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Year : 2023  |  Volume : 16  |  Issue : 1  |  Page : 98-102  

Acquired ectropion uveae: A case series

Department of Glaucoma, Dr. Shroff's Charity Eye Hospital, New Delhi, India

Date of Submission22-Jan-2022
Date of Decision08-Sep-2022
Date of Acceptance17-Dec-2022
Date of Web Publication21-Feb-2023

Correspondence Address:
Suneeta Dubey
Dr. Shroff's Charity Eye Hospital, 5027 Kedarnath Lane, Daryaganj, New Delhi - 110 002
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ojo.ojo_24_22

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Acquired ectropion uveae (AEU) is a common sequela to various inflammatory and ischemic conditions, though it is not well recognized. There is a paucity of literature describing AEU. So here, we present five cases where we documented ectropion uveae following chronic inflammation. Patients with ectropion uveae following chronic inflammation and ischemia were retrospectively reviewed. Their medical records and clinical findings were analyzed. Five patients of various ages were found to have AEU, of which 1 was post trabeculectomy with phacoemulsification and posterior chamber intraocular lens, 1 following neovascular glaucoma (NVG), 1 following uveitic glaucoma, and 2 following iridocorneal endothelial syndrome. Patients with NVG and uveitic glaucoma also had undergone glaucoma filtration surgeries. AEU may be seen secondary to inflammatory and ischemic processes and should be looked for carefully as they may cause progressive glaucoma.

Keywords: Ectropion uveae, glaucoma, inflammation, iridocorneal endothelial syndrome, neovascular glaucoma, uveitic glaucoma

How to cite this article:
Daga D, Dubey S, Gandhi M. Acquired ectropion uveae: A case series. Oman J Ophthalmol 2023;16:98-102

How to cite this URL:
Daga D, Dubey S, Gandhi M. Acquired ectropion uveae: A case series. Oman J Ophthalmol [serial online] 2023 [cited 2023 Mar 26];16:98-102. Available from: https://www.ojoonline.org/text.asp?2023/16/1/98/370041

   Introduction Top

Ectropion uveae is defined by the presence of iris pigment epithelium on the anterior surface of the iris on the pupillary margin. It can be congenital or acquired, of which congenital ectropion uveae (CEU) is a rare association which presents with angle dysgenesis and anterior insertion of the iris root along with glaucoma.[1] The relatively more common type is the acquired ectropion uveae (AEU), resulting from excessive inflammation or ischemic pathological processes involving the anterior part of the iris. It may be seen secondary to neovascular glaucoma (NVG), iridotrabecular dysgenesis, trauma, uveitis, and surgery. It is frequently missed and not well recognized in the setting of excessive inflammation. Here, we present a case series of AEU associated with different clinical scenarios.

   Case Reports Top

Case 1

A 65-year-old woman presented to us with complaints of poor vision in OD with a diagnosis of cataract with glaucoma on treatment with brimonidine and timolol maleate combination therapy. On examination, her best-corrected visual acuity (BCVA) was OD 6/24 and OS 6/9, and Goldmann applanation tonometer (GAT) OU 18 mmHg. Her anterior segment was unremarkable. On gonioscopy, angles were open till scleral spur in OU. She had nuclear sclerosis grade 3 in OD and grade 2 in OS. On posterior segment evaluation OD, optic nerve head (ONH) had a cup–disc ratio (CDR) of 0.8:1 with superior neuroretinal rim (NRR) thinning, OS ONH had a CDR of 0.6:1. She was diagnosed with OU primary open-angle glaucoma with cataract, and she underwent OD phacoemulsification with trabeculectomy and posterior chamber intraocular lens (PCIOL) and OS phacoemulsification with trabeculectomy with PCIOL a few months later. She gained good visual acuity of OU 6/6, and intraocular pressure (IOP) was well controlled in mid-teens. She was on regular follow-up. Five years later, in one of her follow-ups, her BCVA OU was documented as 6/6, IOP by GAT OU as 16 mmHg. OU had a diffuse bleb, deep anterior chamber with patent surgical peripheral iridectomy. In OS, she had a 1 clock hour of ectropion uveae present temporally [Figure 1]a. Upon dilation, she was noted to have posterior pigment epithelium adhered to anterior capsular opacification in OS [Figure 1]b and [Figure 1]c. OU optic disc and visual fields were stable. This is not a typical ectropion uveae, but the adherence of the posterior pigment epithelium of the iris to anterior capsular opacification was probably due to inflammatory process in the anterior capsule post surgery causing contraction and adherence of the posterior pigment epithelium of the iris appearing as if it is detached from the iris stroma. Although this was not causing any symptoms and her BCVA and IOP and visual fields were stable, we kept her on regular follow-up, as ectropion uveae may be associated with glaucoma progression.
Figure 1: (a) Ectropion uveae present temporally in undilated (red arrow); (b and c) On dilation posterior pigment epithelium of iris adhered to anterior capsular opacification (yellow arrow)

