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Anaesthetic management of emergency caesarean section in a parturient with systemic mastocytosis
International Journal of Obstetric Anesthesia (2013) 22, 243–254
CASE REPORTS
www.obstetanesthesia.com
Anaesthetic management of emergency caesarean section in a parturient with systemic mastocytosis F. Ulbrich, H. Engelsta¨dter, N. Wittau, D. Steinmann Department of Anaesthesia and Critical Care Medicine, University Medical Center Freiburg, Freiburg, Germany ABSTRACT Mastocytosis is a rare disorder caused by the proliferation and accumulation of mast cells in various organs. It has a broad variety of clinical manifestations, including cardiovascular collapse. Diverse stimuli trigger the release of vasoactive substances and parturients with systemic mastocytosis are at high risk for precipitating mast cell degranulation. As a result, women with systemic mastocytosis should have an anaesthetic plan for labour and delivery. Anxiety, stress, sleep deprivation, pain and numerous pharmacological agents are all triggers for mast cell degranulation. For pain relief in labour, epidural analgesia is recommended. Pharmacological agents with a high potential for triggering mast cell degranulation should be avoided. This is particularly important in the case of an emergency caesarean section. Resuscitation equipment must be available should life-threatening haemodynamic instability occur during surgery. We report the case of a pregnant woman with systemic mastocytosis who required emergency caesarean section. c 2013 Elsevier Ltd. All rights reserved.
Keywords: Pregnancy; Systemic mastocytosis; Caesarean section; General anaesthesia
Introduction Mastocytosis is a rare disorder of haematopoietic stem cells. The exact incidence is unknown although it is estimated that annually 5–10 new cases per million are diagnosed.1 Mastocytosis is caused by the proliferation and accumulation of mast cells in one or more organs. Frequently affected are the skin and bone marrow; rarely the gastrointestinal tract, liver, spleen and lymph nodes.1,2 Cutaneous mastocytosis, frequently known as urticaria pigmentosa, is the most common form and usually occurs in childhood.1,3 Approximately 10% of patients with urticaria pigmentosa also have systemic manifestations;4 this condition is more common in adults and clinical presentation varies immensely. A number of stimuli such as trauma, mechanical irritation, emotional stress, pain, fluctuating body temperature, nutrients, venom and pharmacological agents trigger the release of histamine, prostaglandin and other vasoactive substances. Minor symptoms include weakness, fatigue, urticaria, pruritus, abdominal cramps, vomiting, diarrhoea, mental confusion and fever.5 Patients Accepted March 2013 Correspondence to: F. Ulbrich, Department of Anaesthesia and Critical Care Medicine, University Medical Center Freiburg, Hugstetter Strasse 55, D-79106 Freiburg, Germany. E-mail address: [email protected]
are at risk of fulminant mast cell degranulation, which can lead to bronchospasm, profound anaphylactoid reactions and even cardiovascular collapse.1,4–6 Parturients with systemic mastocytosis are at particularly high risk for developing mast cell degranulation because of the physiological stress of labour.5 An anaesthetic plan for delivery should be established at the earliest opportunity. We describe the case of a pregnant woman with systemic mastocytosis requiring emergency caesarean section.
Case report A 33-year-old woman with systemic mastocytosis presented to the preoperative assessment clinic at our anaesthetic department at 29 weeks of gestation. A repeat caesarean section was planned at term. The woman reported having multiple exacerbations of her condition during pregnancy with abdominal pain, nausea and vomiting, bronchospasm, tachycardia and low blood pressure. She reported no anaphylactoid reactions. At the time of assessment she was on multiple medications (Table 1) and her condition was stable. Two years previously, the patient delivered a healthy infant by caesarean section under spinal anaesthesia. For this she had received intrathecal hyperbaric bupivacaine with fentanyl together with intravenous midazolam 2 mg for sedation.
