Surgical management of severe cervical kyphosis with myelopathy in osteogenesis imperfecta - A case report

Study Design. A case of a 9-year-old child with Osteogenesis Imperfecta and severe cervical kyphosis associated with wedged vertebrae and progressive neurological deterioration is presented. Objective. To highlight the difficulties in surgical management of this condition and to discuss the appropriate surgical approach. Summary of Background Data. This case demonstrates an unusual case of Osteogenesis Imperfecta with associated wedged vertebrae causing a quadriparesis. Surgical decompression and stabilization can be performed with resolution of symptoms even in this age group with the appropriate approach and implants. 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. Radiological and laboratory investigations confirmed the diagnosis of Osteogenesis Imperfecta, and radiographs of the cervical spine revealed a kyphotic deformity of 120 degrees. Magnetic resonance imaging and computerized tomography scans showed anterior cord compression attributable to wedged vertebrae at C3 and C4. Magnetic resonance imaging-angiography was performed before surgery to identify the anatomic position of the vertebral arteries. A modified anterolateral approach to the upper cervical spine was performed, and anterior C3 and C4 corpectomies with interbody cage and plate fixation were carried out. Results. After surgery the patient made a full neurological recovery, and significant correction of the deformity was achieved and maintained at follow-up. Conclusions. Cervical kyphotic deformity in Osteogenesis Imperfecta is uncommon. Association of this condition with wedged vertebrae is rare. Surgical decompression of the upper cervical spine is a challenging problem in the presence of this deformity. Which surgical approach to use is controversial. There are difficulties exposing wedged vertebrae by a standard anterior approach, and hence we have used a modified anterolateral approach to address this surgical problem, because a posterolateral approach was impossible with the intervening vertebral arteries. Spinal stabilization in children with Osteogenesis Imperfecta and poor bone stock is a challenge. We have used a small diameter MOSS cage ("Harms mesh cage") with maxillofacial plate and screws to achieve stabilization and fusion.