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DISCLAIMER--PLEASE READ While every attempt has been made to have dosages, medications, and treatment recommendations as accurate and up to date as possible, this manual is no substitute for clinical judgement and knowledge. Tthe authors are not liable for any action taken on the basis of this manual. Pediatric Ophthalmology Edited and Revised by Balaji Gupta MD, Meagan Celmer MD

1. Congenital abnormalities Eyelids

Ankyloblepharon

fusion of lids AD with incomplete penetrance surgery Blepharophimosis AD with almost 100% penetrance congenital tetrad syndrome with epicanthus inversus, telecanthus, blepharoptosis and phimosis strabismus, nystagmus, microphthalmus, lacrimal drainage problems, short PF, brow hair crossing in midline, flat brow, infertile females, hypoplastic tarsal plate with tight lower skin leading to ectropion chromosomes are usually normal Diff dx when no family history: Waardenburgs syndrome Williams syndrome FAS Trisomy 18 oculodigital syndrome Crytophthalmos Skin covers all ocular structures Distichiasis pull, electrolysis, cryo double freeze thaw surgery split tarsus and pull our each hair Ectropion usually with Downs or blepharophimosis variation with total bilateral eversion of upper lids in newborns with marked chemosis and prolapse of conjunctiva Entropion rare, by defn is isolated

surgery based on corneal status

congenital horizontal tarsal kink 180 degree bend of the upper tarsus to appose globe needs early surgery due to infection and corneal scarring Epiblepharon bilateral mild skin excess in lower lid with vertical cilia, most commonly asians r/o frank entropion observe and lubricate surgery for corneal problems with simple excision of skin or Quickert sutures Epicanthus rare at birth but up to 20% by 2 yo palpebralis (simple) equal, most common inversus - below, often with other abnormalities tarsalis - above, asians supraciliaris - from eyebrow Euryblepharon horizontally widened PF with anterior and downward placed lateral canthus and ectropion laterally observe since usually improves, consider tarsal strips procedure Eyelid Coloboma usually full thickness isolated defect in upper lid at inner 1/3 junction 20% are bilateral lower lid at lateral 1/3 often with systemic syndromes, especially AD Treacher Collins lower lid often partial thickness with adjacent margin deformities such as trichiasis Cornea

Megalocornea >13 mm (or > 12 mm in newborn) nl endothelial density r/o congenital glaucoma

simple usually isolated, nonprogressive anterior megophthalmos bilateral, most commonly XLR normal lens is too small for ciliary ring and leads to subluxation F carriers may have slightly larger cornea

iris TI defects ectopic pupil increased IOP cataracts rarely associated with renal cell cancer microcornea <10mm (<9 mm in newborn) hyperopes, AD>AR 20% with angle closure glaucoma, usually eye nl r/o nanophthalmos, microphthlamos Anterior segment dysgenesis AD Posterior embryotoxin

Axenfeld- Rieger syndrome ocular Iris strands and hypoplasia with posterior embyrotoxin Can look like ICE syndrome Glaucoma in 50% high iris insertion cryptless iris iris TI defects corectopia ectropion uvea microcornea megalocornea systemic craniofacial anomalies mental retardation maxillary hypoplasia telecanthus microdontia skeletal and spine deformities hypospadias pituitary gland anomalies redundant periumblical skin Peters anomaly Posterior corneal defect, stromal opacity, and iris strands 80% bilateral

often the leukoma clears with time associated with the PAX6 gene Congenital opacities facets, nebula, macula, leukoma corneal keloid probably from intrauterine trauma Corneal dermoid, congenital glaucoma sclerocornea bilateral or unilateral central clearer than peripheral cornea often with other ocular problems Forceps injury Localized unilateral opacity with tears of Descemets membrane Astigmatism and amblyopia Mucopolysaccharidosis, mucolipidosis Bilateral diffuse clouding with smooth epithelium MPS IS (Scheies syndrome) Mucolipidosis IV Conjunctival bx reveals abnormal cytoplasmic vacuoles CHED Bilateral diffuse opacity with corneal edema and increased corneal thickness CHSD AD flaky or feathery stromal clouding with normal thickness and smooth epithelium Optic Nerve hypoplasia non genetic, non-progressive loss of axons before full maturity

small disc with yellow ring surrounding it with or without pigment borders superior segment hypoplasia may be associated with maternal diabetes variable amounts with variable vision trial of patching in unilateral cases associations maternal DM young maternal age first child intrauterine infections LSD ethanol anticonvulsants systemic basal encephaloceles cerebellar vemis hypoplasia 4th ventricle dilation posterior fossa cysts visual pathway tumors septo-optic dysplasia (De Morsiers syndrome) short stature, nystagmus, bilateral ON hypoplasia less than 10% have useful vision (>20/100) midline defects of chiasm, septum pellucidum, corpus callosum, hypothalamus, 3rd ventricle, pituitary gland cerebral/cerebellar atrophy, hydrocephalus, porencephalic cysts 15-60% have pituitary hypofunction, especially growth hormone

Coloboma sporadic, but AD genetic forms have been identified ocular posterior embryotoxin, exudative RD, posterior lenticonus Systemic Dandy Walker cyst, absent corpus callosum, encephaloceles CHARGE syndrome includes coloboma, heart defects, atresia of choanae, retarded growth, genitourinary abnormalities, and ear problems Aicardi syndrome with infantile spasms, no corpus callosum, double X chromosome, multiple punched out lesions genetic Chromosomal abnormalities include trisomy 18, 22, 13-15 Mecek Gruber, Goltz, and Lenz Microphthalmia syndrome Morning Glory unilateral, nongenetic usually isolated without other findings enlarged, excavated ON with central core of glial tissue with a pigmented raised annulus of subretinal tissue that may have a blue color usually very poor vision variety of other ocular signs may be found up to 30% get serous RD systemic basal encephalocele, absent corpus callosum, cleft palate, renal abnormalities Pupil Miosis Abnormal dilator muscle or persistent pupil membrane Unilateral or bilateral, sporadic or hereditary

microcornea megalocornea iris atrophy iris TI defects myopia abnormal angle congenital rubella Lowes syndrome Mydriasis Many are the form fruste of aniridia, esp if central iris structures are missing Must consider neurologic, pharmocologic, and traumautic etiologies Retinal Situs inversus bilateral in 80%, often in tilted discs congenital tortuosity usually arterial, may be AD, associated hyperopia consider Tetralogy of Fallot, heart defects prepapillary arterial loops 1:2000, bilateral in 10%, usually inferior artery, not a hyaloid remnant associated artery obstruction, VH, hyphema av malformation and Wyborn Mason syndrome aka racemose angiomas, macrovessels Wyborn Mason has CNS vasculopathy Congenital hypertrophy of RPE (CHRPE) >4 lesions, FH indicative of polyposis

lesions <0.5 DD to >quandrant present in 2/3 of all kindreds grouped are called bear tracks can have thin rim of hypopigmentation hypopigmented spots inside which slowly enlarge peripheral lesions may be elevated combined harmartoma 90% present with decreased vision with tumors of posterior pole +FA leakage from vessels usually no growth, but contraction of glial tissue variable pigment in differential of melanoma and other tumors persistant hyaloid artery association with PHPV, prepapillary hemorrhage 2. Ectopia lentis

