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Chapter 6. Cornea
Roderick Biswell, MD Sections in this chapter: Cornea: Introduction Corneal Ulceration Epithelial Keratitis Degenerative Corneal Conditions Miscellaneous Corneal Disorders Corneal Pigm entation Contact Lenses Corneal Transplantation Refractive Corneal Surgery References SEE ALSO on AccessMedicine - band -shaped keratopathy - corneal diseases - labrador keratopathy -m acular dystrophy

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DEGENERATIVE CORNEAL CONDITIONS

KERATOCONUS
Keratoconus is an uncomm on degenerative bilateral disease that m ay be inherited as an autosom al recessive or autosomal dom inant trait. Unilateral cases of unknown cause occur rarely. Sym ptoms appear in the second decade of life. The disease affects all races. Keratoconus has been associated with a num ber of diseases, including Down's syndrom e, atopic derm atitis, retinitis pigm entosa, aniridia, vernal catarrh, Marfan's syndrom e, Apert's syndrom e, and Ehlers-Danlos syndrom e. Pathologically, there are disruptive changes in Bowm an's layer with keratocyte degeneration and ruptures in Descem et's m em brane. Blurred vision is the only sym ptom . Many patients present with rapidly increasing m yopic astigm atism . Signs include cone-shaped cornea Figure ( 6 11 ); linear narrow folds centrally in Descem et's m embrane (Vogt's lines), which are pathognom onic; an iron ring around the base of the cone (Fleischer's ring); and, in extreme cases, indentation of the lower lid by the cornea when the patient looks down (Munson's sign). There is an irregular or scissor reflex on retinoscopy and a distorted corneal reflection with Placido's disk or the keratoscope even early in the disease. Colorcoded topography provides more accurate inform ation on the degree of corneal distortion Figure ( 2 25 ). Generally, the fundi cannot be clearly seen because of corneal astigmatism.
Figure 6 11. Keratoconus. Add to 'My Saved Images'

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Acute hydrops of the cornea m ay occur, manifested by sudden diminution of vision associated with central corneal edem a. This arises as a consequence of rupture of Descem et's m em brane and m ay be triggered by the patient rubbing the eye. The condition may be m istaken for extreme thinning with im pending perforation. Acute hydrops usually clears gradually without treatm ent but often leaves apical and Descemet m em brane scarring. Rigid contact lenses will m arkedly im prove vision in the early stages by correcting irregular astigm atism . Keratoconus is one of the m ost comm on indications for penetrating keratoplasty. Surgery is indicated when a contact lens can no longer be effectively worn or when peripheral thinning will affect the surgery. Keratoconus is often slowly progressive between the ages of 20 and 60, although

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an arrest in progression of the keratoconus m ay occur at any tim e. If a corneal transplant is done before extrem e corneal thinning occurs, the prognosis is excellent; about 8095% obtain good best-corrected sight.

CORNEAL DEGENERATION
The corneal degenerations are a rare group of slowly progressive, bilateral, degenerative disorders that usually appear in the second or third decades of life. Som e are hereditary. Other cases follow ocular inflam matory disease, and som e are of unknown cause.

Marginal Degeneration of the Cornea

TERRIEN'S DISEASE Terrien's disease is a rare bilateral sym m etric degeneration characterized by m arginal thinning of the upper nasal quadrants of the cornea. Males are m ore com m only affected than fem ales, and the condition occurs more frequently in the third and fourth decades. There are no sym ptom s except for m ild irritation during occasional inflam m atory episodes, and the condition is slowly progressive. The clinical picture consists of marginal thinning and peripheral vascularization with lipid deposition. Perforation is a known com plication, especially from traum a. Tectonic (structural) keratoplasty m ay be required. Histopathologic studies of affected corneas have revealed vascularized connective tissue with fibrillary degeneration and fatty infiltration of collagen fibers. Because the course of progression is slow and the central cornea is spared, the prognosis is good. BAND (CALCIFIC) KERATOPATHY Band keratopathy is characterized by the deposition of calcium salts in alike bandpattern in the anterior layers of the cornea Figure ( 6 12 ). The keratopathy is usually lim ited to the interpalpebral area. The calcium deposits are noted in the basem ent m embrane, Bowm an's layer, and anterior strom al lam ellas. A clear m argin separates the calcific band from the lim bus, and clear holes m ay be seen in the band, giving the Swiss cheese appearance. Sym ptom s include irritation, injection, and blurring of vision.
Figure 6 12. Add to 'My Saved Images' Calcific band keratopathy. (Courtesy of M Hogan.)