244 Table 1
Systemic mastocytosis Current medications
Regular
As required
Budesonide 3 mg oral 8-hourly Prednisolone 7.5 mg oral daily Ranitidine 300 mg oral 12-hourly Budesonide inhalation Prednisolone 200 mg oral Diphenhydramine 50 mg oral Ondansetron 4 mg oral Flunitrazepam 0.5 mg oral Vitamin C 750 mg oral
Postoperatively she had mild pruritus that did not require treatment. After discussion, an anaesthetic plan was formulated for her caesarean delivery. The preferred option was for spinal anaesthesia with the possibility of general anaesthesia, if required. A list of anaesthetic agents considered safe in systemic mastocytosis was placed in her medical notes (Table 2). At 39 weeks of gestation the woman presented as an emergency to the hospital with severe continuous abdominal pain. Agitation made communication difficult and cardiotocography could not be performed. At first she refused medical investigation but was convinced Table 2
of the necessity by her midwife. Auscultation of the fetal heart rate revealed decelerations and on vaginal examination her cervix was 3 cm dilated. Ultrasonography showed no fetal heart rate. The duty anaesthetist was notified of the need for emergency caesarean section 19 min after the patient arrived in the hospital. In the operating room the patient was uncooperative and complained of severe and continuous abdominal pain, possibly due to labour and an acute exacerbation of her systemic mastocytosis. Intravenous midazolam 3 mg was administered for sedation and to facilitate preoxygenation. Baseline blood pressure was 142/72 mmHg and heart rate 128 beats/min. Her weight was estimated to be 76 kg. With thiopental and suxamethonium contraindicated in systemic mastocytosis, general anaesthesia was induced with a fractionated, large dose of propofol (200 + 100 + 100 mg) and tracheal intubation was carried out without the use of a muscle relaxant. Laryngoscopy and intubation were uneventful (Cormack and Lehane grade 1). Cricoid pressure was not applied. After verification of correct tube position by capnography, the obstetricians started the operation, six min after raising the emergency alarm. Anaesthesia was maintained with 1.0% end-tidal concentration of isoflurane in oxygen.
Safety of medications in systemic mastocytosis Safe
Alpha-2 adrenoreceptor agonists Analgesics
Antibiotics Anticholinergics Antihypertensives
Benzodiazepines Catecholamines Hypnotics Local anaesthetics Minerals Muscle relaxants Tocolytics Uterotonics
Volatile agents
Clonidine Alfentanil Fentanyl Sufentanil Remifentanil Piritamid Paracetamol Erythromycin Urapidil Dihydralacine Glyceryl trinitrate Midazolam Flunitrazepam Phenylephrine Ephedrine Propofol Ketamine Amide local anaesthetics Magnesium Vecuronium Cisatracurium Fenoterol
Contraindicated
Uncertain
Codeine Morphine Pethidine Tramadol NSAIDs Metamizole Penicillin Atropine Beta blockers
Etomidine Thiopental Ester local anaesthetics Mivacurium Suxamethonium
Rocuronium
Oxytocin Methylergonovine Prostaglandins Isoflurane Sevoflurane Desflurane
F. Ulbrich et al. Four minutes after incision a lifeless newborn was delivered. Immediate resuscitation was initiated by a team of neonatologists, without success. During surgery the patient was haemodynamically stable. For analgesia she received intravenous piritramid 10.5 mg and clonidine 150 lg and for antibiotic prophylaxis erythromycin 1 g. In addition, we gave intravenous hydrocortisone 200 mg and 6 IU oxytocin as an infusion. Following a somewhat delayed emergence from anaesthesia and uneventful extubation, the patient was transferred to the intensive care unit and monitored for 24 h. Analgesia was continued with intravenous piritramid and oral paracetamol. The subsequent hospital stay was uncomplicated and the patient was discharged on the seventh postoperative day. The patient reported no recall or awareness. At autopsy, severe placental insufficiency was diagnosed as the reason for the baby’s demise. An incidental finding was umbilical cord torsion.
