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.

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.

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.

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.

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.

Implante Ex-PRESS® como primera opción quirúrgica en el síndrome iridocorneo-endotelial / Ex-PRESS® implant as the first surgical option in the iridocorneal endothelial syndrome

Se plantea como objetivo dar a conocer los resultados a mediano plazo del implante Ex-PRESS® como primera opción quirúrgica en pacientes con síndrome iridocorneo-endotelial (ICE). Se incluye la descripción de dos mujeres con diagnóstico de síndrome ICE y glaucoma no controlado por medicación, tratadas con el implante Ex-PRESS® y con cirugía de catarata asociada en una de ellas. La presión intraocular se mantiene estable tres años después de la intervención sin necesidad de tratamiento médico adicional anti-glaucoma, con un implante bien colocado y rodeado por una ampolla de filtración difusa, sin eventos adversos documentados que pudieran suponer una amenaza para la vista, y sin un progreso registrado en el curso de la patología. El presente informe propone considerar los implantes Ex-PRESS® como primera opción quirúrgica, al posibilitar la existencia de un ostium permeable, a pesar del cierre progresivo del ángulo sinequial típico del síndrome ICE (AU)

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 (AU)

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.

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.

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.

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.

Cirugía combinada de catarata y queratoplastia lamelar endotelial en el síndrome iridocórneo endotelial / Combined cataract surgery and lamellar endothelial keratoplasty in iridocorneal endothelial síndrome

Reportamos el caso de una mujer de 59 años, que refería disminución de agudeza visual (AV) en el ojo izquierdo (OI). Mediante la exploración, se objetivó en dicho ojo una AV corregida de cuenta dedos a 30 cm, y en la lámpara de hendidura se observó la presencia de edema corneal moderado, con pliegues en la membrana de Descemet. También se apreciaron sinequias iridianas anteriores, atrofia de iris y corectopia. Se diagnosticó de síndrome iridocórneo endotelial (ICE). Se decidió un abordaje terapéutico quirúrgico mediante una cirugía combinada de catarata y queratoplastia endotelial automatizada con disección de la membrana de Descemet (DSAEK). No se reportaron complicaciones intraoperatorias. La recuperación anatómica y funcional fue exitosa, presentando una AV corregida al año de 0,8. Este resultado apoya la eficacia de la DSAEK en el síndrome ICE animando a la realización de más estudios que soporten igualmente su eficacia en este síndrome

The case is reported of a 59-year-old woman, who reported decreased visual acuity (VA) in the left eye (LE). On examination, a corrected VA of finger count/ 30cm was observed in LE. Corneal oedema and folds in the Descemet's membrane were observed using the slit lamp. Anterior synechiae, iris atrophy, and corectopia were also found. ridocorneal endothelial syndrome (ICE) was diagnosed. The treatment option was a combination of cataract surgery and automated endothelial keratoplasty with Descemet's membrane dissection (DSAEK). There were no complications during the operation, with a successful anatomical and functional recovery, with a VA of 0,8 being observed after one year. This result supports the efficacy of DSAEK in ICE syndrome, encouraging more studies to be carried out that should also support its efficacy in this syndrome

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.

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.