Harlequin syndrome in a pediatric population: a case series.

Harlequin syndrome is a rare condition, presenting with unilateral facial flushing and hyperhidrosis in response to physical exercise, heat or emotional stressors and has scarcely been reported in pediatric patients. It is caused by a dysfunction of vasomotor and sudomotor sympathetic fiber activity inhibiting the ability to flush on the affected side, causing the neurologically intact side to appear red. We present three pediatric cases of this uncommon syndrome, each of them of different origin and displaying distinct associated (neurological) symptoms, and review medical literature. Insight into the anatomical structure of the thoracocervical and facial sympathetic nervous system is pivotal as it dictates symptomatology. About half of Harlequin syndrome cases are complicated with ocular symptoms and a minority may be part of more extensive partial dysautonomias affecting facial sudomotor, vasomotor and pupillary responses, such as Holmes-Adie syndrome and Ross syndrome. Etiology is generally idiopathic, however, cases secondary to surgery, trauma or infection have been described. Considering its predominantly self-limiting nature, treatment is usually unnecessary and should be restricted to incapacitating cases.

Use of beta-blockers for rosacea-associated facial erythema and flushing: A systematic review and update on proposed mode of action.

BACKGROUND: Flushing and erythema are frequent skin symptoms in rosacea. Because their adequate treatment remains a clinical challenge, new treatment options are explored, such as oral ß-blockers. OBJECTIVES: To evaluate the efficacy of oral ß-blockers for rosacea-associated facial flushing and erythema. METHODS: PubMed, Embase, Web of Science, and Cochrane Library were systematically searched, including studies providing original data on the efficacy of oral ß-blockers in rosacea patients with facial flushing and/or persistent erythema. Risk of bias was assessed using the Cochrane Risk of Bias tool, Newcastle-Ottawa scale, and Quality in Prognosis Studies tool. RESULTS: Nine studies evaluating the use of carvedilol, propranolol, nadolol, and ß-blockers in general were included. Articles studying carvedilol and propranolol showed a large reduction of erythema and flushing during treatment with a rapid onset of symptom control. Bradycardia and hypotension were the most commonly described adverse events. LIMITATIONS: Most studies had a retrospective design with a small sample size, and outcome measurement was often subjective. CONCLUSIONS: Oral ß-blockers could be an effective treatment option for patients with rosacea with facial erythema and flushing that does not respond to conventional therapy. Larger prospective trials with objective outcome assessment are needed to validate the promising results of these studies.

Detection of subclinical Harlequin syndrome in pediatric patients.

BACKGROUND: Harlequin syndrome presents as differences in facial coloring due to unilateral flushing. This is the result of the inability to flush on the affected side due to the disruption of vasomotor and sudomotor sympathetic activity. The neurologically intact side appears flushed. A 2°C temperature difference between the flushed and nonflushed sides of the face has been detected in patients presenting with Harlequin syndrome. This difference in temperature might be detectable even in the absence of unilateral flushing, and this subclinical manifestation of the syndrome may occur more often than realized. AIM: To measure and compare the difference in the change in temperature on both sides of the face in patients with a thoracic epidural. METHODS: Fifteen pediatric patients receiving thoracic epidurals for the correction of pectus excavatum via Nuss procedure were enrolled. Temperature measurements on each side of the face were collected at three time points: prior to epidural placement in the holding area, one hour after epidural analgesia had been instituted, and after the patient awakened in the recovery area. The primary outcome is whether or not a temperature difference occurred between the two sides of the face over time. RESULTS: Comparing the pre-op temperature change to post-op temperature change for each side of the face, patient 2 had a large increase in temperature on the left side of the face with a decrease in temperature on the right side of the face. The largest observed difference between the changes in temperature from pre-op to post-op between the right and left sides of the face was 1.85°C in patient 2. This was more than two standard deviations from the mean difference in the patient population. Patient 15 also had a large difference in change in temperature from pre-op to post-op between the right and left sides of the face with an observed difference of 1.14°C, although this was not more than two standard deviations from the mean. None of the patients had unilateral facial flushing. CONCLUSION: Asymmetric effects or distribution of local anesthetic used in thoracic epidurals may result in asymmetric blockade of efferent sympathetic nervous system activity. This may cause differences in temperature between the two sides of the face without unilateral flushing. This phenomenon has previously been termed subclinical Harlequin syndrome. Subclinical Harlequin syndrome may be more common than anticipated and may be detected by comparing temperature differences in patients.

