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