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Man urgently referred for swelling and redness in left eye

A demarcated area of erythema and edema of the lids was observed, and CT scan revealed diffuse inflammation, swelling and fat stranding.

Issue: November 1, 2007

ByKatrinka L. Heher, MD

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A 41-year-old man was urgently referred to the oculoplastic service at the New England Eye Center for a swollen, red left eye. The patient complained of irritation and progressive redness of the eye and of both the upper and lower lids for the past 1.5 days. He also noted mild mucoid discharge and epiphora but denied any current fever, trauma, sinus infection or change in vision. His medical history was significant only for a knee surgery and a subsequent febrile illness within the past month. This was managed with a course of doxycycline for presumed Lyme disease, although serologies were negative. Ocular history was unremarkable. He was taking no medications and had no allergies.

Examination

On initial workup, visual acuity with correction was 20/20^-2 in the right eye and 20/25⁺² in the left eye. The pupil exam, IOP, Ishihara color tests and confrontational visual fields were within normal limits. Hertel measurements revealed the left eye to be 4 mm proptotic. Extraocular movements were full in the right eye but limited in all directions of gaze in the left eye, most significantly in abduction (70%). There was a well-demarcated area of erythema and edema of the left lids, as well as mild ptosis (Figure 1). There was no significant warmth or lagophthalmos. The conjunctiva was diffusely injected but particularly intense at 3 o’clock and inferiorly. The remainder of the slit lamp examination was remarkable only for trace nuclear sclerotic cataracts, but no anterior chamber cell, flare or vitritis was appreciated.

Chemistry panel, complete blood count with differential, sedimentation rate, Lyme serologies and blood cultures were all within normal limits. A CT scan with contrast of the orbits was obtained (Figure 2). The radiology read on the CT scan revealed axial and coronal views of orbits showing soft tissue swelling with fat stranding and enhancement in the preseptal tissues of the left orbit, which extended postseptally to the lacrimal gland and lateral rectus muscle. There was mild intraconal fat stranding but no bone erosion or remodeling. The right orbit was unremarkable, and there was no active sinus disease.

What is your diagnosis?

Redness, swelling, pain

The differential diagnosis for a middle-aged patient with progressive redness, swelling and mild pain of the eyelids and eye must include orbital cellulitis. Etiologies to consider include bacterial spread from superficial skin wounds, aspergillus, fungus and mucormycoses. This patient had only mild pain, no history of periorbital trauma, dermal or sinus infection, no fever and only minimal pain. On CT, there was no evidence of abscess formation, sinus disease or bony deformation.

Thyroid-associated orbitopathy is characterized by eyelid retraction, lagophthalmos, proptosis and a restrictive myopathy. Vision, motility abnormalities and optic nerve involvement are late, insidious findings. Pain is not a prominent feature of thyroid orbitopathy.

Other less likely etiologies to consider include lymphoma, metastatic lesions, an inflammatory response to trauma or foreign body, arteriovenous malformations, carotid-cavernous fistulas, Wegner’s disease, giant cell arteritis and sarcoidosis.

The patient was diagnosed with idiopathic orbital inflammatory syndrome based on his presentation and clinical exam, negative laboratory workup and CT scan findings.

Treatment

The mainstay of treatment for idiopathic orbital inflammatory syndrome involves corticosteroids. Commonly reported starting doses of prednisone are 60 mg to 100 mg orally for 1 to 2 weeks, although some may choose to initially use an equivalent one intravenous dose of methylprednisolone. A prompt and dramatic response to steroids should be expected within the first 3 to 4 days. Lack of this response should alert the physician to explore other diagnoses. Oral prednisone is then tapered over a 6- to 12-week course. The primary-care physician should be involved to help monitor for systemic side effects such as weight gain, diabetes, osteoporosis, myopathies, peptic ulcer disease, adrenal suppression, opportunistic infections, psychosis and cushingoid appearances.

