In Vivo Confocal Microscopy and Anterior Segment Optical Coherence Tomography Findings of Patients with Iridocorneal Endothelial Syndrome

This case report aims to present the findings of in vivo confocal microscopy (IVCM) and anterior segment optical coherence tomography (AS-OCT) in three patients with iridocorneal endothelial (ICE) syndrome. Three female patients 37, 50, and 57 years of age presented with complaints of unilateral visual impairment and elevated intraocular pressure (IOP). Biomicroscopy revealed unilateral pupil irregularities and anterior synechiae, and gonioscopy demonstrated synechiae in the iridocorneal angle. IOP was within normal limits with medical treatment in two patients, while one patient had an IOP of 44 mmHg despite maximal antiglaucomatous treatment. IVCM revealed large, polymorphic, and hyperreflective cells in the corneal endothelial layer of the affected eyes and normal corneal epithelium, stroma, and endothelium in the fellow eyes. AS-OCT findings were normal in healthy eyes, while the affected eye showed synechiae in the iridocorneal angle and a hyperreflective, thickened endothelial layer. The patient with refractory glaucoma underwent trabeculectomy surgery with 5-fluorouracil. In conclusion, IVCM and AS-OCT allow a detailed examination of endothelial cell abnormalities and iridocorneal membranes in ICE syndrome, which is characterized by unilateral pupil and iris irregularities and anterior synechiae mainly in women.

Iridocorneal Endothealial Syndrome. Case Reports.

AIM: Iridocorneal endothelial (ICE) syndrome is a rare disease characterized by abnormal proliferation and structural changes of the endothelium, obliteration of the iridocorneal angle, and anomalies of the iris. The consequence of these changes is secondary glaucoma and corneal decompensation. The etiology is unclear, and the syndrome more commonly affects middle-aged women. CASE REPORTS: In this article we present two different case studies of young patients diagnosed with ICE syndrome with complications. The first case report is about a young woman in whom surgical treatment of glaucoma and corneal edema was successful. On the other hand, the second report presents a complicated case of a 29-year-old patient whose treatment was not successful despite repeated interventions. CONCLUSION: This text highlights the complexity of ICE syndrome, the difficulty of its therapy and the importance of early diagnosis.

Two-Step Iridocorneal Endothelial Syndrome Management: Endocapsular Intraocular Lens and Artificial Iris Followed by Descemet's Stripping Automated Endothelial Keratoplasty.

A 54-year-old female presented with complaints of glare and progressive visual loss OS with a corrected distance visual acuity (CDVA) OS of 20/100. The patient had grade 1 corneal edema with a "beaten bronze" appearance on specularly reflected light, pseudopolycoria, and a nuclear sclerotic cataract. The diagnosis of nuclear cataract and progressive iris atrophy iridocorneal endothelial (ICE) syndrome was made, and the patient underwent uneventful phacoemulsification with capsular bag placement of an AcrySof SA60AT intraocular lens combined with pseudopolycoria repair using an endocapsular Model A REPER artificial iris. Six months later, the patient was submitted to a Descemet's stripping automated endothelial keratoplasty (DSAEK) procedure, and 6 months after that the CDVA was 20/32 with no corneal edema and normal intraocular pressure. This two-step surgical approach, combining phacoemulsification and endocapsular foldable iris prosthesis placement followed by DSAEK, may be considered a promising option to successfully treat progressive iris atrophy ICE syndrome patients.

Detection of Nocturnal Elevation in Intraocular Pressure Using a Home Tonometer in a Patient With Iridocorneal Endothelial Syndrome.

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.

Comparison of penetrating and endothelial keratoplasty in patients with iridocorneal endothelial syndrome: A registry study.

BACKGROUND: To compare graft survival of endothelial keratoplasty (EK) versus penetrating keratoplasty (PK) in patients with iridocorneal endothelial (ICE) syndrome and identify ocular features associated with graft survival. METHODS: Observational, prospective, cohort study. A total of 30 806 first grafts performed between 1985 and 2020 were identified through the Australian Corneal Graft Registry and included in this observational, prospective cohort study. A total of 196 eyes underwent a primary corneal graft for ICE syndrome. Kaplan-Meier graft survival plots and Chi-squared tests were performed to identify graft survival rates for EK and PK. A history of raised intraocular pressure (IOP) was also recorded and analysed. Graft survival of eyes with ICE syndrome were compared to that of other indications. RESULTS: Grafts performed for ICE syndrome increased to 0.8% of all cases during the 2005 to 2020 period compared with 0.5% between 1985 to 2004 (χ2 =9.35, p = 0.002). From 2010, EK surpassed PK as the preferred graft type. Survival of primary grafts in eyes with ICE syndrome was lower than for other indications (log-rank = 56.62, p < 0.001). Graft survival was higher following PK than Descemet stripping (automated) endothelial keratoplasty (DS(A)EK) (log-rank = 10.56, p = 0.001). Graft survival was higher in eyes without a history of raised IOP compared to those with a reported history of raised IOP (log-rank = 13.06, p < 0.001). CONCLUSIONS: ICE syndrome carries a poor prognosis for graft survival. DS(A)EK had a poorer prognosis than PK. A history of raised IOP is associated with higher risk of graft failure.

