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OC DISEASE > deck > Flashcards

deck Flashcards

1
Q

List the major cell types in the epi:

A

keratocytes:

  • basal cell layer
  • prickle cell or squamous
  • granular (keratin)
  • stratified squamous (lack nuclei)

clear cells:
-melanocytes

  • merkel cells
  • Langerhans cells
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2
Q

what is this? what are some associated characteristics? characteristic histo finding? what cell layer is being affected

A

seborrheic keratosis: raspberry waxy stuck on

pseudo horn cysts: accum of keratin (must be diff from pearls of squam cell carc.)

  • proliferation of basal cells with various amounts of acanthosis (proliferation of squamous cells) -> usually broad-based and sessile
    • greasy-looking lesions, “brown wax dripped on a background”
    • varying amount of pigmentation
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3
Q

T/F: seborrheic keratosis is actinic

A

false

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4
Q

what causes seborrheic keratosis?

A

Idiopathic

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5
Q

what is Leser-Trelat sign:

A
  • Leser-Trelat sign: fairly young and suddenly many seb keratosis lesions are appearing is more worrisome – linked to visceral cancers
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6
Q

tx for seborrheic keratosis?

A
  • if small: observe, excision/curettage, cryosurgery involving “treat and retreat” with liquid nitrogen, electrodissection
  • if 5mm+: debulk/shave, treat base with cryosurgery
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7
Q

what is this?

what are some associated characteristics?

characteristic histo finding?

what cell layer is being affected?

describe the typical time course of a keratoacathoma

A

keratoacanthoma;

  • nodular: cup-shape, keratin core, pedunculated, rolled borders
  • rapid growth – peak size within 6-8 weeks
  • spontaneous involution/resolution may occur
  • inflammation: micro-abscesses and inflammatory cells within and at base of lesion -> cells at base probably preventing from invading into lower tissue (mixed leukocytes w/ some dermal invasion)
  • *very little cell atypia but mitotic figures can be seen
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8
Q

what is pseudoepitheliomatous hyperplasia of eyelids? what other eyelid growth might it resemble? what will histo reveal?

A
  • a type of keratocanthoma, but mimics basal cell carcinoma bc edges are raised and center is umbilicated / necrotic
  • BIOPSY!
    • path/histology: mixed leukocytes, some dermal invasion
    • little cell atypia, but some mitotic features because rapidly growing
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9
Q

what causes keratoacanthoma?

A

probably UV light, possibly carcinogen exposure, HPV (some biopsies but this may be comorbidity rather than truly causal)

ALSO: usually originates from hair follicle

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10
Q

tx for keratoacanthoma? also include alt therapies.

are steroids effective?

A
  • small: observation (usually go away by themselves), cryosurgery (if taking too long to resolve)
  • 5mm or more: excisional biopsy (if persisting for more than 8 weeks) – needs to be deep to reveal normal tissue underneath
  • alternative therapies:
  • 5-FU, an anti-metabolite
  • mitomycin C (alkylating agent),
  • intralesional steroid bc of inflammatory component, but not very effective
  • oral eretinate (can be used if many lesions, i.e. in cases of HIV/AIDS)
  • oral methotrexate (antimetabolite blocks folic acid synthesis)
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11
Q

HPV is what kind of virus? where does it live in epidermis? what does it do/inactivate?

A
  • live in basal epithelium, not activated until infected cell begins migration upward
  • inactivates the p53 protein -> disables apoptosis, causes massive proliferation of the cells
    • tons of viral replication in the highly differentiated keratinocytes
    • may become cancerous via other mutations
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12
Q

what changes in epi layer occur in benign squamous papilloma (AKA skin tag)?

how is it different from verruca?

A

variable keratosis over fibrovascular core (key point)

  • viral often have looping vascular pattern in fibrous tissue
  • verruca will have viral inclusions and koilocytes on histology
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13
Q

pathogen. of papilloma? verruca?

A
  • verruca: HPV, wart, p53 activation
  • benign squamous papilloma: genetic damage because of environmental factors
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14
Q

tx for papilloma or verruca:

list some alt tx therapies as well

A
  • small: observe, cryosurgery, keratolytic agents (acids), electrodessication, excision and curettage
  • 5mm+: debulk, treat base with cryo

alternate topical therapies:

  • cantharidin
  • podophyllin (astringent from may-apple tree),
  • retin A cream
  • cidofovir (developed in HIV era for CMV, works as a DNA chain terminator to interrupt all cell division)
  • alternate oral therapies: isoretinoin with caution
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15
Q

patho of molluscum:

how to tx? list some alt tx forms, too

A
  • DNA pox virus
  • self-limiting but highly contagious
  • *varying degrees of inflamm, crops or singular

TX:

  • small: observe, cryosurgery, cantharidin, podophyllin, bichloroacetic acid
  • alternate therapies: Australian lemon myrtle oil, tea tree oil, duct tape (works in some people)
  • SilverCure device, Zymadem (OTC-naturopathic found in tea tree and other natural oils)
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16
Q

telangiectasia vs angioma

A
  • Telangiectasia – dilated but normal blood vessels that have become damaged and cannot recover
  • Angioma – new vessel growth
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17
Q

what is this precursor to squam cell carc? what are some key histo findings?

A
  • act. keratosis (but resembles SCC)
  • histo: inflamm, various keratosis + pigment; exagg. surface topo
  • typically full thickness dysplasia (cells irregular, loss of polarity)
    • squamous eddies: epi cells swirling around the keratin due tohigh turnover
    • usually intact BM w/inflammatory cells below
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18
Q

list one of the possible benign DDx of actinic keratosis:

A
  • DDx: roasacea, but this would have thickened, ruddy skin with papules and pustules
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19
Q

what % of act keratosis proceed to squam cell carc

A

5%

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20
Q

patho of act keratosis

A
  • Pathogenesis: UV exposure -> mild atypia, dysplasia, polarity loss, elastosis, base inflammation
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21
Q

tx for act keratosis (small vs large)

A
  • if small: cryotherapy
  • if broad based:
    • 5-FU cream-causes epithelium to slough off
    • PDT 5-ALA
    • Solarize – an NSAID with unclear mechanism in this case (COX-2 inhibits PGE2)
    • Imiquimod cream – suppresses the upregulation of growth via some opioid receptors
    • Ingenol gel (plant extract) – 3d course with amazing results, but an unknown mechanism
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22
Q

what is Bowen’s disease? *

A

carcinoma in-situ -> not a UV-light induced lesion, can occur in non-sun-exposed areas

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23
Q

what is Xeroderma pigmentosum? predisposed to what condition?

A
  • AR trait, most common in the Japanese
    • defective enzymes can’t replace DNA damage in cell

predisposed to squam cell carc

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24
Q

what are some classic features of squamous cell carc? histo feat?

A

squam cell carc, NO classic features, gotcha bitch

  • keratin pearls on histology
    • suggest squam vs BCC
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25
Q

second most common mal lid lesion?

A

squam cell carc

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26
Q

patho of squam cell carc?

A

UV exposure, irritation (i.e. tobacco/esophageal), inflammation (i.e. colitis)

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27
Q

tx for squam cell?

A
  • MOHS or wide-margin excision with frozen section
    • alternate: cryosurgery or radiation
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28
Q

2 forms of squam cell?

A

1) ulcerative
2) nodular

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29
Q

3 histo types of basal cell carc:

A

1) nodular:

  • can be ulcerative, cystic, or differentiated (other proliferating cell types within the lesion)
    • ulcerative or non-ulcerative
    • pearly borders
    • slow growth
    • local invasion

2) superficial

3) sclerosing

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30
Q

histo feature of basal cell:

A
  • do not show exaggerated surface topography (are pearly instead)

“palisading of nuclei” = blunt leading edge of cohesive abnormal cells

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31
Q

where are you more likely to find BCC vs seb cell carc?

A

more commonly BCC are on the lower lid due to increased sun exposure in this location (compared to sebaceous cell carcinomas from the Meibomian glands that are more likely to occur on the upper lid)

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32
Q

what is one of the classic features of superficial BCC?

what does superficial BCC respond to as tx?

A

thread-like borders

Imiquimoid; other options are PDT or 5-FU therapy

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33
Q

morpheoform sclerosing BCC displays what kind of histo activity

A
  • extends with fibrous bands and cords -> “cordlike infiltration”
  • deeper penetration, skin buckling
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34
Q

Gorlin syndrome: what is it, genetics,

A
  • AD tumor suppressor gene-related cancer, involving sonic hedgehog protein
  • pts tend to develop multiple neoplasms including BCC and medulloblastoma
  • pts are very sensitive to ionizing radiation/sunlight, high proliferation of cells in UV-light exposed areas
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35
Q

what are some risk factors for BCC?

A

UV/carcinogen exposure,

fair skin or albinism,

xeroderma pigmentosum,

p53 overexpression,

Gorlin syndrome

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36
Q

how to tx BCC?

A
  • MOHs or wide margin excision with frozen section
  • imiquimod if superficial/very thin/very early, cryosurgery if early lesion, radiation, 5-FU
  • off-label: PDT with 5-ALA
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37
Q

ephelis/freckle has increased melanin or melanocytes?

A

increased melanin, but normal number of melanocytes

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38
Q

lentigo: increased melanin or melanocytes

A

increased melanocytes that do not change from summer to winter, do not darken with UV exposure

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39
Q

what are the 3 types of nevi?

A
  1. junctional nevus – along epidermal/dermal junction
  2. compound nevus – above and below border of BM: elevated but with normal skin markings, no exaggerated surface topography is seen
  3. intradermal nevus – nevus cells in dermis, mostly below dermal-epidermal border
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40
Q

what causes blue nevi?

