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The formation of the foramen magnum and its role in developing ventriculomegaly and Chiari I malformation in children with craniosynostosis syndromes.
J Craniomaxillofac Surg. 2015 Sep; 43(7):1042-8.JC

Abstract

OBJECT

Craniosynostosis syndromes are characterized by prematurely fused skull sutures, however, less is known about skull base synchondroses. This study evaluates how foramen magnum (FM) size, and closure of its intra-occipital synchondroses (IOS) differ between patients with different craniosynostosis syndromes and control subjects; and whether this correlates to ventriculomegaly and/or Chiari malformation type I (CMI), intracranial disturbances often described in these patients.

METHODS

Surface area and anterior-posterior (A-P) diameter were measured in 175 3D-CT scans of 113 craniosynostosis patients, and in 53 controls (0-10 years old). Scans were aligned in a 3D multiplane-platform. The frontal and occipital horn ratio was used as an indicator of ventricular volume, and the occurrence of CMI was recorded. Synchondroses were studied in scans with a slice thickness ≤1.25 mm. A generalized linear mixed model and a repeated measures ordinal logistic regression model were used to study differences.

RESULTS

At birth, patients with craniosynostosis syndromes have a smaller FM than controls (p < 0.05). This is not related to the presence of CMI (p = 0.36). In Crouzon-Pfeiffer patients the anterior and posterior IOS fused prematurely (p < 0.01), and in Apert patients only the posterior IOS fused prematurely (p = 0.028).

CONCLUSION

The FM is smaller in patients with craniosynostosis syndromes than in controls, and is already smaller at birth. In addition to the timing of IOS closure, other factors may influence FM size.

Authors+Show Affiliations

Department of Plastic and Reconstructive Surgery, Dutch Craniofacial Center, Erasmus University Medical Center/Sophia Children's Hospital, Rotterdam, The Netherlands. Electronic address: b.rijken@erasmusmc.nl.Department of Paediatric Radiology, Erasmus University Medical Center/Sophia Children's Hospital, Rotterdam, The Netherlands.Department of Paediatric Neurosurgery, Erasmus University Medical Center/Sophia Children's Hospital, Rotterdam, The Netherlands.Department of Biostatistics, Erasmus University Medical Center, Rotterdam, The Netherlands.Department of Plastic and Reconstructive Surgery, Dutch Craniofacial Center, Erasmus University Medical Center/Sophia Children's Hospital, Rotterdam, The Netherlands.

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

26051848

Citation

Rijken, Bianca F M., et al. "The Formation of the Foramen Magnum and Its Role in Developing Ventriculomegaly and 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. 7, 2015, pp. 1042-8.
Rijken BF, Lequin MH, Van Veelen ML, et al. The formation of the foramen magnum and its role in developing ventriculomegaly and Chiari I malformation in children with craniosynostosis syndromes. J Craniomaxillofac Surg. 2015;43(7):1042-8.
Rijken, B. F., Lequin, M. H., Van Veelen, M. L., de Rooi, J., & Mathijssen, I. M. (2015). The formation of the foramen magnum and its role in developing ventriculomegaly and 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(7), 1042-8. https://doi.org/10.1016/j.jcms.2015.04.025
Rijken BF, et al. The Formation of the Foramen Magnum and Its Role in Developing Ventriculomegaly and Chiari I Malformation in Children With Craniosynostosis Syndromes. J Craniomaxillofac Surg. 2015;43(7):1042-8. PubMed PMID: 26051848.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - The formation of the foramen magnum and its role in developing ventriculomegaly and Chiari I malformation in children with craniosynostosis syndromes. AU - Rijken,Bianca F M, AU - Lequin,Maarten H, AU - Van Veelen,Marie-Lise C, AU - de Rooi,Johan, AU - Mathijssen,Irene M J, Y1 - 2015/05/08/ PY - 2015/01/17/received PY - 2015/04/29/revised PY - 2015/04/30/accepted PY - 2015/6/9/entrez PY - 2015/6/9/pubmed PY - 2017/1/11/medline KW - Chiari I malformation KW - Craniosynostosis syndromes KW - Foramen magnum KW - Ventriculomegaly SP - 1042 EP - 8 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 - 7 N2 - OBJECT: Craniosynostosis syndromes are characterized by prematurely fused skull sutures, however, less is known about skull base synchondroses. This study evaluates how foramen magnum (FM) size, and closure of its intra-occipital synchondroses (IOS) differ between patients with different craniosynostosis syndromes and control subjects; and whether this correlates to ventriculomegaly and/or Chiari malformation type I (CMI), intracranial disturbances often described in these patients. METHODS: Surface area and anterior-posterior (A-P) diameter were measured in 175 3D-CT scans of 113 craniosynostosis patients, and in 53 controls (0-10 years old). Scans were aligned in a 3D multiplane-platform. The frontal and occipital horn ratio was used as an indicator of ventricular volume, and the occurrence of CMI was recorded. Synchondroses were studied in scans with a slice thickness ≤1.25 mm. A generalized linear mixed model and a repeated measures ordinal logistic regression model were used to study differences. RESULTS: At birth, patients with craniosynostosis syndromes have a smaller FM than controls (p < 0.05). This is not related to the presence of CMI (p = 0.36). In Crouzon-Pfeiffer patients the anterior and posterior IOS fused prematurely (p < 0.01), and in Apert patients only the posterior IOS fused prematurely (p = 0.028). CONCLUSION: The FM is smaller in patients with craniosynostosis syndromes than in controls, and is already smaller at birth. In addition to the timing of IOS closure, other factors may influence FM size. SN - 1878-4119 UR - https://www.unboundmedicine.com/medline/citation/26051848/The_formation_of_the_foramen_magnum_and_its_role_in_developing_ventriculomegaly_and_Chiari_I_malformation_in_children_with_craniosynostosis_syndromes_ DB - PRIME DP - Unbound Medicine ER -