Bilateral optic nerve compression caused by lymphomatous infiltration of all extraocular muscles

Orbital lymphoid tumors may arise within one or more extraocular muscles, although multiple and bilateral presentation is rare. Further more, bilateral optic nerve compression is an exceptional finding in orbital lymphoma. A 28-year-old man experienced bilateral proptosis and loss of vision, He had suffered from a stage IV non-Hodgkin lymphoma, not involving the orbit, which had been quiescent for 3 years. Ocular motility was impaired in all directions, Bilateral optic disc swelling was apparent. Orbital MRI revealed bilateral enlargement of all extraocular muscles and of lacrimal glands, causing apical optic nerve compression. Incisional biopsy confirmed the diagnosis of orbital small-cell lymphoma, Combination chemotherapy and oral prednisone administration resulted in a complete regression of orbital masses, Bilateral lymphomatous infiltration of all extraocular muscles with papilledema requires a differential diagnosis with other causes of bilateral papilledema and of extraocular muscle enlargement. A previous history of lymphoma, associated lacrimal gland involvement, and clinical features may help the diagnosis, Biopsy is required both for the differential diagnosis and for lymphoma grading.