Ectopia lentis et pupillae AR, similar to simple ectopia with oval slitlike pupils often asymmetric and displaced in the opposite direction of the lens atophic looking, may poorly dilate, iris transillumination defects up to 30% with nuclear/cortical cataract that can rapidly progress axial myopia with potential retinal detachment Homocystinuria AR, 1/200,000 newborns, five times higher in Irish absence of cystathionine synthetase resulting in increased homocystine in urine and blood

ocular subluxated lenses inferonasally not present at birth but progressive 90% have subluxation by the third decade often spherophakic lens which can dislocate into anterior chamber long axial length predisposes to retinal detachment, esp after surgery peripheral chorioretinal degeneration systemic fair coarse hairfair skinmalar flushpoor peripheral circulationmental retardationmarfanoid habitusdecreased platelet lifearterial/venous thrombiosteoporosispancreatitisgeneral anesthetia increases risk of arterial and venous thrombi dietary management (low methionine and high cystine) may help Marfan syndrome AD, most frequent heritable cause of lens dislocation, 4/100,000 15% have no family history ocular up to 60% of patients have superotemporal subluxated lenses can be present at birth, progressive or stationary iris hypoplasia iridodonesis iris transillumination defects myopia high astigmatism keratoconus miosis fetal angle with glaucoma increased axial length and dislocated lenses predispose to retinal detachment

systemic skeletal and cardiovascular defects antibiotics for SBE prophylaxis prior to surgery echocardiogram, increased urine hydroxyproline long extremities long fingers joint laxity pectus excavatums coliosis aortic insufficiency dilation of ascending aortaaortic aneurysm Simple ectopia lentis AD, often present at birth bilateral, symmetric, superotemporal subluxation with iridodonesis often small spherical lenses no systemic problems glaucoma more common with later onset Weill Marchesani Syndrome AR, 1:100,000 spherophakia, ectopic lens inferonasally with eventual dislocation high myopia up to 20 diopters episodic pupil block glaucoma are almost inevitable and is worsened with miotics cycloplegics can induce pupil block stubby broad hands and feet joint stiffness

muscular appearance thick skin Misc congenital glaucoma Ehlers Danlos hyperlysinemia sulfite oxidase deficiency Lowes syndromeaniridia 3. Glaucoma 1/2 of all childhood glaucoma is secondary and acquired Congenital M>>F, worse prognosis if at birth (10%), infantile much better over 80% are diagnosed by 12 months of age

tearing photophobia blepharospasm bilateral in 75% IOP >20 peripheral iris vessels iris processes high iris insertion corneal edema corneal enlargement Haabs striae

genetics most cases are sporadic affected parent has 5% chance of having affected child no hereditary relationship to POAG or steroid response measurements normal eye at birth has <10.5 mm corneal diameter and <16 mmHg IOP anesthetics lower IOP except ketamine, succinylcholine Descemet's breaks are often horizontal and should be drawn check axial length and refraction for myopia gonioscopy more difficult than adults wide indistinct ciliary body band thin anterior stroma at periphery with flat iris attachment look for iris insertion at or anterior to scleral spur trabecular meshwork is pale and may have a stippled orange-peel appearance Schwalbes line is indistinct

50% have trabecular meshwork dysgenesis (best prognosis) others with iris hypoplasia and malformation of collarette, analmous iris vessels (tunica vasculosa lentis or wandering iris vessels), and structural defects. Rx Pediatric glaucomas need, in general, surgical intervention Associations Ocular anterior dysgensis

ectopia lentis aniridia congenital cataract trauma familial iris hypoplasia(AD) aphakia Metabolic Lowes syndrome mucopolysaccharidosis Systemic Neurofibromatosis type 1 Rubella chronic uveitis Down syndrome Rubinstein-Taybi syndrome Oculodentodigital syndrome Aniridia 1:100,000 20/200 Visionnystagmusmacular hypoplasiaoptic nerve hypoplasia (75%)congenital cataractssubluxationmicrophakiacorneal hazeprogressive pannus glaucoma common cause of blindness in 30-50% of pts due to angle closure as well as mesodermal tissue in angle poor surgical results with vitreous loss and cataracts AN1 (85%) familial with isolated ocular manifestations in AD pattern with complete penetrance but variable expressivity 2/3 of patients have affected parents usually punctate cortical opacities needing surgery 30-40 yo AN2 (13% ) del of short arm chrm 11p-

sporadic nonfamilial associated with Wilms tumor (nephroblastoma) may get visually significant nuclear cataracts 1/4 with Wilms tumor which is often diagnosed before age 5 GU abnormalitiessevere mental retardationfacial dysmorphismhemihypertrophylong facieslow set ears with poor lobesMillers syndrome WAGR (Wilms tumor, aniridia, genitourinary abnormalities, mental retardation) AN3 (3%) Gillespiess syndrome, AR, with mental retardation and cerebellar ataxia no Wilms tumor, CT shows abnormal structure of brain normal macula and optic nerve

4. Infections

Ophthalmia Neonatorium Conjunctivitis in the newborn period Affects only 0.1% of neonates with prophylaxis all prophylaxis effective against GC, not against TRIC 10% of cervix with GC or TRIC

W/U gram/giemsa (cytoplasmic inclusions), blood and chocolate conjunctival cultures, DFA or chlamidiazyme GC and TRIC infections may be associated with systemic disease

CLINICAL EXAM CANNOT DEFINE ETIOLOGY GC Hyperacute with purulent discharge PCN IV 100,000 units/kg/day qid x 7days in isolated setting note the emergence of some resistant strains to penicillin with an alternative regimen IV cefotaxime irrigate with saline q4, AS 0.5% bid

RPR, FTA, and notify public health TRIC 3-13 weeks of age TC ungt 1% tid x 3 wks, Erythromycin 50mg/kg P.O. qid x 2 wks if fails (10%) repeat/Bactrim 0.5 ml/kg/day bid x 2 wks treat the parents check for otitis media, pneumonitis HSV only in a systemic disease setting chemical (toxic) conjunctivitis Misc many bacteria have been cultured, but ? pathogens if w/u negative, use topical agents of choice Preseptal/Orbital cellulitis Staphylococcus, Streptococcus, and H. flu most common sinusitis is a common cause of orbital cellulitis other causes include URI, trauma, minor skin faruncle, dental disease blood cultures positive only 5% in post septal cases age may suggest a more benign course with <9 yo having less need for surgical intervention CT/MRI consider Ceftriaxone IM, IV with Nafcillin, nasal decongestants must carefully watch while on antibiotics Signs of orbital involvement Proptosis Pain on movement

Limitation of movement APD, color vision loss demarcation of skin findings at end of orbital septum TORCHS

Toxoplasmosis 3,000 children affected every year 70% pregnant women seronegative although placental transfer common in infected mothers, many infants will be normal IgM in infants is highly suggestive systemic disease hepatosplenomegaly diarrhea vomiting anemia convulsions hydrocephalus CT with intracranial calcifications ocular 80% of patients with systemic disease have ocular involvement up to 1/3 of children have bilateral visual loss will have unilateral visual loss isolated eye involvement is rare bilateral retinochoroiditis involving the macula microphthalmos

Cataract (10%) Nystagmus (25%) Strabismus (30%) optic atrophy Can even consider treating prior to birth in cases of known acquisition during pregnancy Rubella 80% infected if mom gets it 1st trimester of these 80% get defects, 50% have ocular problems salt/pepper retinopathy cataracts glaucoma iridocyclitis corneal opacities iris hypoplasia microphthalmia rare to have glaucoma and cataracts all are over 20 yr old, uncommon congenital condition in U.S. CMV 2% of all newborns get it (most common congenital infection) but 95% is subclinical maternal viremia is most common method of transmission, but can occur during birth consider lab studies in any newborn with congenital ocular defects not explained by other causes systemic fever hepatosplenomegaly jaundice

deafness pneumonitis seizures periventricular calcifications motor retardation microcephaly ocular variable and nonspecific necrotizing retinitis hemorrhage cataracts iridocyclitis optic nerve atrophy Peter's strabismus nystagmus up to 1/4 of severely affected infants have bilateral retinochoroiditis in multiple areas Lab IgM in a neonate is strong presumptive evidence liver bx and studies of cells in the urine CT calcifications Congenital HSV is type 2 Acquired either transplacentally or retrograde through vagina Up to 50% of infants can acquire it if mother has active lesions at birth Some are asymptomatic with ocular symptoms later on

skin vesicles, conjunctivitis, keratitis, cataracts, retinitis Syphilis 90% infected if mom has primary or secondary syphilis systemic bone bone marrow lungs hepatosplenomegaly CNS failure to thrive moist lesions of anus and mouth rhinitis saber shins Hutchinson's triad: peg teeth, sensorineural hearing loss, interstitial keratitis ocular bilateral salt/pepper retinopathyglaucomairitiscataractsVaricella rare clinically CR scars, Horner's, optic nerve hypoplasia, microphthalmia can have severe vitritis/retinitis, anterior uveitis

5. Leukocoria

Cataracts 1/3 genetic, 1/3 diseases, 1/3 idiopathic monocular are not metabolic or genetic

capsule forms in 5th week and limits entry of organisms opacities >3mm are visually significant

