Harlequin syndrome
From IDWiki
Background
- Rare dysautonomic syndrome
- Caused by unilateral injury of sympathetic nerves
- Often idiopathic, but may be caused by cervical or upper thoracic diseases
Etiologies
- Primary/idiopathic (Harlequin syndrome)
- Secondary (Harlequin sign)
- Structural lesions: pancoast tumour, cervical sympathitic tumour, cervical carotid artery dissection, birth injury, thoracic syrinx, medullary infarction, thalamic hemorrhage
- Post-anaesthesia: thoracic epidural, paravertebral block, multilevel intercostal bnerve block, interscalene block, erector spinae plan block, central line of jugular vein
- Post-surgery: carotid endarterectomy, thyroidectomy, anterior cervical discectomy, excision of neck schwannoma, rotational spine surgery, ECMO line insertion, thoracic sympathetcomy, excision of cervical or mediastinal mass
- Harlequin syndrome of neonates
Clinical Manifestations
- Hemifacial discolouration that is sharply demarcated at the midline
- The affected side is pale, cold, and anhidrotic
- May be triggered by exercise, strong emotions, spicy food, and heat
- Lasts minutes to hours, then self-resolves
