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114 Cards in this Set
- Front
- Back
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sherrington's law
What condition violates this law? |
innervation to ipsilateral antagonist muscle decreases as the innervation to agonist muscle increase
Duane's syndrome |
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Hering's law
what condition violates this law? |
There is equal and simultaneous innervation to all the synergistic muscles
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1. primary deviation
2. secondary deviation |
1. deviation measure with normal eye fixating
2. deviation measured with paretic eye fixing, larger than primary deviation |
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Panum's fusional area
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objects seen in panum's area are seen at 1 object while they fall in disparate areas in the retina.
outside panum's area --> double vision |
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EOM functions
Medial lateral superior inferior superior oblique inferior oblique |
medial - adductor
lateral - abductor superior - elevator, adductor, intorter inferior - depressor, adductor, extorter superior oblique - intorter, abductor, depressor inferior oblique - extortor, abductor, elevator |
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duction
versions vergences saccades |
1. monocular eye rotation
2. conjugate binocular eye movement in same direction 3. disconjugate movement of opposite direction 4. rapid eye movement of eye to place image to fovea |
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agonist
antagonist synergist yoke muscle |
1. primary muscle moving eye in a direction
2. muscle in same eye that oppose agonist 3. muscle in same eye that acts together with agonist 4. muscle in opposite eye that moves eye in same direction |
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angle kappa
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the angle between the anatomical axis and the visual axis
+ angle K: temporal postiion of fovea relative to pupil axis, cause temporal globe rotation. light reflex is nasal cause pseudoexotropia - angle k: nasal position of fovea relative to pupil axis, nasal rotation of globe, light reflex temporal cause pseudoesotropia |
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blood supply to eom
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inferior and superior muscular branches of ophthalmic artery provide all blood supply to all EOM except for lateral rectus
Lateral rectus supplied by vessel branch of lacrimal artery Each EOM is accompanied by 2 anterior ciliary anteries except lateral rectus |
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different methods measuring deviation
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krimsky - prism used to center corneal reflex
hirschberg - corneal reflex decentration helps estimate deviation. 1mm->7deg->15PD pupil border 15PD, pupil body 30PD, sclera 45PD Prism cover/cover uncover - measure tropia alt cover test - measures tropia and phoria bruckner test - use red reflex to determine strabismus, brighter reflex in deviated eye |
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Measure deviations with glasses
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Minus lens - measures more
plus lens - measure less %difference = 2.5(D) |
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amblyopia
-prevalence -types -treatment |
decrease vision not from structural abnormality, from fovea disuse. prevalence 2-4%
Strabismic - most common cause, crowding phenom, density filters don't reduce vision refractive - high refractive errors deprivation - media opacity, occlusion, ptosis. critical period for development of deprivation last longer. also shorter period needed to cause amblyopia organic - nerve/retina problem treat: remove anything blocking vision. occlude good eye, 1 week occlusion / 1year age. cont until no improvement, reeval at 9years old |
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monofixation syndrome
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peripheral fusion absent central fusion. develop foveal suppresion scotoma, minute small angle ARC, small angle deviation < 10. amblyopia common. caused by: 1ry condition, anisometropia, macular lesion, strabismus sx. use 4 PD base out prism test
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congenital esotropia
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onset by 6mo, have Fhx, neuro/developmental problem.
esotropia >30PD, can have cross fixation (VA both eyes equal). amblyopia occurs only if constant inturn of one eye only. assoc. DVD, Inf oblique overaction, latent nystagmus. asymmetry of smooth pursuit. treat: will not resolve itself. correct amblyopia if any, perform early surgery. aim within 10PD BL medial recession, MR recesion with ipsilateral LR resection. |
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accomodative esotropia
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from activation of accomodative reflex, onset avg 2.5yrs. amplyopia common
can be triggered by trauma/illness. Types: refractive (normal AC/A)- high hyperopia (avg +4.75) eso large angle 20-30 dist=near, full cycloplegic refraction corrects, treat amblyopia non-refractive-(high AC/A), eso near>dist, sight hyperopia like normal child, treat bifocals (+2.50 or +3.00 top of bifocal cross lower pupil border)/miotics partial or decompensated - have residual esotropia even after hyperopic correction, surgery |
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What is criteria for prescribing bifocals in accomodative esotropia?
