Transcript Duane`s Retraction Syndrome

Duane’s Retraction Syndrome
Dr Sunayana Bhat
Consultant
Paediatric ophthalmology , Strabismus and Neuro ophthalmology
Vasan eye care , Mangalore
Ph : 9611102754
[email protected]
Historical Background
• Duane syndrome is a rare, congenital disorder of eye
movement
• Stilling and Turk : described it first
• Duane popularised it
Classic features
Limitation of abduction
Slight limitation of adduction
Retraction of globe on adduction
Adduction narrowing of palpebral fissure
Upshoot / downshoot of eye in adduction
Pathophysiology
Fibrosis of LR/MR
“ Musculo Fascial
Innervational
Anomaly ”
Fibrous bands
Co contraction –
paradoxical innervation
Duane’s Syndrome Type I: OS
-limited abduction
-retraction in adduction
Duane’s Syndrome Type II: OS
-limited adduction
-retraction in adduction
Duane’s Syndrome Type III: OS
-limited adduction and abduction
-retraction in adduction/abduction
History
•
•
•
•
•
•
Squinting
Head tilt
Loss of binocular vision
Reduced ocular movement
Facial asymmetry
Picture of paralytic squint
Epidemiology
•
•
•
•
•
1% of all cases of strabismus
Sporadic
Unilateral
60%
Left eye
Female preponderance
Clinical Picture
•
•
•
•
AHP
Strabismus ( 77% of cases)
Globe retraction
Palpebral fissure narrowing
• Anisometropia /amblyopia
• Sensory adaptation with excellent binocular functions
-directions of gaze where visual axes are aligned
-suppression without diplopia in gaze of abnormal muscle
Associated Anomalies
• Skeletal
• Auricular
• Ocular
-dysplasia of the iris stroma,
-pupillary anomalies
-cataracts
- heterochromia
- Marcus Gunn jaw-winking
-coloboma
- crocodile tears
-microphthalmos.
• Neural
Syndromes
•
•
•
•
•
•
Okihiro syndrome
Wildervanck syndrome
Holt-Oram syndrome
morning-glory syndrome
Goldenhar syndrome.
congenital labyrinthine deafness.
Klippel-Feil anomaly
Variants
• Bilateral DRS
Inverse DRS
Differentials
DRS
Sixth N palsy
• ET small angle
• ET large angle
• Narrowing
• -ve
• Saccadic velocities
• Slow in abduction only
Differentials
• Mobius syndrome
Treatment
• Treat refractive error /amblyopia
• Conservative : Age 5-6 yrs
Indications for surgery
•
•
•
•
Anomalous head posture
Strabismus in primary gaze
Significant upshoot or downshoot in adduction
Cosmetically significant palpebral fissure narrowing in
adduction.
“ Limited Goals ”
Made simple …
Small angle <15
PD
Single MR
recession
Larger angle
Bilateral MR
recession
Eso DRS
Asymmetric
Small angle <15
PD
Single LR
recession
Larger angle
Bilateral LR
recession
Exo DRS
Symmetric
Recession of MR
and LR
Upshoots
Downshoots
Y split
Globe retraction
Posterior fixation
suture to LR
Transposition of
SR /IR
Duction
Improvement
FOSTER’S
modification
Carry home message
• Diagnosis often elusive SO LOOK for it !
• Treat Ref Error , amblyopia
• Risk benefit ratio of surgery to be well understood by patients
and surgeon
• Tailor made surgery required for every case
Thank u