Cornea & External Disease

Front 1

ANATOMY: Development
-From which cells does the optic cup develop and during which week of gest age?
-What structures does the surface ectoderm give rise to?
-What do the neural crest cells of the surface ectoderm form?
-When does Bowman's layer form?
-What % of adult size is the infant globe at birth?

Back 1

Development
-4 wks GA: optic vesicle forms a double-layered optic cup of neuroectoderm
-Surface ectoderm forms lens placode, corneal and conjunctival epithelium, eyelid epidermis
-Neural crest cells of surface ectoderm form corneal endothelium, stroma and sclera
-Bowman's layer forms at 4 months GA (all other components formed at 3 months)
-At birth, infant globe is 80% of adult size

Front 2

ANATOMY: Eyelids
-How often are cilia replaced when cut or pulled out?
-What type of glands are Moll and Zeiss?

Back 2

Eyelids
-Cilia regrow in 2 weeks if cut, 2 months if pulled out
-Moll: apocrine sweat glands
-Zeiss: sebaceous glands

Front 3

ANATOMY: Conjunctiva
-What do the tarsal and bulbar conj adhere to (respectively) and are they tightly or loosely adherent?
-What are the palisades of Vogt?
-What type of conj epithelium is found in the palpebral, bulbar and forniceal areas?
-Where are goblet cells most numerous in the conjunctiva?
-Which part of the conj has the most numerous lymphoid tissue (CALT)?
-What is the blood supply and innervation of the conj?

Back 3

Conjunctiva
-Tarsal conj adheres tightly to tarsus; bulbar conj adheres loosely to Tenon's
-Palisades of Vogt: radiating ridges at the corneal limbus, location of stem cells
-Palpebral conj: stratified cuboidal
-Bulbar conj: squamous
-Fornices: columnar
-Goblet cells most numerous in the tarsal conj, inferonasal bulbar conj
-CALT most numerous in fornices
-Palpebral conj supplied by eyelid circulation; bulbar conj supplied by anterior ciliary arteries
-Sensory innervation:CN V

Front 4

ANATOMY: Cornea
-What is the horizontal and vertical diameter of a normal cornea?
-What is the refractive index, average radius of curvature and amount of dioptric power contributed by the cornea?
-How does the cornea get it's nutrition?
-What nerves supply corneal innervation?

Back 4

Cornea
-11-12 mm horizontally; 10-11 mm vertically
-Refractive index: 1.3375
-Radius of curvature: 7.8 mm
-Total dioptric power: 43.25 D
-Nutrition: glucose diffuses from aqueous humor; O2 diffuses through tear film
-Sensory innervation: long ciliary nerves

Front 5

ANATOMY: Cornea
-What type of epithelium does the cornea have, how much does it contribute to total thickness of cornea and how thick is the epithelial basement membrane?

Back 5

Cornea
-Epithelium: stratified squamous
-Makes up 5% (0.05 mm) of total K thickness
-Basement membrane 50 nm thick

Front 6

ANATOMY: Cornea
-Where are keratocytes found in the cornea and what type of arrangement are they in?
-What type of collagen is found in the stroma?
-What type of proteoglycans are in the stroma?
-What is the difference between the stromal lamellae of the anterior and posterior cornea?
-What is the water content of the cornea and how is it maintained?

Back 6

Cornea
-Keratocytes form a spiraling 3D network throughout the cornea, found as flattened fibroblasts between collagen lamellae
-Collagen: Type I, type V intertwined with filaments of type VI
-Proteoglycans: decorin (dermatan sulfate), lumican (keratan sulfate)
-Ant corneal lamellae: short, narrow sheets with extensive interweaving
-Post corneal lamellae: long, wide, thick sheets, minimal interconnections
-Water content maintained at 78% by intact epith/endothel barrriers and endothel Na,K-ATPase pump

Front 7

ANATOMY: Cornea
-What changes does endothelium undergo during life?
-Where is the endothel cell concentration highest?
-How thick is Descemet's membrane?

Back 7

Cornea
-Endothel cells decline throughout life --> results in enlargement, spread of neighboring cells to cover defective areas
-Highest concentration in periphery
-Descemet's: BM of endothelial cells; 10-12 um thick in adults

Front 8

ANATOMY: Sclera
-What type of collagen is found in sclera?
-What nerves innervate sclera?
-What is an Axenfeld loop?
-What are senile scleral plaques?

Back 8

Sclera
-Type I collagen and proteoglycans make up sclera
-Axenfeld loop: nodule overlying ciliary body, represents long posterior ciliary nerve (innervates sclera)
-Senile scleral plaques: CaPhos deposits located anterior to insertions of MR/LR muscles --> become dehydrated and reveal blue color of underlying uvea

Front 9

EXAM: Visual acuity
-How can VA reduced by corneal irregularity be overcome to determine visual potential of an eye?

Back 9

Visual acuity in setting of corneal irregularity
-RGP over-refraction: use RGP CL with base curve halfway b/w the 2 powers obtained from K readings, and power near pt's spherical equivalent --> perform refraction

Front 10

EXAM: Specular reflection
-What can be seen with specular microscopy?

Back 10

Specular reflection/microscopy
-Specular reflection = normal light reflexes bouncing off a surface
-Specular microscopy: used to evaluate posterior cornea (produces faint specular reflection)
-Used to evaluate endothelial cell density,morphology, presence of guttae and KPs (look like nonreflective dark areas)

Front 11

EXAM: Stains
-How is TBUT measured?
-What is positive and negative staining?
-How does fluorescein affect AC evaluation?
-What is the dye disappearance test?

Back 11

Stains
-TBUT: instill fluorescein --> ask pt to hold lids open after 1-2 blinks --> count seconds until dry spot appears (<10 seconds = abnl)
-Positive staining: seen with fluorescein collecting in epithelial defects
-Negative staining: fluorescein highlights NONstaining lesions projecting through tear film (elevated lesions)
-When fluorescein collects in epi defect --> can diffuse into stroma and AC --> green flare visible in AC
-Dye disappearance test: instill fluorescein --> observe tear meniscus for disappearance of fluorescein (if prolonged, suggests blockage of tear drainage)

Front 12

EXAM: Stains
-When do rose bengal and lissamine green stain the ocular surface?
-What are side effects of rose bengal?

Back 12

Stains
-Rose bengal & lissamine green: stain cornea/conj when disruption occurs in protective mucin coating
-Rose bengal has more toxic effects on corneal epithelium, less well tolerated compared to lissamine green

Front 13

EXAM: Stains
-Describe staining patterns seen in: viral conjunctivitis, toxicity, lagophthalmos, trichiasis, drye eye syndrome,exposure keratopathy, SLK, CL wear

Back 13

Staining patterns
-Diffuse (all over K, conj): viral conjunctivitis, toxicity
-Inferior K, conj: lagophthalmos, trichiasis
-Interpalpebral: DES, exposure, neurotrophic keratopathy
-Superior K, bulbar conj: SLK, FB under lid
-3/9 o clock staining: CL wear

Front 14

EXAM: Inflammation
-Where are papillae most often seen, what is their clinical appearance?
-Distinguish between mild and severe papillae
-Name 2 causes of papillary conjunctivitis

Back 14

Inflammation: Papillae
-Most often seen on superior palpebral conj
-Clinical: raised lesions with dilated central vessel with spokelike capillaries surrounded by edema, mixed inflamm cell infiltrate
-Mild papillae: smooth, velvety appearance
-Severe papillae: elevated mounds separated by fibrous septae (achoring fibers of palpebral conj), may collect mucus and pus in septae
-2 causes of papillary conjunctivitis: allergic, bacterial

Front 15

EXAM: Inflammation
-Which population of patients do not display follicular reaction?
-What are follicles composed of?
-What is benign lymphoid folliculosis?
-What is the clinical appearance of a follicle?

Back 15

Inflammation: Follicles
-Neonates do not have visible follicles
-Follicles are composed of round/oval clusters of lymphocytes; have vessels surrounding but not prominently visible within the follicle (unlike papillae)
-Benign lymphoid folliculosis: clusters of enlarged, noninflamed follicles in inferotemporal palpebral/forniceal conj of children/adolescents

Front 16

EXAM: Corneal inflammation
-Where do stromal blood vessels originate in neovascular disease?
-How do inflammatory cells get into the cornea?
-What are 3 factors used to describe stromal inflammation?

Back 16

Corneal inflammation
-Stromal blood vessels originate from limbal vascular arcades --> migrate into peripheral cornea
-Inflammatory cells: enter stroma from tear film, limbus, through epi defect, through endothel defect from aqueous humor or from stromal blood vessels in neovascular disease
-Description of stromal inflammation: 1) suppurative vs nonsuppurative; 2) focal vs multifocal; 3) central, paracentral, peripheral

Front 17

EXAM: Corneal inflammation
-Describe 3 different types of KPs and their etiologies
-How does the cornea become opacified?
-What is a pannus?

Back 17

Corneal inflammation
-Small dots/strands of KP: fibrin and other proteins
-Punctate KP: neutrophils, lymphocytes
-Mutton-fat (large) KP: macrophages
-Corneal opacification: altered keratocytes produce abnormal collagen fibers that are disorganized, scatter light, form nontransparent scar
-Pannus: subepithelial fibrous ingrowth into peripheral cornea (can be vascularized)

Front 18

EXAM: Inflammation
-Name 3 causes of PEE
-Name 3 causes of PEK
-Name 3 causes of nonsuppurative stromal keratitis

Back 18

Corneal inflammation
-PEE: DES, toxicity, atopic keratoconjunctivitis
-PEK: Thygeson's SPK, HSV, Adenovirus keratoconjunctivitis
-Nonsupp stromal keratitis: HSV, VZV, Syphilis

Front 19

EXAM: Pachometry
-What is the average CCT?
-In which regions is the cornea thicker and where is it thinnest?

Back 19

Pachometry
-Average CCT: 540 um
-Thickest areas of cornea: paracentral and peripheral zones
-Thinnest area: 1.5 mm temporal to geographic center

Front 20

EXAM: Pachometry
-When are Descemet's folds seen?
-When is epithelial edema seen?
-What CCT indicates possible corneal decompensation after intraocular surgery?

Back 20

Pachometry
-Descemet's folds: seen when K thickness inc by 10% or more
-Epi edema: seen when thicknesss > 700 um
-Thickness > 650 = high risk of K edema after surgery

Front 21

EXAM: Pachometry
-How does the endothelium maintain corneal clarity (2 ways)?
-What # of endothel cells generally results in por endothel function?
-What is the most common cause of ACUTE K edema? CHRONIC K edema?

Back 21

Pachometry
-Endothel prevents edema and maintains clarity via 1) physical barrier b/w aqueous and corneal stroma and 2) metabolic pump of fluid out of stroma
-Less than 500 endothel cells --> dysfunction (not absolute #)
-ACUTE K edema: altered barrier effect of endothelium
-CHRONIC K edema: altered pump function

Front 22

EXAM: Pachometry
-What are Waite-Beetham lines?
-What are early signs of K edema?
-What effect does corneal edema have on IOP readings?

Back 22

Pachometry
-Waite-Beetham lines: faint, deep stromal wrinkles
-Early signs of K edema: diffuse epith haze, mild stromal thickening, Waite-Beetham lines, Descemet's folds, patchy/diffuse collagenous layer posteriorly
-K edema causes artificially low IOP readings (cornea has decreased rigidity and resistance to deformation)

Front 23

EXAM: Esthesiometry
-What is esthesiometry and when is it primarily used?
-How is corneal sensation recorded using this device?

Back 23

Esthesiometry
-Measurement of K sensation
-Primarily used in eval of neurotrophic keratopathy
-Record sensation as normal, reduced, absent for each quadrant of cornea

Front 24

EXAM: Specular microscopy
-What are the 3 parameters that can be calculated from specular/confocal microscopy?
-What are normal values for each parameter and what are the abnormalities?

Back 24

Specular microscopy
-Endothel cell density: normally >3500 in children, 2400 in adults (can decline to 2000 in elderly) --> If < 1000, may not tolerate intraocular surgery
-Coefficient of variation: standard deviation of mean cell area divided by mean cell area; normally <0.3 --> polymegathism (inc variation in cell area) seen with CL wear --> if >0.4, may not tolerate surgery
-% Hexagonal cells (6 apices): should be 100% --> lower % indicades decreasing health of cells --> pleomorphism (inc variability in cell shape) --> if >50% nonhexagonal (high pleomorphism) may not tolerate surgery

Front 25

EXAM: Confocal microscopy
-What is this modality used to study?
-What are 4 uses of confocal microscopy?

Back 25

Confocal microscopy
-Used to study cellular layers of cornea even in presence of edema/scarring
-4 uses: infectious crystalline keratopathy, fungal keratitis, amebic keratitis, following refractive surgery pts

Front 26

EXAM: Topography
-What is the difference in steepness between the central and peripheral cornea?
-Describe the size of the central, paracentral and apical zones of the cornea? What are these areas responsible for?

Back 26

Topography
-Central cornea is 3D steeper than peripheral cornea
-Central zone: 1-2 mm (spherical shape)
-Paracentral zone: 3-4 mm donut with 7-8 mm outer diameter
-Apical zone: central + paracentral zones --> used for CL fitting, responsible for refractive power of eye

Front 27

EXAM: Topography
-Where are the peripheral zone, limbus and transitional zones?
-Where is the optical zone? what is it's size?
-What is the difference between the corneal apex and corneal vertex? How can the vertex be seen clinically?

Back 27

Topography
-Peripheral (AKA transitional) zone: outer diameter of 11 mm (adj to paracentral zone) --> area of greatest flattening, asphericity of normal cornea
-Limbus: outer diameter of 12 mm
-Optical zone: portion of cornea overlying entrance to pupil (5.4 mm size)
-Corneal apex: point of maximum curvature (temporal to pupil center)
-Corneal vertex: located at intersection of pt's line of sight and corneal surface (seen as pupillary light reflex on exam)
-Note: vertex does not necessarily correspond to apex!

Front 28

EXAM: Topography
-What is the corneal refractive index used for calculating power?
-What is the avg refractive power of the central cornea?
-What interface is responsible for most of the eye's refraction?

Back 28

Topography
-Cornea refractive index: 1.3375
-Avg refractive power of central cornea: +43D
-Air-tear interface is responsible for most of the eye's refractive power

Front 29

EXAM: Topography
-How does the keratometer measure corneal power?
-Is this estimation clinically useful? What is it used for?

Back 29

Topography
-Keratometer: reads 4 points of central 2.8 - 4 mm zone of cornea to estimate power --> clinically useful
-Uses: CL fitting, IOL power calculation, detection of irregular astigmatism

Front 30

EXAM: Topography
-What does keratoscopy measure?
-How is keratoscopy different from keratometry?
-What is the appearance of the mires in steeper and flatter areas of the cornea, respectively?

Back 30

Topography
-Keratoscopy gives info about corneal curvature --> instruments reflect images of multiple concentric circles from corneal surface
-Diff b/w keratometry and keratoscopy: keratoscopy can be used to analyze K curvature in both center and periphery
-Steeper areas: mires are closer together, thinner
-Flatter areas: mires further apart, thicker

Front 31

EXAM: Topography
-What are benefits/ disadvantages of axial curvature in placido disk topography? instantaneous radius of curvature (i.e., tangential power)? mean curvature map?

Back 31

Topography
-Axial curvature: closely approximates power of central 1-2 mm but does not describe true shape/curve of peripheral K
-Instantaneous radius of curvature: more sensitive to peripheral changes
-Mean curvature map: uses infinite # of spheres to fit curvature at that point -->more sensitive to peripheral changes of curvature

Front 32

EXAM: Topography
-What are some normal patterns of topography?
-What are abnormal patterns?
-What are some uses for topography?

Back 32

Topography
-Normal patterns: round, oval or bow-tie
-Vertical bow-tie: with the rule astigmatism
-Horiz bow-tie: against the rule astigmatism
-Crab-claw: pellucid marginal degeneration, subclinical (forme fruste) keratoconus
-Assymetric bow-tie: keratoconus
-Topography can detect irregular astigmatism from CL wear, keratoconus/other ectasias, K surgery
-Also used for pre-op cataract/refractive surg pts

Front 33

EXAM: Topography
-How does retinoscopy help detect irregular astigmatism?

Back 33

Topography
-Retinoscopy shows nonlinear or multiple reflexes that cannot be completely neutralized with a spherocylindrical lens

Front 34

DIAGNOSIS: DES
-What are the primary systems involved in DES?
-What is the normal function of these systems (together as a whole)?

Back 34

DES
-Lacrimal functional unit (LFU): lacrimal glands, ocular surface (K, conj, Meibomian glands), eyelids, nerves
-Function of LFU: tear film integrity, maintaining K transparency and stem cell population, best quality image projected onto retina

Front 35

DIAGNOSIS: DES
-What are 3 ways in which the tear film can be compromised?
-What are the two main mechanisms of dry eye?

Back 35

DES
-Tear film stability is threatened with: decreased secretion, delayed clearance, altered composition of tears
-Mechanisms of DES: increased TF osmolarity, TF instability

Front 36

DIAGNOSIS: DES
-Describe the cycle in which TF osmolarity worsens DES
-What are major causes of increased TF osmolarity? Examples?

Back 36

DES
-TF hyperosmolarity --> surface damage --> inflammation --> loss of goblet cells and reduced mucin --> TF instability --> worsens hyperosmolarity and completes the cycle
-Causes of hyperosmolarity: reduced aqueous tear flow (lacrimal gland damage/dysfunction, cicatrical scarring, decreased sensory drive to lacrimal gland from ocular surface), increased evaporation of TF (2/2 MGD)

Front 37

DIAGNOSIS: DES
-Describe the algorithm for determining the etiology of DES
-Describe Non-Sjogren drye eye causes
-Describe intrinsic and extrinsic causes of evaporative tear loss

Back 37

DES
1) Aqueous deficient: Sjogren syndrome DES vs NON-Sjogren syndrome DES
-Non-Sjogren DES: lacrimal deficiency, lacrimal gland duct obstruction, reflex block, systemic drugs
2) Evaporative: Intrinsic vs extrinsic
-Intrinsic: MGD, lid aperture disorders, low blink rate (Parkinson's), Drugs (Accutane)
-Extrinsic: Vit A deficiency, preservatives in topical meds, CL wear, allergy or other ocular surface disease

Front 38

DIAGNOSIS: DES
-What % of adults age 30-60 and adults > 65 yo (respectively) have DES?
-Is there a higher prevalence of DES among men or women?

Back 38

DES
-10% of adults 30-60 yo
-15% of adults >65 yo
-F>M

Front 39

DIAGNOSIS: Tear Film
-What is the normal tear meniscus height, shape? What is abnormal?
-What is the purpose of the Schirmer test? How is it performed?

Back 39

Tear Film
-Tear meniscus normally 1 mm height, convex shape
-If < 0.3 mm --> abnormal
-Schirmer test: used to quantify aqueous tear production
-Instill topical anesthetic --> blot out residual fluid --> place filter paper strip at the jxn of mid-lateral 1/3 of lower lids --> wait 5 minutes and measure amt of wetting

Front 40

DIAGNOSIS: Tear Film
-What is an abnromal Schirmer test?
-What does a Schirmer I test measure?
-What does a Schirmer II test measure?
-What are normal and abnormal values for each of these tests?

Back 40

Tear Film
-Abnormal Schirmer test: < 5 mm wetting (normal is >/= 10 mm) --> suggests aqueous tear deficiency
-Schirmer I: no topical anesthetic used --> measures both basal and reflex tear secretion
-Schirmer II: No topical anesthetic used and nasal mucosa irritated with cotton applicator --> measures primarily reflex secretion
-Abnormal Schirmer I: < 10 mm wetting after 5 minutes
-Abnormal Schirmer II: < 15 mm wetting after 5 minutes

Front 41

DIAGNOSIS: Tear Film
-What is decreased tear lysozyme or lactoferrin suggestive of?

Back 41

Tear Film
-Decreased tear lysozyme/lactoferrin suggests dry eye --> seen in lacrimal gland dysfunction states (decreased proteins produced by lacrimal gland)

Front 42

DIAGNOSIS: ATD
-What are 3 findings on exam that support a diagnosis of aqueous tear deficiency?
-What are typical symptoms?

Back 42

ATD
-Clinical findings: abnormal Schirmer test, exposure staining pattern, filamentary keratopathy
-Sx: worse towards end of day or with prolonged use of eyes/environmental extremes, FB sensation, PEK, burning, photophobia, blurry VA

Front 43

DIAGNOSIS: ATD
-What are typical findings on exam reflective of ATD?
-What is the typical appearance of filamentary keratopathy?

Back 43

ATD
-Exam: bulbar conj injection, conjunctivochalasis, decreased tear meniscus, irreg K surface, debris in tear film
-Filamentary keratopathy: stands of epith cells attached to K surface over a core of mucus --> painful if removed as these are attached firmly to cornea

Front 44

DIAGNOSIS: ATD
-What do rose bengal and lissamine green stain?
-What are some advantages of lissamine green versus rose bengal?

Back 44

ATD
-Rose bengal/lissamine green: stain dead/devitalized cells and mucus as well as epith cells inadequately protected by mucin
-in KCS, staining seen at nasal and temporal limbus and/or inf paracentral cornea (exposure pattern)
-Lissamine green: does not stain healthy conj epithelium, less irritating, does not inhibit viral growth

Front 45

DIAGNOSIS: ATD
-What types of medical management can be considered for ATD (7)?

Back 45

ATD: Medical managment
-PF lubricants, warm compresses, humidifier/moisture shield
-Smoking cessation (risk factor!)
-Punctal occlusion, lat tarsorrhaphy
-Demulcent solutions (mucomimetic agents)
-Restasis (modulates inflammatory response --> reduced destruction of lacrimal gland)
-Short course topical steroids

Front 46

DIAGNOSIS: ATD
-How is filamentary keratopathy treated?
-What topical glaucoma meds can worsen DES?

Back 46

ATD
-Filamentary keratopathy tx: control underlying condition (i.e., GVH disease), 10% acetylcystine (mucolytic agent), topical anti-inflammatory meds, goggles/shields
-Topical glaucoma meds that worsen DES: miotics, topical beta blockers (dec goblet cell density), PO CAI's (dec tear production)

Front 47

DIAGNOSIS: ATD
-What are some methods of surgical mgmt of ATD?

Back 47

ATD: Surgical mgmt
-Reversible/irreversible punctal occlusion (plugs, cautery)
-Correct eyelid malpositions
-Tarsorraphy

Front 48

DIAGNOSIS: Sjogren Syndrome
-What are features assoc with SS?
-What is the difference between primary and secondary SS?
-What ist he pathophysiology of SS?

Back 48

Sjogren Syndrome
-Features; dry eyes, dry mouth, hypergammaglobulinema, RA, ANA+
-Primary: dry eyes/mouth but no evid of immune dysfunction or conn tissue disease
-Secondary: most commonly assoc with RA
-Pathophys: T-cell mediated inflammation --> lacrimal gland destruction

Front 49

DIAGNOSIS: Sjogren Syndrome
-What is the histologic appearance of the lacrimal gland in SS?
-What role do peripheral sensory nerves, androgenic hormones and viral infections play in SS?

