Ocular Pathology Flashcards
(288 cards)
1
Q
What is the pathology of senile calcific plaque?
A
• The pathology of senile calcific plaques shows
basophilic calcific deposits in sclera anterior to the
lateral rectus muscle
2
Q
What is trilateral retinoblastoma?
A
● Bilateral retinoblastoma + ectopic intracranial
retinoblastoma (pineal gland or parasellar region)
● Histology: numerous round blue cells surrounded
by pink necrotic cells
3
Q
What is the term for epithelium growing on both sides of the cornea?
A
● Epithelium growing on both sides of the cornea is
termed epithelial downgrowth
● Most commonly seen from multiple prior surgeries
such as ECCE and PKP
4
Q
What are the characteristic findings in Peters Anomaly?
A
● Peters anomaly is characterized by:
○ The absence of Descemet’s membrane
○ Adhesions of iris and lens material to the
posterior surface of the cornea
5
Q
What are the pathologic findings of ICE Syndrome?
A
● Corneal edema
● Inappropriate proliferation of endothelium and/or
Descemet’s membrane along anterior segment
structures
6
Q
What are the characteristic findings in Granulomatosis with Polyangiitis (GPA) and what vessels are involved?
A
● Chronic sinusitis
● Saddle Nose deformity
● Granulomatous inflammation with necrosis
● Small and medium size blood vessels are usually
involved
7
Q
What is the most common cause of proptosis in adults?
A
● Thyroid-associated orbitopathy
● Pathology: muscle bundles separated by fluid and
infiltrated by a mixture of mononuclear
inflammatory cells
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8
Q
What conditions are associated with weakened zonules?
A
● Congenital
○ Marfan’s
○ Homocystinuria
○ Hyperlysinemia
○ Ehler-Danlos Syndrome
○ Scleroderma
○ Weill-Marchesani
● Other
○ Pseudoexfoliation
○ Prior vitreoretinal surgery
○ History of trauma
○ High myopia
9
Q
What is the most common extraocular tumor that spreads into the eye via direct extension?
A
● Squamous Cell Carcinoma of the Conjunctiva
○ Typically mucoepidermoid carcinoma or
spindle cell variant
10
Q
What is the management of a brown pigmented lesion of the palpebral conjunctiva?
A
● A brown pigmented lesion of the palpebral
conjunctiva is more likely to be Primary Acquired
Melanosis with atypia which requires immediate
work up with excisional biopsy given its high
malignant potential
11
Q
What is the presentation, prognosis, and management of lymphangioma?
A
● Typically present in children in the superior and
nasal quadrant
● Can bleed into themselves causing cysts of blood,
“chocolate cysts”
● Enlarge in association with upper respiratory
infection
● Benign
● Treatment:
○ Observation unless optic nerve compression
or corneal exposure
○ Debulking, complete removal is difficult
12
Q
What was the main conclusion of the LARGE choroidal melanoma trial?
A
● Adjunctive radiotherapy did not improve overall
survival
13
Q
What was the main conclusion of the MEDIUM choroidal melanoma trial?
A
● Mortality was similar between brachytherapy and
enucleation groups
● Histologically confirmed metastases were found in
approximately 10% patients treated with
enucleation
14
Q
What was the main conclusion of the SMALL choroidal melanoma trial?
A
● Mortality was 1% at 5 years with observation only
15
Q
What cell type produces the myelin sheath of the optic nerve?
A
● Oligodendrocyte
16
Q
What is the function of microglial cells?
A
● “Clean-up” function due to phagocytic activity
17
Q
What function do astrocytes have?
A
● Support and nutrition to the optic nerve and retina
18
Q
What are the similarities and differences between the pathology of pterygia and pingueculae?
A
● Similarities:
○ Both have fragmented stromal collagen with
basophilic “elastotic” degeneration
○ Degeneration is due to chronic UV light
exposure
● Differences:
○ Pterygia = involves the cornea and
destruction of Bowman’s layer
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19
Q
What is stained by periodic acid-Schiff (PAS) stain?
A
● Type IV collagen, glycogen, and proteoglycans
20
Q
What is stained by Prussian blue stain?
A
● Iron
21
Q
What is stained by Von Kossa stain?
A
● Calcium
22
Q
What is stained by cytokeratin stain?
A
● Squamous epithelium
23
Q
What are the favorable and unfavorable risk factors in conjunctival melanoma?
A
● Favorable:
○ Epibulbar
● Unfavorable
○ Location:
■ Palpebral, caruncle, eyelid margin,
fornix (non-limbal)
○ Invasion into deeper tissues
○ Thickness > 1.8 mm
○ Pagetoid or full thickness intraepithelial
spread
○ Lymphatic invasion
○ Mixed cell type
○ De novo origin
○ Residual involvement at the surgical margin
24
Q
What is a Mittendorf dot and where is it most commonly located?
A
● Remnant of the hyaloid artery of primary vitreous
at its attachment to the tunica vasculosa lentis
● Most commonly located inferonasally
25
What are the criteria of the Reese Ellsworth Classification for retinoblastoma? What is it useful for?
● Classification by:
○ Number
○ Size
○ Location
○ Presence or absence of vitreous seeding
● Used after external beam radiation for RB lesions,
does not give prognostic information
26
What is stained by Verhoeff-van Gieson Stain?
● Elastin
27
What is HMB-45 a marker for?
● Marker for melanocytic lesions
28
What cells does CD3 stain identify?
● T cells
29
What cells does CD20 stain identify?
● B cells
30
What does Warthin-Starry stain identify?
● Bartonella henselae
31
What is the rate of metastasis of conjunctival melanoma?
● 25%
32
What is the presentation of a ciliary body melanoma?
● Can range from completely asymptomatic to visual loss, photopsias, and visual field loss
33
What is a dermolipoma and where is it typically located?
● Type of choristoma
● Most commonly in the superotemporal quadrant
● Extends posteriorly into the orbit
34
What are the average ages of retinoblastoma diagnosis in patients with family history and patients without family history with unilateral or bilateral disease?
● Positive family history: 8 months
● Negative family history, bilateral disease: 12
months
● Negative family history, unilateral disease: 24
months
35
What is the pathology of a xanthelasma and what systemic problem is it associated with?
● Pathology shows macrophages with large amount
of fatty cytoplasm
● Associated with hyperlipoproteinemia
36
What happens during a spasm of the near reflex?
● Excessive convergence, accomodation, miosis
● Normal ductions, abduction deficit on version
movements
37
What stains can be used to visualize acanthamoeba?
● Stains:
○ H&E
○ PAS
○ Calcofluor white
○ Acridine orange
○ Gridley stain
○ Giemsa
38
Where are Koeppe iris nodules located and what conditions are they associated with?
● Located at the pupillary margin
● Associated with granulomatous uveitis:
○ Sarcoidosis
○ Tuberculosis
○ Syphilis
○ Lyme
39
What is adenoma sebaceum and what condition is it associated with?
● Facial angiofibromas (red papules)
● Adenoma sebaceum is found in 80-90% of
individuals with tuberous sclerosis which is
inherited in an autosomal dominant pattern
40
What condition are cerebellar hemangioblastomas associated with and what other problems are associated with this condition?
● Von Hippel Lindau Syndrome
● Also associated with retinal hemangioblastomas
and increased risk of renal cell carcinoma and
pheochromocytoma
41
What is the structure of the cells in the outer plexiform layer and what pathology can this result in?
● The outer plexiform layer is composed of neuronal
synapses that are obliquely oriented AKA the nerve
fiber layer of Henle
● The oblique orientation in this area accounts for
flower-like (petalloid) pattern of leakage seen on
IVFA for macular edema
42
What is stained by the Mart-1 immunostain?
● Melanocytes
43
What histologic features of choroidal melanoma are associated with increased rate of metastasis?
