What is the classic triad for congenital glaucoma and the modality of imaging and exam findings to monitor this condition?;"Congenital glaucoma triad • Blepharospasm • Photophobia • Epiphora Imaging and exam findings to monitor the disease • A scan • Reversible cupping is seen as the disease process is treated appropriately" What is a “phoria”?;" • Latent deviation that is controlled by fusional mechanism so eyes remain aligned under binocular conditions • Apparent when fusional control is lost" What is a “tropia”?;" • Manifest deviation that cannot be controlled by fusional mechanism • Eyes are misaligned even under binocular conditions" What are the different ways of measuring visual acuity in a preverbal child?;" • Blink to light • Fix and follow • Central steady maintained • Teller card • VEP • OKN" What is the origin of the superior rectus?;"Annulus of Zinn at orbital apex" What is the functional origin of the superior oblique?;"Trochlea" What is cerebral visual impairment?;" ● Retrogeniculate visual impairment ● Visual deficits resulting in pathology posterior to lateral geniculate nucleus ● Common causes ○ Periventricular leukomalacia (most common) ○ Intracranial hemorrhage ○ Perinatal hypoxia ○ Intrauterine infection ○ Structural abnormalities ○ Seizures ○ Hydrocephalus ○ Trauma ○ Meningitis ○ Encephalitis ● Exam findings ○ Variable level of visual inattentiveness ○ Subtle optic nerve cupping (similar to glaucomatous cupping) in preterm infants ● Work up: neuroimaging" What are the findings in unilateral and bilateral superior oblique (SO) palsy?;"Unilateral SO palsy • Little if any V pattern • Less than 10 degrees of extorsion in downgaze • Head tilt toward should opposite side of weakness • Subjective incyclodiplopia is uncommon unless palsy is severe Bilateral SO palsy • V pattern • Extorsion is 10 degrees or more in downgaze; >20 degrees is highly suggestive of bilateral involvement • Chin down position • Subjective incyclodiplopia is common in acquired cases" What is an important risk when simultaneous surgery on three or more rectus muscles occurs and its clinical presentation?;" ● Anterior segment ischemia occurs because majority of blood supply to anterior segment derives from anterior ciliary arteries which travel in the 4 rectus muscles ● Clinical findings: ○ Cell and flare (earliest sign) ○ Corneal edema ○ Pupil irregularity" What is the pathophysiology of midface hypoplasia?;"Cranial malformation due to premature closure of skull base suture" What is hypertelorism?;"Excessive distance between medial orbital walls" What is telecanthus?;"Greater than normal distance between medial canthus" What is craniosynostosis?;"Premature closure of one or more cranial sutures during embryonic period or early childhood" What is plagiocephaly?;" ● Most common craniosynostosis ● Means “oblique head” ● Cause: external compressive forces, occurring prenatally or during infancy ● Types ○ Deformational plagiocephaly → due to intrauterine constraint (oligohydramnios), ipsilateral occipital flattening ○ Unilateral coronal suture synostosis → also due to intrauterine constraint. Synostotic side → forehead and supraorbital rim are depressed, interpalpebral fissure wider, orbit is often higher than nonsynostotic side" What is brachycephaly?;" • Bilateral closure of coronal sutures • Wide and flat forehead" What is scaphocephaly?;" • Premature closure of sagittal suture • Long head" Which cycloplegic eye drop can cause the most allergic or hypersensitivity reactions?;" • Atropine • Systemic side effects: fever, dry mouth, flushing, tachycardia, urinary retention, delirium, nausea, dizziness" What is the panum's area of single binocular vision?;" • A visual system that can combine slightly disparate points within a limited area surrounding the horopter • Objects in this space on disparate retinal points but are physiologically seen single • Objects outside Panum’s area fall on widely disparate retinal points and are seen as lying in 2 visual directions, results in physiologic diplopia" What is Brown syndrome?;" • Positive forced duction (restriction from trochlea superior oblique complex) • V pattern • None or minimal superior oblique muscle overaction • No torsion • Negative head tilt test • Deficient elevation in adduction (due to various abnormalities of tendon trochlea complex)" What are the clinical findings in inferior oblique muscle palsy?;" • Intorsion of hypotrophic eye (due to superior oblique overaction) • Positive head tilt test • Deficient elevation in adduction" What are primary iris stromal cysts?;" • Often diagnosed in infancy • Due to sequestration of epithelium during embryologic development • Epithelium lined stromal cysts containing goblet cells • If enlarged → associated with blockage of visual axis, corneal decompensation, glaucoma, iritis due to leakage of cysts of contents • Treatment: surgical excision" Polychromatic lenticular deposits (“Christmas tree” cataract) is seen in which condition?;"Myotonic dystrophy" What is Myotonic dystrophy and its findings?;" • Inability to relax muscles when stimulated • Ptosis • CPEO • Hypotony due to ciliary body detachments • Peripheral pigmentary retinopathy cardiac conduction abnormalities • Christmas tree cataracts • Sluggish pupils • EMG (diagnostic) myotonic discharges •""hatchet face"" wasting of temporalis and master muscle, frontal balding, low intelligence" What is the most common location of a limbal dermoid?;" • Inferotemporal limbus • Associated with Goldenhar syndrome (oculoauriculovertebral syndrome) = hemifacial microsomia, ear deformities, upper eyelid colobomas, vertebral anomalies" What is the difference between atopic keratoconjunctivitis and vernal keratoconjunctivitis?;" • AKC: superior and inferior conjunctiva with papillae and possible scarring. Occurs year around. Susceptible patient → asthma, eczema, atopic dermatitis • VKC: seasonal propensity. Affects superior palpebral conjunctiva • BOTH VKC and AKC preferentially affect males" What is congenital CN4 palsy?;" • Head tilt • Facial asymmetry • Large vertical fusional amplitudes" What is JIA?;" • Most common cause of uveitis in pediatric age group 1. Oligoarthritis (4 or less joints) most likely to have anterior uveitis, 10 30% 2. RF negative polyarthritis uveitis in 10% 3. Psoriatic arthritis uveitis in 10% 4. Systemic arthritis/Still's disease uveitis rarely occurs" What is chemical ophthalmia neonatorum?;" • First 24 hours, due to silver nitrate, resolves by day 2 of life" What is ophthalmia neonatorum due to Neisseria gonorrhoeae?;" • Occurs 3 4 days of life up to 3 weeks after birth • Most amount of purulent discharge compared to other forms • Acquired during passage through birth canal (occurs with C section if amniotic membranes rupture prior)" What is ophthalmia neonatorum due to Chlamydia?;" • Occurs at around 7 days of life • Can occur earlier if there is rupture of maternal amniotic membranes • Acquired during passage through birth canal (occurs with C section if amniotic