Abstract Number ‐ 48: Cerebral Sinus Venous Thrombosis Secondary to Chronic Inflammatory Disorders in Children

Abstract

Introduction Cerebral Sinus Venous Thrombosis (CSVT) is a rare form of venous thromboembolism that can lead to significant morbidity and mortality predominantly in young people. While previous literature has identified prothrombotic and infectious pathologies as significant risk factors for CSVT, chronic systemic conditions such as connective tissue diseases (CTD) are less studied. The purpose of the current study is to describe the clinical features, neuroimaging characteristics, treatment, and long‐term neurological outcomes in children with CSVT secondary to CTD. Methods We reviewed electronic medical records of patients under 18 years of age diagnosed with CSVT secondary to CTD between 2002–2018 in a single center. We analyzed data regarding demographics, comorbidities, neuroimaging and neurological examination findings during the initial encounter. Subsequent long‐term follow‐up neurological examinations, lab and imaging workup, and medical management was also analyzed. Results Eight patients (Male:Female: 6:2) met the criteria and were included in the analysis. The patient’s age ranged from 6 weeks to 17 years. CTDs include Behcet’s (n = 2), Kawasaki (n = 1), systemic lupus erythematosus (n = 1), Wegener granulomatosis (n = 1), sarcoidosis (n = 1), and inflammatory bowel disease (n = 1). Five patients had a normal neurological examination on initial presentation, two had papilledema, and one had hypotonia and poor suck reflex. On initial neuroimaging, the most common thrombosis locations were transverse sinuses (n = 7). All patients received anti‐thrombolytic therapy (heparin, warfarin, or Lovenox) between 2.5 to 6 months. Three patients had complete resolution and one had partial resolution of thrombosis on follow‐up neuroimaging (average = 4.3 months, range 3–8 months). On long‐term clinical follow‐up (average: 41.3 months, SD: 62.1), all patients had normal neurological examinations. Conclusions Our case series demonstrated that 50% of patients with CSVT secondary to CTD showed full/partial resolution of thrombosis on follow‐up neuroimaging. In addition, they also had good clinical outcomes on long‐term clinical follow‐up with anticoagulation or antithrombotic treatment. This study uncovers the favorable outcomes of CSVT associated with CTD in children, but more cohort studies are required in order to make accurate conclusions.