title: Avoiding Cardiovascular Collapse: Pediatric Cutaneous Mastocytosis and Anesthetic Challenges.
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.
authors.affiliation: From the Department of Anesthesiology, Duke University Medical Center, Durham, North Carolina.
authors.forename: Shannon
authors.initials: S
authors.lastname: Tew
authors.forename: Brad M
authors.initials: BM
authors.lastname: Taicher
mesh_terms: D000768:Anesthesia, General / Q000379:methods*; D002320:Cardiovascular Physiological Phenomena; D002648:Child; D005167:Factor VII / Q000627:therapeutic use; D005168:Factor VII Deficiency / Q000150:complications; D005260:Female; D006801:Humans; D007796:Laminectomy / Q000379:methods; D008159:Lumbar Vertebrae; D008407:Mast Cells; D008738:Methyl Ethers; D000077149:Sevoflurane; D014552:Urinary Tract Infections / Q000150:complications; D014582:Urticaria Pigmentosa / Q000150:complications*
chemical_list: D008738:Methyl Ethers; D000077149:Sevoflurane; D005167:Factor VII