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Calcific band keratopathy has been described in a num ber of inflam m atory, m etabolic, and degenerative conditions. It is characteristically associated with juvenile rheum atoid arthritis. It has been described in longstanding inflam m atory conditions of the eye, glaucom a, and chronic cyclitis. Band keratopathy m ay also be associated with hyperparathyroidism , vitam in D intoxication, sarcoidosis, and leprosy. Treatm ent consists of rem oval of the corneal epithelium by curettage under topical anesthesia followed by irrigation of the cornea with a sterile 0.01m olar solution of ethylenediam inetetraacetic acid (EDTA; edetate calcium ) or application of EDTA with a cotton applicator. The excim er laser has shown particular value in the treatment of band keratoplasty, or the band can be rem oved surgically.

Climatic Droplet Keratopathy (Labrador Keratopathy, Spheroid Degeneration of the Cornea)

Clim atic droplet keratopathy affects m ainly men who work out of doors Figure (6 13 ). The corneal degeneration is thought to be caused by exposure to ultraviolet

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light and is characterized in the early stages by fine subepithelial yellow droplets in the peripheral cornea. As the disease advances, the droplets becom e central, with subsequent corneal clouding causing blurred vision. Treatm ent in advanced cases is by corneal transplantation.
Figure 6 13. Add to 'My Saved Images' Two photos showing climatic droplet (Labrador) keratodystrophy. Inset at left shows slitlam p view. (Photo at left courtesy of A Ahm ad.) View Large

Salzmann's Nodular Degeneration

This disorder is usually preceded by corneal inflam mation, particularly phlyctenular keratoconjunctivitis or trachom a. Sym ptoms include redness, irritation, and blurring of vision. There is degeneration of the superficial cornea that involves the strom a, Bowm an's layer, and epithelium with superficial whitishgray elevated nodules som etim es occurring in chains. Corneal transplantation is rarely required; superficial lam ellar keratectomy or phototherapeutic (laser) keratectom y can result in visual im provem ent. Rigid contact lenses will significantly im prove visual acuity in m ost cases.

ARCUS SENILIS (CORNEAL ANNULUS, ANTERIOR EMBRYOTOX ON)

Arcus senilis is an extrem ely comm on, bilateral, benign peripheral corneal degeneration that m ay occur at any age but is far more com mon in elderly people as part of the aging process. Arcus senilis in people under age 50 is often associated with hypercholesterolemia; blood lipid studies should be done. Pathologically, lipid droplets involve the entire corneal thickness but are m ore concentrated in the superficial and deep layers, being relatively sparse in the corneal stroma. There are no symptom s. Clinically, arcus senilis appears as a hazy gray ring about 2m m in width and with a clear space between it and the lim bus Figure ( 6 14 ). No treatm ent is necessary, and there are no com plications.
Figure 6 14. Add to 'My Saved Images' Arcus senilis. (Photo by Diane Beeston.)

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HEREDITARY CORNEAL DYSTROPHIES

This is a group of rare hereditary disorders of the cornea of unknown cause characterized by bilateral abnorm al deposition of substances and associated with alteration in the normal corneal architecture that m ay or may not interfere with vision. These corneal dystrophies usually manifest them selves during the first or second decade but som etim es later. They m ay be stationary or slowly progressive throughout life. Corneal transplantation, when indicated, im proves vision in m ost patients with hereditary corneal dystrophy. Anatomically, corneal dystrophies m ay be classified as epithelial, strom al, and posterior lim iting m em brane dystrophies.

