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Seminars in Ophthalmology, 23:291–293, 2008

Copyright 
c Informa Healthcare USA, Inc.
ISSN: 0882-0538
DOI: 10.1080/08820530802505971

Management of Brown Syndrome


John Lee
Moorfields Eye Hospital ABSTRACT Brown syndrome is a challenging management problem. Con-
London, UK genital Brown syndrome may show sontaneous resolution, and conservative
management is successful in around 75% of cases. Inflammatory acquired
Brown syndrome may respond to peri-trochlear injection of steroids or oral
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non-steroidal inflammatory agents. Post-traumatic acquired Brown syndrome


is not as common as it was in the “pre-seat belt” era. Surgical management can
be undertaken, but results are disappointing.

KEYWORDS Brown Syndrome, eye muscle surgery

INTRODUCTION
Harold Brown described his eponymous syndrome in 19501 , ascribing it to an
For personal use only.

underaction of the inferior oblique muscle, but identifying the classical clinical
features of limited elevation of the affected eye in adduction, downdrift in
adduction, a “V” pattern and a positive forced duction test for passive elevation
in adduction.
The pathology is now generally agreed to be in the superior oblique ten-
don/trochlear complex, and the syndrome is subdivided into primary congen-
ital, and acquired. Acquired cases may be further classified as inflammatory,
traumatic and iatrogenic, but many other secondary causes have been reported.
This paper sets out some of our experience with primary and acquired Brown
syndrome.

PRIMARY CONGENITAL BROWN SYNDROME


Although most textbooks advocate surgical management of this condition,
it is generally agreed that surgery is unpredictable, and there is a high risk of
subsequent surgery, typically for an iatrogenic superior oblique palsy induced
by superior oblique tenectomy or tenotomy.
However, there are some reports suggesting that conservative management
may result in spontaneous improvement in some cases, thereby avoiding surgery
for either the primary motility defect or consecutive deviations.
Declaration of interest: The author
The first of these was by Waddell, in 19822 , who reported 36 cases, followed
reports no conflicts of interest. The over a period of 1–14 years. Twenty -four (67%) showed partial or complete im-
author alone is responsible for the provement. However, 14 patients underwent surgery, so the true spontaneous
content and writing of the paper.
improvement rate is not clear from the data reported, although clearly sponta-
Address correspondence John Lee,
FRCS, MRCP, FCOphth neous improvement occurred.
Moorfields Eye Hospital Gregersen, in 19933 , reported 10 cases followed over 13 years. Nine improved,
City Road, London
UK, EC1V 2PD.
and in 3 cases improvement was complete. Kaban et al.,4 also in 1993, reported
E-mail: john.lee@moorfields.nhs.uk full recovery in 10 of 60 cases with a follow-up period of 48 months.
291
We have recently reviewed our own patients and article by Wilson, Eustis and Parks6 lists many of the
experience.5 Our management philosophy has been to references.
practice a conservative approach, with careful monitor- Presumed inflammatory Brown syndrome is charac-
ing but with surgery withheld unless the patient clearly terized by typical ocular motility findings of Brown syn-
showed no sign of recovery over many years observa- drome typically associated with pain which patients lo-
tion, or requested surgery themselves. It has been our calize very accurately to the region of the trochlea of
experience that most children with Brown syndrome are the affected eye. There is usually local tenderness to
remarkably unconcerned by their compensatory head palpation as well. The condition does not seem to be
posture, and our main task has been to reassure their associated with any other systemic disorders, and may
parents that all was well, and that their child was main- resolve spontaneously, although relapses are common.
taining good control of its eye position and good binoc- Some time ago we reviewed our experience with 9
ular vision. cases of acquired Brown syndrome seen between Jan-
We retrospectively reviewed all cases referred to us of uary 1994 and December 1999. Six had a characteris-
primary congenital Brown syndrome, without previous tic presumed inflammatory etiology; three were post-
surgical treatment, seen between 1992 and 2002. There
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traumatic. We treated all of them with peri-trochear


were 32 patients, 22 female and 10 male. The age at injections of Dexamethasone (4 mg in 1 ml.) to
first attendance was from 1 to 14 years, with a mean the affected side. In two cases, a second injection
of 6 years. This wide range reflects the nature of our was performed, followed by a short course of non-
institution, with many of the older children being re- steroidal anti-inflammatory drugs such as Flurbiprofen
ferred for second opinions. The age at last attendance by mouth.
was from 4 to 16 years, mean 10 years. Three of six presumed inflammatory cases obtained
Twenty-nine had demonstrable stereopsis on first at- good relief of both pain and restricted motility after one
tendance, ranging from gross stereopsis to 30 arc sec- injection. Three others failed to improve. None of the
onds. Nineteen had an abnormal head posture docu- post-traumatic cases showed any improvement.
For personal use only.

