Woman presents with eye pain, periorbital swelling and vision loss

Fundus examination showed a serous detachment in the macula of the left eye.

Mark E. Patron

Andre J. Witkin

A 32-year-old woman presented to the New England Eye Center at Tufts Medical Center with a 2-week history of left eye pain accompanied by periorbital swelling and progressive central vision loss. Review of systems was positive for periorbital tenderness, left eye pain with movement, tearing and irritation of the left eye. She denied fever, and there was no history of trauma.

Examination

On examination, the patient’s visual acuity was 20/20 in the right eye and counting fingers at 7 feet in the left eye. IOPs measured 13 mm Hg and 14 mm Hg. Pupils were equally round and reactive with no relative afferent pupillary defect. She missed two of 14 Ishihara color plates with the left eye. Her extraocular movements were full in both eyes, but she felt discomfort with movement of her left eye. There was no diplopia or exophthalmos. Confrontational visual field of her left eye revealed central and temporal defects. External examination showed tender periorbital edema of the left eye (Figure 1). Slit lamp examination was remarkable for 1+ injection of the left conjunctiva. There were no cells in the anterior chamber.

Figure 1. External examination showed tender periorbital edema of the left eye. Images: Semela LB, Kapadia MK

Figure 2. Dilated fundus examination showed a serous detachment in the macula of the left eye.

Figure 3. Serous detachment in the macula of the left eye documented with OCT.

Dilated fundus examination (Figure 2) showed a serous detachment in the macula of the left eye, which was documented with optical coherence tomography (Figure 3). The vitreous was clear in both eyes.

The patient had initially presented 2 years prior with similar findings on the right side.

What is your diagnosis?

Periorbital edema with serous macular detachment

At the patient’s initial presentation with right-sided symptoms, a CT scan of the orbits showed scleral thickening and lacrimal gland inflammation with no sinusitis (Figure 4). Laboratory studies were unremarkable except for a slightly elevated white blood cell count. B-scan ultrasound demonstrated scleral thickening and retrobulbar edema (T-sign) consistent with posterior scleritis (Figure 5). A presumptive diagnosis of idiopathic orbital inflammation was entertained, and a lacrimal gland biopsy was performed for histologic confirmation. The specimen showed no evidence of granulomatous inflammation suggestive of Wegener’s granulomatosis or sarcoidosis.

Figure 4. At the patient’s initial presentation with right-sided symptoms, a CT scan of the orbits showed scleral thickening and lacrimal gland inflammation with no sinusitis.

Figure 5. B-scan ultrasound demonstrated scleral thickening and retrobulbar edema (T-sign) consistent with posterior scleritis.

Figures 6a and 6b. At the patient’s subsequent presentation with left-sided symptoms, fluorescein angiography was performed and revealed early pinpoint hyperfluorescence with late leakage.

At the patient’s subsequent presentation with left-sided symptoms, fluorescein angiography was performed and revealed early pinpoint hyperfluorescence with late leakage (Figures 6a and 6b).

Differential diagnosis

Idiopathic orbital inflammation may be clinically similar to orbital cellulitis. Most commonly, orbital cellulitis arises from sinusitis, but the lack of sinus disease on imaging studies suggests inflammation over infection. Other disorders to consider in the differential diagnosis include inflammatory reaction to trauma or a foreign body, inflammatory diseases such as thyroid associated orbitopathy, sarcoidosis, vasculitis and neoplastic causes including metastases. In Wegener’s granulomatosis, which features necrotizing inflammation and vasculitis, ocular involvement is seen in about 50% of cases. Ocular and orbital manifestations include conjunctivitis, marginal ulcerative keratitis, scleritis and uveitis. With sarcoidosis, ocular involvement occurs in 25% to 50% of cases and may include infiltration of the lacrimal gland, extraocular muscles, orbital fat, optic nerve and uveal tract.

Discussion

Idiopathic orbital inflammation, also known as orbital pseudotumor, is a benign, noninfectious clinical syndrome characterized by features of nonspecific inflammatory conditions of the orbit without identifiable local or systemic causes. It accounts for 4.7% to 6.3% of orbital disorders and is the third most common orbital disease, following Graves’ orbitopathy and lymphoproliferative diseases. The etiology is unclear, but an immune-mediated process involving B- and T-cells seems likely. Viral, genetic and environmental factors have been implicated as possible triggers. Idiopathic orbital inflammation can occur in any age group and has no gender or racial predilection.

Because the disease can affect all structures within the orbit, the clinical features are highly variable. The most commonly used classification scheme is based on the anatomic structures that are involved and include dacryoadenitis, myositis, anterior and posterior scleritis, optic neuritis, diffuse orbital fat inflammation, and inflammation localized to the cavernous sinus (Tolosa-Hunt syndrome). Idiopathic orbital inflammation can be focal or diffuse and most often presents with an abrupt onset of pain, photophobia, proptosis, lid swelling, chemosis, conjunctival injection and extraocular muscle restriction. A unilateral presentation is typical; however, bilateral presentations are not uncommon, especially in children. Involvement of the optic nerve or sclera can lead to severe visual loss.

The initial workup is directed toward eliminating other local or systemic causes for orbital disease, utilizing laboratory studies and neuroimaging accordingly. Patients are sometimes treated empirically based on their clinical and radiographic findings. However, a confirmatory biopsy is often helpful in patients with severe vision loss, poor response to treatment or multiple recurrences. The orbital lobe of the lacrimal gland often provides a straightforward anatomic location for tissue diagnosis if lacrimal gland enlargement is present on imaging studies or clinical examination. The characteristic pathologic findings are a mixed infiltrate with plasma cells, lymphocytes, macrophages and polymorphonuclear cells. More chronic forms are associated with fibrosis.

Corticosteroids are the mainstay therapy for idiopathic orbital inflammation and are administered for several months to ensure remission. The response is typically rapid, with pain and proptosis resolving as quickly as 24 to 48 hours after treatment onset. Typically, oral prednisone is started at a dose of 60 mg to 100 mg daily and tapered slowly over weeks to months. Intravenous steroid therapy is usually reserved for patients with severe vision loss. A lack of response to prednisone makes the diagnosis of idiopathic orbital inflammation suspect, and a tissue biopsy should be considered.

Follow-up

The patient was started on 80 mg of prednisone daily. Two weeks after initiation of therapy, the patient had no pain, and her vision in the left eye improved to 20/60. The subretinal fluid causing the exudative retinal detachment was significantly reduced.

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• Linda Brenner Semela, MD, and Mitesh K. Kapadia, MD, PhD, can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; Web site: www.neec.com.

• Edited by Mark E. Patron, MD, and Andre J. Witkin, MD. Drs. Patron and Witkin can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; Web site: www.neec.com.