II. Definitions

  1. Strabismus
    1. Eye misalignment in children

III. Epidemiology

  1. Accounts for 50% of Amblyopia (most common cause)
  2. Affects up to 4% of children in U.S.

IV. Types

  1. Esotropia or Esodeviation (Crossed Eyes, Convergent Strabismus)
    1. Both eyes turn inward
    2. Most common Strabismus cause, accounting for >50% of ocular deviations in children
    3. Typically present by age 18 to 36 months of age
    4. Accommodative Esotropia occurs when a child with uncorrected Farsightedness looks at a near object
    5. Congenital Esotropia is due to excessive convergence and is high risk for Amblyopia
  2. Exotropia or Exodeviation (Walleye, Divergent Strabismus)
    1. One eye turns outward while other eye faces forward
    2. Deviation most evident with distant Vision and therefore only occurs intermittently
    3. Typical onset in preschool aged children
    4. Parents often note changes on a bright day, or when children are tired or inattentive
      1. Eye deviates outward
      2. Child closes one eye
    5. Often children are able to self correct Exotropia with concentration
    6. Accommodative Exotropia occurs when a child with uncorrected Nearsightedness looks at a distant object
  3. Fourth Cranial Nerve palsy (superior oblique palsy)
    1. Eye rises when Head Tilted to side of palsy
    2. Findings
      1. Hypertropia (deviated eye turns up)
      2. Hypotropia (deviated eye turns down)

V. Signs: Screen at every well child check

  1. Test Specifically for Strabismus
    1. Only 50% children with Strabismus have obvious defect

VI. Signs: Organized by Strabismus type

  1. Manifest (Tropia): Strabismus that is always present
    1. Corneal Light Reflex
    2. Cover-Uncover exam
    3. Bruckner Test (Red Reflex)
    4. Fixation and following
  2. Intermittent Strabismus
    1. Healthy newborns develop alignment after age 4 weeks
    2. Intermittent exodeviation under 6 months
    3. Esodeviation much more likely to be pathologic
  3. Latent (Phoria): Present if binocular Vision blocked
    1. Only found when specifically tested
    2. Cover-Uncover exam

VII. Signs: Small-Angle Strabismus (Accommodative Esotropia)

  1. Identified with Cover Testing
  2. Seen in Farsighted toddlers
  3. Corrected with glasses or bifocals
  4. Abnormal use of accommodative convergence
    1. Normal reflex for near Vision
    2. Farsighted children use convergence all the time

VIII. Screening

  1. General
    1. See testing above
    2. Photoscreening (picture of eyes and Red Reflex)
      1. May be used in future for mass screening
  2. Timing
    1. Screen frequently in first 3 years of life
    2. Example protocol: 8, 12, 18, 25, 31, 37 months of age
    3. Early diagnosis results in best outcome
  3. References
    1. Williams (2002) BMJ 324: 1549-51 [PubMed]

IX. Diagnosis: Immediate Ophthalmology Consult Indications

  1. Manifest (constant) deviation in any age
  2. Intermittent Exodeviation >6 months
  3. Intermittent Esodeviation >2 months

X. Diagnosis: Pitfalls - Pseudostrabismus

  1. Esotropia may be apparent despite normal alignment
  2. Iris appears to be surrounded by different white
  3. Illusion of different amount white on each side of iris
    1. Flat Nasal Bridge
    2. Large epicanthal folds
  4. Differentiate from Strabismus with proper testing
    1. See Strabismus signs above
  5. Refer to Ophthalmology for any question of misalignment

XI. Management

  1. Early ophthalmology Consultation is critical
  2. Amblyopia treated prior to realignment surgery
  3. Accommodative Esotropia
    1. Farsightedness correction with bifocals
    2. Surgical correction if refractory
  4. Congenital Esotropia
    1. High risk for Amblyopia if not corrected by age 2 years old
    2. Onabotulinumtoxin A injection is an initial option in some cases
    3. Surgical Realignment of eyes
      1. Repair under age 1 year offers best chance of near-normal binocular Vision
      2. Repair over age 1 to 2 years may result in significantly worse prognosis for binocular Vision
  5. Exotropia
    1. Surgical correction if constant, frequent or large exodeviation
  6. Hypertropia or Hypotropia
    1. Refer all cases to ophthalmology or pediatric neurology