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Case 2

A 39-year-old woman presented with complaints of decreased vision in OD for the past 1 year, along with watering and redness in the same eye. She had noticed an irregular increase in the size of her pupil in OD. She had no history of trauma or any previous surgeries. She was diagnosed elsewhere with glaucoma and started on brimonidine and timolol maleate combination therapy in OD. On examination, the visual acuity in OD was 6/12 improving to 6/6 with pinhole and OS was 6/6. IOP with GAT was 22 mmHg in OD on IOP-lowering medication and OS was 12 mmHg. Her OD had conjunctival congestion, clear cornea, irregular anterior chamber depth, and shallow inferiorly. The superior iris appeared stretched with loss of normal pattern and iridodonesis. Ectropion uveae was present in the inferior pupillary margin pulling the posterior pigment epithelium toward the angle [Figure 2]a and [Figure 2]b. The pupil was irregular in shape and had no reaction to light. On gonioscopy, her superior angle was open till scleral spur [Figure 2]f while nasal [Figure 2]d, inferior [Figure 2]e, and temporal [Figure 2]g angles were closed with broad-based PAS. The anterior segment of OS was within normal limits [Figure 2]c with angles open to scleral spur on gonioscopy. OU had a clear lens, ONH had a CDR of 0.3:1 with healthy NRR with no obvious RNFL defect. A diagnosis of OD ICE syndrome with essential iris atrophy was made. She was continued on IOP-lowering agents and lubricants, although there was no glaucomatous change in the optic disc. However, as we learned in the previous case, glaucomatous damage could occur and progress quite quickly, as a precautionary measure, she was kept on IOP-lowering agents and on regular follow-up. This case was similar to the previous one, where ectropion uveae was secondary to inflammatory and atrophic changes in the iris.
Figure 2: (a and b) Irregularly shaped pupil with ectropion uveae present inferiorly (red arrow) in OD; (c) Normal anterior segment in OS; (f) Gonioscopic image of superior angle open till scleral spur; (d) Nasal angle; (e) Inferior angle; and (g) Temporal angle closed with PAS formation (yellow arrow). PAS: Peripheral anterior synechiae

