Iridoplasty of fixed-dilated pupil (Urrets-Zavalia syndrome) after deep anterior lamellar keratoplasty.

A 38-year-old woman with advanced keratoconus initially developed Urrets-Zavalia Syndrome (UZS) in the left eye after deep anterior lamellar keratoplasty. During the uneventful surgery, a 7-mm-wide pupil unresponsive to light was noticed. On the first postoperative day, intraocular pressure (IOP) was elevated up to 45 mmHg with shallow anterior chamber (AC). A peripheral iridotomy in 2 o'clock position was conducted and a fixed combination of brinzolamide 1% and timolol 0.5% was administered topically. In the subsequent postoperative period, IOP was successfully reduced, but the patient reported severe photophobia, glare, and decreased vision. Twelve months after surgery, her best-corrected visual acuity (BCVA) was 20/200, the fixed-dilated pupil persisted, iris was atrophic, and lens opacities were detected. She was submitted for phacoemulsification cataract surgery combined with iridoplasty using the closed chamber slipping suture technique. Three months after surgery, her BCVA was 20/25, pupil diameter remained stable at 4 mm, and glare symptoms were significantly reduced. A very adequate cosmetic outcome was also achieved. Iridoplasty in postkeratoplasty patient with unilateral UZS was effective in improving patient's visual function disability and restoring residual anisocoria. This technique can be applied as a single procedure or combined with another one for the management of UZS.

Introduction

Urrets-Zavalia Syndrome (UZS) is a well-recognized although rare postoperative complication, originally described as the appearance of a fixed-dilated pupil with iris atrophy following penetrating keratoplasty for keratoconus patients.[1] Since the occurrence of UZS has additionally been reported after deep anterior lamellar keratoplasty (DALK), Descemet stripping endothelial keratoplasty, goniotomy, and after implantation of phakic intraocular lenses.[2345] Diseased atrophic irises can cause visual distortion, photophobia, and intolerable glare. We report a case of established UZS during uneventful left DALK, in a patient treated for keratoconus, who also developed subsequent lens opacities. The restoration course included combined phacoemulsification cataract surgery with iridoplasty through closed-chamber technique. To our knowledge, this association has not been previously described for the management of UZS.

Case Report

A 38-year-old woman presented with bilateral keratoconus and was submitted to undergo left DALK. The preoperative uncorrected visual acuity (VA) was 20/64 and the best-corrected distance VA (BCDVA) was 20/40 (−3, 0 sph, −2, 50 cyl × 90): The central corneal thickness was 441 μm and intraocular pressure (IOP) was 15 mmHg. The pupil in the involved eye was circular, normally reacting to light with normal iris texture. Her medical history was inconspicuous. Using 7.50/7.75 mm graft into a 7.50 mm diameter recipient bed, DALK was performed under general anesthesia. Disposable trephines were used and deep lamellar dissection was performed after air injection into deep stroma to achieve the big bubble as described by Anwar and Teichmann.[6] No signs of intraoperative Descemets membrane (DM) perforation were visible. The donor lenticule was secured with 16 interrupted 10:0 monofilament nylon sutures. A small air bubble was introduced into the AC to reduce the risk of aqueous seepage between the posterior corneal lamella and the graft, while pupil dilation (7 mm) was noticed. On the first postoperative day, the AC was shallow, IOP was 45 mmHg, and a peripheral iridotomy was conducted using Nd:YAG laser in 2 o’clock position. No mydriatic drops were used. She was advised moxifloxacin 0.5% four times a day for 10 days along with dexamethasone drops 0.1% to be tapered to once a day 8 weeks after the operation. A fixed combination of brinzolamide 1% and timolol 0.5% was also administered. In the following weeks, a clear graft was observed, but the pupil was atrophic, remained fixed-dilated, and unresponsive to light stimulus. Four weeks after surgery, she presented with uncorrected distance VA 20/100, IOP was 15 mmHg with pigment deposition in endothelium and glaukomflecken. Three months postoperatively, BCDVA was 20/64 (−1.0 sph, −6.0 cyl × 80), cornea was clear but the characteristic features of UZS persisted. Eight months after surgery, BCDVA was 20/32 (−1.50 sph, −2.0 cyl × 110), sutures had been gradually removed, and IOP was 14 mmHg without medication. During the following months, lens opacities were noticed, VA was gradually decreasing and 15 months after DALK, the patient underwent uncomplicated phacoemulsification cataract surgery with posterior chamber intraocular lens implantation [Figure 1]. Simultaneously, iridoplasty was performed by four peripheral paracentesis and two iris sutures were placed using Siepser's slipping suture technique,[7] creating a superior and an inferior pupillary margin [Figures 2 and 3]. Three months postoperatively, the final outcome was quite sufficient. Slit lamp examination revealed a pupillary diameter up to 4 mm resulting in only slight residual anisocoria without complications such as ocular hypertension, posterior synechiae, and iridocyclitis. Patient's BCDVA was 20/25 (−5.0 cyl × 120) with reduction regarding photophobia and glare symptoms.

Figure 1

Anterior segment photograph of the left eye showing a fixed-dilated pupil before placing the first iris suture. Uncomplicated phacoemulsification and posterior chamber intraocular lense implantation was successfully completed

Figure 2

Superior pupillary margin created after placing the first iris suture

Figure 3

Postoperative anterior segment photograph showing the reconstruction of the pupillary margin after placement of two iris sutures. The cornea graft is clear and intraocular pressure is not elevated

Discussion

In cases of UZS following lamellar keratoplasties, the etiology remains uncertain and several causative mechanisms have been described in literature. Some authors have reported cases of UZS developed after DALK, that were complicated with intraoperative perforation of DM and air tamponade possibly induced pupillary block, provoked early onset ocular hypertension leading to secondary iris ischemia and fixed-dilated pupil.[8] Urrets-Zavalia supported the role of atropine in developing fixed dilation of pupil and iris atrophy.[1] Ischemic trauma due to pupillary dilation or increased IOP may be associated with iris ischemia as etiology of UZS. Interestingly, there may be a difference between UZS following penetrating keratoplasty compared to DALK patients where other causes may be associated with air pupillary block. In our case, we detected sign of pupillary block (shallow postoperative AC) which emphasizes its possible role in UZS. Although no visible perforation of DM was noticed intraoperatively, we cannot exclude the possibility of undetected microperforation of DM to have occurred leading to this complication. There may be a correlation between air injection in AC and the toxic effect of oxygen on the crystalline lens leading to cataract formation, iris ischemia, and pupil dilation.[9]

Decreased BCDVA and visual symptoms (glare and photophobia) are late sequelae of UZS. In addition, residual anisocoria contributes to irregular cosmetic appearance which disturbed our young patient who experienced the previous symptoms as well. Surgical treatment of UZS has been described using circular iris suture in open-sky technique.[10] In our case, the closed chamber slipping suture technique originally described by Siepser was preferred since anterior lamellar keratoplasty was previously performed, regrafting was unnecessary, and iridoplasty was combined with phacoemulsification cataract surgery. This technique seems to be efficient for the restoration of disturbed anterior segment anatomy caused by UZS following DALK as a single procedure or in association with another one.

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Conflicts of interest

There are no conflicts of interest.