Cerebral blood flow in children with syndromic craniosynostosis: cohort arterial spin labeling studies

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  • 1 Department of Plastic and Reconstructive Surgery and Hand Surgery, Dutch Craniofacial Center,
  • 3 Department of Radiology,
  • 4 Department of Biostatistics,
  • 8 Pediatric Intensive Care Unit, and
  • 9 Department of Neurosurgery, Sophia Children’s Hospital, Erasmus MC, University Medical Center Rotterdam;
  • 2 Department of Radiology, University Medical Center Utrecht, The Netherlands;
  • 5 Department of Radiology and Radiological Science, Section of Pediatric Neuroradiology, Division of Pediatric Radiology, The Johns Hopkins University School of Medicine, Baltimore, Maryland;
  • 6 Institute of Diagnostic and Interventional Radiology, University Hospital Zurich, University of Zurich, Switzerland;
  • 7 Department of Diagnostic Imaging, Division of Neuroradiology, The Hospital for Sick Children, Toronto, ON, Canada; and
  • 10 Departments of Neurology and Anesthesiology (Pediatrics), Harvard Medical School and Boston Children’s Hospital, Boston, Massachusetts
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OBJECTIVE

In comparison with the general population, children with syndromic craniosynostosis (sCS) have abnormal cerebral venous anatomy and are more likely to develop intracranial hypertension. To date, little is known about the postnatal development change in cerebral blood flow (CBF) in sCS. The aim of this study was to determine CBF in patients with sCS, and compare findings with control subjects.

METHODS

A prospective cohort study of patients with sCS using MRI and arterial spin labeling (ASL) determined regional CBF patterns in comparison with a convenience sample of control subjects with identical MRI/ASL assessments in whom the imaging showed no cerebral/neurological pathology. Patients with SCS and control subjects were stratified into four age categories and compared using CBF measurements from four brain lobes, the cerebellum, supratentorial cortex, and white matter. In a subgroup of patients with sCS the authors also compared longitudinal pre- to postoperative CBF changes.

RESULTS

Seventy-six patients with sCS (35 female [46.1%] and 41 male [53.9%]), with a mean age of 4.5 years (range 0.2–19.2 years), were compared with 86 control subjects (38 female [44.2%] and 48 male [55.8%]), with a mean age of 6.4 years (range 0.1–17.8 years). Untreated sCS patients < 1 year old had lower CBF than control subjects. In older age categories, CBF normalized to values observed in controls. Graphical analyses of CBF by age showed that the normally expected peak in CBF during childhood, noted at 4 years of age in control subjects, occurred at 5–6 years of age in patients with sCS. Patients with longitudinal pre- to postoperative CBF measurements showed significant increases in CBF after surgery.

CONCLUSIONS

Untreated patients with sCS < 1 year old have lower CBF than control subjects. Following vault expansion, and with age, CBF in these patients normalizes to that of control subjects, but the usual physiological peak in CBF in childhood occurs later than expected.

ABBREVIATIONS ANCOVA = analysis of covariance; ASL = arterial spin labeling; CBF = cerebral blood flow; ICH = intracranial hypertension; ICP = intracranial pressure; MANCOVA = multivariate ANCOVA; PLD = postlabeling delay; ROI = region of interest; sCS = syndromic craniosynostosis; VP = ventriculoperitoneal.

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Contributor Notes

Correspondence Priya N. Doerga: Sophia Children’s Hospital, Dutch Craniofacial Center, Erasmus MC, University Medical Center Rotterdam, The Netherlands. p.doerga@erasmusmc.nl.

INCLUDE WHEN CITING Published online December 27, 2019; DOI: 10.3171/2019.10.PEDS19150.

Disclosures Dr. Hernandez-Tamames received support of non–study-related clinical or research effort from GE Healthcare. This study was funded by financial support provided by Stichting Hoofdzaak, a foundation that collects donations for research for patients with craniofacial disorders; they had no involvement in any aspect of the study.

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