Introduction We review a patient with a known occipital arteriovenous malformation. He subsequently presented with worsening symptoms and was found to have elevated CSF pressure. We review the pathophysiology for the development of intracranial hypertension in patients with arteriovenous malformations and the implications for management. A literature review identified a small number of previous cases.

Case presentation 12 months after diagnosis of an AVM a 28 year-old male complained of worsening headaches and blurred vision. A repeat scan was unchanged. At lumbar puncture the CSF opening pressure was grossly elevated. He was referred to the neurosurgical unit where the decision was made to place a ventriculoperitoneal shunt.

Discussion Raised CSF pressure which is not due to AVM growth, swelling or bleeding is an uncommon but previously documented risk. A number of theories have been suggested for the development of raised intracerebral pressure in these cases, although it remains poorly understood. In patients with persistently raised CSF pressure there is a significant risk of visual loss; therefore cases need to be identified promptly and receive definitive management. The decision to operate should be made electively, and only when the risk of surgery is outweighed by the risk of the lesion itself.