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The role of the posterior fossa in developing Chiari I malformation in children with craniosynostosis syndromes.
J Craniomaxillofac Surg. 2015 Jul; 43(6):813-9.JC

Abstract

OBJECTIVE

Patients with craniosynostosis syndromes are at risk of increased intracranial pressure (ICP) and Chiari I malformation (CMI), caused by a combination of restricted skull growth, venous hypertension, obstructive sleep apnea (OSA), and an overproduction or insufficient resorption of cerebrospinal fluid. This study evaluates whether craniosynostosis patients with CMI have an imbalance between cerebellar volume (CV) and posterior fossa volume (PFV), that is, an overcrowded posterior fossa.

METHODS

Volumes were measured in 3D-SPGR T1-weighted MR scans of 28 'not-operated' craniosynostosis patients (mean age: 4.0 years; range: 0-14), 85 'operated' craniosynostosis patients (mean age: 8.0 years; range: 1-18), and 34 control subjects (mean age: 5.4 years; range: 0-15). Volumes and CV/PFV ratios were compared between the operated and not-operated craniosynostosis patients, between the individual craniosynostosis syndromes and controls, and between craniosynostosis patients with and without CMI. Data were logarithmically transformed and studied with analysis of covariance (ANCOVA).

RESULTS

The CV, PFV, and CV/PFV ratios of not-operated craniosynostosis patients and operated craniosynostosis patients were similar to those of the control subjects. None of the individual syndromes was associated with a restricted PFV. However, craniosynostosis patients with CMI had a significantly higher CV/PFV ratio than the control group (0.77 vs. 0.75; p = 0.008). The range of CV/PFV ratios for craniosynostosis patients with CMI, however, did not exceed the normal range.

CONCLUSION

Volumes and CV/PFV ratio cannot predict which craniosynostosis patients are more prone to developing CMI than others. Treatment should focus on the skull vault and other contributing factors to increased ICP, including OSA and venous hypertension.

Authors+Show Affiliations

Department of Plastic and Reconstructive Surgery and Hand Surgery, Dutch Craniofacial Center, Erasmus University Medical Center/Sophia Children's Hospital, Rotterdam, The Netherlands. Electronic address: b.rijken@erasmusmc.nl.Department of Pediatric Radiology, Dutch Craniofacial Center, Erasmus University Medical Center/Sophia Children's Hospital, Rotterdam, The Netherlands.Department of Radiology, Erasmus University Medical Center, Rotterdam, The Netherlands; Department of Medical Informatics, Erasmus University Medical Center, Rotterdam, The Netherlands.Department of Pediatric Neurosurgery, Dutch Craniofacial Center, Erasmus University Medical Center/Sophia Children's Hospital, Rotterdam, The Netherlands.Department of Biostatistics, Erasmus University Medical Center, Rotterdam, The Netherlands.Department of Epidemiology, Erasmus University Medical Center, Rotterdam, The Netherlands.Department of Radiology, Erasmus University Medical Center, Rotterdam, The Netherlands; Department of Medical Informatics, Erasmus University Medical Center, Rotterdam, The Netherlands; Faculty of Applied Sciences, Delft University of Technology, The Netherlands.Department of Plastic and Reconstructive Surgery and Hand Surgery, Dutch Craniofacial Center, Erasmus University Medical Center/Sophia Children's Hospital, Rotterdam, The Netherlands.

