What is the pathology of senile calcific plaque?;"• The pathology of senile calcific plaques shows basophilic calcific deposits in sclera anterior to the lateral rectus muscle" What is trilateral retinoblastoma?;"● Bilateral retinoblastoma + ectopic intracranial retinoblastoma (pineal gland or parasellar region) ● Histology: numerous round blue cells surrounded by pink necrotic cells" What is the term for epithelium growing on both sides of the cornea?;"● Epithelium growing on both sides of the cornea is termed epithelial downgrowth ● Most commonly seen from multiple prior surgeries such as ECCE and PKP" What are the characteristic findings in Peters Anomaly?;"● Peters anomaly is characterized by: ○ The absence of Descemet’s membrane ○ Adhesions of iris and lens material to the posterior surface of the cornea" What are the pathologic findings of ICE Syndrome?;"● Corneal edema ● Inappropriate proliferation of endothelium and/or Descemet’s membrane along anterior segment structures" What are the characteristic findings in Granulomatosis with Polyangiitis (GPA) and what vessels are involved?;"● Chronic sinusitis ● Saddle Nose deformity ● Granulomatous inflammation with necrosis ● Small and medium size blood vessels are usually involved" What is the most common cause of proptosis in adults?;"● Thyroid-associated orbitopathy ● Pathology: muscle bundles separated by fluid and infiltrated by a mixture of mononuclear inflammatory cells " What conditions are associated with weakened zonules?;"● Congenital ○ Marfan’s ○ Homocystinuria ○ Hyperlysinemia ○ Ehler-Danlos Syndrome ○ Scleroderma ○ Weill-Marchesani ● Other ○ Pseudoexfoliation ○ Prior vitreoretinal surgery ○ History of trauma ○ High myopia" What is the most common extraocular tumor that spreads into the eye via direct extension?;"● Squamous Cell Carcinoma of the Conjunctiva ○ Typically mucoepidermoid carcinoma or spindle cell variant" What is the management of a brown pigmented lesion of the palpebral conjunctiva?;"● 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" What is the presentation, prognosis, and management of lymphangioma?;"● 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" What was the main conclusion of the LARGE choroidal melanoma trial?;"● Adjunctive radiotherapy did not improve overall survival" What was the main conclusion of the MEDIUM choroidal melanoma trial?;"● Mortality was similar between brachytherapy and enucleation groups ● Histologically confirmed metastases were found in approximately 10% patients treated with enucleation" What was the main conclusion of the SMALL choroidal melanoma trial?;"● Mortality was 1% at 5 years with observation only" What cell type produces the myelin sheath of the optic nerve?;"● Oligodendrocyte" What is the function of microglial cells?;"● “Clean-up” function due to phagocytic activity" What function do astrocytes have?;"● Support and nutrition to the optic nerve and retina" What are the similarities and differences between the pathology of pterygia and pingueculae?;"● 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 " What is stained by periodic acid-Schiff (PAS) stain?;"● Type IV collagen, glycogen, and proteoglycans" What is stained by Prussian blue stain?;"● Iron" What is stained by Von Kossa stain?;"● Calcium" What is stained by cytokeratin stain?;"● Squamous epithelium" What are the favorable and unfavorable risk factors in conjunctival melanoma?;"● 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" What is a Mittendorf dot and where is it most commonly located?;"● Remnant of the hyaloid artery of primary vitreous at its attachment to the tunica vasculosa lentis ● Most commonly located inferonasally" 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" What is stained by Verhoeff-van Gieson Stain?;"● Elastin" What is HMB-45 a marker for?;"● Marker for melanocytic lesions" What cells does CD3 stain identify?;"● T cells" What cells does CD20 stain identify?;"● B cells" What does Warthin-Starry stain identify?;"● Bartonella henselae" What is the rate of metastasis of conjunctival melanoma?;"● 25%" What is the presentation of a ciliary body melanoma?;"● Can range from completely asymptomatic to visual loss, photopsias, and visual field loss" What is a dermolipoma and where is it typically located?;"● Type of choristoma ● Most commonly in the superotemporal quadrant ● Extends posteriorly into the orbit" 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" 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" What happens during a spasm of the near reflex?;"● Excessive convergence, accomodation, miosis ● Normal ductions, abduction deficit on version movements" What stains can be used to visualize acanthamoeba?;"● Stains: ○ H&E ○ PAS ○ Calcofluor white ○ Acridine orange ○ Gridley stain ○ Giemsa" 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" 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" 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" 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" What is stained by the Mart-1 immunostain?;"● Melanocytes" 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" 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" 