Discussion Systemic mastocytosis is most commonly generated by a point mutation in the gene for the tyrosine kinase receptor Kit D816V.6 Mutations cause activation of mast cell progenitor cells with abnormal growth and differentiation. Pathological mast cell infiltrates may appear in various organs. Systemic mastocytosis is diagnosed by biopsy of skin, bone marrow or other mast cell infiltrates with immunohistochemistry analysis of specimens identifying aberrant surface antigens of mast cells.1,2,6 The severity of the disease is dependent on the degree of systemic involvement and on the location of mast cell infiltration.2 Clinical symptoms are caused by mediator release or organ dysfunction. As the disease can have a benign course without any restrictions to everyday life, diagnosis is often delayed.4 The risk of anaphylactoid reactions is high in all patients irrespective of symptom severity.3 The prevalence of perioperative anaphylactoid reactions is not known, but cases of grade III to IV reactions have been described in the literature.7 Approximately 12% of patients with systemic mastocytosis undergoing surgery sustain complications requiring epinephrine.6 For this reason certain anaesthetic medications which are potent triggers of allergic reaction are contraindicated. With the increased physical and psychological stress of pregnancy, one third of women with systemic mastocytosis experience a worsening of systemic symptoms during pregnancy.5,8 Parturients are at high risk of suffering severe exacerbations: anxiety, stress, lack of sleep, pain and many pharmacological agents are trigger factors for mast cell degranulation. Therefore a tailored anaesthetic plan is necessary with regard to analgesia and anaesthesia for delivery.5 The anaesthetic department should be consulted early to discuss management.
245 Patients should be questioned with respect to individual drug tolerance, trigger factors and clinical symptoms. Existing medical documents can also be requested at this time. The anaesthetist can then prepare an individual plan to minimize the risk of triggering an exacerbation. Some general precautions, such as the creation of a quiet and relaxing atmosphere, are advisable. It is important to inform all medical staff about medications that may cause mast cell degranulation (Table 2).7 Intradermal skin testing as a predictor of drug reaction is valuable in some cases but not mandatory for all patients.6 Baseline serum tryptase is increased in patients with systemic mastocytosis and can be used for monitoring.3,7 Early epidural analgesia is recommended in order to provide optimal labour pain relief to the pregnant woman with systemic mastocytosis.8 As metabolism of ester-linked local anaesthetics produces paraaminobenzoic acid, a possible trigger for an anaphylactoid reaction, amide-linked local anaesthetics are preferable. In patients with systemic mastocytosis both spinal and epidural anaesthesia are appropriate techniques for caesarean section.5,6 Sedative (benzodiazepines or promethazine) and antihistamine premedication is advisable in patients undergoing neuraxial anaesthesia for caesarean section.7 Corticosteroids, as inhibitors of mast cell degranulation and suppressors of cytokine and inflammatory mediators, are recommended to minimize reactions.6 However, some authors state that they fail to suppress anaphylactoid reactions sufficiently.9,10 Ropivacaine has been suggested to be the local anaesthetic of choice for neuraxial anaesthesia. Although some authors avoid bupivacaine, its use seems to be safe and there have been many case reports published where it has been used without complications.6,9 Sudden haemodynamic instability may arise during surgery. Continuous blood pressure monitoring should thus be considered. In case of mast cell degranulation fractionated epinephrine is the first medication of choice. In addition, corticosteroids and antihistamines are indicated for stabilisation. Patients are not candidates for ambulatory surgery; observation for 24 h is indicated, as reactions up to four hours postoperatively have been described.9 In cases of emergency caesarean section, general anaesthesia may be required. Thiopental, frequently used in this emergency situation, is a potent trigger of histamine release and is contraindicated. In contrast, the use of midazolam, propofol, etomidate or ketamine is considered safe.9 Volatile anaesthetics generally do not induce mast cell degranulation and are a good choice for maintaining anaesthesia.9 Fentanyl, sufentanil and remifentanil are all considered safe.7 Piritramid, a synthetic opioid, is routinely used in Germany for postoperative analgesia, has a potency about 0.75 times