Pub Type(s)

Comparative Study
Journal Article

Language

eng

PubMed ID

25979575

Citation

Rijken, Bianca Francisca Maria, et al. "The Role of the Posterior Fossa in Developing Chiari I Malformation in Children With Craniosynostosis Syndromes." Journal of Cranio-maxillo-facial Surgery : Official Publication of the European Association for Cranio-Maxillo-Facial Surgery, vol. 43, no. 6, 2015, pp. 813-9.
Rijken BF, Lequin MH, van der Lijn F, et al. The role of the posterior fossa in developing Chiari I malformation in children with craniosynostosis syndromes. J Craniomaxillofac Surg. 2015;43(6):813-9.
Rijken, B. F., Lequin, M. H., van der Lijn, F., van Veelen-Vincent, M. L., de Rooi, J., Hoogendam, Y. Y., Niessen, W. J., & Mathijssen, I. M. (2015). The role of the posterior fossa in developing Chiari I malformation in children with craniosynostosis syndromes. Journal of Cranio-maxillo-facial Surgery : Official Publication of the European Association for Cranio-Maxillo-Facial Surgery, 43(6), 813-9. https://doi.org/10.1016/j.jcms.2015.04.001
Rijken BF, et al. The Role of the Posterior Fossa in Developing Chiari I Malformation in Children With Craniosynostosis Syndromes. J Craniomaxillofac Surg. 2015;43(6):813-9. PubMed PMID: 25979575.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - The role of the posterior fossa in developing Chiari I malformation in children with craniosynostosis syndromes. AU - Rijken,Bianca Francisca Maria, AU - Lequin,Maarten Hans, AU - van der Lijn,Fedde, AU - van Veelen-Vincent,Marie-Lise Charlotte, AU - de Rooi,Johan, AU - Hoogendam,Yoo Young, AU - Niessen,Wiro Joep, AU - Mathijssen,Irene Margreet Jacqueline, Y1 - 2015/04/14/ PY - 2015/03/01/received PY - 2015/03/31/revised PY - 2015/04/02/accepted PY - 2015/5/17/entrez PY - 2015/5/17/pubmed PY - 2016/12/15/medline KW - Cerebellum KW - Chiari I malformation KW - Craniosynostosis syndromes KW - Posterior fossa SP - 813 EP - 9 JF - Journal of cranio-maxillo-facial surgery : official publication of the European Association for Cranio-Maxillo-Facial Surgery JO - J Craniomaxillofac Surg VL - 43 IS - 6 N2 - OBJECTIVE: Patients with craniosynostosis syndromes are at risk of increased intracranial pressure (ICP) and Chiari I malformation (CMI), caused by a combination of restricted skull growth, venous hypertension, obstructive sleep apnea (OSA), and an overproduction or insufficient resorption of cerebrospinal fluid. This study evaluates whether craniosynostosis patients with CMI have an imbalance between cerebellar volume (CV) and posterior fossa volume (PFV), that is, an overcrowded posterior fossa. METHODS: Volumes were measured in 3D-SPGR T1-weighted MR scans of 28 'not-operated' craniosynostosis patients (mean age: 4.0 years; range: 0-14), 85 'operated' craniosynostosis patients (mean age: 8.0 years; range: 1-18), and 34 control subjects (mean age: 5.4 years; range: 0-15). Volumes and CV/PFV ratios were compared between the operated and not-operated craniosynostosis patients, between the individual craniosynostosis syndromes and controls, and between craniosynostosis patients with and without CMI. Data were logarithmically transformed and studied with analysis of covariance (ANCOVA). RESULTS: The CV, PFV, and CV/PFV ratios of not-operated craniosynostosis patients and operated craniosynostosis patients were similar to those of the control subjects. None of the individual syndromes was associated with a restricted PFV. However, craniosynostosis patients with CMI had a significantly higher CV/PFV ratio than the control group (0.77 vs. 0.75; p = 0.008). The range of CV/PFV ratios for craniosynostosis patients with CMI, however, did not exceed the normal range. CONCLUSION: Volumes and CV/PFV ratio cannot predict which craniosynostosis patients are more prone to developing CMI than others. Treatment should focus on the skull vault and other contributing factors to increased ICP, including OSA and venous hypertension. SN - 1878-4119 UR - https://www.unboundmedicine.com/medline/citation/25979575/The_role_of_the_posterior_fossa_in_developing_Chiari_I_malformation_in_children_with_craniosynostosis_syndromes_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1010-5182(15)00096-7 DB - PRIME DP - Unbound Medicine ER -