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Flashcards in Peds Deck (161):
1

Embryology - 22 days

Optic primordium appears in neural folds

2

Embryology - 25 days

Optic Vesicle evaginates. Neural crest cells migrate to surround vesicle.

3

Embryology - 28 days

Vesicle induces lens placode

4

Embryology - 2nd Month

Invagination of optic and lens vesicles
Hyaloid artery fills embryonic fissure
Pigment granules appear in RPE
lateral rectus and superior oblique muscles grow anteriorly
Eyelid folds appear
Retinal differentiation begins
Migration of retinal cells begins
Neural Crest cells of corneal endothelium migrate centrally (corneal stroma follows)
cavity of lens vesicle is obliterated
Secondary vitreous surrounds hyaloid system
Choroidal vasculature develops
Axons from ganglion cells migrate to optic nerve
Glial Lamini cribrosa forms
Bruch's membrane appears

5

Embryology 3rd Month

Precursors of rods and cones differentiate
Anterior rim of optic vesicle grows forward and CB starts to develop.
Sclera condenses
Vortex veins pierce sclera
Eyelid folds meet and fuse

6

3 types of XT

- convergence insufficiency
- divergence excess
- primary/idiopathic

7

Embryology -4th month

retinal vessels grow into RNFL at optic disc
Folds of ciliary processes appear
Iris sphincter develops
Descemets membrane forms
Schlemms canal appears
Hyaloid system starts to regress
Glands and Cilia develop

8

Embryology 5th Month

Photoreceptor inner segments develop
Choroidal vessels form
Iris stroma is vascularized
Eyelids begin to separate

9

Embryology - 6th Month

Ganglion cells thicken in the macula
Recurrent Atrerial brances joing the choroidal vessels
Dilator muscle forms

10

Embryology - 7th Month

Outer segments of photoreceptors differentiate
Central fovea starts to thin
Fibrous lamina cribrosa forms
Choroidal melanocytes produce pigment
Circular muscle forms in CB

11

Embryology - 8th Month

Chamber angle completes formation
Hyaloid system disappears

12

Embryology - 9th Month

Retinal vessels reach periphery
Myelination of nerve fibers are complete to the lamina
pupillary membrane dissapears

13

What is the name for the celluar area of retinal proliferation at the leading edge of the vasculature in the ember

Vascular Anlage

14

Types of abnormal small eyes

Microphthalmos with cyst. - clinically when you open the lids the cyst tends to be infront as a retina

15

Embryonic Fissure

Closure at 6 weeks - closes inferonasally (This is where we get colobomas

16

Ocular features of Trisomy 13

Microphthalmia, lens is catatractous, persistant hyaloid primary vasculature with cartilage behind lens, dyspalastic retina.
Die by 6 months.
Part of ddx of intra-ocular cartilege, and retained lens nuclei

17

Synophthalmia

Associated with Holoprosencephaly and the

18

Acardi Syndrome

rare genetic malformation syndrome characterized by the partial or complete absence of a key structure in the brain called the corpus callosum, the presence of retinal abnormalities (peripapillary retinal colobomas), and seizures in the form of infantile spasms. Aicardi syndrome is theorized to be caused by a defect on the X chromosome as it has thus far only been observed in girls or in boys with Klinefelter syndrome.

19

Peters Anomaly

Type 1 – posterior defect - leukoma plus iridocorneal adhesions
Type 2 – leukoma + iridocorneal adhesions + lenticulocorneal adhesions
Peters Plus – above + renal, cardiac, genitourinary, cleft palate.

Pathology - Look for abscence of DM.
RECALL: DM is PAS +'ve and so is the lens capsule, look for both of them.
AD - PAX6

Long term sequelae includes posterior keratoconnus

20

Tuberous Sclerosis

Astrocytic hamartoma

21

Pilomatrixoma

Occur in children - tend to be in brow

22

Limbal Dermoid

Think about Goldenhaar syndrome

23

Meesman's Juvinille

Kids with tearing and RCE

24

PPMD

endothelium behaving like epithelium, snail tracks.

