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Concept: Arteriovenous malformation

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Object The focus of the present study was the evaluation of outcomes after unstaged and staged-volume Gamma Knife surgery (GKS) in children harboring intracranial arteriovenous malformations (AVMs). Methods Twenty-two children (median age 9.5 years) underwent GKS for AVMs and were followed up for at least 2 years thereafter. The disease manifested with intracranial hemorrhage in 77% of cases. In 68% of patients the lesion affected eloquent brain structures. The volume of the nidus ranged from 0.1 to 6.7 cm(3). Gamma Knife surgery was guided mainly by data from dynamic contrast-enhanced CT scans, with preferential targeting of the junction between the nidus and draining vein. The total prescribed isodose volume was kept below 4.0 cm(3), and the median margin dose was 22 Gy (range 20-25 Gy). If the volume of the nidus was larger than 4.0 cm(3), a second radiosurgical session was planned for 3-4 years after the first one. Nine patients in the present series underwent unstaged radiosurgery, whereas staged-volume treatment was scheduled in 13 patients. Results Complete obliteration of the AVM was noted in 17 (77%) of 22 patients within a median period of 47 months after the last radiosurgical session. Complete obliteration of the lesion occurred in 89% of patients after unstaged treatment and in 62.5% after staged GKS. Four (67%) of 6 high-grade AVMs were completely obliterated. Complications included 3 bleeding episodes, the appearance of a region of hyperintensity on T(2)-weighted MR images in 2 patients who had no symptoms, and reappearance of the nidus in the vicinity of the completely obliterated AVM in 1 patient. Conclusions Radiosurgery is a highly effective management option for intracranial AVMs in children. For larger lesions, staged GKS may be applied successfully. Initial targeting of the nidus adjacent to the draining vein and application of a sufficient radiation dose to a relatively small volume (≤ 4 cm(3)) provides a good balance between a high probability of obliteration and a low risk of treatment-related complications.

Concepts: Brain tumor, Anatomical pathology, Radiobiology, Gamma knife, Neurosurgery, Congenital disorders, Arteriovenous malformation, Cerebral arteriovenous malformation

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BACKGROUND:: Anatomic diversity among cerebellar arteriovenous malformations (AVMs) calls for a classification that is intuitive and surgically informative. Selection tools like the Spetzler-Martin grading system are designed to work best with cerebral AVMs but have shortcomings with cerebellar AVMs. OBJECTIVE:: To define subtypes of cerebellar AVMs that clarify anatomy and surgical management, to determine results according to subtypes, and to compare predictive accuracies of the Spetzler-Martin and supplementary systems. METHODS:: From a consecutive surgical series of 500 patients, 60 had cerebellar AVMs, 39 had brainstem AVMs and were excluded, and 401 had cerebral AVMs. RESULTS:: Cerebellar AVM subtypes were as follows: 18 vermian, 13 suboccipital, 12 tentorial, 12 petrosal, and 5 tonsillar. Patients with tonsillar and tentorial AVMs fared best. Cerebellar AVMs presented with hemorrhage more than cerebral AVMs (P < .001). Cerebellar AVMs were more likely to drain deep (P = .04) and less likely to be eloquent (P < .001). The predictive accuracy of the supplementary grade was better than that of the Spetzler-Martin grade with cerebellar AVMs (areas under the receiver-operating characteristic curve, 0.74 and 0.59, respectively). The predictive accuracy of the supplementary system was consistent for cerebral and cerebellar AVMs, whereas that of the Spetzler-Martin system was greater with cerebral AVMs. CONCLUSION:: Patients with cerebellar AVMs present with hemorrhage more often than patients with cerebral AVMs, justifying an aggressive treatment posture. The supplementary system is better than the Spetzler-Martin system at predicting outcomes after cerebellar AVM resection. Key components of the Spetzler-Martin system such as venous drainage and eloquence are distorted by cerebellar anatomy in ways that components of the supplementary system are not. ABBREVIATIONS:: AICA, anterior inferior cerebellar artery;AVM, arteriovenous malformationmRS, modified Rankin ScalePICA, posterior inferior cerebellar arteryROC, receiver-operating characteristicSCA, superior cerebellar artery.

