OBJECTIVE In addition to craniocerebral disproportion, other factors, such as Chiari malformation type I, obstructive sleep apnea, and venous outflow obstruction, are considered to have a role in the occurrence of intracranial hypertension in craniosynostosis. This pilot study examined cerebral venous flow velocity to better characterize the complex intracranial venous physiology of craniosynostosis. METHODS The authors performed a prospective cohort study of craniosynostosis patients (n = 34) referred to a single national (tertiary) craniofacial unit. Controls (n = 28) consisted of children who were referred to the unit's outpatient clinic and did not have craniosynostosis. Transfontanelle ultrasound scans with venous Doppler flow velocity assessment were performed at the first outpatient clinic visit and after each surgery, if applicable. Mean venous blood flow velocities of the internal cerebral vein (ICVv) and the superior sagittal sinus (SSSv) were recorded and blood flow waveform was scored. RESULTS Preoperatively, SSSv was decreased in craniosynostosis patients compared with controls (7.57 vs 11.31 cm/ sec, p = 0.009). ICVv did not differ significantly between patients and controls. Postoperatively, SSSv increased significantly (7.99 vs 10.66 cm/sec, p = 0.023). Blood flow waveform analyses did not differ significantly between patients and controls. CONCLUSIONS Premature closure of cranial sutures was associated with decreased SSSv but not ICVv; indicating an effect on the superficial rather than deep venous drainage. Further Doppler ultrasound studies are needed to test the hypothesis that at an early stage of craniosynostosis pathology SSSv, but not pulsatility, is abnormal, and that abnormality in both SSSv and the superficial venous waveform reflect a more advanced stage of evolution in suture closure.

Cerebral venous drainage, Craniofacial, Craniosynostosis, Doppler ultrasound, Intracranial hypertension, Superior sagittal sinus
dx.doi.org/10.3171/2018.1.PEDS17480, hdl.handle.net/1765/108768
Journal of Neurosurgery: Pediatrics
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Cornelissen, M.J, de Goederen, R, Doerga, P.N, Cuperus, I. (Iris), van Veelen-Vincent, M.L.C, Leguin, M, … Tasker, R.C. (2018). Pilot study of intracranial venous physiology in craniosynostosis. In Journal of Neurosurgery: Pediatrics (Vol. 21, pp. 626–631). doi:10.3171/2018.1.PEDS17480