Introduction. The Consortium for Dural Arteriovenous Fistula Outcomes Research (CONDOR)

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Objective To explore the impact of outflow patency on radiosurgical outcomes of lateral sinus dural arteriovenous fistulas (DAVFs). Methods We retrospectively examined 83 lateral sinus DAVFs treated with Gamma Knife radiosurgery (GKRS) between 1995 and 2020. Two neuroradiologists blinded to the therapeutic outcomes served as imaging evaluators on pre-GKRS digital subtraction angiography (DSA) and magnetic resonance (MR) images. The sinovenous outflow of lateral sinus DAVF was scored using combined conduit score (CCS), ranging from 0 (total occlusion) to 8 (full patency). The patients’ follow-up MR and DSA images were used to validate the radiosurgical outcomes (obliteration or non-obliteration) of lateral sinus DAVF. Cox regression and Kaplan–Meier analyses were performed to determine the correlations between the variables and outcomes. Results Among the 83 cases, 60 (72%) lateral sinus DAVFs achieved obliteration after GKRS at a median latency period of 24.5 months. After adjustment for aggressive presentation, cortical venous reflux, straight sinus reflux, and optic nerve sheath enlargement, a CCS of >6 was independently associated with lateral sinus DAVF obliteration (hazard ratio: 2.335, p = 0.007). The estimated 36-month probabilities of obliteration were 80% versus 53.6% for lateral sinus DAVFs with a CCS of >6 versus ≤6. Conclusions Lateral sinus DAVFs with a CCS of >6, indicating a nearly patent sinovenous outflow, were more likely to be obliterated after GKRS. Sinovenous outflow patency is a factor associated with therapeutic outcome in radiosurgery for lateral sinus DAVFs.
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Background: Anterior cranial fossa dural arterio-venous fistulas (DAVFs) represent 6% of all intracranial DAVFs; characteristically they show an aggressive behaviour with high risk of intracranial haemorrhage. Peculiar anatomical features, such as feeding by the ethmoidal arteries and the pattern of venous drainage (frequently with varices that mimic aneurysmal dilatation), can be evaluated in detail only by digital subtraction angiography (DSA), which represents the "gold standard" in the diagnosis of such cranial fistulas. Recent technological developments in endovascular management of this type of DAVF have partially reduced the morbidity risk related to this modality of treatment. Our purpose is to present our experience in the surgical management of 14 patients with anterior cranial fossa DAVFs, with attention paid to the possible role of preoperative embolisation in these cases and to the surgical technique. Method: Between 1999 and 2015, 14 patients with anterior cranial fossa DAVFs were submitted to surgery in two neurosurgical departments; the mean age was 63 years old; nine DAVFs caused intracranial haemorrhage (subarachnoid haemorrhage in three cases, intracerebral haemorrhage in six cases). Pre-operative embolisation was attempted in an early case and was successfully done in one recent case. In all patients, the surgical approach chosen was a pterional craniotomy with a low margin on the frontal bone in order to gain the exposure of the anterior cranial fossa and especially of the olphactory groove region; the resection of the falx at its insertion on the crista galli was needed in five cases in order to get access to the contralateral afferent vessels. Cauterisation of all the dural feeders on and around the lamina cribrosa was needed in all cases; venous dilatations were evident in eight patients (in seven out of nine patients with ruptured DAVF and in one out of five patients with unruptured DAVF) and were removed in all cases. One patient harboured an ophthalmic artery aneurysm, which was excluded by clipping. Results: One patient died 5 days after surgery due to the severity of the pre-operative haemorrhage. Postoperative DSA showed the disappearance of the DAVF and of the venous pseudo-aneurysms in all cases. Clinical outcome was favourable (without neurological deficits) in 11 patients; three patients presented an unfavourable clinical outcome, due to the severity of the initial haemorrhage. Conclusions: Surgical exclusion of the anterior cranial fossa DAVFs still represents the gold standard for such lesions, due to low post-operative morbidity and to complete protection against future rebleedings; endovascular techniques may help the surgeon in complex cases.
