The True Dysfunctional Lens

To describe the diagnosis challenges and surgical management of anterior lenticonus in Alport's Syndrome.

Private practice

A 36 year-old patient refers progressive decreased visual acuity and an increased myopia in both eyes. He has been seen by multiple ophthalmologists who could not find the cause of his poor vision. On examination, despite a perfectly clear lens, it is possible to see a severely distorted and bulged anterior capsule. Anterior lenticonus caused by Alport's Syndrome was diagnosed. Clear lens extraction was performed with the implantation of a monofocal IOL. The patient had his vision fully restored with surgery. This case illustrates this rare pathology that can be challenging to diagnose. Surgical management is required and proper technique is mandatory.

The awareness of this condition has to be present everytime a young male patient complains of poor vision with no clear identifiable cause. The careful examination of the crystalinne lens is mandatory and an oil-droplet sign may be the only hint of this diagnosis. Clear lens extraction is the ultimate treatment and can restore vision completely for these patients.