Epithelial Corneal Dystrophies

MEESMAN'S DYSTROPHY This slowly progressive disorder is characterized by m icrocystic areas in the

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epithelium. The onset is in early childhood (first 12 years of life). The m ain sym ptom is slight irritation, and vision is slightly affected. The inheritance is autosom al dom inant. ANTERIOR MEMBRANE DYSTROPHIES (COGAN'S, MAP-DOTFINGERPRINT) Map or fingerprint patterns are seen at the level of the epithelial basem ent m embrane. Debris, cysts, and dots also m ay be noted. Recurrent erosion is com m on. Vision usually is not significantly affected. In Cogan's dystrophy, intraepithelial opacities are seen in the pupillary area. OTHERS Reis-Bcklers dystrophy is a dom inantly inherited dystrophy affecting prim arily Bowman's layer. The disease begins within the first decade of life with symptom s of recurrent erosion. Opacification of Bowm an's layer gradually occurs and the epithelium is irregular. No vascularization is usually noted. Vision m ay be m arkedly reduced. Vortex dystrophy, or cornea verticillata, is characterized by pigm ented lines occurring in Bowm an's layer or the underlying strom a and spreading over the entire corneal surface. Visual acuity is not m arkedly affected. Such a pattern of radiating pigm ented lines may also be seen in patients treated with chlorprom azine , chloroquine , indom ethacin , oram iodaroneas well as in Fabry's disease.

Stromal Corneal Dystrophies

There are three prim ary types of stromal corneal dystrophies: GRANULAR DYSTROPHY This usually asymptom atic, slowly progressive corneal dystrophy m ost often begins in early childhood. The lesions consist of central, fine, whitish "granular" lesions in the strom a of the cornea. The epithelium and Bowm an's layer may be affected late in the disease. Visual acuity is slightly reduced. Histologically, the cornea shows uniform deposition of hyaline material. Corneal transplant is not needed except in very severe and late cases. The inheritance is autosom al dominant. MACULAR DYSTROPHY This type of strom al corneal dystrophy is m anifested by a dense gray central opacity that starts in Bowm an's layer. The opacity tends to spread toward the periphery and later involves the deeper strom al layers. Recurrent corneal erosion m ay occur, and vision is severely impaired. Histologic exam ination shows deposition of acid mucopolysaccharide in the strom a and degeneration of Bowman's layer. Penetrating keratoplasty is often required. The inheritance is autosomal recessive. LATTICE DYSTROPHY Lattice dystrophy starts as fine, branching linear opacities in Bowm an's layer in the central area and spreads to the periphery. The deep strom am ay becom e involved, but the process does not reach Descemet's m em brane. Recurrent erosion m ay occur. Histologic exam ination reveals am yloid deposits in the collagen fibers. Penetrating keratoplasty is com m on, as is recurrence of the dystrophy in the graft. The hereditary pattern for lattice dystrophy is autosom al dominant.

Posterior Corneal Dystrophies

FUCHS' DYSTROPHY This disorder begins in the third or fourth decade and is slowly progressive

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throughout life. W omen are more com monly affected than m en. There are central wart-like deposits on Descem et's m em brane, thickening of Descemet's m embrane, and defects of size and shape of the endothelial cells. Decom pensation of the endothelium occurs and leads to edem a of the corneal strom a and epithelium , causing blurring of vision. Corneal haze is slowly progressive. Histologic examination of the cornea reveals the wart-like excrescences over Descem et's m em brane that are secreted by the endothelial cells. Thinning and pigm entation of the endothelium and thickening of Descem et's m embrane are characteristics. Penetrating keratoplasty, often com bined with extracapsular lens extraction and a posterior lens im plant, is often needed. Cataract surgery alone can trigger endothelial decom pensation in advanced disease. POSTERIOR POLYMORPHOUS DYSTROPHY This is a com m on disorder with onset in early childhood. Polym orphous plaques of calcium crystals are observed in the deep strom al layers. Vesicular lesions m ay be seen in the endothelium . Edema occurs in the deep strom a. The condition is asym ptom atic in m ost cases, but in severe cases epithelial and total strom al edem a may occur. The inheritance is autosom al dom inant. < Previous Section| Next Section >

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