Horner Syndrome Workup

  • Author: Christopher M Bardorf, MD, MS; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Feb 15, 2012
 

Laboratory Studies

  • Lab studies in general do not play a role in the diagnosis and management of Horner syndrome.
  • Depending on the localization and suspected etiology, lab tests one may consider, in conjunction with appropriate medical consultation, include the following: CBC count, fluorescent treponemal antibody absorption (FTA-ABS) test, Venereal Disease Research Laboratory (VDRL) test, purified protein derivative (PPD) placement, and/or urine test (ie, vanillylmandelic acid [VMA], homovanillic acid [HVA]) to rule out neuroblastoma in pediatric Horner syndrome.
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Imaging Studies

  • Imaging studies may be ordered in conjunction with appropriate medical and/or surgical consultation depending on the localization and suspected etiology.[4]
  • Imaging studies may include MRI/MRA, angiography, extracranial Doppler ultrasound, and/or chest x-ray.
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Other Tests

  • Pharmacologic testing is very helpful in the diagnosis of Horner syndrome and localization of lesions causing Horner syndrome.[5]
  • Cocaine (4% or 10% solution)[6]
    • Cocaine inhibits the re-uptake of norepinephrine from the synaptic cleft. Two drops of 4% or 10% cocaine solution are instilled into each eye.
    • Cocaine instilled in an eye with intact sympathetic innervation causes the pupil to dilate. A sympathetically denervated pupil dilates poorly to cocaine, regardless of the level of the sympathetic interruption because of the absence of endogenous norepinephrine in the synapse.
    • The maximum response is seen 40-60 minutes after instillation of the drops. Postcocaine anisocoria greater than 0.8 mm is sufficient to diagnose Horner syndrome.
    • The disadvantages of cocaine drops are as follows: they are difficult to obtain because they must be made at a compounding pharmacy, they are relatively expensive, and they can give equivocal results.
  • Apraclonidine (0.5% or 1%)[7, 6]
    • Apraclonidine is a practical and reliable alternative to cocaine.
    • Apraclonidine is an ocular hypotensive agent. It is a weak, direct-acting alpha-1 and alpha-2 receptor agonist. Apraclonidine has little to no effect on a normal pupil.
    • Patients with Horner syndrome have denervation supersensitivity of the iris dilator muscle. Therefore, the pupil in the affected eye dilates in response to apraclonidine due to the up-regulated alpha-1 receptors. Reversal of anisocoria occurs after bilateral instillation of apraclonidine.
    • False-negative results may occur in acute cases, as it takes time for up-regulation of the alpha-1 receptors to occur.[8]
    • Apraclonidine 0.5% or 1% may cause lethargy, bradycardia, and respiratory depression in infants younger than 6 months due to immaturity of the blood-brain barrier.[9]
  • Hydroxyamphetamine 1% (Paredrine)
    • The localization of a lesion causing Horner syndrome may be aided by the use of 1% hydroxyamphetamine solution. Hydroxyamphetamine may distinguish a postganglionic third-order neuron lesion from a presynaptic second-order or first-order neuron lesion. Hydroxyamphetamine stimulates the release of norepinephrine from presynaptic postganglionic nerve terminals.
    • Two drops of 1% hydroxyamphetamine solution are instilled into each eye. Note that results of this test will be inaccurate if performed within 24-48 hours of the cocaine test.
    • Hydroxyamphetamine instilled into an eye with Horner syndrome with intact postganglionic fibers (ie, first- or second-order neuron lesions) dilates the pupil to an equal or greater extent than the normal pupil. An eye with Horner syndrome with damaged postganglionic fibers (third-order neuron lesions) does not dilate as well as the normal pupil after hydroxyamphetamine drops.
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Contributor Information and Disclosures
Author

Christopher M Bardorf, MD, MS  Ophthalmologist, Children's Eye Physicians

Christopher M Bardorf, MD, MS is a member of the following medical societies: American Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Gregory Van Stavern, MD  Attending Physician, Department of Ophthalmology and Neurology, Washington University School of Medicine

Gregory Van Stavern, MD is a member of the following medical societies: American Academy of Neurology and North American Neuro-Ophthalmology Society

Disclosure: Nothing to disclose.

