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Pediatric Optometry Final Exam > Pediatric Ocular Disease > Flashcards

Pediatric Ocular Disease Flashcards

1
Q

What is the most likely etiology of congenital cataracts

A

unknown (up to 80%)

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2
Q

What are the 4 types of congenital cataracts

A

zonular, polar, nuclear, posterior lentoconus

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3
Q

What are the signs/ symptoms of a congenital cataract?

A

opacity of lens at birth, leukocoria, nystagmus (one or both eyes), strabismus, No APD

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4
Q

When would we want cataract extraction performed?

A

within days to weeks

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5
Q

What are 2 potential complications to congenital cataracts

A

amblyopia, glaucoma

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6
Q

According to the IATS study, what percent of children with congenital cataracts became glaucoma suspects or developed glaucoma?

A

33%

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7
Q

According to IATS, how did the costs of Surgery with IOL implant compare with costs of CL?

A

CL are more than double

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8
Q

Which type of retinoblastoma is curable?

A

Orbital disease

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9
Q

If retinoblastoma is _______________, prognosis is dismal

A

intracranial

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10
Q

What are the characteristics of PHPV?

A

often vascularized, can cause vascular traction, microphthalmos, shallow anterior chamber, cataract and persistent hyaloid artery

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11
Q

PHPV is the failure of the ___________ ___________ to regress

A

primary vitreous

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12
Q

What age is retinoblastoma usually diagnosed?

A

before age 2

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13
Q

Retinoblastoma is usually bilateral/unilateral

A

unilateral (75%)

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14
Q

What patients tend to have more advanced Retinoblastoma in the US?

A

hispanic, lower socionomic status

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15
Q

Retinoblastoma grows under the retina towards _________

A

vitreous

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16
Q

What are the 4 presenting signs of retinoblastoma?

A

leukocoria, strabismus, red painful eye with glaucoma, and poor vision

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17
Q

A DFE done under anesthesia with scleral depression is useful when looking for which disease?

A

Retinoblastoma

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18
Q

genetic causes of retinoblastoma is alteration in which gene?

A

RB1

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18
Q

When retinoblastoma is found in older children and unilateral it is usually n___-____________

A

non-heritable

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18
Q

all b___________ cases are presumed

A

heritable

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19
Q

What type of retinoblastoma causes the most deaths due to Retinoblastoma?

A

trilateral retinoblastoma

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20
Q

Decreased deaths and less eyes requiring enucleation due to retinoblastoma can be attributed to what

A

advances in treatment

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21
Q

What are the intraocular treatments for retinoblastoma?

A

chemotherapy/ chemoreduction; radiation therapy; laser therapy; brachytherapy; cryotherapy; enucleation

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22
Q

What are the extraocular treatments for retinoblastoma?

A

chemo or radiation directed at where the extension is; rarely gets this far in high income countries; often fatal if tumor spreads beyond the eye

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23
Q

Medical conditions (can be severe/ life-threatening); diminished orbital growth; visual field defects and hearing loss are all late effects that can happen following treatment for what disease?

A

Retinoblastoma

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24
Q

What are ocular findings of Stickler’s Syndrome?

A

high myopia, retinal detachments, cataracts, vitreous degeneration, glaucoma, pigmentary changes in the retina

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24
Q

What is the most common cause of retinal detachment in the pediatric population?

A

trauma (others: proliferative retinopathy, myopica, aphakia, ROP, lattice)

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25
Q

Coat’s Disease is a peripheral retinal ______________ with associated ______________

A

telangiectasia; exudation

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26
Q

Is Coat’s Disease unilateral or bilateral?

A

unilateral

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27
Q

What can Coat’s Disease lead to?

A

serous retinal detachment

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28
Q

How does toxocoriasis affect the retina?

A

It causes retinal granuloma associated with inflammation

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29
Q

Toxocoriasis is usually unilateral/ bilateral?

A

unilateral

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30
Q

What do myelinated nerve fibers look like?

A

unilateral, superficial, white retinal lesion with feathery borders

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31
Q

What type of refractive error will a patient with myelinated nerve fibers have if they also have decreased VAs?

