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.

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.

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)

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

PURPOSE: To report the outcomes of primary trabeculectomy with mitomycin-C (MMC) in eyes with glaucoma secondary to iridocorneal endothelial (ICE) syndrome. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

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.

[The irido-corneo-endothelial syndrome. The loss of the control of corneal endothelial cell cycle. A review]. / Le syndrome irido-cornéo-endothélial ou la perte du contrôle du cycle cellulaire de l'endothélium cornéen. Une revue.

The three major symptoms of the irido-corneo-endothelial syndrome are the alterations of the corneal endothelium and of the iris with a loss of the regulation of the cell cycle, and the progressive obstruction of the irido-corneal angle. This rare pathology attacks mainly young adult women. Most of the symptoms and complications originate from the excessive proliferation of the corneal endothelial cells accompanied by the evolution of their phenotype towards that of the epithelial cells. In normal conditions the corneal endothelial cells do not divide, they are blocked in the G1 stage of the cell cycle, mainly because of the action of the inhibitors of cyclin-dependent kinases. Still these cells retain a good capacity for proliferation, which can be induced by the down-regulation of the expression of the inhibitors of the cyclin-dependent kinases. This proliferative capacity declines with age and is also different according to the localization of the cells: it is more intense with those originating from the central area then in those from the peripheral area of the cornea. The age-related decline of the proliferative capacity is not due to the shortening of the telomers, but to the stress-induced accelerated senescence of the cells.

Iridocorneal endothelial syndrome in a Chinese child.

PURPOSE: We reported a rare case of ICE syndrome in a Chinese child. CASE REPORT: A 6-year-old child was admitted to the ophthalmology department of the Affiliated Wuxi Second Hospital of Nanjing Medical University, complaining of elevated intraocular pressure at his first diagnosis in a local hospital. The patient was initially treated with medication. During subsequent follow up, two iris holes were seen in the patient's right eye and he was then diagnosed with ICE syndrome. The child underwent surgery combined with mitomycin C intraoperatively. The intraocular pressure was stably controlled. CONCLUSION: ICE syndrome has been rarely reported in children. Glaucoma filtering surgery combined with intraoperative administration of mitomycin is efficacious for intraocular pressure control.

[Gene therapy in ophthalmology: state of the art]. / Gentherapie in der Ophthalmologie: Stand der Forschung.

The term "gene therapy" denotes the treatment of diseases or gene deficiencies by introduction of genes into cells. To achieve this goal, vectors are used to transfer the genetic information into the cells. Thus, the protein of interest can be overexpressed or silenced. On account of its easy accessibility, the good compartmentalisation and the separation from the main bloodstream by the blood-retina barrier, the eye represents a very attractive target to treat ocular diseases by gene therapy. In this work, we provide an overview of the progress in ocular gene therapy over the last decade and give an outlook on future developments.