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

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.

Unilateral keratoconus associated with iridocorneal endothelial syndrome: Case report.

We report a rare association of unilateral keratoconus (KC) and iridocorneal endothelial syndrome in a 34-year-old female. Slit-lamp examination showed advanced KC with faint apical scarring in her right eye. The pupil was superiorly displaced with superior peripheral anterior synechia. Specular microscopy showed abnormal endothelium with low endothelial cell count in the right eye. Corneal topography confirmed the unilateral KC diagnosis. As the patient did not tolerate hard contact lens, penetrating keratoplasty with pupilloplasty was performed with excellent outcome.

Acquired ectropion uveae: A case series.

Acquired ectropion uveae (AEU) is a common sequela to various inflammatory and ischemic conditions, though it is not well recognized. There is a paucity of literature describing AEU. So here, we present five cases where we documented ectropion uveae following chronic inflammation. Patients with ectropion uveae following chronic inflammation and ischemia were retrospectively reviewed. Their medical records and clinical findings were analyzed. Five patients of various ages were found to have AEU, of which 1 was post trabeculectomy with phacoemulsification and posterior chamber intraocular lens, 1 following neovascular glaucoma (NVG), 1 following uveitic glaucoma, and 2 following iridocorneal endothelial syndrome. Patients with NVG and uveitic glaucoma also had undergone glaucoma filtration surgeries. AEU may be seen secondary to inflammatory and ischemic processes and should be looked for carefully as they may cause progressive glaucoma.

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.

'Progressive peripheral anterior synechiae in iridocorneoendothelial syndrome- a crawling disaster'.

Purpose: Iridocorneal endothelial (ICE) syndrome is well known to cause refractory glaucoma in young adults. Commonly acclaimed mechanism for trabeculectomy failure in these cases include accelerated subconjunctival fibrosis, abnormal endothelial proliferation, and closure of ostium. In the following article, we present a case of Iridocorneal endothelial syndrome that presented with refractory glaucoma after trabeculectomy due to rapidly progressive peripheral anterior synechiae causing angle closure and corneal decompensation that mandated a tailored surgical approach of management. Methods: This is a descriptive case report based on electronic medical records, patient observation, surgical intervention, and follow-ups. Case description: A thirty-eight-year-old-male presented to us with signs suggestive of iridocorneal endothelial syndrome with gonioscopy revealing peripheral anterior synechiae (PAS) over four clock-hours temporally. Uncontrolled intraocular pressure (IOP) despite maximal medical therapy mandated augmented trabeculectomy with anti-fibrotics. The bleb failed within 3 weeks of trabeculectomy, with evidence of progressive crawling PAS causing endothelial decompensation and raised IOP. He underwent Ahmed glaucoma valve (AGV) implant surgery with viscosynechiolysis and sectoral iridectomy under antiviral cover. This helped control IOP and retain corneal clarity, with no recurrence of PAS in the affected area. Conclusion: Progressive peripheral synechiae in ICE syndrome can cause early bleb failure and refractory glaucoma. Careful viscosynechiolysis and sectoral iridectomy alongside a second implant surgery can help salvage visual functions and preserve corneal clarity while preventing further progression of PAS in these eyes.

Outcomes of the Aurolab aqueous drainage implant and trabeculectomy with mitomycin C in iridocorneal endothelial syndrome.

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.

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

Iridocorneal Endothelial Syndrome: Case Report of Essential Progressive Iris Atrophy.

Background: Iridocorneal endothelial (ICE) syndrome is a group of ophthalmic disorders, first reported by Eagle and Yanoff in 1979, a disease characterized by abnormalities of the iris and the corneal endothelium, and mainly occurs unilaterally in young and middle-aged women, with no family history. ICE syndrome comprises a spectrum of three clinical variants: Progressive essential iris atrophy (corectopia, iris atrophy or iris hole), Chandler syndrome (corneal oedema with mild to absent iris change), and Cogan - Reese syndrome (nodular pigmented lesion of the iris). Objective: We are presenting this case because of its rarity, diagnostic intricacy and therapeutic challenge. Case report: We report in this study a case of Essential Progressive Iris Atrophy, an Iridocorneal Endothelial Syndrome variant in a 40 years old patient, female, complaining about the shape of the pupil in the left eye, as well as photophobia in the same side.In the first evaluation, we observed visual acuity of 1.0 in both eyes.Intraocular pressure was 14 mm Hg in the right eye and 12 mm Hg in the left eye. On the biomicroscope, we had a proper right eye finding, on the left eye Iris atrophy with deformity in the direction from 12 to 6 hours. We performed gonioscopy, an ultra sound (UBM), spectral microscopy, pachymetry, OCT and Octopus perimetry. Conclusion: We confirmed the diagnosis of essential iris atrophy based on the clinical findings, and in abnormalities in complementary exams. Nowadays, the patient is being followed in the Ophthalmology department at JZU Brcko District Bosnia and Herzegovina.

