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Mediating the Mediators: Managing Mast Cell Activation Syndrome During Cesarean Delivery
Abstract Number: F5C-1
Abstract Type: Case Report/Case Series
Mast Cell Activation Syndrome (MCAS) is a disorder of inappropriate release of mast cell mediators (1). Exposure to environmental triggers causes hypersensitivity reactions ranging from mild mucocutaneous signs to anaphylaxis. MCAS may also present with collagen disorders such as Ehlers Danlos syndrome (EDS) and dysautonomia, and there may be a genetic association to this disease cluster (2).
A 41yo woman with MCAS, EDS, postural orthostatic tachycardia syndrome (POTS), anxiety/panic attacks, and recent intracranial aneurysm coiling presented for an elective cesarean delivery at 38 5/7 weeks. She had a history of angioedema and anaphylaxis, and was advised by her immunologist to avoid histamine-releasing medications including ester local anesthetics, opioids (especially morphine), and NSAIDs. She was also advised that epidural anesthesia was contraindicated and that spinal anesthesia would be safer, without clear rationale for this statement.
Preoperatively, she was receiving a prophylactic regimen of prednisone, hydroxyzine, ranitidine, and montelukast. We initially planned to perform an epidural or low-dose spinal-epidural anesthetic to avoid sudden autonomic changes in this somewhat frail and anxious patient. However, the patient was strongly opposed to an epidural catheter based on her immunologist's advice; because of this, a spinal anesthetic was performed. A phenylephrine infusion at 50 mcg/min was started prior to spinal placement to prevent hypotension, reflex tachycardia and a potential exacerbation of POTS. Spinal anesthesia was performed with bupivacaine 12mg, fentanyl 15mcg, and morphine 150mcg. Placement was uncomplicated and a T4 sensory level was obtained. During the procedure hypothermia was avoided with warmed blankets and fluids.
Delivery was uncomplicated, and the newborn’s Apgar scores were 8 and 9. Adequate postoperative analgesia was achieved with PO acetaminophen, tramadol, and the intrathecal morphine.
Guidelines on peripartum management of MCAS and similar mast cell disorders (e.g., mastocytosis) are sparse. Strategies include avoidance of known triggers, prophylaxis with mast cell stabilizing medications, and normothermia (1, 3). Given the extensive list of possible triggers, it can be difficult to avoid all “contraindicated” medications and interventions. We chose to include morphine in our spinal anesthetic to minimize postoperative systemic opioid requirement, reasoning that a small dose given into an area without significant numbers of immune cells would be acceptable, and it was well tolerated. The avoidance of epidural anesthesia was probably unnecessary but alleviated patient anxiety. Preemptive initiation of a phenylephrine infusion before spinal placement may have helped avoid an exacerbation of POTS.
1. NEJM 2015; 373:163-172.
2. Nature Genetics 2016; 48:1565-69.
3. Anesthesiology 2014; 120:753-759.