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patient must fuse at distance and esotropic at near with full hyperopic correction.
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Echothiophate and side effects
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a cholinesterase inh, long duration, 3wks.miotic used in accomodative esotropia.
cause obicularis, ciliary spasms, cataract in adults, iris cysts in children, can accentuate succnylcholine effect in GA. |
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consecutive esotropia
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esotropia that occurs post exotropia surgery.
always rule out a slipped muscle. Treat with prisms, correct hyperopia, miotics, possible re-op |
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sensory exotropia
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exotropia occuring when there is a long standing VA loss
in children can be exotropia or esotropia in adults usually develop exotropia the angle of deviation is variable with time |
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intermittent exotropia
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most common childhood exodeviation. onset infant to age 4. stage 1-interminttent exo large at dist than near. stage 2- exo at dist more constant and near becomes exo.
stage 3 - exo at near and dist is constant, suppresion develops. amblyopia is NOT common. treat any amblyopia, overminus, prism base in, surgery via BL lateral rectus recession or LR recession and MR resection in one eye. *overcorrection of 10-15PD desirable, is persistent use base out prism or alt patching. |
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types of intermittent exotropia
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1. Basic- exo near=dist
2. Divergence excess- exo dist>near. Called true if exo dist>near despite 1hour occlusion. To test if high AC/A ratio test with +3D lens Called pseudo(simulated) if after occlusion deviation is same near=dist. 3. convergence insufficiency- onset usually after 10yo, exo is greater at near than dist. best treated with orthoptic exercises (pencil push ups, base out prism). can have blurred vision and HA when reading. |
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indications for intermittend XT surgery
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preserving binocular vision is goal.
1. increase in tropia phase 2. incresae ease of dissociation 3. poor recovery of fusion *size of deviation not a problem if cna fuse. |
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divergence insufficiency
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esotropia greater at distance than near. must rule out divergence paralysis due to pontine tumor, hear trauma, neuro abnormality.
treat with base out prism or surgery |
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A and V pattern strabimus
indication for sx types of surgery |
indication: abnl head position, improve motor alignment
Surgery: 1. no oblique muscle overaction, do vertical transposition of horizontal muscles (MALE) med to apex, lateral to empty space, move 1-1.5 tendon width. 2. if oblique muscle overaction present, inf oblique weakening, BL sup oblique tenotomy. |
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Brown syndrome
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cannot elevate eye in adduction. classic essential feature is exotropia on upgaze (not prominent in every case) aquired (trauma, inflammation, iatrogenic)or congenital. Have restricted force duction. V pattern. assoc with head posture or 1ry hypotropia. Treat: observe can spontaneously resolve, surgery indicated if head turn, hypotropia in 1ry, amblyopia, binocular vision disrupted.
Perform sup oblique weakening (tenectomy, tenotomy, spacer). most common complication is sup obliqie palsy. |
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duane's retraction syndrome
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congenital hypoplasia of CN 6 nucleus with aberrant regeneration to lateral rectus. common Female, left eye
Type 1-most common, limited abduction, narrow fissure on adduction, has esotropia Type 2-limited adduction, has exotropia Type 3-limited abduction and adduction, with globe retraction. surgical indication: significant head turn, primary gaze deviation. For esotropia, recess MR. For globe retraction, BL LR recession. For upshoot or downshoot, split LR or faden. AVOID RESECTION wil worsen globe retraction, up and downshoot. |
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superior oblique palsy
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Most common cyclovertical palsy. underaction of sup oblique iwth overaction inf oblique
Congenital: most common, has large fusional amplitude (>15PD) Acquired: mostly trauma. can be unilateral or bilateral. Unilateral: little V, small excyclotorsion <5, head tilt, + 3 step step to one side Bilateral: Large V, excyclotorsion > 10, 3 step test + both sides. Treat when: head tilt, hypertropia, diplopia. Unilateral: <15 D weaken ipsilateral inf oblique, > 15D weaken ipsilateral inf oblique and recess contralateral IR (contralateral yoke muscle of SO) or tuck the ipsilateral SO. >35D must work on 3 muscles recession of ipsilateral inf oblique, SO tendon tuck, contralateral IR recession. Bilateral: BL inf oblique weakening, to fix excyclotorsion harada ito procedure. |
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Inferior oblique overaction
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Occurs in congenital esotropia. usually bilateral but asymmetric. hypotropic when fixating with normal eye, when fixating invovled eye, hypertropic. deviation worse on gaze toward lesion, and head tilt away from lesion. V pattern. Treat with inf oblique weakening (recession, myectomy, anteriorization).