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The Orphan Drug Act (ODA) [1], first enacted in the United States in 1983, was set up to encourage the development of drugs for rare diseases. At that time, drug therapies for such diseases were rarely developed. Three decades later, a growing proportion of industry research and development (R&D) [2] and regulatory drug approvals [3] target diseases affecting fewer than 200,000 persons in the United States, the prevalence-based threshold of rare disease under the ODA.
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Ethmoidal dural arteriovenous fistulae are rare vascular malformations associated with a high risk of bleeding. We present a multicenter contemporary series of patients treated with microsurgical and endovascular techniques. Sixteen consecutive patients were evaluated and/or treated between 2008 and 2015 at four centers with large experience in the endovascular and surgical treatment of cerebrovascular diseases. We analyzed demographic and clinical data, risk factors for dural fistulas, treatment type, peri- and post-operative morbidity, clinical and radiological outcomes, rates of occlusion, and long-term neurological outcome. Sixteen patients (81 % men, mean age of 58 years) with ethmoidal dural fistulas were included in the analysis. Seven patients had suffered an intracranial hemorrhage; the remaining presenting with neurological signs and symptoms or the fistula was an incidental finding. Three patients were managed conservatively. Among patients who underwent intervention (n = 13), 46.1 % were treated with endovascular therapy and 53.9 % were treated surgically. Complete angiographic obliteration was achieved in 100 % immediately after treatment and at last follow-up evaluation. All patients experienced a favorable neurological recovery (mRS 0–2) at the last follow-up visit (12 months). Ethmoidal dural AVFs are found mostly in male patients. Nowadays, due to wider use of non-invasive imaging, AVFs are discovered with increasing frequency in patients with minimal or no symptoms. Traditionally, these fistulas were considered “surgical.” However, in the modern endovascular era, selected patients can be effectively and safely treated with embolization although surgical ligation continues to have an important role in their management.
Background and purpose: The introduction of liquid embolic agents has revolutionized endovascular approach to cranial vascular malformations. The aim of the study was to retrospectively assess the efficacy and safety of Precipitating Hydrophobic Injectable Liquid (PHIL), a new nonadhesive liquid embolic agent, in the treatment of patients with cranial dural arteriovenous fistulas. The primary end point was the rate of complete occlusion of dural arteriovenous fistulas. Secondary end points included the incidence of adverse events and clinical status at 3-month follow-up. Materials and methods: This was a retrospective multicenter study. Twenty-six consecutive patients with dural arteriovenous fistulas (de novo or previously treated) treated by injection of PHIL only or with PHIL in combination with other embolization products (such as Onyx or detachable coils) were included in the study. Recruitment started in August 2014 and ended in September 2015. Results: Twenty-two (85%) patients were treated with PHIL only, with 3 patients treated with both PHIL and Onyx, and 1, with both PHIL and coils. Immediate complete angiographic occlusion was achieved in 20 (77%) patients. Of the 6 patients with residual fistulas, 3 were retreated with PHIL and 1 achieved angiographic cure. An adverse event was seen in 1 patient who developed worsening of preexisting ataxia due to acute thrombosis of the draining vein. Conclusions: PHIL appears to be safe and effective for endovascular treatment of cranial dural arteriovenous fistulas. Short-term angiographic and clinical results are comparable with those of Onyx, with the added advantage of easier preparation and improved homogeneous cast visualization. The use of iodine as a radio-opacifier also produces considerably less artifacts on CT compared with tantalum-based embolic materials.
