What are the considerations for a patient with Mast Cell Activation Syndrome (MCAS) undergoing rhinoplasty?

Rhinoplasty in MCAS Patients: Perioperative Management

Patients with MCAS can safely undergo rhinoplasty with appropriate perioperative prophylaxis, multidisciplinary coordination, and avoidance of specific triggering agents, as the risk of anaphylaxis is elevated but manageable with proper precautions. 1

Preoperative Risk Assessment and Preparation

Mandatory Preoperative Measures

• Review all prior anesthetic records meticulously to identify any previous triggers of mast cell activation, as careful identification and avoidance of known triggers is critical to preventing perioperative complications. 1

• Prescribe two epinephrine auto-injectors for the patient to have immediately available, as anaphylaxis occurs in 20-50% of patients with systemic mastocytosis and mast cell disorders. 1, 2

• Coordinate care with a multidisciplinary team including the surgeon, anesthesiologist, and allergist/immunologist before the procedure to develop a comprehensive perioperative plan. 1, 2

Preoperative Prophylactic Medication Protocol

• Administer H1 antihistamines (such as cetirizine or fexofenadine at 2-4 times FDA-approved doses), H2 antihistamines (such as famotidine), and anxiolytic agents (benzodiazepines) as pre-anesthetic treatment to reduce the frequency and severity of mast cell activation events. 1, 2

• Consider corticosteroids as part of the premedication regimen, as they help in resolution of mast cell activation symptoms, though the evidence is primarily anecdotal. 1, 2

• Continue the patient's baseline antimediator therapy (H1 antihistamines, H2 antihistamines, mast cell stabilizers like cromolyn sodium, and leukotriene modifiers if applicable) throughout the perioperative period without interruption. 2

Intraoperative Anesthetic Considerations

Safer Anesthetic Agents

• Use propofol for anesthetic induction and sevoflurane or isoflurane for inhalational anesthesia, as these agents are considered safer based on anecdotal evidence, though not evidence-based. 1

• Select fentanyl or remifentanil as analgesics rather than morphine or codeine, as these synthetic opioids are safer alternatives in MCAS patients. 1, 2, 3

• Use lidocaine or bupivacaine for local anesthesia and povidone-iodine for skin antisepsis, as these are considered safer options. 1

Agents to Strictly Avoid

• Avoid the muscle relaxants atracurium, mivacurium, and succinylcholine, as these are known triggers; rocuronium and vecuronium are safer alternatives. 1, 2

• Exercise caution with opiates but never withhold analgesics, as pain itself is a potent trigger for mast cell degranulation and inadequate pain control can worsen the clinical situation. 1, 2, 3

Environmental and Technical Precautions

• Avoid temperature extremes (both hypothermia and hyperthermia) in the operating room, as these can trigger mast cell activation. 1

• Minimize unnecessary trauma during patient positioning and surgical manipulation, as mechanical irritation can lead to mast cell activation symptoms, skin blistering, or even osteolytic fractures in susceptible patients. 1

Management of Acute Intraoperative Reactions

Recognition and Initial Response

• Immediately discontinue the suspected triggering drug or anesthetic agent if signs of mast cell activation develop (flushing, urticaria, hypotension, bronchospasm, angioedema). 1

• Administer intramuscular epinephrine immediately for severe reactions with hypotension or laryngeal angioedema, as this is the cornerstone of acute anaphylaxis management. 1, 2

Supportive Management

• Initiate aggressive fluid resuscitation for hypotensive episodes while maintaining the patient in the supine position. 1

• Administer intravenous corticosteroids and additional H1 and H2 antihistamines to help manage ongoing symptoms and prevent biphasic reactions. 1

• Treat bronchospasm with intramuscular epinephrine or inhaled rapidly acting bronchodilators such as albuterol. 1

Postoperative Monitoring and Management

Immediate Postoperative Period

• Maintain heightened vigilance for delayed reactions in the recovery area, as mast cell activation can occur in the immediate aftermath of the surgical procedure. 1

• Measure serum tryptase levels within 30-120 minutes of any suspected activation event and compare to the patient's baseline tryptase obtained during a symptom-free period to confirm mast cell activation. 2

Pain Management Considerations

• Provide adequate analgesia without hesitation, as inadequate pain control creates a vicious cycle where pain itself triggers further mast cell degranulation. 1, 2, 3

• Prefer intravenous administration of opioids over oral routes to ensure reliable drug delivery and minimize gastrointestinal exposure in MCAS patients. 3

Critical Pitfalls to Avoid

• Never delay or cancel necessary surgery solely based on MCAS diagnosis, as anesthesia is not contraindicated and the condition is manageable with proper precautions. 1

• Never withhold analgesics due to concerns about triggering mast cells, as this creates greater risk by allowing pain to become a trigger itself. 1, 2, 3

• Never assume all patients with suspected MCAS have confirmed disease, as many referred patients have other conditions; however, when MCAS is suspected perioperatively, err on the side of caution and implement full prophylactic protocols. 4, 5

• Never use compounded medications to eliminate additives, as this practice is not recommended and additives have not been validated as problematic triggers in controlled studies. 1