Urrets-Zavalia Syndrome After Deep Anterior Lamellar Keratoplasty: A Case Series

To describe 13 cases of fixed, dilated pupils following deep anterior lamellar keratoplasty.

This retrospective case series presents 13 eyes of 13 patients who underwent deep anterior lamellar keratoplasty, with intraoperative/postoperative air in the anterior chamber, and who subsequently presented with fixed, dilated pupil postsurgery. The patients presented over 10 years, from January 2015 to December 2024, at a tertiary eye care center. Details of demographic data, risk factors, intraoperative complications, anterior segment inflammation, raised intraocular pressure, and pupillary block postsurgery were analyzed.

Urrets-Zavalia syndrome was clinically diagnosed in the postoperative period in 13 cases, associated with Descemet membrane microperforation intraoperatively in nine cases and air/gas injection (perfluoro propane C3F8) postoperatively for a detached graft in five cases (for shallow anterior chamber (AC) in two cases and for double anterior chamber in one case). No known risk factors were noted in one case. In four of 13 cases, the pupil reduced in size and regained some reactivity (pupillary diameter 4 mm at last follow-up) over a period of two years.

Urrets-Zavalia syndrome, a rare complication reported following penetrating keratoplasty performed for keratoconus, can also develop after deep anterior lamellar keratoplasty. Intraoperative iris trauma, Descemet membrane perforation, and full-chamber air bubble are presumed risk factors and should be avoided.