Anterior polar 1/3 are bilateral abnormal separation of lens from surface ectoderm 90% sporadic pyramidal 2 mm projecting cone with surrounding cortical opacity bilateral and sporadic subcapsular idiopathic, trauma, often acquired lenticonus Alports X linked dominant Difficult capsulorhexis Chronic steroid use may present with PSC cataracts hereditary nephritis sensorineural deafness PPMD whitish dots in macular and mid periphery Central Nuclear either the embryonic or fetal nucleus

usually non-progressive bilateral are often AD inherited Sutural Lamellar acquired usually after 6 months progressive hypoglycemia and galactosemia can cause lamellar pattern Posterior Posterior lenticonus looks like PSC, bowl like central outpouching unilateral, progressive at surgery, post capsule often thin wispy and difficult to peel off opacity PHPV posterior capsular associated with Downs syndrome Oil Drop cataract associated with galatosemia Lowe's XLR Systemic hypotonia hyporeflexia mental retardation Aminoaciduria metabolic acidosis

growth retardation renal failure often with early death ocular most commonly small thick opaque bilateral cataracts pupils can be very miotic with lens adhesions female carriers have white punctate opacities throughout their lenses glaucoma in 50% by age 6 but can be early on corneal keloids Hallerman Streif Labs especially to exclude Lowe's, galactosemia consider TORCHS titers even in unilateral cases Urine reducing substances, aminoacids, blood/protein copper, sediment Blood Ca+2/phos, glaucoma, amino acids, TORCHS serology, others

PHPV wide spectrum of presentation congenital, nonhereditary, usually unilateral not associated with other defects except cataracts retinoblastoma is rarely found in microphthalmic eyes

elongated ciliary processes microphthalmos shallow anterior chamber radial iris vessels retinal detachment retrolental plaque intraocular hemorrhage angle closure glaucoma US/CT very diagnostic natural history of untreated eyes with moderately severe disease is progressive shallowing of anterior chamber, cataract formation, and angle closure glaucoma Pseudogliomas benign neoplasm astrocytic hamartoma can mimic RB, involvement of disc, multifocal, bilateral usually with tuberous sclerosis, rarely with neurofibromatosis little postnatal growth

von Hippel angioma medulloepithelioma

uveitis nematode endophthalmitis usually older boys, inflammation more inactive lesions show more changes than RB (cataract, synechiae, etc) ELISA + 90% with 1:8 dil

retinitis, old VH

Non-rhegmatogenous retinal detachment Coats' usually older boys age 8-10 years old anomalous grapelike clusters of leaking blood vessels serous RD with dilated vessels can look like retinoblastoma aneurysmal dilation can look like angioma, but no feeder vessels about one half of untreated patients will progress treatment includes laser and cryotherapy retinal dysplasia juvenile retinoschisis Misc retinal hemorrhage retinal folds colobomas Retinoblastoma 1:20,000 births, becoming more common 6% with FH, 25% have genetic mutation 13q14 with low esterase D levels 1-3 y.o., present older when unilateral 70% are unilateral with 30% bilateral 1/5 of unilateral on presentation get 2nd eye affected later the differential dx of inflammation is the most difficult leukocoria most commonstrabismusinflammation (pseudohypopyon)Ca2+vitreous seedingnormal size globeglaucomahyphemaheterochromiafixed pupilserous RD with dilated vesselssigns different for endophytic/exophytic metastasis late with spinal cord, bone, skull, lymph nodes, abdomen pineal gland trilateral tumor with very poor prognosis up to 50% with secondary cancers especially osteogenic sarcoma, fibrosarcoma, rhabdomyosarcoma many years later Genetic risk history determine if germline or somatic germline in multiplex and multifocal cases simplex disease with unifocal RB has 12% risk for germline mutation remember carrier status due to 80% penetrance

unaffected parents with one child mutifocal RB have 6% risk for second child examine family for regressed retinocytoma Molecular genetic analysis can find the specific gene mutation using Southern blotting RFLPs to do linkage studies Diffuse infiltrating RB (1%) likely to be missed occurs later (6 y.o.), unilateral, grows slower RX CT, MRI: look for calcification, pineal gland U/S: A scan with high internal reflectivity and echo spikes from calcification, B scan with orbital shadowing bone scan, bone marrow, lumbar puncture as needed with massive tumor, enucleation may be primary procedure external beam radiation, Episcleral plaque, Photocoagulation, cryotherapy systemic chemotherapy regression TI cottage cheeseTII fish fleshTIII combo of aboveTIV white sclera5 yr survival >90%, poorer with metastasis Histology Flexner Wintersteiner rosettes -an attempt to make photoreceptors with clear lumen fleurette with outer segs of photoreceptors Homer Wright rosette- lumen with neurofibrillary material, also in medulloblastoma, neuroblastoma pseudorosette-tumor around necrosis viable tumor around vessels with areas of necrosis ROP 85% of ROP is transient disease with spontaneous regression examine all children born under 32 weeks or weighing less than 1,500 gm CRYO-ROP study had 6% of children <1251 gm reach threshold, 20% prethreshold, 60% have some ROP Risk factors include <750 gm, PCA of <28 wks, O2 > 3wks, black < white initial exam at a post-conceptual age of 32 weeks long term risks include pseudostrabismusmyopiaPAS and glaucomaRDcataractmicrophthlamiaphthisis classification stage 1linestage 2ridgestage 3neovascularizationstage 4partial RDstage 5total RDplus disease with ROP Zone I = rush disease, exam q48 hrs rx decreases the risks of a bad outcome by 50% treat for 5 continuous clock hours or 8 total clock hrs of Stage 3 plus disease

cryotherapy or indirect laser

6. Neuroophthalmology CN palsies congenital IIIrd maldevelopment, intrauterine injury, or birth trauma 1/2 of all IIIrd palsies in children usually one eye and isolated with ptosis, ophthalmoplegia, and pupil involved usually dilated but may be miotic with aberrant regeneration esp with adduction other than birth trauma, most are permanent IVth common, compensated until later in life VIth unilateral isolated finding rare and usually due to birth trauma most common congenital disorder with VI nerve is Mobius and Duanes syndromes often is transient acquired VI differential includes myasthenia gravis, myositis, and trauma benign usually recover by two months, otherwise image can be recurrent and even in other eye pseudotumor cerebri pontine glioma will develop other signs usually within two weeks of onset

INO, gaze palsies, VII nerve dysfunction posterior fossa tumors Horners syndrome in infantile cases, the third order neuron may degenerate transynaptically, so always investigate the second order neurons 20% will have some pathology which will develop within the first 18 months heterochromia is a soft sign for pathology consult from pediatrics commonly with physiologic anisocoria, so test with cocaine to confirm diagnosis true congenital birth trauma from forceps or shoulder dystocia aquired almost always with ipsilateral sympathetic chain chest lesions are always left sided since the right side sympathetic chain does not descend into the chest most common cause is neuroblastoma CNS tumorssyrinxinfiltrative lymphomavascular lesionspost ganglionic lesions are less common and often with vascular etiology of ICA workup history, palpate abdomen, pharmocologic localization, CXR, urine levels of metanephrine, brain and neck MRI Hydrocephalus nl LP in children is 180 mm water + venous pulsations means ICP is < 200 mm water most common infantile causes include chiari malformation, dandy walker cyst, aqueductal stenosis, scarring due to meningitis decreased upgazelight near dissociationabduction weaknessON atrophynystagmusXT with A patternNystagmus

warning signs for further workup include vertical componantsintermittant naturenon conjugatenew onsetloss of visionRetractionOptic nerve pallorInanitionCongenital bilateral, conjugate, pedular horizontal nystagmus fixation often makes it worse, may be better at near than far head turn with null point is almost always evidence of congenital origin differentiation is important since congenital nystagmus is a benign condition disappears with sleep Motor No ocular abnormality, normal visual potential decreased foveation time leads to decreased visual acuity jerk with pedular component and null point often have abnormal head positions to achieve null point Latent benign, never pathologic only seen on monocular gaze fast phase toward uncovered eye in reality, most of these have a fine nystagmus at all times test Va with +10 occluder Manifest Latent jerk nystagmus in patients with strabismus due to suppression fast phase in direction of fixing eye can become latent if eyes are aligned if eyes are straight, then one must have visual loss Sensory pathology from cornea to optic nerve such as macular hypoplasia from albinism, aniridia, optic nerve hypoplasia nystagmus starts in the first few months often develop nystagmus with severe sensory loss before age 2 can get nystagmus with visual loss up to age 4, and never after age 6

pendular with occasional jerk movements, slow searching conjugate movements carefully check media, refraction, foveal hypoplasia, and optic nerve atrophy/hypoplasia Spasmus Nutans onset 4-14 months rare to start after 3 years vision good, but can have strabismus, amblyopia nystagmus, torticollis, and head nodding (slow freq, 2-25 degrees) dissociated assymetric high frequency (15 hz) small amplitude nystagmus often have increased head bobbing when focusing on objects as this may suppress nystagmus and improve vision always check for optic atrophy, APD, decreased eating as associated signs of intracranial tumors get MRI due to association with abnormal brain findings usually goes away, often in less than one month, but can last 1-2 years

