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Paediatric cerebral sinus vein thrombosis. A multi-center, case-controlled study
Gili Kenet1, Dalia Waldman, Aharon Lubetsky
1The Israeli National Hemophilia Center, Sheba Medical Center, Tel-Hashomer, 52621, Israel. Gilli.kenet@sheba.health.gov.il
Insights
Cerebral sinus venous thrombosis (CSVT) in children is not strongly linked to thrombophilia. However, thrombophilia screening is beneficial for children with idiopathic CSVT to guide long-term therapy decisions.
Area of Science:
- Pediatric Neurology
- Hematology
- Thrombosis Research
Background:
- Cerebral sinus venous thrombosis (CSVT) in children lacks clear understanding regarding its causes and contributing risk factors.
- The role of thrombophilic conditions in pediatric CSVT requires further investigation.
Purpose of the Study:
- To investigate the prevalence of thrombophilic risk factors in children diagnosed with CSVT.
- To compare thrombophilia markers in pediatric CSVT patients versus healthy controls.
- To assess the impact of thrombophilia on clinical presentation and treatment decisions in pediatric CSVT.
Main Methods:
- Multi-center case-controlled study involving 46 pediatric patients with CSVT and 112 healthy controls.
- Thrombophilia screening performed on all CSVT cases.
- Prospective follow-up of patients for a median of 4.1 years.
Main Results:
- No significant difference in thrombophilia marker prevalence between CSVT cases and controls.
- Co-morbid systemic illness was common in pediatric CSVT patients.
- Idiopathic CSVT cases showed a higher frequency of thrombophilic risk factors compared to those with co-morbidities.
Conclusions:
- Thrombophilia screening may benefit select pediatric patients, particularly those with idiopathic CSVT.
- Thrombophilic risk factors did not influence clinical presentation or initial treatment in this cohort.
- Further research is needed to fully elucidate the role of thrombophilia in pediatric CSVT etiology.
Abstract:
The etiology and pathophysiology of cerebral sinus venous thrombosis (CSVT) in the paediatric population is still poorly understood, and the role of thrombophilic risk factors remains to be elucidated. In our multi-center case-controlled study we studied 46 patients with CSVT diagnosed from April 1996 to December 2003, consecutively referred for thrombophilia work-up. The results of thrombophilia screen were compared to 112 healthy paediatric controls. Anticoagulant therapy was applied according to treating physicians' decisions, and all cases were prospectively followed for a median of 4.1 years. Of 46 children, 8 had CSVT diagnosed in the neonatal period and therefore were analyzed separately. The prevalence of single thrombophilia markers and combinations of thrombophilic risk factors were similar among cases and controls. Among children with CSVT co-morbid systemic illness was present in most patients at diagnosis. Seven out of 8 children with idiopathic CSVT had thrombophilic risk factors as compared to 31/38 patients with co-morbid conditions. Anticoagulation was initiated in most patients, 11/46 patients continued therapy for a total of one year or more post event. Neither clinical presentation nor initial treatment decisions were affected by presence of thrombophilic risk factors in our study group. Thrombophilia prevalence was not increased in children with CSVT as compared to controls, however thrombophilia was more frequent among children with idiopathic CSVT. Thus, those selected patients would benefit most from thrombophilia work-up, required for long-term therapy considerations.