Back 49

Sjogren Syndrome
-Histology: focal/diffuse lymphocytic infiltration assoc with areas of glandular destruction
-Abnormal sensory nerves (from K/conj to lacrimal gland) may be present in 20% of SS pts --> dec lacrimal gland secretion by reflex stimulation
-Androgenic hormone deficiency and viral infection can progress SS

Front 50

DIAGNOSIS: Non-SS DES
-What are primary and secondary causes of lacrimal gland dysfunction (besides SS)?
-What are causes of obstructed lacrimal gland outflow?
-What are causes of decreased nervous stimulation to lacrimal gland?

Back 50

Non-SS DES
-Primary lacrimal gland dysfunction: Riley-Day (dysautonomia), congenital alacrima, Adie syndrome, Shy-Drager (idiopathic autonomic dysfunction)
-Secondary lacrimal gland disease: sarcoid, chronic GVHD, HIV, xerophthalmia
-Lacrimal outflow obstruction: cicatrical conjunctivitis (pemphigoid, SJS, burns, trachoma)
-Decreased nervous stimulation: VZV, HSV, CL wear, peripheral neuropathies (i.e., Bell's palsy, DM), PK, aging, anticholinergic meds

Front 51

DIAGNOSIS: ETD
-What are clinical signs of evaporative tear dysfunction?
-What are typical symptoms?

Back 51

ETD
-Signs: dec TBUT, MGD, abnl aqueous tear production, linear pattern of staining of inf conj, cornea, lid margin
-Sx: filmy vision, burning, FB sensation, recurrent chalazia

Front 52

DIAGNOSIS: ETD
-What is the basis for meibomian gland obstruction and it's consequences for the ocular surface/tear film?

Back 52

ETD: MGD
-Progressive obstruction of meibomian gland orifices 2/2 keratinization --> decreased lipid delivery to ocular surface and tear film --> inflammation, tear film instability (inc evaporation, inc osmolarity)

Front 53

DIAGNOSIS: ETD
-Describe hyposecretory and hypersecretory causes of MGD
-What are some clinical signs of MGD? What do the meibomian glands typically look like?

Back 53

ETD: MGD
-Hyposecretory (obstructive) MGD: blepharitis, acne rosacea, pemphigoid
-Hypersecretory (nonobstructive): meibomian seborrhea
-Clinical: lid margin telangiectasias, pouting of meibomian gland orifices +/- white keratin plug extruding through, meibomian gland dropout (chronic phase), foamy tears, rapid TBUT

Front 54

DIAGNOSIS: ETD
-What are signs of ocular surface inflammation assoc with MGD?
-What are some management strategies for MGD?

Back 54

ETD: MGD
-Ocular inflammation: injection, tarsal papillae, episcleritis, PEE, marginal epith/subepi infiltrates, K pannus, K thinning
-Mgmt: lid hygeine (warm compresses, lid massage and cleansing), short term topical abx, systemic tetracyclines (doxy 100 bid x 4 weeks or e-mycin), short course topical steroids (for mod-severe inflamm), PO omega-3 fatty acids

Front 55

DIAGNOSIS: ETD
-What are the clinical manifestations of rosacea?
-What is the typical presentation?
-What is rhinophyma?

Back 55

ETD: Rosacea
-Cutaneous sebaceous gland dysfunction on face, neck, shoulders --> meibomian glands and other lipid-producing lid glands also affected
-Frequently assoc with blepharitis and aqueous tear deficiency
-Clinical: excessive sebum secretion, chronic blepharitis, lid margin telangiectasias, MGD, recurrent chalazia
-Rhinophyma: thickening of skin/conn tissue of nose

Front 56

DIAGNOSIS: ETD
-What is the epidemiology of rosacea?
-What is the mainstay of management?

Back 56

ETD: Rosacea
-Epi: 30-60 yo, F>M
-Mgmt: systemic tetracyclines (anti-inflammatory)

Front 57

DIAGNOSIS: ETD
-Describe the clinical appearance of seborrheic blepharitis
-What is the typical pattern of keratitis?
-What is the mainstay of mgmt? What if inflammation is a primary component?

Back 57

ETD: Seborrheic blepharitis
-Clinical: lid margin inflammation, turbid meibomian gland secretions, oily/greasy crusting on lids, lashes, brows, scalp
-Keratitis: PEE over inf 1/3 of cornea
-Mgmt: lid hygeine; if inflammation prominent --> topical steroids to lid margins (short course) or systemic tetracycline

Front 58

DIAGNOSIS: ETD
-What is a chalazion and which glands are involved?
-What is the clinical appearance?
-What type of inflammation is present?
-Mgmt?

Back 58

ETD: Chalazia
-Localized lipogranulomatous reaction of meibomian or Zeis glands (prominent epitheliod cells, centered around lipid)
-Slow growth, painless, erythema overlying; contents may drain internally or externally through skin, blurry vision 2/2 astigmatism from globe compression
-Mgmt: hot compresses, attempted expression of contents, intralesional steroid (0.1-0.2 cc triamcinolone 10 mg/mL), I&D or combination

Front 59

DIAGNOSIS: ETD
-What are the most common eyelid, conjunctival, lacrimal gland, corneal and fundus findings assoc with sarcoid?

Back 59

ETD: Sarcoid
-Eyelids: nontender small (millet seed) or large nodules
-Lacrimal gland: KCS (25% of pts)
-Conj granulomas
-Corneal band keratopathy/calcific changes, stromal neovasc, mutton fat KPs
-Fundus: periphlebitis

Front 60

DIAGNOSIS: ETD
-What is Mikulicz syndrome?
-What is Lofgren syndrome?
-What is Heerfordt syndrome?

Back 60

ETD: Sarcoid
-Mikulicz: enlarged lacrimal/salivary glands + DES
-Lofgren: erythema nodosum, hilar adenopathy, iridocyclitis
-Heerfordt: uveitis, fever, facial n palsy

Front 61

DIAGNOSIS: ETD
-What is ichthyosis? How does it present?
-What are typical corneal findings?
-What is the mgmt?

Back 61

ETD: Ichthyosis
-Hereditary skin disorder with excessively dry skin, scale accumulation
-Presentation: eyelid scaling, cicatrical ectropion, conj thickening
-Cornea: dot/filament opacities in deep stroma (vision unaffected)
-Mgmt: hydration of skin/lids (NOT responsive to steroids)

Front 62

DIAGNOSIS: ETD
-What are characteristics of ectodermal dysplasia?
-What are 3 features of anhidrotic ectodermal dysplasia?

Back 62

ETD: Ectodermal dysplasia
-Rare, heterogenous group of conditions noted at birth, diffuse epidermal and epidermal appendage abnormalities (hair, nails, teeth, sweat glands)
-Eyes: abnormal lacrimal system, eyelid/lash abnormalities, dryeye, pterygium, K scarring, limbal stem cell deficiency
-Anhidrotic ectodermal dysplasia: hypotrichosis, anodontia, anhidrosis (no sweating --> leads to hyperpyrexia)

Front 63

DIAGNOSIS: ETD
-What is xeroderma pigmentosum?
-What are findings noted on the skin in this disease?
-What are typical eye findings?
-What is the most common ocular malignancy in these pts?

Back 63

ETD: Xeroderma pigmentosum
-auto recessive, impaired ability to repair sunlight-induced DNA damage
-Skin: focal hyperpigmentation, atrophy, actinic keratoses, telangiectasias --> cutaenous neoplasms
-Eye: photophobia, tearing, blepharospasm, KCS, DES complications
-Most common eye cancer: squamous cell ca (cancer in 11% of pts, usually at limbus)

Front 64

DIAGNOSIS: Conj vascular anomalies
-What are systemic/topical vasodilators that cause conj hyperemia (3)?

Back 64

ETD: Conj hyperemia
-Alcohol, oxygen, carcinoid tumor --> vasodilators --> conj hyperemia

Front 65

DIAGNOSIS: Conj vascular anomalies
-What is Rendu-Osler-Weber syndrome?
-How is it inherited?
-What other areas are involved?

Back 65

Hereditary Hemorrhagic Telangiectasias
-Spontaneous hemorrhage from telangiectatic vessels of palpebral & bulbar conj
-Other organ systems: skin, nose, brain, GI tract, lungs, oral mucosa
-Dominantly inherited
-Onset in early adult life

Front 66

DIAGNOSIS: Lymphangiectasia
-What is the clinical appearance?
-What diagnosis should lymphangiectasias be distinguished from?

Back 66

Lymphangiectasia
-Irregularly dilated, periodically hemorrhage-filled lymphatic channels of bulbar conj w/ surrounding conj edema/heme
-Distinguish from Louis-Bar syndrome (ataxia-telangiectasia): epibulbar/interpalpebral telangiectasias of arteries WITHOUT lymphatic component
-Note: Louis-Bar also has cerebellar and immunologic abnormalities

Front 67

DIAGNOSIS: Vit A deficiency
-What is the ocular appearance of this disease?
-What is a Bitot spot?
-What is the clinical presentation?

Back 67

Vitamin A Deficiency
-Xerosis (dry conj/cornea) with dec mucous production by goblet cells
-Xerophthalmia (blinding condition)
-Bitot spot: superficial, foamy gray area on bulbar conj (interpalpebral region) --> consists of keratinized epithelium, inflammatory cells, bacteria
-Clinical: nyctalopia, xerophthalmic fundus (yellow-white spots in peripheral retina), keratomalacia (K necrosis)

Front 68

DIAGNOSIS: Vit A deficiency
-What are the 3 stages of ocular surface changes in this disease?
-What is the management?

Back 68

Vitamin A Deficiency
-3 surface changes: conj xerosis with or w/o Bitot spots (X1), corneal xerosis (X2), corneal ulceration/keratomalacia (X3)
-Mgmt: both vit A and protein-calorie supplementation (coexists), corneal lubrication, prevention of secondary infection, topical retinoids (not available in US)

Front 69

DIAGNOSIS: Vit C
-What is the concentration of ascorbate in the aqueous versus plasma?
-What ocular consequences does vit C deficiency have?

Back 69

Vitamin C deficiency
-Aqueous contains 15-20x more ascorbic acid than plasma
-Vit C deficiency results in unstable collagen fiber formation
-Xerosis, subconj/orbital hemorrhage, impaired wound healing

Front 70

DIAGNOSIS: Exposure keratopathy
-Name some causes of lagophthalmos
-What are some treatments of exposure keratopathy?

Back 70

Exposure keratopathy
-Causes of lagophthalmos: CN VII palsy, thyroid eye disease, ectropion, circatrizing conditions (SJS), degenerative neurologic disease (i.e., Parkinson's)
-Mgmt: PF AT, ointment at bedtime, temporary tarsorraphy, upper eyelid weights
-BCL use can be hazardous (high risk of infection)

Front 71

DIAGNOSIS: Floppy Eyelid Syndrome
-What patient population typically has this condition?
-What is the pathophysiology?
-What are clinical findings?
-Mgmt?

Back 71

Floppy Eyelid Syndrome
-Obese patients with OSA
-Flimsy, lax upper tarsus --> everts with minimal upward force --> contacts bedsheets while sleeping in face down position --> chronic eye irritation, inflammation, papillae, mucus d/c, keratopathy
-Tx: cover affected eye with shield/tape lids shut at night

Front 72

DIAGNOSIS: SLK
-What is the pathogenesis of SLK?
-What systemic disease is sometimes associated with this diagnosis?
-What is the clinical appearance?
-What patient population?

Back 72

Superior limbic keratoconjunctivitis
-Mechanical trauma transmitted from upper eyelid to superior bulbar/tarsal conj
-Assoc with autoimmune thyroid disease
-Chronic, recurrent; Females, 20-70 yo
-Clinical: superior tarsal papillae, inj/thickening of sup bulbar conj, punctate staining above and below limbus, filamentary keratopathy, superior limbal hypertrophy

Front 73

DIAGNOSIS: SLK
-What lab tests should pts with SLK have?
-What is the histologic appearance?
-Mgmt?

Back 73

Superior limbic keratoconjunctivitis
-Should have thyroid function tests
-Histo: nuclear pyknosis with "snake nuclei", loss of goblet cells, keratinization
-Mgmt: topical anti-inflamm, large diameter BCL, restasis

Front 74

DIAGNOSIS: Recurrent corneal erosions
-Why do recurrent erosions occur?
-The activity of which protein is upregulated in eptihelium with recurrent erosions?

Back 74

Recurrent corneal erosions
-Secondary to poor adhesion of epithelium to basement membrane
-Increased gelatinase activity (MMP2 and 9) noted in pts with recurrent erosions (alter the epi basement membrane during wound healing)

Front 75

DIAGNOSIS: Recurrent corneal erosions
-What is the clinical presentation?

Back 75

Recurrent corneal erosions
-Clinical: sudden onset eye pain upon waking, redness, tearing, photophobia
-K epithelium may be intact upon exam or may appear edematous, heaped-up (pooling of fluorescein over area)
-Minor episodes resolve rapidly
-Examine both eyes: if contralateral eye has epithelial changes also --> likely primary basement membrane defect (not likely post-traumatic)

Front 76

DIAGNOSIS: Recurrent corneal erosions
-What is acute and chronic phase treatment for this condition?

Back 76

Recurrent corneal erosions
-Acute tx: frequent lubrication with antibiotic ointments, cycloplegia
-Hypertonic saline (dec K edema) during day and ointment at bedtimefor 6-12 mo (promotes proper epith reattachment)
-Anti-inflammatories: systemic tetracyclines, topical FML
-Anterior stromal micropuncture: small lesions produced over defect to create subepithelial scars (adhesions between epithelium and stroma)
-Epithelial debridement: procedure of choice in severe secondary basement-membrane related recurrent erosions --> take care not to damage Bowman's layer

Front 77

DIAGNOSIS: Persistent epi defect
-What is the clinical appearance of a persistent epi defect?
-What is a frequent accompanying sign?
-What is a frequent accompanying disease?

Back 77

Persistent corneal epithelial defects
-Central/paracentral areas of nonhealing epithelium with gray edges, rolled under, significant underlying stromal inflammation
-K anesthesia is a frequent accompanying finding
-KCS, prior HSV/VZV infection are frequently accompanying diseases

Front 78

DIAGNOSIS: Persistent epi defect
-What drugs most commonly cause persistent epi defects?
-What are pharmacologic therapies for persistent epi defects?

Back 78

Persistent epi defects
-Causative drugs: topical anesthetics, topical NSAIDs, trifluridine, beta-blockers, CAIs, meds with BAK (benzalkonium chloride preservative)
-Tx: Systemic tetracyclines, frequent nonpreserved lubricants, tarsorraphy

Front 79

DIAGNOSIS: Neurotrophic keratopathy
-What nerve has to be damaged to create corneal anesthesia?
-What happens to tear film s/p corneal denervation?
-What is the appearance of a neurotrophic ulcer from HSV?

Back 79

Neurotrophic keratopathy
-Damage to CN V
-TF osmolarity increases after corneal denervation
-Involves central/paracentral cornea, epi defects persist without stromal inflammation or active virus replication
-Clinical: intense staining, raised/rolled up gray edges

Front 80

DIAGNOSIS: Neurotrophic keratopathy
-What are features of Riley-Day syndrome?
-How does autonomic dysfunction decrease tear production?

Back 80

Neurotrophic keratopathy
-Riley-Day: autonomic neuropathy in Ashkenazi Jews, alacrima, vasomotor instability, dec DTRs, absent lingual papillae, impaired taste, pain or temp sensation
-Also have inc sensitivity to cholinergic/adrenergic agonists
-Autonomic dysfunction decreases lacrimal gland tear production --> secondary xerosis

Front 81

DIAGNOSIS: Trichiasis
-What is trichiasis?
-What is distichiasis?
-What is the preferred surgical treatment for aberrant eyelashes?

Back 81

Trichiasis
-Trichiasis: lashes emerge from normal anterior origin but curve inwards towards cornea (may have subtle cicatrical entropion of lid margin)
-Distichiasis: congenital, usually AD or acquired --> extra row of lashes emerges from ducts of meibomian glands
-Preferred surg tx: tarsotomy with eyelid margin rotation

Front 82

DIAGNOSIS: Mucus fishing syndrome
-What is the staining pattern?
-What is the usual inciting event and pathophysiology?

Back 82

Mucus fishing syndrome
-Staining: well-circumscribed pattern of rose bengal/lissamine green stain on nasal and inf bulbar conj
-Inciting even: KCS
-Pts have increased mucus production 2/2 ocular surface damage --> vigorous eye rubbing and compulsive removal of mucus from fornix (mucus fishing) --> worsened irritation --> more mucus production

Front 83

DIAGNOSIS: Topical anesthetic abuse
-What effects do topical anesthetics have on epithelium?
-What is the primary clue to anesthetic abuse in clinical presentation?
-What is the evolution of corneal changes?

Back 83

Topical anesthetic abuse
-Local anesthetics inhibit epithelial migration/division
-Primary clinical clue: failure of presenting condition to respond to appropriate therapy
-Initially see punctate keratopathy --> becomes neurotrophic ulcer with KPs, hypopyon (mimics infection) --> diffuse edema, infiltrates, large ring opacity

Front 84

DIAGNOSIS: Dellen
-What is the pathopysiologyof dellen formation?
-What do dellen look like?
-Mgmt?

Back 84

Dellen
-In cornea adjacent to limbus and elevated areas (i.e., pterygium, bleb, dermoid) --> may have dessication of epithelium/subepi tissues secondary to interruption of tear film and improper wetting of the surface
-Clinical appearance: saucer-like depressions in corneal surface, epith punctate irregularities, thin/dehydrated stroma
-Rx: frequent ocular lubrication, pressure patching

Front 85

DIAGNOSIS: CL-related problems
-What is a Sattler veil? what does it lead to? how is it managed?
-What is microcystic epitheliopathy? how long does it take to resolve?

Back 85

CL-related problems
-Sattler veil: central epithelial edema; seen after many hours of wear with hard CL --> can lead to epithelial necrosis/ulceration
-Secondary to hypoxia --> lactate accumulation, impaired CO2 efflux
-Microcystic epitheliopathy: fine epithelial cysts best seen w/ retroillumination, extended-wear SCL wearers, may take 6 weeks to resolve after d/c CL

Front 86

DIAGNOSIS: CL related problems
-What is the etiology and appearance of toxic conjunctivitis?
-What preservative causes CL-related allergic reactions? how does this appear clinically?

Back 86

CL-related problems
-Toxic conjunctivitis: injection, PEK, erosions, microcysts --> usually 2/2 proteolytic enzymes/chemicals used in CL cleaners
-Allergic reactions: usually 2/2 thimerosal --> delayed hypersensitivity response --> can also cause CL-induced SLK
-Clinical: superior bulbar conj injection, papillaryconjunctivitis, superficial pannus

Front 87

DIAGNOSIS: CL-related problems
-What degree of pannus is acceptable in the cornea?
-What type of CL more commonly induce pannus?
-What is the pathophysiology?

Back 87

CL-related problems
-Micropannus is acceptable and common in SCL wearers if <2 mm.
-Caused by hypoxia, chronic trauma

Front 88

DIAGNOSIS: Limbal stem cell deficiency
-Where are the corneal and conjunctival stem cells located, respectively?
-What % of the limbus must be present to ensure normal ocular resurfacing?

Back 88

Limbal stem cell deficiency
-Corneal stem cells: located in basal layer of limbus
-Conj stem cells: uniformly distributed throughout bulbar or forniceal conj
-25-33% of limbus must be intact to ensure normal ocular resurfacing

Front 89

DIAGNOSIS: Limbal stem cell deficiency
-What are the normal functions of the limbus?
-What is the presentation of limbal stem cell deficiency?

Back 89

Limbal stem cell deficiency
-Normal function of limbus: acts as barrier against corneal neovasc from conj and invasion of bulbar conj cells
-Absence of limbal cells decreases effectiveness of wound healing
-Presentation: recurrent ulceration, dec vision with irregular K surface (wave-like appearance with fluorescein)

Front 90

DIAGNOSIS: Limbal stem cell deficiency
-What is the treatment of limbal stem cell deficiency - unilateral vs bilateral?

Back 90

Limbal stem cell deficiency
-Unilateral: limbal or conj autograft from fellow eye
-Bilateral: limbal autograft from HLA-matched living related donor OR eye bank donor eye

Front 91

VIROLOGY: Ocular defense mechanisms
-Describe the functions of the following tear components: lysozyme, beta-lysin, lactoferrin, mucin
-What 2 other immunologic molecules are found in tear film?
-Name 3 cytokines present in the tear film

Back 91

Ocular defense mechanisms
-Lysozyme: degrades bact cell walls
-Beta-lysin: disrupts bact plasma membranes
-Lactoferrin: inhibits bact metabolism
-Secretory IgA and complement components are also present in TF
-Cytokines: EGF, TGF-beta, hepatocyte growth factor

Front 92

VIROLOGY: Normal ocular flora
-Which 2 bact species predominate in the 1st two decades of life?
-What bacteria and parasites are more commonly seen with age?

Back 92

Normal ocular flora
-1st 2 decades: streptococci, pneumococci
-Increasing age: gram negatives (but S. epi still most common as well as S. aureus, diphtheroids)
-Also see parasites (demodex folliculorom/brevis) on lid margins with inc age

Front 93

VIROLOGY: Virulence mechanisms
-Name 2 viruses and 1 parasite that use adhesins to adhere to ocular surface
-What is a biofilm?
-Name 4 bacteria that can overcome intact epithelium
-Name 4 organisms that can establish latency

Back 93

Virulence mechanisms
-Adhesins: Candida, HSV (heparan sulfate), adenovirus (sialic acid)
-Biofilm: 3D structure allowing interbacterial communication/signaling, interferes w/ phagocytosis
-Bacteria that overcome intact epithelium: N. gonorrhea, N. meningitidis, C. diphtheria, Shigella spp.
-Latency: VZV, HSV (trigeminal ganglion), Chlamydia (within intracellular phagosomes of host cells), Streptococci(Biofilm --> crystalline keratopathy)

Front 94

VIROLOGY: Corneal culture
-Name 3 indications for corneal culture of ulcers

Back 94

Corneal cultures
-Indications: 1) ulcer >1-2 mm size, 2) suspect atypical organism or 3) no response to therapy

Front 95

VIROLOGY: Culture media/stains
-Name 2 stains and 1 type of culture media for mycobacteria
-Name 2 stains and 2 types of culture media for fungi
-Name 2 stains and 2 types of culture media for acanthamoeba

Back 95

Stains/Culture media
STAINS:
-Mycobacteria: acid-fast, lectin
-Fungi/acanthamoeba: calcofluor white, acridine orange
MEDIA
-Mycobacteria: Lowenstein-Jensen
-Fungi: Brain-heart infusion, Sabouraud's
-Acanthamoeba: Non-nutrient agar with E. coli overlay, Buffered charcoal yeast extract agar

Front 96

VIROLOGY: VIral pathogenesis
-What is the clinical significance of the type of nucleic acid in a virus?
-What is a viral capsid? function?
-What is the function of the lipid bilayer? What agents can damage the lipid bilayer?

Back 96

Viral pathogenesis
-DNA type: confers different susceptibilities to antiviral meds (target viral gene transcription)
-Capsid: protein shell surrounding DNA material, protects genome and helps virus-host cell binding
-Lipid bilayer/envelope: surrounds capsid in some viruses --> vulnerable to damage via UV light, detergents, alcohols, antiseptics; helps neutralize Abs via surface glycoproteins

Front 97

VIROLOGY: Viral pathogenesis
-Name 2 enveloped viruses
-Name 1 non-enveloped virus

Back 97

Viral pathogenesis:
-Enveloped: HSV, HIV
-Non-enveloped: adenovirus

Front 98

VIROLOGY: Herpesviruses
-Structure?
-Latency?
-Location of HSV1 vs HSV2 infections?
-What % of adults in industrialized socieites have serum Abs to HSV-1?