membranes rupture prior)" What is ophthalmia neonatorum due to HSV?;" • Occurs at 2 weeks of life • Very rare • Acquired during passage through birth canal (can occur with C section if amniotic membranes rupture prior)" What is the cause of CN6 palsy in children?;" • 1/3 patients have intracranial lesions • More common in older children than at birth or during infancy • Face turn protective against amblyopia, produce single binocular image" What is the length of the active muscular portion of the medial/inferior/lateral/superior rectus?;" • 40 mm" What is the length of the active muscular portion of the superior oblique?;" • 32 mm" What is the length of the active muscular portion of the inferior oblique?;" • 37 mm" What are the extraocular muscles with the longest tendons?;" • Superior oblique (26 mm) • Levator (14 20 mm)" What is the longest overall extraocular muscle?;" • Superior oblique • Active muscle (32 mm) + tendon (26 mm)" What is the smallest overall extraocular muscle?;" • Inferior oblique • Active muscle (37 mm) + tendon (1 mm)" An "A pattern" difference in measurement between upgaze and downgaze is AT LEAST how many PD?;" • 10 PD" A "V pattern" difference in measurement between upgaze and downgaze is AT LEAST how many PD?;" • 15 PD" What does the mnemonic "MALE” stand for, in relation to strabismus surgery?;" • Medial recti are always displaced toward ""Apex"" of pattern • Lateral recti are displaced toward ""Empty"" part of the pattern" What are the ways to correct V pattern esotropia without oblique dysfunction?;" • Either: 1. bilateral medial rectus recessions displaced inferiorly 2. bilateral lateral rectus resections displaced superiorly" What are the spiral of Tillaux measurements?;" • Medial rectus 5.5 mm • Inferior rectus 6.5 mm • Lateral rectus 6.9 mm • Superior rectus 7.7 mm" What is another name for Neurofibromatosis type 1?;" • von Recklinghausen disease" What is another name for Sturge Weber syndrome?;" • Encephalotrigeminal angiomatosis" What is another name for Wyburn Mason syndrome?;" • Bonnet Dechaume Blanc syndrome" What is another name for tuberous sclerosis?;" • Bourneville’s disease" What is another name for Neurofibromatosis type 2?;" • Multiple inherited schwannomas, meningiomas, ependymomas (MISME)" What are the sporadic, non inherited phakomatoses?;" • Wyburn Mason syndrome • Sturge Weber syndrome" At what age is it normal to start making eye contact and react with facial expressions?;" • 6 weeks of age" What is the differential diagnosis for ectopia lentis?;"• Trauma • Marfan • Homocystinuria • Ehlers Danlos • Aniridia • Congenital glaucoma • Iris coloboma • Syphilis • Weill Marchesani syndrome • Sulfite oxidase deficiency • Hyperlysinemia • Ectopia lentis et pupillae" What is sulfite oxidase deficiency?;"• Deficient in molybdenum cofactor • Ectopia lentis, recurrent seizures, hypertonia, deep set eyes • Most children die before age 5" What protein is affected in Marfans syndrome?;"• Fibrillin protein" What is the enzyme deficiency in hyperlysinemia?;"• Alpha aminoadipic semialdehyde synthase" What is the newborn axial length and corneal horizontal diameter?;"• Axial length: 15 17 mm • Corneal horizontal diameter: 9.5 10.5 mm" What is the myelinated retinal nerve fiber layer?;"● Focal abnormality in myelination ○ Normally, myelination of retinal ganglion cells starts at lateral geniculate ganglion and stops at lamina cribrosa ● Typically incidental and asymptomatic ● Lesions usually follow vascular distribution ● Result in scotoma corresponding to region of myelination ● Can cause pseudopapilledema if it occurs around optic nerve head ● Can reduce visual acuity due to the following: 1. Involvement of macula 2. High myopia 3. Amblyopia (increased risk refractive amblyopia) 4. Nystagmus" What tests diagnose myasthenia gravis?;"• Tensilon test • Ice pack test • Anti MuSK • Anti ACh receptor abs • Single fiber EMG" What is the differential diagnosis for congenital corneal opacity?;"""STUMPED"" 1. Sclerocornea 2. Trauma 3. Ulcer 4. Mucopolysaccharidoses 5. Peters anomaly 6. Endothelial (CHED) 7. Dermoid" What is the Hirschberg test?;"• Qualitative test using corneal light reflex that gives rough idea of what type of deviation is present" Which strabismus surgeries pose the greatest threat to visual acuity if a complication occurs?;"• Inferior oblique advancement • Strengthening of inferior oblique requires reinsertion more posterior which predisposes macular to greater danger if scleral perforation occur" What is iris mammillation?;"• Nodules the same color as surrounding iris • Associated with oculodermal melanocytosis, ciliary body melanoma, and choroidal melanoma DDx: Lisch nodules, ocular/oculodermal melanocytosis, malignant melanoma of iris, Cogan Reese syndrome, iris granulomas" What disease is associated with a spontaneous hyphema?;"• Juvenile xanthogranuloma" What inherited condition is associated with sphenoid dysplasia?;"• NF1" What are the 3 main patterns of nystagmus in congenital sensory nystagmus (due to poor vision from a variety of causes)?;"1. Searching nystagmus, VA worse than 20/200 2. Pendular pattern, most common, VA at least 20/200 in one eye 3. Jerk nystagmus, VA 20/60 20/100" What is Harada Ito procedure?;"● Advancement/strengthening of anterior fibers of superior oblique tendon that are most responsible for ocular torsion ○ Involves splitting the superior oblique tendon and displacing anterior fibers to superior border of lateral rectus incyclotorsion ● Useful for CN 4 palsy with excyclotorsion with ipsilateral hypertropia ● Surgery corrects excyclotorsion with limited effect on vertical alignment" What is the most common strabismus pattern in craniosynostosis syndrome?;"• V pattern exotropia, often accompanied by significant inferior oblique overaction" What are the tertiary actions for the (superior/inferior) recti and the (superior/inferior) obliques?;"• ADDuction is tertiary action for superior/inferior recti • ABDuction is tertiary action for obliques" What is congenital dacryocystocele?;"• Result of distal (at the level of the valve of Hasner) and proximal (at the level of the valve of Rosenmüller) obstruction of nasolacrimal duct that causes distention of lacrimal sac • Kinking of common canaliculus prevents accumulated secretions • Most also have cysts of distal NLD seen under inferior turbinate in nasal cavity • Present at birth or shortly afterwards • Bluish swelling just below and nasal to medial canthus DDx: capillary hemangioma, encephalocele, dermoid cyst" What is the normal IOP in a newborn?;"• 10 12 mmHg" What is the Bruckner test?;"• Shining direct ophthalmoscopy into both eyes to elicit red reflex OU simultaneously • Looking for asymmetry of red reflex • Asymmetric red reflex: lens opacities, strabismus, anisometropia • Red reflex dim in one eye, bright in other eye 🡪 brighter eye is the abnormal eye • Foveation of ophthalmoscope filament dims red reflex" What is the Krimsky test?;"• Prisms to quantify decentration of corneal