Anesthesia Considerations 

Neurofibromatosis

 

 

Background 

 

• Cafe au lait spots are the most characteristic finding 

• 2 types: NF-1 (more common) & NF-2 

• Neurofibromas nearly always involve the skin, but they can also occur in the deeper peripheral nerves & nerve roots & in or on viscera or blood vessels innervated by the autonomic nervous system

 

 

Considerations

 

• Potential difficult airway:

  • Airway obstruction/distortion from laryngeal, pharyngeal, tongue, cervical tumors

  • Macrocephaly & macroglossia

  • Tumors can be very vascular

• Respiratory:

  • Restrictive lung disease (kyphoscoliosis ~25%)

  • Potential for pulmonary fibrosis from parenchymal tumours

  • Pulmonary hypertension/RV failure 

  • Mediastinal neurofibroma: may present with cardiopulmonary complaints 

• Cardiovascular:

  • Hypertension

    • Usually essential, secondary: RAS >> pheochromocytoma (<1%)

  • Dysrhythmias, idiopathic cardiomyopathy

  • RV outflow tract tumour

• CNS:

  • ↑ ICP (5-10% have intracranial tumors, hydrocephalus) +/- contraindication to neuraxial

  • Seizure disorder

  • Cognitive deficits, learning disabilities

  • Peripheral neuropathy 

• Unpredictable response to NMB drugs:

  • Variable responses (sensitivity or resistance to succinylcholine, sensitivity to NDMR)

• Difficult regional & epidural/spinal, may be contraindicated if spinal neurofibroma

• Possible endocrine problems: pheochromocytoma, hypoglycemia, pituitary tumours, hyperparathyroidism, medullary thyroid carcinoma

 

 

Optimization/Consults 

 

• Neurology: optimize ICP, antiepileptics

• Cardiology/endocrine: optimize antihypertensives (phenoxybenzamine) if pheochromocytoma

• Consider MRI (r/o ↑ICP) if planning regional technique

• Respirology: if restrictive lung disease

• Conflicts: 

  • ↑ICP vs neuraxial 

  • Spinal neuromas vs neuraxial

  • ↑ ICP vs difficult airway 

 

 

Pregnancy Considerations 

 

• Higher maternal complications 

• If pelvic/abdominal neurofibromas → cesarean section necessary (dystocia, obstructed labor or respiratory embarrassment)

• Higher fetal complications: preterm labor, IUGR, abortion 

• GA: very cautious of difficult airway & underlying hypertension 

• Neuraxial: must have imaging (MRI/CT) of spine before epidural/spinal