Strabismus, 13:201–204, 2005Copyright ©c 2005 Taylor & Francis Inc.ISSN: 0927-3972 print / 1744-5132 onlineDOI: 10.1080/09273970500379941

CASE REPORT

An Unusual Cause of AcquiredBrown’s Syndrome

Dr. A. O. GarrickMiss McGuiness, andMr. HeavenDepartment of Ophthalmology,Royal Albert Edward Infirmary,Wigan, UK

KEYWORDS Brown’s syndrome; superior oblique tendon sheath syndrome; non-Hodgkinlymphoma; B-cell malignant lymphoma; ocular motility disorder; radiotherapy

CASE REPORTA 56-year-old male presented with complaints of swelling, superiorly in

his left eye, for the past three months. This was associated with pain anddouble vision on elevation, watering, photophobia, and blurring of vision,all in the left eye. The right eye was free of symptoms. The past ocularhistory was unremarkable. He was under treatment for hypertension andhypercholesterolaemia.

Orthoptic investigations revealed best-corrected visual acuity of 6/5 and 6/9in the right and left eye, respectively, with no history of anisometropia. Thecover test revealed a minimal exophoria with good recovery. There was a markedrestriction of movement in the left eye in all elevated positions, which wasgreatest on adduction in elevation. The patient experienced diplopia in eleva-tion and on looking to the right. A Lees screen was performed and reveaeda mechanical field resembling a “dog ear” as in Brown’s syndrome (Fig. 2).The pupils reacted normally to light and accommodation. Saccadic movementswere slightly hypometric on horizontal and vertical gaze. Binocular functionswere within normal limits and the prism cover test measurements were 4∧BI atnear and 1∧BI at distance. He had bifoveal fixation on the 4∧test for centralfixation.

The most significant finding on ocular examination was a fleshy subconjunc-tival mass overlying the superior aspect of the left globe. This was pink, firm,well vascularised and painful to the touch. Intra-ocular pressures (IOP) were16 and 24 mmHg in the right and left eye, respectively. Fundoscopy showed ahealthy disc and macula. No abnormalities were found on general examination,specifically, no palpable lymph nodes.

The following investigations were then done: blood tests (FBC, U&E, LFT’s,LDH), incisional biopsy, and CT-scans of the orbits. The results of the bloodtests were normal. The CT-scans revealed a soft tissue mass, 1.2 cm thick, inthe left superior conjunctival region, extending along the postero-medial as-pect of the globe on the inferior aspect (Fig. 1). There was no extension intothe retro-orbital space. Histology of the biopsy revealed tumour cells that

Accepted 1 October 2005.

Correspondence: Dr. A. O. Garrick,Department of Ophthalmology,Royal Albert Edward Infirmary,Wigan Lane, Wigan WN1 2NN, UK.E-mail:

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FIGURE 1 CT-scans of the orbits.

expressed leukocyte common antigen and the B-cellmarker CD20, leading to a histological diagnosis of B-cell non-Hodgkins malignant lymphoma.

The patient was referred to the oncologist who re-quested CT-scans of the neck, chest, abdomen andpelvis and also a bone marrow trephine and as-pirate. None of these investigations revealed anyabnormalities.

The above results led to a diagnosis of stage IEnon-Hodgkins lymphoma. He subsequently had 8

FIGURE 2 Hess screen chart of extraocular movements before radiotherapy to the left eye.

doses of radiotherapy under the care of the oncolo-gist and was reassessed in our department 15 weekslater, 3 weeks after the last dose of radiotherapy. Hisdiplopia had resolved and the patient felt that he wasable to move his eyes freely in elevation without anydiscomfort.

Visual acuity was 6/5 in both eyes, representing animprovement in the left eye. The reduced VA in theleft eye on initial presentation was presumably dueto induced astigmatism. On ocular examination, the

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FIGURE 3 Hess screen chart of extraocular movements after radiotherapy to the left eye.

superior bulbar conjunctiva was now flat but still pink.The IOP was stable in the right eye but had decreasedto 12 mmHg in the left. The cover test revealed a min-imal exophoria for near and a minimal esophoria fordistance, measuring 4∧BI and 2∧BO, respectively.

