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Ocular Disease part 2 > Corneal Dystrophy > Flashcards

Corneal Dystrophy Flashcards

1
Q

what is a dystrophy?

A

developmental, symmetric and frequently hereditary changes occurring in original corneal tissue because of faulty nutrition, unrelated to other systemic or local diseases

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2
Q

are corneal dystrophies typically autosomal dominant or recessive?

A

dominant - recessive are usually more serious (morbid)

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3
Q

what are some standard characteristics of corneal dystrophies?

A

autosomal dominant, onset by age 20, bilateral, slowly progressive changes, no systemic disease association, no primary ocular disease history, centrally located, primary involvement of single corneal layer

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4
Q

which dystrophy is an exception to the characteristic of being discovered by age 20?

A

Fuch’s dystrophy (age-related degenerative process)

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5
Q

what are 3 anterior corneal dystrophies?

A

cogan microcystic, meesman’s, ries-buckler’s

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6
Q

what are 5 stromal corneal dystrophies?

A

lattice, granular/avellino, macular, schnyder’s, fleck

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7
Q

what are 2 posterior corneal dystrophies?

A

Fuch’s and posterior polymorphous

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8
Q

what is cogan microcystic (EBMD, ABMD) or “map-dot-fingerprint”?

A

epithelial basement membrane dystrophy - most common corneal dystrophy asymptomatic to irritation upon waking, transient VA, photophobia, glare, pain (RCE)

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9
Q

who typically gets cogan microcystic dystrophy?

A

non-hereditary = against the rule

40-70 y/o

male = females

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10
Q

what are the 4 different presentations for cogan microcystic dystrophy?

A

dots, microcysts, fingerprints (least common) and maps (most common)

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11
Q

what causes cogan microcystic dystrophy?

A

abnormal basement membrane (lacking hemidesmosomes) and abnormal attachment of BM to bowmans

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12
Q

what is the dot pathology for EBDM?

A

microcyst filled with cytoplasmic debris - trapped by extra BM material (BM becomes thickened in places and cysts become enveloped)

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13
Q

what is the treatment for acute EBMD?

A

abrasion protocol = bandage CL, pressure patch, antibiotic, NSAID, cycloplegic, doxycycline lubricants

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14
Q

what is the treatment for chronic EBMD?

A

abrasion protocol = bandage CL, pressure patch, antibiotic, NSAID, cycloplegic, doxycycline, lubricatns hypertonic = 5% NaCl gtt, ung (Muro 128)

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15
Q

what is Meesman’s dystrophy?

A

rare - intraepithelial cysts (6 months) = abnormal basal cells and maturation to squamous, thick BM

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16
Q

what happens if the cysts in Meesman’s dystrophy rupture?

A

pain, tearing, photophobia vision minimally affected

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17
Q

what is Reis-Buckler’s dystrophy?

A

rare - painful RCE age 5-20 with decreasing episodes by 30

bowman’s/anterior stroma replaced by fibrocellular tissue, irregular corneal surface, scarring, decrease/fluctuation in VA

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18
Q

what is lattice dystrophy (type 1)?

A

age 2-10, VA reduction, RCE common anterior stromal “inter-lacing” filamentous lesions, white spots, central haze (amyloid deposits)

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19
Q

what is lattice type 2 dystrophy?

A

similar phenotype but not genotype to type 1 central cornea sparing and associated with other conditions (VN7 palsy, peripheral neuropathy, amyloidosis)

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20
Q

what is granular dystrophy?

A

*earliest seen dystrophy - in first decade VA reduction > 40 y/o centrally discrete focal white deposits all stromal depths “cornflakes”, area between lesions is clear (deposits are hyaline-like material) RCE is rare

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21
Q

what is Avellino (granular type 2) dystrophy?

A

unique to area of italy - features are similar to lattice and granular

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22
Q

what is macular dystrophy?

A

most severe and least common dystrophy - VA reduction starts in teens, photophobia, RCE less than lattice exception to “dystrophy rule” - AR extends to periphery

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23
Q

what causes macular dystrophy?

A

(autosomal recessive disease) excess glycosaminoglycans - abnormal keratocytes storage mucopolysaccharide

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24
Q

what does macular dystrophy look like?

A

diffuse, “ground-glass” haze lesions, corneal haze between lesions, gray/white or milky/white opacities throughout stroma and limbus to limbus

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25
Q

what is schnyder’s central crystalline?

A

exception to “dystrophy rule” - associated with systemic hypercholesterolemia (cardiovascular risks)

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26
Q

what are the symptoms of schnyder’s central crystalline dystrophy?

A

mild VA reduction (20/40), no RCE, central crystals from annulus during 1st to 2nd decades, dense arcus ring

27
Q

what is fleck dystrophy?

A

gray/white opacities, odd shaped, incidental findings at all levels of the stroma

28
Q

what is posterior polymorphous dystrophy (PPD)?

