CORNEA & EXTERNAL EYE DISEASES Seborrheic Keratosis

(Richard L. Nepomuceno, MD)  this disorder is often associated with

seborrheic dermatitis
EYELID DISEASES  patients complain of redness, burning, and
 Meibomian gland dysfunction mattering of the eyelids
 Staphylococcal blepharitis  usually bilateral
 Seborrheic keratosis  often associated with meibomian gland
 Angular blepharitis dysfunction
 Molluscum contagiosum  the lashes are covered with yellow, greasy
 Hordeolum scales
 Chalazion  the scales are transluscent and easily
 Viral Papilloma removed
 Seborrheic keratosis
 Xanthelasma
 Basal cell carcinoma
 Capillary hemangioma
 Allergic contact dermatitis

Meibomian Gland Dysfunction

 meibomian glands
o Sebaceous glands of posterior
lamella of the eyelids
 abnormal lipid composition and secretion Seborrheic keratosis
 enlargement, inspissations of orifices
 chronic burning, foreign body sensation, Angular Blepharitis
filmy vision, tearing, crusting of the eyelids  maceration and crusting of the skin at lateral
canthus
 maceration is due to proteolytic enzymes
 injection of conjunctival and epibulbar
vessels
 caused by:
o Staphylococcus
o Moraxella
o Herpes
o Candida
Meibomian gland dysfunction

Staphylococcal Blepharitis
 infection of lid margin, lash bases, and
follicles
 burning, redness, mattering lashes
 ulceration, fibrin, collaretes, crusting
 can cause
o recurrent hordeola
o conjunctivitis
o marginal corneal infiltrates Angular blepharitis
o punctuate epitheliopathy
 chronicity causes thickened and scarred lids

OPHTHALMOLOGY | CORNEA & EXTERNAL EYE DISEASES 1

Molluscum Contagiosum Viral Papilloma
 Pox virus by direct contact  caused by Human Papilloma virus
 often seen in children and young adults  painless, keratinized, cutaneous lesion
 single lesion as papule to waxy nodule with usually without inflammation
umbilicated center  may cause papillary conjunctivitis
 in AIDS, multiple and large
 associated with toxic follicular conjunctivitis,
SPK, SEI, vascular pannus
 self-limited but may need treatment

Viral papilloma

Molluscum contagiosum
Seborrheic Keratosis
 mainly seen in elderly
Hordeolum / Stye
 benign, pigmented, well-demarcated, slightly
 local, infected abscess of a hair follicle or
elevated, warty, crusted, stuck-on-
gland in the lid margin
appearance, with visible keratotic plugs
 external – anterior eyelid margin, often gland
of Zeiss
 internal – meibomian gland

Seborrheic keratosis

Xanthelasma
Hordeolum / stye
 superficial dermis contain histiocytes with
Chalazion cholesterol esters
 chronic lipogranuloma of meibomian gland  associated with elevated serum cholesterol
or hyperlipidemia syndrome
 forms as a discrete mass

Chalazion Xanthelasma

OPHTHALMOLOGY | CORNEA & EXTERNAL EYE DISEASES 2

Basal Cell Carcinoma
 slow-growing tumors found in sun-exposed
areas
 most common eyelid malignancy
 usually located on the lower eyelid
 edges are raised and pearly, with a central
ulceration
Basal cell carcinoma
Squamous Cell Carcinoma
 rare malignancy of the eyelids
 commonly arises in sun-exposed areas and
may resemble other lesions of the eyelid
 the inset shows pearly raised margins of a
squamous cell carcinoma
Squamous cell carcinoma
 eosinophilic cells with large cytoplasms
 (1) keratin pearls and (2) dyskeratotic cells
are seen
 Dyskeratotic cells have small, dark nuclei
and produce keratin
Histology, squamous cell carcinoma
OPHTHALMOLOGY | CORNEA & EXTERNAL EYE DISEASES
Capillary Hemangioma
 appear at or soon after birth
 strawberry nevus
 usually involute spontaneously
 may cause amblyopia if in visual axis
 treatment is by intralesional steroid injection
Capillary hemangioma
Allergic Contact Dermatitis
 environmental or externally applied agents
 cel-mediated type IV hypersensitivity
reaction
 may be cause by drugs: Neomycin, Atropine
Allergic contact Dermatitis
3
LACRIMAL SYSTEM DISORDERS
 Canaliculitis
 Dacryocystitis
 Normal tear film
 Keratoconjunctivitis sicca

