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.

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

PRCIS: Our study highlights the long-term success of trabeculectomy or Ahmed glaucoma valve (AGV) surgery in patients with glaucoma secondary to iridocorneal endothelial (ICE) syndrome. However, many ICE syndrome cases may need multiple glaucoma surgeries to achieve controlled intraocular pressure (IOP), with/without concomitant corneal graft surgery. OBJECTIVE: To evaluate the long-term outcome of trabeculectomy and AGV implantation in ICE syndrome. METHODS: Patients with glaucoma secondary to ICE syndrome who underwent either trabeculectomy or AGV surgery with intraoperative adjunctive mitomycin-C 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 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 a 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. RESULTS: 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 = 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 analysis of variance, P = 0.44). The mean IOP decreased from 35.76 ± 6.36 mm Hg preoperatively to 16.00 ± 3.10 in group A and from 36.12 ± 8.11 mm Hg 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 analysis of variance, P = 0.81). Kaplan-Meier analysis shows complete success in 14 patients (48.3%), 11 patients (37.9%), and 7 patients (24.1%) at 6-month, 1-year, and 2-year follow-up, respectively. The cumulative success rate was 95% at 2 years follow-up for all patients. CONCLUSIONS: In 2-year follow-up, trabeculectomy or AGV significantly reduced the IOP in glaucoma patients secondary to ICE syndrome.

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.

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

PURPOSE: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

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

RATIONALE: 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. PATIENT CONCERNS: 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. DIAGNOSES: The patient was diagnosed with angle-closure glaucoma secondary to ICE in the right eye based on a series of ophthalmic examinations. INTERVENTIONS: Penetrating canaloplasty was performed to manage glaucoma secondary to ICE in the right eye. OUTCOMES: 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. LESSONS: Penetrating canaloplasty could be considered as an option for the treatment of refractory angle-closure glaucoma secondary to ICE with extensive angle adhesion.

'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.

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.

Achieving Inner Aqueous Drain in Glaucoma Secondary to Iridocorneal Endothelial Syndrome: One Year Results of Penetrating Canaloplasty.

PURPOSES: To report the efficacy of a bleb-independent penetrating canaloplasty in the management of glaucoma secondary to iridocorneal endothelial syndrome (GS-ICE). DESIGN: Prospective, non-comparative clinical study. METHODS: Penetrating canaloplasty was performed on 35 eyes from 35 patients with GS-ICE and medically uncontrolled intraocular pressure (IOP) between January 2018 and April 2020. Patients were followed up at 1 week, months 1, 3, 6, 12 postoperatively, and semi-annually thereafter. The IOP, anti-glaucoma medication, and surgery-related complications were recorded. Surgical success was defined as IOP ≥ 5 mmHg and ≤ 21 mmHg without (complete success) or with/without (qualified success) IOP-lowering medication. RESULTS: A total of 29 eyes (82.9%) had 360° catheterization and successfully received penetrating canaloplasty. Of these eyes, 24 (82.8%) achieved qualified success and 22 (75.9%) achieved qualified success at 12 months after surgery. The mean IOP decreased from 39.5 ± 11.8 mmHg on 2.9 ± 1.0 medications before surgery to 16.6 ± 5.3 mmHg (P < .001) on 0.2 ± 0.6 medications (P < .001) at 12 months postoperatively, respectively. Hyphema (37.9%), transient hypotony (34.5%), and transient postoperative IOP elevation (≥ 30 mmHg, 17.9%) were the most commonly observed early complications at the 1 week and 1 month visits. From 1 month and beyond, all treated eyes showed no obvious bleb at the operation quadrant. CONCLUSIONS: Penetrating canaloplasty rescued the inner aqueous outflow in ICE eyes and demonstrated acceptable success in IOP control with few complications, providing a new option for the management of GS-ICE.

[A case of atypical iris corneal endothelial syndrome].

Iridocorneal endothelial syndrome is a rare ophthalmic disease, most of which are unilateral and common in women. The rate of misdiagnosis and missed diagnosis is relatively high due to its various clinical manifestations. In this case, the patient presented uncontrollable high intraocular pressure, corneal edema leading to difficult observation of corneal endothelium morphology, and accompanied by a small amount of iris neovascularization. No clearly diagnosis was made before glaucoma surgery. Further examination was performed after corneal clearance, and the final diagnosis was iris corneal endothelial syndrome (Chandler syndrome).

Descemet Membrane Endothelial Keratoplasty and Goniosynechialysis in Iridocorneal Endothelial Syndrome: Surgical Perspective and Long-Term Outcomes.

PURPOSE: The purpose of this study was to report the long-term outcomes of Descemet membrane endothelial keratoplasty (DMEK) with goniosynechialysis in eyes with iridocorneal endothelial syndrome with an emphasis on the surgical technique. METHODS: This was a retrospective series of 5 eyes of 5 patients undergoing a DMEK procedure at a tertiary eye care institute by a single surgeon. Modifications in the standard surgical technique, including the release of peripheral anterior synechiae, peripheral descemetorhexis, the release of adherent membranes, maneuvers in the anterior chamber with or without cataract extraction were logged. Long-term outcomes of the surgery in these patients are reported. RESULTS: Five DMEK procedures were performed in 5 eyes of 5 patients. The average age of the patients was 48.2 ± 10.8 years, and the donor was 55.2 ± 3.2 years with a mean endothelial cell density (ECD) of 2784.6 ± 231 cells/mm 2 . After a mean follow-up period of 29.6 months (24-41 mo), all the 5 grafts remained clear; the mean BCVA at the last follow-up was 0.18 ± 0.17 logarithm of the minimum angle of resolution with a mean ECD of 1086.2 ± 338 cells/mm 2 with a 61.1% reduction in ECD. intra ocular pressure was normal in all the cases. None of the patients had graft detachments that required rebubbling, and all eyes had a clear graft at the last follow-up. CONCLUSIONS: DMEK can be performed successfully in a case with iridocorneal endothelial syndrome with good visual outcomes with modifications to the surgical technique, including peripheral anterior synechiae release and minimizing surgical manipulation.