A

migration of melanocytes from neural crest was arrested during development – the cells stop in dermis

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41
Q

what is the malignancy potential for nevi?

A
  • low : 5% of large congenital nevi may convert because of the increased number of melanocytes
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42
Q

Should you biopsy a kissing nevus/Spitz nevi?

A

Yeah probably

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43
Q

what do halo nevi indicate? are they good or evil?

A
  • immune cells digesting melanocytes/eating away at nevi (this also occurs in choroidal nevi)
  • halo is good
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44
Q

what is lentigo maligna a precursor to?

A

lentigo maligna/Hutchinson’s freckle =precursor to lentigo malignant melanoma

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45
Q

what does lentiginious mean?

A

along epidermal-dermal junction

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46
Q

describe the appearance of lentigo maligna

A
  • “black paint scattered on a brown background” with hyperemia and irregular borders
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47
Q

describe the classic appearance of nodular melanoma

A

**classic “broken blueberry” appearance – applies to most nodular melanomas

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48
Q

Name 6 DDx for melanoma:

A
  • solar lentigo
  • pig. actinic keratosis
  • seborrheic keratosis
  • keratoacanthoma
  • nevus: common acquired nevi, dysplastic nevi, blue nevi, nevus of Ota
  • pigmented BCC
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49
Q

Stage 0 Melanoma

A
  • melanoma in situ, superficial and confined to the epithelium
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50
Q

Stage 1 Melanoma

A

still pretty superficial, not very far into the dermis

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51
Q

Stage 2 melanoma:

A

tumor has penetrated below the dermal/epidermal border

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52
Q

Stage 3:

A
  • below epidermal junction, but even further and has encountered lymphatic vessel – is now travelling on its way to the sentinel node (first node) -> biopsy is needed to determine if the cancer has reached the node
    • any thickness with or without ulceration
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53
Q

Stage 4:

A

has travelled through the node to another organ – places such as lung, liver, brain, bone, soft tissue, GI tract, or other places in skin far from original location

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54
Q

most melanomas metastasize to what organ first?

A

most melanomas metastasize to the liver and then to the lungs

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55
Q

Malignant melanoma patho:

A

UV exposure (chronic or intermittent), genetic predisposition

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56
Q

tx for melanoma:

A
  • excision w/ narrow margins
  • interferon alpha 2b adjunct
  • zelboraf (vemurafenib)
  • yervoy (ipilimumab)
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57
Q

congenital nevi are usually what kind of nevi? (junct, compound, or intradermal)

A

compound

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58
Q

what kind of biopsy is performed for a lentigo maligna?

A

punch biopsy

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59
Q

3 major forms of malignant melanoma:

A

1) lentigo maligna melanoma
2) superficial spreading: most common (upper back)
3) nodular melanoma(de novo or out of LMM or SMM)

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60
Q

What is this?

A

lentigo maligna (at risk for lentigo maligna melanoma)

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61
Q

risk factors for malignant melanoma:

A

atypical mole syndrome, 1st deg relative, northern european, UV exposure, age

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62
Q

how does the Zelboraf (vemurafenib) tx for malig melanoma work? what does it target?

A

Zelboraf (vemurafenib)“designer drug” = chemically synthesized

~50% of melanomas have oncogenic BRAF mutations at position V600 which allows for continuous proliferation of cells

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63
Q

how does the Yervoy (Ipilimumab tx for malig melanoma work? what does it target?

A

monoclonal antibody that blocks CTLA-4 receptor in sensitized T-cells

to turn on the T-cell, CD28 and B7 are coregulatory

to turn off the T-cell, CTLA-4 and B7 are coregulatory -> CTLA-4 activation leads to inhibitory signal production

there is a sudden burst of immune activity that CTLA-4 then shuts down

Ipilimumab allows CTLA-4 to prevent shut down of immune response

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64
Q

How does interferon alpha 2b tx malig melanoma?

A
  • Excite NKs and T-1 helper cells >
  • tumor cells increase MHC II expression for immune system to quickly recognize tumor
  • suppress angiogenesis – robs the tumor of blood supply
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65
Q

What is Muir-Torre syndrome?

A
  • sudden appearance of crops of sebaceous gland hyperplasias, even if benign-looking, can be a marker of internal malignancy
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66
Q

Describe the appearance of sebaceous gland hyperplasia on skin

A

-flesh-colored papular/placoid lesion on cheek

*may have umbilications and look like incipient basal cell carcinoma

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67
Q

what are eccrine/sweat gland hydrocystomas?

A
  • little pockets of sweat retained in skin, occurring in hot and humid weather
    • basically retention cysts in sweat glands, often called hydrocystomas because contain clear fluid
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68
Q

tx for eccrine/sweat gland hydrocystomas?

A

1) 1% atropine cream: sweat glands are under sympathetic control (Ach at first synapse and at effector), so reducing stimulus for sweat dries skin out
2) CO2 pulsed dye lasers – a little skin insult causes better epithelial regrowth
3) Botox (unknown mechanism)

**often go away on their own, but can be semi-permanent during the summer

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69
Q

tx for sudoriferous cysts:

A
  • needle puncture – short term solution, but cyst will regrow
  • electrodissection – older method of removal
  • excision and curettage – removes the cyst and destroys the involved epithelial cells
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70
Q

What is this? where do they typically occur? size range?

A
  • sudoriferous cyst, cyst of Moll -> clear fluid contents
  • often translucent, may have bluish or purplish tinge due to hemorrhage if the vessels leak some blood as they are stretched
  • often occur off to the side, at the inner or outer canthi
  • rarely larger than 1-6mm
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71
Q

Describe the appearance of an eccrine syringoma:

A
  • small fibrous growths, yellow or flesh colored papules or plaques
    • appear as “buried grains of rice” wrapping around the eyelids/canthi
    • sometimes become inflamed – esp will have large amount if auto-immune response occurs
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72
Q

What is this? where do they originate? What pop do they usually affect?

What are some DDx’es?

A

eccrine syringoma: originate from epidermal eccrine sweat ducts

  • most often seen in young women, usually start in 20s and develop for 20y
  • long lived and stable, without regression
    • DDx: milia, sarcoidosis
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73
Q

what is the most common eyelid tumor from the appendages?

A

eccrine syringioma

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74
Q

List some possible appearances of sebaceous gland carcinomas:

A
  • nodular – yellow (lipidized) firm to touch, rubbery
  • nonulcerating
  • can mimic many other findings, i.e. blepharitis, conjunctivitis, recurrent chalazion
  • may destroy lashes
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75
Q

what pop is sebaceous gland carc most likely to affect? How lethal is it?

A
  • most commonly: older females, Asians, upper lids
  • lethal: 30%
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76
Q

List 3 glands/locations where sebaceous gland carc may affect. which has best prognosis? which has worst?

A

1) Zeiss gland – best prognosis
* mimics Zeiss cyst
2) caruncle – vascularization, non-ulcerating
3) Meibomian: mimic chalazion

  • expansive and metastatic with high mortality
  • may extend into the orbit, require removal of the entire orbit
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77
Q

what is the tx for sebaceous gland carc?

A
  • MOHs micrographic necessary
  • orbital exenteration may be necessary if has infiltrated far enough
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78
Q

visceral malignancy can occur in what % of sebaceous gland carcinoma patients?

A

up to 40%

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79
Q

what might this be?

A

sebaceous gland carcinoma

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80
Q

what are the 3 diff types of capillary hemangioma?

A

1) placoid
2) nodular
3) cherry hemangioma

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81
Q

where do you usually find capillary hemang? what do the placoid ones look like?

A
  • on face, trigeminal dermatome
  • superficial, placoid, with crepe paper appearance
    • begins as small red lesion, usually on the upper lid
    • may be associated with intraorbital extension
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82
Q

Describe the course of placoid capillary hemangiomas: who are they more common in?

A
  • usually present at birth, may grow rapidly in first 12 months but often significantly regress (sponatenously) by age 5
  • more common in females
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83
Q

How do you tx capillary hemangioma?

A
  • steroid injections -> steroids are anti-angiogenic, suppresses growth of capillaries (nodular not as responsive as placoid)
    • ?anti-VEGF in the future
  • may respond to propranolol – direct, multiple applications – unclear mechanism
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84
Q

How is a nodular capillary hemangioma diff from a placoid?

A
  • nodular variety: still a ball of capillaries, just growing in a slightly different form
    • also present early in life with rapid initial growth, but less regression than with the placoid type
    • less resp to steroids
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85
Q

what is a naevus flammeus / port-wine stain? where are they found?

A
  • telangiectasia
    • usually segmental and unilateral, following the trigeminal dermatome
      • does not blanch with pressure
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86
Q

naevus flammeus / port-wine stain have a mutation in what gene? what are some assoc of importance?

A
  • mutation in GNAQ gene

associated with:

  • ipsilateral glaucoma (30%)
  • Sturge-Weber syndrome (5%) which is neuro-cutaneous brain+skin issues
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87
Q

what’s one form of tx for port wine stains?

A

yellow pulsed dye laser, early in life

  • endothelial cells (there are many in blood vessels)
  • the laser is very superficial and causes thrombosis/shriveling of the vessels
  • can debulk and lead to a decrease in color of the lesion
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88
Q

describe how port wine stains may change in appearance over time:

A

initially flat and red -> darkening and hypertrophy of the skin -> skin becomes coarse, nodular, and friable (easily broken up, like very dry and crumbly cheese)

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89
Q

what is this?

A

(placoid) capillary hemangioma

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90
Q

what is this?

A

(nodular) capillary hemangioma

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91
Q

when do cavernous hemangiomas occur in life? how do you tx?

