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Woman complains of foreign body sensation, decreased vision in left eye
The patient had a history of chronic pseudodendritic keratitis, increased IOP and granulomatous uveitis.
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 Jeffrey Chang |  Vivek Chaturvedi | ||
An 84-year-old Catholic nun initially presented to our eye clinic with foreign body sensation for 3 weeks. Her medical history included uncomplicated bilateral extracapsular cataract extraction 15 years prior, coronary artery disease, atrial fibrillation, hypertension and bladder cancer, for which she was receiving intravesicular injections of bacillus Calmette-Guerin, or BCG.
Examination
On examination, the patient’s best corrected visual acuity was 20/25 in the right eye and 20/30 in the left eye. Her IOPs were 16 mm Hg in the right eye and 30 mm Hg in the left eye. Her pupils were relatively symmetric, with no relative afferent pupillary defect noted.
On slit lamp examination, 1+ ciliary flush, granulomatous keratic precipitates, and 2+ anterior chamber cell and flare were noted in the left eye. A well-positioned posterior chamber IOL was present bilaterally. The right eye was quiet. Fundus examination demonstrated no vitreous cells, Weiss rings and mild inferior peripheral chorioretinal atrophy in both eyes. The patient was started on prednisolone acetate 1% in the left eye every hour, cyclopentolate 1% in the left eye three times a day and brimonidine 0.15% in the left eye three times a day. Blood testing revealed an elevated angiotensin converting enzyme (ACE) level of 82 (normal range: 9 to 67) and a lysozyme level of more than 32 (normal range: 9 to 17). A QuantiFERON-TB Gold test for tuberculosis was negative. A chest X-ray was normal.
Over the next 6 weeks, the anterior uveitis quieted, IOP normalized, and the topical steroids were tapered. On her return visit 6 weeks later, slit lamp examination of the left eye revealed pseudodendritic keratitis, trace cells in the anterior chamber and absence of vitreous cells with an unchanged posterior segment examination (Figure 1). Oral acyclovir was started at 800 mg five times per day, and topical steroids four times per day and brimonidine three times per day were continued in the left eye.
Despite continuation of high-dose oral acyclovir, pseudodendritic keratitis persisted over the next 5 months in the left eye. The anterior chamber reaction decreased and the prednisolone was tapered to once daily. Six months after initial presentation, despite persistence of a small pseudodendrite, the oral acyclovir was discontinued due to a decrease in renal function.
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| Color photograph of the left eye 6 weeks after initial presentation, showing presence of a pseudodendrite highlighted with fluorescein stain. | A. Color fundus photograph of the left eye 2 months after discontinuation of oral acyclovir, demonstrating dense vitritis. B. Color photograph of the inferotemporal retina of the left eye, showing a discrete area of retinitis. Images: Witkin AJ, Soukiasian SH | |
Two months later, the patient returned to the eye clinic noting 1 week of a painless decrease in vision in the left eye. BCVA had decreased from 20/30 to 20/400. IOP was 16 mm Hg in both eyes. There was no relative afferent pupillary defect in either eye. Slit lamp examination of the left eye revealed trace conjunctival injection, intact corneal epithelium, granulomatous keratic precipitates, 2+ cell and flare in the anterior chamber, and diffuse iris stromal atrophy. Left fundus examination revealed 4+ vitritis with a difficult view of the posterior pole. Peripheral retinal examination showed multiple discrete areas of retinitis in all four quadrants in the left eye, most notably inferotemporally (Figure 2). The right eye remained quiet.
What is your diagnosis?
Decrease in vision
The differential diagnosis for this patient includes noninfectious inflammatory causes of vasculitis and retinitis such as sarcoidosis and Behçet’s disease; infectious causes of retinitis such as tuberculosis, toxoplasmosis, syphilis, endogenous endophthalmitis, cytomegalovirus and acute retinal necrosis; and a masquerade syndrome from primary intraocular lymphoma or leukemic infiltration of the retina.
