DR. KIRANDEEP KAUR

DR BHARAT GURNANI, DR. VEENA KANNVSAMY

Abstract

Aim-To report three cases of familial microspherophakia.Methods-13 years old girl presented with a BCVA of 20/120 in the RE and 20/80 in LE. Slit lamp examination (SLE) revealed Van Herick, grade 1, with iridophacodonesis.The elder sibling had BCVA in RE of 20/1200 and denied PL in LE.SLE of RE revealed a corneal scar, a cataractous lens in AC, and iridocorneal adhesions.LE revealed axial proptosis, ciliary staphyloma with scleral thinning, hazy cornea, and cataractous anteriorly dislocated lens.The eldest sibling also has reduced visual acuity in BE.BCVA in RE was 20/80, and PL in OS. SLE in RE revealed iridodonesis with aphakia and lens in the vitreous.LE had hazy cornea, fixed dilated pupil, iridodonesis with freely mobile lens from AC to the vitreous. Result –All were diagnosed as microspherophakia, and a tailored approach was used to manage these cases.

Conclusion-Kaleidoscopic presentations should be born in mind, and a tailored approach used to diagnose and manage such cases.