University of Iowa Health Care

Ophthalmology and Visual Sciences

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Acute Acquired Brown Syndrome:

38-year-old Male with Ethmoidal Sinus Mucocele

Rahul Bhola, MD

December 1, 2005

Chief Complaint: 38-year-old male presented with binocular vertical diplopia

History of Present Illness (History of Present Illness): The patient noted the sudden onset of vertical binocular diplopia which he noticed was greatest in left gaze and was relieved with a chin-up position. There was no history of trauma or any medical illness.

Medical History and Past Ocular History: Unremarkable

Family and Social History: Non-contributory

Ocular Exam:

  • General: Well-appearing male patient in no acute distress
  • Visual Acuity: OD-- 20/20; OS-- 20/20
  • Intra-ocular pressure: Within normal limits, OU
  • Motility: Under-elevation in adduction of the right eye mimicking Brown Syndrome (see Figure 1). A right hypotropia of 2 prism diopters (2 PD) in primary gaze increasing to 4 PD in left and upgaze
  • External exam, eyelids, adnexa, and anterior ocular examination:
    • Right: A soft compressible tender mass measuring 2x2 cm on the superonasal aspect of the right upper lid extending from the trochlea to the medial canthal tendon was noted. Normal anterior ocular exam.
    • Left eye: Normal
  • Pupils:
    • Equally reactive, OU
    • No relative afferent pupillary defect (- RAPD)
  • Dilated fundus exam (DFE):
    • OU-- Normal macula, vitreous, and peripheral retina
Figure 1: Versions in the nine cardinal gazes at the time of presentation. Note the under-elevation in adduction of the right eye, which is worse in attempted gaze to the patient's upper left.
Versions in the nine cardinal gazes at the time of presentation. Note the under-elevation in adduction of the right eye, which is worse in attempted gaze to the patient's upper left

Course: The patient's symptoms and motility examination are consistent with restriction of motility involving the right superior oblique. This is in the same anatomical location where a soft, tender mass was noted. Magnetic resonance imaging (MRI) with contrast (see Figure 2) revealed an expansile, nonenhancing 3-4 cm mass of the right ethmoidal sinus with an intraorbital and extraconal extension consistent with a mucocele. The mass appeared to be pressing on the globe causing effacement of the right medial rectus and superior oblique tendon with anterolateral displacement of the trochlea.

Figure 2: Axial section of T1 contrast MRI showing right-sided ethmoidal mass consistent with a mucocele.
Axial section of T1 contrast MRI showing right-sided ethmoidal mass consistent with a mucocele.

Course:

The cause of vertical binocular diplopia in this patient was a ethmoidal sinus mucocele pressing on the globe resulting in effacement of the superior oblique tendon with anterolateral displacement of the trochlea thus causing a limitation to elevation in adduction classically known as Brown Syndrome. Following endoscopic removal of the mass the patient was relieved of diplopia and ugze ability in adduction was improved, but moderately limited. The histopathology of the lesion was consistent with a mucocele.

Discussion:

Brown Syndrome was first characterized in 1950 by Harold Whaley Brown as a restrictive limitation to elevation in adduction. On the basis of surgical findings Brown implicated a shortened superior oblique (SO) tendon sheath as the cause of the syndrome. Most subsequent reports have alternatively proposed an abnormality in the trochlear-SO tendon complex as the etiology of restriction to elevation in adduction.

Various etiologies of acquired Brown Syndrome have been described including frontal sinus surgery (Rosenbaum et al, 1985), blepheroplasty (Levine et al, 1975), chronic sinusitis, trauma, inflammatory (Hermann, 1978), systemic lupus erythematosis (Whitefield et al, 1995), and restrictive fibrous bands. High-resolution MRI demonstrated varied abnormalities in both congenital and acquired Brown syndrome such as traumatic or iatrogenic scarring, avulsion of the trochlea, cyst in the superior oblique tendon, inferior displacement of the lateral rectus pulley and fibrous restrictive bands extending from the trochlea to the globe (Bhola et al, 2005).