25

CHED

Type 1 - no nystagmus but tearing, photophobia, AD, presents later
Type 2 -

26

Rubella Keratitis

congenital corneal opacifcation

27

Cystinosis

crystaline keratopathy

28

Marfan Syndrome

AD - FBN1 gene CHromosome 15, Encodes forfibrillin 1. 25% are sporadic.
Ocular findings, abnormal ciliary processes and absent or disorganized zonules (tend to sublix superotemporally.
Risk of: bilateral ectopic lentis
RD
Lattice
early vitreous liquéfaction
Choroidal and scleral thinning,
flat cornea
KC
Glaucoma, strab

29

diagnostic criteria for Marfans

Aortic root z score >than 2 SD above the norm + ectopic lentis or + FBN1 gene or + systemic signs (learn a list of 6 or 7)

30

Ectopia Lentis

Trauma
Homocystinuria - AR - methionine - (develop seizures, osteoporosis, MR, prone to thromboembolism)
Hyperlysinemia
Weiil Marchesani syndrome
Sulfite oxidase
Others - ectopia lentis et pupillae (eccentric pupils, AR)

31

Dacaryoadenitis

Infectious - Virus (EBV, CMV,HSV, Mumps), bacterial (S. Aureus,
Inflammatory - Sjogrens, Sarcoid, Wegners, thyroid
Infiltrative - pleomorphic adenoma, lymphoma, adenoid cystic ca.

32

How do you differentiate between a Browns and Inferior Oblique palsy

In Brown's syndrome you see a V pattern - (Recall the OBliques are working so they will Abbduct on up gaze . Whereas, in an inferior oblique palsy the abductors are not working so on upgaze when you are in upgaze they will adduct seeing an A pattern

33

Ddx of congenitally reduced vision with normal exam

Retina:
- LCA
- rod or cone dystrophy
- achromatopsia
- blue cone monochromatism
- CSNB
- albinism

ON
- subtle hypoplasia
- optic atrophy

Occipital
- infarcts
- hypoplasia
- congenital TORCH
- delayed visual maturation

34

Leukocoria

Pink Roses Can Calm The Night

PHPV
RB, RD, ROP
Coats, Coloboma, Cataract
Toxocara
Norrie's

35

High myopia in children

Idiopathic
Congenital glaucoma
ROP
Down's
CSNB
spherophakia
Marfan's, Stickler's

36

Pseudostrabismus ddx

Dragged macula (ROP, toxocara, incontinentia pigmenti)
Wide-nasal bridge
Positive or negative angle kappa
Epicanthus
Hypertelorism

37

Abnormal head posture

Strabismus (horizontal, vertical, torsional)
Nystagmus
Infantile VF defect
Ptosis

Neck problems
Glasses

38

Conditions with torsion

SO palsy / overaction
IO palsy / overaction
IO myokymia
Torsional nystagmus
Restriction

39

Child with left hypotropia. DDx?

Left eye hypo:
- left superior orbital mass
- left SR weakness
- left IO weakness
- left IR tethering
- left orbital floor #

Right eye hyper:
- right SO palsy
- right IR palsy
- right IO overaction
- right inferior orbital mass
- right SR tethering

40

Overelevation in adduction

ipsi IO overaction
ipsi SO palsy
DVD

41

Features of congenital nystagmus

CONGENITAL

- better with Convergence
- no Opsoclonus
- there is a Null point
- no change with Gaze position
- Equal OU
- Near va better than distance (because it dampens with convergence)
- Inversion of OKN
- head Turn is present (for null point)
- Absent during sleep
- there is Latent nystagmus

42

Ddx of congenital nystagmus

- Congenital motor nystagmus
- Brain lesion causing nystagmus
- Sensory deprivation (lens, vitreous, retina, fovea, ON)

43

Type of JRA most likely to get anterior uveitis

RF -
ANA +
pauciarticular (

44

Ddx of congenital ptosis

Levator dehiscence (trauma)
Levator dysgenesis (idiopathic/familial)
Neurogenic (CN 3 palsy, Horner's)
NMJ (Myaesthenia)
Mechanical (lid tumors, chalazia, neurofibroma, etc)

45

Eye syndromes with deafness

Usher
Syphillis
Polyarteritis nodosa
Goldenhaar
Cogan
Alport
Wardenberg

46

What is Herman Bieloschowsky and what do you worry about

Monocular nystagmus of childhood
Worry about ON/chiasm lesion - image

47

Secondary tumors in RB

Melanoma
Osteogenic sarcoma
Breast
Lymphoma, leukemia

48

Paradoxical pupil response in (3)

