Optic Disc Swelling and Vision Loss in a Patient with Cystic Fibrosis and Diabetes

Advances in cystic fibrosis management have significantly improved life expectancy in these patients. However, we are now faced with a growing number of long-term extrapulmonary consequences of this disease, including ophthalmic complications of diabetes in cystic fibrosis patients. We present a unique report that documents a case of diabetic papillopathy progressing to nonarteritic anterior ischemic optic neuropathy resulting in vision loss in a patient with CF and diabetes. It highlights the potentially devastating consequences of longstanding diabetes in CF patients. 1. Introduction Cystic fibrosis-related diabetes (CFRD) is a well-known complication of cystic fibrosis (CF) and is often diagnosed in early adulthood [1]. With recent increases in patient survival, ophthalmic complications of diabetes are beginning to emerge. To our knowledge, we report the first case of diabetic papillopathy (DP) in a CF patient, which was complicated by nonarteritic anterior ischemic optic neuropathy (NAION) and permanent vision loss. We report this unique case to draw attention to the clinical presentation of this potential ophthalmic complication of diabetes in CF patients. 2. Case Presentation We present a 37-year-old woman with moderate-to-severe CF lung disease since the age of 5. She was diagnosed with diabetes at a different institution, when she presented with mild DKA at 24 years of age. Her specific diabetes diagnosis was ambiguous due to a paucity of information available to our center, as well as poor recollection of events. Her c-peptide was 408 one month after her diagnosis with diabetes, and subsequently she was treated as CFRD. Her c-peptide became undetectable 4 years after her initial diabetes diagnosis. She has been suboptimally controlled on a multiple daily injection (MDI) regimen with insulin glargine and insulin lispro. She has also been using carbohydrate counting and correction factor insulin dosing. Her hemoglobin A1C was consistently between 8.7 and 11.2%. Her diabetes was difficult to control due to variable nutritional intake, hypoglycemia and the fear of such, and resistance to proper monitoring. Her history was significant for left-eye diabetic retinopathy identified on fundoscopic screening, pancreatic exocrine insufficiency, and microalbuminuria. She presented with a two-day history of sudden-onset severe bilateral headache, periorbital pain, and pressure, followed by a superior visual field deficit involving her left eye. On physical exam, she was normotensive. There was no evidence of focal neurological deficits. Fundoscopic