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Anterior chamber inflammation, iris depigmentation noted 25 years after cataract extraction
A woman had decreasing vision in her right eye over a 5-year period.
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A 64-year-old Caucasian woman was referred to the New England Eye Center after her ophthalmologist noted gradually decreasing vision in the right eye. The patient had enjoyed 20/25 vision for many years after uncomplicated cataract surgery in the right eye 25 years ago, but she had a decrease in vision to 20/50 over the last 5 years.
Background
The ophthalmologist’s exam showed diffuse iris atrophy, an enlarged and slightly irregular pupil, and posterior capsular opacification. A YAG capsulotomy was then performed in the right eye, but low-grade iritis was found after the procedure. The patient was started on prednisolone acetate 1% five times a day and hyoscine daily in her right eye with no improvement in her anterior chamber inflammation or vision.
The patient’s ocular history was otherwise significant for Fuchs’ corneal dystrophy and blepharitis. Her medical history was notable for Lyme disease 9 months prior that presented as Bell’s palsy and was treated with 1 month of intravenous antibiotics. She also suffered from rosacea, irritable bowel syndrome and lumbar spondyloarthropathy. She denied other joint pain but admitted to rare cold sores. Her medications included metronidazole cream and omeprazole.
Examination
On examination, the patient’s vision was 20/80 in the right eye and 20/30 in the left eye with a myopic prescription and 2.5 D of astigmatism in her right eye only. Her pupil was 5 mm and fixed in the right eye without a relative afferent pupillary defect, and her extraocular movements and IOPs were normal. Her slit lamp exam was significant for rosacea, mild blepharitis and 3+ guttata without corneal edema in both eyes. She had blue irises with diffuse depigmentation in her right eye (Figure) as well as trace cell and flare in the right anterior chamber. A sulcus IOL was well positioned in the right eye with an open posterior capsule. She had a mild nuclear sclerosis cataract in the left eye. Her fundus exam was unremarkable.
Figure. External exam revealed a pharmacologically dilated pupil in the right eye and a slightly depigmented iris with transillumination defects. Slit lamp exam showed trace anterior chamber inflammation in the right eye.
Image: Renz J, Raizman MB
A full work-up by a rheumatologist was done and revealed a high ANA in a 1:640 speckled pattern and elevated SSA and SSB antibodies. Her rheumatoid factor was also mildly elevated, and her lysozyme titer was high-normal. All other laboratory tests were unremarkable.
What is your diagnosis?
Iris depigmentation
Although it was difficult to detect obvious heterochromia in this patient, her affected iris appeared diffusely depigmented with transillumination defects. It was considered to be hypochromic in this patient, although atrophy in blue irises tends to appear hyperchromic due to exposure of the retinal pigment epithelium.
The differential diagnosis for this patient included inflammatory causes such as Fuchs’ heterochromic uveitis, Posner-Schlossman syndrome, and chronic autoimmune uveitis such as that related to Lyme disease or lupus. However, with normal IOP, a full treatment for Lyme disease, and refractory low-grade uveitis atypical of rheumatologic disease, the patient was diagnosed with Fuchs’ heterochromic uveitis. Also on the differential was trauma because IOLs can sometimes cause sectoral chafing of the iris, herpes iritis, congenital Horner’s syndrome and asymmetrical pigment dispersion syndrome, although these seemed less likely.
Discussion
Fuchs’ heterochromic uveitis, also known as Fuchs’ heterochromic iridocyclitis, accounts for 3.5% to 8% of all uveitis and is characterized by chronic low-grade uveitis without formation of synechiae or acute exacerbations, iris heterochromia, early cataract formation, small stellate keratic precipitates and, in some cases, glaucoma. It is unilateral in 90% to 95% of cases. Our patient did not have keratic precipitates, but this may have been confounded by her corneal dystrophy or ongoing corticosteroid treatment. Vitreous opacities are also often seen, and floaters are one of the most common presenting symptoms. Vitreoretinal scarring is sometimes noted, but these patients typically have positive toxoplasma serology.
