Brown syndrome is a relatively rare condition of the eye apparatus, in which various factors render the superior oblique muscle unable to perform its respective function - elevation of the eye while being in adduction, principally as a result of mechanical limitation [1] [2] [3] [4]. Numerous conditions have been implicated in the pathogenesis of this ocular disorder, including juvenile and rheumatoid arthritis, systemic diseases (scleroderma, Sjogren's syndrome, systemic lupus erythematosus), orbital metastatic deposits, but also trauma, inflammation, and surgery, all leading to restriction of superior oblique tendon movement [1] [2] [3] [5]. On the other hand, many individuals develop Brown syndrome from birth, indicating that congenital forms of the disease also exist [1] [2] [4] [5]. Furthermore, familial Brown syndrome, estimated to occur in approximately 1 in 20,000 live births, was also recognized in the literature [1] [3]. The clinical presentation is characterized by the inability of patients to elevate the eye in adduction, producing predominantly unilateral (although 10% of cases exhibit bilateral) changes in eye movement [1] [2] [3]. Consequently, a slight downward deviation of the eye on adduction is noted [1] Studies did not show a predilection toward gender or either of the eyes [1]. In the setting of the congenital Brown syndrome, spontaneous resolution is seen in 11-76% of children, but complications such as hypertropia, amblyopia, and other visual deficits may develop [1] [4] [6].

• Head Tilted Backward

  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 [ophthnotes.com]

  Brown characterized the syndrome in many ways such as:[2] 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 [en.wikipedia.org]

• Localized Edema

  edema occurring within the confined area of the trochlea. [emedicine.com]

• Cape Verde

  Verde","region_id":1},{"id":126,"name":"Cayman Islands","region_id":1},{"id":43,"name":"Central African Republic","region_id":1},{"id":217,"name":"Chad","region_id":1},{"id":48,"name":"Chile","region_id":1},{"id":50,"name":"China","region_id":1},{"id [radiopaedia.org]

• Pediatric Disease

  Results in Superior Oblique Sharpening in Brown Syndrome and Systemic Pediatric Diseases. International Journal of Clinical Pediatric Surgery. [article.sciencepublishinggroup.com]

• Skin Thickening

  Figure 1: (a) Skin thickening of the fingers extending proximal to the metacarpophalangeal joints and severe flexion contractures of the fingers. [ijo.in]

• Pupillary Abnormality

  Brown syndrome - syndrome occurring in individuals with light complexion, blond hair, light eyes and characterized by loss of pain sensitivity, pupillary abnormalities, neurogenic anhidrosis, vasomotor instability. Synonym(s): neural crest syndrome [medical-dictionary.thefreedictionary.com]

• Hip Pain

  Musculoskeletal disease was found in 2 patients (8.3%), including recurrent hip pain and lumbar vertebral column scoliosis. [article.sciencepublishinggroup.com]

• Flexion Contracture

  Figure 1: (a) Skin thickening of the fingers extending proximal to the metacarpophalangeal joints and severe flexion contractures of the fingers. [ijo.in]

• Frontal Headache

  By age 5 years, he complained to his mother of daily, intermittent diplopia, eye pain in both eyes (OU), and frontal headaches. Additional strabismus surgeries OU were done at ages 6¾ years and 8¼ years. [ncbi.nlm.nih.gov]

• Temporal Headache

  Case report Case 1 A 44-year-old female with past medical history of rheumatoid arthritis presented with a 2-week history of left retro-orbital pain and temporal headaches. She also developed transient diplopia with rightward or downward gaze. [ncbi.nlm.nih.gov]

The diagnosis of Brown syndrome can be made only after an extensive workup. Because the initial diagnosis of an eye-related pathology might be made by a simple evaluation of eye movement, the role of a properly performed physical examination is pivotal in recognizing this condition. Apart from the obvious inability to perform eye elevation while being in adduction, several other features that are seen during the exam are a downward deviation in adduction, widening of the palpebral fissure when the patient adducts the eye and the absence of over-activation of the superior oblique muscle [1]. Additionally, the forced duction test, during which the limbus is grasped by a forceps with the attempt to move the eye mechanically, will be positive - restricted movement will be observed due to the mechanical nature of the disease. In that case, the diagnosis of Brown syndrome is almost definite [1]. To identify the exact cause of this condition, a thorough patient history is helpful in distinguishing between congenital and acquired forms. Age of onset, as well as a history of previous conditions or events that could lead to Brown syndrome, should be assessed. Imaging studies, such as computed tomography (CT) and magnetic resonance imaging (MRI) have been regarded as potentially useful tools in solidifying the diagnosis [5]. Thickening of the superior oblique tendon on T1 and high signal intensity on T2-weighed imaging have been reported in this condition [5].

1. Olusanya BA. Brown syndrome with severe amblyopia: a case report from Africa. Pan Afr Med J. 2015;20:56.
2. Cho YA, Kim S, Graef MH. Surgical Outcomes in Correction of Brown Syndrome. Korean J Ophthalmol. 2006;20(1):33-40.
3. Kenawy N, Pilz DT, Watts P. Familial unilateral Brown syndrome. Indian J Ophthalmol. 2008;56(5):430-434.
4. Lambert SR. Late spontaneous resolution of congenital Brown syndrome. J AAPOS. 2010;14(4):373-375.
5. Currie S, Goddard T. MR imaging features of acquired Brown syndrome. AJNR Am J Neuroradiol. 2009;30(9):1778-1779.
6. Kent JS, Makar I. Hypertropia following Spontaneous Resolution of Brown’s Syndrome. Case Rep Ophthalmol. 2012;3(2):230-235.