● High mitotic index
● High cell proliferation
● Complex microvascular patterns
● Tumor infiltration
● Lymphocytes
● Trisomy 8
● Monosomy 3
● Predominance of epithelioid cells
44
What is the difference between a tumor predominantly composed of epithelioid cells versus spindle cells?
● Tumor that is predominantly epithelioid is more
aggressive than a tumor predominantly spindle
cells
45
What makes up the primary, secondary, and tertiary vitreous?
● Primary: hyaloid artery, vasa hyaloidea propia,
mesenchymal cells, tunica vasculosa lentis
● Secondary: adult vitreous body; replaces primary
around 9th week of gestation
● Tertiary: zonular fibers
46
What is a choroidal osteoma? What is the epidemiology? What is the classic presentation and hallmark feature?
● Benign bony tumor of the choroid
● Found in young/adolescent patients, commonly in
females, can be bilateral
● Usually near the optic nerve, appears
orange-yellow with pseudopod margins
● When involving the macula: subretinal
neovascularization can negatively impact vision
● Hallmark feature: high amplitude echo on B scan
due to calcification with loss of normal orbital
echoes behind the lesion
47
What features are unique to sarcoid lesions of the optic nerve compared to sarcoid lesions of the eye itself?
● Sarcoid granulomas of the optic nerve may have
necrosis (caseating granulomas or noncaseating)
48
What are the features of choroidal melanoma on A scan and B scan?
● A scan: low amplitude internal reflections
● B scan: mushroom shaped mass or dome shaped;
can see invasion through Bruch’s membrane
49
What are the two growth patterns of retinoblastoma?
● Endophytic: grow from the retinal surface and
project into the vitreous cavity; more associated
with vitreous seeding
● Exophytic: grow underneath the retina and toward
sclera; more associated with serous RD
50
What is the pathology, appearance, and genetics of Lattice Corneal Dystrophy?
● Pathology: fusiform deposits within the corneal
stroma. Stain red with congo red; see
metachromasia with crystal violet and apple green
birefringence under polarized light
● Cornea has glass-like branching lines in the stroma
● Autosomal dominant inheriteance of TGFB1
mutation
● Of the stromal dystrophies, it has the highest rate
of recurrence in corneal transplant patients
51
What corneal dystrophies are associated with TGFB1 mutations?
● TGFB1 mutations cause abnormal keratoepithelin
● LARGE
○ Lattice (Type 1)
○ Avellino
○ Reis-Bucklers and Thiel-Behnke
○ Granular (Groenouw type 1)
○ Empty
52
Rank the stromal corneal dystrophies from highest to lowest rate of recurrence.
● Lattice > Granular > Macular
53
What percentage of women with choroidal metastases have history of breast cancer?
● 90% of women have been treated for breast cancer
in the past
54
What percentage of patients with very ischemic CRVO develop NVI/NVA and what is the most important predictive factor?
● High, up to 60%
● Over a mean of 3-5 months after the onset of
symptoms
● Most important predictive risk factor is poor visual
acuity
55
What is Meretoja syndrome? What gene mutation is it associated with? How does it present?
● Also known as lattice corneal dystrophy type 2, but
is not a true dystrophy due to the systemic
involvement
● Caused by mutation of gelsolin gene
● Presentation: corneal lattice-like changes with
systemic amyloidosis, masked facies,
dermatochalasis, lagophthalmos, pendulous ears,
dry/lax skin, cranial/peripheral nerve palsies,
orthostasis hypotension, cardiac conduction
abnormalities, problems with perspiration
56
What are the similarities and differences of Reis-Bucklers Corneal Dystrophy and Thiel-Behnke Corneal Dystrophy?
● Both are due to TGFB1 mutations
● Reis-Bucklers: autosomal dominant, painful
recurrent erosions, 1st/2nd decade, affects
Bowman’s layer, more common to have recurrent
erosion episodes and more severe stromal scarring
● Thiel Behnke: also affects Bowman’s layer; has
curly fibers on electron microscopy
57
What is the appearance of an eosinophil and what pathology are they most commonly associated with?
● Bilobed nucleus with abundant intracytoplasmic
eosinophilic granules
● Found in allergic reaction-type process like vernal
keratoconjunctivitis
58
What intraocular location is the most common site to have metastases?
● Bilobed nucleus with abundant intracytoplasmic
eosinophilic granules
● Found in allergic reaction-type process like vernal
keratoconjunctivitis
59
What are Dalen-Fuchs nodules and what conditions are they found in?
● Small, discrete yellowish infiltrate at the level of
the RPE
● Made up of aggregates of inflammatory cells,
usually epithelioid histiocytes, that collect between
the RPE and Bruch’s membrane
● Seen in sympathetic ophthalmia and VKH
60
What neoplasm is most likely to spread systemically with fine needle aspiration biopsy?
● Retinoblastoma
61
What is deposited in band keratopathy and what layer is it deposited in? What are the risk factors for band keratopathy? What is the treatment for band keratopathy?
● Calcium hydroxyapatite deposits in Bowman’s
membrane
● Systemic risk factors: hypercalcemia
(hyperparathyroidism, excessive vitamin D intake,
renal failure, milk-alkali syndrome, Paget disease,
sarcoidosis)
● Ocular risk factors: chronic uveitis, phthisis bulbi,
end-stage glaucoma, anterior mosaic dystrophy,
silicone oil tamponade
● Medication induced: steroid phosphate,
pilocarpine containing, mercury based
preservatives
● Rx: EDTA with superficial debridement
62
What is the process by which a retinal scar is formed?
● For example: after PRP
● Laser demarcation is produced by glial cells (Muller
cells and fibrous astrocytes)
● The internal limiting membrane and Bruch’s
membrane provide plans for glial scarring
● RPE cells undergo fibrous metaplasia giving rise to
dense black clumps seen in retinal scars
63
What is Masson trichrome used to stain?
● Hyaline in corneal stroma, for example in granular
dystrophy
64
What substance does Alcian blue stain and what pathology is this associated with?
● Stains acid mucopolysaccaride in corneal stroma
blue
● Used to diagnose Macular dystrophy
65
What are the three attachment sites of the uveal tract to the sclera?
● Scleral spur
● Internal ostia of vortex veins
● Peripapillary tissue
● This explains the dome-shaped configuration of
choroidal hemorrhages
66
What are the risk factors for a nevus being a malignant melanoma?
● TFSOM
● Thickness
● Subretinal fluid
● Symptoms
● Presence of orange pigment
● Juxtapapillary location (within 2 disc diameters)
● Absence of drusen or RPE changes
● Hot spots on IVFA
● Homogeneity on ultrasound
● Large size
● Flat lesion diameter of < 10 mm is nearly always
benign
67
What is snowflake degeneration?
● Long term complication of IOLs made of PMMA
material and an indication for explantation
68
What is a postoperative complication associated with hydrogel material?
● Calcification
69
What is a post-op complication of silicone-based IOLs in eyes with asteroid hyalosis?
● Deposits of calcium oxalate after a YAG cap
70
What is the appearance of nfectious crystalline keratopathy? What is the most commonly associated bacterium? What are the most common comorbidities? What is the treatment?
● Appears as white, branching, crystalline opacities
within the corneal stroma with little to no
associated inflammatory response
● Most common bacterium: strep viridans
● Most common comorbidities: PKP, corticosteroid
use, immunocompromised corneas, contact lens
wear
● Treatment: Stop topical steroid; requires
prolonged use of antibiotics. It is difficult to treat
and often requires repeat PKP or lamellar
keratectomy
71
What is the treatment for primary acquired melanosis?
● Excision with cryotherapy
72
What is the difference between UBM and B scan?
