Brown syndrome (named after Dr. Harold W. Brown) is also known as Superior Oblique Tendon Sheath syndrome.(see figure 1) It is a mechanical problem in which the superior oblique muscle/tendon (on the outside of the eyeball) is unable to lengthen and therefore does not move freely. This makes looking up and in with the affected eye difficult. Brown Syndrome may be present at birth (congenital) or begin later. It may be constant or intermittent.
The eyes usually look normal except in side gaze positions. In side gaze (looking away from the affected side), one eye appears higher than the other, particularly when looking up. A vertical misalignment is sometimes noted when looking straight ahead.
Often the higher eye is mistakenly presumed to be the abnormal eye, but it is actually the lower eye that is affected. Brown syndrome causes the affected eye to have trouble looking upward and inwards towards the nose. Essentially the affected eye is “tethered” or held down by the tight superior oblique tendon.
Brown syndrome may be acquired or congenital. Acquired Brown syndrome is uncommon but may be seen following surgery, after trauma or in association with inflammatory diseases. Trauma can cause a Brown Syndrome if a blunt object hits the eye socket in the upper inside corner near the nose. Surgery for the eyelid, frontal sinus, eyeball (retinal detachment) and teeth (dental extraction) have been linked to acquired Brown syndrome. Inflammation of the tendon-trochlea complex (from adult and juvenile rheumatoid arthritis, systemic lupus erythematosus and sinusitis) can be associated with development of the problem. Sometimes the cause is never identified.
Hereditary cases of Brown syndrome are rare. Most cases arise without a family history (sporadic).
The eyes are usually straight when looking directly ahead and down. The hallmark sign of Brown syndrome is decreased ability to look upward and inward with the affected eye(s). In some situations the eyes turn outward (exotropia) when looking up.
Brown syndrome can be associated with an abnormal head position (chin up, face turn, head tilt) to help allow the eyes to work together better. The affected eye can get “stuck” after looking up or down for long periods of time. When the eye becomes unstuck, a click is often heard and may be accompanied by pain or discomfort.
Brown syndrome may be more noticeable in children since they often look upward toward adults.
Ninety percent of patients have only one affected eye, more commonly the right.
Some children with Brown syndrome have poor binocular vision (which can result in poor depth perception) and/or amblyopia.
Brown syndrome can be classified according to severity. In mild cases there is a reduced ability to look up and in with the affected eye. In moderate cases, there is also a tendency for the eye to move downward as it moves inward. In severe cases there is a tendency for the affected eye to turn downward when the patient looks straight ahead.
Spontaneous resolution sometimes occurs in acquired and intermittent cases. In the congenital form of Brown syndrome, the eye movement problem is usually constant and unlikely to resolve spontaneously.
Treatment recommendations for Brown syndrome vary according to the cause and severity of the movement disorder. Close observation alone is usually sufficient in mild cases. Visual acuity and the ability to use both eyes at the same time (binocular vision) should be monitored closely in young children. Nonsurgical treatment is often advised for recently acquired, traumatic and variable cases. Systemic and locally injected corticosteroids have been used to treat inflammatory cases of acquired Brown syndrome. Non-steroidal anti-inflammatory agents (like ibuprofen) have also been used. Surgical treatment is usually recommended if any of the following are present: eye misalignment when looking straight ahead, significant double vision, compromised binocular vision or pronounced abnormal head position. More than one surgery may be needed for optimal management.
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