[Diagnosis and therapy of cerebral sinus and venous thrombosis].
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Expression of angiogenic growth factors in dural arteriovenous fistula.
OBJECT: Although various mechanisms of the development of dural arteriovenous fistula (AVF) have been described, the exact course of its pathogenesis, including molecular processes mediating its genesis, is still unknown. Recently, the importance of sinus thrombosis and venous hypertension has been reported in experimental and clinical studies. Additionally, a role of angiogenic growth factors in the pathogenesis of vascular malformations of the central nervous system has been reported. In this study, the authors investigated the existence of sinus thrombosis in dural AVF and the expression of angiogenic growth factors (basic fibroblast growth factor [bFGF] and vascular endothelial growth factor [VEGF]) in nine patients with dural AVFs that were surgically resected. METHODS: The authors examined histological features of dural AVFs that involved the transverse/sigmoid sinus in seven patients and the superior sagittal sinus in two. Sinus thrombosis was verified angiographically in seven cases and histologically in all cases. In surgically resected specimens the angiogenic growth factors bFGF and VEGF were examined immunohistochemically in nine patients with dural AVFs, with five dural sinuses from cadavers with unrelated central nervous system diseases serving as a normal control group. The media and perivascular connective tissues of the arteries in the wall of the normal dural sinuses stained faintly for bFGF; on the other hand, the expression of VEGF was not detected. In all patients with dural AVFs, the thick wall of the dural sinus stained strongly for bFGF, mainly in the subendothelial layer and media of the strongly proliferative vessels in the sinus wall, in addition to the perivascular connective tissues. In all nine cases VEGF was expressed in the endothelium of the sinus and perivascular connective tissues. In two cases, VEGF was expressed in many capillaries proliferating in the granulation-like tissues in sinuses that were obliterated by organized thrombi. CONCLUSIONS: It is concluded that the pathogenesis of dural AVF is still unknown, but that angiogenic growth factors, which might be produced by the healing process due to sinus thrombosis, may participate in the genesis of dural AVF. Understanding the mechanism of molecular pathogenesis in the development of dural AVF might aid in the establishment of a new therapeutic strategy for this dynamic vascular disease.
Adult↗
Importance of anatomical asymmetries of transverse sinuses: an MR venographic study.
Time-of-flight (TOF) magnetic resonance venography (MRV) is often used to examine the intracranial dural sinuses, particularly in the evaluation of dural sinus thrombosis. The goal of the study was to evaluate the use of TOF MRV in assessing the normal anatomy of dural sinuses and their variations as sources of potential pitfalls in the diagnosis of venous sinus thrombosis. Cerebral TOF MRV obtained in 105 persons with normal MR studies were reviewed to determine the presence, aplasia and hypoplasia of the transverse sinuses. Twenty-one (20%) aplasias of the left sinus, 41 (39%) hypoplasia of the left sinus, 33 (31%) symmetric, 6 (6%) hypoplasia of the right sinus, and 4 (4%) aplasias of the right sinus cases were determined in the asymmetry in sizes of transverse sinuses. These results suggested that transverse sinus flow gaps or aplasias can be observed in approximately 24% of normal population on MR imaging. The rate of these gaps in normal subjects must be kept in mind because it can be a source of misdiagnosis in cases of suspected dural sinus thrombosis.
Adult↗
[Cerebral venous thrombosis disclosing Behçet disease].
BACKGROUND: Behçet's disease is very occasionally revealed by thrombophlebitis in children. Dural sinus thrombosis can be one of its complication. CASE REPORT: A 14 year-old boy of Mediterranean origin was admitted for acute meningitis with temperature of 40 degrees C. His CSF contained 24 cells/ml and 0.33 g/l protein; it was sterile. The RBC sedimentation rate was 84 mm. Other investigations, including brain scan, were negative. The condition became worse, with a deterioration of visual acuity, attack of aphthous stomatitis and skin lesions at points of puncture. There was papilledema with retinal vascularitis. A second brain scan and MRI showed sagittal sinus thrombosis. The condition improved immediately after treatment with prednisolone and ticlopidine. The brain MRI taken 3 months later showed partial permeability of the sagittal sinus. The patient had several attacks of meningoencephalitis and stomatitis during the following 2 years. The last attack was complicated by iridocyclitis; this required cyclosporin treatment. CONCLUSION: Phlebothrombosis is a classic complication of Behçet's disease and can reveal the disease. Sagittal sinus thrombosis has never before been reported as the first manifestation of the disease in children.
