Surgical treatment of spinal CSF-venous fistulas: a systematic review

Abstract

CSF-venous fistula is a relatively new and unexplored cause of spontaneous intracranial hypotension syndrome (SIH). The syndrome was first described in 1974 in a patient with chronic lumbar puncture-associated headache after digital subtraction myelography. After 40 years, CSF-venous fistula was considered as the cause of the SIH syndrome in patients with orthostatic headache. CSF-venous fistula is a pathological connection of the subarachnoid space and the adjacent epidural vein or veins. It is one of the top three causes of SIH along with dural defects and meningeal diverticulum. Despite the insufficient assessment of the prevalence of CSF-venous fistulas, some authors consider the syndrome to be the cause of 25 % of the cases. Positional headaches, as well as headaches associated with cough and Valsalva maneuver, are the most common symptoms in patients with CSF-venous fistulas. These patients are characterized by typical MRI signs of intracranial hypotension, such as a clear visualization of pachymeninx, venous congestion, an increase in the size of the pituitary gland, brain sagging syndrome, tonsillar herniation, etc. However, due to the rarity of this disease, algorithms for its diagnosis and treatment have not been established. Management of patients is usually limited to conservative treatment as the first-line method. This includes EBP alone or in combination with fibrin glue. Open surgery for ligation or coagulation of the fistula is used in cases of failed conservative treatment. Endovascular occlusion of the draining vein is a relatively new and less common treatment. The article presents a systematic review of the literature and analysis of the clinical results of various types of treatment for CSF-venous fistulas (EBP, surgical excision and endovascular embolization).