Cerebral Sinovenous Thrombosis Research Articles

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.

Tachyarrhythmia-induced Cerebral Sinovenous Thrombosis in a Neonate Without Cardiac Malformation

Tachyarrhythmia-induced Cerebral Sinovenous Thrombosis in a Neonate Without Cardiac Malformation

Inaugural Cerebral Sinovenous Thrombosis Revealing Homocystinuria in a 2-Year-Old Boy

Cerebral sinovenous thrombosis is unusual during childhood and requires early and accurate management because of its detrimental consequences. We report on the case of a 2-year-old boy with mild psychomotor delay, who presented with nonfebrile acute ataxia. A brain computed tomographic (CT) scan showed complete thrombosis of the superior sagittal sinus, confirmed by magnetic resonance angiography and associated with a right frontal hemorrhagic infarction. Systematic screening for thrombophilia revealed homocystinuria linked to cystathionine β-synthase deficiency with underlying compound heterozygosity. The evolution was favorable after anticoagulant therapy, specific diet, and vitamin supplementation. This case is of interest because of the unusual clinical presentation as a pediatric cerebral sinovenous thrombosis. Furthermore, homocystinuria is rarely revealed by cerebral sinovenous thrombosis at the onset of the disease and should systematically be ruled out in pediatric stroke.

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.

Spontaneous intestinal perforation and multiple thrombotic events in a neonate with thrombophilia

Abstract Spontaneous intestinal perforation (SIP), a rare complication in neonates, was diagnosed in a preterm newborn. After surgery, small bowel histology showed acute ischemic changes and one area of perforation. Fibrin thrombus in the submucosal capillary vessels suggested a thrombotic mechanism in the pathogenesis. Subsequently, two severe asynchronous thrombotic events developed: cerebral sinovenous thrombosis and renal vein and inferior vena cava thrombosis. The neonate presented with hypercoagulability and factor V Leiden heterozygosity. This is the first association of SIP with inherited thrombophilia and thrombotic events in a neonate, suggesting hypercoagulability as a new research focus for pathogenesis of this enigmatic disease.

Neonatal stroke: a review of the current evidence on epidemiology, pathogenesis, diagnostics and therapeutic options

Neonatal stroke, including perinatal arterial ischaemic stroke and cerebral sinovenous thrombosis, remains a serious problem in the neonate. This article reviews the current evidence on epidemiology, pathogenesis, diagnostics and therapeutic options. Although our understanding of the underlying mechanisms and possible risk factors has improved, little progress has been made towards therapeutic options. Considering the high incidence of neurological sequelae, the need for therapeutic options is high and should be the focus of future research.

Extensive cerebral Sinovenous Thrombosis in a 5 Year Old Girl, Following Mild Dehydration. (Case Report and Review of Literature)

Cerebral sinovenous thrombosis (CSVT) in children has rarely been reported in the literature, especially without underlying disorder. It has increasingly been diagnosed due to clinical awareness and sensitive neuroimaging techniques. The aim of this article was to report a case of cerebral sinovenous thrombosis without underlying disorder. We reported a 5 year old girl, presented with severe headache and seizure. She had a history of fever and diarrhea before the onset of headache. Neuroimaging showed evidence of CSVT on MRI and magnetic resonance venography. Investigations showed no inherited thrombophilia. The patient was treated with low molecular weight heparin (LMWH) which continued by warfarin. This case illustrated severe complications of dehydration in pediatrics without any evidence of underlying disorders.

The Development Of Venous Thromboembolism May Increase The Risk Of Osteonecrosis In Children With Acute Lymphoblastic Leukemia

