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Year : 2021  |  Volume : 1  |  Issue : 2  |  Page : 234-236

Coincident iridocorneal endothelial syndrome and keratoconus

1 Department of Ophthalmology, Faculty of Medical and Health Sciences, University of Auckland, New Zealand
2 Corneoplastic Unit and Eye Bank, Queen Victoria Hospital, NHS Trust, East Grinstead, West Sussex, United Kingdom
3 Department of Ophthalmology, Faculty of Medical and Health Sciences, University of Auckland, New Zealand; Corneoplastic Unit and Eye Bank, Queen Victoria Hospital, NHS Trust, East Grinstead, West Sussex, United Kingdom

Date of Submission16-May-2020
Date of Acceptance04-Sep-2020
Date of Web Publication01-Apr-2021

Correspondence Address:
Dr. James McKelvie
Department of Ophthalmology, Private Bag 92019, University of Auckland, Auckland

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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_1535_20

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This report describes a rare case coincident keratoconus and Iridocorneal Endothelial (ICE) syndrome in a 43-year-old male with atopy who presented with progressively declining vision. A beaten silver appearance of the right endothelium with a dark-light reversal pattern and endothelial pleomorphism was consistent with the Chandler's syndrome variant of ICE. Keratoconus was demonstrated by irregular astigmatism with thinning and steepening on corneal tomography. He remained stable throughout 14 years with no evidence of secondary glaucoma or progression in keratoconus with conservative management. Coincident ICE and keratoconus is a rare entity with only one other published case of the Chandler's syndrome variant.

Keywords: Chandler's syndrome, corneal dystrophy, iridocorneal endothelial syndrome, keratoconus

How to cite this article:
Li Y, McLintock C, Lake D, McKelvie J. Coincident iridocorneal endothelial syndrome and keratoconus. Indian J Ophthalmol Case Rep 2021;1:234-6

How to cite this URL:
Li Y, McLintock C, Lake D, McKelvie J. Coincident iridocorneal endothelial syndrome and keratoconus. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Dec 5];1:234-6. Available from: https://www.ijoreports.in/text.asp?2021/1/2/234/312332

Iridocorneal endothelial (ICE) syndrome is a rare disorder characterized by abnormal endothelial cell proliferation that may be associated with corneal edema, angle-closure glaucoma, and characteristic iris changes. There is no conclusive prevalence; however, it is likely to be significantly less than the reported incidence of corneal endothelial dystrophies of 54 per 100,000.[1] Its precise etiology remains unknown; however, it has been suggested that viral infections, such as Herpes simplex and Epstein-Barr virus, may be an underlying or predisposing factor.[2] The three variations of ICE, Cogan-Reese syndrome, essential iris atrophy, and Chandler's syndrome, are differentiated by the degree of corneal and iris involvement.

Keratoconus is a form of corneal ectasia characterized by progressive myopic irregular astigmatism, with an incidence of 54 per 100,000.[3] It is often associated with atopy, eye rubbing, genetic syndromes, connective tissue disorders, and rarely other types of corneal dystrophy.[3]

  Case Report Top

A 43-year-old male presented with progressively declining visual acuity. He had a history of allergic conjunctivitis and hay fever and a family history of primary open-angle glaucoma.

On examination, unaided visual acuity was 6/12 and 6/24 correcting to 6/9.6 and 6/6 with spectacles, and autorefraction was -0.75/-1.00 × 60 and -1.25/-1.75 × 180 in the right and left eye, respectively. Blepharitis was present with papillae bilaterally. A “beaten silver” endothelial reflex was noted in the right eye only. Intraocular pressure (IOP) was 15 and 16 mmHg in the right and left eye, respectively. Gonioscopy and dilated examination demonstrated open angles, no peripheral anterior synechiae, a deep anterior chamber, and healthy optic nerves with a cup to disc ratio of 0.2 bilaterally. Apart from mild corneal haze in the right eye, there was no sign of endothelial decompensation, IOP elevation, or disc changes to suggest secondary glaucoma.

Endothelial cell count and morphology were normal in the left eye; however, specular microscopy demonstrated a light-dark reversal pattern [Figure 1]a, characteristic of Chandler's syndrome in the right eye. Endothelial pleomorphism was present with a coefficient of variation (CV) of 0.62 and 0.39, with an endothelial cell density (CD) of 2170 and 2368 in the right and left eye, respectively.
Figure 1: Specular microscopy, corneal topography, and ABCD progression. First presentation in 2014 demonstrating ICE cells in the right eye (1ai); the left eye (1aii) is unremarkable. A follow-up scan in 2018 showed no significant progression in pleomorphism and stable endothelial cell count in the right and left eye, respectively (1aiii and iv). Corneal topography of the right (1b) and left eye (1c) shows corneal thinning and irregular astigmatism. ABCD progression of the right (1d) and left (1e) eye over 2 years, demonstrating stable keratometry and minimal fluctuation in the BAD D scores, with values shifting from 2.23 to 2.39 in the right eye and 3.22 to 3.09 in the left eye

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Corneal tomography revealed bilateral corneal thinning with irregular astigmatism [Figure 1]b and [Figure 1]c, with minimum corneal thickness of 544 and 496 μm in the right and left eye, respectively. Optical coherence tomography demonstrated hyperreflectivity of the endothelium in the right eye only [Figure 2]. Management consisted of 0.3% hypromellose lubricating eye drops (Artelac; Bausch & Lomb Ltd, Surrey, UK), 2% sodium cromoglycate eye drops (Opticrom; Aventis Pharma Ltd, Surrey, UK), and oral antihistamines.
Figure 2: Optical coherence tomography. The endothelium of the right eye appears hyper reflective (2a), whereas the left eye (2b) appears unremarkable. White scale bar (bottom right) = 250 μm

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Over 14 years of follow-up, conservative management with annual review yielded stable visual acuity of 6/12 and refraction of +0.75/-1.25 × 75 in the right eye, with an IOP in the normal range and unchanged optic discs (cup to disc ratio 0.4). Corneal pachymetry and specular microscopy remained stable with a CD of 2157 and CV of 0.58 [Figure 1]a, [Figure 1]b, [Figure 1]c, [Figure 1]d, [Figure 1]e.

