Comparison of Descemet Membrane Endothelial Keratoplasty for Iridocorneal Endothelial Syndrome and Fuchs Endothelial Dystrophy.

PURPOSE: To evaluate the clinical outcomes of Descemet membrane endothelial keratoplasty (DMEK) for treating eyes with iridocorneal endothelial (ICE) syndrome and comparing the outcomes with those achieved after treating Fuchs endothelial dystrophy (FED). DESIGN: Prospective interventional comparative case series. METHODS: Sixty-three patients (68 eyes) with ICE syndrome or FED were enrolled at the Zhongshan Ophthalmic Center between March 10, 2014 and May 11, 2018. Eligible patients were divided into 2 groups: ICE group (eyes, 24; patients, 24) and FED group (eyes, 44; patients, 39). DMEK was performed in all cases. Corrected distance visual acuity (CDVA), endothelial cell loss (ECL), intraocular pressure (IOP), graft survival, and surgical complications were documented. RESULTS: In the ICE and FED groups, the mean follow-up duration was 24.9 ± 5 months and 25.2 ± 7.7 months, respectively. At 1 year postoperatively, Kaplan-Meier survival analysis demonstrated 85.7% and 100% cumulative graft success rates (P = .017) in patients with ICE and FED, respectively. Postoperative CDVA level was comparable between the 2 groups through 12-month follow-up; thereafter CDVA was better in the FED group than in the ICE group (P < .001). Moreover, postoperative ECL was significantly higher in the ICE group than in the FED group throughout the follow-up period (P < .001). A discernable increase in structural abnormalities of the anterior chamber angle was observed in 2 eyes (8.3%) in the ICE group; however, no significant differences were observed in the incidence of principal complications between the 2 groups. CONCLUSIONS: DMEK provides comparable short-term visual outcomes in the treatment of ICE to those observed in the treatment of FED, with higher postoperative ECL over FED.

Cogan-Reese syndrome with Iris cyst: A novel presentation.

PURPOSE: To report an atypical case of Cogan-Reese syndrome associated with iris cyst in a young adult male. METHODS: Slit-lamp biomicroscopic examination, swept-source anterior segment optical coherence tomography (ASOCT) and ultrasound bio-microscopy (UBM) were done to evaluate and characterize the nature of the iris cyst. Gonioscopy, specular microscopy and confocal microscopy were attempted, but unsuccessful due to the large corneal opacity. RESULTS: On slit-lamp biomicroscopy, a large nasal corneal opacity with overlying band-shaped keratopathy was noted, with history suggestive of a trivial non-penetrating trauma and likely healed corneal ulcer. Through the temporal clear cornea, the iris displayed altered pattern with overlying shiny membrane and multiple, small, discrete, hyperpigmented, irregular nodules suggestive of Cogan-Reese syndrome. On the nasal side, an iris cyst with typical 'stuck-on appearance' onto the endothelium was visible. ASOCT and UBM failed to show any evidence of epithelial downgrowth or Descemet membrane disintegrity, ruling out the possibility of a post-traumatic implantation iris cyst. CONCLUSION: The occurrence of iris cyst in this case of Cogan-Reese syndrome is unique, and could be related to the disease pathogenesis, or a rare co-incidental finding.

Descemet membrane endothelial keratoplasty in iridocorneal endothelial syndrome and posterior polymorphous corneal dystrophy.