246
Systemic mastocytosis
that of morphine and has been used successfully in patients with systemic mastocytosis.6 In contrast, morphine and pethidine are potent histamine-releasing agents which can lead to death in these patients.7 As the depolarizing muscle relaxant, suxamethonium, is a potent allergen and major trigger of allergic reactions, it is contraindicated.7 Benzylisochinoline muscle relaxants can lead to mast cell activation while the steroid muscle relaxants can block histamine-N-methyltransferase causing accumulation of histamine and mast cell degranulation. Therefore, these are contraindicated in patients with systemic mastocytosis. The safe use of the steroid relaxant rocuronium has been published.5,11 However, there are inconsistent data relating to allergic potency.7 Vecuronium and pancuronium are considered to be of medium risk, atracurium and mivacurium of high risk. Cisatracurium is associated with minimal mast cell activation and should be the preferred muscle relaxant.1 In our case a large dose of propofol was administered for induction of general anaesthesia. Consequently, tracheal intubation was performed without a muscle relaxant. Rocuronium was not applied in this situation due to uncertainty regarding its negative effects. However, after a further review of the literature, the risk of anaphylactoid reactions appears to be low and rocuronium should be safe for general anaesthesia in these patients.11 In this case the anaesthetist’s intention was to minimize the risk of degranulation as much as possible. Other than the fact that the patient was pregnant, no other indication of difficult airway was observed and so a difficult tracheal intubation was not expected. In summary, parturients with systemic mastocytosis are a particular challenge for anaesthetists. A well-structured anaesthetic plan and back-up plan should be made and communicated to all involved. During labour, life-
0959-289X/$ - see front matter c 2013 Elsevier Ltd. All rights reserved. http://dx.doi.org/10.1016/j.ijoa.2013.03.011
threatening complications may occur and anaesthesia is associated with increased risk of triggering an acute exacerbation. Anaesthetic management should focus on avoidance of trigger factors. Glucocorticoids, antihistamines and epinephrine should be ready for the duration of hospital stay, especially during the critical periods of labour and the early postpartum period.
References 1. Ahmad N, Evans P, Lloyd-Thomas AR. Anesthesia in children with mastocytosis – a case based review. Paediatr Anaesth 2009;19:97–107. 2. Amon U, Hartmann K, Horny HP, Nowak A. Mastocytosis – an update. J Dtsch Dermatol Ges 2010;8:695–711. 3. Goldfinger MM, Sandadi J. Undiagnosed systemic mastocytosis in a teenager revealed during general anesthesia. Paediatr Anaesth 2010;20:290–1. 4. Vaughan ST, Jones GN. Systemic mastocytosis presenting as profound cardiovascular collapse during anaesthesia. Anaesthesia 1998;53:804–7. 5. Villeneuve V, Kaufman I, Weeks S, Deschamps A. Anesthetic management of a labouring parturient with urticaria pigmentosa. Can J Anaesth 2006;53:380–4. 6. Chaar CI, Bell RL, Duffy TP, Duffy AJ. Guidelines for safe surgery in patients with systemic mastocytosis. Am Surg 2009;75:74–80. 7. Konrad FM, Unertl KE, Schroeder TH. Mastocytosis. A challenge in anaesthesiology. Anaesthesist 2009;58:1239–43. 8. Worobec AS, Akin C, Scott LM, Metcalfe DD. Mastocytosis complicating pregnancy. Obstet Gynecol 2000;95:391–5. 9. Konrad FM, Schroeder TH. Anaesthesia in patients with mastocytosis. Acta Anaesthesiol Scand 2009;53:270–1. 10. Lerno G, Slaats G, Coenen E, Herregods L, Rolly G. Anaesthetic management of systemic mastocytosis. Br J Anaesth 1990;65:254–7. 11. Hinkelbein J, Feller-Heppt G, Lenz C, Hensel M, Frietsch T. Mastocytosis: pathology and practical implications for anesthesia. Anaesth Intensivmed 2009;50:508–18.
