J CATARACT REFRACT SURG - VOL 32, JANUARY 2006
Fixed, dilated pupil after phakic intraocular lens implantation Erdal Yuzbasioglu, MD, Firat Helvacioglu, MD, Sadik Sencan, MD
A 26-year-old man with degenerative high myopia had bilateral anterior chamber phakic intraocular lens (IOL) implantation under general anesthesia. The preoperative slitlamp examination was normal. No mydriatic drops were used before, during, or after the procedure. Postoperatively, the intraocular pressure (IOP) in the right globe increased to 60 mm Hg. After the IOP was controlled, the pupil became fixed and dilated. Iris fluorescein angiography was obtained and delayed filling of the iris capillary plexus with large areas of no perfusion was observed in the right eye. Based on these findings, a diagnosis of Urrets-Zavalia syndrome was made. To our knowledge, this is the first report of Urrets-Zavalia syndrome after phakic IOL implantation. J Cataract Refract Surg 2006; 32:174–176 Q 2006 ASCRS and ESCRS
The incidence of paralytic mydriasis after penetrating keratoplasty (PKP) owing to keratoconus was reported first by authors such as Castroviejo and Paufique.1 Later, Urrets-Zavalia2 indicated his belief that the association of paralytic mydriasis, iris atrophy, and secondary glaucoma after keratoplasty represented a specific syndrome, which was named after him. We describe a patient who had a fixed, dilated pupil after phakic intraocular lens (pIOL) implantation. CASE REPORT A 26-year-old man with degenerative high myopia had anterior chamber pIOL implantation under general anesthesia in both eyes. Preoperatively, the slitlamp examination was completely normal. Both pupils were isocoric, light reactive, and 5.5 mm in diameter. Anterior chamber depth was 3.50 mm in the right eye
and 3.70 mm in the left eye. Limbus-to-limbus measurements were 11.5 mm in both eyes. The best corrected visual acuity was 0.05 with ÿ33.00 lenses in the right eye and 0.4 with a ÿ29.00 lens in the left eye. Intraocular pressure (IOP) was 15 mm Hg in the right eye and 14 mm Hg in the left eye. After a clear temporal corneal incision, the IOLs were implanted on the iris with the help of sodium hyaluronate 1% (Healon). After the iridedctomy, the ophthalmic viscosurgical device was removed from the anterior chamber by irrigation and aspiration with balanced salt solution. No mydriatic medication was given before, during, or after surgery. On the first postoperative day, the IOP in the right eye increased to 60 mm Hg. A corneal epithelial edema-dilated pupil and blurred visual acuity worse than 0.05 were seen. After the IOP was controlled, the pupil remained fixed and dilated (Figure 1). There was no reaction to light or pilocarpine 0.125% and 0.4%. Because the pupil remained fixed and dilated for 6 months, iris fluorescein angiography was performed and delayed filling of the iris capillary plexus with large areas of no perfusion was observed (Figures 2 and 3). Based on these findings, UrretsZacalia syndrome was diagnosed.
Accepted for publication January 24, 2005. From the Department of Ophthalmology, Bakirkoy Dr. Sadi Konuk Education and Research Hospital, Istanbul, Turkey. Presented as a poster at the 38th Turkish Society of Ophthalmology Annual National Congress (poster in Turkish), October 9–13, 2004 Antalya, Turkey, and the 8th Mediterranean Ophthalmology Society Congress (poster in English), October 13–16, 2004, Antalya, Turkey. No author has a financial or proprietary interest in any material or method mentioned. Reprint requests to Erdal Yuzbasioglu, MD, 4. Levent, Konaklar mahallesi Sogut sok. Koza sitesi, 14. blok D:16 Besiktas, Istanbul, Turkey 34330. E-mail:
[email protected]. Q 2006 ASCRS and ESCRS Published by Elsevier Inc.
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DISCUSSION
Urrets-Zavalia syndrome was first identified as an uncommon complication after PKP for keratoconus in a series of 6 cases that had the associated symptoms of fixed, dilated pupil, iris atrophy, and secondary glaucoma. Mydriasis following PKP was first described by Casterviejo.2–4 Gasset5 estimated that this syndrome occurred in 5.8% of the patients he treated. He reported that in 24 of 445 eyes a fixed, dilated pupil developed, and he described glaucoma as a secondary phenomenon, not integral to the syndrome. Davies and Ruben1 found similar incidences,4 0886-3350/06/$-see front matter doi:10.1016/j.jcrs.2005.11.016
CASE REPORTS: FIXED, DILATED PUPIL WITH PHAKIC IOL
Figure 1. Fixed dilated pupil in the right eye.
