Anesthesia and Surgery with Mast Cell Disease

Anaphylactic and anaphylactoid reactions during the perioperative period
"Anaphylactic reactions in the peri-operative period are often serious and potentially life-threatening conditions, involving multiple organ systems in which the clinical manifestations are the consequence of the release of preformed mediators from mast cells and basophils. Anaphylaxis is an immune mediated type I allergic reaction following the massive release of mediators from mast cells and basophils as a response to an allergen. Anaphylactoid reactions are defined as those reactions that produce the same clinical picture with anaphylaxis but are not IgE mediated, occur through a direct nonimmune-mediated release of mediators from mast cells and/or basophils or result from direct complement activation. The occurrence of these reactions during anesthesia, although quite rare, remains a major concern for the anesthesiologists."

Perioperative Anaphylaxis
"Perioperative anaphylaxis is a potentially life-threatening and under-recognized event most commonly caused by antibiotics, neuromuscular blocking agents, dyes, latex, and disinfectants. This review provides updates in the epidemiology and pathogenesis of perioperative anaphylaxis, discusses culprit agents, and highlights the tenets of management including a comprehensive allergy evaluation."

Kounis syndrome or allergic coronary vasospasm in a two-year-old
"Kounis syndrome is coronary vasospasm because of mast cell degranulation in the context of an allergic reaction. The syndrome has known associations with several drugs used during anesthesia, including rocuronium and isoflurane."

Rocuronium-induced coronary vasospasm--"Kounis syndrome"
"A 49-year-old male became hypotensive, bradycardic, and suffered myocardial injury during induction of anesthesia with lidocaine, propofol, and rocuronium in the operating room. Coronary arteriography revealed coronary vasospasm in coronary arteries otherwise free of disease. In the ICU, the patient was again administered rocuronium for a procedure with subsequent hypotension, bradycardia, and ST elevation on telemetry that resolved with administration of diphenhydramine and hydrocortisone. An allergic reaction to rocuronium with coronary vasospasm is suspected, suggestive of the Type 1 variant of Kounis syndrome. This is the first report to describe a case of rocuronium-induced Type 1 Kounis syndrome."

Intraoperative "Kounis syndrome" that improved electrocardiography changes and hemodynamic situation after administering nitroglycerine
"A 58-year-old female without cardiovascular risk factors, was going to be operated to repair the rotator cuff. Induction and interscalene brachial plexus block were uneventful, but after her placement for surgery the patient started with severe bronchospasm, hypotension, cutaneous allergic reaction and ST elevation on the electrocardiogram. An anaphylactic shock was suspected and treated but until the perfusion of nitroglycerina was started no electrocardiographic changes resolved. After necessary diagnostic test the final diagnosis was variant I of Kounis syndrome due to cefazolin and rocuronium. Ephinephrine is the cornerstone of treatment for anaphylaxis but should we use it if the anaphylactic reaction is also accompanied by myocardial ischemia? The answer is that we should not use it because myocardial ischemia in this syndrome is caused by vasospasm, so it would be more useful drugs such as nitroglycerin."

Multiple drug allergies: Recommendations for perioperative management
"Life-threatening hypersensitivity reactions are more likely to occur in patients with a history of allergy, atopy, or asthma. Hence, in a patient who presented with a history of multiple drug allergies (MDA), an allergological assessment should be performed prior to surgical procedure. Drug allergies, being one of the causes of catastrophic events occurring in the perioperative period, are of major concern to anesthesiologists. Neuromuscular blocking agents are regularly used during anesthesia and are one of the most common causes of perioperative anaphylaxis. They are estimated to be responsible for 50%-70% of perioperative hypersensitivity reactions. Antibiotics and latex represent the next two groups of drug allergy. Allergic reactions to propofol are rare with an incidence of 1:60,000 exposures. Although intraoperative drug anaphylaxis is rare, it contributes to 4.3% of deaths occurring during general anesthesia. These recommendations discuss pathophysiology of MDA, preoperative evaluation, and anesthesia considerations as well as the prevention and management of allergic reactions in anesthetized patients with a history of MDA."

Perioperative approach of allergic patients
"Severe, generalized allergic reactions called anaphylaxis are estimated to have a mortality of 3.5-4.8%. Adequate recognition and handling of a severe perioperative anaphylactic reaction result in better outcomes, including less hypoxic-ischemic encephalopathy and death. The diagnosis of a perioperative allergic reaction can be difficult as the list of possible culprits of a perioperative allergic reaction is extensive. Making an informed guess on the causative agent and avoiding this agent in future anesthesia procedures is undesirable and unsafe. Therefore, to ensure future patient safety, a thorough investigation following a perioperative allergic reaction is mandatory. A collaborate approach by allergists and anesthesiologists is advised. In this article, we discuss the basic approach of the allergic patient and of patients with a suspected allergy to perioperatively administered medication."

