Bilateral Iridocorneal Endothelial Syndrome

Objectives@#We presented a case of bilateral iridocorneal endothelial (ICE) syndrome with secondary glaucoma and discussed its clinical presentation and management.@*Study design@#This is a case report.@*Results@#A 12-year old female consulted for a one-year history of progressive blurring of vision in both eyes associated with abnormal pupils and occasional eye pain. Polycoria, shallow anterior chambers, increased intraocular pressures (IOP), areas of closed angles on gonioscopy, and increased cup-to-disc ratio in both eyes indicative of ICE syndrome were present. Management included anti-glaucoma medications and implantation of glaucoma drainage devices (GDD).@*Conclusion@#Early detection with regular follow-ups leading to prompt management of the ICE syndrome are necessary. IOP control may be challenging due to the nature of the disease and may require multiple surgeries including GDD implantation to achieve successful outcomes. Early use of GDD may be beneficial for pediatric patients with ICE and glaucoma, and may require additional surgeries to adequately manage the IOP.

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 iridocorneoendotelial: variedad Chandler / Iridocorneal endothelial syndrome in Chandler variety

En el síndrome iridocorneoendotelial se unifican diferentes variaciones clínicas de una misma entidad. El denominador común de todas ellas es una peculiar anormalidad de las células endoteliales, alteraciones del iris y el desarrollo de glaucoma, generalmente unilateral y más común en mujeres. Se presenta un caso clínico de la variedad Chandler, quien acudió a la consulta de catarata, con este diagnóstico en el ojo derecho. Se realiza cirugía de catarata en ambos ojos con excelentes resultados y control de la presión intraocular(AU)

Iridocorneal endothelial syndrome comprises different clinical variations of the same disease. The common features of all them are peculiar anomaly of the endothelial cells, alterations of the iris and the onset of glaucoma, generally unilateral and more common in women. A clinical case of the Chandler variety is presented, which is a woman with cataract, and iridocorneal endothelial syndrome diagnosis on the right eye. Cataract surgery was performed in both eyes with excellent results and control of the intraocular pressure(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)

Síndrome de Cogan Reese / Cogan Reese syndrome

RESUMO Estudo realizado para descrever um caso da síndrome de Cogan Reese. Paciente do sexo feminino, 55 anos, com diagnóstico de síndrome de Cogan Reese. Melhor acuidade visual de 0,67 em olho direito e 0,2 em olho esquerdo. Pelo exame biomicroscópico não se detectou anormalidades no olho direito. No olho esquerdo haviam nódulos pigmentados na superfície anterior da íris, irregularidades corneanas e buraco iriano (pseudopolicoria). A pressão intra-ocular era 18 mmHg no olho esquerdo e havia atrofia óptica glaucomatosa no disco óptico. A paciente havia sido submetida à trabeculectomia três anos atrás. Recentemente o tratamento medicamentoso possibilitou o controle relativo da pressão intraocular. Gonioscopia revelou sinéquias anteriores periféricas. A microscopia especular eletrônica mostrou ICE-cells e baixa contagem de células. A cirurgia filtrante para tratamento do glaucoma usualmente tem sucesso quando realizada precocemente, mas ela pode falhar devido à endotelização da fístula pela membrana anormal do endotélio corneano. O tratamento medicamentoso foi efetivo apesar da falha na trabeculectomia.

ABSTRACT Study conducted to report a case of Cogan Reese syndrome. Female patient, 55 years old with diagnosis of Cogan Reese syndrome. Best visual acuity of 0.67 in the right eye and 0.2 in the left eye. By biomicroscopic examination there was no abnormalities in the right eye. In the left eye there were pigmented nodules on the anterior surface of the iris, corneal irregularities and iris hole (pseudopolycoria). The intraocular pressure was 18 mmHg in the left eye and there was glaucomatous optic atrophy of the optic disk. The patient had been subjected to trabeculectomy three years ago. Recently medical treatment allowed the relative control of intraocular pressure. Gonioscopy revealed peripheral anterior synechiae. Corneal specular microscopy showed ICE-cells and low cell count. Glaucoma filtering surgery is usually successful when done early, but it may fail due to endothelialization of the fistula by the abnormal corneal endothelium. Medical treatment was effective despite a fail trabeculectomy.

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

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.

