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Postoperative Harlequin Syndrome: Case Report of a Rare but Clinically Striking Condition

Kydes, Ashley, T., MD*; Kelley, Ashley, M., MD; Pandya, Samir, R., MD; Barst, Samuel, M., MD

doi: 10.1213/XAA.0000000000000669
Case Reports

We present a case of a 2-year-old boy who underwent thoracoscopic resection of a left paraspinal mediastinal mass and developed Harlequin syndrome postoperatively. Harlequin syndrome is a rare neurological condition characterized by unilateral hyperhidrosis and erythema of the head and neck. Our discussion highlights this condition and other differential diagnoses that may present similarly in the postoperative period.

From the Departments of *Anesthesiology, Pediatric Anesthesiology, and Pediatric General Surgery, New York Medical College at Westchester Medical Center, Valhalla, New York.

Accepted for publication September 25, 2017.

Funding: None.

The authors declare no conflicts of interest.

Address correspondence to Ashley M. Kelley, MD, Department of Anesthesiology, New York Medical College at Westchester Medical Center, 100 Woods Rd, Valhalla, NY 10595. Address e-mail to

Harlequin syndrome is an autonomic disorder caused by dysfunction in the upper cervical or thoracic sympathetic nervous system pathway. It is characterized by asymmetric, unilateral flushing on the face, neck, and upper thoracic region of the chest with a sharp midline demarcation. It is a rare condition, affecting fewer than 200,000 people in the United States.1 The “Harlequin Sign,” defined as unilateral flushing with or without sweating of the face and neck is usually noted after strenuous activity, exposure to heat, or during emotional duress.

Harlequin syndrome was first described by Neligan and Strand2 in a 1952 Lancet article. The name was inspired by the resemblance of patients’ half-flushed faces to colorful harlequin masks used by the character of the classical Italian theater, “Commedia dell’Arte.”3 Although benign in most cases, it can be distressing for patients and a diagnostic challenge for physicians. As described by Nagasaka et al,4 transient cases of Harlequin syndrome can be caused by procedures including regional anesthetic techniques or attempted vascular access, in addition to surgical procedures.

The patient’s parents have consented to the publication of this case report.

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A healthy 2-year-old boy undergoing workup for a persistent cough was diagnosed with a left-sided paraspinal mass located at T2–6. An extensive workup including serum and urine studies and positron emission tomography-computed tomography scans were performed. The lesion was biochemically inert and its appearance on imaging studies was thought to be most consistent with a ganglioneuroma or a ganglioneuroblastoma. There was no extension of the mass into the neural foramen. The child was scheduled to undergo a thoracoscopic resection of the paraspinal mass with general anesthesia. Standard American Society of Anesthesiologists monitors were applied and a 22-gauge left radial arterial line was placed intraoperatively. After induction, the patient was positioned in the right lateral decubitus position on a forced air warming blanket. An axillary roll was used for positioning and pressure points were padded. The case proceeded uneventfully and the child was extubated without complication at the conclusion of the operation.



On arrival at the postanesthesia care unit, the patient was noted to have a flat, erythematous rash on the right side of his face and thorax. Initially, this was attributed to exposure of the child’s dependent side to the forced air warming blanket. After an hour, it was noted that the rash was not improving, but rather becoming more delineated, with a distinct midline demarcation on his face and upper chest (Figure). At this time, differential causes were discussed between the pediatric anesthesia and pediatric surgical teams, and a diagnosis of Harlequin syndrome was made.

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Harlequin syndrome is caused by an injury to the sympathetic nervous system, particularly in the face and neck. The asymmetric sweating and flushing of skin involves the face, neck, and upper thoracic region. The sympathetic deficit on the denervated side causes flushing of the opposite side to appear more pronounced; thus, the affected side appears pale. It is unclear whether the response of the undamaged side is normal or excessive, but it is believed that it may be the result of the body attempting to compensate for the damaged side and maintain homeostasis.5 Females are affected considerably more often than males, and social embarrassment is the chief factor in seeking medical advice.6

Harlequin syndrome can be classified as primary idiopathic, secondary Harlequin syndrome, or iatrogenic Harlequin syndrome. Most cases of Harlequin syndrome are classified as primary idiopathic, where the cause of the injury is unknown or no structural lesion can be identified.

