Clinical Profile of Iridocorneal Endothelial Syndrome at a Tertiary Care Eye Centre

Irido-Corneal Endothelial (ICE) syndrome is an uncommon clinical entity which includes corneal endothelial damage, obstruction of anterior chamber angle, iris atrophy, and polycoria.ICE syndrome is a spectrum of clinical entities namely Progressive Iris Atrophy (PIA), Cogan Reese Syndrome (CRS), and Chandler Syndrome (CS). It is considered to be sporadic, usually unilateral and diagnosed in young adults, females and occasionally in children. Corneal decompensation and glaucoma are the commonest causes of vision loss in patients with ICE syndrome.Medical management of this condition is usually with topical antiglaucoma medication. In most cases medical therapy fails and requires Glaucoma Filtration surgery. Glaucoma drainage devices are the mainstay of treatment and many patients require keratoplasty. We wanted to study the clinical profile of ICE syndrome patients, its association with glaucoma in Indian population and compare the findings with those of previous studies. METHODSIn this retrospective study, data of 50 consecutive patients having ICE syndrome at a tertiary eye care centre was collected. RESULTSOut of 50 patients, 28 (56%) were females, aged 7 to 65 years (median - 40.72 years). 49 patients (98%) had unilateral presentation, and one had bilateral involvement (2%). Progressive Iris Atrophy (PIA) was seen in 20 (40%), Chandlers Syndrome (CS) in 20 (40%) and 10 eyes (20%) had Cogan-Reese Syndrome (CRS). 46 (92%) eyes had glaucoma. Mean Intra-Ocular Pressure (IOP) was 23.70 ± 11.7 mm of Hg. Mean AGM used was 2.44 ± 1.3. 44 (88%) eyes were diagnosed to have glaucoma at presentation, 1 (2%) eye was diagnosed with glaucoma during follow up. 20 (40%) eyes were managed medically for IOP control. 8 (16%) eyes had undergone previous glaucoma surgery. 20 (40%) eyes underwent glaucoma surgery for IOP control. 15 (30%) eyes had Trabeculectomy with Mitomycin C, 5 (10%) had Ahmed glaucoma valve. 4 (8%) eyes underwent diode cyclophotocoagulation. 6 (12%) eyes required more than 1 surgery for IOP control. 6 (12%) eyes underwent penetrating keratoplasty (PK) for corneal oedema. Mean period of follow up was 8.4 ± 6.4 months. There were no cases of sight threatening complications like suprachoroidal haemorrhage, blindness or endophthalmitis. CONCLUSIONSGlaucoma is strongly associated with ICE syndrome. ICE patients may require more than 1 surgery for IOP control. They are commonly associated with corneal complications which may require penetrating keratoplasty.

Clinical research status of iridocorneal endothelial syndrome / 国际眼科杂志(Guoji Yanke Zazhi)

@#Iridocorneal endothelial(ICE)syndrome presents a group of ocular pathologies mostly characterized by corneal endothelial abnormalities and iris atrophy. Iridocorneal endothelial syndrome is typically a unilateral and generally not involving genetic condition, frequently affecting women in early to middle adulthood. There is no treatment for absence of symptoms in early stages. When complicated with corneal edema and secondary glaucoma, treatment is mainly symptomatic. In this group of diseases, there are many similarities and some differences in their pathogenesis, clinical manifestations and treatment. So far no universal information on these features has been published. Recent clinical studies on iridocorneal endothelial syndrome are reviewed in this paper.

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.

Posterior keratoconus and iris atrophy: a fortuitous association? / Ceratocone posterior e atrofia de iris: uma associação incidental?

ABSTRACT The authors describe an unusual association between posterior keratoconus and iris atrophy, confirmed by a complete ocular evaluation, scheimpflug imaging and pachymetric curve. A hypothesis for concomitant findings is discussed.

RESUMO Os autores descrevem a rara associação entre ceratocone posterior e atrofia de íris, confirmada por avaliação oftalmológica completa, imagens de scheimpflug e curva paquimétrica. Sugere-se uma hipótese que explique a concomitância de ambas as alterações.

