Spheno-orbital Encephalocele: Eyeball is touching the nostril!


Eye in this neurological condition is the eye your eyes have never seen before!

Here is a case of young man aged 22 years. According to him the upper lid of his right eye had a swelling because of which the eye protruded since he was 7 years old. He managed living with it all those years but in the last six months he observed increase in the size of swelling. The increase in the size was accompanied with gradual decrease in the vision.
The swelling was quite large and eventually pushed the eyeball medially and downwards. The eyeball literally touched the nostril! The swelling was coupled with redness, loss of vision, pain and watering of right eye. Conjunctival chemosis was also associated with this condition.

Upon conducting plain radiograph a hint towards real culprit was found. The sphenoid wing and occipital region in the skull had a defect which resulted in the widening of right orbital fissure as clearly shown in the figure below.

Asian Journal of Neurosurgery

For further investigation, USG of right eye was performed. The results revealed presence of a large retro-ocular mass with solid and cystic areas with internal septations possibly representing herniated neural tissue. Spheno-orbital encephalocele was diagnosed.

Spheno-orbital encephalocele is an extremely rare congenital condition in which intracranial contents herniate into the cranial cavity with or without meninges and CSF. Thus, the real culprit was caught by USG. In this case, temporal bone had herniated with CSF through greater wing of sphenoid on the right side into the right orbital space causing anterior displacement of right eyeball.

Now only climax remained to this investigation which was brought by surgery. Right frontal craniotomy was performed. During the operation, it was found that the bony defect previously seen in USG results was present on the frontal and sphenoid bone which together make up the roof of right orbit. The bony defect extended up to the petrous temporal bone. Ultimately, when encephalocele was exposed it was opened and drained. The dysplastic brain tissue was excised. As far as the skull base was concerned, it was repaired using a pericranial flap.

The surgery was a success and the protruding eyeball became normal. The patient experienced slight ptosis in the right eye. He could count fingers at a distance of 4 feet and conjunctival chemosis had also resolved. However, the range of movement remained unchanged.


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