This 21-year-old with type 1 neurofibromatosis presents with a four-year history
of a right progressive non-axial painless proptosis and swelling around the
orbit (Figure 1A-D). Examination reveals a non-pulsatile exophthalmos. The right
eye is only light perception due to corneal scarring from years of exposure
keratopathy and possible optic nerve compression. The left eye has normal vision
and slit-lamp shows multiple Lisch's nodules.
His past medical history includes three temporal operations for plexiform
neurofibroma and a Fasanella Servat operation for right ptosis.
He works as a welder and his main concern is his inability to wear the eye
shields to protect his left eye due to the left orbital mass.
Figure 1 Right non-axial
proptosis and swelling around the orbit.
a. What are
the eyelid abnormalities seen in these pictures?
He is keen
for surgical excision of the mass to allow him to wear shield during welding and
also to improve his appearance. CT scans of the orbit are shown below.
Figure 2. CT scan of the right orbit.
b. What are
the main abnormalities seen in these CT scan?
c. How would
you reduce the mass and improve the appearance of this patient?