Neurofibromatosis is an autosomal dominant trait with new mutations being common. The hallmark of the disease is café-au-lait spots (more than six that are greater than 1.5cm in diameter) and neurofibromas.

Associated Conditions:

• Laryngeal and tracheal compression secondary to tumor
• High incidence of kyphosis and progressive scoliosis
• Increased incidence of neural tumors-glioma, meningioma, acoustic neuroma and pheochromocytoma
• Compression of spinal roots, cerebello-pontine angle, medulla oblongata, and other nonneural structures
• Impaired mental function (usually mild)
• Megalocephaly
• Malformations of the greater wing of the sphenoid bone with pulsating exophthalmos
• Congenital pseudoarthrosis (commonly tibia and radius)
• Increased incidence of cancer

Anesthetic Considerations:

• Airway abnormalities may lead to difficulties with intubation and ventilation
• Patients may have a prolonged response to nondepolarizing muscle relaxants
• Hypertension, tachycardia or arrhythmias may indicate pheochromocytoma
• Increased intracranial pressure may require appropriate treatment