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Posterior polymorphous corneal dystrophy associated iridocorneal endothelial syndrome

Poster Details

First Author: B.García-Valcarcel Gonzalez SPAIN

Co Author(s):    S. Miraflores Gómez   A. Baeza Autillo   L. Garcia Padilla   C. Chau Ramos   P. Balado Vazquez  

Abstract Details



Purpose:

To remember the various alterations in the iridocorneal angle, and its consequences, which may occur associated with posterior polymorphous corneal dystrophy (PPCD)

Setting:

Gregorio Marañon General Universitay Hospital. Madrid. Spain

Methods:

Posterior Polymorphous Corneal Dystrophy is one of the Descement Membrane and Endothelial Dystrophies. His inheritance is autosomal dominant and penetrance and variable expressivity. Many patients may remain asymptomatic: edema,and elevated IOP may occur. .Specular microscopy confocal microscopy and Gonioscopy are helpful in the Diagnosis of PPCD. It may be asociated with Iridocorneal Endothelial Syndrome To do this we present two familial cases of posterior polymorphous dystrophy (DPP) associated with iridocorneal endothelial syndrome. IOP, gonioscopy, specular microscopy, confocal microscopy and fundus studies were performed.

Results:

Clinical Case 1 40 year old patient with impaired Visual Acuity OS. Best Corrected VA: OR: 0.9 OS:0.4-0.5 Slit Lamp OR: endothelial vesíulas (crater-like). Image support with Posterior Polymorphous Corneal Dystrophy OS: endothelial corneal decompensation IOP OR: 11 mmHg OS: 13 mmHg Gonioscopy: Angle grade 0-I in the four quadrants. Prominent Schwalbe line, iris processes with increased pigmentation. Endothelial Count OR: 1150 - 1300 cells. Crater-like lesions with decreased cell density. Fundus: Normal. OR: Iridotomies were performed OS: Penetrating keratoplasty (PK) was performed One year after the PK, the best corrected VA was 0.7. The IOP remains controlled by time without antihypertensive treatment Clinical Case 2 Pediatric patient with bilateral corneal opacities. BMC: corneal opacities with calcium deposits; distribution bilateral band (OR was more affected). Embriontoxon both eyes. IOP both eyes: 25 mmHg. Gonioscopy: anterior iridocorneal adhesions at 1 o'clock in OS. Iris processes in Both Eyes (Disgenesic angle). Normal fundus examination. Commenced treatment with timolol maleate 0.5%, remaining with the IOP controlled at the moment, although not ruled out the need for trabeculoplasty in the future.

Conclusions:

PPCD is one of the subsequent corneal dystrophies with Fuchs dystrophy and congenital hereditary endothelial dystrophy His inheritance is autosomal dominant and penetrance and variable expressivity. It is usually bilateral and often asymmetrical. Many patients may remain asymptomatic throughout life . However, 30% may have corneal edema, and 15 % may be associated with elevated IOP. Clinically, the first changes are already being observed in the first decade of life. Characteristic lesions affecting PPCD Descemet's membrane and endothelium presenting great variability. Vesicles, lesions and diffuse band opacities may occur. Less often can be seen corneal edema, peripheral anterior synechiae and endothelial Guttas Specular microscopy and confocal provide a useful complementary tool . In gonioscopy observed : prominent Schwalbe line and real iris processes or peripheral iridocorneal adhesions. Occasionally seen : iris atrophy , remains of pupillary membrane, uveal ectropion and corectopia , which , combined with previous findings suggesting a resemblance to the iridocorneal endothelial syndrome, or a mesenchymal dysgenesis of the anterior chamber. Glaucoma associated with DPP ( ' secondary angle closure glaucoma with anterior traction mechanism without pupillary block ' ) is difficult to manage and poses a therapeutic dilemma for the ophthalmologist . Appears much as 60 % in patients requiring keratoplasty as treatment FINANCIAL INTEREST: NONE

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