Idiopathic spinal cord herniation: report of eight cases and review of the literature

Abstract

Study design: A case series of eight patients with idiopathic spinal cord herniation and a review of the literature.

Objective: To report on this rare entity, provide insight on its natural history, and propose an optimal management strategy.

Summary of background data: Idiopathic spinal cord herniation is a rare disease with 50 cases reported before the current study.

Methods: Eight cases (follow-up 1 month to 8 years) are reported using available information from patient charts, interviews, and assessments. All imaging studies are reviewed. The review of the literature was performed using PUBMED.

Results: Four patients, followed without surgical intervention, have not progressed. Of the three patients who underwent surgical repair by one of the authors, two improved and one was unchanged. A fourth patient, who was initially treated by another surgeon who failed to identify the dural defect and herniation, had a poor outcome.

Conclusion: The pathophysiology of the dural defect is still uncertain. The typical presentation is Brown-Séquard syndrome. Microsurgical repair in cases with progression of neurologic deficits is usually successful in achieving recovery of function or arrest of progression.