Zonular weakness in patients with gyrate atrophy of the choroid and retina

We describe 2 white female siblings (ages 35 and 38 years), both with a history of gyrate atrophy of the choroid and retina, who presented with complaints of decreased vision. The preoperative ophthalmic examination showed visually significant posterior subcapsular cataracts with no evidence of zonular weakness in either eye. We noted a previously unreported finding of concurrent extensive zonular weakness in both patients when their pupils were dilated and the patients were placed in the supine position for surgery. All 4 eyes received capsular tension rings (CTRs) for zonular stabilization during cataract extraction. Our finding suggests that zonular weakness is a common feature in patients with gyrate atrophy and may contribute to late in-the-bag intraocular lens (IOL) dislocations. The use of CTRs in this patient population may stabilize the capsular bag intraoperatively and serve as a circumferential fixation point if late in-the-bag IOL dislocation occurs.