The authors report a case of a developmentally normal child with a congenital complex torcular dural arteriovenous fistula (DAVF) who later, in his teenage years, developed several vermian cavernomas within a large cerebellar developmental venous anomaly (DVA). The patient had initially presented with an abnormally large head circumference but no neurological deficits. He underwent several partial embolization procedures in an attempt to decrease the blood supply of the fistula over the course of 8 years. Nine years following initial presentation, he presented with a fourth ventricular hemorrhage, due to development of a new vermian cavernoma adjacent to a previously known vermian DVA and suffered subsequent mild left-sided hemiataxia from which he later recovered. CT angiographic images demonstrated that the vermian DVA drained into the left transverse sinus, which also drained the torcular arteriovenous fistula. A routine follow-up MRI examination 10 years following initial presentation demonstrated interval development of several large cavernomas in the cerebellum, all within the DVA. The patient had no new symptoms at that time and was neurologically intact. This case report highlights the de novo development of multiple cavernous malformations potentially secondary to DAVF-induced venous congestion in a preexisting DVA.
Waleed Brinjikji, Kelly D. Flemming and Giuseppe Lanzino
Pui Man Rosalind Lai and Nirav J. Patel
Lorenzo Rinaldo, Waleed Brinjikji and Leonardo Rangel-Castilla
An 80-year-old female presented with a long history of severe pulsatile tinnitus, vertigo, and decreased hearing. She was found to have a large right-sided tentorial arteriovenous fistula (AVF) with enlarged deep draining veins, including the vein of Rosenthal. The patient underwent Onyx embolization of the fistula via a combined transarterial and transvenous approach resulting in complete obliteration of the fistula. Her symptoms improved immediately after the procedure and at 6-months’ follow-up she was clinically asymptomatic with no evidence of residual fistula on neuroimaging. Transvenous embolization of AVF is at times necessary when transarterial access is not possible.
The video can be found here: https://youtu.be/uOMHY7eaOoQ.
Waleed Brinjikji, Harry J. Cloft, Giuseppe Lanzino, Leonardo Rangel-Castilla and Pearse P. Morris
Arteriovenous fistulae of the internal maxillary artery are exceedingly rare, with less than 30 cases reported in the literature. Most of these lesions are congenital, iatrogenic, or posttraumatic. The most common presentation of internal maxillary artery fistulae is pulsatile tinnitus and headache. Because these lesions are single-hole fistulae, they can be easily cured with endovascular techniques. The authors present a case of a patient who presented to their institution with a several-year history of pulsatile tinnitus who was found to have an internal maxillary artery arteriovenous fistula, which was treated endovascularly with transarterial coil and Onyx embolization.
The video can be found here: https://youtu.be/fDZVMMwpwRc.
Embolization of middle cerebral artery aneurysms: ready for prime time?
Giuseppe Lanzino and Waleed Brinjikji
Deena M. Nasr, Waleed Brinjikji, Michelle J. Clarke and Giuseppe Lanzino
Spinal epidural arteriovenous fistulas (SEDAVFs) constitute a rare but treatable cause of vascular myelopathy and are a different subtype from the more common Type I spinal dural AVFs. The purpose of this study was to review a consecutive series of SEDAVFs from a single institution and report on the clinical presentations, functional status, and treatment outcomes.
The authors identified all SEDAVFs treated at their institution from 2005 to 2015. SEDAVFs were defined as spinal AVFs in which the fistulous connection occurred in the epidural venous plexus. The clinical presentation, functional status, immediate treatment outcomes, and long-term neurological outcomes were analyzed.
Twenty-four patients with SEDAVFs were included in this study. The patients' mean age at presentation was 66.9 years. The most common presenting symptoms were pain and numbness (22 patients, 91.7%), followed by lower-extremity weakness (21 patients, 87.5%). The mean duration of symptoms prior to diagnosis was 11.8 months. Eighteen patients (75.0%) were treated with endovascular therapy alone, 4 (16.7) with surgery, and 2 (8.3%) with a combination of techniques. There was 1 major treatment-related complication (4.2%). Fifteen patients (62.5%) had improvement in disability, and 12 patients (54.5%) had improvement in sensory symptoms.
