Urrets - Zavalia Syndrome After Deep Anterior Lamellar Keratoplasty For Keratoconus

To describe a case of fixed dilated pupil ( Urrets-Zavalia Syndrome) following Deep Anterior Lamellar Keratoplasty (DALK) for keratoconus.

 A 42-year-old high myopic female patient with CF 1m vision in Right Eye, underwent Deep Anterior Lamellar Keratoplasty for keratoconus. We failed to achieve big bubble and hence manual Deep Anterior Lamellar Keratoplasty was done. Air bubble was injected into the anterior chamber during dissection. At the end of the procedure, during side port hydration, the air bubble reduced to 3/4th of the chamber and was left in situ.

On postop day1 patient presented with edematous graft, shallow anterior chamber, intense inflammation and high Intra Ocular Pressure. Anterior Segment Optic Coherence Tomography showed inferior appositional angle closure with pupillary block. On dilatation, the air bubble migrated to the front of iris and the anterior chamber deepened . With antiglaucoma medications, the Intra Ocular Pressure was controlled. Inflammation was treated with intense steroids.At 2 weeks postop, graft cleared, inflammation and Intra Ocular Pressure were under control. However, patient complained of glare and on examination,there was a fixed dilated pupil, ectropion uveae and a BCVA of 6/36. Her Intra Ocular Pressure is under control with antiglaucoma medication.

RESULTS:
Although patient had a Best corrected Visual acuity of 6/36 at the end of 1 month, the functional vision was not satisfactory due to glare.She also need to be on antiglaucoma medications for control of Intra Ocular Pressure.

Intra Ocular Pressure monitoring intra and post operatively in Deep Anterior Lamellar Keratoplasty patients is important to prevent iris ischemia resulting in dilated pupil. At the end of surgery if air injection is required, the pupil should be left widely dilated and air bubble should be mobile and well above the inferior pupillary border to prevent pupillary block. Another alternative is to do an inferior peripheral iridotomy in an undilated pupil. Postoperatively our patient had a fairly regular topography (vision : 6/36), but still was not satisfied with the quality of vision due to excessive glare.