Elsevier

Pediatric Neurology

Volume 49, Issue 5, November 2013, Pages 305-312
Pediatric Neurology

Original Article
Endovascular Treatment of Children With Cerebral Venous Sinus Thrombosis: A Case Series

https://doi.org/10.1016/j.pediatrneurol.2013.07.008Get rights and content

Abstract

Background

Cerebral venous sinus thrombosis is a potentially serious condition affecting 0.56 to 0.67 per 100,000 children annually; adverse outcomes are common. The standard of care is anticoagulation with heparin. A proportion of patients, however, remain in a severe clinical condition and in these, endovascular therapy is an alternative treatment. There is little published literature on the use of endovascular treatments in children with cerebral venous sinus thrombosis.

Methods

We retrospectively reviewed case notes and imaging in a consecutive series of nine children treated using endovascular therapy after diagnosis of cerebral venous sinus thrombosis. Clinical presentation, decision to escalate therapy, methods of recanalization, and clinical outcome were assessed.

Results

Nine children were treated (age range 18 months to 16 years). Diagnosis was made by computed tomography, computed tomography venography, magnetic resonance imaging, or magnetic resonance venography. Seven children were in a coma; one had signs of raised intracranial pressure with progressive cranial nerve palsies; and one was drowsy with a fluctuating hemiparesis. Eight children had been treated with heparin without improvement. Several endovascular methods were used including local tissue plasminogen activator, microguidewire and catheter disruption, balloon angioplasty, and thromboaspiration using the Penumbra device. Eight children had good functional outcomes. One child died as a result of uncontrolled intracranial hypertension secondary to cerebral venous sinus thrombosis.

Conclusion

Endovascular therapy may have a role in the treatment of cerebral venous sinus thrombosis in children when medical therapy has failed and the patient is in a poor clinical condition.

Introduction

See related articles on pages 301 and 313

Cerebral venous sinus thrombosis (CVST) is a serious condition affecting between 0.56 and 0.67 per 100,000 children per year.1, 2 In case series, between 2% and 10% of neonates and between 11% and 17% of older children die as a result of CVST and between 22% and 50% suffer neurological deficits.1, 2, 3, 4 Many minimally symptomatic patients may improve spontaneously and therefore go unrecognized, so this may be an overestimation. However, a proportion of patients will follow a severe course. The standard treatment is systemic anticoagulation with heparin and, in large pediatric case series, anticoagulation is associated with improved clinical outcome.1, 2 There is no randomized data to support this practice and little evidence on therapeutic options in the event of failure to respond to anticoagulation. In adults, this situation is considered a possible indication for endovascular treatment with local intrasinus thrombolysis or mechanical thromboaspiration, and favorable clinical outcomes are seen in as many as 92% of adult patients.5 The endovascular approach allows local thrombolytic directly targeted into the thrombus and for mechanical disruption or aspiration of thrombus, both of which contribute to recanalization, primarily through increasing the contact area between the thrombus and lytic agent. Mechanical thromboaspiration is relatively new and achieves more effective, faster restoration of venous flow with reduction in lytic requirement.

The role of endovascular therapy in the management of pediatric CVST is unclear, especially with regard to patient selection, optimal time to intervene, and contraindications to this therapy. We treated nine children using endovascular techniques with severe CVST who had failed to respond to anticoagulation or in whom anticoagulation was contraindicated. We aimed to assess outcomes after endovascular therapy in patients who had failed to show clinical improvement with conventional management.

Section snippets

Methods

Analysis of this cohort was approved by the Institutional Clinical Effectiveness and Audit Department. Nine consecutive children including seven girls and two boys (ages 18 months to 16 years) with severe CVST, treated using endovascular techniques at our institution between 1999 and 2013, were reviewed. Clinical presentation, decision to escalate therapy, methods of recanalization, and clinical outcome were assessed. All patients except one in whom anticoagulation was contraindicated were

Results

A summary of each patient is described in Table 1 and example cases are displayed in Figure 1, Figure 2, Figure 3. Seven of the nine patients were comatose at the time of endovascular treatment. Five had suffered hemiparesis and three had suffered seizures. The distribution of occlusive thrombus is shown in Table 2. The majority had superior sagittal sinus (six of nine) and transverse sinus involvement but six of nine had straight sinus and five of nine had vein of Galen and internal cerebral

Discussion

Conventional treatment of CVST with anticoagulation aims to arrest thrombus propagation, but a proportion of patients continue to demonstrate clinical deterioration and thrombus propagation despite adequate anticoagulation. In these, lysis and/or disruption of existing thrombus may attain sinus recanalization and a reduction in venous pressure. A systematic review6 including 169 adult patients with CTV treated with local thrombolysis showed a possible benefit for those with severe CVST.

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