Iridocorneal Endothelial Syndrome Research Articles

Glaucoma associated with iridocorneal endothelial syndrome in 203 Indian subjects

PurposeTo report the demographic profile, clinical features, and prevalence of glaucoma and its management in patients with Iridocorneal endothelial (ICE) syndrome.MethodsRetrospective review of 203 consecutive subjects with ICE syndrome at a tertiary eye care centre between January 1988 and June 2013.ResultsICE 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.ConclusionsIn 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.

The frequency of secondary glaucoma in patients with iridocorneal endothelial syndrome in correlation to the presence of uveal ectropion

Introduction/Objective. Iridocorneal endothelial (ICE) syndrome incudes 3 clinical forms: progressive iris atrophy, Chandler?s syndrome, and Cogan?Reese syndrome. It is characterized by various degrees of iris atrophy, corneal endothelial changes, uveal ectropion, corectopia, peripheral anterior synechiae (PAS) and secondary glaucoma. The aim of the study was to illustrate forms of ICE syndrome, determine frequency of secondary glaucoma with emphasis on cases with uveal ectropion, analyze response to medicament treatment and the need for surgical treatment in intraocular pressure (IOP) control. Methods. Patients underwent slit lamp examination, applanation tonometry, gonioscopy, ophthalmoscopy, Humphrey visual field testing and Heidelberg retina tomography. Patients were divided into two groups: group I, without uveal ectropion (22 patients) and group II, with uveal ectropion (14 patients). Results. A total of 36 patients were examined in a 10-year period. The average age was 38 years, male to female ratio 1:2. Secondary glaucoma was confirmed in 26 (72.2%) patients, out of which 12 (54.5%) in group I and 14 (100%) in group II. PAS were more frequent in group II. In group I, mean initial IOP was 37 mmHg, and after medicament treatment 26 mmHg. Secondary glaucoma was controlled in 50% and remaining 50% underwent surgical treatment. In group II, mean initial IOP was 49 mmHg, and after medicament treatment 32 mmHg. All 14 patients (100%) underwent surgical treatment in order to achieve IOP control. Conclusion. ICE syndrome is a rare, progressive disease, with high incidence of secondary glaucoma, which is more frequent in cases with uveal ectropion. In these cases, medicament treatment is not effective and trabeculectomy with antimetabolite application is necessary.

Iridocorneal endothelial syndrome: A case report

Iridocorneal endothelial (ICE) syndrome includes a group of rare ocular pathologies with unilateral involvement, frequently affecting young women. The disease complex includes essential iris atrophy, Chandler's syndrome, and Cogan–Reese syndrome. We present a case of ICE, in which there was a combination of features of essential iris atrophy and Cogan–Reese syndrome.

Encysted bleb following ahmed glaucoma valve surgery: A histopathological case report

A 27-year-old male patient, with family history of glaucoma was diagnosed to have iridocorneal endothelial syndrome in the left eye. He was treated initially with topical medications and subsequently underwent trabeculectomy with the express implant. Postoperatively, the bleb was found to be well functioning for few months and later became flat and he was given subconjunctival (SC) 0.1 ml 5-Flourouracil (FU) injection with bleb needling. As the bleb did not form despite these measures, he underwent a second surgery with an Ahmed glaucoma valve (FP7) implantation. On review after 4 weeks, the patient had developed a large encysted bleb. Needling was done again and another dose of SC 5-FU 0.1 ml was given. The bleb became encysted again after 6 weeks, hence bleb wall excision was done, and the cyst wall was sent for histopathological analysis which revealed presence of loculated cysts and goblet cells but no inflammation. To the best of our knowledge, there have been very few histopathological case reports of encysted bleb published in peer-reviewed literature.

