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.

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.

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.

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.

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.

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.

Outcomes of Descemet stripping endothelial keratoplasty combined with near total iridectomy in iridocorneal endothelial syndrome.

To report the outcomes of endothelial keratoplasty (EK) combined with near total iridectomy in the management of iridocorneal endothelial (ICE) syndrome with severely disorganised anterior segment. Three patients with ICE syndrome, who had a severely disorganised anterior segment underwent near total iridectomy, with/without cataract surgery, followed by EK at the same time. Mean age was 35 years. Prior to EK, the intraocular pressure (IOP) was in the range of 12-15 mm Hg. One patient (patient 2) had advanced disc damage prior to EK. Two eyes (patients 1 and 2) had a glaucoma drainage device, and in one, the IOP was controlled with two antiglaucoma medications. All grafts were clear, and IOP was well controlled till the last mean follow-up of 53 (range 30-72) months. The outcomes of EK with this surgical approach are favourable and should be considered in selective cases of ICE syndrome.

Surgical outcomes of Ahmed glaucoma valve implantation in patients with glaucoma secondary to iridocorneal endothelial syndrome.

OBJECTIVES: To evaluate the long-term outcome of patients with iridocorneal endothelial (ICE) syndrome who underwent Ahmed glaucoma valve implantation surgery for uncontrolled glaucoma. METHODS: Eighteen patients who suffered from unilateral ICE syndrome with uncontrolled glaucoma and subsequently underwent Ahmed aqueous shunt surgery at Zhongshan Ophthalmic Center between January 2008 and December 2016 were reviewed. Outcome measures included intraocular pressure (IOP), the use of glaucoma medications, visual acuity, further surgical interventions, and surgical complications. RESULTS: The mean IOP was reduced from 34.8 ± 10.6 mmHg on 3.6 ± 0.5 medications to 17.4 ± 4.9 mmHg (t = 6.791, P = 0.000) on 1.6 ± 1.1 medications (Z = -3.545, P = 0.000) at the last follow-up (42.0 ± 19.3 months). Five eyes (27.8%) achieved complete success, nine (50.0%) achieved qualified success, and the remaining four (22.2%) were considered failures. Survival was 94.4% at 1 year, 88.1% at 2 years, and 73.5% at 3 years. Four cases displayed a flat anterior chamber and were treated with a single anterior chamber reformation surgery with no recurrence. No other complications related to the glaucoma drainage implants occurred in this series. CONCLUSIONS: Ahmed glaucoma valve implantation appears to be a safe and effective method for treating glaucoma secondary to ICE syndrome. Postoperative shallow anterior chamber and hypotony may occur but responds well to the treatment. Early consideration may be given to aqueous shunt surgery in patients with glaucoma secondary to ICE syndrome when trabeculectomy fails.

Cornea thinning in two cases of ICE syndrome.

We present two patients with a new finding of symmetrical corneal thinning early in the clinical course of iridocorneal endothelial syndrome. Patient 1 was evaluated for uncontrolled angle closure glaucoma of the left eye (OS) status post laser peripheral iridotomy (LPI). After placement of an Ahmed glaucomatous valve and trabeculectomy with mitomycin C were performed, the patient was diagnosed with Chandler syndrome. The patient's pachymetry at the time of diagnosis revealed stable central corneal thickness (CCT) of 481 µm of the right eye (OD) (baseline 494 µm) and central cornea thinning with CCT of 407 µm OS (baseline 486 µm). Patient 2 was evaluated for ocular hypertension and Chandler syndrome OS was diagnosed. The patient had a good short-term response to LPI and ocular hypotensive medications. This patient was also found to have thinning of his affected cornea with CCT 523 µm OD and 476 µm OS.

Corneal thinning and cornea guttata in patients with mutations in TGFB2.

OBJECTIVE: Human genome-wide association studies and animal models suggest a role for TGFB2 in contributing to the corneal thickness phenotype. No specific mutations, however, have been reported in this gene that affect corneal thickness. We sought to determine if haploinsufficiency of TGFB2 in humans associated with Loeys-Dietz syndrome type 4 is associated with corneal thinning. DESIGN: Observational cohort study of families with Loeys-Dietz syndrome type 4, caused specifically by TGFB2 mutations, in a tertiary care setting. PARTICIPANTS: Three probands with pathogenic mutations in TGFB2 and family members underwent comprehensive ophthalmic examination. METHODS: Clinical assessment included Scheimpflug imaging, specular microscopy, and slit-lamp biomicroscopy. We measured visual acuity, axial length, refractive error, and central corneal thickness. RESULTS: Clinical evaluation of 2 probands identified corneal thinning and cornea guttata, despite a young age and distinct mutations in TGFB2 (c.905G>A, p.Arg302His; c.988C>A, p.Arg330Ser). In the third family, corneal thinning co-segregated with a TGFB2 mutation (c.1103G>A, p.Gly368Glu), although without apparent guttae. CONCLUSIONS: In this series, participants with TGFB2 mutations associated with Loeys-Dietz syndrome type 4 demonstrated decreased corneal thickness, and in 2 cases with splice site mutations, also demonstrated cornea guttata. The data demonstrate the importance of considering distinct phenotype-genotype correlations within this condition.

Outcomes of Ahmed Glaucoma Drainage Implant in Eyes With Glaucoma Secondary to Iridocorneal Endothelial Syndrome.