Anaphylaxis During General Anaesthesia
"The incidence of anaphylaxis has been estimated at between 1 in 10 000 and 1 in 20 000 anaesthesias in Australia and 1 in 13 000 anaesthesias in France. In the most recent French epidemiological survey, the compounds most frequently involved in anaphylaxis were muscle relaxants (60%), followed by latex (16%)."

"Activation of humoral and cellular pathways resulting from immunoglobulin E-mediated adverse reactions usually produces characteristic respiratory, cardiovascular and skin responses, but effects can be seen in virtually any system. These responses may occur as isolated clinical events. As a result, an anaphylactic reaction restricted to a single clinical symptom (e.g. bronchospasm, tachycardia) can easily be misdiagnosed. Intra- and postoperative investigations must be performed to confirm the nature of the adverse reaction, the role of the suspected drugs, and to define precise recommendations for future anaesthesias. The patient must be fully informed, and given a detailed written account of the anaphylactic episode, the results of the allergological assessment performed and the resulting recommendations. Furthermore, the patient should be strongly advise to wear a warning bracelet or carry a warning card."

Allergic reactions during anesthesia at a large United States referral center
"Thirty-eight patients were found to have an anaphylactic reaction during anesthesia, of which 18 were immunoglobulin (Ig)E-mediated anaphylactic reactions (likely causative agent identified by skin test), 6 were non-IgE-mediated anaphylactic reactions (elevated tryptase levels and negative skin test), and 14 were probable non-IgE-mediated anaphylactic reactions (tryptase levels normal or not obtained and negative skin test). Of the IgE-mediated anaphylactic reactions, antibiotics were the most prevalent likely causative agent (50%) whereas neuromuscular blocking drugs were implicated as a likely causative agent in 11% of reactions."

Practical guidelines for the response to perioperative anaphylaxis
"Muscle relaxants and antibiotics are the most common drugs that cause perioperative anaphylaxis in Japan, as in many countries. In addition, sugammadex appears to be a primary causative agent."

"Even if there are no skin symptoms, anaphylaxis should be suspected, especially when hypotension resistant to inotropes and vasopressors persists. For improving the diagnostic accuracy of anaphylaxis, it is helpful to collect blood samples to measure histamine/tryptase concentrations immediately after the events and at baseline. The first-line treatment for anaphylaxis is adrenaline. In the perioperative setting, adrenaline should be administered through the intravenous route, which has a faster effect onset and is secured in most cases. Adrenaline can cause serious complications including severe arrhythmias if the appropriate dose is not selected according to the severity of symptoms. The anesthesiologist should identify the causative agent after adverse events. The gold standard for identifying the causative agent is the skin test, but in vitro tests including specific IgE antibody measurements and basophil activation tests are also beneficial. The Working Group of the Japanese Society of Anesthesiologists has developed this practical guide to help appropriate prevention, early diagnosis and treatment, and postoperative diagnosis of anaphylaxis during anesthesia." 

A Practical Approach to Systemic Mastocytosis Complications in Cardiac Surgery: A Case Report and Systematic Review of the Literature
"Twelve studies were included describing cases of patients undergoing cardiac surgery who were diagnosed with systemic mastocytosis. An adverse effect, namely anaphylaxis, has happened in three cases. Different strategies of premedication, intraoperative and postoperative management were used. In our case, the patient was admitted for elective biological aortic valve replacement due to severe aortic stenosis. Intraoperatively, the patient developed an anaphylactic shock during the administration of protamine after separation from the cardiopulmonary bypass. This anaphylaxis reaction was a complication of the pre-existing systemic mastocytosis and could be successfully managed by the administration of epinephrine, antihistamines and corticosteroids. (4) Conclusions: This systematic literature search and case report highlight the importance of careful preoperative planning, as well as coordination between cardiac surgeons, anesthesiologists and hemato-oncological specialists, in patients with rare but complication-prone diseases such as systemic mastocytosis."

Anaphylaxis spares no drug: A report of diclofenac-induced anaphylaxis mimicking post-laparoscopy respiratory complication
"In the post-anesthesia care unit, the patient complained of respiratory difficulty. Even after the supplemental oxygen and in absence of any significant finding on respiratory examination, the patient soon developed severe cardiorespiratory collapse. On evaluation, administration of intravenous diclofenac a few minutes before the event was suspected as the trigger for this anaphylactic response. The patient responded to the injection of adrenaline, and her post-surgical progress over the next two days was uneventful. The retrospective tests done for confirming diclofenac hypersensitivity were found to be positive. No drug, however safe, should be given blindly without proper observation and monitoring. The course of development of anaphylaxis can range from a few seconds to minutes and hence, the earliest recognition and prompt action can be the only deciding factor between life and death for such patients."

