Primary Bullous Keratopathy in a Patient With Werner Syndrome Treated With Corneal Transplant.

Here, we present, to the best of our knowledge, the first case of Werner syndrome with corneal blindness due to bilateral primary bullous keratopathy. Werner syndrome is a rare autosomal recessive disorder characterized by features of premature aging, insulin-dependent diabetes mellitus, osteoporosis, atherosclerosis, hypergonadotrophic hypogonadism, hypertriglyceridemia, scleroderma-like skin changes, and sarcomas. Among ocular manifestations, cataracts, cystoid macular edema, and retinal detachment have been reported. Because these patients show features of premature aging, they have decreased corneal endothelial function and delayed fibroblast growth. To date, there are few reports of wound dehiscence, bleb formation, and bullous keratopathy following surgical insult that have usually occurred after cataract surgery in patients with Werner syndrome. There have been no reports in the literature regarding Werner syndrome presenting with primary corneal decompensation without any inciting factor. Our patient with Werner syndrome had primary bilateral bullous keratopathy and bilateral corneal blindness for 10 years and was eventually rehabilitated by corneal transplant. Hence, this case highlights the importance of early referral of such patients to the ophthalmologist for prompt diagnosis and early treatment so that blindness could be avoided.