Purpose: To summarize the clinical profile and response to treatment of 4 biopsy-proven cases of inflammatory orbital pseudotumor extending beyond the orbit.
Design: Retrospective observational case series.
Methods: Four patients' charts were retrospectively reviewed. There were three men with extraorbital extension (two intracranial; one maxillary antrum) and one woman with intracranial disease that extended into the orbit. The men were 40, 41, and 60 years old; the woman was 73 years old.
Results: Two men with orbital myositis and mild discomfort, initially treated with corticosteroids, had asymptomatic intracranial disease 9 and 12 months after initial presentation. A third man had extension into the maxillary antrum after initial symptoms of painless diplopia. The woman had intraorbital disease with minimal discomfort (dacryoadenitis and myositis) 5 years after presenting with intracranial disease in the Meckel cave that subsequently became bilateral. Histopathologic examination in all cases showed nonspecific inflammation without evidence of vasculitis or granulomas.
Conclusions: Unlike typical cases of nonspecific orbital inflammation, two of these four cases did not have pain as a prominent feature. Neuroimaging was essential in diagnosing asymptomatic extraorbital disease. Surgery has a prominent role in confirming this diagnosis, primarily by helping to rule out other diseases, such as those with granulomatous inflammation or vasculitis. Additional therapy was usually not required postoperatively.