Problems in Instrumentation of Syndromic Craniovertebral Junction Anomalies – Case Reports

The aim of this study is to highlight the complications of instrumentation in the setting of syndromic craniovertebral junction (CVJ) anomalies. The records of patients with syndromic CVJ anomalies treated by this author during the period of 2012–2017 were retrospectively reviewed. Patients in whom intraoperative difficulties and complications were encountered were culled out from the database. Complications were divided into (1) technique related, (2) neural injury, (3) vascular injury, (4) instrumentation pull out/breakage, (5) inaccurate screw placement and, (6) where postoperatively, the surgeon felt an alternate surgical technique could have yielded better results. Four patients with either unexpected intraoperative difficulties or complications or in whom the technique could have been refined were identified. There were 2 patients with proatlas segmentation anomalies and 2 with Morquio’s-Brailsford disease. The first patient had cage migration which necessitated a second procedure during craniovertebral realignment, the second had partial penetration of the screw into the transverse foramen, the third with bipartite atlas underwent a C1–2 fixation without a horizontal cross-connector and, the fourth had screw pull outs from the subaxial cervical spine intraoperatively during an attempted occipitocervical fusion. In children with syndromic CVJ anomalies, the surgeon should be aware of the high risk of intraoperative difficulties and complications. Potential pitfalls and the ways to avoid these complications are discussed.