Classification and Management Considerations for Intraosseous Dural Arteriovenous Fistulae.

BACKGROUND: Intraosseous dural arteriovenous fistulas (IODAVFs) are rare DAVFs that communicate with marrow. Given their infrequency, common nomenclature is nonexistent. Patients may present with benign symptoms, such as tinnitus, or venous hypertension symptoms including hemorrhage depending on the venous outflow pattern. OBJECTIVE: To describe all available cases of IODAVF in the literature, in addition to our cases, to better define presentation, and treatment outcomes. To advance a classification system to develop common language for these lesions for clinicians and researchers. METHODS: Neurointerventional procedure logs at 2 high-volume neurovascular centers were reviewed for all cases of IODAVFs, as was the English-based literature available in PubMed. The angioarchitecture, symptoms, management, and demographics were reviewed and summarized. RESULTS: Four institutional cases were identified, 2 of which had shunting within the marrow (clival or petrous), with venous drainage toward the heart. One case involved the dorsum sella with drainage into the superior petrosal sinus with reflux into the anterior and posterior spinal venous plexuses, and one involved the left petroclival junction, resulting in communication with the cavernous sinus with retrograde drainage into the superior ophthalmic veins. Two patients were managed by observation, one was treated with radiosurgery and one with microsurgical skeletonization. Twenty additional cases from the literature are summarized. CONCLUSION: IODAVFs of the cerebrocranial vasculature may present incidentally, with tinnitus, or with symptoms related to mass effect or venous hypertension. We propose a classification which accounts for drainage patterns. Further study is needed for these rare lesions.