mented at first attendance. Limitation of elevation in It has been our experience that post-traumatic Brown
adduction ranged from -5 to -2 a first visit. syndrome is a disappointing entity to treat. It occurred
Over the study period, 24 cases (75%) showed spon- much more frequently in the era when car windscreens
taneous improvement in ocular motility. In three cases were made of toughened, not laminated glass. In ad-
with bilateral Brown syndrome, both sides improved. In dition campaigns to increase seat belt usage, and leg-
eleven of these, we also documented an improvement islation to make it compulsory (passed in the UK in
in stereopsis. Follow up was from 6 months to 9.5 years January 1993), markedly changed the incidence of this
with a mean of 4 years. problem.
Five patients who failed to improve underwent In 1992, we reported6 13 cases of post-traumatic
surgery. Three had superior oblique tendon expanders, Brown syndrome, of which 12 followed road traffic acci-
and two had superior oblique tenotomies. One of these dents, and one a fronto-ethmoidectomy. The age range
developed some iatrogenic superior oblique underac- was from 18 to 46 with a mean of 34. Eight were male.
tion and required a subsequent contralateral inferior All had symptomatic diplopia due to restriction of ele-
rectus posterior fixation procedure. Long-term results vation in the affected eye. Three also showed superior
were good in all cases. oblique underaction on ocular rotations. Twelve of the
These results confirm us in our current management cases had been observed for spontaneous improvement
plan of careful observation while awaiting spontaneous for a period of 9 to 30 months before surgery. One case
improvement, which we anticipate in around three- presented 10 years after the trauma.
quarters of our patients. The remainder are likely not to All cases were initially managed with confirmation of
improve, and may require surgery, although we warn all forced duction testing, exploration of the trochlea and
parents that more than one procedure may be necessary. superior oblique tendon, removal of any glass foreign
bodies and superior oblique tenotomy. Post-operatively
ACQUIRED BROWN SYNDROME patients were asked to do vigorous ocular rotation ex-
Here are many reports of acquired Brown syndrome ercises. In 4 of 13 cases, this strategy worked fairly well,
due to a wide variety of causes. The excellent review and further intervention was not required. However,

J. Lee 292
seven cases underwent a second procedure, and 2 had ble steroids, but may also improve spontaneously. Post-
a total of three operations. traumatic Brown syndrome is happily much rarer than
These involved procedures on ipsilateral and con- it was in the days before laminated windscreens and
tralateral vertical rectus and oblique muscles. Patients compulsory seat belts. Management is difficult and dis-
reported symptomatic improvement in 7 cases, no appointing.
change in 3 cases, and worsening in 3 cases. Percent-
age scored fields of diplopia-free vision improved in 8, REFERENCES
were not recorded in 2, and were worse in 3 at final Brown H. Congenital structural anomalies, in Allen JH (ed): Strabismus
follow-up. Ophthalmic Symposium II. St Louis, Mosby, 1950, p391.
Waddell E. Brown’s syndrome revisited. Br Orthoptic J. 1982;39:17–21.
Gregersen, Rindziunski E. Brown’s Syndrome. Acta Ophthalmologica.
CONCLUSIONS 1993;71:371–76.
Kaban TJ, et al. Natural history of presumed congenital Brown Syndrome.
In primary congenital Brown syndrome, a policy of Arch. Ophthalmol.1993;111:943–46.
observation will allow a substantial majority of children Dawson, EM, Barry J-S, Lee JP. Spontaneous improvement in patients with
congenital Brown syndrome. JAAPOS. In press.
to show spontaneous improvement. Surgery can be re- Wilson ME, Eustis HS, Parks MM. Browns syndrome. Major review. Surv
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served for those who do not improve, but may require Ophthalmol 1989;34:153–72.
Aylward GW, Lawson J, McCarry B, Lee JP, Fells P. The surgical treatment
multiple surgical procedures. Presumed inflammatory of traumatic Brown syndrome. J Pediatr Ophthalmol Strabismus.
Brown syndrome may respond to local injection of solu- Sep-Oct;29(5):276–83.
For personal use only.

293 Management of Brown Syndrome

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