BASICS
DESCRIPTION
Ocular motility disorder characterized by an inability to actively or passively elevate the eye in adduction
EPIDEMIOLOGY
Incidence
Occurs in 1 of 450 strabismus cases
Prevalence
Unknown
RISK FACTORS
• Trauma to superior oblique muscle or trochlea
• Juvenile idiopathic or rheumatoid arthritis
Genetics
Autosomal dominant inheritance has been observed rarely.
PATHOPHYSIOLOGY
Restricted elevation when adducting caused by either an abnormal superior oblique muscle or tendon, or mechanical limitation at the trochlea.
ETIOLOGY
• Congenital: A tight or inelastic superior oblique tendon
• Acquired:
– Due to trauma either to the superior oblique muscle/tendon, or to the trochlea
– Inflammation, in particular juvenile idiopathic and adult rheumatoid arthritis (1)[A], (2)[A]
COMMONLY ASSOCIATED CONDITIONS
• Juvenile idiopathic or adult rheumatoid arthritis
• Trauma
• No other consistent systemic or strabismus associations (3)[A]
DIAGNOSIS
HISTORY
• Unusual eye movement in upgaze in adduction: May not be noticed by parents until later in childhood:
– Acquired: Can be diplopic in upgaze and occasionally in contralateral gaze
– Pain or sensation of a “click” on attempted elevation in adduction
– Inflammatory causes may note supranasal orbital pain, swelling, and tenderness.
• History of arthritis or joint pain
• History of trauma
PHYSICAL EXAM
• Limited elevation in adduction can range from minimal to severe.
• Elevation in abduction is normal.
• Face-turn away from the involved eye or chin-up position to avoid associated diplopia.
• Hypotropia in primary or in gaze away from the involved eye.
• Amblyopia can occur but is rare.
• With acquired, an audible or palpable superior nasal click may be found on attempted elevation in adduction.
• Swelling and tenderness occur in the trochlear space.
• Full dilated eye examination is performed with refraction.
• Uveitis associated with arthritis is checked.
DIAGNOSTIC TESTS & INTERPRETATION
Lab
Initial lab tests
If acquired, testing for possible arthritis is performed (ANA, rheumatoid factor, erythrocyte sedimentation rate, complete blood count with differential, urinalysis) (see the Pediatric Uveitis chapter).
Follow-up & special considerations
Even if initial testing for arthritis is negative, continue follow-up as it may appear after Brown syndrome occurs.
Imaging
MRI scan can show enhancement of the superior oblique/trochlear region in acquired cases, but is not necessary to make the diagnosis (4)[A].
Diagnostic Procedures/Other
• Positive forced duction testing
• Ultrasound of trochlear area may be useful in acquired cases.
DIFFERENTIAL DIAGNOSIS
• Double elevator palsy (see the chapter on this)
• Primary inferior oblique palsy
• Orbital fracture, tumor, or hemorrhage
• Absent/anomalous muscles as in craniosynostosis syndromes
TREATMENT
MEDICATION
First Line
• In acquired nontraumatic cases:
– In patients with associated juvenile idiopathic or rheumatoid arthritis, treatment of the underlying condition often results in resolution of Brown syndrome.
– In patients with no associated condition, anti-inflammatory medication such as oral ibuprofen can be used.
– If inflammation persists, oral nonsteroidal anti-inflammatory drugs can be used.
Second Line
If inflammation persists, oral corticosteroids or local steroid injections in the area of the trochlea may be helpful.
ADDITIONAL TREATMENT
General Measures
Treatment of Brown syndrome is necessary when a hypotropia in primary gaze or a cosmetically significant head turn is present. Many patients do not require treatment as they are comfortable visually, do not have an adaptive head position, and learn to avoid the involved position.
Issues for Referral
Rheumatology consultation may be helpful in acquired cases.
COMPLEMENTARY & ALTERNATIVE THERAPIES
None proven or indicated
SURGERY/OTHER PROCEDURES
• Indications for surgery include the presence of significant head position or a hypotropia in primary gaze.
• The goals of surgery are to allow a more normal head position and to lessen diplopia in the largest possible field of gaze.
• Surgical treatment is to release superior oblique restriction without causing further symptoms, such as a hypotropia due to overaction of the inferior oblique.
• Surgical procedures include:
– Superior oblique tenotomy or tenectomy
– Superior oblique tenotomy with:
Split tendon lengthening
Suture bridge placement
Silicone tendon expander placement
• Goal with the above procedures in addition to tenotomy is to lessen postoperative superior oblique weakness (5)[A], (6)[A].
ONGOING CARE
FOLLOW-UP RECOMMENDATIONS
As needed for monitoring of amblyopia and further diplopia or unusual head position
Patient Monitoring
Visual acuity and overall function
PROGNOSIS
• Congenital:
– Many patients do not require intervention as their related symptoms are not significant.
– Face turn or hypotropia can be corrected with surgery but long-term follow-up is needed to rule out amblyopia or consecutive inferior oblique overaction.
• Acquired:
– When associated with inflammatory disease, often resolves with systemic treatment
– Can resolve spontaneously in acquired nontraumatic cases
REFERENCES
1. Wilson ME, Eustis HS Jr, Parks MM. Brown’s syndrome. Surv Ophthalmol 1989;34(3):153–172.
2. Kaban TJ, Smith K, Orton RB, et al. Natural history of presumed congenital Brown syndrome. Arch Ophthalmol 1993;111(7):943–946.
3. Wang FM, Wertenbaker C, Behrens MM, et al. Acquired Brown’s syndrome in children with juvenile rheumatoid arthritis. Ophthalmology 1984;91(1):23–26.
4. Bhola R, Rosenbaum AL, Ortube MC, et al. High-resolution magnetic resonance imaging demonstrates varied anatomic abnormalities in Brown syndrome. J AAPOS 2005;9(5):438–448.
5. Suh DW, Oystreck DT, Hunter DG. Long-term results of an intraoperative adjustable superior oblique tendon suture spacer using nonabsorbable suture for Brown syndrome. J AAPOS 2003;7(4):274–278.
6. Sprunger DT, von Noorden GK, Helveston EM. Surgical results in Brown syndrome. J Pediatr Ophthalmol Strabismus 1991;28(3):164–167.
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