EYE ANTERIOR STRUCTURES PATHLOGIES

AND DISORDERS

FielmannOptometristIrina Jagiloviche-mail: irina.jagilovica@gmail.com

DISORDERS OF LASHES

1. Trichiasis

2. Metaplastic lashes

3. Distichiasis

4. Phthiriasis palpebrarum

6. Poliosis

5. Madarosis

MadarosisDecrease in number or complete loss of lashes

Local causes

• Chronic anterior lid margin disease

• Infiltrating tumours

• Burns, radiotherapy or cryotherapy

PoliosisPremature localized whitening of hair

Ocular associations

Chronic anterior blepharitis

Sympathetic ophthalmitis

Systemic associations

Vogt-Koyanagi-Harada syndrome

Waardenburg syndrome

Distichiasis

•Second row of lashes arising from meibomian gland orifices

•Congenital

Trichiasis

Most frequently affects lower lidof normal lashes

Most frequently affects lower lid

Inferior punctate epitheliopathy

Phthiriasis palpebrarum

Infestation of lashes by pubic crab louse and its ova (nits)

Typically affects children in poor hygenic conditions

Staphylococcal blepharitis

Chronic irritation worse in morningsScales around base of lashes (collarettes)

Hyperaemia and telangiectasia of anterior lid marginScarring and hypertrophy if longstanding

Complications of staphylococcal blepharitis

poliosis madarosis trichiasis Recurrent styes

Marginal keratitis Tear film instability

Seborrhoeic blepharitis

Shiny anterior lid margin

Hyperaemia of lid margin

Greasy scales

Lashes stuck together

Meibomianitis

Meibomian cyst formation

Inflamed and blocked meibomian gland orifices

Meibomian seborrhoea

Oil globules over meibomian gland orifices Oily and foamy tear film

Herpes simplex

Signs• Crops of small vesicles• Rupture and crust• Heal without scarring after 7 days

Complications • Follicular conjunctivitis • Keratitis

Treatment - topical antivirals

BENIGN EYELID LESIONS1. Nodules

• Chalazion • Acute hordeola

2. Cysts

•

• Cyst of Moll• Cyst of Zeiss• Sebaceous cyst• Hidrocystoma

3. Tumours

Xanthelasma

• Viral wart• Keratoacanthoma• Naevi• Capillary haemangioma• Port-wine stain• Pyogenic granuloma• Cutaneous horn

Molluscum contagiosum•

Signs of chalazion (meibomian cyst)

Painless, roundish, firm lesion within tarsal plate

May rupture through conjunctiva and cause granuloma

Molluscum contagiosum

Painless, waxy, umbilicated nodule

May be multiple in AIDS patients

Chronic follicular conjunctivitis

Occasionally superficial keratitis

Xanthelasma

Common in elderly or those with hypercholesterolaemia

Yellowish, subcutaneous plaques containing cholesterol and lipid

Usually bilateral and located medially

Eyelid cystsCyst of Moll

Cyst of Zeis

Translucent

On anterior lid margin

On anterior lid margin

Opaque

Viral wart (squamous cell papilloma)

Most common benign lid tumour

Raspberry-like surface

KeratosesSeborrhoeic Actinic

Common in elderly

Discrete, greasy, brown lesion

Flat ‘stuck-on’ appearance

Affects elderly, fair-skinned individuals

Most common pre-malignant skin lesion

Rare on eyelids

Flat, scaly, hyperkeratotic lesion

Keratoacanthoma

Uncommon, fast growing nodule

Acquires rolled edges and keratin-filled crater

Involutes spontaneously within 1 year

Naevi

Elevated

May be non-pigmented

No malignant potential

Flat, well-circumscribed

Pigmented

Low malignant potential

Has both intradermal and junctional components

MALIGNANT EYELID TUMOURS

1. Basal cell carcinoma

2. Squamous cell carcinoma

3. Meibomian gland carcinoma

4. Melanoma

5. Kaposi sarcoma

Basal Cell Carcinoma - Important Facts

1. Most common human malignancy

2. Usually affects the elderly

3. Slow-growing, locally invasive

5. 90% occur on head and neck

6. Of these 10% involve eyelids

7. Accounts for 90% of eyelid malignancies

4. Does not metastasize

Nodular basal cell carcinoma

Shiny, indurated nodule

Surface vascularization

Slow progression

May destroy large portion of eyelid

Ulcerative basal cell carcinoma(rodent ulcer)

