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Characteristics of Cogan-Reese syndrome. a Anterior segment image. b Bright membrane-like structure

Characteristics of Cogan-Reese syndrome. a Anterior segment image. b Bright membrane-like structure

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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 see...

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Article
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.
Article
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 IOP between January 2018 and April 2020. Patients were followed up at one week, month 1, 3, 6, 12 postoperatively, and semi-annually thereafter. Intraocular pressure (IOP), number of anti-glaucoma medication and surgery-related complications were recorded. Surgical success was defined as IOP ≥5 and ≤21mmHg without (complete success) or with/without (qualified success) IOP-lowering medications. 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<0.001) on 0.2±0.6 medications (P<0.001) at 12 months post-operation, respectively. Hyphema (37.9%), transient hypotony (34.5%) and transient post-operative IOP elevation (≥30mmHg, 17.9%) were the most commonly observed early complications at the one week and one month visits. From one 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 quite acceptable success in IOP control with few complications, providing a new option for the management of GS-ICE.
Article
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.
Article
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.
Article
We reported uveal effusion and transient myopia as the initial presentation of systemic lupus erythematosus with pulmonary arterial hypertension. Choroidal retinopathy is rare but extremely destructive to visual function. Therefore, prompt diagnosis and effective treatments will result in complete resolution of the uveal effusion and functional restoration of vision.