Introduction
Acute thrombosis in patients with an inherited bleeding disorder (BD)
represents a need to balance treating a thrombus with anticoagulation
while maintaining adequate hemostasis 1. This is
important in managing a critical thrombus such as a cerebral sinus
venous thrombosis (CSVT), especially following trauma, when the risk of
intracranial hemorrhage (ICH) is significantly
heightened2,3. In children with BDs, there are no
standard guidelines that direct management of
thrombosis4. Literature regarding the treatment of
CSVT is variable, with studies demonstrating good neurological outcomes
and thrombus resolution without anticoagulation therapy as well as
studies indicating CSVT propagation in over 1/3 of children not treated
with anticoagulation therapy 5-7. Here we present the
management and outcome of four children with inherited BDs who developed
trauma related CSVT. A summary of lab values, management, complications,
and outcomes are provided in Table 1.