Resolution of syndromic craniosynostosis-associated Chiari malformation Type I without suboccipital decompression after posterior cranial vault release

Case report

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  • 1 Department of Neurosurgery,
  • 2 Division of Plastic Surgery, and
  • 3 Craniofacial Center, University of Washington School of Medicine, Seattle Children's Hospital, Seattle, Washington
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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.

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Contributor Notes

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: jeff.ojemann@seattlechildrens.org.

Please include this information when citing this paper: DOI: 10.3171/2011.11.PEDS11268.

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