Propofol Infusion Syndrome Following Endoscopic Tracheoplasty and Jet Ventilation: Case Report.

OBJECTIVE: We present a case of propofol infusion syndrome (PRIS) following jet ventilation. METHOD: Case report and review of literature. RESULTS: A 70-year-old man required CO2 laser endoscopic tracheoplasty for tracheal and subglottic stenosis due to A-frame deformity. Postoperatively, the patient was reintubated for respiratory distress and propofol was resumed. Over the next two days the patient developed acute kidney injury, leukocytosis, acute primary respiratory acidosis with high anion gap metabolic acidosis, multiple end organ damage, elevated cardiac markers, and worsening lactic acidosis. The patient was recognized as having propofol infusion syndrome and propofol was immediately discontinued and replaced with dexmedetomidine. Unfortunately the patient progressed to multi-organ failure complicated by rhabdomyolysis and distributive intravascular coagulopathy. CONCLUSIONS: Propofol is often used as an anesthetic for jet ventilation during otolaryngologic airway surgery. Propofol related infusion syndrome is an uncommon but life-threatening peri-operative complication that should be considered in any patient with an unusual post-operative recovery characterized by metabolic acidosis, ECG changes, end organ damage, and elevated lactate.

Refractory atrial fibrillation with rapid ventricular response as a heralding sign of propofol infusion syndrome in a patient with COVID-19.

A woman in her 40s was transferred to the medical intensive care unit due to severe COVID-19 infection causing respiratory failure. Her respiratory failure worsened rapidly, requiring intubation and continuous sedation with fentanyl and propofol infusions. She required progressive increases in the rates of the propofol infusion, as well as addition of midazolam and cisatracurium due to ventilator dyssynchrony. To support the high sedative doses, norepinephrine was administered as a continuous infusion. She developed atrial fibrillation with rapid ventricular response, with rates ranging between 180 and 200 s which did not respond to intravenous adenosine, metoprolol, synchronised cardioversion or amiodarone. A blood draw revealed lipaemia, and triglyceride levels were noted to be elevated to 2018. The patient developed high-grade fevers up to 105.3 and acute renal failure with severe mixed respiratory and metabolic acidosis, indicating propofol-related infusion syndrome. Propofol was promptly discontinued. An insulin-dextrose infusion was initiated which improved patient's fevers and hypertriglyceridaemia.

VA-ECMO rescue therapy in propofol infusion syndrome after cardiac surgery.

INTRODUCTION: Propofol infusion syndrome (PRIS) is a rare entity that could lead to profound cardiogenic shock (CS). Mitochondrial toxicity and sympathetic blockade are the mechanisms leading to CS in PRIS. CASE REPORT: We present a 22-year-old woman who developed refractory CS due to PRIS after aortic valve replacement surgery secondary to Coxiella infective endocarditis. She was rescued with VA-ECMO (veno-arterial extracorporeal membrane oxygenation) and was discharged 2 months later with no cardiac dysfunction. DISCUSSION: PRIS diagnosis is difficult even though propofol is frequently used in critical care units. Abrupt refractory CS in patients with recent use of high doses of propofol (> 4 mg/Kg/h) together with rhabdomyolysis should raise the suspicion. Diagnostic confirmation is based on muscle biopsy and fat enzyme analysis. CONCLUSION: Propofol withdrawal and support therapies-including VA-ECMO-are the treatment of choice in severe PRIS. VA-ECMO could increase survival as a bridge to recovery due to reversibility of PRIS.

Síndrome por infusión de propofol en un adolescente / Syndrome due to propofol infusion in an adolescent

Se describe el caso clínico de un adolescente de 18 años de edad, con antecedente de salud aparente, atendido en el cuerpo de guardia del Hospital General Docente Roberto Rodríguez Fernández de Morón, provincia de Ciego de Ávila, con síntomas sugestivos de apendicitis aguda, por lo que fue intervenido quirúrgicamente. Una vez terminado el proceder tuvo parada cardíaca, lo cual se interpretó como un síndrome por propofol. Se decidió transferirlo a la Unidad de Cuidados Intensivos, donde fue evolucionando favorablemente, con mejoría de todos los parámetros. A los 6 días lo trasladaron a la Unidad de Cuidados Intermedios y posteriormente egresó de la institución hospitalaria sin complicaciones.

