Indian Journal of Clinical and Experimental Ophthalmology

Print ISSN: 2395-1443

Online ISSN: 2395-1451

CODEN : IJCEKF

Indian Journal of Clinical and Experimental Ophthalmology (IJCEO) is open access, a peer-reviewed medical journal, published quarterly, online, and in print, by the  Innovative Education and Scientific Research Foundation (IESRF) since 2015. To fulfill our aim of rapid dissemination of knowledge, we publish articles ‘Ahead of Print’ on acceptance. In addition, the journal allows free more...


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Kumar, Gnanadurai, and Huda: ICE syndrome- Progressive iris atrophy with secondary glaucoma


Introduction

Iridocorneal endothelial (ICE) syndrome is a group of overlapping clinical spectrum which can manifest as any of the 3 subgroups with variable amount of corneal edema, abnormalities of iris and secondary angle closure glaucoma without pupillary block. ICE syndrome is typically unilateral, non hereditary, most commonly involving middle-aged females of 20-45 years. Out of the 3 variants, most common being Chandler’s syndrome involving over more than half the cases, followed by essential or progressive iris atrophy (25%) and Iris Nevus or Cogan Reese syndrome (25%). The pathology in all three entities is growth of an abnormal endothelial cell layer which proliferates and migrates across the angle of anterior chamber and over iris surface and thus progressing to glaucoma. Other ocular structures are uninvolved and no systemic disease association been found. Rarely it may have familial involvement. Nearly 40% cases may present with secondary glaucoma. Here I am reporting a case of ICE syndrome with essential progressive iris atrophy associated with secondary glaucoma.

Case Report

A 40 year old female presented to the ophthalmology department with a chief complaint of unusual looking left eye for many years. She had no complaints of diplopia or photophobia with no history of trauma in that eye. No history of defective vision, pain, redness, watering. She was healthy looking with no comorbidities. Her general examination and vitals were within normal limits.

Table 1

Ocular examination

Anterior segment

Right eye (Figure 2)

Left eye (Figure 3)

Visual acuity

6/6, N6

6/6, N6

Extraocular movement

Full and free

Full and free

Eyelid

Normal

Normal

Conjunctiva

Clear

Clear

Cornea

Clear

Clear, NO edema, no haze

Anterior chamber

Normal

Normal

Iris

Normal colour and pattern

Several iris stretch holes at 9 to 12 o’clock, with marked iris thinning noted.

Pupil

2 mm round, centrally located, RTL

Polycoria (more than one papillary opening) and corectopia (displacement of puill from its normal position)

Lens

Clear

Clear

Posterior segment examination (90D lens)

Fundus is normal

CDR – 0.5 (Figure 5)

Other etiologies of secondary open angle glaucoma such as pigment particles (Pigmentary glaucoma), Pseudoexfoliative material (pseudoexfolation glaucoma), fibrovasular tissue (Neovascular glaucoma), ghost cell glaucoma were excluded.

Figure 1

Anterior segment picture showing normal structures (OD)

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Figure 2

Anterior segment picture showing iris atrophy and stretch holes (OS)

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Table 2

Investigations

Intraocular pressure (Applanation Tonometry)

16 mm Hg

24 mm Hg

Gonioscopy (with 4 mirror gonio lens)

Grade IV (wide open)

Trabecular meshwork was visualised. Peripheral anterior synechiae noted in two quadrants at 12 and 9 o’clock position.(Figure 4)

Visual field (automated perimetry)

Normal

Normal

OCT RNFL

Normal

Normal

Specular Microscopy

Normal

Endothelial cell count decreased and polymegathism.

Figure 3

Gonioscopy picture of left: trabecular meshwork was visualised

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Peripheral anterior synechiae noted in two quadrants at 12 and 9 o’clock position

Figure 4

Fundus picture shows 0.5 cup-disc ratio (left eye)

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Figure 5

Visual fields-normal

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Figure 6

OCT - normal

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Follow up and management

Patient was started on eye drop Timolol 0.5%, 1 drop BD in left eye. On follow up the IOP was measured and under control (18 mm Hg) and she is being followed up in our OPD for her glaucoma status every 3 months. Her fundus LE CDR 0.5, Visual fields – Normal and no progression of glaucoma.

Discussion

Iridocorneal endothelial (ICE) syndrome is a group of overlapping clinical spectrum manifesting as any of the three subgroups with varying degrees of corneal edema, abnormalities of Iris & secondary angle closure glaucoma without pupillary block.1 ICE syndrome is mainly unilateral, non familial, commonly in middle aged females. Most common clinical type is Chandler’s syndrome (50%) followed by essential progressive iris atrophy (25%) and iris nevus (Cogan Reese) syndrome (25%).

ICE syndrome involves an abnormal clone of corneal endothelial cells that develop with epithelium like characteristics of desmosomes, microvilli, etc. and can proliferate. These abnormal endothelial cells are dysfunctional, leading to corneal edema. Further more, the corneal endothelium proliferates and migrates like a membrane covering the angle of anterior chamber and anterior surface of iris. This membrane contracts resulting in peripheral anterior synechiae, corectopia, iris atrophy, iris stretch holes, iris nodules & glaucoma. Ischemia may be a secondary phenomenon causing Iris melt holes. The trigger for the epithelialization of these endothelial cells is unknown.2 Though some postulate abnormal neural crest cell proliferation. Histopathological examination of the eyes with ICE syndrome shows thin abnormal corneal endothelium and Descemet’s membrane separated by a thick accumulation of collagen.3

Specular microscopic examination of this group of disorders shows characteristic ICE cells. The cells lose their normal hexagonal appearance of endothelial cells and appear rounded with raised intracellular granularity and a dark spot. The corneal endothelial changes are marked and earliest in Progressive Iris Atrophy entity of ICE syndrome with minimal anterior synechiae.4 The clinically non-involved, contralateral eye of these patients also show such abnormal endothelial cells in few cases.

