DR. VIVEK SINGH

Dr. RAKHI KUSUMESH, Dr. VIDYA BHUSHAN KUMAR, DR. PARUL SHARMA, Dr. AMBASTA ANITA

Abstract

Alport syndrome is a rare genetic disorder, characterized by progressive renal failure, hearing loss, and ocular abnormalities like anterior lenticonus, corneal opacities, cataract, temporal retinal thinning and fleck retinopathies. We report a case of 18 years old female presented as progressive decrease in vision for one year, unaware of hearing loss and kidney disease. We found that she was having bilateral anterior and posterior lenticonus with moderate sensorineural hearing loss and proteinuria without any family history. Herein, we aimed at highlighting the importance of detection of lenticonus in otherwise asymptomatic patients for early diagnosis and management of Alport syndrome and addressing the challenges in performing clear lens extraction with toric IOL to eliminate irregular astigmatism for better visual rehabilitation.