RESUMO
Carotid-cavernous fistulas (CCFs) are pathologic, arteriovenous communications between the carotid artery and cavernous sinus. They cause various complex neuro-ophthalmic symptoms by shunting the flow of arterial blood into the venous system. In this study, a systematic review is conducted on the neuro-ophthalmic presentations associated with CCFs. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses 2020 guidelines were followed during the systematic review. We searched PubMed, Scopus, and Web of Science from inception to December 31, 2023. Articles written in English on patients with confirmed CCFs reporting clinical features, diagnostic modalities, treatment approaches, and outcomes were included. Abstracted data included demography, clinical presentations, venous flow dynamics, trauma history, investigative methodology, approaches to treatment, and outcomes. Overall, 33 studies with a total number of 403 patients were included. The mean age at presentation was 42.99 years for patients with direct CCFs and 55.88 years for those with indirect CCFs. Preponderance was observed in male patients with direct CCFs, constituting 51.56%, while females predominated in those with indirect CCFs, at 56.44%. The clinical symptoms in all patients with CCFs were proptosis in 58 cases (14.39%), conjunctival congestion in 29 patients (7.20%), diplopia in nine patients (2.23%), vision blurring in four patients (0.99%), eyelid swelling in five patients (1.24%), pain in the eye in three patients (0.74%), and an upper lid mass in one patient (0.25%). Endovascular treatments, including coil and Onyx embolization, have been effective in relieving clinical symptoms and arresting the progression of these symptoms. In conclusion, the common clinical features in CCFs usually underline proptosis, congestion, and diplopia, necessitating a comprehensive neuro-ophthalmological review. Prompt identification of the symptoms of blurred vision is crucial to avoid permanent damage. Lid swelling, ocular pain, and an upper lid mass are less common but equally essential presentations for comprehensive evaluation. The recognition of these variable presentations is essential not only for timely intervention but also for the improvement in patient outcomes, thus emphasizing the role of clinician awareness in managing CCF cases.
RESUMO
Background: Cerebral arteriovenous malformations (CAVMs) are either clinically silent or symptomatic. The most common presentation in more than half of all CAVMs presenting patients is hemorrhage which is accompanied by long-standing neurological morbidity and mortality. This report presents a case of an atypical large, slow-flow paramedian AVM with a dilated venous varix managed with surgery. The impact of the intraoperative findings on the diagnosis and the operative technique will be discussed. Case Description: In otherwise, healthy 26-year-old male complained of repeated episodes of generalized seizures and loss of consciousness. Brain magnetic resonance imaging (MRI) revealed a right parietal paramedian arteriovenous malformation (AVM) with signs of an old hemorrhagic cavity beneath it. Digital subtraction angiography demonstrated a slow-filling AVM with dilated venous varix drains into the superior sagittal sinus. However, the exact point of drainage cannot be appreciated. The filling of the AVM occurred precisely with the beginning of the venous phase. Intraoperatively, we noticed a whitish spherical mass, thick hemosiderin tissue, and a large cavity below the nidus; then, a complication-free complete microsurgical resection of this high-grade AVM was performed. Postoperatively, the patient suffered two attacks of seizures in the first few hours after the surgery, for which he received antiepileptics. MRI was clear during follow-up, and the patient was seizure-free and neurologically intact. Conclusion: Parietal convexity AVMs are challenging lesions to tackle. However, the chronicity and the slow-filling of the AVM, in this case, can render the surgical pathway more direct and accessible.