DEGENERATIVE

AND MISCELLANEOUS CORNEAL CONDITIONS B. Band (Calcific) Keratopathy

- Band-like deposition of calcium salts in the Bowman layer, epithelial BM and anterior stroma
THE CORNEA - Limited to the interpalpebral area
ABCDEs of the Corneal Layers - thought to be caused from conditions that increase the levels of calcium in the body such as
A Anterior Epithelium kidney disease, excessive vitamin D, increased levels of certain thyroid hormones, sarcoidosis,
B Bowman Layer lupus and Paget’s disease, a condition where there is excessive breakdown of your bones.
C Central Stroma - Symptoms: Irritation, injection, blurring of vision
D Descemet Membrane - Treatment: Chelation with EDTA
E Endothelium
C. Climatic Droplet Keratopathy
Three Characteristics of Cornea - Exposure to UV light
I. The Corneal Epithelium - Early Stage: Fine yellow droplets in peripheral cornea
- composed of stratified squamous epithelial cells and makes up approximately 5%–10% of - Advance Stage: Droplets become central, corneal clouding and blurring of vision
the total corneal - Asymptomatic, Reduced visual acuity
- Tight junctions between superficial epithelial - Treatment: Prevention (wearing of sunglasses); Advance cases: corneal transplantation
- cells prevent penetration of tear fluid into the stroma.
II. The Stroma D. Salzmann Nodular Degeneration
- The regular arrangement of stromal cells and macromolecules is also necessary for a clear - Superficial gray-white to bluish elevated nodules
cornea. Keratocytes vary in density and size throughout the stroma and form a 3- - Preceded by Corneal inflammation (Trachoma)
dimensional network throughout the cornea. - 1-3 mm are seen anterior to Bowman’s layer of the cornea
III. The Endothelium - Callous-like scar
- The endothelium is made up of closely interdigitated cells arranged in a mosaic pattern of - Dry eye, chronic blerpharitis and chronic allergic keratoconjunctivitis
mostly hexagonal shapes. - Symptoms: Redness, irritation, blurring of vision
- The Descemet membrane is the basement membrane of the corneal endothelium. It increases - Treatment: Rigid contact lenses, Superficial lamellar keratectomy
in thickness from 3 μm at birth to 10–12 μm in adults, as the endothelium gradually lays
down a posterior amorphous non-banded zone. E. Arcus Senilis
- Lipid droplets involve the entire corneal thickness
- The average adult cornea is 550 μm thick in the center, although there are racial variations, and - Most common peripheral corneal opacity
about 11.7 mm in diameter horizontally and 10.6 mm vertically - Hazy ring (2 mm)
- More concentrated in the superficial and deep layers, being relatively sparse in the corneal
Examination of Vision in the Patient with an Abnormal Cornea stroma.
A. External Examination - gross - Extremely common, frequently occurs without any predisposing systemic condition in elderly
B. Slit Lamp Biomicroscopy – biomicroscope is a binocular Galilean Telescope with multiple individuals but maybe associated with dyslipidaemia in younger patients
magnifications. - Prevalence strongly associated with age
C. Stains – Fluorescein, Rose Bengal and Lissamine Green - Associated with hypercholesterolemia and hypertriglyceridemia.
D. Impression Cytology - Blood lipid studies should be performed in affected individuals under age 50.
E. Corneal Pachymetry – measuring of corneal thickness; sensitive indicator of endothelial - Asymptomatic, no complications, no treatment is necessary
physiology
F. Keratometry - The ophthalmometer (keratometer) empirically estimates, but does notdirectly II. MISCELLANEOUS CORNEAL CONDITIONS
measure, the central corneal power. A. Inherited Corneal Dystrophies
1. Epithelial & Subepithelial
I. CORNEAL DEGENERATIONS - bilateral and symmetrical abnormal deposits of unknown cause
- Gradual breakdown of the cornea a. Epithelial Basement Membrane Dystrophy
- Results in gradual loss of vision - Patterns are seen at the level of the epithelial basement membrane
- Rare group of slowly progressing, bilateral degenerative disorder - Recurrent erosion is common
- Usually appear in the 2nd or 3rd decades of life - Vision usually is not significantly affected
- Hereditary, follow ocular inflammatory disease, and unknown cause - Autosomal dominant, onset is on the 2nd decade
- Over time pattern and distribution varies:
A. Terrien Disease a. Dot Like Opacities
- Bilateral symmetric degeneration b. Microcytes
- Marginal thinning of the upper nasal quadrants c. Map Like Patterns
- Peripheral vascularization with lipid deposition d. Whorled Fingerprint like Pattern
- Men are more commonly affected than women - Treatment: Same as that of corneal Erosions
- more frequently in the third and fourth decades
- Asymptomatic; Because the course of progression is slow and the central cornea is spared, the b. Meesman corneal Dystrophy
prognosis is reasonably good. - Tiny intraepithelial cysts of uniform size but variable density
- Complication®Perforation - Onset is early childhood
- Treatment : Tectonic Keratoplasty - Symptoms: Slight irritation, vision slightly affected
- a surgical procedure in which part or all of a damaged or diseased cornea is replaced - Juvenile hereditary epithelial dystrophy
by healthy corneal tissue from a donor - Inheritance is autosomal dominant
- Treatment: not normally required for lubrication

