Bianca F M Rijken1, Maarten H Lequin2, Marie-Lise C Van Veelen3, Johan de Rooi4, Irene M J Mathijssen5. 1. 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. 2. Department of Paediatric Radiology, Erasmus University Medical Center/Sophia Children's Hospital, Rotterdam, The Netherlands. 3. Department of Paediatric Neurosurgery, Erasmus University Medical Center/Sophia Children's Hospital, Rotterdam, The Netherlands. 4. Department of Biostatistics, Erasmus University Medical Center, Rotterdam, The Netherlands. 5. Department of Plastic and Reconstructive Surgery, Dutch Craniofacial Center, Erasmus University Medical Center/Sophia Children's Hospital, Rotterdam, The Netherlands.
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.
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 craniosynostosispatients, 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: B K den Ottelander; R de Goederen; C A de Planque; S J Baart; M L C van Veelen; L J A Corel; K F M Joosten; I M J Mathijssen; M H G Dremmen Journal: AJNR Am J Neuroradiol Date: 2020-12-03 Impact factor: 3.825
Authors: Bianca K Den Ottelander; Marie-Lise C Van Veelen; Robbin De Goederen; Stephanie Dc Van De Beeten; Marjolein Hg Dremmen; Sjoukje E Loudon; Sarah L Versnel; Ans Mw Van Den Ouweland; Marieke F Van Dooren; Koen Fm Joosten; Irene Mj Mathijssen Journal: Dev Med Child Neurol Date: 2020-09-09 Impact factor: 5.449