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Year : 2020  |  Volume : 32  |  Issue : 3  |  Page : 291-294

Iridocorneal endothelial syndrome – A case of contradictions

Department of Ophthalmology, Government Medical College, Kozhikode, Kerala, India

Date of Submission03-Mar-2020
Date of Decision23-Mar-2020
Date of Acceptance18-Apr-2020
Date of Web Publication23-Dec-2020

Correspondence Address:
Dr. Jincy Mariya Paul
Department of Ophthalmology, Government Medical College, Kozhikode, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/kjo.kjo_26_20

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Iridocorneal endothelial syndrome comprises a spectrum of disorders, which present with features of corneal edema, iris atrophy, peripheral anterior synechiae, and secondary glaucoma, in varying combinations. Clinical variants include Chandler's syndrome, progressive iris atrophy, and Cogan-Reese/iris nevus syndrome. Although classically described as a unilateral condition in young women, bilaterality, varied age of presentation, and male gender are also possibilities. Here, we report a case of bilateral progressive iris atrophy in an elderly male.

Keywords: Bilateral, iridocorneal endothelial syndrome, progressive iris atrophy, secondary glaucoma

How to cite this article:
Paul JM, Prabhu PB, Solomon CB. Iridocorneal endothelial syndrome – A case of contradictions. Kerala J Ophthalmol 2020;32:291-4

How to cite this URL:
Paul JM, Prabhu PB, Solomon CB. Iridocorneal endothelial syndrome – A case of contradictions. Kerala J Ophthalmol [serial online] 2020 [cited 2022 Dec 3];32:291-4. Available from: http://www.kjophthal.com/text.asp?2020/32/3/291/304550

  Introduction Top

Iridocorneal endothelial (ICE) syndrome denotes a disorder in which the normal endothelium is altered, and this leads to varying degrees of corneal edema, iris atrophy, and secondary glaucoma. The altered endothelium migrates across the angle structures and the iris, and contraction of this tissue leads to the characteristic high peripheral anterior synechiae (PAS), iris changes, and secondary glaucoma.[1],[2]

It includes three clinical variants:

  1. Chandler's syndrome
  2. Essential/progressive iris atrophy
  3. Cogan-Reese/iris nevus syndrome.

It commonly presents unilaterally in women between the ages of 20 and 50 years.[3],[4],[5]

There have been a few isolated reports in literature of male preponderance as well as bilateral involvement.[1],[4],[6],[7],[8]

We report a case of bilateral progressive iris atrophy in an elderly male.

Cases presenting with bilateral involvement may be mistaken for posterior polymorphous dystrophy. Typical features should, however, direct the diagnosis toward ICE syndrome.

  Case Report Top

A 72-year-old male presented with a history of gradual, painless, and progressive decrease of vision in both eyes for 1 year. He had a recent onset of recurrent episodes of moderately intense eye pain, redness, colored haloes, and headache. There were no associated systemic illnesses or other relevant family, personal, or past histories.

Ocular examination revealed, band keratopathy, iris atrophy, corectopia and multiple PAS in all quadrants, in both eyes. There were stretch holes in the inferior quadrants in both eyes [Figure 1]a, [Figure 1]b and [Figure 2]a, [Figure 2]b.
Figure 1: (a) Finding of corectopia in the right eye. (b) Peripheral anterior synechiae and stretch holes seen in the left eye

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Figure 2: (a) Gonioscopy showing peripheral anterior synechiae in the right eye. (b) Peripheral anterior synechiae in the left eye

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Best-corrected visual acuity was 6/9 (right eye [OD]) and 6/24 (left eye [OS]) with elevated intraocular pressure (IOP) measured by applanation tonometry (OD = 28 mmHg and OS = 32 mmHg).

Fundus evaluation showed glaucomatous cupping in both eyes (cup-to-disc ratio; OD = 0.8 and OS = 0.9) [Figure 3]a and [Figure 3]b.
Figure 3: (a) Fundus photo showing glaucomatous cupping in the right eye. (b) Advanced cupping in the left eye

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Field examination by Humphrey field analyzer showed severely depressed fields in both eyes, and specular microscopy showed typical ICE cells [Figure 4].
Figure 4: Specular microscopy showing iridocorneal endothelial cells

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Routine blood investigations were within normal limits.

The patient was managed with augmented trabeculectomy, following control of IOP with medical management [Figure 5].
Figure 5: Functional filtering bleb seen postoperatively (left eye)

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  Discussion Top

ICE syndrome is thought to be due to a replacement of the normal endothelial cells with cells having epithelial-like characteristic and migratory properties.[1],[4]

The true etiology is not yet known with theories of viral infection being currently investigated.[9]

The endothelium shows a typical hammered silver or beaten-bronze appearance on slit-lamp examination.

The variants are distinguished based on varied clinical findings.[2]

Chandler's syndrome

This is the most common subtype, and it typically presents with corneal edema with little or no iris changes.

Essential/progressive iris atrophy

Iris findings predominate in this subtype with typical findings such as polycoria, corectopia, iris hole formation, ectropion uveae, and iris atrophy.

