What is Brown Syndrome?
Brown syndrome is a condition that can be present at birth or develop later in life. It’s caused by problems with a muscle in the eye called the superior oblique muscle. This issue can be due to damage in the trochlea, a part of the muscle’s tendon, issues surrounding the extraocular muscles, or abnormalities in the superior oblique muscle itself. When present in babies, it affects their ability to focus on objects above their line of sight.
When Brown syndrome happens later in life, it’s often because of inflammation, injury, surgery, or conditions like rheumatoid arthritis. It can show itself in different ways and severity, sometimes posing difficulties in diagnosis and treatment. There’s also a kind known as pseudo-Brown syndrome that is typically seen after implant surgery, but it doesn’t involve the superior oblique muscle or its tendon.
In true Brown syndrome, there’s a constant issue with the eye moving upwards. The medical reason for this is the superior oblique tendon getting stuck as it tries to move through the trochlea, a loop in the eye that acts like a pulley. The tendon may be stiff or abnormally routed.
Symptoms of Brown syndrome involve difficulty moving the eye upwards, a widening of the eye opening when trying to look up, and a unique tilt of the head by the patient to minimize double vision and improve their sight. These signs, often coupled with a clicking or snapping sensation when trying to look up, can be unique to Brown syndrome.
Doctors usually diagnose Brown syndrome by closely studying its symptoms, questioning patients thoroughly, and conducting comprehensive eye exams, including specific tests of eye movement. If the case is complicated or surgery is needed, imaging tests like MRI or CT scans might be used to get a closer look at the eye’s anatomy.
Treatment for Brown syndrome varies. It can be as simple as keeping an eye on the condition or doing exercises, or it may involve using prisms for mild cases. But in certain cases where there is a noticeable abnormal head posture, double vision, or cosmetic issues, surgery might be required. The goal of surgery is to adjust or extend the superior oblique tendon to remove the restriction.
Brown syndrome can impact a person’s vision and emotional health as the condition can be isolating and cause limitations particularly when the individual has to tilt their head significantly to see properly. The condition was first identified by Dr. Harold Brown in 1949, and it has since been acknowledged as a significant reason for vertical misalignment of the eyes, affecting both binocular vision and quality of life.
What Causes Brown Syndrome?
Brown syndrome is a condition that experts believe is caused by issues with the tendon-trochlear complex, specifically the superior oblique. There are two main types of Brown syndrome – one that is present from birth, or ‘congenital’, and one that is acquired later. Acquired Brown syndrome may result from inflammation, injury, or due to doctor interventions. But there might be several other potential causes as well.
Congenital Brown syndrome might occur due to various factors like a too tight or improperly lined up pulley system, a short superior oblique muscle and its tendon, a tight sheath on the superior oblique tendon, a lump on the superior tendon, abnormal nerve signals, or congenital fibrosis of the tendon.
On the other hand, acquired Brown syndrome may stem from whole-body diseases like systemic disease, dysthyroid disease, Hurler-Scheie syndrome, hypogammaglobulinemia, juvenile or regular rheumatoid arthritis, sinusitis, Sjogren syndrome, systemic lupus erythematosus, and others due to doctor interventions.
Furthermore, some eye-related conditions can lead to Brown syndrome, including eyelid surgery, glaucoma implants in the top part of the nose, fractures to the orbit’s floor, retinal encircling band, sinus surgery or sinusitis, superior oblique tuck, inflammation of the trochlea, cancer in the upper nose, and injuries above the nose or to nasal orbit.
In addition to these, other causes that can lead to this condition include Cyclic Brown syndrome, idiopathic (where the cause is unknown), an inflammatory condition called click syndrome, inflammation of the sheath surrounding the tendon of the superior oblique muscle, cardiopulmonary resuscitation, after childbirth, infection, and age-related deterioration.
Risk Factors and Frequency for Brown Syndrome
Back in the 1970s, Raynor and Hiatt estimated that Brown syndrome occurs in 1 out of every 450 cases of strabismus, a condition where the eyes do not properly align with each other. This rate increases when considering cases with issues related to vertical eye muscles. Urist and his team found Brown syndrome in 23 out of 226 patients who had weakness in their vertical eye muscles. All these patients had positive results on a test known as the forced duction test or traction test.
Various studies have shown that women are more likely to have Brown Syndrome, with 10% of cases being present in both eyes. In addition, it’s been found that Brown syndrome is sometimes associated with other conditions like Duane syndrome, crocodile tear syndrome (which causes abnormal tearing when eating), congenital ptosis (a drooping of the upper eyelid), and Marcus Gunn syndrome.
