Brown syndrome

From EyeWiki


Disease Entity

Strabismus/ocular misalignment

Disease

Brown syndrome is a vertical strabismus syndrome characterized by limited elevation of the eye in an adducted position, most often secondary to mechanical restriction of the superior oblique tendon/trochlea complex.

Previously referred to as "superior oblique tendon sheath syndrome," it was considered a dysgenesis of the superior oblique muscle's tendon sheath. More recently, it is thought that the problem is not the sheath, but rather the tendon itself, that undergoes increased tension.

Brown syndrome can be congenital or acquired, is unilateral in 90% of patients, and has a slight predilection for females. There is thought to be a genetic predisposition to congenital Brown syndrome, however, most cases are sporadic in nature.

Etiology

Fundamentally, Brown syndrome results from a limitation of the normal function of the superior oblique tendon-trochlea complex. It most often occurs as a congenital condition. It has been proposed that congenital Brown syndrome is due to a dysgenesis of the muscle tendon or trochlea, and recent work suggests that some cases may be associated with congenital cranial dysinnervation disorders.

There is a large differential for secondary causes of Brown syndrome, including inflammation, trauma, tendon cysts, previous sinusitis, or other iatrogenic causes such as orbital or strabismus surgery. Abnormalities of the fascial anatomy is considered to be a rare cause. For uncertain reasons, Brown syndrome is more commonly found in the right eye than the left eye.

Diagnosis

The diagnosis of Brown Syndrome is based on the clinical findings and history. Workup may be performed if there is concern for an underlying inflammatory etiology.

Physical examination

A complete ophthalmic examination should be performed. Special focus should be given to the sensory-motor examination, including strabismus measurements in all cardinal positions of gaze, ocular motility, and binocular function/stereopsis. In cases of acquired Brown syndrome, a thorough orbital examination should be performed with special attention to the trochlear area.

Signs

The key finding in Brown syndrome is limited elevation in adduction. There is typically minimal or no vertical deviation in primary position, however, moderate cases will have a downshoot in adduction and severe cases may demonstrate a hypotropia in primary position. A compensatory abnormal head position may be present, often patients adopt a chin up position or a head turn away from the affected eye (to keep the affected eye abducted).

Forced duction testing is very useful in the diagnosis of Brown syndrome, and will demonstrate restriction to elevation in adduction.

Amblyopia is generally absent. A down movement of the eye on adduction may mimic superior oblique over-action, however, a V-pattern will be noted in Brown syndrome. Saccadic eye movements should remain unaffected.

A Tendon cyst or a mass may be palpable in the superonasal orbital. Brown Syndrome secondary to an inflammatory condition is frequently associated with orbital pain and tenderness to palpation of the trochlea.

Image added in courtesy of Dr Agathi Kouri, MD, FRCS, Panagiotis and Aglaia Kiriakou Children's Hospital, Athens, Greece

Symptoms

Patients with Brown syndrome may have a variety of symptoms which may be constant, intermittent, or recurring, including:

  • Diplopia
  • Poor binocular vision/stereopsis
  • Orbital pain and tenderness
  • Pain with eye movement
  • Abnormal head position
  • A ‘click’ may be heard or felt by the patient with movement of the eye when attempting to elevate the eye in AD-duction

Differential diagnosis

Brown syndrome should be differentiated from the following conditions:

  • Inferior oblique muscle palsy
  • Double elevator palsy
  • Congenital fibrosis of extraocular muscle
  • Thyroid eye disease
  • Orbital fracture with entrapment
  • Myasthenia gravis

Management

Management of Brown syndrome depends on symptomatology, etiology, and the course of the disease.

Observation is often preferred, as symptoms are often intermittent in nature and do not cause permanent damage. Improvement of congenital Brown syndrome has been described in up to 75% of cases. Of note, as patients are most symptomatic on upgaze, normal growth can decrease symptoms as patients grow taller and have less necessity for upgaze position. Acquired Brown syndrome cases may also undergo spontaneous resolution, and thus early surgical intervention is not recommended.

Brown syndrome due to inflammatory disease with associated pain may transiently benefit from injection of steroids to the trochlear area. This may require recurrent treatments for symptomatic relief.

Systemic steroids and non-steroidal anti-inflammatory agents have also been utilized with variable success.

Surgery

Surgery can be considered in the following circumstances:

  • Hypotropia in primary position
  • Significant abnormal head position
  • Significant diplopia
  • Significant downshoot on adduction
  • Compromised binocularity
  • Significant orbital pain or pain with eye movements

The following surgical procedures could be used:

  • A tenotomy or tenectomy to weaken the superior oblique
  • The superior oblique expansion surgery has been found to have high success rates and can be performed through a variety of techniques, including a silicon expander (e.g. a #240 retinal silicone band), a non-absorbable "Chicken suture", or a superior oblique split tendon lengthening procedure
  • Iatrogenic Brown syndrome secondary to muscle plication may require reversal of the plication
  • In case the primary cause is a muscle cyst, removal of the cyst may be indicated


References

  1. Kushner, Burton J. “Vertical Strabismus.” Taylor & Hoyt's Pediatric Ophthalmology and Strabismus, by Scott R. Lambert and Christopher J. Lyons, Elsevier, 2017, pp. 828–837.
  2. Wright KW, Brown's syndrome: diagnosis and management, Trans Am Ophthalmol Soc. 1999;97:1023-109.
  3. Alexandros Damanakis, Stabismoi 2nd edition, Litsas medical editions, Athens-Greece.
  4. American Academy of Ophthalmology. Pediatric Ophthalmology and Strabismus BCSC, Leo, 2011-2012.
  5. Jack J. Kanski- Brad Bowling, Clinical Ophthalmology- A systematic approach, Seventh Edition, Elsevier, 2011.
  6. JS Crawford, Surgical treatment of true Brown's syndrome, American journal of ophthalmology, 1976
  7. Manley, DR and Rizwan, AA. 2011. Brown Syndrome. Curr Opin Ophthalmol, 22: 432-440.
  8. Stager DR Jr, Parks MM, Stager DR Sr, Pesheva M. Long-term results of silicone expander for moderate and severe Brown syndrome (Brown syndrome "plus"). JAAPOS 1999 Dec;3(6):328-32.
  9. Coussens T, Ellis FJ. Considerations on the etiology of congenital Brown syndrome. Curr Opin Ophthalmol. 2015 Jul;26(5):357-61.