A classic association with Fuchs’ uveitis is Amsler sign, in which a hyphema occurs after cataract surgery or paracentesis, as well as other minute traumas such as gonioscopy, applanation tonometry or peribulbar anesthesia. Interestingly, unilateral astigmatism has also been associated with this disease, with an average of 2.2 D and disparity greater than 0.75 D. The mechanism for this has been proposed to be either an immunologic reaction against the corneal epithelium and stroma or a localized form of neural crest disease.
As many of these signs and symptoms are common with other disease processes and can be easily missed or overlooked, there is often a significant diagnostic delay. The time from presentation to an ophthalmologist to diagnosis ranges from within 3 months to 26 years, with a mean of 3 years. Patients typically present in the third to fourth decade of life with no gender or racial predisposition, most commonly with a complaint of decreased vision and floaters. Cataract is the most common complication, with a higher risk of posterior capsular opacification (20% to 45%) and IOL precipitates after cataract surgery. Glaucoma is the most serious complication and develops in about 20% of patients. It does not appear to be as responsive to medical and surgical therapy as other forms of open-angle glaucoma.
The etiology of Fuchs’ uveitis is not well known; however, recent research has implicated the rubella virus. Quentin and colleagues found rubella antibodies in 52 of 52 patients with clinically defined Fuchs’ uveitis. Epidemiologic evidence is also compelling. In a retrospective study of nearly 4,000 patients by Birnbaum and colleagues, a clinically significant reduction of affected patients was seen in those who had birthdays after the rubella vaccine was instituted. Other associations have also been proposed, including cytomegalovirus, toxoplasmosis and herpes simplex virus. It is possible that Fuchs’ heterochromic uveitis is a syndrome that may result from a variety of infectious agents.
The prognosis for this disease is quite good, with the majority of patients who do not develop glaucoma maintaining vision of 20/40 or better. The treatment of uveitis is sometimes also discontinued because patients may continue to show a small amount of inflammation despite the chronic use of topical corticosteroids. At her 6-month follow-up visit, our patient had stable vision and normal IOPs while being maintained on a low-dose topical steroid medication.
References:
- Ashwin PT, Tambe K, Quinlan M. Unusual presentation of Amsler’s sign in Fuchs’ heterochromic uveitis. J Cataract Refract Surg. 2006;32(9):1579-1580.
- Birnbaum AD, Tessler HH, Schultz KL, et al. Epidemiologic relationship between Fuchs heterochromic iridocyclitis and the United States rubella vaccination program. Am J Ophthalmol. 2007;144(3):424-428.
- Bonfioli AA, Cui AL, Orefice F. Fuchs’ heterochromic cyclitis. Semin Ophthalmol. 2005;20(3):143-146.
- Chee SP, Jap A. Presumed Fuchs heterochromic iridocyclitis and Posner-Schlossman syndrome: comparison of cytomegalovirus-positive and negative eyes. Am J Ophthalmol. 2008;146(6):883-889.
- Cunningham ET Jr, Baglivo E. Fuchs heterochromic iridocyclitis – syndrome, disease, or both? Am J Ophthalmol. 2009;148(4):479-481.
- Faramarzi A, Soheilian M, Jabbarpoor Bonyadi MH, Yaseri M. Corneal astigmatism in unilateral Fuchs heterochromic iridocyclitis. Ocul Immunol Inflamm. 2011;19(3):151-155.
- Javadi MA, Jafarinasab MR, Araghi AS, Mohammadpour M, Yazdani S. Outcomes of phacoemulsification and in-the-bag intraocular lens implantation in Fuchs’ heterochromic iridocyclitis. J Cataract Refract Surg. 2005;31(5):997-1001.
- Norrsell K, Sjödell L. Fuchs’ heterochromic uveitis: a longitudinal clinical study. Acta Ophthalmol. 2008;86(1):58-64.
- Quentin CD, Reiber H. Fuchs heterochromic cyclitis: rubella virus antibodies and genome in aqueous humor. Am J Ophthalmol. 2004;138(1):46-54.
- Rothova A. The riddle of Fuchs heterochromic uveitis. Am J Ophthalmol. 2007;144(3):447-448.
For more information:
- Jennifer Renz, MD, and Michael B. Raizman, 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; website: www.neec.com.
- Edited by Kavita Bhavsar, MD, and Michelle C. Liang, MD. They 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; website: www.neec.com.