Bilateral anterior lenticonus associated with Alport syndrome

Abstract

Anterior Lenticonus is a rare congenital anomaly of the eye characterized by a conical protrusion of the crystalline lens capsule and the underlying cortex in anterior chamber leading to high axial myopia; most cases are associated with Alport syndrome. Alport syndrome (AS) is an inherited disorder of many forms, most commonly X-linked. The main abnormality is deficient synthesis of type IV collagen, the main component of basement membranes. It typically presents with the classic triad of progressive glomerulonephritis, progressive high tone hearing loss, and several ocular signs; the most pathognomonic of which is the presence of anterior lenticonus.We reported a 21 year old female of alport syndrome presenting with gradual, progressive diminished vision in both eyes associated with anterior lenticonus and dot and fleck retinopathy with progressive sensorineural hearing loss.