Iridocorneal Endothelial Syndrome Research Articles

Bilateral iridocorneal endothelial syndrome-Chandler's syndrome: a case report and literature review.

Bilateral iridocorneal endothelial syndrome-Chandler's syndrome: a case report and literature review.

Clinical Features of Iridocorneal Endothelial Syndrome in Korean Patients

Purpose: To document the distribution and clinical features of iridocorneal endothelial (ICE) syndrome in its various subtypes observed in patients who visited a single institution in Korea.Methods: A retrospective analysis was conducted on the medical records of patients diagnosed with ICE syndrome at the Department of Ophthalmology, Yeungnam University Hospital, from 1993 to 2023. The cases were classified into Chandler syndrome, progressive iris atrophy, and iris nevus syndrome based on the severity of corneal and iris abnormalities. We also examined the presence of glaucoma, corneal edema, and iris abnormalities at diagnosis.Results: In all, 22 eyes from 21 patients with ICE syndrome were included. The mean age at diagnosis was 56.6 ± 16.1 years with a female predominance (20 patients, 95.5%). The syndrome was primarily unilateral (20 patients, 95.5%). Subtypes included 9 eyes with Chandler syndrome (40.9%), 7 eyes with progressive iris atrophy (31.8%), and 6 eyes with iris nevus syndrome (27.3%). Glaucoma was present in 15 eyes (68.2%), with 8 eyes (36.4%) undergoing glaucoma surgery. Of the 7 eyes that had trabeculectomy as initial glaucoma surgery, six experienced poor intraocular pressure control leading to Ahmed valve implantation in 4 cases. Corneal edema was noted in 10 eyes (45.5%) at diagnosis progressing to corneal decompensation in 6 eyes (27.3%).Conclusions: ICE syndrome in Korea predominantly affects middle-aged women and occurs unilaterally with Chandler syndrome being the most common subtype. Given the high risk for glaucoma development, suboptimal outcomes of trabeculectomy, and significant risk for corneal decompensation, ongoing management efforts are essential for glaucoma and corneal function preservation.

Outcomes of Trabeculectomy and Ahmed Glaucoma Valve Implantation in Patients with Iridocorneal Endothelial Syndrome.

Our study highlights the long-term success of trabeculectomy or AGV surgery in subjects with glaucoma secondary to ICE syndrome. However, many of ICE syndrome cases may need multiple glaucoma surgeries to achieve controlled IOP, with/without concomitant corneal graft surgery. To evaluate the long-term outcome of trabeculectomy and Ahmed glaucoma valve (AGV) implantation in iridocorneal endothelial syndrome (ICE). Patients with glaucoma secondary to ICE syndrome who underwent either trabeculectomy or AGV surgery with intraoperative adjunctive MMC from 2009 to 2020 were included in this study. All patients were followed for at least 6 months after initial surgery. The main outcome measures were intraocular pressure (IOP), number of IOP lowering medications and surgical success. Surgical success was defined as complete according to the levels of IOP (<18) and at least 20% reduction from preoperative IOP without medications and qualified as complete success but with medications, where the number of medications was less than preoperative numbers. Cumulative success was the sum of the qualified and complete success. Twenty-nine eyes of 29 patients were included. Trabeculectomy was done in 13 patients (group A, 44.8%) and 16 patients underwent AGV surgery (group B, 55.2%). The median age was 50(42-56.50) and 47(36.75-52.75) years in groups A and B, respectively (P value=0.10). All patients completed at least 2 years of follow up. Mean IOP was not significantly different between groups preoperatively (P=0.70) and the effect of the type of surgery on IOP was not statistically significant at multiple follow-up time points (repeated measures ANOVA, P=0.44). The mean IOP decreased from 35.76±6.36mmHg preoperatively to 16.00+3.10 in group A and from 36.12+8.11mmHg to 17.00+3.75 in group B (P=0.449) at year 2 of follow-up. The effect of the type of surgery was not significant on the total number of IOP lowering medications used throughout the study (repeated measures ANOVA, P=0.81). Kaplan-Meier analysis show complete success in 14 patients (48.3%), 11 patients (37.9%) and 7 patients (24.1%) at 6-month, 1 year and 2 years follow-up, respectively. Cumulative success rate was 95% at two years follow-up for all patients. In 2 year follow up, trabeculectomy or AGV could significantly reduce the IOP in glaucoma patients secondary to ICE syndrome.