Diff dx: CNS tumors especially chaismal gliomamonocular blindnesscongenital nystagmus

Downbeat usually seen down/left or down/right more than straight down lesion of cervicomedullary junction Arnold-Chiari TII multiple sclerosis spinocerebellar degeneration lithium, phenytoin CVA (vertebral basiler system) syringomyelia neoplasm

platybasia, basiler invagination Vit B12, thiamine, magnesium def paraneoplastic syndrome associated with Hodgkins lymphoma, breast, ovarian, and small cell Upbeat if seen in primary upgaze, usually pathologic involving cerebellum or brainstem if seen in eccentric gaze, may be gaze evoked Gaze evoked stabilized with fixation, increased with darkness, visual blur posterior fossa or drugs Periodic alternating nystagmus horizontal, jerk changing direction from one side to other over a few minutes often with changing head position may have skew deviation, downbeat nystagmus congenital may be subset of congenital nystagmus (up to 5%) Kestenbaum may not work due to changing AHP consider four muscle recession associated with albinism acquired cerebellar disease, bilateral visual loss, neurodegenerative conditions check posterior fossa and craniocervical areas MR to r/o Arnold Chiari rx with baclofen Superior oblique myokymia

monocular episodic intorsion, oscillopsia or shimmering brief, lasting seconds, but patients very aware of onset and end can be induced by looking into field of action of SO or back to primary can have intermittant SO OA and loss of elevation in adduction can be the only symptom of midbrain tumor, consider imaging natural hx of remission and relapses INO adduction lag with nystagmus in abducting eye from interruption of MLF fibers diff dx of myasthenia, MS, CVA, partial IIIrd, thyroid eye disease See Saw acquired form with intorting eye rises with extorting eye falling often with INO congenital form with either absent torsion or opposite midbrain, chiasm, or otolith craniopharygioma pituitary tumors CVA trauma septo optic dysplasia Convergence-retraction Vestibular nystagmus Peripheral unidirectional, rotary, uniplanar greatest in gaze of fast componant

usually vertigo, tinnitus, deafness labrynthitis, ischemia, trauma, toxic Central unilateral or bidirect, may change direction ear symptoms less severe trauma, CVA, tumor, demylination

Optic neuritis rare, more often bilateral with disc edema may be less likely to develop MS 7. Phakomatoses Ataxia Telangiectasia AR, chromosome 11 ocular nystagmus, strabismus bulbar conjunctival telangiectasia especially at canthi, are venules, usually seen by 5 yo oculomotor apraxia with head thrusts, loss of OKN, poor saccades systemic ataxia in infancy or soon after walking with subsequent neural deterioration telangiectasia of ears, face, bridge of nose, extensor surfaces many cancers including lymphomas and leukemias immunocompromised with low IgA, increased ESR, CEA mortality from sinopulmonary infections carriers for the gene are more likely to develop malignancies and are more sensitive to radiation Incontinentia Pigmenti

patients often referred from dermatology after bx Bloch-Sulzberger type F, congenital X-linked dominant (lethal to males) systemic dental defects (60%) CNS (30%) includes mental retardation, seizures, hydrocephalus, cerebral ischemia and edema ocular (30%) can look like ROP AV anastomoses/ peripheral neoavascular zones exudates VH cataracts optic atrophy palpebral conjunctival nevi patchy retinal mottling foveal hypoplasia peripheral colobomas nystagmus strabismus skin lesions S1 appears like viral exanthem with papules, macules, and bullae over trunk/limbs and bx revealing intraepithelial vesicles filled with eosinophils S2 are verrucous S3 hyperpigmented whorls and streaks from 3-6 months of age and there is absence of pigment in basal epithelium JXG usually diagnosed <3 yo with single or multiple yellow, red, or brown papules 4-20 mm diameter on face, trunk, limbs

M=F, skin heals in 1-2 years without problems in general, a benign condition ocular can occur without skin lesions usually unilateral spontaneous hyphema from rupture of tumor vessels glaucoma secondary to direct trabecular obstruction uveitis can be with minimal anterior chamber involvement only or may simulate posterior scleritis heterochromia secondary to diffuse iris involvement iris or ciliary body tumor with either nodular or diffuse infiltration tumors can occur on eyelids (common), orbit (with bony destruction), cornea, and epibulbar tissues generally without spontaneous involution in eye, so may need to excise, steroids pathology skin or iris biopsy and anterior chamber tap classically with foamy histiocytes and Touton giant cells Touton giant cells can be found in aqueous Oil red O can be positive on frozen sections immunohistochemistry can be useful Neurofibromatosis Type I AD, 1:3000, progressive with variable expression, chromosome 17 Cafe au lait spots present by 1 yr, >6 over 0.5 cm, grow, anywhere on body freckling develops later in intertriginous areas Neurofibromas nodular or diffuse (rare), benign but functional/cosmesis problems

plexiform difficult to treat (see plastics) histo neurons, Schwann cells, fibroblasts, mast cells, vessels increasing superficial subcutaneous involvement is associated with less deep orbital involvement Lisch nodules indicates mutation at NF1 locus on long arm chromosome 17 affecting guanosine diphosphatase activating protein > 90% of pts over 6 y.o., increase in number no correllation with severity or prognosis usually bilateral, well defined dome shaped, gelatinous, elevated with smooth contour typically less pigmented, yellow or tan, variable in size, height, and distribution fleshy or velvety texture and often with glassy translucent character Other findings absence of sphenoid bonemeningiomasoptic nerve gliomaglaucoma especially on side of lid and facial involvementthickened corneal nervesastrocytic harmartomasscoliosisIncreased incidence of JXGoptic nerve glioma aka Juvenile Pilocytic astrocytomas PPLOV with APD, proptosis, increased ICP most often in first decade (avg 3-4 yo) usually more extensive ON and chiasmal involvement with NF1 if normal MRI scan by 2 yo, then unlikely to develop up to 15% of NF1 increased mortality with chiasmal involvement observe, radiation, surgery, biopsy proliferating spindly astroglia or oligocytes, Rosenthal fibers NF TII Chromosome 22 acoustic neuromas may have VII n involvement secondarily ON glioma > 50%PSC and cortical cataractslagophthalmoscorneal hypesthesiadecreased lacrimal secretionRetinal harmartomasSturge-Weber syndrome no hereditary pattern classic form includes facial hemangioma, ipsilateral intracranial hemangioma, ipsilateral choroidal hemangioma, and congenital glaucoma systemic cutaneous usually V1, V2 distribution not crossing midline deep purple in adults with partial blanching facial hemihypertrophy if there is no supraorbital facial involvement, neurologic features are often absent CNS subarachnoid racemose angioma of venules with an associated maldevelopment of nearby cerebral cortex

calcifications of meninges, cortex as early as 1 y.o. and does not progress after age 20 these can form the railroad track sign of curvilinear doubled lines in the occipital and temporal lobes decreased IQ in up to 50% of patients localized seizures of the contralateral side and may become generalized ocular subconjunctival vessels iris heterochromia, retinal tortousity choroidal hemangioma up to 50% of patients have a flat diffuse choroidal hemangioma yellow-orange, minimal elevation, diffuse, most commonly in posterior pole but usually involves over 50% of the choroid in children it may not be apparent except for the deep red coloring which has been labeled tomato catsup fundus slowly growretinal and choroidal degenerationgliosisSRNVMserous retinal detachmentfocal calcificationmarked early hyperfluorescenceglaucoma 1/3 develop glaucoma with congenital > adult onset most often when the eyelid is involved angle abnormalities vs increased episcleral pressure uncommonly bilateral, or bilateral glaucoma, visceral angiomas Rx skin lesions are treated with argon or dye laser, cosmetics surgery risk of hemorrhagic expulsion/effusion standard goniotomy, trabeculotomy have had poor success some success with trabeculectomy with adjuvant MMC associated syndromes Klippel-Trenaunay-Weber syndrome Tuberous Sclerosis AD, chromosome 9 but new mutations are evident in 80% of new cases systemic neurologic mental retardationepilepsyabnormal EEGlarge cortical massesintracranial calcifications of astrocytic harmartomas especially in basal ganglia skin lesions ash leaf signcafe au lait spotsadenoma sebaceum (80% ages 2-5 yo)shagreen patches (leathery, raised)peau d'orange in lumbosacral regionperiungal fibromassubcutaneous nodulesdepigmented nevimisc sclerosis of skullrenal cystsangiomyolipomasrhabdomyomas of heartWilms tumoraortic aneurysm ocular involved in 1/2 of patients giant astrocytic hamartomaVF defectssmall flat gray white astrocytic hamartomas of

