Clinical spectrum of spontaneous carotid-cavernous fistula.

A carotid-cavernous fistula (CCF) is an abnormal communication between the cavernous sinus and the carotid arterial system. A CCF is divided into two categories, direct and indirect. Direct fistulas usually account for 70 to 90% of all CCF. Spontaneous, low-flow fistulas are usually associated with atherosclerosis, hypertension and collagen vascular disease or may develop in females during peripartum period. The elderly age group, especially women are at increased risk. We report three cases of spontaneous CCF presenting with ocular manifestations and hypertension, without any collagen vascular disease. One case was a direct variety and the other two were of indirect variety.