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Avoiding Cardiovascular Collapse: Pediatric Cutaneous Mastocytosis and Anesthetic Challenges
1From the Department of Anesthesiology, Duke University Medical Center, Durham, North Carolina.
Insights
Anesthetic management for pediatric mastocytosis requires careful planning. This case highlights strategies for managing mast cell mediator release, factor VII deficiency, and potential hypersensitivity during surgery.
Area of Science:
- Anesthesiology
- Pediatric Hematology
- Genetics
Background:
- Mastocytosis involves abnormal mast cell infiltration, potentially causing mediator release and hypersensitivity.
- Pediatric patients with mastocytosis present unique anesthetic challenges.
- This case involves a child with cutaneous mastocytosis, factor VII deficiency, and recurrent urinary tract infections.
Abstract:
Mastocytosis includes a spectrum of diseases characterized by abnormal mast cell infiltration in various organs, which can lead to mast cell mediator release and immediate hypersensitivity. We review anesthetic challenges presented by a 6-year-old girl with a history of mast cell mediator release because of the urticaria pigmentosa variant of cutaneous mastocytosis, factor VII deficiency, increasing episodes of urinary tract infections, and pyelonephritis. She underwent spine magnetic resonance imaging, subsequent lumbar laminectomy for fatty filum release, and a cystourethroscopy. Perioperative management included factor VII desensitization, avoidance of triggers, minimizing histamine-releasing medications, mast cell stabilization, and preparation for potential immediate hypersensitivity.