CASE REPORTS
www.obstetanesthesia.com
Anaesthetic management of emergency caesarean section in a parturient with systemic mastocytosis F. Ulbrich, H. Engelsta¨dter, N. Wittau, D. Steinmann Department of Anaesthesia and Critical Care Medicine, University Medical Center Freiburg, Freiburg, Germany ABSTRACT Mastocytosis is a rare disorder caused by the proliferation and accumulation of mast cells in various organs. It has a broad variety of clinical manifestations, including cardiovascular collapse. Diverse stimuli trigger the release of vasoactive substances and parturients with systemic mastocytosis are at high risk for precipitating mast cell degranulation. As a result, women with systemic mastocytosis should have an anaesthetic plan for labour and delivery. Anxiety, stress, sleep deprivation, pain and numerous pharmacological agents are all triggers for mast cell degranulation. For pain relief in labour, epidural analgesia is recommended. Pharmacological agents with a high potential for triggering mast cell degranulation should be avoided. This is particularly important in the case of an emergency caesarean section. Resuscitation equipment must be available should life-threatening haemodynamic instability occur during surgery. We report the case of a pregnant woman with systemic mastocytosis who required emergency caesarean section. c 2013 Elsevier Ltd. All rights reserved.
Keywords: Pregnancy; Systemic mastocytosis; Caesarean section; General anaesthesia
Introduction Mastocytosis is a rare disorder of haematopoietic stem cells. The exact incidence is unknown although it is estimated that annually 5–10 new cases per million are diagnosed.1 Mastocytosis is caused by the proliferation and accumulation of mast cells in one or more organs. Frequently affected are the skin and bone marrow; rarely the gastrointestinal tract, liver, spleen and lymph nodes.1,2 Cutaneous mastocytosis, frequently known as urticaria pigmentosa, is the most common form and usually occurs in childhood.1,3 Approximately 10% of patients with urticaria pigmentosa also have systemic manifestations;4 this condition is more common in adults and clinical presentation varies immensely. A number of stimuli such as trauma, mechanical irritation, emotional stress, pain, fluctuating body temperature, nutrients, venom and pharmacological agents trigger the release of histamine, prostaglandin and other vasoactive substances. Minor symptoms include weakness, fatigue, urticaria, pruritus, abdominal cramps, vomiting, diarrhoea, mental confusion and fever.5 Patients Accepted March 2013 Correspondence to: F. Ulbrich, Department of Anaesthesia and Critical Care Medicine, University Medical Center Freiburg, Hugstetter Strasse 55, D-79106 Freiburg, Germany. E-mail address: [email protected]
are at risk of fulminant mast cell degranulation, which can lead to bronchospasm, profound anaphylactoid reactions and even cardiovascular collapse.1,4–6 Parturients with systemic mastocytosis are at particularly high risk for developing mast cell degranulation because of the physiological stress of labour.5 An anaesthetic plan for delivery should be established at the earliest opportunity. We describe the case of a pregnant woman with systemic mastocytosis requiring emergency caesarean section.
Case report A 33-year-old woman with systemic mastocytosis presented to the preoperative assessment clinic at our anaesthetic department at 29 weeks of gestation. A repeat caesarean section was planned at term. The woman reported having multiple exacerbations of her condition during pregnancy with abdominal pain, nausea and vomiting, bronchospasm, tachycardia and low blood pressure. She reported no anaphylactoid reactions. At the time of assessment she was on multiple medications (Table 1) and her condition was stable. Two years previously, the patient delivered a healthy infant by caesarean section under spinal anaesthesia. For this she had received intrathecal hyperbaric bupivacaine with fentanyl together with intravenous midazolam 2 mg for sedation.
