IDIOPATHIC ORBITAL inflammation encompasses a spectrum of disorders, ranging from mild and steroid-responsive to severely progressive, therapy-resistant, and blinding. We describe a patient with massive bilateral orbital involvement and intracranial extension.
A 36-year-old black female drug abuser noted progressive bulging and blindness in her left eye over 4 months. Her visual acuity was 20/20 OD and no light perception OS. The left eye was totally immobile, with disc pallor and afferent pupillary defect (Figure 1).Orbital biopsy results disclosed patchy lymphoplasmacytic infiltrates, often perivascular, with prominent fibrosis. No granulomatous or xanthogranulomatous inflammation was present. Acid-fast and fungus stains were negative for organisms. Results of systemic evaluation for Wegener granulomatosis, Erdheim-Chester disease, lymphoma, tuberculosis, and sarcoidosis were negative. Subsequently, oral cyclosporin and prednisone were administered.
Charles NC, Turbin RE. Bilateral Sclerosing Orbital Pseudotumor With Intracranial Spread. Arch Ophthalmol. 2003;121(3):412-413. doi:10.1001/archopht.121.3.412