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Patient presents with blurred, decreased vision
The right eye had elevated IOP and extensive trabecular meshwork pigment deposition.
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A 45-year-old woman presented for emergency evaluation of decreased vision and redness of her right eye. The patient said that the blurred vision had begun 2 days before presentation. She denied any associated symptoms, including photophobia, headache, nausea or vomiting. She admitted a similar episode occurred 20 years ago that was treated with eye drops for a prolonged period of time. She was unsure of the diagnosis or treatment from that episode. She had no further episodes in the last 20 years. She was not currently using any eye medications.
She denied any other medical problems and was not currently on any medications. She had no family members with eye problems. Her review of systems was unremarkable. She denied chronic cough, history of back problems, prior joint disease or gastrointestinal disorders.
 Isabel Balderas |  Tom Hsu, MD |
Examination
Examination revealed visual acuity of 20/40+3 in the patient’s right eye and 20/25-2 in the left eye. Her right eye pinholed to 20/25-2. Pupillary exam revealed anisocoria, with her right pupil demonstrating an irregular margin. Her right pupil was irregular, dilated and unreactive (Figure 1), and her left pupil was reactive and normally shaped. Neither eye demonstrated an afferent or relative afferent pupillary defect. Extraocular movements and visual fields by confrontation were intact. Tonometry by applanation revealed a markedly elevated IOP in her right eye of 62 mm Hg. Left eye was normal with IOP of 14 mm Hg.
Slit lamp examination of the right eye showed mild diffuse injection of the conjunctiva with no evidence of scleritis or episcleritis. Corneal exam uncovered Krukenberg spindles (Figure 2) in both eyes as well as pigment deposition in both eyes inferiorly (greater in the right eye.) The cornea showed no signs of edema. The anterior chamber of the right eye had 2+ pigmented cells with occasional white cells. The left eye was quiet. Further examination of the right iris showed diffuse transillumination defects, as well as iris atrophy, that were more pronounced inferonasally (Figure 3). There were no posterior synechiae and only trace nuclear sclerosis. Gonioscopy showed that both angles were open to the ciliary body with a concave approach. However, the right eye had extensive trabecular meshwork pigment deposition in the inferior angle and the left eye had mild pigment deposition. Dilated fundus examination was unremarkable. Cup-to-disc ratio was approximately 0.3 in both eyes. Glaucoma testing, including visual fields, OCT and HRT, were unremarkable.
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Images: Krishnan C |
What is your diagnosis?
High IOP, pigment deposition
The patient had her IOP controlled with topical brimonidine, dorzolamide and timolol, while her inflammation was treated with topical prednisolone acetate 1% taken hourly. Her IOP and visual acuity returned to normal as her inflammation resolved. She received a uveitis workup, which revealed a normal CXR, ESR, CBC, HLA-B27, PPD and urinalysis. ANA testing returned a titer of 1:40 with a speckled pattern, which is inconclusive, as it is often found in patients with no evidence of connective tissue disease. Her serum was positive for HSV-1 IgG antibodies. The patient admitted to a history of cold sores.
At this point we were under the impression that the patient’s entire clinical history was consistent with herpes simplex uveitis, but we could not entirely exclude pigment dispersion syndrome, iridocorneal endothelial syndrome or chronic angle closure. Patients with iridocorneal endothelial syndrome typically have high peripheral anterior synechiae if they have iris findings. Patients with pigmentary glaucoma typically do not have inflammatory cells and keratic precipitates. Patients with chronic angle closure can have the same iris findings, but the angle is not open on gonioscopy. Three months after her original presentation, the patient returned with another elevation in IOP to 27 mm Hg and marked inferior keratic precipitates (Figure 4). At this point, we were fairly convinced that our patient had uveitis secondary to herpes simplex virus.
