RT Book, Section A1 Bissonnette, Bruno A1 Luginbuehl, Igor A1 Engelhardt, Thomas SR Print(0) ID 1164080218 T1 Neurofibromatosis Generalisata T2 Syndromes: Rapid Recognition and Perioperative Implications, 2e YR 2019 FD 2019 PB McGraw-Hill Education PP New York, NY SN 9781259861789 LK accessanesthesiology.mhmedical.com/content.aspx?aid=1164080218 RD 2024/03/29 AB Phakomatoses are multisystem disorders that primarily affect the skin and the CNS. Part of the RASopathies. Neurofibromatosis (NF) Type I (NF-I), also called von Recklinghausen disease, is a rare genetic disorder characterized by the development of multiple neurofibromas of the nerves and skin. The presence of café-au-lait spots on the skin of the trunk and other regions, as well as freckling, particularly in the axillary region and in the inguinal area is a characteristic. Often evident by age 1 year, the café-au-lait spots tend to increase in size and number over time. At birth or early childhood, plexiform neurofibromas may be present. Benign tumor-like nodules of the colored regions of the eyes (Lisch nodules) may be present.