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G. S. Figueiredo, S. S. P. Kolli, S. Ahmad, K. Gales, F. C. Figueiredo; Urrets-Zavalia Syndrome Following Penetrating Keratoplasty for Keratoconus. Invest. Ophthalmol. Vis. Sci. 2007;48(13):4682.
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© ARVO (1962-2015); The Authors (2016-present)
Urrets-Zavalia syndrome (UZS) consists of a fixed dilated pupil associated with iris atrophy which at present is a widely recognised but poorly understood complication following penetrating keratoplasty (PK) for keratoconus (KC). The purpose of this study is to establish the incidence, visual effects and aetiology of UZS.
This is a retrospective single centre study in the United Kingdom of consecutive patients with UZS following PK for KC from February 1997 to October 2006. The PKP procedures have been performed by a single surgeon (FCF). Surgical complications, including raised post-operative intraocular pressure (IOP) and its duration were noted. All UZS patients were invited for further clinical assessment including glare symptoms, visual acuity (VA), contrast sensitivity (CS), pupil size, gonioscopy and pupil reactivity to pilocarpine 2% and slit lamp examination. ICG angiography was performed to assess iris vasculature. Identical assessments were made for the equivalent number of age matched control patients who had undergone PK for KC but did not develop UZS.
The incidence of UZS was 17.7% in the KC population (n=62). Although patients with UZS complained of symptoms of glare and photophobia, there was no significant difference in LogMAR VA or Pelli-Robson CS between UZS and KC controls (p<0.5). There was significantly higher first day IOP (p<0.05) in UZS (mean= 38mmHg, SD= 15.22) compared with controls (mean= 25.3mmHg, SD= 10.7) which normalised within 1 week. The patients with UZS had increased pupil size (6.8 ± 1.4 mm compared to 5.9 ± 1.0 mm; p<0.05) with significantly reduced response to 2% pilocarpine (24.2% ± 20.2 compared to 62.1% ± 6.0; p<0.005). ICG angiography revealed delayed and reduced iris vasculature filling in UZS patients compared with normal filling patterns of controls.
The only significant factor associated with development of UZS is elevated post-operative IOP. Nearly all patients in the UZS group developed long standing dilated pupils with significantly reduced reactivity to topical pilocarpine. ICG angiography confirmed iris vessel ischaemia. These findings would support the theory that the cause of UZS in patients undergoing PK for KC is iris ischaemia resulting from occlusion of iris root vessels within the sclera due to elevated IOP and can be explained by the known lower ocular rigidity in KC patients. This would support the need for particularly aggressive postoperative IOP control in all patients undergoing PK for KC.
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