Rosen & Barkin's 5-Minute Emergency Medicine Consult (352 page)

BASICS

Unilateral sympathetic denervation of the eye produces signs of Horner's syndrome:

• Relaxation of retracting muscles in upper and lower lids:
  
  • Ptosis
    (drooping of the lids)
    
• Loss of pupillary dilator innervation:
  
  • Miosis
    (unopposed pupillary constriction)
    
• Loss of sympathetic stimulation of sweat glands:
  
  • Anhidrosis
    

• 40% unknown (in 1 large series)
  
• Tumors of lung or metastases to cervical nodes:
  
  • May interrupt preganglionic sympathetic fibers (between thoracic sympathetic trunk and superior cervical ganglion)
    
• Trauma: Penetrating neck wounds directly injure sympathetic fibers
  
• Pneumothorax:
  
  • Tension pneumothorax may cause traction on sympathetic fibers owing to shift of mediastinal structures.
    
• Infiltration or infection of cervical nodes:
  
  • Sarcoidosis, tuberculosis
    
• Vascular disorders:
  
  • Migraine or cluster headaches
    
  • Carotid artery dissection
    
• Lateral medullary infarction produces Horner's syndrome as part of the Wallenberg syndrome:
  
  • Presents with vertigo and ataxia, which may overshadow the Horner's syndrome.
    
• Cavernous sinus thrombosis may present with some of the features of Horner's syndrome:
  
  • The condition typically causes headache and/or eye pain.
    
  • Ocular signs include ocular palsies, pain, chemosis, and proptosis.
    

• Hereditary Horner's syndrome:
  
  • Blue iris (or irregular coloration) on affected side
    
  • Brown on unaffected side (heterochromia iridis)
    
• Birth trauma:
  
  • May cause damage to sympathetic chain
    
• New Horner's syndrome in a child should prompt workup for tumor (neuroblastoma).
  

DIAGNOSIS

• Horner's syndrome is characterized by:
  
  • Ptosis:
    Drooping of eyelid on affected side, usually slight
    
  • Miosis:
    Decrease in pupillary size on involved side (pupillary asymmetry ≥1 mm)
    
  • Anhidrosis:
    Lack of sweating on involved side of face
    
• The importance of Horner's syndrome is its association with certain disease states.
  

Focus on pre-existing conditions that predispose to Horner's syndrome or are risk factors for these conditions:

• Tumors, vascular disease, trauma:
  
  • Minor trauma often precedes carotid dissection.
    
• Cardiovascular risks
  
• Exposures:
  
  • For pseudo-Horner
    
• Pain:
  
  • Suggests carotid dissection
    

Concentrate on a focused neurologic exam looking to confirm Horner's syndrome and exclude other neurologic deficits:

• General physical exam should focus on identifying signs of other suspected conditions, such as tumor.
  

• History and physical exam focused on neurologic findings
  
• CXR to screen for tumor or pneumothorax
  

Provocative testing:

• Pharmacologic (cocaine) testing confirms diagnosis of sympathetic ocular lesion:
  
  • 1 drop of 5% ocular cocaine solution is instilled
    into each eye
    .
    
  • Failure of pupil on involved side to dilate as much as other pupil (increase in amount of anisocoria) in 1 hr is confirmatory (positive test).
    

Not useful for Horner per se:

• Often needed as part of the workup of causative or associated conditions.
  

• CXR is usually indicated because of the association of chest pathology and Horner's syndrome.
  
• CT or MRI of head, neck, or chest may be indicated depending on signs and symptoms.
  
• For suspected carotid dissection:
  
  • MRA or CTA of the head and neck:
    
    • Either test is appropriate
      
    • The lesion is expected to be ipsilateral to the Horner's syndrome.
      
• Choice of neuroimaging in suspected stroke depends heavily on local protocols and resources.
  
  • At a minimum, if stroke is suspected CT of the brain is indicated to rule out hemorrhagic stroke.
    
  • If acute stroke (<3 hr) is suspected and patient is a candidate for thrombolysis image on STAT basis
    

Ocular tonometry is indicated if acute glaucoma is suspected.

• Increased intracranial pressure (ICP):
  
  • Almost always associated with altered level of consciousness (LOC), headache
    
• Simple anisocoria (pseudo-Horner's syndrome):
  
  • 15–20% of the population has anisocoria and 3–4% also has miosis and ptosis.
    
  • Cocaine test is negative (both pupils dilate equally).
    
  • Inspect photo ID for pre-existing anisocoria.
    
• Topical medications or exposures are a common cause of miosis.
  
• Migraine or cluster headache
  
• Glaucoma, inflammatory ocular diseases, or ocular trauma
  

TREATMENT

Cautions:

• The importance of Horner's syndrome is its association with more serious underlying conditions.
  
• Patients with increased ICP or tension pneumothorax must be recognized and treated as soon as possible.
  
• If acute stroke suspected transport to designated stroke center when possible
  

• If increased ICP is suspected, initiate measures to control ICP:
  
  • Intubation, osmotic diuretics
    
• Tension pneumothorax:
  
  • Needle thoracostomy followed by chest tube
    

Horner's syndrome per se requires no ED treatment:

• Causative or associated conditions may require treatment.
  

Cocaine: 5% (adult), 2.5% (peds) ophthalmic solution: 1 drop in each eye is diagnostic.

FOLLOW-UP

Admission for isolated Horner's syndrome is not needed:

• Admission may be needed for underlying condition.
  

• Stable patients with isolated Horner's syndrome may be discharged with appropriate follow-up arranged for continued workup as outpatient:
  
  • When Horner's syndrome is suspected, emergencies such as carotid dissection or stroke should be ruled out prior to discharge.
    

Neurologists and ophthalmologists must often be involved in the workup of Horner's syndrome.

Must consider underlying etiology

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• Maloney WF, Younge BR, Moyer NJ. Evaluation of the causes and accuracy of pharmacologic localization in Horner’s syndrome.
  Am J Ophthalmol
  . 1980;90(3):394–402.
  
• Martin TJ. Horner’s syndrome, Pseudo-Horner’s syndrome, and simple anisocoria.
  Curr Neurol Neurosci Rep
  . 2007;7(5):397–406
  
• Proctor C, Chavis PS. Horner's syndrome.
  J Neuroophthalmol.
  2013;33(1):88–89
  
• Thanvi B, Munshi SK, Dawson SL, et al. Carotid and vertebral artery dissection syndromes.
  Postgrad Med
  . 2005;81:383–388.
  
• Wilheim H, Ochsner H, Kopycziok E, et al. Horner’s syndrome: A retrospective analysis of 90 cases and recommendations for clinical handling.
  Ger J Ophthalmol
  . 1992;1(2):96–102.
  

CODES