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Atypical Gout-Associated Band Keratopathy CE Credit CE Credit - Case Report Atypical Gout-Associated Band Keratopathy Sara Moses, OD; Lisa Schifanella, OD, MS; Ellen Prewitt, OD, FAAO Abstract enzyme (ACE), lysozyme and uric acid. A chest x-ray was also Band keratopathy is a chronic condition that involves the ordered to rule out sarcoidosis. The results, summarized in deposition of crystals in the anterior layers of the cornea. There Table 1, revealed no significant abnormalities, particularly no are many etiologies, both ocular and systemic, that can result elevation of uric acid levels. Consequently, the band keratopathy in the formation of these crystals. These conditions include was presumed idiopathic and treated with artificial tears three chronic ocular inflammation, hypercalcemia, and gout. Herein times per day in each eye. The patient was also sent for cataract we report a case of band keratopathy due to gout and discuss surgery in order to improve visual function. the possible systemic etiologies and management of band keratopathy.. Initial best corrected visual acuities following cataract surgery were 20/20 in each eye. However, over the course of a year, Case Report vision was reduced to the 20/25 level OD and 20/40 level OS, A 55-year-old retired African American male presented with dry with no improvement by pinhole testing. Slit lamp examination eye complaints and mild blurry vision. He reported that comfort revealed progression of band keratopathy in each eye and was was improved with the use of artificial tears, but that his vision now within the visual axis of the left eye (Figures 1&2). remained blurry. Over the prior two years he had been followed for a slowly progressing peripheral band keratopathy OU. His Due to the advancement of band keratopathy, blood work medical history was significant for type 2 diabetes, hypertension, was repeated for magnesium, phosphorous, and uric acid sleep apnea, alcohol dependence, and chronic pain. levels. Results of lab testing revealed elevated uric acid levels (Table 2), which suggested a possible diagnosis of gout. Best corrected visual acuities measured 20/40-2 OD and The patient was referred to his primary care physician who 20/30- OS and showed no improvement with pinhole testing. confirmed the diagnosis and began treatment with 100 mg of Extraocular muscle and pupil functions were normal in each allopurinol daily. Subsequent eye examinations have revealed eye. Intraocular pressures were 16 mmHg OD and 14 mmHg OS. no progression of band keratopathy in either eye since initiation of treatment for gout. The patient was referred to a corneal Slit lamp examination revealed normal lids and adnexa. The specialist for evaluation due to the reduction in visual acuity, band keratopathy had progressed across the inferior cornea and the determination was made to continue with artificial tear OU but was not yet within the visual axis of either eye. Dilated therapy and monitoring for the time being. fundus examination revealed significant cataracts and an unremarkable posterior segment in each eye. Due to the progressive nature of the presumed calcific band Table 1. keratopathy, labs were ordered including complete blood count Lab Test Result Reference Range (CBC), basic metabolic panel (Chem-8), angiotensin-converting BUN 11 7-18 mg/dL CO2 31 18.4-27.4 mEq/L Creatinine 1.11 0.7-1.4 mg/dL Glucose 109 70-105 mg/dL Sara Moses, OD- Charlie Norwood Veterans Affairs Medical Center Chloride 103 98-107 mmol/dL Lisa Schifanella, OD, MS - Tuscaloosa Veterans Affairs Medical Center Potassium 4.3 3.5-5.1 mmol/L Ellen Prewitt, OD, FAAO - Tuscaloosa Veterans Affairs Medical Center Correspondence to: Sara Moses OD Sodium 143 136-146 mmol/L Charlie Norwood Veterans Affairs Medical Center Uric acid 6.9 2.6-7.2 mg/dL 950 15th Street, Augusta, GA 30904 E-mail: [email protected] ACE 38 9-67 U/L The authors have no financial or proprietary interest in any material or method Calcium 9.5 8.9-10.4 mg/dL mentioned in this article. This article has been peer reviewed. Atypical Gout-Associated Band Keratopathy 375 Figure 1. Figure 2. Discussion Band keratopathy is an opacification of the cornea due to Table 2. deposition of crystals in Bowman’s layer. Typically, the band begins in the corneal periphery, slowly progressing centrally Lab Test Result Reference Range and toward the visual axis. Eventually, the deposits coalesce Magnesium 2.3 1.6-3 mg/dL in a horizontal band-like plaque from limbus to limbus. The Phosphorus 2.9 2.3-4.3 mg/dL deposits are most frequently composed of calcium, although other particles, such as cystine or uric acid, can also have a Uric acid 8.7 2.6-7.2 mg/dL similar presentation. The appearance of the plaques can range from flat and increase with chronic ocular inflammation, such as in