Acquired Brown syndrome: Report of two casesMasoud Aghsaei Fard, MD, Abulfazl Kasaei, MD, and Hossein Abdollahbeiki, MD

We report 2 cases of acquired Brown syndrome, each associatedwith a mass in the supranasal area of the involved eye. Meticulousendoscopic surgery of an ethmoidal mucocele in the first case re-sulted in the recovery of superior oblique muscle function. Imagingstudies suggested the presence of an orbital venous malformationin the second case, the clinical course of which was stable, withminimal intermittent diplopia over time.

Brown syndrome is a restrictive strabismus charac-terized by limitation of elevation in adduction,normal elevation in abduction, and a positive

forced duction test.1 We present 2 patients with acquiredBrown syndrome associated with a supranasal ethmoidalmucocele in 1 case and an orbital venous malformation inthe other.

Case 1

A 31-year-old man presented to the Farabi Eye Hospi-tal, Tehran, Iran with the chief symptom of a slowlygrowing mass in the anterior supranasal area of the rightorbit associated with binocular vertical diplopia in up-gaze and image tilting of 1 month’s duration. Themass had been present for 5 months without accompa-nying diplopia. There was no history of sinus disease.Uncorrected visual acuity was 20/20 in both eyes. Afirm, nontender mass was found on external examinationof the right eye. The eyes were orthotropic in primaryposition, but duction and version testing demonstrateda �1 limitation of elevation in adduction of the righteye (Figure 1A). A right hypotropia of 6D was presentin left and upgaze. Forced duction testing indicated a re-striction of elevation in adduction of the right eye. Thedouble Maddox rod test showed 5� incyclotorsion. Acomputed tomography (CT) scan showed a nonhomoge-nous mass of the right ethmoidal sinus having an intra-orbital, extraconal extension consistent with a mucocele(Figure 1B). The mass appeared to be compressing thesuperior oblique tendon and the medial rectus muscle.Endoscopic removal of the mass and subsequent patho-logic analysis confirmed the diagnosis of mucocele. Thepatient’s diplopia resolved 3 days after the endoscopicprocedure (Figure 2A and B), and right eye motility im-

Author affiliations: Farabi Eye Research Center, Department of Ophthalmology, TehranUniversity of Medical Sciences, Tehran, IranSubmitted January 23, 2011.Revision accepted May 5, 2011.Reprint requests: Masoud Aghsaei Fard, MD, Farabi Eye Research Center, Quazvin Sq.,

Tehran, Iran 1336616351 (email: masood219@gmail.com).J AAPOS 2011;15:398-400.Copyright � 2011 by the American Association for Pediatric Ophthalmology and

Strabismus.1091-8531/$36.00doi:10.1016/j.jaapos.2011.05.005

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proved in the field of action of the superior obliquemuscle, with no deviations.

Case 2

A 46-year-old man presented to the Farabi Eye Hospitalwith intermittent vertical diplopia that became apparentwhen he was bending over. The frequency of diplopia de-creased over 2 months. Best-corrected visual acuity was20/20 in each eye. Motility examination disclosed �1limitation of elevation in adduction. Bending overinduced vertical diplopia with 5D left hypotropia. CTscanning and magnetic resonance imaging revealed anenhancing, hypointense mass of the left supranasal orbitwithout enlargement on straining (Figure 3A, B). CTangiography failed to reveal the venous malformation(Figure 3C). A color Doppler sonogram of the left orbit(captured using a 7.5 MHz linear ultrasound transducer)identified the supranasal mass as enlarged venous struc-tures with a low flow and partial thrombosis. The flowwas reversed and accentuated during the time that thepatient performed a Valsalva maneuver. Based on clini-cal history and imaging findings, the patient was diag-nosed with orbital varices. No intervention wasrequired but he continued to experience intermittentdiplopia.

