Cerebral venous thrombosis in neonates, children and adolescents; results from the German pediatric surveillance unit for rare pediatric diseases (ESPED)

S. Holzhauer 1, K. Kentouche 2, A. Kruempel 3, C. Heller 4, R. Knoefler5, R. Straeter6, U. Nowak-Göttl 7 (1Berlin , Germany , 2Jena , Germany , 3Muenster, Germany , 4Frankfurt, Germany , 5Dresden , Germany , 6Muenster , Germany , 7Kiel , Germany )

Pediatric Thrombosis
Date: 17.02.2017,
Time: 08:00 - 09:15

Objective: To evaluate the influence of transient risk factors and hereditary thrombophilia on clinical course, treatment practices and early outcome in a population based national cohort of children with CSVT. To study the influence of age (neonatal versus pediatric) on risk factors and outcome in CSVT.

Methods: We conducted a prospective nationwide surveillance study in pediatric patients through the hospital-based German Pediatric Surveillance Unit (ESPED). We included consecutive patients from 0-18 years of age admitted to hospitals in Germany with diagnosis of a first CSVT with an enrolment period between 2001-2010. Diagnosis was confirmed using MRI or CT imaging. Laboratory analysis of coagulation parameters have either been analyzed according to standardized thrombophilia screening protocols in the local hospitals or centrally in study center at the University of Muenster. We followed the course of disease over a period of 36 months. Follow up investigation included MRI or CT imaging and questionnaires on clinical outcome and recurrence.

Results: A total of 599 patients, from birth to 18 years with a diagnosis of CSVT were enrolled in the study. We have observed a male predominance with 61%. 138 (23%) CSVT cases were diagnosed during the neonatal period, 461 (77%) patients were older than one month at time of diagnosis. In our cohort 40% of neonates and 20% of older infants/children developed thrombosis without identified underlying predisposing diseases. The majority of transient triggers associated with thrombosis were local (mastoiditis, 18%) or systemic (sepsis, meningitis, 13%) infections or asparaginase administration during treatment for leukemia or lymphoma (12%). Outcome, dependent both on age at onset and existence of transient triggers was worse in children with spontaneous CSVT compared to triggered CSVT with regard to mortality rate (11 vs. 3%), patency of the veins and neurological impairment. Moreover, presenting symptoms as well as the clinical course differed between neonates and children.

Conclusion: In the pediatric population most of CSVT events were associated with infections as transient trigger. CSVT in neonates and children older than 1 months of age differed both with regard to underlying risk factors, treatment and outcome. Age specific, randomized controlled trials comparing treatment strategies are needed to optimize care in these patients.