Orbital plexiform neurofibromata

Case contributed by Dr Henry Knipe

Presentation

Proptosis.

Patient Data

Age: 20
Gender: Female

Severe left proptosis and stretching of the left optic nerve due to extensive lobulated abnormal enhancing T2 hyperintense soft tissue that is predominantly intraconal in location.

The optic nerve is completely encased by this soft tissue, which extends through the superior orbital fissure and expands the cavernous sinus. Further soft tissue at the superomedial and superior extraconal space.

The left extraocular muscles are swollen, particularly medial and inferior rectus and superior rectus/levator palpebrae.

Right orbit has a normal appearance. No sphenoid wing dysplasia identified.

Solitary high T2/FLAIR focus in the right thalamus with slight intrinsic T1 hyperintensity and no contrast enhancement.

Case Discussion

This patient has a reported history of neurofibromatosis type 1, with the extensive enhancing left orbital soft tissue most likely representing plexiform neurofibromas. Right thalamic signal abnormality consistent with FASI. 

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Case information

rID: 47703
Case created: 30th Aug 2016
Last edited: 31st Aug 2016
Inclusion in quiz mode: Included

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