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DVD
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Intermittent deviation of non-fixing eye. upward, abduction, extortion. Unknown cause, violates herrings law. occur during occlusion or inattention. assoc with cong esotropia, inferior oblique overaction, latent nystagmus
Diagnose: bielschowsky phenomenon (increase light DVD eye drift up, decrease light DVD eye drift down). Red lens phenomenon: red lens on either eye will show red image always below white image. Treat: glasses correction, If increase in size, frequency, abnl head postion perform BL surgery (unilateral sx can reveal occult DVD. surgery: SR recession, IR resection. IO weakening or anterior transposition in IOOA with DVD |
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complications of strabismus surgery
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overcorrection (overcorrection causes diplopia most commonly), undercorrection (undercorrection more common than overcorrection), globe perforation (cause chorioretinal scar no visual problems), infection, suture granuloma, conj inclusion cyst, change in refractive error, dellen, conj scarring, ant segment ischemia, diplopia, change lid position, lost muscle, oculocardiac reflex
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Congenital CN 3 palsy
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can have complete of partial loss of SR, MR, IF, inf oblique, levator function.
Eye is down and out Can cause aberrant regenration. 75% has small pupil in invovled side (anisocoria and ptosi) Surgery to straighten 1ry gaze |
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What are the types of childhood nystagmus?
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congenital nystagmus
latent nystagmus sensory nystagmus spasmus nutans |
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Congenital nystagmus
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BL binocular horizontal nystagmus in all directions
waveform slow phase with exponential increase velocity. worst on fixation, has null pt causing head turn. Improve with convergence causing esotropia (eats up prism) no CN abnormalities. 60% with OKN reversal. nystagmus not in sleep. |
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latent nystagmus
Manifest nystagmus |
horizontal nystagmus occurs when one eye occluded. fast phase to uncovered eye. nystagmus chage direction depending which eye covered.
waveform exponentialy decrease in slow phase. assoc with congenital esotropia and DVD. OKN normal. Manifest nystagmus - latent nystagmus present when both eyes open |
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sensory nystagmus
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most common nystagmus in child. due to abnormal afferent pathway. occcurs in aniridia, rod monochromatism, CSNB, ON coloboma, cataract, ON hypoplasia, macular coloboma, leber's congenital amaurosis
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spasmus nutans
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fine rapid monocular asymetric nystagmus
Triad: head nodding, torticollis, nystagmus. rule out tumor in chiasm, get MRI |
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OKN response
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normal response, slow phase towards direction of moving stimulus, fast phase saccades to refixate to opposite direction. normal response indicates visual input present.
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Paradoxical pupil respoonse
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CSNB, achromatopsia, ON hypoplasia
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infant has poor vision, searching nystagmus, normal exam
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albinism, acrhomatopsia, leber's congenital amaurosis, aniridia, optic nerve hypoplasia
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mobius syndrome
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aplasia of brain stem nuclei, palsy of CN 6,7,9,12
have facial diplegia, gaze palsy, chest, limb and tongue defect. Patietns have esotropia with limited abduction, exposure keratitis from exposure. |
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ddx leukocoria
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retinoblastoma
congenital cataract PHPV coat's toxocariasis toxoplasmosis ROP RD coloboma uveitis retinal dysplasia Vit heme congenital retinal folds |
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ddx intraocular cartilage
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medulloepithelioma
PHPV trisomy 13 teratoma |
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Persistent hyperplastic primary vitreous
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occurs from incomplete regression of tunica vasculosa lentis and primary vitreous.
unilateral microphthalmia, retrolental plaque can have cartilage, elongated ciliary processes, prominent radial iris vessels, shallow AC, cataract, angle closure glaucoma, Vit heme, RD. Treatment- observe, lensectomy w/wo vitrectomy. |
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ROP
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vasoproliferative retinopathy occuring in premature infants.
