PEDIATRIC CEREBRAL SINUS VENOUS THROMBOSIS (CSVT) IN A BRAZILIAN SINGLE-CENTER: A 3-YEAR RETROSPECTIVE ANALYSIS OF A MULTIDISCIPLINARY EXPERIENCE

Abstract

Objectives: CSVT is rare in pediatric patients but is related to considerable morbidity and mortality. Its etiology is multifactorial: infections, head trauma, cancer and some chronic inflamatory diseases are some of the risk factors. Clinical manifestations are variable and non specific: headache, seizure, focal neurologic deficit and impaired consciouness. Pathogenesis involves venous obstructive ouflow which results in impaired drainage of cerebrospinal fluid and increased intracranial pressure. Diagnosis involves a high suspicious index and an adequade neuroimaging. Management consists of suportive care, anticoagulation and treatment of the neurologic complications. This sudy has the purpose of describing a cohort of pediatric CSVT. Materials and methods: Patients under 18 years diagnosed with CSVT between august 2021 to august 2024 were retrospectively included. Demographic informations, signs and symptoms, laboratory parameters, management information, venous recanalization rate, chronic complications and follow-up were evaluated. Results: 22 patients were included, 72.7% male and 27.2% female. Median age at diagnosis was 9.8 years (0.16–17). Headache was the most common clinical manifestation (72.2%). In 90.9% of patients at least one risk factor for CSVT was present and infections were the most common (40.9%). 63.6% had more than one sinus affected and 22.7% had cerebral venous infarct. Median values of aPTT, PT, fibrinogen and platelet count were normal and median D-dimer was 3.598ng/mL (275-21373). Complete treatment and follow-up data were available in 17 patiens. Treatment included anticoagulation with enoxaparin in all patients and 2 completed antithrombotic therapy with rivaroxaban. Acetazolamide and lumbar puncture were necessary in 35.2% for high intracranial pressure control. One patient (5%) needed surgical thrombectomy. Median time of anticoagulation was 5.4 months (2.6-13.5) with 62.5% of patients with complete venous recanalization. There was no death related to CSVT but 11.7% had chonic motor sequelae. Median time of follow-up was 1.4 years (0.3-2.3) with 5.8% of recurrence thrombosis. Discussion: This is the first brazilian cohort of pediatric CVST. Infections were the most common risk factor. There were no clear correlation between number of sinuses affected and acute and chronic neurologic complications. Despite a small number of patients, some patients were treated with direct oral anticoagulants. The cooperation of hematology, neuroradiology, neurology and neurosurgery was essential. This cohort has limitations and some similar findings of other studies, but it highlights the role of pediatric hematologists in the approach of thromboembolic events, and also emphasizes the need of multidisciplinarity from diagnosis to acute and chronic management of pediatric thrombosis. Conclusion: CVST as other pediatric thrombosis needs a multiciplinary team for adequate acute and long term treatment. Multicentric studies are needed to establish more concrete data about CVST in pediatric population in Brazil.