Intracranial dural arteriovenous fistula as a cause for symptomatic superficial siderosis: A report of two cases and review of the literature

• Type of Material: Article
• Authors: Griffin R. Baum, Emory University; Nefize Turan, Emory University; Ferdinando S. Buonanno, Massachusetts General Hospital; Gustavo Pradilla, Emory University; Raul Nogueira, Emory University
• Language: English
• Date: 2016-09-01
• Publisher: Medknow Publications
• Publication Version: Final Published Version
• Copyright Statement: © 2016 Surgical Neurology International; Published by Wolters Kluwer - Medknow.
• License: Creative Commons Attribution-NonCommercial-ShareAlike 3.0 Unported
• Final Published Version (URL): http://dx.doi.org/10.4103/2152-7806.179577
• Title of Journal or Parent Work: Surgical Neurology International
• ISSN: 2229-5097
• Volume: 7
• Issue: Suppl 9
• Start Page: S223
• End Page: S227
• Keywords: Neurology; embolization; Neurosurgery; Dural arteriovenous fistula; superficial siderosis
• Research Categories: Health Sciences, Medicine and Surgery; Biology, Neuroscience

Abstract

Background: Superficial siderosis (SS) is the occult deposition of hemosiderin within the cerebral cortex due to repeat microhemorrhages within the central nervous system. The collection of hemosiderin within the pia and superficial cortical surface can lead to injury to the nervous tissue. The most common presentation is occult sensorineural hearing loss although many patients have been misdiagnosed with diseases such as multiple sclerosis and amyotrophic lateral sclerosis before being diagnosed with SS. Only one case report exists in the literature describing an intracranial dural arteriovenous fistula (dAVF) as the putative cause for SS. Case Description: We describe two cases of SS caused by a dAVF. Both patients had a supratentorial, cortical lesion supplied by the middle meningeal artery with venous drainage into the superior sagittal sinus. In both patients, symptoms improved after endovascular embolization. The similar anatomic relationship of both dAVFs reported presents an interesting question about the pathogenesis of SS. Similar to the pathologic changes seen in the formation of intracranial arterial aneurysms; it would be possible that changes in the blood vessel lining and wall might predispose a patient to chronic, microhemorrhage resulting in SS. Conclusions: We describe the second and third cases of a dAVF as the cause of SS, and the first cases of successful treatment of SS-associated dAVF with endovascular embolization. As noninvasive imaging techniques become more sensitive and easily obtained, one must consider their limitations in detecting occult intracranial vascular malformations such as dAVF as a possible etiology for SS.