Published Online
on August 25, 2003

A more recent version of this article appeared on September 16, 2003

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Submitted on February 11, 2003
Revised on June 23, 2003
Accepted on June 23, 2003

Cerebral Venous Thrombosis in Children. A Multifactorial Origin

Christine Heller MD, Achim Heinecke MD, Ralf Junker MD, Ralf Knöfler MD, Andrea Kosch MD, Karin Kurnik MD, Rosemarie Schobess MD, Arnold von Eckardstein MD, Ronald Sträter MD, Barbara Zieger MD, Ulrike Nowak-Göttl MD^*, and and the Childhood Stroke Study Group

From the Department of Pediatrics/Pediatric Hematology & Oncology, University of Frankfurt am Main (C.H.), Dresden (R.K.), Münster (A.K., R.S., U.N.-G.), Munich (K.K.), Halle (R.S.), and Freiburg (B.Z.), Germany; Institute of Medical Informatics and Biomathematics (A.H.) and Institute of Clinical Chemistry and Institute of Arteriosclerosis Research (R.J.), University of Münster, Münster, Germany; and Institute of Clinical Chemistry, University of Zürich (A.v.E.), Zürich, Switzerland.

^* To whom correspondence should be addressed. E-mail: leagottl{at}uni-muenster.de.

Background--The present study was performed to assess the association of prothrombotic risk factors and underlying conditions (infections, vascular trauma, immobilization, malignancies, autoimmune diseases, renal diseases, metabolic disorders, obesity, birth asphyxia, cardiac malformations, and use of prothrombotic drugs) with cerebral venous thrombosis (CVT) in children.

Methods and Results--From 1995 to 2002, 149 pediatric patients aged newborn to <18 years (median 6 years) with CVT were consecutively enrolled. In patients and in 149 age- and gender-matched children with similar underlying clinical conditions but without CVT, the factor V G1691A mutation, the factor II G20210A variant, lipoprotein(a) [Lp(a)], protein C, protein S, antithrombin, and antiphospholipid antibodies, as well as associated clinical conditions, were investigated. Eighty-four (56.4%) of the patients had at least 1 prothrombotic risk factor compared with 31 control children (20.8%; P<0.0001). In addition, 105 (70.5%) of 149 patients with CVT presented with an underlying predisposing condition. On univariate analysis, factor V, protein C, protein S, and elevated Lp(a) were found to be significantly associated with CVT. However, in multivariate analysis, only the combination of a prothrombotic risk factor with an underlying condition (OR 3.9, 95% CI 1.8 to 8.6), increased Lp(a) (OR 4.1, 95% CI 2.0 to 8.7), and protein C deficiency (OR 11.1, 95% CI 1.2 to 104.4) had independent associations with CVT in the children investigated.

Conclusions--CVT in children is a multifactorial disease that, in the majority of cases, results from a combination of prothrombotic risk factors and/or underlying clinical condition.

Key words: pediatrics • lipoproteins • thrombosis

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