• Jonathan Poggi, Wendy S Chen, Helena O Taylor, Steven Sullivan, Albert S Woo, and Petra M Klinge.
• Department of Neurosurgery, Rhode Island Hospital, The Warren Alpert School of Medicine, Brown University, Rhode Island, USA.
• World Neurosurg. 2020 Nov 1; 143: 158-162.

BackgroundWhile commonly seen in syndromic craniosynostosis, the incidence of Chiari malformation (CM) in nonsyndromic craniosynostosis has been reported at 5% and there is a lack of understanding of the pathophysiology and management of CM in this patient population.Case DescriptionWe present a 5-year-old male patient who underwent a sagittal craniosynostosis repair at the age of 5 months and returned at the age of 5 years with daily headaches associated with behavioral changes. He was found to have pan-synostoses and radiographic evidence of increased intracranial pressure, including a Chiari malformation. Neurologic and genetic workup was unremarkable. A cranial vault reconstruction was performed, and subsequent imaging demonstrated resolution of previously noted Chiari malformation.ConclusionsIn our case, we provided a unique window into the underlying pathophysiology for CM in patients with concurrent nonsyndromic craniosynostosis that we hope will add to the current foundation of literature supporting the intricate relation between cranial vault compliance and Chiari malformation or hindbrain herniation. Furthermore, we provide insight into the management of acquired CM and support isolated cranial vault reconstruction in those who do not appear to have symptomatic suboccipital compression.Copyright © 2020 Elsevier Inc. All rights reserved.

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