Cerebral Sinovenous Thrombosis In Children Research Articles

Cerebral sinovenous thrombosis in children with nephrotic syndrome: systematic review and one new case.

The aim of this review is to provide clinicians with characteristics of children with nephrotic syndrome and cerebral sinovenous thrombosis (CSVT). We have reviewed 37 articles of pediatric cases and provided 1 new case. PRISMA guidelines were followed. Sixty-two patients were included in the review. CSVT was more common in males, usually occurred within 6 months of nephrotic syndrome onset and was found more often in outpatients. The superior sagittal sinus was the most common sinus affected. Non-contrast computed tomography was the most frequent radiologic study performed, with 30% of results negative for CSVT. Headache and vomiting were the most common symptoms while neurologic symptoms were less frequent. Anticoagulation treatment was strongly inconsistent throughout the literature. Thrombosis outcomes were favorable. The most common possible risk factors were corticosteroid treatment, proteinuria and hypoalbuminemia. Four children had a genetic predisposition diagnosed after thrombosis. No markers for anticoagulation prophylaxis seemed to be relevant for the majority of thrombosis occurring in outpatients. Prophylactic anticoagulation does not seem reasonable to prevent CSVT. Knowledge of nonspecific symptoms and of nephrotic syndrome being a state of hypercoagulation and early use of appropriate radiologic study seem to be of major importance.

Post traumatic cerebral sinovenous thrombosis in children: A retrospective and multicenter study.

Cerebral sinovenous thrombosis (CSVT) is a rare but life-threatening condition in the pediatric population and there is no pediatric guidelines regarding anticoagulation for post traumatic CSVT. This study aims to describe a cohort of children with post traumatic CSVT and the use of anticoagulant therapy in this population. A multicenter retrospective study. Patients admitted with post traumatic CSVT in the six participating Pediatric Intensive Care Unit were included. Overall, 29 patients (median age 8.2 years [IQR 4.8-14.6], n=22 (76%) males) were included in the study (Table1). CSVT was observed within the first 24h after admission for a half of the patients (n=14, 50%). Anticoagulation was initiated in 18 patients (62%). No patient received thrombolytic therapy or endovascular treatment. The presence of epidural hematoma was associated with the absence of anticoagulation (n=0 versus n=10, p=0.003). One patient (3%) died of extracranial injury (not related with adverse event of anticoagulation) and in survivors, median Pediatric Overall Performance Category Outcome (POPC) score at discharge from PICU was 2 [IQR 2-4] (i.e., mild disability). Regarding the outcomes of patients, we found no association according to the anticoagulation status (p=1). Overall, 23 patients (79%) had a follow-up cerebral imaging with a median delay of 42 days [IQR 6-63] after admission. CSVT was still seen in 9 patients (31%). We found no difference regarding the persistence of CSVT between patients according to the anticoagulation status (p=0.36). The median duration of anticoagulant treatment was 58 days [IQR 44-91] and one patient (3%) experienced adverse event related to anticoagulation. There were minimal adverse events in patients with post traumatic CSVT treated with therapeutic anticoagulation. However, the effect of anticoagulation on outcomes needs to be confirmed in further studies.