OBJECTIVE: To report the clinical outcome of Descemet membrane endothelial keratoplasty (DMEK) in cases of corneal decompensation secondary to iridocorneal endothelial syndrome (ICE) or posterior polymorphous corneal dystrophy (PPCD). DESIGN: Retrospective interventional case series. PARTICIPANTS: Eight eyes of 7 patients that underwent DMEK due to corneal decompensation secondary to either ICE syndrome or PPCD, and had at least 6 months of postoperative follow-up. METHODS: Data were collected on best corrected visual acuity (BCVA), graft attachment and survival, endothelial cell density (ECD), and intraocular pressure (IOP). BCVA change, ECD loss, and IOP elevations were analyzed. RESULTS: Patients' age was 51.5 ± 13.3years. Four eyes (4 patients) had ICE syndrome and 4 eyes (3 patients) had PPCD. All procedures were uneventful. Follow-up time was 11.3 ± 7.6 months (range 6-24 months). DMEK was combined with goniosynechiolysis in 3 eyes and iridoplasty in 1 eye. BCVA improved in all eyes. Mean BCVA improved from 0.70 ± 0.34 logMAR (Snellen equivalent ∼20/100; range 20/50-20/400) preoperatively to 0.21 ± 0.14 logMAR (Snellen equivalent ∼20/34; range 20/20-20/40) at the final follow-up (p = 0.008). Donor ECD was 2740 ± 193 cells/mm2 preoperatively and 1967 ± 277 cells/mm2 at 6 months after surgery (p = 0.010)-cell loss rate of 27.8%. There were no graft rejections and no graft failures. Postoperative IOP rise (steroid response) was seen in 2 eyes, and was managed successfully with topical medical treatment. There was no evidence of glaucoma progression in any of the cases. CONCLUSIONS: DMEK surgery was effective in treating corneal decompensation secondary to ICE syndrome and PPCD. Adjunct procedures can be simultaneously combined with DMEK to address other disease aspects.

Unique variations and characteristics of iridocorneal endothelial syndrome in China: a case series of 58 patients.

PURPOSE: Iridocorneal endothelial (ICE) syndrome is a rare condition, and unique characteristics in Chinese patients can make diagnosis difficult. Our purpose was to describe the clinical characteristics and variations of ICE syndrome in 58 consecutive Chinese patients. METHODS: The clinical data of consecutive patients with ICE syndrome who were seen between 2008 and 2011 at the glaucoma clinic of our ophthalmology department were retrospectively reviewed. The diagnostic criteria for ICE syndrome were a "hammered-silver" appearance of the corneal endothelium and specular microscopy showing ICE cells characterized by the absence of a hexagonal appearance, dark areas within the cells, and a light-dark reversal pattern. The general characteristics of the cornea, iris, pupil, and anterior chamber angles were compiled and examined. RESULTS: Fifty-eight patients with ICE syndrome were identified: 26 had Chandler's syndrome (CS), 23 Cogan-Reese syndrome, and nine progressive iris atrophy (PIA). The incidence of glaucoma was 98%. Twenty-three (39.7%) patients had atypical ICE syndrome. Intraocular pressure was relatively high in 13 patients with slightly damaged irises, while the corneal endothelium remained relatively intact. Slit lamp examination showed an intact iris in ten patients (17.2%), with massive deposition of black pigment in the anterior chamber angle. In the 25 patients in whom the anterior chamber angle was only partially closed, 80% of the anterior chamber angles were hyperpigmented. CONCLUSIONS: CS is the most common variation of ICE syndrome in Chinese patients, and PIA is the least common. A slight change or an intact iris under slit lamp examination is a characteristic of ICE syndrome.

Clinical outcome of Descemet stripping automated endothelial keratoplasty in 18 cases with iridocorneal endothelial syndrome.

