Background: Giant perimedullary arteriovenous fistulas (GPMAVFs) located in the cervical region are a rare pathology with distinctive characteristics.
Objective: To evaluate clinical presentation and different endovascular treatment options of cervical GPMAVFs and review previously published data in the literature regarding cervical GPMAVFs.
Methods: Six patients with cervical GPMAVFs were found in the spinal vascular malformations database of our group collected between 1990 and 2009. Endovascular techniques and treatment outcomes were evaluated and compared with other published series.
Results: Clinical presentations were progressive motor deficit (5 patients), hematomyelia (1 patient), meningeal syndrome (1 patient), and respiratory arrest and gait apraxia (1 patient). Three patients were treated by the transarterial approach. One patient was treated by the transvenous approach due to previous embolizations resulting in a proximal occlusion and preventing a safe transarterial approach. A transvenous approach was used in another patient due to complex arterial anatomy. In 1 patient, direct percutaneous puncture of the venous pouch was necessary because of previous proximal occlusion of the arteries. All embolizations resulted in complete occlusions with clinical improvement, and there was no recanalization during a mean follow-up of 21 months.
Conclusion: Transarterial embolization of cervical GPMAVFs is safe and effective when it is done in highly experienced centers. Cervical GPMAVFs that cannot be accessed by the transarterial technique due to their complex angioarchitecture can be treated by transvenous embolization or direct puncture of the venous pouch.