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.

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.

'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.

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).

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.

Clinical and Specular microscopy characteristics and corelation in Iridocorneal endothelial syndrome without corneal edema.

PURPOSE: To investigate endothelial imaging patterns in ICE syndrome and correlate these with the observed clinical features in the affected eye. METHODS: Of the 70 patients of ICE syndrome referred from the glaucoma clinic between 2017 and 18, 17 patients had a clear cornea for reliable endothelial imaging were included in the study. RESULTS: Mean age was 47(range 29-63) years; 9 males and 8 females. The right eye was involved in 10 and left eye in 7 patients. Mean best corrected visual acuity was 20/30 (20/20-20/80) in the affected eye. All 17 patients had clear and compact central cornea and reasonably good vision at the time of specular microscopy. Those with best corrected visual acuity <20/20 had cataract as a co-morbidity. Endothelial abnormalities were noted in all patients and were documented using Hirst and modified Sherrard's classification system. As per the modified Sherrard's classification system, 9 eyes were categorised into total ICE, 5 eyes into subtotal ICE, in 3 eyes grading could not be applied. Of the 4 subtotal ICE, 1 was subtotal ICE (+) and 3 were subtotal ICE (-). 12/17 patients had glaucomatous disc. CONCLUSIONS: Specular microscopy provides information on the various morphological pattern of endothelial abnormalities and helps in mapping out the areas with abnormal/diseased endothelium. These have implications in the management of ICE syndrome.

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.

Atypical Case of Bilateral Chandler Syndrome With Recurrent Band Keratopathy.

PURPOSE: To report a unique case of bilateral Chandler syndrome with recurrent band keratopathy. METHODS: This is a retrospective observational case report. RESULTS: A 39-year-old Asian man presented with progressive painless diminution of vision in both eyes for 6 years. Examination revealed diffuse corneal edema, hammered silver appearance of endothelium with guttae-like lesions, and corectopia in the right eye and mild corneal edema, central band keratopathy, and guttae-like lesions on the endothelium and peripheral anterior synechiae in the left eye. Routine specular microscopy, confocal microscopy, and pachymetry were performed. A clinical diagnosis of bilateral Chandler syndrome with band keratopathy was made. Superficial epithelial keratectomy with ethylenediaminetetraacetic acid (EDTA) chelation was performed in the left eye first, followed by Descemet-stripping automated endothelial keratoplasty in the right eye. Histopathological examination of the surgically excised Descemet membrane in the right eye showed multilayered endothelium with adhered epithelial cells consistent with Chandler syndrome. At 9-month follow-up, the right eye showed a clear cornea with an attached graft and the left eye revealed recurrence of central band keratopathy for which repeat EDTA chelation was successfully performed. CONCLUSIONS: Recurrent band keratopathy coincident with endothelial dysfunction in iridocorneal endothelial syndrome can be repeatedly treated with EDTA chelation, whereas endothelial keratoplasty might be delayed until the time point of corneal decompensation.

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.

Atypical variant of Fuchs' endothelial dystrophy mimicking iridocorneal endothelial syndrome: A case report.

To report an unusual presentation of Fuch's endothelial dystrophy (FED) and its management. A 53-year-old male patient presented with unilateral progressive painless diminution of vision. Best-corrected visual acuity of the right and left eyes were 20/20 and 20/400, respectively. Slit lamp examination of the right eye was unremarkable, on left eye examination there was corneal edema, shallow anterior chamber, areas of 360 degrees iridocorneal touch with few synechiae on gonioscopy and age-related nuclear sclerosis. Pachymetry and specular microscopy imaging were performed before and after the surgical procedure. Ultrasound biomicroscopy (UBM) was performed preoperatively to rule out the presence of any angle anomalies. Combined procedure of Phacoemulsification with intraocular lens implantation and Descemet's membrane endothelial keratoplasty (DMEK) with peripheral iridectomy were performed. At 6-weeks follow-up, best-corrected visual acuity improved from 20/400 to 20/20. Slit lamp examination of the left eye showed clear DMEK graft with patent inferior peripheral iridectomy. Specular microscopy showed a cell count of 1761 cells/mm2. In a patient presenting with unilateral corneal edema, shallow anterior chamber depth, and iridocorneal adhesions, one needs to rule out the diagnosis of atypical variant of FED.

Bilateral Chandler's syndrome: Uncommon entity diagnosed by ultrasound biomicroscopy and confocal microscopy.

A 22­year­old female presented with bilateral, progressive diminution of vision. Slit­lamp examination revealed bilateral sectoral corneal edema. Gonioscopy showed broad­based peripheral anterior synechiae and a membrane obscuring angle structure in both the eyes. On ultrasound biomicroscopy (UBM), a membrane extending from corneal endothelium to anterior iris surface causing traction was seen. Confocal microscopy showed an "epithelium­like" transformation of the corneal endothelium. This case demonstrates a bilateral Chandler variant of the iridocorneal endothelial (ICE) syndrome where the diagnosis of Chandler's disease was confirmed by confocal microscopy, after the mechanism of secondary angle closure was demonstrated by the UBM.

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.

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.

Iridocorneal endothelial syndrome: Evaluation of patient demographics and endothelial morphology by in vivo confocal microscopy in an Indian cohort.

Purpose: To evaluate the patient demographics and morphological characteristics of corneal endothelium by in vivo confocal microscopy (IVCM), in patients with Iridocorneal Endothelial (ICE) Syndrome. Methods: In this retrospective observational series, IVCM acquired endothelial images of patients with ICE syndrome were evaluated. 'ICE cells' morphology was classified as "-" or "+" if they were larger or smaller than contralateral normal endothelium. It was correlated with patient demographics and clinical manifestations. Results: IVCM was performed on 41 eyes of 21 patients, with 13 males (62%) and 8 females (38%). The disease was unilateral in 19 (90.5%) and bilateral but asymmetric in two (9.5%) patients. Total ICE was seen in 91% eyes. Eighty percent patients (12 out of 15) with ICE-cells were males while 83.3% (5 out of 6) patients with ICE + cells were females. Mean age of patients with ICE- cell type and ICE + cell type was 45.8 ± 17.8 years and 40.3 ± 9.2 years respectively (P = 0.02). Both ICE - and ICE + eyes had similar incidence (33.3%) of corneal edema. ICE + eyes had more severe (grades 2/3) glaucoma (n = 5/6 eyes, 83.3%) compared to ICE - eyes (n = 8/15 eyes, 53.3%). Conclusion: A male preponderance, predilection of ICE - and + cell variants for male and female gender respectively, lack of association of the endothelial cell morphology with corneal edema, and apparent association of ICE + phenotype with more severe glaucoma occurring at a relatively younger age, are some novel findings of the present study. In the clinical setting correlation of patient demographics with these IVCM findings may help in better long-term prognostication of eyes with ICE syndrome.