Case Series of Urrets-Zavalia Syndrome After Descemet Membrane Endothelial Keratoplasty

Abstract

To present a case series of Urrets-Zavalia syndrome (UZS) that developed after Descemet membrane endothelial keratoplasty (DMEK). A retrospective chart review was performed to identify patients who underwent DMEK by a single surgeon at the Duke Eye Center from 2017 to 2019 and subsequently developed UZS. Demographic data, preoperative history, operative notes, and postoperative course were reviewed. We describe 5 cases of UZS after DMEK (ages 19-74 years; 3 men and 2 women). Onset of UZS was noted at postoperative week 1 (n = 1), 2 (n =3), or 4 (n = 1). Four patients had an underlying diagnosis of Fuchs endothelial dystrophy and 1 had posterior polymorphous corneal dystrophy. Sixty percent (n = 3) of patients had an elevated intraocular pressure on postoperative day 0 or 1, and 40% (n = 2) of patients also had a pupillary block. One patient developed UZS after a rebubbling procedure for partial graft detachment and another developed UZS after repeat DMEK transplantation. Of the 3 patients who underwent bilateral combined DMEK and cataract surgery, 1 developed UZS in the second eye, whereas 2 developed UZS in the first eye. Most patients experienced monocular diplopia or had cosmetic concerns because of their mydriatic pupils. Two patients had spontaneous improvement in mydriasis. UZS after DMEK is rare, with only 2 cases in the literature: Holtmann et al and Isac et al. We present the largest case series of UZS after DMEK to date. Postoperative elevation in intraocular pressure is a common contributing factor. Evaluation of more patients may elucidate additional risk factors for this condition.