[Harlequin syndrome after scoliosis surgery]. / Harlekinsyndrom nach Skoliosechirurgie.

Harlequin syndrome is a rare combination of symptoms, characterized by unilateral facial anhidrosis and paleness on the affected side, becoming obvious by contralateral flushing mainly during sports activity. The syndrome is mostly idiopathic, however it is also described as a complication of thoracic surgery, i.e. superior lobectomy. Here, we report on two cases of Harlequin syndrome following scoliosis surgery at the cervicothoracic junction.

Idiopathic harlequin syndrome: a case report and literature review.

Harlequin's syndrome is a rare dysautonomic syndrome of the face characterized by sweating with flush of one side and anhidrosis of the contralateral side. Mostly idiopathic although several secondary cases have been reported in the literature, the purpose of the treatment is mainly aesthetic and functional. We report the case of a patient having harlequin syndrome in its idiopathic form with a literature review.

Harlequin Syndrome in Acute Thalamic Hemorrhage.

Harlequin syndrome is a disorder of the autonomic nervous system. It clinically presents as a distinct line of hemifacial sympathetic denervation. We describe a case of Harlequin syndrome with co-existing central first-order Horner syndrome in the setting of a large thalamic hemorrhage with intraventricular extension.

Risks of substance uses, alcohol flush response, Helicobacter pylori infection and upper digestive tract diseases-An endoscopy cross-sectional study.

This study examines the effects of environmental hazards, including tobacco, alcohol/alcohol flush response, areca nut, and Helicobacter pylori (H pylori) infection on upper digestive diseases. This is a multi-hospital-based endoscopy-survey cross-sectional study. Subjects were received upper endoscopies in outpatient clinics at four hospitals in Taiwan between 2008 and 2013. Biopsy-based methods or urea breath test were used confirm the status of H pylori infection. In total, 8135 subjects were analyzed. Higher cumulative amounts of alcohol consumption were at higher risk of Barrett's esophagus and esophageal squamous cell carcinoma (ESCC), higher cumulative amounts of tobacco consumption were at higher risk of peptic ulcer, and higher cumulative amounts of areca nut consumption were at higher risk of duodenitis. Alcohol flush response was significant risk for reflux esophagitis and Barrett's esophagus (adjusted odds ratio [aOR] = 1.18 and 1.32, 95% confidence interval [CI] = 1.07-1.31 and 1.06-1.65, respectively). H pylori infection was inversely associated with ESCC risk (aOR = 0.20, 95% CI = 0.10-0.40). In addition, H pylori infection was consistently and significantly risk factors for gastrointestinal diseases, including peptic ulcer, gastric adenocarcinoma, and duodenitis (aOR = 5.51, 1.84, and 2.10, 95% CI = 4.85-6.26, 1.03-3.26, and 1.71-2.56, respectively). Besides the cumulative risk of alcohol, tobacco, and areca nut for Barrett's esophagus, ESCC, and peptic ulcer, respectively, presence of facial flushing was the significant risk for reflux esophagitis and Barrett's esophagus. H pylori infection was positively associated with peptic ulcer, gastric adenocarcinoma, and duodenitis, but inversely associated with ESCC.

University Students' Willingness to Assist Fellow Students Who Experience Alcohol-Related Facial Flushing to Reduce Their Drinking.

