[Thrombophilia and thrombosis in children--lessons from cerebral sinus vein thrombosis registry of a tertiary center].

ABSTRACT

BACKGROUND: Pediatric thrombosis is a rare event, and its pathophysiology is often associated with the presence of thrombophilic risk factors as welt as acute comorbid conditions. Sinus venous thrombosis [SVT] in childhood is a serious disease with reported annual incidence of 0.67 per 100,000 children. The etiology and pathophysiology of SVT in the pediatric population is stilt poorty understood, and the role of thrombophilic risk factors remains to be elucidated. METHODS: In our single tertiary center registry the authors documented all new acute cases of radiologically confirmed SVT from 1996 to 2008. Children were consecutively referred for thrombophilia work-up. Anticoagulant therapy was applied according to treating physicians' decision and all cases were prospectively followed for a median of 5 years. RESULTS: Our patient group included 15 males and 8 females whose median age was 2.9 years. Comorbid systemic illness was present in patients at diagnosis. Thrombophilia results were similar in comparison to pediatric controls previously collected at the said medical center. Short term anticoagulation was immediately initiated in most cases, and neurologic outcome was good. Neither clinical presentation nor acute-phase treatment decisions were affected by the presence of thrombophilic risk factors in the specified study group. Nevertheless, since SVT is a rare multifactorial disorder, imaging studies are recommended in sick "high-risk" children for better diagnosis and early initiation of anticoagulant treatment. Despite the presence of comorbid conditions in most cases, thrombophilia work-up is recommended to determine treatment length and assess potential further future risks.