Intraoperative Harlequin Syndrome

Mashour, George A. MD, PhD; Levine, Wilton MD; Ortiz, Vilma E. MD

doi: 10.1213/01.ANE.0000190793.83526.0F
Letters to the Editor: Letters & Announcements

Department of Anesthesia and Critical Care, Massachusetts General Hospital, Harvard Medical School, Boston, MA,

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To the Editor:

The term “Harlequin syndrome” was introduced by Lance et al. (1) to describe a rare autonomic dysfunction characterized by hemifacial sweating and flushing. The syndrome may be triggered by thermal, gustatory, emotional, or exertional stimuli and may also be found in conjunction with Horner’s syndrome (1-3). Ocular findings in Horner’s are associated with lesions at the level of T1, whereas sudomotor and vasomotor findings of Harlequin syndrome are thought to be associated with the level of T2 and T3 (3,4). In the perioperative setting, Harlequin syndrome has been reported postoperatively after the resection of a neck mass as well as after internal jugular venous catheter placement (5,6). It is thought that interrupted sympathetic outflow results in an ipsilateral flushing and sweating defect. We report a case of Harlequin syndrome in a 77-yr-old female during pancreatic surgery that resolved spontaneously on emergence from general anesthesia (Fig. 1). The patient had no prior autonomic dysfunction and reported no postoperative events. An asymmetrical epidural block demonstrated preoperatively (T2 on left, T4 on right) was suspected as the cause, but the etiology remains unclear. This is the first report of intraoperative Harlequin syndrome.

George A. Mashour, MD, PhD

Wilton Levine, MD

Vilma E. Ortiz, MD

Department of Anesthesia and Critical Care, Massachusetts General Hospital, Harvard Medical School, Boston, MA,

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1. Lance JW, Drummond PD, Gandevia SC, Morris JG. Harlequin syndrome: the sudden onset of unilateral facial flushing and sweating. J Neurol Neurosurg Psychiatry 1988;51:635-42.
2. Fallon KE, May JJ. Harlequin syndrome in two athletes. Br J Sports Med 2005;39e1.
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6. Coleman PJ, Goddard JM. Harlequin syndrome following internal jugular vein catheterization in an adult under general anesthetic. Anesthesiology 2002;97:1041.
© 2006 International Anesthesia Research Society