Anesthesia Considerations for Neurofibromatosis
(Von Recklinghausen Disease)
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
©2000 The Department of Anesthesiology and Intensive Care, The Chaim Sheba Medical Center
Sackler School of Medicine, Tel Aviv University, Tel Hashomer, Israel, 52621.