Evidence-Based Recommendations on the Workup and Treatment Options for Inflammatory Pathologies of the Sella and Orbit: A Systematic Review

Background: Inflammatory pathologies of the sella and orbit are rare but require prompt diagnosis to initiate effective treatment and avoid long term complications. Diagnosis and treatment of these pathologies has largely been guided by a clinician's personal experience as clear, uniform guidelines have yet to be established.

Objective: Identify evidence-based recommendations for the treatment of inflammatory pathologies of the sell and orbit

Methods: PRISMA guidelines were utilized to screen inflammatory pathologies of the orbit and sella. Clinical trials, large case series, or retrospective/prospective cohort studies that involved clinical treatment and associated follow-up were included. Narrative reviews, case reports, preclinical studies, as well as studies focusing on trauma, infection, or inappropriate pathology were excluded.

Results: A total of 169 studies were included and organized by disease pathology. Treatments for specific pathologies, such as surgery, radiation, steroids, targeted treatments, immunomodulators, intravenous immune globulin [JA1], and plasmapheresis, were noted and included ([Fig. 1]). Steroids were the most often employed treatment; studies varied on 2nd line management options as well as timing. Outcomes measured by the studies included improvement in neurological, endocrinological, radiological, visual and functional improvement, and relapse rates. Pathological diagnosis highly impacted treatment selection which allowed for a treatment guideline to be generated ([Fig. 2]). Level 3 evidence was present for most studies except for 13 trials in neuromyelitis optica with level 1 or 2 evidence.

Conclusion: This is the first evidence-based review on treatment recommendations for inflammatory pathologies of the orbit and sella. The presentation and workup of many of these pathologies is often homogeneous, adding to the diagnostic complexity of these cases. We present one of the first reviews to consolidate novel and effective treatments for these rare pathologies as well as a general workup ([Fig. 2]). Trial endpoints, treatment length and dose as well as patient selection varied widely across the studies in this review proving generalization of the findings difficult. The reported data may be useful to help guide future randomized clinical trials and provide a resource for clinical management decisions based on the available evidence. Given that several pathologies included in this review stem from extracranial primary disease, future guidelines should also include appropriate screening and prevention in those with a primary inflammatory pathology outside of the skull base. Future guidelines will aim to improve on our findings to guide treatment across the multidisciplinary team involved in these challenging cases.

Publication History

Article published online:
01 February 2023