Neurofibromatosis
Incidental findings
+ve signs Multiple neurofibromas, ≥6 café au alait patches, axillary freckles, kyphoscloliosis, Lisch nodules
Differential diagnosis
Function
Tests
Extensions

  • Axilla for freckles
  • Visual acuity, fundoscopy for optic nerve glioma
  • Hearing for acoustic neuroma
  • Would like to know blood pressure (renal artery stenosis)
Notes

  • Complications: Malignant transformation, hypertension, spinal cord / nerve root compression
  • Screening: Lisch nodules in 100% of patients >20 years
  • Type 2 usually bilateral acoustic neuroma or unilateral acoustic with menigioma/neurofibroma/schwannoma
  • Other features: lung cysts, retinal hamartomas, skeletal abnormalities, intellectual disability