● UBM = high resolution (40 microns), high
frequency (50 MHz) penetrates less (4 mm)
● B scan = lower frequency, decreased resolution,
penetrates deeper (4 cm)
73
What is the pathology behind Giant Cell Arteritis?
● Granulomatous inflammation that destroys the
internal elastic lamina
● Involves medium to large vessels (i.e. posterior
ciliary, ophthalmic arteries)
● Treatment: IV solumedrol 1 g/day x 3-5 days then
PO prednisone with long taper
74
Marilyn Monroe Always Gets Her Man in LA County
Marilyn - Macular dystrophy
Monroe - Mucopolysaccharide
Always - Alcian blue stain
Gets - Granular dystrophy
Her - Hyaline
Man in - Masson Trichrome stain
L - Lattice dystrophy
A - Amyloid
County - Congo red
75
What is the other name for type 2 granular corneal dystrophy?
● Avellino dystrophy
76
What is the other name for type 3 granular dystrophy?
● Reis Buckler dystrophy
77
What is the most common etiology of anterior capsular fibrosis?
● Posterior synechiae causing hypoxia to lens
epithelium
● Leads to metaplastic response of epithelium, forms
fibrous plaques, mixed in with basement
membrane
78
What exam components should be included in the initial evaluation of a child with suspected retinoblastoma?
● Visual function
● Slit lamp exam
● Corneal diameter
● IOP
● DFE with scleral depression
● B scan (looking for calcifications)
● MRI brain
● Examination of parents/siblings
79
In what pathology is lipogranulomatous inflammation seen?
● Typically seen in chalazia
80
What virus causes molluscum contagiosum? What is the presentation? What is seen on pathology?
● Caused by the pox virus
● Exam shows dome-shaped, waxy nodules with
central umbilication
● Microscopic exam shows abundant granular
eosinophilic cytoplasm with small peripheral
nuclei; Henderson-Patterson corpuscleseosinophilic
inclusion bodies
81
What is the microscopic appearance of seborrheic keratosis?
● Hyperkeratosis, acanthosis (polygonal or basaloid
squamous cell proliferation), and some degree of
papillomatosis
● Pseudohorn cysts (concentrically laminated
keratin)
82
What is the microscopic appearance of papilloma?
● Fibrovascular ores with finger-like projections
going away from epidermis, surrounded by
hyperplastic stratified squamous epithelium
83
What is the histologic appearance of Peters anomaly? How often is Peters anomaly bilateral? How often is it associated with systemic abnormalities? What are some of these abnormalities?
● Histologically shows localized absence of corneal
endothelium and Descemet’s membrane beneath
an area of stromal irregularity
● Associated with iridocorneal adhesions
● Bilateral > 50% of the time
● Associated with systemic abnormalities > 50% of
the time
● Associated with:
○ Axenfeld-Rieger syndrome
○ Congenital rubella
○ Microphthalmia with linear skin defects
○ Pfeiffer syndrome
○ Kivlin Syndrome
○ Trisomies 13-15
84
What locations of ocular melanoma have the best and worst prognosis? What are the most common and least common locations for uveal melanoma? What are the associated mortality rates of these locations?
● Best prognosis: Iris
● Worst prognosis: ciliary body
● Most common location: choroid
● Least common location: iris
● Mortality rates:
○ Iris = 1-4%
○ Conjunctiva = 25%
○ Choroid = 12-50%
○ Ciliary Body = 30-50%
85
What chromosome is implicated in retinoblastoma?
● Chromosome 13
86
What chromosome is involved in neurofibromatosis type 1?
● Chromosome 17
87
What chromosome is involved in neurofibromatosis type 2?
● Chromosome 22
88
What chromosome is involved in tuberous sclerosis?
● Chromosome 9 for TSC1 (hamartin)
● Chromosome 16 for TSC2 (tuberin)
89
What type of tumor is a limbal dermoid? What syndrome is it associated with?
● Choristoma (normal tissue in an abnormal location)
● Associated with Goldenhar syndrome -
oculoauriculovertebral syndrome
○ Ear and mouth malformations
○ Preauricular skin tags
○ Strabismus
90
What is the difference between charged-particle radiation and brachytherapy in the treatment of uveal melanoma?
● Charged particle radiation delivers more
homogenous dose of radiation to tumor as
compared to brachytherapy
● Charged particle radiation delivers higher dose of
energy to anterior segment leading to high
incidence of NVG and vision loss
● Charged particle radiation is effective primary
therapy for uveal melanomas (unlike conventional
external beam radiation)
91
What are the layers of the choroid?
● Three main layers (inner to outer)
○ Choriocapillaris
○ Stroma
○ Lamina fusca (suprachoroidal layer)
● Can also be divided as follows:
○ Bruch’s membrane
○ Vascular layers (choriocapillaris, Sattler’s
layer - medium sized vessels, Haller’s layer -
large vessels)
● The choroid is thickest in the posterior pole (0.25
mm) and thinnest anteriorly and peripherally (0.10
to 0.15 mm)
92
What is the structure of amyloid?
● Final structure is beta-pleated sheet
● Ultrastructure shows nonbranching fibrils with
diameter 75-100 angstroms
93
What is Grocott-Gomori methenamine silver stain used for?
● Used to stain mucormycosis and other nonseptate
large branching hyphae
● Other useful stains for mucormycosis:
○ H&E
○ PAS
○ Calcofluor white
94
What are the most common non-ocular tumors located outside the field of any radiation treatment that occur in Rb survivors?
● Osteosarcoma and cutaneous melanoma
95
What is the pathology of anterior basement membrane dystrophy? What are some of the complications of this dystrophy?
● Pathology shows redundant epithelial basement
membrane, encysted degenerated epithelial cells
● One of the most common hereditary corneal
dystrophies
● Often bilateral
● Complications:
○ Recurrent erosions due to poor adhesion of
corneal epithelium from thickening
basement membrane
○ Problems with LASIK flap creation
96
What is the pathology of sebaceous carcinoma?
● Foamy cytoplasm within tumor cells
● Pagetoid spread = tumor cells that spread through
the epithelium without well-defined borders of the
area of involvement
● Very similar to chronic blepharoconjunctivitis or
chalazion
97
What is the most common orbital tissue to be involved in sarcoidosis?
● Lacrimal gland
98
What is the fixative used for electron microscopy?
● Glutaraldehyde
99
What is the fixative used for routine histopathologic analysis?
● Formalin
100
What are absolute ethanol or methanol media used for?
● Crystals (e.g. corneal urate crystals)
101
What is the fixative used for cytologic preparation?
● Ethyl alcohol
102
What is the fixative used for immunofluorescence?
● Michel fixative
● Zeus
103
What is Saccomanno medium used for?
● Liquid specimens or smears (e.g. vitreous biopsies, fine needle aspirates)
104
What are the acellular layers of the cornea?
● Bowman’s - made of collagen
● Descemet - true basement membrane, collagen
type IV, continuously produced by endothelium,
thickness increases with age
105
What is the pathologic appearance of Dalen Fuchs nodules and what conditions are they found in?
● Located between RPE and Bruch’s membrane
● Compared of lymphocytes and epithelioid
histiocytes
● Seen in VKH and SO
106
What is a plexiform neurofibroma and what condition is it associated with?
● Slow growing non-encapsulated lesions consisting
of endoneural fibroblasts, Schwann cells, nerve
axons
● Dermis infiltrated by numerous spindle shaped
cells arranged in ribbons and cords
● Associated with neurofibromatosis type 1 (von
Recklinghausen disease)
107
What is a seborrheic keratosis and what can it be associated with?
● Well circumscribed, oval, dome shaped to
verrucous benign papillomatous proliferation of
basal cells within epidermis
● Usually pink or brown in color
● “Stuck-on” appearance
● Lesions have cystic collections of keratin
● Pathology shows pseudohorn cysts or cysts line
with acanthotic stratified epithelium
● Sudden appearance of multiple seborrheic
keratoses = Leser-Trelat sign which is associated
with GI adenocarcinoma
108
What is Muir-Torre Syndrome?
