Idiopathic spinal cord herniation at two separate zones of the thoracic spine: the first reported case and literature review

Abstract

Background context: Idiopathic spinal cord herniation (ISCH) is a rare cause of progressive myelopathy. Preoperative diagnosis can be made with magnetic resonance imaging (MRI). Many surgical techniques have been applied by various authors, and ISCH is usually reversible by surgical treatment.

Purpose: To present a case of ISCH in two separate zones at two thoracic levels. To our knowledge, this is the first such case to be published in English literature. We also discuss the clinical findings, surgical procedures, and surgical outcomes for other previously reported cases of ISCH in the literature.

Study design: Case report.

Methods: A 52-year-old woman with bilateral lower extremity weakness underwent thoracic MRI, which revealed transdural spinal cord herniation at two separate zones, namely, the T4-T5 and T5-T6 intervertebral disc levels.

Results: During surgery, the spinal cord was reduced, the two separate dural defects were connected, and the new single defect was restored then reinforced with a thin layer of fascial graft. The posterior dural defect was then closed with interrupted stitches. The patient's neurologic status was characterized by no changing of the preoperative motor status. Follow-up MRI scans showed that the cord was replaced in the dural sac and showed cord hyperintensity in the herniation levels. The patient could move with a cane at the sixth month postoperatively.

Conclusions: Idiopathic spinal cord herniation is a rare clinical condition that should be considered in the differential diagnosis of paraplegia. Although progression of neurologic deficits can be very slow, reduction of the spinal cord and repair of the defect are crucial to stop or reverse the deterioration. The outcome for patients who initially have Brown-Séquard syndrome is significantly better than for patients who presented with spastic paralysis. To our knowledge, this case study represents the first reported instance in which two separate anterior dural defects caused two levels of anterior spinal cord herniation.