Presentation
Left auricular swelling.
Patient Data
Absence of the left sphenoid wing. Consequently, the left temporal lobe with the surrounding prominent CSF bulges anteriorly with subsequent proptosis.
Left auricular subcutaneous irregular fairly defined soft tissue mass lesion eliciting low signal on T1 and high signal on T2 with post-contrast enhancement, suggestive of plexiform neurofibromatosis. It encroaches upon the left external auditory canal.
Absence of the left sphenoid wing. Consequently, the left temporal lobe with the surrounding prominent CSF bulges anteriorly with subsequent proptosis.
A gaping bony defect is seen in the posterior aspect of the right orbit giving the "bare orbit sign".
Absent anatomical landmarks of the optic nerve canal, anterior clinoid, superior, and inferior orbital fissures.
Hypoplasia of the left sphenoid sinus.
Left auricular subcutaneous isodense soft tissue mass lesion which encroaches upon and obliterates the left external auditory canal, suggestive of plexiform neurofibromatosis.
Small bony defect at the left temporal squamous suture.
Side findings: Enlarged nasopharyngeal adenoids. Left otomastoiditis. Bilateral maxillary, ethmoid and frontal sinusitis.
Case Discussion
The case presented with left auricular swelling. On MRI, it showed sphenoid wing dysplasia with mild subsequent proptosis. Hence, neurofibromatosis (NF) was radiologically considered. The left auricular swelling was presumed a plexiform neurofibroma.
Diagnosis of NF was confirmed clinically by the presence of café au lait patches and the diagnosis of plexiform neurofibroma was confirmed by pathology.
In patients with NF, sphenoid wing dysplasia is usually associated with neurofibromas in the ipsilateral superficial temporal fossa which are often contiguous with a radiologically abnormal temporo-squamosal suture.
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