A

High myopia

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32
Q

What type of visual field defect is seen in patients with myelinated nerve fibers?

A

scotoma

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33
Q

What conditions make up the MAC spectrum of ocular malformations?

A

microphthalmia, anophthalmia, coloboma

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34
Q

What is it called when the choroidal fissure fails to close?

A

coloboma

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35
Q

What direction do we typically see colobomas?

A

nasal

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36
Q

When do colobomas typically form?

A

between the 5th and 7th week of pregnancy

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37
Q

What symptom is associated with an iris coloboma?

A

light sensitivity

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38
Q

What symptom is associated with a macular or ON coloboma?

A

vision loss

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39
Q

What type of coloboma is associated with a visual field defect?

A

retinal coloboma

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40
Q

What conditions are associated with colobomas?

A

heterochromia, microphthalmia, cataracts, glaucoma, nystagmus, staphyloma

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41
Q

What genes are associated with colobomas?

A

PAX2, PAX6

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42
Q

What is CHARGE syndrome?

A

coloboma, heart anomaly, atresia of chonae, retardations, genital anomalies, and ear/ hearing abnormalities

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43
Q

What is it called when there is an additional chromosome 22?

A

Cat-Eye Syndrome

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44
Q

Goldenhard syndrome is also known as oculo-auriculo-vertebral syndrome and is associated with skin tags located where?

A

pre-auricular

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45
Q

What are the treatments for colobomas?

A

glasses, amblyopia treatment, CL or surgery for iris colobomas, retinal coloboma management (neo, RD, etc), low vision devices, genetic counseling

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46
Q

Which Retinal diseases present in infancy

A

retinopathy of prematurity, Leber’s Congenital Amaurosis, Congenital Stationary Night Blindness, Achromatopsia, foveal hypoplasia

47
Q

What is growing in the retina of premature infants with ROP?

A

abnormal blood vessels

48
Q

How are retinal blood vessels different in premature infants with ROP vs without ROP?

A

ROP: blood vessel growth stops after birth, so they do not extend to the edges of the retina

49
Q

What is released to stimulate growth of blood vessels in areas of the retina that are avascular?

A

VEGF

50
Q

In an eye with ROP…

What can happen if there is scarring in the retina?

A

retinal detachment and vision loss

51
Q

What are risk factors for ROP?

A

low birthweight (under 1500 grams or 3 lbs is high risk); premature birth (32 weeks or earlier); supplemental oxygen or mechanical ventilation, severe illness at birth

52
Q

Where are the 3 retinal zones for classification of ROP?

A

Zone 1: closest to the optic nerve and macula (worse prognosis)
Zone 2: mid-peripheral
Zone 3: far periphery on temporal side

53
Q

There are 5 stages of ROP, what is a common vascular characteristic of stages 3, 4 and 5

A

Plus Disease: thickened and tortuous blood vessels

54
Q

Which stages of ROP resolve without treatment?

A

1 and 2

55
Q

Which stage of ROP typically requires treatment?

A

stage 4

start to consider treatment at stage 3

56
Q

In stage 1, there is a distinct border between what two types of tissue in the retina?

A

vascularized and avascular

57
Q

What forms in stage 2 of ROP?

A

elevated ridge with neovascularization of the ridge

58
Q

In stage 3 of ROP, the fibrovascular tissue extends from the ridge into the _____________

A

vitreous

59
Q

Stages 4 and 5 are characterized by what retinal complication?

A

retinal detachment

60
Q

What type of retinal detachment occurs in stage 4 of ROP?

A

Partial (may or may not involve the macula)

61
Q

What is the VA during stage 5 of ROP?

A

LP or NLP

62
Q

What type of RD is there in stage 5 of ROP?

A

total RD

63
Q

What treatment was found to prevent severe vision loss and RD in 50% of stage 3 ROP patients?

A

cryotherapy

64
Q

What is the preferred treatment for ROP?

A

laser photocoagulation

65
Q

What are complications of ROP?

A

poor BCVA, Myopia, strabismus, amblyopia, glaucoma, nystagmus, and cataracts

66
Q

What is Leber’s Congenital Amaurosis?

