The exact incidence of immediate anaphylaxis from anesthesia or surgery in mastocytosis patients (or mast cell patients, more generally) is not known. Only a handful of these events have been reported in literature; however, it is likely that the majority of uneventful procedures were not tracked, so statistics are unclear. To date, there have been no controlled trials investigating anesthetics in mast cell patients.
In 2014, a paper was published entitled “Perioperative Management of Patients with Mastocytosis.” This paper is excellent and addresses the specific issues that may arise for mast cell patients before, during and after surgery. I recommend you provide this reference to any doctor involved in your surgical/procedural care that is unfamiliar with mast cell disease.
Mastocytosis patients are at risk for activation by a number of triggers, some of which cannot be avoided in the surgical setting. For this reason, suppression of mediator release in advance of surgery is recommended. The following pre-medication protocol is recommended for mast cell patients for all major and minor procedures and for radiology procedures with and without dyes:
- Prednisone 50mg orally (20mg for children under 12): 24 hours and 1-2 hours before procedure
- Diphenhydramine 25-50mg orally (12.5 mg for children under 12) OR hydroxyzine 25mg orally, 1 hour before procedure
- Ranitidine 150mg orally (20mg for children under 12) 1 hour before procedure
- Montelukast 10mg orally (5mg for children under 5) 1 hour prior to procedure
This protocol was developed for the Mastocytosis Society by Dr. Mariana Castells and the original can be found here: http://www.tmsforacure.org/documents/ER_Protocol.pdf
Common triggers for mast cell patients in this setting include:
- Anxiety and psychological stress regarding the procedure. Administration of medication to mitigate anxiety (benzodiazepines, etc) is recommended to avoid mast cell degranulation. It is preferable for mast cell patients to be the first surgery of the day if possible, and for a quiet, calm atmosphere to be maintained in the OR.
- Temperature changes. Either being too cold or too hot can culminate in a mast cell reaction. Constant monitoring of patient’s temperature is required. Additionally, the OR temperature should be monitored. Head coverings, warming mattresses, and forced-air warming systems can be used to prevent hypothermia. Infusion and irrigation solutions should be warmed, and anesthetic gases should be warmed wherever possible.
- Irritation of the skin (including use of tourniquet). This can cause mast cell degranulation that leads to urticaria, especially in patients with cutaneous mastocytosis. Blisters may form with pressure from tourniquet or face mask. Mast cell degranulation of this type releases chymase which can lead to edema.
- The inherent physical trauma associated with surgery. This is of specific consideration when operating in the GI tract, which has a significant mast cell population relative to other organs.
- Musculoskeletal pain from skeletal involvement in SM patients. Patients should be positioned carefully to avoid causing fractures.
- Pain can cause mast cell degranulation. For this reason, opioid medications should be used for pain relief wherever possible.
Pascale Dewachter, M.D., Ph.D.; Mariana C. Castells, M.D., Ph.D.; David L. Hepner, M.D., M.P.H.; Claudie Mouton-Faivre, M.D. Perioperative Management of Patients with Mastocytosis. Anesthesiology 03 2014, Vol.120, 753-759.