Progress of iridocorneal endothelial syndrome / 国际眼科杂志(Guoji Yanke Zazhi)

@#Iridocorneal endothelial syndrome(ICES)is a rare ocular disease characterized by abnormal structure and proliferation of the corneal endothelium, the anterior chamber angle, and the iris. Common clinical features include corneal edema, secondary glaucoma and iris atrophy. ICES often occurs in young women, and most of them are monocular. Its pathogenesis is still unclear, the symptoms are various, and the blindness rate is high. The disease is difficult to diagnose, and there is no ideal treatment. The purpose of this article is to review the literature on the characteristics, diagnosis and treatment of ICES in order to help the diagnosis and treatment of the disease.

Iridocorneal Endothelial Syndrome with Features of Posterior Polymorphous Corneal Dystrophy

PURPOSE: To report a case of iridocorneal endothelial syndrome, which overlapped with some of the features of posterior polymorphous corneal dystrophy. CASE SUMMARY: A 61-year-old female presented with tearing pain and blurred vision in her left eye, which was aggravated in the morning. The symptom started approximately 1 year prior to her visit. At the initial visit, the visual acuities were 1.0 in both eyes and the intraocular pressures were normal. On slit-lamp examination, a single pair of horizontal parallel lines was observed at the central corneal endothelial layer in the right eye. In contrast, multiple pairs of oblique parallel lines were observed in the left eye. The lines of the lesions were more prominent and wavier in the left eye than those of the right eye. The overlying cornea was clear, and the corneal thicknesses were in the normal range in both eyes. Using a gonioscopic examination, localized peripheral anterior synechiae were observed only in the left eye. The pupil and iris were normal in both eyes. On specular microscopic examination, the corneal endothelial cell size in the right eye increased and the corneal endothelial density decreased to 668 cells/mm². In the left eye, multiple abnormal endothelial cells with dark-light reversal were observed. In conclusion, the patient was subsequently diagnosed with iridocorneal syndrome, rather than posterior polymorphous corneal dystrophy. CONCLUSIONS: Posterior polymorphous corneal dystrophy and iridocorneal endothelial syndrome may present with many similarities. Therefore, in cases of uncertain diagnosis, an understanding of the clinical features is important for proper diagnosis.

Glaucoma neovascular y atrofia esencial de iris / Neovascular glaucoma and essential iris atrophy

Los glaucomas secundarios son de difícil manejo, independientemente de las causas. Entre ellos, el glaucoma neovascular representa un reto para los oftalmólogos por las causas que lo producen y la gran pérdida visual que provoca. De igual manera, el síndrome iridocorneoendotelial es una enfermedad poco frecuente. El glaucoma ocurre aproximadamente en el 50 por ciento de los casos en dependencia del síndrome que predomine, mucho más grave en la atrofia esencial del iris. Presentamos un caso clínico de una paciente femenina de 58 años de edad en quien aparecen en cada ojo, de manera independiente, estos dos tipos de glaucomas secundarios(AU)

Secondary glaucomas are always difficult to manage, no matter what their causes might be. Among them, neovascular glaucomas pose a challenge to ophthalmologists, due to the factors bringing them about and the great visual loss they produce. On the other hand, iridocorneal endothelial syndrome is an uncommon condition. Glaucoma develops in about 50 percent of the cases, depending on the prevailing syndrome, and is much more severe in essential iris atrophy. A clinical case is presented of a 58-year-old female patient with these two types of secondary glaucoma, one type in each eye(AU)

Síndrome Iridocorneana Endotelial: Relato de Caso da Variante de Chandler / Iridocorneal Endothelial Syndrome: Case Report of Chandler's Variant

