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Anterior Segment Disease > 3: Developmental Abnormalities > Flashcards

3: Developmental Abnormalities Flashcards

1
Q

lenticonus

A

cone-shaped lens protruding anteriorly or posteriorly

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2
Q

lenticonus laterality

A
  • anterior: bilateral

- posterior: unilateral

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3
Q

lenticonus treatment

A

surgical removal of lens (lensectomy)

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4
Q

lenticonus pearls:

-can ____ with age

A

progress

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5
Q

microspherophakia

A

small, spherical lens in which the equator of the lens is visible with full pupillary dilation

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6
Q

microspherophakia systemic associations

A

Marfan syndrome

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7
Q

microspherophakia pearls:

-lens may move and _____

A

block the pupil

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8
Q

microspherophakia treatment

A
  • cycloplegics are treatment of choice; miotics may lead to pupillary block
  • treat other complications as they arise
  • clear lens extraction may be necessary
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9
Q

congenital cataract: lamellar

A

alternating clear and white cortical lamellar opacities

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10
Q

congenital cataract: cerulean

A

blue opacities

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11
Q

congenital cataract: sutural

A

opacification of the anterior or posterior Y sutures

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12
Q

congenital cataract: polar

A

central opacity in the anterior or posterior capsule

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13
Q

congenital cataract laterality

A

unilateral or bilateral

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14
Q

congenital cataract pearls:

-typically, opacities are _____ and _____ vision

A

stable;

do not interfere with

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15
Q

Mittendorf dot

A

remnant of the hyaloid artery on the nasal posterior lens capsule

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16
Q

Mittendorf dot laterality

A

unilateral or bilateral

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17
Q

Mittendorf dot pearls:

-typically, ______ vision

A

does not interfere with

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18
Q

epicapsular stars

A

remnant of the tunica vasculosa lentis on the anterior lens capsule

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19
Q

epicapsular stars laterality

A

unilateral or bilateral

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20
Q

epicapsular stars pearls:

-typically, ______ vision

A

does not interfere with

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21
Q

persistent pupillary membrane (PPM)

A

remnant of the tunica vasculosa lentis on the anterior iris

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22
Q

persistent pupillary membrane (PPM) laterality

A

unilateral or bilateral

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23
Q

persistent pupillary membrane (PPM) pearls:

-typically, ______ vision

A

does not interfere with

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24
Q

Brushfield spots

A

aggregation of collagen in the iris stroma

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25
Q

Brushfield spots laterality

A

bilateral

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26
Q

Brushfield spots systemic associations

A

Down syndrome

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27
Q

Brushfield spots pearls:

  • occurs in ____% of normal patients (called _____)
  • more apparent in ____
A

10-24%;
Kunkmann-Wolffian bodies;
lighter irises

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28
Q

aniridia

A

total or near-total absence of the iris

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29
Q

aniridia laterality

A

bilateral

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30
Q

aniridia pearls:

  • 90% of cases develop ____
  • 75% of cases develop ____
  • _____ are common
  • 25% of cases will develop _____
A

aniridic-related keratopathy (corneal changes- pannus, ulceration, scarring- occur in early teenage years);
synechial angle-closure glaucoma;
macular hypoplasia and nystagmus;
Wilms’ tumor (kidney cancer)

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31
Q

hypertelorism

A

increased distance between eyes and orbit; increased pupillary distance

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32
Q

telecanthus

A

increased distance between the medial canthi; normal pupillary distance

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33
Q

telecanthus systemic associations

A

fetal alcohol syndrome

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34
Q

telecanthus pearls:

-may also be the result of _____

A

nasoorbitoethmoidal fracture

35
Q

telecanthus treatment

A

surgical treatment is possible but recurrence is common

36
Q

anopthalmos (anopthalmia)

A

absence of the globe

37
Q

anopthalmos (anopthalmia) laterality

A

unilateral or bilateral

38
Q

anopthalmos (anopthalmia) pearls:

  • the globe may be replaced by _____
  • may also be due to _____
A

a cyst;

enucleation, evisceration, or extenteration

39
Q

microphthalmos (microphthalmia)

A

small, malformed globe

40
Q

microphthalmos (microphthalmia) laterality

A

unilateral or bilateral

41
Q

microphthalmos (microphthalmia) meanagment

A
  • treatment of refractive error is critical; prevent amblyopia
  • surgical reconstruction
42
Q

buphthalmos

A

enlarged globe due to elevated IOP before birth or during the first 3 years of life

