title: Treatment of Basilar Invagination With Klippel-Feil Syndrome: Atlantoaxial Joint Distraction and Fixation With Transoral Atlantoaxial Reduction Plate.
abstract: BACKGROUND
  Klippel-Feil syndrome (KFS) is characterized by congenital vertebral fusion of the cervical spine and a wide spectrum of associated anomalies. In patients with KFS with basilar invagination (BI), compression of the brainstem and upper cervical cord results in neurological deficits, and decompression and occipitocervical reconstruction are required. The highly varied anatomy of KFS makes a posterior occipitocervical fixation strategy challenging. For these patients, the transoral atlantoaxial reduction plate (TARP) operation is an optimal option to perform a direct anterior fixation to achieve stabilization.
  
  
  OBJECTIVE
  To evaluate the effectiveness of TARP internal fixation for the treatment of BI with KFS.
  
  
  METHODS
  Ten consecutive patients with BI and KFS who underwent TARP reduction and fixation from 2010 to 2012 were reviewed. Clinical assessment and image measurements were performed preoperatively and at the most recent follow-up. Nine patients (9/10) were followed for an average of 31.44 months.
  
  
  RESULTS
  Symptoms were alleviated in 9 of 9 patients (100.00%). The odontoid process was ideally corrected with the TARP system. The mean clivus canal angle improved from 124° preoperatively to 152° postoperatively. The average preoperative and postoperative Japanese Orthopedic Association scores were 10.56 (n = 9) and 14.67 (n = 9), respectively, indicating 63.82% improvement. There was bony bridge catenation on the computed tomography scans and no evidence of hardware failure at 6 months.
  
  
  CONCLUSIONS
  The TARP operation is effective and safe for treating patients with BI with KFS. The midterm clinical results were satisfactory.
authors.affiliation: *Southern Medical University, Guangzhou, China;‡Department of Orthopedics, Guangzhou General Hospital of Guangzhou Military Command, Guangzhou, China;§Institute of Traumatic Orthopaedics of People's Liberation Army, Guangzhou, China;¶No. 303 Hospital of People's Liberation Army, Nanning, China.
authors.forename: Gejin
authors.initials: G
authors.lastname: Wei
authors.forename: Zhiyun
authors.initials: Z
authors.lastname: Wang
authors.forename: Fuzhi
authors.initials: F
authors.lastname: Ai
authors.forename: Qingshui
authors.initials: Q
authors.lastname: Yin
authors.forename: Zenghui
authors.initials: Z
authors.lastname: Wu
authors.forename: Xiang Yang
authors.initials: XY
authors.lastname: Ma
authors.forename: Junjie
authors.initials: J
authors.lastname: Xu
authors.forename: Chenglong
authors.initials: C
authors.lastname: Shi
authors.forename: Hong
authors.initials: H
authors.lastname: Xia
mesh_terms: D000293:Adolescent; D000328:Adult; D001268:Atlanto-Axial Joint / Q000601:surgery*; D001860:Bone Plates; D016025:Bone Transplantation; D002648:Child; D003388:Cranial Fossa, Posterior / Q000601:surgery; D019299:Decompression, Surgical; D005260:Female; D006801:Humans; D007091:Image Processing, Computer-Assisted; D016268:Internal Fixators; D007714:Klippel-Feil Syndrome / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D009777:Occipital Bone / Q000002:abnormalities* / Q000601:surgery*; D009809:Odontoid Process / Q000601:surgery; D011182:Postoperative Care; D011300:Preoperative Care; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D055815:Young Adult