Major ArticlesSpontaneous resolution in patients with congenitalBrown syndromeEmma Dawson, DBO, John Barry, FRCOphth, and John Lee, FRCS, FRCOphth

INTRODUCTION Brown syndrome is characterized by a restriction of active and passive elevation inadduction. It can be congenital, acquired, intermittent, or constant. Spontaneous im-provement has been reported in all types but is less common in congenital and constantcases.

METHODS A retrospective review of patients seen between 1992 and 2002 was conducted at ourtertiary referral center of all new referrals with a diagnosis of constant congenital Brownsyndrome that was previously untreated.

RESULTS Thirty-two patients were identified (22 female and 10 male patients), with an age range atreferral of between 1 and 14 years. The right eye was affected in 14 patients, and 3 patientshad bilateral Brown syndrome. Twenty-nine (91%) demonstrated binocular vision. Nine-teen patients had an abnormal head posture. Twenty-four (75%) patients had someimprovement in their ocular movements without any intervention. The best improvementwas from a –3 limitation to full movement. Of 6 patients with a “click,” 5 improved. Painwas a problem in 10 patients, 8 of whom improved. Diplopia was a symptom in 13patients, of whom 11 improved. The final review ranged from 6 months to 9.5 years afterthe first visit. Five patients who had no spontaneous improvement underwent surgery andobtained improvement.

CONCLUSIONS Spontaneous improvement occurred in 75% of patients. Our management is to be cautiouswith treatment and adopt an observational approach with these patients. ( J AAPOS 2009;13:116-118)

B rown syndrome is a well-recognized ocular motilitydisorder that has consistent and characteristic fea-tures, making it an easily recognizable clinical syn-

drome. The common feature and major characteristic isrestricted or abolished elevation in adduction. A wideningof the palpebral fissure often is observed in adduction. Adowndrift of the affected eye is commonly found on con-tralateral version. Also common in patients with Brownsyndrome is the presence of V patterns.

This syndrome was originally classified by Brown as trueand simulated. True Brown syndrome is always congenitalin contrast to the simulated type, which may be congenitalor acquired and which is often variable. It is bilateral inapproximately 10% of patients.

See editorial on page 115.

Author affiliations: Moorfields Eye Hospital, London, United KingdomPresented at the 34th Annual Meeting of the American Association for Pediatric

Ophthalmology and Strabismus, Washington, DC, April 2-6, 2008.Conducted at: Moorfields Eye Hospital, London, UK.Submitted April 4, 2008.Revision accepted September 17, 2008.Published online December 15, 2008.Reprint requests: John Lee, FRCS, FRCS, FRCOphth, Department of Strabismus &

Paediatrics, Moorfields Eye Hospital, City Road, London EC1V 2PD, UK (email:john.lee@moorfields.nhs.uk)

Copyright © 2009 by the American Association for Pediatric Ophthalmology andStrabismus.

1091-8531/2009/$36.00 � 0doi:10.1016/j.jaapos.2008.09.007

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Brown’s original collection of patients, reported in1973,1 consisted of 126 patients. The highlighted syn-drome showed a tendency to affect the right eye (70 cases),and to be more common in female patients (74 cases).There is limited literature on spontaneous improvement inpatients with Brown syndrome, but the numbers are smalland the follow-up is often limited. Therefore, we decidedto review the natural history of our patients with congen-ital Brown syndrome.

Methods and MaterialsWe conducted a retrospective review at our tertiary referral centerof case notes from between 1992 and 2002 by using the orthopticnew case register. This is a register that records the diagnosis ofevery new patient referred to our department. The criteria wereall new referrals that had a diagnosis of constant congenitalBrown syndrome and had no previous treatment. The study wasapproved by our hospital Ethical Committee, which is the equiv-alent of an institutional review board.

ResultsThirty-two patients were identified. There were 22 femaleand 10 male patients. The mean age at first visit was 6years old, with a range of between 1 and 14 years. Themean age at last visit was 10 years, with a range of 4 to 16years. The right eye was affected in 14 (44%) patients, and

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3 patients had bilateral Brown syndrome. Twenty-nine(91%) patients were able to demonstrate binocular visionin free space, ranging from gross stereopsis (1680 arcsec)to 40 arcsec. Free space tests used for measuring stereopsisincluded Wirt and TNO stereotests. Nineteen (59%) pa-tients had a compensatory head posture enabling somefusion and stereopsis.

Twenty-four (75%) patients had a spontaneous im-provement in their ocular movements with time and with-out any treatment except observation (see e-Supplement 1,available at jaapos.org). The best improvement was froma –3 limitation to full movement. In 11 patients (34%),there was an improvement in the level of stereopsis. Thisranged from no stereopsis demonstrable in free space to100 arcsec and from 400 arcsec to 40 arcsec. Six (19%)patients developed a symptomatic “click” during the pe-riod of observation; 5 of these patients experienced animprovement in their ocular movements. In 10 (31%)patients, looking up was painful; 8 of these patients expe-rienced an improvement in their ocular movements. Bothof these symptoms improved with the improvement inthe patient’s ocular movements. Thirteen (41%) pa-tients complained of intermittent diplopia in elevationand sometimes in primary position without a head pos-ture, and 11 of these patients showed some improve-ment in their ocular movements.

