ASHNR '98'
uhrad.com - Case #9
Clinical History: A 27 year old man on a first name basis with the file room personnel and three volumes of film jackets presents with painful, progressive but non-traumatic neck swelling.
Neurofibromatosis With Carotid Pseudoaneurysm
Findings: There is an amorphous soft tissue mass located within the skull base on the left side. There is extension into the cavernous sinus. There is enlargement of the trigeminal cistern on the left representative of dural ectasia. Additionally, there is a fairly homogeneously enhancing mass present in the left side of the neck in the expected position of the carotid sheath. This represents a large carotid pseudoaneurysm. The accompanying angiogram demonstrates the pseudoaneurysm.
Discussion: Giant aneurysms of the central nervous system are associated with coarctation, polycystic kidney, connective tissue disorders, and granulomatous vasculitis such as TB or syphilis. Surprisingly, the association with neurofibromatosis is not strong. In addition, this patient exhibits a plexiform neurofibroma of the orbit. This is a benign overgrowth of perineural elements (hamartoma). This finding is pathognomonic of NF1 and is most common in the face and extremities.
Reference:
Weir. Aneurysms Affecting the Central Nervous System. Williams and Wilkins, 1987.
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