Unclassified Axenfeld-Rieger Syndrome: A CASE SERIES and Review of Literature

ABSTRACTPurpose: To report anterior segment features in unclassified anterior segment dysgenesis with overlapping features of Axenfeld-Rieger syndrome and other developmental anomalies. Methods: This retrospective study included those with atypical or overlapping features in one or both eyes, which were identified as unclassified ASD. Typical ARS was defined as the presence of posterior embryotoxon with or without iris changes like stromal hypoplasia, corectopia, polycoria, or ectropion uvea with or without systemic features. Cases of ARS with overlapping features with other ASD, like aniridia (complete or incomplete absence of iris), iridocorneal endothelial (ICE) syndrome (beaten metal appearance of corneal endothelium), Peters anomaly, isolated trabeculodysgenesis (evidenced by Haab’s striae, buphthalmos, and epiphora) in one or both eyes with other typical ARS features in the same or other eye were included and screened. Results: Of 56 cases of ARS seen over 10 years, a total of 17 eyes of 11 cases (M:F=9:2, unilateral n=3) with unclassified ASD were identified with a median age of patients of 28.45±17.75 years (range 6–30 years). All cases of unclassified ASD had the presence of focal atypical strands of non-progressive anterior synechiae extending from the iris mid-periphery to the cornea with no attachments to the Schwalbe’s line in any case. Adjacent keratic precipitates or pigment were present in three eyes with focal beaten metal appearance in one eye. Three patients developed repeated episodes of anterior uveitis in one eye with stromal involvement seen in all cases, which responded to antiviral therapy. Conclusions: Atypical features like focal strands with differential corneal involvement and onset of viral uveitis in unclassified ARS suggest a possible viral etiology during different periods of eye development.

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.

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

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

A review of the role of ultrasound biomicroscopy in glaucoma associated with rare diseases of the anterior segment.

Ultrasound biomicroscopy is a non-invasive imaging technique, which allows high-resolution evaluation of the anatomical features of the anterior segment of the eye regardless of optical media transparency. This technique provides diagnostically significant information in vivo for the cornea, anterior chamber, chamber angle, iris, posterior chamber, zonules, ciliary body, and lens, and is of great value in assessment of the mechanisms of glaucoma onset. The purpose of this paper is to review the use of ultrasound biomicroscopy in the diagnosis and management of rare diseases of the anterior segment such as mesodermal dysgenesis of the neural crest, iridocorneal endothelial syndrome, phakomatoses, and metabolic disorders.

İridokorneal Endotelyal Sendromlu Bir Olguda Korneanın Biyomekanik Özellikleri ve Korneal Dansitometri

Iridocorneal endothelial (ICE) syndrome is a rare disorder which includes iris nevus syndrome, Chandler syndrome and essential iris atrophy; and represents a spectrum of the similar disorder process predominantly affecting the corneal endothelium, anterior chamber angle and iris. ICE syndrome is often under-diagnosed especially in early stages when there is no apparent corneal edema and glaucoma. The accurate diagnosis is mainly based on clinical examination and in-vivo confocal microscopy by showing epithelium-like transformation of the corneal endothelium with irregularly shaped cells and hyperreflective nuclei. We hereby report corneal biomechanical properties and densitometry in a 49-year-old patient with ICE syndrome and discuss role of these parameters for the diagnosis and follow-up of ICE syndrome.

Trabeculectomy with Mitomycin-C in Patients with Iridocorneal Endothelial Syndrome: A Case Series.

Iridocorneal Endothelial syndrome (ICE) is a rare ocular disorder characterized by abnormal endothelization of angle structure and iris producing characteristic manifestations including secondary angle closure glaucoma. We describe the clinical course of three patients with secondary glaucoma due to ICE syndrome who underwent trabeculectomy with mitomycin-C. At last follow-up, all patients had controlled IOP (<18 mm Hg) off drug. Early postoperative period was accompanied by shallowing of anterior chamber, diffuse vascularisation of bleb, frequent episodes of high IOP which were managed successfully. Trabeculectomy with MMC offers a good treatment choice for the management of secondary glaucoma associated with ICE syndrome.

Features of Progress and Treatment of Postprimary Refractory Glaucoma in a Patient with Progressive Mesodermal Iris Atrophy

The problem of degenerative diseases of the vascular tract of the anterior eye`s segment is one of the insufficiently explored in ophthalmology. The relevance of the problem lies in the tactical difficulties in treatment of patients with atypical current secondary glaucoma, that develops as a result of degenerative changes of the eye`s anterior segment. First of all this refers to diseases combined by term thе iridocorneal endothelial syndrome. On the foot of the syndrome is the proliferation of pathologically altered corneal endothelium and its migration as a membrane on the trabecular area of the anterior chamber angle and anterior surface of the iris. This leads to development of secondary angle-closure glaucoma. Purpose. To optimize the approaches to the treatment of secondary decompensated refractory glaucoma on the basis of the clinical case analysis. Patient and methods. A case of bilateral progressive mezodermal atrophy of the iris and the cornea on 49 years old patient, who appealed to the Kirov clinical Ophthalmology hospital in February 2015 year with repeatedly operated decompensated refractory glaucoma. The local status of the patient included: thinning of the front layer of iris, sometimes up to full atrophy with non-through defects and deformation of the pupil, precipitates and spots on the endothelium of the cornea, the anterior peripheral adhesions, membrane-like tissue on the trabecula and the front surface of the iris, glaucoma excavation of the optic disc. Results and discussion. A rare form of corneal endothelial syndrome: essential progressive atrophy of mezodermal of the iris and the cornea has been diagnosed. Pharmacological correction of the intraocular pressure and original drainage surgery was performed. As a result, the patient’s IOP decreased from 38 mm to 16 mm Hg pressure. Conclusions. The unusual nature of secondary glaucoma, according with the progression of essential mezodermal atrophy of iris and cornea caused difficulties in diagnosing and treatment tactics. Correct diagnosis helped to choose the proper individual tactics of combined treatment that allowed to compensate glaucomatous process and save the visual functions for restore an active lifestyle of the patient.