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" 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" 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)" 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" 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" 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" 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" Rank the stromal corneal dystrophies from highest to lowest rate of recurrence.;"● Lattice > Granular > Macular" What percentage of women with choroidal metastases have history of breast cancer?;"● 90% of women have been treated for breast cancer in the past" 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" 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" 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" 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" 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" 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" What neoplasm is most likely to spread systemically with fine needle aspiration biopsy?;"● Retinoblastoma" 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" 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" What is Masson trichrome used to stain?;"● Hyaline in corneal stroma, for example in granular dystrophy" 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" 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" 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" What is snowflake degeneration?;"● Long term complication of IOLs made of PMMA material and an indication for explantation" What is a postoperative complication associated with hydrogel material?;"● Calcification" What is a post-op complication of silicone-based IOLs in eyes with asteroid hyalosis?;"● Deposits of calcium oxalate after a YAG cap" 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" What is the treatment for primary acquired melanosis?;"● Excision with cryotherapy" 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)" 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" 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" What is the other name for type 2 granular corneal dystrophy?;"● Avellino dystrophy" What is the other name for type 3 granular dystrophy?;"● Reis Buckler dystrophy" 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" 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" In what pathology is lipogranulomatous inflammation seen?;"● Typically seen in chalazia" 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" 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)" What is the microscopic appearance of papilloma?;"● Fibrovascular ores with finger-like projections going away from epidermis, surrounded by hyperplastic stratified squamous epithelium" 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" 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%" What chromosome is implicated in retinoblastoma?;"● Chromosome 13" What chromosome is involved in neurofibromatosis type 1?;"● Chromosome 17" What chromosome is involved in neurofibromatosis type 2?;"● Chromosome 22" What chromosome is involved in tuberous sclerosis?;"● Chromosome 9 for TSC1 (hamartin) ● Chromosome 16 for TSC2 (tuberin)" 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 " 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)" 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)" What is the structure of amyloid?;"● Final structure is beta-pleated sheet ● Ultrastructure shows nonbranching fibrils with diameter 75-100 angstroms" 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" 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" 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" 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" What is the most common orbital tissue to be involved in sarcoidosis?;"● Lacrimal gland" What is the fixative used for electron microscopy?;"● Glutaraldehyde" What is the fixative used for routine histopathologic analysis?;"● Formalin" What are absolute ethanol or methanol media used for?;"● Crystals (e.g. corneal urate crystals)" What is the fixative used for cytologic preparation?;"● Ethyl alcohol" What is the fixative used for immunofluorescence?;"● Michel fixative ● Zeus" What is Saccomanno medium used for?;"● Liquid specimens or smears (e.g. vitreous biopsies, fine needle aspirates)" 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" 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" 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)" 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" What is Muir-Torre Syndrome?;"● A syndrome of multiple sebaceous neoplasms in conjunction with visceral malignancies (GI or GU)" What is Erdheim-Chester Disease?;"● Subtype of xanthogranulomatous disease ● Pathology shows foamy histiocytes → Touton giant cells ● Associated with serious cardiac complications leading to death" What are the indications for enucleation for treatment of retinoblastoma?;"● Tumor that is > 50% of the globe ● Optic nerve involvement ● Anterior segment involvement" What are the three types of giant cells?;"● Langhans ● Touton ● Foreign body" 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 " 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 " What does foreign body giant cells look like on pathology?;"● Haphazard arrangement of nuclei within the cells " 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" 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" 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 " 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" 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 " What does Alizarin red stain?;"● Stains calcium red" 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" What does a choroidal hemangioma show on IVFA?;"● IVFA shows large choroidal vessels seen in the arterial phase and late staining of the lesion " 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 " What is the appearance of the IVFA in Coats disease?;"● Telangiectatic vessels with leakage of dye into the vitreous " What pathologies are associated with corneal pannus?;"● Trachoma ● Molluscum contagiosum ● Contact lens overwear ● Chemical injuries ● Chronic blepharoconjunctivitis" What is a MALT lymphoma?;"● A low grade conjunctival lymphoma" 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)" What is the diameter and thickness of the adult lens?;"● Diameter = 9 mm ● Depth = 5 mm" 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 " What are the causes of noncaseating granulomas?;"● Sarcoidosis ● FB reaction ● Fungal infection" 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" 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”" 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" What intraocular metastases have the best prognosis for patient survival?;"● Breast cancer ● Metastatic carcinoid tumors" 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" 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" 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" 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)" 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) " What is stained by S-100 stain?;"● Stains melanoma cells" What type of inflammation is caused by fungal infection?;"● Necrotizing granulomatous inflammation" 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" 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)" 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" 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" What is the shape of bleeding in the nerve fiber layer?;"● Flame shaped" What is the shape of blood in the inner plexiform, inner nuclear, or outer nuclear?;"● Dot blot, circular" What is the shape of bleeding in the sub-hyaloid or sub-ILM space?;"● Boat shaped" What is the pattern of fluid in the outer plexiform layer?;"● Petaloid/star shaped" 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" 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" 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" 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" 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" 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" What cells synapse in the lateral geniculate nucleus?;"● Ganglion cells" 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" 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" 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 " 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)" Which immunostains are used for melanocytes?;"● HMB-45 ● Melan A" What is stained by Ki-67 stain?;"● Proliferative cells" What is stained by Desmin stain?;"● Smooth and skeletal muscle ● Useful for diagnosing rhabdomyosarcoma" What does Chromogranin stain?;"● Stains cells of neuroendocrine origin ● Positive in Merkel Cell carcinoma" 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" What is stained by colloidal iron stain?;"● Glycosaminoglycans, stain blue ● Used in macular corneal dystrophy" 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" What is stained by Sudan Black B?;"● Lipid ● Useful in diagnosing sebaceous cell carcinoma" What is stained by Congo red stain?;"● Stains amyloid an orange-red color ● Used in lattice corneal dystrophy" 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" What location of basal cell carcinoma is likely to invade into the orbit?;"● Medial canthus" 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" What are the causes of granulomatous conjunctivitis?;"● Sarcoidosis ● Bartonella ● Syphilis" What is the treatment for ocular lymphoma?;"● Radiation ● Intravitreal methotrexate ● Systemic chemotherapy that penetrates the blood-ocular barrier" 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" What are the most common sites of conjunctival melanoma metastasis?;"● Head and neck lymph nodes ● They act more like cutaneous melanomas" What is the most common type of isotope used for brachytherapy to treat choroidal melanoma?;"● Iodine 125" 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)" 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" 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" What is the most common location of squamous cell carcinoma of the eyelid?;"● Lower eyelid ● Arise in sun damaged