Fixed Dilated Pupil (urrets-zavalia Syndrome) After Deep Anterior Lamellar Keratoplasty

Purpose: To report 4 cases that developed fixed dilated pupil after deep anterior lamellar keratoplasty (DALK) for treating keratoconus and granular corneal dystrophy.

Methods: Four patients underwent DALK (either Melles's manual dissection or Anwar's big-bubble technique) with diagnosis of keratoconus (2 cases) and granular corneal dystrophy (2 cases). In Melles's technique, the anterior chamber was fully filled with air at the beginning of procedure. When corneal dissection was complete, air was replaced with balanced salt solution. In Anwar's technique, a large bubble was formed between Descemet's membrane and the deepest stroma. Viscoelastic was not used within the anterior chamber in either technique. For microperforation, air was left in the anterior chamber for a couple of days. Two patients developed severe fibrinous anterior uveitis that was controlled with intense topical and systemic steroid. Other two patients developed high intraocular pressure that was controlled with antiglaucoma medications.

Results: After 1-2 weeks, all 4 DALK patients developed fixed dilated pupil and posterior synechiae; 2 patients developed a faint anterior subcapsular opacity. Of 286 patients who underwent DALK, 6 patients had microperforation (2%) and 4 patients developed fixed dilated pupil (1.4%). Best-corrected visual acuity (BCVA) decreased in all patients; most of them complained about visual symptoms like glare and halo at night.

Conclusions: Fixed dilated pupil (Urrets-Zavalia syndrome) is an uncommon but rather serious complication of corneal transplantation. Iris ischemia is the most common proposing mechanism. Decreased BCVA and visual symptoms at night (glare and halo) are late sequelae.A fixed dilated pupil with iris atrophy (Urrets-Zavalia syndrome) is an uncommon postoperative complication that was originally described after penetrating keratoplasty for keratoconus. In these cases, the iris is fixed and dilated and adheres to anterior lens capsule. Prepupillary iris atrophy is usually seen and may be accompanied by a severe fibrinous reaction and anterior subcapsular lens opacity.