PurposeTo evaluate the clinical outcome of Descemet stripping automated endothelial keratoplasty (DSAEK) in eyes with iridocorneal endothelial (ICE) syndrome.Patients and methodsA retrospective case series study was conducted. Eighteen consecutive Chinese patients with 20 DSAEK grafts were enrolled. Participants were evaluated by anterior segment optical coherence tomography and confocal microscopy. Postoperative complications, graft survival, endothelial cell counts, corneal thickness, and anterior chamber depth were analysed. A Log-rank test in a Kaplan-Meier analysis and a Cox proportional hazard regression were used to analyse potential risk factors of graft failure.ResultsThe mean follow-up duration was 19.0±8.6 months. The donors' endothelial cell density (ECD) (cells/mm2) values at 1, 3, 6, 12, 18, and 24 months were 3342.2±287.0, 1897.6±745.4, 1793.6±755.7, 1618.1±604.3, 1421.9±650.8, 1265.1±844.1, and 1148.2±1217.8, respectively. Eleven of the 20 grafts exhibited secondary graft failure, with a mean estimated graft survival of 23.4 months. Immediate postoperative complications (air bubble ventilation for elevated intraocular pressure or rebubbling for graft detachment) were more common in eyes exhibiting graft failure (P=0.040). Postkeratoplasty glaucoma surgery emerged as a risk factor of graft failure, with a hazard ratio of 5.174. Eyes with a poor prognosis showed statistically greater central corneal thickness at 1 month, greater graft thickness at 3 months, and a shallower anterior chamber at 6 and 12 months.ConclusionsThe long-term outcome of DSAEK in eyes with ICE syndrome is relatively poor. Immediate postoperative complications, postkeratoplasty glaucoma surgery, thicker corneal parameters, and a shallow anterior chamber were all associated with graft failure.

Iridocorneal Endothelial Syndrome Presenting With Large Diurnal Intraocular Pressure Fluctuation.

PURPOSE OF THE STUDY: The purpose of the study was to report a case of iridocorneal endothelial syndrome with an initial presentation of a large diurnal fluctuation of intraocular pressure (IOP) which peaked early in the morning. METHODS: A 31-year-old white man had transient blurry vision oculus sinister (OS) upon awakening in the morning for the past 2 months. The blurry vision improved within 1 to 2 hours. Clinical examination, diurnal IOP measurements, and specular microscopy were performed to investigate the cause of his transient blurry vision. RESULTS: At 6 AM, IOP OS was 38 mm Hg with corneal edema and visual acuity of 20/30. At 8 AM, IOP OS decreased to 25 mm Hg with clear cornea and visual acuity of 20/20. Diurnal IOP measurements revealed a large fluctuation OS (18 mm Hg). Specular microscopy revealed pleomorphism, polymegathism, light peripheral borders and light/dark reversal of the corneal endothelium OS. IOP oculus dexter remained within normal limits with clear cornea and stable visual acuity throughout the diurnal measurements. CONCLUSIONS: In patients with consistent daily episodes of transient blurry vision, clinicians should suspect a large diurnal IOP fluctuation with high peak IOP. If symptoms and signs are unilateral, iridocorneal endothelial syndrome should be included in the differential diagnosis and corneal specular microscopy should be obtained.

ICE-Syndrome: A Case Report of Implantation of a Microbypass Xen Gel Stent After DMEK Transplantation.

PURPOSE: Treatment of glaucoma eyes with iridocorneal endothelial syndrome is complex. Minimally invasive glaucoma surgery, such as one that implements a novel, microinvasive device, known as Xen gel stents, has shown promise in surgical glaucoma treatment and offers a new therapeutic option. METHODS: This is a case report. RESULTS: A successful implantation of Xen45 gel stent in a woman with secondary glaucoma due to unilateral iridocorneal endothelial syndrome after descement membrane endothelial keratoplasty operation, and the follow-up are presented. CONCLUSIONS: Implantation of Xen gel stents may be a promising option for minimally invasive glaucoma surgery in difficult situations, as low adverse effects, good postsurgery visual acuity and sufficient regulation of intraocular pressure can be seen.

Outcomes of Primary Trabeculectomy With Mitomycin-C in Glaucoma Secondary to Iridocorneal Endothelial Syndrome.