● A syndrome of multiple sebaceous neoplasms in
conjunction with visceral malignancies (GI or GU)
109
What is Erdheim-Chester Disease?
● Subtype of xanthogranulomatous disease
● Pathology shows foamy histiocytes → Touton giant
cells
● Associated with serious cardiac complications leading
to death
110
What are the indications for enucleation for treatment of retinoblastoma?
● Tumor that is > 50% of the globe
● Optic nerve involvement
● Anterior segment involvement
111
What are the three types of giant cells?
● Langhans
● Touton
● Foreign body
112
What do Langhans giant cells look like on pathology and what conditions are they associated with?
● Horseshoe shaped ring of nuclei around the cell
● Seen in giant cell arteritis and sarcoidosis
113
What do Touton giant cells look like on pathology and what conditions are they seen in?
● Ring of nuclei that are surrounded by foamy ring of
lipid
● Seen in xanthogranulomatous disease
○ Juvenile X-linked xanthogranuloma
○ Adult onset xanthogranuloma
○ Adult onset asthma
○ Periocular xanthogranuloma
○ Necrobiotic xanthogranuloma
○ Erdheim Chester Disease
114
What does foreign body giant cells look like on pathology?
● Haphazard arrangement of nuclei within the cells
115
What is persistent fetal vasculature (PFV)?
● PFV is when the primary vitreous does not regress
leading to fibrovascular mass that produces
tractional forces on variety of ocular structures
● Exert traction on ciliary process thus elongating
them (characteristic PFV)
● How to distinguish from retinoblastoma:
○ PFV are often microphthalmic
○ Retinoblastoma eyes are normal size
116
What are the most common presenting signs of retinoblastoma in the US in patients < 5 years old? > 5 years old?
● < 5 years old: leukocoria and strabismus
● > 5 years old: leukocoria and decreased vision
117
What are the characteristics of acanthamoeba keratitis?
● Severe pain
● Range from no inflammation to marked
granulomatous reaction
● Contact lens wearer with poor hygiene
● Cysts (C) and trophozoites (T) within corneal
stroma
118
What structure divides the lacrimal gland into the orbital and palpebral lobes?
● Levator palpebrae aponeurosis
● If biopsy of lacrimal gland is needed, biopsy orbital
part because the excretory ducts pass through
palpebral portion
119
What is a pyogenic granuloma? What is its histologic appearance?
● Granulation tissue typically associated with chalazia or
after previous ocular surgery (pterygium removal or
strabismus) or surrounding leftover foreign bodies
● Typically occurs 3-4 weeks after surgery
● Histology: mixture of acute and chronic inflammatory
cells with capillaries in a spoke-wheel pattern
● Treatment: topical or intralesional steroids. If
refractory, cautery to base and frequent postoperative
steroids
120
What does Alizarin red stain?
● Stains calcium red
121
What does a retinal cavernous hemangioma look like on pathology? What does the IVFA show?
● Pathology shows thin-walled, dilated vascular
channels
● IVFA shows slow filling of the lesion with pooling of
dye in the upper part of the vascular space. There
is no leakage on the IVFA
122
What does a choroidal hemangioma show on IVFA?
● IVFA shows large choroidal vessels seen in the arterial phase and late staining of the lesion
123
What is the appearance of a retinal hemangioblastoma on IVFA?
● Rapid AV phase with rapid filling of the tumor; massive leakage of dye into the tumor can also occur
124
What is the appearance of the IVFA in Coats disease?
● Telangiectatic vessels with leakage of dye into the vitreous
125
What pathologies are associated with corneal pannus?
● Trachoma
● Molluscum contagiosum
● Contact lens overwear
● Chemical injuries
● Chronic blepharoconjunctivitis
126
What is a MALT lymphoma?
● A low grade conjunctival lymphoma
127
Where are the Muller cell nuclei located?
● Inner nuclear layer
● Project process in both anterior and posterior
direction
● Form inner limiting membrane (a true basement
membrane)
● Form the outer limiting membrane (not a true
basement membrane, represents adherens
junctions between Muller cells and photoreceptor
cell inner segments)
128
What is the diameter and thickness of the adult lens?
● Diameter = 9 mm
● Depth = 5 mm
129
What are the causes of rupture of Descemet’s membrane?
● Vertical oblique:
○ Forceps injury during delivery
○ Hydrops associated with keratoconus and
pellucid marginal degeneration
● Horizontal
○ Congenital glaucoma (“Haab striae” -
horizontal lines)
● Terrien marginal degeneration
130
What are the causes of noncaseating granulomas?
● Sarcoidosis
● FB reaction
● Fungal infection
131
What are the types of Iridocorneal endothelial (ICE) syndrome?
● Essential Iris Atrophy
● Cogan-Reese Syndrome
● Chandler’s Syndrome
● Classic presentation: middle aged caucasian
woman with unilateral glaucoma whose pupil is
also slightly irregular
● Pathology shows Descemet’s membrane
abnormally covers anterior iris surface
132
What is the nomenclature for neoplasms of epithelium and soft tissue, respectively?
● Epithelium:
○ Benign = hyperplasia or adenoma
○ Malignant = adenocarcinoma
● Soft Tissue:
○ Benign = “-oma”
○ Malignant = “-sarcoma”
133
What are the clinical features of iris melanomas suggestive of malignancy?
● Growth
● Increased vascularity
● Sectoral cataract
● Glaucoma
● Ectropion iridis
● Most are inferior or inferotemporal
● Lower mortality rate compared to ciliary body or
choroidal melanomas
134
What intraocular metastases have the best prognosis for patient survival?
● Breast cancer
● Metastatic carcinoid tumors
135
What is cavernous optic atrophy of Schnabel?
● Occurs in glaucoma patients after acute IOP elevation
● Large cystic spaces with mucopolysaccharides
● Stains with alcian blue or colloidal iron
136
In which areas is the vitreous strongly attached?
● Vitreous base (strongest attachment): 2 mm anterior
and 4 mm posterior to ora serrata
● Macula
● Margin of optic disc
● Along major retinal blood vessels
● Margins of lattice degeneration
● Sites of chorioretinal scars
137
What is the sequence of events for age-related PVD?
● Liquified vitreous enters a shallow separation of
perifoveal cortical vitreous through a cortical tear
and detaches the vitreous overlying the macula =
partial PVD (asymptomatic)
● Separation of vitreous from optic disc margin
(symptomatic and Weiss ring)
● Occasionally vitreous traction at posterior margin
of the vitreous base can cause retinal break
138
What is the epithelial lining of the inner portion of the ciliary body?
● Lined by double layer of epithelial cells
○ Inner non-pigmented (faces posterior
chamber) and outer pigmented layer (faces
ciliary muscle)
139
What is a medulloepithelioma?
● Rare tumor of nonpigmented epithelium of the ciliary body
● Neuroepithelial cell derived from primitive medullary epithelium
(inner layer of optic cup)
● Usually presents in children age 4-12 years old
● Can be benign or malignant
● Rarely causes secondary glaucoma, hyphema, ectopia lentis
● Pathology shows ribbons and cords of primitive round and oval cells,
multiple layers of neuroepithelial tables lined by thin basement
membrane; mucinous cysts and Flexner-Wintersteiner rosettes
● Can be seen in the retina and optic nerve
● B scan shows large cysts
● Treatment: observation or enucleation before orbital invasion (do not
resect as it induces metastatic spread)
140
What is stained by S-100 stain?
● Stains melanoma cells
141
What type of inflammation is caused by fungal infection?
● Necrotizing granulomatous inflammation
142
What are the most common primary tumor sites that give rise to intraocular metastases in men and women?
