Brown's syndrome ghostdogg productions(1)

Ghostdogg Productions
Presents...

Brown's Syndrome

Dedicated to...

Dr. M. Edward Wilson, MD.

Your interest in this subject of Binocular vision & Strabismus is what has kept me interested and enjoying doing what I do...

A. J. Hamilton

History

1928, German ophthalmologist P. A. Jaensch is presented with a child who could not elevate the affected eye in adduction. The case was presented in a medical journal the following year, initially under the disease name Superior oblique tendon sheath syndrome

1950, American Ophthalmologist Harold. W. Brown described a young patient with similar symptoms of those outlined by Jaensch. He labeled the disease Brown's superior oblique tendon sheath syndrome or simply Brown's Syndrome

General characteristics

Limitation or absence of elevation of the affected eye

Limitation of elevation in direct upgaze

Near normal to normal elevation in abduction

A compensatory abnormal head posture to obtain fusion in PPM

Grading of Severity

Mild Restricted elevation in adduction only with no hypotropia or downshoot in primary or adduction

Moderate restricted elevation and downshoot in adduction and direct elevation with minimal hypotropia in primary position and adduction

Severe restriction of elevation and marked downshoot in adduction and direct elevation. Evident hypotropia in primary position with, but not in all cases, adoption of a abnormal head posture.

Abnormal head postures -
Head tilt

This child adopts a head tilt away from the affected eye to compensate for a hypotropia of the right eye.

Abnormal head postures -
Chin-up head tilt

This child has adopted a chin-up head posture to compensate for a hypotropia of the left eye.

Common features of Browns -
Downshoot on elevation and adduction

In moderate to severe forms of Browns, a downshoot of the affected eye can be seen in elevation and adduction. This is caused by a the eye getting stuck by a tight superior oblique muscle.

Common features of Brown's Syndrome -
Widening of the Palpebral lid fissure

Widening of the palpebral lid fissure associated with downshoot of the affected eye which increases in direct elevation giving the falling eye effect. Note this child has tilted her head back in order to elevate her eyes in adduction and direct upgaze.

Variations of Brown's Syndrome

Congenital Right Brown's Syndrome in a 6-year-old girl


Acquired Left Brown's syndrome in a 16-year-old girl


Bilateral Brown's Syndrome in a 7-year-old girl. Note the substantial chin-up head posture to compensate for the severe downshoot of either eye in both adduction and abduction. Also note widening of the palpebral fissure on elevation.

Other Variations of Brown's Syndrome

Canine Tooth Syndrome. First described by Phillip Knapp, this varient of Browns occurs after trauma, particularly to the region of the Superior oblique tendon and trochlea. In most cases, this form is often diagnosed as either a class VII Superior oblique palsy or Iatrogenic Browns syndrome.

Differential diagnosis of Brown's Syndrome

Other forms of paretic or restrictive strabismus have been diagnosed as potential Brown's. These include:

Double Elevator Palsy

Fourth Nerve palsy

Iatrogenic Superior oblique overaction, and

A True Inferior Oblique paresis

Interesting Facts of Brown Syndrome

90% of patients with Browns have unilateral, 10% are bilateral.

The predominance of this syndrome, similarly to Duane's Syndrome occurs 3:2 girls to boys.

Also similar to Duane's, the Right eye is more often affected than the left.

Generally, over 85% of Browns cases can be treated without surgery...given that good binocular vision is maintained and there is no abnormal head posture.


Typically known as Monocular elevation deficiency, this deficit occurs primarily in adduction and abduction, and can mimic Browns in the fact that there is a pronounced limitation of elevation in the paretic eye, as is the case in this child. A difference of this is that in primary gaze, patients often have a ptosis of the eye, and may adopt a chin-up head posture to compensate for the ptosis.


Another example of a patient with Double elevator palsy. This boy clearly demonstrates an elevation deficiency seen at its worst in abduction, but also in adduction. Also he adopts an evident chin-up head posture to compensate for a primary position hypotropia.