Figure 3. Normal iris flourescein angiography of the left eye.
but Naumann6 does not agree with these figures, reporting that in more than 1000 cases he has not seen this syndrome. There are 3 main types of pupil dilation1,4,5: (1) A pupil with normal light and near reaction that is at least 1.5 mm larger than its fellow unoperated eye. It constricts fully with topical miotics. An incidence of approximately 90% has been reported for this type of abnormality. (2) An unreactive pupil that returns slowly to normal. (3) Irreversible pupil dilation with iris atrophy.
The clinical features of this syndrome after PKP for various conditions are well established.2,3,5,6 The etiology is not clearly known. Increased IOP and low ocular rigidity leading to occlusion of the vessels at the root of the iris are considered the primary factors.3 Naumann6 recommends performing a peripheral iridectomy in all patients who have PKP in phakic eyes. Tuft and Buckley7 report that they have observed other large series in which peripheral iridectomy and cycloplegia were not performed and there were no reported cases of the syndrome. They conclude that the absence of cases in Naumann’s series cannot be attributed solely to this aspect of his surgical technique. Minasian and Ayliffe4 and Maurino et al.8 have reported fixed, dilated pupils (Urrets-Zavalia) after air/gas injection following deep lamellar keratoplasty (DLK) for keratoconus. None of the cases of Maurino et al.8 had peripheral iridectomy; these investigators also recommend surgical iridectomy if the air/gas injection has to be done after the DLK to prevent pupil block, raised IOP, or secondary iris ischemia with a dilated fixed pupil. Elevated IOP was not observed in the case reported by Minasian and Ayliffe.4 They claim that the compressive theory cannot play a part in this instance, and this may support the theory of an intrinsic iris abnormality in keratoconus.4 Jain et al.9 report 2 cases of this syndrome after trabeculectomy and determined that a peripheral iridectomy did not protect against this syndrome, as is believed by some investigators. In our case, increased IOP, iris ischemia, and fixed, dilated pupil developed despite a surgical peripheral iridectomy.
Figure 2. Iris flourescein angiography of the right eye shows delayed filling of the iris capillary plexus with large areas of no perfusion.
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CASE REPORTS: FIXED, DILATED PUPIL WITH PHAKIC IOL
To our knowledge, this is the first case that describes the Urrets-Zavalia syndrome after pIOL implantation. Although IOP changes and iris ischemia were thought to be the primary factors, we should be suspicious of intrinsic iris abnormalities in these cases.
REFERENCES 1. Davies PD, Ruben M. The paretic pupil: its incidence and aetiology after keratoplasty for keratoconus. Br J Ophthalmol 1975; 59: 223–228 2. Urrets-Zavalia A Jr. Fixed, dilated pupil, iris atrophy and secondary glaucoma; a distinct clinical entity following penetrating keratoplasty in keratoconus. Am J Ophthalmol 1963; 56:257–265
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3. Tuft SJ, Buckley RJ. Iris ischaemia following penetrating keratoplasty for keratoconus (Urrets-Zavalia syndrome). Cornea 1995; 14:618–622 4. Minasian M, Ayliffe W. Fixed dilated pupil following deep lamellar keratoplasty (Urrets-Zavalia syndrome) [letter]. Br J Ophthalmol 2002; 86:115–116 5. Gasset AR. Fixed dilated pupil following penetrating keratoplasty in keratoconus (Castroviejo syndrome). Ann Ophthalmol 1977; 9:623–628 6. Naumann GO. Iris ischaemia following penetrating keratoplasty for keratoconus (Urrets-Zavalia syndrome) [letter]. Cornea 1997; 16:120 7. Tuft SJ, Buckley RJ. Reply to letter by GOH Neumann. Cornea 1997; 16:120 8. Maurino V, Allan BD, Stevens JD, Tuft SJ. Fixed dilated pupil (UrretsZavalia syndrome) after air/gas injection after deep lamellar keratoplasty for keratoconus. Am J Ophthalmol 2002; 133:266–268 9. Jain R, Assi A, Murdoch IE. Urrets-Zavalia syndrome following trabeculectomy [letter]. Br J Ophthalmol 2000; 84:338–339
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