Boston Type 1 Keratoprosthesis for Iridocorneal Endothelial Syndromes.

To evaluate the outcome of the Boston type 1 keratoprosthesis (KPro-1) in eyes with iridocorneal endothelial syndromes and failed keratoplasties. A retrospective review was performed of every eye with a history of iridocorneal endothelial syndrome and a failed corneal transplant that was treated with a KPro-1 at a tertiary eye care center between January 1, 2008, and July 1, 2014. The main outcome measures were visual outcome, prosthesis retention, and postoperative complications. Four eyes met the inclusion criteria. Two eyes had essential iris atrophy and 2 eyes had Chandler syndrome. All 4 eyes had failed corneal transplants and successful glaucoma drainage devices. The mean patient age at the time of KPro-1 surgery was 68.3 years (range, 60-80 years). The mean postoperative follow-up duration was 47 months (range, 27-69 months). Preoperatively, the best-corrected visual acuity (BCVA) was worse than 20/200 in all 4 eyes, including 2 eyes that had hand motions vision. After KPro-1, all 4 eyes initially obtained a BCVA of ≥20/70. At the most recent examination, the BCVA was still ≥20/100 in 3 eyes. The KPro-1 device was retained in all 4 eyes. Postoperative complications included glaucoma progression (1 eye), a retroprosthetic membrane (1 eye), and sterile vitritis (1 eye). The Boston KPro-1 may offer a better prognosis than repeat traditional keratoplasty in reestablishing corneal clarity in eyes with iridocorneal endothelial syndromes. Despite anatomic success, visual rehabilitation may be compromised by preexisting glaucomatous optic neuropathy and its postoperative progression.

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

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

Iridocorneal Endothelial Syndrome

The iridocorneal endothelial (ICE) syndrome is characterized by abnormalities of the corneal endothelium, iris, and anterior segment leading to secondary angle closure glaucoma. ICE syndrome is usually unilateral, nonheritable and most common in white women in the third to fifth decades.Journal of Nobel College of Medicine Vol.4(1) 2015: 64-65

Graft Failure and Intraocular Pressure Control After Keratoplasty in Iridocorneal Endothelial Syndrome

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

Diagnosis and Management of Iridocorneal Endothelial Syndrome.

The iridocorneal endothelial (ICE) syndrome is a rare ocular disorder that includes a group of conditions characterized by structural and proliferative abnormalities of the corneal endothelium, the anterior chamber angle, and the iris. Common clinical features include corneal edema, secondary glaucoma, iris atrophy, and pupillary anomalies, ranging from distortion to polycoria. The main subtypes of this syndrome are the progressive iris atrophy, the Cogan-Reese syndrome, and the Chandler syndrome. ICE syndrome is usually diagnosed in women in the adult age. Clinical history and complete eye examination including tonometry and gonioscopy are necessary to reach a diagnosis. Imaging techniques, such as in vivo confocal microscopy and ultrasound biomicroscopy, are used to confirm the diagnosis by revealing the presence of “ICE-cells” on the corneal endothelium and the structural changes of the anterior chamber angle. An early diagnosis is helpful to better manage the most challenging complications such as secondary glaucoma and corneal edema. Treatment of ICE-related glaucoma often requires glaucoma filtering surgery with antifibrotic agents and the use of glaucoma drainage implants should be considered early in the management of these patients. Visual impairment and pain associated with corneal edema can be successfully managed with endothelial keratoplasty.