Recurrences of idiopathic orbital inflammatory syndrome are common and can complicate its management. This is seen most commonly as patients are tapered to doses below 50 mg of prednisone. Simple dacryoadenitis or myositis is most likely to be completely cured, while orbital apex involvement and combined dacryoadenitis and myositis are most likely to recur. There is no relation to age or sex with regard to recurrence, but the sclerosing inflammatory subtype carries the worst prognosis.

Other therapeutic modalities being investigated include radiation therapy, immunosuppressive chemotherapies such as cyclophosphamide, chlorambucil, methotrexate and intravenous immunoglobulin, and biologic treatments such as tumor necrosis factor-alpha.

Discussion

Idiopathic orbital inflammatory syndrome, formerly misnamed orbital pseudotumor, is a benign, noninfective clinical syndrome. It is the third most common orbital disease after thyroid-associated orbitopathy and lymphoproliferative diseases, representing approximately 4.7% to 6.3% of orbital diseases.

Idiopathic orbital inflammatory syndrome is a collection of disorders that can further be classified by the tissues affected: anterior, posterior, diffuse, apical, dacryoadenitis, myositis, periscleritis and perineuritis. This patient had dacryoadenitis and myositis

The spectrum for clinical presentations is highly variable. Most commonly the lacrimal gland or a single extraocular muscle is affected, but diffuse orbital involvement can also be seen. Generally, there is an abrupt onset of symptoms, within hours to days. A retrospective study of patients with idiopathic orbital inflammatory syndrome showed that pain, proptosis, swelling and erythema were the most common initial signs and symptoms. Other commonly associated signs include ptosis, chemosis, motility dysfunction and optic neuropathy. In adults, cases are generally unilateral, while in the pediatric population, bilateral presentations are not uncommon.

Neuroimaging plays an important role in the diagnosis of idiopathic orbital inflammatory syndrome. Common findings include diffuse, oblong enlargement and blurred margins of the lacrimal gland. The most commonly involved extraocular muscle is the medial rectus, followed by involvement of either the superior or lateral rectus. In contradistinction to thyroid orbitopathy, the inferior rectus is the least commonly involved and would generally argue against idiopathic orbital inflammatory syndrome as the diagnosis. Either a single or multiple extraocular muscle can be involved. Although tendon involvement is highly suggestive, it is not necessary, diagnostic or specific for idiopathic orbital inflammatory syndrome, as Ben Simon found that 6.4% of 125 patients with thyroid associated orbitopathy had tendon involvement. Other possible findings include diffuse infiltration of the orbital fat, preseptum, episclera, tenon, optic nerve sheath, orbital apex or cavernous sinus. Uveal and scleral thickening is thought to be a specific sign of idiopathic orbital inflammatory syndrome, seen in 33% of cases.

For more information:

• Daniel S. Landmann, MD, and Katrinka L. Heher, MD, 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 Isabel M. Balderas, MD, and Tom Hsu, MD. Drs. Balderas and Hsu 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. Drs. Balderas and Hsu have no direct financial interest in the products mentioned in this article, nor are they paid consultants for any companies mentioned.

References:

• Ben Simon GJ, Syed HM, et al. Extraocular muscle enlargement with tendon involvement in thyroid-associated orbitopathy. Am J Ophthalmol. 2004;137(6):1145-1147.
• Jacobs D, Galetta S. Diagnosis and management of orbital pseudotumor. Curr Opin Ophthalmol. 2002;13(6):347-351.
• Mombaerts I, Goldschmeding R, Schlingemann RO, Koornneef L. What is orbital pseudotumor? Surv Ophthalmol. 1996;41(1):66-78.
• Narla LD, Newman B, et al. Inflammatory pseudotumor. Radiographics. 2003;23(3):719-729.
• Rubin PA, Foster CS. Etiology and management of idiopathic orbital inflammation. Am J Ophthalmol. 2004;138(6):1041-1043.
• Snebold NG. Orbital pseudotumor syndromes. Curr Opin Ophthalmol. 1997;8(6):41-44.
• Yuen SJ, Rubin PA. Idiopathic orbital inflammation: distribution, clinical features, and treatment outcome. Arch Ophthalmol. 2003;121(4):491-499.