Chiari malformations in patients with uncorrected sagittal synostosis

Background: Single suture craniosynostoses were long considered a benign condition with little risk of complications. In many cases, parents may forgo surgical correction because of the inherent risks of surgical intervention. Case Description: We report 2 cases of uncorrected sagittal synostosis associated with the development of Chiari malformations with syringomyelia later in life. To our knowledge, this is the first report of symptomatic Chiari malformations associated with uncorrected sagittal synostosis. We discuss mode of presentation and management in each case. We propose that these patients either have a pathophysiological predisposition to Chiari malformations based on the compensatory bone growth pattern in sagittal synostosis resulting in craniocephalic disproportion or that there may be an underlying genetic alteration accounting for the association of the 2 processes. Conclusions: Further investigations are warranted to investigate whether the incidence of Chiari malformations is decreased in patients who have undergone early correction of an isolated sagittal synostosis. We conclude that patients with sagittal synostosis should be evaluated for Chiari malformations before being discharged permanently from a neurosurgical practice.