Glaucoma Drainage Device Tube Retraction and Blockage in a Patient with Iridocorneal Endothelial Syndrome Treated With Nd:YAG Membranectomy

Purpose: To report on a case that demonstrates the successful treatment of tube blockage of the Ahmed Glaucoma Valve at its aqueous entry site by Nd:YAG laser membranectomy. Method: We report a case of refractory intraocular pressure elevation in a patient with iridocorneal endothelial syndrome after glaucoma drainage device due to blocked tube from the iridocorneal endothelial syndrome membrane. In July 2014, our patient presented with sudden right eye elevated intraocular pressure to 67 mmHg despite a well-controlled intraocular pressure in the range of teens with timolol alone over the last 2 years. Gonioscopy showed total synechial angle closure and increasing her medical treatment failed to control the intraocular pressure. Ahmed glaucoma valve was implanted successfully. However, recurrence of raised intraocular pressure from partial tube retraction and tube blockage at its aqueous entry site by an endothelial membrane was noted at 2 weeks after the surgery. Nd:YAG laser membranectomy was performed on 2 occasions to restore the patency of the tube shunt. After the second laser membranectomy, the patient’s intraocular pressure returned to, and remained at, normal level since. Conclusion: Tube occlusion by membrane is a well-known complication following glaucoma drainage device tube in patients with iridocorneal endothelial syndrome. Nd:YAG membranectomy is effective to restore the patency of tube lumen without subjecting patients to more invasive surgical interventions including tube extender or another glaucoma drainage device. However, more studies are required to ascertain the long term effect of laser membranectomy to a blocked tube, in comparison to other treatment modalities. To our knowledge, this is the first reported case of using laser membranectomy alone in treating Ahmed glaucoma valve tube obstruction due to a retracted tube blocked by endothelial membrane.

Loss of outer retinal neurons and circuitry alterations in the DBA/2J mouse.

The DBA/2J mouse line develops essential iris atrophy, pigment dispersion, and glaucomatous age-related changes, including an increase of IOP, optic nerve atrophy, and retinal ganglion cell (RGC) death. The aim of this study was to evaluate possible morphological changes in the outer retina of the DBA/2J mouse concomitant with disease progression and aging, based on the reduction of both the a- and b-waves and photopic flicker ERGs in this mouse line. Vertically sectioned DBA/2J mice retinas were evaluated at 3, 8, and 16 months of age using photoreceptor, horizontal, and bipolar cell markers. Sixteen-month-old C57BL/6 mice retinas were used as controls. The DBA/2J mice had outer retinal degeneration at all ages, with the most severe degeneration in the oldest retinas. At 3 months of age, the number of photoreceptor cells and the thickness of the OPL were reduced. In addition, there was a loss of horizontal and ON-bipolar cell processes. At 8 months of age, RGC degeneration occurred in patches, and in the outer retina overlying these patches, cone morphology was impaired with a reduction in size as well as loss of outer segments and growth of horizontal and bipolar cell processes into the outer nuclear layer. At 16 months of age, connectivity between photoreceptors and horizontal and bipolar cell processes overlying these patches was lost. Retinal degeneration in DBA/2J mice includes photoreceptor death, loss of bipolar and horizontal cell processes, and loss of synaptic contacts in an aging-dependent manner.

Evolving indications for Descemet's stripping automated endothelial keratoplasty.

Recent advances in the field of endothelial transplantation, including increasing acceptance of Descemet's membrane endothelial keratoplasty, may alter the indications for Descemet's stripping automated endothelial keratoplasty, to a procedure reserved for complex endothelial disorders. Recent literature demonstrates that Descemet's membrane endothelial keratoplasty provides better and faster visual outcomes and decreased immunologic rejection compared to Descemet's stripping automated endothelial keratoplasty. However, Descemet's membrane endothelial keratoplasty may be more challenging in the management of a number of more complex endothelial disorders. While the literature on complex Descemet's membrane endothelial keratoplasty is limited, the utility of Descemet's stripping automated endothelial keratoplasty has been validated in the management of endothelial dysfunction in the setting of a number of comorbid conditions including prior penetrating keratoplasty, prior glaucoma surgery, iridocorneal endothelial syndrome, aniridia, aphakia, and anterior chamber intraocular lenses, among others. The increasing adoption of Descemet's membrane endothelial keratoplast is changing the practice of endothelial keratoplasty. However, limitations of the Descemet's membrane endothelial keratoplasty procedure have also served to crystallize the essential role of Descemet's stripping automated endothelial keratoplasty in many complex endothelial keratoplasty scenarios. This article will review indications for endothelial keratoplasty, along with the current evidence for Descemet's stripping automated endothelial keratoplasty and Descemet's membrane endothelial keratoplasty in their management.

Concurrence of iridocorneal endothelial syndrome in a patient with glaucomatocyclitic crisis.

Concurrence of iridocorneal endothelial syndrome in a patient with glaucomatocyclitic crisis.