ABSTRACT PURPOSE To describe the results of endothelial keratoplasty (EK) in the management of irido-corneal endothelial (ICE) syndrome. DESIGN Retrospective, consecutive, interventional

case series. METHODS Eight eyes of seven patients underwent EK for corneal edema owing to ICE syndrome. Simultaneous cataract surgery was performed in five eyes. The charts were reviewed for

demographics, clinical findings, and outcomes after EK. RESULTS Mean age was 50.4 years (range 35–59 years). Mean follow-up was 12.5 months (range 6–24 months). Corneal clarity was restored

in all eyes and was maintained till the last follow-up. Four patients had acute rise in the intraocular pressure in the post-operative period, which was well controlled on medical

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AFTER DESCEMET MEMBRANE ENDOTHELIAL KERATOPLASTY (DMEK) Article Open access 06 July 2020 INTRODUCTION Irido-corneal endothelial (ICE) syndrome includes a spectrum of clinical manifestations

characterized by abnormal proliferation of corneal endothelium leading to corneal edema.1, 2 Until recently, penetrating keratoplasty was performed for visual rehabilitation in these eyes.3,

4, 5 In this series, we report the clinical outcomes in eight eyes of seven patients who underwent endothelial keratoplasty (EK) in the management of this syndrome. MATERIALS AND METHODS

From January 2009 to December 2011, eight eyes of seven patients were identified and the charts were reviewed for demographics, clinical findings, and outcomes after EK. One case (Patient 4)

has been included in another published case series.6 Table 1 summarizes the demographics, clinical features, and the outcomes in the patients who underwent EK for ICE syndrome. Mean

follow-up was 12.5 months (range 6–24 months). SURGICAL TECHNIQUE The donor cornea dissection was done using Moria ALTK microkeratome system (Moria/Microtek Inc., Doylestown, PA, USA)

equipped with 350 μm microkeratome head. Descemet’s membrane stripping was done in all eyes except one (Case 4). Sheets IOL glide (BD Medical-Ophthalmic Systems, Waltham, MA, USA) was used

and donor lenticle was inserted using the push-in technique protecting the endothelium with sodium hyaluronate 1% (Healon, Abbott Medical Optics Inc., Abbott Park, IL, USA). The graft was

positioned using air tamponade. Additional procedures such as synechiolysis and cataract surgery were performed when needed. Post-operative evaluation was performed at regular intervals and

included assessment of best-corrected visual acuity, slit lamp examination, tonometry, and confocal microscopy (Confoscan 4, Nidek, Gamagori, Japan) whenever possible. The patients were

treated with anti-glaucoma medications based on the intraocular pressure (IOP) and the disc findings after consultation with the glaucoma specialists. CASE REPORTS Six patients had

unilateral and one (Case 2) had bilateral presentation (Figure 1a and b). One patient (Case 4) developed progressive corneal decompensation following trabeculectomy surgery. Five eyes had

glaucomatous disc changes before surgery; in two the disc changes were evident on B scan ultrasonographaphy as the fundus view was obscured owing to corneal edema. The disc changes were

confirmed after the restoration of corneal clarity following EK. The IOP was normal in all eyes at the time of surgical intervention. All eyes were phakic at the time of surgery, but six

eyes had significant cataract. All patients had visual acuity <20/200 at the time of surgery. Corneal clarity was restored and grafts remained clear till the last visit in all eyes.

