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Ghostdogg Productions Presents Browns Syndrome

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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
Outline

History of the disease

Incidence

Common signs & symptoms

Grading of Severity

Variations of Brown's Syndrome

Interesting factors of Brown's Syndrome

Differential Diagnosis

Management & Treatment of Brown's Syndrome

Complications of Surgery

Summary & References
History

In 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”
–
In 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”. Brown initially found that the
syndrome was caused by a shortening of the tendon
sheath, hence the full name of “Brown's superior tendon
sheath syndrome”. Since then, different etiologies have
been discovered, owning to the syndrome now being
simply known as “Brown's syndrome”.
INCIDENCE

Incidence is low – 6 in 2583 (Croswell & Haldi )

Familial occurrence is rare, but is documented

2%(Birgit Lorenz, Michael C. Brodsky)

Usually unilateral but 10% is bilateral
General characteristics

Limitation or absence of elevation of the affected eye

Limitation of elevation in direct upgaze

Near normal elevation in abduction with little to no overaction
of the superior oblique

An abnormal head posture to compensate for enophtalmus
Causes of Brown's Syndrome
Abnormal head postures Face turn
This child adopts a
face turn 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 Brown's, a “downshoot” of the affected eye can
often be seen in elevation and adduction and direct adduction. This is caused
either by the eye getting “stuck” by a tight superior oblique muscle, or
inflammation of that muscle, or by trauma to the 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 carries over in direct elevation due to
the eye “becoming stuck” by a tight or inflammed superior
oblique muscle. Note also that this child has tilted her head back
in order to elevate her eyes in adduction and direct upgaze.
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.
'Mild' Brown's syndrome
This young boy presents with a mild left Browns syndrome. Note
the downshoot of his affected left eye is only evident on elevation in
adduction and slightly in direct upgaze. He doesn't adopt an
abnormal head posture in primary position, although his mother's
hand is there to keep his head still and maintain his focus.
'Moderate' Brown's Syndrome
This young girl presents with a moderate left Brown's syndrome.On
presentation she adopts a chin-up head tilt to fuse in primary gaze.
Visible upon direct adduction and on elevation in adduction is an
evident downshoot of her affected eye. On direct elevation there is an
evident limitation of elevation in her left eye. There is also a slight
hypertropia of her right eye in downgaze right position.
'Severe' Brown's Syndrome
This teenage boy presents with a severe left Brown's syndrome. He adopts a slight chin-up
head posture to compensate for a left hypotropia in primary position. Evident is the
hallmark downshoot of his left eye in direct adduction, elevation in adduction, and direct
elevation. Also noticeable is the slight raising of his chin further in upgaze in an attempt to
bring his affected left eye up to the same level as his unaffected eye. Unlike in moderate
Brown's, there is no hypotropia upon downgaze.
Variations of Brown's Syndrome
Variations of Brown's Syndrome
To date, there are Three major groups of Brown's:
1. ‘congenital’/ childhood
2. 'acquired' / adult, and
3. an overlap of congenital / acquired
associated with 'A' pattern syndromes
and inferior obl. paresis (if that exists)
'Congenital' Brown's Syndrome
This 6-year old girl has a CONGENITAL RIGHT BROWN'S SYNDROME. Note the inability to elevate her eye in
adduction (C) in direct upgaze (B), and near normal elevation in abduction(A). There is no deviation of the eyes in
right gaze (F), left gaze (D), or primary, although she does adopt a chin-up, head turn to the left (E). Depression in
both eyes is normal (G-I).
'Acquried' Brown's Syndrome
(A) This 7-year old boy developed an ACQUIRED LEFT BROWN'S SYNDROME, following a Valsalva maneuver
during a circumcision procedure under local anesthesia. The MRI study, that was held approximately 2 weeks
after the onset of the symptoms revealed enlargement of tendon-trochlea complex on the right side. The tendontrochlea complex showed some high signals on T1 weighted images, which was attributed to a possible
hemorrhage (B). The patient was treated conservatively and his symptoms resolved spontaneously.
'Acquired' Brown's Syndrome
Acquired Brown's Syndrome
Aside from being a congenital ocular abnormality, Brown's can also have numerous
acquired causes, including trauma, sinus infection, inflammatory diseases such as
rheumatoid arthritis, or systemic lupus erythematosus, as well as postoperative
complications. This 35-year-old woman initially presented with a 2-year history of
diplopia. On presentation she showed definite upward gaze impairment of her left eye,
especially in adduction and direct upgaze. Also visible was a mild ptosis of her left lid.
Axial STIR and postcontrast fat-saturated T1 imaging revealed a prominant
enhancement of the Superior Oblique tendon-trochlea complex as well as a nonspecific
inflammatory process of the SO tendon-trochlea complex.
Axial STIR (A) and postcontrast fat-saturated T1 (B) images; corneal STIR (C)
and postcontrast fat-saturated (D) images. There is a subtle increased STIR
signal and mild assymetric thickening in the anterior portion of the left Superior
oblique tendon. There is also prominant enhancement around the trochlea region
(B and D, indicated by the arrows).
Bilateral Brown's Syndrome

