1500 Endovascular treatment of cerebral venous sinus thrombosis in children: a scoping review

Abstract

<h3>Background</h3> Cerebral venous sinus thrombosis (CVST) is a cerebrovascular disease that typically affects children and young children. Its clinical presentation is highly variable and non-specific, making diagnosis extremely difficult. Systemic anticoagulation is the first-line treatment, with the aim of minimising thrombus extension and achieving recanalisation. However, many patients deteriorate despite maximal anticoagulation. The clinical heterogeneity, low incidence and paucity of clinical trials on CVST have created significant uncertainty and variability in how patients not responding to anticoagulation are managed. Endovascular treatment is an option in these patients. However, there is particularly limited evidence for its use in children. We present the first scoping review of endovascular interventions for CVST in children. <h3>Objectives</h3> To collate, describe and assess the literature on endovascular interventions available for CVST in children, with the aim of guiding future research and the development of clinical guidelines. <h3>Methods</h3> A systematic scoping review on both primary and secondary research on endovascular interventions for CVST specifically in children (aged 1 month - 16 years) was conducted according to PRISMA-ScR guidelines. Studies were identified using the databases PubMed, Embase, Cochrane Library and OpenGrey. 226 studies were identified using our search strategy on 16th October 2020. Following application of eligibility criteria, 48 studies were included for analysis. <h3>Results</h3> Case reports (n=15) and case series (n=15) comprised the majority of the studies. 12 narrative reviews and 1 systematic review were identified. Only 1 non-randomised interventional study and 4 observational studies were identified. No randomised controlled studies were identified. 54 unique, individual children with CVST with details of their diagnosis and endovascular intervention reported were identified across 32 studies. 83% of patients had at least one risk factor for CVST, with inflammatory bowel disease (15%) and dehydration (15%) being the most common. 74% of cases had a bland (i.e. non-haemorrhagic) infarct identified on diagnostic imaging, whilst 26% had a haemorrhagic infarct. The majority (65%) of patients received systemic anticoagulation with heparin before endovascular intervention. 11% did not receive any kind of systemic anticoagulation prior to endovascular intervention, mostly due to rapid, progressive neurological deterioration with anticoagulation started afterwards. The most common indications for endovascular treatment were declining GCS (46%) and worsening/non-improving symptoms despite anticoagulation (35%). Local catheter-guided pharmacological thrombolysis with urokinase or recombinant tissue plasminogen activator was the most commonly used intervention (83%). A combination of endovascular interventions was used in 35% of patients. Complete symptom resolution and complete recanalisation was achieved in 63% and 44% of patients, respectively. 9% of patients died despite endovascular treatment. <h3>Conclusions</h3> The literature reports the use of endovascular interventions for children with progressively worsening symptoms or declining neurological status despite anticoagulation and children in whom anticoagulation is contraindicated. However, there is no consensus on how patients are deemed to be unresponsive to anticoagulation and suitable for endovascular treatment. Cohort studies and randomised controlled trials are needed to robustly assess the efficacy and safety of these interventions in children.