Chronic ulceration

Sclerosing basal cell carcinoma

Indurated plaque with loss of lashes

May mimic chronic blepharitis

Spreads radially beneath normal epidermis

Squamous cell carcinoma

Nodular Ulcerative

Less common but more aggressive than BCC

No surface vascularization

Meibomian gland carcinoma

Hard nodule; maymimic chalazion

Diffuse thickening of lid margin and loss of lashes

Very large tumour

Conjunctival invasion; maymimic chronic conjunctivitis

Melanoma

Blue-black nodule withnormal surrounding skin

May be non-pigmented

Plaque with irregular outline

Variable pigmentation

Kaposi sarcoma

Vascular tumour occurring in patients with AIDS

Usually associated with advanced disease

Very sensitive to radiotherapy

Entropion & ectropion

Horner syndrome

Caused by oculosympathetic palsy

Usually unilateral mild ptosis and miosis, anisocoria

Normal pupillary reactions

Important causes of Horner syndromeCentral(first order neurone)

• Brainstem disease (vascular, demyelination)• Spinal cord disease (syringomyelia, tumours)

Pre-ganglionic (second order neurone)

• Intrathoracic lesions (Pancoast tumour, aneurysm)

• Neck lesions (glands, trauma)

Post-ganglionic (third order neurone)

• Internal carotid artery disease• Cavernous sinus mass

Posterior hypothalamus

Ciliospinal centre of Budge( C8 - T2 )

Superior cervicalganglion

Simple bacterial conjunctivitis

Crusted eyelids and conjunctival injection

Subacute onset of mucopurulent discharge

Signs of conjunctivitis

Usually bilateral, acute waterydischarge and follicles

Subconjunctival haemorrhages andpseudomembranes if severe

Signs of keratitis

Focal, epithelial keratitis Focal, subepithelial keratitis

May persist for months Transient

Progression of vernal conjunctivitis

Diffuse papillary hypertrophy, most marked on superior tarsus

Formation of cobblestone papillae

Limbal vernal

Trantas dotsMucoid nodule

1. Benign• Naevus• Papilloma• Epibulbar dermoid• Lipodermoid

2. Pre-malignant• Primary acquired melanosis ( PAM )• Intraepithelial neoplasia (carcinoma in situ)

3. Malignant• Melanoma• Squamous cell carcinoma• Kaposi sarcoma• Lymphoma

CONJUNCTIVAL TUMOURS

Naevus

30% are almost non-pigmented

Most frequently juxtalimbalSharply demarcated and slightly elevated•

Presents in first two decades

Melanoma

PapillomaPedunculated Sessile

• Presents in middle age• Not caused by infection• Single and unilateral

• Presents in childhood or early adulthood• Infection with papilloma virus • May be multiple and bilateral

• Presents in childhood• Smooth, soft mass• Usually juxtalimbal

• Occasionally Goldenhar syndrome

Epibulbar dermoidSigns Association

Lipodermoid

• Presents in adulthood• Soft, movable, subconjunctival mass• Most frequently at outer canthus

Intraepithelial neoplasia(carcinoma in situ)

• Juxtalimbal fleshy avascular mass

• May become vascular and extend onto cornea• Presents in late adulthood• Malignant transformation is uncommon

Signs Progression

Primary acquired melanosis (PAM)

