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52 year old man with intermittent proptosis
Digital Journal of Ophthalmology 1998
Volume 4, Number 16
March 18, 1998
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Jeffrey T. Finer, M.D., Ph.D. | Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA
Diagnosis and Discussion
Anterior Orbital Pseudotumor / Posterior Scleritis

Orbital pseudotumor is a benign idiopathic inflammatory process involving the structures in the orbit which may present in several different patterns. It may be acute, subacute, chronic, or is rare cases recurrent. It may diffusely involve the entire orbit or present in a localized fashion involving either the anterior orbit, posterior orbit, lacrimal gland, or extraocular muscles. Most cases are unilateral, but bilateral cases are seen and are more common in children.

The clinical signs and symptoms vary depending upon the involved tissues. Our patient's presentation was most consistent with acute anterior orbital pseudotumor which typically involves the globe, tenons capsule, sclera, and periscleral tissues. Signs and symptoms of anterior pseudotumor include pain, redness, lid swelling, proptosis, decreased acuity, and diplopia. Uveitis, choroidal effusions, macular edema, papillitis, and increased intraocular pressure may also be seen. Anterior orbital pseudotumor may be difficult to distinguish either clinically or radiographically FROM posterior scleritis as seen in this case and some feel that these two entities may represent the same inflammatory process.

Posterior orbital pseudotumor involves the orbital apex and may present as an optic neuropathy with visual field defects, dyschromatopsia, and an afferent pupillary defect. It may also present as an ophthalmolegia. Orbital myositis involves either single or multiple extraocular muscles and presents with pain on eye movement, diplopia, and proptosis. Acute dacryoadenitis may present as ptosis with swelling of the lateral upper lid.

The diagnosis of orbital pseudotumor is one of exclusion. In an acute presentation, one must first rule out an infectious process. In our case, the patient had a normal WBC, no fever, and symptoms that were present for at least two weeks, making an acute orbital cellulitis very unlikely. Other diagnostic laboratory studies include thyroid function tests, ESR, ANA, ACE, and ANCA. Orbital imaging is indicated in most cases and CT is the most common means. MRI, however, may offer advantages in distinguishing inflammation FROM neoplasia and hemorrhage. B-scan ultrasonography is useful in cases of anterior pseudotumor or posterior scleritis to evaluate choroidal effusions and may SHOW the characteristic T-sign of a sub-tenons effusion. Orbital biopsy is indicated if there is a prior history of systemic malignancy, uncertainty of the diagnosis, or in cases that are refractory to treatment.

Corticosteroids are the mainstay of treatment and the response can be impressive as seen in our case. Oral prednisone at a starting dose of 60-100 mg is used for 2-3 weeks followed by a taper. Alternative treatments for cases that are refractory to steroids and for patients who become steroid-dependent include low dose radiation, cyclosporin, methotrexate, and cyclophosphamide.