Canaliculitis
 tearing, prominent punctum Keratoconjunctivitis sicca
 anaerobic bacteria
 Actinomyces israelii
CONJUNCTIVAL DISEASES
 Follicular conjunctival reaction
 Papillary conjunctival reaction
 Epidemic keratoconjunctivitis
 Membranous conjunctivitis
 Gonococcal conjunctivitis
 Chlamydial conjunctivitis
 Trachoma
 Allergic (Hay fever) conjunctivitis
Canaliculitis
 Atopic keratoconjunctivitis
 Vernal keratoconjunctivitis
Dacryocystitis  Giant papillary keratoconjunctivitis
 acute or chronic  Superior limbic keratoconjunctivitis
 S. penumoniae or S. aureus  Steven-Johnson syndrome
 bacterial infection following obstruction at  Conjunctival nevus
either end of the nasolacrimal canal  Adenochrome deposits
 nasal trauma, scarring from ocular surface  Pinguecula
disease or a developmental anatomy  Pterygium
 acute – treatment is topical antibiotics  Pyogenic granuloma
 chronic – produces permanent scarring or  Phlyctenulosis
fistula formation  Conjunctival viral papilloma
 treatment is DCR  Conjunctival/Corneal intraepithelial
neoplasia
 Conjunctival lymphoma
 Primary acquired melanosis (PAM)

Follicular Conjunctival Reaction

 follicles – lymphoid germinal centers
 smooth nodules which are avascular at the
apices surrounded by fine vessels at their
bases
Dacryocystitis  can be a normal variant if found in the lower
conjunctiva without infection
Keratoconjunctivitis Sicca  etiology
 aqueous tear deficiency o adenoviral conjunctivitis
 acquired disorder mostly in women o primary herpes simplex viral
 40 years old and above infection
 dryness, blurring of vision, photophobia o molluscum contagiosum
 worse at the end of day and with dry windy o enterovirus
weather o chlamydial infection
o toxicity from medications

OPHTHALMOLOGY | CORNEA & EXTERNAL EYE DISEASES 4

  subepithelial infiltrates develop until after at
least 2 weeks after the onset of symptoms
 SEI results from a host immune resonse

Follicular conjunctival reaction

Papillary Conjunctival Reaction

 non-specific response caused by many
agents
 usually seen on the upper tarsal conjunctiva
 fine mosaic pattern of dilated telangiectatic
blood vessels
 each has a central fibrovascular core that
gives rise to a vessel branching out in a
spoke-like pattern
 conjunctival septae surrounding the papillae
are anchored by pale tissue when papillary
hypertrophy occurs

Epidemic keratoconjunctivitis

Membranous Conjunctivitis
Pappilary conjunctival reaction
Epidemic Keratoconjunctivitis
 syndrome of external ocular adenoviral
infection
 adenovirus serotypes 8, 11, 19 are most
common
 acute onset of watery discharge,
photophobia, and mild foreign body
sensation
 preauricular lymphadenopathy and mixed
papillary-follicular conjunctivitis
 may present with subconjunctival
haemorrhage, chemosis, and
pseudomembrane or membrane formation
 mucopurulent discharge when membranous
reaction develops
 maximum intensity in 5 – 7 days after onset
of symptoms
 keratitis is characterized initially by:
o diffuse punctate intraepithelial
lesions fine/coarse punctate
epithelial keratitis  subepithelial
infiltrates (SEI)
 conjunctival membrane or pseudomembrane
can occur in
o severe viral or bacterial
conjunctivitis
o chemical burn
o Steven-Johnson syndrome
o ocular cicatricial pemphigoid
 produced when an inflammatory discharge
of fibrin with PMNs and fibrin coagulates on
the conjunctival surface
 true membrane
o incorporates the epithelium and
bleeds when removed
 pseudomembrane
o more superficial and can be peeled
or scraped away without bleeding
 healing can result in conjunctival scarring