A Challenging case of essensial iris atrophy ICE Syndrome with secondary angle closure glaucoma at Pasar Rebo General Hospital : A Case Report

Introduction : ICE syndrome is a unique ophthalmic disorder that involves an iris atrophy, irregular corneal oedema, and secondary angle-closure mainly occurs unilaterally in young and middle-aged women, with no family history. Approximately 50% of ICE syndrome patients will later develop secondary angle closure glaucoma. The following case report presents a patient with essential iris atrophy and secondary angle closure glaucoma.&#x0D; Case Illustration : A 50 year old woman presented with painful and redness of the right eye (RE) since 1 week. Her visual acuity was 6/40 of RE and 6/30 of left eye (LE). Her intraocular pressure (IOP) was 61,3 mmHg and 19 mmHg for the RE and LE. On the slit lamp, we had a proper RE finding, the iris atrophy with deformity in the direction from 12 to 6 hours, with minimal corneal edema. We performed gonioscopy, fundus photographs and papillary optical coherence tomography. The patient was diagnosed with ICE syndrome with essential iris atrophy type and secondary angle closure glaucoma then was given anti glaucoma agents.DiscussionICE syndrome treatments focus on managing the corneal edema and secondary glaucoma. We treated our patient with maximal oral and topical combination therapy such as carbonic anhydrase inhibitors, beta blockers, cholinergic, prostaglandin analogs for 6 months the IOP and corneal oedema were well maintained. However, maintaining long term success can be challenging, surgical therapy with a filtering procedure can be considered if the IOP is uncontrolled.&#x0D; Conclusion : We confirmed the diagnosis of essential iris atrophy based on its clinical findings and abnormalities in complementary exams.

Triangular pupil: the early sign of ICE syndrome

Triangular pupil: the early sign of ICE syndrome

The Role of Rho Kinase Inhibitors in Corneal Diseases.

The cornea, as the outermost layer of the eye, plays a crucial role in vision by focusing light onto the retina. Various diseases and injuries can compromise its clarity, leading to impaired vision. This review aims to provide a thorough overview of the pharmacological properties, therapeutic potential and associated risks of Rho-associated protein kinase (ROCK) inhibitors in the management of corneal diseases. The article focuses on four key ROCK inhibitors: Y-27632, fasudil, ripasudil, and netarsudil, providing a comparative examination. Studies supporting the use of ROCK inhibitors highlight their efficacy across diverse corneal conditions. In Fuchs' endothelial corneal dystrophy, studies on the application of Y-27632, ripasudil, and netarsudil demonstrated noteworthy enhancements in corneal clarity, endothelial cell density, and visual acuity. In pseudophakic bullous keratopathy, the injection of Y-27632 together with cultured corneal endothelial cells into the anterior chamber lead to enhanced corneal endothelial cell density and improved visual acuity. Animal models simulating chemical injury to the cornea showed a reduction of neovascularization and epithelial defects after application of fasudil and in a case of iridocorneal endothelial syndrome netarsudil improved corneal edema. Addressing safety considerations, netarsudil and ripasudil, both clinically approved, exhibit adverse events such as conjunctival hyperemia, conjunctival hemorrhage, cornea verticillata, conjunctivitis, and blepharitis. Monitoring patients during treatment becomes crucial to balancing the potential therapeutic benefits with these associated risks. In conclusion, ROCK inhibitors, particularly netarsudil and ripasudil, offer promise in managing corneal diseases. The comparative analysis of their pharmacological properties and studies supporting their efficacy underscore their potential therapeutic significance. However, ongoing research is paramount to comprehensively understand their safety profiles and long-term outcomes in diverse corneal conditions, guiding their optimal application in clinical practice.

An Unusual Case of Unilateral Iridocorneal Endothelial Syndrome with Keratoconus and Secondary Angle Closure Glaucoma: A Case Report

We report an unusual association of unilateral keratoconus–Iridocorneal endothelial syndrome with secondary angle closure glaucoma in a 22-year-old female. The patient was advised glaucoma drainage device as she was a breastfeeding mother with limited options for antiglaucoma medications. This case highlights the ophthalmologists need to look for multiple entities linked to keratoconus.

Management of refractory glaucoma in Cogan–Reese variant of ICE syndrome with secondary tube implantation

Glaucoma secondary to iridocorneal endothelial syndrome is refractory to medical treatment, and its management is always challenging. We report the clinical course and management of refractory glaucoma in a 47-year-old male diagnosed with Cogan–Reese syndrome with an 11-year follow-up result. He initially underwent trabeculectomy, which eventually failed after 7 years; hence, an Aurolab aqueous drainage implant (AADI) was placed superotemporally. On subsequent follow-up, as the AADI tube got retracted, secondary AADI implantation was placed in the superonasal quadrant. Later, his vision dropped due to cataract and underwent phacoemulsification with intraocular lens implantation along with tube trimming.