[Clinical characteristics of 114 patients with iridocorneal endothelial syndrome].

Objective: To investigate the clinical features in patients with iridocorneal endothelial (ICE) syndrome. Methods: A retrospective case series study. Data of clinical manifestations of patients with ICE syndrome including clinical subtypes, presenting visual acuity, clinical features and secondary glaucoma were collected from January 2014 to May 2020 in the Eye Hospital, School of Ophthalmology and Optometry, Wenzhou Medical University. The Wald's Chi-square test of generalized estimating equations was performed to analyze the differences in three clinical subtypes. Results: A total of 127 eyes of 114 subjects (64 females and 50 males) were included. Mean±SD age at presentation was (49±13) years. There were 53 patients (46.5%) with Chandler's syndrome (CS), 36 patients (31.6%) with progressive iris atrophy (PIA), 24 patients (21.0%) with Cogan-Reese syndrome (CRS) and one patient (0.9%) with an undetermined subtype. And 101 patients (88.6%) had uniocular ICE syndrome. Approximately 81.7% (49/60), 56.1% (23/41) and 41.7% (10/24) of eyes presented visual acuity <0.3 in patients with CS, PIA and CRS, respectively. Corneal edema was most common in CS (52.5%, 32/61), followed by PIA (29.3%, 12/41) and CRS (20.8%, 5/24). Corectopia was found in 95.8% (23/24) of eyes with CRS, 95.1% (39/41) of eyes with PIA and 78.7% (48/61) of eyes with CS. Polycoria was observed in 29.3% (12/41) of eyes with PIA, 3.3% (2/61) of eyes with CS and 8.3% (2/24) of eyes with CRS. Ectropion uvea was most common in CRS (54.2%, 13/24), followed by 16.4% (10/61) in CS and 12.2% (5/41) in PIA. Glaucoma was found in 94 eyes (74.0%, 94/127). Among them, 60.7% (37/61) of CS, 80.5% (33/41) of PIA and 95.8% (23/24) of CRS had secondary glaucoma. The difference of presenting visual acuity, corneal edema, corectopia, polycoria, ectropion uveae and secondary glaucoma in three clinical subtypes all had statistical significance (Wald χ2=13.87, 10.77, 965.78, 11.45, 15.00, 222.04; all P<0.05). And 86.2% of eyes (81/94) had glaucoma surgeries and 41 eyes (43.6%, 41/94) had the intraocular pressure well controlled with various interventions. Conclusions: ICE syndrome is mostly uniocular and more common in middle-aged patients. CS is the most common clinical subtype with poor presenting visual acuity. About 3/4 of eyes have secondary glaucoma, and the majority of them require surgical interventions, but prognoses are discouraging.

Outcomes of Descemet-Stripping Endothelial Keratoplasty in 52 Eyes With Iridocorneal Endothelial Syndrome.

PURPOSE: To evaluate the long-term outcomes of Descemet-stripping endothelial keratoplasty (DSEK) in 52 eyes with iridocorneal endothelial (ICE) syndrome. METHODS: Retrospective study of 52 eyes of 52 patients who were diagnosed with ICE syndrome and underwent DSEK between January 2010 and December 2019 with a follow-up of at least 6 months. RESULTS: The mean age was 48.8 ± 10.8 years. Female patients (n = 33) constituted 63.5%. The median duration of follow-up was 2.4 years (range, 0.5-9.1 yrs). The mean best-corrected visual acuity improved significantly after surgery and remained stable (∼20/50) through 5 years. The mean endothelial cell loss was 28%, 37.9%, 43.6%, and 56.9% at 6 months and at 1, 2, and 3 years, respectively. Graft rejection was noted in 5 eyes (9.6%). Postoperatively, increased intraocular pressure (IOP) was seen in 17 eyes (32.7%), and 10 eyes (19.2%) underwent glaucoma surgeries. Fourteen eyes (26.9%) had secondary graft failures. The estimates of graft success were 93.6% ± 3.6% at 1 year, 85.6% ± 5.5% at 2 years, 79.3% ± 6.7% at 3 years, 69.0% ± 8.9% at 4 years, and 59.1% ± 11.9% at 5 years. Postoperative increased IOP was found to be the only significant (P = 0.05) risk factor (hazard ratio 8.92) associated with graft failure. The clinical variant of ICE syndrome did not seem to influence the graft survival (P = 0.68). CONCLUSIONS: In this study, DSEK had a success rate of ∼60% at 5 years. Increased IOP post-DSEK is a significant risk factor for graft failure. Graft survival is not affected by the clinical variant of ICE syndrome.