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S. S. Tuli, C. Mickler, L. Butts, T. Barnett; Urretts-Zavalia Pupil - Ischaemic Iris Syndrome. Invest. Ophthalmol. Vis. Sci. 2010;51(13):1311.
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Urrets-Zavalia Syndrome (UZS) is a rare surgical complication; first reported in 1963. It was originally described as a fixed, widely dilated, atrophic iris and secondary glaucoma that developed after penetrating keratoplasty for keratoconus. Since then it has been associated with other surgeries including DSEK, intracameral gas injection, Argon laser iridoplasty, Phakic IOL implantation, and trabeculectomy. Visual acuity is usually not affected adversely but patients experience a significant morbidity from a permanently dilated pupil. The exact cause of this syndrome has not been elucidated.
We reviewed the records of a series of 6 patients who developed UZS after corneal transplantation (PKP/DSEK) and compared them with 30 control patients. Multiple variables were compared between the disease and control groups including age, preoperative diagnosis, systemic medications, preoperative and postoperative intraocular pressure (IOP), mean arterial pressure (MAP) during the surgery, anesthetic agents, postoperative nausea and vomiting and postoperative pain.
Younger age was positively corelated with disease. Postoperative IOP 35 was significantly correlated with Urrets-Zavalia pupil (p=.007). There was trend toward significance for low intraoperative MAP and time of the MAP below 60mmHg. When variables of low MAP and elevated intraocular pressure were combined, the risk of disease was 100%.
Urrets-Zavalia Syndrome is a rare post surgical complication that is associated with significant patient morbidity. A combination of high IOP and low MAP is 100% predictive of development of Urrets-Zavalia pupil postoperatively. This implies that this syndrome occurs due to iris ischemia secondary to decreased perfusion pressure.
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