NFLpoliosishypopigmented iris spotspapilledema

8. Plastics hypertelorism is increased distance between medial orbital walls telecanthus is increased distance between medial canthi exorbitism is widely divergent lateral orbital walls (>90 deg) Document palpebral fissure (PF) Often with poor lid crease and levator function Check tear function and corneal staining PF increases on downgaze Check for jaw winking and Bell's phenomenon Occlusion amblyopia is rare, most often anisometropic amblyopia from induced astigmatism Must carefully observe these infants for fixation preference Rx Indications for early surgery include occlusion and chin up head position If levator function is present, then consider levator resection, aponeurotic surgery Frontalis sling using a variety of suspension materials is commonly performed with poor levator function 1/2 of patients with banked fascia lata have late ptosis Tearing congenital dacryocele refer to ENT to look for polyps, look up the nose as well bluish cystic swelling medially with epiphora in first few wks of life F>M, isolated finding, NLD obstruction with functional proximal obstruction Differential dx encephalocelemucoceledermoidcapillary hemangiomasudoriferous cystRx conservative rx >50% will resolve (soaks, massage, topical antibiotics) surgery (probing, DCR) if dacryocystitis, cellulitis, respiratory distress, no resolution in 1-2 wks can recur with surgery Congenital NLD obstruction evident in 2-4% of babies stating at 1-2 wks after birth 1/3 bilateral, most systems become patent within 6 wks, 90% within one year check puncta, causes for reflex tearing including conjunctivitis, epiblepharon, or congenital glaucoma inspect medial canthus for distended sac or congenital defects digital pressure over the sac revealing mucopurulent discharge is highly suggestive of NLD obstruction usually at level of valve of Hasner rx with observation and antibiotics, probing, repeat probing, intubation, and DCR

Orbital Tumors 90% are benign usually cyts, most mal are rhabdo Rhabdomyosarcoma rapid proptosis with good Va, extraconal, often down palpable often preseptal, 7 yo, M>F CT shows irregular mass with uniform density low to medium amplitude internal reflectivity with ultrasound surgery bone marrow bx, LP fix in formalin and glutaradehyde for EM sections pathology embyonal most common, alveolar (worst, inferior), pleomorphic (best, least), botryoid (sinus invasion) treatment radiation 4-6000 cGys adjuvent chemotherapy (vincristine/doxorubicin/cyclophosphomide) with good survival Lymphangioma 50% present in preschool years most commonly presents with gradual proptosis during the first few years of life episodes of dark blue superonasal swelling, orbital hemorrhage or proptosis (avg 6 mm) during later years indolent growth with recurrent hemorrhage (can have N/V in children) paindiplopiasudden enlargement with URIhemorrhage (chocolate cysts)conjunctival ectatic lymph channelslid ecchymosisepistaxis (lesions in sinus/pharynx)ON edema/atrophyproptosis increases with valsalva 1/3 of posterior lesions are intraconal infiltrative, unencapsulated, large serum spaces, lymph follicles CT lesions often appear as a solid mass with phleboliths (1/2) Rx conservative some lesions arborize, bleed easily, and incorporate arteries anterior lesions more easy to dissect out may consider aspiration of hemorrhage, CO2 laser, debulking anterior lesions Pseudotumor (pediatric) In children, presents more like orbital cellulitis nonspecific, idiopathic, benign inflammatory process with polymorphous lymphoid infiltrate with varying amounts of fibrosis up to 15% of all pseudotumor <20 yo 1/2 children with systemic symptoms such as N/V, fever, abdominal pain, lethargy 1/2 have it bilaterally more anterior uveitis, optic nerve edema

more recurrent in children spontaneous orbital hemorrhage peripheral eosinophilia in 1/3 CT ring sign of scleritis, tendons inflamed, diffuse infiltrate with streaky edges Systemic steroids have dramatic effects Fibrous Dysplasia fibrous bone growth usually of multiple bones, 8 y.o. rapid growth with stability after puberty Albright's syndrome, systemic, skeletal bones, precocious puberty, abnormalities skin pigment observe unless optic nerve compression CT is best to image lesion, tumor has ground glass appearance spicules of immature bone and osteoid with fibrous stroma Ossifying Fibroma only occur in facial bones aggressive growth pattern needs total surgical excision CT sclerotic margin with less radiodense center and foci of calcification spicules of lamellar bone and osteoblasts present Osteoma slow growing along frontal/ethmoid sinus wall or others extremely radiodense well circumscribed masses usually subtotal resection for symptoms Neuroblastoma Mean age at diagnosis is 2 years old in orbital cases prognosis depends on age of onset and bone metastasis ocular up to 1/2 have metastasis to orbit, but only 20% show clinical evidence ecchymotic lids, unilateral or bilateral exophthalmos,Horner's syndrome, opsoclonus bx extraperiosteally to avoid spread and fix in formalin/glutaraldehyde Horner's syndrome may represent localized neuroblastoma in the neck or chest with a better prognosis systemic originates in adrenal glands or the sympathetic ganglion chain in the retroperitoneum or mediastinum systemic metastasis to abdomen, thorax, bones metastasis to lateral orbit with bone destruction and cranial metastasis catecholamines in urine pathology sheets of round cells with neurosecretory granules no Homer Wright rosettes in metastasis Eosinophilic Granuloma

superotemporal quadrant, M>F, <20 y.o., bone pain and local swelling Bx with EM having Langerhans or Birbeck granules very cellular solid tumor radiation, intralesional steroids, spontaneous resolution post bx Ewing's Sarcoma bone CA of long limbs, trunk, or ribs in 10-25 yo with orbital metastasis abrupt hemorrhagic exophthalmos with bony destruction rarely primary in orbit, does not involve the globe round cell tumor with PAS positive cytoplasmic vacuoles Wilm's tumor extremely rare orbital mets Leukemia early school years, proptosis, granulocytic sarcoma orbit involved more in AML cell nests called chloromas but now granulocytic sarcoma preferred < 10 yo, lid ecchymosis, M>F, Asia/African Ectopic tissue masses Choristoma Growths of normal tissues in abnormal areas Dermoid Cyst preschool years anterior to septum, smooth, painless, usually lateral superior rim, approximately 1 cm progress slowly, can grow rapidly with trauma and inflammation keratin filled cyst lined with epithelium, filled with adnexal structures deeper cysts often underestimated in size; may extend intracranially after fossa formation and are usually found in older patients CT image to r/o deeper intracranial involvement focal defects of orbit bony wall, bone attenuation, or bone sclerosis sharply defined margins with central low density, but often heterogenous densities surgery usually fibrous attachments to bone or suture recurrence most often with remnants inside bone may have to remove remnants off of lateral orbital rim Teratomas aberrant pluripotential germ cells with all three embryonic layers may present with massive proptosis at birth unilateral, cystic with rapid growth CT heterogeneous cystic lesion with intracranial spread surgical rx poor vision due to optic nerve damage most teratomas occur in the gonads or sacrococcygeal region

