RESUMO
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.