The final review ranged from 6 months to 9.5 years afterthe first visit, with a mean of 4 years. Five (16%) patientswho had no improvement in their ocular movements andwere troubled with diplopia underwent extraocular musclesurgery. All had a positive traction test under generalanesthesia. All patients were free of symptoms postopera-tively and experienced an improvement in their ocularmovements. Three patients underwent a superior obliquesilicone tendon expander. The 2 remaining patients had asuperior oblique tenotomy. In one case, a second proce-dure of an inferior rectus Faden was also required, becauseof iatrogenic superior oblique underaction requiring con-tralateral yoke muscle surgery. Other treatment involved 3patients having glasses prescribed, 2 for hypermetropiaand 1 for astigmatism. Two patients required some part-time occlusion for amblyopia.

DiscussionIt is well known and has been reported2 that spontaneousregression is common in acquired and intermittent cases ofBrown syndrome and less common in congenital and con-stant cases. All of our patients were congenital and con-stant cases.

Waddell3 reported the stability of congenital Brownsyndrome by evaluating the change in elevation defi-ciency of 36 patients. Unfortunately, surgical and non-surgical cases were grouped together, making analysisdifficult. The study appears to demonstrate that theoperated group shows only slightly better results thanthe nonoperated group. Waddell was the first to men-

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tion that a “click” could represent an intermediate stagein the resolution.

Kaban and colleagues4 discussed 60 patients with con-genital Brown syndrome, but they clearly divide theirpatients into 2 groups, surgical and nonsurgical. They thenfurther divide the nonsurgical group into 2 more groups:those with a vertical deviation in the primary position andthose who were orthotropic in the primary position. Theirresults showed 11% had complete resolution, 11% showedsome improvement, and only 5% showed a worsening.None of our patients deteriorated.

Capasso and colleagues5 reported a single case of bilat-eral Brown syndrome in a female patient. After 7 months, aspontaneous resolution was observed in the right eye. Theproblem in her left eye showed no significant improvementover 36 months. In our series, we had 3 patients bilaterallyaffected.Allcasesimprovedonbothsides,butasymmetrically—only one had unilateral surgery.

Gregersen and Rindziunski6 reported 10 patients withcongenital Brown syndrome. Spontaneous improvementoccurred in 9 patients; 3 were cured. There was an averageof 13 years follow-up, with each patient having an averageof 9 examinations. The remaining 6 had a reduction of thehypotropia and the depression of the adducted eye. Fourpatients demonstrated normal binocularity: 2 had micros-trabismus; 4, alternating suppression.

Leone and Leone7 discussed a patient with congenitalBrown syndrome affecting the left eye. There was nochange up until 15 years of age. At 16 years of age, thepatient started to “exercise” his left eye for the next 2years. He then became aware of an audible “click.” Whenhe finally was assessed at 19 years of age, he demonstratedfull ocular movements without diplopia and without a“click.” They conclude that the “click” may have repre-sented a loosening of a fibrous obstruction within thesheath or the trochlea that finally stretched or thinned out,allowing unimpeded passage of the tendon.

Our findings of having the right eye affected more thanthe left is in agreement with the literature.8 Our study of32 patients showed a spontaneous improvement in 75%.This improvement is greater than any previous report.Our study showed 6 patients had a “click” and that 5 ofthese experienced an improvement in their motility. Sim-ilarly with pain, 10 patients complained of pain whenlooking up, and 8 patients demonstrated an improvementin their ocular motility. Diplopia was a problem in 13 ofour patients, and 11 showed an improvement in theirmotility.

Although our report is about spontaneous improvementin congenital Brown syndrome, 5 of our patients hadsurgery. Sprunger and colleagues9 thought each patientneeds an average of 2 operations to obtain an acceptableresult. In our series of patients who required surgery, only1 patient of a total of 5 required a second operation.

Our management is to be cautious with treatment forpatients with congenital Brown syndrome and adopt theobservational approach with these patients. In addition,

Volume 13 Number 2 / April 2009118 Dawson, Barry, and Lee

the known unpredictability of surgery for Brown syn-drome makes a conservative approach an attractive option.

AcknowledgmentsWe thank Gill Adams and John Sloper for allowing us to present theirpatients.

References1. Brown HW. True and simulated superior oblique tendon sheath

syndrome. Doc Ophthalmol 1973;34:123-36.2. Wilson ME, Eustice HS, Parks MM. Browns syndrome. Major re-

view. Surv Ophthalmol 1989;34:153-72.3. Waddell E. Brown’s syndrome revisited. Br Orthoptic J 1982;39:17-21.

4. Kaban TJ, Smith K, Orton RB, Noe LP, Clarke W, Cadera W.Natural history of presumed congenital Brown syndrome. Arch Oph-thalmol 1993;111:943-6.

5. Capasso L, Torre A, Gagliardi V, Magli A. Spontaneous resolution ofcongenital bilateral Brown’s syndrome. Ophthalmologica 2001;215:372-5.

6. Gregersen E, Rindziunski E. Brown’s Syndrome. Acta Ophthalmo-logica 1993;71:371-6.

7. Leone CR, Leone RT. Spontaneous cure of congenital Brown’s syn-drome. Am J Ophthalmol 1986;102:542-3.

8. Brown HW. Congenital structural anomalies. In: Allen JH, editor.Strabismus Ophthalmic Symposium II. St Louis (MO): Mosby; 1958.p. 391.

9. Sprunger DT, Von Noorden GK, Helveston EM. Surgical results inBrown’s syndrome. J Pediatr Ophthalmol Strabismus 1991;28:164-7.

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