A

severe visual impairment present at/ or within 2 month of birth

67
Q

What type of refractive error is associated with Leber’s Congenital Amarousis

A

high hyperopia

68
Q

The pupillary light response is brisk/poor in Leber’s Congenital Amarousis

A

poor

69
Q

How does a Leber’s fundus change from birth through adolescence?

A
  • birth: normal;
  • after birth: white flecks, pigment clumping, macular pigment dysplasia;
  • by adolescence: pigmentary retinopathy, vessel attenuation, optic atrophy

Looks like Retinitis pigmentosa

70
Q

Vision loss with congenital stationary night blindness is variable, but usually not worse than ________

A

20/200

71
Q

Which inheritance pattern shows symptoms later in congenital stationary night blindness?

A

AD (presents later with night blindness and normal VA)

72
Q

foveal hypoplasia may be an isolated finding or associated with which other conditions?

A

albanism and aniridia

73
Q

What are the ocular findings associated with foveal hypoplasia?

A

reduced vision, nystagmus, poorly developed macula/ fovea

74
Q

Stargardt’s disease, Best disease, X-linked retinoschisis, cone dystrophy and Retinitis pigmentosa are all retinal diseases that show up in infancy/childhood/adulthood

A

childhood

75
Q

Stargardt’s disease progresses ___________

A

slowly

presents between ages 8-14

76
Q

What VA does Stargardt’s eventually progress to by age 30?

A

20/200

77
Q

How does the macula appear early in Stargardt’s versus later in the disease?

A
  • Early: normal fundus;
  • later: loss of foveal light reflex, then round pigmented macular atrophy

differential: retinitis pigmentosa

78
Q

The abnormal accumulation of lipfuscin in the RPE of a patient with Stargardt’s causes what to happen in the periphery of the retina?

A

yellowish/ white flecks

no loss of acuity unless the macula is involved

79
Q

How does vision change when someone has Best Disease?

Best Disease: childhood retinal disease

A

Normal vision early followed by decreased central vision and metamorphopsia with normal peripheral vision

80
Q

This yellow yolk-like macular lesion is found in which childhood retinal disease?

A

Best Disease

usually bilateral, but can occasionally be unilateral

25% of patients may present with multiple lesions outside the macula

81
Q

Consider the EOG findings of a patient with Best Disease

During dark adaptation, the resting potential decreases slightly and during light adaptation, there is a substantial increase of resting potential. What is the effect on the Arden ratio?

Arden Ratio: light peak/ dark trough

A

Decreases

< 1.5; often 1.0-1.3

Normal:~>1.8 but decreases with age

81
Q

What is the inheritance pattern for Best’s Disease?

A

Autosomal dominant

82
Q

A higher stage number for Best Disease indicates milder/ more severe disease

A

more severe

83
Q

Reduced VA’s start at which stage of Best Disease?

A

2a

84
Q

Which stage of Best Disease is the pseudohypopyon phase?

A

stage 3

pseudohypopyon: yellow-colored vitelline substance in lesion
85
Q

What is the VA associated with Stage 4c of Best disease?

A

20/200 or worse

86
Q

4 Treatment options for Best Disease

A
  1. Laser Photocoagulation
  2. Anti-VEGF
  3. Low vision aids
  4. Genetic counseling
87
Q

A spoke like appearance to the fovea is seen in which childhood retinal disease?

A

X-linked Retinoschisis

Also: peripheral retinoschisis (50%: usually inferior temporal) and vitreous hemorrhages, NFL breaks and retinal detachment associated

88
Q

Salt and pepper, bone spicules, optic atrophy and vessel attenuation are all retinal signs of which childhood retinal disease?

A

Retinitis pigmentosa

looks like Leber’s

89
Q

The SC form of Sickle Cell has more systemic/ ocular complications

A

ocular

90
Q

What are 3 anterior segment findings associated with sickle cell?