Cerebral Sinovenous Thrombosis in Greek Children: A Single Centre Experience

Introduction: Cerebral sinovenous thrombosis (CSVT) in children is a rare, often underdiagnosed but serious event. The risk factors in children include head or neck infections, prothrombotic agents such as oral contraceptives and a chronic systemic illness. In the present study, we aimed to investigate the clinical manifestations, neuroimaging findings, risk factors and treatment of children suffering from CSVT in a reference paediatric centre for thrombosis. In addition, we assessed outcomes after CSVT.Methods: Data were retrospectively collected for children with CSVT, referred between 2010 and 2020 to our hospital. There were 103 children that were used as controls concerning thrombophilic factors. The categorical variables were described by frequency distributions and compared with x2-homogeneity test.Results: Sixty-five patients were included in the study (58% males). The mean age of the children at the time of diagnosis was 6.2 years (SD 4 years, range 1 month to 16 years). The most common presenting symptoms were headache (43%), decreased consciousness (32%), vomiting (12%), seizures (15%), diplopia (6%) and torticollis (5%). Papilloedema was found in 14 children (21.5%) and intracerebral haemorrhage in one. The most frequent risk factors were infections (74%), mainly acute otitis media with or without mastoiditis (55% και 19% respectively) and chronic medical conditions, such as polycythemia vera, nephrotic syndrome, arteriovenous malformation, ulcerative colitis-3% each. The use of oral contraceptives was not documented. For the diagnostic evaluation MRI/Magnetic Resonance Venography was performed in 72.3% of children. CT was diagnostic in 24.6% of patients and one infant underwent Power Doppler Ultrasound. Thrombosis was detected in 46% of children on left side, in 34% on right side and in 20% bilaterally. The deep venous system (straight venous, vein of Galen, transverse and sigmoid sinus, jugular veins) was more commonly affected (75%). Notably, sigmoid sinus thrombosis (40%) was predominantly involved, followed by transverse (31%), while extension to ipsilateral jugular vein occurred in 32%. Multiple sinus involvement was found in 64% of patients. Interestingly, simultaneous localization in the transverse and sigmoid sinuses had an increased probability of being accompanied by papilloedema (p <0.05). Venous infarctions were noticed in two children, while one child with hypoplastic venous sinus had an abdominal peritoneal CNS drain. Laboratory investigations for prothrombotic risk factors were available for all patients. One or more prothrombotic risk factor were found in 18 of the 65 (28%) children. To be more accurate, heterozygosity for FVLeiden and FII20210A mutations were found in 8% and 5% of patients, respectively and homozygosity for MTHFR-C776T in 15% (without raised homocysteine). Thrombophilic factors did not attain statistically significant results, apart from a trend for heterozygosity for FVLeiden and FII20210A mutations in patients (in controls 5% and 3% respectively). All patients received anticoagulation (68% coumarin anticoagulants, 32% Low Molecular Weight Heparin) for a mean duration of 7.5 ±3.3 months and 18.8 ±32.4 months respectively, while 7 children still receive anticoagulation. The duration of anticoagulation therapy was based on clinical outcome and follow-up investigations. No patient developed hemorrhagic events during the therapy. Follow-up imaging studies were available in most of the children. Six children showed no recanalization on 3.5 ±0.5 months, 19 children showed partial recanalization on 5±3 months and 20 children showed complete recanalization on 7± 5 months. No child died or had persisting neurological sequelae, apart from signs of attention deficit disorder, during a median follow-up of 4±3 years. One patient underwent remission of thrombosis in other site (pulmonary emboli) in adulthood.Conclusions: Physicians should be suspicious of CSVT in children with otitis media/mastoiditis or chronic diseases, when referred for headache or other neurological signs. In a quarter of the cases a thrombophilic factor had eventually some contribution to the event. Longer follow-up may reveal the incidence of cognitive or behavioral disabilities due to CSVT. DisclosuresKattamis: Novartis: Consultancy, Honoraria, Research Funding; CRISPR/Vertex: Consultancy, Honoraria; Chiesi: Honoraria; BMS/Celgene: Consultancy, Honoraria, Research Funding; Agios Pharmaceuticals: Consultancy; IONIS: Consultancy; VIFOR: Consultancy; Amgen: Consultancy.

