Background GENetics of mOyaMoyA (GEN-O-MA) project is a multicenter observational study implemented in Italy aimed at creating a network of centers involved in moyamoya angiopathy (MA) care and research and at collecting a large series and biorepository of MA patients, finally aimed at describing the disease phenotype and clinical course as well as at identifying biological or cellular markers for disease progression. The present paper resumes the most important study methodological issues and preliminary results. Methods Nineteen centers are participating to the study. Patients with both bilateral and unilateral radiologically defined MA are included in the study. For each patient, detailed demographic and clinical as well as neuroimaging data are being collected. When available, biological samples (blood, DNA, CSF, middle cerebral artery samples) are being also collected for biological and cellular studies. Results Ninety-eight patients (age of onset mean ± SD 35.5 ± 19.6 years; 68.4% females) have been collected so far. 65.3% of patients presented ischemic (50%) and haemorrhagic (15.3%) stroke. A higher female predominance concomitantly with a similar age of onset and clinical features to what was reported in previous studies on Western patients has been confirmed. Conclusion An accurate and detailed clinical and neuroimaging classification represents the best strategy to provide the characterization of the disease phenotype and clinical course. The collection of a large number of biological samples will permit the identification of biological markers and genetic factors associated with the disease susceptibility in Italy.
Introduction: Dural arteriovenous fistulas (dAVFs) account for 10–15% of all intracranial arteriovenous lesions. Different classification strategies have been proposed in the course of the years. None of them seems to guide the treatment strategy. Objective: We expose the experience of the vascular group at Niguarda Hospital and we propose a very practical classification method based on the location of the shunt. We divide dAVF in sinus and non-sinus in order to simplify our daily practice, as this classification method is simply based on the involvement of the sinuses. Material and Methods: 477 intracranial dural arteriovenous fistulas have been treated. 376 underwent endovascular treatment and 101 underwent surgical treatment. Cavernous sinus DAVFs and Galen ampulla malformations have been excluded from this series as they represent a different pathology per se. 376 dAVFs treated by endovascular approach: 180 were sinus and 179 were non-sinus. 101 dAVFs treated with surgical approach: 15 were sinus and 86 were non-sinus. Discussion: Of the 477 intracranial dAVF the recorded mortality and severe disability was 3% and morbidity less than 4%. All patients underwent a postoperative DSA with nearly 100% of complete occlusion of the fistula. At a mean follow-up of 5 years in one case there was a non-sinus fistula recurrence, due to the presence of a partial clipping of “piè” of the vein. Conclusions: The sinus and non-sinus concept has guided our institution for years and has led to good clinical results. This paper intends to share this practical classification with the neurosurgical community.
In AVM surgery perioperative complications can arise and can have serious perioperative consequences. Surgically related complications in AVM treatment, in many cases, can be avoided by paying attention to details:1. Careful selection of the patient: - addressing a patient with eloquent AVM to Gamma Knife treatment - preoperative treatment with selective embolization of the accessible deep feeders - preoperative gamma knife or embolize those patient with an over-expressed venous pattern2. Meticulous coagulation of deep medullary feeders: - Using dirty coagulation - Using dry non-stick coagulation - Using micro clips - Using laser - Reaching the choroidal vessel in the ventricle when possible - Avoiding occlusive coagulation with hemostatic agents3. Check and avoiding any residual of the AVM4. Keep the patient under pressure control during postoperative periodFulfilling these steps contributes to reduce complications in this difficult surgery, leading to a safer treatment that compares favorably with natural history of brain arteriovenous malformations.
Background:In order to better define the pathogenic role of cerebrospinal fluid (CSF) drainage catheters in postoperative patients, we comparatively analyze the clinical course of device and non-device-related meningitis.Methods:This is an observational, partially prospective, study on consecutive adult patients who developed meningitis after undergoing neurosurgical procedures at the Neurosurgery and Neurointensive care Departments, Spedali Civili, Brescia, Italy, between January 1999 and August 2007.Results:All 77 consecutive post-neurosurgical meningitis events in 65 patients were included in the analysis. Most were classified as external ventricular drainage (EVD)-related meningitis (23 cases, group A), external spinal drainage (ESD)-related meningitis (12 cases, group B), and non-device-related post-neurosurgical meningitis (30 cases, group C). Proven meningitis was identified in 78.3%, 91.7% and 56.7% of the events, respectively. ESD-related meningitis had a shorter onset time vs EVD and non-device-associated meningitis (3 days versus 6 and 7 days, respectively). Median antibiotic treatment duration was 20, 17, and 22.5 days in groups A, B, and C, respectively. Overall, 8 patients (34.8%) in group A, 3 (25.0%) in group B, and 3 (10.0%) in group C died. Median time to become afebrile was shorter in group C than in group A (10 days versus 12 days, P = 0.04). Removal of the device later than 48 hours after meningitis onset, as well as implantation of a second device were associated with a slower time of meningitis resolution.Conclusions:Early device removal and avoiding implantation of a second device were associated with short illness duration. Larger studies are warranted to confirm the conclusions of this study.
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