● Men:
○ 1st - lung
○ 2nd - unknown
● Women
○ 1st - breast
○ 2nd - lung
143
What are the three layers of sclera?
● Outermost to innermost:
○ Episclera
○ Stroma
■ Makes up the majority of the sclera
■ Made of type I collagen
■ Affected by osteogenesis imperfecta
○ Lamina fusca (most strongly attached to
uvea)
144
What is true exfoliation of the lens?
● Lamellar delamination of the lens capsule leads to
splitting of anterior capsule
● Lens capsule is thickened and superficial portion of
anterior lens capsule splits from deeper layer
● Main risk factor: intense infrared radiation
145
What is the appearance of the lens of rubella cataract?
● Microspherophakia lens
● Retained lens fiber nuclei
● During surgery there can be a significant
inflammatory response since there usually is virus
still present within lens
146
What is the shape of bleeding in the nerve fiber layer?
● Flame shaped
147
What is the shape of blood in the inner plexiform, inner nuclear, or outer nuclear?
● Dot blot, circular
148
What is the shape of bleeding in the sub-hyaloid or sub-ILM space?
● Boat shaped
149
What is the pattern of fluid in the outer plexiform layer?
● Petaloid/star shaped
150
What is the most common association with sclerocornea? What is the typical course of sclerocornea? What are the genetics behind sclerocornea?
● 80% of sclerocornea associated with cornea plana
● Sclerocornea is nonprogressive and
noninflammatory
● Ranges from involving the periphery to affecting
the entire cornea
● Most severe form is inherited, autosomal recessive
● 50% of the cases are sporadic
151
What is the cause of mucopolysaccharide material in cystic spaces of cavernous optic atrophy of Schnabel?
● Mostly seen in nonglaucomatous elderly patients
with generalized arteriosclerotic disease
● Commonly unilateral and in elderly women
● Mucopolysaccharide is produced in situ within
atrophic spaces of the optic nerve
152
In the Collaborative Ocular Melanoma (COM) Trial, what is the definition of a large choroidal melanoma? What were they studying in large melanomas? What were the results?
● Large choroidal melanoma:
○ > 16 mm basal diameter
○ > 10 mm apical height
● Compared enucleation alone vs external beam
radiotherapy followed by enucleation
● Findings:
○ Adjunctive radiotherapy did not impact
overall survival
○ Primary enucleation alone is sufficient to
manage large melanomas
153
In the Collaborative Ocular Melanoma (COM) Trial, what is the definition of a medium choroidal melanoma? What were they studying in medium melanomas? What were the results?
● Medium choroidal melanoma:
○ 6-16 mm basal diameter
○ 2.5-10 mm apical height
● Studied enucleation vs iodine 125 brachytherapy
● Findings:
○ All cause mortality and metastases at 5 years
were equivalent between the two groups
154
In the Collaborative Ocular Melanoma (COM) Trial, what is the definition of a small choroidal melanoma? What were they studying in small melanomas? What were the results?
● Small choroidal melanoma:
○ 4-8 mm basal diameter
○ 1-2.4 mm apical height
● Studied mortality in small choroidal melanomas
● Findings:
○ Melanoma specific mortality of 1% at 5 years
155
What are the predictive factors for malignant transformation of a choroidal nevus to a melanoma?
● TFSOM-UHHD
○ Thickness > 2 mm
○ Fluid, subretinal
○ Symptoms: flashes and floaters, decreased
visual acuity
○ Orange pigment
○ Margin within 3 mm of the optic disc
○ Ultrasound hollowness
○ Halo absence
○ Drusen absence
● Monitoring:
○ If no features: monitor q6 months initially and
then annually if no changes
○ 1-2 features: monitor q4-6 months
○ 3 or more features: refer to ocular melanoma
center
156
What cells synapse in the lateral geniculate nucleus?
● Ganglion cells
157
What are the three main groups of retinal ganglion cells?
● Tonic cells stimulated by L or M cones
○ Project to the parvocellular layer of the
lateral geniculate nucleus (LGN)
○ Important in high resolution and color vision
● Tonic cells stimulated by S cones
○ Designed to detect successive color contrast
○ Excited by short waves entering and long
waves leaving their receptive fields
● Phasic cells
○ Larger, less concentrated in the govea
○ Project to the magnocellular layer of the LGN
○ Important for the detection of movement
158
What is a pleomorphic adenoma and how should they be managed?
● Benign epithelial tumor of the lacrimal gland
● Recurrent pleomorphic adenomas can lead to
adenoid cystic carcinoma (malignant)
● Incisional biopsies should not be performed
because can recur if the capsule is incised
○ Recurrences can be very difficult to remove
159
What is a Schwannoma and what does it look like on histology?
● Encapsulated, benign tumor that arises from Schwann cells
● Rarely transforms into a malignant tumor
● Histology: Biphasic pattern
○ Antoni A: nuclear palisading; spindle cells arrange in
interlacing cords, whorls, or palisades that form verocay
bodies (*)
○ Antoni B: loose myxoid association of cells; stellate cells that have
mucous stroma
○ Antoni B: loose myxoid association of cells; stellate cells that have
mucous stroma
160
What layer of the cornea makes Descemet’s membrane? What are the layers of Descemet’s membrane? What substance makes up Descemet’s membrane?
● Produced by corneal endothelium constantly
throughout life, thus it becomes thicker with age
● Anterior banded zone and posterior nonbanded
zone
● Made of Type IV collagen (it is a true basement
membrane)
161
Which immunostains are used for melanocytes?
● HMB-45
● Melan A
162
What is stained by Ki-67 stain?
● Proliferative cells
163
What is stained by Desmin stain?
● Smooth and skeletal muscle
● Useful for diagnosing rhabdomyosarcoma
164
What does Chromogranin stain?
● Stains cells of neuroendocrine origin
● Positive in Merkel Cell carcinoma
165
What does an adenoid cystic carcinoma look like on histology? What is the prognosis? What is the management?
● Swiss cheese (“cribriform”) pattern on histology
● Usually in women
● Highly malignant with significant pain and poor
prognosis
● Exenteration is recommended with removal of
adjacent bone
● Usually in women
● Highly malignant with significant pain and poor
prognosis
● Exenteration is recommended with removal of
adjacent bone
166
What is stained by colloidal iron stain?
● Glycosaminoglycans, stain blue
● Used in macular corneal dystrophy
167
What is Oil red O stain used for and what does it help diagnose?
● Used to stain fresh tissue specimens
● Stains lipid
● Helpful in identifying sebaceous cell carcinoma
168
What is stained by Sudan Black B?
● Lipid
● Useful in diagnosing sebaceous cell carcinoma
169
What is stained by Congo red stain?
● Stains amyloid an orange-red color
● Used in lattice corneal dystrophy
170
What is the typical presentation of choroidal metastases?
● Flat, ill-defined, yellow-White to gray-yellow lesions
● Associated with overlying RPE changes in leopard
spotting pattern
171
What location of basal cell carcinoma is likely to invade into the orbit?
● Medial canthus
172
What is the most common cell origin for optic nerve glioma? What is the histologic appearance of an optic nerve glioma? What are optic nerve gliomas associated with? What is the typical prognosis? What are the treatment options?
● Pilocytic astrocyte
● Histology shows Rosenthal fibers (enlarged, deeply
eosinophilic filaments)
● Associated with neurofibromatosis type 1
● Slow growing, with long term survival
● Typically observed unless progressive visual loss or
growth more posterior into the CNS
● Treatment of gliomas itself can result in vision loss
in the affected eye
173
What are the causes of granulomatous conjunctivitis?
● Sarcoidosis
● Bartonella
● Syphilis
174
What is the treatment for ocular lymphoma?
