Urrets-Zavalia Syndrome After Lamellar Corneal Transplant: Two Case Reports

1. Urrets-Zavalia Syndrome After Lamellar Corneal Transplant: Two Case Reports Timothy Y. Chou, MD, Sujata P. Prabhu, MD, Justin Dexter, MD Department of Ophthalmology SUNY at Stony Brook Stony Brook, New York, USA The authors have no financial interests to disclose

2. Purpose Urrets-Zavalia originally described the syndrome of persistent pupillary dilation in keratoconus patients following penetrating keratoplasty. The cause is uncertain. Herein we report two cases analogous to the Urrets-Zavalia syndrome, but occurring in atypical clinical settings.

3. Methods A retrospective review of the medical records of two patients noted to have persistent pupillary dilation following lamellar corneal transplant surgery. Data collected included age, sex, pre-operative history and medications, surgical events and complications, and post-operative course.

4. Results An 76 year old Asian male underwent uncomplicated Descemet’s stripping with endothelial keratoplasty (DSEK) OS for pseudophakic bullous keratopathy after phacoemulsification cataract surgery. He had underlying Fuchs’ endothelial corneal dystrophy. Preoperative medications included tamsulosin for benign prostatic hypertrophy.

5. Results • The second patient was a 35 year old African American male who had corneal scarring after a chemical injury complicated by keratomycosis of the left eye. He underwent deep anterior lamellar keratoplasty (DALK), using the “big bubble” technique. • During surgery there was a microperforation, requiring the placement of an intracameral air bubble.

6. Results Air was injected at the end of each case, maximally filling the anterior chamber for ten minutes. Thereafter the air was partially aspirated to restore normal intraocular pressure (IOP). Both patients were given topical atropine 1% (which was the mydriatic readily available in the operating room) to prevent pupillary block. Postoperatively, in both cases, the pupils became persistently dilated (figures 1 & 2). Intraoperative pressure was elevated on the first day only, to 26 mmHg after the DALK, and treated with acetozolamide.

7. Figure 1 Persistent pupillary dilation after DSEK

8. Figure 2 Persistent pupillary dilation after DALK

9. Conclusions These two case reports demonstrate that persistent pupillary mydriasis can occur in patients following lamellar transplant surgeries where intracameral air is injected and atropine 1% drops are instilled.

10. Conclusions The DALK patient with corneal scarring had no other inherent corneal disease. This indicates that Urrets-Zavalia syndrome can occur in the absence of an underlying corneal disorder such as keratoconus or a dystrophy.

11. Conclusions There was preoperative use of Tamsulosin, and mild postoperative IOP elevation in the DSEK patient. It is unclear whether these may represent additional risk factors for the syndrome.

12. References Urrets Zavalia A Jr. Fixed pupil, iris atrophy and secondary glaucoma: a distinct clinical entity following penetrating keratoplasty for keratoconus. Am J Ophthalmol 1963;56:257-265 Niknam S, Rajabi MT. Fixed dilated pupil (Urrets-Zavalia syndrome) after deep anterior lamellar keratoplasty. Cornea 2009; 28:1187-1190 Maurino V, Allan BD, Stevens JD, et al. Fixed dilated pupil (Urrets-Zavalia Syndrome) after air/gas injection after deep lamellar keratoplasty for keratoconus. Am J Ophthalmol 2002;133:266-268 Fournié P, Ponchei C, Malecaze F, et al. Fixed dilated pupil (Urrets-Zavalia syndrome and anterior subcapsular cataract formation after Descemet’s stripping endothelial keratoplasty. Cornea 2009;28:1184-1186.