CSNB
Achromatopsia
ON hypoplasia

49

Orbital cellulitis in child, indications for surgery

- >9 yo
- gas on CT (shows its anaerobic)
- any signs of optic nerve compromise
- dental source
- chronic sinusitis
- frontal extension (frontal sinusitis, brain involvement)
- recurrent orbital infxn

50

Capillary hemangiomas - 3 things to worry about

Kassabach Meritt - consumptive coagulopathy (plt's)
Left sided heart failure
May have other hemangiomas - e.g. subglottic - always get ENT consult pre-op

51

Benign orbital masses in kids (big categories)

Vascular (hemangiomas, varix, lymphangiomas)
Neural tissue (glioma, neurofibroma, Schwannoma)
Choristomas (dermoid, lipo-dermoid, epidermoid)
Other (fibrous dysplasia, IOI, mucocele, thyroid)

52

Malignant orbital masses in kids

Primary or secondary
Primary: RB!!!, glioma, meningioma, histiocytosis
Mets: neuroblastoma, Ewing sarcoma
Secondary extension: Burkitt's lymphoma from maxillary sinus

53

Telecanthus ddx

FAS
Blepharophimosis
Wardenburg

54

Lacrimal fistula - symptoms, how to investigate and treat

Tearing at MEDIAL canthus (epiphora is always lateral)

May have recurrent dacryocystitis
Mucous on skin near nose

Look for the fistula
Check puncta
Dye disappearance test

Treat:
- probing may resolve it
- cautery has poor outcomes
- you can excise it (excise completely including the whole tract) +/- stenting if needed
- DCR if needed

55

Nevus of ota - risks to eye

Glaucoma
Uveal/orbital melanoma

56

Chlamydia - complicaitons and rx

Gastritis, pneumonitis, otitis, nasopharyngitis
Rx with topical erythro, oral erythro
Treat mother and sexual partners with doxy or azithro

57

Gonorrhea - rx

IV ceftriaxone, topical vigamox
Treat for chalamydia at the same time

58

VKC features

GLHT SRK

- male>female
- warm months

- GPC
- Limbal follicles
- Horner Trantas dots
- Shield ulcer
- Ropy mucous
- Filamentary keratitis

59

Ligneous features

Girls > boys
Bilateral or unilateral
Pseudomembranous conjunctivitis
Woody plaque
May bleed if excised - highly vascular
Same process affects all mucous membranes (mouth, vagina, etc) --> get an ENT consult to ensure no throat involvement before removing; if it bleeds a lot they may need GA with intubation

60

axial length at birth

16 - 17 mm

61

lens dimensions at birth

6.5 x 3.5 mm

62

corneal diam in infancy

10, 11, 12 mm @ birth, 1, and 2 years (respectively)

63

Why do all infantile pupils seem big

Constrictor pupillae is immature at birth

64

Congenital lid colobomas - upper or lower

upper

65

Congenital ectropion/entropion - upper or lower

lower

66

Blepharophimosis features

Epicanthus inversus
Ptosis
Telecanthus
Lid phimosis

67

Are most prego's suspectible or immune to toxoplasmosis

70% seronegative

68

What is the most common cause of posterior uveitis in kids

Toxoplasmosis

69

Rubella - ocular manifestations

Cataract or glaucoma - don't get both together because they occur at different points in gestation
Salt and pepper retinopathy

70

Do rubella ever get glaucoma and cataracts together?

No - only one or the other, depending on when in gestation the infection happens

71

Be careful of rubella cataract sx because...