Bilateral Browns is a rare form of Brown's which occurs in about 5-10% of all
Browns cases. In this case, the features commonly associated with true
Browns occur in both eyes. In this 7-year-old girl, note that she adopts a
substantial chin-up head posture to compensate for a severe downshoot of
either eye in both adduction and abduction. Also note widening of the both
palpebral fissures on elevation in adduction and abduction. There is very little
elevation of either eye beyond midline gaze.
Traumatic Brown's Syndrome
First described by Phillip Knapp, this varient of Browns occurs following 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. Ths 57-year-old woman developed a right
Browns syndrome following a MVA causing a deep laceration in the area of the trochlea, resulting in
damage to the right SO tendon. Note her inability to elevate (C) or depress (G) the right eye in all
adducted positions.
Interesting Facts of Brown Syndrome

90% of patients with Browns have unilateral, 10% is bilateral.

Occurs in 10 to 24% of vertical muscle anomalies.



The predominance of this syndrome occurs 3:2 girls to boys,
similar to Duane's Syndrome .
Also similar to Duanes, the Right eye is more often affected
than the left.
Generally, over 85% of Browns cases can be treated without
surgery...given that good stereopsis is maintained and there is
no abnormal head posture.
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
“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.
Double elevator palsy
This 7-year-old boy presents with a definite chin-up head tilt upon presentation to
compensate for a left hypotropia and ptosis in primary position. Evident upon both
adduction and elevation in adduction is the depression of his left eye also in direct upgaze.
Downgazes were fairly normal in rotation of the eyes. This could be diagnosed as a left
Brown's syndrome, except for the visible right hypertropia seen behind a translucent
occluder on head tilt to his left shoulder, thus making this a Double elevator palsy.
Fourth Nerve Palsy
Fourth 'Trochlear' Nerve Palsy
This 9-year-old girl presents with a bilateral fourth nerve palsy. She adopts a chin-up
head tilt in order to fuse in primary position. Evident is the visible overaction of the
inferior obliques on both adduction and abduction, as well as a limitation of elevation
of her right eye in direct upgaze, as well as a ‘V’ pattern esotropia more evident in
direct upgaze.
“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 Hypertropia in primary position, rather than
hypotropia
A marked superior oblique overaction
An evident A-Pattern convergence, noticeable in direct upgaze,
and...
A positive Bielschowsky head tilt test
“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, which increases on left gaze, and overshoots on right gaze. On diagnostic versions she shows an “A”
pattern convergence, and marked overaction of her right superior oblique muscle.
Positive Bielschowsky 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, thus confirming her
diagnosis as a Right Inferior oblique paresis rather than a right Brown's syndrome.
Iatrogenic Superior oblique overaction
An iatrogenic superior oblique overaction can very easily be mis-diagnosed as an acquired
Brown's if following are not taken into consideration: Intorsion and abduction in downgaze; An
increase of vertical deviation on adduction to the contralateral side; A left superior oblique
overaction which causes a right hypertropia on right gaze; and most often patients with this
condition will adopt a very evident chin-up abnormal head posture in the direction of the opposite
side in order to fuse binocularily.
This 9-year-old girl presents with a
very evident chin-up head posture. On
versions she shows a clear downshoot
of her right eye on adduction, and an
overaction of the left superior oblique
on abduction. Together with an 'A'
pattern strabismus, this child has an
evident Iatrogenic Superior oblique
Management of Brown's