BackgroundOsteonecrosis (ON) is a severely disabling complication of anti-leukemic therapy, specifically long-term corticosteroid use. A hypercoagulable state is thought to underlie corticosteroid-related ON. Children with acute lymphoblastic leukemia (ALL) are also at increased risk of venous thromboembolism (VTE), indicating underlying hypercoagulability in this disease entity. Hence, we explored the relationship between ON and VTE, along with the association of ON with other variables, including age and asparaginase (ASP) therapy, in children with ALL. MethodsHealth records of children (< 18 yrs.) with de novo ALL treated at McMaster Children's Hospital from 1992 to 2010 were reviewed. Patients were treated according to Dana-Farber Cancer Institute (DFCI) ALL Consortium Protocols. Data regarding demographics, leukemia diagnosis and therapy, development and characteristics of ON and VTE, and thrombophilia work-up, if any, were collected from computer records and chart review.Osteonecrosis was diagnosed by plain X-ray, computed tomography (CT), magnetic resonance (MR) imaging, and/or technetium-99m (99mTc) bone scan. We included ON diagnosed during therapy and/or at any point during post-treatment follow-up.Standard radiological measures, including venous Doppler ultrasound and/or venography (conventional, CT, MR), confirmed VTE. We included only clinically significant thromboembolic events, defined as symptomatic VTE, or asymptomatic VTE requiring anticoagulation, developing during ALL therapy.Logistic regression analyses were performed to identify possible predictors of ON. Odds ratios (ORs) with 95% confidence intervals (CIs) and corresponding p-values were determined. ResultsMean age of the study cohort (n = 208) was 5.4 years and male/female ratio 1.2:1. Seventy-eight (37.5%) patients had high-risk (HR) ALL and 127 (61.1%) received dexamethasone (DEX) as post-induction steroid. One hundred and sixty-two (77.9%) patients received E. coli ASP, 19 (9.1%) Erwinia ASP, and 27 (13.0%) PEG ASP.Twenty-one (10.1%) children developed ON. Joints affected by ON included the ankle in 11 subjects, knee in 10, hip in 8, and heel in one. Fourteen of the 21 patients (66.7%) had involvement of more than one joint. All patients were diagnosed with ON during ALL treatment, with the average being 69.2 weeks following ALL diagnosis.Forty-two (20.2%) subjects had a VTE while receiving therapy at an average of 29.4 weeks after ALL diagnosis. Nine patients had cerebral sinovenous thrombosis, 7 deep vein thrombosis (DVT), and one pulmonary embolism (PE). Twenty-six patients developed a central venous line (CVL)-related VTE.Results of univariate logistic regression analyses for osteonecrosis are presented in Table 1. VTE strongly predicted development of ON – OR 8.85 (95% CI 3.37–23.25, p< 0.001). Thirteen (31.0%) patients with VTE developed ON compared to 8 (4.8%) of 166 subjects without VTE. In 10 of 13 (76.9%) patients who developed both VTE and ON, the diagnosis of VTE preceded that of ON.Table 1Univariate logistic regression for ONFactorTotal nON, n (%)OR95% CIp-valueAge (yrs.)< 1017710 (5.6)>= 103111 (35.5)9.183.47–24.32< 0.001GenderM1158 (7.0)F9313 (14.0)2.170.86–5.490.10Risk-categorySR13010 (7.7)HR7811 (14.1)1.970.80–4.880.14SteroidPRED815 (6.2)DEX12716 (12.6)2.190.77–6.230.14ASPE. coli16213 (8.0)Erwinia191 (5.3)0.640.08–5.160.67PEG277 (25.9)4.011.43–11.24< 0.01Cranial radiationN11310 (8.8)Y877 (8.0)0.900.33–2.470.84VTEN1668 (4.8)Y4213 (31.0)8.853.37–23.25< 0.001Given that older age is a known risk factor for both VTE and ON, we conducted a multivariate analysis, which confirmed that age, ASP type, and VTE were independent, significant risk factors for ON (Table 2).Table 2Multivariate logistic regression for ONFactorOR95% CIp-valueAge (>= 10 yrs.)6.112.11–17.72< 0.001ASP (PEG vs. E. coli)3.831.19–12.310.02VTE (Y)5.692.00–16.140.001 ConclusionIn addition to the known impact of older age, we identified VTE and type of ASP as independent risk factors for ON in children with ALL. These observations suggest overlap in the etiopathogenesis of ON and VTE. We recommend larger, prospective studies to confirm the association of VTE and PEG ASP with ON and to assess the impact of hypercoagulability on the development of ON. This in turn may help develop preventive strategies (e.g., thromboprophylaxis) for ALL-associated ON. Disclosures:No relevant conflicts of interest to declare.

Cerebral Sinovenous Thrombosis Associated With Mastoiditis Due to Recurrent Otitis Media

Cerebral sinovenous thrombosis (CSVT) in childhood is a rare reported. In this era of widespread antibiotic use for acute otitis media, the incidence of otogenic CSVT has markedly declined but has not been completely prevented. The current therapies for CSVT include anticoagulation, thrombolysis, hydration, surgery, and supportive care that were based on adult studies, pediatric case studies, and expert opinion. We describe the case of a 3-year-old boy who presented with manifestations of CSVT associated with mastoiditis secondary to otitis media. He completed a 3-month course of combination antibiotic and anticoagulation therapy; the CSVT was recanalized, and the mastoiditis had partially improved.

