Elderly woman presents with painful, swollen left eye

After pain was relieved in the left eye with a lateral canthotomy and inferior cantholysis, a subsequent CT scan found an orbital mass.

Jeffrey Chang

Vivek Chaturvedi

A 65-year-old Asian woman presented to the Tufts Medical Center emergency room with an acute, painful swollen left eye for approximately 15 hours and acute worsening over the past 4 hours.

The patient emigrated from China 5 years ago and spoke only Cantonese, but the patient’s daughter provided the pertinent history. Discomfort in the left eye started insidiously 3 days prior, and the patient denied recent trauma, fever or illness. There was no significant ocular history, and the patient was not taking any ocular medications. Medical history was significant for hyperthyroidism and hypertension; the patient was taking calcitriol, Tapazole (methimazole) and atenolol. The patient had no known drug allergies.

Examination

The patient was afebrile and in moderate distress on initial presentation. External exam revealed an edematous and ecchymotic left upper eyelid (Figure 1). Near card visual acuity with a +2.5 D lens was 20/50 in the right eye and 20/100 in the left eye. The right pupil was briskly reactive, while the left pupil was sluggishly reactive with a 1+ relative afferent pupillary defect. The right eye demonstrated full motility, while the left showed no motility in any direction. Proptosis of the left globe measured approximately 5 mm. Tonometry at the bedside via Tono-Pen (Reichert) was 16 mm Hg in the right eye and 74 mm Hg in the left eye.

Figure 1. External exam with left blepharoptosis. Images: Chang J, Laver N, Kapadia M

Examination at the bedside with a portable slit lamp revealed an unremarkable anterior segment exam in the right eye except for a moderate nuclear sclerotic cataract. Left eye examination revealed trace conjunctival injection, clear cornea and a quiet anterior chamber, but it was shallower compared with the right eye. There was also a moderate nuclear sclerotic cataract in the left eye.

Figure 2. CT scan of orbits without contrast, axial cut showing left orbital mass.

Figure 3. CT scan of orbits without contrast, coronal cut showing left superior orbital mass.

Following hospital policy, written consents were obtained, procedure side and site were marked, and a left lateral canthotomy and inferior cantholysis were performed with local anesthesia at the emergency department bedside. The patient’s pain was immediately relieved, and IOP in the left eye by Tono-Pen was 16 mm Hg. Subsequent dilated fundus exam in both eyes was unremarkable. The patient received a CT scan of the orbits without contrast ordered by the emergency department staff. An axial cut revealed a large posterior orbital mass in addition to ipsilateral ethmoid sinus opacification (Figure 2). A coronal cut on the CT scan further revealed the orbital mass to be superior in location without evidence of bony destruction (Figure 3).

What is your diagnosis?

Painful, swollen eye

The differential diagnosis for the cause of the orbital compartment syndrome included trauma and retrobulbar hemorrhage, infectious causes (bacterial and fungal orbital cellulitis), inflammatory causes including vasculitic and granulomatous entities (sarcoidosis, Wegener’s), neoplastic causes (lymphoma, lymphangioma, lacrimal gland tumors and cavernous hemangioma), vascular causes (carotid cavernous fistula, cavernous sinus thrombosis) and idiopathic causes (idiopathic orbital inflammation, Tolosa-Hunt syndrome).

Diagnosis

The patient’s history was not consistent with acute trauma, and more likely diagnoses included bacterial and fungal orbital cellulitis given the ipsilateral sinus opacification; lymphoma given the superior location of the orbital mass; and also idiopathic causes such as idiopathic orbital inflammation (orbital pseudotumor).

Initial emergency department blood work revealed that the patient had a normal white blood cell count. Blood cultures were sent to microbiology. Due to the difficulty in differentiating between infectious and inflammatory causes with different and potentially harmful treatments if improperly diagnosed, an urgent diagnostic orbitotomy was planned for the next morning. No steroids were given to the patient.

Orbital mass specimens were sent for pathology, and the orbitotomy also served to debulk the orbit. The patient was continued on intravenous antibiotics while awaiting pathology results. Hematoxylin and eosin staining revealed a mixed acute and chronic inflammatory infiltrate with necrosis, which is unusual for idiopathic orbital inflammation (Figure 4). Special stains were used to rule out infectious etiologies, and Gomori’s methenamine silver stain revealed branching septate hyphae characteristic of Aspergillus fungal species (Figure 5).

Figure 4. Pathology specimen stained with hematoxylin and eosin showing acute and chronic inflammation with necrosis.

Figure 5. Pathology specimen with Gomori’s methenamine silver stain showing branching septate hyphae.

The otolaryngology and infectious disease services were consulted. Endoscopic frontal ethmoid sinus surgery was performed, including left frontal recess opening and sinusotomies with amphotericin B irrigation and endoscopic ethmoidectomy. There was no evidence of bony invasion on the endoscopic exam, and Aspergillus was detected in the direct sinus samples. Treatment for the Aspergillus was initiated by the infectious disease service, and the patient was started on intravenous voriconazole with the eventual plan for long-term oral voriconazole for 6 months. Amphotericin B is the gold standard medication due to experience, but recent reports have used itraconazole and voriconazole with similar results and less toxicity, although there are no controlled comparison studies. Due to the fungal orbital infection, hemoglobin A1c was checked to make sure the patient was not diabetic, and a serum electrophoresis was also checked to make sure the patient was not immune compromised. Both test results were within normal limits.

Discussion

Sino-orbital aspergillosis is usually caused by the Aspergillus fumigatus species (90% of cases), a ubiquitous mold found on decaying vegetation and an opportunistic invader in immune-compromised hosts. Aspergillosis is the most common infection of the paranasal sinuses. Aspergillus has characteristic 45° septate branching hyphae seen microscopically with special stains such as Gomori’s methenamine silver stain. Treatment is modified based on the presence or absence of fungal invasion of surrounding structures. Prognosis is poor if blood vessel wall or bone invasion is present. In an older case series of 47 patients, 13 patients died and there was an 80% mortality rate for patients with central nervous system involvement.

Our patient is currently doing well after surgical debridement and a full 6-month course of intravenous and oral voriconazole. Best corrected vision in the left eye is counting fingers at 2 feet. She is being closely followed by our team along with the infectious diseases and otolaryngology teams with a guarded prognosis.

References:

• Hedges TR, Leung LS. Parasellar and orbital apex syndrome caused by aspergillosis. Neurology. 1976;26(2):117-120.
• Heier JS, Gardner TA, Hawes MJ, McGuire KA, Walton WT, Stock J. Proptosis as the initial presentation of fungal sinusitis in immunocompetent patients. Ophthalmology. 1995;102(5):713-17.
• Lee JH, Lee HK, Park JK, Choi CG, Suh DC. Cavernous sinus syndrome: clinical features and differential diagnosis with MR imaging. AJR Am J Roentgenol. 2003;181(2):583-590.
• Rootman J. Diseases of the Orbit: A Multidisciplinary Approach. Philadelphia: Lippincott; 1988.
• Sivak-Callcott JA, Livesley N, Nugent RA, Rasmussen SL, Saeed P, Rootman J. Localised invasive sino-orbital aspergillosis: characteristic features. Br J Ophthalmol. 2004;88(5):681-687.

• Jeffrey Chang, MD, Nora Laver, MD, and Mitesh Kapadia, MD, PhD, can be reached at Tufts Medical Center, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; Web site: www.neec.com.

• Edited by Jeffrey Chang, MD, and Vivek Chaturvedi, MD. Drs. Chang and Chaturvedi 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.