Management of Brown
syndrome varies widely.
Imaging of the orbit (If the
cause of an acquired Brown's
syndrome is in question) with
special attention to the region
of the trochlea adds
significantly to the workup of
the patient with acquired
Brown syndrome.
In many cases, acquired Brown's
syndrome will spontaneously
resolve over several months to
even several years. Surgery
should only be considered
after the patient has been
observed for at least 6 months
Surgical consdieration for Congenital
Brown's syndrome





If there is a hypodeviation in primary position that causes a significant chin elevation,
surgery should be reserved for children older than 4 years of age; older children are less
likely to develop postoperative Suppression and amblyopia.
Operating on a child under the age of 4 years of age should be considered, but only if the
hypodeviation is large enough to disrupt fusion.
Management of congenital Brown's syndrome is based on lengthening the superior
oblique tendon.
Procedures such as tenotomy and tenectomy release the restriction but are not controlled
Superior Oblique muscle is not overacting and, therefore, procedures such as tenotomy
or tenectomy often result in a secondary superior oblique paresis
Complications of Surgery
Very often, complications can arise following surgery of Browns. This 6-year
old girl has an evident Browns syndrome of the Right eye. Limitation of
elevation in adduction is evident even in forced head posture.
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.
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.
In Summary




Brown's syndrome stands as one of the most recognizable
forms of vertical strabismus to date.
The hallmark feature of Brown's syndrome is the decreased
ability to look up and inward with the affected eye(s),
accounting for approximately 2% of all reported strabismus
cases.
May be present from birth (congenital) or may develop as a
result of inflammation or developmental problem
(acquired).
Management and treatment of Brown's most often depends
on the cause and severity of the condition. Optimal
management may require several surgical operations, again
depending on the cause and severity.
References
Online References:
•
•
•
The Strabismus Minute:
http://www.cybersight.org
The Canadian Neuro-Ophthalmology Group Textbook: Brown's Syndrome
http://www.neuroophthalmology.ca/textbook/disorders-of-eye-movements/iv-neuropathiesand-nuclear-palsies/iii-browns-syndrome
•
McGill University Pediatric & Adult Strabismus:
http://www2.medicine.mcgill.ca/strabismus/atlas/patients/atlasNav/atlas.php
•
Medscape: Brown’s Syndrome:
•
https://emedicine.medscape.com/article/1199301-overview
•
Brown Syndrome – NORD – North American Organization for Rare Diseases:
References (con’t)
Misc. Journal Papers:
Brown's syndrome, Survey of Ophthalmology, Volume 34, Issue 3, Pages 153-172, M. Edward
Edit
Master text styles

Wilson,
H. Sprague
Second
levelEustis, Marshall M. Parks
Third
level
Grave
complications
after Superior oblique tenectomy for Brown Syndrome, Journal of AAPOS,
Fourth level
Volume
Number 1, Pages 8-15, Alvina Pauline Santiago, Arthur L. Rosenbaum
Fifth1,
level

Bilateral idiopathic Brown's syndrome with delayed onset in the second eye, Journal of

AAPOS, Volume 4, number 3 Pages 158-63. Kraft, SP, Nabi, NU, Wilson, ME,
Some aspects of Paralytic strabismus, Optometry Today, March 23 2001, Pages 38-40. Peter G.

Swann
Textbooks:

Binocular Vision & Ocular Motility – Theory & management of Strabismus, 2002 Gunter K. Von

Noorden, Mosby Harcourt Health Sciences Press
Clinical Ophthalmology – A Systematic approach, 7th edition. Jack J. Kanski, Brad Bowling.

ExpertConsult, Elsevier Saunders, 2011
References (con’t)
Textbooks
(con’t):
Edit
Master
text styles

Clinical
Strabismus
Second
level management: Principles and Surgical Management, 1999Arthur L.

Third levelAlvina Pauline Santiago, David Hunter WB Saunders
Rosenbaum,
Fourth level
Colour
Atlas of Strabismus Surgery: Strategies and Techniques, 2014 Kenneth W. Wright, Yi
Fifth level
Ning J. Strube, Springer.

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

Strabismus Surgery: Basic and Advanced Strategies, 2004 David A. Plager, Edward G.

Buckley, Oxford University Press
Pediatric Clinical Ophthalmology: A Colour Handbook, 2012 Scott Olitsky, Leonard B.

Nelson, CRC Press
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