Back 98

Herpesviruses
-dsDNA, icosahedral capsid, enveloped
-HSV1/2/VZV: latency in dorsal root ganglia
-EBV: latency in B cells
-HSV1 usually above waist, HSV2 usually below (but either can infect either location)
-40-80% of adults hvae HSV1 Abs

Front 99

VIROLOGY: HSV
-How is it spread?
-How does primary HSV disease manifest?
-What % of adults have history of ocular HSV? How many of these develop stromal keratitis?

Back 99

HSV
-Spread via direct contact with infected lesions/secretions
-Primary HSV1: on skin/mucosa innervated by CN V or nonspecific URI
-0.15% of US population has h/o ocular HSV; 20% of these develop stromal keratitis

Front 100

VIROLOGY: HSV
-Describe primary ocular HSV findings
-Name 3 ways to distinguish acute HSV ocular infection from adenovirus

Back 100

HSV - Primary
-Primary ocular infection: unilateral blepharoconjunctivitis (follicular), palpable preauricular node, skin/lid vesicles
-HSV vs adeno: HSV has vesicles and ulcers on lid margin/bulbar conj as well as dendrititic epithelial changes (adeno does not); adeno produces conj membranes/pseudomembranes (HSV does not)

Front 101

VIROLOGY: HSV
-What type of serologic testing is helpful for primary ocular HSV?
-When is lab testing suggested?
-What type of testing should be performed?
-Mgmt of primary HSV?

Back 101

HSV - primary
-Serologic testing only helpful when negative (most adults have seropositivity)
-Indication for testing: neonatal infection, unclear clinical diagnosis
-Test via viral culture or PCR
-Mgmt: self-limited (PO antivirals speeds resolution)

Front 102

VIROLOGY: HSV
-HEDS findings re: triggers for HSV recurrence?
-Name 4 types of clinical presentations of ant segment HSV (recurrent)

Back 102

HSV - Recurrent
-HEDS: no factors (environmental, exposure) identified as proven triggers of HSV recurrence (i.e., stress, CL wear, systemic infection, menstruation, trauma)
-4 presentations of ant segment recurrent HSV: blepharoconjunctivitis, epi keratitis, stromal keratitis, iridocyclitis

Front 103

VIROLOGY: HSV
-Clinical presentation of blepharoconjunctivitis (recurrent)?
-Clinical presentation of epi keratitis? (sx, signs, staining pattern)

Back 103

HSV - recurrent
-Blepharoconjunctivitis: may be identical to primary infection, self limited but antivirals speed resolution
-Epi keratitis SX: FB sensation, photophobia, blurred VA, redness
-Epi keratitis signs: punctate epi keratitis, may coalesce into arborizing dendritic epi ulcers WITH terminal bulbs --> swollen K epithelium at edge of ulcer stains w/ rose bengal/lissamine green; bed of ulcer stains with fluorescein
-Other signs: ciliary flush, conj injection, mild stromal edema and subepi infiltrates; may see ghost dendrite after resolution; dec K sensation (worsens with increased recurrences)

Front 104

VIROLOGY: HSV
-DDx of dendritiform epithelial lesions?
-Management of epi keratitis?
-What medication is contraindicated in epi keratitis?
-What side effect can occur with valacyclovir?
-HEDS: Does PO antiviral dec incidence of stromal keratitis in pts with epi keratitis?

Back 104

HSV - recurrent
-Ddx of dendritiform lesions: VZV, EBV, adeno, neurotrophic keratopathy, SCL wear (thimerosol)
-Mgmt: spontaneous resolution but tx shortens course and ? shortens neuropathy; use topical (trifluridine 8x/day) vs PO (acyclovir, valacyclovir --> no ocular toxicity)
-Avoid corticosteroids!
-Valacyclovir can cause TTP/HUS in immunocompromised pts

Front 105

VIROLOGY: HSV
-Which form of keratitis is the most common cause of infectious corneal blindness in US?
-Pathogenesis of stromal keratitis?
-Clinical presentation? (diff types of keratitis, staining pattern)

Back 105

HSV - recurrent
-Stromal keratitis: most common infectious cause of blindness in US
-Pathogenesis: inflammatory response (as opposed to active infection)
-Clinical: nonnecrotizing (interstitial vs disciform) or necrotizing forms
-Signs: no epi ulceration but +stromal whitening and edema (may be hard to see active disease in area of previous scar), neovasc of cornea
-Disciform keratitis (primary endotheliitis): round/oval shape of stromal/epi edema, KPs, +/- AC reaction (milder) --> cannot distinguish disciform keratitis 2/2 VZV from HSV!
-Necrotizing keratitis: cannot distinguish from bact/fungal keratitis; common to see overlying epi defect and neovasc

Front 106

VIROLOGY: HSV
-HEDS: findings re: treatment of stromal keratitis?
-HEDS: does PO acyclovir provide additional benefit in pts on topical steroids + topical antiviral? What was the rationale for this study?
-Why are pts with stromal keratitis treated w/ antivirals?
-What is the preferred treatment for necrotizing stromal keratitis?

Back 106

HSV - recurrent
-HEDS: topical STEROIDS decrease inflammation/shorten duration of stromal keratitis; PO ACYCLOVIR ppx decreases risk of recurrent episodes
-HEDS: PO acyclovir does NOT provide additional benefit in treating stromal keratitis if pt already on topical antiviral + topical steroid (however, disciform keratitis not analyzed as separate group)
-Rationale for this study: topical antivirals have poor penetration through intact K epithelium --> PO antivirals can penetrate intact cornea and reach AC, so may be better for stromal keratitis (and deeper levels of inflammation)
-Treat pts w/ stromal keratitis w/ antivirals b/c pt may shed active virus while on steroids --> antivirals prevent epi keratitis in this setting
-Necrotizing stromal keratitis: prefer PO antivirals (due to toxicity of topical trifluridine) + topical steroids

Front 107

VIROLOGY: HSV
-Clinical presentation of HSV iridocyclitis?
-Presentation of topical antiviral toxicity?
-Presentation of neurotrophic keratopathy?
-Treatment for neurotrophic keratopathy?

Back 107

HSV - recurrent
-Iridocyclitis: granulomatous (or non-granulomatous) iridocyclitis, focal iris TI defects, elevated IOP (trabeculitis)
-Topical antiviral toxicity: diffuse PEE, injection of conj
-Neurotrophic keratopathy: diffuse PEE, vortex pattern f punctate staining, chronic epi regeneration lines, frank ulcer --> note NO staining with rose bengal (unlike epi keratitis)
-Mgmt of neurotrophic keratopathy: liberal lubricating ointments, gtts, tarsorrhaphy if fails tx

Front 108

VIROLOGY: HSV
-What is a metaherpetic ulcer?
-What are the etiology & consequences of persistent bullous keratopathy?

Back 108

HSV - recurrent
-Metaherpetic ulcer: interstitial stromal keratitis assoc with chronic epi defect that doesn't stain w/ rose bengal
-Persistent bullous keratopathy: longstanding disciform keratitis --> permanent K scarring, irreg astigmatism --> use RGP CL, topical steroids

Front 109

VIROLOGY: HSV
-What are indications, indicators of successful outcome in PK for corneal HSV?

Back 109

HSV - recurrent
-Indications for PK: visually significant stromal scarring, astigmatism uncorrected by spectacles/CL
-Mgmt: use PO antivirals (less toxic than topical) --> prolong graft survival, allow liberal use of steroids
-Indicators of successful outcome: no signs of active inflammation for at least 6 months prior to surg (80% success rate)

Front 110

VIROLOGY: VZV
-Presentation of primary VZV infection?
-Ocular involvement in primary infection?
-Risk of reactivation? risk of ophthalmic involvement w/ reactivation?

Back 110

VZV
-Primary infection: direct contact w/ skin lesions/respiratory secretions from infected pt --> maculopapular, pustular systemic rash, fever, malaise
-Ocular involvement UNCOMMON in primary infection: follicular conjunctivitis, vesicular lesions on lids/bulbar conj (may also have epi keratitis, stromal keratitis, uveitis --> less common)
-Risk of reactivation (anywhere): 20%
-Risk of V1 reactivation: 15% (of reactivated cases)

Front 111

VIROLOGY: HSV VS VZV
-Distinguish the 2 entities based on: pain level, dendrite morphology, freq of postherpetic neuralgia, laterality of disease, severity of corneal hypoesthesia, freq of recurrent epi keratitis

Back 111

HSV VS VZV
-More common in VZV: severe pain, postherpetic neuralgia, worse corneal hypoesthesia, dendrites with no terminal bulbs
-More common in HSV: central ulcers with terminal bulbs, bilateral involvement (although uncommon)

Front 112

VIROLOGY: VZV
-Where does VZV most commonly establish latency?
-What is Ramsay Hunt syndrome?
-Which part of trigeminal ganglion most commonly affected?

Back 112

VZV
-Dorsal root ganglion (T3 - L3): most common location
-Ramsay Hunt: re-activation of VZV in geniculate ganglion (Bell's palsy, vertigo, deafness, pain)
-Trigeminal ganglion: V1 most commonly affected

Front 113

VIROLOGY: HZO
-Epidemiology?
-What type of infiltrate is characteristic of VZV stromal keratitis?
-What are some orbital/optic n problems with HZO?

Back 113

HZO
-Pts in 6-9th decades, mostly healthy, increased risk in pts on immunosuppressive tx & HIV (6x higher risk)
-Stromal keratitis: nummular corneal infiltrates
-Dec K sensation, severe postherpetic neuralgia more common in VZV than HSV
-Orbital problems: occlusive arteritis (ptosis, orbital edema, proptosis), papillitis/retrobulbar optic neuritis, CN palsies in 1/3 of pts (CN III most common), orbital apex syndrome

Front 114

VIROLOGY: HZO
-Is PO antiviral tx helpful?
-What meds can be used for postherpetic neuralgia?
-VZV vaccine: % reduction in incidence and % reduction in post-herpetic neuralgia?
-How are PO antivirals dosed in HZO?
-Mgmt of cutaneous lesions?
-Role for topical antivirals?
-Is antiviral ppx useful?

Back 114

HZO
-PO antivirals dec viral shedding from skin lesions, dec risk of systemic dissemination, dec incidence/severity of ocular complications, dec duration of postherpetic neuralgia (if begun within 72 hrs)
-Post-herpetic neuralgia tx: amitriptyline, acyclovir, gabapentin, pregabalin (lyrica), carbemazepine
-VZV vaccine: 50% dec incidence of herpes zoster, 66% dec in postherpetic neuralgia
-PO antivirals (no role for topical antivirals): use double dose compared to HSV dosing (i.e., valtrex 1000 tid, acyclovir 800 5x/day)
-Cutaneous lesions: warm compresses, topical antibiotic ung
-Antiviral ppx NOT useful (unlike w/ HSV)

Front 115

VIROLOGY: EBV
-Where does EBV establish latency?
-What are the ocular manifestations of EBV?
-Describe 3 principal forms of EBV stromal keratitis (types 1 - 3)

Back 115

EBV
-Latency in B cells & pharyngeal mucosal epithelial cells
-Ocular disease: uncommon; most common cause of acute dacryoadenitis (inflammatory enlargement of 1 or more lacrimal glands)
-3 forms of stromal keratitis: 1) multifocal (looks like adeno); 2) blotchy, multifocal, pleomorphic, granular-ring shaped opacities; 3) multifocal deep/full thickness peripheral infiltrates (looks like syphilis)

Front 116

VIROLOGY: EBV
-Serologic changes with EBV?
-Mgmt of EBV infection?

Back 116

EBV
-Acute infection: IgM first, then IgG appears to viral capsid antigens (VCA) --> IgG may persist lifelong
-Mgmt: acyclovir NOT effective for mononucleosis; unclear benefit in corneal disease; use steroids with prophylactic antiviral in case of HSV infection

Front 117

VIROLOGY: Adenovirus
-Genetic material form? Structure?
-Subtype assoc with EKC?
-Transmission?

Back 117

Adenovirus
-dsDNA, nonenveloped, has projecting capsid protein (ligand for cellular adenovirus receptor)
-EKC: Subgroup D
-Transmission: contact with ocular, respiratory secretions, fomites, contaminated swimming pools

Front 118

VIROLOGY: Adenovirus
-Describe serotypes and sx for:
1) simple follicular conjunctivitis
2) pharyngoconjunctival fever
3) EKC (sx, complications)

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Adenovirus
1) Simple follicular conjunctivitis: multiple serotypes, self-limited, no systemic disease
2) Pharyngoconjunctival fever: fever, HA, pharyngitis, follicular conjunctivitis, preauricular adenopathy; serotype 3 or 7
3) EKC: serotypes 8,19,37, subgroup D; only syndrome with K involvement!
-EKC: URI preceding eye sx, bilateral, PEE, follicles, chemosis, preauricular nodes, pseudomembranes/membranes
-EKC sx: tearing, FB sensation, photophobia, multifocal subepi infiltrates (likely immunologic)
-Complications of EKC: subepi conj scarring, symblepharon, chronic dry eye

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VIROLOGY: Adenovirus
-General mgmt?
-Mgmt of membranes?
-Indications for steroids?
-How long does viral shedding persist?

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Adenovirus
-General mgmt: supportive --> cool compresses, AT (use topical abx only when suspicious for bact infection)
-Membranes: manual removal q2-3 days, judicious topical steroids (may prolong viral shedding)
-Steroid indications: subepi K infiltrates, membranes
-Viral shedding persists 10-14 days after sx begin (hygiene!)

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VIROLOGY: Poxvirus
-Structure, DNA?
-Molluscum: presentation?
-What are Henderson-Patterson bodies?
-Mgmt?

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Poxvirus (Molluscum)
-Enveloped, dsDNA
-Clinical: umbilicated nodules on skin, lid margin, occ conj; follicular conjunctivitis (many lesions may be seen in AIDS pts)
-Henderson-Patterson bodies: eosinophilic intracytoplasmic inclusions in epithelial cells
-Mgmt: excision, cryo, incision of central portion of lesion

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VIROLOGY: HPV
-Structure? DNA?
-Difference b/w subtypes 6,11 & 16,18?

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HPV
-Small, nonenveloped, dsDNA
-6,11: latent within epithelial cells, cause conj papilloma/skin wart
-16,18: intregrate viral genome into host chromosomal DNA --> risk of malignant transformation, squamous cell ca

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VIROLOGY: RNA viruses
Give structure & family members
-Picornavirus
-Togavirus
-Orthomyxovirus
-Typical clinical scenario of infection
-Measles: classic triad, corneal findings
-Mumps: ocular manifestation?

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RNA viruses
-PIcornavirus: ssRNA, no envelope; enterovirus, rhinovirus
-Togavirus: ssRNA, no envelope; rubella, encephalomyelitis, yellow fever, dengue
-Orthomyxovirus: ssRNA, enveloped; influenza
-Presentation: follicular conjunctivitis assoc with URI
-Measles: paramyxovirus; triad of cough, coryza, follicular conjunctivitis; corneal ulceration (seen in vit A deficient pts)
-Mumps: paramyxovirus, dacryoadenitis, parotiditis

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VIROLOGY: AHC
-What are 3 causes of acute hemorrhagic conjunctivitis?
-Presentation?
-Systemic consequences?

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AHC
-Causes: enterovirus type 70, coxsackievirus A24, adenovirus type 11
-Presentation: sudden onset follicular conjunctivitis, petechial hemorrhages of bulbar/tarsal conj, lid edema, preauricular nodes, PEK
-Highly contagious, rapidly spreading epidemics
-Rarely w/ enterovirus 70, can have polio-like paralysis (may be permanent in 1/3)

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INFECTIONS: Bacteria
-What part of bacteria cell forms basis of gram stain reaction? What is a + or - reaction?
-Describe differences b/w gram + and gram - cell walls
-How do bacteria replicate?
-Function of the following: flagella, pili, fimbriae, adhesins?

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Bacteria
-Gram stain reaction based on cell wall type of bacteria (gram pos = blue, gram neg = red)
-Gram pos cell walls: have peptidoglycan + techoic acid
-Gram neg cell wall: thin peptidoglycan layer, external lipid membrane containing lipopolysaccharide (aka endotoxin)
-Flagella (movement), pili (conjugation), fimbriae (attachment b/w bacterial cells and b/w bact & eukaryotic cells), adhesins (mediate mucosal surface attachment)

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INFECTIONS: Gram + cocci
-Staph: culture/smear appearance? virulence factors?
-Strep: smear appearance? main virulence factors? S. pneumo virulence factors and appearance?
-Enterococcus: smear appearance? where found? disease caused?

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GPCs
-Staph: culture shows grapelike clusters; smears show single,pairs, short chains; can form biofilm, lantibiotics (bactericidal effects on other competing bacteria in environment)
-Strep: smears show pairs/chains; have M proteins (help resist phagocytosis), Steptolysin (lyses RBCs, plts, PMNs), C5a peptidase (destroys C5a), Hyaluronidase (tissue invasion)
-S. pneumo: lancet-shaped diplococci --> has capsule that resists phagocytosis & pneumolysin toxin (inhibits WBC function)
-Enterococcus: pairs/short chains, found in GI/GU tracts; E. faecalis = important cause of endophthalmitis

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INFECTIONS: Gram - cocci
-What type of infection is N. gonorrhea known for?
-Name a diffference b/w N. gonorrhea & N. meningitidis

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GNC
-N. gonorrhea: hyperacute severe conjunctiviti --> can lead to K perforation
-N. gonorrhea is always a pathogen but N. meningitidis may be commensal in pharynx without causing disease

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INFECTIONS: Gram + rods
-C. diphtheriae: smearpattern? type of disease? assoc with vit A deficiency?
-P. acnes: appearance? disease caused?
-Bacillus: found where? type of disease?

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GPRs
-C. diphtheriae: smears show palisading, cuneiform pattern; produecs acute membranous conjunctivitis; C. xerosis found in Bitot spots (vit A deficiency) but unknown significance in this setting
-P. acnes: slender, slightly curved GPR; causes chronic postop endophthalmitis, rarely microbial keratitis
-Bacillus: may be gram + or gram variable, found in soil, produces resistant spores; causes explosive posttraumatic endophthalmitis

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INFECTIONS: Gram - rods
-Pseudomonas: where is it found? virulence factors?
-Enterobacteriacae: important strains?
-Haemophilus: growth media? appearance? type of diseases caused? main virulence factor for HiB?
-B. henslae: aerobic or anaerobic? Staining type? natural reservoir?

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GNRs
-Pseudomonas: contaminant in water; has polar flagella, adhesins, surface pili, toxins
-Enterobacteriacae: Klebsiella, Enterobacter, Citrobacter, Serratia, Proteus
-HFlu: coccobacilli/short rods, grow on chocolate agar, cause bleb infections s/p filterig surgery, Hib: capsule is major virulence factor
-B. henslae: aerobic, use Warthin-Starry staining of bx tissue, cats = natural reservoir

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INFECTIONS: Gram + filaments
-Mycobacteria: staining agent, growth media, which ones are slow vs fast growers (and which of these cause ulcerative keratitis s/p refractive surg)?
-Nocardia & actinomyces: which has acid fast staining? What disease is caused by actinomyces?
-Chlamydia: type of life cycle, which form is infectious?
-Spirochetes: what type of flagella? staining? how are they seen on culture? appearance of T. pallidum?
-B. burgdorferi: stain?

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Gram + filaments
-Mycobacteria: use acid fast stain, Lowenstein-Jensen agar; M. tuberculosis/leprae = slow growers; M. chelonei/fortuitum = fast growers (ulcerative keratitis s/p refractive surg)
-Nocardai: gram variable or gram +, weakly acid fast
-Actinomyces: NO acid fast staining, cause canaliculitis
-Chlamydia: dimorphic life cycle --> elementary body (survives outside host, infectious), reticulate body (non-infectious)
-Spirochetes: have periplasmic flagella (endoflagella), use silver staining, dark field illumination; T. pallidum: fine, corkscrew shaped, rigid, uniform spirals
-B. burgdorferi: Giemsa stain

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INFECTIONS: Fungi
-Components of fungal cell walls?
-Reproduction of yeasts vs molds?
-Which fungus takes up gram stain?
-Stain used for fungal cell walls?
-What component of fungi do most antifungals target?

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Fungi
-Cell walls: chitin, polysaccharides
-Yeasts: reproduce by budding, may form pseduohyphae
-Molds: grow by branching, apical extension
-Candida: takes up gram stain
-Cell walls stain w/ Gomori methenamine silver
-Most antifungals target sterol-containing cell membrane within cell wall

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INFECTIONS: Fungi
-Name 3 important yeasts that cause eye infections
-Where are each found?

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Fungi
-Candida: ubiquitous
-Cryptococcus: acquired through inhalation, subclinical pulmonary infections
-Rhinosporidium: found in soil/groundwater, cause conj, nasal, pharyngeal lesions

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INFECTIONS: Fungi
-Which type of septate filamentous fungus causes a fulminant keratitis?
-What predisposing factor is most common for fungal keratitis?
-Give examples of nonseptate filamentous fungi? what types of infections do they cause?
-What type of disease does P. carinii cause?

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Fungi
-Fusarium causes fulminant keratitis, warm/humid climates (other species cause more indolent keratitis)
-Most cases followed by exposure to vegetative matter
-Mucor, Rhizopus, Absidia: nonseptate filamentous fungi --> cuse life threatening infections of paranasal sinuses, brain, orbit in pts immunocompromised/diabetic/renal failure (acidotic states)
-P. carinii: choroiditis

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INFECTIONS: Parasites - Protozoa
-Acanthamoeba: life cycle forms (which is infectious?)
-Microsporidia: how do they enter eukaryotic cells?
-Toxoplasma gondii: how do humans acquire infection, what is the most common manifestation?

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Parasites - Protozoa
-Acanthamoeba: motile trophozoite (infectious),dormat cyst (double walled, highly resistant to stressors)
-Microsporidia: enter cells by polar tube --> opens hole in eukaryotic cell membrane
-Toxo: cats shed oocytes in feces, swine ingest oocytes --> meat contains cysts --> eaten by humans; causes chorioretinitis

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INFECTIONS: Parasites
-Leismania: vector, geographic distribution of cases, pathogenesis, staining, type of inflammation

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Parasites - Leismania
-Vector: female sandfly
-Locations: tropical Asia, Africa, Latin America
-Hide within phagolysosomal system of macrophages
-May have granulomatous inflammation within infected lid ulcer
-Giemsa, immunofluorescent stains

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INFECTIONS: Onchocerciasis
-Vector?
-Endemic areas?
-Pathophysiology?
-Manifestations of ocular onchocerciasis?
-What factors does disease severity depend on?
-Treatment?

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Onchocerciasis
-Vector: blackfly (lays eggs in trailing vegetation on fast moving rivers --> river blindness)
-Endemic to sub-Saharan Africa, Middle East, Latin America
-Pathophys: penetrates skin --> matures in nodules at site of fly bite (can live for 15 yrs in host)
-Eye manifestations: keratitis (PEE, "snowflake" corneal opacities), uveitis, chorioretinitis --> occur upon death of microfilariae
-Disease severity depends on degree of exposure to blackfly bites & density of microfilariae in tissues
-Rx: nodulectomy, oral ivermectin, control of blackfly population

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INFECTIONS: Helminths
-Loa loa: vector, pathophysiology
-Toxocara: another name for this? where do organisms naturally reside?
-Taenia solium: how acquired? name of disease?