Iridocorneal endothelial syndrome with rare case of posterior inflammation

Iridocorneal endothelial (ICE) syndrome is a group of iris and corneal disorders. Its presentation is the mixture of iris and corneal anomalies which may lead to low vision or blindness due to secondary glaucoma or corneal decompensation. We report a case of ICE syndrome in one eye and primary angle closure suspect in the other eye, both eyes having controlled intraocular pressure but low vision due to pars planitis/intermediate uveitis with macular oedema and also evaluating more potential inflammatory causes.

Corneal edema caused by undiagnosed iridocorneal endothelial syndrome after SMILE

The aim. To present a clinical case of a patient with corneal edema caused by undiagnosed iridocorneal endothelial syndrome after SMILE surgery. Iridocorneal endothelial syndrome is characterized by abnormal proliferation of the corneal endothelium beyond the Schwalbe line, blockade of the iridocorneal angle, specific changes in the shape of the pupil and the formation of synechiae between adjacent structures of the anterior chamber angle of the eye, which is accompanied by the development of pretrabecular retention of intraocular aqueous humour, increased intraocular pressure (IOP) and subsequent development of glaucoma in 46–82 % of patients. One of the forms of the disease is Chandler’s syndrome, which clinically in the initial stages may be accompanied by the formation of only moderate ectropion. At the same time, endothelial dysfunction is characterized by a significant decrease in the number of cells, a change in their shape and the appearance of epithelioid cells with a hyper-reflective nucleus (the so-called “ICE (iridocorneal endothelial) cells”), with a violation of their natural pumping function. The article presents a clinical case of a patient who underwent a standard examination and symptomatic treatment after laser keratorefractive surgery for myopia correction. Low visual acuity, diffuse corneal edema, IOP decompensation of unclear genesis were observed in one operated eye. Conclusion. Keratorefractive operations can act as a trigger that stimulates the transition of the latent form into a clinically developed pathological process, which causes a decrease in corrected visual acuity due to the formation of corneal edema and glaucoma optic neuropathy.

Ex-PRESS shunt combined with ab-interno peripheral iridectomy: A new surgical procedure for iridocorneal endothelial syndrome

PurposeIridocorneal 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. MethodsThree 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. ResultsNo 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 importanceEx-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.

Corneal Collagen Cross-Linking for Late Onset Progressive Keratoconus in Iridocorneal Endothelial Syndrome.

The aim of this study was to report the first case of successful use of corneal collagen cross-linking (CXL) to halt the progression of keratoconus in a patient with concurrent iridocorneal endothelial (ICE) syndrome. A 30-year-old White man was referred to our subspecialty corneal clinic for further investigation of right corectopia. The patient was asymptomatic and was otherwise fit. Slit-lamp examination revealed a right oval-shaped pupil decentered superiorly, a transillumination defect at the 5-o'clock position, minimal gutta-like changes in the corneal endothelium, and few inferior peripheral anterior synechiae. Dilated fundoscopy was normal in both eyes, with healthy optic discs, maculae, and peripheral retinae. Specular microscopy and slit-lamp findings suggested ICE syndrome. Subsequent investigation with corneal tomography showed progressive signs of inferior corneal ectasia with steepening, and thinning in the right eye, consistent with keratoconus. The left eye was unremarkable. Epithelium-off accelerated corneal CXL was performed in the affected eye. Early post-CXL follow-up (1 week) was unremarkable, and further follow-ups were arranged at 3, 6, 12, and 24 months, respectively. In subsequent reviews, the patient's vision and corneal tomography findings were stable. We describe the first case of corneal CXL for progressive keratoconus with ICE syndrome. co-existing keratoconus and ICE syndrome can occur, and corneal cross-linking was used successfully in this case to halt keratoconus progression. However, further studies will need to establish the impact of epithelium-off corneal cross-linking, especially in more severely affected eyes.

Clinical Outcomes of the Intraocular Lens Injector and Busin Glide for Descemet Stripping Automated Endothelial Keratoplasty in Patients with Iridocorneal Endothelial Syndrome.

