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CONGENITAL ESOTROPIA

CONGENITAL ESOTROPIA. CAUSE . Subtle neurological developmental problem Nearly always in isolation. CONGENITAL ESOTROPIA CET. ONSET < 3 MONTHS: RARE USUAL ONSET 3+ MONTHS. CORE DEFECTS. NOT ET! Sensory: N-T asymmetry Motor: N-T asymmetry, LMLN [T&H] poor devpt of binocularity.

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CONGENITAL ESOTROPIA

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  1. CONGENITAL ESOTROPIA

  2. CAUSE • Subtle neurological developmental problem • Nearly always in isolation Kowal 2005

  3. CONGENITAL ESOTROPIA CET • ONSET < 3 MONTHS: RARE • USUAL ONSET 3+ MONTHS Kowal 2005

  4. CORE DEFECTS • NOT ET! • Sensory: N-T asymmetry • Motor: N-T asymmetry, LMLN [T&H] • poor devpt of binocularity Kowal 2005

  5. CONGENITAL ESOTROPIA CET • Large angle ET • N- T asymmetry • Amblyopia ?30% • Cross fixation : LE used for right gaze, RE for L gaze Kowal 2005

  6. CONGENITAL ESOTROPIA CET • Usual range of refraction • 25% caucasian neonates > +4 • ? Higher + more prone to CET Kowal 2005

  7. RACIAL • Caucasians: poor binocularity + hyperopia : cong ET is commonmest type of cong strab • No good comparative population studies Kowal 2005

  8. ASSOCIATIONS • Down’s • Severe neonatal course IVH /HC • PVL Kowal 2005

  9. 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 Kowal 2005

  10. 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. Kowal 2005

  11. 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. Kowal 2005

  12. 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. Kowal 2005

  13. 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 Kowal 2005

  14. VERTICALS IN CET • > 2 types: • 1. DVD: Non fixing eye drifts up • 2. Oblique dysfunction Usu IO OA Can be SO OA Kowal 2005

  15. VERTICALS IN CET : DVD Kowal 2005

  16. 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 Kowal 2005

  17. CONGENITAL ESOTROPIA CET • Head turns / face tilts Kowal 2005

  18. 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 Kowal 2005

  19. 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 Kowal 2005

  20. The clinical spectrum of early-onset esotropia: • If it looks like CET: is it CET? Kowal 2005

  21. 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. Kowal 2005

  22. 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 Kowal 2005

  23. 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 Kowal 2005

  24. 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. Kowal 2005

  25. 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 Kowal 2005

  26. TIMING OF TREATMENT • Early • Very early • Late • How late Kowal 2005

  27. 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 Kowal 2005

  28. 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 Kowal 2005

  29. Kowal 2005

  30. 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. Kowal 2005

  31. 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. Kowal 2005

  32. Kowal 2005

  33. 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 Kowal 2005

  34. OVERVIEW 2 • Measure angle ≥ 2 times • Check refraction • >+3 : try anti- accommodative Rx • Gls / pilo / phospholine • AIM: alignment within a few months of onset Kowal 2005

  35. OVERVIEW • Many variables • Bimedial recession - reliable to 50∆ • Recess / resect • Augment for very large angles - botox, 1-2 extra muscles Kowal 2005

  36. OVERVIEW • Day surgery • Check within 24-36 hours re: slipped stitch • Recurrent / residual ET often accommodative • Consceutive XT with time Kowal 2005

  37. Kowal 2005

  38. 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? Kowal 2005

  39. 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. Kowal 2005

  40. 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% " Kowal 2005

  41. Kowal 2005

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