Fourth Nerve Palsy

True Inferior oblique paresis

Patients with a True inferior oblique paresis generally present with the following symptoms, which differentiate it from Browns:

A limitation of elevation in adduction, with a large vertical deviation in primary position, usually more than 10 PD.

A marked superior oblique overaction

An evident A-Pattern convergence, noticeable in direct upgaze

A positive Bielschowsky head tilt test

Negative forced ductions test


This 15-year old girl has a Inferior oblique paresis of the right eye. Primary position shows an evident left hypertropia. On diagnostic versions she shows an A pattern convergence, marked overaction of her right superior oblique, and hypotropia on left gaze.


Positive Bielschowsky's Head tilt test. On tilting her head to her left shoulder, there is an evident increase of the right hypertropia. This imbalance is rectified upon tilting her head to the opposite side.

Complications of Surgery

Very often, complications can arise following surgery of Browns. This 10-year old girl has an evident Browns syndrome of the Right eye. Limitation of elevation in adduction is evident even in forced head posture.


At three days post surgery following a right superior oblique tenectomy, the right Browns is still present, while care was taken to avoid disturbance of the intermuscular septum. Four weeks postoperatively the limitation is still present, though now greatly improved.


At six months post surgery, the child's limitation of elevation and adduction has been eliminated as was the child's hypotropia and abnormal head posture. Given the characteristic nature of Browns, this helps to differentiate an undercorrection from a missed tendon.

In Conclusion...

To date, Browns stands as one of the more prevalent forms of restrictive Strabismus.

More commonly seen in childhood, however still can be seen in adulthood, either acquired or untreated from childhood.

Can be Familial

Can and should be observed by parents if children are assuming a chin-up head posture for fusion.

References

Wilson ME, Eustis HS, Jr, Parks MM. Brown's syndrome. Surv Ophthalmol. 1989 Nov-Dec;34(3):153172

Clinical Strabismus management: Principles and Surgical Management, 1999 Arthur L. Rosenbaum, Alvina Pauline Santiago, David Hunter, W.B. Saunders Company

Colour Atlas of Strabismus Surgery: Strategies and Techniques, 2014

Kenneth W. Wright, Yi Ning J. Strube, Springer Press

Optometry: Science, Techniques, and Clinical Management , 2009

Mark Rosenfield, Nicola Logan, Keith, H. Edwards, Elsevier Health Sciences

Postgraduate Ophthalmology, Volume 2, 2012,

Zia Chaudhuri, Murugesan Vanathi, Jaypee Highlights Medical Publishers Inc.

Strabismus Surgery: Basic and Advanced Strategies, 2004

David A. Plager, Edward G. Buckley, Oxford University Press

Binocular Vision & Ocular Motility, 2002

Gunter K. Von Noorden, Mosby

Pediatric Clinical Ophthalmology: A Colour Handbook, 2012

Scott Olitsky, Leonard B. Nelson, CRC Press

http://www.neuroophthalmology.ca/textbook/disorders-of-eye-movements/iv-neuropathies-and-nuclear-palsies/iii-browns-syndrome

http://www.cybersight.org/bins/content_page.asp?cid=1-3

References

Pediatric Ophthalmology and Strabismus, Expert Consultant, Online & Print, volume 4; 2012, Creig Simmons Hoyt, David Taylor, Elsevier Health Sciences

Wright KW. Brown's syndrome: diagnosis and management. Trans Am Ophthalmol Soc. 1999. 97:1023-109

Parks MM, Brown M. Superior oblique tendon sheath syndrome of Brown. Am J Ophthalmol. 1975 Jan. 79(1):82-6

Clarke WN, Noel LP. Brown's syndrome with contralateral inferior oblique overaction: a possible mechanism. Can J Ophthalmol. 1993 Aug. 28(5):213-6.

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