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7 year old girl with a left face turn and binocular diplopia
Digital Journal of Ophthalmology 1997
Volume 3, Number 13
February 18, 1997
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Mitchell S. Fineman, M.D. | Wills Eye Hospital, Philadelphia, PA
Joseph Calhoun, M.D. | Wills Eye Hospital, Philadelphia, PA
Diagnosis and Discussion
Brown's Syndrome

Brown's syndrome is characterized clinically by the following: deficient elevation of the affected eye in adduction, improved elevation in the midline, and normal elevation in abduction. Superior oblique overaction, if present, should be minimal at most. Midline elevation produces divergence, manifesting as a V pattern exotropia. Finally, positive forced duction test of the affected eye demonstrating restricted passive elevation in adduction confirms the diagnosis.

Other clinical manifestations which may be seen include: downshoot of the affected eye in adduction, widened palpebral fissure on adduction, anomalous head posture, and primary position hypotropia.

Brown's syndrome is classified as congenital or acquired depending on the age of onset, and constant or intermittent depending on the clinical course.

Most cases of congenital and constant Brown's syndrome are thought to be caused by developmental anomalies of the superior oblique tendon and/or trochlea apparatus. Most cases of acquired Brown's syndrome are idiopathic. However, Brown's syndrome may also develop secondary to inflammatory conditions such as juvenile rheumatoid arthritis, trauma to the superomedial orbit, mass lesions involving the superomedial orbit, and postoperatively following surgery on the superior oblique tendon, orbit, retina, or sinus.

The indications for treatment of Brown's syndrome are abnormal head position and significant hypotropia in primary position. However, most acquired cases should be observed for approximately one year since spontaneous improvement has been reported.

Treatment options include observation alone, injection of steroids near the trochlea in inflammatory conditions, superior oblique tenotomy, superior oblique tenectomy, simultaneous superior oblique tenotomy and inferior oblique muscle recession, and superior oblique tendon silicone expander.

Complications of treatment include signs and symptoms of a superior oblique muscle palsy and improved but persistent Brown's syndrome.

Key Points:
- The diagnosis of Brown's syndrome can be made when the classic motility defect is seen. The diagnosis is confirmed with positive forced duction testing in the affected eye.
- Acquired Brown's syndrome may be secondary to various inflammatory, neoplastic and post-traumatic conditions. Imaging studies and serologic testing are useful in the evaluation of underlying conditions.
- The most common management for all types of Brown's syndrome is observation. Acquired and intermittent cases have a higher rate of spontaneous regression than congenital and constant cases.
- The main indications for surgery in Brown's syndrome are primary position hypotropia and/or an anomalous head posture.