risk low birth <1500g <1251g 65%ROP. early gestational age <33 weeks, supplemental oxygen. |
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ROP Zones
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zone 1- the posterior pole, 2x disc foval distance, centered around disc. worst prognosis
Zone 2- zone 1 to nasal periphery, temporally equidistant from disc. Zone 3- remaining temporal periphery |
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ROP stages
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Stage 1- demarcation line
Stage 2- Ridge Stage 3- Ridge with fibrovascularization Stage 4- subtotal RD Stage 4- Total RD |
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ROP definitions
Plus disease Rush disease threshold prethreshold |
plus: venous engorgement and arterial tortuosity in poterior pole
rush: plus at zone 1 threshold: Stage 3+ in zone 1 or 2, 5 continious clock hours or 8 non-continious clock hours Prethreshold: any stage less than threshold in zone 1, or stage 2+ or 3 in zone 2. |
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Treatment of ROP
When to serial exam |
Treatment: if reach threshold ROP, must treat within 72 hours with laser or cryo.
For stage 4, 5- RD repair with scleral buckle, vitrectomy or both. Serial exam: serial weekly exam in prethreshold patients |
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Children with hx ROP have higher incidence of:
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myopia, strabimus, amblyopia, macular dragging, cataract, glacucoma, RD.
Untreated patient needs follow up at 6 months to rule out these complications |
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Study: Cryotherapy for ROP Study (CRYO-ROP)
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Evaluated whether cryo for ROP in preterm <1251g prevented complicatons.
-cryo preserved VA in eyes with threshold disease. initial exam is 4-6 weeks post birth or 36 weeks gestaton. then every 2 weeks until vessels with zone 3. If prethrehold exam every week until regression or threhold reached. If threshold, treat with cryo within 72hours. 90% regression, retreat in 1 week if worsens. |
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Commitant esodeviatons
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measure angle of esodeviation is nearly constant in all fields of gaze.
congenital esotropia, accomodative esotropia, sensory esotropia, divergence insufficiency |
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incommitant esodeviation
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measured angle of esodeviation varies with direction gaze.
thnk of a neurological disorder (get head imaging), medial rectus restriction or lateral rectus paralysis |
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Exodeviation of children
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intermittent exotropia
sensory exotropia convergence insufficiency duane syndrome type 2 third nerve palsy orbital disease myasthenia gravis |
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Double elevator palsy (monocular elevation deficiency)
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limitation of elevation on primary gaze and on adduction and abduction. paralysis of inf oblique and SR. Have hypotropia of invovled eye worst on upgaze, chin up position, fusion on downgaze, ptosis or pseudoptosis.
types: 1. inferior rectus restiction (+force duction, no muscle paralysis, absent bells, extra or deeper eyelid fold on affected side) 2. With elevator weakness free force duction, muscle paralysis, bells normal 3. combination of inf rectus restriction and weak elevators (+positive force duction with muscle palsy) treat: indicated in large vertical deviation, chin up posture. If inf rectus restricted: recess IR. If no restriction, MR and LR transpose toward the superior rectus (Knapp procedure). defer any ptosis repair until vertical deviation corrected |
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Congenital cataract
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Bilateral, most common idiopathic, familial AD, consider galactosemia, PHPV, lowe's, rubella, Opacities of >3mm usually visually significant. need metabolic work-up and treatment by 3 months old. Remove cataract within days or weeks of discovery to prevent amblyopia if: risk of amblyopis, lens is responsible for eye disease, cataract progression threatens healh of eye. perform lensectomy, ant vitrectomy, post capsulotomy. Contact lens fitting or epikeratophakia to correct aphakia.
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most common type congenital cataract
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anterior axial embryonial
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congenital cataract assoc with metabolic disorder
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hypocalcemia
hypoglycemia - at pregnancy galactosemia - deficient galactose 1 P uridyl transferase mannosidosis - alpha mannosidase deficiency fabry's - alpha galactosidase deficiency alport's - x-linked, triad of ant lenticonus, deafness, hemorrhagic nephropathy lowe's - x-linked, defect amino acid metabolism, catarct, RTA, aminoaciduria, muscle hypotonia, MR |
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Lowe's syndrome
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x-linked, defect in amino acid metabolism, M>F. have BL congenital cataracts, microphakia, glaucoma.