This study explored bystanders’ willingness to help a friend who flushes when drinking to reduce his/her drinking. Alcohol-related facial flushing is an indicator of an inherited variant enzyme, aldehyde dehydrogenase (ALDH), that impairs alcohol metabolism and increases drinkers’ lifetime risk of certain aerodigestive cancers. Individuals who flush should reduce their alcohol exposure, but they may continue to drink if social pressures and rules of etiquette make not drinking socially risky. The analysis used data from 2912 undergraduate students from 13 universities in southwestern, central and northeastern China from a survey asking how they respond to someone’s flushing in various scenarios. Latent class analysis grouped students by similar responses to flushing. A multinomial logistic regression explored how class membership was associated with knowledge, drinking status, and reactions to one’s own flushing. Five classes were derived from the latent class analysis, ranging from always intervene to mostly hesitate to help; in between were classes of students who were willing to help in some scenarios and hesitant in other scenarios. Only 11.6% students knew the connection between facial flushing and impaired alcohol metabolism, and knowledgeable students were somewhat more likely to assist when they saw someone flushing. In the absence of knowledge, other factors—such as drinking status, the gender of the bystander, the gender of the person who flushed, and degree of friendship with the person who flushed—determined how willing a person was to help someone reduce or stop drinking. Class membership was predicted by knowledge, gender, drinking status, and reactions to one’s own flushing. Of these 4 factors, knowledge and reactions to one’s own flushing could be influenced through alcohol education programs. It will take some time for alcohol education to catch up to and change social and cultural patterns of drinking. Meanwhile, motivational strategies should be developed to increase the willingness of bystanders to assist friends and to create a social expectation that flushers should stop or reduce their drinking.

Association Between Excessive Alcohol Consumption and Echocardiographic Parameters According to the Presence of Flushing Reaction in Korean Men: A Community-Based Study.

BACKGROUND: The objective of this study was to investigate the effect of excessive alcohol consumption on heart reflected by various echocardiographic parameters according to the presence or absence of flushing reaction that might reflect acetaldehyde metabolism. METHODS: A total of 854 Korean men without significant cardiovascular diseases who underwent echocardiography and participated in the Korean Healthy Twin Study were used as subjects of this study. These subjects were classified into 3 categories: nondrinker, moderate drinker (≤196 g/wk), and heavy drinker (>196 g/wk) within 2 strata of flushing reaction to alcohol drinking. Association between echocardiographic measurements and categories of the amount of alcohol consumption considering flushing reaction were evaluated using mixed linear regression model. RESULTS: The proportion of flushers among drinkers was 39.5% (278 of 703). In stratified analysis by flushing reaction, nonflushers showed significantly higher left ventricular mass index (ß: 4.605; 95% CI: 0.966, 8.243) and significantly lower ratio of peak early diastolic velocities (E peak) over peak late diastolic velocities of mitral inflow (ß: -0.103; 95% CI: -0.198, -0.008) in heavy drinkers compared to nondrinkers. Flushers showed significantly higher left atrial (LA) volume index (ß: 2.712; 95% CI: 0.456, 4.968) in heavy drinkers and significantly lower ratio of E peak over the peak early diastolic mitral annular velocities (ß: -0.493; 95% CI: -0.902, -0.085) in moderate drinkers compared to nondrinkers. However, the interaction according to flushing reaction was only statistically significant for the association between alcohol consumption and LA volume index (p for interaction = 0.004). CONCLUSIONS: Alcohol consumption is associated with changes in cardiac structure and function. Such association might be influenced by acetaldehyde metabolism.

Harlequin syndrome following upper lobectomy.

A 58-year-old women developed unilateral facial flushing and sweating on the left side of her face immediately after a right thoracotomy upper lobectomy and paravertebral block. She was diagnosed with Harlequin syndrome in the absence of any other neurological signs or symptoms. She had recovered completely from this episode on follow-up.

Harlequin sign concomitant with Horner syndrome after anterior cervical discectomy: a case of intrusion into the cervical sympathetic system.

Harlequin syndrome is a rare autonomic disorder referring to the sudden development of flushing and sweating limited to one side of the face. Like Horner syndrome, associating miosis, ptosis, and anhidrosis, Harlequin syndrome is caused by disruption of the cervical sympathetic pathways. Authors of this report describe the case of a 55-year-old female who presented with both Harlequin sign and Horner syndrome immediately after anterior cervical discectomy (C6-7) with cage fusion and anterior spondylodesis. They discuss the pathophysiology underlying this striking phenomenon and the benign course of this condition. Familiarity with this unusual complication should be of particular interest for every specialist involved in cervical and thoracic surgery.

