Background: A craniocervical junction arteriovenous fistula (CCJAVF) is a rare vascular malformation, and its etiology remains unclear. Here, to the best of the authors' knowledge, they present the first case of CCJAVF associated with thrombus formation in the ipsilateral internal jugular vein.
Observations: An 80-year-old man presented with a sudden occipital headache. Computed tomography revealed a subarachnoid hemorrhage surrounding the brainstem and upper cervical cord. Digital subtraction angiography showed a CCJAVF fed by the left C2 radiculomeningeal artery with ascending intracranial drainage and epidural plexus. After endovascular treatment, the authors retrospectively found that his ipsilateral internal jugular vein and innominate vein were occluded with a huge thrombus at admission.
Lessons: This case suggested a restricted antegrade venous flow due to thrombus-induced progressive retrograde intracranial drainage causing hemorrhage. Venous hypertension should be considered one of the causes of hemorrhage due to CCJAVF as well as intracranial arteriovenous fistulas.
Keywords: arteriovenous fistula; craniocervical junction; internal jugular vein; subarachnoid hemorrhage; venous thrombosis.