Respiratory failure caused by Chiari I malformation with associated syringomyelia

The authors report a case of a 38-year-old male with a Chiari I malformation and syringomyelia that presented with acute respiratory distress. Pulmonary function testing was consistent with respiratory muscle weakness. A suboccipital craniectomy and C1, 2, 3 laminectomies were performed on an emergent basis. Postoperatively the patient had significant improvement in his respiratory status associated with a reduction in the size of the syringomyelia on follow-up imaging.