Systematic Review of Treatment Options and Therapeutic Responses for Lesions of the Sella and Orbit: Evidence-Based Recommendations.

Abstract

Inflammatory pathologies of the sella and orbit are rare but require prompt diagnosis to initiate effective treatment. Because uniform recommendations for treatment are currently lacking, we performed an evidence-based review to identify recommendations. We performed a literature search of the PubMed, Embase, and Web of Science databases to identify papers evaluating treatment of inflammatory pathologies of the sella and orbit. We used PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines to define recommendations, specifically examining aggregated sample sizes, disease-specific patient follow-up, and clinical trials focused on inflammatory diseases of the sella and orbit. A total of 169 studies were included and organized by disease pathology. Treatments for various pathologies were recorded. Treatment options included surgery, radiation, steroids, targeted treatments, immunomodulators, intravenous immune globulin, and plasmapheresis. Steroids were the most often employed treatment, second-line management options and timing varied. Pathological diagnosis was highly associated with treatment used. Most evidence were level 3 without available control groups, except for 13 trials in neuromyelitis optica with level 1 or 2 evidence. This is the first evidence-based review to provide recommendations on specific treatments for pathologies of the orbit and sella. The reported data may be useful to help guide randomized clinical trials and provide resource for clinical management decisions based on the available evidence.