Brown syndrome

1. Brown syndrome is a rare form of strabismus characterized by limited elevation of the affected eye.  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. 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.The disorder may be congenital (existing at or before birth), or acquired.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).
2. Harold W. Brown characterized the syndrome in many ways such as:

   • Limited elevation in the eye when head is straight up
   • Eyes point out in a straight up gaze (divergence in up gaze)
   • Widening of the eyelids in the affected eye on adduction
   • Head tilts backwards (compensatory chin elevation to avoid double vision)
   • Near normal elevation in abduction.

   
   

   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.
3. In Brown's original series there was a 3:2 predominance of women to men. Ninety percent of patients have only one affected eye, more commonly the right. 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.The goal of surgery is to restore free ocular rotations. Various surgical techniques have been used:
• Harold Brown advocated that the superior oblique tendon be stripped. A procedure named sheathotomy. The results of such a procedure are frequently unsatisfactory because of reformation of scar tissue.
• Tenotomy of the superior oblique tendon (with or with out a tendon spacer) has also been advocated. This has the disadvantage that it frequently produces a superior oblique paresis.
• Weakening of the inferior oblique muscle of the affected eye may be needed to compensate for iatrogenic fourth nerve palsy. 

1. It's also known as Superior Oblique Tendon Sheath syndrome. Not to be confused with Brown-Vialetto-Van Laere syndrome.