TY - JOUR T1 - Perioperative Management of Neurofibromatosis Type 1 JF - Ochsner Journal JO - Ochsner J SP - 111 LP - 121 VL - 12 IS - 2 AU - Charles J. Fox AU - Samir Tomajian AU - Aaron J. Kaye AU - Stephanie Russo AU - Jacqueline Volpi Abadie AU - Alan D. Kaye Y1 - 2012/06/20 UR - http://www.ochsnerjournal.org/content/12/2/111.abstract N2 - Neurofibromatosis type 1 (neurofibromatosis-1), a relatively common single-gene disorder, is caused by a mutation of the NF1 gene that results in a loss of activity or in a nonfunctional neurofibromin protein. Clinical anesthesiologists may find patients with neurofibromatosis-1 challenging because this condition may affect most organ systems and result in a wide variety of presentations and clinical implications. Current neurofibromatosis-1 research studies include genotype-phenotype correlations, investigation of the pathoetiology behind the different clinical manifestations of neurofibromatosis-1, and the search for treatment options for the different features of the disorder. Neurofibromatosis-1–associated complications of the central nervous, respiratory, cardiovascular, musculoskeletal, and gastrointestinal and genitourinary systems all present various degrees of considerations for anesthesiologists. Additionally, neurofibromatosis-1 has dramatic implications for pregnant women. ER -