Brown Syndrome: Symptoms, Types, Causes and Treatment

Brown Syndrome is a complex eye movement disorder that challenges both patients and clinicians. Characterized by difficulty elevating the eye in certain directions, it can disrupt daily activities and impact quality of life. Understanding its symptoms, types, causes, and management options is crucial for those affected and their caregivers. This article provides a comprehensive overview, synthesizing current research and clinical insights for an accessible, in-depth guide.

Symptoms of Brown Syndrome

Brown Syndrome's symptoms can be subtle or striking, often leading to confusion with other eye movement disorders. Recognizing its hallmark features is the first step toward accurate diagnosis and effective management.

Symptom	Description	Additional Features	Source(s)
Limited Elevation	Eye cannot move upward when turned inward (adduction)	More pronounced in adduction	1 2 3 11
Downshoot	Affected eye moves downward in adduction	May be accompanied by lid widening	1
Head Posture	Abnormal head tilt or turn to compensate	Reduces double vision (diplopia)	1 2
Hypotropia	Eye appears lower than the fellow eye	Especially in primary gaze in severe cases	1 2 3

Table 1: Key Symptoms

Limited Elevation in Adduction

The defining symptom of Brown Syndrome is a restriction in elevating the affected eye when it is turned toward the nose (adduction). This limitation is resistant to both voluntary effort and passive movement, distinguishing it from other forms of strabismus like inferior oblique palsy 1 2 3.

• Most noticeable when looking up and inward
• May be mistaken for nerve palsy but does not improve with forced duction testing

Downshoot and Lid Widening

A characteristic “downshoot” may occur—when the affected eye moves downward instead of upward during adduction. This can be accompanied by a visible widening of the eyelid (palpebral fissure) on the same side 1. These features can help clinicians distinguish Brown Syndrome from other ocular motility disorders.

Abnormal Head Position

To avoid double vision or compensate for the eye movement limitation, individuals may adopt an abnormal head posture, such as tilting or turning the head 1 2. This adaptation helps align the eyes in a way that minimizes symptoms.

Hypotropia and Sensory Fusion Loss

In more severe cases, the affected eye may rest lower than the other eye (hypotropia), even when looking straight ahead (primary position). Some individuals may also lose the natural ability to fuse images from both eyes, leading to double vision 1 2 3.

Types of Brown Syndrome

Brown Syndrome does not present the same way in every patient. Understanding its different forms helps guide diagnosis and management.

Type	Onset	Course	Notable Traits	Source(s)
Congenital	Present at birth	Often stable or improves	May be associated with other cranial disorders	2 3 5 9
Acquired	Develops later	May resolve or persist	Often linked to trauma, inflammation, or disease	2 3 4 6 8 9 10 11
Intermittent	Variable	Symptoms fluctuate	Elevation restriction not always present	3
Constant	Persistent	Ongoing symptoms	Consistent motility limitation	3

Table 2: Brown Syndrome Types

Congenital Brown Syndrome

Present from birth, congenital Brown Syndrome is the more common form. It is usually stable but can spontaneously resolve in some cases 2 3 5 9. There is growing recognition that congenital Brown Syndrome may be linked to broader cranial nerve development disorders, such as congenital cranial dysinnervation disorders (CCDDs) 5.

• Often discovered in early childhood
• May be associated with other eye movement disorders (e.g., Duane syndrome, ptosis)

Acquired Brown Syndrome

Acquired Brown Syndrome develops later in life and can have a variety of causes, including trauma, inflammation, or systemic diseases 2 3 4 6 8 9 10 11. Acquired forms are more likely to resolve on their own or with medical treatment, especially if inflammation is the underlying cause 2 3 8 9 10.

• Can occur at any age
• May be transient or persistent
• Sometimes associated with pain or discomfort around the trochlea (the pulley-like structure above the eye)

Intermittent and Constant Forms

Brown Syndrome may be constant—with symptoms always present—or intermittent, where symptoms fluctuate over time 3. Intermittency can complicate diagnosis but may offer hope for spontaneous improvement.

Causes of Brown Syndrome

The underlying cause of Brown Syndrome is a restriction or abnormality in the movement of the superior oblique tendon as it passes through the trochlea. The reasons for this vary depending on the type of syndrome.

Cause Type	Mechanism	Examples/Notes	Source(s)
Mechanical	Physical restriction of tendon	Tight tendon, fibrosis, scarring	1 2 3 6 7
Developmental	Abnormal nerve or muscle development	Trochlear nerve issues, CCDDs	5
Inflammatory	Swelling or inflammation of trochlea	Trochleitis, autoimmune diseases	6 8 10 11
Traumatic	Injury to orbital area	Fractures, surgical complications	2 4 6 9 11
Neoplastic	Space-occupying lesion	Orbital tumors or metastases	4
Iatrogenic	Result of medical intervention	Surgery-related damage	11

Table 3: Causes of Brown Syndrome

Mechanical Restriction

Most commonly, Brown Syndrome is caused by a mechanical problem that prevents the superior oblique tendon from gliding smoothly through the trochlea. This may be due to a congenitally tight tendon, scar tissue, or fibrotic bands 1 2 3 6 7.

