A Teenager with Vernal Keratoconjunctivitis and Pellucid Marginal Degeneration, Presenting with Exotropia

BACKGROUND: Vernal keratoconjunctivitis (VKC), an allergic disease, has a known association with ectatic disorders of the cornea. Pellucid marginal degeneration (PMD) is a bilateral, asymmetrical, ectatic disorder of the cornea characterized by inferior corneal thinning. We report a case of sensory exotropia due to PMD in association with VKC. CASE DETAILS: A 19-year old boy with a history of VKC presented with exotropia of the right eye of 3 years’ duration. His unaided vision in his right eye was 1/60 and in his left eye it was 6/36. On examination, both eyes had high against the rule astigmatism, which was more in the right eye. There was exotropia of 15º (40 prism diopter base in) in the right eye with suppression (Worth four dot test). Corneal examination in both eyes showed inferior band thinning, 2 mm above the inferior limbus, extending from 4 to 8 clock hours, with bulging of the cornea just above the thinning. The clinical features were suggestive of PMD, which was supported by his corneal scans – Atlas, Pentacam, and Optovue. Although he was undergoing treatment for VKC, the onset of PMD and decrease in vision went unnoticed. The asymmetric error which was not corrected during the sensitive period of visual development led to sensory exotropia. CONCLUSION: A child with VKC should undergo regular refraction so as not to miss any ectatic changes occurring in the cornea. A delay in diagnosing corneal ectasia may negate the possibility of collagen cross-linking which prevents progression of ectasia. If visual rehabilitation is delayed beyond the age of visual maturation, it can lead to strabismus, suppression, and loss of binocular function.