Resumo O presente estudo tem por objetivo relatar um raro caso da variante de Chandler da Síndrome Iridocorneana Endotelial em uma paciente de 56 anos. Esta referia baixa acuidade visual em olho direito há 2 anos, acompanhada de fotofobia e prurido. Ao exame oftalmológico, no primeiro atendimento, apresentava em olho direito acuidade visual de conta dedos à 0,5 metro e se observava à biomicroscopia policoria, edema corneano com microcistos e hiperemia conjuntival. O olho esquerdo não apresentava alterações. A pressão intraocular era de 16mmHg no olho direito e 10mmHg no olho esquerdo. Iniciou-se tratamento tópico com dorzolamida, maleato de timolol e dexametasona, sendo então, alcançado o controle da pressão intraocular. Constatou-se no exame de microscopia especular corneana a presença de ice cells. Na biomicroscopia atual, apresenta, no olho acometido, edema corneano com opacidade central, policoria, cristalino não visível e fundo de olho indevassável. Diante da confirmação do diagnóstico de Síndrome de Chandler, pelo quadro clínico compatível e alterações nos exames complementares, a paciente está em acompanhamento no serviço de Oftalmologia do Hospital Federal Servidores do Estado.

Abstract We report in this study a case of Chandler's Syndrome, an Iridocorneal Endhotelial Syndrome variant in a 56 years old patient, female, complaining about low vision in the right side, as well as itching and photophobia in the same side. In the first evaluation, we observed visual acuity of counting fingers at 0,5 meters, corneal swelling with microcystus and conjunctival hyperemia. The left eye was normal. Intraocular pressure was 16mmHg in right eye and 10 mmHg in left eye. We initiated topic treatment with Dorzolamide, Timolol and Dexamethasone, with good control of intraocular pressure. We noticed in corneal specular microscopy the presence of ice cells. In current biomicroscopy it is remarkable the corneal swelling, with central opacity and lens was not visible, as well as the fundoscopy is impossible. We confirmed the diagnosis of Chandler' Syndrome based on the clinical findings, and in abnormalities in complementary exams. Nowadays, the patient is being followed in the Ophthalmology department at Hospital Federal Servidores do Estado.

Síndrome iridocorneoendotelial: síndrome de Cogan Reese / Iridocorneal endothelial syndrome: Cogan Reese syndrome

El síndrome iridocorneoendotelial comprende entidades que presentan afectación del endotelio corneal, el iris y el ángulo de la cámara anterior, razón por la que se desarrolla el glaucoma. Es generalmente unilateral, progresivo y afecta con mayor frecuencia a los adultos del sexo femenino. Se describen tres variantes con sus características peculiares: atrofia esencial del iris, síndrome de Chandler y el síndrome de Cogan Reese, que es una entidad rara. Se presenta una mujer de 53 años con diagnóstico de síndrome de Cogan Reese de 6 años de evolución y glaucoma de difícil control, por lo que recibió tratamiento quirúrgico(AU)

The iridocorneal endothelial syndrome includes conditions that affect the corneal endothelium, the iris and the anterior chamber angle. It is unilateral, progressive and mainly affects adult females. Three variants are described with their peculiar characteristics: essential atrophy of iris, Chandler´s syndrome and Cogan Reese syndrome that is a rare disease. Here is the case of a 53 year-old woman with Cogan Reese syndrome for six years and uncontrolled glaucoma and finally, she was surgically treated(AU)

Iridocorneal endothelial syndrome: iris naevus (Cogan-Reese) syndrome. A case report.

The purpose is to present a case of iridocorneal endothelium Syndrome with glaucoma and discuss clinical presentation and management strategies. A 45 year old female presented with redness, pain and diminision of vision in left eye. The patient was completely evaluated for the complaints. Slit lamp biomicroscopy revealed semidilated pupil not reacting to light and variation in iris colour pattern. Intraocular pressure was 14mmHg in the right eye and 46mmHg in the left eye. Gonioscopy of the left eye revealed broad based peripheral anterior synechiae. The optic disc of the left eye had a cup of 0.7. Specular microscopy of the left eye showed pleomorphism and polymegathism with multiple guttatae. This case reports the importance of specular microscopy in the evaluation of ICE syndrome and to plan the appropriate management strategies. We performed a trabeculectomy surgery for this patient with a well-functioning bleb to reduce the intraocular pressures following failure of topical anti-glaucoma medications.