Encephalocele infancy or childhood with superomedial exophthalmos that may pulsate often protrudes with valsalva or dependent position cystic, fluctuant and reducible, and easily transilluminates must have skull deformity of frontoethomoidal area or sphenoid dysplasia other signs of CFA with hypertelorism, broad nasal bridge, and malar and orbital floor depression common may be associated with colobomas, optic nerve atrophy, microphthalmos, and optic nerve gliomas r/o neurofibromatosis may be ptotic with pulsating enophthalmos due to inferior floor loss greater injury with mild trauma imaging, neurosurgery Limbal Dermoid Inferotemporal with minimal postnatal growth May contain hair follicles Amblyopia due to astigmatism Lipodermoid Overlying epithelium is normal and hair follicles are absent Can be extensive Surgical excision should be conservative Capillary hemangioma 1-2% of newborn children have it with 40% in head and neck, F>M 1-2 wks postnatal, blanch, invasive, swell with crying, compressible grow up to 12 months and regress after age 2 (1/2 completely by 4 y.o. and 3/4 by 7 y.o.) upper lid, supranasal orbit with proptosis treat to prevent amblyopia secondary to occlusion, strabismus, and anisometropia severe cases can cause thrombocytopenia with platelet entrapment, optic atrophy, exposure keratitis, and skin ulceration steroids Oral: 1.5 - 2.5 mg/kg prednisone over weeks Injection: 40 - 80 mg triamcinolone with 6 - 12 mg of betamethasone minimal response in 10% risks include emboli, lid necrosis, fat atrophy, pigmentary changes, adrenal suppression Topical: surgery lid crease or transconjunctival, avoid entry into lobular mass which is usually encapsulated cauterize feeder vessels best for isolated orbital masses rather than large areas of skin

risks include blood loss, scarring

9. Retina Vitreoretinopathies All AD exept Goldman Favre Stickler's high myopiacataractperipheral vitreal veilsstrabismusperivenous radial lattice with RDEctopia lentiscleft palatePierre-Robin micrognathiahyperextensibilityArthritisCardiac defectsmid facial flattening FEVR AD with variable expression dragged macula with temporal traction abrupt end of vessels in a scalloped edge temporally can look like ROP with neovascularization and RD in up to 30% intraretinal hemorrhage/lipid in less then 10%, thus can mimic Coat's rare nystagmus/strabismus most RD occur before age 10 and then patients are stable over 50% of patients will have normal vitreous examine family members for subtle areas of peripheral avascular zones Goldman Favre AR present between 10 and 20 years old with nyctolopia often 20/200 in childhood extensive peripheral retinoschisisbeaten copper maculapoor visionRPE changesbone spiculesvitreal strandsPSC cataractsHVF similar to RP with ring scotomas Albinism See Retina Leber's congenital amaurosis (rod cone dystrophy) Hyperopia, sluggish pupils AR Most with normal posterior pole exam in infancy Oculodigital reflexcataractskeratoconuskeratoglobuslate bone spicule pigmentblood vessel attenuationretinal white dotsextensive chorioretinal atrophymarbelized retinadisc edemaMacular "coloboma"nystagmus ERG is reduced Because of reported cases of Refsum disease and Bassen Kornzweig syndrome, consider blood lipid profile, peripheral blood smear looking for acanthocytes, and serum phytanic acid levels Achromatopsia stable VA complete (AR) and incomplete (AR and XL)

better VA in the darkphotophobiahyperopic astigmatismparodoxic pupilsno color visionpoor central visionnystagmuscone ERG reduced, rod normal CSNB XL, AR, AD myopiastable but decreased VAnystagmusnyctalopiaparodoxic pupilsAttenuated B wave (never gets back to baseline) ERG similar to XL retinoschisis

10. Syndromes Craniofacial syndromes Craniosynostosis simple cases are sporadic, syndromes are usually AD Ocular ON ALL disk swelling should be evaluated proptosis shallow orbits can cause severe corneal exposure strabismus often extortion of orbits may have absent SO, SR treat refractive error and amblyopia misc variety of other malformations noted including coloboma, cataract, medullated NFL, keratoconus, iris TI defects frequently have NLD obstruction with dacryocystitis Apert AD but most cases are sporadic midfacial hypoplasia, syndactyly digits 2-4 coronal suture closed Pfeiffer similar, shallow orbits especially, short broad thumbs and toes Carpenter similar, extra thumb or big toe, obesity and shallow orbits AR Crouzons AD with 2/3 being familial coronal suture most commonly affected proptosis, ON swelling more frequently plagiocephaly unilateral coronal or lamboidal suture

ocular problems generally with the coronal closure head assymetry best viewed by looking down ear and orbit elevated, nasal root deviated toward the side, the forehead is flattened vertical strabismus common with desagitilization of the trochlea usually without ON compromise Treacher Collins AD with variable expression systemic mandibular/malar hypoplasialarge mouthhighly arched palatedeformed downward earshairline projection to cheekblind fistulas from ear to mouthpreauricular tagsnormal intelligenceocular downward slanting PF (antimongoloid) with lower lid colobomas variable amount of lower lid tissue can exist tearing from punctal and lid abnormalities minimal ocular problems Hemifacial microsomia sporadic Goldenhars syndrome is a varient systemic unilateral facial involvement R>Lmandible usually greatly smallermalformed earspreauricular tags/pitsV and VII nerve palsiescervical spine abnmacrostomiaheart/lung/kidney abnocular epibulbar dermoidslipodermoidsstrabismusDuanes syndromelacrimal duct stenosiscolobomas of upper lids dermoids are usually small (<5mm) and solitary and 60% of the time, are isolated defects Hallermann-Streiff sporadic systemic short from 2-5 S.D. below the mean for height alopecia with hypotrichosis small face with thin beak nosehypoplastic mandiblehigh arched palatedouble chindental problemsmental retardation in 1/3ocular congenital cataracts in 90% with spontaneous resorption microphthalmia, strabismus,uveitis, downslanting palpebral fissures FAS 1:750 of all births, but in alcoholic mothers, 30% have it at risk if > 6 drinks/ day systemic thin vermilion border of upper lipsmental retardationlow birth wt and htcardiac and skeletal abnormalitiesCNS abnormalitieshyperactiveocular primary telecanthus is characteristic most commonly horizontal short PF, ptosis, and epicanthal folds up to 1/2 with decreased Va tortuous vesselsmild optic nerve hypoplasia (40%)high refractive error (+ and -)long eyelashesanterior

segment dysgenesis

11. Uveitis JRA RF-, HLA B27Systemic (1/4) 60% male, 1/4 with destructive arthritis systemic fever, exanthem, pericarditis, leukocytosis ANA rarely have uveitis Polyarticular (1/3) 3 yo, 80% female, >5 joints, symmetric arthritis of small and large joints systemic malaise, anorexia, anemia ANA+ 25% infrequent uveitis Pauciarticular Type I (1/4) early onset < 4 y.o., 80% female, <4 joints up to 50% have iritis ANA + 90% of those with iritis asymmetric arthritis of large joints without destruction or radiologic damage, evanescent systemic fatigue, mild fever, anemia HLA DR5/DR8 chronic iritis with 60% having relapsing course, 20% unremitting, and 20% with single episode most are bilateral within one year silent in 50% of patients no relationship between joint involvement and iritis prognosis dependent upon initial presentation pauciarticular late onset (1/8) see HLA B27+ RX 20% are unresponsive to steroids, 1/2 need up to six months to control with intensive steroids Subtenon's and systemic steroids may need to be used major side effect is intraocular pressure and cataract formation use oral and topical NSAIDs glaucoma b-blockers effective long term in only 1/3 CAI more effective in children but with ASA may cause metabolic acidosis

watch out for iris bombe, consider LPI filters have poor prognosis HLA B27+ early diagnosis difficult with spondylitis late and often peripheral arthritis ankylosing spondylitis 90% are male, after age 8 FH of JRA, Reiters syndrome, iritis, or ankylosing spondylitis Hip, sacroilitis, and Achilles tendonitis 20% have pauciarticular lower limb inflammation only 2% present initially with iritis Cervical spondylitis in females mimics adult disease with polyarthritis symmetric with wrists and fingers ANA+ can have chronic uveitis like JRA Psoriatric arthritis F>M, >10 yo, FH of psoriasis at presentation, monoarticular arthritis affecting knees changing to asymmetric polyarthritis over time skin disease and arthritis are correlated iritis in 10% with 80% being ANA+ arthritis presents early 3-5 yo and the skin changes late 13 yo so often with diagnosis of JRA Juvenile Reiters, IBD Chronic iridocyclitis in young girls like JRA without arthritis can happen in boys usually more severe Acute iritis (1/4) with older children, symmetric, deforming polyarthritis. Pars Planitis Similar to adult uveitis Tends to involve the optic nerve more frequently Strabismus I. Basic Science Anatomy rectus muscles 12 mm posterior to insertion the rectus muscles penetrate posterior Tenon's capsule which fuses with the intramuscular septum to form muscle sheath tendon length: 4-8 mm long muscle length: 40 mm muscle width: 10 mm horseshoe shaped insertion so hook them posteriorly