Concepts: Cerebral cortex, Cerebellum, Anterior inferior cerebellar artery, Arteries of the head and neck, Arteriovenous malformation, Basilar artery, Superior cerebellar artery, Inferior cerebellar veins

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OBJECTIVES: To evaluate the safety and efficacy of a new liquid embolic agent in brain arteriovenous malformation (bAVMs) embolisation. METHODS: A prospective, multicentre series was conducted at 11 interventional centres in Europe to evaluate embolisation of bAVMs with the new liquid embolic agent. Technical conditions, complications, clinical outcome and anatomical results were independently analysed. RESULTS: From December 2005 to December 2008, 117 patients (72 male; 45 female, aged 18-75 years) were included. Clinical presentation was mostly haemorrhage (34.2 %) and seizures (28.2 %). Most AVMs were located in the brain hemispheres (85.5 %). AVMs were <3 cm in 52.1 % of patients and ≥3 cm in 47.9 %. Morbidity was observed in 6/117 patients (5.1 %), related to haemorrhagic events in 2 cases and non-haemorrhagic complications in 4 cases. Five patients (4.3 %) died in relation to the treatment (bleeding in 4 patients and extensive venous thrombosis in 1). Complete occlusion of the AVM by embolisation alone was obtained in 23.5 % of patients. Complementary treatment was performed in 82.3 % of patients with partial AVM occlusion, mostly radiosurgery. CONCLUSIONS: In this prospective, multicentre, European, observational series, the new liquid embolic agent proved to be suitable for BAVM embolisation, with acceptable morbidity and mortality and good efficacy. KEY POINTS: • Numerous interventional techniques have been used to embolise brain arteriovenous malformations (AVMs). • This prospective multicentre study demonstrates the suitability of a liquid embolic agent. • The safety of treatment using Onyx is acceptable. • Such embolisation leads to complete AVM occlusion in 23.5 % of patients.

Concepts: Blood, Stroke, Cerebral cortex, Bleeding, Centers for Disease Control and Prevention, Congenital disorders, Arteriovenous malformation, Cerebral arteriovenous malformation

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BACKGROUND:: Accurate target delineation has significant impact on brain arteriovenous malformations (AVMs) obliteration, treatment success, and potential complications of stereotactic radiosurgery. OBJECTIVE:: We compare the nidal contouring of AVMs using fused images of contrasted computerized tomography (CT) and magnetic resonance (MRI) with matched images of three-dimensional (3-D) cerebral angiography for Cyberknife radiosurgery (CKRS) treatment planning. METHODS:: Between May 2009 and April 2012, 3-D cerebral angiography was integrated into CKRS target planning for thirty consecutive patients. The AVM nidal target volumes were delineated using fused CT and MRI scans versus fused CT, MRI, and 3-D cerebral angiography for each patient. RESULTS:: The mean volume of the AVM nidus contoured with the addition of 3-D cerebral angiography to the CT/MRI fusion (9.09 cm, 95% CI 5.39-12.8 cm) was statistically smaller than the mean volume contoured with CT/ MRI fused scans alone (14.1 cm, 95% CI 9.16-19.1 cm), with a mean volume difference of δ=5.01 cm (p=0.001). Diffuse AVM nidus was associated with larger mean volume differences in comparison to a compact nidus (δ=6.51 vs. 2.11 cm, p=0.02). The mean volume difference was not statistically associated with the patient’s gender (male δ=5.61, female δ=5.06, p=0.84), prior hemorrhage status (yes δ=5.69, no δ=5.23, p=0.86), or prior embolization status (yes δ=6.80, no δ=5.95, p=0.11). CONCLUSION:: For brain AVMs treated with CKRS, the addition of 3-D cerebral angiography to CT/MRI fusions for diagnostic accuracy results in a statistically significant reduction in contoured nidal volume as compared to standard CT/MRI fusion-based contouring.

Concepts: Medical imaging, Brain tumor, Radiosurgery, Radiology, Neurosurgery, Stereotactic surgery, Arteriovenous malformation, Cyberknife

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Abstract We report a case of a patient with a high flow arteriovenous malformation (AVM)i n the orbit, who developed a severe compartment syndrome, and was successfully treated with manual carotid compressions. The patient suffered a progressive proptosis, restriction of ocular motility and decreased of the visual acuity of the right eye. Embolization had a high risk of serious complications due to fistula location, so an alternative treatment consisting in carotid compressions was prescribed. Manual carotid compression is a non-invasive alternative technique to treat AVMs in patients with high risk of embolization episodes.

Concepts: Patient, Patience, Visual acuity, Arteriovenous malformation

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BACKGROUND AND PURPOSE: Epileptic seizures account for 24-40% of all clinical onsets in patients with brain arteriovenous malformations (AVMs). METHODS: We retrospectively reviewed the angioarchitectural features of AVMs associated with seizures in 168 patients admitted to our Department from 1997 to 2012. Patients were dichotomized according to demographic characteristics, type of treatment, bleeding occurrence, and morphological and topographic features. Clinical status at admission and discharge was also recorded. The association of each one of these variables with seizures occurrence was statistically tested. Continuous variables and outcome were compared with Student’s t-test, whereas categorical ones were compared using Fisher’s exact test. The independent contribution of some seizures predictors was assessed with a logistic regression model. Associations were considered significant for P < 0.05. RESULTS: About 29% patients showed seizures and 47% bleeding. No significant difference in age and sex was observed between patients with and without seizures. AVMs > 4 cm (P = 0.001) and those fed by dilated arterial feeders (P = 0.02) were associated with increased risk of seizures. A higher risk of seizures occurrence was also observed in cortical AVMs compared with deeper ones (75.5% vs. 55.4%; P = 0.01), and in AVMs fed by middle and posterior cerebral arteries branches compared with the other vessels (81.6% vs. 45.3%; P < 0.001 and 48.9% vs. 23.5%; P = 0.002, respectively). No lobar predisposition was observed. A nidus > 4 cm also appeared as an independent risk factor of seizures occurrence (OR 2.82; 95% CI, 1.26-6.31; P = 0.009) at logistic regression analysis. CONCLUSIONS: AVM morphology, especially nidus dimension, appeared to more significantly influence seizures occurrence than their topography.

