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HEREDITARY BROWN SYNDROME
DR. JOSEPH KURIANPG STUDENT , ACME PARIYARAM
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”
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
• Syndrome can be : Congenital Acquired Constant Intermittent
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
SUPERIOR OBLIQUE MUSCLE
• Actions : Primary action Intorsion Secondary actions Abduction Depress the eyeball
AETIOLOGY
AETIOLOGY1. Anomalies of Superior Oblique Tendon
Sheath
2. Anomalies of Superior Oblique tendon
► Shortening of tendon due to development anomalies
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
AETIOLOGY5. Acquired causes
► Post operative after superior oblique tucking surgery, scleral buckling & Molteno implant
► Trauma to orbital roof
► Inflammatory conditions like Rheumatoid arthritis
CLINICAL FEATURES CONSISTENT
• Absence of elevation in adduction
• Normal elevation in abduction
• Forced duction test shows restriction in adduction
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
DIAGNOSISI. Inability to elevate eye in adduction
II. Normal elevation in abduction
III. Forced Duction Test shows resistance in adducted position to elevation
DIFFERENTIAL DIAGNOSISi. Paralysis of Inferior oblique muscle
ii. Congenital fibrosis of inferior rectus muscle
iii. Grave’s ophthalmopathy
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.
ON EXAMINATION DaughterHead posture Normal
Hirschberg Test Central
Cover Test Orthophoria in primary gaze
ON EXAMINATION Daughter RE LE
Extra ocular Restriction in Equal & fullmovement elevation on adduction
Forced duction Resistance to Normal test elevation on adduction
ON EXAMINATION FatherHead posture Normal
Hirschberg Test Central
Cover Test Orthophoria in primary gaze
ON EXAMINATION Father
RE LE
Extra ocular Equal & full Restriction inmovement elevation on adduction
Forced duction Normal Resistance to test elevation on adduction
TREATMENTINDICATIONS:
i. Involved eye hypotropic in primary gaze position
ii. Significant anomaly in head posture Head tilt towards affected side Chin lift
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.
TREATMENTSilicon superior oblique tendon expanders
to lengthen tendon * Preferred procedure * Advantage: No symptoms of SO
palsy
REFERENCES• BINOCULAR VISION & OCULAR
MOTILITY 5th edition by Gunter K von Noorden
• DELHI JOURNAL OF OPHTHALMOLOGY vol 22
• KANSKI BOWLING 7th edition
THANK YOU