title: Arnold Chiari Malformation With Sponastrime (Spondylar and Nasal Changes, With Striations of the Metaphyses) Dysplasia: A Case Report.
abstract: SPOndylar and NAsal changes, with STRIations of the Metaphyses (SPONASTRIME) dysplasia (SD) is a dwarfing autosomal recessive syndrome, characterized by a variety of clinical and radiographic features, which form the basis for diagnosis. We describe the presentation of an Arnold Chiari malformation in a patient with a clinical diagnosis of SD. The malformation was successfully treated by decompression of the foramen magnum and elevation of the cerebellum, with complete resolution of pain.We report a rare case of Arnold Chiari malformation in a patient presenting with clinical and radiographic features strongly suggestive of SD and be successfully treated.
authors.affiliation: From the Department of Neurosurgery (JHJ, S-BI), Department of Radiology (ALL), Soonchunhyang University Bucheon Hospital, Bucheon, Korea; and Department of Pediatrics (SYC, DKJ), Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
authors.forename: Je Hoon
authors.initials: JH
authors.lastname: Jeong
authors.forename: A Leum
authors.initials: AL
authors.lastname: Lee
authors.forename: Sung Yoon
authors.initials: SY
authors.lastname: Cho
authors.forename: Dong Kyu
authors.initials: DK
authors.lastname: Jin
authors.forename: Soo-Bin
authors.initials: SB
authors.lastname: Im
mesh_terms: D000328:Adult; D001139:Arnold-Chiari Malformation / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery; D002574:Cervical Vertebrae / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000601:surgery; D019299:Decompression, Surgical / Q000379:methods*; D005539:Foramen Magnum / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000601:surgery; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D010009:Osteochondrodysplasias / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery; D011859:Radiography / Q000379:methods; D013116:Spinal Cord / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000601:surgery; D013595:Syringomyelia / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery; D016896:Treatment Outcome