• Can be congenital (present at birth) or result from injury or surgery
• Tissue surrounding the tendon is now understood to play less of a role than the tendon-trochlea interface itself 3

Developmental Anomalies

Congenital forms may result from abnormal development of the trochlear nerve or superior oblique muscle. These anomalies are sometimes part of a broader group of conditions known as congenital cranial dysinnervation disorders (CCDDs), which affect the nerves controlling eye movement 5.

Inflammatory Causes

Inflammation in the area of the trochlea (trochleitis) can lead to swelling and mechanical restriction of the tendon 6 8 10 11. This is especially relevant in acquired Brown Syndrome and may be linked to systemic inflammatory or autoimmune diseases.

• Responds well to medical treatment in many cases
• Inflammation can be identified with imaging techniques such as MRI 11

Traumatic and Iatrogenic Factors

Trauma, such as fractures or surgical complications, can damage the superior oblique tendon or its pulley system, leading to acquired Brown Syndrome 2 4 6 9 11. Iatrogenic cases arise as unintended consequences of medical procedures, particularly orbital or sinus surgery 11.

Neoplastic Causes

Rarely, tumors or metastatic lesions in the orbit can mechanically interfere with tendon movement, resulting in Brown Syndrome 4.

Treatment of Brown Syndrome

Treatment strategies for Brown Syndrome are tailored to the underlying cause, severity of symptoms, and the likelihood of spontaneous improvement. The approach ranges from conservative observation to targeted medical or surgical interventions.

Treatment Type	Approach/Method	Effectiveness/Indications	Source(s)
Conservative	Observation, monitoring	High rate of spontaneous resolution, especially congenital	2 3 9 11
Medical	Steroid injections, NSAIDs	Effective for inflammatory cases	8 9 10 11
Optical	Prism glasses	Alleviates diplopia in some cases	10
Surgical	Tendon lengthening, tenotomy, silicone expander	Reserved for severe, persistent cases or failed conservative therapy	2 7 9 11

Table 4: Treatment Options

Conservative Management

For many patients, especially those with congenital or mild acquired Brown Syndrome, watchful waiting is recommended. Symptoms often improve or resolve spontaneously over time 2 3 9 11.

• Up to 75% of congenital cases managed conservatively experience improvement or resolution 9
• Regular follow-up is important to monitor for changes or complications

Medical Therapy

Inflammatory acquired Brown Syndrome responds well to medical treatments:

• Steroid Injections: Local injection of corticosteroids into the trochlear area can rapidly reduce inflammation and restore movement 8 10. Studies show high rates of complete or significant remission, particularly when treatment is initiated early 8.
• Oral NSAIDs: Non-steroidal anti-inflammatory drugs may be beneficial in milder or less localized cases 9 11.

Medical therapy is typically the first-line approach for inflammatory cases before considering surgery.

Optical Correction

In some instances, prism glasses can help align images and relieve double vision, especially in children or those not fully responsive to medical or surgical treatments 10.

Surgical Management

Surgery is considered only when conservative and medical therapies fail, or when symptoms significantly impact quality of life. Several surgical techniques have been developed:

• Silicone Tendon Expander: A procedure that lengthens the superior oblique tendon using a silicone spacer. It has shown high success rates and durability in long-term follow-up 2.
• Tenotomy or Tenectomy: Cutting or removing part of the superior oblique tendon to relieve restriction. This approach is effective but can risk overcorrection, leading to other eye movement problems 7.
• Z Tenotomy or Split Tendon Lengthening: Alternative surgical techniques aimed at adjusting tendon length or tension 7.

Surgical outcomes are generally favorable when performed for the right indications, though complications such as scarring or implant extrusion can occur 2 7 11.

Conclusion

Brown Syndrome is a multifaceted ocular motility disorder with distinct diagnostic and management challenges. Key points to remember include:

• Symptoms: Limitation of elevation in adduction, downshoot, lid widening, abnormal head posture, and occasionally hypotropia are characteristic features.
• Types: The syndrome can be congenital or acquired, constant or intermittent, with spontaneous improvement possible in many cases.
• Causes: Mechanical restriction, developmental anomalies, inflammation, trauma, and rarely tumors are among the diverse causes.
• Treatment: Conservative management is effective for most, with medical and surgical options reserved for persistent, symptomatic, or inflammatory cases.

By understanding the nuances of Brown Syndrome, patients and clinicians can better navigate diagnosis, treatment, and long-term management—leading to improved outcomes and quality of life.

Main Points Summary:

• Brown Syndrome limits upward eye movement in adduction and may cause abnormal head posture and hypotropia.
• It presents in congenital and acquired forms, with intermittent or constant symptoms.
• Causes range from tendon restriction and developmental anomalies to inflammation, trauma, and tumors.
• Most cases improve with observation or medical therapy; surgery is reserved for refractory or severe cases.