Ординатура / Офтальмология / Английские материалы / Rapid Diagnosis in Ophthalmology Series Anterior Segment_Macsai, Machado Fontes_2007

Cornea • 5 SECTION

Salzmann Nodular Degeneration

Key Facts

•Occurs typically in eyes with chronic inflammation or dryness

•Unilateral or bilateral

•More common in women, usually asymptomatic (unless the visual axis is affected or astigmatism is induced)

•Associated with many diseases, such as:

•immune stromal keratitis • vernal keratoconjunctivitis • trachoma • herpetic keratitis • keratoconjunctivitis sicca • phlyctenular disease

Clinical Findings

•Single or multiple white, gray-white, or bluish elevated corneal lesions, often annular in location

•Each nodule is separated from other by clear cornea, and iron lines may be present

•More commonly adjacent to an area of corneal scarring, edema, and neovascularization

•Irregular astigmatism

Ancillary Testing

•Usually a clinical diagnosis (history and slit-lamp examination)

•Topography helpful in evaluating level of irregular astigmatism

Differential Diagnosis

•Corneal scar or keloid

•Band keratopathy

•Lipid keratopathy

•Conjunctival intraepithelial neoplasia

•Amyloidosis

•Epithelial basement membrane dystrophy

Treatment

•Lubrication

•Superficial or phototherapeutic keratectomy

•Rigid gas-permeable lenses for visual rehabilitation

•Lamellar or penetrating keratoplasty for severe scarring

Prognosis

•Epithelial erosions may overlie the lesion

•Corneal grafts have an excellent prognosis for survival, but recurrence has been reported

Fig. 5.58 Gray-white elevated Salzmann nodules adjacent to recurrent pterygium.

Fig. 5.59 Slit lamp: corneal opacifi cation secondary to Salzmann nodular degeneration.

Fig. 5.60 Numerous bluish nodules in an annular pattern. Note that each nodule is separated by a clear space.

Degeneration Nodular Salzmann

Cornea • 5 SECTION

Terrien Marginal Degeneration

Key Facts

•Slowly progressive bilateral peripheral corneal inflammatory and degenerative disorder

•Rare disease with unknown etiology

•More common in men (3 : 1 ratio) between 20 and 40 years of age

•Can begin in area of oblique pterygium

Clinical Findings

•Small white opacities in anterior stroma (usually in supranasal peripheral cornea)

•Superficial vascularization from limbal arcades

•Peripheral corneal thinning with an intact epithelium

•Lipid deposits can be seen in the advancing edge

•Against the rule astigmatism

Ancillary Testing

•Corneal topography

•Corneal pachymetry

Differential Diagnosis

•Idiopathic furrow degeneration

•Mooren ulcer

•Corneal dellen

•Pellucid marginal degeneration

Treatment

• No treatment to prevent disease from advancing

•Spectacles or contact lenses (usually rigid gas-permeable lenses give best vision)

•Eccentric lamellar or penetrating keratoplasty for resection of thinned area

Prognosis

•Thinning may progress circumferentially or be stationary

•In most cases, thinning does not progress to perforation

•Surgery generally unsatisfactory, with high postoperative complications (e.g. irregular astigmatism, neovascularization, and graft rejection)

Fig. 5.61 Superior corneal involvement in Terrien marginal degeneration: neovascularization, stromal thinning, and central lipid deposition.

Fig. 5.62 Peripheral corneal thinning. No associated ocular infl ammation.

Fig. 5.63 Terrien marginal degeneration beginning in an area of oblique pterygium. Peripheral corneal thinning with intact epithelium.

Degeneration Marginal Terrien

Cornea • 5 SECTION

Cornea Verticillata

Key Facts

•Consists of deposits in the corneal epithelium

•Usually seen as side effect of certain systemic medications or secondary to sphingolipidoses (such as Fabry disease)

•Related medications include:

•amiodarone • tamoxifen • atovaquone • chloroquine • chlorpromazine

•indomethacin

•In Fabry disease (X-linked recessive disease), female carriers can develop these corneal alterations and affected males can present with:

•periorbital edema • conjunctival vessel tortuosity and dilation • papilledema

•retina edema • optic atrophy • spoke-like posterior cataracts

•Amiodarone, tamoxifen, and chloroquine are reported to be associated with optic neuropathy and maculopathy

