From the Guidelines
Patients with mastocytosis require special surgical precautions due to their risk of mast cell activation during procedures, and a multidisciplinary management approach is recommended with the involvement of surgical, anesthesia, and perioperative medical teams. Before surgery, patients should continue taking their regular medications including H1 and H2 antihistamines, and leukotriene inhibitors. Additionally, premedication with corticosteroids is recommended to reduce the frequency and/or severity of mast cell activation symptoms 1.
Key Precautions
- Patients should avoid triggers that can cause mast cell degranulation, including certain anesthetic agents (such as morphine, codeine, and vancomycin), temperature extremes, friction, and stress 1.
- The surgical team should be informed about the mastocytosis diagnosis and have emergency medications readily available, including epinephrine, IV antihistamines, and IV corticosteroids 1.
- Regional anesthesia is often preferred when possible, and certain perioperative drugs are considered safer, although the supporting data are anecdotal and not evidence-based 1.
- Agents to be avoided include the muscle relaxants atracurium and mivacurium, and succinylcholine, while caution should be exercised with the use of opioids (such as codeine or morphine) 1.
Management of Mast Cell Activation Symptoms
- Management of mast cell activation symptoms depends on the severity of the symptoms, and relies upon discontinuation of the suspected drug or anesthetic agent, fluid resuscitation, and intravenous epinephrine for severe reactions 1.
- Corticosteroids and antihistamines (H1 and H2 blockers) may be used as adjuncts, and a full allergic workup should be initiated in the event of anaphylaxis or other mast cell activation event 1.
From the Research
Surgical Precautions for Mastocytosis
To minimize the risk of anaphylaxis during surgical procedures, several precautions can be taken for patients with mastocytosis:
- Preoperative assessment and management are crucial to prevent mast cell activation and anaphylaxis 2, 3, 4, 5
- Patients with mastocytosis should receive chronic antimediator therapy and/or perioperative prophylactic medications, such as antihistamines and steroids, to reduce the risk of anaphylaxis 2, 3, 5
- The choice of anesthetic agents is important, with recommendations for propofol, etomidate, ketamine, fentanyl-type opioids, cis-atracurium, or pancuronium for induction of general anesthesia 5
- Maintenance of anesthesia can be achieved with a total intravenous technique or with a volatile anesthetic such as sevoflurane 5
- Avoidance of certain physical stimuli and adequate premedication can further decrease the risk of anaphylaxis by 10-fold 4
- Patients with systemic mastocytosis (SM) may require extra precautions, especially those with elevated baseline serum tryptase, a marker of mast cell burden 6
Specific Considerations for Pediatric Patients
- Pediatric patients with cutaneous mastocytosis (CM) do not appear to be at significant risk of widespread mast cell degranulation during anesthesia, but the risk cannot be ascertained with confidence due to the limited number of reported cases 6
- Children with SM and high baseline serum tryptase may merit extra precautions, but experience in this subgroup is limited 6
- Drugs that cause minimal histamine release can be selected for pediatric patients with mastocytosis without compromising the anesthetic technique 6