Brown's Syndrome

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Ghostdogg Productions Presents...

• Brown's Syndrome

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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

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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

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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

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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.

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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.
• ??

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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.

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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 the
  eye getting stuck by a tight superior oblique
  muscle.

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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.

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Variations of Brown's Syndrome
Congenital Right Brown's Syndrome in a 6-year-old
girl
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Variations of Brown's Syndrome
Acquired Left Brown's syndrome in a 16-year-old
girl
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Variations of Brown's Syndrome
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.
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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.
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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

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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 32 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.

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Double Elevator Palsy
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.
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Double Elevator Palsy
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.
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Fourth Nerve Palsy
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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

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True Inferior oblique paresis

• 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.

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True Inferior oblique paresis

• 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.

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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.

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Complications of Surgery

• 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.

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Complications of Surgery

• 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.

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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.

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References

• Wilson ME, Eustis HS, Jr, Parks MM. Brown's
  syndrome. Surv Ophthalmol. 1989
  Nov-Dec34(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/disorder
  s-of-eye-movements/iv-neuropathies-and-nuclear-pal
  sies/iii-browns-syndrome
• http//www.cybersight.org/bins/content_page.asp?ci
  d1-3

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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.
  971023-109
• 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.