Clinical and Radiological Findings in Children with Cerebral Sinovenous Thrombosis

Objective: Cerebral sinovenous thrombosis (CSVT) is increasingly recognized in children with advanced use of neuroimaging techniques. In this study, we aimed to present clinical and radiologic features of CSVT in children. Material and Methods: Children aged 1 month to 18 years old diagnosed with CSVT were retrospectively analyzed. Demographic and clinical data of patients including age at diagnosis, sex, presenting symptoms and signs, risk factors for thrombosis, treatment and prognosis were collected. Results: Twelve children were included in the study, 8 of them were male. Headache (n=7), seizures (n=5) and visual impairment (n=4) were the most common presenting symptoms. The superficial venous system was involved in all patients, in five patients deep venous system was also involved. Parenchymal involvement was present in three patients. Two children had isolated cortical vein thrombosis. All patients had underlying risk factors. Five children had recent infections, six children were diagnosed with Behçet’s disease, three with mastoiditis. High lipoprotein (a) level was found in two patients. One patient had a diagnosis of acute lymphoblastic leukemia and received l-asparaginase treatment. All patients were treated with anticoagulants. One patient received tissue plasminogen activator, complicated with intracranial bleeding. Three patients with parenchymal involvement had moderate to severe motor impairment. All patients presenting with seizure had parenchymal involvement and followed up with epilepsy. Conclusion: CSVT causes significant morbidity. Especially in older children presenting with CSVT, symptoms and signs of Behçet’s disease should be questioned. Mastoiditis is still an important reason for CSVT in children.

Cerebral sinovenous thrombosis in children: A single-center experience.

The study aimed to evaluate the patients with a diagnosis of cerebral sinovenous thrombosis in terms of clinical findings, etiology and underlying risk factors, imaging findings, treatment, and prognosis in the long term. Medical records of 19 patients whose ages ranged between 0 days and 17 years with clinical and radiological cerebral sinovenous thrombosis in Ege University Department of Child Neurology were retrospectively evaluated. Nine of nineteen cases were female (47.3%). The median age was 84 months (0-201 months). The most common complaint at the presentation was headache (n=12) and the most common physical examination finding was papilledema (n=11). In etiology, otitis/mastoiditis in three cases, iron deficiency anemia in three cases, sinusitis in two cases, catheter use in four cases, Behçet's disease in three cases were determined. The most common observed genetic factors causing thrombosis was methylenetetrahydrofolate reductase mutation. The transverse sinus (68.4%) is the sinus where thrombosis is most frequently observed. As a result of an average follow-up of 12 months (2-72 months), hemiparesis (n=3/19, 15.7%) and epilepsy (n=5/19, 26.3%) were recorded as sequelae findings, and no mortality was observed. In cases presenting with headache, evaluation of papilledema on funduscopic examination should not be skipped. Neurological imaging should be performed in the change of consciousness of poor feeding infants and children with infections in the head and neck area or underlying chronic diseases. When cerebral sinovenous thrombosis is detected, anticoagulant therapy should be started immediately.

Cerebral sinovenous thrombosis in children: clinical presentation, locations, and acquired and inherited prothrombotic risk factors.

Cerebral sinovenous thrombosis (CSVT) in children is a rare and life-threatening cerebrovascular disease. Hence, we evaluated its clinical presentations, inherited and acquired prothrombotic risk factors along with the accompanying diseases, the thrombosis locations as well as the outcomes of anticoagulant therapy in children with CSVT. The medical records of pediatric CSVT patients treated between January 2011 and September 2018 were analyzed retrospectively. The study included 29 children, 15 boys (51.7%) and 14 girls (48.3%), with the median age being 11 years (range:3 days-17 years). The most commonly presented complaint in neonates was seizures and in the non-neonatal age groups was a headache. Also, at least one acquired and/or inherited thrombophilic risk factor was identified in 89.7% of the patients. The most commonly acquired prothrombotic risk factors along with the accompanying diseases included infections, central venous catheter, and dehydration, while the most commonly inherited thrombophilic risk factors included heterozygous factor-V Leiden mutation and elevated lipoprotein (a). The most common thrombosis location was found to be the transverse sinus. Also, none of the patients died due to the thrombotic episode. Complications included epilepsy in five patients, hydrocephalus in one patient, and intracranial hypertension in another patient. Clinicians need to be well aware of the inherited and acquired prothrombotic risk factors in CSVT. It should also be kept in mind that at-risk patients may also present with nonspecific signs and symptoms with no apparent neurological manifestation. The risk of acute complications and long-term sequelae can be substantially reduced if diagnosed early and initiated with appropriate treatment at the early stages.

