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

Orbital MRI is the most important diagnostic test, with the findings varying according to the affected orbital structures. For example, extraocular muscle involvement causes enlargement of muscles and tendons, as in this case, producing an abnormal tubular configuration (a characteristic not found in thyroid ophthalmopathy, in which the extraocular tendons are normal).[8,17,18] Imaging studies may also reveal infiltration occurring in the orbital fat or along the optic nerve, the presence of a diffuse orbital mass, enhancement of the sclera, and an increase in the size of the Tenon space.[8]

While not typically necessary in orbital pseudotumor, biopsy can be used in patients if there is a great deal of doubt regarding the diagnosis or if the condition is recurrent or unresponsive to steroids. Biopsy specimens reveal nonspecific, polymorphic, lymphocytic infiltrates with macrophages, polymorphonuclear leukocytes, and eosinophils. In sclerosing orbital pseudotumor, the biopsy reveals extensive fibrosis formation.[1,2,15,16]

A rare condition, calcifying orbital pseudotumor, may develop when the orbit is affected by a chronic, idiopathic inflammatory process. Chronic granulomatous types of orbital pseudotumor can give rise to the sclerosing form of the disease.[1,2,15,16] Serologic studies are required to rule out a systemic cause of orbital pseudotumor.[8]

Corticosteroid therapy typically causes symptoms to quickly regress (more than two thirds of patients demonstrate great improvement within 24-48 hours of treatment) and reduces the proportion of patients who suffer permanent disability from sclerosis.[3,4,7,8] Starting dosages of prednisone of 1-2 mg/kg/day are usually sufficient.[15] Once improvement is noted, dosages should be slowly tapered according to the clinical response. Alternatively, patients can initially be treated with IV pulse steroid therapy, followed by gradually tapered dosages of oral steroids.

Inflammation may improve in orbital pseudotumor even in the absence of treatment, but patients commonly suffer recurrent inflammatory episodes and residual fibrosis.[2,8] Recurrence is not unusual even in treated cases, occurring in 23-56% of patients who have undergone therapy.[2,8] Recurrence is especially common with bilateral disease.

Other treatment options include cytotoxic agents (cyclophosphamide, chlorambucil), immunosuppressants (methotrexate, cyclosporine, azathioprine), IV immunoglobulins, tumor necrosis factor alpha (TNF-α) inhibitor, monoclonal antibody (infliximab, adalimumab), and mycophenolate mofetil.[2,15] Surgical debulking is a possibility for large, sclerotic lesions.[8,19] Low-dose radiation is an option for patients who are elderly or do not respond to systemic corticosteroids, or when steroids are contraindicated. Radiation doses of 20 Gy in 10 fractions seem to reduce the symptoms of orbital myositis but do not offer effective long-term symptom control.[15,20]

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