A
  • begin later in life, just sort of showing up after 6mo – don’t spontaneously regress
    • also difficult to surgically remove – steroids may help a little bit, but overall do not make much difference
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92
Q

Describe how cavernous hemangiomas differ in appearance and formation from capillary hemag:

A
  • form in deeper tissue than capillary hemangioma
    • these lesions are deep purple or blue (if more superficial) or more flesh colored (if deeper)
    • lumpy appearance
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93
Q

what are cherry hemangiomas? when do they occur?

A
  • another subtype of capillary hemangioma, but this one occurs with age
    • usually just pop up and stay there, benign and dormant
    • common for adults to have 20-30 cherry hemangiomas by age 70y
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94
Q

what are AV malformations?

A
  • arteriovenous malformation: no capillary beds, artery plugs directly into a vein with high pressure – blows up the vein like a balloon, blood doesn’t flow and becomes deoxygenated, appears dark
    • slow growing, may not be noticed until later in lifetime
    • can occur in any vascularized structure, including brain and retina (in which they look all screwy like a bag of intestines)
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95
Q

what are venous malformations:

A
  • abnormal veins being fed by normal capillaries
    • weak-walled veins that stretch out under normal amounts of pressure
    • deep purple hue, commonly small
    • do not regress, actually grow slowly with time
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96
Q

lymphangioma affects what part of the eye? how can you treat?

A
  • lymphangioma (growth of lymph vessels) – hard to diagnose
    • if lymphangioma of lid, often affects orbit, conj, or both -> often this spreads to lids, sometimes to other parts of the face
    • may have fistulas with the blood supply, which can give a chocolate color
    • uncommon, and surgical removal is complicated because the visible lymphangioma is only “the tip of the iceberg” and the lesion is quite extensive
      • tend to wrap around, deep into the orbit and skin
      • sometimes respond to steroids
    • usually benign
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97
Q

what causes a pyogenic granuloma? what is it made of?

A
  • neither pyogenic (pus-producing) or a granuloma (clump of inflammatory cells)
    • aberrant wound healing response to minor trauma, surgery (i.e. for chalazion) or inflammation
    • 5% of pregnancies
    • granulation tissue is made of inflammatory cells, fibroblasts, neo– uncontrolled angioleading to a nodule of immature blood vessels that are very fragile
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98
Q

how do you tx this?

A
  • pyogenic granuloma:

shave biopsy, then pulsed dye laser

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99
Q

what is Kaposi’s sarcoma (made of?)

A
  • vascular and lymphatic angioma of skin, mucous membranes, viscera
    • seen in HIV, immunosuppression, certain races (Mediterranean, Jewish ancestry, Sicily and Sardina)
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100
Q

Kaposi’s are associated with what virus?

A
  • 90% of lesions have HHV8 – pro-angiogenic in nature, usually suppressed by the immune system in normals
    • advanced AIDS/ immunosuppression
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101
Q

what is this? how do you treat?

A

Kaposi’s:

*begin as small reddish-brown macules

  • if AIDS-related : HAART
  • radiotherapy because many vessels are involved and these have tons of endothelial cells
    • cryotherapy
  • topical retinoid cream – unclear mechanism but slows growth
  • chemotherapeutic injections
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102
Q

what is this? describe its appearance and characteristics

A

Epidermal Inclusion Cyst:

freely movable, solid cysts with cheesy contents but no keratosis, area may be inflamed

  • often have a yellow-ish hue, but are not sebaceous
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103
Q

how do you tx epidermal inclusion cysts?

A

marsupalization: cyst is removed within fibrous capsule – want to make sure no epithelial cells get left behind and grow back

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104
Q

what is this? how do you tx?

A

milia

  • easily removed via surgical excision
  • stable and do not change over time (rule out molluscum and herpes)
    • may also mimic syringoma
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105
Q

what is this? how do you remove it? describe the appearance

A

Zeiss Cyst

  • derives from epithelium of the gland
  • can grow out of any sebaceous gland, typically on the lid margin
  • appear pearly, nodular, yellow-ish because they contain sebum
    • non-translucent*
  • remove via marsupalization
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106
Q

what is the difference between a choristomatous and a hamartomatous growth?

A
  • choristomatous growths = tissue elements that don’t necessarily belong are included in the growth, i.e. hair/teeth
  • hamartomatous growth = tissue elements that are normally present in the location of the lesion
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107
Q

where do dermoid cysts typically occur?

A

frontozygomatic suture and are rather superficial

  • entrapped epi/subepi tissue within the facial clefts during development
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108
Q

How do you tx dermoid or epidermoid cysts?

A
  • remove via endoscopic surgery – minimal scarring
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109
Q

what kind of deposits are pictured here? what are the deposits made of?

A
  • amyloid: non-collagenous protein deposited from some cellular/vascular abnormality
    • can be induced by trauma, etc.
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110
Q

what kind of deposits are lipoid proteinosis? what are they made of?

A

lipoid proteinosis: hyalinized lipids forming glassy smooth-looking tissue bulbs, can be mistaken for granuloma or other things

  • tends to occur along lid margin
  • also occurs in voice box giving raspy voice
  • fairly uncommon
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111
Q

what is CALT?

A
  • conjunctival accessory lymphoid tissue
    • patchy groupings of lymphocytes and plasma cells that produce IgA to respond to immune challenges
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112
Q

describe the difference bw an exudate and transdudate (and discharge)

A
  • transudate: acellular fluid, fibrin, protein leakage
    • occurs first, before exudative response
  • exudative response: fluid with cellular material (WBC) forms pus in early stages
  • discharge: exudate or transudate released from/out of the body
    • i.e. can be picked up/removed from the tissue
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113
Q

what is ocular pemphigus? what is occurring at the cellular level? where is the inflamm occurring?

A
  • autoimmune response to intercellular proteins that form tight junctions
    • destroys glue (desmoglein) to allow epi cells to separate and become loose
    • acantholysis- separation of epi cells - occurs between the walls of cells
    • interepithelial bullous inflammation results
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114
Q

what is ocular phemigoid? where is the inflamm occurring?

A
  • more severe than pemphigus: autoimmune response against adhesion components and the laminar component of the basement membrane
    • destroys BM which exposes sticky collagenous fibers
      • symblepharon more likely
    • inter- and subepithelial bullous inflammation
    • ulcerated and exposure of sticky material can lead to scarring, etc. and secondary destruction of the goblet cells -> advanced dessication can lead to keratinization
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115
Q

what is a pseudopemphigoid: what systemic conditions cause it?

A
  • autoimmune/allergy induced by an exogenous material, usually meds
    • Toxic Epidermal Necrosis and Steven’s Johnson syndrome -> more often due to meds, i.e. sulfonamides and some antibacterials
    • Erythema Multiforme -> another autoimmune from exogenous cause
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116
Q

what differentiates SJS from TEN

A

TEN covers more than 30% of the body area and is more severe than SJS which covers 10% or less

-typically caused by sulfa drugs

117
Q

what is erythema multiforme? what can cause it?

A

bulls-eye rash with elevated papules, usually more distal (hands/legs) than trunk (usually TEN or SJS)

HSV and other viruses or bacteria can cause, but most often result of medications

118
Q

what kind of pop. usually get pemphigus vulgaris:

A
  • usually seen after 50y
    • same amount of males and females (versus pemphigoid which is 3:1 female) – but if occurring in a younger patient, pemphigus is more common in females
    • Mediterranean descent/Jewish
119
Q

what is this? does it typically affect conj? where in the eye does it affect? what can form/what side effects occur with repeated attacks?

A
  • pemphigus vulgaris:
  • small to fairly large areas of epithelial blisters and bullae of the skin -> transudates into the area, heal and scar
    • rarely, mucous membranes are involved (usually not conj, but rather skin)
  • blepharitis, rarely conjunctivitis, usually spares the cornea but may result in dry eye in a severe case
    • repeated attacks may give symblepharon (rarely), fornix shortening (scarring between tarsal plate and conjunctiva), dry eye, persistent epithelial defects
    • mucous membranes slough off and underlying tissue blends together
120
Q

what kind of pop does cicatricial pemphigoid affect?

A
  • females 3:1 males
  • age 30-90, but most occur during 7th decade; rare in kids
121
Q

where does cicatricial typically affect the body?

A
  • involves mucous membranes more than dermis
    • bilateral
    • 85% oro-pharyngeal, 65% conjunctival, 25% skin
    • *unlike pemphigus vulgaris which is more likely to affect the skin
122
Q

what is the major concern with ocular cic pemphigoid? why is it worse than pemphigus? what changes occur with the eye long term?

A
  • major concern: tear deficiency because goblet cells are wiped out so the eye cannot lubricate itself
  • when epi sloughs off and full tissue thickness involved: parenchymatous
  • more damaging than pemphigus because it attacks the basement membrane, causes substantia propria scarring, causes goblet cell loss
    • shortening of fissue – eyelids zipper shut from ends, progressive over time

tylosis, madarosis (from destruction of root follicle), lash metaplasia (replacement of one tissue with another – not mucocutaneous lids, rather regular skin has grown there), poliosis, scarring of Meibomian orifices

123
Q

what is epidermialization and when do you see it with oc cic pemph?

A

severe: metaplasia, where conj/cornea can be replaced with skin-like tissue

124
Q

why does erythema multiforme occur and what age range is typically affected?

A
  • erythema multiforme: unknown cause, possibly due to deposition if immune complexes (mostly IgM)
    • peak incidence 2nd and 3rd decades
125
Q

Describe the pathogen. of pemph vulg vs oc cic pemph:

A
  • PV – auto-antibodies against intercellular keratinocyte surface membranes results in acantholysis (skin falls apart)
  • OCP – auto-antibodies against basement membranes give severe blistering, erosion, scarring (as tissues are sticking together)
126
Q

How should you tx oc cic pemph, pemph vulg or pseudopemph?