systemically: renal tubular acidosis, aminoaciduria, muscle hypotonia, MR |
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Galactosemia
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Autosomal recessive, defect in galactose 1 P uridyl transferase, cannot convert galactose to glusose, excess galactose converted to galactitol (osmotic agent)
Cause BL oil droplet cataract (reversible early). systemically: hepatomegaly, jaundice. treat by restricting lactose (is fatal otherwise) |
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Congenital syphilis
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maternal transmission after 4th month gestation. have intersitial keratitis, anterior uveitis, secondary cataract, salt and pepper fundus, optic atrophy, argyll robertson pupil. systemically hutchingson's teeth, frontal bossing, saddle nose, deaf, arthorpathy, scar in angle mouth, MR. Dx: RPR, VDRL, FTA-abs (syphillis free infant can have + testing for 15mo if mother passed treponemal antibodies), LP
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Treatment indication for congenital syphillis
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Indicated when +VDRL with ons of the following:
1. clinical evidence syphillis 2. long bone radiologicla change 2. +CSF VDRL 3. unexplained increase CSF protein or WBC 4. blood VDRL 4x greater than moms 5. +FTA-abs |
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ophthalmia neonatorum
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newborn conjunctivitis, in first month of life. papillary conjunctivitis ( no follicles in neonates)
1. chemical- within 24-36hrs, commonly from silver nitrate. No treatment, lubrication 2. N.gonorrhoeae- seen in first few days of life hyperpurelent, see gram neg diplococci. treat with IV ceftriaxone 3. chlamydia trachomatis - most common infectous neonatal conjunctivitis. usually 2week, basophilic intracytoplasmic inclusion bodies in conj epithelial cells on Giemsa. assoc pneumonitis, otitis. treat with topical erythromycin with erythro syrup. treat mother and sex partners with doxy. 4. Bacterial - occur 4-5 days, staph, strep, gram neg on gram stain. treat bacitracin, erytho ointment 5. HSV - 5-14 days, herpetic vesicle on lid, see multinucleated cells in giemsa. treat with viroptic 9x/day and IV acyclovir. |
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Congenital lacrimal duct obstruction
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up to 5% infant have NLDO due to imperforate valve of hasner. most open spontaneously 4-6 week post birth. cause tearing, discharge, can have dacryocistitis, conjunctitivitis. dx: digital pressure over lacrimal sac cause reflux of mucus. treat by lacrimal massage, probing by 13mo age, if probing not work then silicone stent placement, DCR if multiple failure.
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Congenital glaucoma
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Mutation CYP1B1 accounts 85% congenital glaucoma. 70% BL 70% M, affected parent 5% change to have child with glaucoma.
symptoms: tearing, photophobia, blepharospasm, eye rubbing. have >21 IOP, high C/D (reversible), buphthalmos, enlarge cornea >13mm, haab striae, lens subluxation. Can be assoc with angle malformations (neural crest abnormalities). Diagnosis: EUA. definite treatment is surgical, goniotomy, trabeculotomy |
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congenital glaucoma with associated syndromes
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sturge weber
neural crest abnormalities (axenfield/riegers/peters) lowe's rubella aniridia neurofibromatosis homocysteinuria PHPV |
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Effect of halothane and ketamine in IOP
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halothane reduces IOP
katamine raises IOP |
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Megalocornea
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nonprogressive corneal enlargemnet, >13mm.
anterior megalophthalmos- x-linked, BL assoc with abnormalities iris, angle, lens, glaucoma. Have large cornea, weak zonules, can have lens subluxation. complications: glaucoma, ectopia lentis, cataract |
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posterior embryotoxon
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anterior displaced schwalbes line
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axenfeld anomaly
Alagille's syndrome |
1. posterior embryotoxon with iris strands over angle, 60% glaucoma
2. axenfeld with pigmentary retinopathy |
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rieger anomaly
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posterior embryotoxon with iris strands over angle and iris thinnig and distortion, 60% glaucoma
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rieger syndrome
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rieger's anomaly with dental, craniofacial, skeletal abnormalities can have short stature, cardiac defect, deaf, MR
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peter's anomaly
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PAX6, central corneal leukoma (absent descemet and corneal endothelium), with iris adhesions, can have cataract, 50% glaucoma, with dental, craniofacial, skeletal abnormalities
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DDX Ectopia lentis
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marfan's
homocysteinuria weill marchesani hyperlysiniemia sulfite oxidase deficiency ehlers danlos aniridia trauma congenital glaucoma megalocornea sticker's medulloepithelioma tertiary syphillis rarely PXE |
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Marfan's syndrome
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AD, defect in fibrillin. Cause superotemporal ectopia lentis. Assoc: glaucoma, keratoconus, cornea plana, myopia, salt pepper fundus, RD. Tall, spidery hands, arm span > ht, heart disease, aortic dissection
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homocysteinuria
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AR, deficient cystathionine B synthase, accumulation of homocysteine. deficient cysteine and weak sulfhydryl links cause weak zonule. cause inferonasal lens displacement, myopia, retina degeneraton, RD risk in cataract sx. patients tall, blond, osteoporosis, MR. have hypercoagulability increase risk of thromboembolic event in GA. Diagnosis: nitroprusside test, urine test (high homocysteine), amino acid assay, low folate. treat with Vit B6, restrict methionine, supplement cysteine.
