1. CONGENITAL ESOTROPIA

2. CAUSE Subtle neurological developmental problem Usually in isolation
[selection bias]
CONGENITAL ESOTROPIA
Kowal 2008

3. Sensory: N-T asymmetry Motor: N-T asymmetry, LMLN [T&H]
CORE DEFECTS
NOT ET! ALL CORTICAL
Sensory: N-T asymmetry
Motor: N-T asymmetry, LMLN [T&H]
CONGENITAL ESOTROPIA
Kowal 2008

4. Secondary effects Large angle ET with tight medial rectus
Amblyopia ?30%
Cross fixation : LE used for right gaze, RE for L gaze.
X-fixation usually reflects the mechanical situation, and not = vision
CONGENITAL ESOTROPIA
Kowal 2008

5. Secondary effects: VERTICALS IN CET
2 types:
1. DVD:
Non fixing eye drifts up
2. Oblique dysfunction
Usu IO OA
Can be SO OA
? Innervational ?orbital - prob both
CONGENITAL ESOTROPIA
Kowal 2008

6. VERTICALS IN CET : DVD
CONGENITAL ESOTROPIA
Kowal 2008

7. 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 2008

8. ASSOCIATIONS 1 REFRACTION
Usual range of infant refraction
25% caucasian neonates > +4
? Higher + more prone to CET
CONGENITAL ESOTROPIA
Kowal 2008

9. Severe neonatal course IVH / HC 100% PVL ‘delayed devpt’ ~20%
ASSOCIATIONS 2 BRAIN
Down’s 30%
Severe neonatal course IVH / HC 100%
PVL
‘delayed devpt’ ~20%
CONGENITAL ESOTROPIA
Kowal 2008

10. ~ 25% incidence in many chromosomal disorders
ASSOCIATIONS 3 GENETIC
William’s syndrome 100%
~ 25% incidence in many chromosomal disorders
CONGENITAL ESOTROPIA
Kowal 2008

11. The clinical spectrum of early-onset esotropia:
If it looks like CET: is it CET?
CONGENITAL ESOTROPIA
Kowal 2008

12. PEDIG CET Observational Study
ET with onset in early infancy frequently resolves in patients
first examined < 20 w of age
ET < 40 ∆
ET intermittent or variable.
CONGENITAL ESOTROPIA
Kowal 2008

13. PEDIG CET Observational Study
ET ≥40 ∆ presenting after 10 w of age : low likelihood of spontaneous resolution.
Surgery at 3-4 mo of age could reasonably be considered in some CETs
CONGENITAL ESOTROPIA
Kowal 2008

14. Early Very early Late How late TIMING OF TREATMENT
CONGENITAL ESOTROPIA
Kowal 2008

15. DOM Duration of misalignment
< 4 mo DOM:
Stereo, reduced need for 2nd surgery, reduced incidence DVD [Birch]
<12 mo DOM & age:
Stereo better than >12 mo [Ing, 2002]
CONGENITAL ESOTROPIA
Kowal 2008

16. OVERVIEW OF MGMT Check vision - any obvious amblyopia
Amblyopia Rx: patch 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 [mechanical barrier]
CONGENITAL ESOTROPIA
Kowal 2008

17. OVERVIEW 2 Measure angle ≥ 2 times, consistent or increasing
Check refraction
>+3 : try anti- accommodative Rx
Gls / pilo / phospholine
THEN: alignment as soon as convenient
CONGENITAL ESOTROPIA
Kowal 2008

18. OVERVIEW Bimedial recession - reliable to 50∆
Recess / resect prob = BMR to 35∆
Augment for very large angles - botox, 1-2 extra muscles
CONGENITAL ESOTROPIA
Kowal 2008

19. OVERVIEW Day surgery Check within 24-36 hours re: slipped stitch
Recurrent / residual ET often accommodative
Consceutive XT with time ~1% p.a.
CONGENITAL ESOTROPIA
Kowal 2008

20. RESULTS Orthotropia [for D and/ or N] @ 2 mo: 80%
Subsequent careful mngmt for recurrent ET, amblyopia
CONGENITAL ESOTROPIA
Kowal 2008

21. LK RESULTS Selection bias: Private pts Healthy infants
Multiple visits [> than feasible in public setting]
…other unrecognised bias
2nd surgery for 12 mo:
LK: %
CONGENITAL ESOTROPIA
Kowal 2008