insertion from limbus is approximately: 5.5 mm (MR) 6.5 mm (IR) 7 mm (LR) 8 mm (SR)MR easiest to lose without attachments to obliques tendon is only 4 mm long, therefore resection is often bloody LR Faden procedure ineffective since it already has long arc of contact easiest to expose if lost, look at the inferior oblique insertion tendon is 9 mm long superior oblique SO muscle is the shortest EOM, only32 mm long, but the tendon is the longest at 20 mm As it passes underneath the SR, it becomes attached to the SR muscle sheath insertion is variable, but usually at the lateral border of the SR vortex vein is located posteriorly inferior oblique IO tendon 1 mm inserts almost directly over macula about 12 mm posterior to the lower LR insertion inferotemporal vortex vein very close nerve enters as IO passes below IR Blood supply 30% of anterior perfusion by 2 long posterior ciliary arteries at 3 and 9 oclock 70% by 7 anterior arteries in rectus muscles LR only rectus muscle with one anterior ciliary artery, all others have two after surgery, anterior ciliary arteries do NOT recannulate because there are no posterior ciliary vessels superiorly and inferiorly, iris angiograms are more disrupted when IR or SR are operated on iris angiograms do not predict ischemia older patients may be more prone to ischemia perilimbal conjunctival vessels may play a role in circulation CN nuclei Remember IIIrd and VIth nuclei have ipsilateral innervation, whereas the IVth nerve nucleus has contralateral innervation Physiology plane of rotation is13 mm behind cornea (slightly behind the equator) The torque exerted by a muscle on the globe is where it becomes first tangent with the eye The MR is tangent at or anterior to the equator while the LR is tangent just posterior to the equator 1 nerve fiber: 5 muscle fibers (highest in body) Tension

resting tension is 25g saccades generate 50g maximum isotonic contraction is 120g ARC and suppression Defense against diplopia Acquired adult strabismus have minimal sensory adaptation, whereas children generally adapt suppression eliminates central diplopia small scotoma with ET hemiretinal with XT ARC eliminates peripheral diplopia test with bagolini lenses Exam Stereo 20-50 arc seconds equals normal bifoveal fixation 80-3000 arc seconds is peripheral fusion must have adequate illumination to test properly Titmus Fly is 3000 arc seconds animals 400-200-100 arc seconds circles 800-400-200-140-100-80-60-50-40 arc seconds has monocular clues If you rotate 90 deg, lose stereo (as a check) Randot less false positives, more false negatives positive testing equals high grade stereopsis Diplopia tests Red filter red filter over one eye, do they see a pink/red/white fixation light or double? prism neutralization if sees double with prism neutralization, then patient has ARC W4D can test strength of fusion by darkening the room and increasing the dissociation dots are separated by 6 degrees at 1/3 m, and 1.25 degrees at 6 m, and 12 degrees at 1/6 m alternating suppression can be confused with diplopia Normal phorias adults6 M2 pd E1/3 M6 pd Xchildren6 M8 pd E1/3 M8 pd EAngle Kappa an abnormal angle kappa is different than eccentric fixation because the fovea is still used positive= normal, nasal negative= abnormal, temporal Fusional amplitudes

horizontalconvergenceD 14pddivergenceD 6pdN16pdvertical2.5pdBruckner test direct ophthalmoscope at arms length, full brightness perform Hirschbergs, then compare brightness of red reflex assess fixation or hypoaccomodation asymmetric red reflexes are due to strabismus, anisometropia, anisocoria, posterior pole abn/media opacities II. Amblyopia not a defense against diplopia 2% of population with neutral density filter, the difference between the eyes diminish crowding phenomenon eccentric fixation can be found with dense amblyopia Strabismic often with refractive componant present earlier to doctor density of amblyopia is not correlated with time of recovery during therapy Refractive meridional > +3.00 ametropic usually >+5.00 give correct Rx may take a long time for vision to get better--be patient anisometropia very common > 1.5 D difference in hyperope, >2 D in myopes children with better initial vision will do better anisekonia doesn't occur with correct rx very small incidence of ET developing while patching Organic often a functional componant is also present deprivation (amblyopia ex anopsia) media opacity such as cataract or corneal opacity structural eg, optic atrophy, macular scar small APD, abnormal PERG Occlusion less than 10% usually reversible with cessation of patch or brief patching of other eye Monofixation syndrome < 8 pd of strabismus (cover/uncover or simultaneous prism and cover) and 1/3 have no deviation

alt cover > cover-uncover, prism and cover anisometropia common small macular scotoma (debate about ARC and suppression) usually good binocular vision, NRC, and abnl stereoacuity (<60%) may have HARC (cover test normal), macular lesion most have amblyopia Dx for central scotoma use 4pd BO test (no movement in one eye) high false negatives Bagolini lenses Worth 4 dot (up close, normal periphery, back off at 1/3 -1 meter will show scotoma and pt will see only 3 dots) Rx for amblyopia rarely need surgery have stable alignment

III. Esotropia

Congenital Esotropia congenital absence of motor fusion characteristics large angle ET, usually 40-50 pd, but range 10-90 pd usually normal neurological status, but more common with cerebral palsy, hydrocephalus usually normal refraction may have amblyopia decreased sensory status OAIO with V OASO with A DVD latent nystagmus

asymmetric OKN Rx treat amblyopia and trial of glasses if > +3.00 surgery by 18 months to achieve single binocular vision (>90%) if surgery after 2 yo, < 1/2 have binocularity

Diff Dx accomodative ET Duane's T1 Mobius syndrome neurological abnormalities nystagmus blockage bilateral abducens palsy sensory ET Nystagmus blockage manifest congenital nystagmus dampened by convergence eyes straighten under general anesthesia variable angles, but nystagmus often disappears with convergence Accomodative ET 6 months to 7 yrs, avg 2.5 yrs, +FH starts intermittent, precipitating illness, trauma amblyopia frequent no diplopia since suppression or ARC

Refractive

+3.00 - +10.00 D, avg +4.00 hyperopia increases till age 7, then decreases deviation same distance and near, 20 - 30 pd If delayed RX, may not respond If > +5.00 D, can get bilateral ammetropic amblyopia

RX patch amblyopia, full time Rx repeat refraction 2 months post glasses since ciliary relaxation if overcorrected post surgery, try over minus therapy High AC/A near >10pd than distance is a rough clinical measurement usually hyperopic (+2.25 D avg, but can be myopic or +10.00) MUST BE ACCOMODATING TO GET FULL ET can get nonaccommodative ET

measurement normal AC/A 4:1 Gradient method: (ET near with +3.00 lens - ET n)/3 (dioptric distance) Heterophoria method: (PD (cm) + ET near - ET distance)/ D (of near)

RX: +3.00 large flattop bifocals with the segment splitting the pupils

bring target close to child and if they reflexively assume chin up posture, then they are using bifocals

Diff dx nonaccomodative (stress, cyclic )paralytic sensory deprivation divergence insufficency (ET > dist than near) Spasm of convergence Incomitant ET MR restriction thyroid, blowout, post op forced ductions are useful guide to surgical planning VI nerve paresis often with head turn for fusion common in children Check neurological exam, trauma, CT, MRI patch, fresnel prisms, botulinum for diplopia short term Post XT surgery usually improves spontaneously RX prisms, miotics, full CRx, patch, surgery

Surgery chances for consecutive exotropia increase with anisometropia, high hyperopia, amblyopia, or cerebral palsy Tables may do symmetrical surgery of weakening MR or strengthening LR by same amount OU

in adults with >45pd of esotropia, consider three or four muscle surgery to avoid limitation of ductions Note that these are a starting point and should be continuously monitored

ET angle (pd)recess MR in mmresect LR in mm<153-5 (only one)154203.55254.06304.57355.08405.59506.010>6010 recess-resect may weaken MR and strengthen LR of same eye by reading across table used if one eye has much better vision not as good for large deviations Duane's syndrome no VI nerve nucleus innervation of LR by III nerve sporadic usually, but AD in 1/10 F > M, OS > OD (slightly) usually unilateral children rarely complain, present usually with head turn adults complain of asthenopia, intermittant diplopia, and vague discomfort ocular decreased ABduction variable loss of ADduction upshoots and downshoots on adduction paresis of convergence uveal colobomas crocodile tears amblyopia On ADd, PF narrows, globe retracts secondary to activation of LR

systemic spinal defects Goldenhar syndrome Wildervanck syndrome deafness, Klippel Feil anomaly (web neck), and Duanes syndrome F>>M Types most commonly, patients have TI Duanes with ET in primary and minimal enophthalmos/leash, good stereo the less ET in primary position, the greater the enophthalmos TI: decreased ABd, straight to ET in primary, bilateral 20% TII: XT in primary and limit ADD TIII: decreased ABD and ADD Mobius syndrome VI, VII nerve palsies, abn PPRF some can also have III, IV, V, IX, X, and XII may have variety of motility disturbances limitation of adduction that improves with convergence fissure changes vertical muscle involvement mask like facies sporadic, although rare AD varients have been reported systemic Polands anomaly (absent pectoralis muscle) limb, chest, and tongue defects micrognathia, small mouth, hypodontia