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Leber's congenital amaurosis
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AR, like infantile RP cause severe blindness in infancy.
assoc with keratoconus, hyperopia, cataract, coloboma, MR, deafness, seizures, muscle abnl. have CF vision, nystagmus, poorly reactive pupil, oculodigital sign, retinal can look normal. ERG is flat, pathology shows absent photoreceptors |
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Albinsim
Ocular albinism |
AR, oculocutaneous, no pigment in skin, hair, eyes. tyrosinate neg: no pigmentation, decrease VA, TI defect, foveal hypoplasia, hypopigmented fundus, nystagmus, strabismus, myopia
tyrosinase positive: some pigment, ocular and systemic is less severe. Ocular albinism:xlinked, limited to the eye, less iris defect, female carriers ahve mosiac pattern of retinal pigment ahve normal skin pigment. |
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1. Chediak Higashi
2. Hermanski Pudlak |
1. Have large melanosomes in skin biopsy, reticuloendothelial dysfunction. cause recurrent infection and malignancy.
2. common in PR, clotting disorder due to abnormal platelet leads to bleeding |
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Aniridia
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BL absence of iris, usually have a rudimentary iris present. 3 types
AN1: most common, AD only eye invovled AN2: sporatic assoc. wilms tumor. WAGR AN3: AR, MR with ataxia have poor VA, foveal nad ON hypoplasia, nystagmus, amplyopia, strabismus Assoc cataract, glaucoma, corneal pannus. |
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juvenile retinoschisis
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x-linked, males, bilateral
present at birth, cause split at NFL have: foveal retinoshesis, lookslike CME, does not leak on FA. retinal vessels remain in cavities can break causing Vit heme. complications: RD, Vit heme. carriers have normal retina. ERG normal A wave until late, reduced B wave |
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morning glory disc
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unilateral in females, often in high myopia, cranial defects, ocular anomalies. find a cup filled glial tissue surrounded by pigment ring. retinal fold common. can develop peripapillary RD
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congenital rubella
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infection from mother in later first trimester cause a pearly white cataract. Live virus in lens, so it cataract surgery performed can release virus causeing AC inflammation. have salt pepper fundus (most commonly associated), glaucoma, microphthalmos, necrotizing iridocyclitis.
can have cardiac defect, deafness, MR. patients have either cataract or glaucoma rarely both. |
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toxoplasmosis
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infection by toxoplasma gondii, usually congenital, infection in early pregnancy. most common cause post uveitis, most common cause pediatric uveitis. tachyzoites cause inflammation. cause retinal coagulative necrosis and granulomatous choroiditis w/ vitritis. intraretinal cyst can cause reccurent disease. classic finding: inactive chorioretinal scar in the post pole often the macula. active lesions fluffy white, head light in fog, adjacent to old scar. pathology will have round toxoplasma cysts. diagnosis with ELISA, toxo IgG, IgM
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Toxoplasmosis treatment indication and treatment
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indications:
1. decrease vision 2. severe vitrous inflammation 3. lesion threatens macula, optic nerve, papillomacular bundle ** peripheral lesions can be observed Use of antibiotics kill tachyzoites in active phase but not the cysts Treatment: clindamycin, sulfadiazine, pyrimethamine, (can use bactrim). steroids. |
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congenital hereditary endothelial dystrophy
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corneal clouding from defect of endothelium and descemets.
