RESUMO
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.
Assuntos
Adenoidectomia , Anestesia Geral/métodos , Mastocitose/complicações , Obstrução Nasal/cirurgia , Síndrome de Noonan/complicações , Doenças de von Willebrand/complicações , Adenoidectomia/métodos , Adenoidectomia/enfermagem , Anestesia Geral/enfermagem , Criança , Desamino Arginina Vasopressina/uso terapêutico , Hemostáticos/uso terapêutico , Antagonistas dos Receptores Histamínicos/uso terapêutico , Humanos , Cuidados Intraoperatórios/métodos , Cuidados Intraoperatórios/enfermagem , Masculino , Mastocitose/prevenção & controle , Obstrução Nasal/complicações , Síndrome de Noonan/prevenção & controle , Enfermeiros Anestesistas , Planejamento de Assistência ao Paciente , Pré-Medicação/métodos , Pré-Medicação/enfermagem , Cuidados Pré-Operatórios/métodos , Cuidados Pré-Operatórios/enfermagem , Doenças de von Willebrand/prevenção & controleRESUMO
A large, three generation-family with firm evidence of autosomal dominant transmission of the cardio-faciocutaneous (CFC) syndrome is reported. This observation questions once more the validity of separating the CFC syndrome from the Noonan syndrome as two distinct entities.