Risk Factors for Pediatric Cerebral Sinovenous Thrombosis: A Case-Control Study with Pediatric Neuroradiologist Case Validation

BACKGROUND: Cerebral sinovenous thrombosis (CSVT) is a rare but serious disease process among all patients, with a slightly higher prevalence in pediatric patients. Despite its low frequency, CSVT represents the second most common type of venous thromboembolism (VTE) in children, next to deep vein thrombosis (DVT) of the limbs. Current literature lacks robust evidence on risk factors for CSVT, especially in the pediatric population. We sought to determine risk factors of CSVT in pediatric patients, via a single-institutional case-control study. METHODS: A case-control study was conducted at Johns Hopkins All Children's Hospital from patients admitted between March 31st 2006 and April 1st 2018. Cases were identified based on ICD-9 and ICD-10 codes and confirmed via electronic medical record (EMR) review and neuroradiologist confirmation of radiologic testing. Two controls were then randomly selected for each CSVT case, matched by month and year of admission. Clinical and demographic parameters were collected via abstraction from the EMR, including: age at admission, prior history of VTE, previous hospitalization within 30 days, head/neck surgery, other major surgery, congenital or acquired heart disease, dehydration, cancer, serious infection (included meningitis, sepsis, pneumonia, osteomyelitis, fungemia, pyelonephritis, head/neck infection, abscess involving anatomic sites other than skin or head/neck), prematurity, mechanical ventilation, chronic inflammatory disease, cystic fibrosis, nephrotic syndrome, obesity, and head/neck trauma. Associations between CSVT and putative risk factors were evaluated via logistic regression, using odds ratios (ORs) and 95% confidence intervals (95%CIs). Those risk factors with P-values &lt;0.1 in univariate logistic regression were included in adjusted (multivariate) logistic regression modeling; results of the latter employed a threshold of P&lt;0.05 for statistical significance. RESULTS: A total of 60 CSVT cases and 120 controls were identified. Median (and interquartile range in) age was 4.79 years (0.02-13.56 years) for cases and 5.58 years (1.16-10.78 years) for controls. Factors putatively associated with CSVT in unadjusted analyses were: presence of central venous catheter; serious infection; mechanical ventilation; chronic inflammatory disease; and head/neck trauma. In the multivariate model, mechanical ventilation (OR=9.01, 95%CI=2.19-37.02) and head/neck trauma (OR=11.52, 95% CI=2.63-50.41) remained independent, statistically-significant risk factors for CSVT. CONCLUSIONS: This single-institutional case-control study reveals that mechanical ventilation and head/neck trauma are independent risk factors for pediatric CSVT. These findings will be further investigated via the multicenter Children's Hospital-Acquired Thrombosis (CHAT) registry dataset, by which a risk model for CSVT in children will be further developed and validated, in order to inform future preventive strategies in pediatric patients at heightened risk of CSVT. Table Disclosures Goldenberg: NIH: Other: research support and salary support.

Seizures and Outcome One Year After Neonatal and Childhood Cerebral Sinovenous Thrombosis