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), in comparison with those using the Busin glide. Methods: In this retrospective, interventional comparative study, we evaluated the outcomes of DSAEK performed using the injector (n = 12) or the Busin glide (n = 12) for patients with ICE syndrome. Their graft position and postoperative complications were recorded. Their best-corrected visual acuity (BCVA) and endothelial cell loss (ECL) were monitored over a 12-month follow-up period. Results: DSAEK was conducted successfully in the 24 cases. The BCVA improved from the preoperative 0.99 ± 0.61 to 0.36 ± 0.35 at 12 months after operation (p < 0.001), with no significant difference between the two groups (the injector group and the Busin group) (p = 0.933). ECL at 1 month after DSAEK was 21.80 ± 15.01% in the injector group, which was significantly lower than 33.69 ± 9.75% of the Busin group (p = 0.031). No surgery-related complications were observed in the 24 cases intraoperatively or postoperatively except that one case suffered from postoperative graft dislocation, without statistical difference between the two groups. Conclusions: At 1 month after surgery, the use of graft injector for delivering DSAEK-based endothelial graft may cause significantly less endothelial cell damage than the pull-through technique used in the application of Busin glide. The injector allows safe endothelial graft delivery without the need of anterior chamber irrigation, which increases the ratio of successful graft attachment.

Penetrating canaloplasty in angle-closure glaucoma secondary to iridocorneal endothelial syndrome following multiple failed filtering surgeries: A case report

Angle-closure glaucoma secondary to iridocorneal endothelial syndrome (ICE) is challenging to treat, especially in patients who have already undergone multiple surgical procedures. Long-term success is difficult to achieve with traditional filtration surgery again. This case report describes a novel nonbleb-dependent surgery for managing such a young patient. A 30-year-old male with glaucoma secondary to ICE was referred to West China Hospital, Sichuan University for uncontrolled intraocular pressure following multiple failed filtering surgeries under maximum topical antiglaucoma medications in his right eye. The patient was diagnosed with angle-closure glaucoma secondary to ICE in the right eye based on a series of ophthalmic examinations. Penetrating canaloplasty was performed to manage glaucoma secondary to ICE in the right eye. The patient's visual acuity improved, the intraocular pressure was reduced to 11 to 15 mm Hg through 30 months of follow-up, and no antiglaucoma medication or additional surgical procedures were needed. Penetrating canaloplasty could be considered as an option for the treatment of refractory angle-closure glaucoma secondary to ICE with extensive angle adhesion.

Outcomes of Descemet stripping endothelial keratoplasty in cases of corneal endothelial dysfunction.

Automated microkeratome is commonly used to get donor lenticules for Descemet stripping endothelial keratoplasty (DSEK); however, manual dissection of donor lenticules is also being done with good outcomes. The aim of this study was to describe the results of manual DSEK performed in cases of corneal endothelial dysfunction caused due to pseudophakic bullous keratopathy, iridocorneal endothelial syndrome, and postpenetrating keratoplasty graft failure. This was a retrospective observational study. The medical records of all patients with corneal decompensation who underwent DSEK at a tertiary care center performed by the same surgeon were reviewed. A standard DSEK with manually dissected donor lenticules was performed in all cases with the exception of the Descemet membrane not being removed in two cases. A comprehensive ophthalmic examination was performed preoperatively and at each postoperative visit in all patients. Eight eyes of seven patients (four males and three females) were included. The mean age was 64.8 years (range, 49-74 years). The average follow-up was 10.9 months (range, 5-22 months). There was one case of primary graft failure which was managed by repeat DSEK. In the rest, corneas remained clear at the last follow-up. No rebubbling was done as none of the cases showed graft detachment. The preoperative best-corrected visual acuity (BCVA) was 20/2000 or less, and postoperatively, BCVA attained was 20/30 in four eyes and one eye achieved 20/80. Manual DSEK performed in eyes with corneal decompensation allowed rapid restoration of corneal clarity while minimizing intraoperative and postoperative complications.

Spokewise iridotomy combined with Descemet stripping automated endothelial keratoplasty in iridocorneal endothelial syndrome.

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. 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. 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). SI can help control intraocular pressure, improve visual acuity, and increase graft survival after DSAEK in ICE syndrome patients.

