|ECR 2019 / C-1382|
|Cerebral sinovenous thrombosis in paediatric age|
Aims and objectives
Cerebral sinovenous thrombosis (CSVT) is a rare disorder increasingly diagnosed, especially in paediatric population where it has an incidence of 1:100.000 and represents almost approximately 20% of cerebral vascular diseases.
Most symptoms are nonspecific, so neuroimaging techniques, especially magnetic resonance imaging (MRI), are essential for its management.
Across all pediatric age groups, seizures remain the most common presentation of CSVT. Apart from seizures, irritability and hypotonia are especially observed in neonates and headache and motor symptoms predominate in the non-neonatal patients. The location of the thrombus and whether it is partial or complete, acute or chronic influences the clinical presentation.
Predisposing factors in pediatric population differ from those observed in adulthood. Idiopathic cases are very rare in children (3% vs 25% in adults). In neonates, risk factors include maternal factors such as maternal factors (placenta, premature rupture of membranes, maternal infections or gestational diabetes,hypoxic-ischemic encephalopathy, dehydration and sepsis.
In children and adolescents, the main predisposing factors are infectious and non-infectious processes. First ones include acute otomastoiditis, peritonsillar abscess, bacterial meningitis, and orbital cellulitis. The non-infectious processes include cyanotic heart disease, primary or metastatic tumours and hematological disorders, especially leukemia. Thrombosis in patients with leukemia is secondary to cerebral and meningeal infiltration, leukostasis and the use of chemotherapeutic agents, mainly L-asparaginase and vincristine.
CSVT is also associated with congenital and acquired coagulation disorders. The first ones includes mutations of anticardiolipin antibody, protein C deficiency, antithrombin III, factor V Leiden and Prothrombin G20210A. The seconds included patients with liver disease, the nephrotic syndrome or disseminated intravascular coagulation.
The use of anticoagulant therapy in CSVT is based in clinical trials performed in adults. However, several studies proved that it is not associated with serious hemorrhage in selected patients.
Seizures and coma at presentation along with hemorrhagic infarctions and hydrocephalus is associated with worse outcome.
To revise the characteristics of patients with CSVT in a tertiary-level healthcare hospital, including clinical and radiological features, etiologies, treatment approaches and morbi-mortality rates.
To provide a comprehensive review of normal intracranial venous anatomy.
To highlight the imaging clues for their early identification and differential diagnosis.