BackgroundPediatric cerebral sinovenous thrombosis is a treatable cause of brain injury, acute symptomatic seizures, and remote epilepsy. Our objective was to prospectively study epilepsy and outcomes in neonates and children one year after cerebral sinovenous thrombosis diagnosis. MethodsPatients with cerebral sinovenous thrombosis were enrolled prospectively from 21 international sites through the Seizures in Pediatric Stroke Study. Clinical data, including acute symptomatic seizures and cerebral sinovenous thrombosis risk factors, were collected at diagnosis. A neuroradiologist who was unaware of the diagnosis reviewed acute imaging. At one year, outcomes including seizure recurrence, epilepsy diagnosis, antiepileptic drug use, and modified Engel score were collected. Outcomes were assessed using the modified Rankin score and the King's Outcome Scale for Childhood Head Injury. ResultsTwenty-four participants with cerebral sinovenous thrombosis were enrolled (67% male, 21% neonates). Headache was the most common presenting symptom in non-neonates (47%, nine of 19). Nine (37.5%) presented with acute symptomatic seizures. Six (25%; 95% confidence interval, 10% to 47%) developed epilepsy by one-year follow-up. No clinical predictors associated with epilepsy were identified. King's Outcome Scale for Childhood Head Injury and modified Rankin scores at one year were favorable in 71%. Half of the patients who developed epilepsy (three of six) did not have infarcts, hemorrhage, or seizures identified during the acute hospitalization. ConclusionOur study provides a prospective estimate that epilepsy occurs in approximately one-quarter of patients by one year after diagnosis of cerebral sinovenous thrombosis. Later epilepsy can develop in the absence of acute seizures or parenchymal injury associated with the acute presentation.

Treatment and outcome of childhood cerebral sinovenous thrombosis.

To test our hypothesis that anticoagulation is associated with better neurologic outcomes in childhood cerebral sinovenous thrombosis (CSVT), we analyzed treatment and outcomes in a population of 410 children from the International Pediatric Stroke Study (IPSS). We included patients enrolled in the IPSS registry with a diagnosis of CSVT at age >28 days with radiologic confirmation, in isolation or with concomitant arterial ischemic stroke. The primary outcome was the neurologic status at discharge. We defined unfavorable outcome as severe neurologic impairment or death at discharge. The Pediatric Stroke Outcome Measure was used for long-term outcome in those with follow-up. Predictors of anticoagulation use and outcome were analyzed by logistic regression. Most children (95%) had identifiable risk factors, and 82% received anticoagulation. Shift analysis demonstrated better outcomes at discharge in children who were anticoagulated, and this persisted with longer-term outcomes. In multivariable analysis, anticoagulation was significantly associated with favorable outcomes (adjusted odds ratio [aOR] unfavorable 0.32, p = 0.007) whereas infarct was associated with unfavorable outcome (aOR unfavorable 6.71, p < 0.001). The trauma/intracranial surgery was associated with a lower odds of anticoagulation use (aOR 0.14, p < 0.001). Within the IPSS registry, children with risk factors of trauma or intracranial surgery were less likely to receive anticoagulation for CSVT. Anticoagulation was associated with a lower odds of severe neurologic impairment or death at hospital discharge, but this finding is limited and needs further confirmation in randomized, controlled, prospective studies.

Cerebral Sinovenous Thrombosis.

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.

Deep cerebral venous thrombosis: Rare cause of stroke in children

Clinical presentations of childhood cerebral sinovenous thrombosis are nonspecific and variable. Computed tomography venography and magnetic resonance imaging with magnetic resonance venography are the investigation of choice for the diagnosis of sinovenous thrombosis. We present an 8-month-old female child with a history of loose stools and vomiting. She subsequently developed altered consciousness and abnormal jerky movements. Magnetic resonance imaging showed sub acute hemorrhagic bithalamic infarcts with deep sinus venous thrombosis. A brief review of the deep sinus venous thrombosis including the present case is presented.

Anticoagulant therapy in head injury-associated cerebral sinovenous thrombosis in children.