Discussion

Acquired Brown syndrome can be caused by traumatic,neoplastic, infectious, autoimmune, inflammatory, andiatrogenic processes.1 Two cases of acquired Brownsyndrome caused by ethmoidal mucocele similar to Case1 have been reported previously.2,3 In both cases, a masscompressed the superior oblique tendon; however,immediate and complete recovery of elevation inadduction after surgery occurred in 1 case,3 suggestinga purely mechanical cause. Bhola and colleagues2 reporteda case in which the spread of inflammation from adjacentsinus disease might have been responsible for residual lim-itation after endoscopic surgery.

In our case, intorsion was also present. We believe thatthis was caused by a change in superior oblique tendon vec-tor force due to themass’s enhancing torsional action of themuscle.

Orbital venous malformations and varices are rare andbelieved to be congenital, but patients typically do not be-come symptomatic until adulthood. Typically these lesionspresent due to intermittent diplopia or proptosis duringepisodes of straining or prone positioning. If a lesion is sus-pected, contrast CT should be performed both at rest andwith a Valsalva maneuver. The varix will also show

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FIG 3. Orbital imaging of second patient at presentation showing leftsupranasal mass with enhancement on axial CT (A) and hypointensesignal on coronal T1-weighted magnetic resonance imaging (B). Sag-ittal CT angiography did not show any connection of the mass with thesuperior ophthalmic vein (C).

FIG 1. A, Clinical photograph of patient’s right eye at initial presenta-tion showing supranasal mass and limiting elevation in adduction andcausing Brown syndrome. B, Coronal computed tomography scan(CT) shows ethmoidal mucocele.

FIG 2. Clinical photograph obtained 3 days after endoscopic surgeryshowing improvement of elevation in adduction (A) and resolutionof the mucocele on coronal CT (B).

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enhancement with contrast on CT images and will be hy-perintense on T2-weighted images. The lesions in our pa-tient would have been missed on standard orbitalvenography since theymay have no connection with the su-perior ophthalmic vein. The advantage of color Dopplersonography over other imaging modalities is its ability todetect blood flowwithin a lesion. Previous series have dem-

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onstrated the utility of color Doppler in identifying vascu-lar lesions of the orbit including varices.4,5 We believe thatCase 2 case showed no evidence of distensibility because ofpartial thrombosis, a small superficial lesion, and probablyless extensive communication with the systemic venoussystem.6

References

1. Wright KW. Brown’s syndrome: Diagnosis and management. TransAm Ophthalmol Soc 1999;97:1023-109.

2. Bhola R, RosenbaumAL. Ethmoidal sinus mucocele: An unusual causeof acquired Brown syndrome. Br J Ophthalmol 2005;89:1069.

3. Lacy PD, Rhatigan M, Colreavy MP, Lyons BM, Irani BN,McNab AA. Acquired Brown’s syndrome caused by a fronto-ethmoidal mucocoele. Aust NZ J Surg 2000;70:688-9.

4. Kawaguchi S, Nakase H, Noguchi H, Yonezawa T, Morimoto T,Sakaki T. Orbital varix diagnosed by color Doppler flow imaging—case report. Neurol Med Chir (Tokyo) 1997;37:616-9.

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5. Hatton MP, Remulla HD, Tolentino MJ, Rubin PA. Clinical applica-tions of color Doppler imaging in the management of orbital lesions.Ophthal Plast Reconstr Surg 2002;18:462-5.

An Eye on the Art

“‘This is how I remember Pop,’ I said.

Colton took the frame, held it in bothaminute or so. I waited for his face to lighfact, a frown crinkled the space betwee‘Dad, nobody’s old in heaven,’ Colton s

—Todd Burpo with Lynn Vincent (fromtounding Story of His Trip to Heaven andTennessee, 2010)

6. Lacey B, Rootman J,MarottaTR.Distensible venousmalformations ofthe orbit: Clinical and hemodynamic features and a new technique ofmanagement. Ophthalmology 1999;1061:1197-209.

s—The Arts on the Eye

hands, and gazed at the photo fort up in recognition, but it didn’t. Inn his eyes and he shook his head.aid. ‘And nobody wears glasses.’”

Heaven is for Real: A Little Boy’s As-Back, Thomas Nelson, Nashville,

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