1. CONGENITAL ESOTROPIA

2. Kowal 20053 CAUSE Subtle neurological developmental problem Nearly always in isolation

3. CONGENITAL ESOTROPIA Kowal 20054 CONGENITAL ESOTROPIA CET ONSET < 3 MONTHS: RARE USUAL ONSET 3+ MONTHS

4. CONGENITAL ESOTROPIA Kowal 20055 CORE DEFECTS NOT ET! Sensory: N-T asymmetry Motor: N-T asymmetry, LMLN [T&H] poor devpt of binocularity

5. CONGENITAL ESOTROPIA Kowal 20056 CONGENITAL ESOTROPIA CET Large angle ET N- T asymmetry Amblyopia ?30% Cross fixation : LE used for right gaze, RE for L gaze

6. CONGENITAL ESOTROPIA Kowal 20057 CONGENITAL ESOTROPIA CET Usual range of refraction 25% caucasian neonates > +4 ? Higher + more prone to CET

7. CONGENITAL ESOTROPIA Kowal 20058 RACIAL Caucasians: poor binocularity + hyperopia : cong ET is commonmest type of cong strab No good comparative population studies

8. CONGENITAL ESOTROPIA Kowal 20059 ASSOCIATIONS Down’s Severe neonatal course IVH /HC PVL

9. CONGENITAL ESOTROPIA Kowal 200510 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

10. CONGENITAL ESOTROPIA Kowal 200511 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.

11. CONGENITAL ESOTROPIA Kowal 200512 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.

12. CONGENITAL ESOTROPIA Kowal 200513 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.

13. CONGENITAL ESOTROPIA Kowal 200514 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

14. CONGENITAL ESOTROPIA Kowal 200515 VERTICALS IN CET > 2 types: 1. DVD: Non fixing eye drifts up 2. Oblique dysfunction Usu IO OA Can be SO OA

15. CONGENITAL ESOTROPIA Kowal 200516 VERTICALS IN CET : DVD

16. CONGENITAL ESOTROPIA Kowal 200517 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

17. CONGENITAL ESOTROPIA Kowal 200518 CONGENITAL ESOTROPIA CET Head turns / face tilts

18. CONGENITAL ESOTROPIA Kowal 200519 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

19. CONGENITAL ESOTROPIA Kowal 200520 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

20. CONGENITAL ESOTROPIA Kowal 200521 The clinical spectrum of early-onset esotropia: If it looks like CET: is it CET?

21. CONGENITAL ESOTROPIA Kowal 200522 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.

22. CONGENITAL ESOTROPIA Kowal 200523 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

23. CONGENITAL ESOTROPIA Kowal 200524 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

24. CONGENITAL ESOTROPIA Kowal 200525 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.

25. CONGENITAL ESOTROPIA Kowal 200526 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

26. CONGENITAL ESOTROPIA Kowal 200527 TIMING OF TREATMENT Early Very early Late How late

27. CONGENITAL ESOTROPIA Kowal 200528 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

28. CONGENITAL ESOTROPIA Kowal 200529 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

29. CONGENITAL ESOTROPIA Kowal 200530

30. CONGENITAL ESOTROPIA Kowal 200531 Why does early alignment improve stereoacuity outcomes in CET? J AAPOS. 2000 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.

31. CONGENITAL ESOTROPIA Kowal 200532 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.

32. CONGENITAL ESOTROPIA Kowal 200533

33. CONGENITAL ESOTROPIA Kowal 200534 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

34. CONGENITAL ESOTROPIA Kowal 200535 OVERVIEW 2 Measure angle ≥ 2 times Check refraction >+3 : try anti- accommodative Rx Gls / pilo / phospholine AIM: alignment within a few months of onset

35. CONGENITAL ESOTROPIA Kowal 200536 OVERVIEW Many variables Bimedial recession - reliable to 50∆ Recess / resect Augment for very large angles - botox, 1-2 extra muscles

36. CONGENITAL ESOTROPIA Kowal 200537 OVERVIEW Day surgery Check within 24-36 hours re: slipped stitch Recurrent / residual ET often accommodative Consceutive XT with time

37. CONGENITAL ESOTROPIA Kowal 200538

38. CONGENITAL ESOTROPIA Kowal 200539 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?

39. CONGENITAL ESOTROPIA Kowal 200540 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.

40. CONGENITAL ESOTROPIA Kowal 200541 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% "

41. CONGENITAL ESOTROPIA Kowal 200542