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Clinical Neuroscience News, June 2013

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Case Study: Chiari 1 Malformation with Syringomyelia

Harold L. Rekate, MD, FACS, FAAP and
Salvatore Insinga, DO


A 13-year-old female with Ehlers-Danlos syndrome, an inherited disorder of connective tissue, came to us for evaluation after undergoing two failed operations for Chiari I malformation with syringomyelia at an out-of-state institution. The child presented with incapacitating headache, neck pain and loss of balance that confined her to a wheelchair. Through advanced imaging and other diagnostic tests, we identified a pseudomeningocele and an anterior compression of the brainstem due to severe instability of the cervical spine in the regions of C1, C2 and C3.

Our team used a combined procedure to correct the existing defects. In the first stage, the anesthetized patient was put in a Mayfield head holder under mild flexion while she underwent a posterior fossa decompression revision. Several holes in the dural patch attributed to the earlier craniectomies were found to be leaking spinal fluid and were closed. At this point intraoperative ultrasound was brought in, revealing that the anterior brainstem was being distorted by the odontoid process, significantly reducing ventral CSF flow. We then performed an occipitocervical fusion. Adjusting the head holder, the patient’s neck was put in significant extension. When we determined that sufficient space was opened between the brainstem and the odontoid process to achieve decompression, the fusion was completed.

The patient’s recovery was uneventful and on the fifth day, postoperatively she was discharged home. Beginning physical therapy six weeks later, she subsequently improved to the point that she was able to return to school and resume normal activities.

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Case Study: A.D.

Preoperative sagittal cervical MRI showing basilar invagination

Case Study: A.D.

X-ray of the cervical spine showing the instruments used to fix the position of the skull and spine to prevent damage to the front of the brain

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