PAM without atypia is benign

PAM with atypia is pre-malignant Unilateral, irregular areas of flat, brown pigmentation

May involve any part of conjunctiva

Presents in late adulthood

Signs Types

Conjunctival melanoma

From PAM with atypia

Sudden appearance of nodules in PAM

From naevus

Sudden increase in size or pigmentation

Primary

Solitary nodule

Frequently juxtalimbal but may be anywhere•

Very rareMost common type

Squamous cell carcinoma

• Rarely metastasizes

• Arises from intraepithelial neoplasia or de novo

• Frequently juxtalimbal

• Slow-growing

• Presents in late adulthood• May spread extensively

Signs Progression

Kaposi sarcoma

• Most frequently in inferior fornix

• Affects patients with AIDS• Vascular, slow-growing tumour of low malignancy• Very sensitive to radiotherapy

Lymphoma

• Salmon-coloured, subconjunctival infiltrate

• Usually presents in adulthood• Benign or malignant

PERIPHERAL CORNEAL INFLAMMATION

1. Marginal keratitis

2. Rosacea keratitis

3. Phlyctenulosis

4. Acute stromal keratitis

Marginal keratitis

Subepithelial infiltrate separated by clear zone

Circumferential spread Vascularization followed by resolution

Progression

Rosacea keratitis

Peripheral inferiorvascularization

Subepithelial infiltration Thinning and perforation if severe

• Affects 5% of patients with acne rosaeca• Bilateral and chronic

Progression

Phlyctenulosis

• Small pinkish-white nodule near limbus• Usually transient and resolves spontaneously

• Starts astride limbus• Resolves spontaneously or extends onto cornea

• Uncommon, unilateral - typically affects children• Severe photophobia, lacrimation and blepharospasm

Conjunctival phlycten Corneal phlycten

Acute stromal keratitis

Superficial or mid-stromal infiltration Opacification and vascularization

• Uncommon, usually unilateral• Associated with non-necrotizing scleritis

Progression

CORNEAL INFECTIONS

1. Bacterial keratitis

2. Fungal keratitis

3. Acanthamoeba keratitis

4. Infectious crystalline keratitis

5. Herpes simplex keratitis-Epithelial-Disciform

6. Herpes zoster keratitis

Bacterial keratitis• Contact lens wear• Chronic ocular surface disease• Corneal hypoaesthesia

Expanding oval, yellow-white, dense stromal infiltrate

Stromal suppuration and hypopyon

Fungal keratitis

Frequently preceded by ocular trauma with organic matter

Greyish-white ulcer which may be surrounded by feathery infiltrates

Slow progression and occasionally hypopyon

Acanthamoeba keratitis• Contact lens wearers at particular risk• Symptoms worse than signs

Small, patchy Anterior stromal infiltrates

Perineural infiltrates (radial keratoneuritis)

Ulceration, ring abscess& small, satellite lesions

Stromal opacification

Treatment

Infectious crystalline keratitis• Very rare, indolent infection (Strep. viridans)

• Particularly following penetrating keratoplasty

White, branching, anterior stromal crystalline deposits

• Usually associated with long-term topical steroid use

Herpes simplex epithelial keratitis

• Dendritic ulcer with terminal bulbs

• Stains with fluorescein

• May enlarge to become geographic

Herpes simplex disciform keratitis

Central epithelial and stromal oedema

Folds in Descemet membrane

Small keratic precipitates

Occasionally surrounded by Wessely ring

Signs Associations

Herpes zoster keratitis

Develops in about 50% within 2 days of rash

Small, dendritic or stellate epithelial lesions

Tapered ends without bulbs

Resolves within a few days

Develops in about 30% within 10 days of rash

Multiple, fine, granular deposits just beneath Bowman membrane

Halo of stromal haze

Nummular keratitisAcute epithelial keratitis

May become chronic

CORNEAL DEGENERATIONS

1. Age-related• Arcus senilis• Vogt white limbal girdle

2. Lipid keratopathy

• Crocodile shagreen • Cornea guttata

• Primary• Secondary

3. Band keratopathy

4. Spheroidal degeneration5. Salzmann nodular degeneration

Arcus senilis• Innocuous and extremely common in elderly

• Occasionally associated with hyperlipoproteinaemia

• Bilateral, circumferential bands of lipid deposits

• Diffuse central and sharp peripheral border

• Peripheral border separated from limbus by clear zone

• Clear zone may be thinned ( senile furrow)

