Fleck. Pre-Descemet Dystrophies (generally good vision and comfort) Primary Pre-Descemet Dystrophy

Transcription

1 Fleck Etiology: bilateral, sometimes asymmetric, autosomal dominant opacities located in all levels of stroma as early as 1 st decade Slit lamp: well demarcated, small round gray-white doughnut-like, wreath-like opacities extend throughout the otherwise normal cornea stroma Subjective symptoms: vision usually not affected, may have photophobia and opacities are usually incidentally noted during a careful slit lamp exam Treatment: none Pre-Descemet Dystrophies (generally good vision and comfort) Cornea farinata Etiology: maybe a degenerative process than dystrophic one, older individuals Slit lamp: small, gray, flour-like, comma-shaped, circular, linear, filiform and dotlike opacities, distributed axially or angularly Subjective symptoms: none Primary Pre-Descemet Dystrophy Etiology: primary (unknown or hereditary) or secondary (ocular or systemic disease) degeneration or dystrophy? Slit lamp: resembles cornea farinata but has larger and more polymorphous opacities, six types of opacities (dendritic, boomerang, circular, coma, linear, filiform) Subjective symptoms: vision is unaffected 1

2 Endothelial Dystrophies Manifest themselves in three ways Stressed endothelial cells produce a new tissue; the posterior collagen layer (PCL); clinically this tissue appears as thickening of Descemet s membrane--corneal guttata Endothelial cells become larger and more disrupted by guttata Stromal and epithelial edema occur with breakdown of the endothelial barrier and pump Clinical Tests Specular microscope: size; number and cell shape Pachometry: degree of corneal thickness (stromal edema) Corneal Guttata Slit lamp: dew-droplike, wart-like endothelial prominences that are focal mushroom-like excrescences on Descemet s membrane, located axially: typical beaten metal appearance; specular reflection reveals dark spots and irregular (size and shape) cells 2

3 Primary guttata in three clinical patterns A few guttata; normal endothelial aging; middle to older age; slowly progressive Larger number (accompanied by endothelial pigment): endothelial dystrophy Increasing number of guttata accompanied by corneal edema: Fuchs Hassal-Henle' bodies Secondary guttata Associated with degenerative corneal disease, trauma, or inflammation On removal of causative agent if subsides Fuchs Endothelial Dystrophy History and etiology Bilateral, autosomal dominant (variable expression), central, sometimes asymmetric, female>male, age years, postmenopausal Unknown, progressive corneal disease in which corneal edema results from the primary metabolic incompetence of endothelial cells; endothelial barrier and pump fail Slit lamp (three stages) Guttata, endothelial pigment, moderate graying and thickening of Descemet s membrane, sometimes a grayish membrane Stromal edema (ground glass appearing and folds in Descemet--1.0mm thickness); epithelial edema (fine dewdrop pattern with microcysts & bullae), earlier and worse if IOP elevated= bullous keratopathy Subjective symptoms Subepithelial irregular gray, swirling sheets of scar tissue and stromal scarring with neovascularization Asymptomatic with only guttata and endothelial pigment Hazy vision and symptoms of glare with stromal and epithelial edema (especially in AM), attacks of severe pain with bullous keratopathy Vision deterioration (worse in a.m initially) with stromal and subepithelial scarring but less attacks of pain: hand motion vision 3

4 Management None- monitoring and maintaining normal IOPs With epithelial edema-topical hypertonic drops & ointment, warm air (hair dryer)= may dehydrate eye; loosely fit, thin, high water content bandage soft contact lenses (comfort & decrease pain); decrease IOPs Penetrating keratoplasty (duration of graft clarity limited): combined procedure (I.O.L. and keratoplasty) with cataracts, new deep lamellar endothelial keratoplasty (DLEK) 4

5 5