244 Table 1
Systemic mastocytosis Current medications
Regular
As required
Budesonide 3 mg oral 8-hourly Prednisolone 7.5 mg oral daily Ranitidine 300 mg oral 12-hourly Budesonide inhalation Prednisolone 200 mg oral Diphenhydramine 50 mg oral Ondansetron 4 mg oral Flunitrazepam 0.5 mg oral Vitamin C 750 mg oral
Postoperatively she had mild pruritus that did not require treatment. After discussion, an anaesthetic plan was formulated for her caesarean delivery. The preferred option was for spinal anaesthesia with the possibility of general anaesthesia, if required. A list of anaesthetic agents considered safe in systemic mastocytosis was placed in her medical notes (Table 2). At 39 weeks of gestation the woman presented as an emergency to the hospital with severe continuous abdominal pain. Agitation made communication difficult and cardiotocography could not be performed. At first she refused medical investigation but was convinced Table 2
of the necessity by her midwife. Auscultation of the fetal heart rate revealed decelerations and on vaginal examination her cervix was 3 cm dilated. Ultrasonography showed no fetal heart rate. The duty anaesthetist was notified of the need for emergency caesarean section 19 min after the patient arrived in the hospital. In the operating room the patient was uncooperative and complained of severe and continuous abdominal pain, possibly due to labour and an acute exacerbation of her systemic mastocytosis. Intravenous midazolam 3 mg was administered for sedation and to facilitate preoxygenation. Baseline blood pressure was 142/72 mmHg and heart rate 128 beats/min. Her weight was estimated to be 76 kg. With thiopental and suxamethonium contraindicated in systemic mastocytosis, general anaesthesia was induced with a fractionated, large dose of propofol (200 + 100 + 100 mg) and tracheal intubation was carried out without the use of a muscle relaxant. Laryngoscopy and intubation were uneventful (Cormack and Lehane grade 1). Cricoid pressure was not applied. After verification of correct tube position by capnography, the obstetricians started the operation, six min after raising the emergency alarm. Anaesthesia was maintained with 1.0% end-tidal concentration of isoflurane in oxygen.
Safety of medications in systemic mastocytosis Safe
Alpha-2 adrenoreceptor agonists Analgesics
Antibiotics Anticholinergics Antihypertensives
Benzodiazepines Catecholamines Hypnotics Local anaesthetics Minerals Muscle relaxants Tocolytics Uterotonics
Volatile agents
Clonidine Alfentanil Fentanyl Sufentanil Remifentanil Piritamid Paracetamol Erythromycin Urapidil Dihydralacine Glyceryl trinitrate Midazolam Flunitrazepam Phenylephrine Ephedrine Propofol Ketamine Amide local anaesthetics Magnesium Vecuronium Cisatracurium Fenoterol
Contraindicated
Uncertain
Codeine Morphine Pethidine Tramadol NSAIDs Metamizole Penicillin Atropine Beta blockers
Etomidine Thiopental Ester local anaesthetics Mivacurium Suxamethonium
Rocuronium
Oxytocin Methylergonovine Prostaglandins Isoflurane Sevoflurane Desflurane
F. Ulbrich et al. Four minutes after incision a lifeless newborn was delivered. Immediate resuscitation was initiated by a team of neonatologists, without success. During surgery the patient was haemodynamically stable. For analgesia she received intravenous piritramid 10.5 mg and clonidine 150 lg and for antibiotic prophylaxis erythromycin 1 g. In addition, we gave intravenous hydrocortisone 200 mg and 6 IU oxytocin as an infusion. Following a somewhat delayed emergence from anaesthesia and uneventful extubation, the patient was transferred to the intensive care unit and monitored for 24 h. Analgesia was continued with intravenous piritramid and oral paracetamol. The subsequent hospital stay was uncomplicated and the patient was discharged on the seventh postoperative day. The patient reported no recall or awareness. At autopsy, severe placental insufficiency was diagnosed as the reason for the baby’s demise. An incidental finding was umbilical cord torsion.
