Urrets-Zavalia Syndrome Following Descemet Stripping Endothelial Keratoplasty Claire Y. Chu, MD Pawan Prasher, MD Eric Dai, MD R. Wayne Bowman, MD V. Vinod Mootha, MD Department of Ophthalmology University of Texas Southwestern Medical Center at Dallas, Texas

Introduction  Descemet stripping endothelial keratoplasty (DSEK) has emerged as an elegant alternative to penetrating keratoplasty (PK) for the treatment of endothelial disorders such as Fuchs endothelial dystrophy.  DSEK minimizes the risk of PK associated postoperative complications such as wound dehiscence, refractive astigmatism, prolonged recovery time.  However, pupillary block glaucoma continues to be reported in DSEK outcomes studies as a rare complication ( %) associated with the introduction of the air bubble for graft-host apposition.  Pupillary block had previously been described in association with iris atrophy and a fixed nonreactive pupil, collectively designated Urrets- Zavalia syndrome.  UZ occurs as a rare complication of penetrating keratoplasty, laser peripheral iridotomy, and deep lamellar keratoplasty.  We describe Urrets-Zavalia syndrome in a series of patients undergoing DSEK for Fuchs endothelial dystrophy.

Purpose  To examine risk factors associated with Urrets-Zavalia syndrome in patients undergoing DSEK  Four patients undergoing DSEK for Fuchs endothelial dystrophy with postoperative changes consistent with Urrets-Zavalia syndrome were analyzed retrospectively.  All eyes underwent clinical examination, with followup between 4 and 8 months Materials and Methods

Results  Mean age was /- 8.1 years  Three patients had a history of prior ocular surgery, including CEIOL, scleral buckle for retinal detachment repair, and strabismus surgery  Preoperative BCVA ranged from 20/40 to 20/70  All patients required rebubbling during the postoperative course for management of graft dislocation  Of the three patients who developed pupillary block glaucoma, one required rebubbling whereas two others required rebubbling in addition to repeat DSEK for graft failure and dislocation  One patient had no history of prior ocular surgery and did not develop pupillary block glaucoma, but still developed UZ syndrome. This patient was a glaucoma suspect based on family history and optic atrophy in the affected eye  Postoperative BCVA improved, ranging from 20/25 to 20/50

Postoperative Pupillary Defects A. Pupillary changes after DSEK with postoperative pupillary block glaucoma, graft dislocation, and reapposition of the graft in the operating room. B. Fellow eye. A B

Patient Clinical Data *Glaucoma suspect based on positive family history and increased optic atrophy in the affected eye. Age Gender Preoperative BCVA Postoperative BCVA Prior Ocular Surgery Operative Course Pupillary block glaucoma Nonreactive pupil 182 yo M20/7020/30Cataract extraction DSEK Graft dislocation yo M20/7020/50None DSEK Graft dislocation Repeat DSEK yo F20/7020/50Scleral buckle DSEK Graft dislocation Repeat DSEK ++ 4*74 yo F20/6020/25Strabismus DSEK Graft dislocation +

Discussion  The most common postoperative complication of DSEK is graft dislocation ( %)  Pupillary block glaucoma has been reported as a rare postoperative complication of DSEK at a rate between 0 -3%.  The pathogenesis of Urrets-Zavalia syndrome is thought to be pupillary block glaucoma resulting in iris ischemia, which in turn leads to a fixed dilated pupil. 2  Risk factors predisposing patients to iris ischemia may thus increase the likelihood of UZ -- increased age, multiple intraocular manipulations, postoperative pupillary block glaucoma, glaucoma suspect.  Techniques to minimize graft dislocation in DSEK may minimize the need for additional manipulation and also the risk of pupillary block glaucoma -- peripheral recipient bed scraping, removal of interface fluid, thorough irrigation of viscoelastic from the interface. 3,4,5

Conclusions  A dilated pupil is a rare complication following penetrating keratoplasty and deep anterior lamellar keratoplasty, but can also be associated with DSEK.  Patients with Fuchs endothelial dystrophy with a complicated postoperative course may be at greater risk of iris ischemia and Urrets-Zavalia syndrome.  Continued evolution of surgical techniques will enhance outcomes and minimize complications of DSEK. References 1. Price MO, Price FW. Descemet's stripping endothelial keratoplasty. Curr Opin Ophthalmol : Koenig SB, Covert DJ. Early Results of Small-Incision Descemet’s Stripping and Automated Endothelial Keratoplasty. Ophthalmology : Maurino V, Allan BDS, Stevens JD, Tuft SJ. Fixed Dilated Pupil (Urrets-Zavalia Syndrome) After Air/Gas Injection After Deep Lamellar Keratoplasty for Keratoconus. Am J Ophthalmol : Terry MA, Shamie N, Chen ES, Hoar KL, Friend DJ. Endothelial Keratoplasty: A Simplified Technique to Minimize Graft Dislocation, Iatrogenic Graft Failure, and Pupillary Block. Ophthalmology (5): Silvera DA, Fabrizio MJ, Goins KM. The Characterization of Interface Haze Following DLEK. IOVS 2007;48:ARVO E- Abstract 4715.