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Pulsatile proptosis from sphenoid wing hypoplasia (neurofibromatosis). Emory Eye Center. 50 yo RH W Woman. “My right eye has been moving for as long as I can remember” Good vision, no diplopia, no neurologic symptoms. Neuro-Ophthalmic Examination. BP 104/68, HR 80

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pulsatile proptosis from sphenoid wing hypoplasia neurofibromatosis

Pulsatile proptosis from sphenoid wing hypoplasia (neurofibromatosis)

Emory Eye Center

50 yo rh w woman
50 yo RH W Woman
  • “My right eye has been moving for as long as I can remember”
    • Good vision, no diplopia, no neurologic symptoms
neuro ophthalmic examination
Neuro-Ophthalmic Examination
  • BP 104/68, HR 80
  • VA sc 20/20 20/20
  • Color vision 14/14 14/14
  • Pupils Normal Normal
  • EOM Full Full
  • Fundus Normal Normal
  • HVF Normal Normal
neurofibromatosis 1
Neurofibromatosis 1
  • Café au laitmacules (>6)
  • Neurofibromas (>2)
  • Freckling (axillary, inguinal)
  • Optic gliomas
  • Lisch nodules (iris hamartomas)
  • Sphenoid dysplasia
  • First degree relative with NF-1
absence of sphenoid wing
Absence of sphenoid wing
  • Pulsatile
    • Exophthalmos
    • Enophthalmos
  • Herniation of dura, CSF, brain into orbit (encephalocele)
    • Compression EOMs, optic nerve