Vogt white limbal girdle

Innocuous and very common in elderly

Bilateral

White, crescentic line along nasal and temporal limbus

Type 1 - separated from limbus by clear zone

Type 2 - not separated by clear zone

Crocodile shagreen

• Uncommon and innocuous

• Usually bilateral

Polygonal stromal opacities separated by clear space Most frequently involve anterior stroma (anterior crocodile shagreen)Occasionally involve posterior stroma (posterior crocodile shagreen)

Cornea guttata• Common, bilateral and usually innocuous• Rarely progression to Fuchs dystrophy

Tiny dark spots on central endothelium

Lipid keratopathy

Usually unilateral stromal deposits without vascularization

Rare, occurs spontaneously in avascular cornea

Unilateral stromal deposits with vascularization

Common, secondary to previous disciform keratitis

Primary Secondary

Band keratopathy

Interpalpebral limbal opacification

• Common, unilateral or bilateral depending on cause

• Subepithelial calcification

Central spread of calcification

Small holes within calcified area Separated by clear zone

Progression

CORNEAL DYSTROPHIES1. Anterior

• Cogan microcystic• Reis-Bucklers• Meesmann• Schnyder

2. Stromal• Lattice I, II, III• Granular I, II, III (Avellino)• Macular

3. Posterior• Fuchs endothelial• Posterior polymorphous

..

Cogan microcystic dystrophy• Most common of all dystrophies• Neither familial nor progressive• Recurrent corneal erosions in about 10% of cases

Dots

Fingerprints

Cysts

Maps

MicrocystsDots

Maps Fingerprints

Signs of Cogan dystrophy

Onset - early childhood with recurrent corneal erosions

Superficial polygonal opacities Honeycomb appearance

keratoplasty if severe

Reis-Bucklers dystrophy..

Meesmann dystrophy

Onset - first decade with mild visual symptoms

Treatment - not required

• Clear in retroillumination• Grey in direct illumination

• Tiny, epithelial cysts, maximal centrally

Onset - second decade with visual impairment

Treatment - excimer laser keratectomy

Schnyder dystrophy

Subepithelial ‘crystalline’ opacities

Onset - late first decade with recurrent corneal erosions

Treatment - penetrating keratoplasty if severe

Lattice dystrophy type I

Fine, spidery, branching lines within stroma

Later general haze may submerge lesions

Progression

Onset - first decade with recurrent corneal erosions

Treatment - penetrating keratoplasty if severe

Granular dystrophy type I

Eventual confluenceInitial superficial andcentral crumb-like opacities

Later deeper and peripheral spread but limbus spared

Progression

Onset - old age

Treatment - penetrating keratoplasty if advanced

Fuchs endothelial dystrophy

Eventually bullous keratopathy

Later central stromal oedema

Gradual increase in cornea guttata with peripheral spread

Progression

1. Keratoconus

2. Keratoglobus

3. Pellucid marginal degeneration

CORNEAL ECTASIAS

Morphological classification of keratoconus

Nipple cone Oval cone Globus cone

Small and steep curvature Larger and ellipsoidal Largest

Signs of keratoconusBilateral in 85% but asymmetrical

Oil droplet reflex Prominent corneal nervesVogt striae

Acute hydrops Munson sign Fleischer ring & scarring

Bulging of lower lids on downgaze

Keratoglobus

Bilateral protrusion and thinning of entire cornea Associations - Leber congenital amaurosis and blue sclera

