Rare finding of bilateral Urrets-Zavalia syndrome post Descemet’s stripping endothelial keratoplasty in congenital hereditary endothelial dystrophy in a child

A female patient in her middle childhood who was initially referred to us with congenital glaucoma was later diagnosed with congenital hereditary endothelial dystrophy. Due to progressive corneal thickening and haze, Descemet's stripping endothelial keratoplasty (DSEK) was performed consecutively in both her eyes. Postoperative follow-ups included evaluations of corneal thickness, intraocular pressure and disc assessment. On post DSEK, the corneal thickness reduced on subsequent follow-ups and the intraocular pressure was normal. The corneal clarity improved significantly, however, examination revealed a fixed, dilated pupil with posterior synechiae, suggestive of Urrets-Zavalia syndrome. Urrets-Zavalia syndrome should be suspected in cases with a fixed, dilated pupil post-keratoplasty.