● Radiation
● Intravitreal methotrexate
● Systemic chemotherapy that penetrates the
blood-ocular barrier
175
What testing should be done for evaluation for metastatic choroidal melanoma?
● Liver imaging with ultrasound
● Liver function testing
● Chest x-ray
● If these are abnormal then get a liver CT, CT-PET of
abdomen/chest or MRI of abdomen/chest
176
What are the most common sites of conjunctival melanoma metastasis?
● Head and neck lymph nodes
● They act more like cutaneous melanomas
177
What is the most common type of isotope used for brachytherapy to treat choroidal melanoma?
● Iodine 125
178
Where are goblet cells most commonly found? What is their function?
● Most common in the fornices and inferonasal
bulbar conjunctiva (near the medial canthus)
● Produce mucin (the deep layer of the tear film)
179
What are the functions of the mucin produced by goblet cells?
● Fills spaces between microvilli of corneal epithelial
cells to provide uniform surface
● Lower surface tension of tear film and stabilize it
● Lubricate the eyelids as they traverse the globe
during blinking
● Trap foreign bodies on the surface of the eye
● Converts the hydrophobic surface of the corneal
epithelium to a hydrophobic one which allows for
an even distribution of the tear film
180
What is the differential for leukocoria?
● Persistent fetal vasculature
● Retinal detachment
● Congenital cataracts
● Coats disease
● Retinopathy of prematurity
● Coloboma of the choroid or optic disc
● Toxocariasis
● Uveitis
181
What is the most common location of squamous cell carcinoma of the eyelid?
● Lower eyelid
● Arise in sun damaged skin
182
What is a Fuchs adenoma? What does it look like on pathology?
● Benign mass of the ciliary body epithelium
● Usually an incidental finding of autopsy and does
not cause problems
● Histology shows a glistening white, irregular mass
that is made up of a benign proliferation of
nonpigmented ciliary epithelium and accumulation
of basement membrane material
183
What are the indications for frozen sections?
● Determine whether margins are clear of tumor
cells
● Provide fresh tissue for molecular genetics
● Determine whether representative tissue has been
biopsied for diagnosis
● Ensure tissue conservation (Mohs surgery)
● Definitive diagnosis of a lesion requires permanent
sections, not a frozen section
184
What are the stains used to diagnose amyloid?
● Congo red
● Thioflavin T (stains fluorescent yellow)
● Crystal violet (stains bluish and polarized light
makes it violet)
185
What cultures can be used for aerobic bacteria?
● Blood agar
● Chocolate agar
● Thioglycollate broth
186
What cultures can be used for anaerobic bacteria?
● Anaerobic blood agar
● Phenylethyl alcohol agar in anaerobic chamber
● Phenylethyl alcohol agar in anaerobic chamber
● Thioglycollate broth
● Chopped meat broth
187
What cultures can be used for mycobacteria?
● Blood agar
● Lowenstein-Jensen agar
188
What cultures can be used for fungi?
● Blood agar (25°C)
● Sabouraud dextrose agar (25°C)
● Brain-heart infusion agar
189
What cultures can be used for acanthamoeba?
● Non-nutrient agar with bacterial overlay (E. coli or
Enterobacter aerogenes)
● Blood agar
● Buffered charcoal-yeast extract agar
190
What are the common associations and symptoms of the different types of cataracts?
● Nuclear: second sight, greater impairment of
distance than near vision; seen after hyperbaric
oxygen therapy
● PSC: decreased vision with near more than
distance; seen after prednisone usage
● PSC/Cortical: glare
● PSC/cortical/nuclear: monocular diplopia
● Cortical: common in African Americans, cortical
clefts occur with extreme glucose shifts
● Sunflower: seen in Wilson’s disease
● Christmas tree: Myotonic dystrophy
191
What is the most common cause of fungal keratitis in the US?
● Fusarium
● Most occur in southern US
● Treatment is natamycin 5% until cultures are back
(commercially available)
192
What organisms is Amphotericin B used to treat?
● Used for candida and aspergillus
● Made by a compounding pharmacy
193
What is pagetoid spread?
● Individual cell proliferation (i.e. malignant
melanocytes) in the upper levels of the epidermis
of tissue (i.e. conjunctiva)
194
What is the risk of progression to melanoma in PAM With atypia?
● 46%
● PAM without atypia rarely progresses to
melanoma
195
What causes “lacy vacuolation of iris pigment epithelium?”
● Diabetes
● Blood sugars > 200 at the time of death
demonstrate lacy vacuolation of iris pigment
epithelium on H/E attributed to excess stores of
glycogen
196
What are the strongest point of sclerouveal attachment?
● Major emissarial canals
● Anterior base of the ciliary body
● Juxtapapillary region
197
What is the thickness of the sclera posterior to the extraocular muscle insertion?
● 0.3 mm
198
What part of the sclera merges with the dura of the optic nerve sheath?
● Outer two-thirds of the sclera
199
What is Meesmann corneal dystrophy? What does histology show? What are the genetic associations?
● Epithelial corneal dystrophy that shows punctate,
blister-like, round to oval opacities within the
corneal epithelium, best seen on retroillumination
● Typically presents early in life with mild foreign
body sensation and slight decreased visual acuity
● Histology shows coarse basement membrane,
fibrillogranular material within epithelial cells and
cysts = “peculiar substance”
● Mutations of KRT3 or KRT12
● Autosomal dominant inheritance
200
During the dissection of an enucleated globe, What is the desired section of the majority of the specimen?
● Pupil-optic nerve (PO section)
201
What is the appropriate method for processing with formalin fixative?
● Do not open the eye prior to soaking in formalin
● Entire eye should be suspended in formalin for at
least 12 hours prior to processing (usually > 24
hours)
202
What are the risk factors for posterior subcapsular cataract?
● Chronic topical steroids
● Ionizing radiation
● Chronic vitritis
● Posterior migration of lens epithelial cells from the
equator, during which time the cells swell to 5x the
normal size. These are called bladder cells/Wedl
cells
203
What pathology are Rosenthal fibers associated with?
● Optic nerve gliomas
204
What pathologies are psammoma bodies associated with?
● Optic nerve meningioma
● Pituitary prolactinoma
205
What pathology are Homer Wright rosettes associated with?
● Neuroblastoma
● Medulloblastoma of the cerebellum
● Retinoblastoma
206
What cytokines are elevated in neoplastic processes? In inflammatory processes?
● IL-10 is elevated in neoplastic processes
● IL-6 is elevated in inflammatory processes
● IL-10 > IL-6 is suggestive of lymphoma
207
What is a hemangiopericytoma of the orbit?
● Uncommon, encapsulated tumors that present in
midlife with proptosis with or without pain
● Tumor composed of pericytes with ill-defined
borders, large cytoplasm, and round nuclei
● Pericytes surround “staghorn-shaped” blood
channels
208
What is the most common type of malignant intraocular (or orbital) tumor?
● Metastases
209
What is the most common type of primary malignant intraocular tumor in adults?
● Choroidal melanoma
210
What is the most common primary intraocular tumor in children?
● Retinoblastoma
211
What is the most common route from which retinoblastoma spreads outside the eye?
● Optic nerve
212
What are the two types of lymphoma that spread to the eye?
● Primary central nervous system lymphoma
○ Significantly more common
○ Involves the retina and vitreous
● Visceral or nodal lymphoma
○ Involves the uveal tract (posterior choroid,
iris) like other systemic mets
● Histology of intraocular lymphoma shows
non-Hodgkin B cell
213
What are the two types of peripheral cystoid degeneration?
● Typical
○ Cystic spaces in the retina that form at the
level of the outer plexiform layer
● Reicular
○ Cystic spaces in the retina at the level of the nerve fiber layer
● When cystic cavities coalesce they form schisis
214
What are Henderson-Patterson corpuscles and what are they associated with?