Concepts: Regression analysis, Logistic regression, Statistics, Statistical significance, Prediction interval, Fisher's exact test, Arteriovenous malformation, F-test

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Arteriovenous malformation (AVM) refers to a vascular anomaly where arteries and veins are directly connected through a complex, tangled web of abnormal AV fistulae without a normal capillary network. Hereditary hemorrhagic telangiectasia (HHT) types 1 and 2 arise from heterozygous mutations in endoglin (ENG) and activin receptor-like kinase 1 (ALK1), respectively. HHT patients possess AVMs in various organs, and telangiectases (small AVMs) along the mucocutaneous surface. Understanding why and how AVMs develop is crucial for developing therapies to inhibit the formation, growth, or maintenance of AVMs in HHT patients. Previously, we have shown that secondary factors such as wounding are required for Alk1-deficient vessels to develop skin AVMs. Here, we present evidences that AVMs establish from nascent arteries and veins rather than from remodeling of a preexistent capillary network in the wound-induced skin AVM model. We also show that VEGF can mimic the wound effect on skin AVM formation, and VEGF-neutralizing antibody can prevent skin AVM formation and ameliorate internal bleeding in Alk1-deficient adult mice. With topical applications at different stages of AVM development, we demonstrate that the VEGF blockade can prevent the formation of AVM and cease the progression of AVM development. Taken together, the presented experimental model is an invaluable system for precise molecular mechanism of action of VEGF blockades as well as for preclinical screening of drug candidates for epistaxis and gastrointestinal bleedings.

Concepts: Blood, Blood vessel, Model organism, Telangiectasia, Congenital disorders, Arteriovenous malformation, Cerebral arteriovenous malformation, Hereditary hemorrhagic telangiectasia

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The standard technique for the transcatheter treatment of pulmonary arteriovenous malformations (PAVMs) involves deploying coils into the feeding arteries. We investigated whether venous sac embolization would also be a safe and useful treatment method.

Concepts: Heart, Vein, Congenital disorder, Congenital disorders, Arteriovenous malformation

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Onyx(®) (ev3, Irvine, CA, USA) is a liquid embolic agent composed of ethylene vinyl alcohol copolymer dissolved in dimethyl sulphoxide used for the treatment of intracranial arteriovenous malformations. Onyx is a preferred embolizing agent due to its unique properties, non-adhesive nature, and durability. In addition to its approved intracranial application, Onyx is also being used successfully in extracranial embolization in areas including extracranial aneurisms and vascular malformations, trauma, gastrointestinal bleeding, and neoplasms. Because of its increasing utilization, it is important for reporting radiologists to be able to recognize its extracranial appearance across different imaging techniques and to be familiar with its uses. The goal of this review is to describe the extracranial uses of Onyx and its appearance in various extracranial locations at radiography and CT, while providing didactic examples. Onyx appears radiodense at CT and plain radiography and has a curvilinear pattern following the expected path of the vessel embolized. At CT, Onyx creates streak artefact that may obstruct the view of surrounding tissues consistent with descriptions of other tantalum devices.

Concepts: Stroke, Medical imaging, Radiology, Projectional radiography, Congenital disorders, Arteriovenous malformation, Radiologic technologist

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Study Design Case report. Objective Present a case of Foix-Alajouanine syndrome that presented as acute cauda equina syndrome and discuss the pathophysiology and management. Methods An adult male patient developed sudden onset of back pain and leg pain with weakness of the lower limbs and bladder/bowel dysfunction typical of cauda equina syndrome. Emergency magnetic resonance imaging revealed no compressive lesion in the spine but showed tortuous flow voids and end-on blood vessels in the peridural region suggesting spinal arteriovenous malformation resulting in Foix-Alajouanine syndrome. Results The case was managed by endovascular embolization with excellent results. The pathophysiology, imaging features, management, and literature review of the syndrome is discussed. Conclusion The authors conclude that this condition may be an important differential diagnosis for cauda equina syndrome.

Concepts: Medical terms, Nuclear magnetic resonance, Magnetic resonance imaging, Radiology, Differential diagnosis, Cauda equina, Arteriovenous malformation, Cauda equina syndrome