XII. Prognosis

  1. Up to 40% of children with Strabismus will progress to Amblyopia

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Related Studies

Ontology: Esotropia (C0014877)

Definition (MSHCZE) Esotropie – manifestní, přetrvávající forma strabismu. Jeden nebo oba oční bulby jsou deviovány konvergentně. (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (MSH) A form of ocular misalignment characterized by an excessive convergence of the visual axes, resulting in a "cross-eye" appearance. An example of this condition occurs when paralysis of the lateral rectus muscle causes an abnormal inward deviation of one eye on attempted gaze.
Concepts Disease or Syndrome (T047)
MSH D004948
ICD9 378.00, 378.0
ICD10 H50.0 , H50.00
SnomedCT 194074002, 194085000, 155193001, 16596007
English Convergent Strabismus, Esotropia, Esotropias, Strabismus, Convergent, Esotropia, unspecified, Constant comitant esotropia, Convergent concomitant strabismus, Manifest convergent squint, crossed eyes, eyes cross, eyes are crossed (symptom), eyes are crossed, esotropia (diagnosis), esotropia, Esodeviation, Esodeviations, Esotropia NOS, Unspecified esotropia, Esotropia [Disease/Finding], crossing eyes, cross eye, cross eyes, cross-eye, crossings eyes, disorders esotropia, esotropias, convergent squint, crossed eye, esodeviation, crossing eye, Cross Eye, Cross-Eye, Cross-Eyes, Internal Strabismus, Strabismus, Internal, Internal strabismus, Unspecified esotropia (disorder), Esotropia NOS (disorder), Cross-eye, Convergent strabismus, Convergent squint, Esotropia (disorder), concomitant; strabismus, convergent, convergent; strabismus, strabismus; concomitant, convergent, strabismus; convergent, Esotropia, NOS, Crossed Eyes
Dutch convergent scheelzien, convergent bijkomend strabismus, esotropie, niet-gespecificeerde esotropie, concomitans; strabismus, convergens, convergens; strabismus, strabismus; concomitans, convergens, strabismus; convergens, Strabismus concomitans convergens, Esotropie, Scheelzien naar binnen, Strabismus convergens, Strabismus internus
French Strabisme convergent concomitant, Esotropie, Esotropie, non précisée, Ésotropie, Strabisme convergent
German konvergentes Begleitschielen, Esotropie, unspezifisch, konvergentes Schielen, Esotropie, Konvergenter Strabismus, Strabismus concomitans convergens, Strabismus convergens, Innenschielen, Strabismus convergens internus, Strabismus, konvergenter, Einwärtsschielen
Italian Esotropia non specificata, Esodeviazione, Occhio strorto, Strabismo interno, Strabismo convergente, Esotropia
Portuguese Esotropia NE, Estrabismo concomitante convergente, Estrabismo convergente, Esotropia, Estrabismo Convergente, Estrabismo Interno
Spanish Esotropía no especificada, Mirada bizca convergente, Estrabismo concomitante convergente, esotropía, SAI, esotropía no especificada, esotropía, SAI (trastorno), esotropía no especificada (trastorno), bizquera, esotropía (trastorno), esotropía, estrabismo convergente, Estrabismo Convergente, Estrabismo Interno, Esotropía
Japanese 内斜視、詳細不明, 輻輳性共同斜視, ナイシャシ, ナイシャシショウサイフメイ, フクソウセイキョウドウシャシ, 内斜視, 内斜視-輻輳, 輻輳内斜視
Swedish Inåtskelning
Finnish Esotropia
Russian EZOTROPIIA, KONVERGIRUIUSHCHEE KOSOGLAZIE, KOSOGLAZIE KONVERGIRUIUSHCHEE, KOSOGLAZIE SKHODIASHCHEESIA, КОНВЕРГИРУЮЩЕЕ КОСОГЛАЗИЕ, КОСОГЛАЗИЕ КОНВЕРГИРУЮЩЕЕ, КОСОГЛАЗИЕ СХОДЯЩЕЕСЯ, ЭЗОТРОПИЯ
Czech Ezotropie, blíže neurčená, Konvergentní konkomitantní strabismus, Ezotropie, Konvergentní šilhání, konvergentní strabismus, konvergentní sbíhavé šilhání, ezotropie, esotropie
Korean 공동 내사시
Croatian EZOTROPIJA
Polish Zez zbieżny
Hungarian Esotropia, nem meghatározott, Konvergáló kancsalság, Esotropia, Konvergáló concomittáló strabismus
Norwegian Esotropi, Innoverskjeling, Konvergent strabisme

