Brown syndrome is a rare disorder of the eye distinguished by pathological changes of the superior oblique muscle and its tendon, resulting in the inability to elevate the eye while being in adduction. Both congenital and acquired forms are described, and the diagnosis can be made only after a thorough physical examination and a detailed patient history.
Presentation
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].
Workup
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].
Treatment
Treatment for Brown Syndrome depends on the severity of the symptoms. In mild cases, observation and regular follow-up may be sufficient. For more severe cases, treatment options include:
- Prism Glasses: To help alleviate double vision.
- Surgical Intervention: Surgery may be considered to correct the mechanical restriction of the superior oblique tendon.
- Steroid Injections: In cases where inflammation is a contributing factor, corticosteroid injections may be used.
Prognosis
The prognosis for Brown Syndrome varies. Many patients experience stable symptoms without significant progression. In some cases, especially in children, the condition may improve over time. Surgical intervention can be effective in alleviating symptoms, but the outcome depends on the individual case. Regular monitoring by an eye specialist is essential to manage the condition effectively.
Etiology
The exact cause of Brown Syndrome is not always clear. Congenital cases are often due to abnormalities in the superior oblique tendon or its sheath. Acquired cases can result from trauma, inflammation, or surgery affecting the eye muscles. In some instances, systemic conditions like rheumatoid arthritis can contribute to the development of the syndrome.
Epidemiology
Brown Syndrome is a rare condition, with an estimated prevalence of 1 in 400 to 1 in 450 strabismus cases. It affects both males and females equally and can occur at any age, though congenital cases are more common. The condition is not associated with any particular ethnic or geographic population.
Pathophysiology
The pathophysiology of Brown Syndrome involves a mechanical restriction of the superior oblique tendon. This restriction prevents the eye from moving freely, particularly in upward and inward directions. In congenital cases, this may be due to a short or inelastic tendon sheath. In acquired cases, inflammation or scarring can lead to similar restrictions.
Prevention
Currently, there are no known preventive measures for Brown Syndrome, especially for congenital cases. For acquired cases, managing underlying conditions such as inflammation or trauma may help reduce the risk. Regular eye examinations can aid in early detection and management of symptoms.
Summary
Brown Syndrome is a rare eye movement disorder characterized by restricted upward and inward eye movement. It can be congenital or acquired and presents with symptoms like double vision and abnormal head posture. Diagnosis involves a detailed eye examination, and treatment options range from observation to surgery. The prognosis varies, with some cases improving over time.
Patient Information
For patients, understanding Brown Syndrome involves recognizing the symptoms of restricted eye movement and potential double vision. It's important to follow up with an eye specialist for regular monitoring and management. Treatment options are available, and many patients can lead normal lives with appropriate care. If you notice any changes in your vision or eye movement, consult with your healthcare provider for further evaluation.
References
- Olusanya BA. Brown syndrome with severe amblyopia: a case report from Africa. Pan Afr Med J. 2015;20:56.
- Cho YA, Kim S, Graef MH. Surgical Outcomes in Correction of Brown Syndrome. Korean J Ophthalmol. 2006;20(1):33-40.
- Kenawy N, Pilz DT, Watts P. Familial unilateral Brown syndrome. Indian J Ophthalmol. 2008;56(5):430-434.
- Lambert SR. Late spontaneous resolution of congenital Brown syndrome. J AAPOS. 2010;14(4):373-375.
- Currie S, Goddard T. MR imaging features of acquired Brown syndrome. AJNR Am J Neuroradiol. 2009;30(9):1778-1779.
- Kent JS, Makar I. Hypertropia following Spontaneous Resolution of Brown’s Syndrome. Case Rep Ophthalmol. 2012;3(2):230-235.