Airway Evaluation For Magnetic Resonance Imaging Sedation In Pediatric Patients With PlexiformNeurofibroma

Neurofibromatosis type 1 (NF1) or Von Recklinghausen disease, the most common form of NF, is an autosomal dominant disease with a variable expressivity and a wide variety of clinical manifestations. In one half of cases, NF-1 can result from a de novo mutation, with no previous family history of disease. It affects males and females equally with a disorder frequency of 1 in 4,0001. The gene affected in NF-1, is located on the long arm of the chromosome 17 (q11.2). Neurofibromatosis modifies neurofibromin, a tumor suppressor protein, allowing rapid growth of cells, especially around the nervous system. This leads to the common symptoms of neurofibromatosis. The incidence of head and neck involvement in patients with NF varies between 14% and 37%2. Plexiform neurofibroma of the neck is a cause of morbidity in the affected individual with possible airway abnormalities. Challenges in the care of these patients include the evaluation and the determination of airway patency prior to anesthesia/sedation for the MRI exam