CLINICAL ARTICLE J Neurosurg Pediatr 21:626–631, 2018 C raniosynostosis occurs in approximately 1 in 1500 births and results in abnormal shape of the crani- um and increased risk of intracranial hypertension (ICHT). 1 In published series, the prevalence of ICHT rang- es from 1% to 85%, and it is particularly high in syndromic cases of craniosynostosis. 2,3,13,17 Historically, the develop- ment of ICHT in craniosynostosis was attributed solely to skull growth restriction (i.e., craniocerebral dispropor- tion). 18 Now, however, the accumulated evidence suggests that other factors may also be relevant, 5,6,8,9,19 including: cranial vault venous outfow obstruction, ventriculomeg- aly (or hydrocephalus if progressive), tonsillar herniation or presence of Chiari malformation type I, and obstructive sleep apnea (OSA). These pathophysiological features are ABBREVIATIONS CSF = cerebrospinal fluid; ICHT = intracranial hypertension; ICP = intracranial pressure; ICV = internal cerebral vein; ICV v = mean venous blood flow velocity of the ICV; MANOVA = multivariate analysis of variance; OFC = occipitofrontal head circumference; OSA = obstructive sleep apnea; P SSS = SSS pressure; SSS = superior sagittal sinus; SSS v = mean venous blood flow velocity of the SSS. SUBMITTED August 27, 2017. ACCEPTED January 11, 2018. INCLUDE WHEN CITING Published online April 6, 2018; DOI: 10.3171/2018.1.PEDS17480. Pilot study of intracranial venous physiology in craniosynostosis Martijn J. Cornelissen, MD, PhD, 1 Robbin de Goederen, MD, 1 Priya Doerga, MD, 1 Iris Cuperus, BSc, 1 Marie-Lise van Veelen, MD, PhD, 2 Maarten Lequin, MD, PhD, 3 Paul Govaert, MD, PhD, 4 Irene M. J. Mathijssen, MD, PhD, MBA-H, 1 Jeroen Dudink, MD, PhD, 5 and Robert C. Tasker, MA, MD, FRCP(UK) 6 Departments of 1 Plastic and Reconstructive Surgery and Hand Surgery and 2 Neurosurgery, Sophia Children’s Hospital, Erasmus MC, University Medical Center, Rotterdam; 3 Department of Radiology and 5 Neonatology Division, Department of Pediatrics, Wilhelmina Children’s Hospital, University Medical Center, Utrecht, The Netherlands; 4 Neonatology Division, Department of Pediatrics, Koningin Paola Children’s Hospital, Antwerp, Belgium; and 6 Departments of Neurology and Anesthesia (Pediatrics), Harvard Medical School, Boston Children’s Hospital, Boston, Massachusetts OBJECTIVE In addition to craniocerebral disproportion, other factors, such as Chiari malformation type I, obstructive sleep apnea, and venous outfow obstruction, are considered to have a role in the occurrence of intracranial hyperten- sion in craniosynostosis. This pilot study examined cerebral venous fow 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 fow velocity assessment were performed at the frst outpatient clinic visit and after each surgery, if applicable. Mean venous blood fow velocities of the internal cerebral vein (ICV v ) and the superior sagittal sinus (SSS v ) were recorded and blood fow waveform was scored. RESULTS Preoperatively, SSS v was decreased in craniosynostosis patients compared with controls (7.57 vs 11.31 cm/ sec, p = 0.009). ICV v did not differ signifcantly between patients and controls. Postoperatively, SSS v increased signif- cantly (7.99 vs 10.66 cm/sec, p = 0.023). Blood fow waveform analyses did not differ signifcantly between patients and controls. CONCLUSIONS Premature closure of cranial sutures was associated with decreased SSS v but not ICV v ; indicating an effect on the super fcial rather than deep venous drainage. Further Doppler ultrasound studies are needed to test the hypothesis that at an early stage of craniosynostosis pathology SSS v , but not pulsatility, is abnormal, and that abnormal- ity in both SSS v and the superfcial venous waveform refect a more advanced stage of evolution in suture closure. https://thejns.org/doi/abs/10.3171/2018.1.PEDS17480 KEYWORDS intracranial hypertension; craniosynostosis; Doppler ultrasound; superior sagittal sinus; cerebral venous drainage; craniofacial J Neurosurg Pediatr Volume 21 • June 2018 626 ©AANS 2018, except where prohibited by US copyright law Unauthenticated | Downloaded 05/16/22 09:10 PM UTC