Head injury is a risk factor for cerebral sinovenous thrombosis (CSVT) in children. Literature concerning head injury-associated CSVT (HIA-CSVT) is scarce. Data supporting safety and efficacy of anticoagulant therapy (ACT) in childhood CSVT is emerging. However, intracranial hemorrhage (ICH) occurs frequently in children with HIA-CSVT at diagnosis making initiation of ACT controversial due to the fear of worsening of ICH. We conducted a retrospective descriptive review of a consecutive cohort of children with HIA-CSVT from 1998 to 2012. Twenty patients (14 males, mean age 7 years) with HIA-CSVT were identified. Most (19/20 [95%]) had significant ICH at diagnosis. None received ACT at diagnosis. Fourteen (70%) were later (median 7 days post-trauma, range 2-48 days) treated with ACT due to CSVT persistence (nine) and propagation (five), despite ICH in 13. None of the treated patients, including the 13 with pre-existing ICH, had significant worsening of hemorrhage. Three (21%) treated patients had minor asymptomatic extension of their hemorrhage and further ACT was withheld. No patient died while on ACT. No patient experienced CSVT propagation on ACT. Clinical outcomes were normal (no neurologic deficits) in 5/20(25%), mild neurological deficits in 10/20(50%), and moderate-severe neurological deficits in 5/20(25%). Small sample size did not permit assessment of the effect of ACT on outcome. Anticoagulant therapy is safe in selected children with HIA-CSVT. ICH is not an absolute contraindication to ACT in children with HIA-CSVT.

Cerebral Sinovenous Thrombosis in Children and Neonates

Our aim is to present the etiology and risk factors for cerebral sinovenous thrombosis (CSVT) and the radiological findings, anticoagulant therapy used, and treatment outcome of patients with CSVT. This study included 12 patients who were treated for CSVT at the Ankara University, School of Medicine, Department of Pediatric Neurology. This study included 5 girls (41.7%) and 7 boys (58.3%) with a mean age of symptom onset of 5.2 ± 6.29 years (range: 0-18 years), who were followed at our institution for a mean of 1.8 ± 1.73 years (range: 0-6.5 years). Among the patients, 3 had no risk factors, 2 had 1 risk factor, and 7 had multiple risk factors. Anticoagulant therapy was administered to 4 patients, of which 1 had neurological sequelae; neurological sequelae or exitus occurred in 4 of the 8 patients who did not receive anticoagulant therapy. The present findings showed that appropriate prophylaxis in appropriately selected patients reduced the rate of recurrence of CSVT.

P339 – 1563 Cerebral sinovenous thrombosis in children and neonates: clinical experience, laboratory findings, treatment, and outcome

P339 – 1563 Cerebral sinovenous thrombosis in children and neonates: clinical experience, laboratory findings, treatment, and outcome

Place des antithrombotiques dans l’accident vasculaire cérébral de l’enfant

Each year, 500 to 1000 children experience a stroke in France. Long-term follow-up demonstrates persistent neurologic deficit in most of them. Despite the lack of studies with high level of evidence, paediatric stroke guidelines are regularly updated based on observational data, analogy with recommendation for adults, and expert opinion. It is reasonable to initiate anticoagulation during the acute phase of cerebral sinovenous thrombosis in children; the duration of anticoagulation needs to be individually tailored. It is not yet possible to draw any conclusions regarding neonates. Intravenous or intra-arterial thrombolysis and mechanical devices have the potential to provide instant and safe recanalization under optimal circumstances, but no generalization about their efficacy in childhood ischemic stroke can be drawn. Aspirin is effective in reducing the risk of recurrence in children with arterial ischemic stroke. There is a need to develop new studies with easy for use of antithrombotic drugs, such as aspirin or oral direct factor Xa inhibitors. A specific approach for rare disease population, which follows guidance for the design and conduct of paediatric trials, is required.

