CONGENITAL ESOTROPIA.

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Presentation transcript:

CONGENITAL ESOTROPIA

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

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

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

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

VERTICALS IN CET : DVD CONGENITAL ESOTROPIA Kowal 2008

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

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

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

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

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

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

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

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

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

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

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

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

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

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

LK RESULTS Selection bias: Private pts Healthy infants Multiple visits [> than feasible in public setting] …other unrecognised bias 2nd surgery for BMR @ 12 mo: LK: 10 -15% CONGENITAL ESOTROPIA Kowal 2008