Ocular surface disease secondary to vitamin A deficiency in the developed world: it still exists.

Report of Cases. Case 1. A 79-yearold woman with a 15-year history of primary biliary cirrhosis and secondary Sjögren syndrome presented with a 1-year history of chronic conjunctivitis refractory to treatment with topical antibiotics. Three months prior, the patient underwent a tectonic penetrating keratoplasty in her right eye because of a perforated corneal ulcer. She had nyctalopia for 2 years. Her visual acuity was 20/200 OD and counting fingers at 3 ft OS. In addition to diffuse punctate epithelial erosions in both eyes, she had a peripheral corneal ulcer in the left eye, which was treated with topical antibiotics. Five days later, the ulcer had progressed to a small perforation. This was managed with cyanoacrylate glue and a bandage contact lens. Her vitamin A level was 20 μg/dL (to convert to micromoles per liter, multiply by 0.0349) (reference range, 38-98 μg/dL). After intramuscular vitamin A administration, the patient’s ocular condition stabilized, and she was discharged to follow up with her local ophthalmologist. Case 2. A 54-year-old woman with a 10-year history of primary biliary cirrhosis was referred for severe dry eyes starting 6 months prior, which were unsuccessfully treated with cyclosporine A, 0.05%, and frequent lubrication. On examination, her visual acuity was 20/25 OD and 20/30 OS. Schirmer I test results were abnormal in both eyes. She had diffuse punctate epithelial erosions with filaments in both eyes and interpalpebral lissamine green staining. Her vitamin A level was 11 μg/dL, and she started treatment with vitamin A ointment, 0.01%, once daily at bedtime and oral vitamin A supplementation. Her symptoms and staining pattern improved significantly over 1 month. Case 3. A 9-year-old autistic boy was referred for visual loss, in addition to red eyes and decreased night vision, over the past 4 months as noted by his mother. He had been diagnosed as having chronic conjunctivitis, which was unsuccessfully treatedwithacorticosteroid-antibiotic combination.Onexaminationunder anesthesia, he had chronic bilateral nonhealing epithelial defects, a relativeafferentpupillarydefect inthe left eye, and profound optic atrophy in the left eye greater than in the right eye. His visual acuity was not attainable because of lack of cooperation. It was later discovered that his diet consisted exclusively of french fries. His vitamin A level was 3 μg/dL. His epithelialdefectshealedwithfrequent lubricationandremainedhealedwith oral vitamin A supplementation. Case 4. A 26-year-old homeless and severely depressed woman presented with recurrent corneal ulcers and corneal perforation necessitating penetrating keratoplasty in the right eye. Her ocular condition was thought to be secondary to severe atopic disease. On examina-