Intracranial Volume and Head Circumference in Children with Unoperated Syndromic Craniosynostosis

Objective: When analysing intracranial volume gain due to operative intervention in craniosynostosis, it is necessary to understand the underlying growth. We sought to create comprehensive intracranial volume (ICV) and occipitofrontal circumference (OFC) growth charts, as measured on unoperated craniosynostotic children. Furthermore, we aimed to investigate whether ICV and OFC could act as a proxy measure for each other. Methods: All pre-operative Great Ormond Street Hospital (GOSH) patients with a diagnosis of Apert, Crouzon-Pfeiffer or Saethre-Chotzen syndrome from 2004 onwards were considered for this study. A control group of unaffected GOSH patients were also measured. ICV and OFC were measured on the same scans. To study correlation between IVC and OFC, logarithmic fits were assessed. Results: 147 craniosynostotic children with 221 preoperative scans were included (81 Apert, 81 Crouzon, 31 Pfeiffer, and 28 Saethre-Chotzen). The control group comprised 56 patients with 58 scans. Apert ICV curves were significantly larger than other syndromes from 206 days onwards, OFC curves were not significantly different. The correlation coefficient between ICV and OFC for all syndromes combined was R2 = 0.87, for the control group R2 = 0.91. Conclusions: Apert children have a larger intracranial volume than other syndromic craniosynostotic conditions and unaffected children but maintain a similar occipitofrontal circumference. This study demonstrates high correlation between intracranial volume and OFC with clinical care implications. Reference growth curves have been created which can be used to monitor intracranial volume change over time and correct operative change for underlying growth. Author Contributions: RWFB, PGMK, GJ, NUOJ and SS designed the study, contributed to data acquisition, analysis and interpretation before drafting the manuscript. AB, NRF, JOH and DJD critically revised the manuscript. Acknowledgements: This research was supported by Great Ormond Street Hospital Children's Charity FaceValue programme (no. 12SG15), The Royal College of Surgeons of England, and the EPSRC (EP/N02124X/1). This report incorporates independent research from the National Institute for Health Research Biomedical Research Centre Funding Scheme. The views expressed in this publication are those of the authors and not necessarily those of the NHS, the National Institute for Health Research or the Department of Health. None of the authors has a financial interest in any of the products, devices or drugs mentioned in this manuscript This paper was presented at the 17th Congress of the International Society of Craniofacial Surgery on October 27th 2017 in Cancun, Mexico Corresponding Author: William Breakey, UCL Great Ormond Street Institute of Child Health, 30 Guilford Street, WC1N 1EH, London, UK. +44 20 7242 9789, William.breakey@ucl.ac.uk ©2018American Society of Plastic Surgeons

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