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Year : 2023  |  Volume : 3  |  Issue : 2  |  Page : 364-367

A rare case of Urrets-Zavalia syndrome following trabeculectomy in a pediatric patient

Glaucoma Services, Dr. Shroff's Charity Eye Hospital, Delhi, India

Date of Submission03-Oct-2022
Date of Acceptance04-Nov-2022
Date of Web Publication28-Apr-2023

Correspondence Address:
Tripti Johri
Dr. Shroff Charity Eye Hospital, Daryaganj, Delhi - 110 002
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJO.IJO_2558_22

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A child with bilateral advanced steroid-induced glaucoma and uncontrolled intraocular pressure (IOP) on maximum medical therapy underwent trabeculectomy surgery. The eye developed a dense anterior chamber inflammation with a high IOP in the postoperative period, which was managed medically. The IOP got controlled in the following visits, but the pupil remained mid-dilated, fixed, and nonreactive to pilocarpine, and a provisional diagnosis of Urrets-Zavalia syndrome was made. Urrets-Zavalia syndrome is a rare postsurgical complication characterized by a fixed dilated pupil with iris atrophy. This is the first such case reported in a pediatric patient following trabeculectomy.

Keywords: Steroid induced glaucoma, Trabeculectomy, Urrets-Zavalia syndrome

How to cite this article:
Johri T, Dubey S. A rare case of Urrets-Zavalia syndrome following trabeculectomy in a pediatric patient. Indian J Ophthalmol Case Rep 2023;3:364-7

How to cite this URL:
Johri T, Dubey S. A rare case of Urrets-Zavalia syndrome following trabeculectomy in a pediatric patient. Indian J Ophthalmol Case Rep [serial online] 2023 [cited 2023 Jun 5];3:364-7. Available from: https://www.ijoreports.in/text.asp?2023/3/2/364/374955

Urrets-Zavalia syndrome (UZS) is an uncommon postoperative complication characterized by a fixed and dilated pupil. It was first described by Urrets-Zavalia in the year 1963 in keratoconus patients who underwent penetrating keratoplasty and were treated with mydriatic agents.[1] It has also been found after various eye procedures like after cataract surgery,[2] deep anterior lamellar keratoplasty, Descemet stripping automated endothelial keratoplasty, trabeculectomy,[3] argon laser peripheral iridoplasty, goniotomy,[4] C3F8 injection,[5] and others. There is only 1.9% reported incidence of developing UZS post trabeculectomy.[6] There are numerous postulated mechanisms for development of fixed dilated pupil, but the exact cause is not known. We report a case of complication of trabeculectomy in an 8-year old-child diagnosed with steroid-induced glaucoma who presented with an episode of high intraocular pressure (IOP) postoperatively and developed a fixed dilated pupil, nonreactive to pilocarpine.

  Case Report Top

A young male child aged 8 years presented to our hospital with the complaint of blurring of vision in both eyes. The patient had been on antiglaucoma medications (AGMs) started elsewhere for high IOP. The child had a history of use of over-the-counter topical steroids for ocular allergy in both eyes. There was no family history of glaucoma. The vision in the right eye was 6/9 and in the left eye was perception of light. The anterior segment examination was normal, and gonioscopy demonstrated open angles with normal angle structures in both eyes. The patient was using three AGMs (brinzolamide, brimonidine, and latanoprost) and had an IOP of 34 and 30 mmHg in the right and left eye, respectively. On fundus examination, the right eye had a cup: disk ratio of 0.8:1 and the left eye had near-total cupping with disk pallor [Figure 1]. The optical coherence tomography (OCT) examination [Figure 2] and [Figure 3] of both the eyes revealed marked thinning of ganglion cell complex (GCC) and retinal nerve fiber layer (RNFL). A diagnosis of bilateral advanced steroid-induced glaucoma was made, and timolol eye drop was added to the existing regimen. The IOP remained uncontrolled on maximum medical management, and he was planned for left eye trabeculectomy surgery for IOP control. The surgery went uneventfully, and the postoperative IOP was well controlled. Later, the right eye underwent trabeculectomy. The postoperative examination on the next day revealed a dense anterior chamber inflammation and IOP of 26 mmHg with a bleb grading of E2H2V2S0. The patient was started on frequent topical steroids, atropine ointment, and AGMs (brinzolamide and timolol). A week later, the anterior chamber reaction resolved, but the IOP remained high (24 mmHg). The releasable suture was removed, and systemic acetazolamide was added for 5 days. The IOP remained controlled in the subsequent visits. However, 3 weeks after suture removal, the IOP was again found to be elevated (28 mmHg), for which laser suturolysis was done and travoprost along with a short course of oral acetazolamide was added. Atropine was stopped at this stage. The IOP got controlled in mid-teens on three AGMs subsequently, but the pupil remained mid-dilated, fixed, and nonreactive to 2% topical pilocarpine drops [Figure 4]. A provisional diagnosis of UZS was made.
Figure 1: Optic disk appearance of the right and left eyes

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Figure 2: OCT of retinal nerve fiber layer (RNFL). OCT = optical coherence tomography, RNFL = retinal nerve fiber layer

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Figure 3: OCT of GCC. GCC = ganglion cell complex, OCT = optical coherence tomography

