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Young woman experiences painful decline in vision
A creamy white peripheral iris mass was seen in her right eye.
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A 25-year-old Caucasian woman with no ocular history was referred to Tufts New England Eye Center from an outside ophthalmologist for 5 days of pain, redness and progressive decline in vision in the right eye. She had a history of intravenous drug use and, 1 week before presentation, was hospitalized for endocarditis with methicillin-sensitive Staphylococcus aureus bacteremia. She had subsequently been transferred to a facility to continue IV vancomycin and ceftriaxone. Five days before presentation at Tufts, she began having redness and pain in the right eye for which she was started on tobramycin drops without improvement. She denied drug use since hospital admission, recent steroid use, ocular trauma, history of autoimmune disease, cough, shortness of breath, history of sexually transmitted diseases or rashes.
Examination
Upon examination at Tufts, the patient’s best corrected visual acuity was 20/100 in the right eye and 20/25 in the left eye. Pupils were equal, round and reactive without afferent pupillary defect. IOPs were 14 mm Hg in both eyes. In the right eye, slit lamp examination revealed mild upper lid edema with 3+ diffuse conjunctival injection. There were inferior nonpigmented keratic precipitates. The anterior chamber showed 4+ cell with an inferior sliver hypopyon and trace fibrin at the pupillary margin. There was iris and limbal vessel dilatation with a creamy white peripheral iris mass located at approximately 11 o’clock (Figures 1 and 2). Dilated fundus exam demonstrated no vitreous cell, retinal lesions or vasculitis. Anterior and posterior examination of the left eye was unremarkable.
Source: Lauren Bierman, MD, and Caroline Baumal, MD
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Iris nodule
Anterior uveitis with an iris nodule in the setting of recent bacteremia and endocarditis is highly suspicious for an infectious septic embolus. Differential diagnosis of anterior uveitis with an iris nodule includes infectious, autoimmune and malignant etiologies. In infectious cases, nodules may represent septic emboli or be present in association with infectious uveitis due to herpes simplex or herpes zoster, cytomegalovirus, mycobacterium, tuberculosis, syphilis and rickettsial infections. Fungal associations have also been reported, namely Cryptococcus, coccidioidomycosis and Candida. Autoimmune etiologies including sarcoidosis, Vogt-Koyanagi-Harada syndrome and multiple sclerosis, and malignant etiologies including lymphoma, leukemia and metastatic disease should also be considered. Fuchs’ heterochromic iridocyclitis is another form of anterior uveitis associated with iris nodules.
Discussion
Iris nodules can be present in many forms of anterior uveitis, including infectious, autoimmune and neoplastic etiologies. In general, iris nodules associated with infectious uveitis appear creamy-white, fluffy and soft. The nodules are typically unilateral and persist or grow despite corticosteroid therapy. Typically, there is a marked inflammatory response in the anterior chamber and/or the vitreous. There has been a wide variety of bacterial and fungal species reported in the literature as the infectious agents. In particular, ocular tuberculous is an important consideration. Ocular tuberculosis is a chronic granulomatous disease with varied ocular manifestations, the most common of which is disseminated choroiditis with multiple, discrete, yellow lesions in the posterior pole, with or without macular edema and varying degrees of associated vitritis. Iris nodules are a rare manifestation of ocular tuberculosis, yet do appear in the literature. Sarcoidosis should be considered in the differential diagnosis of any patient with intraocular inflammation because, like tuberculosis, it has numerous manifestations and can affect any ocular tissue. Classically, sarcoidosis causes bilateral chronic uveitis with mutton fat keratic precipitates. Unlike tuberculosis, iris nodules are a common finding in ocular sarcoid and are present in about 10% of patients. These nodules are characterized by granulomatous inflammation at the pupillary margin (Koeppe nodules) or in the iris stroma (Busacca nodules). Sarcoid often has cutaneous involvement such as orbital, eyelid or conjunctival granulomas, and the presence of these can provide a useful clue as to the etiology of the iris nodule. Iris nodules due to Vogt-Koyanagi-Harada syndrome occur during the acute uveitic phase, while those due to multiple sclerosis are often present in conjunction with pars planitis and, much more rarely, granulomatous anterior uveitis. Iris nodules in Fuchs’ heterochromic iridocyclitis tend to be small and transparent, while metastatic neoplasms appear more gray-white and may be accompanied by hyphema, iris atrophy, hypopyon and secondary glaucoma. Anterior uveitis with iris nodules can also occur in lymphoma and leukemia; however, this presentation is much less common than is involvement of the posterior segment.
Due to the rarity of this presentation, there is no real consensus on whether iris nodule biopsy is necessary for diagnosis or whether intravitreal antibiotics are beneficial. In the largest review of more than 1,300 uveitis cases evaluated at a tertiary care facility, three cases of infectious uveitis with iris nodules were identified. This accounts for 0.22% of all uveitis patients evaluated at this facility from 1985 to 2001. Anterior chamber paracentesis was performed in all three cases, and one case also included aspiration of a portion of the iris nodule. All three patients were admitted to the hospital to identify a systemic source for a suspected intraocular infection, and they were treated with parenteral antibiotics. Anterior chamber paracentesis was a useful tool in establishing a diagnosis and treatment in these cases.
While infectious uveitis is an uncommon cause of iris nodules, early recognition and treatment are important. Steroids and other immunosuppressive agents used to treat noninfectious uveitis can prolong or worsen the symptoms and outcomes of a patient with infectious uveitis. Therefore, clinical suspicion and a thorough history and review of systems are critical in differentiating patients with noninfectious and infectious uveitis.
The patient immediately underwent an anterior chamber and intravitreal tap and injection to obtain fluid samples and to deliver vancomycin and ceftazidime directly to both chambers. Ophthalmology consulted the Infectious Disease service, which recommended that no further infectious workup was indicated at that time in the setting of known recent methicillin-sensitive S. aureus bacteremia and endocarditis. While awaiting the results of aqueous and vitreous culture, the patient was started on topical steroids and Vigamox (moxifloxacin, Alcon) every 2 hours and cyclopentolate three times daily in the right eye. Unfortunately, at this point, the patient refused all further ophthalmology care so full clinical course and outcome remain unknown.
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- For more information:
- Lauren Bierman, MD, and Caroline Baumal, MD, can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; website: www.neec.com.
- Edited by Aubrey R. Tirpack, MD, and Astrid C. Werner, MD. They can be reached at the New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; website: www.neec.com.