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Chapter 43

Scoliosis and Kyphosis in Osteogenesis Imperfecta

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FIGURE 43.1 MRI images showing the pathology of an OI patient with basilar invagination. (A) Preoperative sagittal T1-weighted image shows evidence of basilar invagination, narrowing of the foramen magnum, and compression of the anterior surface of the medulla oblongata resulting in neurologic symptoms on examination. (B) Preoperative sagittal T2-weighted image demonstrates a multiloculated syringomyelia in the cervical cord extending from C2 through at least C6. (C) Following upper cervical laminectomy with dorsal occipitocervical fusion, this T2-weighted image demonstrates resection of the anterior arch of C1 and partial resection of the body of C2 (odontoid process) with relief of the cord compression.

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FIGURE 43.2 OI patient with spondylolisthesis. One can observe anterior subluxation of L5 on S1. Pedicle screws from the fusion construct are seen in L4.

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FIGURE 43.3 OI patient with severe scoliosis. (A and B) Coronal (A) and sagittal (B) preoperative radiographs show severe scoliosis. In this image, the patient demonstrates right thoracic scoliosis of 68° and left thoracolumbar scoliosis of 48°. (C and D) Coronal (C) and sagittal (D) postoperative radiographs 20 months post-surgery.

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FIGURE 43.4 OI patient with severe scoliosis. The patient was started on cyclic pamidronate (bisphosphonate) therapy prior to surgery. (A and B) Coronal (A) and sagittal (B) preoperative CT show severe scoliosis and low quality bone. The major curve would progress to be 75° at the time of surgery. (C and D) Coronal (C) and sagittal (D) postoperative radiographs 5 months after surgery.