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Helminths
-Loa loa: mango horsefly vector, migrates through conn tissues to reach eye (causes transient hypersensitivity reactions)
-Toxocara AKA visceral larval migrans: resides in dogs/cats
-Taenia solium: caused by ingestion of undercooked pork --> hyatid cysts form --> cysticercosis

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INFECTIONS: Arthropods
-Disease caused by phthriasis pubis?
-Demodex: which species infects eye, appearance of lashes?
-Ophthalmomyiasis: cause of disease, how acquired?

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Arthropods
-Phthiriasis pubis: venereally acquired crab louse, causes blepharoconjunctivitis
-Demodex: follicularis & brevis inhabit normal lashes --> inc colonization with age; cause cylindrical sleeves around lash bases --> blepharitis sx
-Ophthalmomyiasis: maggot infestation of eye; female lands on host & deposits eggs/larvae

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INFECTIONS: Staph blepharitis
-2 most common causes of blepharitis?
-Clinical sx?
-Clinical signs?
-Appearance of chronic conjunctivitis?
-Difference in appearance b/w chronic conjunctivitis 2/2 staph vs moraxella?

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Staph blepharitis
-2 most common causes of blepharitis: staph infection OR irritation from oily meibomian gland secretions
-Sx: younger pts; AM burning, itching, crusting --> improves over day
-Signs: hard scales/crusts at lash margin (collarettes), lid margin telangiectasias, poliosis (white lashes), madarosis, trichiasis
-Chronic conjunctivitis: > 4 weeks, chronic tarsal papillae, injection, scant mucopurulent d/c
-S. aureus chronic conjunctivitis: matted golden crusts/ulcers on lid margin
-Moraxella lacunata: chronic angular blepharoconjunctivitis (crusting, ulcers of lateral angle skin)

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INFECTIONS: Staph blepharitis
-Pathophysiology of phylencticulosis?
-Most common etiology in industrialized/non-industrialized countries?
-Clinical appearance, progression?

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Staph blepharitis: Phylenticulosis
-local corneal/conj inflammation --> hypersensitivity reaction induced by microbial antigens
-S. aureus (industrialized), M. Tb (nonindustrialized)
-Clinical: perilimbal small, rounded, gray/yellow elevations (focal inflammatory nodules) with zone of hyperemic conj vessels --> become necrotic and ulcerate --> spontaneous involution --> wedge-shaped fibrovascular scars along limus (tends to be recurrent)

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INFECTIONS: Staph blepharitis
-Characteristic lab finding on culture?
-Mgmt?
-Mgmt if prominent conjunctivitis?
-Mgmt if phylenticulosis or marginal infiltrates?

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Staph blepharitis
-Lab finding: heavy, confluent growth of S. aureus on culture
-Mgmt: lid hygeine (wet washcloth, baby shampoo, abx ung, AT)
-Prominent conjunctivitis: topical abx
-Phylencticules, marginal infiltrates: topical steroids (strong immunologic component)

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INFECTIONS: Hordeolum
-Which glands involved in external vs internal hordeola?
-Clinical course/presentation?
-Mgmt?

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Hordeolum
-Internal: occur in posterior lid, 2/2 meibomian gland inspissation
-External: anterior eyelid, 22/ glands of Zeis or lash follicles
-Clinical: painful, nodular, erythematous mass --> spontaneously resolve over 1-2 weeks; may evolve into chalazia (chronic granulomatous nodules around sebaceous glands) if internal
-Mgmt: Warm compresses (facilitate drainage), use systemic abx only if secondary eyelid cellulitis, topical abx not effective, may need I&D if persistent or large

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INFECTIONS: Bact conjunctivitis
-How is infection acquired?
-Which 2 organisms can cause severe, sight threatening infection?
-Characteristics of acute bat conjunctivitis?
-3 most common pathogens? What are characteristics & populations assoc with each?

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Bacterial conjunctivitis
-Infection acquired through direct contact, spread from nasal/sinus mucosa, or NLD (examine NLD system if unilateral!)
-Sight-threatening: N. gonorrhea, S. pyogenes
-Presentation: <3 wks conj inflammation, purulent d/c
-Most common causes: S. aureus, S. pneumo (hemorrhages, inflammatory membranes), H. flu (smokers, COPD, kids w/ otitis media)

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INFECTIONS: Bact conjunctivitis
-Name drugs that can be used for initial medical tx
-What abx should be used for H. flu?
-When are supplemental PO abx recommended?

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Bact conjunctivitis
-Initial tx: polymixin, aminoglycoside, FQ, bacitracin qid x 7days
-H.flu: use polymixin B-trimethoprim
-Supplemental PO abx: conjunctivitis-otitis syndrome, pharyngitis assoc w/ acute conjunctivitis

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INFECTIONS: Gonococcal conjunctivitis (adults)
-Clinical presentation?
-What % of cases have keratitis?
-2 types of growth media?
-Mgmt of pts w/ and w/o K ulceration?
-What if PCN allergy?

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Adult gonococcal conjunctivitis
-Clinical: explosive severe, purulent conjunctivitis, severe chemosis, lid edema, rapid progression --> can lead to K ulceration, melting, perf
-Preauricular lymphadenopathy, keratitis (30-40%)
-Culture media: chocolate agar, Thayer-Martin media
-Mgmt: 1 g IM ceftriaxone (no K ulceration), 1 g IV ceftriaxone q12h x 3 days (K involved)
-If PCN allergic: spectinomycin or fluoroquinolone
-Other mgmt: topical abx, copious irrigation of conjunctiva w/ saline, prophylactic chlamydia tx

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INFECTIONS: Neonatal gonococcal conjunctivitis
-Presentation? Timing after birth?
-Mgmt?

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Neonatal gonococcal conjunctivitis
-Presentation: bilateral conj discharge 3-5 days after birth --> may be serosanguinous in 1st few days --> then comes purulent
-Mgmt: single IM ceftriaxone (nondisseminated) vs IV ceftriaxone (disseminated - consult ID) + hourly saline irrigation until d/c eliminated

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INFECTIONS: Neonatal chlamydial conjunctivitis
-Name 5 ways in which neonatal chlamydial conjunctivitis differs from adult
-Mgmt?

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Neonatal chlamydial conjunctivitis
-Differs from adult chlamydial conj in that neonates: 1) have no follicular response, 2) more purulent discharge, 3) propensity to develop tarsal membranes, 4) more specimens show intracytoplasmic inclusions with Giemsa stain, 5) more likely to respond to topical meds
-Mgmt: systemic erythromycin x 14 days (b/c risk of other systems involvement)

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INFECTIONS: Chlamydial conjunctivitis (adults)
-Which serotypes responsible for trachoma, adult/neonatal conjunctivitis & lymphogranuloma venereum (respectively)?
-Diagnostic methods?

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Adult chlamydial conjunctivitis
-Trachoma: serotypes A-C
-Adult/neonatal conjunctivitis: erotypes D-K
-Lymphogranuloma venereum: serotypes L1, L2, L3
-Labs: Giemsa stain, culture, PCR

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INFECTIONS: Chlamydial conjunctivitis (adult) - Trachoma
-Epidemiology?
-Clinical presentation?
-What is an Arlt line?
-What are Herbert pits?
-Sequelae of chronic infection?

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Trachoma
-Endemic to Middle East
-Leading cause of preventable blindness worldwide
-Transmission: eye-to-eye contact, flies, fomites
-Clinical: severe follicular reaction (greatest in sup tarsal conj), epith/stromal keratitis, fibrovascular pannus in sup 1/3 of cornea
-Arlt line: linear/stellate scarring of sup tarsus
-Herbert pits: limbal depressions 2/2 involution/necrosis of follicles
-Chronic infection --> aqueous tear deficiency, trichiasis, entropion

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INFECTION: Chlamydial conjunctivitis (adult) - Trachoma
-What are the features needed to make diagnosis (need 2/4)?
-What are the 5 key signs to note on WHO severity grading scale?
-Mgmt?

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Trachoma
-Diagnosis: 1) superior tarsal conj follicles, 2) limbal follicles/Herbert pits, 3) Arlt line, 4) Superior corneal fibrovascular pannus
-WHO grading scale: 5 key signs: 1) follicular inflammation, 2) diffuse conj inflammation, 3) tarsal conj scarring, 4) aberrant lashes, 5) K opacification
-Mgmt: topical + PO tetracycline/erythromycin (topical x 2 months, PO x 3 wks) --> use PO erythromycin if resistant to tetracyclines; can also use single dose azithromycin

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INFECTIONS: Chlamydial conjunctivitis (adult)
-How acquired?
-Clinical presentation?
-Mgmt?

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Adult chlamydial conjunctivitis
-Sexually transmitted (found in conjunction with urethritis, cervicitis)
-Onset 1-2 weeks after ocular inoculation
-Clinical: follicular conj response (lower palpebral conj, fornix), scant mucopurulent d/c and preauricular nodes
-NO inflammatory membranes
-K involvement: epi/subepi infiltrates (superior cornea most commonly involved), micropannus
-Mgmt: spontaneous resolution 6-18 months; use tetracycline, azithromycin or erythromycin, evaluate for coinfection (syphilis, gonorrhea)

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INFECTIONS: Parinaud oculoglandular syndrome
-Primary causative agent?
-Transmission?
-Clinical presentation?
-Testing?
-Mgmt?

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Parinaud oculoglandular syndrome
-B. henslae
-Transmitted by cats & fleas (cat bite, scratch)
-Clinical: unilateral granulomatous conjunctivitis (gelatinous granulomatous lesion on conj), unilateral regional adenopathy (may become suppurative), mild systemic sx (fever, multisystem disease in minority)
-Dx: serologic testing (for Ab's)
-Mgmt: unknown ideal mgmt (same as chlamydia, can use adjuvant Rifampin)

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INFECTIONS: Bact keratitis
-Most common predisposing factor in US?
-Pathogenesis?

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Bacterial keratitis
-CL wear: most common predisposing factor in US (15x inc risk if worn overnight; positive correlation w/ # of days lenses worn consecutively w/o removal)
-Pathogenesis: bacteria bind, proliferate --> invade stroma --> PMNs migrate in from periphery --> host enzymes and MMPs cause inflammatory necrosis --> antimicrobials given, wound healing/scarring begins (or if no tx, can cause perforation)

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INFECTIONS: Bact keratitis
-Presentation?
-What is infectious crystalline keratopathy?
-Predisposing factors for crystalline keratopathy?
-5 most common organisms?

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Bacterial keratitis
-Rapid onset pain, conj injection, dec vision, photophobia
-Well demarcated epi defect, poorly defined dense underlying stromal infiltrate & edema
-Infectious crystalline keratopathy: caused by slow growing, fastidious organisms --> nonsuppurative infiltrate, intact epithelium (densely packed, white branching aggregates of organisms, no host inflammation)
-Seen in steroid use, CL wear, infected K grafts
-Most common organisms: S. aureus, S. epi, Strep species, Pseudomonas, Enterobacteriacae

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INFECTIONS: Bact keratitis
-What is the benefit of using fortified antibiotics?
-When should oral abx be started?
-What clinical parameters are useful to monitor response to therapy?

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Bacterial keratitis
-Fortified abx: produce therapeutic abx concentrations in corneal stroma (unlike other abx)
-PO abx: use when suspected scleral/intraocular extension (FQ have good ocular penetration)
-Clinical parameters for response to therapy: 1) blunting perimeter of stromal infiltrate, 2) dec density of stromal infiltrate, 3) dec edema, endothel inflammatory plaque, 4) dec AC inflammation, 5) re-epithelialization, 6) dec K thinning

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INFECTIONS: Bact keratitis
-When is it appropriate to use FQ monotherapy?
-What can be used to treat documented strep infections?

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Bacterial keratitis
-FQ monotherapy: small ulcers (<3mm), noncentral location, no significant thinning
-Strep: cell wall active agent --> vanc, cefazolin, PCN G, bacitracin

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INFECTIONS: Bact keratitis
-What are criteria for instituting steroids in bact keratitis?
-What are indications for PK in bacterial keratitis?

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Bacterial keratitis
-Steroids: not in initial phase, wait until etiologic agent identified, need freq follow up and good compliance, no hard-to-eradicate organisms, favorable response to abx documented
-PK: progressive disease despite tx, perf,descemetocele (need simultaneous PI to avoid pupillary block from inflammatory membranes)

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INFECTIONS: Fungal keratitis
-Name some risk factors for fungal keratitis
-Clinical presentation?

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Fungal keratitis
-Risk factors: trauma w/ plant/vegetable matter, steroid use, CL wear, corneal surgery, chronic keratitis
-Presentation: dec inflammation and sx compared to bact keratitis in initial phase
-Filamentous fungi: gray-white feathery infiltrate; +/- multifocal/satellite infiltrates; may see infiltrate with intact epithelium, hypopyon
-Yeast (candida): superficial white raised colonies

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INFECTIONS: Fungal keratitis
-Stains, culture media?
-Mgmt of fungal keratitis?

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Fungal keratitis
-Stains: Gomori methenamine silver
-Culture: Sabouraud's, blood, brain-heart infusion media
-Filamentous keratitis: Natamycin 5% (esp for Fusarium), topical Ampho B for Aspergillus
-Yeast keratitis: topical Ampho B
-Use topical voriconazole if no response to traditional tx
-PO antifungals: ketoconazole (filamentous), fluconazole (yeast) --> use if severe infection
-Other methods of mgmt: mechanical debridement (superficial infection)

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INFECTIONS: Acanthamoeba keratitis
-What are acanthamoebae?
-What are major risk factors for infection?
-Clinical presentation?

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Acanthamoeba keratititis
-Free living, ubiquitous protozoa in freshwater, soil
-Resist dessication, freezing, chlorine
-Risk factors: 70% of cases assoc with CL use, homemade saline solution
-Clinical: severe eye pain, photophobia, protracted course
-Early: may look like PEE or dendritic
-Later: central stromal infiltrate (gray, white nonsuppurative)--> may form paracentral ring infiltrate; can see radial perineuritis (enlarged corneal nerves)

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INFECTIONS: Acanthamoeba keratitis
-Stain/culture media?
-What is the most important prognostic indicator of successful treatment?
-How to distinguish b/w Acanthamoeba vs HSV?

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Acanthamoeba keratitis
-Stains: Giemsa, PAS, calcofluor white, acridine orange
-Culture: Non-nutrient agar with E. coli overlay (preferred), buffered charcoal yeast extract, blood agar
-Early diagnosis = most important prognostic indicator of tx success
-Acanthamoeba vs HSV: 1) noncontiguous, multifocal epithelial/subepi lesions, 2) pain disproportionatel severe 2/2 perineural inflammation, 3) +risk factors such as CL use, contaminated freshwater exposure, 4) failed response to initial antiviral tx

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INFECTIONS: Acanthamoeba keratitis
-Prognosis? (epith vs stromal stage of disease)
-Treatment?
-Prognosis s/p PK? Pathophysiology of recurrence s/p PK?
-When should PK be performed?

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Acanthamoeba keratitis
-Prognosis: 1) epithelial stage: debridement f/b 3-4 months tx; 2) stromal infiltrates: 6-12 mo tx
-Meds: diamidines, biguanides, aminoglycosides, azoles (most have efficacy in killing free living trophozoite form, but not for cysts)
-PK: High risk of recurrence; thought to be 2/2 occasional residual viable cyst in immunocompromised eye s/p PK (topical steroids used after PK)
-Indications for PK: s/p completion of full course amebicidal tx, minimum 3-6 mo disease free follow up

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INFECTIONS: Microsporidiasis
-Which pt population is prone do disease?
-What type of organism is microsporidia?
-Characteristics of keratoconjunctivitis?
-Histologic appearance?
-Mgmt?

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Microsporidiasis
-Intracellular protozoa
-Pt population: AIDS pts (conjunctivitis, epithelial keratopathy, disseminated disease), immunocompetent pts (stromal keratitis)
-Keratoconjunctivitis: superficial "mucoid" nonstaining opacities, dense areas of fine punctate staining, clear stroma/AC
-Histo: use Brown-Hopps stain, small gram+ spores in conj epithelial cells
-Meds: topical fumagillin, PK if severe/perforation

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INFECTIONS: Loiasis
-What is Loa Loa?
-What is the vector? How does disease reach eye?
-Endemic areas?
-Mgmt?

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Loiasis
-Filarial nematode
-Vector: female deer fly
-Endemic areas: West/Central Africa
-Reaches eye by burrowing subcutaneously into eye --> conjunctivitis
-Mgmt: extraction of filarial worm --> antiparasitic tx (if widespread infection) w/ Diethylcarbamazine or Ivermectin

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INFECTIONS: Microbial scleritis
-Pathophysiologic mechanisms of disease?
-Mgmt?

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Microbial scleritis
-Most cases 2/2 extension from peripheral cornea, trauma, contaminated FB (i.e. scleral buckle), s/p pterygium surgery (esp when beta irradiation or mitomycin used)
-Mgmt: smears/cultures to identify source; topical, subconj or IV abx

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IMMUNOLOGY: Cells
-What Ab do lacrimal gland plasma cells produce?
-What structures comprise the ocular surface?
-What are Langerhans cells?

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Immune cells
-Lacrimal gland plasma cells produce secretory IgA
-Ocular surface: conj, cornea, meibomian glands
-Langerhans cells: dendritic APCs in conj stroma (sentinel cells of ocular surface immune system) --> can uptake Ags and sensitize naive Ag-inexperienced T cells
-Corneal Langerhans cells: in K epithelium of limbus & periphery --> are activated, mature cells (express MHC II Ags) --> capable of effectively stimulating T cells

Front 166

IMMUNOLOGY: Cornea
-What factors confer the cornea's immunopriveleged status?

Back 166

Cornea - immunoprivelege
-No blood vessels or lymphatics
-Expresses immunosuppressive factors (TGF-beta, alpha-MSH)
-Expresses Fas ligand (CD95) --> mediates apoptosis of activated lymphocytes

Front 167

IMMUNOLOGY: Cells
-What are chemokines? Examples?

Back 167

Cells
-Chemokines = chemotactic cytokines --> provide trafficking signals to immune cells (i.e., IL-8 for PMNs, MIP-1 beta for TH1 cells, MCP-1 for monocytes, eotaxin for eosinophils)

Front 168

IMMUNOLOGY: Hypersensitivity reactions
-What are hypersensitivity reactions?
-Pathophys of Type I reaction? Examples of disease?

Back 168

Hypersensitivity reactions
-Normal adaptive protective mechanisms that become so amplified (2/2 increased Ag exposure or heightened immune status) that they cause pathologic changes
-Type I (Anaphylactic/atopic): APCs interact w/ TH2 cells --> cytokines released --> Ags combine w/ IgE bound to mast cells --> degranulation (histamine, mediator release)
-Type I diseases: allergic conjunctivitis, atopy (mutation in IL-4 (cytokine) receptor --> inc IgE production, inc T cells)

Front 169

IMMUNOLOGY: Hypersensitivity reactions
-Pathophys of Type II reaction? Examples of disease?

Back 169

Hypersensitivity reactions
-Type II (Cytotoxic): Igs interact w/ Ags closely assoc w/ cell membranes --> complement activation --> cell lysis
-Type II disease: ocular cicatrical pemphigoid (IgG/A interacts w/ Ags along conj basement membrane)

Front 170

IMMUNOLOGY: Hypersensitivity reactions
-Describe pathophys of Type III reactions? Examples of disease?
-Describe pathophys of Type IV reactions? Examples of disease?

Back 170

Hypersensitivity reactions
-Type III: Ab-Ag complexes deposited in tissues --> secondary complement/effector cell activation
-Arthus reaction: Type III reaction within small blood vessels --> vasculitis
-Type III reactions: Scleritis, Stevens Johnson syndrome
-Type IV: T cells interact with Ags --> become sensitized --> release lymphokines
-Type IV diseases: corneal graft rejection, contact dermatitis, phylenticulosis

Front 171

IMMUNOLOGY: Conjunctiva/Cornea
-What confers conjunctival humoral and cell mediated immunity?
-What types of immune reactions can the cornea have and NOT have?
-What is a Wessely immune ring?

Back 171

Conj/Cornea
-Conj humoral immunity: mediated by IgA
-Conj cell-mediated immunity: mediated by CD4 cells
-Cornea CANNOT have allergic reactions (no mast cells) or Arthus reactions (no blood vessels) --> can have humoral/cellular responses (enter cornea from limbal vessels in periphery)
-Wessely immune ring: ring-shaped stromal infiltrate containing complement, PMNs --> occurs in response to Ags deposited in cornea (drugs, foreign body, etc)

Front 172

IMMUNOLOGY: Cytological cues
-What is the most likely etiology of disease if the most significant cytological finding is...
-PMNs
-Lymphocytes/monocytes
-Eosinophils
-Basophils/mast cells
Keratinized epithelial cells

Back 172

Cytology
-PMNs: severe acute/subacute ocular surface inflammation
-Lymphocytes: chronic toxic or allergic conjunctivitis
-Eosinophils: acute allergic conjunctivitis
-Basophils/mast cells: vernal conjunctivitis
-Keratinized epithelial cells: ocular cicatrical pemphigoid, SJS, KCS, GVH disease

Front 173

IMMUNOLOGY: Contact dermatoblepharitis
-What types of hypersensitivity reactions are responsible?
-What is the difference in clinical presentation of the two different reactions?
-What meds commonly cause this?

Back 173

Contact dermatoblepharitis
-Type I (IgE-mediated acute anaphylactic reaction): within minutes after exposure, itching, lid edema/erythema, chemosis, +/- systemic anaphylaxis
-Type IV (delayed): within 24-72 hrs after re-exposure to Ag, acute eczema w/ leathery thickening/scaling, skin hyperpigmentation, papillae, mucoid d/c
-Drugs that cause: cycloplegics, aminoglycosides, antivirals, preservatives (thimerosal, EDTA)

Front 174

IMMUNOLOGY: Contact dermatoblepharitis
-Mgmt?

Back 174

Contact dermatoblepharitis
-Mgmt: avoid allergen, cold compresses, topical mast cell stabilizers/antihistamines, NSAIDs, steroids (if severe)
-Note: avoid topical vasoconstrictors --> provide sx relief but do not use chronically

Front 175

IMMUNOLOGY: Atopic dermatitis
-What is the pathophysiology?
-What other atopic conditions may be associated?
-What other ocular surface disorders can coexist?

Back 175

Atopic dermatitis
-Pathophys: inc IgE hypersensitivity --> inc mast cell histamine release; impaired cell mediated immunity
-Other atopic conditions: allergic rhinitis, nasal polyps, aspirin hypersensitivity
-Ocular surface diseases: keratoconus, pellucid marginal degeneration, staph/HSV eye infections

Front 176

IMMUNOLOGY: Atopic dermatitis
-Clinical findings?
-Mgmt?

Back 176

Atopic dermatitis
-Clinical: pruritis, lesions on lids/other sites (i.e., extensor surfaces), periorbital darkening, exaggerated lid folds, ectropion, chronic conjunctivitis
-Mgmt: allergen avoidance, topical steroid cream/ung (acutely), topical tacrolimus, PO antihistamines (may exacerbate dry eyes)

Front 177

IMMUNOLOGY: Perennial/Hay fever allergic conjunctivitis
-Pathophysiology?
-Clinical presentation? Hallmark symptom?
-Mgmt?

