Harlequin Syndrome

PROBLEM: Despite advances in Venoarterial Extracorporeal Membrane Oxygenation (VA-ECMO), a significant mortality rate persists due to complications. The non-physiological blood flow dynamics of VA-ECMO may lead to neurological complications and organ ischemia. Continuous retrograde high-flow oxygenated blood enters through a return cannula placed in the femoral artery which opposes the pulsatile deoxygenated blood ejected by the left ventricle (LV), which impacts upper body oxygenation and subsequent hyperoxemia...

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PURPOSE: The Harlequin syndrome may occur in patients treated with venoarterial extracorporal membrane oxygenation (VA-ECMO), in whom blood from the left ventricle and the ECMO system supply different parts of the body with different pa CO2 -levels. The purpose of this study was to compare two variants of pa CO2 -analysis to account for the Harlequin syndrome during apnea testing (AT) in brain death (BD) determination. MATERIALS AND METHODS: Twenty-seven patients (median age 48 years, 26-76 years; male n = 19) with VA-ECMO treatment were included who underwent BD determination...

No abstract text is available yet for this article.

No abstract text is available yet for this article.

No abstract text is available yet for this article.

No abstract text is available yet for this article.

Cardiogenic shock (CS) is a clinical condition characterized by inadequate tissue perfusion in the setting of low cardiac output. CS is the leading cause of death following acute myocardial infarction (AMI). Several temporary mechanical support devices are available for hemodynamic support in CS until clinical recovery ensues or until more definitive surgical procedures have been performed. Veno-arterial (VA) extracorporeal membrane oxygenation (ECMO) has evolved as a powerful treatment option for short-term circulatory support in refractory CS...

Background and Objectives : Sensory ganglionopathy is a rare neurological disorder caused by degeneration of the neurons composing the dorsal root ganglia. It manifests as various sensory disturbances in the trunk, proximal limbs, face, or mouth in a patchy and asymmetrical pattern. Harlequin syndrome is characterized by unilateral flushing and sweating of the face, neck, and upper chest, concurrent with contralateral anhidrosis. Here, we present and discuss a clinical case of sarcoidosis-associated ganglionopathy and Harlequin syndrome...

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BACKGROUND: Harlequin syndrome is a rare disorder caused by autonomic nervous system dysfunction. It manifests as asymmetric facial flashing and sweating with contralateral anhidrosis. It may be primary (idiopathic) with a benign course or can occur secondary to structural abnormalities or iatrogenic factors. To our knowledge, there has been no report of idiopathic Harlequin syndrome published from Ethiopia. We are reporting this case since it signifies the existence of idiopathic Harlequin syndrome in our setting and the need to properly diagnose this condition...

The utilization of extracorporeal membrane oxygenation (ECMO) for cardiopulmonary support continues to increase globally, with > 190,000 ECMO cases reported to the international Extracorporeal Life Support Organization Registry. The present review aims to synthesize important contributions to the literature surrounding the management of mechanical ventilation, prone positioning, anticoagulation, bleeding complications, and neurologic outcomes for infants, children, and adults undergoing ECMO in 2022. Additionally, issues related to cardiac ECMO, Harlequin syndrome, and anticoagulation during ECMO will be discussed...

Surgical resection of obstructive tracheal tumors can be challenging to cardiothoracic surgeons and anesthesiologists. It is often difficult in these cases to maintain oxygenation by face mask ventilation during induction of general anesthesia. Also, the extent and location of these tracheal tumors can preclude conventional induction of general anesthesia and subsequent successful endotracheal intubation. Peripheral cardiopulmonary bypass (CPB) under local anesthesia and mild intravenous sedation may be safe to support the patient until securing a definitive airway...

INTRODUCTION: Pulmonary hemorrhage is a life-threatening complication of VA-ECMO occasionally presenting with Harlequin syndrome. CASE REPORT: We present a case of a VA-ECMO patient complicated with pulmonary hemorrhage, complete right lung atelectasis and differential hypoxia refractory to conventional treatment including optimal mechanical ventilation and bronchoscopy interventions. Patient was successfully managed by conversion of VA to VAV-ECMO. DISCUSSION: Pulmonary hemorrhage and atelectasis treatment in a VA-ECMO patient includes transfusion, hold and reversal of anticoagulation, bronchoscopy interventions and optimization of VA-ECMO and ventilator support...

Harlequin ichthyosis (HI) is a rare, life-threatening genodermatosis that is characterized by thick, scaly, hyperkeratotic plaques throughout the skin and is typically associated with severe ectropion, eclabium, flexion contractures, and dysplastic ears. HI is thought to be caused by a loss-of-function mutation in the ABCA12 gene. It has traditionally been thought to be difficult to treat, as there are currently no treatments available that are approved by the Food and Drug Administration (FDA). We present a case of a 15-year-old boy with HI and a complex medical history who was treated with a trial of off-label ustekinumab...

A 56-year-old man presented to the clinic with episodic headaches for several years which had been worsening over a few months prior to the presentation. He described headache as sharp, stabbing pain around the left eye associated with nausea, vomiting, photophobia, and phonophobia lasting for hours associated with flushing on the left side of the face. The picture of his face during these episodes showed flushing of the left side of the face, ptosis of the right eyelid, and miosis (panel A). Flushing in his face would resolve with the abortion of the headache...

INTRODUCTION: ExtraCorporeal Membrane Oxygenation (ECMO) in pediatric patients with COVID-19 has a survival rate similar to adults. Occasionally, patients may need to be cannulated by an ECMO team in a referring hospital and transported to an ECMO center. The ECMO transport of a COVID-19 patient has additional risks than normal pediatric ECMO transport for the possible COVID-19 transmissibility to the ECMO team and the reduction of the ECMO team performance due to the need of wearing full personal protective equipment...

Autosomal recessive congenital ichthyosis (ARCI) is a non-syndromic congenital disorder of cornification characterized by abnormal scaling of the skin. The three major phenotypes are lamellar ichthyosis, congenital ichthyosiform erythroderma, and harlequin ichthyosis. ARCI is caused by biallelic mutations in ABCA12 , ALOX12B , ALOXE3 , CERS3 , CYP4F22 , NIPAL4 , PNPLA1 , SDR9C7 , SULT2B1 , and TGM1 . The most severe form of ARCI, harlequin ichthyosis, is caused by mutations in ABCA12 . Mutations in this gene can also lead to congenital ichthyosiform erythroderma or lamellar ichthyosis...

Harlequin syndrome is a rare syndrome characterized by hemifacial flushing and altered facial sweating, with only a few case reports related to intercostal blockades. We present a case of Harlequin syndrome in a 65-year-old woman after intercostal blockade for video-assisted thoracoscopic lobectomy. One hour postoperatively, the patient became nauseated and presented with flushing of the right half of the face with a clear line of demarcation. Within 3 hours, the flushing disappeared. In this case report, we discuss Harlequin syndrome in relation to intercostal blockade and encourage clinicians to consider this syndrome in the differential diagnosis when encountering similar symptoms...

The use of ECPELLA in patients with severe lung disease may result in an unfavorable phenomenon of differential hypoxia. The simultaneous evaluation of three arterial blood samples from different arterial line (right radial artery, left radial artery, ECMO arterial line) in patients at risk of Harlequin syndrome (also called differential hypoxemia (DH)) can localize the "mixing cloud" along the aorta. Focusing the attention on the "mixing cloud" position instead of on isolated flows of Veno-Arterial Extracorporeal Membrane Oxygenation (VA ECMO) and Impella CP makes the decision making easier about how to modify MCSs flows according to the clinical context...