Successful management of spinal cord ischemia in a pediatric patient with fibrocartilaginous embolism: illustrative case

Abstract

Background: Fibrocartilaginous embolism (FCE) is a rare cause of ischemic myelopathy that occurs when the material of the nucleus pulposus migrates into vessels supplying the spinal cord. The authors presented a case of pediatric FCE that was successfully managed by adapting evidence-based recommendations used for spinal cord neuroprotection in aortic surgery.

Observations: A 7-year-old boy presented to the emergency department with acute quadriplegia and hemodynamic instability that quickly progressed to cardiac arrest. After stabilization, the patient regained consciousness but remained in a locked-in state with no spontaneous breathing. The patient presented a diagnostic challenge. Traumatic, inflammatory, infectious, and ischemic etiologies were considered. Eventually, the clinical and radiological findings led to the presumed diagnosis of FCE. Treatment with continuous cerebrospinal fluid drainage (CSFD), pulse steroids, and mean arterial pressure augmentation was applied, with subsequent considerable and consistent neurological improvement.

Lessons: The authors proposed consideration of the adaptation of spinal cord neuroprotection principles used routinely in aortic surgery for the management of traumatic spinal cord ischemia (FCE-related in particular), namely, permissive arterial hypertension and CSFD. This is hypothesized to allow for the maintenance of sufficient spinal cord perfusion until adequate physiological blood perfusion is reestablished (remodeling of the collateral arterial network and/or clearing/absorption of the emboli).

Keywords: CSF = cerebrospinal fluid; CSFD = CSF drainage; DWI = diffusion-weighted imaging; FCE = fibrocartilaginous embolism; MAP = mean arterial pressure; MRI = magnetic resonance imaging; SCBF = spinal cord blood flow; SCI = spinal cord ischemia; SCPP = spinal cord perfusion pressure; fibrocartilaginous embolism; spinal cord ischemia; spinal cord perfusion pressure.

FIG. 1.

A and B: MRI sequences obtained after initial presentation. Hyperintensity in T2-weighted sequences (A) as well as diffusion restriction (B, green arrow) can be seen. Those findings suggest an ischemic etiology. The white arrow (A) indicates an annular tear in the C6–C7 intervertebral disc. C and D: Follow-up MRI study (C, 4 months after event; D, 11 months after event). Postischemic changes in the medulla oblongata and the spinal cord are visible.

FIG. 2.

The timeline of the case from arrival to the emergency department to discharge from pediatric intensive care unit. GPT = glucocorticoid pulse therapy; IVIG = intravenous immunoglobulin.

FIG. 3.

Upper: Arterial supply of the cervical spinal cord. The anterior spinal artery supplies the anterior two-thirds of the spinal cord and low medulla. Paired posterior spinal arteries supply the posterior third of the spinal cord. Arterial vasocorona provides delicate anastomoses on the pial surface that send branches supplying the peripheral portions of the lateral funiculi. Lower: Illustration of the arterial mechanism of FCE. During activities that involve elevation of axial load (with subsequent elevated intradiscal pressure), material from the intervertebral disk (depicted in green) travels in a retrograde fashion to the radicular arteries, then anterograde to the arterial system of the spinal cord, thus leading to anterior spinal artery syndrome. The resulting ischemic territory is shaded in red.