Signs and Symptoms of Brown Syndrome
: Brown syndrome is a condition affecting eye movement. Depending on the cause, certain symptoms and physical findings may be evident, guiding how it’s assessed and managed. The most common sign is a problem with moving the eye upwards when looking towards the nose. Other symptoms can include misalignment of the eyes, an abnormal head posture (tilting the head back and turning the face to the other side to avoid looking down), double vision, and sometimes pain and tenderness in the upper nose area. Some people may also notice a clicking sound or sensation when moving the affected eye. The severity of this condition can vary greatly, and treatment can range from simple monitoring to surgical intervention in more severe cases.
- Difficulty moving the eye upward when looking towards the nose
- Misalignment of the eyes
- Abnormal head posture (head tilted back and face turned to the other side)
- Double vision
- Potential pain and tenderness in upper nose area
- Possible clicking sound or sensation when moving the affected eye
In milder cases, people may not have any symptoms and the condition may only be found during a routine eye examination. In severe cases, the disease can profoundly affect vision. To diagnose this condition, doctors typically use different techniques to observe how the eyes move. Imaging studies such as MRI or CT scans can provide a more detailed look at the structures of the eye in more complex cases.
Testing for Brown Syndrome
The diagnosis of Brown syndrome, a rare eye disorder, involves a detailed process which may sometimes require the use of specialized imaging. This process is crucial for confirming whether or not a person indeed has the disorder, understanding to what extent they’re affected, and deciding the best course of action for treatment. It can be difficult for typical eye doctors to detect Brown syndrome in children, and an expert in pediatric ophthalmology is generally necessary for a diagnosis.
The first step of the process is a thorough history taking followed by a detailed eye examination, which will test for any specific features of Brown syndrome. This may involve conducting a so-called ‘forced duction’ test, where the eye’s ability to move in certain directions is checked. While this test can be uncomfortable, it can be done either with local anesthesia or in an operating room. If the eye’s movement is restricted in certain ways, this is considered a positive test outcome and suggests the presence of the syndrome.
Further tests might be required depending on the cause of the Brown syndrome, such as a stricter examination using X-rays if the syndrome was caused by eye trauma. For cases possibly connected to inflammation, other medical specialists may need to be involved in the diagnosis process.
A range of factors is considered during the initial background check:
– Age of Onset: The doctor establishes if the condition has been present since birth or developed over time.
– Symptoms: Any issues such as trouble moving the eye, double vision or usual head postures are documented.
– Related Conditions: Questions regarding any past eye surgeries, trauma or other diseases like rheumatoid arthritis that could contribute to Brown syndrome might be asked.
Following this, a series of eye examinations are conducted:
– Visual Acuity Test: Each eye’s vision quality is evaluated to check for any blurring or issues.
– Binocular Function Tests: The cooperation of both eyes is evaluated, specifically looking for instances of double vision.
– Ocular Motility Assessment: The eye’s range of motion is checked. Inability to move the eye upwards while looking straight is a common sign of Brown syndrome.
– Forced Duction Test: This test checks if there is physical restriction to eye movement.
– Slit Lamp Examination: This test is conducted to examine the front part of the eye.
– Fundus Examination: This is done to check the back of the eye, particularly if there as suspicion of other eye conditions.
Additional Tests might include:
– Synoptophore Examination: This is done to check how the eyes work together and if any head tilting is used to compensate.
– V-Pattern Evaluation: Checks if the eyes turn outward or inward disproportionately when looking up or down. This V-pattern squint happens commonly in Brown syndrome.
More complex cases or those considering surgery may require MRI or CT scans to get a detailed view of the structures within the eye.
Treatment Options for Brown Syndrome
Brown syndrome is usually monitored and managed without surgery. This eye condition often improves on its own, especially if acquired later in life. The main goals of treatment are to improve eye coordination and restore normal vision. Non-surgical treatments can include wearing prism glasses to manage double vision, doing eye exercises, and treating any underlying conditions. For example, if inflammation is causing Brown syndrome, steroids may be given orally or as an injection. Eye exercises have improved this condition in some cases. For Brown syndrome that’s developed due to another condition like sinusitis or arthritis, treating the underlying condition is key. Steroids and other immunosuppressants have been successful in treating Brown syndrome that hasn’t responded to other treatments.
