Pediatric patient presents with unilateral lid erythema and pain with extraocular movements

There was periorbital edema of the upper and lower lids, superolateral scleral injection and an enlarged lacrimal gland in the left eye.

Catherine A. Cox

Jordana F. Goren

A 12-year-old girl was referred to Tufts Medical Center after 2 weeks of worsening left upper eyelid pain and left periorbital erythema plus edema.

Two weeks before presentation, the patient’s mother had noticed a mildly ery-thematous left upper eyelid and took her to see her primary care physician, who diagnosed a left upper lid chalazion. Due to worsening over the subsequent 24 to 48 hours, the patient was referred to and examined by an outside ophthalmologist, and she was found to have increased left upper lid erythema and edema, a violet hue of the left temporal sclera and mild swelling of the left lacrimal gland. Oral ibuprofen was started for a diagnosis of scleritis.

Ten days later, the patient presented to an outside emergency department, and she was found to have left lateral conjunctival chemosis, pain with extraocular movements of the left eye, and intermittent intensity 8 out of 10 generalized headaches in addition to her previous symptoms. She was diagnosed with orbital cellulitis, started on intravenous ceftriaxone and transferred to Tufts Medical Center for further care.

Of note, the patient had no significant medical history, and her review of systems was negative for fevers, upper respiratory infection and/or trauma.

On examination, best corrected visual acuity was 20/20 in both eyes, pupils were equally round and reactive with no afferent pupillary defect, confrontation visual fields were full in both eyes, Ishihara color plates were 11/11 in both eyes, and IOPs were within normal limits. The patient was noted to have minimally decreased abduction of the left eye, as well as tenderness to palpation of the left globe. There was mild tenderness to palpation over the left maxillary sinus; however, no mass or fluctuance was appreciated. Slit lamp biomicroscopy of the left eye showed periorbital edema of the upper and lower lids, superolateral scleral injection and an enlarged lacrimal gland (Figure 1). Hertel exophthalmometry indicated 2 mm of left eye proptosis. Fundus examination revealed a normal optic nerve in both eyes with no edema or pallor, as well as a normal-appearing posterior pole and peripheral examination in both eyes.

Figure 1. External photographs showing superior-lateral scleral injection and enlarged lacrimal gland in the left eye. Images: Monson BK, Hedges TR, Chen V

What is your diagnosis?

Erythema, edema, pain

The patient was continued on intravenous ceftriaxone, but her clinical examination showed only mild improvement after 72 hours of treatment. There was suspicion of an underlying inflammatory process; therefore, she was started on 70 mg of intravenous methylprednisolone. Twenty-four hours later, a significant reduction in the size and tenderness of her left lacrimal gland was seen, as well as near complete resolution of her left eye edema, erythema and pain with extraocular movements. She was discharged from the hospital with a presumed diagnosis of orbital pseudotumor and continued on 60 mg of daily oral prednisone plus weekly subcutaneous treatments of methotrexate.

Two months later, the patient re-presented to Tufts Medical Center with new complaints of left eye blurry vision and a temporal “black shadow” in her left visual field. She reported 4 days of intermittent intensity 10 out of 10 generalized headaches, as well as recurrent left eye pain with upper and lower lid erythema plus edema. Upon discussion with the patient, she disclosed that she had self-discontinued her prednisone and methotrexate treatments due to unwanted side effects (ie, facial fullness).

On examination, significant findings included persistent left eye proptosis, left temporal conjunctival injection and a new left temporal serous retinal detachment. No vitritis, vasculitis or disc swelling was present (Figures 2 and 3).

Figure 2. Axial CT image demonstrating a focal, enhancing, anterior intraconal mass with irregular unencapsulated borders and lateral rectus muscle enlargement in the left eye.

Figure 3. Fundus photograph (top) and Carl Zeiss Meditec Cirrus HD-OCT scan (below) of the left eye showing exudative retinal detachment encroaching on the macula.

An orbital ultrasound of the left eye revealed a positive T-sign. A follow-up CT scan with contrast of the orbits and paranasal sinuses showed a focal-enhancing anterior intraconal mass with irregular unencapsulated borders in the left eye. The left lateral rectus muscle, its tendinous sheath and the left lacrimal gland were enlarged. Orbital fat enhancement was also seen along the left posterior sclera. The cavernous sinus did not appear to be involved. Laboratory tests, including ESR, CRP, MPO, ANCA, ANA, ACE, DS-DNA, HSV, HIV antibody, Lyme antibody, lysozyme, PPD, CXR, FTA-ABS, and urinalysis, were all found to be within normal limits.