A

“comma” shaped conjunctival sickling sign; iris atrophy, hyphema

91
Q

Salmon patches and black sunburst spots are non-proliferative signs of sickle cell found in the anterior/posterior segment

A

posterior

92
Q

Name 2 proliferative posterior segment associated with sickle cell disease

A

AV anastomoses (hair-pin loop); neovascularization (sea fan)

93
Q

What age should we start screening children with the SC form of sickle cell for ocular findings

SC: more ocular findings

perform DFE annually; FA on abnormal eyes

A

beginning at age 9

94
Q

Is Papilledema or pseudopapilledema more common in pediatrics?

A

pseudopapilledema

causes: hyperopia, optic nerve drusen; hyperplastic glial tissue

95
Q

Derangements of mitochondrial metabolism causes the majority of what?

A

Optic neuropathies

96
Q

What is the most common form of hereditary optic atrophy?

A

autosomal dominant optic atrophy

mutations in OPA1 and OPA3 gene

Vision loss occurs between 4-8 years old

97
Q

Which form of optic atrophy is associated with nystagmus?

A

recessive optic atrophy

98
Q

Is toxic/ nutritional optic neuropathy bilateral and asymmetric or symmetric?

A

symmetric

99
Q

Why does radiation therapy cause optic neuropathy in children?

radiation therapy administered for retinoblastoma or craniopharyngioma tumors of childhood

A

difficult to shield optic nerves from the field of radiation

cumulative dose can increase risk of ON

ON can occur weeks to years after treatment

100
Q

Atrophic discs are normal/ large/ small in size

A

normal

diminution of number of axons, with normal vessels and glial tissue

cuased by late gestational injury

101
Q

Optic Nerve Hypoplasia has a normal/ smaller/ larger optic disc than expected

A

smaller

caused by early gestational injury

presents bilaterally

102
Q

What is pathognomonic for ONH?

A

Double ring sign: hypo/hyperpigmented area surrounding the disc

may also present with pendular nystagmus

103
Q

How can yoked prism help a child with nystagmus?

A

May be able to move eyes to null point: dampen abolish nystagmus and have best VA

104
Q

Which type of nystagmus has the following characteristics?
* reversed OKN
* absence of oscillopsia
* Null point
* reduced VA’s before 24 months
* nystagmus less evident with age

A

Idiopathic infantile nystagmus

105
Q

A frosted occluder or high plus lens is helpful when refracting for which type of nystagmus?

A

latent

106
Q

What are the 5 A’s?

common conditions associated with sensory nystagmus

_s_ensory nystagmus is _s_econdary

A

1.** A**niridia
2. Achromatopsia
3. Albinism
4. Optic Nerve Atrophy
5. Leber’s Amaurosis

sensory nystagmus is ALWAYS associated with another ocular condition

107
Q

What is the triad for spasmus nutans?

A
  1. nystagmus
  2. head torticollis
  3. head nodding

onset: always after 6 months; between 1-3 years most common

spontaneously disappears by 5-12 years of age

108
Q

What is the frequency and amplitude for spasmus nutans?

A

high frequency, small amplitude

worse in abducting eye

asymmetric, intermittent

109
Q

What type of imaging needs to be ordered for all children with high frequency dissociated nystagmus?

A

MRI of anterior visual pathway

110
Q

Is acquired nystagmus more common in children or adults?

Types: upbeat and downbeat; gaze-evoked; see-saw; periodic alternating; dissociated

A

adults

also associated with oscillopsia

111
Q

What are the benefits of Botox for nystagmus?

A

reduces nystagmus; improves vision

111
Q

When would BU prism be used for nystagmus?

A

When nystagmus dampens with convergence

112
Q

Is ocular abinism more common in boys or girls?

A

Boys

x-lined inheritance

113
Q

Which type of oculocutaneous albanism has a more reduced BCVA?

A

OCA1

no pigment; no active tyrosinase

VA: 20/200-20/400

114
Q

OCA1b is more/less severe

A

less

pigment increases slightly throughout life

residual tyrosinase activity and some pigment

115
Q

Which is the most common type of albanism?

A

OCA2

more pigment, better vision

fair skin, not as pale as OCA1

116
Q

How does nystagmus associated with albanism change throughout life?

A

starts out pendular, followed by jerk type

any type is possible

Pediatric Optometry Final Exam (3 decks)

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