OPHTHALMOLOGY | CORNEA & EXTERNAL EYE DISEASES 5

 Chlamydial (Inclusion) Conjunctivitis
 Chlamydia trachomatis
 common in sexually active and in
conjunction with urethritis or cervicitis,
although urogenital symptoms may not be
present
 characterized by prominent follicular
response with non-tender preauricular
lymphadenopathy
Membranous conjunctivitis

Gonococcal (Bacterial) Conjunctivitis

 Neisseria gonorrheae
 hyperacute purulent conjunctivitis
 direct contact with infected secretions or
from genital-hand-ocular transmission
 gonococci and meningococci are the only
bacteria causing conjunctivitis with
periauricular lymphadenopathy and Chlamydial conjunctivitis
conjunctival membranes
 punctate epithelial keratitis often noted
superiorly and can evolve to marginal or
subepithelial infiltrates
 micropannus can occur
 one of the most common forms of neonatal
conjunctivitis in the newborn associated with
pneumonitis
 neonates have no follicular response making
diagnosis more difficult
 treatment are
Gonococcal conjunctivitis (mucopurulent discharge)
o oral Tetracycline 250 mg 4x a day
for three weeks
 corneal involvement may consist of diffuse o Erythromycin 500 mg 4x a day for
epithelial haze, epithelial defects, marginal three weeks (Azithromycin)
infiltrates, and peripheral ulcerative keratitis
that rapidly progresses to perforation
 classic cause of neonatal conjunctivitis
 treatment is systemic + topical antibiotics
 Ceftriaxone 1 g IM
 with corneal perforation = Ceftriaxone IV
every 12 hours for 3 days
 co-treatment for Chlamydia

Chlamydial conjunctivitis Inclusion body

Gonococcal conjunctivitis (perforation)

