CONGENITAL ESOTROPIA

CONGENITAL ESOTROPIA


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CAUSE

• Subtle neurological developmental problem

• Nearly always in isolation


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CONGENITAL ESOTROPIA CET

• ONSET < 3 MONTHS: RARE

• USUAL ONSET 3+ MONTHS


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CORE DEFECTS

• NOT ET!• Sensory: N-T asymmetry• Motor: N-T asymmetry, LMLN [T&H]• poor devpt of binocularity


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• Large angle ET• N- T asymmetry• Amblyopia ?30%• Cross fixation : LE used for right gaze,

RE for L gaze


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• Usual range of refraction• 25% caucasian neonates > +4• ? Higher + more prone to CET


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RACIAL

• Caucasians: poor binocularity + hyperopia : cong ET is commonmest type of cong strab

• No good comparative population studies


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ASSOCIATIONS

• Down’s• Severe neonatal course IVH /HC• PVL


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IS IT CONGENITAL : SMOOTH PURSUIT

ASYMMETRY• All neonates develop N-T asymmetry,

sensory and motor• Age 6-8 mo: asymmetry lost in normals• Persists CET• a/w reduced potential for sensory &

motor fusion


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Motion detection: normal infants & CET infants

• Bosworth & Birch. Vision Res. 2005 • Asymmetry in detection of horizontal

motion in normals and CET• Motion detection thresholds measured

in 75 normals and 36 eyes of 27 infants with CET

• FPL with random-dot patterns.


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Motion detection: normal infants

• Asymmetries in sensitivity for nasalward (N) vs. temporalward (T) directions of motion were compared in normals & CETs, age 1 mo to 5 y.

• NORMALS : N = T under 2.5 mo• N > T motion preference between 3.5 and 6.5 mo.• N advantage gradually diminished to T = N by 8 mo

= adults.


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Motion detection: normal infants & CET infants

• No asymmetry in 15 normal infants who performed the task binocularly, hence, the asymmetry was not a L - R bias.

• In the youngest CET patients tested [5 mo], a nasalward superiority in motion detection was observed and was equivalent to that of same-age normal infants.


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Motion detection: normal infants and infants with CET• Unlike normals, this asymmetry persists in

older CET patients and is close to the ‘root’ cause / association of CET


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VERTICALS IN CET

• > 2 types:• 1. DVD: Non fixing eye drifts up• 2. Oblique dysfunctionUsu IO OACan be SO OA


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VERTICALS IN CET : DVD


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VERTICALS IN CET : DVD

• Common pattern:• Right fixation: L• L fixation: R

• End result of ‘braking’ the torsional component of LMLN in the fixing eye to try and improve acuity


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• Head turns / face tilts


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INFANTILE ET COCHRANE

• Cochrane Database Syst Rev. 2005 • ? most effective type of intervention • ? age at intervention• SELECTION CRITERIA: Randomised

trials comparing any surgical or non-surgical intervention for infantile esotropia


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INFANTILE ET COCHRANE

• NO adequate studies were found • CONCLUSIONS: ..literature on interventions

for IE are either retrospective studies or prospective cohort studies.

• ..not been possible to resolve controversies regarding type of surgery, non-surgical intervention and age of intervention …need for good quality trials to be conducted to improve the evidence base


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The clinical spectrum of early-onset esotropia:

• If it looks like CET: is it CET?


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The clinical spectrum of early-onset esotropia:

• Congenital Esotropia Observational Study.PEDIG. Am J Ophthal. 2002

• RESULTS: 175 infants. 3 1 mo.• 55% constant, 25% variable, 20% intermittent • 50% ≥ 40∆ • Most larger angle ET constant • Most smaller angle ET intermittent or variable.


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CET Observational Study - PEDIG #1

• Most first seen > 12 w constant ET (65%)• Most seen <12 w intermittent or variable ET(57%)• Amblyopia in 19% of patients• CONCLUSION: ET in early infancy shows more

variation in size & character than previously appreciated.

• A minority of infants diagnosed < 20 w have the commonly accepted profile for congenital esotropia of a large-angle constant ET.

• Amblyopia is frequent


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CET Observational Study - PEDIG #2

• Am J Ophthalmol. 2002• PURPOSE: To determine the probability of

spontaneous resolution of CET• Eligibility:ET≥ 20∆ @ age 4 to < 20 w.• Primary outcome : alignment at 28 to 32 w. • ET ‘resolved’ : ≤ 8 ∆ with/-out glasses


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CET Observational Study - PEDIG #2 RESULTS

• 170/ 175 followed up. 27% resolved• Most ‘resolved’ : intermittent or variable at

enrollment. • ‘Resolved’ #1: 1/ 42 cases that had constant ET

≥40∆ on both baseline & first follow-up examination & refraction ≤ +3DS.

