증례 : Noonan 증후군 환아의 마취경험

Noonan syndrome (NS) is an autosomal dominant disorder characterized by dysmorphic facial features in association with short stature and heart disease. A webbed neck, chest deformities, mental retardation, and bleeding disorders are also frequently associated with this pathology. The syndrome is named after Dr. Jacqueline Noonan. Patients with Noonan`s syndrome present a multiplicity of challenges to the anaesthetist, particularly with regard to spinal and airway abnormalities. Alternative methods of management should be discussed fully with patients. Careful preoperative consultation and determination of the degree of associated anomalies will help to prepare the anesthesiologist for potential problems. We describe the anesthetic management of a eight-year-old girl with Noonan syndrome for dental management under general anesthesia. We report successful management of a patient with Noonan syndrome, suspected the difficult airway, undergoing dental surgery using the induction agent with propofol and remifentanil, the muscle relaxant used after the intubation.