Adolescent↗
Evaluation of the intracranial dural sinuses with a 3D contrast-enhanced MP-RAGE sequence: prospective comparison with 2D-TOF MR venography and digital subtraction angiography.
BACKGROUND AND PURPOSE: The diagnosis of dural sinus thrombosis is often difficult because of its variable and nonspecific clinical presentation and the overlapping signal intensities of thrombosis and venous flow on conventional MR images and MR venograms. We compared 3D contrast-enhanced magnetization-prepared rapid gradient-echo (MP-RAGE) sequences with 2D time-of-flight (TOF) MR venography, digital subtraction angiography (DSA), and conventional spin-echo (SE) MR imaging for the assessment of normal and abnormal dural sinuses. METHODS: In a phantom study, a plastic tube with pulsating flow was used to simulate the intracranial dural sinus. With 3D MP-RAGE, a variety of flow velocities, contrast material concentrations, and angulations between the phantom flow tube and the plane of acquisition were tested to measure their relationship to signal-to-noise ratio (SNR). In a clinical study, 35 patients, including 18 with suspected dural sinus thrombosis, were studied with both MR imaging and DSA. Receiver operating characteristic (ROC) analysis was performed in a blinded fashion using DSA as the reference standard. RESULTS: With the phantom, the SNR of flow increased with increasing contrast concentration, but was not affected by the angle between the tube and scan slab. There was no relationship between SNR and velocity when the contrast concentration was 1.0 mmol/L or greater. In the clinical study, dural sinus thrombosis as well as the normal anatomy of the dural sinuses were seen better with 3D contrast-enhanced MP-RAGE than with 2D-TOF MR venography. Three-dimensional contrast-enhanced MP-RAGE showed the highest diagnostic confidence on ROC curves in the diagnosis of thrombosis. CONCLUSION: Three-dimensional contrast-enhanced MP-RAGE is superior to 2D-TOF MR venography and conventional SE MR imaging in the depiction of normal venous structures and the diagnosis of dural sinus thrombosis, and is a potential alternative to DSA.
Adult↗
[Sinus vein thrombosis. A rare complication of heparin-induced thrombocytopenia type II].
In the past 10 years numerous reports of cases referring to complications and their outcome with heparin-induced thrombocytopenia type II (HIT II) have been published. Clinically these symptoms are manifested as a combination of arterial and venous thromboembolisms. Mostly affected are the vessels of the limbs, the abdomen, kidneys and coronary arteries. We present the most rare initial manifestations of cerebral symptoms with headache, nausea, change of character and generalised convulsion, which have found their origin in sinus vein thrombosis and the treatment with the heparinoid danaparoid.
Aged↗
Alterations in ventricular size and intracranial pressure caused by sagittal sinus pathology in man.
Lumbar cerebrospinal fluid (CSF) pressure and ventricular size were determined in six patients with impairment of cerebral venous outflow caused by either sagittal sinus thrombosis or arteriovenous shunting into the sagittal sinus. None of the patients had enlargement of the ventricular system, but all six had elevated CSF pressure (mean, 30 mm Hg). At least two mechanisms sufficient to prevent ventricular enlargement and significant hydrocephalus are suggested by these cases: 1) intracranial pressure elevations that occur as a result of cerebral venous outflow impediment maintain a positive pressure gradient between the intracranial CSF and the sagittal sinus contents, thereby increasing bulk CSF outflow; 2) in adults, increased function of alternative CSF outflow pathways occurs secondary to sagittal sinus thrombosis across the arachnoid villi of other intracranial vascular structures and in the spine. These mechanisms may have general importance in the generation of hydrocephalus caused by other disease states in adults but not in children.
