Abstract
Background: A 9- year-old child with osteogenesis imperfecta and severe cervical kyphosis associated with wedged vertebrae and progressive neurological deterioration is presented. There is no report of upper cervical kyphosis associated with wedged vertebrae in osteogenesis imperfecta in the literature. We discuss the methods and difficulties in the surgical management of this condition and to highlight the appropriate surgical approach.
Methods: Methods:A 9-year-old girl presented with progressive cervical kyphosis and quadriparesis. At the age of 3 years she underwent posterior cervical fusion (C1–C6) for instability and deformity. Radiological and laboratory investigations confirmed the diagnosis of osteogenesis imperfecta. Radiographs of the cervical spine revealed a kyphotic deformity of 120° Magnetic Resonance Imaging (MRI) and Computerised Tomography (CT) scans showed anterior cord compression due to wedged vertebrae at C3 and C4. MRI-Angiography was performed pre-operatively to identify the anatomical position of the vertebral arteries. A modified anterolateral approach to the upper cervical spine was performed. Anterior C3 and C4 corpectomies with interbody fusion with cage and plate fixation was carried out.
Results: Postoperatively the patient made a full neurological recovery and significant correction of the deformity was achieved and correction was maintained at final follow-up.
Conclusion: Cervical kyphotic deformity in Osteogenesis Imperfecta is uncommon. Association of this condition with wedged vertebrae is rare. Surgical decompression of the upper cervical spine with severe kyphosis is a challenging problem. Which surgical approach should be used is controversial? There are difficulties exposing wedged vertebrae by a standard anterior or chin split approach to perform vertebrectomy. Costo-transversectomy has been used successfully in patients with Gibbous deformity in the thoracic spine but due the presence of vertebral artery in the cervical spine posterolateral approach is impossible. We have used a modified anterolateral approach to overcome this problem. Spinal stabilisation in children with Osteogenesis Imperfecta and poor bone quality is another challenge. We have used a small diameter MOSS cage with maxillofacial plate and screws to achieve stabilisation and fusion. The purpose of this report is to highlight the importance of diagnosis of progressive cervical kyphotic deformity in children with osteogenesis imperfecta and also to describe the difficulties encountered with surgical management of this condition.
The abstracts were prepared by Mr Colin E. Bruce. Correspondence should be addressed to Colin E. Bruce, Consultant Orthopaedic Surgeon, Alder Hey Children’s Hospital, Eaton Road, Liverpool, L12 2AP.
