice thickness, no slice gap; field of view 22.4cm; matrix size 224×224; in plane resolution of 1mm; echo time 3.1ms; and repetition time 9.9ms …”

Section: Methodsmentioning

confidence: 99%

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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“…All MRI scans were performed on a 1.5 T scanner (GE Healthcare, MR Signa Excite HD, Little Chalfont, UK) with the imaging protocol, including a three-dimensional fast spoiled gradient echo T1-weighted MR sequence. Imaging parameters for craniosynostosis patients were the following: 2 mm slice thickness, no slice gap; field of view (FOV): 22.4 cm; matrix size: 224 × 224; in plane resolution of 1 mm; echo time : 3.1 ms; and repetition time: 9.9 ms. 22 MRI was the imaging modality of choice in this study because of its ability to adequately distinguish between tissue densities (white matter, grey matter, and dura) critical to the calculation of cerebral cortical thickness.…”

Section: Methodsmentioning

confidence: 99%

Cortical Thickness in Crouzon–Pfeiffer Syndrome: Findings in Relation to Primary Cranial Vault Expansion

Wilson

Planque

Yang

et al. 2020

Plastic and Reconstructive Surgery - Global Open

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Background: Episodes of intracranial hypertension are associated with reductions in cerebral cortical thickness (CT) in syndromic craniosynostosis. Here we focus on Crouzon–Pfeiffer syndrome patients to measure CT and evaluate associations with type of primary cranial vault expansion and synostosis pattern. Methods: Records from 34 Crouzon–Pfeiffer patients were reviewed along with MRI data on CT and intracranial volume to examine associations. Patients were grouped according to initial cranial vault expansion (frontal/occipital). Data were analyzed by multiple linear regression controlled for age and brain volume to determine an association between global/lobar CT and vault expansion type. Synostosis pattern effect sizes on global/lobar CT were calculated as secondary outcomes. Results: Occipital expansion patients demonstrated 0.02 mm thicker cortex globally (P = 0.81) with regional findings, including: thicker cortex in frontal (0.02 mm, P = 0.77), parietal (0.06 mm, P = 0.44) and occipital (0.04 mm, P = 0.54) regions; and thinner cortex in temporal (−0.03 mm, P = 0.69), cingulate (−0.04 mm, P = 0.785), and, insula (−0.09 mm, P = 0.51) regions. Greatest effect sizes were observed between left lambdoid synostosis and the right cingulate (d = −1.00) and right lambdoid synostosis and the left cingulate (d = −1.23). Left and right coronal synostosis yielded effect sizes of d = −0.56 and d = −0.42 on respective frontal lobes. Conclusions: Both frontal and occipital primary cranial vault expansions correlate to similar regional CT in Crouzon–Pfeiffer patients. Lambdoid synostosis appears to be associated with cortical thinning, particularly in the cingulate gyri.

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The role of the posterior fossa in developing Chiari I malformation in children with craniosynostosis syndromes

Cited by 32 publications

References 31 publications

Chiari Type 1 Deformity in Children: Pathogenetic, Clinical, Neuroimaging, and Management Aspects

Chiari Type 1 Deformity in Children: Pathogenetic, Clinical, Neuroimaging, and Management Aspects

Intracranial hypertension and cortical thickness in syndromic craniosynostosis

Cortical Thickness in Crouzon–Pfeiffer Syndrome: Findings in Relation to Primary Cranial Vault Expansion

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