A

asymptomatic, rare reduction in VA, polymorphous opacities at level of Descemet’s, maybe corneal edema

29
Q

which dystrophy has a glaucoma risk?

A

posterior polymorphous dystrophy (PPD) - 15% may develop increased IOP (abnormal endothelium grows across TM onto iris = anterior synechiae)

30
Q

why is Fuch’s dystrophy an exception to the “dystrophy rule”?

A

exceptions to rule = higher in females (postmenopausal), age-related (>40), may extend to periphery and appears multi-layered

31
Q

what causes Fuch’s dystrophy?

A

progressive corneal disease in which corneal edema results from primary metabolic incompetence of endothelial cells - endothelial barrier and pump fail

32
Q

what are the symptoms for Fuch’s dystrophy?

A

VA reduction in advancing cases and RCE

33
Q

what are some objective clinical features in Fuch’s dystrophy?

A

Guttata, stroma edema, and epithelial edema

34
Q

how can you evaluate the corneal thickness in Fuch’s?

A

stromal and epithelial edema occur with breakdown of the endothelial barrier and pump = use pachymetry (stromal edema)

35
Q

how do you know if your patient has guttata or fuch’s?

A

some guttata = normal endothelial aging

slowly progressive fuchs = increasing number of guttata with corneal edema

36
Q

what does stromal edema look like?

A

hazy in appearance, folds in Descemet’s and bullous keratopathy

37
Q

what is the treatment for Fuch’s dystrophy?

A

lubricants, hypertonics (5% gtt or ung) NaCl, RCE/abrasion protocol, keratoplasty

38
Q

what is congenital hereditary endothelial dystrophy?

A

not typical dystrophy = autosomal recessive, present at birth or 1st decade, no guttata, diffuse stromal edema and poor results with keratoplasty

39
Q

when are the onset of deposits, onset of symptoms, and reduced VA with granular dystrophies?

A

deposits = 1st decade

symptoms = 3rd decade

asymptomatic VA = 4th or 5th decade

40
Q

when are the onset of deposits, onset of symptoms, and reduced VA with macular dystrophies?

A

deposits = 1st decade

symptoms = 1st decade

VA = 1st or 2nd decade

41
Q

when are the onset of deposits, onset of symptoms, and reduced VA with lattice dystrophies?

A

deposits = 1st decade

symptoms = 2nd decade

VA = 2nd or 3rd decade

42
Q

what type of hereditary pattern are granular, macular and lattice dystrophies?

A

granular = AD

macular = AR

lattice = AD

43
Q

are erosions common in granular, macular or lattice dystrophies?

A

granular = uncommon

macular = very common

lattice = very common

44
Q

what type of opacities are present in granular dystrophy?

A

discrete sharp boarders, intervening stroma is clear early but becomes hazy, not to limbus

45
Q

what type of opacities are present in macular dystrophy?

A

indistinct margins, hazy intervening stroma, extends to limbus, endothelium affected

46
Q

what type of opacities are present in lattice dystrophy?

A

early refractive lines and dots, supepithelial spots, diffuse central haze, limbal zone clear in mild cases

47
Q

what is the corneal thickness in granular, macular and lattice dystrophies?

A

granular = normal macular = thinned lattice = normal

48
Q

what is the deposit type in granular, macular and lattice dystrophies?

A

granular = hyaline macular = glycosaminglycan lattice = amyloid

49
Q

what are the clinical features in granular, macular and lattice dystrophies?

A

granular = clear limbal zone macular = opacities reach limbus, cornea thinned lattice = lattice lines

50
Q

what type of dystrophy is this?

A

EBMD - dots/maps/microcysts

51
Q

what type of dystrophy is this?

A

Schnyder’s central crystalline dystrophy

52
Q

what is wrong with this cornea?

A

stromal edema

53
Q

what type of EBMD is seen here?

A

fingerprints

54
Q

what type of EBMD is seen here?

A

negative staining map

55
Q

what dystrophy is in this photo?

A

filamentous lattice lesions

56
Q

what is seen in this photo and which dystrophy gets it?

A

Guttata - Fuch’s

57
Q

what type of dystrophy is seen here?

A

Meesman’s dystrophy

58
Q

what type of dystrophy is seen in this photo?

A

Meesman’s

59
Q

what type of dystrophy is seen here?

A

posterior polymorpheous (PPD)

60
Q

what is seen in this photo?

A

recurrent epithelial errosion

61
Q

what type of dystrophy is seen here?

A

Reis-Bucklers dystrophy

62
Q

what dystrophy is seen here?

A

macular dystrophy

63
Q

what type of dystrophy is this?

A

granular dystrophy

64
Q

what type of dystrophy is this?

A

lattice type 1

Ocular Disease part 2 (7 decks)

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