Ontology: Exotropia (C0015310)

Definition (MSHCZE) Manifestní, přetrvávající forma strabismu. Jeden nebo oba oční bulby jsou deviovány divergentně. (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (MSH) A form of ocular misalignment where the visual axes diverge inappropriately. For example, medial rectus muscle weakness may produce this condition as the affected eye will deviate laterally upon attempted forward gaze. An exotropia occurs due to the relatively unopposed force exerted on the eye by the lateral rectus muscle, which pulls the eye in an outward direction.
Concepts Disease or Syndrome (T047)
MSH D005099
ICD9 378.10, 378.1
ICD10 H50.1 , H50.10
SnomedCT 194086004, 155194007, 75400001, 194098009, 399054005, 399252000
English Divergent Strabismus, Exodeviation, Exodeviations, Exotropia, Strabismus, Divergent, Exotropia, unspecified, exotropia, exotropia (diagnosis), Exotropia NOS, Unspecified exotropia, Exotropia [Disease/Finding], divergent squint, exodeviation, divergent strabismus, Exotropia NOS (disorder), Unspecified exotropia (disorder), Divergent concomitant strabismus, Divergent strabismus, External strabismus, Divergent squint, Divergent concomitant strabismus (disorder), Exotropia (disorder), concomitant; strabismus, divergent, divergent; strabismus, strabismus; concomitant, divergent, strabismus; divergent, Exotropia, NOS, XT - Exotropia
Spanish exotropía (trastorno), exotropía, Mirada bizca divergente, Exotropía no especificada, Estrabismo concomitante divergente, exotropia (trastorno), exotropia, exotropía, SAI, exotropía no especificada, estrabismo divergente, estrabismo concomitante divergente, exotropía no especificada (trastorno), exotropía, SAI (trastorno), estrabismo externo, estrabismo divergente concomitante (trastorno), estrabismo divergente concomitante, Estrabismo Divergente, Exotropía
Dutch divergente bijkomende strabismus, niet-gespecificeerde exotropie, divergent scheelzien, exotropie, concomitans; strabismus, divergens, divergens; strabismus, strabismus; concomitans, divergens, strabismus; divergens, Strabismus concomitans divergens, Exotropie, Scheelzien naar buiten, Strabismus divergens
French Strabisme divergent concomitant, Exotropie, non précisée, Exotropie, Strabisme divergent
German divergentes Schielen, divergentes Begleitschielen, Exotropie, unspezifisch, Strabismus concomitans divergens, Exotropie, Strabismus divergens, Auswärtsschielen, Schielen, Auswärts-
Italian Exotropia, non specificata, Exodeviazione, Strabismo divergente, Exotropia
Portuguese Estrabismo concomitante divergente, Exotropia NE, Estrabismo divergente, Estrabismo Divergente, Exotropia
Japanese 開散性共同斜視, 外斜視、詳細不明, ガイシャシショウサイフメイ, ガイシャシ, カイサンセイキョウドウシャシ, 斜視-外, 外転(眼), 斜視-開散, 外斜視, 開散斜視
Swedish Utåtskelning
Czech exotropie, Exotropie, blíže neurčená, Divergentní konkomitatní strabismus, Divergentní šilhání, Exotropie, exodeviace, divergentní strabismus
Finnish Uloskarsastus
Russian EKZOTROPIIA, STRABIZM DIVERGIRUIUSHCHII, KOSOGLAZIE RASKHODIASHCHEESIA, KOSOGLAZIE DIVERGIRUIUSHCHEE, КОСОГЛАЗИЕ ДИВЕРГИРУЮЩЕЕ, КОСОГЛАЗИЕ РАСХОДЯЩЕЕСЯ, СТРАБИЗМ ДИВЕРГИРУЮЩИЙ, ЭКЗОТРОПИЯ
Korean 공동 외사시
Polish Zez rozbieżny
Hungarian Exotropia, nem meghatározott, Divergáló concomittáló strabismus, Divergáló kancsalság, Exotropia
Norwegian Utoverskjeling, Eksotropi, Strabismus divergens, Strabisme, divergerende