Síndrome de Chandler / Chandler's syndrome

El síndrome de Chandler es una de las variaciones clínicas del síndrome iridocorneoendotelial, donde el denominador común es una alteración del endotelio corneal cuyo diagnóstico es, en ocasiones, muy difícil de realizar por la complejidad y la variabilidad de sus signos y síntomas. Se presenta una paciente de 67 años con disminución importante de la visión del ojo izquierdo. A la exploración existía un edema corneal severo, una midriasis pupilar, una corectopia leve hacia el sector temporal con tracción iridiana hacia el ángulo y sinequias anteriores angulares en el lado de la corectopia. La realización de un recuento endotelial demostró alteraciones cuali y cuantitativas del endotelio en el ojo izquierdo. Se concluyó que la microscopia especular es fundamental para el diagnóstico y la evaluación en casos sospechosos de síndrome de Chandler(AU)

Chandler's syndrome is a clinical variant of the iridocorneal endothelial syndrome in which the common aspect is the altered corneal endothelium that is sometimes very difficult to be diagnosed on account of the complexity and variability of signs and symptoms. This article was aimed at showing the effectiveness of specular microscopy for diagnosis. Here is a 67 years-old female patient who had significant reduction of vision in her left eye. On examination, there were observed severe corneal edema, pupillary mydriasis, mild corectopia towards the temporal sector with angled iridal traction and anterior angle synechias on the corectopia side. The endothelial counting revealed qualitative and quantitative alterations in the left eye. It was concluded that the specular microscopy is the key to diagnosis and assessment of suspected Chandler's syndrome cases(AU)

Síndrome iridocorneal endotelial: presentación de un caso / Iridocorneal endothelial syndrome: a case report

Introducción: el síndrome iridocorneal endotelial es un conjunto de trastornos no hereditarios, generalmente unilateral y de evolución progresiva, que se presenta con mayor frecuencia en mujeres jóvenes o de mediana edad. Se encuentra dentro del grupo de glaucomas secundarios de causa corneal, y puede iniciarse con un glaucoma agudo, aunque el paciente puede haber notado previamente alteraciones en el iris o en la córnea. Caso clínico: se presentó el caso de una paciente femenina de 49 años de edad que acudió a la Consulta Provincial de Glaucoma de Pinar del Río con ojo derecho nulo doloroso, atrofia del iris, policoria y glaucoma secundario con hipertensión ocular marcada .Después de valorarlo de conjunto en el Servicio de Oftalmología se diagnosticó un síndrome endotelial iridocorneal. Conclusiones: el síndrome iridocorneal endotelial se observa con mayor frecuencia en el sexo femenino. Es una entidad poco frecuente dentro de las enfermedades oftalmológicas y en el grupo de los glaucomas, de muy mal pronóstico visual. Se hace necesaria la realización de más estudios que permitan obtener mayores evidencias sobre este síndrome. De vital importancia resulta la adecuada evaluación de estos pacientes, para lograr la identificación temprana y aplicar métodos oportunos de tratamiento.

Introduction: the iridocorneal endothelial syndrome is a constellation of non-hereditary disorders, generally unilateral and of progressive evolution, which more frequently present in young and middle-aged women. It is one of a group of secondary types of glaucoma of corneal cause, and it can start with a acute glaucoma, although the patient may previously have noticed alteration in the iris or the cornea. Clinical case: a 49-year-old patient presented to the Pinar del Río Province Glaucoma Department with painful blind right eye, iris atrophy, polycoria and secondary glaucoma with marked ocular hypertension. After being jointly evaluated in the Ophthalmology Service, the patient was diagnosed with the iridocorneal endothelial syndrome. Conclusions: the iridocorneal endothelial syndrome is more frequently manifested in the female sex. It is a little frequent entity among the ophthalmologic diseases and among the glaucoma group it is of very low visual prognosis. It is necessary to carry out more studies permitting to obtain greater evidences about this syndrome. The adequate evaluation of these patients is vitally important, for early diagnosis and timely treatment.

