Anesthesia in a pediatric patient with systemic mastocytosis

Abstract

Systemic mastocytosis is a rare disorder of mast cells which carries considerable risk in the perioperative period. Unintended release of mast cell mediators has the potential to cause signi! cant hypotension, multi-system organ dysfunction, and death. Many factors have been implicated in mast cell degranulation including heat, stress, and many medications that may be commonly used anesthetic care. We present the case of a 10 year old girl with systemic mastocytosis who required general anesthesia for reduction of a dislocated elbow. The perioperative care of such patients is reviewed and strategies for intraoperative anesthesia are discussed.