Spinal cord infarction remote from maximal compression in a patient with Morquio syndrome

Case report

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Morquio syndrome, or mucopolysaccharidosis type IV, is a rare enzyme deficiency disorder and results in skeletal dysplasia. Odontoid dysplasia is common among affected patients, resulting in atlantoaxial instability and spinal cord compression. Surgical treatments include decompression and prophylactic fusion, during which intraoperative neuromonitoring is important to alert the surgical team to changes in cord function so that they can prevent or mitigate spinal cord injury. This report describes a 16-year-old girl with Morquio syndrome who developed paraplegia due to thoracic spinal cord infarction during foramen magnum and atlantal decompression. This tragic event demonstrates the following: 1) that patients with Morquio syndrome are at risk for ischemic spinal cord injury at levels remote from areas of maximal anatomical compression while under anesthesia in the prone position, possibly due to impaired cardiac output; 2) the significance of absent motor evoked potential responses in the lower limbs with preserved upper-limb responses in an ambulatory patient; 3) the importance of establishing intraoperative neuromonitoring baseline assessments prior to turning patients to the prone position following induction of anesthesia; and 4) the importance of monitoring cardiac output during prone positioning in patients with chest wall deformity.

Abbreviations used in this paper:MEP = motor evoked potential; MPS = mucopolysaccharidosis; SSEP = somatosensory evoked potential.
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Contributor Notes

Address correspondence to: D. Douglas Cochrane, M.D., F.R.C.S.C., Division of Neurosurgery, Department of Surgery, University of British Columbia, 4480 Oak Street, Vancouver, British Columbia, Canada V6H 3V4. email: dcochrane@cw.bc.ca.Please include this information when citing this paper: DOI: 10.3171/2012.2.PEDS11522.
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