AR (CHED2): at birth, non progressive, nystagmus, no tearing, pain, photophonia AD (CHED1): later in life, progrssive, no nystagmus, +pain, tearing, photophobia |
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Congenital hereditary stromal dystropy
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AD, diffuse opacification of superficial stroma, looks feathery and flaky, peripheral clear. no cornea edema.
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kawasaki's disease
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systemic childhood inflammatory vasculitis with mucocutaneous involvement
onset <5yo common Japan siblings 10x risk Diagnostic criteria (5 of 6) Fever, BL conjunctivits, BL nongranulomatous uveitis, rash, cervical lymphadenopathy, oral lesions, lesions of extremities. Treat with Aspirin. Steroids will cause coronary aneurysm. 15% develop coronary aneurysm |
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Juvenile xanthogranuloma
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Histiocytic proliferation, have yellow orange nodules in skin, can affect iris, which cause spontaneous hyphema. lesions can regress spontaneously in 5 years. pathology: diffuse non necrotizing proliferation of histiocytes with touton giant cells.
treat iris lesions with steroid, RT, excision |
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Waardnburg's syndrome
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AD, lateral displacement of inner canthi and punta, have confluent brows, heterochromia iridis, fundus hypopigmentation. have sensorineural deafness and white forelock
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Dermoid cyst
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Most common orbital tumor in childhood. Arise from neural crest lined by keratinizing epithelium with dermal appendages. located superotemporal usually. killed with keratin. can cause bone erosion, if ruptured cause inflammation. treat with en-bloc incision
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neuroblastoma
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most common metastatic orbital tumor in children. 40% orbital mets. Origintates from adrenal gland, sympathetic ganglion, mediatinum, neck. Cause sudden proptosis, periorbital ecchymosis. If horner's and opsoclunus present can have better prognosis. Pathology: sheets of indiscrete round cells, little cytoplasm, mitotic figures, tumor necrosis, bony invasion. CT-scan look for bone destruction. Treat with chemo and RT. Prognosis is poor if onset >1 yr old and mets to bone. If mets to liver, bone marrow, spleen survival 85%
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capillary hemangioma
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most common benign tumor of orbit in children. shows in the first few weeks of life, regress by 5-8yrs. common in superonasal quadrant of orbit, medial upper lid. It is a high flow lesion. skin is elevated red and blanches. treat with lesion is risk for amblyopia.
treat: observe, steroid inj (risk hypopigmentation, CRAO, fat atrophy, adrenal suppresion) kassanbach merrit - consumptive coagulopathy due to platelet trap, cause thrombocytopenia and cardiac failure |
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Leukemia
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most common malignancy in childhood, unilateral, affect the choroid with retinal heme. cause blurry vision and floaters. can have infiltration of vitreous, retina, optic n, uvea. ON infiltration can cause vision loss and ON swelling. Infiltration of orbit cause proptosis, lid swelling, ecchymosis. treat with ugent RT for ON infiltraton. have increase risk of radiation optic neuropathy is radiation and chemo used concurrently.
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malingnant hyperthermia
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acute metabolic disorder fatal if not treated early. mortality rate <10% incidence higher in children with (strabismus, myopathy, ptosis). diagnosis via clinical signs: tachy, increase end tidal CO2, trismus, increase temp, resp/metabolic acidosis, hyperkalemia, hypercalcemia, renal failure, DIC, cardiac arrest. triggered by succinylcholine, amide anesthetics. treat by stopping trigger agent, hyperventilate, IV dantroline.
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eye popping reflex
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neonatal reflex, widening of palpebral fissure after decrease light or loud noise. present first 3 weeks of like
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List phakomatosis
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All phakomatosis are AD except for Sturge Weber (sporatic), Wyburn Mason (sporatic), and Ataxia telangiectasia (AR)
Neurofibromatosis Sturge Weber Ataxia Telangiectasia Wyburn Mason Tuberous Sclerosis Von Hipple Landau |
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Neurofibromatosis
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AD, schwann cell and melanocyte disorder. Get hamrtoma in skin, eye, CNS
NF-1 chromosome 17. most common phakomatosis. criteria for diagnosis (2 or more) >6 cafe au lait spots >2 neurofibromas or 1 plexiform neurofibroma freckling intertriginous O.N glioma > lisch nodules osseous lesions (sphenoid wing dysplasia) first degree relative NF-2 Chromosome 22 have BL acoustic neuroma, heairng loss, ataxia, HA. assoc with pheochromocytoma, no lisch nodules present |
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Sturge Weber
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Sporatic, have port-wine stain (V1, V2). have tortous vessel in conj, sclera. heterochromia iridis, diffuse cavernous choroidal hemangioma. can have glaucoma from elevated EVP. can have leptomeningial vascular malformation, brain calcifications, seizures, MR.
