Congenital Lens Opacities Flashcards

1
Q

what shape are the lens? is it cellular or acellular? It is a optically _____ structure?

A

biconvex, acellular, optically transparent structure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

how does the lens change shape?

A

by contraction of ciliary body

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

what is transparent basement membrane that surrounds the entire lens?

A

lens capsule

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

the lens capsule is mostly made up of?

A

collagen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

where do zonular fibers insert?

A

at the equator of the lens capsule

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

what happens to the anterior lens capsule as we age?

A

it thickens

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

where are mitotic epithelial cells located?

A

anterior lens only

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

where are new fibers generated? where do they migrate to? where do they meet?

A

at the equator → migrate to anterior & posterior lens → meet at the sutures

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

name the structures of the lens from innermost to outermost

A

embryonic nucleus → fetal nucleus → adult nucleus → cortex → anterior/posterior capsule

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

most congenital cataracts are unilateral or bilateral?

A

bilateral

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

what are congenital cataracts caused by?

A
  • chromosomal abnormalities
  • metabolic disorders
  • intrauterine infections (TORCH)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

what findings will you find with unilateral congenital cataracts?

A

no additional findings

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

which one has a better visual prognosis?

  • isolated congenital cataract
  • congenital cataract with ocular/systemic cormorbidity
A

isolated congenital cataract

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

what is the most common type of congenital cataracts?

A

cerulean (blue-dot)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

which congenital cataract is nonprogressive and presents as multiple, tiny blue scattered dots?

A

cerulean (blue-dot)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

in cerulean cataracts, where are the blue dots scattered in?

A

in the lens cortex

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

in cerulean cataracts, what causes the bluish color?

A

dispersion of light

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

when the opacities in a cerulean cataract occur in a ring-like configuration, what is that called?

A

coronary cataract

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

how do cerulean cataracts impact visual acuity?

A

no effect on VA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

which congenital cataract is caused by metaplasia of epithelial cells?

A

anterior polar cataract

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

which congenital cataract consists of fibrous connective tissue beneath an intact anterior lens capsule?

A

anterior polar cataract

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

which congenital presents as a sharply demarcated opacity beneath the anterior lens capsule?

A

anterior polar cataract

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

are anterior polar cataracts progressive or nonprogressive?

A

nonprogressive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

are anterior polar cataracts unilateral or bilateral?

A

can be either or

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

how do anterior polar cataracts affect visual acuity?

A

can be amblyogenic due to refractive error when cataracts are unilateral

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

what are the refractive errors causing amblyopia in a unilateral anterior polar cataract?

A

hyperopic anisometropia & astigmatism

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

which congenital cataract can be sporadic or familial, & caused by dysplastic lens fibers that adhere to the posterior capsule?

A

posterior polar cataract

28
Q

posterior polar cataracts can be associated with what?

A
  • persistent hyaloid remnants (Mittendorf dots)
  • posterior lenticous
  • persistent anterior fetal vasculature
29
Q

which congenital cataract presents as a round discoid plaque that is clearly demarcated from the rest of the lens?

A

posterior polar cataract

30
Q

what impact do posterior polar cataracts have on visual acuity?

A

usually no effect on VA, unless they progress

31
Q

if posterior polar cataracts do progress, when do they start to progress?

A

usually in middle age

32
Q

what is the risk in cataract surgery for a posterior polar cataract?

A

high risk of posterior capsule rupture

33
Q

which congenital cataract is usually bilateral & heriditary?

A

zonular (lamellar) cataract

34
Q

what are zonular (lamellar) cataracts associated with?

A
  • low vitamin D
  • hypocalcemia
  • maternal malnutrition
35
Q

which congenital cataract presents as an opacification of the zone around the embryonic nucleus?

A

zonular (lamellar) cataracts

36
Q

where are zonular (lamellar) cataracts usually found?

A

usually in area of the fetal nucleus

37
Q

zonular (lamellar) cataracts may also present with what kind of opacity? what are they called?

A

linear opacities may run outwards (aka riders)

38
Q

what impact do zonular (lamellar) cataracts have on visual acuity?

A

decreased VA is common → necessitating surgery

39
Q

which congenital cataract will present with a clear area of lens within & outside of the opaque zone?

A

zonular (lamellar) cataracts

40
Q

which congenital cataract is usually caused by a genetic mutation?

A

nuclear cataract

41
Q

which cataract is associated with ocular abnormalities such as microphthalmia, nystagmus, strabismus, & iris hypoplasia?

A

nuclear cataracts

42
Q

nuclear cataracts involve which structure(s) of the lens?

A

can involve entire nucleus or portion of the nucleus

43
Q

what impact do nuclear cataracts have on visual acuity?

A

results in decreased VA

44
Q

which congenital cataract is caused by an autosomal recessive disorder that impairs galactose utilization caused by absence of the enzyme?

A

oil droplet cataract

45
Q

in oil droplet cataracts, what must be done to prevent severe systemic problems that will result in early death?

A

withholding galactose products from diet

46
Q

in oil droplet cataracts, exclusion of galactose in diet can do what?

A

may reverse early lens changes

47
Q

what impact do oil droplet cataracts have on visual acuity?

A

results in lenticular myopia

48
Q

what congenital cataract presents as an opacity following the anterior/posterior Y suture?

A

sutural cataract

49
Q

where are the Y sutures located?

A

in the fetal nucleus

50
Q

are sutural cataracts progressive or nonprogressive?

A

nonprogressive

51
Q

what impact do sutural cataracts have on visual acuity?

A

no effect on vision, unless associated with other lens opacities that can affect VA

52
Q

what are the three congenital opacities that aren’t cataracts?

A
  • Mittendorf Dot
  • epicapsular star
  • persistent fetal vasculature
53
Q

which congenital lens opacity is a posterior capsule remnant of hyaloid artery?

A

Mittendorf dot

54
Q

are Mittendorf dots progressive or nonprogressive?

A

nonprogressive

55
Q

where are Mittendorf dots frequently located?

A

inferonasally on posterior lens surface

56
Q

what impact do Mittendorf dots have on visual acuity?

A

no effect on VA

57
Q

what congenital lens opacity presents as brown stellate opacities on the anterior capsule?

A

epicapsular star

58
Q

what congenital lens opacities are remnants of embryonic vascular network?

A

epicapsular stars

59
Q

are epicapsular stars progressive or nonprogressive?

A

nonprogressive

60
Q

what impact do epicapsular starts have on visual acuity?

A

no effect on VA

61
Q

what congenital lens opacity is caused by failure of the fetal hyaloid vasculature to regress?

A

persistent fetal vasculature

62
Q

is persistent fetal vasculature unilateral or bilateral?

A

almost always unilateral

63
Q

what is persistent fetal vasculature associated with?

A

microphthalmia

64
Q

what is the persistence of anterior hyaloid artery & abnormal tissue proliferation (retinal glial cells)?

A

persistent fetal vasculature

65
Q

what are the variable degrees of vascular remnants?

A
  • isolated retrolenticular membrane

- complete stalk connecting the optic nerve to the lens