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Occipitocervical Fusion in Skeletal Dysplasia: A New Surgical Technique

Sitoula, Prakash MBBS, MS (Ortho); Mackenzie, William G. MD; Shah, Suken A. MD; Thacker, Mihir MD; Ditro, Colleen APN; Holmes, Laurens Jr PhD, DrPH; Campbell, Jeffrey W. MD; Rogers, Kenneth J. PhD, ATC

doi: 10.1097/BRS.0000000000000381

Study Design. Retrospective cohort study.

Objective. This study describes clinical and radiological results of a new cable technique for occipitocervical fusion (OCF) in children with skeletal dysplasia (SD).

Summary of Background Data. Anatomical variability and poor bone quality make upper cervical surgery technically challenging in patients with SD. We present a new cable technique for OCF in children with SD when the posterior elements are not of a size or quality for other types of instrumentation.

Methods. Retrospective review of 24 patients with SD (8 boys, 16 girls) who underwent OCF between 2001 and 2011. In this technique, cables provide compression across a bone graft that is prevented from entering the canal and the graft resists excessive lordosis. Demographic and radiographical data are presented. All patients were followed for initial outcomes of surgery, and 20 patients (83%) were followed for 2 years or more for mid- and long-term outcomes.

Results. Mean age at surgery was 6.5 years and mean follow-up was 4.1 ± 2.4 years. This technique was used as a primary procedure in 20 and a revision procedure in 4 patients. Diagnoses included Morquio syndrome (6), spondyloepiphyseal dysplasia (9), spondyloepimetaphyseal dysplasia (5), metatropic dysplasia (3), and Kniest syndrome (1). Ten patients had upper cervical instability and features of cervical myelopathy, and the remaining 14 patients had instability and signal changes on magnetic resonance image. Fusion extended from occiput to C2 in 71% patients, and upper cervical decompression was needed in 92% patients. Postoperatively, all patients were immobilized in a halo vest for mean duration of 12 weeks. Fusion was achieved in all patients. Complications included halo pin-tract infections (7), junctional instability (2), and extension of fusion (4).

Conclusion. This new cable technique is a good alternative for OCF in patients with SD who have altered anatomy at the craniocervical junction not amenable to rigid internal fixation.

Level of Evidence: 4

We describe a new technique for occipitocervical fusion in children with skeletal dysplasia when the posterior elements are not of a size or quality to allow the use of other instrumentation. All 24 patients who were treated with this new technique had successful occipitocervical fusion with minimal complications.

From the Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE.

Address correspondence and reprint requests to William G. Mackenzie, MD, Department of Orthopedics, Nemours/Alfred I. duPont Hospital for Children, Box 269, Wilmington, DE 19899; E-mail:

Acknowledgment date: August 30, 2013. First revision date: December 8, 2013. Acceptance date: December 10, 2013.

The device(s)/drug(s) that is/are the subject of this manuscript is/are not FDA-approved for this indication and is/are not commercially available in the United States.

No funds were received in support of this work.

Relevant financial activities outside the submitted work: board membership, consultancy, grants/grants pending, patents, royalties, travel/accommodations/meeting expenses and stock/stock options.

© 2014 by Lippincott Williams & Wilkins