Abstract WP412: Anticoagulant Therapy in Head Injury-associated Cerebral Sinovenous Thrombosis in Children

Background - Head injury is a rare risk factor associated with cerebral sinovenous thrombosis (CSVT). Literature concerning head injury-associated CSVT (HIA-CSVT) is scarce. There is emerging data to support the safety and efficacy of anti-coagulant therapy (ACT) in childhood CSVT. However, intracranial hemorrhage (ICH), which is seen frequently in children with HIA-CSVT at diagnosis, makes initiation of ACT controversial due to the fear of extension of pre-existing ICH. boldMethods - We conducted a retrospective review of a consecutive cohort of children with HIA-CSVT from 1998-2009. Results - Seventeen patients (11 males, mean age 7-years) with HIA-CSVT were identified. Most [16/17 (94%)] had significant ICH at diagnosis. None received ACT at diagnosis. 12/17 (71%) were later treated with ACT due to CSVT persistence (8) and propagation (4) despite ICH in 11. None of the 12 treated patients, including the 11 with pre-existing ICH, had significant worsening of hemorrhage on ACT. Three (25%) of the 12 treated patients had minor extension of their hemorrhage; further ACT was withheld in these patients due to physician preference. No patient died on ACT. No patient experienced CSVT propagation on ACT. Neurological outcome was normal in 3/17 (18%), mildly abnormal in 9/17 (53%) and moderate-severely abnormal in 5/17 (29%). Due to small sample size, influence of ACT on clinical and recanalization outcome could not be measured. Conclusions - Anticoagulant therapy is safe in selected children with HIA-CSVT. Head injury is not an absolute contraindication to ACT in children with HIA-CSVT.

EPNS/SFNP guideline on the anticoagulant treatment of cerebral sinovenous thrombosis in children and neonates

Anticoagulation of cerebral sinovenous thrombosis (CSVT) is recommended in adults and has been also approved in the paediatric setting. Some controversies remain however between the existing paediatric professional consensus, notably about its use in children with intra-cranial haemorrhage and in neonates. The publication of further original studies prompted the French Society for Paediatric Neurology (SFNP) in association with a panel of EPNS experts, to update the level of evidence and the knowledge in this domain.A bibliographic analysis revealed that anticoagulants are widely used in paediatrics. Anticoagulation is well tolerated by children (Class I, level of evidence B) and also probably by neonates (Class IIa, level of evidence B). During the acute phase, anticoagulation is probably effective in reducing the risk of death and sequelae in children (Class IIa, level of evidence B). It is not yet possible to draw any conclusions regarding neonates (Class IIb). Anticoagulation is also effective in reducing the risk of recurrence (Class I, level of evidence B). This risk is dependent on several individual factors such as the age of the child, the cause of the thrombosis, the persistence or the recurrence of thrombogenic factors, and the speed of sinus recanalisation. The duration of anticoagulation needs therefore to be individually tailored (Class I, level of evidence B).These observations have led to the following recommendations:-In the absence of any contraindication, it is reasonable to initiate anticoagulation during the acute phase of CSVT in children. Prolonged treatment over 3–6 months is justified according to individual factors.-In the absence of any contraindication, anticoagulation may be considered individually during the acute phase of CSVT in neonates for a duration of 6–12 weeks.

