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.

'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.

Diseases of the corneal endothelium.

The corneal endothelial monolayer and associated Descemet's membrane (DM) complex is a unique structure that plays an essential role in corneal function. Endothelial cells are neural crest derived cells that rest on a special extracellular matrix and play a major role in maintaining stromal hydration within a narrow physiologic range necessary for clear vision. A number of diseases affect the endothelial cells and DM complex and can impair corneal function and vision. This review addresses different human corneal endothelial diseases characterized by loss of endothelial function including: Fuchs endothelial corneal dystrophy (FECD), posterior polymorphous corneal dystrophy (PPCD), congenital hereditary endothelial dystrophy (CHED), bullous keratopathy, iridocorneal endothelial (ICE) syndrome, post-traumatic fibrous downgrowth, glaucoma and diabetes mellitus.

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.

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.

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

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.

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.

XEN Gel Stent to Treat ICE Syndrome: 4 Cases.

PRéCIS:: This case series reports safe, effective implantation of XEN gel stents to treat iridocorneal endothelial (ICE) syndrome. The stents continue to function well and have not been occluded by membranes or peripheral anterior synechiae, but continued follow-up is necessary. PURPOSE: ICE syndrome-related glaucoma is often refractory to medical treatment, and traditional surgical treatment has lower success rates than typical for other types of glaucoma. We present a series of patients who were treated with XEN gel stent implantation. PATIENTS AND METHODS: Retrospective case series of 4 patients with ICE syndrome who underwent XEN with subconjunctival mitomycin C injection. RESULTS: Average preoperative intraocular pressure was 28.5 mm Hg on 3.8 glaucoma medications, and average postoperative intraocular pressure was 10.5 mm Hg on 1.0 medication. No patients required return to the operating room for additional procedures over an average of 6.9 months of follow-up. One patient had shallow anterior chamber that resolved with conservative management. Another had shallow anterior chamber that resolved with anterior chamber reformation with viscoelastic and developed nonappositional choroidal effusions that had resolved at most recent follow-up of 7 months after surgery. No XEN implants have been occluded by membrane formation or peripheral anterior synechiae. CONCLUSIONS: XEN is a safe and effective option for surgical management of ICE syndrome-related glaucoma. Further follow-up surveillance is necessary.

Atypical Presentation of Iridocorneal Endothelial Syndrome With Band Keratopathy but No Corneal Edema Managed With Descemet Membrane Endothelial Keratoplasty.

PURPOSE: To report an unusual presentation of iridocorneal endothelial (ICE) syndrome associated with band keratopathy and its management with ethylenediamine-tetraacetic acid (EDTA) chelation and Descemet membrane endothelial keratoplasty (DMEK). METHODS: A 57-year-old female patient presented with unilateral progressive painless visual impairment, corneal band keratopathy, and morphological corneal endothelial changes without corneal edema or any previous ophthalmic, medical, or family history. Routine specular and confocal microscopy imaging, as well as biomicroscopy, best-corrected visual acuity, and pachymetry measurements were performed before and after the surgical procedures. Histopathologic and immunohistochemical evaluations of the surgically excised diseased DM-endothelium were performed. RESULTS: Superficial epithelial keratectomy with EDTA chelation was performed. After an initial period of a few months of corneal clearance, the patient presented with recurrence of visually significant band keratopathy. After 1 year, she underwent retreatment with superficial epithelial keratectomy and EDTA chelation, followed by DMEK. Histopathologic and immunohistochemical analysis showed ICE syndrome. Two years after DMEK surgery, the cornea was still clear and band keratopathy had not recurred. CONCLUSIONS: To the best of our knowledge, this is the first case in the literature that reports the association of ICE syndrome with band keratopathy. As band keratopathy recurred shortly after EDTA chelation, endothelial keratoplasty (DMEK) may be indicated to successfully treat such cases.

The iridocorneal endothelial syndrome.

The iridocorneal endothelial syndrome represents a unique group of ocular pathologies (Chandler syndrome, progressive iris atrophy, and Cogan-Reese syndrome) characterized by the proliferation of corneal endothelial cells that migrate toward the iridocorneal angle and iris surface causing, to a degree varying according to the subtype, corneal edema and decompensation and secondary glaucoma, whether by obstructing the angle or producing peripheral anterior synechiae by contraction of the basement membrane of the migrating cells over the surface of the iris. A triggering factor, possibly viral, induces the corneal endothelial cells to proliferate and behave like epithelial cells. Diagnosis is made based on typical ocular findings on the cornea and iris. Iridocorneal endothelial syndrome is more frequent in young women, with unilateral involvement in most cases. In vivo confocal microscopy is an excellent diagnostic tool, especially in borderline presentations like early cases of Chandler syndrome, which affects the cornea predominantly. Typical clinical management consists of treating the corneal edema and decompensation, where endothelial keratoplasty techniques have replaced in many cases the need for a penetrating keratoplasty and treating the secondary glaucoma, which usually requires surgical intervention.