Clinical Findings

•Subepithelial or epithelial fine lines that swirl from a point below the center of the cornea and radiate to the periphery in a vortex-like pattern

Ancillary Testing

• Clinical diagnosis (history and slit-lamp examination)

Differential Diagnosis

•Fabry disease

•Multiple sulfatase deficiency

•Generalized gangliosidosis

•Multiple myeloma

•Medication deposit

•Striate melanokeratosis

•Iron depositions

•Anterior crocodile shagreen

Treatment

• Usually no ocular treatment is required

Prognosis

•Usually does not cause visual disturbances (rarely glare and haloes at night)

•Deposits generally reversible with discontinuation of medication

•In Fabry disease, the visual prognosis is excellent and death is usually secondary to renal, cardiac, or cerebrovascular disease

Cornea • 5 SECTION

Thygeson Superficial Punctate Keratitis

Key Facts

•Uncommon corneal epitheliopathy of unknown etiology

•Chronic condition with spontaneous remissions and transient exacerbations

•Usually bilateral disease

•No race or gender predilection in most reports

•Associated with HLA-DR3

•Usually diagnosed during second or third decade (range 2.5–85 years)

•Healing without corneal scarring, lack of response to antibiotics and antivirals

•Lesions entirely epithelial and do not incite neovascularization

Clinical Findings

•Discrete round or oval grouped punctate intraepithelial deposits, usually in central to paracentral cornea (range 1–50 lesions)

•Each deposit consists of numerous discrete, fine, granular, white to gray dot-like opacities

•Mild epithelial and subepithelial edema

•Lesions slightly elevated during exacerbations

• Epithelium between lesions is normal • Little or no conjunctival hyperemia

Ancillary Testing

• Clinical diagnosis (history and slit-lamp examination)

Differential Diagnosis

• Staphylococcal inflammatory marginal keratitis

•Seborrheic blepharitis

•Keratoconjunctivitis sicca or exposure keratopathy

•Herpetic keratitis

•Recurrent erosion syndrome

•Keratitis secondary to blepharitis, meibomian gland dysfunction, or contact lenses

Treatment

•Low-dose topical steroids (during exacerbations)

•Topical non-steroidal anti-inflammatory drugs may play a role

•Therapeutic (bandage) contact lenses

•Topical cyclosporin A 2%

•Tinted lenses during exacerbations

Prognosis

•Visual prognosis is good (non–sight-threatening condition)

•Typically the more spots seen, the more symptomatic the patient

•The disease usually has a chronic course and can last more than a decade, but signs and symptoms often resolve spontaneously

Fig. 5.67

Epitheliopathy: discrete, grouped round and oval deposits in central cornea with mild surrounding edema.

Fig. 5.68 Detail of a single elevated lesion. Negative staining with fluorescein dye.

Fig. 5.69 Another example of negative staining. Bilateral disease with no neovascularization or conjunctival injection.

Fig. 5.70 Thygeson superfi cial punctate keratitis: note that the epithelium between lesions is normal.

Keratitis Punctate Superficial Thygeson

Cornea • 5 SECTION

Band Keratopathy

Key Facts

•Chronic degenerative condition with calcium deposition at Bowman’s membrane, epithelial basement membrane, and anterior corneal stroma

•Typically seen in eyes with chronic inflammation

•Can be associated with systemic diseases

Clinical Findings

•Grayish opacification of cornea, typically beginning in peripheral cornea (at 3 and 9 o’clock positions)

•Most frequently seen in interpalpebral zone, separated from the limbus by a clear area

•Swiss cheese appearance due to scattered holes inside the band, where corneal nerves penetrate through Bowman’s layer

•A fibrous pannus may separate the epithelium and Bowman’s layer

Ancillary Testing

•Clinical diagnosis (history and slit-lamp examination)

•Systemic work-up

Differential Diagnosis

•Arcus senilis

•Limbal girdle of Vogt

•Salzmann nodular degeneration

•Spheroidal degeneration

Treatment

•Ocular lubrication

•Disodium EDTA chelation (0.05 molar concentration)

•Phototherapautic keratectomy with excimer laser

•Lamellar keratoplasty

Prognosis

•Usually asymptomatic unless extends to visual axis

•Generally slow progression (dry eye can exacerbate the disease)

•Accumulation of calcium can disrupt the ocular surface, causing irritation, photophobia, or recurrent erosions

•Recurrences after treatment are somewhat common, especially when underlying causative condition persists