OPHTHALMOLOGY | CORNEA & EXTERNAL EYE DISEASES 6

Trachoma
 caused by chronic and recurrent Chlamydial
infections
 Chlamydia serotypes A, B, C
 acute phase – follicular conjunctivitis and
epithelial keratitis
 these lead to conjunctival scarring and
pannus formation
 dry eye, trichiasis, and entropion
 Herbert’s pits are small depressions at the
limbus that represent areas of slight thinning
at the sight of necrotic follicles
 can lead to secondary bacterial keratitis
Trachoma (Arlt’s lines)
Trachoma (Herbert’s pits)
Giant Papillary Keratoconjunctivitis
 chronic inflammation of the conjunctiva with
prominent papillary hypertrophy of the
superior tarsus
 associated with soft contact lens material,
protein debris, accumulating on the lens
surface, or chemicals involved in lens
cleansing
 also seen in ocular prosthesis, loose nylon
sutures, filtering blebs
Gian papillary keratoconjunctivitis
OPHTHALMOLOGY | CORNEA & EXTERNAL EYE DISEASES
Superior Limbic Keratoconjunctivitis
 chronic, idiopathic, recurrent condition
characterized by ocular irritation and
redness
 women between ages 20 and 70 years old
 recurrences of disease can occur over 1 to
10 years
 can sometimes resolve spontaneously
 often bilateral, but one eye more severely
affected
 signs:
o fine papillary reaction on the
superior conjunctiva
o hypertrophy of superior limbus
 filamentary keratitis involving the superior 5
th
of the cornea is seen frequently
 associated with hyper- or hypothyroidism
Superior limbic keratoconjunctivitis
Steven-Johnsons Syndrome
 a.k.a. Erythema multiforme major
 acute, inflammatory, vesiculobullous
reaction of the skin and mucous membrane
 more commonly seen in children and young
adults, and more prevalent in women
 acute onset of fever, arthralgia, malaise, and
upper or lower respiratory tract symptoms
 cutaneous eruptions follows within days
 bullous eruptions with membranes or
pseudomembranes formation occur
 mucopurulent discharge is common
 late ocular complications include
symblephera, trichiasis, and dry eye
Steven-Johnson Syndrome
7
Conjunctival Nevus
 congenital hamartomas that consists of
nests of modified melanocytes (nevus cells)
 junctional, compound, and subepithelial nevi
occur in the conjunctiva
 an important variation in the conjunctiva is
the frequent occurrence of small epithelial
inclusion cysts within the nevi, particularly
with compound or subepithelial nevi
 rarely undergo malignant transformation
Conjunctival nevus
Pinguecula
 these elevated, fleshy conjunctial masses
are located in the interpalpebral region, most
commonly on the nasal side
 yellow or light brown
 associated with chronic actinic (UV)
exposure, repeated trauma, dry and windy
conditions
 histologically, they are composed of
abnormal collagen bundles with staining
characteristics similar to elastic tissue
 elastotic degeneration, but the tissue is not
actually composed of elastin
Pinguecula
Pterygium
 benign proliferation of fibrovascular tissue
covered by conjunctival-like epithelium
extending onto the peripheral cornea
 risk factors are UV exposure, wind, and dust
 histopathology: subepithelial fibrovascular
tissue and elastotic degeneration of collagen
OPHTHALMOLOGY | CORNEA & EXTERNAL EYE DISEASES
 can occur above or within Bowman’s layer
 corneal epithelial iron line (Stocker’s line)
can be seen in advance of the head of a
pterygium on the cornea
 indications for removal
o reduced vision due to invasion of
visual axis
o irregular astigmatism
o significant ocular irritation
Pterygium
Pyogenic Granuloma
 a misnomer, because the lesion DOES NOT
represent granulomatous inflammation
 a reactive proliferation of vascular
endothelial cells and granulation tissue
 raised, fleshy, red, pedunculated lesion that
can arise from skin or conjunctiva
 often accompanied by mucopurulent
discharge
 this occurs after inflammatory conditions,
such as chalazia or chemical burns, or after
conjunctival surgery
Pyogenic granuloma
8
Suture Granuloma
 clinically appears similar to a pyogenic
granuloma after corrective surgery
Suture granuloma
Conjunctival / Corneal Phlyctenules
 focal translucent lymphocytic nodules
located at the limbus
 neutrophils enter the nodule a few days after
onset as necrosis develops
 result from delayed cell-mediated
hypersensitivity reaction to staphylococcal
antigens or tubercle bacilli
 limbus may result in fibrosis and
vascularization of the peripheral cornea
 may wander across the cornea producing
vascularization and scarring
Conjunctival / Corneal phlyctenules
Conjunctival Viral Papilloma
 benign lesion of the conjunctiva consists of
multiple fibrovascular connective tissue
cores with an overlying epithelium
 lesions may be sessile or pedunculated
 they are caused by human papilloma virus
 in older adults, these lesions can be
malignant
 sessile is seen more in older patients and
can be premalignant
 treatment is excision and cryotherapy
OPHTHALMOLOGY | CORNEA & EXTERNAL EYE DISEASES
Conjunctival viral papilloma
Conjunctival / Corneal Intraepithelial Neoplasia
 localized to the epithelium and do not invade
the epithelial basement membrane
 spectrum of malignant change from a benign
appearance to severe dysplasia and
anaplasia throughout the entire epithelial
thickness
 gelatinous, sessile appearance with
numerous superficial corkscrew-like blood
vessels typically located at the limbus
Conjunctival / Corneal intraepithelial neoplasia
 corneal involvement appears as irregular
opalescent appearance with small, white,
intraepithelial opacities and a central
fimbriated edge
 leukoplakia (white plaque of keratin protein)
may overlie a portion of the neoplasia
 rose bengal is the stain being used
 HPV strains have been detected in many of
these lesions
 invasion to basement membrane =
squamous cell carcinoma
Severe form of conjunctival intraepithelial neoplasia
9
Benign Lymphoid Hyperplasia
 composed of a mixture of T and B
lymphocytes
 looks similar to a malignant lymphoma
 similar histopathologic appearance
 similar immunohistochemical studies
Benign lymphoid hyperplasia
Conjunctival Lymphoma
 localized lymphoma on the bulbar
conjunctiva
 biopsy was consistent with B-cell lymphoma
 compsed of monoclonal B-lymphocyte
proliferation
 southern blot hybridization studies looking
for Ig gene rearrangements are often
required to diagnose lymphoma
 R/O systemic lymphoma
Conjunctival lymphoma
Systemic Lymphoma
 conjunctival lesion in a patient with systemic
lymphoma
 lesions elevated and salmon colored
 surrounding tissue is not inflamed
 treated with systemic chemotherapy
OPHTHALMOLOGY | CORNEA & EXTERNAL EYE DISEASES
Primary Acquired Conjunctival Melanosis (PAM)
 intraepithelial melanocytes proliferate to
produce multiply flat, brown patches of
unilateral pigmentation within the superficial
conjunctiva
 occurs in middle age
 PAM is different from acquired racial
melanosis and from secondary acquired
melanosis
 caused by Addison’s disease, radiation,
pregnancy, and other causes
 PAM have a premalignant potential
 melanoma develops in 20-30%
 nodules that develop in previously flat
lesions
Primary acquired conjunctival melanosis (PAM)
Racial Melanosis
 benign lesion common among dark-skinned
individuals
 brown pigmentation of the conjunctiva,
usually bilateral
 does not have malignant potential
Racial Melanosis
Conjunctival Concretions
 represent trapped foreign body such as dust
in the conjunctival epithelium
 presents as foreign body sensation if
numerous and very elevated
Conjunctival concretions
10
Kaposi’s Sarcoma Subconjunctival Hemorrhage
 20% of patients with AIDS  rupture of conjunctival blood vessels due to
 7% affect the conjunctiva trauma, valsalva, or blood dyscrasia
 lesion is elevated and highly vascular  similar to bruise injuries
 conjunctival lesions are nodulr or diffuse,
blue-red or deep brown, and often elevated
 Kaposi’s sarcoma arises from vascular
elements, including endothelial cells and
pericytes

