[A Case of Dural Arteriovenous Fistula that Developed 21 Months after Cerebral Venous Sinus Thrombosis].

A 56-year-old man experienced a sudden onset of left hemiparesis. The computed tomography(CT)scan revealed a lobar hemorrhage in the right fronto-parietal lobe. After his admission, deep vein thrombosis was detected in his left lower limb, and angiograms taken on the 36th day of hospitalization revealed cerebral venous sinus thrombosis. Anticoagulant treatment was induced. After 21 months, he experienced a sudden onset of left hemiparesis again. The CT scan revealed a new lobar hemorrhage in the right frontal lobe, and angiograms revealed that two dural arteriovenous fistulas(dAVF)developed in the superior sagittal sinus(SSS)and the left transverse-sigmoid sinus. The one in the SSS had retrograde drainage from the bilateral middle meningeal artery, and we performed transarterial embolization with 50% n-butyl-cyanoacrylate. Postoperative course was uneventful and no further stroke occurred. Intracranial dAVF is known to be an acquired disease caused by venous hypertension, but we rarely find new development of dAVFs after venous diseases. This patient's case will help to elucidate the pathophysiology of dAVF.

Curative glubran 2 embolization of cerebral arteriovenous malformations patient selection and initial results.

The liquid embolic agents currently used for embolization of cerebral arteriovenous malformations are Onyx and NBCA. Glubran 2, a cyanoacrylate-based synthetic glue, has recently been applied for embolization of cerebral arteriovenous malformations (AVMs). We report the clinical results of selected cerebral AVMs treated with Glubran 2 targeting for curative embolization. Between October 2011 and March 2013, 31 patients with cerebral AVMs were selected for curative embolization with Glubran 2. There were 19 men and 12 women with a mean age of 32 years (range 4-65 years). Initial clinical presentation included hemorrhage in 28 and seizures in three patients. AVM location was frontal in eight patients, parietal in four, occipital in eight temporal in six, cerebellar in two and cerebellar vermis in three patients. Follow-up was performed clinically and with angiography examination at three to six months. Clinical outcomes were evaluated based on the modified Rankin Scale (mRS). A mean of 2.5 (range, 1-12) feeding pedicles were embolized per patient. Complete angiographic obliteration of AVM was achieved in 27 patients. A hemorrhagic complication was observed in one patient, an ischemic complication in one patient and technical complications in four patients. There was no procedure-related disabling neurological deficit or death at discharge. Additional gamma knife radiosurgery was performed in five patients, including one patient with recurrent AVM. All of the patients had favorable clinical outcomes at three to six month follow-up (mRS≤2). The curative embolization technique with Glubran 2 for selected cerebral AVMs achieved a high initial complete obliteration rate with an acceptable complication frequency.

MicroRNA-18a improves human cerebral arteriovenous malformation endothelial cell function.

BACKGROUND AND PURPOSE: Cerebral arteriovenous malformation (AVM) is a vascular disease that disrupts normal blood flow and leads to serious neurological impairment or death. Aberrant functions of AVM-derived brain endothelial cells (AVM-BECs) are a disease hallmark. Our aim was to use microRNA-18a (miR-18a) as a therapeutic agent to improve AVM-BEC function. METHODS: Human AVM-BECs were tested for growth factor production and proliferation under different shear flow conditions and evaluated for tubule formation. Thrombospondin-1, inhibitor of DNA-binding protein 1, and vascular endothelial growth factor (VEGF) isotype mRNA levels were quantified by quantitative real-time polymerase chain reaction. Thrombospondin-1, VEGF-A, and VEGF-D protein expression was measured using enzyme-linked immunosorbent assay. Proliferation and tubule formation were evaluated using bromodeoxyuridine incorporation and growth factor-reduced Matrigel assays, respectively. RESULTS: miR-18a increased thrombospondin-1 production but decreased inhibitor of DNA-binding protein 1, a transcriptional repressor of thrombospondin-1. miR-18a reduced VEGF-A and VEGF-D levels, both overexpressed in untreated AVM-BECs. This is the first study reporting VEGF-D overexpression in AVM. These effects were most prominent under arterial shear flow conditions. miR-18a also reduced AVM-BEC proliferation, improved tubule formation, and was effectively internalized by AVM-BECs in the absence of extraneous transfection reagents. CONCLUSIONS: We report VEGF-D overexpression in AVM and the capacity of miR-18a to induce AVM-BECs to function more normally. This highlights the clinical potential of microRNA as a treatment for AVM and other vascular diseases.

Transarterial treatment with Onyx of Cognard type IV anterior cranial fossa dural arteriovenous fistulas.

