Chiari malformation Type I (CM-I) is associated with syndromic and nonsyndromic craniosynostosis in pediatric patients, and the surgical management of CM-I in such cases is controversial. Previous guidelines have recommended simultaneous cranial vault expansion and suboccipital decompression. However, spontaneous resolution of CM-I has been observed, and the combined procedure carries additional surgical risks. The authors report the case of a 6-month-old boy with Crouzon syndrome, CM-I, and a cervical syrinx who underwent posterior cranial vault release without suboccipital decompression. Imaging at the 3-month follow-up visit demonstrated complete resolution of the CM-I, improvement in CSF flow, and reduction in the size of the syrinx. This case suggests that up-front suboccipital decompression may not be necessary in patients with craniosynostosis and CM-I. A strategy of initial cranial vault release, followed by watchful waiting and radiographic surveillance, is proposed.
Abbreviations used in this paper:CM-I = Chiari malformation Type I; ICP = intracranial pressure.
Address correspondence to: Jeffrey G. Ojemann, M.D., Department of Neurological Surgery, Seattle Children's Hospital, 4800 Sand Point Way NE, Seattle, Washington 98105. email: firstname.lastname@example.org.
Please include this information when citing this paper: DOI: 10.3171/2011.11.PEDS11268.
CinalliGRenierDSebagGSainte-RoseCArnaudEPierre-KahnA: Chronic tonsillar herniation in Crouzon's and Apert's syndromes: the role of premature synostosis of the lambdoid suture. J Neurosurg83:575–5821995
SandbergDINavarroRBlanchJRaghebJ: Anomalous venous drainage preventing safe posterior fossa decompression in patients with Chiari malformation Type I and multisutural craniosynostosis. Report of two cases and review of the literature. J Neurosurg106:6 Suppl490–4942007
TaylorWJHaywardRDLasjauniasPBrittoJAThompsonDNJonesBM: Enigma of raised intracranial pressure in patients with complex craniosynostosis: the role of abnormal intracranial venous drainage. J Neurosurg94:377–3852001
ThompsonDNHarknessWJonesBMHaywardRD: Aetiology of herniation of the hindbrain in craniosynostosis. An investigation incorporating intracranial pressure monitoring and magnetic resonance imaging. Pediatr Neurosurg26:288–2951997
TubbsRSEltonSBlountJPOakesWJ: Preliminary observations on the association between simple metopic ridging in children without trigonocephaly and the Chiari I malformation. Pediatr Neurosurg35:136–1392001
WaldauBDomeshekLFLeighFALumKCFuchsHEMarcusJR: Spontaneous resolution of a 13-mm Chiari malformation Type I in relation to differential growth of the posterior fossa volume. Case report. J Neurosurg Pediatr3:110–1142009