Subconjunctival hemorrhage

SCLERAL DISEASES
 Episcleritis
 Scleritis
 Scleromalacia perforans
Kaposi’s sarcoma (eye)
 Congenital melanosis oculi

Chemical Injuries
Scleritis
 can either be caused by an acid or alkali
 anterior
 alkali is more damaging than acid chemical
o sectoral
o nodular
o diffuse
o necrotizing
 posterior
 associated with numerous autoimmune
disease
 deep, constant pain
 bilateral in 50%
Chemical injury
 predominantly in women
 vessels do not move when applicator
Conjunctival Sarcoid
applied to conjunctiva
 sarcoidosis is a multisystem disorder
 complications include:
 more common in blacks than whites
o keratitis
 characterized by the presence of non-
o uveitis
caseating granulomas
o cataract
 multiple sarcoid nodules in the inferior fornix o glaucoma
 pulmonary involvement is the most frequent o scleral thinning
form of the disease, and usually present as o marginal keratolysis
bilateral hilar adenopathy with or without o scleral perforation
parenchymal involvement
 ocular involvement is the second most
common manifestation (50% of affected
patients)

Scleritis

Conjunctival sarcoid

OPHTHALMOLOGY | CORNEA & EXTERNAL EYE DISEASES 11

Scleromalacia Perforans Bacterial Keratitis
 nodular scleritis
o commonly seen in rheumatoid
arthritis
 scleromalacia perforans
o painless form with no obvious
inflammatory sign
o seen in rheumatoid arthritis
 etiology
o trauma
o contact lens wear
o dry eye
o use of contaminated topical
medications
 laboratory confirmation is essential
 virulent Gram-negative organisms
(P. aeruginosa)
 rapid evolution
 tenacious mucopurulent discharge
 opacification and edema adjacent to ulcer
 stromal necrosis due to proteolytic enzymes
 seen among contact lens wearer