BACKGROUND AND PURPOSE: Cognard type IV anterior cranial fossa dural arteriovenous fistulas (DAVFs) are rare lesions with a high risk of intracranial hemorrhage. We present our experience with the use of Onyx via the arterial route in these aggressive lesions. MATERIALS AND METHODS: Between October 2009 and October 2011, six consecutive patients diagnosed with Cognard type IV anterior cranial fossa DAVFs were treated transarterially with Onyx in our department. All patients were male; mean age was 55 years (range 38-68). Four patients presented with intracranial hemorrhage as the initial manifestation; one patient presented with seizures at the time of diagnosis and experienced intracranial hemorrhage during the antiepileptic therapy; and the other patient was asymptomatic. RESULTS: In five patients, complete obliteration was achieved with transarterial Onyx injection in a single treatment session; in the remaining patient, subtotal occlusion was achieved and gamma knife treatment was followed. The average time of injection was 19 min (range 5-28) for every pedicle catheterized and the average amount of Onyx was 3.2 ml (range 0.4-6.3) for each lesion. All patients recovered uneventfully after embolization. No mortality or permanent morbidity was observed in this series. Follow-up digital subtraction or MR angiography confirmed durable obliteration of the fistulas in five cured cases. No patients suffered intracranial hemorrhage during the follow-up period. CONCLUSIONS: In this small series, our experience with the use of Onyx for arterial embolization of Cognard type IV DAVFs is encouraging, with durable complete cure in most lesions without severe complications.

Propranolol use in PHACE syndrome with cervical and intracranial arterial anomalies: collective experience in 32 infants.

The objective of this retrospective study of patients evaluated between July 2008 and October 2011 in seven pediatric dermatology centers was to combine collective clinical experience using oral propranolol therapy in 32 infants with PHACE syndrome (Posterior fossa [brain malformations present at birth], Hemangioma [usually covering a large area of the skin of the head or neck >5 cm]; Arterial lesions [abnormalities of the blood vessels in the neck or head]; Cardiac abnormalities or aortic coarctation [abnormalities of the heart or blood vessels that are attached to the heart]; Eye abnormalities) with cervical or intracranial arterial anomalies. Patients were given an average daily dose of oral propranolol of 1.8 mg/kg divided two or three times per day for an average duration of 12.3 months. The main outcome measure was adverse neurologic events. Seven (22%) patients were categorized as being at higher risk for stroke, defined on magnetic resonance imaging as severe, long-segment narrowing or nonvisualization of major cerebral or cervical vessels without anatomic evidence of collateral circulation, often in the presence of concomitant cardiovascular comorbidities. Only one patient developed a change in neurologic status during propranolol treatment: mild right hemiparesis that remained static and improved while propranolol was continued. An additional three patients had worsening hemangioma ulceration or tissue necrosis during therapy. This is the largest report thus far of patients with PHACE syndrome treated with propranolol. Although no catastrophic neurologic events occurred, serious complications, particularly severe ulcerations, were seen in a minority of patients, and given the sample size, we cannot exclude the possibility that propranolol could augment the risk of stroke in this population. We propose radiologic criteria that may prove useful in defining PHACE patients as being at high or standard risk for stroke. We continue to advise caution in using systemic beta-blockers, particularly for children with vascular anomalies at higher risk for stroke. Use of the lowest possible dosage, slow dosage titration, three times per day dosing to minimize abrupt changes in blood pressure, and close follow-up, including neurologic consultation as needed, are recommended.

Transarterial embolization of intracranial dural arteriovenous fistulas with direct cortical venous drainage using ethylene vinyl alcohol copolymer (Onyx).

PURPOSE: This communication concerns the new possibilities and technical aspects of using ethylene vinyl alcohol copolymer (Onyx, EVAC) for endovascular treatment of intracranial dural arteriovenous fistulas with direct cortical venous drainage (DAVF-CVs). PATIENTS AND METHODS: Five patients with symptomatic DAVF-CVs were treated primarily with transarterial embolization using Onyx. RESULTS: All patients had complete obliteration of their DAVFs with a single Onyx injection that resulted in passage of embolic agent to the draining vein. One asymptomatic technical adverse event occurred (a broken microcatheter on retrieval). On clinical follow-up (mean 12.6 months, range 1-27 months), two patients with intracranial hemorrhage and one patient with cerebellar symptoms improved significantly after treatment, with residual symptoms that did not affect independence. One patient had remission of tinnitus and headache but developed seizures, and one patient was asymptomatic. Imaging follow-up (mean 4 months, range 1-7 months) did not show any revascularization. CONCLUSION: Embolization with Onyx represents a significant improvement in the endovascular treatment of DAVF-CVs. Cases that would not be effectively treated with cyanoacrylate or particles can be cured by embolization alone.