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Clinical photograph showing the 3 variants of ICE syndrome. (a) Eye with progressive iris atrophy after AGV implantation, (a1) shows the same eye after DSEK and intraocular lens implantation. (b) Eye with Cogan-Reese syndrome, (b1) same eye after trabeculectomy with MMC. (c) Eye with Chandler syndrome, (c1) same eye after trabeculectomy with MMC and DSEK. doi:10.1371/journal.pone.0171884.g001
Source publication
Purpose
To report the demographic profile, clinical features, and prevalence of glaucoma and its management in patients with Iridocorneal endothelial (ICE) syndrome.
Methods
Retrospective review of 203 consecutive subjects with ICE syndrome at a tertiary eye care centre between January 1988 and June 2013.
Results
ICE syndrome was present in 223 e...
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Purpose Iridocorneal endothelial (ICE) syndrome is a rare condition, and unique characteristics in Chinese patients can make diagnosis difficult. Our purpose was to describe the clinical characteristics and variations of ICE syndrome in 58 consecutive Chinese patients. Methods
The clinical data of consecutive patients with ICE syndrome who were see...
Purpose: To report the outcomes of Descemet stripping automated endothelial keratoplasty (DSAEK) performed in iridocorneal endothelial (ICE) syndrome patients using the intraocular lens injector (injector), which was compared with those using the Busin glide.
Methods: In this retrospective, case-control study, we evaluated the outcomes of DSAEK per...
One eye with ICE syndrome underwent DSAEK for corneal decompensation after cataract
surgery and was followed up for 24 months. DSAEK was successfully performed in this case.
No graft dislocation occurred. At first post-operative visit patient showed visual improvement
to 20/160 with pinhole. IOP: 10, corneal clarity has significantly improved. At 6...
Background
Axenfeld-Rieger syndrome (ARS) is a rare kind of anterior segment dysgenesis (ASD). The most common ocular features of ARS are posterior embryotoxon and iris hypoplasia, while some patients may manifest as corneal opacity and edema. However, the current understanding of how ARS affects the cornea is still incomplete. This study reports a...
Purpose:
We describe a combined Descemet Membrane Endothelial Keratoplasty (DMEK) using the Cornea-press (C-Press) technique, with implantation of a new sutureless, scleral fixated intraocular lens (IOL) (Carlevale, Soleko), in a case of bullous keratopathy and IOL mispositioning.
Observations:
Two scleral pockets were created along two scleral...
Citations
... Currently, studies of binocular ICE syndrome were dominated by case reports [4][5][6] . Large sample studies of binocular ICE syndrome were rare and mainly concentrated in Asia (Table 1) [7][8][9] . It can be difficult to distinguish ICE syndrome from recurrent viral anterior uveitis, posterior polymorphous corneal dystrophy (PPCD) and Axenfeld-Rieger syndrome, when the clinical manifestations of ICE syndrome are atypical. ...
... While the first line treatment is medical therapy, this is frequently ineffective in adequately lowering IOP in ICE syndrome. 5 A large proportion of patients with ICE syndrome will therefore require surgical intervention. Various IOP-lowering surgical options are available, including trabeculectomy. ...
... A retrospective review of 203 ICE syndrome patients at a tertiary care centre in India showed that despite medical therapy to lower IOP, at least 50% of ICE syndrome patients required surgical intervention. 5 The success of primary trabeculectomy with MMC in their series was 55% at 5 years. 6 Of the eyes that underwent surgical intervention, 27% required repeat surgery to control transplantation, further demonstrating the versatility of these devices in complex glaucoma cases. ...
Purpose
To report on a case of angle-closure glaucoma secondary to iridocorneal endothelial (ICE) syndrome effectively managed with the PreserFlo Microshunt.
Observations
We report successful implantation of a PreserFlo Microshunt in a 57-year-old patient with secondary angle-closure glaucoma in the context of ICE syndrome. Following failure of medical therapy to adequately control intraocular pressure (IOP), the patient was consented for surgical intervention and underwent combined cataract surgery and PreserFlo Microshunt implantation. IOP at the last post-operative follow-up (5 months) was 12 mmHg with the patient on brinzolamide/timolol maleate (Azarga®). We report no complications in the post-operative period.