Ontology: Hypertropia (C0020575)

Definition (NCI) Vertical strabismus in which there is permanent upward deviation of the visual axis of one eye.
Concepts Disease or Syndrome (T047)
MSH D013285
ICD9 378.31
ICD10 H50.2
SnomedCT 194109005, 40608009
English Hypertropias, Unspecified hypertropia, hypertropia (diagnosis), hypertropia, nonparalytic hypertropia, Unspecified hypertropia (disorder), Hypertropia (disorder), Hypertropia
Dutch hypertropie, Hypertropie
Spanish Hipertropía, Hipertropia, hipertropía no especificada (trastorno), hipertropía no especificada, hipertropía (trastorno), hipertropía
Japanese 上斜視, ジョウシャシ
Portuguese Hipertropia
French Hypertropie
German Hypertropie, Fixation nach oben
Czech Hypertropie, hypertropie
Hungarian Hypertropia
Italian Ipertropia
Norwegian Hypertropi

Ontology: Strabismus (C0038379)

Definition (MSHCZE) Obecný pojem pro všechny formy šilhavosti (abnormální souhyb očních bulbů). Skryté, latentní šilhání se obecně nazývá heteroforie, manifestní šilhání heterotropie. Ezoforie (ezotropie) označuje konvergentní, sbíhavé šilhání, exoforie (exotropie) šilhání divergentní. Patognomicky se s. rozděluje na tři základní skupiny. Neparalytický (konkomitující) s. se vyskytuje nejčastěji, je typický u dětí. Paralytický s. je vždy způsoben kompletní nebo částečnou parézou příslušného okohybného nervu, která může být kongenitální nebo získaná (traumata, neuroinfekce, postvirové syndromy, cévní příčiny, systémové onemocnění aj.). Strabické syndromy zahrnují speciální, zvláštní typy s. nejč. způsobené strukturálními anomáliemi okohybných svalů. Šilhá jen jedno oko, popř. se oči střídají. Dvojitému vidění zabrání „vypnutí“ obrazu přicházejícího z odchylujícího se oka, které však může trvale slábnout (vzniká tupozrakost, amblyopie). Léčí se speciálními brýlemi (současně bývá obv. i refrakční vada), speciálními očními „cviky“ (tzv. ortoptika), někdy operativně. Léčit je nutné včas a důsledně. (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (NCI) Strabismus is the intermittent or constant misalignment of an eye so that its line of vision is not pointed at the same object as the other eye. Strabismus is caused by an imbalance in the extraocular muscles which control the positioning of the eyes. Strabismus is normal in newborns but should resolve by the time the baby is 6 months old. In older children with strabismus, the brain may learn to ignore the input from one eye, and this may lead to amblyopia, a potentially permanent decrease in vision in that eye if not corrected.
Definition (MSH) Misalignment of the visual axes of the eyes. In comitant strabismus the degree of ocular misalignment does not vary with the direction of gaze. In noncomitant strabismus the degree of misalignment varies depending on direction of gaze or which eye is fixating on the target. (Miller, Walsh & Hoyt's Clinical Neuro-Ophthalmology, 4th ed, p641)