AIM: To evaluate the success of Ahmed glaucoma valve (AGV) implantation in patients with glaucoma secondary to iridocorneal endothelial (ICE) syndrome. MATERIALS AND METHODS: This retrospective study included 18 eyes of 18 patients with ICE syndrome and glaucoma who had undergone AGV implantation at our institute between January 2008 and April 2019. The primary outcome was the success of AGV, defined as intraocular pressure (IOP) ≥6 and ≤21 mm Hg with or without topical antiglaucoma medication (AGM). Failure was defined as IOP <6 or >21 mm Hg/need for oral AGM/need for additional glaucoma surgery or loss of light perception because of a surgical complication. Corneal clarity and graft survival in these eyes post-AGV were the secondary outcome measure. RESULTS: The median (interquartile range) follow-up was at 20.61 (3.9 to 57) months. Of the 18 eyes, 14 (77.8%) had adequate IOP control with or without AGMs. Postoperatively, the mean (±standard deviation) IOP significantly reduced from 29.7(±10) mm Hg to 16(±3.8) mm Hg and the number of AGMs reduced significantly from a mean of 3.6 (±1) to 1.7 (±0.9). The probability of AGV success was 92.3±7.4%, 66.1±11.0%, and 50.5%±17.3% at 1, 3, and 4 years, respectively. All 4 failures were because of uncontrolled IOP and needed either oral AGM or additional surgery for IOP control. At the last follow-up visit, 16 eyes had clear cornea and 2 eyes had pre-existing corneal scar (eccentric) that persisted after AGV implantation. CONCLUSION: AGV implant was reasonably successful for IOP control in glaucoma secondary to ICE syndrome with a few common but manageable corneal complications.

Graft Survival After Penetrating and Endothelial Keratoplasty in Iridocorneal Endothelial Syndrome.

PURPOSE: To compare the survival of a first penetrating keratoplasty (PK) or endothelial keratoplasty (EK) for iridocorneal endothelial (ICE) syndrome with transplant survival in Fuchs endothelial dystrophy (FED) and pseudophakic bullous keratopathy (PBK). METHODS: We compared graft survival of PK and EK for ICE syndrome for 2 time periods. We then compared graft survival in ICE syndrome with graft survival in FED and PBK. Kaplan-Meier estimates of graft survival up to 5 years posttransplant were calculated with 95% confidence intervals (CI), whereas comparisons between the groups were performed using the log-rank test. RESULTS: We included 86 first transplants for ICE syndrome. There was no difference in graft survival between the 58 PKs and the 28 EKs for up to 5 years after surgery (P = 0.717). For the period from 2009 to 2017, the 5-year graft survival rates for ICE syndrome were 64.3% (CI, 21.8%-88.0%) for the 16 PKs and 66.8% (CI, 41.8%-83.0%) for the 26 EKs (P = 0.469). Between 2009 and 2017, the 5-year survival rate for 42 grafts with ICE syndrome was 62.7% (CI, 39.6%-79.0%), which was lower than 75.9% (CI, 74.2%-77.4%) in 7058 transplants for FED but higher than 55.1% (CI, 52.0%-58.0%) in 3320 transplants for PBK, although the numbers of ICE transplants are too small to tell whether this difference was by chance. CONCLUSIONS: The results indicate no difference in graft survival between PK and EK for ICE syndrome. Graft survival in ICE syndrome is intermediate between that of FED and PBK.

Ex-PRESS mini-shunt implanted in a pregnant patient with iridocorneal endothelial syndrome.

INTRODUCTION: The failure rate of both filtration surgery and of aqueous shunt implantation is higher for iridocorneal endothelial syndrome than in other scenarios, due to the continuous proliferation of abnormal endothelial cells over the trabecular meshwork and the filtration area and also due to the more pronounced cicatrizing response shown by these young patients. We present the first case ever described in the literature of a pregnant patient with iridocorneal endothelial syndrome and uncontrolled ocular hypertension who was implanted an Ex-PRESS mini-shunt. CLINICAL CASE: A 35-year-old female presented with diminution of vision in the left eye for 2 months. She was 20 weeks pregnant. Her visual acuity was 20/20 in right eye and 20/25 in left eye, and intraocular pressure was 11 mmHg in right eye and 34 mmHg in left eye. Slit lamp biomicroscopic examination revealed no alterations in right eye, whereas left eye showed corectopia and uveal ectropion, stroma of iris' sectoral atrophy and moderate corneal epithelial edema. Gonioscopy showed some anterior iris synechiae in left eye. Fundus evaluation was normal. Based on clinical features and examination, the diagnosis of left eye iridocorneal endothelial syndrome with decompensated intraocular pressure was made. She was prescribed topical timolol (0.5%) and dorzolamide. As a result of uncontrolled intraocular pressure and the impossibility to prescribe other hypotensive treatment available due to her being pregnant, it was decided to perform surgery in left eye using an Ex-PRESS mini-shunt and Ologen®; 6 months post surgery, intraocular pressure was 9 mmHg with no need for hypotensive treatment. The cornea was transparent, and the patient maintained her left eye visual acuity. CONCLUSIONS: Ex-PRESS mini-shunt can be considered a surgical option for iridocorneal endothelial syndrome. Its composition allows the ostium to remain open and the device triggers a milder postoperative inflammatory response. In our particular case, taking into account that the subject was a young, phakic, pregnant woman, whose intraocular pressure had to be closely controlled and we had to ensure that her postoperative care included as few drugs and as few reoperations as possible, we thought that using this device was the most appropriate option.