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Case 3

A 40-year-old male presented to us with complaints of change in shape of the pupil of OS which he noticed a month ago. He had a history of corneal refractive surgery done around 8 years ago. On examination, BCVA OU was 6/6, and IOP by GAT was 12 mmHg in OU. Anterior segment was unremarkable in OD [Figure 3]a, in OS cornea was clear, anterior chamber had Van Herick grade 2, iris had ectropion uveae at 12 o'clock and 4 o'clock and 6 o'clock along with Cogan-Reese nodules, a triangular shaped sluggishly reacting pupil [Figure 3]b and [Figure 3]c. On gonioscopy, in OD, all quadrants were open till scleral spur, and in OS, all quadrants were closed with broad-based peripheral anterior synechiae (PAS) except for the nasal quadrant which was open [Figure 3]d, [Figure 3]e, [Figure 3]f, [Figure 3]g. In OU, crystalline lens was clear and OD had a CDR of 0.25:1 whereas OS had a CDR of 0.3:1, with healthy NRR and no obvious retinal nerve fiber layer (RNFL) defect. A diagnosis of OS iridocorneal endothelial (ICE) syndrome with essential iris atrophy with Cogan-Reese syndrome was made. As there was no glaucomatous change in the disc, he was advised lubricants alone and was kept on regular follow-up. He was doing well for 2 years, when he came with complaints of diminution of vision in OS, with VA of 6/18 improving to 6/6 with pinhole. IOP was raised to 34 mmHg. Anterior segment was stable, but there was an increase in cupping of ONH to 0.4:1. He was started on three IOP-lowering agents, namely brimonidine tartrate with timolol maleate combination twice a day with travoprost at bedtime. IOP was controlled on medication for a year, after which he again presented with raised IOP of 34 mmHg and progression of glaucomatous cupping of ONH to 0.85:1. He underwent OS trabeculectomy, following which IOP was controlled in low to mid-teens and stable optic disc on follow-up. Here, the patient has iris atrophy with ectropion uveae and Cogan-Reese nodules present. This ectropion uveae is probably due to inflammatory process involving the iris, causing PAS, angle closure, and glaucoma.
Figure 3: (a) Normal anterior segment in OD; (b and c) Irregular pupil with ectropion uveae and Cogan-Reese nodules (yellow arrow); (d-f) Temporal, inferior, superior quadrants of gonioscopy showing ectropion uveae with PAS formation (red arrow); (g) Nasal quadrant showing open angle. PAS: Peripheral anterior synechiae

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Case 4

A 50-year-old female presented with complaints of diminution of vision in OD for 6 months with a diagnosis of OU proliferative diabetic retinopathy (PDR) and OD glaucoma on IOP-lowering agents. On examination, BCVA OU was 6/24, IOP OD 46 mmHg on 4 IOP-lowering medications, and OS was 16 mmHg. In OD, the cornea had epithelial edema, anterior chamber was regular in depth, neovascularization of the iris (NVI) was present with mild ectropion uveae. On gonioscopy of OD, angles were closed with PAS formation. No neovascularization of angles was present. OS anterior segment was within normal limits. OU was pseudophakic and had PDR with peripheral laser marks. OD ONH had a CDR of 0.85:1 with disc pallor and OU ONH had CDR of 0.5:1. A diagnosis of OU PDR with OD NVG with angle closure was made and was continued on IOP-lowering medications. She underwent OD tube implantation with Ahmed glaucoma valve (AGV). Following this, IOP was well under control in mid-teens with a well-formed posterior bleb of AGV. She was noted to develop ectropion uveae with posterior pigment epithelium pulled toward the tube with some iris atrophy below the tube in OD. Here, ectropion uveae could probably result from chronic inflammation due to rubbing of tube with iris, causing iris atrophy and pulling the ectropion uveae toward the tube.

Case 5

A 14-year-old girl presented to us with complaints of diminution of vision in OS for the past 3 years. She was a known case of juvenile rheumatoid arthritis with associated uveitis with glaucoma in OS, for which she was on 4 IOP-lowering medications. On examination, OD was had BCVA of 6/6 and IOP of 11 mmHg and was within normal limits. OS had BCVA of counting fingers (CFs) with IOP of 46 mmHg, with band-shaped keratopathy (BSK) on the cornea mid-dilated pupil with posterior synechiae, and complicated cataract. Gonioscopy findings could not be documented in view of poor visualization and poor cooperation. Posterior segment details were not visible. She underwent OS ethylenediaminetetraacetic acid chelation for BSK removal followed by OS phacoemulsification with trabeculectomy with PCIOL in September 2019. Following this, IOP was well controlled in low to mid-teens for few months. On follow-up, a thick posterior capsule plaque with ectropion uveae was noted. Her IOP was also raised to mid-thirties, for which IOP-lowering medication was started and IOP was controlled, although BCVA remained CF because of increased glaucomatous cupping of ONH with disc pallor. Here, there are many factors causing excessive inflammation – with the underlying setting of uveitis, the child underwent combined surgery. There is an exaggerated inflammatory response post surgery in children. All these factors together may have exacerbated the inflammation causing ectropion uveae and posterior capsular plaque.

