Urrets-Zavalia syndrome: report of 2 cases

¹Department of Ophthalmology, Central Hospital of the Army, Boite Postale 244, Kouba, 16063, Algeria, ²University of Algiers I Benyoucef Benkhedda, 02 Rue Didouche Mourad, Algiers, 16001, Algeria

^&Corresponding author
Islam Bechakh, Department of Ophthalmology, Central Hospital of the Army, Boite Postale 244, Kouba, 16063, Algeria

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Urrets-Zavalia syndrome characterized by areflexic mydriasis followed by progressive atrophy of the iris can occur afterfull thickness cornea transplant (penetrating keratoplasty). This is often associated with ocular hypertonia. We here report two cases. A 14-year-old girl with bilateral stage IV keratoconus, who underwent penetrating keratoplasty in the right eye. Immediate postoperative outcome was marked by the appearance of an inflammatory reaction of the anterior chamber with double-cross tyndall effect and pupillary fibrin membrane formation characterized by a tone of 23mmhg at day 21. The patient received combination therapy based on corticosteroids (local and bolus), mydriatics, and hypnotics with inflammation recovery. However, mydriasis with pupillary areflexia persisted. Forty years later, the patient had corrected visual acuity 09/10 with areflexic mydriasis and photophobia (A). The second patient was her twin sister who presented with bilateral stage IV keratoconus. She was operated following the same protocol. At day 1 after surgery corneal edema, tyndall effect and mydriasis occurred (B). At the fouth-year follow-up visit, corrected visual acuity was 08/10 with areflexic mydriasis associated with ectropion uvea. Ischemic palsy of the iris sphincter can occur due to direct injury caused by surgical instruments or indirectly by the use of viscoelastic substances. The occurrence of urrets-zavalia syndrome after keratoplasty is not due to graft rejection and does not influence final visual acuity, as is the case with our two patients. Urrets-Zavalia syndrome is still a clinical reality in corneal transplants.

Figure 1: A) white light photographs of the anterior segment of the right eye; color radiograph showing penetrating keratoplasty; the diameter of the graft was 7.75 mm for a recipient cornea trepanned at 7.50 mm, the pupil in reflective mydriasis; B) white light photographs of the anterior segment of the right eye; color image showing full-thickness keratoplasty, areflexic mydriasis 7 mm in diameter associated with ectropion of the posterior uvea