reflex, placed over the fixing eye • Useful if there is poor fixation in one eye (due to dense amblyopia, low vision, blindness) to allow for cover uncover testing and strabismus measurement" What is Marcus Gunn jaw wink?;"•“Congenital Trigemino oculomotor synkinesis” • Eyelid is variably ptotic and elevates with jaw movement • Aberrant connection between CNV3 (external pterygoid muscle) and CN3 (superior branch) • Wink phenomenon elicited with thrusting jaw to contralateral side, jaw protrusion, sucking, chewing, smiling (i.e. bottle feeding)" What is the surgical management for CN6 palsy?;"• Wait minimum of 6 months for any spontaneous resolution to occur 1. If some LR function is regained then large recession of ipsilateral medial rectus along with lateral rectus resection 2. If total paralysis → vertical muscle transposition operation" What is Jensen procedure?;"• A split tendon technique • Superior, inferior, lateral recti are all split one half tendon width • Split is 14mm posterior to insertion • Suture is placed between adjacent muscle halves and tied relatively loosely" What is the Foster procedure?;"• Full tendon technique, entire tendon of superior and inferior recti are cut from their insertions and re inserted next to lateral rectus insertion • Posterior fixation sutures are placed 8 10 mm posteriorly which enhances abducting forces of the transposed vertical recti" What is Sturge Weber syndrome? What are the findings?;"• Leptomeningeal vascular malformation which leads to cortical/subcortical calcifications • Sporadic inheritance • Diffuse choroidal hemangioma (fundus appears redder/pinker) • Ipsilateral to port wine stain • Leads to vision loss due to RPE subretinal hemorrahge, serous RD, CME, retinal pigment atrophy • Stroke like episodes associated with seizures and migraines (consider if occurring in young child after minor head trauma), advised to avoid contact sports • Port wine stain (positively correlates with probability of CNS involvement) • Secondary glaucoma (before age 10 is primary angle abnormality; after age 10 is increased episcleral venous pressure) ipsilateral to port wine stain" What are the interventions to prevent symptomatic superior oblique palsy during treatment of Brown syndrome?;"1. Careful handling of intermuscular septum during tenotomy 2. Simultaneous ipsilateral inferior oblique weakening 3. Sewing in of inert spacer to cut ends of superior oblique tendon" What are the features of orbital rhabdomyosarcoma?;"• Proptosis (80 100%) • Globe displacement (80%) • Blepharoptosis (30 50%) • Conjunctival and eyelid swelling (60%) • Palpable mass (25%) • Pain (10%)" What is congenital motor nystagmus?;"• Horizontal nystagmus with good vision • Typically horizontal on upgaze and downgaze • ONLY seen in CMN: ""paradoxical inversion of OKN response"" right jerk nystagmus with OKN rotation left will dampen or reverse to become left jerk nystagmus" What is Botulinum toxin type A and how is it used in strabismus?;"• BoTox • Blocks release of ACh at neuromuscular junction • Lasts 3 or more months when injected into orbicularis muscle • Lasts 5 8 weeks when injected into extraocular muscles" What is dystopia canthorum?;"• Abnormality whereby inner canthi and lacrimal puncti are displaced laterally • Associated with Waardenburg syndrome" When is chemodenervation (botox) is indicated for strabismus?;"• Small to moderate angle esotropia or exotropia residual strabismus • Acute paralytic strabismus • Active thyroid disease when surgery is inappropriate, no stabilization of TED • Supplemental to medial rectus recession in large angle infantile esotropia" When is chemodenervation (botox) is contraindicated for strabismus?;"• Large deviations • A or V patterns • Dissociated vertical deviations • Restrictive strabismus" What is cyclic esotropia?;"• Rare form of strabismus (1 in 4000 strabismus patients) • Unpredictable response to different forms of therapy EXCEPT for muscle surgery which is usually curative • Fusion is absent on strabismic days as suppression often occurs • “Straight"" days, there is binocular function • Older children who cannot develop suppression, one may get diplopic symptoms on strabismic days. • Patching convert cyclic esotropia into constant manifest esotropia" What are the most common extraocular muscles affected in thyroid eye disease, in order of decreasing of frequency?;"• Inferior rectus (most common) • Medial rectus • Superior rectus • Lateral rectus" What is associated with high myopia and symptomatic strabismus?;"• High myopes → elongated globe can herniate between superior and lateral recti, stretching and dehiscence of intermuscular septum between these 2 muscles • Can cause inferior slippage of lateral rectus pulley • These changes result in progressively worsening hypotropia and esotropia • Medial rectus is frequently tight → worsening esotropia called ""heavy eye syndrome“ Rx: prisms and surgery to address lateral rectus muscle slippage by joining superior and lateral rectus muscles with loop myopexy to reposition the globe and recession of medial rectus may also be needed" What is optical penalization?;"• Involves applying something to sound eye to potentially augment amblyopia therapy • Adding plano lens to sound eye with hypermetropic refractive error on atropine, patient will not be able to accommodate to focus • High plus CL (+13.00) applied to sound eye (rarely done due to risk of infection in essentially monocular patient)" When no other family history of primary congenital glaucoma exists, what is the chance of an affected parent having an affected child?;"• 2%" What is primary deviation and secondary deviation?;"• Primary deviation: deviation measured when normal eye is fixating • Secondary deviation: deviation measured when paretic or restricted eye is fixating • Secondary deviation is larger than primary deviation since more innervation signals are required to bring paretic eye into fixation, results in increased signals to corresponding yoke muscle (Herring's Law)" What is Alagille syndrome?;"• Autosomal dominant, JAG1 gene mutation • Involves liver, heart skeleton, broaden forehead, pointed chin, butterfly hemivertebrae, mild developmental delay • Posterior embryotoxin, hypertelorism, upslanting palpebral fissures, ectopic pupils, chorioretinal atrophy, band keratopathy, cataracts, retinal pigment clumping, axenfeld anomaly, anomalous optic disc, myopia, strabismus, choroidal folds, microcornea" How does one distinguish between Brown syndrome and inferior rectus restriction?;"• Both have deficient elevation on adduction • Forced duction test: retropulse the globe with forceps to test oblique muscles; proptose globe with forceps to test rectus muscles • Retropulsion stretches the superior oblique tendon, whereas restriction would be increased in Brown syndrome • In inferior rectus restriction, restriction would be increased with forceps induced proptosis" What is Marcus Gunn jaw winking syndrome?;"1. Eyelid elevated with mouth OPENING → aberrant