Background and Purpose There are few reports describing the prevalence of dural arteriovenous fistulas (dAVFs). We conducted a nation-wide retrograde survey on the clinical frequency and the status of treatment of dAVFs in Japan. Methods The first questionnaire was sent to 1,236 certified neurosurgery clinics in Japan to ask about the number of patients treated and the location of dAVF during the 5 years from 1998 to 2002. The second questionnaire was sent to 102 large-volume centers to ask the patients’ ages, gender, location of the fistula, clinical presentation, treatment modalities, clinical and radiological results, and recurrence. Results In the first survey, 1,815 cases were chosen from 338 clinics, including 826 cavernous sinus, 514 transverse-sigmoid sinus, 105 spinal, and 370 lesions in other places. The estimated detection rate was 0.29 per 100,000 persons per year in Japan. In the second survey, there were 1,490 cases (mean age: 62.7 ; 628 men and 850 women) that were culled from 68 high-volume centers. The initial clinical presentation was ocular symptoms (45 % of the cases), tinnitus (20 %), intracranial hemorrhage (16 %), and non-hemorrhagic neurological deficits (20 %). Treatment modality was endovascular therapy (76 % of the patients), open surgery (13 %), irradiation (3.4 %), and conservative therapy (11 %). Angiographic results were total obliteration in 59 % of the patients and subtotal obliteration in 16 %. Clinical symptoms disappeared in 54 % of the patients, improved in 16 %, unchanged in 7 %, worsened in 1.5 %, and death occurred in 1.2 %. Recurrence was reported in 5.9 % of the patients. Conclusions This survey delineated the clinical frequency and the epidemiologic features of dAVF in Japan.
Object: In this paper the authors' goal was to review the clinical features and outcome of patients with intracranial dural arteriovenous fistulas (DAVFs) who presented with hemorrhage. Methods: A retrospective study of 28 patients with DAVFs who presented with intracranial hemorrhage to 2 separate institutions was performed. The information reviewed included clinical presentation, location and size of hemorrhage, angiographic features, treatment, and clinical and radiologically documented outcomes. Clinical and radiological follow-up were available in 27 of 28 patients (mean follow-up 17 months). Results: The vast majority of patients were male (86%), and the most common presenting symptom was sudden-onset headache. All DAVFs had cortical venous drainage, and about one-third were associated with a venous varix. The most common location was tentorial (75%). Treatment ranged from endovascular (71%), surgical (43%), Gamma Knife surgery (4%), or a combination of modalities. The majority of fistulas (75%) were completely obliterated, and most patients experienced excellent clinical outcome (71%, modified Rankin Scale score of 0 or 1). There were no complications in this series. Conclusions: Case series, including the current one, suggest that the vast majority of patients who present with intracranial hemorrhage from a DAVF are male. The most common location for DAVFs presenting with hemorrhage is tentorial. Excellent outcomes are achieved with individualized treatment, which includes various therapeutic strategies alone or in combination. Despite the hemorrhagic presentation, almost two-thirds of patients experience a full recovery with no or minimal residual symptoms.
Common estimates of the prevalence rate for pial arteriovenous malformations (AVMs) of the brain vary widely, and their accuracy is questionable. Our objective was to critically review the original sources from which these rates were derived and to establish best estimates for both the incidence and prevalence of the disease. We reviewed all of the relevant original literature: autopsy series, the Cooperative Study of Intracranial Aneurysms and Subarachnoid Hemorrhage and related analyses, and other population-based studies. We also modeled the confidence intervals of estimates for a process of low prevalence such as AVMs. Many of the prevalence estimates (500-600/100,000 population) were based on autopsy data, a source that is inherently biased. Other estimates (140/100,000 population) originated from an inappropriate analysis of data from the Cooperative Study. The most reliable information comes from a population-based study of Olmsted County, MN, but prevalence data specific to AVMs was not found in that study. The estimates for AVM prevalence that are published in the medical literature are unfounded. Because of the rarity of the disease and the existence of asymptomatic patients, establishing a true prevalence rate is not feasible. Owing to variation in the detection rate of asymptomatic AVMs, the most reliable estimate for the occurrence of the disease is the detection rate for symptomatic lesions: 0.94 per 100,000 person-years (95% confidence interval, 0.57-1.30/100,000 person-years). This figure is derived from a single population-based study, but it is supported by a reanalysis of other data sources. The prevalence of detected, active (at risk) AVM disease is unknown, but it can be inferred from incidence data to be lower than 10.3 per 100,000 population.
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