A
  • treatment: stop meds if pseudopemphigoid
    • send to ER – topical and oral steroids, long-term immunomodulators
      • needs to get to dermatologist and figure out long term management
    • prophylactic antibiotics, nutrient supplements

Can be potentially fatal, very serious, but manageable with heavy steroid and antibiotic treatment (supplement high steroids with Ca2+ and vitamin D)

127
Q

how can you tx keratinization that might occur in an oc disease such as oc cic pemph?

A
  • if keratinization, want to slow down keratin production and epi turnover because there is proliferation of cells that do not belong in the cornea -> 0.05% topical retinoic acid
128
Q

what are some aggressive dry eye tx options for something like oc cic pemph? what if you see mucus filaments?

A
  • non-preserved ATs, pulsed topical steroids, oral tetracycline and omega 3s, punctal plugs
  • Restasis (weak), Tacrolimus (stronger brother to Restasis that works in a similar way, a drug for dermatitis that works for ocular findings too) -> suppress T cell activation/interaction
  • 10% acetylcysteine (derived from Mucormyst and compounded to a drop) – if filaments/mucous plaques are found
129
Q

Lst several possible etiologies of conj keratinization:

A
  • actinic damage – UV light
  • inflammation – i.e. severe EKC, trachoma
  • neoplasia
  • chemical insult
  • OCP/pseudopemphigoid
  • nutritional
130
Q

what is this? what causes the “Swiss cheese” appearance seen?

A
  • vitamin A deficiency – Bitot’s spot/xerosis
    • appearance of “Swiss cheese” because of gas bubbles from diptheroid bacteria that are feeding on the keratin
      • sometimes a little melanin is involved, so the spot is not always a classically white color
131
Q

what else can occur that affects the eye after long term vit A def? How long does recovery typically take with nutritional therapy?

A
  • after a long time, vit A deficiency can give night blindness: xerophthalmia fundus
  • resolution: goblet cells start to recover with 2-3w of nutritional therapy, full resolution within 3-6mo
132
Q

what can cause xerosis at the parenchymatous level

A
  • parenchymatous – trachoma, OCP, very severe inflammation i.e. EKC or conj gonorrhea (all epi sloughed off – full-thickness involvement)
133
Q

what are some primary or sec causes of amyloidosis?

what is amyloidosis?

A
  • primary vs. secondary
    • primary – probably genetic/inherited
    • secondary – induced by exogenous processes; i.e. trachoma, leukemia, multiple myeloma
  • amyloidosis: non-collagenous protein deposit in the skin or conjunctiva (rarely cornea)
134
Q

what is this? list several DDX’es

A

amyloidosis

  • lymphoma, leukemia
  • benign lymphoid hyperplasia (also fairly common)
  • carcinoma in situ
  • sebaceous gland hyperplasia
  • recurrent subconjunctival hemorrhages
135
Q

what is argyrosis?

A
  • argyrosis is a silver deposit – recently silver was put back into some make-ups, but it cannot be phagocytosed and can accumulate
136
Q

what are some DDx in the case of pingeuculitis? How can you tx it?

A
  • pingeuculitis
    • mimics: phlyctenule, nodular scleritis, PEH/CIN (if non-responsive to Tx)
    • Tx: topical steroid with low anterior chamber penetration, i.e. FML
      • acular: mild topical NSAID
137
Q

what is causing pteryg and ping?

A
  • actinic (UV light induced)
    • solar elastosis: only occurs in tissues that have elastin protein (lends stretchiness to tissues) -> UV light gives this specific elastin degeneration
138
Q

why are pteryg usually nasal?

A
  • typically nasal origin because limbal stem cells are most disabled here, allowing for progression
139
Q

what is Stocker’s line?

A

iron deposition at head of pterygium

140
Q

what are some things that impact limbal stem cells and give rise to pterygia? (genetic factors?)

A
  • damage to limbal stem cells: a series of events occurs that allows the growth
    • p53 (tumor suppressor) pathway: “guardian of the genome”
    • matrix metalloproteases (MMP) degrade ground substance
      • tissue inihibitors of MMP (TIMP) – if low levels, too much degradation occurs allowing new tissue to come in
    • gene Ku70 may be specifically mutated
      • DNA break repair -> similar to xeroderma pigmentosum, cannot repair DNA so undergo proliferation
141
Q

what are some surgical options for pterygia?

A
  • conjunctival allograft
  • amniotic membrane with fibrin glue that melts with time, so the membrane is held in place until good healing occurs
  • mitomycin C: very weak to slow cell division/repair and prevent overgrowth
  • thiotepa is another alkylating agent to slow recurrence, works in the same way as Mitomycin
142
Q

what is the definition of a cyst?

A

cyst is plug of epi cells on the wrong side of the BM that replicate – the outer bilayer keeps growing as the center dies

143
Q

what is a pseudocyst? what does it contain? how to manage?

A
  • pseudocysts (clinically looks identical to inclusion cyst)
    • fluid pouch – entrapment between layers of the mucous membrane
    • inflammatory debris
    • often transient, resolving on their own
144
Q

what are inclusion cysts? how do you tx them?

A
  • inclusion cysts:
    • true cysts -> needle them, but they come back
    • may trap goblet cells on the internal side, leading to mucous on the inside and a turbid appearance
    • stable -> some go away on their own, but if pt really bothered by the cyst, maybe a surgeon will remove it, but they often come back -> CO2 pulsed laser to base may decrease recurrence
145
Q

what is this? where are they typically found? describe the typical appearance. what is inside this lesion?

A

Epibulbar Dermoid

  • Inferotemporal to limbus
  • Solid dome shape: They often look white and smooth. Often they’ll have something else growing in the lesion, such as hair.
  • Smooth surface
  • Choristomatous: Many things can grow within these lesions.
    • Hair
    • Teeth
146
Q

How can you tx an epidermoid cyst?

A
  • If its small, it can be left alone,
  • a lemallar keratoplasty: area is removed and leave the base of the tissue. Donor tissue is then matched to the space that was removed. apply mitomycin C
147
Q

what is Goldenhar’s syndrome or Oculoauriculoverterbral dysplasia (OAV)?

A
  • Bilateral: Many facial asymmetries and other epibulbar dermoid cysts (in other regions). Mental retardation and physical abnormalities occur as well. Some of the physical abnormalities include:
    • Ear
    • Face
    • Skull
    • Spine
148
Q

How can you differentiate Lipodermoid/Dermolipoma from orbital fat herniation

A
  • Confused with orbital fat herniation: If you put a Q-Tip on it you will see that it’s squishy and responds like dough. It’s often yellow, non-tender, non-vascularized, and non-painful. They can grow larger and can be removed.
149
Q

what is this? where are they typically located? how can you tx?

A
  • This is a fatty tumor growing in the same area as the limbal dermoid cysts.
  • Superior-temporal
  • Temporal
  • Fatty tissue: It can often become inflamed.
    • Squishy
    • Difficult to remove: These are difficult to remove because they go very deep. Most of the time it is preferred to leave them alone.
150
Q

what is this? (hint: associated with organoid nevus syndrome)

A

complex choristoma

  • Organoid nevus syndrome: When we see these in the eye as well as other places, tendency for malignancy. important for the patient to have a brain scan because there are possible neurologic deficits associated with it.
    • Choristomas: Often occurs in superior temporal area. It is a fleshy lesion.
    • Nevi progressing to malignancy
    • Neurologic deficits
151
Q

what pop do you usually see Benign Lymphatic Hyperplasia?

A
  • 6th-7th decade: Most often occurs in the elderly and is unilateral most of the time.
    • Rare in children
152
Q

what is this? what is characteristic of this lesion? when does it occur? describe the appearance

A
  • Benign Lymphoid Hyperplasia
  • There are no real distinctive characteristics of this lesion. It is present at birth and grows slow. It is probably part of a larger syndrome.
  • It is milky hyperplasia. It has a lot of follicles and accessory lymphoid tissue. It also has a milky, juicy look to it. There is no discharge.
153
Q

what is the prognosis for benign lymphoid hyperplasia? (chits vs elderly?)

A
  • Rarely malignant in elderly patients. If you have any concerns (discharge or changes), you can have it biopsied. Remember that tumors are common in the elderly as well.
    • 20% of these progress! Many are signs of systemic conditions while some turn into tumors.
    • Rule of thumb: When you see something like this, make sure to rule out SCC.
  • Children: If this happens in a young person, it is rare. When seeing this lesion in a young person it is therefore important to rule out leukemia, lymphoma, and other systemic diseases.
    • Rule out blood dyscrasias
154
Q

List a few DDX’es for Benign Lymphatic Hyperplasia

A
  • Conjunctivitis
  • CIN
  • Meibomian carcinoma
  • Lymphoma
  • amyloidosis
  • leukemia
155
Q

tx for Benign Lymphatic Hyperplasia

A

excisional biopsy

corticosteroid/beva-mab (monoclonal ab)

156
Q

what is this? what are the causes? describe the characteristic vascular pattern of this lesion

A

Conjunctival Papillomas:

caused by viruses. young person: human papilloma virus (HPV) elderly: also virus but UV too

  • Vascular patterns: They tend to be highly vascularized.
    • Hairpin loops: Most have a hairpin loop pattern, which is a characteristic pattern of slow development as opposed to a fast, disorganized growth such as what happens with cancer.
      • Seeing a well organized vascular pattern is a clue for diagnosis. But, many other lesions may have this pattern as well (see differential Dx for other examples).
    • Arborizing: Looping pattern to blood supply.
157
Q

what are some tx options for conj papillomas?

