Some of the holes occur in quadrants away from the direction of pupillary displacement and are thought to be caused by traction (‘stretch holes’). 189 The iris dissolution begins as a patchy disappearance of the stroma and progresses to full-thickness holes ( Fig. In progressive iris atrophy (known as essential iris atrophy in the past) the clinical picture is dominated by corectopia and progressive dissolution of the iris ( Fig. Michael V Drake MD, in Becker-Shaffer's Diagnosis and Therapy of the Glaucomas (Eighth Edition), 2009 Progressive (essential) iris atrophy In Fuchs' dystrophy there are bilateral guttae which are larger than the endothelial changes in ICE. However, the endothelium is normal, and the cornea is normal except for a prominent Shwalbe's line. Axenfeld–Rieger syndrome is a congenital, bilateral, autosomal dominant condition characterized by marked iris changes including atrophy, holes and corectopia and, frequently, glaucoma. Corectopia, PAS, and glaucoma can occur, but iris changes are milder and glaucoma is less common than in ICE. There is epithelialization of the endothelium. PPD is an inherited, autosomal dominant endothelial dystrophy associated commonly with bilateral focal vesicular changes and scalloped ridges in asymptomatic patients, and less often with diffuse corneal edema. The differential diagnosis of ICE includes posterior poly-morphic dystrophy (PPD) Axenfeld–Rieger syndrome and Fuchs' dystrophy. Confocal microscopy can visualize the endothelium even in the presence of corneal edema, revealing epithelial-like endothelial cells with hyperreflective (white) nuclei. ICE cells correlate with the epithelialization of endothelial cells seen in some cases on histopathology in this condition. ICE cells are not hexagonal and there is a reversal of the normal light/dark pattern, so that the cells look dark and the cell borders appear light. Specular microscopy may demonstrate ‘ICE' cells, unless corneal edema prevents visualization. Abnormal corneal endothelium produces basement membrane that progressively covers the anterior chamber angle and anterior iris and contracts, pulling the pupil toward the membrane and causing iris atrophy away from the membrane and PAS near it. ICE is primarily a clinical diagnosis, although specular and confocal biomicroscopy can be helpful. Corneal changes by slit lamp exam include a fine hammered silver appearance of the endothelium and corneal edema. Iris changes include atrophy, holes, nodules, ectropion uveae ( Figure 281.1 ), and corectopia. Cohen MD, in Roy and Fraunfelder's Current Ocular Therapy (Sixth Edition), 2008 DIAGNOSIS Clinical signs and symptoms Uncontrolled glaucoma may worsen corneal edema and cause increased pain.Įlisabeth J. Intraocular pressure elevation does not correlate with the extent of the PAS. Glaucoma results from the proliferation of the endothelial membrane over the anterior chamber angle and from the subsequent PAS development. Glaucoma develops in most eyes with progressive iris atrophy and Cogan-Reese syndrome, but may be less common with Chandler's syndrome. Patients may have manifestations of more than one of these clinical entities ( Fig. This name is a misnomer since the outcroppings consist of normal iris tissue, and are histologically unrelated to nevi. In Cogan–Reese syndrome, contracture of the proliferating endothelial membrane on the iris face forms multiple pedunculated outcroppings of normal iris tissue.Ĭogan–Reese has been called iris nevis syndrome since these outcroppings resemble small nevi on the iris surface. Later in the disease, the corneal edema, decreased vision, and pain may persist throughout the day. Corneal edema and blurred vision are often worse upon awakening in the early stage of the disease. Patients frequently present with decreased vision and pain. Iris hole formation may also develop without corectopia and may be related to iris ischemia ( Figs 38-2–38-4).Ĭorneal edema is the most common prominent feature of Chandler's syndrome. Contracture of this membrane leads to peripheral anterior synechiae, pupil distortion, and iris hole formation opposite the area of the peripheral anterior synechiae (PAS). The abnormal endothelial layer proliferates to cover the angle and iris. Jody Piltz-Seymour, Tak Yee Tania Tai, in Glaucoma (Second Edition), 2015 Signs and SymptomsĮssential iris atrophy is characterized by corectopia, iris atrophy, iris hole formation, and ectropion uveae.
0 Comments
Leave a Reply. |
AuthorWrite something about yourself. No need to be fancy, just an overview. ArchivesCategories |