Serial Cranial US for Detection of Cerebral Sinovenous Thrombosis in Preterm Infants

Purpose To report the incidence of cerebral sinovenous thrombosis (CSVT) in a prospective cohort of preterm infants with a gestational age of less than 29 weeks. Materials and Methods The local medical ethics review board approved this study, and written parental consent was obtained. Preterm infants with a gestational age of less than 29 weeks who were admitted to the neonatal intensive care unit were prospectively studied with cranial ultrasonography (US). The scanning protocol included visualization with color Doppler imaging of the superior sagittal sinus and transverse sinuses through the anterior (8.5-MHz probe) and mastoid (13-MHz probe) fontanelles. When feasible, magnetic resonance imaging was performed to confirm cranial US-diagnosed CSVT. The differences between preterm infants with and those without CSVT were analyzed by using Mann-Whitney tests for continuous variables and Fisher exact tests for categorical data. Results Cranial US was used to document CSVT in 11 of 249 preterm infants with a gestational age of less than 29 weeks. Transverse sinuses were most frequently affected (in all 11 patients with CSVT). All infants with CSVT were asymptomatic. Postnatal age at diagnosis ranged from 5 to 34 days. The mean gestational age was significantly lower in infants with CSVT (25.9 weeks vs 26.8 weeks, P = .038). Of the risk factors studied, only duration of mechanical ventilation was associated with CSVT; it was significantly longer in the CSVT group. Conclusion Systematic serial cranial US of infants with a gestational age of less than 29 weeks showed a remarkably high incidence of CSVT of 4.4%. Cranial US including color Doppler imaging with scans obtained through the mastoid fontanelle can depict CSVT at an early stage. Treatment of this possibly important condition needs attention. © RSNA, 2013

Serial Cranial US for Detection of Cerebral Sinovenous Thrombosis in Preterm Infants

To report the incidence of cerebral sinovenous thrombosis (CSVT) in a prospective cohort of preterm infants with a gestational age of less than 29 weeks. The local medical ethics review board approved this study, and written parental consent was obtained. Preterm infants with a gestational age of less than 29 weeks who were admitted to the neonatal intensive care unit were prospectively studied with cranial ultrasonography (US). The scanning protocol included visualization with color Doppler imaging of the superior sagittal sinus and transverse sinuses through the anterior (8.5-MHz probe) and mastoid (13-MHz probe) fontanelles. When feasible, magnetic resonance imaging was performed to confirm cranial US-diagnosed CSVT. The differences between preterm infants with and those without CSVT were analyzed by using Mann-Whitney tests for continuous variables and Fisher exact tests for categorical data. Cranial US was used to document CSVT in 11 of 249 preterm infants with a gestational age of less than 29 weeks. Transverse sinuses were most frequently affected (in all 11 patients with CSVT). All infants with CSVT were asymptomatic. Postnatal age at diagnosis ranged from 5 to 34 days. The mean gestational age was significantly lower in infants with CSVT (25.9 weeks vs 26.8 weeks, P = .038). Of the risk factors studied, only duration of mechanical ventilation was associated with CSVT; it was significantly longer in the CSVT group. Systematic serial cranial US of infants with a gestational age of less than 29 weeks showed a remarkably high incidence of CSVT of 4.4%. Cranial US including color Doppler imaging with scans obtained through the mastoid fontanelle can depict CSVT at an early stage. Treatment of this possibly important condition needs attention.

Cerebral Sinovenous Thrombosis Associated With Iron Deficiency Anemia Secondary to Severe Menorrhagia

Cerebral sinovenous thrombosis is a rare condition presenting with a wide spectrum of nonspecific symptoms that can make early diagnosis difficult. Cerebral sinovenous thrombosis has been associated with various etiologies. Iron deficiency anemia associated with cerebral sinovenous thrombosis in teenagers is rare. We present a teenage patient with complete thrombosis of the vein of Galen, straight sinus, and left internal cerebral vein associated with iron deficiency anemia due to severe menorrhagia. Mechanisms that can explain the association between iron deficiency anemia and thrombosis are discussed.

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

Hemorrhagic and Ischemic Stroke in Children With Cancer

Adult survivors of childhood cancer have an increased risk of cerebrovascular disease; little is known about early stroke risk in childhood cancer. Our objectives were to assess stroke prevalence in children with cancer, to establish cancer and stroke type, and to determine if modifiable risk factors for stroke were present. Children with stroke and cancer were compared with all children seen for cancer at a single institution between 2000 and 2009. An International Classification of Disease, 9th version, code search and search of existing pediatric oncology and stroke databases identified children <18 years with ischemic stroke, intracerebral hemorrhage, and cerebral sinovenous thrombosis. Of 1411 children with cancer, 15 had a stroke (1.1%, 95% CI: 0.6-1.7%). Stroke classifications were seven intracerebral hemorrhages, five ischemic strokes (one of which was followed by intracerebral hemorrhage), and three sinovenous thromboses. Stroke occurred at a median of 5 months after cancer diagnosis. Ten children with strokes had hematologic malignancies and five had brain tumors. Thirteen patients died poststroke, eight because of withdrawal of care. White blood cell count ≥48,000/mm3 was found in four children, all with intracerebral hemorrhage. Five of seven children with intracerebral hemorrhage had platelets <50,000/mm3. Stroke has a prevalence of approximately 1% in children with cancer. Hemorrhagic stroke and ischemic stroke occur with approximately equal frequency; children with leukemia and brain tumors are at greatest risk.