connection between external pterygoid muscle and levator palpebrae superioris 2. Eyelid elevated with mouth CLOSING → aberrant connection between internal pterygoid muscle and levator palpebrae superioris" What are the 4 classic features of blepharophimosis syndrome?;"1. Blepharophimosis 2. Ptosis 3. Telecanthus 4. Epicanthus inversus: prominent fold of skin arising from lower eyelid and covering medial canthus Rx: epicanthus inversus and telecanthus are corrected before ptosis repair, medial canthus reconstruction often worsens ptosis" What are earliest signs of malignant hyperthermia?;"• Elevation of end tidal carbon dioxide concentration and tachycardia • Late signs: elevated temp, acid base disorders, renal failure, heart failure" What is ocular and orbital hypertelorism?;"• Ocular hypertelorism: excessive interpupillary distance, implies orbital hypertelorism • Orbital hypertelorism: excessive distance between medial orbital walls as result of lateralization of orbits, diagnosis made radiographically" What is Mobius syndrome?;"• CN 6 and 7 palsies, masked facies, limb abnormalities (clubbed feet), chest wall defects, deformed tongue, may have adduction impairment improved with convergence" What are the symptoms of thyroid eye disease in children?;"• Very rare, milder than adults • Common: proptosis, eyelid retraction, lid lag, soft tissue swelling (preorbital fat pad enlargement) • Uncommon/rarely (unlike adults): extraocular muscle fibrosis, optic neuropathy Rx: medications first then subtotal thyroidectomy/radioiodine ablation" What is Lowe syndrome?;"• Very rare, X linked • Reduction of phosphatidylinositol bisphosphate 5 phosphatase activity • Dense cataracts in ALL patients, glaucoma in 50%, corneal/conjunctival keloids • Severe hypotonia, kidney problems, aggressive behavior (>75%), mild intellectual disability (10%)" How does one diagnose Wilson's disease (hepatolenticular degeneration)?;"• Serum copper and ceruloplasmin • Kayser Fleischer ring (late finding)" What is congenital iris ectropion syndrome?;"• Combination of unilateral congenital iris ectropion, high iris insertion, smooth iris surface, glaucoma • Associated with NF, primary facial hemihypertrophy, Rieger anomaly, Prader Willi syndrome" What is the order of surgery for thyroid eye disease?;"1. orbital decompression 2. strabismus surgery 3. eyelid surgery" What is Sherrington's law?;"• Law of reciprocal innervation, contraction of given muscle (medial rectus) accompanied by decrease in contraction of its antagonist (lateral rectus) • Violated in Duane's syndrome" What is an example that violates Herring's law?;"• Dissociated vertical deviation" What is central and peripheral fusion?;"• Central fusion: area near fovea has small receptive fields, therefore, objects need to be very similar in terms of size and shape to be fused properly • Peripheral fusion: peripheral retina has large receptive field, thus objects can be more dissimilar in size and shape and still be fused" What is vernal keratoconjunctivitis and its findings?;"• Allergic children, occurs within 1st two decades of life mostly in spring or fall • Giant papillary conjunctivitis (>1 mm) • Horner Trantas dots: jelly like limbal nodules = degenerated eosinophils • Shield ulcer, epi defect upper half of cornea" What are Herbert's pits?;"• Small gaps in pannus as a result of healed limbal follicles • Seen in trachoma" What is a Kestenbaum Anderson procedure?;"• Used for correcting head turns associated with nystagmus • Shift null zone closer to primary position • Surgically rotate eye toward the head turn or away from null zone • Total amount of surgery for each eye is equal" What is foreign body granuloma?;"• After strabismus surgery, typically over muscles reattachment site • Localized, elevated, hyperemic, tender mass • More common in the past when sutures were made from gut Rx: topical steroids; if persistent, surgical excision" What is pyogenic granuloma?;"• Occurs after pterygium removal or strabismus surgery, over muscles reattachment site • Reactive hemangioma, contains fibroblasts and proliferating capillaries • “Spoke wheel"" vascular pattern on histology • Onset 3 4 weeks after surgery • Very red Rx: topical or intralesional steroids; if persistent, removed with cautery and frequent post op steroids" Downbeat nystagmus is associated with what lesion/location?;"• Cervicomedullary junction (Arnold Chiari), cerebellar flocculus • In Arnold Chiari malformation, defect of base of skull and upper spinal area • Downward displacement of cerebellar tonsils through foramen magnum" See saw nystagmus is associated with what lesion/location?;"• Parasellar area (craniopharyngioma), midbrain" Opsoclonus nystagmus is associated with what lesion/location?;"• Neuroblastoma (from adrenals)" Periodic alternating nystagmus is associated with what lesion/location?;"• Cervicomedullary junction, cerebellar nodules" Upbeat nystagmus is associated with what lesion/location?;"• Cerebellum, medulla, midbrain" Spasmus nutans nystagmus is associated with what lesion/location?;"• Parasellar/hypothalamic tumors" What is superior oblique myokymia?;"• Monocular oscillopsia with torsional movement of involved eye otherwise neurotically normal Rx: carbamazepine, phenytoin, topical timolol, surgery with superior oblique tenectomy + inferior oblique weakening" What is latent nystagmus?;"• Horizontal jerk nystagmus only when either eye is occluded • Fast phase is toward eye that is NOT occluded • VA is good when both eyes open, worsens when either eye is covered • Common in congenital esotropia and dissociated vertical deviation • Nystagmus: exponential decrease in slow phase velocity (opposite of congenital motor nystagmus)" What is the risk of aphakic glaucoma after surgery for congenital cataracts?;"• Varies from 15 50% • Typically occurs several years after surgery • More frequently when cataract surgery was during infancy • Risk is highest in children with microcornea" What are the findings of Weill Marchesani syndrome?;"• High myopia, Brachydactyly, Short stature, Microspherophakia , Ectopia lentis • Mutation of ADAMTS10 • Risk of small loose lens moving anteriorly and causing pupillary block glaucoma Rx: prophylactic LPI, cycloplegic during acute attack of pupillary block will allow the lens to move back posteriorly" Inferior rectus resection can cause what type of eyelid change?;"• Lower lid elevation • Opposite for inferior rectus recession" What is the inheritance of hereditary cataracts?;"• Autosomal dominant, always bilateral" When should unilateral congenital cataracts be removed?;"• Before 6 weeks of age" When should bilateral congenital cataracts be removed?;"• Before 10 weeks of age" What is Alport syndrome?;"• Mutations in basement membrane (type 4 collagen) COL4A5 gene • X linked (85%) • Blood in urine, hearing loss, eye abnormalities: retinopathy (dot and fleck) and lenticonus (anterior lenticonus = oil droplet, spherophakia, cataracts)" What is Usher syndrome?;"• Congenital sensorineural