Peds Flashcards
Embryology - 22 days
Optic primordium appears in neural folds
3 types of XT
- convergence insufficiency
- divergence excess
- primary/idiopathic
Embryology - 25 days
Optic Vesicle evaginates. Neural crest cells migrate to surround vesicle.
Embryology - 28 days
Vesicle induces lens placode
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
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
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
Embryology 5th Month
Photoreceptor inner segments develop
Choroidal vessels form
Iris stroma is vascularized
Eyelids begin to separate
Embryology - 6th Month
Ganglion cells thicken in the macula
Recurrent Atrerial brances joing the choroidal vessels
Dilator muscle forms
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
Embryology - 8th Month
Chamber angle completes formation
Hyaloid system disappears
Embryology - 9th Month
Retinal vessels reach periphery
Myelination of nerve fibers are complete to the lamina
pupillary membrane dissapears
What is the name for the celluar area of retinal proliferation at the leading edge of the vasculature in the ember
Vascular Anlage
Types of abnormal small eyes
Microphthalmos with cyst. - clinically when you open the lids the cyst tends to be infront as a retina
Embryonic Fissure
Closure at 6 weeks - closes inferonasally (This is where we get colobomas
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
Synophthalmia
Associated with Holoprosencephaly and the
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.
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
Tuberous Sclerosis
Astrocytic hamartoma
Pilomatrixoma
Occur in children - tend to be in brow
Limbal Dermoid
Think about Goldenhaar syndrome
Meesman’s Juvinille
Kids with tearing and RCE
PPMD
endothelium behaving like epithelium, snail tracks.
CHED
Type 1 - no nystagmus but tearing, photophobia, AD, presents later
Type 2 -
Rubella Keratitis
congenital corneal opacifcation
Cystinosis
crystaline keratopathy
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
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)
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)
Dacaryoadenitis
Infectious - Virus (EBV, CMV,HSV, Mumps), bacterial (S. Aureus,
Inflammatory - Sjogrens, Sarcoid, Wegners, thyroid
Infiltrative - pleomorphic adenoma, lymphoma, adenoid cystic ca.
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
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
Leukocoria
Pink Roses Can Calm The Night
PHPV RB, RD, ROP Coats, Coloboma, Cataract Toxocara Norrie's
High myopia in children
Idiopathic Congenital glaucoma ROP Down's CSNB spherophakia Marfan's, Stickler's
Pseudostrabismus ddx
Dragged macula (ROP, toxocara, incontinentia pigmenti) Wide-nasal bridge Positive or negative angle kappa Epicanthus Hypertelorism
Abnormal head posture
Strabismus (horizontal, vertical, torsional)
Nystagmus
Infantile VF defect
Ptosis
Neck problems
Glasses
Conditions with torsion
SO palsy / overaction IO palsy / overaction IO myokymia Torsional nystagmus Restriction
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
Overelevation in adduction
ipsi IO overaction
ipsi SO palsy
DVD
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
Ddx of congenital nystagmus
- Congenital motor nystagmus
- Brain lesion causing nystagmus
- Sensory deprivation (lens, vitreous, retina, fovea, ON)
Type of JRA most likely to get anterior uveitis
RF -
ANA +
pauciarticular (
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)
Eye syndromes with deafness
Usher Syphillis Polyarteritis nodosa Goldenhaar Cogan Alport Wardenberg
What is Herman Bieloschowsky and what do you worry about
Monocular nystagmus of childhood
Worry about ON/chiasm lesion - image
Secondary tumors in RB
Melanoma
Osteogenic sarcoma
Breast
Lymphoma, leukemia
Paradoxical pupil response in (3)
CSNB
Achromatopsia
ON hypoplasia
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
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
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)
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
Telecanthus ddx
FAS
Blepharophimosis
Wardenburg
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
Nevus of ota - risks to eye
Glaucoma
Uveal/orbital melanoma
Chlamydia - complicaitons and rx
Gastritis, pneumonitis, otitis, nasopharyngitis
Rx with topical erythro, oral erythro
Treat mother and sexual partners with doxy or azithro
Gonorrhea - rx
IV ceftriaxone, topical vigamox
Treat for chalamydia at the same time
VKC features
GLHT SRK
- male>female
- warm months
- GPC
- Limbal follicles
- Horner Trantas dots
- Shield ulcer
- Ropy mucous
- Filamentary keratitis
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
axial length at birth
16 - 17 mm
lens dimensions at birth
6.5 x 3.5 mm
corneal diam in infancy
10, 11, 12 mm @ birth, 1, and 2 years (respectively)
Why do all infantile pupils seem big
Constrictor pupillae is immature at birth
Congenital lid colobomas - upper or lower
upper