Venous Cannula Positioning in Arterial Deoxygenation During Veno-Arterial Extracorporeal Membrane Oxygenation-A Simulation Study and Case Report.

Venoarterial extracorporeal membrane oxygenation (VA-ECMO) is indicated in reversible life-threatening circulatory failure with or without respiratory failure. Arterial desaturation in the upper body is frequently seen in patients with peripheral arterial cannulation and severe respiratory failure. The importance of venous cannula positioning was explored in a computer simulation model and a clinical case was described. A closed-loop real-time simulation model has been developed including vascular segments, the heart with valves and pericardium. ECMO was simulated with a fixed flow pump and a selection of clinically relevant venous cannulation sites. A clinical case with no tidal volumes due to pneumonia and an arterial saturation of below 60% in the right hand despite VA-ECMO flow of 4 L/min was described. The case was compared with simulation data. Changing the venous cannulation site from the inferior to the superior caval vein increased arterial saturation in the right arm from below 60% to above 80% in the patient and from 64 to 81% in the simulation model without changing ECMO flow. The patient survived, was extubated and showed no signs of hypoxic damage. We conclude that venous drainage from the superior caval vein improves upper body arterial saturation during veno-arterial ECMO as compared with drainage solely from the inferior caval vein in patients with respiratory failure. The results from the simulation model are in agreement with the clinical scenario.

The efficacy of oral anticholinergics for sympathetic overactivity in a thoracic surgery clinic.

OBJECTIVE: Little is known of the success rates of oral anticholinergics for the treatment of primary hyperhidrosis and facial blushing as alternatives to surgical intervention. We examine predictors of success with these medications. METHODS: A retrospective review was performed at a single institution, including all patients presenting with symptoms of primary hyperhidrosis, facial blushing, or both from 2004 to 2015. All patients were offered a trial of oral anticholinergics. If oral anticholinergic therapy was not successful, patients were offered surgery. Statistical analyses were performed to compare patients who declined surgery given the trial of oral anticholinergics with those who proceeded with surgery. RESULTS: A total of 381 patients presented with symptoms of primary hyperhidrosis (86.6%), facial blushing (2.4%), or both (11.0%). A total of 230 patients (60.4%) declined surgery after using oral anticholinergics, and 151 patients (39.6%) chose surgery. Patients who declined surgery were more likely to have symptoms of primary hyperhidrosis without facial blushing (89.6% vs 82.1%; P = .02) or have primary symptoms involving the axilla, torso, scalp, or groin. Patients who proceeded with surgery had higher rates of palmar symptoms as a primary site (77.6% vs 61.1%; P = .01) and were more likely to have facial blushing alone or in combination with primary hyperhidrosis. Presentation with palmar symptoms and greater number of prior therapy attempts were independent predictors of proceeding with surgery after controlling for concomitant symptom type and location (P = .01 and P < .0001, respectively). CONCLUSIONS: The majority of patients presenting with sympathetic overactivity decline surgery when a trial of oral anticholinergics is included in the treatment algorithm. Facial blushing and palmar symptoms were each associated with choosing surgery.

Episodes of flushing, dyspnea and hypertension after carotid stenting for severe carotid stenosis.

INTRODUCTION: Carotid angioplasty and stenting (CAS) is a treatment option for carotid stenosis in high surgical risk patients. As it implies instrumentation of the carotid bulb, baroreceptors dysfunction may be provoked with consequent hemodynamic instability. REPORT: An 83-year-old woman treated by CAS presented with episodes of flushing, dyspnea and palpitations accompanied by transitory desaturation, hypertension, agitation and anxiety. Symptoms started 12h after the procedure. Each episode lasted 10 min. Five episodes in 3 days were observed. DISCUSSION: A dysfunction of the carotid baroreceptors and chemoreceptors was hypothesized.