Live virus can persist in the cataract for 3 years
- tons of inflammation after surgery
- make sure everyone in the room during surgery is vaccinated

72

Most common congenital infection

CMV

73

Hutchinson's triad of congenital syphillis

Interstitial keratitis
Peg shaped teeth
Hearing loss (CN 8)

74

EKC - type of adenovirus

Type 8

75

Pre-septal cellulitis organism

Staph aureus (from skin trauma)

76

Orbital cellulitis organism in kids

H flu

77

Which muscle is hardest to find if it slips and why

MR - has no fasical attachments

78

IO surgery - what 4 anatomical things do you need to identify

- IO
- IR
- LR
- infero-temporal vortex vein

79

IR/SR act purely vertical in what eye position

23 degrees ABducted

80

IO/SO - act purely vertical in what eye position

51 degrees ADDucted

81

What type of amblyopia is least affected by neutral density filters

Strabismic
(double check this)

82

What type of ambylopia is most affected by crowding

Strabismic

83

What levels of ametropia do you need to start getting amblyopia (hyperopia, myopia, or astigmatism)

Hyperopia > +5
Myopia > -8
Astigmatism > 2.5D

84

What levels of anisometropia do you need to start getting amblyopia

Hyperopia > 1
Myopia > 3
Astigmatism > 1.5

85

What are the 3 causes of amblyopia (broad categories)

Strabismic
Deprivational
Refractive

86

Eye findings in Kawasaki (and systemic)

Conjunctivitis

Fever x 5d
Conjunctivitis
Strawberry tongue
Injected pharynx
Palmar erythema
Desquamating rash
Lymphadenopathy

87

Treatment of kawasaki

ASA

88

Risk of kawasaki

Coronary artery vasculitis --> MI

89

Cannaliculi - measurements

2-8-10 mm

90

Where will a mass of the lacrimal sac present

Under the medial canthus

91

Where is the obstruction in a dacryocystocele

Double obstruction - valve of Hasner (inferior opening) and Valve of Rosenmuller - entry to sac)

92

What is your ddx of a dacryocystocele

Mucocele
Meningocele, encephalocele (present higher)
Vascular malformation
Other orbital tumor

93

What is your investigation of a dacryocystocele

Can ultrasound if you're unsure of the dx
CT/MRI

Usually no investigations are needed as it's a clinical dx

94

What is your management of a dacryocystocele

Get ENT consult to see if its obstructing the airway - esp if breathing or feeding difficulties, FTT, etc

ENT can marsupialise if needed

You can probe/irrigate if not resolving
Otherwise just massage, topical abx if infected

Risks - breathing difficulties or disseminated infxn from dacryocystitis

95

Marfan's - ocular and systemic manifestations

Ocular:
Lens subluxation (up and out)
Myopia, high astigmatism
KC
amblyopia

Systemic:
connective tissue problems
joint problems, arthritis
aortic anneurysms
mitral valve prolapse, aortic insufficiency

96

Homocystinuria - ocular and systemic complications

Ocular:
- lens subluxation (down and in)

Systemic:
- thrombotic vascular events
- MR

97

Optic nerve hypoplasia - risk factors

Materal DM
Ingestion of LD, other substances

98

Optic nerve hypoplasia - investigations

MRI brain for de Morsier's syndrome
Send to endocrine to rule out pituitary problems

99

Optic nerve hypoplasia - management

Send to endocrine to rule out pituitary problems
For the eye - think about amblyopia and treat

100

How does leukemia present in the retina

CWS
Roth spots, hemorrhages

101

What does Lowe's syndrome give you (ocular)

Cataract and glaucoma

102

Ocular albinism - findings

- pale iris
- iris TID (even in carriers)
- pale retina
- foveal hypoplasia
- nystagmus
- photophobia
- high myopia
- strabismus

103

Aicardi - inheritance and findings

X-linked. Lethal in males

Lacunar chorioretinal degeneration
Agenesis of corpus callosum
MR

104

Fibrous dysplasia vs ossifying fibroma

Fibrous dysplasia:
- stabilises after skeletal maturity
- precocious puberty
- pigmented skin macules

Both can have ON compression
Difference is histological

105

Schwannoma - type of spread

Perineural

106

Neurofibroma of upper eyelid. Risks?

