A 70-year-old female with a history of primary angle closure glaucoma (PACG) status post laser peripheral iridotomy (PI) of both eyes (OU) presented for further management. She complained of discomfort and redness of the right eye (OD). Past medical history was significant for hypertension, gastroesophageal reflux disease and a cerebrovascular accident 15
years prior. Medications included atenolol, alprazolam, omeprazole, aspirin and topical prednisolone four times a day OD. The duration of topical prednisolone treatment was approximately 1
week prior to her presentation to our institution. The treatment was deemed necessary by the referring physician for her complaint of discomfort and redness. No other topical medications were given. Intraocular pressure (IOP) was 22 OD and 16 of the left eye (OS). Exam showed mild injection OD, shallow anterior chambers, patent PIs and cataracts OU. Gonioscopy demonstrated narrow angles with extensive peripheral anterior synechiae OU. Dilated fundus exam showed increased vessel tortuosity OU.
Given the significant narrow angles despite patent PIs OU, cataract extraction with intraocular lens implantation OU was performed. She was treated postoperatively with topical moxifloxacin, nepafenac and prednisolone for a month. She did not receive dorzolamide or other sulfa derivatives. Postoperatively, the anterior chambers deepened and the angles opened significantly. IOP was noted to be 19 OD and 18 OS. No hypotony was detected throughout the entire course.
Two months after cataract surgery, she developed 4
mm of proptosis, resistance to retropulsion, tortuous corkscrew blood vessels and an orbital bruit OD. Gonioscopy revealed the recurrence of narrow angles OD.
A CCF was suspected and both computed tomography (CT) of the orbits with and without contrast and computed tomography angiography (CTA) of the head were performed. The CT orbits showed possible asymmetry of the superior ophthalmic veins (SOV). The CTA head showed atherosclerotic disease within the distal cavernous segments of the internal carotid arteries. As neither imaging modality was completely diagnostic and high suspicion for a CCF remained (based on the clinical examination findings), a six vessel cerebral angiogram was performed. The diagnostic cerebral angiogram showed a small indirect Barrow type D right carotid cavernous fistula with retrograde drainage into the right SOV (Figure ). Early filling of the right SOV was seen on right internal carotid artery injection; however, there were no feeders large enough to be actually visualized. On injection of the right external carotid artery, there was filling of the cavernous sinus via small branches of the right accessory meningeal artery. Our patient did not have a suitable endovascular corridor to the CCF via the petrosal sinuses, therefore transfemoral venous embolization did not appear possible. The plan was for transarterial embolization and if satisfactory occlusion could not be achieved from embolization of the right accessory meningeal artery feeder, then an alternative approach through the right SOV was to be considered. When diagnostic cerebral angiography was repeated prior to the planed embolization procedure, it was noted that she had undergone spontaneous partial closure of her CCF and the procedure was aborted.
Figure 1 Cerebral angiogram showing a small indirect Barrow type D right carotid cavernous fistula. Selective injection of the external carotid artery demonstrates filling of the cavernous sinus (arrow) and retrograde drainage into the right superior ophthalmic (more ...)
One month later, our patient developed worsening vision and was noted to have a choroidal detachment OD (Figures
A and B). She declined further angiographic testing and was thus started on self-administered manual carotid jugular compressions. One month later, she was noted to have progressive worsening of her choroidal detachments and angle closure (Figure C and D).
Figure 2 Photographs of the right eye taken one month (A, B) and two months (C, D) after closure of the CCF. (A) External photograph showing corkscrew blood vessels. (B) Fundus photograph showing intraretinal hemorrhages, tortuous retinal vessels and choroidal (more ...)
She eventually opted for repeat surgical intervention but when diagnostic cerebral angiography was performed prior to the embolization procedure, significant thrombosis of the CCF was noted. There was virtually no filling of the SOV on angiography and no intervention was warranted. Examination two months later showed complete resolution of her choroidal detachments and open angles OU.