Glaucoma associated with iridocorneal endothelial syndrome in 203 Indian subjects.

PURPOSE: To report the demographic profile, clinical features, and prevalence of glaucoma and its management in patients with Iridocorneal endothelial (ICE) syndrome. METHODS: Retrospective review of 203 consecutive subjects with ICE syndrome at a tertiary eye care centre between January 1988 and June 2013. RESULTS: ICE syndrome was present in 223 eyes of 203 subjects, 124 (61%) were female and 79 (39%) were male. The median age at presentation was 43 years (1st (Q1) and 3rd (Q3) quartile; 34, 51 years). ICE syndrome was unilateral in 183 (90%) subjects, and bilateral in 20 (10%) subjects. The most common clinical variant was progressive iris atrophy (PIA, 115; 52% eyes), followed by Chandler syndrome (CS, 87; 39% eyes) and Cogan-Reese syndrome (CRS, 21; 9% eyes). Glaucoma was found in 156 eyes (70%) at presentation and the median (Q1, Q3) intraocular pressure in eyes with glaucoma was 24 (16, 38) mm Hg. Seven eyes developed glaucoma during the follow-up period, increasing the percentage of eyes with glaucoma to 73%. Intraocular pressure was managed medically in 81 eyes (50%) and the other 82 eyes (50%) required surgical intervention. Corneal edema was present in 124 eyes (56%) of which, 32 eyes (14%) required keratoplasty. CONCLUSIONS: In our study on ICE syndrome in Indian population, the presentation was predominantly uniocular and more common in middle aged women. Progressive iris atrophy was the most common clinical variant. ICE syndrome was associated with glaucoma in over 70% of the eyes and half of the eyes had corneal edema.

Surgical outcomes of patients with iridocorneal endothelial syndrome: a case series.

The purpose of this study is to report the intermediate-term surgical outcomes of patients with iridocorneal endothelial syndrome-related glaucoma. The medical records of four patients (five eyes) surgery (Ahmed glaucoma valve implantation surgery and EX-PRESS mini shunt) were retrospectively reviewed. Median follow-up after glaucoma surgery was 24 (15-36) months. The preoperative intraocular pressure was significantly reduced from a median of 33 (22.5-36) mmHg on a median of 4 (4-5) glaucoma medications to a median of 12 (10.5-14.5) mmHg on a median of 2 (0-2) medications at last follow-up after surgery (p = 0.043 for IOP and p = 0.042 for glaucoma medications). Median preoperative visual acuity [0.016 (0.008-0.1)] did not change significantly when compared to median visual acuity at last follow-up [0.016 (0.004-0.5)] (p = 0.59). Intraocular pressure control in patients with iridocorneal endothelial syndrome is challenging and may require multiple operations and revisions. Some modifications during glaucoma drainage implant surgery and use of EX-PRESS mini shunt in certain cases could offer an advantage in these patients.

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.

Cystoid macular edema associated with iridocorneal endothelial syndrome: a case report.

BACKGROUND: Iridocorneal endothelial (ICE) syndrome occurs mainly in young and middle-aged women and typically presents as a unilateral disease characterized by abnormalities of the iris and corneal endothelium. While the ICE syndrome is known to be associated with glaucoma and bullous keratopathy, to our knowledge, only two cases of ICE syndrome complicated with cystoid macular edema (CME) have been reported to date. In this paper, we report a case of ICE syndrome complicated with CME treated at our institution. CASE PRESENTATION: The subject was a 51-year-old woman. In October 2013, she was examined by a primary care physician for blurred vision in her left eye. Dyscoria and abnormality of the corneal endothelium were observed, and the patient was diagnosed with ICE syndrome. In November of the same year, she was referred to our institution with a decrease in visual acuity and CME, both in her left eye. At initial examination, her best corrected decimal visual acuity was 1.0 (Snellen equivalent: 20/20) in the right eye and 0.5 (20/40) in the left eye. Intraocular pressure was 12 mmHg in both eyes. She was diagnosed with Cogan-Reese syndrome based on marked ectropion uveae, peripheral anterior synechia, and abnormalities of the corneal endothelium. Marked CME was observed on ophthalmoscopy and optical coherence tomography. A topical non-steroidal anti-inflammatory drug (nepafenac 0.1 %) was applied to the left eye four times daily from January 2014. Four weeks later, the CME had resolved and her visual acuity was 1.0 (20/20). CONCLUSION: While non-steroidal anti-inflammatory drugs and steroids did not appear to be effective in two previously reported cases of ICE syndrome complicated with CME, topical nepafenac was effective in this case. However, more such cases are needed before concluding that topical nepafenac is effective in this situation.

Iridocorneal Endothelial Syndrome in a 14-Year-Old Male.

The iridocorneal endothelial syndrome is a rare cause of unilateral glaucoma that is almost never seen in children. We report a case of iridocorneal endothelial in a 14-year-old boy who did not yet have ocular hypertension or glaucoma.