Facoemulsificación en el síndrome iridocorneal endotelial / Phacoemulsification in the treament of iridocorneal endothelial syndrome

Se presenta el caso de una paciente atendida en el Servicio de Catarata del Centro Oftalmológico Enrique Cabrera de La Habana, con el objetivo de describir el resultado de la cirugía de catarata por facoemulsificación en el síndrome iridocorneal endotelial, por la variante cho choo chop and flip faco. Se muestran las modificaciones biomicroscópicas del segmento anterior, tonometría, densidad celular endotelial y mejor agudeza visual corregida preoperatoria y postoperatoria. Como resultados sobresalientes se señala que biomicroscópicamente hubo mínimas modificaciones en el iris, se logró la normalización de la tensión ocular, se produjo pérdida de menos del 15 por ciento de la densidad celular endotelial y la paciente logró agudeza visual postoperatoria óptima. Se concluye que la cirugía de catarata mediante facoemulsificación resultó una alternativa válida con mejoría visual en un caso con síndrome iridocorneal endotelial, con evolución estable de su enfermedad base

The case of a female patient who was seen at the cataract service of "Enrique Cabrera" ophthalmological center in Havana was presented. The objective was to describe the results of phacoemulsification cataract surgery to treat iridocorneal endothelial syndrome through the cho choo chop and flip phaco variant. The biomicroscopic changes of the anterior segment, the tonometry, the endothelial cell density and the best corrected visual acuity before and after surgery were shown. The main results comprised minimal changes in the iris from the biomicroscopic viewpoint, normalization of ocular tension, loss of less than 15 percent in endothelial cell density and postoperative optimal visual acuity. It was concluded that phacoemulsification cataract surgery proved to be a valid alternative that achieved visual improvement in a case of iridocorneal endothelial syndrome, with stable recovery from the underlying disease

Síndrome iridocórneo-endotelial: presentación de un caso / Iridocorneal-endothelial syndrome: presentation of a case

El síndrome iridocórneo-endotelial agrupa tres síndromes que cuando se presentan en las formas puras son relativamente fáciles de distinguir; sin embargo, es más frecuente que aparezca con un solapamiento considerable dificultando así el establecer una clara definición. Es una entidad nosológica muy poco común en la consulta de Oftalmología del Hospital Universitario Clínico Quirúrgico Comandante Faustino Pérez Hernández, de Matanzas, lo cual motivó la presentación de este caso. Se trata de una paciente de 47 años, que acudió a consulta en el mencionado hospital, que acudió a consulta en el mes de enero de 2012, portadora de un síndrome iridocórneo-endotelial unilateral en ojo derecho, con agudeza visual de 20/20, atrofia del iris, corectopia, pseudopolicoria y glaucoma secundario con hipertensión ocular marcada, a la que se le realizó trabeculectomía como proceder quirúrgico, con evolución satisfactoria.

The iridocorneal-endothelial syndrome comprehends three syndromes that when they appear in their pure forms are relatively easy to distinguish; but more frequently it appears considerably sneaked, making it difficult to stablish a clear definition. It is a nosological entity very uncommon in the consultation of Oftalmology of the Clinica-surgical Teaching Hospital Comandante Faustino Perez Hernandez, of Matanzas. That motivated the presentation of this case. It deals with a female patient, aged 47 years, assisting the consultation of the before mentioned hospital in January 2012, carrying a unilateral iridocorneal-endothelial syndrome in the right eye, with a visual acuity of 20/20, iris atrophy, corectopia, pseudopolychoria and secondary glaucoma with remarked ocular hypertension. She was subject of a trabeculectomy as a surgical procedure, with a satisfactory evolution.

Comparison of Surgical Outcomes between Trabeculectomy and Aqueous Shunt Surgery in Patients with Iridocorneal Endothelial Syndrome

PURPOSE: To compare surgical outcomes and corneal endothelial cell densities in patients with glaucoma secondary to iridocorneal endothelial (ICE) syndrome who underwent trabeculectomy with mitomycin-C (MMC) versus those who underwent Ahmed Glaucoma Valve (AGV) implantation. METHODS: Twenty-one patients with ICE syndrome who underwent either trabeculectomy with MMC or AGV implantation were included in this study. All patients had more than 12 months of follow-up after glaucoma surgery. Intraocular pressure (IOP), visual acuity (VA) and corneal endothelial cell density were compared according to the method of surgery. RESULTS: Ten eyes had undergone a trabeculectomy with MMC, and 11 eyes had an AGV implantation. In eyes that underwent a trabeculectomy with a MMC, there were no statistically significant differences between preoperative and postoperative month 12 in IOP, VA or corneal endothelial cell density. In eyes that underwent AGV implantation, postoperative IOP, VA and corneal endothelial cell density were statistically significantly lower than the preoperative values. CONCLUSIONS: When determining the method of glaucoma surgery in patients with ICE syndrome, the additional procedures needed to maintain the surgical success of trabeculectomy with MMC and the corneal decompensation which is caused by a decrease in corneal endothelial cell density after AGV implantation should be considered.