A
  • Recurrent after surgery: Usually they are removed.
    • Cryosurgery: This usually has the best result, and the base of the lesion is removed.
    • CO2 laser: This is getting more refined and is becoming more popular.
  • Alternatives for treatment.
    • Cimetidine tends to improve T lymphocyte population which might help reduce the spread of the virus. mitomysin C, canthiardin, alpha interferon (a blistering agent), dinitrochlorobenze
158
Q

List several DDx’es for conj papillomas

A
  • PEH, CIN, SCC, BLH Pyogenic granuloma
159
Q

what are koilocytes?

A

squamous epi cell that has undergone number of structural changes due to viral inf (HPV)

may have these changes: -nuc enlargement -irreg of nuc membrane - nuc hyperchromia -clear area around nuc (perinuc halo) -

160
Q

below is a Pseudoepitheliomatous Hyperplasia (PEH); list some characteristics

A
  • Rapid Growth
  • Inflammation
  • Ulceration possible
  • Variable keratosis
  • Variable vascularization
  • Do not involute
  • usually arise rapidly and dissipate within a few weeks after they peak. The conjunctival ones may be very persistent, pesky lesions. lot of inflammation, can cause damage and scarring of the conjunctiva.
161
Q

how do you tx Pseudoepitheliomatous Hyperplasia (PEH)?

A
  • Surgical excision and biopsy
162
Q

what is this? what are some DDx’es? what is seen in this photo?

A

gelatinous acanthosis with a vascular pattern of pinpoint hairpin loops. invasion of the corneal epithelium(conj papillomas don’t invade cornea). This slide resembles SLK with fibrovascular limbal pannus. DDX: Lymphoma and leukemia, BLH, amyloidosis, papilloma, limbal dermoid, or pingueculitis

163
Q

List some predisposing factors to squam cell carc:

A
  • Predisposition: People who have a p53 pathway defect.
    • HPV 16, smoking are the primary things which cause SCC
    • CL wear: Whenever a CL is placed on someone, there is insult to the eye. SCC are distinctly associated with CL wear although they are rare.
    • UV exposure
    • AIDS/Immunocompromised
    • Xeroderma
    • People with a northern European ancestry are more likely to develop a SCC
164
Q

what are some tx options for conj SCC?

A

Surgical excision> topical chemotherapy w/ Mitomycin C –

base may be treated with 100% ethanol

cryosurgery

CO2 laser ablation

IFN alpha

165
Q

what is this? DDx? what is w/in this lesion?

A
  • lymphangiectasia
  • plexus/network of clear lymphatic vessels – dilated and full of lymph fluid
    • these are channels, as opposed to pseudocysts which are collections of fluid all in one area
166
Q

what is this? DDx? when do they occur? describe typical appearance

A
  • lymphangioma: growth of the lymphatic vessels – intertwined into a mess
  • difficult to diagnose – DDx include fat, cyst, CIN, SCC, SGC, lymphoma, leukemia, amyloidosis
  • present early on (usually congenital), grow slowly
  • no feeder vessels or apparent vascular pattern, but blood may fill channels
    • often closely associated with venous tissue – take on a “chocolate cyst” appearance
  • no irritation, looks fleshy, no keratinization, not opaque
167
Q

what is Osler-Weber-Rendu disease?

A
  • genetic disorder: hereditary hemorrhagic telangiectasia
168
Q

what is a hemangioma?

A
  • hemangioma – congenital malformation of blood vessels which often regress
    • capillary hemangioma usually regress by 10y, cavernous hemangiomas usually grow slowly with age
    • rarely occur on the conjunctiva
169
Q

what is the difference btween Ephelis and Lentigo?

A

Ephelis is a collection of pigment. Lentigo is a collection of melanocytes

170
Q

what is this? where is it usually located? what is typical appearance? is it movable? what causes it to darken?

A
  • Congenital Epithelial Melanosis
  • Present at birth and may be bilateral.
  • Darkens with age
    • Hormonal changes such as during puberty.
      • More melanocytes are actually growing.
    • Steroids
  • Freely movable over the globe.
    • It’s in the conjunctiva, so it’s epithelial. You can move it with a Q-Tip. It wouldn’t move if it were subepithelial.
  • When it is deeper it appears gray.
  • Often at limbus
  • Patchy/ragged appearance
  • Name is a misnomer.
  • Near anterior ciliary arteries
171
Q

what is this? when does it occur? is it movable? describe its coloration

A
  • Congenital Subepithelial Melanosis
  • Present at birth
  • Underneath vessels
  • Not movable

·Slate gray or blue black appearance

  • The pigment cells are mixing with white scleral fibers and becoming a different color.
  • Unilateral in Caucasians
    • Increased chance of melanoma and glaucoma

Congenital subepithelial melanosis is actually a misnomer in that the melanin pigment is trapped in the episclera and Tenon’s capsule, rather than in the substantia propria of the conjunctiva

172
Q

what is nevus of Ota? is it malignant?

A
  • Subepithelial congenital melanosis
  • Goes beyond the globe. It spills over to the dermatome so that there is skin involvement.
  • Cutaneous nevus
  • Heterochromia
  • Common in Asians
    • Rarely malignant
  • Caucasians
    • More likely to become malignant (more likely to get uveal melanoma not conj melanoma)
173
Q

what is a good prognostic indicator for nevi of the conj?

A

One helpful thing with nevi is to look for mucous cysts. This is associated with a benign lesion and stability. The mucoid cysts in nevi make the lesion look like different colors, but this is actually good.

174
Q

how can you tx nevus of ota and other pigmented lesions?

A

candela alex trivantage laser can selectively target colors in birthmarks etc

175
Q

what are the 3 types of conj nevi? describe a few of their characteristics

A

1) junctional:

  • Flat patches similar to Ephelis, PAM
  • Lies at the epidermal/dermal junction

2) compound:

  • Elevated
  • Mucin Cysts
  • Compound means you have melanocytes above and below junction.

3) intradermal

176
Q

Should you be more concerned over bilateral or unilateral primary acquired melanosis? what are some key differences between the two?

A
  • Bilateral: This should make us less concerned. We will see this often.
    • Black/Asian
    • Probably present at birth
    • Darkens with age
    • Very low malignant potential
  • Unilateral: high tendency to become malignant
    • Caucasians 20-50
177
Q

what is secondary acquired melanosis? what are some causes? (what are some chemicals and metabolic diseases and conj disorders?)

A
  • Secondary – We know what causes this. It is secondary to something we know.
    • Radiation
    • Metabolic disorders
      • Addison’s disease
      • Pregnancy
    • Chemical toxicity
      • Arsenic
      • Thorazine
      • Latanoprost
    • Chronic conjunctival disorders
      • Trachoma, VKS, Xeroderma
178
Q

when do PAM typically occur? what % become malignant?

A

·Onset 40-50

  • Unilateral is an alert factor.
  • Waxes and wanes
  • 15-20% become malignant
    • Difficult to diagnose, so need to have it biopsied.
  • Peppered patch appearance
  • Spots of hyperpigmentation
179
Q

what % of melanomas arise from PAM?

A
  • 30-75% of CM arise from PAM
    • CM from PAM has highest mortality
180
Q

Describe the stages of PAM:

A
  • Stage 1 PAM (Primary acquired melanosis)
    • Stage 1A: Similar to a junctional nevus.
      • The difference between acquired is cellular atypia and the potential for neoplastic growths.
      • lack of cysts makes these suspicious.
    • Stage 1B: Shows some cellular atypia and partial thickness dysplasia. BM respected.
      • At this point there is a little disruption and abnormality to the tissue. There may be a little bit of increase vascularization.
    • Good Prognosis
  • Stage 2 PAM This is cancer when the basement membrane has been breached.
    • Stage 2B and conjunctival melanoma are basically the same thing.
      *
181
Q

How is PAM tx’ed?

A

excision cryo mitomycin C or 5FU

182
Q

Most conj melanomas occur where? when it is located in the ____, it carries higher risk of metastasis

A

·Most CM touch the limbus and occur in the UV exposed zone

  • Usually occurs out of PAM
  • CM occurring in extralimbal location carries higher risk of metastasis
183
Q

mortality rate for conj melanoma? what are some good prognostic indicators?

A
  • 25% overall mortality
  • Tumor thickness is a good prognostic indicator
    • Less than 1.5mm good
    • More than 1.5mm bad
  • Poor prognosis if extended to fornix or surrounding structures: Bad if it goes beyond what you can see
184
Q

tx for conj melanoma?

A

“no touch” excision followed by cryosurgery and mitomycin C

185
Q

what is megalocornea? what are some assoc conditions?

A

horiz diam >13mm in adults, infants >12mm

associations: pig disp syndrome, zon. stretching>phakodenesis, iridodenesis, iris stromal hyperplasia

“-denesis” means shaky, signifies movement

186
Q

what is microcornea? what are some assoc conditions?

A

cornea <10mm

assoc w/ glaucoma, aniridia, Peter’s anomaly, MCRS syndrome (bilat microcorn, congen cataracts, rod-cone dystrophy, post staphyloma)

187
Q

what is a corneal staphyloma?

A

cornea is lined by iris tissue, can be cong or acquired

188
Q

how big is the cornea at birth? at 2?

A

10 mm at birth

11.75 mm at age 2

189
Q

what is staphyloma?

A

Staphyloma is an ectasia of the globe with a uveal lining

190
Q

what is cornea plana?

A

cornea plana: no curvature to the cornea – it’s a flat cornea. There is corneal scleralization in this case as well

191
Q

what is sclera cornea? when does it occur? bi or unilat? what causes it?