Adult↗
Heparin treatment in acute cerebral sinus venous thrombosis: a retrospective clinical and MR analysis of 42 cases.
The only randomized data on heparin treatment in acute cerebral sinus venous thrombosis (CSVT) are derived from a small number of patients. The rate of intracranial hemorrhages as a complication of high-dose heparin treatment is still unknown. This retrospective study evaluates the clinical features, neuroimaging monitoring and outcome of 42 patients with proven CSVT. Diagnosis was established by DSA, CT, MR tomography and MR angiography. All patients received heparin intravenously guided by doubling the aPTT value for 3 weeks, followed by oral anticoagulation. Partial or complete recanalization was found in 36 cases. 40 patients improved clinically, in 26 of them complete recovery was observed. One patient deteriorated and developed an apallic syndrome, one further patient died of septic multiorgan failure. Only in one patient was hemorrhagic transformation of infarcted brain tissue observed but without clinical deterioration.
Acute Disease↗
Pathophysiology of cerebral venous thrombosis--an overview.
Cerebral venous sinus thrombosis is a disorder with a unique pathophysiology which needs to be described. A Medline search of all articles detailing pathophysiology of CVST was done, using keywords: cerebral venous thrombosis and pathophysiology. In addition, major texts were reviewed for additional references. The pathophysiology of CVST depends on two interconnected events, local signs due to venous infarct, e.g., hemiparesis and global signs due to raised ICP from an obstructed venous system--papilloedema and isolated intracranial hypertension being one of them. Pathophysiology of CVST is diverse and makes it easier to understand the diversity of clinical presentations.
Cerebral Veins↗
Subdural empyema and other suppurative complications of paranasal sinusitis.
Suppurative intracranial infection, including meningitis, intracranial abscess, subdural empyema, epidural abscess, cavernous sinus thrombosis, and thrombosis of other dural sinuses, are uncommon sequelae of paranasal sinusitis. A high index of suspicion is necessary to identify these serious complications. We present a patient with subdural empyema in whom the diagnosis was delayed, followed by a discussion of suppurative complications of sinusitis. The case shows the rapid progression of subdural empyema, which represents a true neurosurgical emergency requiring prompt diagnosis and management.
Empyema, Subdural↗
Cerebral CT venography.
PURPOSE: To produce cerebral venograms with thin-section helical computed tomography (CT) and to assess their diagnostic utility. MATERIALS AND METHODS: Thirty-six CT venograms were obtained in 33 patients after intravenous administration of iodinated contrast material. Eighteen patients had suspected dural sinus thrombosis. Twelve patients had tumor adjacent to a major venous structure. Three patients underwent CT venography during CT angiography. RESULTS: Superior sagittal, transverse, and straight sinuses were identified on every CT venogram. Other veins were seen with high frequency. Dural sinus thrombosis was diagnosed in seven patients, with magnetic resonance (MR) venographic correlation in five patients. CT venograms were easier to interpret and had fewer artifacts than MR venograms. Relationships of tumor to adjacent cerebral venous structures were well shown on CT venograms. CONCLUSION: CT venography yields detailed images of the intracranial venous circulation with consistently high quality. It is a rapid, useful method for diagnosis of dural sinus thrombosis and for preoperative mapping of venous structures in patients with neoplasm.
Adult↗
Thrombosis of the deep venous drainage of the brain in adults. Analysis of seven cases with review of the literature.
