Anaesthetic Management of Neurofibromatosis

Preanesthetic Evaluation and Planning

The American College of Medical Genetics and Genomics recommends preanesthesia neuraxial imaging to evaluate for spinal or paraspinal neurofibromas is probably not needed for routine epidural or spinal anesthesia, with a strength of evidence level of moderate 1, 2
The American College of Medical Genetics and Genomics suggests that if concerns exist about spinal neurofibromas, spinal anesthesia may be considered over epidural, with a strength of evidence level of moderate 1, 2
No significant adverse events have been reported in the literature for women with NF1 undergoing epidural anesthesia, according to the American College of Medical Genetics and Genomics 1, 2
Blood pressure should be measured preoperatively to screen for pheochromocytoma, which can cause intraoperative hypertensive crises, as recommended by the American Heart Association 2, 3, 4

The American Society of Anesthesiologists recommends considering regional anesthesia techniques when anatomically feasible and not contraindicated by tumor location, with a strength of evidence level of high 1, 2

The American Pain Society suggests that chronic pain affects the majority of NF1 adults and requires multimodal analgesia planning, with a strength of evidence level of high 3, 5
Adequate postoperative pain control should be planned, as these patients often have baseline chronic pain, according to the American Pain Society 3, 5

The National Comprehensive Cancer Network recommends maintaining high suspicion for malignant peripheral nerve sheath tumor (MPNST) in patients presenting with progressive severe pain, rapid tumor growth, or new neurologic symptoms, with a strength of evidence level of high 3, 4, 5
Approximately 8-13% of plexiform neurofibromas undergo malignant transformation to MPNST, according to the National Comprehensive Cancer Network 3