CONGENITAL ESOTROPIA

CAUSE • Subtle neurological developmental problem • Usually in isolation [selection bias] CONGENITAL ESOTROPIA Kowal 2008 3

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

ASSOCIATIONS 2 BRAIN • Down’s 30% • Severe neonatal course IVH / HC 100% • PVL • ‘delayed devpt’ ~20% CONGENITAL ESOTROPIA Kowal 2008 10

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

TIMING OF TREATMENT •

Early

•

Very early

• Late • How late 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 BMR @ 12 mo: LK: 10 -15% CONGENITAL ESOTROPIA Kowal 2008 22