Case Of Iridocorneal Endothelial Syndrome Research Articles

Rare case of iridocorneal endothelial syndrome with secondary glaucoma

A rare case of 40 years old female, came with complaints of pain and blurring of vision in the left eye for the past week. The patient had a diminution of vision since birth in her left eye. On examination left eye vision was found to be 6/60 not improving with pinhole, also defective color vision 20/25. The right-eye vision was found to be normal. The left eye showed significant corneal edema, and moderate anterior chamber depth, with iris features of Corectopia, Pseudopolycoria, and iris atrophy. Pressures were found to be 30mm hg in the left eye, gonioscopy revealed high peripheral anterior synechiae extending anteriorly above Schwalbe’s line. Left eye fundoscopy was done and it showed increased CDR of 0.8 to 0.9 following which visual field analysis was done, it showed inferior field defect. The patient was started on e/d timolol and hypertonic saline for a week. The patient was reviewed after a week showing reduced pressures of 22mmg in the left eye with reduced corneal edema and pain. Diagnosis of iridocorneal endothelial syndrome-essential iris atrophy type with secondary glaucoma was made and now the patient is being followed up regularly. An iridocorneal endothelial syndrome is a group of disorders with three clinical variants as Chandler syndrome, Essential iris atrophy, and Cogan Reese syndrome. Pathology is the presence of abnormal endothelial cells, which behave as epithelial cells, able to proliferate and migrate. The iridocorneal endothelial syndrome is frequently associated with secondary glaucoma.

Differential diagnosis and treatment of Chandler iridocorneal endothelial syndrome

The article presents a profound clinical, instrumental and morphological analysis of a family case of iridocorneal endothelial syndrome (Chandler syndrome) notable for atypical aggressive bilateral course and unusual hereditary manifestation. Five stages of the pathological process have been suggested.

Medical and surgical management has a role in control of intraocular pressure in early detected iridocorneal endothelial syndrome: case series

Iridocorneal endothelial syndrome is a rare ocular disorder characterized by abnormal endothelization of angle structure and iris producing characteristic manifestation including secondary angle closure glaucoma. The purpose is to present four cases of iridocorneal endothelial syndrome with glaucoma and discuss clinical presentation and management strategies. Out of four cases, two cases of Cogan-Reese syndrome who present early have well controlled IOP with trabeculectomy with mitomycin-C. One case who presented late with progressive iris atrophy and secondary angle closure glaucoma went to absolute stage in spite of trabeculectomy with mitomycin-C. One case with Chandler’s syndrome has well controlled IOP with topical anti-glaucoma medication.

Clinicopathologic Findings in Iridocorneal Endothelial Syndrome and Posterior Polymorphous Membranous Dystrophy After Descemet Stripping Automated Endothelial Keratoplasty

To report the histopathologic findings of the iridocorneal endothelial (ICE) syndrome and posterior polymorphous membranous dystrophy (PPMD) in 3 patients who underwent Descemet stripping automated endothelial keratoplasty (DSAEK), and to correlate these findings with the clinical diagnosis. Three patients with clinical findings compatible with either ICE syndrome (1 patient) or PPMD (2 patients) underwent DSAEK. The DSAEK specimens were processed for light microscopy, immunhistochemical staining for cytokeratins AE1/3 and MAK6, and electron microscopy. Examination of the DSAEK specimens showed multilayered endothelial cells and thickened Descemet membrane in all cases. Immunohistochemical staining for cytokeratins was positive in the endothelium in all cases. Ultrastructural examination showed a thickened Descemet membrane and wide-spaced collagen in Descemet membrane in 1 case of PPMD but not in 2 cases of ICE syndrome, including 1 case that carried the clinical diagnosis of PPMD. In 2 cases, the histopathologic evaluation of the DSAEK specimen confirmed the clinical diagnosis; however, in 1 case the pathological diagnosis was ICE syndrome, while the clinical diagnosis was PPMD. ICE syndrome and PPMD can be diagnosed and differentiated from one another by histopathologic evaluation of corneal specimens obtained at the time of DSAEK. We recommend submitting the corneal tissue obtained during DSAEK for pathological examination when the etiology of corneal edema is unclear.

Случай иридокорнеального эндотелиального синдрома (Синдром Чандлера)

Случай иридокорнеального эндотелиального синдрома (Синдром Чандлера)

Iridocorneal Endothelial Syndrome in a 16-Year-Old

We report iridocorneal endothelial syndrome in a male who presented at the age of 16 years with a 3-year history of complaints of blurred vision, altered pupillary shape, and monocular diplopia OD. The examination was notable for unilateral effacement of the iris architecture, stretch holes, corectopia, and localized ectropion uveae. Intraocular pressures were 41 mm Hg OD and 10 mm Hg OS. Gonioscopy revealed intermittent areas of broad synechiae anterior to Schwalbe's line alternating with a clinically normal appearance. The left eye and angle were unremarkable. Specular microscopy confirmed the presence of unilateral endothelial pleomorphism and polymegathism. To our knowledge, this is the earliest reported case of iridocorneal endothelial syndrome in a young man.