Spinal Idiopathic Hypertrophic Pachymeningitis: A Systematic Review of Diagnostic Features, Clinical Management, and...

To comprehensively review the diagnostic features, clinical management, and surgical outcomes of spinal idiopathic hypertrophic pachymeningitis (sIHP) through a systematic analysis of existing literature.

A systematic review was conducted using PubMed, EMBASE, Web of Science, and Cochrane databases from inception to October 31, 2024. Studies reporting on adult patients with sIHP, including clinical, treatment, outcome, and follow-up data, were included. Data extraction covered patient demographics, clinical presentation, diagnostic workup, management strategies, and outcomes.

The review included 29 studies, comprising 8 case series and 21 case reports, with a total of 43 patients. The median age at diagnosis was 54 years, with a slight female predominance (53.5%). Common presenting symptoms included lower extremity weakness (51.2%), sensory complaints (48.8%), and back pain (46.5%). Magnetic resonance imaging was the primary diagnostic tool (86% of cases). Histopathological findings typically showed inflammatory infiltrate (74.4%) and fibrosis (59%). Steroid therapy was the primary medical management, with surgical decompression often necessary. The recurrence rate was 27.6%, with a median time to recurrence of 5 months.

sIHP presents unique diagnostic and management challenges compared to its cranial counterpart. While sharing similarities in demographic patterns and diagnostic approaches, sIHP often requires more aggressive initial management due to the risk of irreversible neurological damage from spinal cord compression. The findings underscore the need to consider sIHP in the differential for patients with progressive myelopathic symptoms without signs of neoplasm.