A carotid-cavernous fistula results from an abnormal communication between the arterial and venous systems within the cavernous sinus in the skull. It is a type of arteriovenous fistula. As arterial blood under high pressure enters the cavernous sinus, the normal venous return to the cavernous sinus is impeded and this causes engorgement of the draining veins, manifesting most dramatically as a sudden engorgement and redness of the eye of the same side. Presentation
CCF symptoms include bruit (a humming sound within the skull due to high blood flow through the arteriovenous fistula), progressive visual loss, and pulsatile proptosis or progressive bulging of the eye due to dilatation of the veins draining the eye. Pain is the symptom that patients often find the most difficult to tolerate.Patients usually present with sudden or insidious onset of redness in one eye, associated with progressive proptosis or bulging.They may have a history of similar episodes in the past. Causes
Carotid cavernous fistulae may form following closed or penetrating head trauma, surgical damage, rupture of an intracavernous aneurysm, or in association with connective tissue disorders, vascular diseases and dural fistulas. Diagnosis
This is based on MRI scan, magnetic resonance angiography and CT scan. A cerebral digital subtraction angiography (DSA) enhances visualization of the fistula. CT scans classically show an enlarged superior ophthalmic vein, cavernous sinus enlargement ipsilateral (same side) as the abnormality and possibly diffuse enlargement of all the extraocular muscles resulting from venous engorgement. Selective arteriography is used to evaluate arteriovenous fistulas.
Diagnosis
Fasting plasma somatostatin greater than 30 pg/mL.SRS (Somatostatin Receptor Scintigraphy) – It is a radio-nucleotide scan by giving Octreotide tagged with Indium111 isotope, which shows an increase in uptake by the tumour cells. Treatment
Treatment is by chemotherapy with streptozocin, dacarbazine, doxorubicin or by watchful waiting and surgical debulking via Whipple procedure and other resections of the gastrointestinal organs affected. References

6. Soga J, Yakuwa Y. Somatostatinoma/inhibitory syndrome: a statistical evaluation of 173 reported cases as compared to other pancreatic endocrinomas. J Exp Clin Cancer Res, 1999; 18:13–22. [PMID: 10374671]


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