AIM: To explore the clinical manifestations and diagnostic features of spontaneous carotid-cavernous sinus fistula so as to improve the recognition and avoid misdiagnosis.

METHODS: Clinical data from 14 patients with spontaneous carotid-cavernous sinus fistula including clinical symptoms and signs, imaging, treatment were analyzed retrospectively.

RESULTS: The most common ocular symptoms in all 14 patients were exophthalmos(100%)and eye redness(93%). The CT or MRI scan performed in 12 cases revealed enlargement of the superior ophthalmic veins in 9 cases and distension of the cavernous sinus in 6 cases. The ocular manifestations improved apparently in all 4 cases undergoing embolization therapy.

CONCLUSION: Due to the wide and various clinical spectrum of spontaneous carotid-cavernous fistula, having a good grasp of diagnostic ocular features and imaging findings is significant to improve the diagnostic ability. Embolization therapy is effective in improving the ocular symptoms.