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Horner Syndrome

Horner Syndrome

Introduction

Horner syndrome is a neurological condition characterized by a classic triad of symptoms resulting from disruption of the sympathetic nerve supply to the eye and ipsilateral face.

Key Points

  • Classic triad: Miosis, ptosis, and anhidrosis
  • Caused by interruption of sympathetic innervation
  • Can be congenital or acquired
  • Requires thorough evaluation to identify underlying cause

Clinical Manifestations

Classic Triad:

  • Miosis: Pupillary constriction (2mm or greater difference)
  • Ptosis: Drooping of upper eyelid (2mm or greater difference)
  • Anhidrosis: Decreased sweating on affected side of face

Additional Features:

  • Apparent enophthalmos (sunken appearance of eye)
  • Iris heterochromia (in congenital cases)
  • Reverse ptosis (elevation of lower lid)
  • Vasodilation of affected side

Age-Specific Considerations:

  • Neonates:
    • May present with iris heterochromia
    • Often associated with birth trauma
    • Can be linked to neuroblastoma
  • Children:
    • May be discovered incidentally
    • Important to rule out underlying malignancy

Etiology & Classification

Anatomical Classification

  • First-Order Neuron (Central):
    • Hypothalamus to spinal cord (C8-T2)
    • Causes: Stroke, tumors, syringomyelia
  • Second-Order Neuron (Preganglionic):
    • Spinal cord to superior cervical ganglion
    • Causes: Pancoast tumor, trauma, thyroid surgery
  • Third-Order Neuron (Postganglionic):
    • Superior cervical ganglion to eye
    • Causes: Carotid dissection, cluster headache

Common Causes by Age Group

  • Congenital:
    • Birth trauma
    • Neuroblastoma
    • Genetic syndromes
  • Acquired:
    • Trauma (surgical/non-surgical)
    • Neoplasms
    • Vascular disorders
    • Inflammatory conditions

Diagnosis & Workup

Clinical Evaluation

  • Detailed History:
    • Birth history (for congenital cases)
    • Trauma history
    • Associated symptoms
    • Family history
  • Physical Examination:
    • Pupillary examination in light and dark
    • Measurement of palpebral fissures
    • Assessment of facial sweating
    • Careful neurological examination

Diagnostic Tests

  • Pharmacologic Testing:
    • Cocaine test (diagnostic)
    • Hydroxyamphetamine test (localization)
    • Apraclonidine test (confirmation)
  • Imaging Studies:
    • MRI brain and spine
    • CT chest
    • Carotid imaging
    • Urine catecholamines (for suspected neuroblastoma)

Management Approach

General Principles

  • Treatment directed at underlying cause
  • Urgent evaluation if acute onset
  • Regular monitoring for congenital cases

Treatment Options

  • Conservative Management:
    • Observation for mild cases
    • Regular follow-up
  • Surgical Intervention:
    • Based on underlying cause
    • Ptosis repair if needed
  • Specific Treatments:
    • Anticoagulation for carotid dissection
    • Tumor removal if indicated
    • Management of underlying conditions

Prognosis

  • Varies based on underlying etiology
  • Better outcomes with early intervention
  • May require long-term follow-up
Further Reading


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