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Von Hipple Landau
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AD, have hamartoma in brian, eye, kidney, adrenal. Ocular retinal angioma is orange red mass fed by dilated vessel drained by vein. leaks causing serous RD and (lipid exudation) macular edema commonly cause vision loss. assoc with hemangioblastoma of cerebellum, cyst in kiney, liver, pancreas, adrenal, renal cell carcinoma, pheochromocytoma. Can treat with cryo, laser.
Von Hipple: only eye involved. |
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tuberous sclerosis
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AD, triad of adenoma sebacium, seizure, MR.
Have astrocytic hamartoma of retina or ON (mulberry lesion). in the skin have adenoma sebaceum, ash leaf spot, shagreen patch, periungual fibromas. In brain have brain stones, MR, seizures, calcification. Can have spntaneous pneumothorax due to pleural cysts, and cardiac rhabdomyoma |
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ataxia telangiectasia
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AR, dilated conj vessels, impared convergence, nystagmus, oculomotor apraxia. Has skin telangiectasia, MR, cerebellar ataxia, thymic hypoplasia, Ig! defiency causing increase risk infection and malignancy
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Wyburn Mason
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sporatic, AV malformation in the retina, can have glaucoma, and hemorrhage. Assoc with AV malformation of brain
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retinal cavernous hemangioma
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cluster of intraretinal aneurysm, filled with venous blood. looks like grapes, assoc with cutaneous and CNS hemnagiomas. FA shows fluids levels without leaking
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Incontinentia Pigmenti
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X-linked, occurs exclusively in females, since males dies off
a proliferative retinal vasculopathy. Like ROP. leads to RD and retrolental membrane. skin changes: stage 1- erythematous macules, papules, bullae stage 2-vesicles become verrucous stage 3- lesions become pigmented stage 4-skin atrophies, hypopigmented treat retina with photocoagulation. can have hairloss, CNS abnormalitiy, dental abnormality |
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surgery in which EOM can cause lid changes?
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Sup rectus recession cause lid retraction while resection cause fissure narrowing
Inferior rectus recession cause lower lid retraction, while resection cause fissure narrowing when recession of muscles lid can be released or advanced |
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normal growth and development of eye
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4mm increase in axial length during first 6 months.
corneal diameter at birth 10.5mm increase to 12mm by 2 years age Cornea flattens in the 1st year of life, causing decrease corneal power there is a decrease in lens power in first year of life. |
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acquired unilateral cataracts in full size eyes
ddx of unilateral congenital cataract |
almost exclusively posterior lenticonus, amlyopia common
ddx: PHPV, anterior polar, posterior lenticonus |
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Which topical glaucoma medication is contraindicated in children?
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brimonidine associated with CNS depression under age 3
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anterior segment ischemia following strabismus surgery
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blood supply to anterior segment comes from anterior ciliary artery which travel via rectus muscles. simultaneous surgery of 2 or more muscles has risk of ant. segment ischemia.
Earliest sign is cell and flair in anterior segment. can have K edema, descemet folds, severe cases necrosis and phthisis can occur |
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calculating AC/A ratio
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normal ratio: 3:1 to 5:1
heterophoria method AC/A = ((dev near - dev dist)/(accom demand)) + pupil dist (cm) Lens gradient: AC/A = (dev with lens - dev without lens) / lens power |
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What is the most common congenital infection in humans?
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CMV, occur in 1% of infants 90% asymptomatic
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Inferior oblique muscle most weakened by which procedure?
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anteriorization
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faden operation (posterior fixation suture)
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weakening muscle in field of action, place suture through belly of muscle to sclera. Used in DVD, nystagmus, high AC/A esotropia, non-comintant strabismus
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nystagmus surgery
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indicated to correct head turn by shifting null point closer to primary position.
1. kestenbaum-anderson procedure 2. 4 horizontal muscle recession |