  Discussion Top

This case describes a patient who has a rare combination of concomitant keratoconus and ICE. There are only five reports of coincident ICE syndrome and keratoconus,[4],[5],[6],[7],[8] with only one involving the Chandler's syndrome variant.[4] The other cases involved keratoconus with coincident Cogan-Reese syndrome[6] and three cases of keratoconus with iris atrophy,[5],[7],[8] all of which developed secondary angle-closure glaucoma. Angle-closure glaucoma occurs in 46–82% of ICE patients and is more commonly associated with Cogan-Reese syndrome and essential iris atrophy compared to Chandler's syndrome, which frequently presents with corneal edema and a lesser degree of iris pathology.[2]

Posterior polymorphous corneal dystrophy (PPCD) can present with corneal changes similar to those seen in ICE; however, it typically presents with bilateral signs, whereas ICE is characteristically sporadic and unilateral as highlighted in this case. Fuchs endothelial dystrophy (FED) may also present with progressive decline in visual acuity with endothelial changes. In this case, sequential specular microscopy demonstrated unilateral light-dark reversal pattern that is diagnostic for ICE rather than bilateral guttae as seen in FED, and no clear demarcation between normal and abnormal cells as seen in PPCD.

Corneal anterior chamber depth and narrow angles are risk factors for angle-closure glaucoma. We postulate that keratoconus may have been a protective factor against this due to deep anterior chambers with open angles, as this patient did not develop secondary glaucoma that is commonly associated with peripheral anterior synechiae and a membrane over the angle structures.[9]

The presence of endothelial pathology may pose diagnostic and therapeutic challenges for keratoconus. Corneal thickening secondary to endothelial compromise seen in advanced ICE may in part mask the corneal ectasia characteristic of keratoconus. The nature of the corneal thickness changes can help to differentiate each disorder, as keratoconus tends to have characteristic focal ectasia that manifests central thinning surrounded by an annulus of thickened epithelium and ICE typically presents with a generalized increase in thickness.[10]

Corneal collagen crosslinking, the gold standard treatment for progressive keratoconus, may be relatively contraindicated in the context of ICE, particularly if the corneal thickness is less than 400 μm due to an increased risk of endothelial damage with corneal crosslinking. This patient did not demonstrate progressive tomographic or refractive changes and therefore did not require corneal crosslinking. If secondary endothelial damage develops, an endothelial transplant may be required, similar to advanced cases of ICE that develop secondary corneal decompensation due to endothelial dysfunction. In ICE patients with advanced keratoconus, it may be necessary to perform penetrating keratoplasty to address both pathologies.

  Conclusion Top

We describe a very rare coincidence of iridocorneal endothelial syndrome and keratoconus.

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

Musch DC, Niziol LM, Stein JD, Kamyar RM, Sugar A. Prevalence of corneal dystrophies in the United States: Estimates from claims data. Invest Ophthalmol Vis Sci 2011;52:6959-63.  Back to cited text no. 1
Silva L, Najafi A, Suwan Y, Teekhasaenee C, Ritch R. The iridocorneal endothelial syndrome. Surv Ophthalmol 2018;63:665-76.  Back to cited text no. 2
Romero-Jiménez M, Santodomingo-Rubido J, Wolffsohn JS. Keratoconus: A review. Cont Lens Anterior Eye 2010;33:157-66.  Back to cited text no. 3
Gasset AR, Worthen DM. Keratoconus and chandler's syndrome. Ann Ophthalmol 1974;6: 819-20.  Back to cited text no. 4
Blair SD, Seabrooks D, Shields WJ, Pillai S, Cavanagh HD. Bilateral progressive essential iris atrophy and keratoconus with coincident features of posterior polymorphous dystrophy: A case report and proposed pathogenesis. Cornea 1992;11:255-61.  Back to cited text no. 5
Chakrabarty L. A unique case of keratoconus with Cogan-Reese syndrome and secondary glaucoma. Indian J Ophthalmol 2017;65:64-6.  Back to cited text no. 6
[PUBMED]  [Full text]  
Maria MD, De Maria M, Iannetta D, Moramarco A, Fontana L. Iridocorneal endothelial syndrome in a patient with keratoconus – A case report. BMC Ophthalmol 2019;19:221. doi: 10.1186/s12886-019-1215-x.  Back to cited text no. 7
Gus PI, Araújo BS de, Zelanis S, Schmalfuss TR, Marinho DR. Posterior keratoconus and iris atrophy: A fortuitous association? Arq Bras Oftalmol 2019;82:68-71.  Back to cited text no. 8
Edmonds CR, Wung S-F, Pemberton B, Surrett S. Comparison of anterior chamber depth of normal and keratoconus eyes using Scheimpflug photography. Eye Contact Lens 2009;35:120-22.  Back to cited text no. 9
Reinstein DZ, Gobbe M, Archer TJ, Silverman RH, Coleman DJ. Epithelial, stromal, and total corneal thickness in keratoconus: Three-dimensional display with artemis very-high frequency digital ultrasound. J Refract Surg 2010;26:259-71.  Back to cited text no. 10


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