Man presents with unilateral eyelid swelling, redness

Catherine A. Cox

Jordana F. Goren

A 54-year-old Caucasian man was referred to the New England Eye Center for progressive right lower eyelid swelling. He reported that his symptoms started 10 days before presentation with eyelid swelling and redness, and he subsequently developed discharge and eye pain, exacerbated with extraocular movements and palpation. He also developed blurred vision and constitutional symptoms including decreased appetite and lethargy. He denied any light sensitivity, flashes, floaters, recent illness, fever or recent trauma.

Ocular history was significant for myopia and allergic conjunctivitis for which he took Pataday (olopatadine hydrochloride 0.2%, Alcon) as needed. Medical history was remarkable for Down syndrome, gout and hypothyroidism. He denied any history of sinus disease and had no drug allergies.

On external exam, the patient had right-sided periorbital erythema and swelling (Figure 1). Best corrected visual acuities were 20/40 in the right eye and 20/50 in the left. Pupils were reactive; the right pupil was marginally larger than the left, without an afferent pupillary defect in either eye. Ishihara color plates were full, but the patient noted decreased brightness in the right eye. IOP was 18 mm Hg in the right eye and 17 mm Hg in the left. Extraocular motility was moderately decreased in all fields of gaze in the right eye and full on the left. Hertel ophthalmometry revealed 4 mm of right eye proptosis. Confrontational visual fields were full in both eyes.

Figure 1. Photos demonstrate periorbital edema (left), with diffuse conjunctival injection and chemosis (right). Images: Williams SL, Kapadia M

Figure 2. Posterior pole (left) and mosaic (right) demonstrating macular choroidal folds and temporal choroidal effusion.

Anterior segment exam of the right eye demonstrated diffuse 3+ injection with chemosis and dense central superficial punctate keratitis without infiltrate. The anterior chamber was deep and quiet. Anterior segment exam of the left eye was normal. Posterior segment exam of the right eye demonstrated a large choroidal effusion/detachment that extended into the macula (Figure 2). There was no vitritis or optic nerve head swelling in the right eye, and posterior segment exam of the left eye was normal.

What is your diagnosis?

Red, painful eye

The differential diagnosis for an acute presentation of a red, painful eye with proptosis includes infectious, inflammatory, vascular and neoplastic diseases.

Orbital cellulitis is a common infection and may be due to bacterial or fungal organisms. In the majority of cases, the source of the infection is pre-existing sinusitis, which is visible on imaging studies. Inflammatory diseases in the differential diagnosis include idiopathic orbital inflammation, thyroid-associated orbitopathy, Wegener’s granulomatosis and sarcoidosis. Idiopathic orbital inflammation is the most common inflammatory disease of the orbit and often presents with a red, painful eye. Thyroid-associated orbitopathy can be associated with proptosis and a red eye but rarely presents in such an acute manner. Wegener’s granulomatosis and sarcoidosis are systemic inflammatory disorders, which can affect the orbit. Carotid-cavernous fistula is a vascular disorder that occurs more commonly after orbital trauma. Findings include engorged episcleral vessels and pulsatile proptosis. Dermoid cysts are filled with inflammatory material and can present acutely with a red, painful eye if the cyst ruptures.

Discussion

Idiopathic orbital inflammation, or idiopathic orbital pseudotumor, is a nongranulomatous inflammatory disease often involving the lacrimal gland, extraocular muscles and eyelids. It is seen in all age groups, although it is less common in children. There is no systemic involvement, although it can have a similar presentation to orbital inflammatory disease associated with systemic entities. Typical symptoms include subacute onset of progressive eye pain, lid swelling, blurred vision and diplopia. External examination reveals variable periorbital edema, conjunctival injection and chemosis, proptosis and extraocular motility restriction. Dilated fundus examination may reveal choroidal folds or a choroidal effusion. Assessment of optic nerve function with pupils and visual field testing is essential if compressive optic neuropathy is suspected.

Evaluation with CT or MRI can demonstrate a focal or diffuse pattern of orbital inflammation, including enlargement of the lacrimal gland, inflammation of the extraocular muscles, and orbital fat stranding. Ultrasound may demonstrate scleral thickening and a posterior T-sign, which indicates edema within posterior Tenon’s capsule and is characteristic of posterior scleritis. Although possible with other inflammatory disorders, the findings of posterior scleritis associated with orbital disease are highly suggestive of idiopathic orbital inflammation.

Diagnosis and management

The diagnosis of idiopathic orbital inflammation is usually made clinically, based on a constellation of clinical findings and radiology. In our practice, patients are often treated empirically with oral prednisone starting at a dose of 60 mg to 80 mg daily and tapered over a course of 3 months. The rapid resolution of symptoms within 2 to 3 days after starting corticosteroids is almost pathognomonic of this disease. Biopsy of orbital structures, such as the lacrimal gland, is reserved for patients with atypical presentations or patients with recurrent inflammation as the prednisone is tapered. Recalcitrant cases of idiopathic orbital inflammation often require adjunctive treatment with immunomodulatory agents, such as methotrexate, cyclophosphamide, rituximab or radiation therapy.

Figure 3. CT scan (left) demonstrating diffuse intraconal and extraconal inflammation. Ultrasound (right) demonstrates scleral thickening, posterior T-sign and choroidal effusion.

Our patient had a CT scan that demonstrated diffuse intraconal and extraconal fat stranding, and B-scan ultrasound demonstrated scleral thickening and a posterior T-sign with 360° choroidal detachment (Figure 3). The patient was treated with methylprednisolone 1 g IV daily for two doses and responded immediately. He was discharged on an oral prednisone taper, with complete resolution of his symptoms.

References:

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• Steven L. Williams, MD, and Mitesh Kapadia, MD, PhD, can be reached at New England Eye Center, Tufts University School of Medicine, 800 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; website: www.neec.com.

• Edited by Catherine A. Cox, MD, and Jordana F. Goren, MD, MS. Drs. Cox and Goren can be reached at New England Eye Center, Tufts University School of Medicine, 800 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; website: www.neec.com.