PURPOSE: To report the outcomes of primary trabeculectomy with mitomycin-C (MMC) in eyes with glaucoma secondary to iridocorneal endothelial (ICE) syndrome. METHODS: We included 16 eyes of 15 subjects with ICE syndrome who underwent primary trabeculectomy with MMC between 1991 and 2013. Surgical success was defined as complete when the intraocular pressure (IOP) was ≥5 and ≤21 mm Hg with no additional antiglaucoma medication (AGM) or surgery and as qualified if IOP was controlled with AGM. RESULTS: The median age (interquartile range) of subjects at the time of trabeculectomy was 41 years (37, 44 y) and the median follow-up period was 23 months (7, 79 mo). Postoperatively, the median IOP significantly reduced from 36 (26, 43) to 14 mm Hg (12, 17 mm Hg) (P<0.001) and median number of AGMs reduced from 3 (2, 4) to 0 (0, 0) (P<0.001). The percentage of complete success was 75% at 6 months, 64% at 12 months, 57% at 36 months, and 33% at 60 months. The percentage of qualified success was 94% at 6 months, 82% at 12 months, 71% at 36 months, and 60% at 60 months. Five eyes failed during the follow-up period. The mean (±SD) number of glaucoma surgeries per eye was 1.3±0.5. Eight eyes developed corneal edema at a median follow-up of 78.5 months and 4 eyes underwent keratoplasty. CONCLUSIONS: Primary trabeculectomy with MMC offers moderate surgical success in patients with ICE syndrome. Maintaining long-term IOP control and corneal clarity in these eyes is a big challenge.

Descemet Stripping Endothelial Keratoplasty in Iridocorneal Endothelial Syndrome: Postoperative Complications and Long-Term Outcomes.

PURPOSE: To determine the long-term outcomes of Descemet stripping endothelial keratoplasty (DSEK) in iridocorneal endothelial (ICE) syndrome. METHODS: Retrospective review of a consecutive series of 4 eyes of 4 patients with ICE syndrome treated by DSEK surgery at a single institution with follow-up between 2 and 7 years. RESULTS: Mean follow-up after initial DSEK surgery was 55 months (range, 24-95 months). One eye required 1 repeat DSEK, and 2 eyes required 2 repeat DSEKs for a total of 9 DSEK operations across the 4 eyes. There were no graft dislocations and no primary graft failures. Seven of 9 grafts achieved a visual acuity of 6/12 or better by 6 months. Mean endothelial cell loss at 3, 6, 12, and 24 months was 55 ± 14%, 78 ± 5%, 80 ± 6%, and 83 ± 9%, respectively. Long-term graft survival was poor with 7 of 9 grafts suffering late endothelial failure by a mean of 18 ± 7 months (range, 12-28 months). Mean graft survival on Kaplan-Meier analysis was 19 months (95% confidence interval, 14-24 months). CONCLUSIONS: DSEK has the potential to provide good short-term visual outcomes in eyes with ICE syndrome. However, long-term graft survival beyond 2 years is poor because of late endothelial failure. Patients with ICE considering DSEK surgery should be warned about the high probability of repeat surgery.

Graft failure and intraocular pressure control after keratoplasty in iridocorneal endothelial syndrome.

PURPOSE: To describe and compare graft survival and intraocular pressure (IOP) control after penetrating keratoplasty (PK) and Descemet stripping automated endothelial keratoplasty (DSAEK) in eyes with iridocorneal endothelial (ICE) syndrome. DESIGN: Retrospective case series. METHODS: Multicenter study conducted at the Singapore National Eye Centre (SNEC) and Price Vision Group. Twenty-nine consecutive eyes with ICE syndrome that underwent keratoplasty between 1991 and 2011 were identified from the SNEC transplant database and Price Vision Group patient database and the following data extracted: demographics, graft failure, IOP indices, and glaucoma treatment pre- and postkeratoplasty. The main outcome measures were graft failure and need for additional IOP-lowering treatment after keratoplasty. RESULTS: The mean follow-up duration was 7.0 ± 4.9 years in the PK group (n = 17) and 4.0 ± 2.6 years in the DSAEK group (n = 12). After a mean of 4.1 ± 3.1 years, 37.9% of grafts (11/29) failed: 7 PK compared to 4 DSAEK (P = .72). The graft failure rate was 50% in eyes with prekeratoplasty glaucoma surgery vs 31.6% in those without (P = .43). Additional glaucoma treatment was required in 37.9% of eyes (11/29): 41.2% of PK eyes and 50% of DSAEK eyes (P = .28) Eyes that had undergone glaucoma surgery before keratoplasty were less likely to require escalation of IOP-lowering therapy postkeratoplasty (9.1% vs 50%, P = .03). CONCLUSIONS: One-third of grafts failed after keratoplasty for ICE syndrome at a mean duration of 4 years and additional IOP-lowering treatment was required in 37.9%. Both PK and DSAEK had similar outcomes with regard to graft failure and IOP control.