Iris nevus/Cogan-Reese syndrome

In this variant of ICE syndrome, tan pedunculated nodules or diffuse pigmented lesions are found on the anterior surface of the iris, with iris atrophy being a less common finding.

Specular microscopy is an important diagnostic tool. ICE cells, which appear on a specular photomicrograph as dark, larger than normal endothelial cells, with a bright central spot, have been described as being pathognomonic for the disease.

Malhotra et al. reported a male preponderance in his study, though only one bilateral case was reported.[1]

Sherrard et al., in their series of 57 eyes with ICE syndrome, noted a lack of sex discrimination by the disease, with a male: female ratio of 47%:53%.[10]

Cases of bilateral ICE with the same variant presenting in both eyes or two different variants presenting in both eyes also have been reported.[1],[4],[6],[8],[11]

Posterior polymorphous dystrophy, iris hypoplasia, and Axenfeld–Rieger syndrome are a few of the commonly considered differential diagnoses. These can be differentiated as follows:

Posterior polymorphous dystrophy

It is corneal dystrophy, with autosomal dominant inheritance. Three patterns which are observed are endothelial vesicle-like lesions, band lesions, and diffuse deep stromal opacities. It rarely results in corneal edema or elevated IOP.

Iris hypoplasia

It is easily distinguished from ICE syndrome by the typical finding of a hypoplastic iris. It may appear like aniridia, but rudimentary iris often revealed on gonioscopy.

Axenfeld–Rieger syndrome

This is again an autosomal dominant inherited condition with similar findings to that of ICE syndrome but presents with glaucoma in early infancy or childhood.

Medical management with topical and systemic IOP-lowering agents as well as surgical management options such as glaucoma-filtering surgeries or glaucoma drainage devices may need to be tried.

In the case presented, the patient already suffered significant optic nerve head damage with depressed visual field from secondary glaucoma. He was managed with IOP-lowering agents initially to alleviate his symptoms of eye pain and corneal edema and then taken up for augmented trabeculectomy sequentially in both eyes to prevent further optic nerve head damage.

The age of presentation, male gender, and bilaterality are all atypical in this case reported which shows the possibility of such a rare presentation in ICE syndromes.

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.

Financial support and sponsorship

Guided by my coauthors, Dr. Padma B. Prabhu and Dr. Charmaine B. Solomon, from the Department of Ophthalmology, Government Medical College, Kozhikode.

Conflicts of interest

There are no conflicts of interest.

  References Top

Malhotra C, Seth NG, Pandav SS, Jain AK, Kaushik S, Gupta A, et al. Iridocorneal endothelial syndrome: Evaluation of patient demographics and endothelial morphology by in vivo confocal microscopy in an Indian cohort. Indian J Ophthalmol. 2019;67:604–10.  Back to cited text no. 1
Iridocorneal Endothelial Syndrome and Secondary Glaucoma - EyeWiki [Internet]. Available from: https://eyewiki.org/Iridocorneal_Endothelial_Syndrome_and_Secondary_Glaucoma. [cited 2020 Feb 3].  Back to cited text no. 2
Shields MB. Progressive essential iris atrophy, Chandler's syndrome, and the iris nevus (Cogan-Reese) syndrome: a spectrum of disease. Surv Ophthalmol. 1979;24:3–20.  Back to cited text no. 3
Liu YK, Wang IJ, Hu FR, Hung PT, Chang HW. Clinical and specular microscopic manifestations of iridocorneal endothelial syndrome. Jpn J Ophthalmol. 2001;45:281–7.  Back to cited text no. 4
Le Q-H, Sun X-H, Xu J-J. In-vivo confocal microscopy of iridocorneal endothelial syndrome. Int Ophthalmol. 2009;29:11–8.  Back to cited text no. 5
Hemady RK, Patel A, Blum S, Nirankari VS. Bilateral iridocorneal endothelial syndrome: case report and review of the literature. Cornea. 1994;13:368–72.  Back to cited text no. 6
Kupfer C, Kaiser-Kupfer M, Kuwabara T. Progressive bilateral essential iris atrophy. Trans Am Ophthalmol Soc. 1976;74:341–56.  Back to cited text no. 7
Huna R, Barak A, Melamed S. Bilateral iridocorneal endothelial syndrome presented as Cogan-Reese and Chandler's syndrome. J Glaucoma. 1996;5:60–2.  Back to cited text no. 8
Unilateral Glaucoma: Background, Pathophysiology, Epidemiology. 2019. Available from: https://emedicine.medscape.com/article/1207362-overview. [cited 2020 Mar 3].  Back to cited text no. 9
Sherrard ES, Frangoulis MA, Muir MG. On the morphology of cells of posterior cornea in the iridocorneal endothelial syndrome. Cornea. 1991;10:233–43.  Back to cited text no. 10
Kaiser-Kupfer M, Kuwabara T, Kupfer C. Progressive bilateral essential iris atrophy. Am J Ophthalmol. 1977;83:340–6.  Back to cited text no. 11


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]


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