title: Anesthetic management in a pediatric patient with Noonan syndrome, mastocytosis, and von Willebrand disease: a case report.
abstract: This case report describes anesthetic considerations for a 6-year-old boy, admitted for adenoidectomy under general anesthesia, who had a complicated medical history, including mastocytosis, Noonan syndrome, and von Willebrand disease. Each affected the anesthetic plan and was addressed preoperatively among all surgical and anesthesia providers. Mastocytosis created a major concern, with its increased numbers of histamine-filled mast cells. Each drug that was added or eliminated from the anesthetic plan, to prevent histamine release by the activation of triggers, was considered. Patient handling and temperature control were also concerns. One of Noonan syndrome's characteristics is heart anomalies. This patient had a history of a patent foramen ovale and pulmonary stenosis; therefore, air was carefully removed from all intravenous lines and syringes. The main concern for bleeding difficulties was attributed to the history of von Willebrand disease, which results in prolonged bleeding time and can lead to delayed bleeding or serious postsurgical hemorrhage. Desmopressin was administered preoperatively to increase platelet aggregation and the von Willebrand factor level. The use of aspirin and other nonsteroidal anti-inflammatory drugs was avoided. We discuss the clinical and anesthetic management of this case with a review of pertinent literature.
authors: Critical Health Systems, Raleigh, NC, USA. LynnMacksey@msn.com
authors: Lynn Fitzgerald
authors: LF
authors: Macksey
authors: Beth
authors: B
authors: White
mesh_terms: D000233:Adenoidectomy / Q000379:methods / Q000451:nursing; D000768:Anesthesia, General / Q000379:methods* / Q000451:nursing; D002648:Child; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use; D006490:Hemostatics / Q000627:therapeutic use; D006633:Histamine Antagonists / Q000627:therapeutic use; D006801:Humans; D007430:Intraoperative Care / Q000379:methods / Q000451:nursing; D008297:Male; D008415:Mastocytosis / Q000150:complications* / Q000517:prevention & control; D015508:Nasal Obstruction / Q000150:complications / Q000601:surgery*; D009634:Noonan Syndrome / Q000150:complications* / Q000517:prevention & control; D009719:Nurse Anesthetists; D010347:Patient Care Planning; D011292:Premedication / Q000379:methods / Q000451:nursing; D011300:Preoperative Care / Q000379:methods / Q000451:nursing; D014842:von Willebrand Diseases / Q000150:complications* / Q000517:prevention & control
chemical_list: D006490:Hemostatics; D006633:Histamine Antagonists; D003894:Deamino Arginine Vasopressin