hearing loss and retinitis pigmentosa" What is Saethre Chotzen syndrome?;"• Mild condition type of craniosynostosis • Mutation in TWIST gene • Plagiocephaly, ptosis, mild brachydactyly, mild syndactyly, lateral deviation of big toes" What is Crouzon syndrome?;"• Autosomal dominant • Craniosynostosis (fusion of both coronal sutures leading to ""tower skull”) • Normal intelligence • Findings limited to skull, does NOT have hand/feet/limb deformities" What is Apert’s syndrome?;"• Autosomal dominant • Syndactyly of hands and feet (dominant feature) • Internal organ malformation, abnormally fused skull sutures, intelligence often affected" What is Pfeiffer syndrome?;"• Autosomal dominant, craniosynostosis (cloverleaf shaped skull), syndactyly of hands and feet short, broad thumbs and toes" What is dorsal midbrain syndrome (Parinaud syndrome)?;"● Paralysis of upgaze defective (voluntary) ● Convergence pupillary light near dissociation ● Convergence retraction nystagmus with downward rotating OKN ● Eyelid retraction (""Collier's sign"") ● Most common causes: ○ Children → pinealoma, congenital aqueduct stenosis ○ Young adult women → multiple sclerosis ○ Elderly → midbrain stroke" What is the treatment for persistent NLDO past 12 months of age?;"• Probing, 90% success rate • If epiphora persists then repeat probing, intubation, inferior turbinate infracture • Last resort DCR" What is an example of Pierre Robin sequence?;"• Frequent finding in Stickler syndrome" What is dissociated vertical deviation (DVD)?;"• Present in more than half of congenital esotropia • DVD usually occurs after 2 years of age and occurs regardless of whether original horizontal deviation was surgically corrected • Not true hypertropia, violates Hering's law • No corresponding downward movement of opposite eye as the hypertropic eye moves downward to refixate" What syndromes present with CPEO symptoms?;"• Kearns Sayre syndrome • Oculopharyngeal dystrophy • Myotonic dystrophy" What is chronic progressive external ophthalmoplegia (CPEO)?;"• Progressive ptosis • Ophthalmoplegia mitochondrial disorder mtDNA point deletion • Generalized muscle weakness • Muscle biopsy = ragged red fibers" What is Kearns Sayre syndrome?;"• CPEO + pigmentary retinopathy • Mitochondrial disease • Cardiac evaluation essential to rule out heart block • Associated with cardiomyopathy and cardiac conduction abnormalities" What is oculopharyngeal dystrophy?;"• Autosomal dominant, seen in French Canadian ancestry • Presents later in life 50 60s • Progressive dysphagia, muscle weakness, ptosis, CPEO" What is ectopia lentis et pupillae?;"• Bilateral, lens subluxation opposite direction of eccentric pupils • Most commonly autosomal recessive • Poor dilation, persistent pupillary membranes, TIDs, early cataract, glaucoma, high myopia, retinal detachment" What is DeMorsier syndrome (septo optic dysplasia)?;"• Congenital, marked midline brain development abnormalities • Unilateral/bilateral optic nerve hypoplasia • Vision can range from near normal to severely impaired • Nystagmus frequently present, develops around 1 4 months of age when pathways required for normal fixation typically mature • Fundus exam will show optic nerve hypoplasia • Require MRI and Endocrine referral for hormone deficiency (GH)" What is achromatopsia?;"• Rod monochromatism • Autosomal recessive • Congenital absence of cone cells • Poor central vision, no color, nystagmus" What is Leber's congenital amaurosis?;"• Autosomal recessive • Disorder of rod and cones • Severe form of retinitis pigmentosa • Poor vision, pupils sluggish • Retinal exam normal in early life, then becomes similar to retinitis pigmentosa (bone spicules, optic nerve pallor, attenuated vessels, etc)" What is cortical visual impairment?;"• Poor vision, pathology occurring somewhere from lateral geniculate nucleus to primary visual cortex in occipital lobe • Causes: periventricular leukomalacia, stroke, CNS malformations, utero infections • Usually normal fundus and ERGs" What is the most common cause of amblyopia in congenital ptosis?;"• Anisometropia amblyopia • NOT occlusion amblyopia unless ptosis is markedly severe, even in severe cases, patient compensates with chin up position • Astigmatism persists even after ptosis is repaired • Spectacle correction and occlusion therapy must be maintained after ptosis surgery" What is "Hang back" technique?;"• Recession technique used whereby muscle is suspended to the stump of the original insertion with nonabsorbable suture • Useful for patients with thin sclera (high myopes) where posterior suture pass risks perforation" What is isometropic amblyopia or (bilateral refractive;"• Children with large amount of uncorrected hypermetropia, myopia, astigmatism, or combination in both eyes • Mechanism: pattern vision deprivation in both eyes Treatment: refractive correction alone because bilateral process" What is Monofixation syndrome?;"• Certain sensory state in strabismus • Peripheral fusion but without bimacular fusion due to macular scotoma • Typically have stereoacuity in the range of 200 3000 sec of arc • Can be diagnosed with Worth 4 dot test • When flashlight at 10 feet, light projects on central 1 degree when viewed at this distance, will only see 2 or 3 lights (non fusion response due absence of bimacular fusion) • When flashlight at 2 feet, light projects on peripheral retina, will see 4 lights (fusion response) at near since peripheral fusion present" What is Neurofibromatosis type 2?;"• Autosomal dominant, mutations in NF2 gene, chromosome 22 gene produces protein merlin (aka schwannomin) acts as tumor suppressor gene • Associated with bilateral vestibular schwannomas (hearing loss and tinnitus), intracranial and spinal meningiomas and schwannomas of other cranial nerves • Most common ocular pathology: cataracts (60 80%), posterior subcapsular and wedge cortical • Less common ocular findings: retinal hamartomas, RPE hamartomas, optic nerve sheath meningiomas, epiretinal membranes" What is the order of magnitude of different fusional amplitudes in an average person?;"• Convergence of fusional amplitudes > divergence fusional amplitudes > vertical fusional amplitudes • Distance fixation at 6 meters: 1. Convergence fusional amplitudes 14 prism diopters 2. Divergence fusional amplitudes 6 prism diopters 3. Vertical fusional amplitudes 2.5 prism diopters" What is red glass test?;"• One way to test for anomalous retinal correspondence • Reason for test is to avoid post op diplopia surprises • First measure patient’s deviation objectively with prism • Then red glass placed over non deviating eye while fixates on white light • If sees one light, suppression is present • Sees lights superimposed (pink), harmonious ARC • Sees 2 lights but separation, unharmonious ARC" What is the treatment of primary congenital glaucoma?;"• 1st goniotomy or trabeculotomy • If failed 2nd goniotomy or trabeculotomy • Trabeculectomy (+/ (+/--) MMC or drainage implant • Success rate of 1 or 2 angle surgeries for PCG in