Neurofibromatosis and Dural Ectasia in Teen

Description

This patient is a teenage male with neurofibromatosis (a nervous system disorder characterized by tumors developing from the nerve tissue) who had very substantial upper thoracic back pain. He is neurologically intact. He has dural ectasia, which is expansion and proliferation of the dura (the protective covering of the spinal cord) that eats up surrounding bone and causes deformities. He has a spontaneous dislocation at T4-T5.

On the MRI and CT scan slices, you are looking down the spinal canal of T4. T4 is completed dislocated onto T5. Some surgeons considered a very radical and risky vertebral column resection, which would have been an unnecessarily risky and bloody operation.

Treatment

Dr. Bridwell treated the patient with halo traction for several weeks to partially correct the dislocation. Dr. Bridwell then performed a posterior instrumented fusion from mid-cervical to the thoracic spine. The dural ectasia destroyed much of his fixation points (places where Dr. Bridwell could insert screws) in the thoracic spine, so Dr. Bridwell used fixation points above and below the thoracic spine.

Result

At 5-years post-op, he is pain free and doing well. On the clinical photos, you can see the substantial clinical deformity pre-op.

Note on the Pre-op to Post-op Comparison questionnaire scores that for the pain, function, and mental health questions, the maximum score is 25; for the self-image question, the maximum score is 30; and for the satisfaction question, the maximum score is 10. For the SRS Scores Converted to 100-point Scale, 100 means no pathology.