Discussion Systemic mastocytosis is most commonly generated by a point mutation in the gene for the tyrosine kinase receptor Kit D816V.6 Mutations cause activation of mast cell progenitor cells with abnormal growth and differentiation. Pathological mast cell infiltrates may appear in various organs. Systemic mastocytosis is diagnosed by biopsy of skin, bone marrow or other mast cell infiltrates with immunohistochemistry analysis of specimens identifying aberrant surface antigens of mast cells.1,2,6 The severity of the disease is dependent on the degree of systemic involvement and on the location of mast cell infiltration.2 Clinical symptoms are caused by mediator release or organ dysfunction. As the disease can have a benign course without any restrictions to everyday life, diagnosis is often delayed.4 The risk of anaphylactoid reactions is high in all patients irrespective of symptom severity.3 The prevalence of perioperative anaphylactoid reactions is not known, but cases of grade III to IV reactions have been described in the literature.7 Approximately 12% of patients with systemic mastocytosis undergoing surgery sustain complications requiring epinephrine.6 For this reason certain anaesthetic medications which are potent triggers of allergic reaction are contraindicated. With the increased physical and psychological stress of pregnancy, one third of women with systemic mastocytosis experience a worsening of systemic symptoms during pregnancy.5,8 Parturients are at high risk of suffering severe exacerbations: anxiety, stress, lack of sleep, pain and many pharmacological agents are trigger factors for mast cell degranulation. Therefore a tailored anaesthetic plan is necessary with regard to analgesia and anaesthesia for delivery.5 The anaesthetic department should be consulted early to discuss management.
245 Patients should be questioned with respect to individual drug tolerance, trigger factors and clinical symptoms. Existing medical documents can also be requested at this time. The anaesthetist can then prepare an individual plan to minimize the risk of triggering an exacerbation. Some general precautions, such as the creation of a quiet and relaxing atmosphere, are advisable. It is important to inform all medical staff about medications that may cause mast cell degranulation (Table 2).7 Intradermal skin testing as a predictor of drug reaction is valuable in some cases but not mandatory for all patients.6 Baseline serum tryptase is increased in patients with systemic mastocytosis and can be used for monitoring.3,7 Early epidural analgesia is recommended in order to provide optimal labour pain relief to the pregnant woman with systemic mastocytosis.8 As metabolism of ester-linked local anaesthetics produces paraaminobenzoic acid, a possible trigger for an anaphylactoid reaction, amide-linked local anaesthetics are preferable. In patients with systemic mastocytosis both spinal and epidural anaesthesia are appropriate techniques for caesarean section.5,6 Sedative (benzodiazepines or promethazine) and antihistamine premedication is advisable in patients undergoing neuraxial anaesthesia for caesarean section.7 Corticosteroids, as inhibitors of mast cell degranulation and suppressors of cytokine and inflammatory mediators, are recommended to minimize reactions.6 However, some authors state that they fail to suppress anaphylactoid reactions sufficiently.9,10 Ropivacaine has been suggested to be the local anaesthetic of choice for neuraxial anaesthesia. Although some authors avoid bupivacaine, its use seems to be safe and there have been many case reports published where it has been used without complications.6,9 Sudden haemodynamic instability may arise during surgery. Continuous blood pressure monitoring should thus be considered. In case of mast cell degranulation fractionated epinephrine is the first medication of choice. In addition, corticosteroids and antihistamines are indicated for stabilisation. Patients are not candidates for ambulatory surgery; observation for 24 h is indicated, as reactions up to four hours postoperatively have been described.9 In cases of emergency caesarean section, general anaesthesia may be required. Thiopental, frequently used in this emergency situation, is a potent trigger of histamine release and is contraindicated. In contrast, the use of midazolam, propofol, etomidate or ketamine is considered safe.9 Volatile anaesthetics generally do not induce mast cell degranulation and are a good choice for maintaining anaesthesia.9 Fentanyl, sufentanil and remifentanil are all considered safe.7 Piritramid, a synthetic opioid, is routinely used in Germany for postoperative analgesia, has a potency about 0.75 times