The case report of an 18 years adolescent with history of apparent health is described. He was assisted in the emergency service of Roberto Rodríguez Fernández Teaching General Hospital from Morón, Ciego de Ávila, with suggestive symptoms of acute appendicitis, reason why he was surgically intervened. Once finished the procedure he had a cardiac arrest, which was interpreted as a syndrome due to propofol. It was decided to referred him to the Intensive Cares Unit, where he had a favorable clinical course, with improvement of all parameters. Six days later he was transferred to the Intermediate Cares Unit and later on he was discharged from the hospital institution without complications.

[Propofol Infusion Syndrome Treated Successfully by Early Diagnosis].

Propofol infusion syndrome (PRIS) is one of the severe complications which occur during continuous venous infusion of propofol, and has a high mortality rate. It is featured by high fever, oliguria, myogloblin urine, acute renal failure, hepatomegaly, fatty liver, and so on. We have experienced a case of PRIS who was saved by prompt changing of sedatives from propofol to midazolam and dexmedetomidine. The patient was an 82-year-old man, who underwent off-pump coronary bypass grafting due to effort angina pectoris. After the operation, he suffered from continuous high fever over 38 ℃, acute renal impairment, and high level of creatine kinase (CK) without CK-MB increment, suggesting PRIS. We promptly changed sedatives from propofol to midazolam and dexmedetomidine, then the patient recuperated from these abnormalities. It is strongly suggested that meticulous observation is necessary during propofol infusion.

Propofol infusion syndrome; are high doses always required? / Síndrome por infusión de propofol; ¿realmente se requieren altas dosis?

Propofol infusion syndrome is a rare condition that mainly affects critically ill patients who receive high doses of this hypnotic for a long time. We describe the case of a patient who presented hepatotoxicity in the immediate postoperative period of two surgeries in which she had received conventional doses of propofol for a short period of time. After studying the patient and monitoring her evolution, we arrived at a differential diagnosis of propofol infusion syndrome due to increased susceptibility. This syndrome should be considered in patients presenting hepatotoxicity in the immediate postoperative period, even when low doses of propofol have been administered.

The Effect of Concomitant Sirolimus and Propofol Therapy on Triglyceride Concentrations in Critically Ill Patients.

BACKGROUND: Sirolimus and propofol are both independently associated with the development of hypertriglyceridemia (HTG) during therapy. To date, there are no published reports describing synergistic or additive drug interaction resulting in HTG with concomitant use of these medications. STUDY QUESTION: To identify the occurrence of HTG in patients receiving concomitant sirolimus and propofol infusion therapy. METHODS: Adult patients receiving sirolimus and a continuous propofol infusion for at least 12 hours from January 2005 to August 2009 were retrospectively evaluated. Data included Acute Physiology and Chronic Health Evaluation II score, weight, length of propofol therapy, and baseline triglyceride (TG) concentrations. The major outcome was incidence of HTG (TGs ≥500 mg/dL). Minor outcomes included the change in TG concentration from therapy initiation and manifestations of propofol-related infusion syndrome (PRIS). RESULTS: Sixteen patients were included in the analysis, with 8 (50%) of the patients developing HTG. The patients in this case series had the following mean values: Acute Physiology and Chronic Health Evaluation II score of 20.2 ± 5.3, weight of 76.3 ± 21.2 kg, and baseline TG concentrations of 181.3 ± 89.7 mg/dL. Indications for sirolimus therapy included hematopoietic stem-cell transplantation (n = 15) and heart transplantation (n = 1). Mean length of propofol infusion was 99.8 ± 88.5 hours. The mean TG concentration during infusion was 515.6 ± 468.1 mg/dL. Fourteen (87.5%) patients had an increase of ≥100 mg/dL, 12 (75%) patients had an increase of ≥200 mg/dL, and 6 (37.5%) patients had an increase of ≥300 mg/dL in TG concentrations during therapy. Eleven patients developed one manifestation of PRIS, excluding HTG, and one patient had more than 2 new onset PRIS manifestations during propofol therapy. CONCLUSIONS: Coadministration of propofol and sirolimus can potentially result in HTG, which may warrant more frequent monitoring. Further analysis is needed to examine the mechanism and clinical impact of this interaction.

