Bianca Francisca Maria Rijken1, Maarten Hans Lequin2, Fedde van der Lijn3, Marie-Lise Charlotte van Veelen-Vincent4, Johan de Rooi5, Yoo Young Hoogendam6, Wiro Joep Niessen7, Irene Margreet Jacqueline Mathijssen8. 1. 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. 2. Department of Pediatric Radiology, Dutch Craniofacial Center, Erasmus University Medical Center/Sophia Children's Hospital, Rotterdam, The Netherlands. 3. Department of Radiology, Erasmus University Medical Center, Rotterdam, The Netherlands; Department of Medical Informatics, Erasmus University Medical Center, Rotterdam, The Netherlands. 4. Department of Pediatric Neurosurgery, Dutch Craniofacial Center, Erasmus University Medical Center/Sophia Children's Hospital, Rotterdam, The Netherlands. 5. Department of Biostatistics, Erasmus University Medical Center, Rotterdam, The Netherlands. 6. Department of Epidemiology, Erasmus University Medical Center, Rotterdam, The Netherlands. 7. 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. 8. Department of Plastic and Reconstructive Surgery and Hand Surgery, Dutch Craniofacial Center, Erasmus University Medical Center/Sophia Children's Hospital, Rotterdam, The Netherlands.
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.
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 craniosynostosispatients 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' craniosynostosispatients (mean age: 4.0 years; range: 0-14), 85 'operated' craniosynostosispatients (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 craniosynostosispatients, between the individual craniosynostosis syndromes and controls, and between craniosynostosispatients 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 craniosynostosispatients and operated craniosynostosispatients were similar to those of the control subjects. None of the individual syndromes was associated with a restricted PFV. However, craniosynostosispatients 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 craniosynostosispatients with CMI, however, did not exceed the normal range. CONCLUSION: Volumes and CV/PFV ratio cannot predict which craniosynostosispatients 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.
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Authors: Alexander T Wilson; Bianca K Den Ottelander; Marie-Lise C Van Veelen; Marjolein Hg Dremmen; John A Persing; Henri A Vrooman; Irene Mj Mathijssen; Robert C Tasker Journal: Dev Med Child Neurol Date: 2021-07-15 Impact factor: 4.864