skin" 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" 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" 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)" What cultures can be used for aerobic bacteria?;"● Blood agar ● Chocolate agar ● Thioglycollate broth" 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" What cultures can be used for mycobacteria?;"● Blood agar ● Lowenstein-Jensen agar" What cultures can be used for fungi?;"● Blood agar (25°C) ● Sabouraud dextrose agar (25°C) ● Brain-heart infusion agar" 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" 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" 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)" What organisms is Amphotericin B used to treat?;"● Used for candida and aspergillus ● Made by a compounding pharmacy" What is pagetoid spread?;"● Individual cell proliferation (i.e. malignant melanocytes) in the upper levels of the epidermis of tissue (i.e. conjunctiva)" What is the risk of progression to melanoma in PAM With atypia?;"● 46% ● PAM without atypia rarely progresses to melanoma" 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" What are the strongest point of sclerouveal attachment?;"● Major emissarial canals ● Anterior base of the ciliary body ● Juxtapapillary region" What is the thickness of the sclera posterior to the extraocular muscle insertion?;"● 0.3 mm" What part of the sclera merges with the dura of the optic nerve sheath?;"● Outer two-thirds of the sclera" 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" During the dissection of an enucleated globe, What is the desired section of the majority of the specimen?;"● Pupil-optic nerve (PO section)" 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)" 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" What pathology are Rosenthal fibers associated with?;"● Optic nerve gliomas" What pathologies are psammoma bodies associated with?;"● Optic nerve meningioma ● Pituitary prolactinoma" What pathology are Homer Wright rosettes associated with?;"● Neuroblastoma ● Medulloblastoma of the cerebellum ● Retinoblastoma" 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" 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 " What is the most common type of malignant intraocular (or orbital) tumor?;"● Metastases" What is the most common type of primary malignant intraocular tumor in adults?;"● Choroidal melanoma" What is the most common primary intraocular tumor in children?;"● Retinoblastoma" What is the most common route from which retinoblastoma spreads outside the eye?;"● Optic nerve" 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" 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" What are Henderson-Patterson corpuscles and what are they associated with?;"● Eosinophilic inclusion bodies ● Seen in molluscum contagiosum" What is the treatment of CIN?;"● Complete excision with cryotherapy to surgical margins and adjunctive treatment with antimetabolite (i.e. mitomycin C)" 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 " What is the most common organism causing infectious crystalline keratopathy?;"● Strep viridans" What pathology is associated with elongated ciliary processes?;"● Persistent Fetal Vasculature" “Disinsertion of the longitudinal muscle of the ciliary body from the scleral spur” describes what anterior chamber pathology?;"● Cyclodialysis" A tear between the longitudinal and circular fibers of the ciliary muscle describes what anterior chamber pathology?;"● Angle recession" What is the most common benign orbital tumor in adults?;"● Cavernous hemangioma" What is the most common malignant orbital tumor in adults?;"● Lymphoma" What is the most common primary intraocular tumor in adults?;"● Melanoma" What is the most common intraocular tumor overall?;"● Metastases" What is the most common benign orbital tumor in children?;"● Capillary hemangioma ● Other sources say benign cystic lesions (epidermoid or simple epithelial cysts)" What is the most common malignant orbital tumor in kids?;"● Rhabdomyosarcoma" 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" What are causes of congenital Horner’s syndrome?;"● Birth trauma-related brachial plexus injuries ● Neuroblastoma" 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" 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" 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" 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" What are the ocular adverse effects of reactive oxygen species?;"● Cataract ● ARMD ● Diabetic Retinopathy ● Glaucoma" 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 " What is the histologic appearance of an iris nevus?;"● Proliferation of branching dendrites, melanocytic cells, typically oblong or ovoid, bland appearance in iris stroma" What is the histologic appearance of a Spindle-B cell type melanoma?;"● Plump spindle-shaped melanocytes with granular appearing nuclei and prominent nucleoli " What is the histologic appearance of an epithelioid melanoma?;"● Polyhedral cells with large, round nuclei with clumped chromatin and prominent eosinophilic nucleoli " What is the classic histologic appearance