Back 177

Perennial allergic/hay fever conjunctivitis
-Pathophys: airborne Ags enter tear film --> contacts conj mast cells --> IgE mediated hypersensitivity
-Clinical: intense itching = hallmark sx; lid swelling, injection/chemosis, discharge
-Mgmt: avoid allergens, topical antihistamines/NSAIDs, cold compresses, AT (same as contact dermatoblepharitis)
-Note: AT can wash away Ags and inflammatory mediators from tear film
-Note: Avoid topical vasoconstrictors for sx relief --> can cause rebound hyperemia when d/c'd
-Note: reports of corneal perforation with use of NSAIDS --> caution!

Front 178

IMMUNOLOGY: Vernal keratoconjunctivitis
-Type of hypersensitivity reaction?
-Gender predilection?
-Sx and exam findings?

Back 178

Vernal keratoconjunctivitis
-Type I and IV hypersensitivity reactions; seasonally recurrring and bilateral
-M>F
-Sx: itching, blurry VA, photophobia, copious mucoid d/c
-Exam: upper papillary hypertrophy (may form giant papillae = cobblestones), hyperemia, chemosis; PEE superiorly/centrally, superior pannus, shield ulcers (noninfectious)

Front 179

IMMUNOLOGY: Vernal keratoconjunctivitis
-What is limbal VKC? Population? Exam findings?
-What is one corneal disease associated with VKC?

Back 179

Vernal keratoconjunctivitis
-Limbal VKC: hotter climates, Africans/Asians; limbus with thickened, gelatinous appearance, scattered opalescent mounds and vasc congestion
-Horner Trantas dots: whitish dots that are aggregates of degenerated eosinophils and epithelial cells --> seen in hypertrophied limbus
-VKC has been reported to be associated with keratoconus

Front 180

IMMUNOLOGY: Vernal keratoconjunctivitis
-Mgmt?

Back 180

Vernal keratoconjunctivitis
-Mgmt if mild: topical antihistamines, mast cell stabilizers
-Mgmt if severe: topical steroids or cyclosporine (use if moderate to severe discomfort or dec VA); can do pulse therapy
-Other options: supratarsal steroid injection

Front 181

IMMUNOLOGY: Atopic keratoconjunctivitis
-Pathophysiology?
-Coexisting bact/viral diseases?
-Name some differences b/w atopic and vernal keratoconjunctivitis

Back 181

Atopic keratoconjunctivitis
-Primarily a Type IV hypersensitivity (mast cell stabilizers do not work); pts may have impaired cell-mediated immunity
-More susceptible to HSV keratitis & S. aureus colonization of lids
-AKC vs VKC: AKC is year-round, older pts, smaller papillae in both upper & lower palpebral conj, "milky" conj edema, extensive corneal neovasc and opacification due to chronic epi disease, conj scarring --> symblepharon, lens opacities

Front 182

IMMUNOLOGY: Atopic keratoconjunctivitis
-Mgmt?

Back 182

Atopic keratoconjunctivitis
-Allergen avoidance
-Similar tx to VKC
-Monitor for infectious complications
-In severe cases, may need systemic immunosuppression

Front 183

IMMUNOLOGY: Ligenous conjunctivitis
-Pathophysiology?
-Clinical presentation?
-Mgmt?

Back 183

Ligenous conjunctivitis
-Defect in plasminogen gene on Ch 6 --> severe type I plasminogen deficiency --> hypofibrinolysis is primary problem
-Clinical: ocular irritation, FB sensation, firm ("woody") fibrinous plaques on palpebral conj surfaces visible w/ eversion
-Mgmt: frequent recurrences s/p excision, no single treatment consistently effective

Front 184

IMMUNOLOGY: CL associated conjunctivitis
-Name 4 mechanisms by which CLAC occurs

Back 184

CL-associated conjunctivitis
-Repeated mechanical trauma to superior tarsus by CL surface, prosthesis or suture
-Hypersensitivity reaction to CL polymer
-Dry eye (2/2 dec blinking and inc evaporative tear loss from CL wear)
-Surface deposits on CL

Front 185

IMMUNOLOGY: CL associated conjunctivitis
-Clinical presentation?
-Features of GPC?
-Prognosis of GPC?

Back 185

CL-associated conjunctivitis
-Clinical: mild superior tarsal papillae, PEE, peripheral K infiltrates/neovasc
-GPC: primarily soft CL wearers; CL intolerance, itching, excessive mucous, blurry VA from mucous coating on CL, injection, abnormally large papillae on sup tarsal conj
-Prognosis of GPC: Sx improve w/ discontinuation of CL wear but papillae may persist months --> years

Front 186

IMMUNOLOGY: CL-associated conjunctivitis
-Mgmt?

Back 186

CL-associated conjunctivitis
-CL mgmt: discard offending CL, refitting, improve lens hygeine, use daily-wear rather than extended wear, 1-month CL holiday
-Cleaning CL: use preservative-free agents, disinfection w/ hydrogen peroxide system (best tolerated), periodic replacement of lens cases
-Can also change to RGP CL

Front 187

IMMUNOLOGY: SJS/Toxic epidermal necrolysis
-Most common inciting agents?
-Pathophysiology?
-What is toxic epidermal necrolysis? Population affected?

Back 187

SJS/TEN
-Most common inciting agents: drugs, infections
-Pathophysiology: immune complex deposition in dermis/conj stroma
-Toxic epidermal necrolysis: most severe form of this condition --> seen in children, AIDS pts

Front 188

IMMUNOLOGY: SJS/TEN
-What is erythema multiforme? Difference between major and minor forms?
-Most common pt populations affected?
-Presentation?
-Most common ocular findings?
-What are the late ocular complications?

Back 188

SJS/TEN
-Erythema multiforme: acute inflammatory vesicobullous reaction on skin & mucous membranes
-Classic lesion: "target" with red center, surr by pale ring then red ring
-Minor: skin only
-Major: skin & mucous membranes (SJS)
-Pt population: children/young adults, F>M
-Presentation: fever, arthralgia, malaise, URI --> skin eruption with target lesions --> mucous membrane lesions (lesions cycle q2 weeks)
-Primary ocular findings: mucopurulent conjunctivitis, episcleritis
-Late complications: conj shrinkage, keratinization, trichiasis, tear deficiency

Front 189

IMMUNOLOGY: SJS/TEN
-Mgmt?
-Role of topical steroids?
-Role of PK and amniotic membrane transplantation?

Back 189

SJS/TEN
-Supportive care: lubricants (preservative free), monitor for infection
-Steroids: controversial role (can cause K thinning/perf but do decrease inflamm and surface angiogenesis)
-Amniotic membrane transplantation: does have significant long term benefit when applied over entire ocular surface including lid margins
-PK: poor prognosis --> use only for K thinning or perforation

Front 190

IMMUNOLOGY: Ocular cicatricial pemphigoid
-Pathophysiology?
-What is the main difference b/w pseudopemphigoid and true pemphigoid?
-Name 4 categories in the differential diagnosis?
-What disease is clinically identical to OCP?

Back 190

Ocular cicatricial pemphigoid
-Pathophys: Type II hypersensitivity reaction --> Abs against cell surface antigen in conj basement membrane zone (BP180) --> Ab activates complement --> breakdown of conj membranes
-Pseduopemphigoid: disease progression ceases once offending agent recognized and removed (true pemphigoid does not)
-DDx: postinfectious conditions, autoimmune conditions, prior conj trauma, severe blepharoconjunctivitis
-Linear IgA dermatosis: causes ocular syndrome identical to OCP

Front 191

IMMUNOLOGY: OCP
-Epidemiology?
-How to differentiate b/w OCP and pemphigus vulgaris?
-Clinical presentation? Stages I - IV?

Back 191

OCP
-F>M, >60 yo
-Pemphigus vulgaris affects primarily skin (rarely affects eyes, no conj scarring)
-Clinical presentation: chronic vesicobullous disease primarily involving conj --> recurrent attacks of mild, nonspecific conj inflammation with occasional mucopurulent d/c
-Stage I: mild subepithelial fibrosis, fine linear opacities
-Stage II: rupture of bullae --> subepi fibrosis, loss of goblet cells, shotening of fornices (watch for forniceal depth < 8 mm)
-Stage III: symblepharon
-Stage IV: extensive adhesions b/w globe, lid --> restricted ocular motility

Front 192

IMMUNOLOGY: OCP
-What laboratory tests are useful in diagnosis?
-How is disease severity judged?
-Mgmt of severe disease?
-Initial drug of choice in mild disease?

Back 192

OCP
-Lab eval: conj biopsy --> immunohistochemical staining for complement, IgG/M/A in basement membrane zone of conj
-Stage II: measure shortening of inferior forniceal depth
-Stage III-IV: extent of symblepharon formation along inferior fornix in quartiles
-Mgmt of severe disease: Cyclophosphamide (mainstay of tx, target = reduce WBC to 2000-3000), Etanercept (alternative tx)
-Dapsone: initial drug of choice in mild disease; avoid in G6PD or sulfa allergy

Front 193

IMMUNOLOGY: Ocular GVH
-What pt population is this normally seen in?
-Ocular GVHD occurs mostly in acute or chronic GVHD?
-Pathogenesis?
-Mgmt?

Back 193

Ocular GVHD
-Pts who are s/p allogeneic bone marrow transplantation
-Ocular GVHD usually seen in CHRONIC forms of GVHD
-Pathogenesis: 1) conj inflammation w/ and w/o subepithelial fibrosis; 2) severe KCS from lacrimal gland T-cell infiltration (occurs in 40-60% of cGVHD)
-Mgmt: aggressive lubrication, punctal occlusion, mucolytics (for severe filamentary keratitis), BCL, topical cyclosporine

Front 194

IMMUNOLOGY: Thygeson SPK
-Microscopic appearance?
-Clinical presentation?
-Hallmark finding?
-Appearance on high magnification?

Back 194

Thygeson SPK
-Microscopy: clumps of enlarged epithelial cells & multiple reflective filamentary structures in deeper layers --> may be linear, curled, branching
-Presentation: bilateral, recurrent episodes of FB sensation, tearing, photophobia, reduced VA, minimal injection; signs/sx exceed apparent clinical signs
-Hallmark finding: multiple "crumblike" elevated K lesions with negative stain; wax and wane over time
-High mag: each opacity is cluster of multiple smaller pinpoint opacities

Front 195

IMMUNOLOGY: Thygeson SPK
-Mgmt (mild vs severe)?

Back 195

Thygeson SPK
-Mild: AT
-Persistent: low-potency topical steroids, BCL; consider topical cyclosporine or tacrolimus given fewer sfx compared to steroids

Front 196

IMMUNOLOGY: Interstitial keratitis
-Pathophysiology?
-How does IK occur in context of congenital syphilis?

Back 196

Interstitial keratitis
-Most cases 2/2 type IV hypersensitivity reaction
-Pathophys: nonsuppurative inflammation of K stroma, no primary involvement of endothelium/epithelium
-When IK occurs as part of congenital syphilis --> usually a later, immune-mediated manifestation of disease (if occurs within 1st 2 years of life, more likely infectious)

Front 197

IMMUNOLOGY: Interstitial keratitis
-Nonocular signs of congenital syphilis?
-Presentation of syphilitic IK?
-What is the etiology of a salmon patch in syphilitc IK?
-Mgmt?

Back 197

Interstitial keratitis
-Nonocular manifestations of syphilis: dental deformities (Hutchinson incisors), bone/cartilage abnormalities (saddle nose, frontal bossing, saber shins), deafness, circumoral radiating scars, MR
-Syphilitic IK (congenital): bilateral, pain, tearing, photophobia, injection, sup stromal inflammation and neovasc --> may progress to K edema
-Salmon patch: intense deep stromal neovasculariation --> makes cornea appear pink (salmon patch)
-Acute mgmt: cycloplegia, topical steroids (limit scarring)
-If no treatment --> burns out after several weeks
-If systemic: PCN

Front 198

IMMUNOLOGY: Reactive arthritis
-Classic triad?
-Predisposing infections?
-Genetic association?
-Clinical presentation?
-Mgmt?

Back 198

Reactive arthritis
-Triad: ocular inflammation, oligoarthritis, urethritis
-Infections: gram negative dysentery (Salmonella, Shigella, Yersinia) or Chlamydial urethritis
-More than 75% have HLA-B27 association
-Clinical: bilateral mucopurulent papillary conjunctivitis (especially with negative cultures), self-limited
(can also have keratitis, iritis, episcleritis)
-Mgmt: palliative --> topical steroids

Front 199

IMMUNOLOGY: Cogan syndrome
-Triad?
-Clinical presentation?
-Mgmt?

Back 199

Cogan syndrome
-Triad: hearing loss, vertigo, stromal keratitis
-Clinical: young adults, h/o URI 1-2 weeks prior, bilateral faint white subepi infiltrates (early), then multifocal nodular infiltrates (later)
-Mgmt: diagnosis of exclusion (no specific lab findings), frequent topical steroids
**Note: important to treat early --> deafness can occur if delayed (use PO steroids for vestibuloauditory sx)

Front 200

IMMUNOLOGY: Blepharoconjunctivitis marginal infiltrates
-Presentation? (typical location, predisposing causes, appearance)

Back 200

Blepharoconjunctivitis marginal infiltrates
-Predisposing causes: CL wear, trauma, endophthalmitis, blepharoconjunctivitis
-Location: 10,2,4,8 o'clock positions (where lid margins intersect w/ K surface)
-Appearance: gray-white, well circumscribed, located 1 mm inside limbus w/ clear zone of cornea b/w infiltrate and limbus (characteristic of staph blepharitis)
-K epithelium overlying infiltrates may be intact, ulcerated or w/ PEE

Front 201

IMMUNOLOGY: Peripheral ulcerative keratitis
-What is the most common systemic disease association?
-Is there a correlation w/ systemic disease activity and PUK?
-Presentation?

Back 201

Peripheral ulcerative keratitis
-Rheumatoid arthritis = most common assoc systemic autoimmune disease
-PUK exacerbations correlate w/ systemic disease exacerbations
-Presentation: unilateral, single sector of peripheral cornea within 2 mm of limbus, accomp by vaso-occlusion of adj limbal vascular network

Front 202

IMMUNOLOGY: Peripheral ulcerative keratitis
-Mgmt?
-Role of topical steroids?
-Examples of collagenase inhibitors?

Back 202

Peripheral ulcerative keratitis
-Supportive mgmt: 1) improve lubrication (dilutes cytokines in tear film), 2) promote re-epithelialization (collagenase inhibitors), 3) suppress systemic inflammation, 4) excision of adjacent limbal conj (removes source of cells/enzymes)
-Topical steroids: do not use if significant thinning --> exacerbates melting
-Collagenase inhibitors: tetracyclines, topical medroxyprogesterone 1%, mucomyst 20%, sodium citrate 10%

Front 203

IMMUNOLOGY: Mooren ulcer
-Presumed pathophysiology?
-Precipitating factors?
-Mgmt?

Back 203

Mooren ulcer
-By definition, unknown cause
-Presumed autoimmune pathophysiology --> autoreactivity to K-specific antigen
-Precipitating factors: trauma, surgery, helminthic infections (higher rate of ulcer in endemic areas)
-Mgmt: many strategies used, few effective treatments

Front 204

IMMUNOLOGY: Mooren ulcer
-Presentation?
-What is an important distinguishing feature b/w Mooren ulcer and idiopathic PUK?
-What are the 2 clinical types of Mooren ulcer?

Back 204

Mooren ulcer
-Presentation: chronic, painful, progressive, idiopathic ulcer of periph K epithelium & stroma --> spreads circumferentially, then centripetally
-Can have perforation w/ minor trauma/infection, vascularization, fibrosis
-PUK vs Mooren ulcer: Mooren ulcer has pure corneal involvement (PUK also has scleral involvement)
-Unilateral form: older pts, slowly progressive
-Bilateral form: younger, male African pts w/ coexisting parasitemia; bilateral, rapidly progressive & poor response to tx

Front 205

IMMUNOLOGY: Episcleritis
-Presentation? How to distinguish from scleritis?
-Mgmt?
-Epidemiology? Systemic disease associations?

Back 205

Episcleritis
-ocular redness w/o irritation (may have mild tenderness), in exposure zone of eye, localized to sector of globe in 70%
-Nodular vs simple (diffuse) forms
-Distinguish from scleritis: bright red/salmon pink color (scleritis: violaceous); blanches w/ 2.5% phenylephrine
-Mgmt: benign, self-limited (minority of cases assoc w/ systemic disease); use topical/PO NSAIDs vs lubricants (if severe/no response can use topical steroids)

Front 206

IMMUNOLOGY: Scleritis
-Pathophysiology? Systemic disease association?
-Epidemiology?
-Presentation?

Back 206

Scleritis
-Immune-complex mediated vasculitis
-1/3 of pts w/ diffuse/nodular scleritis & 2/3 of pts w/ necrotizing scleritis have systemic disease
-Epidemiology: rare in children, F>M, 50% bilateral
-Presentation: gradual (over days) severe boring/piercing ocular pain (may wake from sleep and worsen at night), tenderness to palpation, violaceous hue to sclera, edema, vessels cannot be moved w/ CTA

Front 207

IMMUNOLOGY: Scleritis
-What areas are involved in diffuse anterior scleritis?
-What % of pts have visual morbidity, systemic disease with necrotizing scleritis?

Back 207

Scleritis
-Diffuse anterior scleritis: portion of anterior sclera involved in 60% of cases; entire anterior segment involved in 40%
-Necrotizing scleritis: 60% w/ ocular/systemic complications, 40% w/ vision loss

Front 208

IMMUNOLOGY: Scleritis
-Describe appearance of necrotizing scleritis w/ inflammation
-Scleromalacia perforans: most common systemic disease association? Presentation?

Back 208

Scleritis
-Necrotizing scleritis w/ inflammation: blue-gray appearance (thinned sclera), large anastomotic vessels circumscribing involved area; may spread around globe until whole globe involved
-Scleromalacia perforans: longstanding RA, painless scleral thinning, bulging staphyloma --> risk of rupture w/ minimal trauma

Front 209

IMMUNOLOGY: Scleritis
-Posterior scleritis: Presentation? Intraocular complications? Diagnostic imaging? Systemic disease association?

Back 209

Scleritis - Posterior
-Presentation: pain, tenderness, proptosis, visionj loss, restricted EOM
-Intraocular manifestations: choroidal folds, exudative RD, papilledema, angle closure glaucoma (2/2 choroidal thickening)
-Imaging: thickened posterior sclera seen on U/S, CT or MRI
-Most often no systemic disease association

Front 210

IMMUNOLOGY: Scleritis
-Most common complications?
-What is sclerokeratitis? Most common systemic disease association?

Back 210

Scleritis
-Complications: peripheral keratitis (37%), scleral thinning (33%), uveitis (30%), cataract (7%), glaucoma (1%)
-Sclerokeratitis: scleritis + neighboring peripheral corneal fibrosis/lipid deposition --> seen w/ HZO

Front 211

IMMUNOLOGY: Scleritis
-Most common complications?
-What is sclerokeratitis? Most common systemic disease association?

Back 211

Scleritis
-Complications: peripheral keratitis (37%), scleral thinning (33%), uveitis (30%), cataract (7%), glaucoma (1%)
-Sclerokeratitis: scleritis + neighboring peripheral corneal fibrosis/lipid deposition --> seen w/ HZO

Front 212

IMMUNOLOGY: Scleritis
-DDx of assoc systemic conditions?
-Lab workup?

Back 212

Scleritis
-Systemic associations: infectious (Lyme, sphylis, Tb, cat-scratch, Hansen disease), autoimmune (RA, SLE, seronegative spondyloarhtropathies, Wegeners, PAN, GCA), gout
-Labs: CBC w/ diff, ESR, CRP, RF, ANCA, ANA, U/A, uric acid, sarcoid screen, sphylis serology

Front 213

IMMUNOLOGY: Scleritis
-DDx of assoc systemic conditions?
-Lab workup?

Back 213

Scleritis
-Systemic associations: infectious (Lyme, sphylis, Tb, cat-scratch, Hansen disease), autoimmune (RA, SLE, seronegative spondyloarhtropathies, Wegeners, PAN, GCA), gout
-Labs: CBC w/ diff, ESR, CRP, RF, ANCA, ANA, U/A, uric acid, sarcoid screen, sphylis serology

Front 214

IMMUNOLOGY: Scleritis
-Mgmt?
-What characterizes treatment failure?
-Treatment options if fail steroid treatment?

Back 214

Scleritis
-Mgmt: systemic treatment --> PO NSAIDs (diffuse, nonnecrotizing), oral steroids,TNF inhibitors (RA-assoc scleritis)
-Treatment failure = 1) progression to more sever form of disease, 2) failure to achieve response after 2-3 weeks of tx
-Non-steroidal systemic immunosuppressives: antimetabolite (MTX), immunomodulator (cyclosporine) or cytotoxic med (cyclophosphamide)

Front 215

IMMUNOLOGY: Scleritis
-Mgmt?
-What characterizes treatment failure?
-Treatment options if fail steroid treatment?

Back 215

Scleritis
-Mgmt: systemic treatment --> PO NSAIDs (diffuse, nonnecrotizing), oral steroids,TNF inhibitors (RA-assoc scleritis)
-Treatment failure = 1) progression to more sever form of disease, 2) failure to achieve response after 2-3 weeks of tx
-Non-steroidal systemic immunosuppressives: antimetabolite (MTX), immunomodulator (cyclosporine) or cytotoxic med (cyclophosphamide)

Front 216

NEOPLASMS: Epithelial cysts
-Histopathological structure?
-Stimulus for formation?
-Location within the eye?

Back 216

Epithelial inclusion cysts
-Inclusion of conj epithelium into substantia propria --> cavity filled with clear fluid, lined by nonkeratinized conjunctival epithelium
-Stimuli: chronic inflammation, trauma, surgery
-Location: bulbar conj or fornix

Front 217

NEOPLASMS: Conjunctival papilloma
-Pathophysiology? Infectious agent association?
-Clinical presentation of sessile vs pedunculated forms?
-Signs of dysplasia?
-Mgmt?

Back 217

Conjunctival papilloma
-Pathophysiology: HPV (6 - children; 16 - adults) initiates neoplastic growth of epithelial cells w/ vascular proliferation
-If HPV 16, 18 --> can result in dysplasia/carcinoma
-Pedunculated: fleshy, exophytic growth with fibrovascular core, emanates from stalk; clear overlying epithelium w/ numerous corkscrew vessels underneath
-Sessile: usu at limbus; glistening surface w/ numerous red dots --> strawberry appearance
-Signs of dysplasia: keratinization (leukoplakia), symblepharon, inflammation, invasion
-Mgmt: spontaneous regression (mo --> yrs); propensity for recurrence when excised
-Monitor for dysplasia in limbal lesions

Front 218

NEOPLASMS: Conj intraepithelial neoplasia (CIN)
-Epidemiology?
-Clinical variants? Location?
-Mgmt?
-Risk of recurrence?

