Spontaneous Pupillary Recovery of Urrets-Zavalia Syndrome Following Descemet’s Membrane Endothelial Keratoplasty

To report a case of Urrets-Zavalia syndrome (UZS) with spontaneous pupillary recovery following Descemet's membrane endothelial keratoplasty (DMEK). This was an interventional case report with literature review. A 37-year-old female phakic patient presented to our clinic with bilateral decreased vision secondary to worsening Fuch’s endothelial dystrophy. She underwent bilateral inferior peripheral iridotomies prior to undergoing left DMEK surgery under general anaesthesia. The DMEK surgery was uncomplicated but she had a large fixed and dilated left pupil on the following day despite a normal examination with a normal intraocular pressure. A diagnosis of UZS was made. The pupil remained fixed and dilated until 4 months postoperatively, which anisocoria started to improve by time. At 6 months postoperative, anisocoria had fully resolved with normal pupillary reactions and complete resolution of photopic symptoms. UZS is a rare complication of DMEK surgery and, to our best knowledge, only one case has been reported in the literature. Surgeons and patients should be aware of this potential phenomenon following uneventful DMEK surgery. Conservative measures should be considered for initial management of UZS in young patients as spontaneous recovery may sometimes ensue, as occurred in our case.