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2. Epithelial and Stromal b. Posterior Polymorphous Dystrophy
- These are due to mutations of the transforming growth factor beta-induced 1 (TGFB1) - Metaplasia of endothelial cells
gene. - Posterior border of cornea appear gray and nodular
a. Reis –Bücklers Dystrophy - Edema in deep stroma
- Opacity of the epithelium and Bowman’s layer - Associated with iris abnormalities, glaucoma and alport syndrome
- Haziness, relative sparing the periphery - Metaplasia of endothelial cells which seem to acquire some characteristics of
- Vision markedly reduced epithelial cell (stratified squamous epithelium)
- No vascularization is noted - The condition is asymptomatic in most cases, but in severe cases, epithelial and total
- Manifests in childhood as recurrent erosion stromal edema may occur.
- Inheritance is autosomal dominant - Onset in early childhood asymptomatic
- Grey-white geographical subepithelial opacities - Polymorphous plaques of calcium crystals are seen in deep stromal layers
- Corneal sensation is reduced. - Inheritance: autosomal dominant
- Treatment: directed at recurrent erosions. Excimer keratectomy in some - Treatment: not required

b. Lattice Dystrophy B. Corneal Ectasia
- Fine, linear, branching opacities - Progressive thinning of the paraxial cornea
- Histologic examination: Amyloid deposits in the collagen fibers - Stromal shape disorder, prevalent in ethnic group
- Inheritance: autosomal dominant TGFB1 - Bulging forward of the cornea
- Amyloid staining with congo red will exhibit green birefringence with a polarizing - Pathologically: affects Bowman layer, stroma and Descemet membrane
filter - Corneal ectatic disorders or corneal ectasia are a group of uncommon, noninflammatory, eye
- Symptoms: recurrent erosions occur at the end of the first decade in the classic disorders characterised by bilateral thinning of the central, paracentral, or peripheral cornea
form, when typical stromal signs may not yet be present - Accompanied by apical protrusion and irregular astigmatism
- Treatment penetrating or deep lamellar keratoplasty - It can be graded by the highest axis of corneal power of keratometry as mild (<48 D), moderate
(48-54D) severe >54D
c. Granular Dystrophy - Keratoconus is often slowly progressive and usually stabilizes in the fourth decade of life.
- Central, fine, whitish “granular” lesions in the stroma - Symptom: Blurred vision
- Histologically: Uniform deposition of hyaline material - Signs: Cone-shaped cornea,
- The epithelium and Bowman layer may be affected late in the disease. • Vogt’s Lines - Linear vertical narrow folds centrally in Descemet membrane;
- Asymptomatic, slowly progressive Pathognomonic
- Inheritance: autosomal dominant TGFB1 • Fleischer’s ring - an iron ring around the base of the cone
- Treatment: penetrating or deep lamellar keratoplasty. Superficial recurrences may • Munson’s sign - extreme cases, indentation of the lower lid by the cornea when the
require repeated excimer laser keratectomy patient looks down
- Acute hydrops of the cornea
3. Stromal - Sudden diminution of vision associated with central corneal edema due to rupture of
a. Macular Dystrophy Descemet Membrane
- Dense gray central opacity - Clears gradually without treatment but often leaves apical and Descemet Membrane
- Spread toward the periphery and later involves all depths of the stroma scarring
- Histologically: Deposition of acid mucopolysaccharide - Treatment
- Causes deterioration of the most sensitive part of the central retina (macula), which - Rigid contact lenses
has the highest concentration of light-sensitive cells - Trigger collagen cross-linking
- Inheritance: autosomal recessive - Surgery (deep lamellar or penetrating keratoplasty)
- Treatment: penetrating or deep keratoplasty (recurrence is common) - Corneal transplantation

4. Endothelial
a. Fuch’s Dystrophy REFERENCE:
- Death of cells in the endothelium ● Riordan-Eva, P., & James, A.J. (2018). Vaughan & Ausbury’s General Ophthalmology (19th ed.).
- Begins in the 3rd and 4th decade, affects women more and is slowly progressive China: McGraw-Hill Education.
- Gradually worsening blurring glare, and misting of vision (morning) ● Bowling, B. (2015). Kanski’s Clinical Ophthalmology (8th ed.). China: Elsevier.
- bilateral accelerated endothelial cell loss.
- More common in women and is associated with a slightly increased prevalence of
open angle glaucoma
- These cells normally pump fluid from the cornea to keep it clear.
- When they die, fluid builds up and the cornea gets swollen and puffy.
- Epithelial bullae or blisters
- Inheritance: mostly sporadic, some are autosomal dominant
- Corneal guttata: central, irregular, wart-like deposits/ excrescence on descemet
membrane.
- Treatment: conservative – 5% NaCl drops and reduction of IOP; surgical: posterior
lamellar and penetrating keratoplasty

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