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].

Techniques and results of treatment for Brown syndrome at two institutions were reviewed. [ncbi.nlm.nih.gov]

Treatment Generally treatment for individuals with BSS focuses on the underlying cause of the disorder. Early treatment with high-dose steroids may be beneficial in many cases. Other treatment is symptomatic and supportive. [ninds.nih.gov]

Nonsurgical treatment is often advised for recently acquired, traumatic and intermittent cases. [aapos.org]

Prognosis The prognosis for individuals with BSS varies depending on the cause of the disorder. x Prognosis The prognosis for individuals with BSS varies depending on the cause of the disorder. [ninds.nih.gov]

If the cervical spinal cord lesion is incomplete, such as central cord or Brown-Séquard syndrome, younger patients with have a more favorable prognosis for recovery. [emedicine.medscape.com]

Prognosis The prognosis for Brown-Séquard's syndrome is generally poor although it may be better than other forms of spinal cord injury.[13] Aetiology can have a bearing on prognosis. [patient.info]

Preoperative 1 month 12 month P AER (0-3) 2,88 0,92 0,59 Torticollis (%) 79.1 8 4 Triamcinolone injection in trochlea was not related with the prognosis, the existence of torticollis, or the value of AER (P 0.05). [article.sciencepublishinggroup.com]

Acquired nonsuperior oblique Brown syndrome Etiology of acquired nonsuperior oblique Brown syndrome Acquired nonsuperior oblique etiologies: Inferior orbital fibrous adhesions to the posterior globe are caused by the following: orbital floor fracture [emedicine.com]

Congenital Brown syndrome may be associated with an absent trochlear nerve and hypoplastic superior oblique muscle suggesting an etiologic mechanism of congenital cranial dysinnervation disorder. [ncbi.nlm.nih.gov]

Terminology Also called sideropenic dysphagia Epidemiology Occurs mostly in Scandinavia and Great Britain Usually women ages 30 years Treatment Iron supplements, mechanical dilation and monitor for upper GI carcinoma Clinical images Images hosted on other [pathologyoutlines.com]

Epidemiology[edit] In Brown's original series there was a 3:2 predominance of women to men and nearly twice as many cases involved the right eye as the left. 10% of cases showed bilaterality. [en.wikipedia.org]

CT is a valuable tool in understanding the pathophysiology and management of acquired Brown syndrome, showing thickening and inflammatory changes of the reflected portion of the superior oblique tendon. [ncbi.nlm.nih.gov]

CT is a valuable tool in understanding the pathophysiology and management of acquired Brown syndrome, showing thickening and inflammatory changes of the reflected portion of the superior oblique tendon. Article History Published in print: 1985 [pubs.rsna.org]

While its pathophysiology is still unknown, it was initially described in aphakic patients classically after intracapsular lens extraction.[1] [2] Most patients were elderly although patients as young as 12 years old have been reported.[8] [9] It typically [eyewiki.aao.org]

Surgical exploration revealed adhesions that prevented the normal sliding of the superior oblique tendon beneath the superior rectus muscle. Forced ductions became normal and downgaze improved after removal of the silicone band. [ncbi.nlm.nih.gov]

Prognosis and prevention[3, 12] Correction of iron deficiency will correct the condition if malignancy has not yet occurred. Prevention of iron deficiency prevents the disease. Historical notes[2] Henry Plummer (1874-1937) was an American internist. [patient.info]

In all cases, the child’s vision is closely monitored to prevent other problems down the road. Takeaways Having one eye that cannot move and ends up stuck in the upward position could be diagnosed as Brown syndrome. [bundoo.com]

This prevents the lower eye from raising the eye inward. Brown’s Syndrome can be congenital or acquired in adulthood. It may be constant or intermittent. In 90% of cases, only one eye is affected, more often, the right one. [mariaelisascarale.it]

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.