Discussion
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Herpes simplex virus (HSV) is a double-stranded DNA virus that can lead to a multitude of eye disorders including blepharitis, conjunctivitis, dendritic epithelial keratitis, stromal keratitis, trabeculitis, iridocyclitis and acute retinal necrosis syndrome. The elevated IOP associated with HSV uveitis is considered to be caused by inflammation of the trabecular meshwork as well as blockage of the meshwork by inflammatory cells.
HSV is a relatively common cause of uveitis and has been estimated to occur in approximately 9% of nontraumatic iritis cases. The average age of onset has been estimated at 46 years. Elevated IOP is remarkably common in HSV uveitis, which is interesting considering that many forms of uveitis show no elevation in IOP. This is postulated to be due to the shutdown of the ciliary body secondary to inflammation. One study showed that more than 90% of patients with herpetic uveitis had an elevation in IOP of more than 23 mm Hg. An intriguing finding in HSV uveitis is that the majority of patients (about 71%) will have a recurrence, as was the case with our patient.
A study by Van der Lelij et al evaluated 31 patients with anterior uveitis, sectoral iris atrophy and no history of keratitis, characteristics that were similar to those of our patient. They were able to obtain aqueous samples in 24 of these patients; 83% of patients were found to have evidence of HSV in their aqueous, while 13% had evidence of varicella zoster virus (VZV). The VZV patients were found to have an average age of 65 years, while the HSV patients had an average age of 33.7 years. This implies that these clinical features are suggestive of HSV in younger patients and VZV in older patients. The patients in this study also had an appearance of pigment dispersion that was seen in our patient. The pigment dispersion is believed to be due to stromal iris atrophy.
The arm of the Herpetic Eye Disease Study that was published in 1996 on the treatment of uveitis secondary to HSV was unable to recruit its target of 104 patients. In the 50 patients who were recruited and treated with oral acyclovir 400 mg by mouth five times per day, the researchers were unable to show a statistically significant benefit of treatment. However, there was a trend toward significance; 50% of the patients treated with acyclovir were considered to be treatment failures, while 68% of the placebo-treated patients were considered treatment failures.
Treatment
Our patient was not treated with acyclovir and instead was treated with topical corticosteroids and topical IOP-lowering medications. She responded well, and visual acuity and IOP again returned to normal. The keratic precipitates resolved. These results are considered typical in HSV uveitis. It typically responds well to minimal intervention and rarely leads to significant permanent loss in visual acuity.
For more information:
- Matthew Swanic, MD, and Chandrasekharan Krishnan, 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.
- Edited by Isabel M. Balderas, MD, and Tom Hsu, MD. Drs. Balderas and Hsu 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. Balderas and Hsu have no direct financial interest in the products mentioned in this article, nor are they paid consultants for any companies mentioned.
References:
- Green LK, Pavan-Langston D. Herpes simplex ocular inflammatory disease. Int Ophthalmol Clin. 2006;46(2):27-37.
- Herpetic Eye Disease Study Group. A controlled trial of acyclovir for iridocyclitis caused by herpes simplex virus. Arch Ophthalmol. 1996;114(9):1065-1072.
- Jones R 3rd, Pasquale LR, Pavan-Langston D. Herpes simplex virus: an important etiology for secondary glaucoma. Int Ophthalmol Clin. 2007;47(2):99-107.
- Miserocchi E, Waheed NK, et al. Visual outcome in herpes simplex virus and varicella zoster virus uveitis: A clinical evaluation and comparison. Ophthalmology. 2002;109(8):1532-1537.
- Tugal-Tutkun I, Urgancioglu M. Bilateral acute depigmentation of the iris. Graefe’s Arch Clin Exp Ophthalmol. 2006;244(6):742-746.
- Van Der Lelij A, Ooijman FM, Kijlstra A, Rothova A. Anterior uveitis with sectoral iris atrophy in the absence of keratitis: A distinct clinical entity among herpetic eye diseases. Ophthalmology. 2000;107(6):1164-1170.
- Yamamoto S, Pavan-Langston D, et al. Possible role of herpes simplex virus in the origin of Posner-Schlossman syndrome. Am J Ophthalmol. 1995;119(6):796-798