Neurofibromatosis (NF)

Airway involvement - Neurofibromas of the airway, larynx, or mediastinum can cause a difficult airway or compression; evaluate for hoarseness or stridor, image if concerned, and prepare for a difficult airway.

Screen for pheochromocytoma - NF1 is associated with pheochromocytoma; send plasma free metanephrines or 24-hour urine metanephrines in the hypertensive patient, and be alert to catecholamine-driven lability.

Neuraxial caution - Intraspinal neurofibromas and vertebral deformity can complicate or contraindicate neuraxial techniques; review spine imaging (MRI) for intraspinal tumors and deformity before attempting.

Kyphoscoliosis and lungs - Spinal deformity and restrictive lung disease may be present; check pulmonary function testing when the curve is significant.

Neuromuscular blockers - Both resistance and sensitivity have been reported; titrate with the twitch monitor.

Neurofibromatosis (type 1 most commonly) is an inherited neurocutaneous disorder causing neurofibromas along nerves, café-au-lait spots, and a predisposition to tumors.

Its anesthetic relevance is its unpredictable multisystem reach: airway, laryngeal, or mediastinal neurofibromas can cause a difficult airway; intraspinal neurofibromas complicate neuraxial anesthesia; there's an association with pheochromocytoma (catecholamine crisis) and with kyphoscoliosis and restrictive lung disease; and altered responses to neuromuscular blockers have been reported.