Concepts Disease or Syndrome (T047)
MSH D013285
ICD9 378.40, 378.30, 378.4
ICD10 H50.5 , H50.9, H50.40, H50.50
SnomedCT 14619009, 155196009, 194107007, 194114009, 194116006, 246726008, 194137003, 267745004, 155192006, 22066006, 128602000
LNC MTHU013559
English Phorias, Squint, Strabismus, Phoria, Heterophoria, HETEROTROPIA, SQUINT, STRABISMUS, Disord binoc eye movem, Heterophoria, unspecified, Heterotropia, unspecified, Strabismus NOS, Strabismus, unspecified, strabismus, heterotropia (diagnosis), strabismus (diagnosis), heterotropia, squinting (symptom), squinting, heterophoria (diagnosis), heterophoria, Binocular eye movement disorder NOS, Unspecified disorder of binocular eye movements, Heterophoria NOS, Heterotropia NOS, Unspecified heterotropia, Unspecified heterophoria, Unspecified strabismus, Strabismus [Disease/Finding], eye deviation, crossed eye, Squinting, phoria, phorias, deviations eye, Unspecified heterotropia (disorder), Heterophoria NOS (disorder), Ocular dissociation (finding), Disorder of binocular eye movements (disorder), Strabismus NOS (disorder), Unspecified heterophoria (disorder), Eye deviation, Binocular eye movement disorder, Heterotropia, Disorder of binocular eye movements, Ocular dissociation, Heterotropia (disorder), Strabismus (disorder), eye; deviation, eye; movement disorder (binocular), Heterophoria, NOS, Heterotropia, NOS, Squint, NOS, Strabismus, NOS, squint
French STRABISME, Strabisme SAI, Hétérotropie, Anomalie non précisée des mouvements binoculaires, Anomalie des mouvements conjugués des yeux SAI, Hétérophorie, non précisée, Hétérotropie, non précisée, DEFAUT CONVERGENCE AXES VISUELS, Anomalie des mouvements conjugués des yeux, Hétérophorie, Strabisme, Phories
Portuguese ESTRABISMO, Estrabismo NE, Heterotropia NE, Alteração binocular dos movimentos do olho NE, Heteroforia NE, Heterotropia, Alteração dos movimentos binoculares do olho NE, HETEROTROPIA, PISCAR DE OLHOS, Estrabismo não Manifesto, Estrabismo Paralítico, Estrabismo Manifesto, Heteroforia, Alteração binocular dos movimentos do olho, Estrabismo, Forias, Vergo Estrabismo
Spanish ESTRABISMO, estrabismo latente, Estrabismo NEOM, Heteroforia no especificada, Heterotropía, Heterotropía no especificada, Trastorno de los movimientos binoculares de los ojos NEOM, Bizco, Trastorno no especificado de los movimientos binoculares de los ojos, HETEROTROPIA, heteroforia, SAI (trastorno), heteroforia, SAI, heterotropía no especificada, heteroforia no especificada (trastorno), heterotropía no especificada (trastorno), heteroforia no especificada, estrabismo, SAI, estrabismo, SAI (trastorno), Estrabismo no Manifesto, Estrabismo Paralítico, Estrabismo Manifesto, estrabismo (trastorno), estrabismo, heterotropía (trastorno), heterotropía, Heteroforia, Trastorno de los movimientos binoculares de los ojos, Estrabismo, Estrabismo Curvo, Foria