uveitis visually insignificant gray-white opacities to bulky flakes and with corneal endothelial compromise. with devastating visual consequences.18 The plaques characteristically have a “cheesy” appearance with clear holes Ocular Associations in the opacified band which aids in the differentiation between A strong association exists between band keratopathy and band keratopathy and other corneal dystrophies or opacities. chronic ocular inflammation from sarcoidosis, Vogt Koyanagi These holes occur where corneal nerves pass through Harada syndrome, and HLA B-27-related disorders such as Bowman’s membrane and inhibit the deposition of crystals.5,18 juvenile idiopathic arthritis and ankylosing spondylitis. In Visual acuity and ocular discomfort depend on the severity the case of sarcoidosis, the link is theorized to be related to and location of the disease. As the deposits collect within the elevated serum calcium and vitamin D levels.9,16 Although cornea, the epithelium can become rough and irregular. If the ocular involvement with sarcoidosis commonly presents as corneal epithelium degrades, recurrent corneal erosions may uveitis, iris granulomas, or periphlebitis, band keratopathy occur. occurs in 4-5% of ocular sarcoidosis cases. For patients with HLA-B27 associated uveitis, Verhagen et.al showed that band The development of the plaques in band keratopathy is thought keratopathy tends to develop in eyes with reduced vision, and to be a result of a chemical imbalance between the cornea and that the band keratopathy itself is rarely the cause of acuity the tear film.21 Precipitation of the plaques into the cornea occur loss.20 with an increase in calcium levels in the tear film or when the pH of the ocular surface becomes basic; occasionally both Ocular surgeries and injections are also known to be associated occur simultaneously.13 Broadly speaking, the levels of tear film with band keratopathy. The long-term presence of silicone oil calcium increase with elevated serum calcium levels as well in the eye after retinal detachment repair has been shown to as with significant evaporation of the tears. These conditions increase the risk of band keratopathy. This is especially true arise in keratitis sicca and a variety of systemic conditions that when the silicone oil enters the anterior chamber and contacts produce hypercalcemia such as end-stage renal disease and the corneal endothelium. Doostdar et.al proposed that because hyperparathyroidism. Ocular surface pH has been shown to silicone is known to promote calcium absorption in bones, it 376 Clinical & Refractive Optometry 32.3, 2021 Table 3. Band keratopathy associated Systemic Associations conditions2,16,17,18,21 Band keratopathy is associated with a variety of ocular and systemic conditions (Table 3). These conditions primarily revolve Ocular Idiopathic End-stage glaucoma Chronic corneal edema Ophthalmic drops around two factors: ocular inflammation and hypercalcemia. - Endothelial - With phosphates When there is no evidence of an ocular inflammatory condition compromise - Phosphate-based present in the setting of band keratopathy, it becomes necessary Chronic uveitis irrigation after alkali to assess for systemic cause. Lab testing is extremely useful - Herpes simplex burn in detecting the systemic conditions associated with band - Herpes zoster Phthisis bulbi - Acute retinal necrosis Aphakia with silicone oil keratopathy. A typical panel of labs for band keratopathy Corneal dystrophies Intraocular injection will include CBC, Chem-8, uric acid, ACE, and lysozyme. The Neurotrophic keratitis - Tissue plasminogen Chem-8 will provide information relating to kidney function and Keratitis sicca activator (tPA) electrolyte levels, including serum calcium levels. The CBC, Interstitial keratitis ACE, and lysozyme assist in assessing for chronic systemic Systemic Hypercalcemia: Gout inflammation. ACE and lysozyme, particularly when combined - End stage renal Cystinosis with a chest x-ray, are useful in detecting sarcoidosis, while uric disease Chemical exposure acid evaluates for gout. - Hyperparathyroidism - Mercury fumes - Sarcoidosis - Calcium bichromate - Milk-alkali syndrome Lithium Hypercalcemia is the primary etiology for band keratopathy - Paget disease Familial band keratopathy due to a systemic condition. The list of possible disease entities - Discoid lupus Vogt Koyanagi Harada that can result in hypercalcemia is extensive and includes end erythematosus syndrome stage renal disease, hyperparathyroidism, sarcoidosis, milk-alkali - Malignancy Juvenile Idiopathic - Tuberous sclerosis arthritis syndrome, Paget disease of bone, discoid lupus, erythematosus, - Excessive vitamin D Denosumab malignancy, tuberous sclerosis, and excessive vitamin D.9 As - Excessive calcium serum calcium levels
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