Paraplegia after epidural-general anesthesia in a Morquio patient with moderate thoracic spinal stenosis

Abstract

Purpose: We describe an instance in which complete paraplegia was evident immediately postoperatively after apparently uneventful lumbar epidural-general anesthesia in a patient with Morquio Type A syndrome (Morquio A) with moderate thoracic spinal stenosis.

Clinical features: A 16-yr-old male with Morquio A received lumbar epidural-general anesthesia for bilateral distal femoral osteotomies. Preoperative imaging had revealed a stable cervical spine and moderate thoracic spinal stenosis with a mild degree of spinal cord compression. Systolic blood pressure (BP) was maintained within 20% of the pre-anesthetic baseline value. The patient sustained a severe thoracic spinal cord infarction. The epidural anesthetic contributed to considerable delay in the recognition of the diagnosis of paraplegia.

Conclusion: This experience leads us to suggest that, in patients with Morquio A, it may be prudent to avoid the use of epidural anesthesia without very firm indication, to support BP at or near baseline levels in the presence of even moderate spinal stenosis, and to avoid flexion or extension of the spinal column in intraoperative positioning. If the spinal cord/column status is unknown or if the patient is known to have any degree of spinal stenosis, we suggest that the same rigorous BP support practices that are typically applied in other patients with severe spinal stenosis, especially stenosis with myelomalacia, should apply to patients with Morquio A and that spinal cord neurophysiological monitoring should be employed. In the event that cord imaging is not available, e.g., emergency procedures, it would be prudent to assume the presence of spinal stenosis.

Fig. 1

A Sagittal T2/STIR-weighted image of the thoracic and lumbar spine obtained seven months preoperatively. Characteristic Morquio A syndrome features are evident, including platyspondyly and relatively large intervertebral disks. Obliteration of the CSF space anterior to the spinal column caused by disk protrusion is evident at T3–4 and T4–5 (white arrows). There was similar protrusion at T2–3 (see Fig. 1B), which is not well seen in this image because mild thoracic dextroconvex scoliosis results in variation of the sagittal plane within the image. Increased cord signal indicative of myelomalacia was not evident. The white dot identifies the body of T3. STIR = short T1 inversion recovery; CSF = cerebrospinal fluid. B Sagittal T2/STIR-weighted image of the thoracic spine obtained 21 hours after emergence from anesthesia. The spinal stenosis observed in Fig. 1A is again evident. The bracket identifies the extent of the abnormal T2/STIR “bright signal” consistent with spinal cord infarction and edema. The white dot identifies the body of T3

Fig. 2

Axial T2-weighted image at the level of the T3–4 intervertebral disk obtained seven months preoperatively. There is cerebrospinal fluid (CSF) (white) around the posterior portion of the spinal cord but not the anterior. The anterior surface of the cord has a “tented” rather than a smooth convex configuration, reflecting bilateral ventrolateral pressure on the anterior surface of the cord (solid white arrows). The trachea and the esophagus are identified for orientation