Onset usually at birth

Pellucid marginal degeneration

• Bilateral crescent-shaped inferior corneal thinning

• Onset between 20 and 40 years

EPISCLERITIS AND SCLERITIS

1. Episcleritis• Simple• Nodular

2. Anterior scleritis• Non-necrotizing diffuse • Non-necrotizing nodular• Necrotizing with inflammation• Necrotizing without inflammation ( scleromalacia perforans )

3. Posterior scleritis

Applied anatomy of vascular coats

Scleritis

• Maximal congestion of deep vascular plexus

• Slight congestion of episcleral vessels

• Maximal congestion of episcleral vessels

EpiscleritisNormal

• Radial superficial episcleral vessels• Deep vascular plexus adjacent to sclera

Simple episcleritis• Common, benign, self-limiting but frequently recurrent

• Typically affects young adults

• Seldom associated with a systemic disorder

Simple sectorial episcleritis Simple diffuse episcleritis

Nodular episcleritis• Less common than simple episcleritis

• May take longer to resolve

• Treatment - similar to simple episcleritis

Localized nodule which can be moved over sclera

Deep scleral part of slit-beam not displaced

Diffuse anterior non-necrotizing scleritis

• Widespread scleral and episcleral injection

• Relatively benign - does not progress to necrosis

Complications of uveitis

Posterior synechiae - 30% Cataract -20%

Glaucoma due to PAS - 15% Band keratopathy - 10%

UVEAL TUMOURS 1. Iris melanoma

2. Iris naevus

6. Choroidal haemangioma

7. Choroidal metastatic carcinoma

8. Choroidal osseous choristoma

• Circumscribed• Diffuse

9. Melanocytoma

3. Ciliary boy melanoma

4. Choroidal melanoma

5. Choroidal naevus

Iris Melanoma

1. Very rare - 8% of uveal melanomas

2. Presentation - fifth to sixth decades

3. Very slow growth

4. Low malignancy

5. Excellent prognosis

Iris melanoma

Usually pigmented nodule at least 3 mm in diameter Invariably in inferior half of iris

Occasionally non-pigmented Surface vascularization

Angle involvement may cause glaucoma

Pupillary distortion, ectropion uveae and cataract

Differential diagnosis of iris melanoma

Large iris naevus distorting pupil Leiomyoma

Adenoma of pigment epithelium

Primary iris cyst Ciliary body melanoma eroding iris root

Metastasis to iris

Iris naevusTypical Diffuse

• Diameter usually less than 3 mm• Occasionally mild distortion of pupil an ectropion uvea

• May cause ipsilateral hyperchromic heterochromia

• May be associated with Cogan-Reese syndrome

• Pigmented, flat or slightly elevated

Ciliary body melanoma

• Rare - 12% of uveal melanomas

• Presentation - 6th decade

• May be discovered by chance

• Prognosis - guarded

Anterior uveitis

Symptoms: acute iritis causes a sudden onset of unilateral photophobia, redness, pain and blurred vision. In chronic anterior uveitis symptoms may initially be absent or mild. Signs: Circumcorneal injection and a small pupil in acute iritis. Keratic precipitates may be small in acute iritis or large and mutton fat in granulomatous inflammation. Hypopyon and a fibrinous exudate.

Anterior uveitis

Posterior synechiae Hypopyon

Acute anterior uveitis in young adults

Fibrinous exudate Residual pigment on lens

• Majority are men

• Initially no systemic disease

• Minority subsequently develop ankylosing spondylitis

• 45% are positive for HLA-B27

IMPORTANT SYSTEMIC ASSOCIATIONS OF UVEITIS1. Spondylarthropathies

• Systemic features• Ocular features

2. Juvenile idiopathic arthritis

3. Sarcoidosis

4. Behçet disease5

5. Vogt-Koyanagi-Harada syndrome5

7. Tubulointerstitial nephritis

6. Inflammatory bowel disease• Ulcerative colitis• Crohn disease

• Systemic features

• Ocular features

ACQUIRED CATARACT

• Morphological1. Classification of age-related cataract

• According to maturity

• Diabetes• Myotonic dystrophy• Atopic dermatitis• Trauma

2. Other causes of cataracts

3. Surgery

• Secondary (complicated)

