Carotid cavernous fistula

Case contributed by Dr Mona Mohamed Naguib Sabala


Known hypertensive. Acute confusion and expressive dysphasia? CVA.

Patient Data

Age: 80 years
Gender: Female

Initial CT brain without contrast revealed intraventricular hemorrhage (IVH), more on the left side. 

 CT cranial angiogram was then advised to specify the cause of hemorrhage and rule out an underlying aneurysm. Abnormal and intense enhancement of the left superior ophthalmic vein was identified on the arterial phase, the vein is otherwise minimally dilated and there is no proptosis. 

Case Discussion

Caroticocavernous fistula (CCF) is an abnormal communication between the carotid arterial circulation and the cavernous sinus. Various classifications have been proposed for CCF, the most frequently used and most broadly divides caroticocavernous fistula into two main types: direct and indirect CCF. Direct CCFs are often secondary to trauma and most commonly seen in young male patients. Whereas, indirect CCFs usually result from a degenerative process in elderly female patients with systemic hypertension and atherosclerosis. 

Despite the similarity of symptoms and imaging findings between both types of CCFs, it is still possible to differentiate one type from another. Amongst the available imaging modalities, digital subtraction angiography DSA remains the gold standard for the diagnosis and classification of caroticocavernous fistula.  The patient's clinical history is also crucial for a correct diagnosis. In patients with subtle and slow progressing symptoms, an indirect CCF should be considered. In a patient with a history of trauma, exuberant and fast-progressing neuro-ophthalmic symptoms, a direct CCF should be initially considered. 

In this case, the age, sex, insidious onset of symptoms and absence of a history of trauma along with mild severity of imaging findings all favor the diagnosis of indirect CCF. 

Intracranial hemorrhage may occur as a complication of CCF and generally, the bleeding is adjacent to the ectatic veins (as in the above patient).

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