Download - Hereditary brown syndrome
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HEREDITARY BROWN SYNDROME
DR. JOSEPH KURIANPG STUDENT , ACME PARIYARAM
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DEFINITION• Brown syndrome is a disorder of
elevation involving mechanical restriction of extra ocular movement.
• Brown was the first to describe this anomaly
• He suspected a congenitally short superior oblique tendon sheath to be the cause hence
“superior oblique tendon sheath syndrome”
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INCIDENCE
• Incidence is low – 6 in 2583 (Croswell & Haldi )
• Familial occurrence is rare 2%(Birgit Lorenz, Michael C. Brodsky
)
• Usually unilateral but 10% is bilateral
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• Syndrome can be : Congenital Acquired Constant Intermittent
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SUPERIOR OBLIQUE MUSCLE
• Origin : Annulus of Zinn at the orbital apex, medial to optic canal
• Insertion :Outer posterior quadrant of the eyeball (insertion line 11mm)
• Artery : Lateral muscular branch of the ophthalmic artery
• Nerve : Trochlear nerve
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SUPERIOR OBLIQUE MUSCLE
• Actions : Primary action Intorsion Secondary actions Abduction Depress the eyeball
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AETIOLOGY
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AETIOLOGY1. Anomalies of Superior Oblique Tendon
Sheath
2. Anomalies of Superior Oblique tendon
► Shortening of tendon due to development anomalies
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AETIOLOGY3. Anomalies of Inferior oblique & adjacent
structures
► Dense attachments causing restriction of action
4. Impaired slippage of tendon through Trochlea
► Anomalies of Trochlea ►Retro bulbar thickening of tendon
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AETIOLOGY5. Acquired causes
► Post operative after superior oblique tucking surgery, scleral buckling & Molteno implant
► Trauma to orbital roof
► Inflammatory conditions like Rheumatoid arthritis
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CLINICAL FEATURES CONSISTENT
• Absence of elevation in adduction
• Normal elevation in abduction
• Forced duction test shows restriction in adduction
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CLINICAL FEATURES VARIABLE• Mild limitation of elevation in primary gaze
• Down shoot in adduction
• Widening of palpebral fissure
• Hypotropia in primary position
• Compensatory head posture>Head Tilt ,Chin lift
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DIAGNOSISI. Inability to elevate eye in adduction
II. Normal elevation in abduction
III. Forced Duction Test shows resistance in adducted position to elevation
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DIFFERENTIAL DIAGNOSISi. Paralysis of Inferior oblique muscle
ii. Congenital fibrosis of inferior rectus muscle
iii. Grave’s ophthalmopathy
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CASE HISTORY 8yr old girl presented to our ophthal OPD
. Her mother had noticed squinting in the RE in certain gaze positions. No complaints of diplopia .
Family History: Father gives history of squint in LE. He is a diagnosed case of Browns syndrome in LE.
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ON EXAMINATION DaughterHead posture Normal
Hirschberg Test Central
Cover Test Orthophoria in primary gaze
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ON EXAMINATION Daughter RE LE
Extra ocular Restriction in Equal & fullmovement elevation on adduction
Forced duction Resistance to Normal test elevation on adduction
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ON EXAMINATION FatherHead posture Normal
Hirschberg Test Central
Cover Test Orthophoria in primary gaze
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ON EXAMINATION Father
RE LE
Extra ocular Equal & full Restriction inmovement elevation on adduction
Forced duction Normal Resistance to test elevation on adduction
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TREATMENTINDICATIONS:
i. Involved eye hypotropic in primary gaze position
ii. Significant anomaly in head posture Head tilt towards affected side Chin lift
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TREATMENTPROCEDURES Complete Tenectomy of Superior Oblique
muscle. * Forced duction test becomes negative * In case of symptoms of superior oblique
paralysis consider recession of contra lateral IR muscle or ipsilateral IO muscle.
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TREATMENTSilicon superior oblique tendon expanders
to lengthen tendon * Preferred procedure * Advantage: No symptoms of SO
palsy
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REFERENCES• BINOCULAR VISION & OCULAR
MOTILITY 5th edition by Gunter K von Noorden
• DELHI JOURNAL OF OPHTHALMOLOGY vol 22
• KANSKI BOWLING 7th edition
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THANK YOU