Cerebral venous sinus (sinovenous) thrombosis (CSVT) is a serious and rare disorder, whose etiology and pathophysiology has not yet been completely clarified. Unlike in adults with CSVT, management in children and neonates remains controversial. Nonetheless, mortality and morbidity associated with neurologic sequelae among survivors are significant.
Stroke and cerebrovascular diseases are increasingly recognized and diagnosed in pediatric patients, including neonates, due to mounting awareness and advances in neuroimaging techniques. Stroke is defined as the sudden occlusion or rupture of cerebral arteries or veins resulting in brain injury. Stroke due to vascular occlusion is broadly divided into arterial ischemic stroke (AIS) and cerebral sinovenous thrombosis (CSVT). In AIS, brain injury occurs due to arterial occlusion secondary to thromboembolism resulting in cerebral infarction. In CSVT, brain dysfunction occurs due to thrombotic occlusion of cerebral veins and/or dural venous sinuses resulting in venous congestion and intracranial venous hypertension, which may or may not be associated with “venous infarction.”
Although there is overlap in the conditions predisposing to neonatal AIS and CSVT, the clinical and radiographic features, management, and outcomes are distinct. The condition of CSVT is underrecognized in neonates due to the subtle and nonspecific presentation, resulting in delayed or missed. diagnosis. While randomized controlled trials (RCTs) have established the usefulness of anticoagulant therapy for adults with CSVT, pediatric data are limited to multicenter (childhood CSVT) and single center (neonatal CSVT) cohort studies. Important developmental differences in the hemostatic, sinovenous, and neurologic systems in children and neonates prevent the direct extrapolation of adult treatment data to pediatrics. Current knowledge, though limited due to sample size or single-center enrolment, addresses the epidemiology, symptoms, neuroimaging diagnostic findings, treatment practices, and outcome of pediatric and neonatal CSVT, and will be summarized in this chapter.