A
  • congenital
  • may be bilateral or unilateral
  • may be full or partially extensive where it shows part of the pupil. It may be fully or partially opaque
  • derangement of corneal fibers so that they are not parallel and arranged as orderly – they therefore take on a scleral look. maybe neural crest developmental anomaly?

* fairly rare

**assoc w/ ant chamber dysgen

192
Q

what are some meds that can cause band kerat?

A

Lithium, HCTZ, eyedrops w/ mercury

193
Q

what is this? what is the tx?

A

band keratopathy

tx: epitheliolectomy/chelate (EDTA), scrape off calcium plaque, consider PTK, amniotic membrane

*Vogt’s limbal girdle type 1 is a type of band keratopathy. Type 2 is just an elastoid degeneration.

194
Q

what pop are affected by spheroidal degen/actinic keratopathy?

what are some risk factors/types?

A

outdoor equat region, men more than women (4:3)

Types

1) strictly UV and environmental related
2) there was some type of trauma/inflamm/dystrophy (such as a burn or trachoma) and the epithelium healed poorly
3) extra corneal, ex: top of a pterygium/ping (with yellow gelatinous material)

195
Q

what changes are occuring on a cellular level with spheroidal degen/act keratopathy? what layer of cornea affected?

A

hyaline spheroids are deposited extracellularly at or bellow the epithelium, elastotic degen in Bowman’s/ant stroma

“like non-calcific band keratopathy”

196
Q

what is the tx for spheroidal degen/act keratopathy?

A

scraping or amoils brush (followed by PTK)

lamellar or pen keratoplasty

197
Q

what is this? is it typ uni or bilat? affects what types of people?

A

Salzmann’s nodular degen

-bilat

>50yo

females>males

198
Q

what is the pathogen of Salzmann’s? what layer of the cornea is it affecting?

A

persist oc surface disease:

  • chronic inflamm
  • inc fibroblast
  • hyalin degen, excess collagen

**occurs first in ant stroma

199
Q

what other ocular findings might accompany Salzmann’s?

A

pannus

band keratopathy

spheroidal degen

200
Q

tx for Salzmann’s, medical vs surgical: should you use mitomycin C

A

aggressive dry eye tx:

Lotemax:

  • QID x30d, then BID x30d

oral doxy (counter MMP activity):

  • 100mg x7d, then 50mg x60d

restasis

mitomycin C: inhibits the fibroblast activity, be careful, risk for stromal melt, prob shouldn’t use

surgery: alcohol to anesthetize, create flap/peel flap (used to use coke/tetracaine), resurface w/PTK

201
Q

Stage 1 Fuch’s:

A

asymptomatic, central guttata+pigment, VA norm

202
Q

Stage 2 Fuch’s:

A

symptoms due to corn edema (blurry va, glare haloes etc), stromal then epi edema, bedewing/microcystic, loss of corn sens, bullae RCE

pachy-thickening

203
Q

Stage 3 Fuch’s

A

pannus for, chronic edema, periph vasc, pt is actually more comfy than stage 2 due to loss of corn sensation

204
Q

Fuch’s tx: what medication should you avoid?

A

hair dryer 2-3X/day, 5% NaCl 4-8x/day, lower IOP **avoid CAI since they inhib endo pump** take care of RCE, endo keratoplasty, penetrating keratoplasty

205
Q

what kind of pts does Fuch’s affect?

A

females 3:1, 5-6 decade, 4% of people over 40

206
Q

descemet’s takes on what kind of appearance in Fuch’s?

A

blue grey sheen beaten metal due to edema

207
Q

Pts with Fuchs are more prone to what after cataract surg?

A

pseudophakic bullous keratopathy

208
Q

what is causing post polymorph dystrophy?

A

endo cells behaving like epi cells (proliferating and migrating) can cause iris and angle abnormalities

209
Q

describe presentation and progression of post polymorph dystrophy: (include slit lamp appearance)

A

usualy asympt, bilat asymmetric, early in life but slowly progressive

endo vesic, donut shaped lesions, snail track bands, putty gray dots, sheets, curvilinear lesions, cornea may be thin centrally *Hyperopic shift*, angle/iris abnorm like ICE

210
Q

describe the pathogen of RCE:

A

abnorm relationship bw epi and BM (risk factors: trauma, EBMD, inflammation, pers edema), chronic low grade inflamm

211
Q

how do you manage post polymorph dystrophy?

A

observe, manage bullous keratopathy/edema, glaucoma issues (tx similar to Fuch’s)

212
Q

post polmorph dystrophy may appear similar to what other clinical syndrome?

A

ICE: both conditions can cause endo cells to take on epi cell character. can migrate into angle and stroma, can get membranes that contract to form corectopia, synech, iris atrophy

213
Q

what are the 1st,2nd, 3rd line tx for RCE?

when is doxy contraindic?

what are some possible additional methods?

A

1st: hyperosmotics, lubes, LOtemax QID x30d, BIDx30d, oral doxy 100mgX7d, 50mgX60d, (CI: SLE, chits, preg, kidney, cand), BCl
2nd: ant stromal puncture
3rd: PTK
possibly: Azasite BID, tranquil-eyes, autolog serum tears

214
Q

what is the Gray line? where are the meibomian glands in relatin to it?

A

sup portion of orbicularis, (muscle of Riolan)

meib orif open post to gray line

215
Q

what are meib glands? how many do you have throughout life?

A

mod sebaceous glands

16yo: upper 30-40 lower 20-30

10-20% drop out by 60

216
Q

what is meib seborhhea:

A

hypersecretory MGD, assoc with rosacea and seb demr

217
Q

what are some etiologies of MGD:

A

1) high chol esters: sterols have higher melting point than waxes
2) bacteria: staph and propion on lid margin
3) bac lipases and esterases hydrolyze etsers in meib secretions: produces irritating free fatty acids/inflamm

218
Q

describe pannus vs degenerative pannus:

A
  • pannus = collagen deposition on top of Bowman’s beneath epithelium
  • degenerative pannus is without inflammation or associated vessels – it looks like frosted glass
219
Q

describe type 1 vs 2 of limbal girdle of vogt: what is major diff bw two forms?

A
  • type 1=early band keratopathy – calcium deposition
    • Swiss cheese appearance, lucid interval
    • if becomes more severe can progress to band keratopathy
  • type 2 is elastotic degeneration – UV light induced (same as actinic keratopathy)
    • stringy/chalky appearance with NO lucid interval/can abut limbus

*type 2 has NO lucid interval

220
Q

what is the diff bw prim and second lipid degen

A
  • primary lipid degeneration: without corneal trauma or disease – can be seen with serum lipoprotein abnormalities
  • secondary lipid deposition: condition causing corneal vascularization allows leakage of lipids from vessels
221
Q

prim vs second amylodosis of cornea: what causes each and which is more common?

A
  • primary: enzymes cannot properly fold proteins so they do not become water soluble and therefore cannot exit the cell
    • systemic amyloidosis rarely involves cornea
    • primary corneal amyloid is usually extension of conj amyloid to cornea
  • secondary: most common cause of corneal amyloidosis
    • seen after damage to cells (can’t get amyloid out of tissue) – i.e. trauma, infection, uveitis, hydrops, some dystrophies
222
Q

what is the pt profile of someone with keratocon? when does it start, who does it affect?

A
  • incidence of 1:20,000 ; sporadic inheritance
    • 68% male
    • onset 10-20, commonly at 13y (puberty), usually stable by 30y
      • occasional later onset
223
Q

List several associations with keratocon:

A
  • collagen diseases, i.e. Ehlers Danlos
  • floppy eyelid syndrome
  • Leber’s congenital amaurosis -> lose vision early in life due to a gene defect (not the same as Leber’s optic neuropathy)
    • babies with this condition rub their eyes a lot, giving mechanical trauma…
224
Q

what are some proposed mechanisms of keratocon?

A
  • endocrine system – puberty, hypothyroidism, pregnancy are implicated in development/progression
  • defective collagen – more prone to keratoconus
    • Ehlers-Danlos, floppy eyelid syndrome, osteogenesis imperfect
  • mechanical: eye rubbing in VKC, hard CL wear, Down’s syndrome pts, Leber’s congenital amaurosis
  • breakdown of Bowman’s layer is common
225
Q

what is a descemetocele?

A
  • fluid causes such erosion that the cornea thins in one area and the stroma tissue erodes away - Descemet’s bulges and herniates through – bubble is a Descemetocele
    • epi sloughs off / ulcerates, stroma thin or absent
    • Descemet’s is probably close to rupturing at this point
226
Q

what is the dresden protocol?

A
  • Dresden protocol (corn cross linking) takes about 1-2h:
    • pre-op pilo>small pupil can prevent UV to retina
      • >400um thickness of cornea protect endo from UV radiation
    • remove epi – anesthetic, alcohol debridement
    • riboflavin (0.1% sol’n w/ 20% dextran) q2-3min for 30min
    • UV-A radiation for 30min, stil with riboflavin q2-3min
    • ab ung, BCL until healed
    • may partially regress, usually stable and permanent
227
Q

what conservative or surgical measure can you take to manage hydrops?

A
  • # 1 get a cornea specialist on the phone to decide what will be done
  • conservative/observation approach: continue this until cornea heals, endo must grow before can attempt PK
    • hyperosmotics: 5% NaCl drops 4-6x/day and 5% NaCl ointment at night
    • calm inflammation: prednisolone phosphate 1% QID or Lotemax 0.5% QID
    • cycloplegia
    • prophylactic antibiotic: polytrim or tobramycin TID
  • surgical/gas bubble: newer, work with the pt and specialist because may not bubble right away; variable response but generally leads to faster healing than if the pt is not bubbled
    • instill miotics and remove aqueous to allow for gas bubble
    • inject gas bubble, pt stays supine for 24-36h -> tamponades the cornea and helps with deturgescence
      • gas better than fluid because fluid influx is what’s causing the issue
    • try to draw H2O out: 5% NaCl gtt 4-6x/day, 5% NaCl ointment at night
    • prednisolone phosphate 1% QID or Lotemax 0.5% QID
    • antibiotic drops QID
228
Q

what are some associations with keratoglobus?