German STRABISMUS, unspezifische Stoerung der binokulaeren Augenbewegungen, Heterotropie, Heterophorie, unspezifisch, Heterotropie, unspezifisch, binokulaere Augenbewegungsstoerung NNB, Strabismus NNB, BEGLEITSCHIELEN, SCHIELEN, Strabismus, nicht naeher bezeichnet, Heterophorie, binokulaere Augenbewegungsstoerung, Phorie, Schielen, Strabismus
Italian Disturbo del movimento binoculare dell'occhio, Eteroforia, Disturbo del movimento binoculare dell'occhio NAS, Eteroforia, non specificata, Eterotropia, Disturbi non specificati dei movimenti binoculari dell'occhio, Eterotropia, non specificata, Strabismo NAS, Forie, Strabismo
Dutch scheelzien, heteroforie, niet-gespecificeerd, heterotropie, niet-gespecificeerde stoornis van binoculaire oogbeweging, niet-gespecificeerde heterotropie, binoculaire oogbewegingsafwijking NAO, strabismus NAO, Strabisme, oog; bewegingsstoornis (binoculair), oogstand; deviatie, Heteroforie, Strabismus, niet gespecificeerd, binoculaire oogbewegingsafwijking, heteroforie, strabismus, Strabismus, Scheelzien
Japanese 眼球斜位、詳細不明, 斜視、詳細不明, 両眼球運動障害NOS, 眼球斜位, 両眼球運動障害, 斜視NOS, 詳細不明の両眼の眼球運動の障害, ガンキュウシャイショウサイフメイ, リョウガンキュウウンドウショウガイ, シャシショウサイフメイ, ガンキュウシャイ, シャシ, シャシNOS, リョウガンキュウウンドウショウガイNOS, ショウサイフメイノリョウガンノガンキュウウンドウノショウガイ, やぶにらみ, 上斜位, 上斜視, 斜位(眼球), 斜視
Swedish Skelning
Czech šilhání, strabismus, Strabismus, Heterotropie, blíže neurčená, Heteroforie, blíže neurčená, Heteroforie, Šilhání, Binokulární okohybná porucha, Blíže neurčené binokulární okohybné poruchy, Binokulární okohybná porucha NOS, Heterotropie, Strabismus NOS, šilhavost
Finnish Karsastus
Russian KOSOGLAZIE, STRABIZM, КОСОГЛАЗИЕ, СТРАБИЗМ
Korean 상세불명의 사시, 사위
Croatian STRABIZAM
Polish Zez
Hungarian Heterophoria, Kancsalság, Heterotropia, nem meghatározott, Binocularis szemmozgás-betegség, Binocularis szemmozgás zavar k.m.n., Heterophoria, nem meghatározott, Heterotropia, Strabismus k.m.n., Nem meghatározott binocularis szemmozgás zavar
Norwegian Skjeling, Strabisme, Fori

Ontology: Hypotropia (C0152208)

Definition (NCI) Vertical strabismus in which there is permanent downward deviation of the visual axis of one eye.
Concepts Disease or Syndrome (T047)
ICD9 378.32
SnomedCT 29491004
English hyop - Hypotropia, hypotropia (diagnosis), hypotropia, deviation downward eye, downward deviation of eyes, Downward deviation of eyes, Hypotropia, Hypotropia (disorder), Downward Ocular Deviation, Sunset Sign
Dutch neerwaartse afwijking van de ogen, hypotropie
French Hypotropie, Déviation du regard vers le bas
German Hypotropie, nach unten gerichtete Abweichung der Augen
Italian Ipotropia, Deviazione degli occhi verso il basso
Portuguese Hipotropia, Desvio dos olhos para baixo
Spanish Hipotropía, Desviación hacia abajo de los ojos, hipotropía (trastorno), hipotropía
Japanese 下斜視, 眼の下方偏位, カシャシ, メノカホウヘンイ
Czech Deviace očního bulbu dolů, Hypotropie
Hungarian Hypotropia, Szemek lefele irányuló deviatiója