Urrets-Zavalia Syndrome after Descemet Membrane Endothelial Keratoplasty

Abstract

Purpose

To report a case of Urrets-Zavalia syndrome (UZS) after Descemet membrane endothelial keratoplasty (DMEK).

Methods

A 74-year-old woman with Fuchs endothelial dystrophy and inconspicuous ocular history developed UZS after DMEK surgery. The intraoperative and postoperative course is presented.

Results

After uneventful DMEK surgery, intraocular pressure was elevated up to 40 mm Hg on the first postoperative day. A small bleed from the peripheral wide-open iridectomy in the 12 o'clock position in the otherwise deep anterior chamber was observed. On the sixth postoperative day, a 4-mm-wide pupil, nonreactive to light, was noted. One year after surgery, the fixed medium mydriasis (4 mm) persisted and best-corrected vision was 0.1 logMAR. No pupillary reaction was noted after application of 0.2% or 2% pilocarpine.

Conclusions

Filling the anterior chamber with air to secure fixation of a grafted Descemet membrane carries the risk of early acute postoperative ocular hypertension. This can lead to iris sphincter defects resulting in a fixed dilated pupil after DMEK surgery. Large patent iridectomy in the 12 o'clock position is insufficient to prevent this. Patients undergoing DMEK surgery should be informed about this potential complication.