15% have MR Convergence Spasm Intermittent ET, miosis, and myopia (accommodation) Symptoms include headache, asthenopia, diplopia, photophobia May appear to have bilateral VI th nerve palsies, but will have miosis with abduction Functional vs organic (posterior fossa, vestibulopathy, pituitary tumors, diffuse metabolic disease, MS, and trauma) Baclofen has been tried in MS IV. Exotropia

Types PseudoXT + angle kappa ROP dragged macula Toxacara telecanthus Duanes TII incomitant Basic same at distance and near Divergence excess distance > near by 15 pd high AC/A check post occlusion (break fusion) simulated excess if becomes basic with +3.00D after patching for one hour

most do not become ET at near post op if straight at distance

Convergence insufficiency near > distance by 15 pd near point of convergence (NPC) is remote low AC/A blurred near vision often only with exophoria traumatic often resolve within one year (up to 2/3) remaining are difficult to treat usually prisms, orthoptics are ineffective Rx pencil pushups reading or TV with BO prism to increase convergence amplitudes chronically, can treat with BI prism often have significant overcorrection early on, especially with bimedial resections a recess/resect procedure provides some incomitance so the patient can fuse without diplopia Exophoria NPC may be remote asthenopia if controlled by accommodative convergence most commonly a problem with convergence insufficiency NOT treatable by surgery, use convergence exercises Intermitant XT

most idiopathic, +FH, F>M usually starts around age 2 close one eye in bright sunlight ARCV pattern only 1/4 improve or are stable often complain about diplopia when driving or moving must measure with accommodative target bilateral temporal hemiretinal suppression progressive with decrease tonic convergence, developed suppression, decreased accommodation, and orbital divergence with age most are pseudo-divergence excess, so patch 45min or check with +3.00d Rx give full myopic Rx minus lenses, base in prisms work for small amounts timing of surgery is determined by how often the eye is deviated, not by how much

Constant XT older Adults with monocular vision loss often with sensory XT decompensated intermittant or sensory XT often alternate freely rare diplopia congenital (infantile) XT associated with organic disease, craniofacial abnormalities, or neurological problems. If healthy, then commonly with X patterns, DVD rx bilateral LR recessions or unilateral recess/resect

Consecutive XT much check adduction to see if MR has slipped Surgery wait until 2 1/2 to 4 year old to promote fusion if in lateral gaze measures10 pd < straight, then decrease recession by 1mm/muscle BLRR should aim for 10 - 20 pd of ET early on resect/recess should aim for a few pd overcorrected early on for long standing cases, need to release contracted conjunctiva and Tenon's Tables may do symmetrical surgery of weakening LR or strengthening MR of both eyes may weaken LR and strengthen MR of same eye by reading across table at larger angles, may prefer to do four muscle surgery with the numbers in parenthesis

XT angle (pd)recess LR in mmresect MR in mm1543205425653076357.56.54087509 (6)7 (5)6010 (7)8 (6)7011 (7.5)9 (6.5)8012 (8)10 (7) consecutive ET usually resolve with time may patch, prisms, full plus glasses, phospholine iodide rare to have persistance, especially when ductions are full if incomitant, after a resect/recess, may require reoperation

V. Vertical Deviation

DVD updrift of nonfixating eye especially with cover-uncover

deviated eye often aBDucts and EXtorts and almost bounces back into position asymmetrical, spontaneous or with occlusion equal deviation in gazes etiology unknown, almost always bilateral Usually NO hyPOtropia when hyPERtropic eye fixates Herring's law does NOT apply associated with latent nystagmus, congenital ET measure with base down prism under occluder often becomes evident after ET surgery natural history with resolution in 15-25% diff dx- IO overaction, hypertropia SO palsy Most common cause of vertical diplopia Longstanding palsies with contraction of IO and vertical concomitance pay careful attention to versions for the diagnosis when fixating with paretic eye, can mimic a SR palsy of opposite side especially in ABDuction (inhibitional paresis of the contralateral antagonist), ptosis Three Step Test patient prefers head tilt to OPPosite side with chin depression to minimize diplopia HyPERtropia IPSIlaterally with elevation on ADDuction + Bielschowsky test: head tilt to IPSIlateral side with increased HYPER Do Biechowsky head tilt supine and standing. Often, acute acquired IVth nerve will have normal exam in supine position and hyperdeviation in standing. Cyclotorsion often worse in downgaze double maddox rod

often less than the true tropia due to sensory adaptation pay attention only to the net torsion between the eyes fundus exam gives the true cyclotropia Congenital long standing head tilt amblyopia uncommon, may indicate absent SO facial asymmetry with affected side being more full can decompensate at later age often with subtle complaints of intermittant diplopia, asthenopia, neck ache with reading no torsion on Maddox rod secondary to sensory compensation may have mild decrease in stereo forced ductions in OR show laxity and it is loose upon inspection Acquired traumatic, CVA, sinusitis, tumor complain of tilting of objects discrete hx of onset can measure torsion with Maddox rod usually < 20pd of hyperopia in primary ischemic causes usually are in older patients with 5-10 pd of hypertropia forced ductions in OR reveal normal SO of both sides Bilateral uncommon, associated with trauma V-pattern ET downgaze often with chin down posture Double Maddox rod > 10-15 degrees excylotorsion

Head tilt shows either alternating hypertropia or is reduced to very little or none can be masked

Double elevator Palsy chin up position decreased elevation hypotropia worse on upgaze ptosis or pseudoptosis asymmetric Bell's IR restriction +forced ductions, normal forced generation, normal saccades superiorly elevator weakness -forced ductions, decreased force generation, decreased saccades superiorly Monocular Supranuclear palsy Rare acquired palsy due to lesions of the high midbrain interrupting supranuclear input to SR and IO of one eye Looks like double elevator palsy except normal Bell's and doll's eyes maneuver for upgaze May have convergence weakness, pupil abnormalities, and decreased downgaze R/O myasthenia Brown's syndrome congenital, tenosynovitis, IO abnormalities Decreased elevation on ADDuc usually DEPr on ADDuc + forced duction V pattern, straight in primary bilateral in 1/10

r/o IO paresis

acquired watch and wait often resolves trochlear bursitis JRA adult RA sinusitis/surgery blepharoplasty mets orbital surgery SLE post partum scleroderma primary SO overaction there is true muscular overcontraction, so tenotomy does not produce secondary SO palsy be careful if patients have fusion

Congenital Fibrosis Syndrome bilateral ptosis, static external ophthalmoplegia, and downward eye deviation often incomplete with aberrant regeneration may be familial CPEO bilateral asymmetric ptosis usually before adolescence few yrs later, symmetric PEO with sparing of downgaze

early on can have exotropia with convergence insufficiency pattern may have diplopia may have weakness of orbicularis, slow saccades, and nystagmus serum CK may be elevated mitochondrial cytopathy classic ragged red muscle fibers on trichrome stain may have increased sensitivity to induction agents and nondepolarizing muscle relaxants Kearns-Sayre post pole mottled RPE and may have spicules ERG normal or mildy decreased systemic arrhythmias, congestive cardiomyopathy deafness intracranial calcifications growth retardation diabetes mellitis thyroid abnormalities dementia endocrine dysfunction CSF protein skeletal muscle weakness ataxia

Myotonic dystrophy AD, onset in second decade presenting with blepharospasm

ocular ptosis orbicularis weaknesss low saccades ophthalmoplegia miotic pupils light/near dissociation christmas tree cataracts in all systemic cardiac disease dysphagia MR incontinence baldness scoliosis testicular atrophy Oculopharygeal dystrophy French Canadians with ptosis and dysphagia in third and fourth decade increased risk of aspiration, starvation ophthalmoplegia, orbicularis and facial weakness, limb girdle muscle weakness palatal voice Skew Deviation Acquired supranuclear cause of a vertical deviation May have torsional and horizontal componants Classically described as comitant, skew deviation may present in many ways and can look like CN palsies

Other signs of brainstem involvement small degrees of incomitance if anynormal doll's eyes and Bell's reflex Most commonly pontine lesion, but midbrain, cerebellum, and medulla can also be affected Misc it is less helpful in chronic paresis where vertical deviations are more comitant fundus torsion can help to distinguish which vertical muscles are involved clinically, vertical rectus muscles cause minimal complaints of tilting forced ductions may help Top of Form
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