title: Surgical treatment of Klippel-Feil syndrome with basilar invagination.
abstract: BACKGROUND
  Klippel-Feil syndrome (KFS) is a congenital cervical vertebral union caused by a failure of segmentation during abnormal development and frequently accompanies conditions such as basicranial malformation, atlas assimilation, or dens malformation. Especially in basilar invagination (BI), which is a dislocation of the dens in an upper direction, compression of the spinomedullary junction from the ventral side results in paralysis, and treatment is required.
  
  
  METHODS
  We present the case of a 38-year-old male patient with KFS and severe BI. Plane radiographs and computed tomography (CT) images showed severe BI, and magnetic resonance image (MRI) revealed spinal cord compression caused by invagination of the dens into the foramen magnum and atlantoaxial subluxation. Reduction by halo vest and skeletal traction were not successful. Occipitocervical fusion along with decompression of the foramen magnum, C1 laminectomy, and reduction using instruments were performed. Paralysis was temporarily aggravated and then gradually improved. Unsupported walking was achieved 24 months after surgery, and activities of daily life could be independently performed at the same time. CT and MRI revealed that dramatic reduction of vertical subluxation and spinal cord decompression were achieved.
  
  
  CONCLUSIONS
  Reduction and internal fixation using instrumentation are effective techniques for KFS with BI; however, caution should be exercised because of the possibility of paralysis caused by intraoperative reduction.
authors.affiliation: Department of Orthopaedic Surgery, School of Medicine, Shinshu University, 3-1-1 Asahi, Matsumoto-city, Nagano, 390-8621, Japan. ogiogi@kif.biglobe.ne.jp
authors.forename: Nobuhide
authors.initials: N
authors.lastname: Ogihara
authors.forename: Jun
authors.initials: J
authors.lastname: Takahashi
authors.forename: Hiroki
authors.initials: H
authors.lastname: Hirabayashi
authors.forename: Keijoro
authors.initials: K
authors.lastname: Mukaiyama
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Kato
mesh_terms: D000328:Adult; D019299:Decompression, Surgical / Q000379:methods; D006801:Humans; D007714:Klippel-Feil Syndrome / Q000150:complications / Q000601:surgery*; D008297:Male; D009809:Odontoid Process / Q000002:abnormalities* / Q000601:surgery*; D019637:Orthopedic Procedures / Q000379:methods*; D010985:Platybasia / Q000150:complications / Q000601:surgery*; D013117:Spinal Cord Compression / Q000601:surgery