eMedicine Specialties > Ophthalmology > Extraocular Muscles

Brown Syndrome: Differential Diagnoses & Workup

Author: Kenneth W Wright, MD, Director, Wright Foundation for Pediatric Ophthalmology and Strabismus; Director, Pediatric Ophthalmology Research and Education, Cedars-Sinai Medical Center; Clinical Professor of Ophthalmology, University of Southern California Keck School of Medicine
Coauthor(s): Maria Gabriela Salvador, MD, Consulting Staff, Department of Ophthalmology, Division of Pediatric Ophthalmology and Strabismus, Colonia Polanco Chapultepec Morales
Contributor Information and Disclosures

Updated: Dec 3, 2007

Differential Diagnoses

Trochlear Nerve Palsy

Other Problems to Be Considered

The following signs of superior oblique overaction help differentiate it from Brown syndrome (see Table below):

  • Mild limitation of elevation in adduction
  • No limitation of elevation in abduction
  • Common bilateral involvement
  • Superior oblique overaction
  • A-pattern, lambda-subtype with divergence in downgaze
  • Fundal examination reveals intorsion in primary position that increases in downgaze.
  • Negative forced ductions test

The following signs occur with inferior oblique paresis, differentiating it from Brown syndrome (see Table below):

  • Limitation of elevation in adduction occurs, with a large vertical deviation in primary position, usually more than 10 PD.
  • Marked superior oblique overaction
  • A-pattern, convergence in upgaze
  • Fundal examination reveals intorsion in primary position, which increases in upgaze.
  • Positive head-tilt test
  • Negative forced ductions test

Double elevator palsy: In Brown syndrome, limitation of elevation in adduction is invariable; yet, this sign is also present in 70% of patients with double elevator palsy. The difference between elevation in adduction versus elevation in abduction differentiates Brown syndrome from double elevator palsy, where elevation is equal to or worse in abduction.

Differential Diagnoses: Clinical Features of Brown Syndrome, Inferior Oblique Paresis, and Superior Oblique Overaction

Open table in new window

Table
 Brown syndrome (inelastic superior oblique muscle-tendon complex)Primary superior oblique overactionInferior oblique paresis
Limitation of elevation in adductionUsually severe (-3 to -4)Usually mildUsually severe (-3 to -4)
Limitation of elevation in adductionCommon (mild to moderate)NoNo
Bilateral involvementRare (5-10%)CommonUnusual
Vertical deviationNone or small (<10 PD)Bilateral small (<10 PD)Unilateral large (>10 PD)
Superior oblique overactionNone or minimalYes, markedYes, marked
PatternNone or V-pattern Y-subtype with divergence in upgazeA-pattern Lambda-subtype with divergence in downward gazeA-pattern, often convergence in upgaze
Fundus torsionNone in primary or downgaze, intorsion in upgazeIntorsion in primary, increasing in downgazeIntorsion in primary, increasing in upgaze
Head title testNegativeNegativePositive
Forced ductionsPositiveNegativeNegative
 Brown syndrome (inelastic superior oblique muscle-tendon complex)Primary superior oblique overactionInferior oblique paresis
Limitation of elevation in adductionUsually severe (-3 to -4)Usually mildUsually severe (-3 to -4)
Limitation of elevation in adductionCommon (mild to moderate)NoNo
Bilateral involvementRare (5-10%)CommonUnusual
Vertical deviationNone or small (<10 PD)Bilateral small (<10 PD)Unilateral large (>10 PD)
Superior oblique overactionNone or minimalYes, markedYes, marked
PatternNone or V-pattern Y-subtype with divergence in upgazeA-pattern Lambda-subtype with divergence in downward gazeA-pattern, often convergence in upgaze
Fundus torsionNone in primary or downgaze, intorsion in upgazeIntorsion in primary, increasing in downgazeIntorsion in primary, increasing in upgaze
Head title testNegativeNegativePositive
Forced ductionsPositiveNegativeNegative


Workup

Laboratory Studies

  • No laboratory tests are specifically required in the workup of congenital Brown syndrome.
  • In cases of acquired, nontraumatic Brown syndrome, tests to exclude autoimmune diseases, such as lupus, juvenile rheumatoid arthritis (JRA), and rheumatoid arthritis, may need to be ordered.

Imaging Studies

  • Consider MRI of the orbit for acquired Brown syndrome, especially if associated with pain, discomfort, signs of inflammation, or an atypical pattern of strabismus.
  • In some cases, imaging studies may identify pathology in the area of the trochlea, even superior nasal orbital tumors and sinusitis.

More on Brown Syndrome

Overview: Brown Syndrome
Differential Diagnoses & Workup: Brown Syndrome
Treatment & Medication: Brown Syndrome
Follow-up: Brown Syndrome
Multimedia: Brown Syndrome
References

References

  1. Brown HW. True and simulated superior oblique tendon sheath syndromes. Doc Ophthalmol. Feb 21 1973;34(1):123-36. [Medline].