Síndrome por Infusión de propofol en un caso de estatus epiléptico refractario / Propofol infusion syndrome in a refractory epileptic status case

INTRODUCCIÓN: El síndrome por infusión de propofol (SIP) es una reacción adversa poco frecuente, pero potencialmente letal descrita por la utilización de dicho fármaco en infusión intravenosa (IV) continua. El diagnóstico se basa en la combinación de acidosis metabólica, rabdomiolisis, hiperkalemia, hepatomegalia, insuficiencia renal, hiperlipidemia, arritmias e insuficiencia cardiaca rápida mente progresiva. OBJETIVO: Presentación de un caso clínico de SIP y revisión de literatura. CASO CLÍNICO: Paciente femenino de 6 años de edad con antecedentes de epilepsia secundaria a extensa alteración del desarrollo cortical hemisférico derecho. Presentó estatus epiléptico refractario que requirió ingreso a Unidad de Cuidados Intensivos para soporte vital y tratamiento, el que incluyó como terapia de tercera línea infusión intravenosa continua de propofol en dosis progresivas hasta alcanzar una tasa 10 mg/kg/h. Cursó con compromiso hemodinámico y a las 24 h de iniciado el tratamiento se observó alza de la creatinifosfokinasa (CK), acidosis metabólica y lactacidemia elevada, y luego de descartar otras causas se planteó el diagnóstico de SIP por lo que se suspendió la droga, logrando estabilización hemodinámica a las 24 h. DISCUSIÓN: El diagnóstico de SIP es complejo, se debe considerar en pacientes que estén recibiendo el fármaco y presenten acidosis metabólica o insuficiencia cardiaca. Los factores que más influyen en la mortalidad son la dosis acumulativa de la droga, la presencia de fiebre y lesión encéfalo craneana. En el caso descrito la paciente recibió una dosis mayor a 4 mg/ kg/h que es la dosis máxima recomendada y respondió favorablemente luego de 12 h después de la suspensión del fármaco.

INTRODUCTION: Propofol Infusion Syndrome (PRIS) is a rare but potentially lethal adverse reaction secondary to the continuous intravenous infusion of this drug. The diagnosis is based on the com bination of metabolic acidosis, rhabdomyolysis, hyperkalemia, hepatomegaly, renal failure, hyperli pidemia, arrhythmias, and rapidly progressive heart failure. OBJECTIVE: To report a case of PRIS and literature review. CLINICAL CASE: A 6-year-old female patient with history of epilepsy secondary to large malformation of cortical development of the right hemisphere. The patient presented a refractory status epilepticus that required admission to the Intensive Care Unit for life support and treatment, which included continuous intravenous infusion of propofol at 10 mg/kg/h. She developed hemo dynamic instability, and after 24 h of treatment an increase of creatine phosphokinase (CPK) levels, metabolic acidosis and elevated lactacidemia were observed. After ruling out other causes, PRIS was diagnosed; therefore, the drug was suspended, achieving hemodynamic stabilization after 24 hours. DISCUSSION: The diagnosis of PRIS is complex and should be considered in patients who are receiving this drug and present metabolic acidosis or heart failure. The factors that most influence mortality are the cumulative dose of the drug, the presence of fever, and cranial brain injury. In the case described, the patient received a dose higher than 4 mg/kg/h, which is the maximum recommended dose, and responded favorably 12 hours after stopping the drug.

