A 36-Year-Old Woman With Painful Swelling and Ophthalmoplegia of the Right Eye

Sumaira Nabi, MBBS; Sadaf Khattak, MBBS; Mansoor Iqbal, MBBS, FCPS

Disclosures

June 11, 2014

Discussion

This patient was administered high-dose oral corticosteroid therapy in the form of prednisolone at 2 mg/kg/day. Her symptoms resolved within 48 hours, with significant improvement in proptosis and ptosis. However, her ocular motility showed only mild improvement. She was then referred to an ophthalmology hospital for specialized treatment. The consultant ophthalmologist confirmed the diagnosis of orbital pseudotumor on the basis of clinical and radiologic evidence, as the patient did not give consent for biopsy and histopathology.

Orbital pseudotumor, also called “idiopathic orbital inflammatory syndrome” or “idiopathic nonspecific orbital inflammation,” is an inflammatory condition of the orbit characterized by a mixed infiltrate and fibrosis. The lacrimal glands (the most frequently affected structures), globe, extraocular muscles, and optic nerve are among the many structures that can be affected by the disease. The condition may result in diffuse orbital involvement or, alternatively, it can have more focused consequences, affecting specific orbital structures, such as the lacrimal glands or specific muscles.[1,2] Orbital pseudotumor can therefore be subclassified on the basis of the anatomic target areas within the orbit, clinically presenting itself primarily as lacrimal pseudotumor, anterior pseudotumor, posterior pseudotumor, diffuse pseudotumor, and myositic pseudotumor (which mimics thyroid-associated orbitopathy, as seen in our patient).[3,4,5]

The disease’s symptoms tend to be nonspecific ones that simulate those of a number of intraorbital tumors, rather than manifesting as the classic triad of pain, ophthalmoparesis, and proptosis.[2] Thus, the clinical features of orbital pseudotumor—which include inflammation, infiltration, and/or signs of mass effect—demonstrate wide variation. Ocular manifestations commonly include pain, proptosis, lid edema, erythema, local swelling, and conjunctival injection; other symptoms can include vision loss, diplopia, ptosis, and extraocular dysmotility.[3,4,5,6,7,8] Symptoms usually develop over a period of hours or days, but development has been known to take place over weeks, or even months. Orbital pseudotumor often occurs in just one eye, but it is not unusual for bilateral symptoms to occur.[8]

One of the most common orbital diseases, orbital pseudotumor occurs mainly in adults but has no race or sex predilection.[8,9,10,11] It was first described by Birch-Hirschfeld, in 1905.[12]

The disease’s pathogenesis has not been fully elucidated, although immune-mediated processes are thought to be the likely underlying ocular mechanism. The etiology of orbital pseudotumor is also unknown, although infection, autoimmune disease, and aberrant wound healing have been suggested in the literature as possible underlying causes. Infectious diseases (eg, streptococcal pharyngitis, viral upper respiratory infection, and infection with the spirochete Borrelia burgdorferi ) may also be linked to orbital pseudotumor.[13,14,15] Nonspecific findings—benign lymphoid hyperplasia and inflammatory cell infiltration with necrotizing vasculitis—may be the only pathologic results.[15,16]

The diagnosis is challenging, since the differential diagnosis is extremely varied, ranging from orbital cellulitis to Graves disease.[8] Therefore, orbital pseudotumor is diagnosed through exclusion, based on the history of the case and on the examination, clinical course, and radiologic, serologic, and histologic findings, as well as on the patient’s response to steroid therapy.

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