PT  - JOURNAL ARTICLE
AU  - Charles J. Fox
AU  - Samir Tomajian
AU  - Aaron J. Kaye
AU  - Stephanie Russo
AU  - Jacqueline Volpi Abadie
AU  - Alan D. Kaye
TI  - Perioperative Management of Neurofibromatosis Type 1
DP  - 2012 Jun 20
TA  - Ochsner Journal
PG  - 111--121
VI  - 12
IP  - 2
4099  - http://www.ochsnerjournal.org/content/12/2/111.short
4100  - http://www.ochsnerjournal.org/content/12/2/111.full
SO  - Ochsner J2012 Jun 20; 12
AB  - 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.