Conclusions and importance
The PreserFlo Microshunt may be a promising option for patients with ICE syndrome who fail medical therapy. Implantation of this device was well tolerated in the presented case.
... The pathogenesis involves the production of a Descemet's membrane-like proliferative film that extends and proliferates across the Schwalbe line into the angle and iris surface by abnormal corneal endothelial cells [1]. ICE syndrome is frequently complicated by severe secondary glaucoma, often requiring surgical treatment in relatively young people [2,3]. However, even in patients with undiagnosed glaucoma, temporary elevations of intraocular pressure (IOP) not observed during the day have been reported, and its presence is therefore difficult to confirm in medical clinics [4]. ...
... It has been reported that 70%-74% of patients with ICE syndrome have glaucoma at the time of initial diagnosis [2,3] Generally, aqueous humor production is higher in the morning than during the rest of the day, and therefore, peak diurnal IOP fluctuation occurs predominantly in the morning hours [5]. Baskaran et al. reported that in patients with primary angle closure and primary angle closure glaucoma, the degree of PAS was associated with diurnal IOP fluctuations [6]. ...
We report on a patient with iridocorneal endothelial (ICE) syndrome in whom intraocular pressure (IOP) elevation during the night was detected using a home tonometer. A 44-year-old woman was diagnosed with ICE syndrome in the left eye. Angle-closure attack-like symptoms, including blurred vision and headache, appeared and spontaneously resolved irregularly at bedtime. Daytime examination indicated normal IOP and no obvious signs of glaucoma such as visual field defects or fundus abnormalities. However, nocturnal IOP measurements using a home tonometer revealed temporary high IOP at the time of symptom onset. A home tonometer may be a useful tool to detect transient IOP elevation at night, even if the IOP is normal during daytime examinations.
... etiology and pathophysiology. Therefore, the clinical management of ICE syndrome is mainly symptomatic to deal with corneal edema and glaucoma, which are the most common causes of vision loss in this disease (1)(2)(3). ...
... A recent large retrospective case analysis shows that corneal edema occurs in ∼56% of patients, and 14% require keratoplasty (3). Penetrating keratoplasty (PK) can significantly improve visual acuity; however, the long-term outcomes were heterogeneous in different studies, with high allograft rejection and the need for multiple corneal transplants (4)(5)(6)(7). ...
... Approximately 73% of patients with ICE syndrome have secondary glaucoma, and 50% require surgical intervention (3). Glaucoma control after keratoplasty is crucial for graft survival (18)(19)(20), especially in ICE syndrome (12,13). ...
Purpose
Iridocorneal endothelial (ICE) syndrome is a progressive anterior segment disorder that can be tricky to treat. Keratoplasty is commonly used to treat corneal edema in ICE syndrome. However, glaucoma is an important risk factor affecting graft survival. To address this question, we designed a retrospective cohort study to evaluate the effect of Spokewise Iridotomy (SI) on Descemet Stripping Automated Endothelial Keratoplasty (DSAEK) Grafts in Iridocorneal Endothelial (ICE) Syndrome.
Methods
This was a retrospective cohort study. A total of 29 patients were included; 31 eyes with ICE syndrome underwent DSAEK at Peking University Third Hospital between June 2015 and June 2022, including 11 eyes with combined SI during DSAEK. The aim was to explore the effect of SI on vision, glaucoma control, complications, peripheral anterior synechiae recurrence, endothelial cell count, and graft survival.
Results
The median follow-up time was 30.83 months (mo.) in the SI+Endothelial Keratoplasty (EK) group and 6.17 mo in the EK group. The 2-year cumulative survival rate of grafts in the SI+EK group was 100%, compared with the 6-month and 1-year cumulative survival rates of 80.2 and 63.2%, respectively, in the EK group ( p = 0.043). The SI+EK group had a lower incidence of immediate postoperative complications ( p = 0.005), fewer postoperative anti-glaucoma medications (AGMs) ( p = 0.029), smaller peripheral anterior synechiae recurrence ( p = 0.001), and significant visual acuity improvement ( p < 0.05). More AGMs were used in failed grafts ( p = 0.002).
Conclusion
SI can help control intraocular pressure, improve visual acuity, and increase graft survival after DSAEK in ICE syndrome patients.
