A Study on the Distinctive Clinical Profile and Thrombophilia in Pediatric Cerebral Venous Sinus Thrombosis

The objective of the study was to systematically bring out the clinical presentations, neuro-imaging features, treatment given, and long-term outcomes of children with cerebral venous sinus thrombosis (CVST).

Data were obtained by retrospective analysis of electronic records spanning 13 years, of children (<18 years) with a confirmed diagnosis of CVST based on magnetic resonance imaging of the brain and managed at a tertiary care children's hospital in the UK.

Seventeen patients with pediatric CVST were identified over a 13-year study period, highlighting the uncommon prevalence of this entity. This study comprised 10 males and seven females. The age range at presentation was between 2 days and 17 years with a median age of 5.5 years. Headache was the commonest presenting symptom in 10 of 17 children and focal neurological signs were seen in 11 of 17 patients. Among risk factors, six patients had an antecedent infection of the ear/mastoid, three children had acute leukemia, and two patients had central venous catheters. Para-infectious CVST (seven of 17 patients) responded well to appropriate antibiotic therapy. Thrombophilia screens were available in 10 of 17 patients with noninfectious CVST and returned abnormal in four patients (two with Factor V Leiden mutations and one each with deficiency of protein C and anti-thrombin III). Anticoagulants were used in only six of 17 cases and were generally well tolerated. Follow-up data revealed, 11 of 17 patients had a complete recovery and four of 17 patients had residual neurological deficits. Two children died in the entire cohort.

Pediatric CVST is uncommon and has a different spectrum from adults, with unique clinical triggers and thrombophilic states. Management varies significantly among clinicians, due to the paucity of trial evidence and also due to the heterogeneity of this condition in children.