deamino-arginine-vasopressin and Noonan-Syndrome

This case report describes a 9-year-old Caucasian girl who required comprehensive dental treatment under general anaesthesia but gave a history of Noonan's syndrome. The patient was extremely needle phobic. Because of the association between Noonan's syndrome and underlying coagulopathies, for which no previous investigations were evident, dental treatment had to be postponed pending further investigation. The patient was referred to a haematologist and underwent coagulation screening, which revealed the presence of von Willebrand's disease. The patient was prescribed Desmopressin to raise plasma levels of factor VIII: C and von Willebrand's factor (VWF) in order that dental treatment, including extractions, could be carried out under an in-patient general anaesthetic.. Congenital heart defects and bleeding diatheses are regarded as a common association of Noonan's syndrome. Witt et al estimated that around one-third of the patients have an associated bleeding disorder, although a later report suggested that as many as 74% of the coagulation profiles could be abnormal. Most of the bleeding problems are reported to be mild, and resolve with age in some patients, but, clearly, they may cause problems during dental treatment, necessitating haematological investigations and a multidisciplinary approach.

Topics: Anesthesia, General; Child; Comprehensive Dental Care; Deamino Arginine Vasopressin; Dental Care for Chronically Ill; Female; Hemostatics; Humans; Noonan Syndrome; von Willebrand Diseases

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.

Topics: Adenoidectomy; Anesthesia, General; Child; Deamino Arginine Vasopressin; Hemostatics; Histamine Antagonists; Humans; Intraoperative Care; Male; Mastocytosis; Nasal Obstruction; Noonan Syndrome; Nurse Anesthetists; Patient Care Planning; Premedication; Preoperative Care; von Willebrand Diseases