Post-operative visual acuity ranged from 20/50–20/30. No intra-operative and immediate post-operative complications were encountered in any patient except one patient, who had an exaggerated

anterior segment inflammation, which was controlled with steroid treatment. In one patient (Case 4), anterior chamber was very shallow despite performing synechiolysis. Descemet stripping

was deferred in this patient to avoid inadvertent trauma to the clear lens. The interface was clear throughout the post-operative period. Four of the seven patients had increased IOP in the

post-operative follow-up period. The IOP rise was controlled medically with short course of acetazolamide and addition of one or two topical anti-glaucoma medication. The patients who had

post-operative increase in IOP and/or had clinical evidence of glaucomatous disc changes were continued on one or two topical anti-glaucoma medications based on the IOP control. In one

patient (Case 1), there was a significant progression of disc and field changes in post-operative period due to non-compliance to the anti-glaucoma medications. No apparent increase in

anterior chamber angle abnormalities after keratoplasty was noted on clinical slit lamp examination during the follow-up visits. DISCUSSION ICE syndrome includes progressive essential iris

atrophy, Chandler’s syndrome, and the Iris-nevus syndrome.1 It is typically considered as unilateral, although cases of bilateral involvement of the same or two different forms might occur.7

Price and Price8 first reported the management of these eyes with EK with good results in three patients with a follow-up range of 1–14 months. In our series, 5/7 (71.4%) patients had

pre-existing glaucomatous disc changes. Four of the five patients had episodes of rise in IOP in the post-operative period, which was managed medically. One patient was non-compliant with

anti-glaucoma medications and progressed to advanced field loss. As EK effectively restores the corneal clarity, the paramount factor in maintaining good visual acuity in these eyes is

adequate control of glaucoma, which is seen episodically in this syndrome and may not be associated with symptoms such as pain or headaches. Another reason for the rise in IOP could be

attributed to a steroid response, although this is difficult to differentiate from the natural course of the disease in these eyes. Hence, patients undergoing EK for ICE syndrome should be

followed up regularly and probably at more frequent intervals to closely monitor for IOP control. This is particularly important in eyes with pre-existing glaucomatous disc changes. We did

not observe any remarkable changes in iris and angle abnormalities following EK. A longer period of follow-up is needed for better understanding. In conclusion, surgical intervention in the

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Research Foundation, India. AUTHOR CONTRIBUTIONS Involved in design, conduct of study, and data collection: SCH; analysis and interpretation of results: SCH; preparation of manuscript and

literature search: SCH and MR; and review of manuscript and final approval: SCH, MR, SIM, SIR, PG, and VSS). Institutional Review Board, LV Prasad Eye Institute, Hyderabad, approved the

study. AUTHOR INFORMATION AUTHORS AND AFFILIATIONS * Cornea and Anterior Segment Services, LV Prasad Eye Institute, Hyderabad, India S Chaurasia, M Ramappa, P Garg, S I Murthy & V S

Sangwan * VST Glaucoma center, LV Prasad Eye Institute, Hyderabad, India S Senthil Authors * S Chaurasia View author publications You can also search for this author inPubMed Google Scholar

* M Ramappa View author publications You can also search for this author inPubMed Google Scholar * P Garg View author publications You can also search for this author inPubMed Google Scholar

* S I Murthy View author publications You can also search for this author inPubMed Google Scholar * S Senthil View author publications You can also search for this author inPubMed Google

Scholar * V S Sangwan View author publications You can also search for this author inPubMed Google Scholar CORRESPONDING AUTHOR Correspondence to S Chaurasia. ETHICS DECLARATIONS COMPETING

INTERESTS The authors declare no conflict of interest. RIGHTS AND PERMISSIONS Reprints and permissions ABOUT THIS ARTICLE CITE THIS ARTICLE Chaurasia, S., Ramappa, M., Garg, P. _et al._

Endothelial keratoplasty in the management of irido-corneal endothelial syndrome. _Eye_ 27, 564–566 (2013). https://doi.org/10.1038/eye.2012.298 Download citation * Received: 30 April 2012 *

Accepted: 13 December 2012 * Published: 25 January 2013 * Issue Date: April 2013 * DOI: https://doi.org/10.1038/eye.2012.298 SHARE THIS ARTICLE Anyone you share the following link with will

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content-sharing initiative KEYWORDS * endothelial keratoplasty * ICE syndrome * glaucoma