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We report a Japanese infant with Horner syndrome whose clinical examination and testing suggested the location of the causative lesion. A 4-year-old Japanese girl had an acquired right ocular ptosis and unequal pupils presenting shortly after birth. She also exhibited left hemifacial flushing and loss of sweating on the contralateral side (harlequin sign). Physical examination demonstrated 2.0 mm of ptosis of the right upper lid with normal elevator function. The diameters of the pupils were 4 mm on the left and 2.5 mm on the right. No sweating was induced in the right frontal region at 40 degrees C for 15 minutes of sweat challenge test. Otherwise, no abnormalities were found by the neurophysiologic examinations or magnetic resonance imaging of the brain. Based on the clinical examination, we speculated that the responsible lesion might be in the preganglionic areas. Harlequin sign was informative for making the diagnosis of Horner syndrome.
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PMID:Harlequin sign (hemifacial flushing and contralateral hypohidrosis) in a 4-year-old girl with Horner syndrome. 1691 33

Harlequin syndrome, the presentation of hemifacial flushing and sweating, is a well recognized, though rarely reported, phenomenon associated with cervical sympathetic trauma. It is thought to result from disruption to sudomotor and vasomotor neurons present in the cervical sympathetic chain. The more common Horner's syndrome classically comprises the triad of unilateral miosis, ptosis and ipsilateral facial anhydrosis, and may also present as a sequela of cervical sympathetic denervation. We report a 26-month-old child with concomitant Horner's and harlequin syndromes, following neck dissection to address a large cervical lymphatic malformation. To our knowledge this is the first reported case of both syndromes resulting from surgery, and illustrates the particular challenge of lymphatic malformations in neck surgery due to their non-adherence to anatomical planes.
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PMID:Coexisting harlequin and Horner syndromes after paediatric neck dissection: a case report and a review of the literature. 1908 11

Interscalene brachial plexus blockade is regularly used for postoperative pain management following shoulder surgery. A known but generally benign side effect of this technique is Horner syndrome. Another syndrome known as harlequin syndrome exists but does not appear to be as common. This syndrome consists of contralateral facial flushing and sweating secondary to ipsilateral sympathetic chain inhibition. Despite the alarming presentation in the perioperative setting, this syndrome appears to be benign and self-limiting when precipitated by regional anesthetic technique. This article describes an occurrence of harlequin syndrome without observed ptosis or miosis following a postoperative interscalene nerve block.
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PMID:Isolated Harlequin Syndrome Following Brachial Plexus Nerve Block via Interscalene Approach: A Case Report. 3157 94