Anesthetic considerations in pediatric mastocytosis: a review.

Mastocytosis is an orphan disease rarely encountered by practicing anesthesiologists. Children with mastocytosis often present for procedures or surgery requiring anesthesia. Because many of the medications commonly used in pediatric anesthesia have been reported to initiate mast cell activation, parents are often very anxious about their child's perioperative experience. Laboratory investigations of serum histamine assays associated with different anesthetic drugs have not been shown to predict mast cell degranulation in these patients. However, the pediatric literature suggests that children with disease limited to the skin rarely suffer serious side effects from anesthesia, and there are no reported fatalities. Preoperative prophylaxis is usually based on expert opinion and case reports. Detailed tables summarizing reports of anesthetic medications used for children with mastocytosis undergoing anesthesia, reported side effects, and suggested prophylaxis regimens are included in this review.