According to the anatomic abnormalities noted by MRI, four distinct mechanisms of Brown syndrome have been identified: trochlear damage, superior oblique (SO) tendon abnormalities, abnormalities of rectus extra ocular muscle pulleys, and congenital abnormalities of superior oblique muscle (Bhola et al, 2005). MRI can delineate the pathological anatomical abnormalities causing Brown syndrome thereby individualizing surgical management without reliance on extensive exploratory surgery.

Diagnosis: Acquired Brown Syndrome secondary to Ethmoidal Mucocele

EPIDEMIOLOGY

Incidence: 1 in 400-450 strabismus cases

Etiology based on MRI:

  • Congenital Abnormality of SO muscle/tendon
    • Inelastic muscle/tendon
    • Anomalous orbital adhesions with SO tendon anomalies
  • Trochear Damage
    • Traumatic: Vehicle accident, dog bite, sports injury
    • Iatrogenic: frontal sinus surgery, blepharoplasty, glaucoma implant, scleral buckle
    • Inflammatory: chronic sinusitis, trocheilits, rheumatoid arthritis, systemic lupus erythematosis
    • Orbital masses: mucocele, orbital tumors
  • SO tendon abnormalities
    • Inflammatory
    • SO tendon cyst
    • Iatrogenic: SO tucking
  • Abnormality of rectus extraocular muscle pulleys
    • Pulley heterotopy
    • Pulley Instability

SIGNS

  • Limitation to elevation in adduction
  • "Y-pattern" with divergence in upgaze
  • Positive forced duction for restricted elevation in adduction
  • +/- anomalous head posture
  • +/- ipsilateral hypotropia in primary position
  • Minimal or no SO overaction
  • Trochlear tenderness and/or palpable click (inflammatory cases)

SYMPTOMS

  • Inability to elevate the eye in adduction
  • Diplopia
  • Abnormal head position
  • Supranasal orbital pain in traumatic or inflammatory conditions

TREATMENT

  • Managment in Brown syndrome should be tailored to the pathophysiology of the individual patient
  • In an acquired case of Brown syndrome, high-resolution MRI can define in great detail the pathological anatomical abnormalities causing Brown syndrome, allowing rational individualization of surgical management without reliance on extensive exploratory surgery.

Differential Diagnosis of diplopia and elevation deficit:

  • Brown syndrome
  • Elevation deficit in adduction
  • Inferior oblique palsy
  • Primary superior oblique overaction
  • Double elevator palsy

References

  1. Rosenbaum AL, Astle WF. Superior oblique and inferior rectus muscle injury following frontal and intranasal sinus surgery. J Pediatr Ophthalmol Strabismus 1985;22:194-202.
  2. Levine MR, Boynton J, Tenzel RR, Miller GR. Complications of Blepharoplasty. Ophthalmic Surg 1975; 6:47-53.
  3. Hermann JS. Acquired Brown’s syndrome of inflammatory origin. Arch Ophthalmol 1978;96:1228-32
  4. Whitefield L, Isenberg DA, Brazier DJ, Forbes J. Acquired Brown’s syndrome in systemic lupus erythematosus. Br J Rheumatol 1995;34:1092-4
  5. Bhola R, Rosenbaum AL, Ortube M, Demer JL. High resolution MRI demonstrates varied anatomic abnormalities in Brown’s Syndrome. J AAPOS 2005 Oct;9(1):438-48.
  6. Brown HW. Congenital structural muscle anomalies. In: Allen JH, ed. Strabismus Ophthalmic Symposium (I). St. Louis: Mosby; 1950:205-36.

Suggested citation format: Bhola RL: Acute Acquired Brown Syndrome: 38-year-old Male with Ethmoidal Sinus Mucocele. Eyerounds.org. December 1, 2005; Available from: http://www.EyeRounds.org/cases/50-Acquired-Brown-Syndrome-Ethmoid-Mucocele.htm.

last updated: 12-01-2005
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