• Large incision extracapsular extraction• Phacoemulsification

• Drugs

Classification of Age-related Cataract According to Morphology

1. Subcapsular• Anterior• Posterior

2. Nuclear

3. Cortical

4. Christmas tree

Subcapsular cataract

Anterior Posterior

Nuclear cataract

• Exaggeration of normal nuclear ageing change• Causes increasing myopia

• Increasing nuclear opacification

• Initially yellow then brown

Progression

Cortical cataract

Initially vacuoles and clefts Progressive radial spoke-like opacities

Progression

Christmas tree cataract

Polychromatic, needle-like opacities May co-exist with other opacities

Classification according to maturity

Immature Mature

Hypermature Morgagnian

Other causes of cataract - diabetesJuvenile

• White punctate or snowflake posterior or anterior opacities

• May mature within few days

Adult

• Cortical and subcapsular opacities• May progress more quickly than in non-diabetics

Other causes of cataract - atopic dermatitis

• Cataract develops in 10% of cases between 15-30 years

• Bilateral in 70% • Frequently becomes mature

• Anterior subcapsular plaque (shield cataract)

• Wrinkles in anterior capsule

Causes of traumatic cataract

Penetration

Concussion

‘Vossius’ ring from imprinting of iris pigment Flower-shaped

• Ionizing radiation

• Electric shock

• Lightning

Other causes

DrugsChlorpromazine

• Long-acting mioticsOther drugs

• Amiodarone

• Busulphan

- initially posterior subcapsularSystemic or topical steroids

- central, anterior capsular granules

Secondary (complicated) cataract

• Chronic anterior uveitis

• High myopia

Posterior subcapsular

• Hereditary fundus dystrophies• Central, anterior subcapsular opacities

Glaukomflecken

• Follows acute angle- closure glaucoma

LenticonusPosterior

• Posterior axial bulge• Unilateral - usually sporadic• Bilateral - familial or in Lowe syndrome

Anterior

• Anterior axial bulge

• Associated with Alport syndrome

ECTOPIA LENTIS

1. Acquired

2. Isolated familial ectopia lentis

3. Associated with systemic syndromes• Marfan syndrome

• Weill-Marchesani syndrome

• Homocystinuria

4. Treatment options

Acquired ectopia lentisTrauma

• Buphthalmos• Megalocornea

Anterior uveal tumours Degenerate eye

Stretched zonules

Ocular features of Marfan syndrome

Upward subluxation

Zonule usually intact Axial myopia

Blue scleraCornea planaAngle anomaly and glaucoma

Lattice degeneration

Weill-Marchesani syndromeSystemic features

Short stature

Ocular features

Short stubby fingers (brachydactyly)

Mental handicap

Microspherophakia

Angle anomaly and glaucoma

Usually anterior lens subluxation

Homocystinuria• Defect in cystathio beta-synthase

Systemic features

Malar flush and fine, fair hair

Marfanoid habaitus

Increased platelet stickiness

Mental handicap

Ocular features

Downward lens subluxation

Disintegration of zonule

Treatment Options for Ectopia Lentis

• For induced astigmatism

1. Spectacle correction

• For aphakic portion

• Associated cataract

3. Surgical removal

• Lens-induced glaucoma

2. Nd:YAG laser zonulysis to displace lens out of visual axis

• Endothelial touch

• When other methods are inappropriate

Refrerencfes

Jack J Kanski. Clinical Ophtalmology. A Systemic approach

Jack J Kanski. Illustrated Tutorials