Cerebral Venous Thrombosis in Children A Multifactorial Origin

• Published in: Circulation (New York, N.Y.) Vol. 108; no. 11; pp. 1362 - 1367
• Main Authors: Heller, Christine, Heinecke, Achim, Junker, Ralf, Knöfler, Ralf, Kosch, Andrea, Kurnik, Karin, Schobess, Rosemarie, von Eckardstein, Arnold, Sträter, Ronald, Zieger, Barbara, Nowak-Göttl, Ulrike
• Format: Journal Article
• Language: English
• Published: Hagerstown, MD Lippincott Williams & Wilkins 16.09.2003; American Heart Association, Inc
• Subjects: Adolescent; Biological and medical sciences; Cerebral Veins; Child; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Intracranial Thrombosis - diagnosis; Intracranial Thrombosis - etiology; Male; Medical sciences; Neurology; Risk Factors; Vascular diseases and vascular malformations of the nervous system; Vascular Patency; Human; Nervous system diseases; Cerebral vein; Cardiovascular disease; Thrombosis; Cerebral disorder; Venous disease; Vascular disease; Etiology; Central nervous system disease; Risk factor; pediatrics; Diagnosis; Child; Cerebrovascular disease; lipoproteins
• ISSN: 0009-7322; 1524-4539; 1524-4539
• DOI: 10.1161/01.CIR.0000087598.05977.45

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.