of Juvenile xanthogranuloma?;"● Large histiocytes with foamy cytoplasm, Touton giant cells, lymphocytes, and occasional eosinophils " What is the classic histologic appearance of Ghost cell glaucoma?;"● Ghost cells (small, khaki-colored RBCs) with Heinz bodies " What is the classic histologic appearance of phacolytic glaucoma?;"● Lipid-laden macrophages " 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 " 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) " 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" What is the classic histologic appearance of tuberculosis?;"● Caseating granulomas " What are paradoxical pupils and what are the causes?;"● Constriction in response to darkness ● Causes: ○ Retinal dystrophies ○ Optic neuropathies" What is the appearance of mixed-cell type melanomas?;"● Spindle-shaped and polyhedral melanocytic cells with prominent atypia and pleomorphism" 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" What is the appearance of Dalen-Fuchs nodules on histology?;"● Epithelioid histiocytes and lymphocytes between Bruchs membrane and the RPE (predominantly histiocytes) ● Pigment phagocytosis " 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 " What are the mesenchymal tumors?;"● Fibrous histiocytoma ● Osteoma ● Solitary fibrous tumor ● Fibrous dysplasia ● Rhabdomyosarcoma" 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 " 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" 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" What is the name of the antigenic site on an antibody? On a foreign molecule?;"● Antibody: idiotope ● Foreign molecule: epitope" 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" 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)" What determines the color of the iris?;"● The size and number of melanin pigment granules in the anterior stromal melanocytes" What is the histologic appearance of sarcoidosis?;"● Noncaseating granulomas and conjunctival nodules " 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" 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 " What is the appearance of a fibrous histiocytoma on histology?;"● Proliferation of spindle fibroblasts and plump histiocytic cells in a focal storiform (matlike) architecture " 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 " 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)" What is the appearance of a compound nevus on histology?;"● Epithelial cysts in substantia propria surrounded by pigmented cells " What are the regulators for anterior chamber associated immune deviation (ACAID)?;"● Transforming growth factor beta (TGF-beta) and vasoactive intestinal peptide (VIP)" What is the immune function of interferon gamma?;"● Causes infiltration of neutrophils and macrophages" What role does Substance P play in immunity?;"● Plays a role in vascular permeability ● Causes leukocyte infiltration" What role does TNF-alpha play in immunity?;● Involved in mononuclear cell infiltration What syndrome are RPE hamartomas associated with?;"● Familial Adenomatous Polyposis ● Autosomal dominant ● Develop colon cancer" What is Gardner syndrome and what is it characterized by?;"● Familial Adenomatous Polyposis ● Cutaneous cysts ● Stemmas ● Odontomas ● Extracolonic cancers ● Atypical CHRPE" 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 " 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" What is an apocrine hidrocystoma?;"● Cyst arising from the glands of Moll (AKA cyst of Moll) ● Appears blue or translucent and transilluminates" What is an epidermoid cyst?;"● White, rubbery or firm ● Lined by stratified squamous keratinized epithelium and containing keratin" How should you biopsy a sebaceous adenocarcinoma?;"● Wide surgical excision is mandatory ● A conjunctival map biopsy is helpful to eliminate potential of pagetoid spread" What is conjunctival intraepithelial neoplasia (CIN)?;"● Full thickness dysplasia of the epithelial layer ● Associated with HPV 16 & 18 and sessile papillomas " What is the differential for pseudopapilledema?;"● Optic nerve drusen ● Hyperopic disc ● Prominent glial tissue ● Optic nerve myelination" What is the histologic definition of the macula?;"● Where the ganglion cell layer is thicker than a single cell" What is the histologic definition of the foveola?;"● Contains only cone photoreceptors, ganglion cells, and other nucleated cells (Muller cells) ● Blood vessels are absent" 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" What pathology has zonal granulomatous inflammation? Nodular granulomatous inflammation? Diffuse granulomatous inflammation?;"● Zonal = phacoantigenic uveitis ● Nodular = sarcoidosis ● Diffuse = sympathetic ophthalmia" 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" 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" What is the role of fibroblast growth factor in the eye?;"● Accelerates corneal wound repair after surgery, chemical burns, or ulcers" 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" 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" 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" 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"