Scleromalacia perforans

INFECTIOUS CORNEAL DISEASES

 Bacterial keratitis
o S. pneumonia
o P. aeruginosa S. aureus P. aeruginosa
 Fungal keratitis
 Acanthamoeba keratitis Fungal Keratitis
 Primary herpes simplex virus infection  filamentous (Fusarium) or yeast (Candida)
 Herpes simplex virus dendiritic keratitis  dirty gray white, dry infiltrate with feathery
 Herpes simplex virus geographic keratitis borders
 Herpes simplex virus necrotizing stromal  multiple satellite infiltrates
keratitis  large ulcers have endothelial plaque and
 Herpes zoster dendritiform keratitis hypopyon (pus in the eye)
 Marginal keratitis associated with  can extend into the anterior chamber and
staphylococcal blepharitis perforation
 Luteic interstitial keratitis
 Corneal epithelial membrane dystrophy
(map-dot-fingerprint dystrophy)
 Lattice corneal dystrophy
 Macular dystrophy
 Fuch’s endothelial dystrophy
 Keratoconus
 Corneal arcus
 Calcific band keratopathy
 Secondary lipid keratopathy Fungal keratitis

 Salzmann’s nodular degeneration

 Neutrophic keratopathy
 Mooren’s ulcer
 Others
o Corneal foreign body
o Alkali burn

OPHTHALMOLOGY | CORNEA & EXTERNAL EYE DISEASES 12

Acanthamoeba Keratitis
 seen in contact lens wearer who make their
own solutions
 severe pain, photophobia, protracted
progressive course
 early form – dendritiform
 enlarged corneal nerves
 stroma – gray white superficial infiltrate in
central cornea Herpes simplex virus dendritic keratitis

 partial or complete ring infiltrate in late

phase Herpes Simplex Virus Geographic Keratitis
 commonly misdiagnosed as HSV  lysis of desquamated cells result to an ulcer
 stains with rose bengal dye
 geographic map ulcers develop by
centrifugal spread of HSV
 subepithelial infiltrates serve as markers of
past infection and leave as scars
 sectoral or diffuse reduction in corneal
sensation
 diagnosis mainly on signs
 tissue culture or antigen detection
techniques
Acanthamoeba keratitis

Primary Herpes Simplex Virus Infection

 acquired from the environment
 unilateral vesicular blepharoconjunctivitis
 pruritic vesicles of lids, skin, and eyelid
margins
 follicular conjunctivitis and palpable
preauricular lymph node
 treatment is oral Acyclovir and topical
Trifluridine or Acyclovir Herpes simplex virus geographic keratitis

Herpes Simplex Virus Disciform Keratitis

 focal circular area of microcystic edema
overlying a mild stromal inflammatory
infiltrate and edema with descemet folds and
underlying keratic precipitates (KP)
 photophobia and decreased vision
 cell-mediated immune response to viral
Primary herpes simplex virus infection antigens in the stroma

Herpes Simplex Virus Dendritic Keratitis

 majority of patients have a recurrent ocular
disease
 asymptomatic, mild, foreign, body sensation,
photophobia, redness, and blurred vision
 starts as discrete punctuate epithelial
keratitis, coalesce, dendritic lesion (swollen
opaque epithelial cells) commonly in the
center
 characteristic terminal bulbs
Herpes simplex virus disciform keratitis