Surgery for Brown syndrome is typically considered if the condition causes noticeable eye alignment issues, or if a patient is uncomfortable with their appearance. One type of surgery involves cutting the tendon which pulls the eye upward, which has been more successful than removing the sheath around the tendon. However, if the entire tendon is cut, it could cause other problems like palsy. Some surgeons suggest combining this procedure with another surgery to reduce the risk of these issues. There’s also a method involving a silicone expander, which helps to lengthen the tendon in a controlled manner, still used today. If the silicone expander isn’t enough, adjustable stitches can be used during surgery to further tweak the tendon length. After the surgery, eye exercises are recommended. Depending on the severity of Brown syndrome, other procedures involving reconstructing the trochlea (a part of the eye) and removing adhesions may be considered.
What else can Brown Syndrome be?
Understanding the right condition is vital for curative steps. Let’s look at some
vital and often-confused situations that need to be thought through when
diagnosing Brown Syndrome:
- Weakness or paralysis of the inferior oblique muscle, this doesn’t
occur often. - Orbital fractures can make eye muscles get stuck and limit their lifting motion.
Knowing the symptoms of a fractured orbit – like severe restriction of lifting in both eye abduction and adduction, history of injury, numbness or “pins and needles” under the eyes, or a sinking of the eye into the socket – can help differentiate it from Brown Syndrome. - Superior oblique muscle working too much (with or without inferior oblique muscle weakness) shows no restraint in forced lifting test and an “A” pattern when looking up. This differs from Brown Syndrome.
Inborn fibrosis syndromes can influence multiple eye muscles. The
muscle that lifts the eyelid can also be impacted, resulting in a drooping eyelid. This, along with
deficiencies in lifting both during abduction and adduction, can help differentiate
from Brown Syndrome. Double elevator palsy is when the lifting deficiency in abduction is equal to or larger than in
adduction. Forced lifting tests can be positive and are made clear by the protrusion of the eyeball. Among health disorders, Congenital cranial
dysinnervation disorders occur when cranial nerves and the muscles they manage have abnormal development. One example is Duane Retraction
Syndrome which can show as restricted eye movement.
Myasthenia gravis is an autoimmune condition that can cause variable weakness of
the extraocular muscles, leading to symptoms that could be mistaken for Brown
Syndrome. However, the changeability and fatigability of symptoms are key distinguishing features. Strabismus fixus is a severe form of irregular eye alignment
with a marked restriction of movement in one or more directions, potentially
resembling Brown syndrome. Changes after surgery
especially after strabismus surgery or surgeries involving the orbit, can
sometimes lead to restricted ocular motility, similar to Brown syndrome. Orbital tumors or inflammation in the orbit can restrict eye movement due to mechanical obstruction or muscle involvement.
Stiff Eye Syndrome is a rare condition characterized by a generalized
restriction in eye movements due to hardening of extraocular muscles.
Diagnosing the condition, a detailed clinical history, comprehensive eye examination, and, in some cases, imaging studies are essential to differentiate
Brown syndrome from these conditions. The specific features of eye movement limitation, the presence or absence of pain, systemic signs and symptoms, and the result of diagnostic tests like the forced lifting test can help arrive at
the correct diagnosis. Working together with neurologists, rheumatologists, or
other specialists may also be required in complex cases.
What to expect with Brown Syndrome
Many cases of congenital Brown syndrome, a condition present since birth, naturally get better with time without the need for treatment. As kids get older, the need to look upward decreases unless they end up being shorter than average in height.
Acquired Brown syndrome, which develops later in life, also usually has a good outcome. Symptoms often improve when the root cause is treated correctly. Some children with Brown syndrome may not need any specific treatment, as they may outgrow it as they get older. Even when surgery is required, usually it doesn’t result in long-term damage to vision or eye health. By managing it well, either through non-invasive methods or surgery, eye alignment can be corrected, symptoms can be relieved, and a majority of the kids with Brown syndrome can develop good vision and depth perception in both eyes.
Surgery might be necessary in cases where the eyes are significantly misaligned or when there is a severe restriction in looking upward. The goal of the surgery is to loosen the superior oblique muscle to improve eye functioning. However, this procedure tends to be unpredictable, and additional surgeries might be required in some cases.
Brown syndrome caused by inflammation may present with pain around the affected eye region. Pain might fluctuate with episodes of inflammation and tenderness when the area is touched. Steroid injections around the eye area can be beneficial to reduce inflammation.
Brown syndrome caused by trauma has been decreasing in countries with strict road safety rules. Surgical treatment for this type might require additional procedures to correct the eye muscles to improve binocular vision, which is the ability to maintain visual focus on an object with both eyes.