... It causes refractory glaucoma because of abnormal corneal endothelial cells, which are responsible for peripheral anterior synechia (PAS), pupillary deviation, iris atrophy, and iris nodules, by producing membrane-like materials on the surface of the anterior chamber angle and iris. [1][2][3] Although trabeculectomy [4][5][6][7][8] and tube shunt surgery 9,10 are indicated as surgical treatments for ICE syndrome, surgical outcomes are relatively poor, and postoperative complications are common. The Ex-PRESS shunt, which does not require peripheral iridectomy, has been used in some patients with the ICE syndrome. ...
Purpose
Iridocorneal endothelial (ICE) syndrome may cause refractory glaucoma due to progressive synechial closure or membrane formation at the anterior chamber angle. Filtration surgeries are often required but are associated with a higher rate of surgical failure or complications than other types of glaucoma. Herein, we report a new and effective surgical procedure for glaucoma secondary to ICE syndrome: Ex-PRESS shunt combined with ab-interno peripheral iridectomy using a small-gauge vitreous cutter.
Methods
Three patients with ICE syndrome who underwent surgery were included. Intraoperatively, an ab-interno peripheral iridectomy was performed using a small-gauge vitreous cutter through a corneal incision in the superior-nasal or superior-temporal quadrants to create space for the insertion of Ex-PRESS shunt free from the iris tissue. The shunt was inserted under the scleral flap. The first patient underwent combined cataract surgery, whereas patients 2 (pseudophakia) and 3 (phakia) underwent Ex-PRESS alone.
Results
No intraoperative complications were observed. The intraocular pressure remained stable until the final postoperative visits at approximately 7, 4, and 1 year in Cases 1, 2, and 3, respectively. Case 2, with mild preoperative corneal edema due to graft failure in Descemet's stripping automated endothelial keratoplasty (DSAEK), underwent reDSAEK 6 months postoperatively.
Conclusions and importance
Ex-PRESS shunt combined with ab-interno peripheral iridectomy using a small-gauge vitreous cutter may be a safe and effective surgical procedure in patients with ICE syndrome, regardless of the lens status.
... The prevalence of glaucoma in eyes with ICE syndrome has been reported to range from 46% to 82%. 5 ...
... The prevalence of glaucoma in ICE syndrome has been reported to range from 46 to 82% [4,8,9]. Glaucoma is usually refractory, requiring multiple medications and surgical procedures [10][11][12]. ...
... The rate of reoperation for glaucoma and complications trended higher in the trabeculectomy group relative to the tube group. Secondary glaucoma associated with ICE syndrome is frequently refractory to medical management and commonly requires glaucoma surgery for IOP control [8][9][10][11][12]. Patients with ICE syndrome have been reported to have a higher failure rate after conventional filtration surgery than most other types of glaucoma [4,[8][9][10][11][12][13][14]. ...
... Secondary glaucoma associated with ICE syndrome is frequently refractory to medical management and commonly requires glaucoma surgery for IOP control [8][9][10][11][12]. Patients with ICE syndrome have been reported to have a higher failure rate after conventional filtration surgery than most other types of glaucoma [4,[8][9][10][11][12][13][14]. The relatively younger age of onset with robust healing and scarring and the progressive membrane proliferation blocking the internal ostium and formation of anterior synechiae may predispose to long-term failure of trabeculectomy [13]. ...
Purpose
To compare the surgical outcomes of the Aurolab aqueous drainage implant (AADI) and trabeculectomy with mitomycin C (MMC) in patients with glaucoma secondary to iridocorneal endothelial (ICE) syndrome.
Materials and methods
This retrospective comparative case series included 41 eyes of 41 patients with ICE syndrome and glaucoma who underwent either a trabeculectomy with MMC (n = 20) or AADI surgery (n = 21) with a minimum of 2 years follow-up. Outcome measures included intraocular pressure (IOP), the use of glaucoma medications, visual acuity, additional surgical interventions, and surgical complications. Surgical failure was defined as IOP > 21 mmHg or reduced < 20% from baseline, IOP ≤ 5 mmHg, reoperation for glaucoma or a complication, or loss of light perception vision.
