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Brown Syndrome Differential Diagnoses

  • Author: Kenneth W Wright, MD; Chief Editor: Hampton Roy, Sr, MD  more...
 
Updated: Aug 26, 2014
 
 

Diagnostic Considerations

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.

Table. Differential Diagnoses: Clinical Features of Brown Syndrome, Inferior Oblique Paresis, and Superior Oblique Overaction (Open Table in a new window)

  Brown syndrome (inelastic superior oblique muscle-tendon complex) Primary superior oblique overaction Inferior oblique paresis
Limitation of elevation in adduction Usually severe (-3 to -4) Usually mild Usually severe (-3 to -4)
Limitation of elevation in adduction Common (mild to moderate) No No
Bilateral involvement Rare (5-10%) Common Unusual
Vertical deviation None or small (< 10 PD) Bilateral small (< 10 PD) Unilateral large (>10 PD)
Superior oblique overaction None or minimal Yes, marked Yes, marked
Pattern None or V-pattern Y-subtype with divergence in upgaze A-pattern Lambda-subtype with divergence in downward gaze A-pattern, often convergence in upgaze
Fundus torsion None in primary or downgaze, intorsion in upgaze Intorsion in primary, increasing in downgaze Intorsion in primary, increasing in upgaze
Head title test Negative Negative Positive
Forced ductions Positive Negative Negative

Differential Diagnoses

 
 
Contributor Information and Disclosures
Author

Kenneth W Wright, MD Director, Wright Foundation for Pediatric Ophthalmology and Strabismus Ophthalmology, Cedars-Sinai Medical Center; Clinical Professor of Ophthalmology, Keck School of Medicine of the University of Southern California

Kenneth W Wright, MD is a member of the following medical societies: American Academy of Ophthalmology, American Academy of Pediatrics, American Ophthalmological Society

Disclosure: Received royalty from Titan Surgical for inventor; Received royalty from Oxford University Press for author; Received royalty from Springer for author.

Coauthor(s)

Maria Gabriela Salvador, MD, MD 

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

J James Rowsey, MD Former Director of Corneal Services, St Luke's Cataract and Laser Institute

J James Rowsey, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for the Advancement of Science, American Medical Association, Association for Research in Vision and Ophthalmology, Florida Medical Association, Sigma Xi, Southern Medical Association, Pan-American Association of Ophthalmology

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, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Acknowledgements

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.

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

  2. Parks MM, Brown M. Superior oblique tendon sheath syndrome of Brown. Am J Ophthalmol. 1975 Jan. 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. 1982 Feb. 89(2):124-33. [Medline].

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

  5. Bhola R, Rosenbaum AL, Ortube MC, Demer JL. High-resolution magnetic resonance imaging demonstrates varied anatomic abnormalities in Brown syndrome. J AAPOS. 2005 Oct. 9(5):438-48. [Medline].

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

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

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

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

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

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

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

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

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

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

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

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

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

  19. 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. 1992 Mar-Apr. 29(2):92-7; discussion 98-9. [Medline].

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

 
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A 3-year-old patient with acquired right Brown syndrome. Marked limitation of elevation in adduction is present in the right eye. Pseudo-overaction of the left inferior oblique is present. Courtesy of Kenneth Wright, MD.
The same patient as in the image above, 6 years later. The patient shows normal eye movements, and no signs of Brown syndrome. A spontaneous resolution occurred over a 2-year period. Courtesy of Kenneth Wright, MD.
This patient has the longest follow-up in the silicone tendon expander group at 11 years. A. Preoperative composite photograph of eye movements, showing right Brown syndrome. The patient underwent silicone tendon expander, 6 mm right eye. B. Postoperative photograph 3 years after surgery, showing full ocular motility. C. Postoperative photograph 11 years after surgery, showing continued normal ocular motility. Courtesy of Kenneth Wright, MD.
Composite photographs, showing left Brown syndrome with marked limitation of elevation in adduction. Courtesy of Kenneth Wright, MD.
Fundus torsion (direct view). The bottom set of fundus photographs represents downgaze; the center photographs, primary position; and the top photographs, upgaze. Note that in the top set of photographs, the left fundus is intorted as the foveal fixation is slightly above the top of the optic disc. Courtesy of Kenneth Wright, MD.
Table. Differential Diagnoses: Clinical Features of Brown Syndrome, Inferior Oblique Paresis, and Superior Oblique Overaction
  Brown syndrome (inelastic superior oblique muscle-tendon complex) Primary superior oblique overaction Inferior oblique paresis
Limitation of elevation in adduction Usually severe (-3 to -4) Usually mild Usually severe (-3 to -4)
Limitation of elevation in adduction Common (mild to moderate) No No
Bilateral involvement Rare (5-10%) Common Unusual
Vertical deviation None or small (< 10 PD) Bilateral small (< 10 PD) Unilateral large (>10 PD)
Superior oblique overaction None or minimal Yes, marked Yes, marked
Pattern None or V-pattern Y-subtype with divergence in upgaze A-pattern Lambda-subtype with divergence in downward gaze A-pattern, often convergence in upgaze
Fundus torsion None in primary or downgaze, intorsion in upgaze Intorsion in primary, increasing in downgaze Intorsion in primary, increasing in upgaze
Head title test Negative Negative Positive
Forced ductions Positive Negative Negative
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