● Eosinophilic inclusion bodies
● Seen in molluscum contagiosum
215
What is the treatment of CIN?
● Complete excision with cryotherapy to surgical
margins and adjunctive treatment with antimetabolite (i.e. mitomycin C)
216
What is a dermoid cyst? What causes it? Where are they most commonly located?
● Cyst lined by stratified squamous epithelium with keratinization
● May contain keratin and hair (choristoma)
● Wall of cyst has adnexal structures: sebaceous glands, sweat glands, hair follicles
● Rupture of cyst produces prominent granulomatous inflammatory reaction
● Result of abnormal embryonic epithelial nest that becomes entrapped
● Most often in superotemporal orbit near frontozygomatic suture
217
What is the most common organism causing infectious crystalline keratopathy?
● Strep viridans
218
What pathology is associated with elongated ciliary processes?
● Persistent Fetal Vasculature
219
“Disinsertion of the longitudinal muscle of the ciliary body from the scleral spur” describes what anterior chamber pathology?
● Cyclodialysis
220
A tear between the longitudinal and circular fibers of the ciliary muscle describes what anterior chamber pathology?
● Angle recession
221
What is the most common benign orbital tumor in adults?
● Cavernous hemangioma
222
What is the most common malignant orbital tumor in adults?
● Lymphoma
223
What is the most common primary intraocular tumor in adults?
● Melanoma
224
What is the most common intraocular tumor overall?
● Metastases
225
What is the most common benign orbital tumor in children?
● Capillary hemangioma
● Other sources say benign cystic lesions
(epidermoid or simple epithelial cysts)
226
What is the most common malignant orbital tumor in kids?
● Rhabdomyosarcoma
227
What were the criteria for intervening on carotid stenosis per the “North American Symptomatic Carotid Endarterectomy Trial?”
● Amaurosis fugax or hemispheric TIAs with 70-99%
carotid stenosis benefitted from carotid
endarterectomy to reduce risk of future stroke
● Endarterectomy for complete occlusion carotid
artery has low success rate for re-establishing flow
and high mortality
● Asymptomatic carotid stenosis is less reliable
228
What are causes of congenital Horner’s syndrome?
● Birth trauma-related brachial plexus injuries
● Neuroblastoma
229
What is Waardenburg Syndrome and what are the classic findings?
● Rare genetic disorder due to abnormal neural crest
migration
● Presentation:
○ Sensorineural deafness
○ Dystopia canthorum
○ Heterochromia
○ White forelock of hair arising from anterior scalp
○ Hirschprung Disease
230
What are the types of rhabdomyosarcoma?
● Alveolar
○ Most malignant
○ Think “Alveolar Awful”
● Embryonal
○ Most Common
○ Think “Embryonal Everyone”
● Pleomorphic
○ Best prognosis
○ Think “Pleomorphic Please”
● Botryoid
231
What is a capillary hemangioma? What is the typical prognosis?
● Proliferation of capillary endothelial cells
● Rapid growth during the first 12 months of life
● Typically regresses and involutes with complete
regression around 5 years of age
232
What is the Goldmann-Witmer Coefficient? What is it used for?
● Test that compares intraocular antibody
production to serum as measured by ELIZA or radioimmunoassay
● GW ratio > 3 is diagnostic of local antibody
production to a specific microbial pathogen
● GW ratio > 3 is diagnostic of local antibody
production to a specific microbial pathogen
233
What are the ocular adverse effects of reactive oxygen species?
● Cataract
● ARMD
● Diabetic Retinopathy
● Glaucoma
234
What is posterior embryotoxon and what is it associated with?
● Prominent and anterior displacement of Schwalbe
line
● Schwalbe line is the junction of Descemet
membrane and trabecular meshwork
● Can occur as an isolated finding
● Also seen in Axenfeld-Rieger Syndrome and Alagille syndrome
235
What is the histologic appearance of an iris nevus?
● Proliferation of branching dendrites, melanocytic cells, typically oblong or ovoid, bland appearance in iris stroma
236
What is the histologic appearance of a Spindle-B cell type melanoma?
● Plump spindle-shaped melanocytes with granular appearing nuclei and prominent nucleoli
237
What is the histologic appearance of an epithelioid melanoma?
● Polyhedral cells with large, round nuclei with clumped chromatin and prominent eosinophilic nucleoli
238
What is the classic histologic appearance of Juvenile xanthogranuloma?
● Large histiocytes with foamy cytoplasm, Touton
giant cells, lymphocytes, and occasional eosinophils
239
What is the classic histologic appearance of Ghost cell glaucoma?
● Ghost cells (small, khaki-colored RBCs) with Heinz bodies
240
What is the classic histologic appearance of phacolytic glaucoma?
● Lipid-laden macrophages
241
What is the classic histologic appearance of CMV infection?
● Retinal necrosis with large eosinophilic
intranuclear (Owl’s eye inclusions, see below) or
intracytoplasmic inclusion bodies
242
What is the classic histologic appearance of retinoblastoma?
● Cells with round or oval nuclei, approximately
twice the size of a lymphocyte; high mitotic
activity; necrosis, calcification, and pseudorosettes
consisting of viable tumor cells (asterisk)
surrounding a blood vessel (arrow);
Flexner-Wintersteiner rosettes (central lumen lined
by refractile structure corresponding to ELM);
fleurettes (differentiated photoreceptors)
243
What is the classic histologic appearance of Bilateral Diffuse Uveal Melanocytic Proliferation (BDUMP)?
● Infiltration of the uveal tract with benign nevoid or spindle-shaped cells
244
What is the classic histologic appearance of tuberculosis?
● Caseating granulomas
245
What are paradoxical pupils and what are the causes?
● Constriction in response to darkness
● Causes:
○ Retinal dystrophies
○ Optic neuropathies
246
What is the appearance of mixed-cell type melanomas?
● Spindle-shaped and polyhedral melanocytic cells with prominent atypia and pleomorphism
247
Rank the risk for systemic lymphoma from lowest to highest based on the following locations: eyelid, orbital, conjunctiva.
● Conjunctiva < orbital < eyelid
● Bilateral involvement also increases the risk of
systemic disease greatly
● Risk of systemic disease increases for decades after
original lesion is diagnosed
248
What is the appearance of Dalen-Fuchs nodules on histology?
● Epithelioid histiocytes and lymphocytes between
Bruchs membrane and the RPE (predominantly histiocytes)
● Pigment phagocytosis
249
What is the typical course of a keratoacanthoma?
● Rapid growth over 4-8 weeks
● History of spontaneous regression over several months
● Considered to be a variant of differentiated
squamous cell carcinoma
● Possibility of perineural invasion and metastases
250
What are the mesenchymal tumors?
● Fibrous histiocytoma
● Osteoma
● Solitary fibrous tumor
● Fibrous dysplasia
● Rhabdomyosarcoma
251
What is the genetic association of persistent fetal vasculature? How often is it unilateral? What are the mild findings? What are the severe findings?
● Congenital anomaly that is sporadic
● Unilateral in ⅔ of cases
● Mild:
○ Prominent hyaloid vessels
○ Mittendorf dot
○ Bergmeister papilla
● Severe:
○ Microphthalmos
○ Ciliary process elongation
○ Retrolenticular plaque
○ Thick, fibrous persistent hyaloid artery
○ Prominent radial iris vessels
252
What is a duplication cataract and what is it caused by?
● Metaplasia of lens epithelium causing anterior
subcapsular fibrous plaques
● Occurs after injury to the lens epithelium
253
What are the advantages and disadvantages of flow cytometry?
● Advantages
○ Shows percentage of particular cells in a specimen
● Disadvantages
○ Failure to show location and distribution of these cells
○ Sampling errors
254
What is the name of the antigenic site on an antibody? On a foreign molecule?