Encephalopathy associated with propofol infusion syndrome: A case report.

INTRODUCTION: Propofol infusion syndrome (PRIS) is a rare but potentially fatal complication of propofol infusion. It is clinically characterized by metabolic acidosis, refractory bradycardia, rhabdomyolysis, renal failure, hyperlipidemia, and hepatomegaly. Brain lesion was only reported once in a pediatric patient. We present the 1st adult case with colon polyp and cancer who was diagnosed with PRIS. Her brain magnetic resonance imaging (MRI) and computed tomography (CT) scans reveal prominent bilateral brain lesions, matching with the proposed pathophysiologic mechanism of the syndrome. The patient received prompt acidosis correction and cardiorespiratory support. At last, she died from refractory circulatory failure. CONCLUSION: It may be necessary to order a prompt neuroimaging examination in patients suspected with PRIS to judge whether brain lesions exist or not.

Propofol Related Infusion Syndrome: Ultrastructural Evidence for a Mitochondrial Disorder.

OBJECTIVE: The objective of this report of a fatal propofol-related infusion syndrome in a young adult was to present-to our knowledge for the first time-direct ultrastructural evidence for the central role of mitochondrial damage in the pathogenesis of this syndrome. DATA SOURCES: Histological and electron microscopical analysis of liver, skeletal, and heart muscle obtained by autopsy and blood obtained from patient. STUDY SELECTION: Case report. DATA EXTRACTION: In addition to conventional macroscopical and histological investigations, electron-microscopical analysis of myocardial- and skeletal muscle and liver tissue obtained at autopsy from a young man was performed in order to search for ultrastructural changes of mitochondria. Acylcarnitine concentrations of his blood were determined by ultra-high performance liquid chromatography mass spectrometry. DATA SYNTHESIS: A 19-year-old male was admitted with acute left-side hemiparesis. The patient was intubated, then propofol infusion started, and a craniotomy was performed to remove an intracerebral hematoma. In the postoperative period, the patient presented with elevated intracranial pressure and brain edema. After repeat surgery, the patient showed impaired systolic left ventricular function, increasing fever, anuria, hyperkalemia, and metabolic acidosis, and he finally expired. Electron microscopy revealed dark, electron dense amorphous structures associated with mitochondria in heart muscle and liver tissue obtained at autopsy. Peripheral blood analysis revealed increased levels of acetyl-, propionyl-, butyryl-, malonyl-, and valeryl-carnitine as an indicator for propofol-related infusion syndrome, as well as for propofol-mediated inhibition of free fatty acid uptake into mitochondria, affecting beta-oxidation. CONCLUSIONS: Electron dense bodies found in association with mitochondria in muscle and liver cells probably correspond to accumulation of free fatty acid provide direct morphological evidence for the mitochondrial damage in propofol-related infusion syndrome.

Síndrome de infusión por propofol en el adulto / Propofol infusion syndrome

Propofol infusion syndrome is a rare but lethal complication of propofol administration. The most common clinical presentation is high anion-gap metabolic acidosis, heart failure, rhabdomyolysis and hyperkalemia in patients receiving a prolonged propofol infusion. It has an estimated incidence of 1,1% in patients receiving propofol and its mortality varies between 18 and 81%. The most important risk factor is an infusion over 4 mg/kg/h of propofol and a critically sick patient. Management of a diagnosed PRIS is based on immediate discontinuation of propofol and support therapy.

