title: Resolution of syndromic craniosynostosis-associated Chiari malformation Type I without suboccipital decompression after posterior cranial vault release.
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.
authors.affiliation: Department of Neurosurgery, University of Washington School of Medicine, Seattle Children’s Hospital, Seattle, Washington 98105, USA.
authors.forename: Michael R
authors.initials: MR
authors.lastname: Levitt
authors.forename: Toba N
authors.initials: TN
authors.lastname: Niazi
authors.forename: Richard A
authors.initials: RA
authors.lastname: Hopper
authors.forename: Richard G
authors.initials: RG
authors.lastname: Ellenbogen
authors.forename: Jeffrey G
authors.initials: JG
authors.lastname: Ojemann
mesh_terms: D001139:Arnold-Chiari Malformation / Q000150:complications / Q000473:pathology / Q000601:surgery*; D003394:Craniofacial Dysostosis / Q000150:complications; D003398:Craniosynostoses / Q000150:complications / Q000473:pathology / Q000601:surgery*; D003399:Craniotomy; D019299:Decompression, Surgical / Q000379:methods*; D006801:Humans; D007091:Image Processing, Computer-Assisted; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D019635:Neurosurgical Procedures / Q000379:methods*; D011182:Postoperative Care; D019651:Plastic Surgery Procedures; D012886:Skull / Q000473:pathology / Q000601:surgery*; D013131:Spine / Q000473:pathology; D014057:Tomography, X-Ray Computed