Case Of Igg4-Related Eye Disease Requiring Differentiation From Carotid-Cavernous Fistula

Purpose We report our findings in a case of IgG4-related eye disease that was confused with carotid-cavernous fistula (CCF). Methods Case report: A 61-year-old man visited to our department for exophthalmos with dilated conjunctival vessels in both eyes. He had hyperthyroidism. At the first visit, his ocular movements were disturbed, and MRI showed funicular-like appearance of the superior ophthalmic veins. However, enlargements of the extraocular muscles were not detected, and thyroid ophthalmopathy was excluded. Cerebral angiography showed no flow from either internal carotid arteries to the cavernous sinus, and CCF was also excluded. Blood tests showed high level of IgG4 (281 mg/dl) and a IgG4-related eye disease was considered. Systemic examinations showed no other lesions. Although histological examinations could not be performed, we treated the patient with 30 mg prednisolone as a possible IgG4-related eye disease. However, the reduced vision and critical fusion frequency did not improve. Then we switched to steroid pulse therapy, and all findings including the MR images were improved. A biopsy could not be performed because of the high risk of bleeding. Thus, malignant lymphoma could not be completely excluded. The patient is being followed continuously to detect any recurrences. Results Conclusions We report our findings in a very rare case of IgG4-related eye disease with funicular-like appearance along the superior ophthalmic veins requiring differentiation from CCF. Ophthalmologists should consider IgG4-related eye diseases in cases with signs and symptoms of CCF and thyroid ophthalmopathy.