Idiopathic spinal cord herniation: Clinical review and report of three cases

Abstract

Idiopathic spinal cord herniation (ISCH) is a rare condition, of unknown pathogenesis, that primarily affects the thoracic spinal cord. It is characterized by ventral displacement of the spinal cord through a dural defect. The aim of this study was to review the literature and to present a retrospective single center experience with three cases of ISCH. The literature review analyzed a total of 78 relevant publications on ISCH, which included a total of 171 patients, supplemented with 3 patients treated at our institution. Numerous case reports have demonstrated improvement in clinical outcomes after surgery; however, follow-up is predominantly short, data are incomplete, the condition is frequently misdiagnosed, and it is difficult to predict which patients will benefit from surgery. We identified 159 cases treated with surgical management. The mean symptom duration was 54 months, and the mean follow-up 33 months. The result at follow-up was improved neurological outcome in 74%, unchanged result in 18%, and worse outcome in 8%. There were 15 cases of conservative management, with mean symptom duration 52 months and mean follow-up 33 months. The neurological outcome was unchanged in 100%. ISCH is a rare condition causing progressive thoracic myelopathy, and the natural history is unknown. There is a lack of evidence-based treatment strategies, and the majority of cases are treated with surgical management at diagnosis. Management of ISCH needs to be individualized for each patient, and clinicians should be encouraged to report new cases, standardize case reports, and ensure long-term follow-up.