Back 218

CIN
-Epi: older male smokers, petroleum or chronic sunlight exposure, light complexion; if young, suspect HIV
-Location: limbus, interpalpebral zone
-3 clinical variants: papilliform (sessile papilloma w/ dysplastic cells), gelatinous, leukoplakic --> if accompanied by large feeder vessels, higher probability of invasion beneath epith BM
-Mgmt: Excise 3-4mm beyond clinically apparent margins + adjunctive cryo
-Topical IFN/mitomycin C/5-FU
-Recurrence: 33% of completely excised lesions (negative margins); 50% of incompletely excised

Front 219

NEOPLASMS: Corneal intraepithelial neoplasia
-Clinical appearance?
-Mgmt?

Back 219

Corneal intraepithelial neoplasia
-Clinical: granular, translucent, gray sheet of epithelium w/ broad base @ limbus; edges have fimbriated margins, pseudopodia extensions; no neovascularization (helps differentiate from limbal stem cell failure)
-Mgmt: apply absolute EtOH to affected cornea x 30-40 seconds, extend 1-2 mm onto normal cornea --> copious irrigation --> remove devitalized epithelium (leave Bowman's) --> remove adjacent limbus (even if lesion is primarily corneal) --> cryo margins of lesion

Front 220

NEOPLASMS: Squamous cell carcinoma
-Pathogenesis?
-Clinical appearance?

Back 220

Squamous cell carcinoma
-Pathogenesis: UV radiation, viral/genetic factors, compromised immunity, xeroderma pigmentosum
-Clinical: limbal lesion w/ outward growth (sharp borders), leukoplakic appearance, stays superficial (rare penetration of sclera/Bowman's), engorged feeding vessels
-Clinical forms: plaquelike, gelatinous, leukoplakic

Front 221

NEOPLASMS: Squamous cell carcinoma
-Mgmt?
-When is stem cell transplantation required?

Back 221

Squamous cell carcinoma
-Mgmt: complete local excision 4 mm beyond margins; cryo to margins; remove thin lamellar scleral flap; treat remaining sclera w/ absolute EtOH
-Stem cell transplant: needed when >2/3 of limbus removed

Front 222

NEOPLASMS: Other conj/corneal neoplasms
-Mucoepidermoid carcinoma: location, histology, prognosis?
-Spindle cell carcinoma: location, histologic appearance?

Back 222

Other conj/corneal neoplasms
-Mucoepidermoid ca: limbal conj, fornix or caruncle; use mucin stain to show malignant goblet cells; more likely to invade globe/orbit than squamous cell ca
-Spindle cell ca: rare, highly malignant, on bulbar/limbal conj; cells look like spindle-shaped fibroblasts

Front 223

NEOPLASMS: Glandular conj tumors
-Oncocytoma: cells of origin? appearance?
-Sebaceous gland carcinoma: predisposing exposure? differential diagnosis?

Back 223

Glandular conj tumors
-Oncocytoma: originates from ductal/acinar cells of lacrimal glands; reddish-brown nodule on surface of caruncle
-Sebaceous gland ca: older/younger pts w/ radiation exposure; look like chronic chalazia or unilateral blepharoconjunctivitis

Front 224

NEOPLASMS: Benign melanosis
-Epidemiology?
-Appearance?
-What is striate melanokeratosis?

Back 224

Benign (racial) melanosis
-Middle-aged pts w/ dark skin
-Light brown pigmentation of perilimbal, interpalepbral bulbar conj
-Striate melanokeratosis: streaks, whorls --> can extend into peripheral corneal epithelium

Front 225

NEOPLASMS: Ocular melanocytosis
-Epidemiology?
-Pathogenesis? Location?
-Clinical appearance?

Back 225

Ocular melanocytosis
-Epidemiology: Blacks, Hispanics, Asians; congenital lesion
-Pathogenesis: proliferation of subepithelial melanocytes (blue nevus); episcleral location
-Clinical appearance: patches of slate gray episcleral pigmentation; immobile; unilateral; may also have inc pigmentation of iris/choroid

Front 226

NEOPLASMS: Ocular melanocytosis
-What is a nevus of Ota?
-What is oculodermal melanocytosis?
-Mgmt? Other ocular conditions?

Back 226

Ocular melanocytosis
-Nevus of Ota: ipsilateral dermal melanocytosis, proliferation of dermal melanocytes in periocular skin (V1 & V2 dermatomes)
-Oculodermal melanocytosis = combined ocular & cutaneous pigmentations
-Mgmt: 10% of pts have secondary glaucoma; higher lifetime risk of malignant melanoma of uvea, orbit, skin, conj

Front 227

NEOPLASMS: Nevus
-In which population are pure intraepithelial nevi seen?
-Clinical appearance? Location?
-Mgmt?
-Which types of nevi should be biopsied rather than observed?

Back 227

Nevus
-Pure intraepithelial nevi seen primarily in children
-Clinical: flat (near limbus) vs elevated (bulbar conj, semilunar fold, caruncle, eyelid margin) vs cobblestone (subepithelial)
-Intralesional epithelial inclusion cysts: seen in 50% of all conj nevi (compound, subepithelial types)
-Cysts may enlarge 2/2 mucin secretion by goblet cells --> falsely appear malignant
-Mgmt: q6-12 mo followup (rare malignant transformation)
-Biopsy if located on palpebral conj, caruncle, plica or fornix

Front 228

NEOPLASMS: Primary acquired melanosis
-Epidemiology?
-Clinical appearance?
-Signs of malingant transformation?
-Mgmt?

Back 228

Primary acquired melanosis
-Epidemiology: light skinned pts (called BENIGN acquired melanosis in dark skinned pts)
-Clinical: unilateral multiple, flat brown patches of pigmentation
-Signs of malignancy: increased nodularity, enlargement, vascularity --> definitively established only w/ biopsy (shows cellular atypia)
-Biopsy lesions on palpebral conj, fornix, caruncle, plica
-Mgmt: biopsy --> if PAM w/o atypia, follow q6-12 mo --> if atypia present, excisional biopsy

Front 229

NEOPLASMS: Melanoma
-Overall mortality rate?
-Pathogenesis?
-Clinical appearance?
-Mets?

Back 229

Melanoma
-25% overall mortality
-Pathogenesis: arise from PAM (70%), de novo (10%) or nevi (2%)
-Clinical: most commonly bulbar conj or limbus; 25% amelanotic; heavy vascularity, nodular growth, can invade globe/orbit
-Mets to regional lymph nodes, brain, lungs, liver, bone

Front 230

NEOPLASMS: Melanoma
-Mgmt?
-Poorest prognostic factors?

Back 230

Melanoma
-Mgmt: excision 4 mm beyond visible margins, removal of thin lamellar scleral flap, absolute EtOH to sclera, cryo to margins, topical mitomycin C
-Poorest prognostic factors: de novo melanomas, no limbal involvement, residual margin disease s/p surgical resection

Front 231

NEOPLASMS: Hemangioma
-Most common location?
-2 systemic diseases assoc with conj hemangioma?

Back 231

Hemangioma
-Palpebral conjunctiva is most commonly involved (represents extension of eyelid hemangioma)
-If diffuse hemangiomatosis of palpebral conj/fornix --> think orbital capillary hemangioma
-Sturge Weber: nevus flammeus (port wine stain), capillary & cavernous hemangiomas, leptomeningeal hemangiomatosis
-Ataxia telangiectasia: epibulbar telangiectasias, cerebellar disease, immune alterations
-

Front 232

NEOPLASMS: Inflammatory vascular tumors
-Pyogenic granuloma: clinical appearance, predisposing factors, treatment?

Back 232

Inflammatory vascular tumors
-Pyogenic granuloma: stimulated by trauma/surgery, chalazion --> exuberant healing granulation tissue, proliferating radial capillaries, red color, smooth, easy bleeding, +stain w/ fluorescein
-Mgmt: topical/intralesional corticosteroids vs excision

Front 233

NEOPLASMS: Kaposi sarcoma
-Pathogenesis? Cells of origin?
-Clinical appearance? Most common locations?

Back 233

Kaposi sarcoma
-Pathogenesis: malignant neoplasm of vascular endothelial cells --> 2/2 HHV-8 infection (AIDS pt if young)
-Clinical: eyelids (purplish nodule), conjunctiva (reddish, highly vascular subconj lesion similar to subconj hemorrhage)

Front 234

NEOPLASMS: Lymphatic tumors
-Lymphangiectasia vs lymphangioma? Differences in appearance?

Back 234

Lymphatic tumors
-Lymphangiectasia: irregularly dilated lymphatic channels in bulbar conj --> may spontaneously fill w/ blood if anomalously connected w/ venous channels
-Lymphangioma: proliferations of lymphatic channel elements (looks like patch of vesicles w/ edema)

Front 235

NEOPLASMS: Lymphocytic tumors
-Lymphoid hyperplasia: pathogenesis? clinical appearance? mgmt?
-Lymphoma: What % are bilateral? Epidemiology?

Back 235

Lymphoid hyperplasia
-Pathogenesis: most likely a low grade B cell lymphoma
-Clinical: indistinguishable from conj lymphoma --> salmon-colored subepith tumor, pebbly appearance (like follicles), minimal elevation
-Mgmt: spontaneous resolution; can also treat w/ excision, topical steroids, radiation
-Lymphoma: 20% bilateral; most pts >50 yo or immunosuppressed

Front 236

NEOPLASMS: Epibulbar dermoid
-Pathogenesis?
-Clinical appearance? Location?

Back 236

Epibulbar dermoid
-Pathogenesis: congenital, faulty development of eyelid folds --> displaced embryonic tissue destined to become skin, located on conj epithelium
-Clinical: well circumscribed, porcelein white lesions at inferotemporal limbus (also seen in orbit, subconj space, central cornea)
-If located on cornea, may have arcuslike deposition of lipid along anterior corneal border

Front 237

NEOPLASMS: Epibulbar dermoid
-Important systemic association?
-Mgmt?

Back 237

Epibulbar dermoid
-Goldenhar syndrome: sporadic or AD; first branchial arch derivatives --> epibulbar dermoid, upper lid coloboma, preauricular skin tags, aural fistulae, vertebral anomalies
-Mgmt: no malignant potential, grow along with child & eye

Front 238

CONGENITAL: Cryptophthalmos
-Pathogenesis?
-Main syndromic form?
-Mgmt?

Back 238

Cryptophthalmos
-Pathogenesis: bilateral, ablepharon (failed formation of eyelids/lashes), cornea merged w/ epidermis, ant chamber/iris/lens variably formed or absent
-Pseduocryptophthalmos: eyelids and assoc structures form but fail to separate (ankyloblepharon)
-Fraser syndrome: aurofacial & urogenital abnormalities w/ or w/o cryptophthalmos (FRAS1 gene)
-Mgmt: surgery for cosmesis or pain relief 2/2 absolute glaucoma

Front 239

CONGENITAL: Microphthalmos
-Pathogenesis?
-Associated systemic conditions?

Back 239

Microphthalmos
-Pathogenesis: small disorganized globe 2/2 failed closure of fetal fissure
-May also have colobomas of iris, ciliary body, uvea, optic nerve
-Associated w/ trisomies of every chromosome (esp trisomy 13), maternal infections, exposure to toxins/radiation

Front 240

CONGENITAL: Nanophthalmos
-Pathogenesis?
-Clinical appearance? Associated ocular conditions?
-Genetics?

Back 240

Nanophthalmos
-Pathogenesis: small, functional eye w/ NORMAL internal organization/proportions
-Genetics: sporadic vs hereditary (nanophthalmos 1 = dominant; nanophthalmos 2 = recessive, membrane-type frizzled protein gene mutation)
-Clinical: short axial length --> high hyperopia (7-15D), high lens:eye volume --> ant segment crowding and glaucoma
-Other associated ocular conditions: steep corneal curvature, thickened sclera, narrow palpebral fissures

Front 241

CONGENITAL: Nanophthalmos
-Histologic appearance?
-Mgmt? Major complication of intraocular surgery?

Back 241

Nanophthalmos
-Histology: frayed collagen fibrils, glycogen-like deposits --> scleral rigidity --> decreased intraocular volume, high propensity for spontaneous choroidal effusion
-Mgmt: cataract surgery complicated by uveal effusion/hemorrhage --> exudative RD; need high IOL power to achieve emmetropia

Front 242

CONGENITAL: Blue sclera
-Why does the sclera appear blue?
-Name 2 syndromes assoc with blue sclera, genetic defects in each, systemic features in each?

Back 242

Blue sclera
-Generalized scleral thinning --> increased visibility of underlying uvea
-Osteogenesis imperfecta, type 1: decreased type I collagen --> bone fragility, blue sclera
-Ehlers Danlos, type VI: defect in lysyl hydroxylase gene --> joint hyperextensibility, kyphoscoliosis, cardiac/skin abnormalities

Front 243

CONGENITAL: Microcornea
-Definition?
-Inheritance?
-Pathogenesis?
-Associated ocular conditions?

Back 243

Microcornea
-K diameter < 10 mm (or 9 mm in newborn)
-AD > AR inheritance
-Pathogenesis: arrest of corneal growth in 5th month of gestation OR overgrowth of anterior tips of optic cup (less space for cornea to develop)
-Assoc eye conditions: hyperopia, angle closure glaucoma (20% develop open angle glaucoma later in life)

Front 244

CONGENITAL: Megalocornea
-Definition?
-Inheritance?
-Pathogenesis?
-Mgmt?
-Assoc ocular conditions?

Back 244

Megalocornea
-K diameter 13-16.5 mm diameter
-X-linked recessive inheritance (M>F)
-Pathogenesis: bilateral, nonprogressive K enlargement thought 2/2 to failed optic cup growth (larger space for cornea to fill) vs arrested buphthalmos (exaggerated K growth compared to rest of eye)
-Mgmt: rule out congenital glaucoma (IOP testing); implant IOL into capsular bag in cataract surgery
-Assoc eye conditions: central cloudy dystrophy of Francois, glaucoma (not congenital glaucoma), iris translucency, etc

Front 245

CONGENITAL: Cornea plana
-Definition?
-Genetics & Pathogenesis?
-Assoc eye conditions?

Back 245

Cornea plana
-K radius of curvature 30-35 D (less than 43 D) --> flat cornea
-Corneal curvature is same as scleral curvature (pathognomonic)
-Genetics: KERA gene (ch12q22) -> keratan sulfate proteoglycans --> role in spacing of collagen fibrils
-Majority of isolated cases in Finnish pts
-Assoc eye conditions: hyperopia (or other refractive error), angle closure glaucoma (shallow AC)

Front 246

CONGENITAL: Posterior embryotoxon
-Definition?
-What % of normal eyes have this finding?
-Inheritance?

Back 246

Posterior embryotoxon
-Thickened, centrally displaced Schwalbe line (may be visible by external exam)
-Schwalbe line = junction of trabecular meshwork and termination of Descemet's membrane
-8-30% of normal eyes have posterior embryotoxon
-Autosomal dominant

Front 247

CONGENITAL: Axenfeld-Rieger syndrome
-What are the 3 characteristic findings?
-What systemic abnormalities are present?
-Genetics & inheritance?

Back 247

Axenfeld-Rieger
-3 findings: posterior embryotoxon, attached iris strands, iris hypoplasia
-Glaucoma occurs in 50% of pts
-Systemic findings: skeletal, cranial, dental, facial abnormalities
-Genetics: chromosome 6p25 (forkhead genes) --> govern anterior chamber development; 75% dominantly inherited

Front 248

CONGENITAL: Peters anomaly
-Definition?
-What % of cases are bilateral? What % of cases have associated systemic malformations?
-What other ocular abnormalities may be present?
-Histology?
-Genetics?

Back 248

Peters anomaly
-Central corneal opacity present at birth, varying degrees of iridocorneal adhesions
-60% of cases bilateral, 60% assoc with systemic abnormalities
-Other eye findings: keratolenticular touch, PFV, cataract, congenital glaucoma, microcornea, aniridia
-Histology: localized absence of K endothelium & Descemet's beneath area of opacity
-Most cases sporadic; genetic mutations: PAX6, PITX2, FOXC1, CYP1B1

Front 249

CONGENITAL: Posterior keratoconus
-Clinical appearance?
-Laterality, genetics, risk of progression?

Back 249

Posterior keratoconus
-Localized central/paracentral indentation of posterior cornea w/o anterior protrusion
-Overlying stromal haze and thinning (up to 1/3 of normal cornea)
-Descemet's & endothelium are PRESENT (unlike Peters)
-Most cases unilateral, nonprogressive, sporadic

Front 250

CONGENITAL: Sclerocornea
-What is the most common associated ocular abnormality?
-What % of cases are bilateral?
-Inheritance, risk of progression?
-Clinical appearance?

Back 250

Sclerocornea
-80% of cases also have cornea plana (most common assoc abnormality)
-90% bilateral
-Clinical appearance: ill-defined limbus w/ superficial vessels crossing cornea (scleral, episcleral or conj vessels)
-Sporadic, nonprogressive, noninflammatory

Front 251

CONGENITAL: Keratectasia & congenital anterior staphyloma
-Pathogenesis? How to differentiate b/w the 2 conditions?
-Clinical appearance?
-DDx?

Back 251

Keratectasia & congenital anterior staphyloma
-Pathogenesis: intrauterine corneal perforation 2/2 infection/vitamin deficiency --> failure of neural crest cell migration
-Dermoid transformation of cornea to stratified squamous epithelium (spares lids, conj)
-Difference b/w the 2 conditions: Descemet's and endothelium absent in both conditions; posterior uveal lining present only in congenital anterior staphyloma
-DDx: unilateral Peters anomaly
-Clinical: opaque, bulging cornea b/w eyelids at birth; deep AC, unilateral, sporadic

Front 252

CONGENITAL: CHSD, CHED
-Clinical appearance of congenital hereditary stromal dystrophy?
-Clinical appearance of CHED? Pathophysiology?
-Difference b/w CHED1 & CHED2?

Back 252

Congenital dystrophies
-CHSD: rare, dominant; bilateral central K clouding WITHOUT tearing, photophobia, edema --> flaky, feathery appearance
-CHED: diffuse, blue-gray, ground glass cornea --> 2/2 degeneration of endothelial cells during/after 5th month GA
-CHED1: dominant, slowly progressive K edema; WITH tearing, photophobia WITHOUT nystagmus
-CHED2: autosomal recessive, nonprogressive, WITH nystagmus; cornea may be 2-3x thicker than normal; NO tearing, photophobia

Front 253

CONGENITAL: Intrauterine keratitis (syphylitic)
-What is a von Hippel internal corneal ulcer?
-When and how does syphylitic interstitial keratitis present?

Back 253

Intrauterine keratitis
-von Hippel internal corneal ulcer: posterior corneal defect, follows intrauterine corneal inflammation
-Syphilitic interstitial keratitis: untreated congenital syphilis, develops in 1st decade of life; rapidly progressive edema followed by abnormal deep stromal neovascularization adjacent to Descemet's (salmon patch) --> later, vessels regress ("ghost vessels")

Front 254

CONGENITAL: Congenital corneal keloid
-Histological appearance?
-Predisposing factor?
-Systemic associations?

Back 254

Congenital corneal keloid
-Histo: thick collagenase bundles, haphazard arrangement, focal myofibroblastic proliferation
-Occur following corneal trauma/perforation
-Bilateral
-Systemic associations: Lowe disease, ACL syndrome

Front 255

CONGENITAL: Congenital corneal anesthesia
-Clinical presentation?
-DDx?
-Differences b/w groups I, II & III?
-Mgmt?

Back 255

Congenital corneal anesthesia
-Clinical: bilateral painless corneal opacities, sterile epithelial ulcerations in infancy/childhood
-DDx: HSV keratitis, recurrent corneal erosions, dry eye
-Group I: isolated trigeminal anesthesia
-Group II: mesenchymal abnormalities (Goldenhar, Mobius, Riley-Day)
-Group III: focal brainstem signs w/o mesenchymal dysplasia
-Mgmt: systemic w/u, neuroradiology studies, lubrication, tarsorrhaphy, punctal occlusion, contact lens, amniotic membrane transplant

Front 256

CONGENITAL: Congenital glaucoma
-Definition?
-Clinical presentation?
-Haab striae appearance?
-Frequency of corneal edema?

Back 256

Congenital glaucoma
-Definition: dysplasia of anterior chamber angle without other ocular/systemic abnormalities
-Clinical: triad of epiphora, photophobia, blepharospasm; buphthalmos (K diameter >12 mm), K edema
-Haab striae: horizontal or concentric tears in Descemet's membrane
-K edema in 25% of affected infants at birth, 60% by six months

Front 257

CONGENITAL: Other congenital corneal problems
-Corneal signs of birth trauma?
-What is an arcus juvenilis?

Back 257

Other congenital corneal problems
-Birth trauma: vertical/oblique striae --> healing leaves hypertrophic ridge of Descemet's --> spontaneous edema can occur at any time in life
-Arcus juvenilis: lipid deposition in peripheral corneal stroma (sector of cornea); no associated serum lipid abnormalities

Front 258

DYSTROPHIES: Overview
-What are general characteristics of all dystrophies?
-Name all dystrophies by location affected within the cornea

Back 258

Dystrophies
-All are bilateral, +/- symmetric, mostly inherited
-Epithelial: ABMD, Lisch, Meesmann, Gelatinous drop-like dystrophy
-Bowman's: Reis-Bucklers, Thiel-Behnke
-Stromal: Lattice, Granular, Macular, Schnyder, Congenital stromal dystrophy, Fleck, Posterior amorphous, Central cloudy dystrophy of Francois, Pre-Descemet dystrophy
-Endothelial: Fuchs, posterior polymorphous, CHED1 & 2

Front 259

DYSTROPHIES: ABMD
-Genetics?
-Histology?
-Clinical?

Back 259

ABMD (aka Map-Dot-Fingerprint)
-Genetics: usually sporadic
-Histology: thickened epithelial BM, fibrillar material b/w epithelial BM and Bowman's, epithelial cells with microcysts
-Clinical: F>M, >50 yo, four types of lesions (fingerprint lines, map lines, dots/microcysts, blebs/cobblestones)

Front 260

DYSTROPHIES: ABMD
-Symptoms?
-Mgmt?
-How does anterior stromal puncture work?

Back 260

ABMD
-Symtpoms: 10% of pts have recurrent K erosions --> 50% of these have AMBD
-Mgmt: 5% NaCl, lubricants, epithelial debridement, patching, BCL
-If fails conservative mgmt, can try anterior stromal puncture
-Anterior stromal puncture: multiple small punctures to disturb Bowman's --> promotes tight adhesion, stimulates cornea to produce functional BM complexes

Front 261

DYSTROPHIES: Meesmann
-Histology?
-Clinical appearance?

Back 261

Meesmann
-Histology: epithelial cells contain granular substance ("peculiar substance") & microcysts containing PAS+ degenerated cell products
-Cells also exhibit frequent mitoses, basement membrane projects into basal epithelium
-Clinical: tiny, bubbble-like blebs in interpalpebral region --> mild irritation and dec VA (generally no tx required)

Front 262

DYSTROPHIES: Lisch
-Histology?
-Clinical appearance?
-Mgmt?

Back 262

Lisch
-Histology: epithelial cells show cytoplasmic vacuoles, +Ki67 staining (but no increased mitotic activity)
-Clinical: gray, band-shaped, feathery lesions in whorled pattern
-Retroillumination: intraepithelial, densely crowded microcysts in area of lesions
-Mgmt: pts pain free, may have mild dec VA

Front 263

DYSTROPHIES: Gelatinous droplike dystrophy
-Histology? Deposited material?
-Clinical manifestations?