El síndrome de infusión de propofol es una complicación rara, pero posiblemente letal de la administración de propofol (Propofol Infusion Syndrome, PRIS) Generalmente, se presenta como una acidosis metabólica con anion Gap aumentado, falla cardiaca rápidamente progresiva, rabdomiólisis e hiperkalemia, junto con el antecedente claro de una infusión prolongada de la droga. La incidencia se estima en un 1,1% en los pacientes que reciben propofol y su mortalidad varía entre el 18 al 81%. Los factores de riesgo son: una infusión de propofol mayor a 4 mg/kg/h y pacientes críticos. El tratamiento se basa en la suspensión de la droga y medidas de soporte hemodinámico.

Síndrome por infusión de propofol: reporte de un caso / Propofol infusion syndrome: a case report

El propofol es un anestésico ampliamente utilizado en las unidades de cuidados intensivos. Su empleo puede provocar un efecto adverso poco frecuente pero extremadamente grave, conocido como síndrome por infusión de propofol (SIP), caracterizado por inestabilidad hemodinámica, rabdomiólisis, acidosis láctica y disfunción multiorgánica. Presentamos un caso de SIP con desenlace fatal, discutiendo su fisiopatología, clínica y consideraciones a tener en cuenta al emplear propofol.

Propofol is a widely used anesthetic in the Intensive Care Unit. At times it may cause rather an unusual adverse reaction known as propofol infusion syndrome (PRIS), characterized by hemodynamic instability, rhabdomyolysis, lactic acidosis, and multiple-organ dysfunction. The study presents a case of PRIS with fatal outcome, including a discussion of its physiopathology, clinical aspects and considerations to be borne in mind when using propofol.

O propofol é um anestésico amplamente utilizado nas Unidades de Cuidados Intensivos. Sua utilização pode provocar um efeito adverso pouco frequente mas extremamente grave conhecido como síndrome por infusão de propofol (SIP), caracterizado por instabilidade hemodinâmica, rabdomiólise, acidose lática e disfunção múltipla de órgãos. Apresentamos um caso fatal de SIP e discutimos a fisiopatologia, clínica e os aspectos que devem ser considerados quando se utiliza esta droga.

[Malignant hyperthermia syndrome in the intensive care unit : Differential diagnosis and acute measures]. / Maligne hypertherme Syndrome auf der Intensivstation : Differenzialdiagnostik und Akutmaßnamen.

Malignant hyperthermia is a life-threatening disease caused by derangement of the autonomic nerve system and hypermetabolism of the peripheral musculature. Commonly body core temperatures of more than 40 °C will be found in this disease which is caused mostly by psychopharmacological drugs like antidepressants, neuroleptics but also antibiotics, pain killers, anti-Parkinson drugs, and volatile anesthetics. The inducers of malignant hyperthermia interact with postsynaptic receptors (serotonin, anticholinergics) or muscular intracellular structures responsible for calcium utilization (volatile anesthetics, succinylcholine). Rarely malignant hyperthermia is a consequence of mental stress or vigorous exercise and or heat. Malignant hyperthermic syndromes lead to a severe dysbalance of the autonomic nerve system accompanied by rhabdomyolysis, disseminated intravascular coagulopathy, and finally multi-organ failure. Accordingly, medical management is primarily directed to stabilize vital functions, withdrawal of the causing drug, and if possible antagonizing toxic substances. The leading symptom hyperthermia needs to be treated physically with available cooling systems.

[Fatal hyperpyrexia in an adolescent patient with severe burns after a traffic accident]. / Letale Hyperpyrexie bei einem jugendlichen Schwerbrandverletzten nach Verkehrsunfall.

After a motorcycle accident a 16-year-old patient suffered severe burns to 40.5 % of the total body surface area (TBSA) of which 37 % were deep subdermal burns. After tangential and partly epifascial necrosectomy, Integra® was used as a temporary dermis replacement material for the lower extremities, combined with extensive negative pressure wound therapy (NPWT). In the further course of the treatment the patient developed uncontrollable hyperpyrexia with a fatal outcome. Possible influencing factors, such as the dermis replacement material combined with NPWT over large areas as well as the differential diagnoses propofol infusion syndrome, heatstroke and malignant hyperthermia are discussed.