Inflammatory diseases such as Behçet’s and sarcoidosis often cause retinal vasculitis, with perivascular sheathing, exudates, hemorrhages and neovascularization later in the disease course. Retinal whitening may be present due to ischemia secondary to vascular occlusion. In our patient, retinal whitening was present without discrete vasculitis or hemorrhages. Patients with Behçet’s disease also typically have recurrent oral ulcerations as well as ulcerations of other mucosal tissue, which our patient did not have. Our patient had elevated ACE and lysozyme levels found on initial work-up of anterior uveitis. However, because these tests are not specific for sarcoidosis, it is possible that ACE and lysozyme levels were elevated secondary to the BCG injections she had been receiving as treatment for her bladder cancer, as BCG stimulates a T-cell mediated immunological response similar to that found in sarcoidosis.
Infectious retinitis appears as retinal whitening, often with overlying vitritis. It may be difficult to distinguish between causes of retinitis, and the history may often be helpful. In our case, the patient had a history of pseudodendritic keratitis, a classic finding in herpes zoster ophthalmicus. QuantiFERON-TB Gold testing was negative, making tuberculosis unlikely. She was a Catholic nun and unlikely to have contracted syphilis. Acute infection with toxoplasmosis may appear similar. Recurrent cases of toxoplasmosis typically also have areas of chorioretinal scarring. Cytomegalovirus retinitis is often associated with retinal hemorrhages and typically found in severely immunocompromised patients. Endogenous endophthalmitis is typically painful, the patients are sick from systemic infection, and the area involved is primarily the choroid as opposed to the retina.
A consideration in any older patient is primary intraocular lymphoma or leukemic infiltration of the retina. Vitreoretinal lymphoma may look similar to the retinal lesions in our patient and typically appears as cellular infiltration of the vitreous and the subepithelial space of the retina. Bilateral involvement is common. Diagnosis may be difficult, and diagnostic vitreous tap may be recommended in challenging cases.
Discussion
Our patient was diagnosed with acute retinal necrosis based on her history of pseudodendritic keratitis, granulomatous uveitis and increased IOP. Acute retinal necrosis is one of the most devastating consequences of ocular infection by members of the herpesviridae family of viruses. It is typically seen in otherwise healthy individuals of both sexes and any age.
Acute retinal necrosis typically presents with a severe uveitis with keratic precipitates and vitritis, retinal vasculitis and peripheral retinal necrosis. It is rapidly progressive and often leads to retinal detachment within a few weeks of initial presentation. Acute retinal necrosis often involves the contralateral eye at some point during the disease course if left untreated. Diagnosis may be made based on clinical examination alone, but viral culture and polymerase chain reaction may be used to confirm the diagnosis.
Acute retinal necrosis is usually treated with high-dose intravenous acyclovir for 7 days, followed by high-dose oral acyclovir, valacyclovir or famciclovir for another 6 to 8 weeks. Other adjunctive therapies include oral steroids to reduce ocular inflammation and prophylactic laser retinopexy to prevent retinal detachment. Despite therapy, the prognosis for this disease remains poor in many patients due to the propensity for retinal detachment.
Our patient was admitted to the hospital for intravenous acyclovir 700 mg a day for 1 week (dose adjusted for renal function). After 1 week, the vitritis had started to improve, and BCVA had increased to 20/60 in the left eye. The patient was discharged on oral valacyclovir 1,500 mg a day. The patient continues to improve 2 months after presenting with acute retinal necrosis.
For more information:
- Andre J. Witkin, 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.
- Sarkis H Soukiasian, MD, is affiliated with Tufts Medical Center and Lahey Clinic and can be reached at the Lahey Clinic, 41 Mall Road, Burlington, MA 01805; 781-744-8555; Web site: www.lahey.org.
- 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. Drs. Chang and Chaturvedi have no direct financial interest in the products mentioned in this article, nor are they paid consultants for any companies mentioned.
References:
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- Freilich JM, Ryan EA, et al. Acute retinal necrosis syndrome: findings, management, and differential diagnosis. Int Ophthalmol Clin. 1996;36(3):141-146.
- Kaiser PK, Lowder CY, et al. Chest computerized tomography in the evaluation of uveitis in elderly women. Am J Ophthalmol. 2002;133(4):499-505.
- Liesegang TJ. Herpes zoster ophthalmicus natural history, risk factors, clinical presentation, and morbidity. Ophthalmology. 2008;115(2 Suppl):S3-12.
- Magone MT, Nasser RE, Cevallos AV, Margolis TP. Chronic recurrent varicella-zoster virus keratitis confirmed by polymerase chain reaction testing. Am J Ophthalmol. 2005;139(6):1135-1136.