A 64-year-old man was admitted to our hospital with gradually worsening nausea, dysphagia, double vision, grogginess, and gait disturbances. On admission, the patient showed dysarthria, horizontal ocular nystagmus to the left, paresis of cranial nerves IX and X, and ataxia on the right side. Magnetic resonance imaging (MRI) revealed an isolated lesion in the medulla. Cerebral angiography (CAG) showed a CCJAVF with the coexistence of intradural AVF and dural AVF, fed by the right first cervical radiculomedullary, right vertebral, and intradural posterior inferior cerebellar arteries, which were drained by the anterior spinal vein in an ascending direction. The patient underwent direct surgery to occlude dural and intradural fistulas. Postoperatively, the patient returned to work with full recovery from the neurological deficits via rehabilitation. MRI revealed vanishing brainstem congestion, and CAG revealed complete disappearance of the AVF.

CCJAVFs with venous drainage around the brainstem, regardless of their direction (ascending or descending), can cause isolated brainstem congestion, although this condition is rare. Keywords : Arteriovenous fistula (AVF), Brainstem congestion, The craniocervical junction (CCJ), Drainage