   Discussion Top

AEU is the presence of posterior pigment epithelium of the iris on the anterior surface of the iris in the pupillary margin, secondary to various conditions such as NVI, NVG, uveitis and uveitic glaucoma, ICE syndromes, inflammation post surgery, trauma, or other ischemic or neoplastic procedures.

CE is a rarer, nonprogressive condition with similar clinical appearance, along with iris stromal hypoplasia, iridotrabecular dysgenesis, and anterior insertion of the iris root and glaucoma. It generally involves the entire pupillary rim. CEU is commonly associated with neurofibromatosis or facial hemihypertrophy. It is proposed to be a manifestation of the general tendency for hyperplasia and dysplasia associated with these conditions.[1],[2]

AEU is a progressive condition, secondary to ischemia or inflammation. It may involve a part of pupillary rim-partial AEU, seen with iris ischemia or in ICE syndrome. When the whole circumference of the pupillary rim is involved, it is complete AEU, seen commonly with NVG or uveitis. Probable pathogenesis of AEU is formation of fibrovascular membrane, secondary to inflammation on the surface of the iris with atrophy of the iris. Gradual traction of this membrane causes anterior curling of the posterior pigment epithelium and iris stroma. When this membrane reaches and blocks the iridocorneal angle, it acts as a barrier to aqueous outflow causing elevation of IOP.[3],[4] Another probable mechanism of AEU is epithelial-mesenchymal transition (EMT)-like phenomenon. Prolonged inflammation causes the release of cytokines which induces conversion of anterior lens epithelial cells and iris epithelial cells into myofibroblasts. This conversion of epithelial cells into myofibroblasts results in fibrous membrane formation over the iris and angle, resulting in glaucoma and ectropion uvea at the pupil. EMT-like phenomenon could also result in abnormalities in the aqueous outflow pathway causing secondary glaucoma.[5] EMT-like phenomenon is observed more in pediatric age group, as they have increased propensity of inflammation postsurgical procedure. In adults, it is seen in the setting of secondary glaucoma which also has excessive inflammation.

In the above-described cases, we documented AEU, following various reasons of chronic inflammation, with glaucoma managed medically or surgically. A thorough examination and regular follow-up is important for the timely diagnosis and management of ectropion uveae and associated glaucoma, which may get exacerbated causing severe progression of the disease and intractable glaucoma.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patients have given their consent for 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

Ritch R, Forbes M, Hetherington J Jr., Harrison R, Podos SM. Congenital ectropion uveae with glaucoma. Ophthalmology 1984;91:326-31.  Back to cited text no. 1
Kumari R, Saha BC. Congenital ectropion uveae with glaucoma: A case report. Int Ophthalmol 2018;38:2623-6.  Back to cited text no. 2
Markovic V, Vukovic D, Radosavljevic A, Marjanovic I. Acquired ectropion uveae and secondary glaucoma due to trauma: Report of 3 cases. Eur J Ophthalmol 2017;27:e1-4.  Back to cited text no. 3
Dowling JL Jr., Albert DM, Nelson LB, Walton DS. Primary glaucoma associated with iridotrabecular dysgenesis and ectropion uveae. Ophthalmology 1985;92:912-21.  Back to cited text no. 4
Takahashi E, Inoue T, Fujimoto T, Kojima S, Tanihara H. Epithelial mesenchymal transition-like phenomenon in trabecular meshwork cells. Exp Eye Res 2014;118:72-9.  Back to cited text no. 5


  [Figure 1], [Figure 2], [Figure 3]


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