Posterior scleritis, orbital fat inflammation, myositis involving the tendinous sheaths and an enlarged lacrimal gland were thought to be most consistent with left orbital pseudotumor. The patient received 3 days of intravenous methylprednisolone in hospital and showed significant clinical improvement. She was discharged home on weekly subcutaneous methotrexate injections, as well as a 3-month oral prednisone taper. At 1 month all symptoms and clinical findings were resolved.

Discussion

The initial differential diagnosis for this patient included inflammatory, infectious and/or neoplastic processes. Possible inflammatory diseases included idiopathic orbital inflammation (ie, orbital pseudotumor), Graves’ disease, Wegener’s granulomatosis or sarcoidosis. Infectious processes might include preseptal vs. orbital cellulitis or an orbital abscess. Neoplastic processes might include orbital lymphoma, an eosinophilic granuloma (histiocytosis X), a ruptured dermoid cyst or a lymphangioma. Unreported trauma or an occult foreign body must also always be considered in a pediatric patient.

Orbital pseudotumor typically presents as recurrent episodes of acute, unilateral orbital swelling. These episodes can be accompanied by pain, lacrimal gland enlargement, conjunctival chemosis, restricted motility, proptosis, binocular diplopia, elevated IOP and/or decreased vision. Inflammation within the orbit may present as myositis, scleritis, tendonitis or dacryoadenitis. The inflammatory process is idiopathic, with no known underlying disease, no localized recognizable cause within the orbit, and no reported racial or gender predilection. Pediatric orbital pseudotumor is relatively rare, comprising only 6% to 16% of all pseudotumor cases. In contrast to adult orbital pseudotumor, which typically presents unilaterally, children can have bilateral presentations in 33% of cases.

Like orbital pseudotumor, Graves’ disease can present with extraocular muscle enlargement, but multiple muscles are typically involved and there is sparing of the tendinous sheaths.

In Wegener’s granulomatosis, ocular involvement is often accompanied by multisystemic problems: pulmonary consolidation, necrotizing inflammation of the upper respiratory tract and glomerulonephritis. Isolated orbital symptoms can occur, with the characteristic finding of bony orbital destruction on imaging, but isolated orbital cases are less common. Systemic manifestations of Wegener’s can be fatal if left untreated. Given a negative ANCA and lack of systemic findings, Wegener’s was lower on our differential.

Sarcoidosis causes noncaseating granulomatous inflammation and is well-described in children. Like orbital pseudotumor, orbital sarcoidosis may present with proptosis secondary to an orbital mass, serous retinal detachment, and inflammation involving both the eyelid and lacrimal gland. There is often hilar and/or mediastinal lymphadenopathy on imaging, an elevated ACE and/or lysozyme level, and the patient may complain of respiratory difficulty.

Orbital cellulitis is a sight-threatening emergency. Orbital signs may include proptosis, decreased sensation in a V1-V2 distribution, impaired ocular motility, pain with extraocular movements, decreased visual acuity, elevated IOP and an afferent pupillary defect. Orbital cellulitis may also present with a serous retinal detachment. Sinusitis can be an underlying etiology of orbital cellulitis and may lead to recurrent episodes.

While unusual in children, orbital lymphomas can also present with isolated orbital inflammation. Low-grade mucosa-associated lymphoid tissue (MALT) lymphomas tend to follow an indolent course and remain localized to the mucosal tissues of the orbit, without bony destruction or invasion of the sinuses. Serous retinal detachment associated with orbital MALT lymphomas has been reported in the literature but is rare.

Conclusion

Our patient initially presented with unilateral orbital inflammation and was subsequently found to have dacryoadenitis, myositis and posterior scleritis with a serous retinal detachment. Poor response to antibiotic treatment prompted treatment with systemic steroids, which resulted in rapid improvement of symptoms. The negative blood work and imaging studies helped confirm a diagnosis of orbital pseudotumor. There is a high incidence of relapse in cases of orbital pseudotumor, particularly if immunosuppressive treatment is stopped too early. Our patient’s relapse and worsening of symptoms underscores this point and should be kept in mind when educating patients about their disease and treatment course, as well as when scheduling follow-up visits.

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• Bryan K. Monson, MD, Thomas R. Hedges III, MD, and Vicki Chen, MD, can be reached at New England Eye Center, Tufts University School of Medicine, 800 Washington St., Box 450, Boston, MA 02111; 617-636-4219; 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; website: www.neec.com.