Enrique Garcia-Valenzuela, MD, PhD  Clinical Assistant Professor, Department of Ophthalmology, University of Illinois Eye and Ear Infirmary; Consulting Staff, Vitreo-Retinal Surgery, Midwest Retina Consultants, SC, Parkside Center

Enrique Garcia-Valenzuela, MD, PhD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, Association for Research in Vision and Ophthalmology, Retina Society, and Society for Neuroscience

Disclosure: Nothing to disclose.

Specialty Editor Board

Gerhard W Cibis, MD  Clinical Professor, Director of Pediatric Ophthalmology Service, Department of Ophthalmology, University of Kansas School of Medicine

Gerhard W Cibis, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, and American Ophthalmological Society

Disclosure: Nothing to disclose.

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

Disclosure: Medscape Salary Employment

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, Pan-American Association of Ophthalmology, Sigma Xi, and Southern Medical Association

Disclosure: Nothing to disclose.

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.

References

  1. Reede DL, Garcon E, Smoker WR, Kardon R. Horner's syndrome: clinical and radiographic evaluation. Neuroimaging Clin N Am. May 2008;18(2):369-85, xi. [Medline].

  2. Smith SJ, Diehl N, Leavitt JA, Mohney BG. Incidence of pediatric Horner syndrome and the risk of neuroblastoma: a population-based study. Arch Ophthalmol. Mar 2010;128(3):324-9. [Medline].

  3. Pirouzian A, Holz HA, Ip KC, Sudesh R. Acquired infantile Horner syndrome and spontaneous internal carotid artery dissection: a case report and review of literature. J AAPOS. Apr 2010;14(2):172-4. [Medline].

  4. Almog Y, Gepstein R, Kesler A. Diagnostic value of imaging in horner syndrome in adults. J Neuroophthalmol. Mar 2010;30(1):7-11. [Medline].

  5. Mughal M, Longmuir R. Current pharmacologic testing for Horner syndrome. Curr Neurol Neurosci Rep. Sep 2009;9(5):384-9. [Medline].

  6. Perez-Inigo MA, Gonzalez I, Fernandez FJ, Díaz S, Ferrer C, Alias E, et al. [Usefulness of apraclonidine in the diagnosis of Horner syndrome]. Arch Soc Esp Oftalmol. Feb 2009;84(2):105-8. [Medline].

  7. Freedman KA, Brown SM. Topical apraclonidine in the diagnosis of suspected Horner syndrome. J Neuroophthalmol. Jun 2005;25(2):83-5. [Medline].

  8. Dewan MA, Harrison AR, Lee MS. False-negative apraclonidine testing in acute Horner syndrome. Can J Ophthalmol. Feb 2009;44(1):109-10. [Medline].

  9. Watts P, Satterfield D, Lim MK. Adverse effects of apraclonidine used in the diagnosis of Horner syndrome in infants. J AAPOS. Jun 2007;11(3):282-3. [Medline].

  10. Albert DM, Jakobiec FA. Principles and Practice of Ophthalmology. 1994;4:2473-4.

  11. American Academy of Ophthalmology. Basic and Clinical Science Course: Neuro-ophthalmology. 1999-2000;5:97-99, 109-111.

  12. Fraunfelder, FT. Drug-Induced Ocular Side Effects and Drug Interactions. 1996;4:553.

  13. Gutman I, Levartovski S, Goldhammer Y, et al. Sixth nerve palsy and unilateral Horner's syndrome. Ophthalmology. Jul 1986;93(7):913-6. [Medline].

  14. Loewenfeld I. The Pupil: Anatomy, Physiology and Clinical Applications. 1993;2:1131-1177.

  15. Mokhtari F, Massin P, Paques M, et al. Central retinal artery occlusion associated with head or neck pain revealing spontaneous internal carotid artery dissection. Am J Ophthalmol. Jan 2000;129(1):108-9. [Medline].

  16. Roy FH. Ocular Syndromes and Systemic Diseases. 1989.

 
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