A
  • occurs in families with h/o keratoconus and pellucid marginal degen
  • associations: collagen defects i.e. osteogenesis imperfect and Ehlers-Danlos; also Leber’s congenital amarurosis but unclear (?eye rubbing)
229
Q

what is the pathogenesis of keratoglobus? what is happening?

A
  • pathogenesis: uniform thinning from limbus to limbus, to 100-200um
    • focal breaks in Bowman’s
    • corneal hydrops and Descemet’s fractures are possible
230
Q

what can you do surgically to tx keratoglobus?

A
  • epikeratophakia: strip epithelium and button a graft on top -> reasonably effective/reasonable results
231
Q

posterior embryotoxon: what is it? what does it look like and where is it commonly seen?

A
  • posterior embryotoxon: prominent, anteriorly-displaced Schwalbe’s line (15% of adults)
    • circumlinear gray/white elevation of endothelium of peripheral cornea
      • most easily seen infero-temporal
232
Q

Axenfeld’s anomaly:

A

posterior embryotoxon and iris processes over the TM extending between Schwalbe’s line and scleral spur

233
Q

what is axenfeld’s syndrome?

A
  • Axenfeld’s syndrome = Axenfeld’s anomaly + secondaryglaucoma (or high IOP, depending on which doctor you ask)
234
Q

what is Axenfeld Reigers?

A
  • Axenfeld-Reiger’s: Axenfeld’s anomaly or syndrome and iris stromal hypoplasia
235
Q

what is Reiger’s syndrome? do they have glaucoma, and if so what % of pts have it?

A
  • Reiger’s syndrome = Reiger’s anomaly + associated systemic deformities
    • developmental defects of: face, teeth, bones -> hypoplasia of malar bones, broad flat nasal root, prominent supraorbital ridges, microdontia
      • mental developmental delay
    • 50% early glaucoma and cataracts
236
Q

how do you manage ICE conditions and conditions like Peter’s anomaly?

A
  • Tx inc IOP/glaucoma:
    • don’t use outflow enhancers: pilo and epi
    • reduce inflow: ABCs: beta blockers, carbonic anhydrase inhibs, alpha agonists
    • enhance uveoscleral outflow: prostaglandins help to some extent
    • surgery: trabeculectomy, because drops won’t work forever
  • cataract extraction
  • penetrating keratoplasty if bilateral corneal opacities
    • i.e. Peter’s syndrome, Chandler’s iridocorneal endothelial syndrome
237
Q

what are the 2 types of Peter’s anomaly:

A
  • type 1 = iridocorneal adhesions but no cornea/lens contact
  • type 2 = more severe = iridocorneal and lenticulocorneal adhesions
    • corneal leukomas (blindness results from these) can be seen
    • fairly severe, but even the less severe cases are sight-threatening
238
Q

what is Peter’s anomaly? what causes it? inheritance pattern?

A
  • Peter’s anomaly: a different disorder than anterior chamber dysgenesis
    • caused by homeobox genes -> present at birth
      • usually AR or sporadic, but dominant forms do occur
      • less common than Axenfeld-Rieger (which is less common than just Axenfeld)
239
Q

describe the pathogenesis of Peter’s: is it typically bilateral? what is the % of patients with glaucoma

A
  • 80% bilateral, 50% of patients have glaucoma
  • pathogenesis: iridocorneal or lenticulocorneal adhesions distinguish Peter’s from Axenfeld-Rieger
    • endo decompensated in areas of corneal touch – pumps not working
    • corneal edema/clouding (beyond fibrosis areas) -> scarring -> vascularized due to inflammatory processes
240
Q

Describe the typical ICE pt: age, gender…bi or unilateral?

A
  • patient profile: ~3:1 females, usually 30-50y
    • clinically unilateral, but probably have subclinical signs in fellow eye
241
Q

Describe the patho of ICE: what layer of the cornea is affected?

A
  • pathogenesis: aberrant endothelial cells
    • anomalous mesectodermal differentiation -> endothelial cells take on epithelial cells characteristics (i.e. actin filaments, etc.)
      • migration and contraction toward angle structures
      • get pleomorph, polymegath
242
Q

what do you see clinically w/ Chandler’s syndrome?

A
  • Chandler’s syndrome:
    • endothelial atrophy/“beaten metal” appearance, like very dense endothelial guttata
    • predominant corneal edema
    • 40% have iris atrophy (not key feature)
    • aberrant endo cells migrate over the angle and onto the iris – obstruct outflow which can increase IOP
      • can get PAS
243
Q

what do you see with essential iris atrophy?

A
  • 100% have iris atrophy
    • corectopia (dragged/displaced iris) – as the membrane contracts, it pulls the iris into the angle giving PAS and stresses the stroma (may not always be right next to the synechiae)
      • angle abnormalities can decrease outflow, give glaucoma
    • sometimes atrophy gives full-thickness defect -> pseudopolycoria
      • light shines through on retroillumination
244
Q

what do you see clinically w/ Cogan-Reese iris nevus syndrome? do they have iris atrophy?

A
  • iris surface nodules and/or matted whorl-like appearance -> loss of iris crypts and film of scaffolding over top
    • membrane over large area of iris plane – breaks up/atrophies in some places but not others
  • large sectoral nevi, heterochromia, ectropian uveae, 2* glaucoma
  • 50-60% have iris atrophy
245
Q

How do you manage/tx ICE?

A
  • tends to progress rapidly – change in angle, etc. within weeks to months
  • 100% develop glaucoma:
    • reduce aqueous inflow with B-block, CAIs, A-agonists
    • prostaglandins
    • usually end up at surgery, but it can be difficult because the anterior chamber may have a very shallow angle
  • penetrating keratoplasty if corneal edema is severe
  • deep lamellar endothelial keratoplasty -> this may be preferred to the glaucoma surgery also because of the angle complications
    • leftover endo cells may migrate in a few years -> this surgery is not a permanent fix for the syndrome
246
Q

what are some differentials for iris mamillations:

A

rule out Lisch nodules (if in a young pt, indicative of neurofibromatosis), Cogan-Reese syndrome, tapioca melanoma (flat lesion with lumpy parts), and inflammatory nodules

247
Q

what pop gets PMD? (pellucid)

A
  • 20-40y at onset (slightly older than keratoconus)
  • more often have family history of keratoconus or keratoglobus
248
Q

what are some causes of PMD? what layer is being affected?

A
  1. pathogenesis: unknown
    • Bowman’s is disrupted in the thin area
    • stroma thin also, but Descemet’s and endothelium are intact
    • has been known to develop after hyperopic LASIK because some of the peripheral corneal tissue is removed during this surgery
249
Q

describe presentation of PMD

A
  • bilateral, inferior ectasia of globe, crescent-shaped, very close to (not at) limbus
250
Q

TX for PMD?

A

cresentic wedge shaped resection – only in appropriate patients – cannot be too severe a case, many advantages over PK (quicker healing, endo sparing, less rejection, glauc, astig)

  • lamellar keratoplasty
  • penetrating keratoplasty
251
Q

Describe senile furrow degen and how it differs from PMD

A
  • older patients, 80y+
    • intact epithelium
    • no vascularization in area of thinning
    • right at the limbal border, as opposed to PMD – basically has no zone of normal cornea between thinning and limbus
      • also no corneal ectasia
252
Q

what is occurring in TMD (Terrien’s)? what is typical pt? where is it affecting the eye?

A
  • bilateral (86%) marginal thinning – starts shallow, leads to an abrupt ridge with lipid deposit in toward the pupil
  • pt: 20-30y (but can occur at any age – perhaps just doesn’t present to clinic until this age), 3:1 male
  • usually superior/sup-nasal at onset
253
Q

what are the 2 forms of TMD? what age ranges are affected?

A
  • quiescent: non-painful and slowly progressive, no infiltrates/redness, really more of a degeneration
    • usually seen in older patients
    • acuity may decrease due to ATR cyl
  • inflammatory: 10-30% of cases, occurs in younger patients; decreases VA
    • ± painful episcleritis or superficial scleritis – can lead to serious peripheral corneal thinning
254
Q

Describe the pathogen of TMD:

A
  • formerly grouped with senile furrow degeneration and PMD, but more recently this idea has changed
    • low-grade/subclinical inflammation against corneal antigens, similar to what occurs in Mooren’s ulcer
      • unclear what exactly is being responded to
255
Q

Describe the presentation and possible progression of TMD:

A
  • presentation: usually begins superior
    • starts as fine punctate speckled stromal opacities near the limbus
    • pannus also, leads to opacities of lipid deposition
    • progressive thinning
    • pseudopterygium may form
      • breakdown of limbal stem cells
      • MMP activation dissolves stromal glue to allow tissue wasting
    • breaks in Descemets = intracorneal aqueous pockets
    • rare for hydrops or perforation to manifest
256
Q

what is the goal in treating TMD? should you use steroids? what kind of surgery is available?