Childhood Cerebrovascular Disease

Cerebrovascular disease was selected as the topic for the 10th Neurobiology of Disease in Children Symposium, which was held October 13, 2010, in Providence, Rhode Island. Program directors Drs Gabrielle deVeber, Donna Ferriero, Steve Roach, and Zinaida Vexler prepared an outstanding agenda with individual presentations by and a panel discussion with highly respected leaders in the field of cerebrovascular disease. The conference covered the clinical aspects, pathogenesis, and current management of various forms of childhood stroke, followed by a session on controversial topics and unanswered questions, and concluded with a panel discussion consisting of many of the day’s speakers. Dr deVeber opened the symposium with a talk highlighting recent insights in diagnosis and initial treatment of pediatric stroke. One of the main forms of childhood stroke, arterial ischemic stroke, was introduced by Dr Adam Kirton, who emphasized the importance of using imaging to more efficiently diagnose children with stroke. Dr Kirton made the point—echoed by many speakers throughout the day—that childhood stroke is far more common than has previously been thought. Dr Heather Fullerton continued the discussion of arterial ischemic stroke, focusing on common symptoms of stroke and how to recognize them as well as basic management. Dr Fullerton gave a second presentation later in the day that covered secondary stroke prevention in children, covering not only the role of antithrombotics but other treatment options for secondary stroke prevention. Cerebral sinovenous thrombosis was covered by both Dr Linda DeVries and Dr Fenella Kirkham. Dr DeVries focused on the importance of imaging for diagnosis of perinatal cerebral sinovenous thrombosis and statistical data from the International Pediatric Stroke Study. Dr Kirkham’s talk centered on childhood cerebral sinovenous thrombosis, presenting the interesting finding that iron deficiency is common in children with this condition. In an afternoon session, Dr Mahendra Moharir spoke on the clinical management and challenges of treating neonatal sinovenous thrombosis and addressed the debate regarding the use of anticoagulant therapy as treatment. The third and final form of childhood stroke, hemorrhagic stroke, was covered by Dr Warren Lo, who focused specifically on intracranial and subarachnoid hemorrhage. Dr Lo presented a broad view of hemorrhagic stroke, including incidence, identified risk factors, diagnosis, and management, touching on the American Heart Association guidelines that were presented in detail during a later session. A session on the pathogenesis of childhood stroke provided valuable insight into several topics. Dr Frank Sharp presented data on gene expression profiling of blood cells in ischemic stroke and introduced several gene profiles that have been useful in diagnosing stroke, particularly when the cause of ischemia is unknown. Dr Eng Lo covered the topic of neurovascular niche, positing the entire neurovascular unit as a target for therapy and highlighting recent investigations into neuroprotection, including the cerebral endothelium as a neuroprotective organ. The role of inflammation in stroke was presented by Dr Zinaida Vexler, who discussed not only the detrimental effects of inflammation in the poststroke cellular environment but also its contribution to recovery neurogenesis and investigations into how to enhance this effect. Dr Patricia Massicotte presented on thrombus formation, the inciting event of arterial ischemic stroke, highlighting the differences in thrombus formation in children from that in adults, particularly that vitamin K–dependent factors are decreased in children, as are thrombin, protein C, and protein S, although plasminogen activator inhibitor levels are increased. Finally, Dr Michael Rivkin shared insights into nervous system plasticity based on new developments in neuroimaging, particularly into cortical synaptogenesis and pruning. In a session on management and therapy, Dr Steve Roach shared the latest American Heart and Stroke Association Guidelines on diagnosis and management of childhood stroke.

Cerebral Sinovenous Thrombosis in a Toddler With Iron Deficiency Anemia

Cerebral sinovenous thrombosis (CSVT) is an increasingly recognized cause of neurological morbidity and mortality within the pediatric population. Unlike its manifestation in the adult population, CSVT in children has a broad and often multifactorial cause. Our case describes an 18-month-old young boy presenting to the emergency department twice during a 48-hour period with vomiting and lethargy. The child was found to have a sagittal sinus venous thrombosis caused by severe dietary iron deficiency anemia. Severe iron deficiency anemia has been linked to CSVT formation in several recent case reports.

Cerebral sinovenous thrombosis in children: Clinical presentation and extension, localization and recanalization of thrombosis

Many important questions regarding pathophysiology and treatment of cerebral sinovenous thrombosis need clarification and may depend on further knowledge on the etiology, site, extension and recanalization of the thrombosis. We studied these variables in a cohort of children and adolescents from seven Portuguese Centers. We conclude from our results that the deep venous system and the superior longitudinal sinus are less frequently affected with thrombosis but have a greater potential for serious neurologic disease and for major sequelae. Non-recanalization, at least in the long term, is not an adverse prognostic factor. Extensive propagation of the thrombus from the initial site of origin seems to be common. The early identification of risk factors and their treatment coupled with an aggressive attitude towards diagnosis and treatment for thrombosis involving the deep venous system would be warranted.