• #2: ET 35 ∆ @ baseline and 40 ∆ @ at the outcome examination, ET resolved subsequent to the outcome examination.


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CET Observational Study - PEDIG #2 RESULTS

• CONCLUSIONS: ET with onset in early infancy frequently resolves in patients first examined at less than 20 w of age when the deviation is < 40 ∆ and is intermittent or variable.

• ET ≥40 pd presenting after 10 w of age have a low likelihood of spontaneous resolution.

• surgical correction at 3 to 4 mo of age could reasonably be considered in some CETs


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TIMING OF TREATMENT

• Early• Very early• Late • How late


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Stereopsis & duration of misalignment in CET

• .Ing M, JAAPOS 2002• Titmus c.f. duration of misalignment [DOM]

and age @ alignment • 90 pts surgically aligned by 24 m. • Patients aligned by 6 or 12 m or w/in 6 or 12

m of DOM did not differ in % with stereopsis.• Alignment after 12 m of age did show a

decrease percentage with stereopsis


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Ing : Stereo, age @ alignment, DOM

• The quality of stereo decreased for pts DOM ≥ 12 m

• CONCLUSION: • Alignment within 1 year of age or within 12 m

of misalignment favorably affects the % of CET patients who develop stereo.

• The quality of the stereopsis result is affected by DOM rather than the age @ alignment


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Why does early alignment improve stereoacuity

outcomes in CET?• J AAPOS. 2000 Birch EE, Fawcett S,

Stager DR.• 129 consecutive patients enrolled in a

prospective study of infantile esotropia who were followed up for a minimum of 5 years. At ages 5 to 9 years : Randot stereo

• DOM [but not age at alignment or onset] was a significant factor in determining RDS outcomes.

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Search&term=%22Birch+EE%22%5BAuthor%5D

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Search&term=%22Fawcett+S%22%5BAuthor%5D

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Search&term=%22Stager+DR%22%5BAuthor%5D


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Why does early alignment improve stereoacuity

outcomes in CET?• patients with stereo less likely to need a 2nd

surgery [p=0.05] and less likely to have DVD (P <.001).

• better stereopsis occurs because early surgery minimizes DOM, not because alignment is achieved during an early critical period of visual maturation

• RDS can also be achieved if DOM is not prolonged.


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OVERVIEW OF MGMT

• Check vision - any obvious amblyopia• Amblyopia Rx: FTO 1w/y of life then

review eg age 10 mo: patch for 50+% of waking hours for 5 days before the next visit

• Amblyopia may not respond with large ET


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OVERVIEW 2

• Measure angle ≥ 2 times• Check refraction• >+3 : try anti- accommodative Rx• Gls / pilo / phospholine• AIM: alignment within a few months of

onset


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OVERVIEW

• Many variables• Bimedial recession - reliable to 50∆• Recess / resect• Augment for very large angles - botox,

1-2 extra muscles


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OVERVIEW

• Day surgery• Check within 24-36 hours re: slipped

stitch• Recurrent / residual ET often

accommodative• Consceutive XT with time


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Case 1

• >I saw today a 15 week old baby with typical cong ET.• >Confident exam findings• >Little / no amblyopia.• >Accurate measurement of misalignment of 45^.• >Cyclo +4-2x180 OU• >• >My normal practice would be to tentatively book BMR 2-4 weeks hence and• >see child again pre-op to confirm measurements• >• >This is however the youngest child I have seen with cong ET• >Previously operated a 21 week child many years ago - ended up with• >random dot stereo• >• >Any tips / thoughts about operating in 2-3 weeks at age 17-18 weeks?


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Case 1 - Alan Scott• 1 Glasses trial for 2 weeks with over correction, say, +4, ou. • Forget the astigmatism, it changes all the time at this age. Yes it

could be accommodative and I have seen glasses work at this age. You may well need them later in any case.

• 2 Botox 3 units to each MR. This has a 60-80% chance of correction under age 6 mo. An office procedure under local as with adults.

• 3 BMR recession if the Botox doesnt hold.


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Consec XT - Ciancia• > Thank you for your interest in my results in operated Infantile Esotropia.• >The percent of secondary XT was as follows:

• >Immediate 1%• >At 6 months 2,3%• >At 1 year 3,5% • >At 2 years 5,4%• >At 3 years 10%• >At 4 years 8,2%• >At 5 years 10% (roughly)• >At 10 years 20% "• >At 15 to 27 years 30% "


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CONGENITAL ESOTROPIA