NM-218
Mastocytosis in Our Pediatric Patients: A Rare Disease with Specific Anesthetic Challenges and Considerations
Clerizier S, Blasius K, Ross E
University of North Carolina, Chapel Hill, NC, USA
Introduction
Mastocytosis consists of a variety of clinical features characterized by increased mast cells in systemic organs. Patients present with either cutaneous mastocytosis [(CM) - typically in childhood] or systemic mastocytosis (SM). Incidence is estimated at 1/150,000 (2). The cause is a mutation of the mast cell receptor which induces constitutive activation (found in 50% of CM cases and >90% of SM cases) (2). These mast cells have the potential to degranulate and release inflammatory mediators such as histamine, tryptase, and cytokines (1). Anesthetic management requires knowledge of triggers/drugs that can cause mast cell degranulation and preparedness to treat cardiovascular collapse.
Case Presentation
17-year-old female presents with worsening CM and new systemic symptoms over the last year for a diagnostic bone marrow biopsy to evaluate for SM. She was diagnosed with CM in childhood with symptoms of urticarial rash flares 2-3 times a year. For the last 12 months, flares occur 2-3 times weekly with gastrointestinal discomfort, flushing, headaches and arthralgias. Home medications include: ranitidine, montelukast and an Epi Pen. The anesthetic goals are to maintain normothermia, pain control, minimize stimulation, and encourage anxiolysis to prevent mast cell degranulation and histamine release. The patient was premedicated with: midazolam and dexmedetomidine for anxiolysis, fentanyl for analgesia, and diphenhydramine and dexamethasone to prevent histamine release. The case was done under monitored anesthesia care with a native airway. Propofol was used for induction and maintenance of anesthesia. Hemodynamic stability was constant throughout. A new urticarial rash, however, was observed at the site of betadine prep for the biopsy. Additionally, a pre-existing rash on her neck substantially grew in size. Afterwards, the patient was monitored under the care of an anesthesiologist in the postoperative area for 1 hour with no further issues.
Discussion
As illustrated by this case, mastocytosis patients are sensitive to triggers of mast cell degranulation. It is essential to avoid stress, pain, changes in temperature, light anesthesia, or friction to the skin. Knowledge of patient specific triggers is important and drugs that can cause histamine release should be avoided or administered with caution (3). While many anesthetic drugs can instigate mast cell degranulation (ex opioids, muscle relaxants, analgesics, and volatile anesthetics), studies have shown that routine anesthetic techniques can be used in these patients as long as providers premedicate patients thoughtfully, are aware of potential consequences, and are prepared to treat cardiovascular collapse (1). Patients should be monitored after the procedure because symptoms may take 1-2 hours to appear.
References
1. Carter, Melody C., et al. “Pediatric Mastocytosis: Routine Anesthetic Management for a Complex Disease.†Anesthesia & Analgesia, vol. 107, no. 2, 2008, pp. 422–427
2. Dewachter, Pascale, et al. “Perioperative Management of Patients with Mastocytosis.†Anesthesiology, vol. 120, no. 3, 2014, pp. 753–759
3. Tew, Shannon, and Brad M. Taicher. “Avoiding Cardiovascular Collapse.†A & A Case Reports, vol. 5, no. 10, 2015, pp. 179–181
Top