  2. Parks MM, Brown M. Superior oblique tendon sheath syndrome of Brown. Am J Ophthalmol. Jan 1975;79(1):82-6. [Medline].

  3. Helveston EM, Merriam WW, Ellis FD, Shellhamer RH, Gosling CG. The trochlea. A study of the anatomy and physiology. Ophthalmology. Feb 1982;89(2):124-33. [Medline].

  4. Wright KW. Brown's syndrome: diagnosis and management. Trans Am Ophthalmol Soc. 1999;97:1023-109. [Medline].

  5. Parks MM. Bilateral superior oblique tenotomy for A-pattern strabismus in patients with fusion (commentary). Binoc Vis. 1988;3:39.

  6. Buckley EG, Flynn JT. Superior oblique recession versus tenotomy: a comparison of surgical results. J Pediatr Ophthalmol Strabismus. May-Jun 1983;20(3):112-7. [Medline].

  7. Clarke MP, Bray LC, Manners T. Superior oblique tendon expansion in the management of superior oblique dysfunction. Br J Ophthalmol. Jul 1995;79(7):661-3. [Medline].

  8. Clarke WN, Noel LP. Brown's syndrome with contralateral inferior oblique overaction: a possible mechanism. Can J Ophthalmol. Aug 1993;28(5):213-6. [Medline].

  9. George JL, Maalouf T, Cordonnier MO, Angioi-Duprez K. [Abnormal eyelid positions in Brown syndrome]. J Fr Ophtalmol. Jun 2004;27(6 Pt 1):579-87. [Medline].

  10. Helveston EM, Birchler C. Superior oblique palsy: subclassification and treatment suggestions. Am Orthopt J. 1982;32:104-110.

  11. Prieto-Diaz J. Posterior tenectomy of the superior oblique. J Pediatr Ophthalmol Strabismus. Sep-Oct 1979;16(5):321-3. [Medline].

  12. Scott AB, Knapp P. Surgical treatment of the superior oblique tendon sheath syndrome. Arch Ophthalmol. Sep 1972;88(3):282-6. [Medline].

  13. Scott WE, Jampolsky AJ, Redmond MR. Superior oblique tenotomy: indications and complications. Int Ophthalmol Clin. 1976;16(3):151-9. [Medline].

  14. Von Noorden GK. Binocular Vision and Ocular Motility. St. Louis: CV Mosby; 1996:437-442.

  15. Wright KW. Color Atlas of Ophthalmic Surgery-Strabismus. Philadelphia, Pa: Lippincott; 1991:201-219.

  16. Wright KW. Superior oblique silicone expander for Brown syndrome and superior oblique overaction. J Pediatr Ophthalmol Strabismus. Mar-Apr 1991;28(2):101-7. [Medline].

  17. Wright KW. Surgical procedure for lengthening the superior oblique tendon. Invest Ophthamol Vis Sci. 1989;30(sup):377.

  18. Wright KW, Min BM, Park C. Comparison of superior oblique tendon expander to superior oblique tenotomy for the management of superior oblique overaction and Brown syndrome. J Pediatr Ophthalmol Strabismus. Mar-Apr 1992;29(2):92-7; discussion 98-9. [Medline].

  19. Wright KW, Silverstein D, Marrone AC, Smith RE. Acquired inflammatory superior oblique tendon sheath syndrome. A clinicopathologic study. Arch Ophthalmol. Nov 1982;100(11):1752-4. [Medline].

Further Reading

Keywords

Brown's syndrome, BS, superior oblique tendon sheath syndrome, true sheath syndrome, simulated sheath syndrome, strabismus, amblyopia

Contributor Information and Disclosures

Author

Kenneth W Wright, MD, Director, Wright Foundation for Pediatric Ophthalmology and Strabismus; Director, Pediatric Ophthalmology Research and Education, Cedars-Sinai Medical Center; Clinical Professor of Ophthalmology, University of Southern California Keck School of Medicine
Kenneth W Wright, MD is a member of the following medical societies: American Academy of Ophthalmology, American Academy of Pediatrics, and American Ophthalmological Society
Disclosure: Nothing to disclose.

Coauthor(s)

Maria Gabriela Salvador, MD, Consulting Staff, Department of Ophthalmology, Division of Pediatric Ophthalmology and Strabismus, Colonia Polanco Chapultepec Morales
Disclosure: Nothing to disclose.

Medical Editor

Anastasios J Kanellopoulos, MD, Assistant Program Director, Clinical Associate Professor, Department of Ophthalmology, Manhattan Eye, Ear, and Throat Hospital, New York University
Anastasios J Kanellopoulos, MD is a member of the following medical societies: American Academy of Ophthalmology, Association for Research in Vision and Ophthalmology, Eye Bank Association of America, and International Society of Refractive Surgery
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

J James Rowsey, MD, Director of Corneal Services, St Luke's Cataract and Laser Institute, Florida
Disclosure: Nothing to disclose.

CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

 
 
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