Intradermal skin testing in the investigation of suspected anaphylactic reactions during anaesthesia--a retrospective survey
"Sixty-five patients suffered a suspected anaphylactic reaction between 1976 and 2001. In 47 patients skin testing was performed and 43 of these patients had positive skin tests: neuromuscular blockings drugs and succinylcholine more specifically, were the most frequently incriminated drugs. After the anaphylactic reaction 19 patients had surgery on 26 occasions with the use of a skin-test-negative neuromuscular blocking drug; no problems occurred. Skin testing proved to be a reliable tool to investigate suspected anaphylactic reactions during anaesthesia and to guide the future use of neuromuscular blocking drugs."

Timing of skin testing after a suspected anaphylactic reaction during anaesthesia
"A delay of 4 to 6 weeks after a suspected anaphylactic reaction has commonly been recommended before performing skin testing. However, sometimes surgery cannot be delayed, and investigation must be done earlier. Recent recommendations suggest that skin testing can be performed immediately after a reaction."

"Review of the literature did not give a definite answer to the optimal timing of skin testing after a suspected anaphylactic reaction during anaesthesia."

"Only positive skin tests can be taken into account, and there is little safety data to provide confidence in early skin testing. A protocol of how to act if urgent surgery is necessary is suggested."

Suspected Anaphylactic Reactions Associated with Anaesthesia

CONTRAST MEDIA

Current Knowledge and Management of Hypersensitivity to Perioperative Drugs and Radiocontrast Media
"Several mechanisms are implicated, including IgE- and non-IgE-mediated mechanisms. Perioperative anaphylaxis tends to be severe and has a higher mortality rate than anaphylaxis in other settings. This is partly due to factors that impair early recognition of anaphylaxis. Neuromuscular blocking agents, latex containing products, and antibiotics are the most common etiology. Chlorhexidine and dyes are increasingly culprits. The newest emerging cause is sugammadex, which is used for reversal of the effects of steroidal neuromusclar agents, such as rocuronium. Latex-induced allergy is becoming less common than in the 1980s due to primary and secondary prevention measures. Serum tryptase levels during the time of anaphylaxis and skin testing to suspected agents as an outpatient are necessary to confirm the diagnosis. Management includes epinephrine and aggressive fluid therapy. With radiocontrast media allergy, patients with a history of immediate hypersensitivity reactions to radiocontrast media should receive steroid and antihistamine premedication before re-exposure. Because IgE-mediated anaphylaxis to radiocontrast media is rare, there is a universal consensus that routinely skin testing all patients with a past reaction is not effective."

Rapid collapse of the inferior vena cava in a patient with cardiac arrest induced by anaphylactic shock after iodinated contrast medium injection
"Anaphylactic shock to contrast media can progress to cardiac arrest despite appropriate treatment. During anaphylactic shock to contrast media, rapid vasodilation and a massive fluid shift can occur. Here we report a patient who developed cardiac arrest induced by anaphylactic shock to iodinated contrast medium and exhibited rapid collapse of the inferior vena cava (IVC) on enhanced abdominal computed tomography (CT) images. The patient underwent postsurgical unenhanced and contrast-enhanced abdominal CT follow-up of cecum cancer. She had neither allergy nor medical history except for the cancer. She did not complain of any symptoms immediately after completion of the CT. However, she developed anaphylactic shock and pulseless electrical activity cardiac arrest only 2 minutes after finishing the CT despite appropriate treatment. Emergency physicians successfully treated the patient using advanced life support and targeted temperature management."

"The collapsed IVC is a good indicator of hypovolemia in patients with trauma. In this case, we considered that rapid vasodilation and a massive volume shift might have caused the collapsed IVC. This finding suggests the importance of aggressive volume resuscitation as well as epinephrine injection in patients with anaphylactic shock to contrast media. Furthermore, this finding occurred before the onset of clinical symptoms, and there is a possibility that it could be used as an indicator of anaphylactic shock to contrast media. "

A rapid caliber change in the inferior vena cava during multiphasic contrast-enhanced computed tomography may signal an acute anaphylactic reaction to nonionic contrast medium
"Severe anaphylactic reactions to an intravenous nonionic iodine contrast medium (NICM) are uncommon but can result in permanent morbidity or death if not managed appropriately. An anaphylactic reaction to an NICM typically manifests as clinical symptoms that include an itchy nose, sneezing, and skin redness. To our knowledge, a rapid change in the caliber of the inferior vena cava (IVC) during multiphasic contrast-enhanced computed tomography (CT) has not been reported. Here, we report the computed tomographic findings in three cases of hypovolemic shock caused by an anaphylactic reaction to an NICM. We suspect that a decrease in caliber of the IVC during multiphasic contrast-enhanced CT may be a predictor of an allergic-like reaction to an NICM. Patients in whom physicians and radiographers detect a rapid caliber change in the IVC during multiphasic contrast-enhanced CT should be managed carefully. "