This patient was diagnosed with anterior lenticonus, which was responsible for the drop in vision in the right eye. The patient was referred out for a complete physical and work-up, with the ultimate diagnosis being as suspected, Alport Syndrome.

Also known as Hereditary Nephritis, Alport Syndrome is an X-linked inherited disorder that involves damage to the kidneys, blood in the urine, and occasionally loss of hearing, as well as the anterior lenticonus seen here.

These patients develop chronic glomerulonephritis with progressive loss of kidney function and eventually can lead to end-stage renal disease at an early age, anywhere from adolescence to age 40 or so.

Any young male with anterior lenticonus should be worked up for Alport Syndrome, especially if kidney trouble and/or hearing loss are also present. A careful family history is important, as well. In cases of significant vision loss, cataract surgery can be performed.