Síndrome endotelial iridocorneal: reporte de un caso / Iridocorneal endothelial syndrome: report of a case

Se presenta un caso clínico en el municipio Cristóbal Rojas, estado Miranda, República Bolivariana de Venezuela, con el diagnóstico de síndrome endotelial iridocorneal (atrofia esencial de iris) en un paciente del sexo masculino, con una buena evolución de su cuadro clínico, controlado solo con hipotensores oculares. Este síndrome se observa en el sexo femenino fundamentalmente, y es una entidad poco frecuente, no solo dentro de las enfermedades oftalmológicas sino también dentro del grupo de los glaucomas, y de muy mal pronóstico.

We present a clinical case from the municipality Cristobal Rojas, Miranda State, Bolivarian Republic of Venezuela, with the diagnostic of iridocorneal endothelial syndrome (the iris essential atrophy) in a male patient, with a good evolution, only controlled with ocular hypotensors. This syndrome is observed mainly in the female genre, and it is a little frequent entity, not only among the ophthalmologic diseases but also among the glaucoma group. It has a very bad prognosis.

A Case of Iridocorneal Endothelial Syndrome with Pigmentary Glaucoma

PURPOSE: To report a case of iridocorneal endothelial syndrome with pigmentary glaucoma. METHODS: We encountered a 44-year-old unilateral glaucoma patient who complained of intermittently decreased visual acuity in the right eye. For differential diagnosis, we carried out ophthalmic and systemic examination. RESULTS: Binocular corrected visual acuity was 1.0. His intraocular pressure, measured using a Goldmann applanation tonometer, was 50 mm Hg in the right eye and 18mm Hg in the left. Upon examination of the right eye, we found pigments with a Krukenberg's spindle appearance on the corneal endothelium, peripheral anterior synechia extending beyond Schwalbe's line, trabecular hyperpigmentation, endothelial pleomorphism, polymegathism, cell loss, dark area within the cells, a light central spot and light peripheral zone, retinal nerve fiber layer defects, and visual field defects, which together led to the diagnosis of iridocorneal endothelial syndrome with pigmentary glaucoma. We observed progressed glaucomatous injury upon examination of the disc stereo photograph, retinal nerve fiber layer photograph, and visual field test, even though we used topical IOP reducers. Eventually, we performed a trabeculectomy in the right eye. CONCLUSIONS: We experienced a case of iridocorneal endothelial syndrome with unilateral pigmentary glaucoma. The glaucoma was not well-controlled with topical IOP reducers. The glaucoma was then treated by a trabeculectomy. We report this case with a review of the literature.

Surgical Result in Glaucoma Associated with Iridocorneal Endothelial Syndrome

Iridocorneal endothelial(ICE) syndrome is charaterized by unilateral glaucoma, abnormal corneal endothelium with proliferation, and iris stromal abnormalities. Uncontrolled glaucoma in ICE syndrome has been treated with filtering surgery, but the late failures occurred frequently. The medical records of nine patients with ICE syndrome that underwent glaucoma surgery were retrospectively reviewed. 6 eyes were progressive iris atrophy, and 3 eyes were Cogan-Reese syndrome. The success rates of primary trabeculectomy at 1 and 2 years were 57.1% and 33.3%, respectively. Ahmed glaucoma valve implant oeprations were performed on 6 eyes with failed previous filtering operation. 4 of 6 eyes resulted in controlled IOP until last follow up visit(mean ; 18.8 months). Overall, 1.9 times operations were needed to control IOP. Operations were performed only one time in 4 patients and two times in 3 patients to control IOP.