Fifteen percent of patients display secondary Harlequin syndrome, when the disorder is caused by an underlying disease or structural lesion. Most frequent are neurogenic tumors compressing the sympathetic trunk in the neck region.7 It is important to note that even if the underlying lesion is successfully removed surgically, this does not guarantee that the symptoms of Harlequin syndrome will disappear postoperatively. Other identifiable causes are Pancoast syndrome secondary to spinal invasion by an apical lung tumor, medullary infarction, sympathetic chain compression by an elongated inferior thyroid artery, brachial plexopathies, and dissection of the cervical carotid artery.7 In addition, it is thought that torsion of the thoracic spine can cause blockage of the anterior radicular artery leading to Harlequin syndrome.5

Iatrogenic Harlequin syndrome occurs when there is inadvertent injury to the sympathetic nervous system. This may occur after surgical dissection, central line insertion, or performance of a peripheral nerve block. The phenomenon may be transient or permanent. In this patient, the etiology was a lesion of the sympathetic chain ganglia, caused during resection of the patient’s left-sided T2–T6 paraspinal tumor.

Of importance to the anesthesiologist are the differential diagnoses for unilateral facial flushing. They include a varied etiology, from relatively benign as with Harlequin syndrome, to life threatening such as a cerebral vascular accident. Neurovascular disease, including cerebral vascular accident or intracranial hemorrhage can present with unilateral facial flushing, possibly due to failure of the autonomic nervous system.8 Other less acute diagnoses include malignant neoplasm of the brain or lung, venous or arterial thrombus, trigeminal neuralgia, Frey syndrome, or Horner syndrome. Frey syndrome, or auriculotemporal syndrome, is characterized by recurrent episodes of gustatory flushing and sweating in the cutaneous distribution of the auriculotemporal nerve. It is thought to result from misdirection of regenerated parasympathetic nerve fibers and can occur after parotid surgery or injury, perinatal birth injury, or facial trauma in childhood.9 Similarly, Horner syndrome is characterized by unilateral facial flushing, but is accompanied by ocular phenomena, including ptosis and miosis. The preganglionic fibers responsible for the innervation of the vasomotor reflexes of the face arise from the upper thoracic branches at the level of T2–T3, whereas oculomotor branches arise at T1.10 Horner syndrome can also present in conjunction with Harlequin syndrome. The constellation of symptoms present in a patient will be determined by the extension of the lesion. Involvement of the upper extremity implies injury of the superior thoracic sympathetic ganglia, whereas concomitant Horner syndrome indicates an injury to the superior cervical ganglia.

Transient cases of Harlequin syndrome have been reported secondary to procedures performed by the anesthesiologist. These include neuropraxia of the sympathetic innervation of the face after internal jugular catheterization, administration of extrapleural bupivacaine infusion, high thoracic paravertebral anesthesia, and asymmetrical epidural anesthesia.4,9 All these causes, when applicable, should be investigated by the anesthesiologist. Furthermore, some drugs, especially local anesthetics and prostaglandin E, are thought to enhance harlequin color change expression through their influence on the capillary tone in the peripheral vascular bed.11 This effect is reversible with discontinuation of the drug.

Although idiopathic cases do not require intervention, contralateral sympathectomy can be performed if symptoms are distressing to the patient. However, this surgical procedure may lead to compensatory flushing and sweating in other locations.7

The rash in our patient resolved within several hours in the postoperative period. Microscopic examination of the specimen confirmed the diagnosis of ganglioneuroma. At his 1-month follow-up visit, he was reported to have similar self-limiting episodes after physical exertion. These were accompanied with asymmetrical perspiration. At the patient’s 18-month postoperative visit, his symptoms had not changed.

In summary, Harlequin syndrome is a rare neurological condition characterized by unilateral hyperhidrosis and erythema of the head and neck. It is important for the anesthesiologist to be familiar with the presentation because it may be seen following regional anesthetic techniques or attempted vascular access, in addition to surgical procedures. It may be transient or permanent depending on the etiology and must be included in the differential diagnosis for patients presenting with similar symptoms.

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Name: Ashley T. Kydes, MD.

Contribution: This author helped write and edit the manuscript.

Name: Ashley M. Kelley, MD.

Contribution: This author helped write and edit the manuscript.

Name: Samir R. Pandya, MD.

Contribution: This author helped write and edit the manuscript.

Name: Samuel M. Barst, MD.

Contribution: This author helped write and edit the manuscript.

This manuscript was handled by: Mark C. Phillips, MD.

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