To summarize the clinical profile and response to treatment of 4 biopsy-proven cases of inflammatory orbital pseudotumor extending beyond the orbit.

Retrospective observational case series.

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.

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.

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.