OBJECTIVES: To characterize the clinical, radiographic, and pathologic findings in thrombosis of the deep venous drainage of the brain. To highlight clinical and radiographic findings that may lead to the diagnosis of disease and distinguish it from dural sinus thrombosis. To review the published literature on this disorder. DESIGN: Retrospective review of the medical and radiographic records of seven patients from three institutions over the past 10 years. Review of the English language literature from 1971 to the present. RESULTS: All seven patients had risk factors for cerebral venous thrombosis. Five patients presented with a short, rapidly progressing course characterized by headache, nausea and vomiting, and decline in level of consciousness. All five patients died or were rendered severely disabled. Computed tomographic scans, magnetic resonance imaging, and magnetic resonance angiography showed findings associated with deep cerebral vein thrombosis in three of four, in five of five, and in three of three patients, respectively. Transfemoral catheter angiography was diagnostic in two of two patients. Twenty-one reported cases of deep cerebral venous thrombosis were identified in the literature. CONCLUSIONS: When the two populations are combined and compared with large series of patients with dural sinus thrombosis, patients with deep venous system thrombosis are more commonly women, tend to present with a more rapidly declining time course, altered consciousness, and long tract signs. Death or long-term sequelae are far more likely to occur in internal cerebral vein thrombosis than with dural sinus thrombosis. Unenhanced computed tomography can demonstrate findings that are strongly suggestive of the diagnosis. Magnetic resonance imaging and magnetic resonance angiography are confirmatory. Angiography may still be necessary when the diagnosis is not clear.
Adolescent↗
Otogenic intracranial complications: a review of 28 cases.
CONCLUSIONS: Antibiotic treatment does not absolutely prevent the development of otogenic intracranial complications (ICC); however, their incidence is relatively low (0.36%). Various pathogens can be isolated in cultures of patients with these complications, but combinations of third- or fourth-generation cephalosporins with chloramphenicol, vancomycin, metronidazole or aminoglycosides can provide good results. Underlying cholesteatoma is common and is usually associated with intracranial abscess or sinus thrombosis. High morbidity rates warrant long-term follow-up. OBJECTIVE: To evaluate the cause and nature of otogenic ICC in patients treated at 1 medical center over an 18-year period. MATERIAL AND METHODS: This was a retrospective chart review of 28 patients admitted to Sheba Medical Center, Israel with otogenic ICC between 1984 and 2002. RESULTS: Meningitis was the commonest complication (46.4%), followed by brain abscess, epidural abscess, sigmoid sinus thrombosis, subdural empyema, perisinus abscess and transverse and cavernous sinus thrombosis. Twelve patients (42.9%) had received antibiotic treatment prior to admission. Chronic otitis media, cholesteatoma and brain abscess were diagnosed mainly in adults, while acute otitis media and epidural abscess were more frequent in children. Twenty-one patients underwent mastoidectomy to eradicate the source of infection. The commonest finding at surgery was granulations (81%). Cholesteatoma was seen in 38.1% of cases. Cholesteatoma and brain abscess were usually associated with Gram-negative bacterial infection. Meningitis, however, was caused by Streptococcus pneumoniae in 40% of cases. CT showed a sensitivity of 92.75% for diagnosing otogenic ICC. There was no mortality. The morbidity rate was high (71.4%) and included hearing impairment, hemiparesis, hydrocephalus, mental retardation, polyneuropathy and epilepsy.
Adolescent↗
[Prognosis of vena angularis and sinus cavernosus thrombosis in the past and present].
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Adolescent↗
[Ulcerative colitis complicated by sinus cavernous thrombosis and necrotizing vasculitis].
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Adult↗
Genetically-induced deep venous thrombosis presenting as acute mastoiditis.
Sigmoid sinus thrombosis (SST) usually follows acute as well as chronic otitis media with coalescent mastoiditis. A singular case of noncoalescent mastoiditis complicated with thrombosis of deep cerebral sinuses occurring in a young child is presented. A genetic thrombophilic disorder (prothrombin G20210A allele mutation) was identified as the predisposing factor for this unusual complication. Particular emphasis is placed on the course of the disease, which showed regression only after surgical exploration and additional anticoagulant therapy. We conclude that a thorough and early assessment of coagulation factors should always be performed, especially in the population at risk, in order to rule out unusual aetiologies of these rare but still life-threatening pathological processes.
Acute Disease↗
Predictors of outcome in patients with cerebral venous thrombosis and intracerebral hemorrhage.
