title: Chiari malformation and odontoid panus causing craniovertebral stenosis in a child with Crouzon's syndrome.
abstract: Crouzon's disease is a well-known disorder affecting multiple organ systems, specifically a craniofacial disorder with highly variable penetrance and severity of deformity. Crouzon's patients typically have anomalies of the skull base leading to gross distortion of the cranium and in some cases the cervicocranium. We present a 5-year-old girl with Crouzon's disease who suffered from an acquired Chiari I malformation after insertion of a ventriculoperitoneal shunt and a coexistent ventral odontoid panus. Both these lesions were causing cervicomedullary compression. The literature is controversial on the surgical management of anterior and posterior compression at the craniocervical junction. We review the literature on surgical options for decompression at the craniocervical junction and offer our surgical case as a treatment option for patients in this rare clinical situation.
authors.affiliation: Denton Regional Medical Center and Plano Presbyterian Hospitals, Plano, Texas, USA. drrdd@yahoo.com
authors.forename: Rob D
authors.initials: RD
authors.lastname: Dickerman
authors.forename: Michael
authors.initials: M
authors.lastname: Lefkowitz
authors.forename: Suzanne A
authors.initials: SA
authors.lastname: Arinsburg
authors.forename: Steven J
authors.initials: SJ
authors.lastname: Schneider
mesh_terms: D001139:Arnold-Chiari Malformation / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology*; D002675:Child, Preschool; D003251:Constriction, Pathologic / Q000209:etiology / Q000601:surgery; D003394:Craniofacial Dysostosis / Q000601:surgery*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D009333:Neck / Q000473:pathology* / Q000601:surgery; D009809:Odontoid Process / Q000473:pathology*; D012886:Skull / Q000473:pathology; D013131:Spine / Q000473:pathology; D014057:Tomography, X-Ray Computed; D017287:Ventriculoperitoneal Shunt / Q000009:adverse effects