Iris Nevus (Cogan-Reese) Syndrome Presenting with Zonular Dehiscence during Cataract Extraction.

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.

Macular edema in Cogan-Reese syndrome.

PURPOSE: Iridocorneo-endothelial (ICE) syndrome is known as a rare spectrum causing glaucoma, corneal and iris damages. Retinal complications are uncommon. OBSERVATIONS: We report the case of a middle-aged woman suffering from a Cogan-Reese Syndrome (CRS) with refractory ocular hypertension (OHT) who presented a cystoid macular edema (CME) during follow up. CONCLUSIONS AND IMPORTANCE: We suspect the CME to be inflammatory linked to the pathophysiological hypotheses of the CRS. The CME was successfully treated with topical nonsteroidal anti-inflammatory drugs (NSAID). No consensus is available on its duration. A recurrence happened when treatment was stopped, its reintroduction was successful.

Posterior Polymorphous Corneal Dystrophy in a Patient with a Novel ZEB1 Gene Mutation.

Posterior polymorphous corneal dystrophy (PPCD), a rare, bilateral, autosomal-dominant, inherited corneal dystrophy, affects the Descemet membrane and corneal endothelium. We describe an unusual presentation of PPCD associated with a previously unknown genetic alteration in the ZEB1 gene. The proband is a 64-year-old woman diagnosed with keratoconus referred for a corneal endothelium study who presented endothelial lesions in both eyes suggestive of PPCD, corectopia and iridocorneal endothelial synechiae in the right eye and intrastromal segments in the left eye. The endothelial count was 825 in the right eye and 1361 in the left eye, with typical PPCD lesions visible under specular and confocal microscopy. In the next generation sequencing genetic analysis, a heterozygous c.1A > C (p.Met1Leu) mutation was found in the ZEB1 gene (TCF8). The PPCD3 subtype is associated with corneal ectasia, and both can appear due to a pathogenic mutation in the ZEB1 gene (OMIM #189909). However, our patient had a previously unreported mutation in the ZEB1 gene, which mediates the transition between cell lines and provides a pathogenic explanation for the epithelialisation of the corneal endothelium, a characteristic of PPCD.

Bilateral laser peripheral iridotomy in a co-occurrence of unilateral iridocorneal endothelial syndrome and chronic angle closure glaucoma.

PURPOSE: To report a case of unilateral Iridocorneal endothelial (ICE) syndrome- Progressive iris atrophy (PIA) with an overlapping chronic angle closure glaucoma (CACG) and to highlight the effect of bilateral Laser peripheral iridotomy (LPI) in such a co-occurrence. CASE DESCRIPTION: A patient presented to us with bilateral gradual painless progressive diminution of vision. Both eyes (BE) had a clear cornea, shallow peripheral anterior chamber depth, grade 2 nuclear sclerosis, raised intraocular pressure and glaucomatous optic neuropathy. In addition, the Left eye (LE) had an irregular anterior chamber, peripheral anterior synechiae (PAS) extending to cornea, patchy iris atrophy, subtle corectopia and a low endothelial cell count on specular microscopy. Indentation gonioscopy led to the diagnoses of CACG BE with ICE syndrome- PIA LE. LPI was performed bilaterally. On Anterior Segment Optical Coherence Tomography (ASOCT), there was evident widening of the angle away from PAS in the Right eye as well as in the LE with PIA post LPI. CONCLUSION: This is a unique case of unilateral PIA with an associated CACG in BE. It is the first case demonstrating the effect of bilateral LPI in such a case scenario. Though not indicated in ICE syndrome, LPI did show short term evidence of significant widening of the angle away from areas of PAS even in the eye with PIA having a limited high PAS and a concurrent primary (chronic) angle closure disease.

Ex-PRESS® implant as the first surgical option in the iridocorneal endothelial syndrome.

Our purpose is to report the intermediate-term results of the Ex-PRESS® implant as the first surgical option in patients with Iridocorneal Endothelial (ICE) Syndrome. We describe two patients diagnosed from ICE syndrome with medically uncontrolled glaucoma and finally treated with an Ex-PRESS® implant, associating a cataract surgery in one of them. Three years after surgery, intraocular pressure remains stable without any additional medical antiglaucomatous treatment, with a well-located implant surrounded by a diffuse filtering bleb and no sight-threatening adverse events have been documented. In addition, no progression of the disease has been registered. This report encourage the Ex-PRESS® implant to be considered the first surgical option in this patients as it allows a permeable ostium in spite of the progressive synechial angle closure typical of the ICE syndrome.