title: Foramen magnum decompression and expansile duroplasty for acquired Chiari type I malformation in craniometaphyseal dysplasia.
abstract: Craniometaphyseal dysplasia (CMD) is a rare genetic bone remodelling disorder characterized by undertubulation of the long bones and sclerosis and hyperostosis of the skull base with cranial nerve impairment. To our knowledge there are no previous reported cases of autosomal dominant CMD and acquired Chiari type 1 malformation with cervicomedullary compression. We outline the clinical and radiological features of this condition and describe the surgical management.
authors.affiliation: Department of Neurosurgery, St Bartholomew's & The Royal London Hospitals, London, UK.
authors.forename: M D
authors.initials: MD
authors.lastname: Sewell
authors.forename: H
authors.initials: H
authors.lastname: Akram
authors.forename: J
authors.initials: J
authors.lastname: Wadley
mesh_terms: D000328:Adult; D001139:Arnold-Chiari Malformation / Q000000981:diagnostic imaging / Q000601:surgery*; D015519:Bone Density; D019465:Craniofacial Abnormalities / Q000473:pathology / Q000601:surgery*; D019299:Decompression, Surgical / Q000379:methods*; D003937:Diagnosis, Differential; D005260:Female; D005539:Foramen Magnum / Q000473:pathology / Q000601:surgery*; D006801:Humans; D008279:Magnetic Resonance Imaging; D011859:Radiography; D016896:Treatment Outcome