Dissecting Aortic Aneurysm 55 Years after Diagnosis of Iris Flocculi

Purpose: The aim of this study is to report the clinical features, imaging studies, surgical removal, and pathology of congenital iris flocculi in a patient who developed a life-threatening dissecting aortic aneurysm after 55 years of follow-up. Methods: Documentation with iris photography was performed from age 18 to 55 years, at which time anterior segment optical coherence tomography (OCT) was performed. At the time of subsequent cataract surgery, the iris flocculi were removed in each eye and submitted for light and electron microscopy. Results: The size and shape of the iris flocculi waxed and waned over the years but caused no visual disturbance. Anterior segment OCT demonstrated clear, round cysts with thin lining and no solid component. At the age of 55, congestive heart failure from a dissecting aortic aneurysm was discovered and surgically repaired. Subsequent bilateral cataract surgery and cyst removal were performed, and the iris flocculi were studied with light and electron microscopy. Conclusions: Congenital iris flocculi cause little, if any, visual impairment in most cases. However, they are occasionally associated with dissecting aortic aneurysm due to a shared mutation in smooth muscle that affects both the iris and the aorta. Patients with iris flocculi should be monitored periodically for aortic abnormalities.