BACKGROUND AND PURPOSE: Although intracerebral hemorrhages are frequent in patients with cerebral venous thrombosis, and lead to worse outcome, predictors of outcome in cerebral venous thrombosis patients with intracerebral hemorrhages have never been evaluated in adequately powered studies. METHODS: This study was conducted as a part of the International Study on Cerebral Vein and Dural Sinus Thrombosis. We evaluated predictors of outcome in cerebral venous thrombosis patients who had an "early intracerebral hemorrhage," ie, intracerebral hemorrhages already present at time of diagnosis of cerebral venous thrombosis by a logistic regression analysis, with a modified Rankin scale 3 to 6 at month 6 as dependent variable. The same analysis was performed with "delayed intracerebral hemorrhages," ie, intracerebral hemorrhages that occurred after the diagnosis of cerebral venous thrombosis, as dependent variable. RESULTS: Of 624 patients recruited in International Study on Cerebral Vein and Dural Sinus Thrombosis, 245 (39%) had an early intracerebral hemorrhage: at month 6, 51 (21%) of them had a modified Rankin Scale 3 to 6. Independent predictors of having modified Rankin scale 3 to 6 at month 6 were older age (adjusted odds ratio for 1-year increase in age, 1.05; 95% CI, 1.02 to 1.08); male gender (adjusted odds ratio, 3.25; 95% CI, 1.29 to 8.16); having a deep cerebral venous system thrombosis (adjusted odds ratio, 5.43; 95% CI, 1.67 to 17.61) or a right lateral sinus thrombosis (adjusted odds ratio, 2.56; 95% CI, 1.03 to 6.40); and having a motor deficit (adjusted odds ratio, 2.94; 95% CI, 1.21 to 7.10). Of the 36 patients who had a delayed intracerebral hemorrhage, those who had a modified Rankin scale 3 to 6 at month 6 were less likely to have received heparin at the acute stage, and more likely to have had early intracerebral hemorrhage. CONCLUSIONS: Among patients with early intracerebral hemorrhage, those who were older, men, had a thrombosis of the deep cerebral venous system or of the right lateral sinus, and a motor deficit were at higher risk for death or dependency at month 6. This subgroup of patients with predictors of poor outcome can be the target for new therapeutic strategies.
Adolescent↗
Increased incidence of thrombophilic abnormalities in patients with cranial dural arteriovenous fistulae.
Cranial dural arteriovenous fistulae (DAVF) may occur post-traumatic or sporadic. The physiopathologic mechanisms of sporadic DAVF are still unclear. A dural sinus thrombosis followed by an increase in venous pressure and/or an increased procoagulatory activity of the coagulation system are associated at least with some DAVF. The objective of this study was to investigate the coagulation profile in patients with DAVF. Thus the association of thrombophilic abnormalities, sinus thrombosis and DAVF should be analyzed. A total of 15 patients with cranial DAVF were included in this study. Blood samples were analyzed for 20210A mutation of the prothrombin gene, resistance to activated protein C and factor V Leiden mutation. Fibrinogen (Fib), Textarin time (TT), antithrombin (AT), protein C and protein S activity, von Willebrand factor antigen (vWF:Ag), Ristocetin cofactor activity (vWF:RCo), D-Dimer (DD) and coagulation factor VIII-activity (F VIII) were determined in all patients. Blood was screened for the occurrence of lupus antiphospholipid antibodies and cardiolipin antibodies. Thrombophilic risk factors were found in 5 (33%) of the 15 patients with cranial DAVF. Four patients had a heterozygote 20210A mutation of the prothrombin gene and one patient had a heterozygote FV Leiden mutation. Sinus thrombosis was detected in two patients with grade 2b DAVF and was associated with a 20210A mutation of the prothrombin gene in both patients. Additionally, one patient had deficient protein C activity and screening for cardiolipin antibodies was positive in three patients. In the current series the frequency of prothrombin Gene 20210A mutation was higher in patients with DAVF compared to the general population, whereas the incidence of Factor V Leiden mutation was not. Therefore in patients with cranial DAVF thrombophilic abnormalities should be considered in the post-operative/post-interventional management.
Adult↗