CONGENITAL ESOTROPIA

Kowal CAUSE Subtle neurological developmental problem Nearly always in isolation

CONGENITAL ESOTROPIA Kowal CONGENITAL ESOTROPIA CET ONSET < 3 MONTHS: RARE USUAL ONSET 3+ MONTHS

CONGENITAL ESOTROPIA Kowal CORE DEFECTS NOT ET! Sensory: N-T asymmetry Motor: N-T asymmetry, LMLN [T&H] poor devpt of binocularity

CONGENITAL ESOTROPIA Kowal CONGENITAL ESOTROPIA CET Large angle ET N- T asymmetry Amblyopia ?30% Cross fixation : LE used for right gaze, RE for L gaze

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

CONGENITAL ESOTROPIA Kowal RACIAL Caucasians: poor binocularity + hyperopia : cong ET is commonmest type of cong strab No good comparative population studies

CONGENITAL ESOTROPIA Kowal ASSOCIATIONS Down’s Severe neonatal course IVH /HC PVL

CONGENITAL ESOTROPIA Kowal 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

CONGENITAL ESOTROPIA Kowal Motion detection: normal infants & CET infants Bosworth & Birch.Vision Res 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.

CONGENITAL ESOTROPIA Kowal 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.

CONGENITAL ESOTROPIA Kowal 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.

CONGENITAL ESOTROPIA Kowal 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

CONGENITAL ESOTROPIA Kowal VERTICALS IN CET > 2 types: 1. DVD: Non fixing eye drifts up 2. Oblique dysfunction Usu IO OA Can be SO OA

CONGENITAL ESOTROPIA Kowal VERTICALS IN CET : DVD

CONGENITAL ESOTROPIA Kowal 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

CONGENITAL ESOTROPIA Kowal CONGENITAL ESOTROPIA CET Head turns / face tilts

CONGENITAL ESOTROPIA Kowal INFANTILE ET COCHRANE Cochrane Database Syst Rev ? most effective type of intervention ? age at intervention SELECTION CRITERIA: Randomised trials comparing any surgical or non- surgical intervention for infantile esotropia

CONGENITAL ESOTROPIA Kowal 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

CONGENITAL ESOTROPIA Kowal The clinical spectrum of early-onset esotropia: If it looks like CET: is it CET?

CONGENITAL ESOTROPIA Kowal The clinical spectrum of early-onset esotropia: Congenital Esotropia Observational Study.PEDIG. Am J Ophthal 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.

CONGENITAL ESOTROPIA Kowal 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

CONGENITAL ESOTROPIA Kowal CET Observational Study - PEDIG #2 Am J Ophthalmol PURPOSE: To determine the probability of spontaneous resolution of CET Eligibility:ET≥ age 4 to < 20 w. Primary outcome : alignment at 28 to 32 w. ET ‘resolved’ : ≤ 8 ∆ with/-out glasses

CONGENITAL ESOTROPIA Kowal 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.

CONGENITAL ESOTROPIA Kowal 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

CONGENITAL ESOTROPIA Kowal TIMING OF TREATMENT Early Very early Late How late

CONGENITAL ESOTROPIA Kowal Stereopsis & duration of misalignment in CET.Ing M, JAAPOS 2002 Titmus c.f. duration of misalignment [DOM] and 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

CONGENITAL ESOTROPIA Kowal Ing : Stereo, 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 alignment

CONGENITAL ESOTROPIA Kowal

CONGENITAL ESOTROPIA Kowal Why does early alignment improve stereoacuity outcomes in CET? J AAPOS Birch EE, Fawcett S, Stager DR.Birch EEFawcett SStager 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.

CONGENITAL ESOTROPIA Kowal 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.

CONGENITAL ESOTROPIA Kowal

CONGENITAL ESOTROPIA Kowal 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

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

CONGENITAL ESOTROPIA Kowal OVERVIEW Many variables Bimedial recession - reliable to 50∆ Recess / resect Augment for very large angles - botox, 1-2 extra muscles

CONGENITAL ESOTROPIA Kowal OVERVIEW Day surgery Check within hours re: slipped stitch Recurrent / residual ET often accommodative Consceutive XT with time

CONGENITAL ESOTROPIA Kowal

CONGENITAL ESOTROPIA Kowal 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 weeks?

CONGENITAL ESOTROPIA Kowal 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.

CONGENITAL ESOTROPIA Kowal 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% "

CONGENITAL ESOTROPIA Kowal