OPHTHALMOLOGY | CORNEA & EXTERNAL EYE DISEASES 13

Neurotrophic / Metaherpetic Ulcer
 recurrent HSV can damage the epithelial
basement membrane and anterior stroma
 postinfectious (sterile) keratopathy
 round or oval ulcer with smooth, thickened,
edges that often overlie an area of stromal
inflammation or inactive stromal scarring
Ocular rosacea
 risk for stromal melting and perforation
Corneal Epithelial Basement Membrane
Dystrophy
(Map-Dot-Fingerprint Dystrophy)
 most common anterior corneal dystrophy
 bilateral and can have dominant inheritance
(often incomplete penetrance)
 more common in women, 2% of the general
population
 gray patches, microcysts, or fine lines are
Neurotrophic / Metaherpetic ulcer
seen in the central epithelium
 abnormal epithelial turnover, maturation,
Herpes Zoster Dendritiform Keratitis
and production of basement membrane
 pseudodendrites
 have heaped up epithelium with unusually
branching patterns but lack the terminal
bulbs
 represently lytically infected corneal
epithelial cells
 complications:
o mucous plaques
o nummular keratitis
o disciform keratitis
o deep stromal keratitis
o perforation Corneal epithelial basement membrane dystrophy
o postherpetic neuralgia
 histologically
o map – thickened basement
membrane with extension to the
epithelium
o dot – abnormal epithelial cells with
microcytes (Cogan’s microcystic
dystrophy)
o fingerprint – fibrillar material
Herpes zoster dendritiform keratitis
between the basement membrane
and Bowman’s layer
Ocular Rosacea
 symptoms of recurrent pain, photophobia,
 oculodermatologic disease of facial
tearing, and blurred vision
telangiectasia and erythema
 more common after age of 30 years
 sebaceous gland dysfunction of the face,
 10% of these patients have recurrent
neck, and shoulders
erosion
 type IV hypersensitivity reaction
 50% of patients with recurrent erosion
 recalcitrant meibomian gland dysfunction
syndrome (RES) have MDF dystrophy
and/or staphylococcal blepharitis
 corneal neovascularization

OPHTHALMOLOGY | CORNEA & EXTERNAL EYE DISEASES 14

Lattice Corneal Dystrophy
 auto dominant with variable expression
 first decade of life
 type I is the most common
 cornea has refractile lines, small white dots,
and haze in the central superficial stroma
 represent amyloid deposit in the axial
anterior stroma
 can present as recurrent eye syndrome
(RES)
 vision diminished by 30’s
 birefringence of the Congo red stain is seen
with polarized light
Macular Dystrophy
 least common
 autorecessive
 early loss of vision
 deposits of glycosaminoglycans
(mucopolysaccharides)
 gray white irregular feathery lesions in the
axial stroma going deep and peripheral
 Alcian blue staining of extracellular and
intracellular
 mucopolysaccharides occur in all layers of
the cornea, including the epithelium,
endothelium, and Descemet’s membrane
 treatment is penetrating keratoplasty (PKP)

Lattice corneal dystrophy

Granular Dystrophy Macular dystrophy

 autodominant
 discrete, chalky, granular opacities in the Cornea Guttata
axial cornea with clear areas of intervening  peripheral
stroma o Hassall-Henle bodies
 granular deposits represent hyaline deposits  central
(granules with phospholipid and microfibrillar o cornea guttae
protein)  no inheritance pattern
 blurred vision middle to late 30’s  round, dark, drop-like prominences at
 the hyaline material stains red with Descemet’s membrane and endothelium
Masson’s trichrome stain  fine, brown, pigment granules on the
posterior surface of the cornea
 guttae represent thickend localized anvil
shaped excrescences of Descemet’s
membrane
 beaten-metal appearance resembling skin of
an orange

Granular dystrophy

Cornea guttata

OPHTHALMOLOGY | CORNEA & EXTERNAL EYE DISEASES 15

Fuch’s Endothelial Dystrophy
 hereditary atrophy of the endothelium
 autodominant
 women more than 50 years old
 significant density of cornea guttae
 attenuation and dysfunction of endothelial
cells
 leads to corneal edema
 stromal edema  epithelial edema
 risk of corneal decompensation after
cataract surgery Keratoconus

Vogt’s Limbal Girdle

 mild form of limbal calcific band keratopathy
 usually irregular and slightly elevated and
may contain small “Swiss cheese” holes