Efeitos da restrição calórica nas vias de sinalização por insulina e óxido nítrico: implicações para biogênese, morfologia e função mitocondriais / Calorie restriction restriction effects on insulin and nitric oxide signaling: implications to mitochondrial biogenesis, morphology and function

A restrição calórica (RC) estende a expectativa de vida de muitos organismos por mecanismos ainda em estudo. Entre os vários efeitos fisiológicos da RC encontra-se o aumento na biogênese mitocondrial, dependente de óxido nítrico (NO•), sintetizado pela enzima óxido nítrico sintase endotelial (eNOS). Um dos indutores fisiológicos mais potentes da eNOS é a insulina, cujos níveis plasmáticos são consideravelmente reduzidos nos organismos em RC. O objetivo deste trabalho foi investigar os mecanismos associados ao aumento da sinalização por NO• durante a RC in vivo e in vitro, e as conseqüências celulares do aumento de massa mitocondrial no que diz respeito à longevidade e capacidade respiratória celulares. Submetemos camundongos Swiss fêmeas à RC de 40% e observamos um considerável aumento tecido-específico na fosforilação basal de Akt e eNOS em músculo esquelético, tecido adiposo visceral e cérebro, os quais também apresentaram maior massa mitocondrial. A associação entre a sinalização por insulina, NO• e biogênese mitocondrial foi adicionalmente confirmada em um grupo de camundongos tratados com o desacoplador mitocondrial dinitrofenol (DNP), que também reduz a insulinemia e aumenta a longevidade em camundongos. Para o estudo mecanístico deste fenômeno, usamos soros de ratos Sprague-Dawley submetidos à RC de 40% ou alimentados ad libitum (AL) em cultura celular de células vasculares da musculatura lisa (VSMC), reproduzindo um protocolo descrito para RC in vitro. O uso do soro RC aumentou a fosforilação do receptor de insulina e Akt, a expressão de eNOS e nNOS (forma neural da NOS) e a fosforilação de eNOS, o que se refletiu em maior liberação de nitrito (NO2) no meio de cultura. Inibindo-se a Akt, todos os efeitos promovidos pela RC na sinalização por NO• foram revertidos. Ao se imunoprecipitar do soro a adiponectina, citocina conhecida por aumentar a sensibilidade à insulina, aumentada durante a RC, os efeitos do soro RC na via de sinalização de insulina foram abolidos e, conseqüentemente, os efeitos na sinalização por •NO foram prevenidos. Neurônios de células granulosas de cerebelo, que não expressam eNOS, apenas nNOS, foram cultivados com os soros AL ou RC, e também apresentaram considerável aumento na sinalização por •NO. Estas alterações induziram a biogênese mitocondrial e capacidade respiratória, e foram associadas à maior longevidade celular. Os mesmos efeitos mitocondriais foram observados em células secretoras de insulina, INS1, entretanto a secreção de insulina em resposta à glicose tornou-se inibida, por um mecanismo desconhecido, porém associado a reduzidos níveis intracelulares de espécies oxidantes, moléculas-chave para a secreção de insulina; e à alteração da morfologia mitocondrial, provavelmente devido à maior expressão de mitofusina-2 (Mfn-2). Ao se nocautear a Mfn-2, houve um aumento na geração de EROs e as células em RC passaram a secretar insulina a níveis comparáveis aos das células controle. Concluímos que durante a RC a maior sensibilidade à insulina aumenta a atividade de eNOS, via Akt, associada à maior biogênese mitocondrial. A adiponectina é uma molécula-central nestes eventos. A expressão de nNOS também é afetada, por mecanismos desconhecidos. O aumento de biogênese mitocondrial eleva a capacidade respiratória celular e impacta positivamente a longevidade in vitro. A alteração da morfologia mitocondrial associa-se a alterações na produção de oxidantes intracelulares e mudanças na secreção de insulina

Calorie restriction (RC) is known to extend the lifespan in many organisms, and its mechanisms of action are still under investigation. Enhanced mitochondrial biogenesis driven by nitric oxide (•NO), synthesized by the endothelial nitric oxide synthase (eNOS), is proposed to be a CR central effect. Insulin is one of the most potent physiological activators of eNOS. However, plasmatic insulin levels are dramatically reduced in organisms under CR. The goal of this work was uncover the mechanisms associated with enhanced •NO signaling during CR, in vivo and in vitro, as well as the cellular consequences of increased mitochondrial mass, regarding lifespan and reserve respiratory capability. Female Swiss mice were submitted to 40% of CR. A tissue-specific (skeletal muscle, abdominal adipose tissue and brain) increment in basal Akt and eNOS phosphorylation, which was related to enhanced mitochondrial biogenesis, was observed. Indeed, this association was also verified in tissues from mice treated with low doses of a mitochondrial uncoupler, dinitrophenol (DNP). To unveil the mechanism behind the insulin signaling effects on •NO levels, serum from Sprague-Dawley rats submmited to 40% of CR was used to culture in VSMC cells, an in vitro CR protocol. CR sera enhanced insulin receptor (IR) and Akt phosphorylation, as well as nitrite (NO2-) accumulation in the culture media, the expression of eNOS and nNOS (neural NOS isoform) and eNOS phosphorylation. The effects of CR sera were reversed by Akt inhibition. The immunoprecipitation of serum adiponectin, a cytokine known to improve peripheral insulin sensitivity, also reversed the CR serum effects on insulin and •NO signaling. Cerebellar neurons, which do not express eNOS, just nNOS, were also cultured with CR or AL serum and also presented striking increments in •NO signaling, associated with mitochondrial biogenesis, increased reserve respiratory capability and lifespan extension. The mitochondrial effects promoted by CR were also observed in insulin secreting cells (INS1). However, under the CR condition, insulin secretion stimulated by glucose was impaired. The likely explanations are reduced mitochondrial reactive oxygen species (ROS) generation, or the alteration in mitochondrial morphology, associated, in our model, with enhanced mitofusin-2 expression (Mfn-2). In cells which the Mfn-2 was knocked down, insulin secretion in CR and AL groups was responsive to glucose at the same level, and the intracellular oxidants levels were much higher. Overall, CR improves •NO signaling due to enhanced insulin sensitivity, through Akt, and results in mitochondrial biogenesis. Adiponectin is a key molecule in this phenomenon. Increments in mitochondrial mass enhance the cellular reserve respiratory capability and lifespan. Mitochondrial morphology alterations are associated with possible decreases in ROS generation and impaired insulin release, maintained the low levels of plasmatic insulin

