Sinovenous thrombosis in children
Section snippets
Epidemiology
The Canadian Pediatric Ischemic Stroke Registry, a population-based national registry, provides the first data on the incidence of sinovenous thrombosis in children [1]. In the Registry, based on 160 pediatric patients with CSVT, the incidence is 0.6 per 100,000 children per year aged term birth to 18 years. Neonates comprise 43% of patients in the Registry, resulting in an incidence for neonatal CSVT of 40.7 per 100,000 live births per year. No prior estimates are available; however, the
Anatomy and physiology
As in adults, the venous drainage of the brain via the “superficial” or “deep” sinovenous systems consist of networks of cerebral sinuses and veins. The location of venous sinuses along suture lines makes them susceptible to mechanical injury during birth in the neonate. The rigid attachment of sinuses and lack of venous valves results in a passive drainage of blood flow in the cerebral venous system [5]. As a result of these anatomic factors, reduction in systemic blood pressure can result in
Pathophysiology of venous infarction
In sinovenous thrombosis, the mechanism for venous infarction is obstruction of venous drainage with increasing venous pressure in the affected region of the brain. The venous congestion results in significant extravasation of fluid into the brain, producing focal cerebral edema and hemorrhage. The edema may be transient, if venous flow is re-established, or be associated with permanent tissue infarction if the increased venous blood pressure eventually exceeds the arterial blood pressure. In
Clinical features
The clinical features of childhood CSVT are subtle, diffuse, and, in very young infants and children, dominated by seizures. Signs of raised intracranial pressure typically develop gradually over hours, days, or even weeks. The clinical presentation is influenced by the age of the child, the extent and location of the thrombus, and the presence or absence of associated venous infarction. In some infants and children, accompanying asphyxia or meningitis may produce neurologic signs that
Risk factors
In childhood CSVT, thrombosis results from a combination of intravascular and vascular factors. Within individual patients, certain underlying risk factors including prothrombotic states may predispose to thrombosis, and other states including acute illnesses or prothrombotic medications act as triggering factors. Dehydration is a major intravascular risk factor at all ages. Vascular malformations including vein of Galen or cerebral arteriovenous malformations can also be associated with CSVT.
Imaging
The diagnosis of sinovenous thrombosis in infants and children is challenging. Findings and radiologic appearances are more variable and nonspecific compared with adults.
A number of radiographic techniques are available to study the cerebral veins and sinuses, and each has advantages and disadvantages (Table 2). But there have been very few systematic studies assessing diagnostic strategies, including magnetic resonance and CT techniques, against a gold standard. The recent advent of
Treatment
Therapy for sinovenous thrombosis includes nonantithrombotic and antithrombotic therapy. In the acute phase of CSVT, nonantithrombotic therapy is aimed at maintaining adequate perfusion of the brain and minimizing the metabolic demands within cerebral tissue in order to minimize the extent of cerebral damage. Approaches include maintenance of blood pressure, correction of hyper- or hypo-glycemia, and prevention of recurrent seizures. In addition, specific primary treatment for all reversible
Outcome
The outcome of CSVT in children is difficult to assess because no standardized outcome measures have been developed, and most studies have had an insufficient duration of follow-up. In addition, given the inconsistent use of anticoagulants and other treatments, it is difficult to adjust for the potential influence of treatment on outcome. A number of factors influence outcome from CSVT, including age at the time of the event, the rapidity of diagnosis, extent of the thrombosis, presence of
Summary
Sinus thrombosis in children is increasingly recognized; however, the diagnosis is still frequently missed. Children may have an increased incidence of this disorder compared with adults, and neonates are at greatly increased risk compared with older children. Childhood CSVT carries significant long-term sequelae that include death or neurologic deficits in nearly 50% of cases. Neonates are not spared from these sequelae. At present, the approach to treatment is empiric but in the past decade
Acknowledgements
The authors are grateful to Dr. Susan Blaser, Staff Neuroradiologist at The Hospital for Sick Children, for her help and expert advice in the preparation of this article.
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2022, Seminars in Pediatric NeurologyCitation Excerpt :Imaging approach for accurate radiological diagnosis of CSVT differs between neonates and non-neonates. Table 1 provides a description of advantages and limitations/nuances of various modalities.26 MRI with venography (MRV) is the diagnostic modality of choice at all ages as it permits assessment of venous flow, thrombus, infarction, underlying parenchymal lesions, hemorrhage, and concurrent intracranial conditions.26
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2019, European Journal of Paediatric NeurologyCitation Excerpt :In most cases, the diagnosis of CSVT can be made on the basis of CT findings as it demonstrates direct signs of thrombosis (cord sign, dense triangle sign, or empty delta sign), edema, cerebral infarction, or parenchymal hemorrhage. However, conventional CT may miss the diagnosis of CSVT in up to 40% of children and underestimate both the extent of the thrombus and the presence of venous infarcts.15 Brain MRI with MRV is considered to be the method of choice for the diagnosis of childhood stroke, visualizes flow and thrombus and is sensitive to parenchymal changes.
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2017, Swaiman's Pediatric Neurology: Principles and Practice: Sixth EditionEasily overlooked sonographic findings in the evaluation of neonatal encephalopathy: Lessons learned from magnetic resonance imaging
2014, Seminars in Ultrasound, CT and MRICitation Excerpt :Often unrecognized in the past, sinus vein thrombosis (SVT) in neonates is being increasingly diagnosed owing to advancements in imaging techniques, and HII has been established as the most common cause for SVT in this age group.97 The symptoms are usually nonspecific and commonly include seizures, jitteriness, and lethargy.97,98 Although computed tomography and MRI are excellent modalities for the detection of venous thrombosis, each has its own pitfalls and its own advantages for the SVT detection.99
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