The corneal endothelial changes are most prominent & present with blurring of vision and silver hammered or beaten bronze appearance of cornea due to corneal edema. It can rarely be bilateral. Slit lamp Biomicroscopy can identify the junction between normal and abnormal endothelium on careful examination using specular reflection.5

This clinical entity has marked Iris changes characterized by corectopia and progressive dissolution of the iris. The iris dissolution starts as a patchy atrophy of corneal stroma leading to full thickness holes. Iris holes are located away from the direction of pupillary displacement, caused due to traction and known as stretch holes.6 Melt holes occur without corectopia and stretching are thought to be ischemic in nature. Peripheral anterior synechiae (PAS) are mostly broad based & located anterior to Schwalbe's line resulting in corectopia towards the PAS. These PAS, lift the iris off the surface of the lens and produce ectropion uveae. As PAS becomes more extensive, leading to secondary angle closure and rise in IOP.3

This can be distinguished by the presence of pigmented iris lesions or pedunculated nodules on anterior surface of Iris. There are patches of normal iris pinched by the contracting endothelial membrane.7 Other features include heterochromia and ectropion uvea. Iris atrophy is absent in half the cases and in the remaining half it is usually mild-moderate, although corectopia may be severe.

Symptoms and signs of ICE syndrome

  1. Intermittent blurred vision and change in the appearance of iris/pupil.

  2. Halos around lights and photophobia seen in patients with corneal edema

  3. Conjunctival hyperemia and pain is seen in cases of corneal edema and increase in IOP.

  4. Varying degrees of iridocyclitis with KP.

Differential diagnosis

  1. Posterior polymorphous dystrophy

  2. Fuch's endothelial Dystrophy

  3. Iris abnormalities like iridoschisis and malignant melanoma

  4. Developmental disorders like Reigers syndrome and aniridia

  5. Neurofibromatosis and anterior uveitis with nodules (most of these conditions are bilateral).

Treatment

Corneal edema may respond to topical hyperosmotic solution or ointment or soft contact lens. In many cases if IOP improves, corneal edema reduces Penetrating keratoplasty is indicated if vision is reduced significantly or causes pain from bullous keratopathy or secondary infectious keratitis.2

Glaucoma

Medical management has generally found to be inefficient over long-term. If the entire angle is covered by the membrane or sealed by synechiae, clinician must rely on β adrenergic antagonists, α adrenergic agonists and carbonic anhydrase inhibitors. As a general rule medical treatment fails because the condition is progressive.4 Filtering surgery is often required though it fails after 2 to 5 years, perhaps related to proliferation of a membrane over the internal opening of the scelerostomy even when adjunctive use of antimetabolite therapy. In such cases, repeat trabeculectomy with MMC application or other shunt operations can be attempted.2 Immunotoxin has been shown to inhibit proliferation of endothelium in tissue culture. Perhaps antiviral therapy may play some role if the presence of viral particles can be confirmed and positively identified.

Conclusion

  1. The case stands out due to its complexity. It predominantly had progressive changes in the iris and can be classified as essential iris atrophy but there were Glaucoma features as well (LE CDR-0.5).

  2. She had a feature of progressive iris atrophy associated with secondary Glaucoma.

  3. Long-term follow-up is necessary because the disease itself is progressive in nature.

Source of Funding

None.

Conflict of Interest

The authors declare that there is no conflict of interest.

References

1 

R Allingham K Damji S Freedman RR Allingham K Damji S Freedman Glaucomas associated with disorders of the corneal endotheliumShield’s Textbook of Glaucoma5th editionLippincott Williams and WilkinsPhiladelphia200428896

2 

H Laganowsky K Muir KA Hitchings Glaucoma and iridocorneal endothelial syndromeArch Ophthalmol199211034650

3 

M Yanoff Iridocorneal endothelial syndrome: Unification of a disease spectrumSurv Ophthalmol19792411210.1016/0039-6257(79)90142-5

4 

MC Wilson MB Shields A comparison of the clinical variations of the iridocorneal endothelial syndromeArch Ophthalmol19891071014658

5 

MB Hamill JH Krachmer MJ Mannis EJ Holland Mechanical injuryCornea, Vol 1MosbyPhiladelphia2005

6 

JH Weaver A needle for corneal foreign body removalTrans Am Acad Ophthalmol Otolaryngol19717536601

7 

D G Wilsoncampbell R J Simmons The essential iris atrophiesAm J Ophthalmol1978857498209



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© This is an open access article distributed under the terms of the Creative Commons Attribution License - Attribution 4.0 International (CC BY 4.0). which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.


Article type

Case Report


Article page

159-163


Authors Details

M Sathish Kumar, J Samuel Cornelius Gnanadurai, Rubina Huda


Article History

Received : 19-08-2020

Accepted : 28-08-2020

Available online : 31-03-2021


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