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Anesthetic considerations in pediatric mastocytosis: a review
Norma J Klein1, Shad Misseldine
1University of California, Davis, Sacramento, CA 95817, USA. incline@comcast.net
Insights
Children with mastocytosis rarely experience severe anesthesia side effects, even with common anesthetic drugs. This review summarizes anesthetic options and safety for pediatric mastocytosis patients.
Area of Science:
- Pediatric Anesthesiology
- Hematology
- Rare Diseases
Background:
- Mastocytosis is a rare disease impacting pediatric patients requiring anesthesia.
- Anesthetic medications can trigger mast cell activation, causing parental anxiety.
- Current diagnostic tests do not reliably predict perioperative mast cell degranulation.
Purpose of the Study:
- To review anesthetic management for pediatric mastocytosis.
- To summarize reported side effects and prophylaxis strategies.
- To provide guidance for anesthesiologists encountering these patients.
Main Methods:
- Systematic review of pediatric literature on anesthesia and mastocytosis.
- Analysis of case reports and expert opinions on anesthetic drug use.
- Compilation of data on perioperative outcomes and adverse events.
Main Results:
- Children with skin-limited mastocytosis generally tolerate anesthesia well.
- No fatalities have been reported in pediatric mastocytosis patients undergoing anesthesia.
- Preoperative prophylaxis is typically guided by expert consensus and case data.
Conclusions:
- Anesthesia is generally safe for pediatric mastocytosis patients, particularly those with skin-limited disease.
- Careful consideration of anesthetic agents and potential triggers is essential.
- Further research is needed to establish evidence-based prophylaxis guidelines.
Abstract:
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.
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