Clinical Characteristics of Fast and Slow Progressors of Glaucoma Secondary to Iridocorneal Endothelial Syndrome

Clinical Characteristics of Fast and Slow Progressors of Glaucoma Secondary to Iridocorneal Endothelial Syndrome

Iridocorneal Endothelial Syndrome – A Case Report

Iridocorneal endothelial (ICE) syndrome is a rare ophthalmic disorder, wherein the basic pathology is an abnormal corneal endothelium that leads to varying degrees of corneal oedema, iris atrophy, and secondary angle closure glaucoma.[1] This syndrome typically affects young women unilaterally with no family history.[2] The true aetiology of ICE syndrome is unclear. Viral cause for the disease has been proposed, based on a history of inflammation in certain cases and on the presence of inflammatory cells on histological analysis.[3] The abnormal endothelial cells may migrate posteriorly, forming a membrane that covers the adjacent structures, iris and trabecular meshwork.[4] The contraction of this membrane leads to characteristic iris changes, iridotrabecular synechiae, and corectopia with the pupil being drawn towards the area where the synechiae are most prevalent and to secondary angle-closure glaucoma.[5]

Rare case of iridocorneal endothelial syndrome with secondary glaucoma

A rare case of 40 years old female, came with complaints of pain and blurring of vision in the left eye for the past week. The patient had a diminution of vision since birth in her left eye. On examination left eye vision was found to be 6/60 not improving with pinhole, also defective color vision 20/25. The right-eye vision was found to be normal. The left eye showed significant corneal edema, and moderate anterior chamber depth, with iris features of Corectopia, Pseudopolycoria, and iris atrophy. Pressures were found to be 30mm hg in the left eye, gonioscopy revealed high peripheral anterior synechiae extending anteriorly above Schwalbe’s line. Left eye fundoscopy was done and it showed increased CDR of 0.8 to 0.9 following which visual field analysis was done, it showed inferior field defect. The patient was started on e/d timolol and hypertonic saline for a week. The patient was reviewed after a week showing reduced pressures of 22mmg in the left eye with reduced corneal edema and pain. Diagnosis of iridocorneal endothelial syndrome-essential iris atrophy type with secondary glaucoma was made and now the patient is being followed up regularly. An iridocorneal endothelial syndrome is a group of disorders with three clinical variants as Chandler syndrome, Essential iris atrophy, and Cogan Reese syndrome. Pathology is the presence of abnormal endothelial cells, which behave as epithelial cells, able to proliferate and migrate. The iridocorneal endothelial syndrome is frequently associated with secondary glaucoma.

Surgical Outcomes of Glaucoma Drainage Device Implantation in Refractory Glaucoma Patients in Thailand.

To study the surgical outcomes of glaucoma drainage device (GDD) implantation in refractory glaucoma patients. Retrospective chart review of glaucoma patients undergoing GDD implantation, Ahmed glaucoma valve (AGV), Baerveldt glaucoma implants (BGI), and Aurolab aqueous drainage implantation (AADI) from January 2012 to June 2021. Glaucoma patients were classified into two groups: primary glaucoma including: primary open angle glaucoma (POAG), primary angle closure glaucoma (PACG) and juvenile open angle glaucoma (JOAG) and secondary glaucoma including: neovascular glaucoma (NVG), ocular surgery (vitreoretinal surgery, scleral buckling procedure, postoperative extra capsular cataract extraction, scleral fixation intraocular lens, penetrating keratoplasty), intraocular trauma, uveitis glaucoma, lens-induced glaucoma, pseudoexfoliation glaucoma (PXG), iridocorneal endothelial (ICE) syndromes and Axenfeld Rieger Syndrome. Surgical outcomes were studied. Primary glaucoma included 57 eyes from 49 patients. Secondary glaucoma included 87 eyes from 85 patients. The cumulative probability of complete or qualified success of refractory glaucoma patients at five years were 53.4% (95%CI: 38.4%, 66.3%). Higher success rate was discovered in primary glaucoma at 65.8% (95%CI: 38.4%, 83.3%) than 45.2% (95%CI: 26.9%, 61.9%) in secondary glaucoma group significantly with p=0.003. While the results among success rate, adverse events and complications was not different between types of GDD. Predictors for failure were neovascular glaucoma with unadjusted hazard ratio (HR) 3.62 (95%CI: 1.45, 9.04) with p=0.006, and lens-induced glaucoma with adjusted HR 4.19 (95%CI: 1.10, 15.86) with p=0.035 in multivariable analysis. Tube malposition and occlusion were the most frequent adverse events at 11.11%, corneal decompensation at 5.5%, hypotony at 2% in the nonvalved group, and endophthalmitis at 0.69%. Surgical success in refractory primary glaucoma was superior to secondary glaucoma with no difference between nonvalved and valved GDD implantation. Lens-induced glaucoma was a strong predictor for failure in GDD implantation.