Possible Complications When Diagnosed with Brown Syndrome
Complications that could occur during eye surgery may include excessive bleeding, muscle loss, perforation of the globe (or tearing of the eye), and harm to nearby eye structures. After your surgery, you may experience issues like scarring, being over or under-corrected, superior oblique palsy (an eye muscle disorder), the need for more surgeries, and implant extrusion (or the implant coming out of place), particularly when a silicone tendon expander is used.
Complications during and after surgery :
- Excessive bleeding
- Lost muscle
- Perforation of the globe
- Damage to surrounding ocular structures
- Scarring
- Under and over-correction
- Superior oblique palsy
- Need for further surgery
- Implant extrusion (in cases of a silicone tendon expander)
Recovery from Brown Syndrome
After surgery for Brown syndrome, good care and rehabilitation play a significant role in a successful recovery, especially after invasive procedures. Here’s a clear guide to care following surgery:
After the operation, managing any pain is crucial. This may involve medications and advice on resting the eye. Patients are also educated on how to spot signs of infection, and antibiotic eye drops may be given if necessary. Protecting the eye from further harm and reducing strain while it heals are also essential. Regular check-ups are scheduled to keep an eye on the healing process and spot any complications early, such as double vision or strabismus, which might need extra treatment.
During rehab, visual therapy and specific eye exercises can be beneficial to improve eye coordination and movement after surgery. Regular checks on the individual’s visual sharpness can help catch any potential vision loss or development of “lazy eye”, particularly in children. Patients are given advice on how to adjust their daily activities during their recovery and offered emotional support and counseling, as dealing with visual impairment can be tough emotionally.
Treatment might include working with rehabilitation specialists, such as an eye movement expert or occupational therapist, if the patient is struggling with daily tasks because of vision changes. Jointly coming up with a recovery plan and emphasizing the importance of sticking to the rehab protocol are key for the best outcome. In some cases, devices to help with tasks that require good vision, like reading, could be helpful.
Lasting management strategies could include changes to the individual’s lifestyle if there are lasting limitations in eye movement. Regular eye check-ups are key for tracking any changes or long-term effects of the surgery. And connecting patients with community resources and support groups, who can provide extra help and understanding, might also be beneficial.
Optimal recovery from Brown syndrome surgery requires a full-scale approach that combines medical management with rehab strategies. A cross-disciplinary team, consisting of surgeons, general doctors, nurses, therapists, and counselors, can offer a strong network that addresses all sides of the patient’s health and emotional wellbeing.
Preventing Brown Syndrome
Teaching both the patient and their family about the condition, different ways to treat it, and why it’s crucial to keep up with appointments is very important. For children, it’s vital to let parents know that the condition can naturally get better over time and that consistent check-ups are necessary. If the patient isn’t bothered by how they have to position their head to see properly and they aren’t seeing double, simple management is usually sufficient.
In the case of acquired Brown syndrome, treating the root cause can alleviate symptoms. If surgery is required, the patient must fully understand the risks, benefits and possibility of symptoms getting worse before going ahead. It’s also helpful to give the patient relevant educational materials and information on support groups, which can greatly help their general wellbeing and recovery process.
Avoiding the condition getting worse and patient education are critical parts in managing Brown syndrome. Part of these preventive measures include regular eye exams to monitor the condition and catch any changes early. Additionally, preventing any injuries to the head or eye that could worsen the condition is important. Managing inflammation through medication or other medical advice can aid in preventing complications.
Educating patients about Brown syndrome – what it is, how it affects the eyes, and what could potentially cause it – is important for overall care. This education should also cover the different treatment options available, such as observation, physical therapy, and possible surgery. Recognizing worsening symptoms, like increasing difficulty moving the eyes or pain, should be emphasized as a reason to immediately seek medical help. Patients can improve their quality of life by practicing self-care techniques, like exercises they can do at home to maintain eye movement and comfort.
Patients should also understand how the condition affects their lifestyle. Strategies should be offered to help them manage these challenges, such as specific head postures to help with vision. Support services should be offered, including counseling or therapy if needed, to cope with the emotional and psychological aspects of living with this chronic condition. The family of the patient should be educated on the possibility of inheriting the syndrome as well, and the importance of genetic counseling should be explained. Visual aids can help patients and their families understand the eye’s anatomy and how Brown syndrome impacts it. This understanding can demystify the condition and empower patients with knowledge.
When providing education and prevention strategies, the information should be tailored to the patient’s age and level of understanding. For children, the information should be presented in an easy and engaging way. Adults often benefit from detailed explanations and written materials. Regular follow-up appointments are essential to assess the patient’s understanding and their compliance with management strategies.