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Figure 4: Postoperative photograph 2 weeks after the surgery showing dilated and fixed pupil

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  Discussion Top

UZS is a rare postsurgical complication with an unknown exact mechanism and is characterized by a fixed dilated pupil with iris atrophy. Multiple theories have been proposed, of which ischemic atrophy of the sphincter muscle after an acute attack of postoperative increase in IOP and subsequent pupillary dilatation is the most popularly accepted. The other presumed risk factors for developing UZS could be use of mydriatics and intraocular inflammation. The presentation of UZS may range from focal pupillary due to injury of the posterior pigment layer to diffuse or sector atrophy with injury of the iris stroma.[7] The morphological changes in iris vessels and filling defects seen on fluorescein angiography and indocyanine green angiography of the anterior segment are comparable to the changes seen in iris ischemia.

UZS has been associated with corneal surgeries (especially penetrating keratoplasty) and glaucoma surgeries. Chelnis et al reported a case of a child who underwent goniotomy procedure and subsequently developed a dilated pupil.[4] Gharbiya reported a similar complication in a 74 year old patient who had a glaucoma drainage device implantation.[8] Jain et al published 2 cases of trabeculectomy who developed a fixed dilated pupil following an uncomplicated surgery.[3] Recently, Klezlova[9] et al reported a case of UZS in a young child of juvenile open angle glaucoma who underwent an uncomplicated trabeculotomy and trabeculectomy with mitomycin C. The child developed episodes of high IOP after bleb revision surgery for postoperative hypotony, following which he developed a fixed and dilated pupil eventually.

Gi Seok Park et al[10] reported a case of UZS in a 51 year old patient with uveitic glaucoma who underwent an uneventful trabeculectomy surgery. The patient developed a dilated pupil 1 month after the surgery, even with well-controlled IOP. This case report highlights that UZS can even develop following an uneventful intraocular procedure with controlled IOP.

Various mechanisms of development of UZS have been postulated. Raised IOP in the pre- or postoperative period seems to be the most consistent risk factor for development of UZS. Tuft et al., speculated that raised intraocular pressure compresses the iris vessels and results in development of iris ischemia and a fixed dilated pupil as seen in three patients of penetrating keratoplasty, who had transient elevated intraocular pressures after the surgery.[11]

Another hypothesis discusses the possibility of use of long-acting mydriatic agent to be a risk factor predisposing to the development of UZS. Mydriatic agents are frequently used in trabeculectomy patients to deepen the anterior chamber depth, relax the ciliary body, and prevent a cilio-lenticular block, whereas there are studies that disregard the contribution of mydriatic use in the mechanism of UZS.

  Conclusion Top

This is the first reported case of a young patient with steroid-induced glaucoma developing UZS characterized by a fixed and dilated pupil following trabeculectomy surgery.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Urrets Zavalia A Jr. Fixed, dilated pupil, iris atrophy and secondary glaucoma. Am J Ophthalmol 1963;56:257-65.  Back to cited text no. 1
Tan AK, Humphry RC. The fixed dilated pupil after cataract surgery: Is it related to intraocular use of hypromellose Br J Ophthalmol 1993;77:639-41.  Back to cited text no. 2
Jain R, Assi A, Murdoch IE. Urrets-Zavalia syndrome following trabeculectomy. Br J Ophthalmol 2000;84:338-9.  Back to cited text no. 3
Chelnis JG, Sieminski SF, Reynolds JD. Urrets-Zavalia syndrome following goniotomy in a child. J AAPOS 2012;16:312-3.  Back to cited text no. 4
Aralikatti AK, Tomlins PJ, Shah S. Urrets-Zavalia syndrome following intracameral C3F8 injection for acute corneal hydrops. Clin Experiment Ophthalmol 2008;36:198-9.  Back to cited text no. 5
Magalhães OA, Kronbauer CL, Müller EG, Sanvicente CT. Update and review of Urrets-Zavalia syndrome. Arq Bras Oftalmol 2016;79:202-4.  Back to cited text no. 6
Lagoutte F, Thienpont P, Comte P. Proposition de traitment du syndrome dUrrets-Zavalia. A propos d'un cas reversible. J Fr Ophthalmol 1983;6:291-4.  Back to cited text no. 7
Gharbiya M, Colica C, Campagna O, De Vico U, Cruciani F. Urrets- Zavalia Syndrome after glaucoma filtration device implantation. Clin Ter 2013;164:e381-2.  Back to cited text no. 8
Klezlova A, Liebezeit S, Prokosch-Willing V, Gericke A, Pfeiffer N, Hoffmann EM. Urrets-Zavalia syndrome after combined trabeculotomy-trabeculectomy surgery. J Glaucoma 2018;27:e80-3.  Back to cited text no. 9
Park GS, Won YK, Kim KN, Lee YH, Kim CS. Urrets-Zavalia syndrome following trabeculectomy. J Korean Ophthalmol Soc 2020;61:221-5.  Back to cited text no. 10
Tuft SJ, Buckley RJ. Iris ischaemia following penetrating keratoplasty for keratoconus (Urrets-Zavalia syndrome) Cornea. 1995;14:618-22.  Back to cited text no. 11


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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