Glaucoma
Amblyopia (ptosis or astigmatism)

107

Neuroblastoma - where is the primary most often

Abdomen (adrenal gland)
2nd = retroperitoneal

mets @ presentation in 1/3 of cases

108

What part of the brain controls pursuit

Parietal (that's why abnormal OKN towards the side of the lesion)

109

What part of the brain controls saccades

Frontal (FEF)

110

What is cogan's dictum

Homonymous hemianopia
- with abnormal OKN = parietal
- with symmetric OKN = occipital

111

What do you rule out in spasmus nutans

Can look like monocular nystagmus of childhood so rule out nerve or chiasmal tumor (image)

112

Ocular findings in axenfeld reiger's

Posterior embryotoxin (anteriorly displaced Schwalbe's line)
Iris hypoplasia with holes

113

What is Allagile's

Axenfeld Reiger + pigmentary retinopathy + systemic findings

114

What is Peter's syndrome

Opacified cornea

115

Name 5 iris nodules

- Brushfield spots - in Down's - stromal hyperplasia surrounded by hypoplasia
- Lisch nodules - NF1 - hamartomas
- Iris nevi in ICE - heaped up iris stroma due to the endothelial membrane on top that contracts and tents up the stroma
- Iris cysts - often from phospholine iodide use
- JXG: yellow-orange spots. Touton on path. Bleed spontaneously.

116

How do you treat a medulloepithelioma

Watch
Excise
Enucleate

117

What do you find in Alport's syndrome

Cataract
Hearing loss

118

Microspherophakia - ddx and management

Weill-Marchesani
Alport
Lowe's
Hyperlysinemia
Rubella
Peter's

Use cycloplegics to flatten the lens and pull it back to relieve ACG

119

Cataracts - work up for bilateral

Hearing tests: Alport, Rubella
Urine tests: reducing sugars (galactosemia) and amino acids (Lowe's)
Genetics: Trisomy 13, 18, 21
Blood tests: blood sugar (DM), calcium, TORCH titres, RBC galactokinase (galactosemia)

*Alport & Lowe's are x-linked so only w/u boys*

120

Cataracts - work up for unilateral

TORCH titres
family history
do a good eye exam (both eyes)

121

Cataracts, bilateral - when do you intervene

Visually sig is >3 mm usually
Operate on BETTER eye first. Aim for 6-8 weeks old, then second eye 1 week later

122

Ddx of cataract + glaucoma

Lowe's
Hallerman-Streiff
Rubella (very rare)

123

ROP - screening criteria (canada)

birth weight

124

ROP - when do you do their first exam

Either 4 weeks after birth, or 31 weeks GA, whichever is LATER

125

ROP - what is threshold dz

Zone I or II
Stage 3
With plus
5 continuous or 8 cumulative clock hours

126

ROP - what do you do for threshold dz and when

3 options:
- cryo
- laser
- anti-VEGF

Treat within 72 hours. Treat whole avascular retina

127

ROP - what is pre-threshold dz

Think of it as anything that doesn't quite meet threshold:

- Zone I, any stage with plus
- Zone I, stage 3 no plus
- Zone 2, stage 2 with plus

128

What did we learn from CRYO-ROP

Defined threshold dz
And said cryo worked

129

What did we learn from ETROP

Said treating pre-threshold dz is also probably a good idea
Used laser (?)

130

How often do you follow ROP kids and when do you stop

Every 1 or 2 weeks depending on the severity (it's kind of complicated....)
Stop when zone 3 is vascularized
Or if all good at 45 weeks

131

How can you make an ROP exam easier on the newborn

Cooperative nursing staff
Swaddle
Soothers
Use sugar
Topical anaesthesia
Stop if the HR drops

132

What are treatment options for RB

Chemo or enucleation are mainstays of treatment
RT/plaque are second line

133

If you enucleate RB, how much ON do you want to remove

>10 mm

134

What imaging do you want to get in RB

Look at pineal gland

135

What is your initial metastatic w/u in RB

Bone scan, LP

136

How do you investigate an RB

Anterior chamber LDH: plasma LDH >1.0
Check both eyes carefully
B-scan = calcium, high internal reflectivity
FA = early hyper, late leakage
Orbital CT looking for calcium
Brain MRI for pineal tumor
Metastatic workup (bone scan, LP)

137

What are the second malignancies in RB

Melanoma
Osteogenic sarcoma
Breast ca
Lymphoma, leukemia

138

What are poor prognostic factors in RB

Optic nerve invasion
Uveal invastion
Extra-scleral extension
Multifocal tumors

Any metastatic dz at presentaiton
Unilateral = bilateral prognosis

139

What's more common in kids, leukemia or lymphoma?

