Cerebral Sinovenous Thrombosis

Abstract

Cerebral sinovenous thrombosis (CSVT) is a rare but serious cerebrovascular disorder affecting children from the newborn period through childhood and adolescence. The incidence is estimated at 0.6/100,000/year, with 30-50% occurring in newborns. Causes are diverse and are highly age dependent. Acute systemic illness is the dominant risk factor among newborns. In childhood CSVT, acute infections of the head and neck such as mastoiditis are most common, followed by chronic underlying diseases such as nephrotic syndrome, cancer, and inflammatory bowel disease. Signs and symptoms are also age related. Seizures and altered mental status are the commonest manifestations in newborns. Headache, vomiting, and lethargy, sometimes with 6th nerve palsy, are the most common symptoms in children and adolescents. Recent multicenter cohort studies from North America and Europe have provided updated information on risk factors, clinical presentations, treatment practices, and outcomes. While systemic anticoagulation is the most common specific treatment used, there are wide variations and many uncertainties even among experts concerning best practice. The treatment dilemma is especially pronounced for neonatal CSVT. This is due in part to the higher prevalence of intracranial hemorrhage among newborns on the one hand, and the clear evidence that newborns suffer greater long-term neurologic morbidity on the other hand. With the advent of widespread availability and acceptance of acute endovascular therapy for arterial ischemic stroke, there is renewed interest in this therapy for children with CSVT. Limited published evidence exists regarding the benefits and risks of these invasive therapies. Therefore, the authors of current guidelines advise reserving this therapy for children with progressive and severe disease who have failed optimal medical management. As research focused on childhood cerebrovascular disease continues to grow rapidly, the future prospects for improving knowledge about this disorder should be good.

Keywords: cerebrovascular disorders; childhood; neonatal; stroke; thrombosis.

Figure 1

Normal MR venogram, sagittal view (A), axial view (B). Case of cerebral sinovenous thrombosis, acute MR venogram sagittal (C) and axial (D), showing absent flow signal in SS, both TS and StS, with ischemic change (arrow) in cortical white matter on DWI (H). Computed tomography venogram (E) of child with mastoiditis and occlusive thrombus of left SiS and TS (*) and distal SS (**), compared to patent right SiS and TS (arrow). MR venogram 6 months later (F,G), with recanalization of SS, StS, and TS. SS, sagittal sinus; StS, straight sinus; TS, transverse sinus; SiS, sigmoid sinus; JV, jugular vein.