infants presenting from 3 months to 1 year is approximately 80%" What is the change in a child's refraction from birth?;"• Humans begin life hyperopic due to relative steepness of cornea and short axial length at birth • Hyperopia continues to increase until it peaks around 6 8 years of age • Then myopic shift towards emmetropia process in which refractive power of eye is adjusted during childhood = emmetropization" What is the definition of amblyopia?;"• Visual acuity 2 lines worse than normal eye" Manipulation of which extraocular muscle is most likely to result in oculocardiac reflex?;"• Medial rectus" What is an OKN drum?;"• Elicit optokinetic reflex whereby patient has quick refixation movement in opposite direction of rotating bars if vertical reflex occurs 🡪 VA 20/400 or better" What is optic nerve hypoplasia in children associated with?;"● In general, most commonly maternal ingestion of phenytoin, LSD, quinine, alcohol ● Superior segmental optic nerve hypoplasia most commonly associated with maternal diabetes, also occurs spontaneously ○ Dense inferior VF defects" What is Kawasaki disease?;"● Characteristic findings: Fissured lips, bilateral conjunctival injection, inflamed tongue papillae (""strawberry ● Systemic vasculitis affecting medium sized vessels, including coronary arteries ○ Risk of death from coronary artery aneurysms → obtain echocardiogram ● Anterior uveitis is self limited and does not require treatment Rx: aspirin, IVIg Avoid systemic steroids → risk of worsening rate of coronary artery aneurysm" How to perform strabismus surgeries in thyroid associated ophthalmopathy?;"• NEVER involve resecting antagonist to restricted muscle • In general, NEVER perform resection for TAO strabismus" How is the light reflex related to estimation of deviation?;"• 1 mm of corneal light reflex equals approximately 7 degrees, 15 PD of deviation • Corneal light reflex at pupillary margin = 2 mm off = 15 degrees off = 30 PD off • Corneal light reflex at mid iris = 4 mm off = 30 degrees off = 60 PD off • Corneal light reflex at limbus = 6 mm off = 45 degrees off = 90 PD off" What are the findings of sarcoidosis in children?;"• Keep in mind ACE level in normal children is typically higher compared to adults • <5 yr old: associated with rash and arthritis more commonly whereas lung involvement less frequent • Same as adults: anterior uveitis more common than panuveitis" What is optic nerve hypoplasia associated with and its findings?;"• Double ring sign • De Morsier syndrome = septo optic dysplasia, ONH, absence of septum pellucidum, agenesis of corpus callosum, hypopituitarism 🡪 GH deficiency • ONH can occur with hypopituitarism independent of presence or absence of septum pellucidum" What is Stargardt disease?;"• AKA fundus flavimaculatus, most common of juvenile macular dystrophy (onset typically early teenage years) • VA 20/50 20/200 range • Bull's eye maculopathy • Loss of foveal reflex • Polymorphic sheen/beaten bronze appearance • Yellow deposits = pisciform flecks in paramacular area, relative sparing of peripapillary retina ""Dark choroid"" = retinal vessels are highlighted against a hypofluorescent choroid in early phase of FA due to blockage from diffusely distributed lipofuscin" What is microcornea?;"•<9 mm horizontal diameter in newborn • <10 mm horizontal diameter at 2 years of age • Seen in PHPV, colobomas, nanophthalmos • Clear cornea of normal thickness • Typically hyperopia because corneas are flat → more prone to angle closure glaucoma • Patients without angle closure glaucoma → 20% develop open angle glaucoma" What is Aicardi syndrome?;"• X linked dominant • Oval chorioretinal lacunae (lethal in male), agenesis of corpus callosum infantile spasms (before age 5 months) • Colobomas • Microphthalmos • Developmental delay" What is Batten's disease?;"• Optic atrophy, macular pigmentary deposits, low/absent ERG • Neuronal ceroid lipofuscinosis results in seizure" What is a Duane's syndrome?;"• Nucleus of CN 6 absent • Aberrant branch of CN 3 that innervates lateral rectus associated with Goldenhar syndrome • Caused by defect in development during 4th week of gestation" What are the quantitative VA measurements in preverbal infants and toddlers?;"• Preferential looking (Teller acuity cards II or Cardiff acuity test) • Visually evoked potential" What are the methods to measure VA in 2 5 year old?;"• Allen cards • HOTV cards • E game" What two diseases have similar retinal findings to retinopathy of prematurity?;"• Incontinentia pigmenti/Bloch Sulzberger syndrome • Familial exudative vitreoretinopathy (FEVR) FEVR: genetic (autosomal dominant), affects growth and development of blood vessels in retina → vitreous traction, peripheral exudation, peripheral retinal breaks, incomplete peripheral retinal vascularization. PREMATURITY NOT A RISK FACTOR. IP/Bloch Sulzberger syndrome: X linked DOMINANT. Affects eyes, skin, and brain. Lethal in males. Bullae on extremities and hyperpigmented macules on trunk. Microcephaly, seizures, intellectual disability. Incomplete peripheral vascularization leading to neovascular membranes leading to retinal detachments" What are the 2 X linked dominant conditions?;"• Incontinentia pigmenti • Aicardi syndrome" What is posterior lenticonus?;"• Outpouching of central posterior capsule due to thinning in that area • ""Oil droplet"" appearance on retroillumination" What syndrome is associated with anterior lenticonus?;"• Associated with Alport syndrome Alport syndrome: glomerulonephritis, high tone deafness, dot and fleck retinopathy (>80%)" What is periodic alternating nystagmus (PAN)?;"• Congenital or acquired • Jerk nystagmus lasts for 60 90 seconds in one direction, then period of no nystagmus 10 20 seconds, then jerk nystagmus in opposite direction • Alternating head turning due to state of vestibular overactivity • Congenital PAN oculocutaneous albinism • Acquired PAN lesion of cervicomedullary junction, multiple sclerosis, Chiari malformation, cerebellar degeneration" What is the VA of a newborn at birth?;"• 20/400 • At birth, fovea does not have density of cone that is found in adult retina, reaches same density of cones after 1 year of life" The following types of nystagmus are associated with which corresponding systemic conditions? 