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

Purpose: To present a case series of Urrets-Zavalia syndrome (UZS) that developed after Descemet membrane endothelial keratoplasty (DMEK).

Methods: 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.

Results: 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.

Conclusions: 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.