Leukemia

140

What is the inheritance pattern of phakomatoses? What are the exceptions?

All AD with incomplete penetrance except the Weird ones: sturge Webber and Wyburn mason are AR

- other exception: ataxia telangiectasia is sporadic

141

Dx criteria for NF1 (7)

- 1st degree relative with NF1
- >2 lisch nodules
- >6 cafe au lait spots (>5 mm pre-pubertal, >15 mm post-pubertal)
- 1 plexiform or 2 neurofibromas
- osseous dysplasia (e.g. sphenoid wing)
- intertiginous freckling
- optic nerve glioma

142

Dx criteria for NF2

Either
- 2 acoustic neuromas
- 1st degree relative with NF2 and either 1 acoustic neuroma or 2 of: meningioma, schwannoma, neurofibroma, glioma, PSCC

143

SWS - what do you need to rule out

Leptomeningeal vascular malformations

144

SWS - what is the mechanism of glaucoma

Combined:
- increased EVP from the conj lesions
- angle abnormalities from the hemangiomas growing over/in the angle
- neovascular

145

VHL - list all the findings (systemic + ocular)

Chromosome 3

Ocular:
- retinal angiomas
- may leak
- treat only if causing a serous RD

Systemic:
- cerebellar angiomas
- cysts and tumors of kidney, pancreas, liver, adrenal glands
- renal cell carcinoma
- pheochromocytoma

146

Tuberous sclerosis - retinal finding

Retinal astrocytoma

147

Tuberous sclerosis - triad

Adenoma sebaceum, mental retardation, and seizures

148

Tuberous sclerosis - skin findings

Ash leaf spots
Shagreen patches
Adenoma sebaceum
Peri-ungal fibromas
+/- cafe au lait spot

149

Ataxia telangiectasia - ocular findings

Telangiectactic conj vessels
Impaired convergence
Nystagmus
Ocular motor apraxia

150

Ataxia telangiectasia - systemic concerns

Defective T cell function and IgA deficiency can cause recurrent infections or malignancies (leukemia/lymphoma)

Also get ataxia and MR

151

Retinal cavernous hemangioma - systemic association

None!

Unlike retinal capillary hemangiomas (VHL) or choroidal hemangiomas (SWS)

152

Brown's features vs IO palsy
Brown's Rx

Can't adduct in upgaze
May have palpable click or audible click
May be associated with systemic inflammatory dz
No associated SO overaction

Brown's will give you a V-pattern (eye can't adduct in upgaze so it's out) while IO palsy will give you an A pattern

Rx Brown's with SO tenectomy, tenotomy, or silicone spacer

153

Epinephrine dose for anaphylaxis

IM: 0.3 - 0.5 mL of 1:1000

always remember ABC, call code, stop offending agent, etc

154

FAS features

Think of it mostly as lids:
- ptosis
- telecanthus
- short palpebral fissures

- microphthalmos
- ET
- refractive error
- amblyopia
- on hypoplasia
- ant seg dysgenesis

155

Crouzon features

This is a craniosynostosis:

Inferior scleral show
Proptosis, globes can luxate forward
Exposure keratitis
V-pattern XT
Therefore amblyopia
Refractive error

High pressure in the head from suture closure can give you papilledema
And always look for ON pallor from the optic canal becoming narrowed

156

What is Apert

Craniosynostosis. It's Crouzon + syndactycly

157

Goldenhaar - ocular and systemic features

Remember the whole thing is one side of the face is dysplastic. That side of body can have issues too.

Ocular:
- limbal dermoid
- lower lid coloboma

Ear:
- pre-auricular skin tag
- poor hearing

Systemic:
- cleft lip/palate
- vertebral anomalies
- can have dysplastia of the organs on that side of body - heart, lung, kidneys

158

Treacher Collins

Mandibular dysplasia

159

Pierre Robin - ocular association

Sticklers

think "Pierre Robin & Stickler are all friends" :)

160

Infantile vision at birth and 6 months

20/200-20/400 at birth
20/20 at 6 months (by VEP)

161

How do you measure infantile vision

VEP
or preferrential looking

Preferrential looking doesn't give you 20/20 until a couple years old (may involve more than just vision i.e. processing, motor capabilities, etc)
VEP is 20/20 by 6 months