Peds Flashcards

0
Q

Embryology - 22 days

A

Optic primordium appears in neural folds

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1
Q

3 types of XT

A
  • convergence insufficiency
  • divergence excess
  • primary/idiopathic
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2
Q

Embryology - 25 days

A

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

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3
Q

Embryology - 28 days

A

Vesicle induces lens placode

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4
Q

Embryology - 2nd Month

A

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

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5
Q

Embryology 3rd Month

A

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

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7
Q

Embryology -4th month

A
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
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8
Q

Embryology 5th Month

A

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

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9
Q

Embryology - 6th Month

A

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

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10
Q

Embryology - 7th Month

A
Outer segments of photoreceptors differentiate
Central fovea starts to thin
Fibrous lamina cribrosa forms
Choroidal melanocytes produce pigment
Circular muscle forms in CB
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11
Q

Embryology - 8th Month

A

Chamber angle completes formation

Hyaloid system disappears

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12
Q

Embryology - 9th Month

A

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

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13
Q

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

A

Vascular Anlage

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14
Q

Types of abnormal small eyes

A

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

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15
Q

Embryonic Fissure

A

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

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16
Q

Ocular features of Trisomy 13

A

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

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17
Q

Synophthalmia

A

Associated with Holoprosencephaly and the

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18
Q

Acardi Syndrome

A

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.

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19
Q

Peters Anomaly

A

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

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20
Q

Tuberous Sclerosis

A

Astrocytic hamartoma

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21
Q

Pilomatrixoma

A

Occur in children - tend to be in brow

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22
Q

Limbal Dermoid

A

Think about Goldenhaar syndrome

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23
Q

Meesman’s Juvinille

A

Kids with tearing and RCE

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24
Q

PPMD

A

endothelium behaving like epithelium, snail tracks.

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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