Back 263

Gelatinous droplike dystrophy
-Histology: amyloid deposits in epith/subepithelium --> disrupted epithelial tight junctions --> increased permeability
-Clinical: 1st-2nd decade, can be mulberry lesions, band keratopathy-like or kumquat-like

Front 264

DYSTROPHIES: Reis-Bucklers
-Histology: stains used? how to distinguish b/w RBCD & TBCD?
-Clinical appearance? effect on vision?

Back 264

Reis-Bucklers
-Histology: Bowman's layer replaced by sheet-like connective tissue w/ granular Masson trichrome-red deposits (these stain + for TGFB1)
-RBCD vs TBCD: need electron microscopy to distinguish --> TBCD has curly fibers
-Clinical: confluent, irregular, geograhic opacities at Bowman's layer (mostly central); posterior cornea normal
-Vision loss secondary to anterior scarring, surface irregularity, anterior stromal edema

Front 265

DYSTROPHIES: Thiel-Behnke
-Pathognomonic histological findings?
-What do the curly fibers stain positively for?
-Name one clinical difference b/w RBCD & TBCD

Back 265

Thiel-Behnke corneal dystrophy
-Histology: Bowman's layer replaced by fibrocellular material in wavy "saw-toothed" pattern
-Curly fibers stain positive for TGFB1 protein keratoepithelin
-Clinical: decreased frequency and severity of erosions than with RBCD (otherwise clinically indistinguishable from RBCD)

Front 266

DYSTROPHIES: Stromal dystrophies
-Name 4 dystrophies associated w/ TGFB1
-Name 7 non-TGFB1 stromal dystrophies

Back 266

Stromal dystrophies
-TGFB1: lattice, granular, avellino (lattice-granular), lattice dystrophy type 2 (aka Meretoja syndrome, gelsolin type dystrophy)
-NON-TGFB1: macular, Schnyder, congenital stromal, posterior amorphous, fleck, central cloudy dystrophy of Francois, pre-Descemet dystrophy

Front 267

DYSTROPHIES: Lattice corneal dystrophy
-Histology: material deposited? location of deposits? stains?
-Clinical appearance?

Back 267

Lattice corneal dystrophy
-Histology: anterior stromal, subepithelial AMYLOID deposits; eosinophilic layer b/w epithelial BM and Bowman's
-Stains: Congo red (orange-red), crystal violet (metachromatic), dichroism and birefringence
-Clinical: glass-like branching stromal lines (lattice lines) best seen w/ retroillumination --> start central and spread centrifugally, then centripetally
-Peripheral cornea remains clear

Front 268

DYSTROPHIES: Gelsolin type lattice corneal dystrophy (Meretoja syndrome)
-Why is this not a true corneal dystrophy?
-Does amyloid stain for AA or AP?
-Extraocular sits of amyloid deposition?
-Clinical presentation? (systemic findings, location of cornea involved, age of presentation)

Back 268

Gelsolin-type lattice corneal dystrophy (Meretoja syndrome)
-Systemic involvement, so not a true corneal dystrophy
-Amyloid does NOT stain for AA or AP
-Extraocular amyloid: arterial walls, peripheral nerves, glomeruli
-Clinical: 3-4th decade, lattice lines more peripheral (space center), facial mask, dry/lax skin, dermatochalasis, lagophthalmos, CN and peripheral nerve palsies, pendulous ears

Front 269

DYSTROPHIES: Granular dystrophy
-Histology: material deposited? stains? electron microscopy findings?
-Clinical appearance?

Back 269

Granular dystrophy
-Histology: HYALINE deposits, stain w/ Masson trichrome stain
-Electron microsocopy: rod-shaped bodies immersed in amorphous matrix
-Clinical: crumb-like opacities that may broaden into disks (clear stroma b/w opacities) --> progressive glare, photophobia, recurrent erosions and decreasing vision

Front 270

DYSTROPHIES: Granular-lattice dystrophy (Avellino)
-Histology: deposited material? stains?
-Clinical appearance?

Back 270

Granular-lattice dystrophy (Avellino)
-Histology: both HYALINE (Masson trichrome) and AMYLOID (Congo red) deposited
-Clinical: stellate-shaped, snowflake-like, icicle-like opacities in superficial & mid-stroma; may also see lattice lines in deeper stroma

Front 271

DYSTROPHIES: Macular corneal dystrophy
-Histology: deposited material? stains?
-How does macular dystrophy differ from other stromal dystrophies?
-Clinical appearance?

Back 271

Macular dystrophy
-Histology: glycosaminoglycan deposits --> stain w/ Alcian blue, colloidal iron
-Macular dystrophy is autosomal RECESSIVE, least common and earliest presenting of the 3 classic stromal dystrophies
-Clinical: focal gray-white superficial stromal opacities with indefinite edges; intervening stroma is cloudy; Descemet's/endothelium may be involved --> guttae

Front 272

DYSTROPHIES: Macular dystrophy
-What are differences between type I, IA and II macular dystrophies?
-What is a laboratory test that can help with diagnosis of macular dystrophy?

Back 272

Maculary dystrophy
-Type I (most prevalent): lack antigenic keratan sulfate in cornea, serum & cartilage
-Type IA: have AgKS in keratocytes but not in serum
-Type II: normal ratio of keratan sulfate: dermatan sulfate but total synthesis 30% below normal
-Lab test: ELISA --> measures total sulfated keratan sulfate

Front 273

DYSTROPHIES: Schnyder dystrophy
-What is the pathophysiology? Stains?
-What is the clinical course (4 stages)?
-How is vision limited?
-Mgmt?

Back 273

Schnyder corneal dystrophy
-Pathophysiology: local disorder of corneal lipid metabolism --> accumulation of lipid & dissolved cholesterol in epithelium, Bowman's, stroma
-Oil red O stain highlights opacities (stains phospholipids red)
-Clinical: 1) central K opacification; 2) dense K arcus lipoides; 3) midperipheral K opacification; 4) decreased K sensation
-Effect on vision: disproportionate reduction of photopic vision (maintain excellent scotopic vision) --> may need transplant
-Mgmt: check fasting lipid profiel to r/o hyperlipoproteinemia

Front 274

DYSTROPHIES: Congenital stromal dystrophy
-Histology?
-Clinical appearance?

Back 274

Congenital stromal dystrophy
-Histology: regular separation of stromal lamellae; abnormal lamellar layers (thin filaments in electron-dense ground substance) separate the nromal layers
-Normal keratocytes & endothelium
-Clinical: congenital, diffuse, bilateral corneal thickening and clouding with flakelike whitish opacities throughout stroma --> mod to severe vision loss

Front 275

DYSTROPHIES: Fleck dystrophy
-Histology: abnormal substances? stains?
-Clinical appearance? Which K layers are NOT involved?

Back 275

Fleck dystrophy
-Histology: keratocytes containing excess GAG (colloidal iron, Alcian blue) & lipids (Sudan black, Oil red O)
-Clinical: discrete dandruff-like gray-white opacities throughout stroma
-Only stroma involved (epithelium, Bowman's, Descemet's, endothelium normal)
-Nonprogressive
-May have decreased K sensation

Front 276

DYSTROPHIES: Posterior amorphous corneal dystrophy
-Histology?
-Clinical appearance? Associated corneal/ocular abnormalities?

Back 276

Posterior amorphous dystrophy
-Histology: focal endothelial cell attenuation, irregular stromal architecture anterior to Descemet's, disorganized posterior stromal lamellae
-Clinical: diffuse, sheet-like posterior corneal opacity
-Assoc with flat cornea (<41D), thin (<380 um), hyperopia (may also have other ocular abnormalities but NO associated glaucoma)

Front 277

DYSTROPHY: Central cloudy dystrophy of Francois
-Clinical appearance?

Back 277

Central cloudy dystrophy of Francois
-Clinical: multiple nebulous, polygonal, gray areas separated by crack-like intervening clear zones (most dense centrally and posterioly)
-Other corneal layers are normal

Front 278

DYSTROPHIES: Pre-Descemet corneal dystrophy
-Histology?
-Clinical appearance?

Back 278

Pre-Descemet corneal dystrophy
-Histology: large keratocytes containing vacuoles and inclusions w/ lipidlike material
-Clinical: focal, gray-white opacities in deep stroma anterior to Descemet's
-Remainder of cornea WNL and vision unaffected

Front 279

DYSTROPHIES: Fuchs dystrophy
-Histological appearance of endothelium and Descemet's?
-Presumed pathophysiology?
-Clinical appearance?

Back 279

Fuchs' dystrophy
-Histology: endothelial polymegathism, pleomorphism (larger, more polymorphic) & thickened Descemet's (secondary to increased collagen production due to endothelial stress)
-Pathophysiology: decreased Na/K-ATPase sites or function in endothelium
-Clinical: central (later, also peripheral) guttae, thickened Descemet's --> stromal edema --> CCT may approach 1 mm --> epithelial microcystic edema --> bullae (may rupture)

Front 280

DYSTROPHIES: Fuchs dystrophy
-Symptoms?
-Mgmt?
-Diagnosis and monitoring of disease?

Back 280

Fuchs dystrophy
-Symptoms: edema --> decreased vision, pain (2/2 ruptured bullae); sx worse upon awakening due to decreased surface evaporation during sleep
-Presents in 5th to 6th decade
-Mgmt: 5% NaCl gtts/ointment, lower IOP, BCL (for bullae), endothelial keratoplasty vs PK
-Specular microscopy: endothelial cell count < 1000
-Pachometry: indicator of endothelial function; if >640, may decompensate w/ intraocular surgery

Front 281

DYSTROPHIES: Posterior polymorphous corneal dystrophy (PPMD)
-What is the most distinctive histological finding?
-Clinical appearance? Associated ocular abnormalities?
-Mgmt?

Back 281

Posterior polymorphouse corneal dystrophy (PPMD)
-Histology: abnormal, multilayered endothelial cells --> look/behave like epithelial cells
-Cells have +keratin stain, microvilli, rapid growth, proliferative tendencies, intercellular desmosomes
-Clinical: may see isolated grouped vesicles, geographic-shaped discrete gray lesions, broad bands w/ scalloped edges
-Associated ocular abnormalities: stromal edema, corectopia, iridocorneal adhesions, glaucoma
-Mgmt: most pts asymptomatic, may need anterior stromal puncture for localized swelling

Front 282

DYSTROPHIES: CHED1
-Histology?
-Clinical findings?

Back 282

CHED1
-Histology: sparse, atrophic endothelial cells w/ diffuse thickening/lamination of Descemet's
-Endothelium may be replaced by keratinized stratified squamous epithelium
-Stroma is thickened
-Clinical: diffuse corneal clouding & thickening (progressive); blurry VA, photophobia, tearing worse in AM

Front 283

DYSTROPHIES: CHED2
-Which is more severe: CHED1 or CHED2?
-What are some differences b/w CHED1 & 2?

Back 283

CHED2
-CHED2 is more severe than CHED1
-Differences b/w CHED2 & 1: CHED2 has massive K thickening (may be 2-3x normal), rare subepithelial band keratopathy & IOP elevation, +nystagmus, minimal tearing/photophobia

Front 284

DYSTROPHIES: Keratoconus
-Pathophysiology?
-Environmental risk factors?
-Histology?

Back 284

Keratoconus
-Pathophysiology: central or paracentral cornea undergoes progressive thinning, bulging
-Environmental risk factors: eye rubbing, inflammation, oxidative stress
-Histology: fragmented Bowman's, thinning of stroma & epithelium, folds/breaks in Descemet's, variable scarring

Front 285

DYSTROPHIES: Keratoconus
-Clinical appearance?
-What finding is considered the minimal manifestation of keratoconus?
-What is Rizzutti sign?
-What is Munson sign?

Back 285

Keratoconus
-Clinical: bilateral (may be asymmetric) apical thinning of central cornea --> progresses during adolescent years into mid 20s/30s --> may have extreme irregular astigmatism
-Minimal manifestation of keratoconus: high astigmatism
-Rizzutti sign: conical reflection on nasal cornea when penlight shone from temporal side
-Munson sign: protrusion of lower eyelid upon downgaze

Front 286

DYSTROPHIES: Keratoconus
-What is a Fleischer ring?
-What is a Vogt line?
-What is acute hydrops?
-What systemic conditions are associated w/ increased prevalence of keratoconus?

Back 286

Keratoconus
-Fleischer ring: iron deposits in epithelium at base of cone (see w/ cobalt blue filter)
-Vogt line: "stress lines" (fine reticular striations) in posterior strmoa
-Acute hydrops: spontaneous rupture in Descemet's --> sudden corneal edema
-Systemic associations: Down's, Marfan's, floppy eyelid, Leber congenital optic neuropathy, MV prolapse

Front 287

DYSTROPHIES: Keratoconus
-What does Placido-disk based topography typically show? What does pachometry mapping typically show?
-Conservative mgmt strategies?
-Criteria for PK?

Back 287

Keratoconus
-Placido disk-based topography: shows inferior steepening
-Pachometry mapping: shows thinnest area is in paracentral area (visual axis), not at the steepest point
-Conservative mgmt: glasses, RGP CL
-Indications for PK: CL intolerace or failure to correct vision, unstable CL fit, progressive thinning to periphery

Front 288

DYSTROPHIES: Keratoconus
-What is an alternative procedure to PK for surgical mgmt?
-What surgery is contraindicated in the presence of keratoconus?
-Mgmt of hydrops?

Back 288

Keratoconus
-DALK (deep anterior lamellar keratoplasty): does not disrupt host endothelium --> decreased rejection
-Refractive procedures are contraindicated w/ keratoconusp ts
-Hydrops: topical hypertonic agents, patching, bandage CL
**Hydrops is NOT an indication for immediate surgery**

Front 289

DYSTROPHIES: Pellucid marginal degeneration
-Definition?
-How to distinguish b/w keratoconus and pellucid marginal degeneration?
-Main cause of decreased vision?
-Mgmt?

Back 289

Pellucid marginal degeneration
-inferior, peripheral corneal thinning in absence of inflammation
-Pellucid vs keratoconus: keratoconus shows protrusion at point of maximal thinning but pellucid shows protrusion ABOVE point of maximal thinning
-High irregular astigmatism --> decreased vision
-Mgmt: more difficult to manage surgically and w/ CL (poor CL fit, need for large grafts closer to limbus due to location of thinning --> increased risk of rejection, technically difficult surgery)

Front 290

DYSTROPHIES: Keratoglobus
-How is keratoglobus different from pellucid or keratoconus?
-What is the strongest systemic association?
-Histology?
-Clinical findings?
-Mgmt? Prognosis for PK?

Back 290

Keratoglobus
-Differs from pellucid/keratoconus in that it is present at birth
-Strongest systemic association: blue sclera, Ehlers Danlos type VI
-Histology: absent/fragmented Bowman's, thinned but organized stroma, thin Descemet's
-Clinical: deep AC, steep curvature (as high as 50-60 D), generalized thinning esp in midperiphery, increased K diameter
-Fleisher rings usually NOT present
-K may perforate w/ minimal trauma
-Mgmt: scleral CL; poor prognosis s/p PK

Front 291

DYSTROPHIES: Overview
-Which corneal dystrophies are autosomal recessive?
-Which dystrophies have an unknown genetic pattern?
-Which dystrophy is X-linked dominant?
-Which are mostly spontaneous?

Back 291

Genetics of dystrophies
-Autosomal recessive: macular, gelatinous droplike, CHED2
-Unknown genetics: central cloudy dystrophy of Francois
-X-linked dominant: Lisch epithelial corneal dystrophy
-Spontaneous: AMBD, Fuchs, Pre-Descemet dystrophy

Front 292

METABOLIC: Mucopolysaccharidoses
-What is the missing enzyme in this group of disorders?
-What are the accumulated metabolites?
-Inheritance?
-Clinical appearance?

Back 292

Mucopolysaccharidoses
-Deficient enzymes: lysosomal acid hydrolases (catabolize GAGs) --> lysosomal storage disease
-Accumulated metabolites: heparan/dermatan/keratan sulfate
-Inheritance: all are AR (except Hunter's - X-linked recessive)
-Clinical: variable degrees of corneal clouding, retinopathy, optic neuropathy (Hunter's rarely assoc w/ K clouding)

Front 293

METABOLIC: Mucopolysaccharidoses
-Histologic appearance?
-What are gargoyle cells?
-Prognosis for PK?

Back 293

Mucopolysaccharidoses
-Histology: fibrillogranular accumulation in vacuoles of epithelial, subepithelial cells and keratocytes (clouding is proportional to GAG accumulation)
-Gargoyle cells: histiocytes laden w/ abnormal storage material
-Prognosis: may recur again in grafted cornea if PK performed

Front 294

METABOLIC: Diabetes mellitus
-Most common corneal manifestations?
-Pathogenesis?

Back 294

Diabetic corneal disease
-Most common corneal manifestations: PEE, epithelial irregularity (resembles EMBD), dec K sensation, prolonged epithelial healing, Descemet's folds, Waite-Beetham lines
-Pathogenesis: ultrastructural basement membrane abnormalities --> abnormal epithelial -stromal adhesion

Front 295

METABOLIC: Hyperlipoproteinemia
-What are the 2 ocular hallmarks?
-Pathogenesis?
-Labs?

Back 295

Hyperlipoproteinemia
-2 ocular hallmarks: xanthelasma & corneal arcus (asymmetric arcus in pt < 40 yo --> suggests lipid abnormality)
-Pathogenesis: extracellular deposits of cholesterol, phospholipids, triglycerides
-Labs: fasting, EtOH-restricted lipid profile

Front 296

METABOLIC: Hypolipoproteinemias
-Which 3 disorders in this category cause corneal disease?
-Inheritance?
-Clinical appearance of each?
-Lab finding?

Back 296

Hypolipoproteinemias
-3 disorders causing K disease: fish eye, Tangier, LCAT deficiency
-Inheritance: autosomal recessive
-Clinical: 1) LCAT: peripheral arcus, nebular stromal haze; 2) fish eye: minute gray-white-yellow dots causing K clouding; 3) Tangier: large orange tonsils, very low HDL in plasma, diffuse K clouding but no arcus
-Labs: low HDL levels

Front 297

METABOLIC: Sphingolipidoses
-Name 3 diseases in this category that involve the cornea
-Inheritance?
-Pathphysiology
-Clinical appearance?
-Lab eval of Fabry disease?

Back 297

Spingolipidoses
-3 disease involving cornea: Fabry disease (X-linked recessive), generalized gangliosidosis (AR), multiple sulfatase deficiency (AR)
-Pathophysiology: alpha-galatosidase A deficiency (Fabry), beta-galactosidase (generalized gangliosidosis)
-Clinical: verticillata (whorl-like lines) in basal epithelium; spoke-like cataracts, periorbital edema, retinal/optic n disease
-Labs: Fabry disease - decreased level of alpha-galatosidase A in plasma/urine

Front 298

METABOLIC: Mucolipidoses
-Pathogenesis?
-Inheritance?
-Clinical presentation?

Back 298

Mucolipidoses
-Pathogenesis: defects in lysosomal acid hydrolases --> abnl carbohydrate & lipid metabolism --> deposition of mucopolysaccharides & sphingolipids in tissue
-Autosomal recessive
-Clinical: corneal clouding, progressive (poor success w/ PK)

Front 299

METABOLISM: Bietti crystalline corneal dystrophy
-Pathogenesis?
-Clinical appearance?
-Testing?

Back 299

Bietti crystalline corneal dystrophy
-Pathogenesis: autosomal recessive defect in lipid metabolism --> crystalline deposits, but no abnormal accumulation of cholesterol --> peripheral K opacities, tapetoretinal dystrophy
-Clinical: sparkling spots at limbus, superficial stroma, subepithelium (75% of pts); retinal crystals, RPE atrophy (may look like RP)
-Testing: ERG decreased in advanced cases

Front 300

METABOLIC: Cystinosis
-Pathogenesis?
-Difference b/w adult and infantile/nephropathic forms?
-Corneal appearance?
-Mgmt?

Back 300

Cystinosis
-Pathogenesis: autosomal recessive defect in cystine transport --> accumulation within lysosomes
-Infantile (nephropathic): dwarfism, prog renal dystunction
-Adult form: normal life expectancy
-Corneal findings: deposition of fine, polychromatic cystine crystals in conj, stroma (densest peripherally), other parts of eye incl TM --> no effect on VA
-Mgmt: topical cysteamine drops --> dec density of deposits, dec K pain (fewer erosions)

Front 301

METABOLIC: Tyrosinemia
-Pathogenesis?
-Systemic findings?
-Ocular findings?
-Mgmt?

Back 301

Tyrosinemia
-Pathogenesis: defective tyrosine aminotransferase enzyme --> excess tyrosine in blood/urine
-Systemic findings: MR, hyperkeratotic lesions of palms, soles, elbows
-Ocular findings: photophobia, tearing, injection, tarsal papillary hypertrophy, recurrent K pseudodendrites (poor staining)
-Consider this in the DDx of recurrent HSV keratitis in a child
-Mgmt: restrict dietary tyrosine, phenylalanine

Front 302

METABOLIC: Alkaptonuria
-Pathogenesis?
-Clinical findings (corneal, systemic)?
-Labs?

Back 302

Alkaptonuria
-Pathogenesis: deficiency of enzyme for degradation of tyrosine & phenylalanine --> homogentisic acid (metabolic product) accumulates --> alkapton polymer (pigmented) deposits in connective tissue
-Clinical: K homogentisic acid lesions, arthropathy, renal calculi, pigmentation of cartilage
-Labs: urine turns dark upon standing, testing shows homogentisic acid oxidase deficiency

Front 303

METABOLIC: Amyloidosis
-What is amyloid?
-5 staining characteristics?
-What is the composition of the folllowing types of amyloid: AL, AA, SAA, AF

Back 303

Amyloidosis
-Amyloid: eosinophilic hyaline material
-Staining: 1) Red w/ Congo red dye; 2) dichroism, birefringence; 3) metachromasia w/ crystal violet; 4) UV fluorescence w/ thioflavin T; 5) filamentous appearance on electron microscopy
-AL = amyloid composed if immunoglobulin
-AA = nonimmunoglobulin amyloid
-SAA = serum-related protein of AA
-AF = noncollagenous protein, can be deposited in eye

Front 304

METABOLIC: Amyloidosis
-What is the most common form of ocular amyloidosis?
-What is the most common form of amyloidosis in the cornea?
-Name examples of primary localized amyloidosis
-What are presenting features of primary systemic amyloidosis in the eye?
-When does secondary localized amyloidosis occur in the eye?
-What are examples of secondary systemic amyloidosis? What type of amyloid protien is deposited? How is the cornea involved?

Back 304

Amyloidosis:
-Most common ocular amyloidosis = primary localized
-Most common cornea-involving amyloidosis: secondary localized
-Primary localized: gelatinous droplike dystrophy, lattice dystrophy, conj amyloid plaques
-Primary systemic amyloidosis: waxy, ecchymotic eyelid papules, vitreous opacities, pupil light:near dissoc (see Meretoja syndrome - lattice dystrophy type 2)
-Secondary localized amyloidosis: occurs in eyes w/ longstanding chronic inflammation
-Secondary systemic amyloidosis: cornea NOT involved; AA deposits in pts w/ RA, leprosy, Mediterranean fever, bronchiectasis

Front 305

METABOLIC: Immunoglobulin synthesis disorders
-Name 4 disorders in this category
-What are M proteins? Bence Jones proteins?
-Name 2 types of corneal findings?