Ocular movements showed a slight overaction of theright eye in laevo-elevation and a minimal limitation ofthe left eye in adduction with no diplopia. There wasno obvious limitation of the left eye in adduction inelevation. A second Lees screen revealed a improvementin the mechanical limitation of the left eye in adductionin elevation with only minimal restriction (Fig. 3).

DISCUSSIONThe superior oblique tendon sheath syndrome or

Brown’s syndrome, as it is commonly called, is char-acterised clinically by limited elevation in adduction,while the depression in adduction is normal. Whenboth elevation and depression in adduction are limitedit is known as a trapped superior oblique tendon.

The aetiology of Browns syndrome is usually anabnormality in the superior oblique tendon or thetrochlear complex (Ansons & Davis, 1991). This canbe congenital or acquired (in early childhood or later),permanent, transient or temporary. Common congen-ital causes are developmental anomalies, i.e. a fibrousattachment between the superior rectus and the supe-

rior oblique tendon (Ansons & Davis, 1991). Acquiredcases are caused by a plethora of conditions, whichcharacteristically result in a mechanical restriction ofthe eye in elevation, greatest in adduction in elevation.The literature reports various aetiologies that have pro-duced an acquired Brown’s syndrome, including supe-rior oblique surgery (Mein & Harcourt, 1986), trauma(Mein & Harcourt, 1986), inflammation in the trochlearregion (Mein & Harcourt, 1986), a nodule or swellingof the tendon (Ansons & Davis, 1991), SLE (Mein &Harcourt, 1986) and scleritis (Mein & Harcourt, 1986).Documented unusual causes of Brown’s syndrome areorbital metastatic deposits in the extraocular muscles(Booth-Mason et al., 1985), cysticercosis (Pandey et al.,2001), Hurler-Scheie’s syndrome (Bradbury et al., 1989),mucopolysaccharidosis, repair of an orbital roof frac-ture (Laucer et al., 1998), juvenile arthritis (Wang etal., 1984), and blunt orbital trauma (Baker & Conklin,1987). However, the literature search for this case reportindicated that documented cases of orbital lymphomapresenting as a Brown’s syndrome appear to be non-existent.

Orbital lymphoma presenting clinically as a Brown’ssyndrome indicates that the location of the lymphomamust be in the anatomical region of the superior obliquemuscle, tendon or trochlear, i.e. located superiorly inthe orbit. With some imagination it is easy to under-stand the mechanism by which this occurs. The pressure

203 Unusual Cause of Brown’s Syndrome

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effect of a superior orbital lymphoma (or mass) wouldsimulate an attachment between the superior rectus andthe superior oblique tendon, thus limiting the move-ment of the superior oblique. If the mass were locatedelsewhere in the orbit (medial, lateral or inferior), theorthoptic findings would be different.

REFERENCESAnsons AM, Davis H. Diagnosis and Management of Ocular Motility Dis-

orders, 3rd ed. London: Blackwell Science Ltd., 1991;431–434.Baker RS, Conklin JD Jr. Acquired Brown’s syndrome form blunt orbital

trauma. J Pediatr Ophthalmol Strabismus. 1987;24(1):17–21.

Booth-Mason S, Kyle GM, Rossor M, Bradbury P. Acquired Browns Syn-drome: an unusual cause. Br J Ophthalmol. 1985;69:791–794.

Bradbury JA, Martin L, Strachan IM. Acquired Brown’s Syndromeassociated with Hurler-Scheie’s Syndrome. Br J Ophthalmol.1989;73:305–308.

Laucer SA, Sauer H, Pak SM. Brown’s syndrome diagnosed following repairof an orbital roof fracture: case report. J Craniomaxillofac Trauma.1998;4(4):20–22.

Mein J, Harcourt B. Diagnosis and Management of Ocular Motility Disor-ders, 1st ed. London: Blackwell Science Ltd., 1986;301–302.

Pandey PK, Chaudhuri Z, Bhatia A. Extraocular muscle cysticercosis pre-senting as Brown’s syndrome. Am J Ophthalmol. 2001;131(4):526–527.

Wang FM, Wertenbaker C, Behrens MM, Jacobs JC. Acquired Brown’ssyndrome in children with juvenile rheumatoid arthritis. Ophthal-mology. 1984;91(1):23–26.

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