Results
The cumulative probability of failure at 2 years was 50% in the trabeculectomy group (95%CI = 31–83%) and 24% in the AADI group (95%CI = 11–48%) (p = 0.09). The IOP was consistently lower in the AADI group compared with the trabeculectomy group at 6 months and thereafter. Surgical complications occurred in 13 eyes (65%) in the trabeculectomy group and 12 eyes (57%) in the AADI group (p = 0.71). Reoperations for glaucoma or complications were performed in 12 eyes (60%) in the trabeculectomy group and 5 patients (24%) in the tube group (p = 0.06). Cox proportional hazards showed that AADI had a 53% lower risk of failure at 2 years (p = 0.18; HR = 0.47; 95%CI = 0.16–1.40).
Conclusion
AADI surgery achieved lower mean IOPs than trabeculectomy with MMC in managing glaucoma secondary to ICE syndrome. A trend toward lower rates of surgical failure and reoperations for glaucoma and complications was observed following AADI placement compared with trabeculectomy with MMC in eyes with ICE syndrome.
... Cogan-Reese syndrome, otherwise known as iris nevus syndrome, is the least common variant of ICE syndrome [3]. In Cogan-Reese syndrome, iridic stromal matting and stromal loss, fine iris nodules, and unilateral angle-closure glaucoma often develop secondary to endothelial membrane formation on the anterior portion of the iris [4]. ...
The aim of this study was to report a novel presentation of Cogan-Reese syndrome presenting with zonular dehiscence during cataract extraction with intraocular lens placement. A 49-year-old woman presented with worsening vision over 2 years. The examination was significant for bilateral pupil miosis, visually significant cataracts, and unilateral glaucoma. No iris nodules or corneal endothelial disease was observed through slit-lamp examination and specular microscopy bilaterally. Cataract extraction on the left eye was complicated by significant zonular dehiscence. An iris sample taken during the procedure demonstrated histopathologic findings consistent with an iris nodule composed of melanocytic nevoid cells. Cogan-Reese, or iris nevus syndrome, is a subset of iridocorneal endothelial syndrome that usually presents with iridic stromal matting and stromal loss, nodule formation, and secondary unilateral angle-closure glaucoma. Here, we describe a presentation of Cogan-Reese syndrome that presented with pupillary miosis and glaucoma preoperatively, and zonular dehiscence during cataract extraction with no underlying corneal pathology.
... Glaucoma is strongly associated with ICE syndrome. 2,13 Mechanism of glaucoma is that altered corneal endothelial cells have migratory characteristics and hence it moves posteriorly onto the trabecular meshwork and peripheral iris. Peripheral anterior synechiae results from contraction of this tissue within the angle, causing secondary angle closure glaucoma. ...
... CRS (20%) was the least common variant of ICE syndrome which was similar to Chandran et al in which PIA (52%) was the predominant variant followed by CS and the least was CRS. 13 Laganowski et al case series also showed the similar frequency 2 . Wilson et al 10 reported that Cogan-Reese was the commonest in Caucasian population, whereas Teekhasaenee et al reported that Cogan-Reese syndrome was common in the Thai population. ...
... In other published series it ranged from 12-40%. 1,2,13,14 These differences can be possibly explained by the variations in the severity of the disease and the evolution of anti-glaucoma medications over time. Chandan et al studied a case series of 202 patients and found an inter eye variance in the presentation of ICE syndrome. ...
Clinical Profile of Iridocorneal Endothelial Syndrome at a Tertiary Care Eye Centre
... Progressive or Essential iris atrophy (EIA) is the most common irido-corneal endothelial syndrome (ICE) in Indian eyes [1]. The basic pathology lies in the corneal endothelium, which proliferates and migrates across the anterior chamber angle and on to the anterior surface of the iris. ...
... Though our patient presented to us with bilateral POAG, over the years developed features of EIA and secondary angle closure component in his left eye. Chandran P., et al. showed that 52% of their patients with ICE syndrome had EIA, and 90% of them had uniocular disease [1]. ...
... Though right eye responded to the medical management, IOP in left eye could not be controlled with maximum tolerable AGM, owing to progressive iris atrophy with PAS formation. Glaucoma in ICE syndrome responds poorly to medical management (50%) [7] more so, if associated with EIA [1]. Filtering surgery and GDD failed to control IOP in our patient. ...
