Abstract

INTRODUCTION- Alport syndrome (AS) is a genetically acquired condition characterized by triad
of impaired renal function, sensorineural hearing loss and Anterior Lenticonus (AL) resulting from a
weakened anterior lens capsule.
PRESENTATION- A 25-year-old gentleman with pseudophakic right eye presented with gradually progressing diminished vision of the left eye. Slit lamp examination revealed AL, posterior polymorphous corneal dystrophy and bilateral macular lamellar hole with associated sensory neural hearing loss. On suspicion a renal workup was done which was deranged and USG abdomen showed bilateral contracted kidneys. He and his sibling with similar findings underwent hemodialysis followed by renal transplant.
CONCLUSION- AL apart from the evident lenticular myopia also has typical Higher Order abberations. The ophthalmic findings should raise the suspicion of AS helping diagnose life threatening systemic complications for which intervention can be done at the earliest.