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

J Neurosurg Pediatr. 2012 Feb;9(2):111-5. doi: 10.3171/2011.11.PEDS11268.

Abstract

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.

Publication types

  • Case Reports

MeSH terms

  • Arnold-Chiari Malformation / complications
  • Arnold-Chiari Malformation / pathology
  • Arnold-Chiari Malformation / surgery*
  • Craniofacial Dysostosis / complications
  • Craniosynostoses / complications
  • Craniosynostoses / pathology
  • Craniosynostoses / surgery*
  • Craniotomy
  • Decompression, Surgical / methods*
  • Humans
  • Image Processing, Computer-Assisted
  • Infant
  • Magnetic Resonance Imaging
  • Male
  • Neurosurgical Procedures / methods*
  • Plastic Surgery Procedures
  • Postoperative Care
  • Skull / pathology
  • Skull / surgery*
  • Spine / pathology
  • Tomography, X-Ray Computed