[The irido-corneo-endothelial syndrome. The loss of the control of corneal endothelial cell cycle. A review]. / Le syndrome irido-cornéo-endothélial ou la perte du contrôle du cycle cellulaire de l'endothélium cornéen. Une revue.

The three major symptoms of the irido-corneo-endothelial syndrome are the alterations of the corneal endothelium and of the iris with a loss of the regulation of the cell cycle, and the progressive obstruction of the irido-corneal angle. This rare pathology attacks mainly young adult women. Most of the symptoms and complications originate from the excessive proliferation of the corneal endothelial cells accompanied by the evolution of their phenotype towards that of the epithelial cells. In normal conditions the corneal endothelial cells do not divide, they are blocked in the G1 stage of the cell cycle, mainly because of the action of the inhibitors of cyclin-dependent kinases. Still these cells retain a good capacity for proliferation, which can be induced by the down-regulation of the expression of the inhibitors of the cyclin-dependent kinases. This proliferative capacity declines with age and is also different according to the localization of the cells: it is more intense with those originating from the central area then in those from the peripheral area of the cornea. The age-related decline of the proliferative capacity is not due to the shortening of the telomers, but to the stress-induced accelerated senescence of the cells.

Management of detached graft donor by SF6 injection following Descemet stripping automated endothelial keratoplasty of an eye with iridocorneal endothelial syndrome and Ahmed glaucoma drainage tube.

A 65-year-old woman with iridocorneal endothelial syndrome and a history of Ahmed glaucoma drainage (AGD) tube implantation underwent Descemet stripping automated endothelial keratoplasty (DSAEK) in her right eye. During the procedure, filling the anterior chamber with air was quite difficult due to escape of air via the AGD tube and a complete air fill of the anterior chamber could only be managed after multiple attempts. On operation night, there was no air left in the anterior chamber. On postoperative day 1, graft detachment was determined by slit-lamp biomicroscopy. For rebubbling, sulfur hexafluoride (SF(6)) 20 % was injected into the anterior chamber. Two days later, there was still some SF(6) in the anterior chamber and the graft was completely attached. At postoperative week 2, visual acuity was 2/10. SF(6) use may be considered for DSAEK in cases of previous AGD tube implantation history due to its potential for longer duration in order to obtain a better tamponade with the bubble due to its expanding nature.

Descemet stripping automated endothelial keratoplasty combined with phacoemulsification in Chandler syndrome.

PURPOSE: To report a case of Descemet stripping automated endothelial keratoplasty (DSAEK) in combination with phacoemulsification and implantation of intraocular lens (IOL) (triple procedure) in a patient with Chandler syndrome. METHODS: A patient with visually significant corneal edema due to Chandler syndrome and cataract underwent DSAEK combined with phacoemulsification and IOL implantation (DSAEK triple procedure). RESULTS: One year postsurgery, the patient's uncorrected distance visual acuity improved from counting fingers to 20/63, and the corrected distance visual acuity to 20/32. The cornea was clear with absence of corneal edema. CONCLUSIONS: In the presented case, DSAEK triple procedure was an effective treatment for corneal edema secondary to Chandler syndrome in the presence of cataract.

A case of essential iris atrophy in a male patient.

Essential iris atrophy is a rare disease of abnormal proliferative corneal endothelium which usually presents with iris atrophy, corectopia, pseudopolycoria, corneal oedema and glaucoma. The disease occurs typically in one eye of a young to middle aged woman. The unusual feature in this case was that, the patient was a 36-year-old male presenting with iris atrophy, corneal oedema and uniocular glaucoma.