Back 305

Immunoglobulin synthesis disorders
-Multiple myeloma
-Waldenstrom macroglobulinemia (IgM production --> hyperviscosity, needle-like crystals in stroma)
-Benign monoclonal gammopathy
-Cryoglobulinemia (proteins that precipitate on exposure to cold --> hyperviscosity, limbal masses, crystalline corneal deposits)
-M protein = overproduction of both light & heavy chains
-Bence Jones = overproduction of light chains only
-Corneal findings: crystalline deposition in all layers of cornea/conjunctiva; copper deposition in cornea

Front 306

METABOLIC: Ehlers-Danlos
-Systemic features?
-Type VI: corneal manifestations? inheritance?
-Type I: corneal manifestations?
-Laboratory testing?
-What type of collagen is defective in this disease?

Back 306

Ehlers-Danlos
-Systemic features: hyperextensibility of joints/skin, easy bruising, "cigarette paper" scars
-Type VI: autosomal recessive; brittle cornea (easily ruptured), keratoconus, keratoglobus
-Type I: keratoconus
-Lab: deficiency of hydroxylysine
-Pathogenesis: defects in type I & III collagen synthesis

Front 307

METABOLIC: Marfan syndrome
-Which organ systems are involved?
-Inheritance?
-Pathogenesis: which gene affected? where is this gene product found in the eye?
-Ocular manifestations?

Back 307

Marfan syndrome
-Affects eye, heart (aortic root aneurysms), skeletal system (arachnodactyly, pectus excavatum, kyphoscoliosis)
-Autosomal dominant
-Pathogenesis: fibrillin gene defect (fibrillin found in corneal BM, lens zonular fibers, sclera, lens capsule)
-Eye findings: blue sclera, lens subluxation, cornea plana (35 D) in 20% of pts (rarely keratoconus, keratoglobus)

Front 308

METABOLIC: Location of various corneal deposits
-Name 4 types of pigmented deposits that are found in superficial layers?
-Name 3 pigm deposits in stromal layers?
-Name 6 deposits in endothelial/deep stromal layers?

Back 308

Location of pigmented corneal deposits
-Superficial: calcium (band keratopathy), verticillata, iron lines, adrenochrome, spheroidal degeneration
-Stromal: phenothiazines, bloodstaining, siderosis (iron)
-Endothelial: mercury, silver, gold, copper (Wilson's, chalcosis), krukenberg spindle

Front 309

METABOLIC: Gout
-What causes gout/hyperuricemia?
-Causes?
-Ocular/corneal findings?
-Labs?
-Mgmt?

Back 309

Gout
-Disorder of purine metabolism --> increased uric acid --> crystals deposited into joints/kidney
-Causes: obesity, excessive EtOH, chem, myeloproliferative disesae, chemotx, Lesch-Nyhan syndrome
-Eye findings: orange-brown or white band keratopathy, acute inflammation of sclera, episclera, conj
-Labs: elevated uric acid (may be normal even in presence of keratopathy)
-Mgmt: manual removal of superficial deposits

Front 310

METABOLIC: Porphyria
-Pathogenesis?
-Which form is most commonly associated w/ eye disease?
-Ocular findings?
-Mgmt?

Back 310

Porphyria
-Pathogenesis: disorder of porphyrin production (pigments involved in heme synthesis) --> accumulated porphyrins in liver, circulation --> accumulation in skin/mucous membranes, iron overload
-Most common type affecting eye: porphyria cutanea tarda (sporadic or autosomal dominant)
-Ocular disease: interpalpebral injection, vesicles, necrosis, scarring (mimics bullous pemphigoid), necrotizing scleritis, exposure/thinning of cornea --> sun-exposed areas affected
-Urine turns dark upon standing
-Mgmt: avoid UV exposure, phlebotomy to reduce iron

Front 311

METABOLIC: Wilson's disease
-Sites of copper deposition?
-Inheritance?
-Systemic sx?
-What is the appearance & location of a Keyser-Fleischer ring?
-What type of cataract can form?
-Lab eval?
-Mgmt?

Back 311

Wilson's disease
-Copper deposited in liver, kidneys, brain & cornea (Descemet's)
-Autosomal recessive
-Systemic sx: muscle rigidity (Parkinsonism), dementia
-Keyser-Fleischer ring: golden brown/ruby red/green pigment ring in peripheral Descemet's --> begins superiorly, grows to meet deposits inferiorly (copper deposits)
-Sunflower cataracts
-Note: of all disorders resulting in Keyser-Fleischer ring, only Wilson's disease has decreased serum ceruloplasmin & neurologic sx
-Labs: dec serum ceruloplasmin, high nonceruloplasmin-bound copper, high urinary copper
-Mgmt: penicillamine --> Keyser-Fleischer ring disappears gradually w/ therapy (use to monitor tx)

Front 312

METABOLIC: Miscellaneous disorders
-What type of corneal findings are associated with hemochromatosis?
-What disorder is associated w/ enlarged corneal nerves?

Back 312

Miscellaneous disorders
-Hemochromatosis: NO corneal deposits or changes
-MEN 2B (medullary thyroid, pheochromocytoma, mucosal neuroma): assoc w/ enlarged corneal nerves
(note: may also see in MEN 2A)

Front 313

DEGENERATIONS: Pinguecula
-Predisposing factor?
-Location?
-Clinical appearance?
-Mgmt?

Back 313

Pinguecula
-UV-light exposed areas (interpalpebral bulbar conj)
-Location: nasal>temporal interpalpebral bulbar conj
-Appearance: yellow-white, amorphous, subepithelial deposits
-Mgmt: lubricant (mainstay of tx), excision if cause irritation, topical steroids

Front 314

DEGNERATIONS: Pterygium
-Predisposing factor?
-Histology?
-Clinical appearance?
-What is a Stocker line?
-Criteria for excision?

Back 314

Pterygium
-UV light =strong correlation w/ development (increased prevalence in areas close to equator)
-Histology: elastotic degeration of stromal collagen, subepithelial fibrovacular tissue
-Stocker line: pigmented iron line at central, anterior edge of pterygium on cornea
-Excision: if causes irritation, approaches visual axis or causes irregular astigmatism --> vision loss

Front 315

DEGENERATIONS: Concretions
-Clinical appearance?
-What is conjunctival lithiasis?

Back 315

Concretions
-Clinical: yellow-white deposits (epithelial inclusion cysts filled w/ epithelial & keratin debris)
-Usually secondary to chronic conjunctivitis in older pts
-Conjunctival lithiasis: concretions w/ secondary calcification

Front 316

DEGENERATIONS: Conjunctivochalasis
-Name some clinically significant consequences of conjunctivochalasis?
-Mgmt?

Back 316

Conjunctivochalasis
-Consequences: irritation from exposure of redundant conj, secondary tearing from lower punctal occlusion
-Mgmt: lubricants, anti-inflammatory meds, anti-histamines, nocturnal patching

Front 317

DEGENERATIONS: Aging changes
-Changes in shape/refractive index?
-Descemet's changes?
-Endothelial cell changes?

Back 317

Aging changes
-K becomes flatter, thinner, less transparent --> higher index of refraction
-Descemet's becomes thicker (3 mm birth --> 10 mm adult)
-Hassall-Henle bodies (peripheral guttae)
-Endothelial cell loss (100,000 cells lost during 1st 50 yrs of life) --> 4000 cells at birth to 2500-3000 cells with aging
-0.6% per year rate of endothelial cell loss

Front 318

DEGENERATIONS: Coats white ring
-Predisposing factor?
-Clinical appearance?
-Mgmt?

Back 318

Coats white ring
-Metallic foreign body = predisposing factor
-Clinical appearance: small (<1mm diameter) circle/oval area of discrete gray-white dots in superficial stroma
-Represents iron-containing remnants of metallic FB
-No treatment

Front 319

DEGENERATIONS: Spheroidal degeneration
-Clinical appearance?
-Are these composed of lipids?
-Difference b/w primary and secondary spheroidal degeneration?

Back 319

Spheroidal degeneration
-Clinical appearance: bilateral, interpalpebral, translucent, golden-brown, spheroidlike deposits in superficial stroma
-NOT composed of lipid (but look like oil droplets)
-Males > females
-Primary: bilateral, nasal & temporal cornea, may extend to conj
-Secondary: assoc with ocular injury/inflammation, aggregate near corneal scars/vascularized areas

Front 320

DEGENERATIONS: Iron deposition
Define the following...
-Ferry line
-Hudson-Stahli line
-Fleischer ring/line
-Stocker line

Back 320

Iron deposition
-Ferry line: epithelial iron line anterior to filtering bleb
-Hudson-Stahli line: epithelial iron line in aging cornea
-Fleischer ring/line: keratoconus, located at base of cone; assoc w/ epithelial irregularity
-Stocker line: epithelial iron line anterior to head of pterygium

Front 321

DEGENERATIONS: Dystrophy vs Degenerations
-How do they differ w/ regard to: location, symmetry, age at presentation, progression?

Back 321

Dystrophy:
-central cornea
-symmetric, bilateral
-early age (hereditary)
-slow progression
Degeneration
-peripheral cornea
-asymmetric
-assoc with aging
-progression may be slow or rapid

Front 322

DEGENERATIONS: White limbal girdle
-Type I appearance?
-Type II appearance?
-Histology?

Back 322

White limbal girdle
-Type I: narrow, concentric whitish band along limbus in interpalpebral area; lucid interval separates limbus & girdle; may have small clear areas (swiss cheese)
-Type II: small, fleck-like, needle-like deposits at nasal/temporal limbus w/ no clear interval b/w girdle & limbus
-Histology: elastotic degeneration

Front 323

DEGENERATIONS: Arcus
-Describe appearance & progression of arcus
-Frequency in older adults? By race?
-What is unilateral arcus associated with?

Back 323

Arcus
-Clinical appearance: peripheral stromal lipid deposition w/ clear interval b/w limbus & arcus; starts at superior & inferior poles --> progresses to entire circumference
-Higher frequency in blacks
-60% of pts 50-60 yo, 100% of pts >80yo
-Unilateral arcus: may be assoc w/ contralateral carotid artery disease or ocular hypotony

Front 324

DEGENERATIONS: Crocodile shagreen & cornea farinata
-Crocodile shagreen: location, appearance, DDx?
-Farinata: location, appearance, DDx, composition, visual consequence?

Back 324

Crocodile shagreen
-Bowman's layer, central cornea, mosaic of polygonal gray opacities separated by clear zones --> posterior variant looks like central cloudy dystrophy of Francois
-Farinata: deep stroma, dot & comma-shaped opacities composed of lipofuscin, may be variant of pre-Descemet dystrophy

Front 325

DEGENERATIONS: Polymorphic amyloid degeneration
-Clinical appearance?
-Vision affected?

Back 325

Polymorphic amyloid degeneration
-Clinical: stellate flecks or irreg filaments in stroma w/ clear intervening stroma --> resembles early lattice dystrophy
-Normal visual acuity

Front 326

DEGENERATIONS: Senile furrow degeneration
-location, appearance, mgmt?

Back 326

Senile furrow degeneration
-Corneal thinning within lucid interval of arcus (b/w arcus and limbus) --> NOT assoc with neovasc, perforation or inflammation; epithelium intact
-Aging pts
-No mgmt needed

Front 327

DEGENERATIONS: Terrien marginal degeneration
-Age group affected?
-Clinical appearance?
-Topography findings?
-DDx?
-Indications for surgery?

Back 327

Terrien marginal degeneration
-2nd-3rd decade
-Clinical: slowly progressive peripheral corneal thinning; starts superiorly, spreads circumferentially (rarely involves inferior limbus)
-Lipid deposits at leading edge of pannus, fine vascular pannus traverses area of thinning, intact epithelium
-Easily perforates w/ minor trauma!
-Topography: flattening of peripheral thinned cornea, steepening of corneal surface 90 from midpoint of thinned area --> high against-the-rule or oblique astigmatism
-DDx: Fuchs superficial marginal keratitis (inflammatory progressive thinning w/o epithelial ulceration)
-Indications for surgery: imminent perforation, vision-limiting astigmatism --> use patch grafts

Front 328

DEGENERATION: Salzmann nodular degeneration
-Predisposing factors?
-Epidemiology?
-Clinical appearance?
-Mgmt?

Back 328

Salzmann nodular degeneration
-Predispoing factors: idiopathic, old longstanding keratitis (trachoma, interstitial keratitis, phylenticulosis)
-Middle-aged to older females
-Clinical: gray/white/blue nodules in circular configuration in central/paracentral cornea; may have assoc pannus
-Mgmt: lubrication, superficial keratectomy (if dec vision 2/2 irreg astigmatism)

Front 329

DEGENERATIONS: Other postinflammatory degenerations
CORNEAL KELOID:
-Causative factors?
-Clinical appearance?
LIPID KERATOPATHY
-Clinical appearance?
-Mgmt?

Back 329

Corneal keloid
-Secondary to vigorous fibrotic response to perforation/injury
-Clinical: white, glistening (jelly-like) masses resembling dermoids
Lipid keratopathy
-Clinical: yellow/cream lipids deposited superficially & deep --> secondary epithelial ulceration (may be assoc w/ longstanding corneal inflammation)
-Mgmt: argon laser, VEGF may be helpful

Front 330

DEGENERATIONS: Calcific band keratopathy
-Deposited material?
-Location of deposits?
-6 main known causes?

Back 330

Calcific band keratopathy
-Calcium hydroxyapatite
-Bowman's layer
-6 known causes: chronic inflammation, hypercalcemia, hyperphosphatemia w/o hypercalcemia (renal failure), hereditary, chronic mercury exposure, silicone oil in aphakic eye

Front 331

DEGENERATIONS: Calcific band keratopathy
-Clinical appearance?
-Workup?
-Mgmt?
-Why should PTK not be used initially?

Back 331

Calcific band keratopathy
-Clinical: Lucid interval b/w limbus & lesion --> deposits may coalesce to form horizontal band of calcific plaques
-Workup: serum electrolytes, urinalysis -> r/o metabolic/renal disease
-Mgmt: EDTA chelation to remove Ca from Bowman's (remove overlying epithelium first) --> avoid damage to Bowman's
-PTK not advised as primary procedure b/c Ca ablates at different rate from stroma --> irregular surface may be produced

Front 332

DEGENERATIONS: ICE syndrome
-Pathogenesis?
-Chandler syndrome?
-Essential iris atrophy?
-Cogan-Reese (iris nevus)?
-Epidemiology?
-Mgmt?

Back 332

ICE syndrome
-Pathogenesis: abnormal clone of endothelial cells --> has characteristics of epithelial cells --> "endothelialization" of AC angle, iris surface 2/2 proliferation of abnl endothelial cells
-Chandler syndrome: corneal edema only (abnl cells confined to cornea) --> may see border b/w nl and abnl endothelium
-Essential iris atrophy: abnl endothelium involves AC angle (PAS formation --> glaucoma) & iris (atrophy, corectopia, polycoria)
-Cogan-Reese (iris nevus): multiple pigmented iris nodules caused by contracting endothelial membrane
-Mgmt: PK or DSAEK
-Epidemiology: unilateral, F>M, middle-aged

Front 333

DEGENERATIONS: Peripheral guttae
-What is another name for peripheral corneal guttae?
-Clinical appearance?
-When do they become pathologic?

Back 333

Peripheral guttae
-AKA Hassall-Henle bodies
-Clinical: normal aging change --> overproduction of endothelial basement membrane
-Pathologic when appear in central cornea (then called "corneal guttae")

Front 334

DEGENERATIONS: Scleral senile plaques
-Clinical appearance?
-Histology?

Back 334

Scleral senile plaques
-Clinical: ovoid/rectangular zones of grayish translucency
-Histology: focal calcified plaque surrounded by acellular collagen (noninflammatory)

Front 335

DEGENERATIONS: Drug depositions
-Verticillata: clinical appearance? most common causes? affect on vision?

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Drug depositions: corneal verticillata
-Clinical: whorl-like pattern of brown/gray deposits in inferior interpalpebral cornea, clockwise (vortex keratopathy)
-Etiology: AMIODARONE (most common), Fabry disease, hydroxychloroquine/chloroquine, indomethacin, phenothiazines
-No symptoms or change in vision --> if decreased vision, rule out drug-induced optic neuropathy

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DEGENERATIONS: Drug depositions
-Name one drug that causes epithelial microcysts
-What deposition may be seen with topical ciprofloxacin?

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Drug depositions
-Cytarabine: epithelial microcysts (toxic to epithelium)
-Cipro: chalky white precipitate (cipro crystals) within epithelial defect --> resolves when med d/c'd

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DEGENERATIONS: Drug depositions
-Clinical appearance of adrenochrome deposits?
-What deposits are seen with chlorpromazine?
-What compounds are most often associated w/ deep stromal/Descemet's deposits?

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Drug depositions
-Adrenochrome deposits: black/dark brown deposits in conj/cornea 2/2 epinephrine compounds (harmless)
-Chlorpromazine: brown opacities in posterior stroma, Descemet's & endothelium
-Deep stroma/Descemet's deposits: metallic compounds --> gold, silver

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TRAUMA: UV radiation
-Most common causes?
-Clinical presentation?

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UV Radiation
-Causes: sun lamps, arc welding, snow blindness (UV light reflected from snow), prolonged outdoor exposure
-Clinical: sx begin a few hours after exposure --> self limited

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TRAUMA: Ionizing radiation
-Causes?
-Which are worse: longer or shorter wavelengths?
-Hallmark of ionizing radiation injuries?
-Acute and chronic complications?
-Prognosis for PK?

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Ionizing radiation
-Causes: nuclear explosions, x-rays, radioisotopes
-Longer wavelengths penetrate less deeply (more intense reaction in superficial layers); shorter wavelengths penetrate to deeper tissues
-Hallmark of ionizing radiation injury: poor wound healing
-Acute: conj edema, chemosis, scarring, shrinkage, dec tear production, conj telangiectasias, PEE
-Chronic: dry eye, neurostrophic cornea, keratitis/neovascularization, poor wound healing
-Poor prognosis for PK given chronic ocular surface disease

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TRAUMA: Alkali burns
-Describe the 4 stages of the Hughes classification of ocular alkali burns

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Alkali burns: Hughes classification
-Grade I: epi defect, no limbal ischemia
-Grade II: epi defect + stromal haze + ischemia of <1/3 limbus
-Grade III: total epi defect + stromal haze + ischemia of 1/3-1/2 limbus
-Grade IV: opaque cornea + ischemia > 1/2 limbus

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TRAUMA: Alkali burns
-What is the general consensus re: treatment of chemical burns?
-What is the relationship b/w chemical burns & aqueous humor ascorbate levels?
-Why do steroids help decrease ocular damage?

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Alkali burns
-No general consensus re: optimal mgmt of chemical injuries!
-In animals, severe alkali burns assoc w/ dec aqueous ascorbate levels to < 1/3 normal (ascorbate thought to promote collagen synthesis in alkali-burned eyes)
-Steroids inhibit PMN function (PMNs are source of proteolytic enzymes that dissolve stromal collagen)

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TRAUMA: Toxic keratoconjunctivitis from medications
-Consequence of topical anesthetic use?
-What preservative in ocular solutions is thought to be very toxic to the surface?
-Name drugs that cause toxic keratoconjunctivitis
-Name drugs that cause toxic follicular conjunctivitis

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Toxic keratoconjunctivitis from medications
-Topical anesthetics: epithelial loss, stromal edema/infiltrates/opacities
-Most toxic preservative: benzalkonium chloride
-Toxic keratoconjunctivitis: aminoglycosides, trifluridine, mitomycin C, topical anesthetics, preservatives
-Toxic follicular conjunctivitis: miotics, alpha agonists, cycloplegics

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TRAUMA: Toxic keratoconjunctivitis secondary to meds
--Clinical presentation? (difference b/w follicular & papillary reactions due to meds)
-Mgmt?

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Toxic keratoconjunctivitis due to meds
-Papillary reaction: generalized injection, tarsal papillae, mucoid d/c, PEE (may see vortex keratopathy), peripheral K infiltrates & limbal stem cell deficiency (esp 2/2 mitomycin C)
-Follicular reaction: upper & lower palpebral follicles; bulbar follicles highly associated w/ toxic etiology
-Mgmt: d/c offending agent --> may take months to resolve
-Rule out pseudopemphigoid w/ biopsy

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TRAUMA: Vegetative matter
-Name 2 types of tropical trees known to cause acute keratoconjunctivitis
-What is a common houseplant assoc w/ keratoconjunctivitis
-What drugs should be avoided in treatment?

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Vegetative matter
-Trees: pencil tree, manchineel tree
-Houseplant: dieffenbachia (causes calcium oxalate crystal deposition in cornea)
-Avoid steroids --> promotes fungal infections (high risk w/ vegetative matter exposure)

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TRAUMA: Iridodialysis/cyclodialysis
-What is iridodialysis? Symptoms?
-What is cyclodialysis? Effect on IOP? Diagnosis and mgmt?

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Iridodialysis
-Traumatic separation of iris root from ciliary body
-If large --> polycoria, monocular diplopia
Cyclodialysis
-Separation of ciliary body from scleral spur --> formation of cleft
-Effect on IOP: increased uveoscleral outflow & aqueous hyposecretion --> chronic hypotony, macular edema
-Diagnosis: ultrasound to define extent/location
-Mgmt: closure w/ topical cycloplegics or laser/cryo/suture

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TRAUMA: Hyphema
-Mechanism of traumatic hyphema?
-What % of pts w/ rebleeds develop elevated IOP?
-Mechanism of corneal bloodstaining?
-Clinical appearance?
-Pattern of resolution?

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Hyphema
-Mechanism: blunt force to globe --> posterior displacement of lens/iris diaphragm, scleral expansion in equatorial zone --> disruption of major iris arterial circle, ciliary body arteries, recurrent choroid arteries/veins
-50% of pts w/ rebleeds develop elevated IOP
-Mechanism of bloodstaining: RBCs release hemoglobin --> penetrates into posterior stroma --> converted to hemosiderin within keratocytes --> keratocyte death
-Early bloodstaining: yellow changes in posterior stroma, decreased transparency
-Clears in centripetal pattern starting in periphery

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TRAUMA: Hyphema
-What are guidelines for surgical intervention for prevention of 1) optic atrophy; 2) corneal bloodstaining; 3) PAS; 4) sickle hemoglobinopathies?

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Hyphema
-Optic atrophy: IOP > 65 x 2 days or > 35 x 7 days
-Corneal bloodstaining: early bloodstaining on exam or IOP > 25 avg x 5 days
-PAS: Total hyphema x 5 days or any hyphema fails to resolve to < 50% volume by 8 days
-Sickle disease: IOP > 25 avg x 24 hrs, repeated transient IOP elevations to > 30 x 2-4 days

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TRAUMA: Sickle cell complications of hyphema
-Causes of glaucoma, optic neuropathy?
-What drugs should be avoided?

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Sickle cell complications of hyphema
-Glaucoma 2/2 decreased outflow of sickled cells through trabecular meshwork
-Optic neuropathy: decreased blood flow to optic nerve w/ sickling --> nerve is at higher risk for damage even w/ modest IOP elevation in SCD pts
-Avoid carbonic anhydrase inhibitors, osmotic agents --> reduce pH, cause hemoconcentration --> more sickling