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J.A. King, J.C. Affeldt, M.R. Agarwal; Etiologic Profile of Vortex Keratopathy . Invest. Ophthalmol. Vis. Sci. 2003;44(13):1381.
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Purpose: The distinctive whorl-shaped epithelial pattern of vortex or hurricane keratopathy (cornea verticillata) has been ascribed to a number of etiologies including Fabry disease, drug toxicity, corneal transplants, band keratopathy, corneal edema, and healing abrasions. The purpose of this review was to identify for the first time the etiologic spectrum, frequency, and diagnostic significance of vortex keratopathy in a large consecutive series of vortex keratopathy patients. Methods: Retrospective chart review. Diagnosis of neurotrophic keratitis required classic Mackie system corneal degenerative changes as highlighted by fluorescein stain, coupled with significant loss of corneal sensation versus controls as quantitated by Cochet-Bonnet aesthesiometry (5.6 mm versus 56.3 mm respectively; P=0.0001). Results: Seventy-five eyes were identified with vortex keratopathy. In descending order of frequency, the etiologies included neurotrophic keratitis (59; 79%), drug toxicity (10; 13%), epidemic keratoconjunctivitis (3; 4%), Thygeson's superficial punctate keratitis (2; 3%), and pellucid marginal degeneration (1; 1%). Four eyes with corneal transplantation and four with band keratopathy were also identified, but all were primarily affected with neurotrophic keratitis. Conclusions: The previously unidentified entity of neurotrophic keratitis was by far the most common cause of vortex keratopathy in this series, accounting for 79% of all cases. Included within this group were the previously distinct conditions of corneal transplantation and band keratopathy. This overwhelming neurotrophic keratitis etiologic preponderance suggests that vortex keratopathy may represent a useful if not pathognomonic clinical marker for the diagnostically challenging disease of neurotrophic keratitis.
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