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.

A Case of Chandler's Syndrome Associated with Anterior Uveitis

PURPOSE: To report a case of Chandler's syndrome associated with anterior uveitis. METHODS: We encountered a 39-year-old woman who complained of decreased visual acuity in the left eye with anterior uveitis and increased intraocular pressure. On examination, we found corectopia, endothelial pleomorphism and peripheral anterior synechiae, leading to the diagnosis of Chandler's syndrome with anterior uveitis. RESULTS: The anterior uveitis and increased intraocular pressure in Chandler's syndrome were well controlled with the use of Beta-blocker, Latanoprost and topical corticosteroids. We report this case with a review of the literatures.

A Case of Chandler's Syndrome Associated with Anterior Uveitis

PURPOSE: To report a case of Chandler's syndrome associated with anterior uveitis. METHODS: We encountered a 39-year-old woman who complained of decreased visual acuity in the left eye with anterior uveitis and increased intraocular pressure. On examination, we found corectopia, endothelial pleomorphism and peripheral anterior synechiae, leading to the diagnosis of Chandler's syndrome with anterior uveitis. RESULTS: The anterior uveitis and increased intraocular pressure in Chandler's syndrome were well controlled with the use of Beta-blocker, Latanoprost and topical corticosteroids. We report this case with a review of the literatures.

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.

Iridocorneal Endothelial Syndrome: iris nevus syndrome

Iridocorneal endothelial syndrome (ICE syndrome) embraces a group of rare diseases with similar characteristics in the anterior segment, namely Essential iris atrophy, Chandler`s syndrome, and Iris nevus syndrom (Cogan Reese syndrome). The origin and pathogenesis of ICE syndrome remain unkown, but it appears that it is an abnormality of the corneal endothelial cells that is fundamental to all three conditions and leads to characteristic changes in the cornea, iris, and angle. Finding by specular microscopy of abnormal cells on the posterior corneal surface has led to the speculation of possible pathogenensis of this condition. We have experienced two patients with Iris nevus syndrome. Both cases presented with glaucoma as the chief clinical problem, and they illustrated difficult challenges in the glaucoma managemant in this disorder.

Progressive Iris Atrophy

Progressive iris atrophy is a disease characterized by marked iris atrophy, iris hole, corneal endotherial abnormality, perripheral anterior synechia, and glaucoma. It is considered as a variant of iridocorneal endotherial syndrome with Chandler's syndrome and Cogan-Reese syndrome. We have experianced a 34 year old male patient complaning visual blurring in his left eye. At examination, we found characteristics of progressive iris atrophy. After filerring surgery, associated glaucoma was well controlled. Then we report a case with review of the literatures.

A Case of Cogan-Reese Syndrome

Cogan-Reese syndrome is a disease characterized by glaucoma in an eye with peripheral anterior synechia, multiple pigmented iris nodules, and ectopic Descemet's membranes. Cogan-Reese syndrome is a variant of iridocorneal endothelial syndrome and shares common properties whcih are corneal endothelial degeneration, iris atrophy and secondary glaucoma, with progressive iris atrophy and Chandler's syndrome. We have experienced a 43-year-old female patient complaining of visual dimness in the left eye. On examination, we found characteristics of Cogan-Reese syndrome which were corneal endothelial pleomorphism and cell loss, corneal edema, iris atrophy and multiple pigmented nodules on the surface of the iris, peripheral anterior synechia, and increased intraocular pressure in the left eye. We report a case of Cogan-Reese syndrome which has not been reported in Korea.

A Case of Essential Iris Atrophy

Essential iris atrophy is a disease characterized by corectopia, iris atrophy, iris hole formation, corneal endothelial degeneration, peripheral anterior synechia, and glaucoma. This disease shares common properties with Chandler's syndrome and Cogan-Reese syndrome, so above three diseases are catagorized by iridocorneal endothelial(ICE) syndrome. We have experienced a patient who had a complaint of visual dimness in right eye. On examination, she had corectopia, iris hole and atrophy, and increased IOP in right eye. Specular microscopic finding was consistent with essential iris atrophy.