43
Q

buphthalmos laterality

A

unilateral or bilateral

44
Q

buphthalmos management

A

management of IOP is critical

45
Q

cryptophthalmos

A

hidden globe due to poor formation of the eyelids; skin stretches from forehead to cheek

46
Q

cryptophthalmos laterality

A

unilateral or bilateral

47
Q

microcornea

A

adult horizontal corneal diameter < 11 mm

48
Q

microcornea laterality

A

unilateral or bilateral

49
Q

microcornea pearls:

-increased incidence of ____

A

angle closure/narrow angle glaucoma

50
Q

megalocornea

A

adult horizontal corneal diameter > 13 mm

51
Q

megalocornea laterality

52
Q

sclerocornea

A

scleralization and vascularization of the peripheral or entire cornea

53
Q

sclerocornea laterality

54
Q

cornea plana

A

severely flat corneal curvature, where the sclera and cornea have the same curvature

55
Q

cornea plana laterality

56
Q

cornea plana treatment

A
  • manage refractive error (hyperopia)

- monitor for glaucoma

57
Q

posterior embryotoxin

A

anteriorly displaced Schwalbe’s line; seem as creamy-white thickened line in the corneal periphery

58
Q

posterior embryotoxin laterality

59
Q

posterior embryotoxin pearls:

  • occurs in _____% of normal patients
  • spectrum of conditions that have posterior embryotoxin as a finding: ______
A

~15;

  • Axenfeld anomaly (posterior embryotoxin + attached iris strands)
  • Axenfeld syndrome (Axenfeled anomaly + glaucoma; 50% of Axenfeld anomaly develop glaucoma)
  • Rieger anomaly (Axenfeld anomaly + iris stromal hypoplasia; corectopia and entropion uveae may also be present)
  • Rieger syndrome (Rieger’s anomaly + dental and/or facial malformations)
60
Q

Peters’ anomaly

A

central corneal opacity, usually with iris strands that extend from the collarette to a posterior corneal defect behind the scar

61
Q

Peters’ anomaly laterality

62
Q

Peters’ anomaly treatment

A

standard treatment is PKP

63
Q

Peters’ anomaly pearls:

-50% of cases develop _____ due to ____

A

glaucoma; abnormal development of the TM and Schlemm’s canal and/or the presence of a shallow anterior chamber

64
Q

epiblepharon

A

horizontal fold of skin across the eyelid margin

65
Q

epiblepharon laterality

66
Q

epiblepharon pearls:

  • more commonly affects the ____
  • common in _____
A

LL;

Asian infants, typically outgrown with facial bone growth

67
Q

epicanthal folds

A

vertical fold of skin at the medial (and sometimes lateral) canthus

68
Q

epicanthal folds laterality

69
Q

epicanthal folds pearls:

  • can create the appearance of _____
  • common finding in _____
A

esotropia;

Asians and infants (in infants, typically outgrown with facial bone growth)

70
Q

ablepharon

A

absence of eyelids

71
Q

ablepharon laterality

72
Q

microblepharon

A

vertical shortening of the eyelids

73
Q

microblepharon laterality

74
Q

euryblepharon

A

horizontal elongation and vertical shortening of the eyelids

75
Q

euryblepharon laterality

76
Q

euryblepharon management

A
  • conservative; topical lubrication

- surgical correction may be indicated

77
Q

ankyloblepharon

A

partial or complete fusion of upper and lower eyelid margins

78
Q

ankyloblepharon laterality

A

unilateral or bilateral

79
Q

ankyloblepharon pearls:

-may also be the result of ____

A

cicatrizing disease

80
Q

blepharophimosis syndrome

A

4 major facial features:

  • blepharophimosis: horizontal shortening of the palpebral fissures (eyes appear more narrow)
  • epicanthus inversus
  • telecanthus
  • ptosis
81
Q

blepharophimosis syndrome laterality

82
Q

blepharophimosis syndrome treatment

A

surgical management

83
Q

coloboma

A

defect in the eyelid or iris (can also affect the retina, choroid, and ON) due to failure of complete closure of the embryonic fissure

84
Q

coloboma laterality

A

unilateral or bilateral

Anterior Segment Disease (22 decks)

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