EyeNet Magazine


 
Morning Rounds
Behind the Masquerade
By Rohit Adyanthaya, MD, and Robert Honkanen, MD
Edited by Thomas A. Oetting, MD
 
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(PDF 248 KB)

When we first saw Milagro Rodriguez,* she was frustrated and worried. After four months of persistent blurry vision and pain in her left eye, the 42-year-old Hispanic woman had finally consulted an optometrist. He diagnosed inflammatory glaucoma and began treating her. However, after two weeks, her glaucoma showed no signs of responding to his treatment regimen, which included prednisolone, timolol/dorzolamide, brimonidine and latanoprost eyedrops.

At that point, the optometrist referred Ms. Rodriguez to us for further evaluation and treatment. He reported that Ms. Rodriguez had no medical or ocular history of note other than a positive purified protein derivative test for tuberculosis 12 years ago; her TB was successfully treated at that time. 


We Get a Look

When we examined Ms. Rodriguez, her BCVA was 20/20 in the right eye and 20/200 in the left, and her IOP was 10 mmHg in the right eye and 40 mmHg in the left. The slit-lamp examination revealed marked keratic precipitates and debris in the inferior cornea and angle of the left eye (Figs. 1 and 2). She had 2+ cellular reaction in the anterior chamber, with large clumps of cells. In addition, we noted neovascularization of the iris, along with a few iris nodules.

On gonioscopy, the left eye had approximately 120 degrees of peripheral anterior synechiae, notably in the inferior and superior quadrants, and a small amount of neovascularization of the angle in the superotemporal region. The fundus exam was unremarkable.

At this point, our working diagnosis was uveitic glaucoma, and we began investigating the etiology. We ordered antinuclear antibody, erythrocyte sedimentation rate, human leukocyte antigen–B27 and prolactin tests as well as tests for chlamydia, Lyme disease and syphilis. All of these tests came back within normal limits. The serum chemistry profile found that her alkaline phosphatase and triglyceride levels were elevated, and results of the complete blood count indicated that she had mild anemia.

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The Next Step

As it was our opinion that the cellular reaction seen in our patient’s left eye—notably the large circulating cell clusters and “clumping” of cellular debris— was atypical for uveitis, we performed a diagnostic paracentesis of the anterior chamber. The results showed numerous cell clusters with nuclear atypia and cytoplasmic vacuoles typical of adenocarcinoma (Fig. 3).

These worrisome findings prompted a full medical workup. Indeed, a chest x-ray and CT scan revealed a large mass in the lower lobe of the left lung with nodules in the right lung, and an endoscopic biopsy confirmed the diagnosis of adenocarcinoma of the lung. Subsequent oncologic evaluation revealed multiple metastases to the brain (Fig. 4).

Ms. Rodriguez received whole brain and left orbit irradiation and systemic chemotherapy. We also treated her with diode laser cyclophotocoagulation for glaucoma. Two weeks after this procedure, she reported that her ocular pain was significantly reduced. The IOP in her left eye dropped to 26 mmHg, although her visual acuity was unchanged. No further glaucoma treatments were needed.

Ms. Rodriguez remained comfortable for the next year without experiencing any additional visual complaints or ocular pain. Unfortunately, at the end of that year, she passed away.

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What’s Your Diagnosis?

LEFT EYE. (1) The diffuse slit-beam photo of the anterior segment of the left eye shows areas of fine iris neovascularization, and settled cellular debris is easily visible in the inferior angle. (2) Keratic precipitates are present on the inferior corneal endothelium.
CRITICAL DETAILS. (3) This composite of four pathology images shows cell clusters from the anterior chamber aspirate. The clusters of basophilic cells with nuclear atypia and vacuolated cytoplasm are typical of adenocarcinoma. (4) A CT scan of the patient’s brain reveals multiple metastases (a separate CT scan of her chest had pinpointed the primary lung tumor).

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Discussion

The uveitic masquerade syndromes (UMS) are a group of disorders that present as inflammatory processes but are, in fact, noninflammatory in origin. A high frequency of malignant disease among patients with a masquerade syndrome has been reported, up to almost 50 percent of cases in one review.1 Given the potential impact of an incorrect diagnosis, UMS should not be overlooked during the differential diagnosis of anterior uveitis.

The primary cancer may be undiagnosed at the time a patient consults the ophthalmologist. In a survey of 520 eyes with uveal metastases, almost one-third of patients had no history of primary cancer at the time of diagnosis; the breasts and lungs represented the majority of primary tumor sites found on subsequent evaluation.2

Use of paracentesis. In cases where an atypical anterior chamber reaction raises clinical suspicion for a masquerade syndrome, diagnostic anterior chamber paracentesis can be used to obtain a biopsy specimen.3 Paracentesis is a safe, nonaggressive and quick procedure with fewer complications and a lower risk of morbidity than intraocular biopsy.4 For patients with a UMS, paracentesis of the anterior chamber remains an essential diagnostic procedure.

Use of laser. Roughly 50 percent of patients with metastatic disease in the eye will develop secondary glaucoma.5 Although incisional glaucoma procedures can lower IOP, they may lead to orbital seeding of malignant cells and thus are relatively contraindicated. In contrast, external diode laser cyclophotocoagulation both is effective at lowering IOP and does not violate ocular integrity, thus reducing the risk for orbital spread of malignant cells.

Additional advantages of the procedure are that it can be done in the office using a retrobulbar block and that patients recover quickly and require less frequent postoperative care, although narcotic pain relief may be needed postoperatively. These are important advantages when treating a patient who has a poor prognosis or is a high anesthetic risk.

Avoidance of enucleation. Studies have found a mean survival of 5.4 months after presentation with metastases to the anterior segment.1 If the patient can be kept comfortable, the need for enucleation can be avoided.1,2
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1 Rothova A et al. Ophthalmology. 2001;108(2):386-399.
2 Shields CL et al. Ophthalmology. 1997;104(8):1265-1276.
3 Augsburger JJ et al. Ophthalmology. 1985;92(1):39-49.
4 Van der Lelig A, Rothova A. Br J Ophthalmol. 1997;81(11):976-979.
5 Ferry AP, Font RL. Arch Ophthalmol. 1975;93(7):472-482.
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* Patient name is fictitious.
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Dr. Adyanthaya is a vitreoretinal fellow at the Valley Retina Institute in McAllen, Texas; Dr. Honkanen is assistant professor of ophthalmology at Stony Brook University Medical Center in New York.

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