1. Downbeat, 2. See saw, 3. Opsoclonus, 4. Periodic alternating nystagmus, 5. Upbeat;"1. Downbeat: cervicomedullary junction (Chiari type 1), cerebellar 2. See saw: parasellar, midbrain 3. Opsoclonus: neuroblastoma 4. Periodic alternating nystagmus: cervicomedullary junction, cerebellar 5. Upbeat: midbrain, medulla, cerebellum" What is Alexander's law?;"• Nystagmus more pronounced when gaze is directed toward side of fast beating component" What syndrome is associated with upper eyelid coloboma?;"• Goldenhar syndrome • Additional findings: limbal dermoids, preauricular skin tags, vertebral abnormalities, congenital heart disease, CNS abnormalities" What syndrome is associated with coloboma of lower eyelid?;"• Treacher Collins syndrome" What is the medical treatment of accommodative esotropia?;"• Echothiophate iodide • Suppress convergence in patients with high AC/A ratio • Stimulates ciliary muscle, reducing central demand for accommodation which in turn reduces excessive convergence of eyes • Causes prolonged paralysis with depolarizing general anesthetic like succinylcholine • Iris cyst formation prevented by co administration of phenylephrine 2.5%" What is the difference between dermoid cyst and epidermoid cyst?;"• Dermoid cyst wall has adnexal structures (sebaceous glands or sweat glands, hair follicles) • Epidermoid cysts do not have adnexal structures" What is Megalocornea?;"• X linked condition • Carrier mothers have slightly enlarged corneal diameters • 90% are male • Not associated with increased IOP • AC angle can be seen directly without gonioscope Associated with: lens subluxation, iris hypoplasia radial (transillumination defects), ectopic pupil" What are the common features of congenital (infantile) esotropia?;"● Onset before 6 months of age ● Cross fixation ● Dissociated vertical deviation ● Latent nystagmus ● Large angle of deviation ● Similar angles at distance and near" What is the PD difference between up and down gaze in "A" and "V" pattern deviations?;"•""A"" pattern at least 10 PD • ""V"" pattern at least 15 PD" What are the stages of ROP?;"• Stage 0: immature vascularization (no ROP) • Stage 1: demarcation line (no height) • Stage 2: ridge with or without small tufts of fibrovascular proliferation (has height) • Stage 3: ridge with extraretinal fibrovascular proliferation • Stage 4: subtotal retinal detachment • Stage 5: total RD" What are the different ROP zones?;"• Zone 1: posterior pole, circle centered on optic disc • Zone 2: edge of zone 1 to point tangential to nasal oral serrate and around area near temporal equator • Zone 3: residual crescent anterior to zone 2" What is dominant optic atrophy (Kjer optic atrophy)?;"• Most common hereditary optic atrophy • Autosomal dominant • Mutation in OPA1 gene • Blue yellow dyschromatopsia (tritan) • Temporal pallor with area of ""triangular excavation“ • Slow loss of vision • Absence of nystagmus • Mild to moderate vision loss (20/40 to 20/200)" What is autosomal recessive optic atrophy?;"• Begins in early childhood (before 5 years old) • More severe vision loss • Nystagmus 50%" What is Behr optic atrophy?;"• Hereditary optic atrophy • Onset of vision loss in early childhood (< 10 years old) • Associated with ataxia, intellectual disability, urinary incontinence" What distinguishes preseptal cellulitis from orbital cellulitis?;"1. Lack of restriction of ocular movements 2. Lack of proptosis 3. Lack of ocular congestion (chemosis, injection, increased IOP)" What is the Knapp procedure?;"• Transpose medial and lateral recti muscles to superior rectus • Indications for this surgery: monocular elevation deficiency with large hypotropia on primary gaze and chin up head position" What is the arterial blood supply for the extraocular muscles?;"• Inferior and superior recti carry bulk of blood supply to anterior segment • Each rectus muscle has 1 3 anterior ciliary arteries which are branches of muscular arteries which themselves are branches of ophthalmic artery • Lateral muscular branch supplies lateral rectus, superior rectus, superior oblique, levator palpebrae muscles • Medial muscular branch supplies inferior rectus, inferior oblique • Infraorbital artery partially supplies inferior oblique and inferior rectus muscles" What is methotrexate used for and what are the common side effects?;"• Commonly used for JIA • Most common side effect is GI upset • Other side effects: ulcerative stomatitis, hepatotoxicity, and pneumonitis" What genes are associated with different types of glaucoma?;"• Primary congenital glaucoma: CYP1B1, GLC3A, GLC3B, GLC3C • Juvenile onset glaucoma: GLC1A (aka MYOC) • Pseudoexfoliation glaucoma: LOXL1 • Normal tension glaucoma: OPTN" What effect can be seen with eyelids after inferior rectus resection?;"• Upward advancement of lower eyelid" What effect can be seen with eyelids after inferior rectus recession?;"• Pulling lower eyelid down" What is facultative suppression?;"• Intermittent misalignment • Suppression when eyes are deviated (no double vision) • Suppression absent when eyes are aligned" What is constant suppression?;"• Suppression present when eyes aligned or deviated (present all the i.e. amblyopia" What is central suppression?;"• Prevents 2 different images on the two fovea from reaching the brain • E liminates visual confusion" What is peripheral suppression?;"• Suppression of the image falling on the peripheral retina in the deviated eye • This is for elimination of diplopia • Only develops in immature visual system" What is the normal cup to disc ratio of a newborn?;"• Less than 0.3 • Usually less than 0.2 difference between cup to disc ratios between two eyes" What are the symptoms of neonatal chlamydia conjunctivitis?;"• Papillary reaction (NOT follicular reaction as seen in adults) due to immaturity of neonates lymphoid system • Follicular reaction occurs after 1 month of age when immune system is more mature. • Present at around 1 week of life • Risk of pneumonitis and otitis media Rx: oral macrolides" What is are the ocular findings associated with aniridia?;"• Bilateral hypoplastic irides, foveal hypoplasia, poor vision, nystagmus, photophobia (more light entering eye) • Glaucoma • Optic nerve hypoplasia • Cataracts • Corneal scarring later secondary to limbal stem cell deficiency PAX6 gene mutations Wilms tumor (only in sporadic)" What are Allen cards?;"• A method to test visual acuity, shows pictures instead of letters" What is a HOTV test?;"• A visual acuity test • Projecting the letters HOTV then point to the same letter on a card" What is a Teller card?;"• Visual testing for preverbal child • One side blank while other side has series of vertical black and white stripes • Small peephole in the middle through which examiner looks to observe which direction child gazes" What is the Vogt triad in tuberous sclerosis?;"1. Seizures (due to cortical tubers/calcified astrocytic hamartomas) 2. Intellectual disability 3. Facial angiofibromas (sebaceous adenomas) • Associated with retinal astrocytomas, ash leaf spot, subungual/periungual fibromas" What are the classic ocular findings of Axenfeld Rieger syndrome?;"1. Posterior embryotoxon 2. Iris strands 3. Corectopia with possible hole formation • Autosomal dominant mutation of PITX2 and FOXC1" What is Kasabach Merritt syndrome?;"• Sequestration of platelets within a large vascular lesion, such as a hemangioma, leading to thrombocytopenia • High mortality rate" What findings are there in Neurofibromatosis 2?;"• Bilateral acoustic neuromas • Multiple meningiomas • Posterior sub capsular or or wedge shaped cortical cataracts • Lisch nodules of the iris can occur in NF2 but are infrequent The gene for NF2 is located on chromosome 22" What findings are there in Neurofibromatosis 1?;"• Iris/Lisch nodules • Cafe au lait spots • Optic glioma • Osseous defects • Sphenoid wing dysplasia The gene for NF1 is located on chromosome 17 Also called Von Recklinghausen disease" What diseases are associated with congenital iris ectropion?;"• Neurofibromatosis 1 • Prader Willi • Facial hemihypertrophy" What effect does exotropia have on angle kappa?;"• Positive angle kappa (angle between visual axis and pupillary axis)" What is a posterior embryotoxon?;"• A thickening and anterior displacement of the Schwalbe line • May be an isolated finding in 15% of patients • Other associated conditions: Axenfeld Reiger syndrome, arteriohepatic dysplasia (Alagille syndrome), velocardiofacial syndrome" What is Chediak Higashi syndrome?;"• Autosomal recessive mutation in lysosomal trafficking regulator protein that renders inadequate phagocytosis • Recurrent pyogenic infection • Patients rarely survive past 10 years of age, die from infection • Albinism" What is Hermansky Pudlak syndrome?;"• Autosomal recessive • Low platelets • Interstitial lung disease • Granulomatous colitis • Albinism • Common among Swiss or Puerto Rican patients" What are the ocular manifestations of congenital rubella?;"• Pigmentary retinopathy • Cataract • Glaucoma • Poor visual acuity secondary to cataracts and microphthalmia, rarely CNV • Deafness • Strabismus" What are the blood supply to the extraocular muscles?;"• Medial muscular branch of ophthalmic artery (larger of the 2): medial rectus, inferior rectus and inferior oblique • Lateral muscular branch of ophthalmic artery: lateral rectus, superior rectus, superior oblique, and levator • Lacrimal artery: lateral rectus • Infraorbital artery: inferior oblique and inferior rectus" What are the causes of fetal intracranial calcifications?;"• Congenital toxoplasma gonfi: diffuse intracranial calcifications, hosts and reservoirs are cats • CMV: intracranial calcifications and hydrocephalus • Lymphocytic choriomeningitis virus (LCMV): host and reservoir are rats. Occurs earlier in pregnancy, from food contaminated w/rodent excrement or bite of infected rodent or airborne. Macrocephaly, hydrocephalus, periventricular calcifications w/seizures, chorioretinal scars, optic atrophy, nystagmus, strabismus • Cysticercosis: leukocoria, hydrocephalus • Tuberous sclerosis: intracranial calcified astrocytic hamartomas • Sturge Weber syndrome: ipsilateral leptomeningeal intracerebral calcifications, seizures, neurologic deficits, cognitive impairment" What are the risk factors for retinopathy of prematurity in premature babies?;"• Blood transfusions • Poor postnatal weight gain • Sepsis" What are the different types of neonatal conjunctivitis?;"• Within 24 hours of birth: chemical • 3 4 days after birth: gonococcal • 1 week after birth: chlamydia • 2 weeks after birth: HSV" What association is there with sporadic aniridia?;"• WAGR complex: Wilms tumor, aniridia, genitourinary malformations, mental retardation" What are the findings of Goldenhar syndrome?;"• Duane syndrome • Microphthalmia • Eyelid colobomas • Iris abnormalities • Corneal hypoesthesia • Epibulbar (limbal) dermoids • Dermolipomas" What is an A pattern deviation?;"• Difference in measurement between upgaze and downgaze is at least 10 PD • Measurements in upgaze and downgaze are recorded 25 degrees from primary position" What is a V pattern deviation?;"• Difference in measurement between upgaze and downgaze is at least 15 PD • Measurements in upgaze and downgaze are recorded 25 degrees from primary position" What are the milestones for visual development?;"• 6 8 weeks: maintain visual fixation and react w/facial expressions • 2 3 months: interested in bright objects • 3 4 months: orthotropic; fix and follow to small objects; nasal bias for smooth pursuit resolved" What findings are associated with morning glory disc anomaly?;"• Retinal detachment • Basal encephalocele • Midface anomalies • Moyamoya disease (constriction of internal carotid arteries, recurrent strokes) • PHACE (posterior fossa malformations, hemangioma, arterial lesions, cardiac, eye anomalies)" Where do neuroblastomas originate from?;"• One of most common childhood cancers and most frequent source of childhood orbital mets • Originates either from adrenal gland or sympathetic ganglion chain in retroperitoneum or mediastinum • Interrupting of papillary sympathetic innervation can cause Horner syndrome" What are the findings in congenital CMV and congenital toxoplasmosis;"• Intraventricular calcifications • Microcephaly • Retinitis" What are the findings in Weill Marchesani syndrome?;"• Short status • Stocky body • Brachydactyly (short fingers and toes) • Microspherophakia" What is CHARGE syndrome?;"• Coloboma • Heart defects • choAnal Atresia • mental Retardation • Genitourinary abnormalities • Ear abnormalities • Associations: iris, optic nerve, chorioretinal colobomas" What is Möbius syndrome?;"• Congenital • Esotropic 50%, exotropia 10% orthophoria 40% • Lagophthalmos, limb/pectoral abnormalities • Commonly affected nerves CN6, CN7 • May also affect PPRF or CN6 nucleus" What are the ROP screening guidelines?;"● 1 week or less ○ Immature vascularization in zone I or posterior zone II ○ Stage 1 or 2 ROP in zone I ○ Stage 3 ROP in zone II ○ Presence or suspected presence of aggressive posterior ROP ● 1 2 weeks ○ Immature vascularization in posterior Zone II ○ Regressing ROP in zone I ○ Stage 2 ROP in Zone II ● 2 weeks ○ Immature vascularization in Zone II ○ Regressing ROP in zone II ○ Stage 1 ROP in Zone II ● 2 3 weeks ○ Regressing ROP in zone III ○ Stage 1 or 2 ROP in zone III" How far are the rectus muscles located from the limbus?;"• Medial rectus 5.5 mm • Inferior rectus 6.5 mm • Lateral rectus 6.9 mm • Superior rectus 7.7 mm The spiral made from the insertion distances is called the Spiral of Tillaux" What are the 6 cardinal positions?;"" What is the differential diagnosis for congenital corneal opacities?;"• Sclerocornea • Tears in Descemet’s (trauma) • Ulcer • Mucopolysaccharidosis/Metabolic • Peter's anomaly • Edema: Congenital hereditary endothelial dystrophy • Dermoid • STUMPED" What is the triad seen in congenital syphilis (Hutchinson’s triad)?;"• Interstitial keratitis • CN VIII deafness • Hutchinson teeth" What are the different subtypes of Duane’s retraction syndrome?;"• Type 1: abduction affected • Type 2: adduction affected • Type 3: abduction and adduction affected" What is the chance of a child getting retinoblastoma if their parent is affected?;"" What is Hering’s law of equal innervation?;"• Conjugacy of saccades is due to innate connections in which eye muscles responsible for each eye’s movements are innervated equally" What is the 4 diopter base out prism test?;"• Documents presence of a small macular scotoma in pt with monofixation syndrome and either micro strabismus or no manifest deviation • The prism is placed over non fixating eye, there is no movement of eye and no convergence movement of contralateral eye • Least reliable test to document macular suppression scotoma"