A
  • goal is to prevent corneal perforation
    • topical steroids if inflammation is present (not very common)
      • cautious because steroids can reduce keratoblast activity and perpetuate thinning
      • you don’t want to induce more melting by completely or long-term inhibiting fibroblasts
    • surgical approaches: cresentic full-thickness keratoplasty, cresentic lamellar keratoplasty
257
Q

Describe Mooren’s presentation: uni or bilat? what pop does it usually affect? age, gender, race etc

A
  • Mooren’s ulcer
    • unilateral or bilateral
    • painful peripheral corneal ulceration and ectasia
      • limbal flush
    • males 2-5:1 over females
    • highest incidence: China and Africa
    • any age of onset possible, but most between 40 and 70y.
258
Q

Describe Mooren’s patho:

A
  • many etiologies
    • vicious and aggressive immune attack against corneal antigens
    • immune complex deposition/helminth infections
    • molecular mimicry with hepatitis C virus
259
Q

describe presentation/prog of Mooren’s:

A
  • “exquisitely painful”
  • begins at limbus -> progresses circumlinearly and centrally towards pupil
  • overhanging central epithelial flap
  • rate of progression is variable -> recurrent, waxing and waning, or chronic
  • 13% perforation
    • descemetocele within ulcer possible
  • anterior uveitis is accompanying condition
260
Q

Describe 1st, 2nd, 3rd line tx for Mooren’s: Surgical tx?

A
  • 1st line: aggressive topical steroids and cycloplegia, systemic immune-suppression
    • short term: systemic steroids
    • long term: methotrexate (probably too strong), cyclophosphamide (alkylating agent), azathioprine and cyclosporine
    • oral tetracycline
  • 2nd line: likely better – lamellar keratoplasty, 1% cyclosporine A
    • removes antigenic tissue
  • 3rd line: anti-tumor necrosis factor agents, topical interferon alpha
  • surgery: conj resection, keratoepithelioplasty
261
Q

when do you show signs vs symptoms w/ MEESman’s?

A
  • clinical signs by 1, but clinical symptoms (RCEs) in middle age most commonly
  • AD condition
262
Q

Describe appearance of Meesman’s on slit lamp:

A
  • on retroillumination, looks like oil droplets
    • with direct focal, may be optically dense appearing gray and putty-like
263
Q

describe pathogen of Meesman’s: what layer of cornea is affected? what went wrong? was it me? are mom and dad getting a divorce?

A
  • epithelial BM nodular thickening – something is wrong with manufacture
  • problem with structural rigidity of epithelial cells -> defective encoding of cytoskeletal intermediate filaments -> cell walls are fragile, degenerate prematurely -> crumble and the debris coalesces into vacuoles/microcysts
  • *Bowman’s layer is normal
264
Q

what are some assoc symptoms w/ Meesman’s?

A
  • Sx later in life: glare, photophobia, recurrent erosions, decreased VA
265
Q

List some diff for Meesman’s:

A
  • DDx: microcystic edema, Map-Dot dystrophy
    • Meesman’s is caused by epithelial cell wall structural defects, is present early in life
266
Q

How might you tx Meesman’s?

A

good results with PTK

267
Q

describe pt profile of EBMD:

A
  • usually onset after 40y
  • bilateral but asymmetric and occurring in a single layer
  • many sporadic cases, but autosomal dominant with incomplete penetrance
268
Q

describe what gets fucked up in EBMD:

A
  • incompetent hemidesmosomes do not anchor basal epithelial cells properly to an aberrant BM
  • epithelial cell maturation and migration is altered
  • BM reduplicates, inter-epi cysts form
269
Q

app what % of RCE are caused by EBMD?

A
  • ~50% of RCE
270
Q

what is this?

A

spheroidal degen

271
Q

describe 1st, 2nd, 3rd line tx for RCE:

A
  • 1st line management:
    • hyperosmotics (5% NaCl) and lubricants -> maybe for ~30d
    • Lotemax QID x30d, then BID x30d
    • oral doxycycline 100mg x7d, then 50mg x60d
    • bandage CL if large and not healing in 24-48h
  • 2nd line: anterior stromal micropuncture
    • most patients can manage without, but very effective Tx
    • indicated in RCE due to trauma, PBK, EMBD
  • 3rd line: phototherapeutic keratectomy
    • “expensive but elegant and effective”
272
Q

when do you usually notice Reis Buckler/Thiel Behnke? (age?) genetics?

A
  • autosomal dominant, both RBD and TBD map to BIGH3 gene on 5q21 – a big homeobox gene controlling embryogenesis of the eye
  • onset between 1st and 2nd decade
273
Q

what layer is affected in Reis Buckler/TB? what is happening/!?

A
  • Bowman’s fragmented, thinned, totally absent -> keratocytes can go thru
  • irregular multilaminar sheets of fibrous pannus are deposited to replace Bowman’s – irregular pattern can give hills and valleys that disrupt epi
  • epi adherence is weak, can thin to 1-2 cells, leading to RCE
274
Q

How do you manage RB/TBenke?

A
  • RCE tx
  • best long-term solution may be PTK
  • penetrating and lamellar keratoplasty – risk of regrowth into the graft
    • most dystrophies start more anteriorly in the stroma and move backward through the cornea
    • don’t want to wait too long to refer to the surgeon – may be able to use lamellar keratoplasty if still within anterior 1/3 of stroma
275
Q

how are Reis-Buckler Dystrophy / Thiel Behnke Dystrophy diff?

A
  • RBD irregular or map pattern of comma-shaped gray/white opacities
    • TBD more reticular mesh/honeycomb opacity pattern

aside, both:

  • begin centrally -> progress to mid-periphery -> variable loss of acuity
  • decreased corneal sensitivity (DES, decreased blinks) – RCE common and easier to occur
276
Q

describe pt profile for Schnyder crystalline dystrophy: who does it affect, genetic, bi or unilat?

A
  • autosomal dominant
  • mostly affects N. European (esp. Sweden, Norway, Denmark, Finland) but is seen in all races
  • ~50% do not show crystals
  • bilateral, usually fairly symmetric
277
Q

describe pathogen of Schnyder crystalline dystrophy

A
  • local abnormal cholesterol mechanism -> lipids, neutral fats, cholesterol are deposited in the anterior stroma
278
Q

Describe the 3 stages of Schnyder’s

A
  • stage 1 (age 1-23y) -> central opacification, ±visible crystals (many times crystals too fine to see separately giving cloudy appearance), normal VA and corneal sensitivity
    • usually begins centrally
  • stage 2 (age 24-40y) -> arcus, limbal girdles, decreased VA secondary to increased central deposits, decreased corneal sensation as the nerves are affected by the crystal deposition
    • maybe xanthelasma and systemic cholesterol issues
  • stage 3 (age 40y+) -> dense central haze that is spreading peripherally, no edema but acuity and corneal sensation are decreased
279
Q

How do you tx Schnyder’s?

A
  • PTK
  • deep anterior lamellar keratoplasty – because this progresses from front to back, must be done early (i.e. during stage 1 or 2)
  • penetrating keratoplasty
  • systemic testing – serum lipid profile is important
280
Q

How can you distinguish gran dystrophy from mac dystrophy?

A
  • distinguishing features (from macular dystrophy):
    • crisp fleck borders ->flat-ish and irregular borders, or look like bread crumbs
    • clear intervening cornea
    • far periphery is not involved – clustering of deposits is central
281
Q

when do you see signs/symptoms for granular dystrophy?

A
  • signs usually by 2nd decade, but relatively slow progression with RCE/decreased VA not occurring until ~40y
  • pts not symptomatic if deposits are not dense or are outside visual axis
282
Q

what is Avellino dystrophy?

A
  • Avellino dystrophy-sub group of granular dyst
    • may appear more like Schnyder’s with crystalline deposits -> sometimes called combined granular-lattice dystrophy
    • only way to really know Avellino is via genetic test
283
Q

how do you tx granular dyst?

A
  • standard RCE protocol
  • if early – maybe superficial keratectomy or PTK but likely will recur
  • if dense, full thickness involvement:
    • penetrating keratoplasty
    • deep lamellar anterior keratoplasty
284
Q

Describe the 3 types of lattice dystrophy:

A
  • type 1 LD AD without systemic ass.
  • type 2 LD (Meretoja syndrome) is AD with systemic amyloidosis
    • decreased corneal and general sensation, stereotypical facial appeaarances, etc.
    • Dutch, Finnish, Scottish populations
    • ass w/ pseudoexfoliation and glaucoma
  • type 3 LD is AR and found in Japanese
285
Q

describe clinical appearance of lattice dystrophy, along with clin signs and symptoms:

A
  • **most common stromal dystrophy
  • dec VA
  • RCEs in the first decade
  • starts as beads > come together to more well-formed linear lattice lesions-> intervening stroma cloudy
    • amyloid is refractile so patients are very sensitive to lights and to glare due to scatter of incident light
  • lose corneal sensation
286
Q

how to tx lattice dystrophy?

A
  • early- PTK or superficial keratectomy – recurrence likely to occur
  • if dense and full-thickness involvement – PK
287
Q

tx for macular dystrophy?

A
  • standard RCE protocol
  • superficial involvement only: PTK or lamellar keratoplasty
  • penetrating keratoplasty -> more often PK than PTK or LK because macular dystrophy tends to involve the full stromal thickness
    • grafts often have MD recurrence
    • macular dystrophy is most common reason for PK in Iceland
288
Q

when does mac dystrophy start? what distinguishes it from gran dystrophy? what symptoms will pt have? what layers are affected?

A
  • onset 3-9y
  • at first, looks like granular dystrophy with nodules + fleck-like lesions but will change with time
    • by age 20, peripheral cornea will be involved
    • in between the opacities, the cornea will look cloudy
    • photophobia is pronounced, pts have a lot of glare sensitivity
    • edges of lesions are less clearly defined than in granular dystrophy
  • epi surface is irregular, cornea has thin and thick zones, change in contour
  • endothelial changes lead to guttata

OC DISEASE (2 decks)

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