● Antibody: idiotope
● Foreign molecule: epitope
255
What organisms are able to invade through an intact corneal epithelium?
● Neisseria gonorrhoeae
● Neisseria meningitidis
● Corynebacterium diphtheriae
● Listeria monocytogenes
● Shigella
● Haemophilus influenzae, biotype III (formerly
Haemophilus aegyptius)
● Fusarium
256
What is the definition of a choristoma? A hamartoma?
● Choristoma: normal tissue in an abnormal location
(eg limbal dermoid)
● Hamartoma: abnormal tissue in a normal location
(eg capillary hemangioma, Lisch nodules)
257
What determines the color of the iris?
● The size and number of melanin pigment granules
in the anterior stromal melanocytes
258
What is the histologic appearance of sarcoidosis?
● Noncaseating granulomas and conjunctival nodules
259
What are the similarities and differences of MHC class I and class II?
● Similarities:
○ Both are heterodimers
○ Both are made up of alpha and beta chains
○ Alpha subunits for both are encoded by the
MHC gene
● Differences:
○ Beta subunit in MHC I is encoded by beta-2
microglobulin gene and is constant across all
MHC I molecules
○ Beta subunit in MHC II is encoded by the
MHC gene
260
What is ocular melanocytosis (oculodermal melanocytosis, nevus of ota) and what is the epidemiology and genetic associations?
● Hyperpigmentation of the episclera and uvea
● Histology shows increased dendritic melanocytes in
the episclera, sclera, and dermal layers of the skin
● Associated with cutaneous hyperpigmentation along
CN V
● Common in Asian and African Americans
● In white populations it increases the risk of uveal
melanoma to 1:400 (from 1:13,000 in general white
population)
● Associated with hyperpigmentation of the iris and
choroid
● Mutation in GNAQ and GNA11
261
What is the appearance of a fibrous histiocytoma on histology?
● Proliferation of spindle fibroblasts and plump
histiocytic cells in a focal storiform (matlike)
architecture
262
What is the epidemiology, risk factors, histology, and indications for treatment/excision of iris melanomas?
● Iris melanomas make up 3-5%% of all uveal melanomas
● Low mortality: 1-4%
● Risk factors:
○ Light skin, light iris, oculodermal melanocytosis in daily
pigmented patents
● Most are spindle cell melanomas, unlike choroidal melanomas
which are mostly mixed
● Can cause extrascleral extension and seeding/secondary
glaucoma which can be treated with local radiation or
enucleation
● Indication for excision is growth involving the angle/TM up to
3-4 clock hours
263
What histologic feature is high risk for malignancy of a conjunctival pigmented lesion?
● Epithelioid morphology (large, hyperchromatic
nuclei, prominent nucleoli, and moderate to
abundant cytoplasm) and pagetoid spread
(migration into the superficial epithelium)
264
What is the appearance of a compound nevus on histology?
● Epithelial cysts in substantia propria surrounded by
pigmented cells
265
What are the regulators for anterior chamber associated immune deviation (ACAID)?
● Transforming growth factor beta (TGF-beta) and
vasoactive intestinal peptide (VIP)
266
What is the immune function of interferon gamma?
● Causes infiltration of neutrophils and macrophages
267
What role does Substance P play in immunity?
● Plays a role in vascular permeability
● Causes leukocyte infiltration
268
What role does TNF-alpha play in immunity?
● Involved in mononuclear cell infiltration
269
What syndrome are RPE hamartomas associated with?
● Familial Adenomatous Polyposis
● Autosomal dominant
● Develop colon cancer
270
What is Gardner syndrome and what is it characterized by?
● Familial Adenomatous Polyposis
● Cutaneous cysts
● Stemmas
● Odontomas
● Extracolonic cancers
● Atypical CHRPE
271
What is a CHRPE?
● Solitary, well demarcated, flat darkly pigmented
subretinal plaque
● Usually between 1-19 mm
● Non-hereditary and sporadic
● Areas of depigmentation = lacuna
● Mild growth can be seen
● Cells are taller than normal RPE
● Densely packed with large, spherical melanin
pigment granules
● Hyperplasia and hypertrophy
272
What is an eccrine hidrocystoma?
● Cyst that develops from eccrine glands
● Tend to cluster around lower eyelids and canthi of
the face
● They often enlarge in heat or humidity
273
What is an apocrine hidrocystoma?
● Cyst arising from the glands of Moll (AKA cyst of
Moll)
● Appears blue or translucent and transilluminates
274
What is an epidermoid cyst?
● White, rubbery or firm
● Lined by stratified squamous keratinized
epithelium and containing keratin
275
How should you biopsy a sebaceous adenocarcinoma?
● Wide surgical excision is mandatory
● A conjunctival map biopsy is helpful to eliminate
potential of pagetoid spread
276
What is conjunctival intraepithelial neoplasia (CIN)?
● Full thickness dysplasia of the epithelial layer
● Associated with HPV 16 & 18 and sessile papillomas
277
What is the differential for pseudopapilledema?
● Optic nerve drusen
● Hyperopic disc
● Prominent glial tissue
● Optic nerve myelination
278
What is the histologic definition of the macula?
● Where the ganglion cell layer is thicker than a
single cell
279
What is the histologic definition of the foveola?
● Contains only cone photoreceptors, ganglion cells,
and other nucleated cells (Muller cells)
● Blood vessels are absent
280
What are the features of a phthisical globe?
● Think “SAD” Shrinkage, Atrophy, and
Disorganization
● Low IOP due to shrinkage of the eye
● Intraocular ossification occurs late: osseous
metaplasia of the RPE with bone formation
281
What pathology has zonal granulomatous inflammation? Nodular granulomatous inflammation? Diffuse granulomatous inflammation?
● Zonal = phacoantigenic uveitis
● Nodular = sarcoidosis
● Diffuse = sympathetic ophthalmia
282
What is the differential for macular drusen in patients younger than 50 years of age?
● Familial dominant drusen
● Cuticular drusen
● Large colloid drusen
● Sorsby macular dystrophy
● Membranoproliferative glomerulonephritis type 2
● Malattia leventinese
283
What is the role of insulin-like growth factor in the eye?
● Responsible for abnormal hyperplastic response of
lens epithelium and corneal endothelium observed
in inflammatory conditions
284
What is the role of fibroblast growth factor in the eye?
● Accelerates corneal wound repair after surgery,
chemical burns, or ulcers
285
What are the mucopolysaccharidoses (MPSs)?
● Errors in metabolism with impaired lysosomal
storage
● Associated with corneal clouding due to
accumulation of incompletely degraded
glycosaminoglycans
● Most MPSs are autosomal recessive
○ The exception is MPS II, AKA Hunter
Syndrome, which is X-linked recessive
286
What are glaukomflecken?
● Necrotic lens epithelial cells and degenerated
subepithelial cortex that results from a hypoxic
insult
● Due to metaplasia that produces fibrous plaques
intermixed with basement membrane
287
What is xeroderma pigmentosum?
● Autosomal recessive disorder characterized by
impaired ability to repair sunlight-induced damage
to DNA
● Patients’ skin can have hyperpigmentation,
atrophy, actinic keratosis, and telangiectasias
● Cutaneous neoplasms are common later in life
● Ocular changes include keratoconjunctivitis sicca,
blepharospasm, conjunctival telangiectasias,
hyperpigmentation, symblepharon, eyelid atrophy,
scarring, trichiasis, ocular neoplasms (in 11% of
patients and more common at the limbus
288
What are the ichthyoses?
● Congenital, autosomal dominant skin disorder
characterized by desquamation
● Does not respond to steroids
● Can cause primary corneal opacities, BK, and
corneal vascularization
● Eyelids are frequently involved with scaling and
cicatricial ectropion