246
Systemic mastocytosis
that of morphine and has been used successfully in patients with systemic mastocytosis.6 In contrast, morphine and pethidine are potent histamine-releasing agents which can lead to death in these patients.7 As the depolarizing muscle relaxant, suxamethonium, is a potent allergen and major trigger of allergic reactions, it is contraindicated.7 Benzylisochinoline muscle relaxants can lead to mast cell activation while the steroid muscle relaxants can block histamine-N-methyltransferase causing accumulation of histamine and mast cell degranulation. Therefore, these are contraindicated in patients with systemic mastocytosis. The safe use of the steroid relaxant rocuronium has been published.5,11 However, there are inconsistent data relating to allergic potency.7 Vecuronium and pancuronium are considered to be of medium risk, atracurium and mivacurium of high risk. Cisatracurium is associated with minimal mast cell activation and should be the preferred muscle relaxant.1 In our case a large dose of propofol was administered for induction of general anaesthesia. Consequently, tracheal intubation was performed without a muscle relaxant. Rocuronium was not applied in this situation due to uncertainty regarding its negative effects. However, after a further review of the literature, the risk of anaphylactoid reactions appears to be low and rocuronium should be safe for general anaesthesia in these patients.11 In this case the anaesthetist’s intention was to minimize the risk of degranulation as much as possible. Other than the fact that the patient was pregnant, no other indication of difficult airway was observed and so a difficult tracheal intubation was not expected. In summary, parturients with systemic mastocytosis are a particular challenge for anaesthetists. A well-structured anaesthetic plan and back-up plan should be made and communicated to all involved. During labour, life-
0959-289X/$ - see front matter c 2013 Elsevier Ltd. All rights reserved. http://dx.doi.org/10.1016/j.ijoa.2013.03.011
threatening complications may occur and anaesthesia is associated with increased risk of triggering an acute exacerbation. Anaesthetic management should focus on avoidance of trigger factors. Glucocorticoids, antihistamines and epinephrine should be ready for the duration of hospital stay, especially during the critical periods of labour and the early postpartum period.
References 1. Ahmad N, Evans P, Lloyd-Thomas AR. Anesthesia in children with mastocytosis – a case based review. Paediatr Anaesth 2009;19:97–107. 2. Amon U, Hartmann K, Horny HP, Nowak A. Mastocytosis – an update. J Dtsch Dermatol Ges 2010;8:695–711. 3. Goldfinger MM, Sandadi J. Undiagnosed systemic mastocytosis in a teenager revealed during general anesthesia. Paediatr Anaesth 2010;20:290–1. 4. Vaughan ST, Jones GN. Systemic mastocytosis presenting as profound cardiovascular collapse during anaesthesia. Anaesthesia 1998;53:804–7. 5. Villeneuve V, Kaufman I, Weeks S, Deschamps A. Anesthetic management of a labouring parturient with urticaria pigmentosa. Can J Anaesth 2006;53:380–4. 6. Chaar CI, Bell RL, Duffy TP, Duffy AJ. Guidelines for safe surgery in patients with systemic mastocytosis. Am Surg 2009;75:74–80. 7. Konrad FM, Unertl KE, Schroeder TH. Mastocytosis. A challenge in anaesthesiology. Anaesthesist 2009;58:1239–43. 8. Worobec AS, Akin C, Scott LM, Metcalfe DD. Mastocytosis complicating pregnancy. Obstet Gynecol 2000;95:391–5. 9. Konrad FM, Schroeder TH. Anaesthesia in patients with mastocytosis. Acta Anaesthesiol Scand 2009;53:270–1. 10. Lerno G, Slaats G, Coenen E, Herregods L, Rolly G. Anaesthetic management of systemic mastocytosis. Br J Anaesth 1990;65:254–7. 11. Hinkelbein J, Feller-Heppt G, Lenz C, Hensel M, Frietsch T. Mastocytosis: pathology and practical implications for anesthesia. Anaesth Intensivmed 2009;50:508–18.