Fuch’s endothelial dystrophy

Posterior Polymorphous Dystrophy

 autosomal dominant disorder of the corneal
endothelium is almost always bilateral
 broad band-like opacities can occur at the
Vogt’s limbal girdle
level of Descemet’s membrane and the
endothelium
Spheroidal Degeneration
 mild condition
 multiple, golden-brown, advanced
 brownish-yellow deposits are more confluent
and are located in the central cornea in this
case
 spheroidal degeneration characteristically
occurs in the interpalpebral zones
 associated with chronic actinic exposure and
dry and windy conditions
Posterior polymorphous dystrophy
 similar to band keratopathy, it may also be
associated with chronic localized ocular
Keratoconus inflammation
 central or paracentral cornea undergoes
progressive thinning and bulging
 cone shape cornea
 no associated inflammation
 unknown cause relation to atopy, Down
syndrome, congenital amaurosis, and eye
rubbing
 tends to progress during adolescent years
 cone shape induces myopia, irregular,
astigmatism, and decreased vision Spheroidal degeneration
 corneal findings
o Vogt’s striae
o Fleischer’s rings
o Irregular scissoring

OPHTHALMOLOGY | CORNEA & EXTERNAL EYE DISEASES 16

Calcific Band Keratopathy
 characteristically this occus in the
intrapalpebral region
 calcium deposits in this region result from
localized elevations of pH favouring calcium
precipitation and increased evaporation,
which increases the local concentration of
calcium
 this condition may be idiopathic
 it is usually associated with localized ocular
inflammatory processes or systemic
hypercalcemia
Calcific band keratopathy
Salzmann’s Nodular Degeneration
 these elevated bluish-white superficial
nodules
 more common in females and most
th
commonly occurring in the 5 decade of life
or even later
 the condition may be associated with
localized corneal inflammation
 histopathology shows subepithelial hyaline
nodules that replace Bowman’s layer
Salzmann’s nodular degeneration
OPHTHALMOLOGY | CORNEA & EXTERNAL EYE DISEASES
Neurotrophic Keratopathy
 involves the central or inferior paracentral
cornea
 epithelial defects stains with fluorescein
heavily
 horizontal oval
 raised, rolled-up gray edges
 progressive sterile ulceration or
superinfection can result in perforation
 factors leading to this are:
o decreased sensitivity to corneal
trauma
o dessication
o cellular functional impairment
 due to a loss of the trophic
influence from the nerves
o etiology
 cranial + peripheral nerve
problems
 anesthetic abuse
 neurosurgical procedures
Neurotrophic keratopathy
Mooren’s Ulcer
 autoimmune, chronic progressive, idiopathic
necrotizing ulceration of the peripheral
stroma and epithelium
 spreads circumferentially and then
centripetally with a leading undermined edge
of deepithelialized tissue
 inflamed eye with intense pain
 spontaneous perforation
 both humoral and cell-mediated immunity
mechanisms play a role
 epithelium is absent in areas of active
ulceration
 vascularized pannus leading up to areas of
active ulceration
 abrupt transition between involved and
uninvolved cornea with an overhanging
edge
Mooren’s ulcer
17
Corneal Foreign Body Corneal Abrasion
 common corneal injury  epithelium of cornea removed by trauma or
 imperative to evert the upper lid foreign body
 removal of the offending agent is essential  pain, photophobia, and blurred vision
 it can lead to abrasion
 may also cause secondary bacterial
infection

Corneal abrasion

Corneal foreign body Thyroid Eye Disease

 infiltration of the extraocular muscles and
Vitamin A Deficiency connective tissue  severe proptosis and
 Bitot’s spot orbital inflammation
o small, white-gray irregular plaque  patient had chronic exposure keratitis and
that usually occurs near the limbus developed in an indolent ulcer
in the intrapalpebral region  exophthalmos and lid retraction predispose
 a gas-producing bacteria, to corneal exposure
Corynebacterium xerosis, is responsible for
the foamy appearance in this lesion
 marked keratinization of the inferior cornea
 corneal surface is dry, and light reflex is
irregular
 goblet cell function is impaired in this
disorder, and there is also lack of mucin

Thyroid eye disease

Vitamin A deficiency

OPHTHALMOLOGY | CORNEA & EXTERNAL EYE DISEASES 18