SEDAVFs often present with lower-extremity motor dysfunction and sensory symptoms. With the availability of newer liquid embolic agents, these lesions can be effectively treated with endovascular techniques. Surgery is also effective at treating these lesions, especially in situations where endovascular embolization fails or is not safe and in patients presenting with mass effect from compressive varices.
Stephen P. Lownie and David M. Pelz
Waleed Brinjikji, Vivek N. Iyer, Christopher P. Wood and Giuseppe Lanzino
Patients with hereditary hemorrhagic telangiectasia (HHT) are known to suffer from high rates of cerebral arteriovenous malformations (AVMs). The authors performed a systematic review and meta-analysis of the literature examining prevalence rates, characteristics, and clinical presentation of cerebral AVMs in the HHT population.
To identify studies on AVM prevalence and characteristics in the HHT population, 4 databases (MEDLINE, EMBASE, Scopus and Web of Science) were searched by a reference librarian with over 30 years experience in systematic reviews and meta-analysis. The search period was January 1, 1990–March 2016. The following search terms were used: hereditary hemorrhagic telangiectasia, Osler-Weber-Rendu syndrome, AVM, brain AVM, arteriovenous malformation, arteriovenous fistula, prevalence, and epidemiology. The authors identified studies that examined the prevalence rates, characteristics, and clinical presentation of cerebral AVMs in patients with HHT. They assessed overall AVM prevalence rates as well as prevalence rates by age, sex, HHT type, and country/region. They also systematically reviewed the characteristics of AVMs, including rupture status, location, clinical presentation, angioarchitecture, and Spetzler-Martin grade. Data were analyzed using a random-effects meta-analysis model.
Thirty-nine studies were included in this meta-analysis. Thirty studies examined brain AVM prevalence rates in various HHT patient populations, and 18 studies examined AVM clinical and angiographic characteristics (9 studies examined both prevalence rates and AVM characteristics). The prevalence of brain AVMs in HHT patients was 10.4% (95% CI 7.9%–13.0%) with no significant difference between males (8.5%, 95% CI 4.9%–12.0%) and females (11.0%, 95% CI 5.9%–16.1%). Patients with HHT Type 1 (HHT1) had a significantly higher brain AVM prevalence (13.4%, 95% CI 9.5%–17.4%) compared with those with HHT Type 2 (HHT2) (2.4%, 95% CI 1.0%–3.8%) (p < 0.0001). In 55.2% (95% CI 38.3%–72.1%) of cases, the AVMs were symptomatic. Spetzler-Martin grade was 2 or less in 86.9% (95% CI 67.5%–95.2%) of patients.
The prevalence of brain AVMs in the HHT population is about 10%. HHT1 patients are significantly more likely to have brain AVMs than HHT2 patients. Most AVMs in the HHT population are symptomatic. The Spetzler-Martin grade for these lesions is 2 or less in nearly 90% of patients.
Thomas J. Sorenson, Waleed Brinjikji, Kelly D. Flemming and Giuseppe Lanzino
Incidental vascular lesions are being discovered at an increasing frequency due to widespread noninvasive brain imaging studies. One of these lesions has recently been termed a “pure arterial malformation” (PAM), which is defined as dilated, overlapping, and tortuous arteries forming a mass of arterial loops with a coil-like appearance in the absence of arteriovenous shunting. The pathogenesis of these lesions is not known, but a congenital etiology is suspected. The authors report the case of a 17-year-old female who was found to have a PAM of the posterior inferior cerebellar artery with adipose tissue interspersed within the arterial loops. The authors believe that this abnormal intracranial association between blood vessel and adipose tissue lends further support to the theory that PAMs are the result of a congenital malformation and are therefore safe to manage conservatively given their presumed benign natural history. Far from offering conclusive evidence, this unique case report adds to the growing body of PAM literature and strengthens an increasingly supported congenital theory of genesis.