Dr.GAURAV CHAUHAN

Dr. SUBHRA NAG

Abstract

Alport syndrome is a hereditary disease with characteristic ocular and systemic manifestations due to defective type 4 collagen. We present a case of Alport syndrome with spontaneous absorption of crystalline lens. A 14-year-old boy presented with painless, progressive diminution of vision for 4 years in both eyes (OU). On examination, best corrected visual acuity (BCVA) was OD HM, OS 5/60. On slit lamp examination, OD had anterior lenticonus, thinning of anterior capsule and posterior subcapsular cataract. OS had centrally deficient anterior capsule with peripheral fibrosis; crystalline lens was absent with few cortical remnants over posterior capsule. On systemic examination, sensorineural deafness and parenchymal kidney disease were detected, leading to the diagnosis of AIport syndrome. After cataract surgery, patient achieved BCVA of 6/6 in OU. Although spontaneous rupture of anterior capsule has been reported in Alport syndrome, spontaneous absorption of lens is rare.