We report an interesting case of a spontaneous occlusion of a carotid cavernous fistula (CCF) causing a paradoxical worsening of orbital symptoms. A 59-year-old woman presented to our institution with conjunctival injection, raised intraocular pressures (IOP) and mild exophthalmos of her left eye. A digital subtraction angiography (DSA) demonstrated a Type-D CCF draining into the left superior ophthalmic vein (SOV). The patient declined endovascular treatment. She presented 15 months later with acute exacerbation of her orbital signs and symptoms. A DSA showed no evidence of arteriovenous fistula, and a brain MRI was consistent with spontaneous thrombosis of the SOV. At her 2-week clinical assessment, the patient showed clinical improvement and her IOP were within normal limits. Spontaneous thrombosis of the SOV can trigger the obliteration of a CCF with possible paradoxical worsening of orbital symptoms. DSA is the gold standard of diagnosis and management is directed toward decreasing IOP.


We present an interesting case of a type-D carotid cavernous fistula (CCF) that closed spontaneously with a paradoxical worsening of the symptoms due to thrombosis of the superior ophthalmic vein (SOV). The authors also give directives for the management of these extremely rare cases.

issue art.gif