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.

Accidente vascular encefálico: Presentación clínica y factores de riesgo

ABSTRACT Clinical presentation and risk factors of cerebrovascular accident Introduction: Acute focal brain dysfunction in adults serves to diagnose and to provide early rescue of the brain tissue. In children, the clinical differences of this condition compared to adults results in delayed diag-nosis and treatment. Objective: To describe the clinical presentation and risk factors of CVA in 156 children and related to age and type of CVA. Patients and Method: A CVA registry of newborn to 18 years old was started in January 2003 were analized. Diagnosis was confirmed by brain imaging. Results: The median of age was 4.9 month (33% was newborn, 46% 6 years old); 64% were males. Arterial ischemic stroke represented 85%, the rest were cerebral sinovenous thrombosis. In the clinical manifestations the diffuse signs were more frequently seen (67%) in children < 6 years of age (p 0.001). Seizures were observed in 61% in children < 6 years old (p = 0.03), 33% presented focal signs. The most common risk factors were acute sys-temic diseases and heart disease. 14.7% of the patients did not present risk factors. Conclusions: Acute focus signals of CVA in children have no diagnostic value, unlike adults; however absence doesn't exclude focal ischemic vascular disease. The acute systemic disease was the most common risk factor for stroke.(Key words: Acute Brain Dysfunction, pediatrics, ischemic stroke, risk factors, venous sinus thrombosis).Rev Chil Pediatr 2013; 84 (4): 396-402RESUMENIntroduccion: La alteracion focal aguda de la funcion cerebral en el adulto permite diagnosticar un accidente vascular encefalico (AVE) e iniciar precozmente el rescate de tejido cerebral. En los ninos, las diferencias clinicas de esta patologia en relacion al adulto generan atraso, subdiagnostico e impiden el tratamiento en la etapa aguda. Objetivo: Describir la presentacion clinica y factores de riesgo del AVE isquemico en 156 ni-nos, comparar la presentacion clinica segun edad y tipo de AVE. Pacientes y Metodo: Se analiza un registro

Abducens palsy as a clue of unexpected contralateral cerebral sinovenous thrombosis

Abducens palsy as a clue of unexpected contralateral cerebral sinovenous thrombosis

Cerebral sinovenous thrombosis in a child with homocystinuria

Cerebral sinovenous thrombosis (CSVT) is a potentially fatal condition which can have multiple etiologies and varied presentation in children. Management of CSVT is incomplete without a search for possible etiology. Sebire et al (2005) found that the incidence of pediatric CVST is at least 0.67 per 100,000 children per year.1 We present an interesting case of a six-year old-boy with extensive CSVT who was diagnosed to be suffering from homocystinuria.

Long-Term Outcomes of Pediatric Ischemic Stroke in Adulthood

This population-based study assesses the long-term impact of childhood stroke on function and independence in young adults. We undertook a cross-sectional outcome study of patients with arterial ischemic stroke and cerebral sinovenous thrombosis, beyond 18 years of age. We studied 26 patients; 21 arterial stroke, 5 cerebral sinovenous thrombosis, with 16 females. Mean age at assessment was 21.5 years, and mean follow-up time was 10.8 years. According to the modified Rankin Scale, final outcomes were 37% normal, 42% mild, 8% moderate, and 15% severe deficits. Risk factors for abnormal functional outcome included arterial ischemic stroke, presence of arteriopathy, and 1-year poststroke Pediatric Stroke Outcome Measure score ≥ 2 (P < .05). Most (77-84%) were independent in driving, relationships, and employment. Functional status at 1 year poststroke strongly predicts long-term outcome. Mental illness in one-quarter of young adults surviving childhood stroke represents an important direction for research.

Neonatal thalamic hemorrhage is strongly associated with electrical status epilepticus in slow wave sleep

Thalamic hemorrhage has been associated with neonatal cerebral sinovenous thrombosis (CSVT), especially when the straight sinus is involved, and often presents with neonatal seizures. Early thalamic injury has previously been shown to predispose to epilepsy and electrical status epilepticus in slow wave sleep (ESES). The objective of this study was to assess the prevalence of sleep-induced epileptic electroencephalography (EEG) abnormalities and postneonatal epilepsy after neonatal thalamic hemorrhage associated with CSVT, in the absence of more widespread cerebral damage. Between 2003 and 2008 15 neonates were diagnosed with a thalamic hemorrhage due to suspected or proven CSVT. Neurodevelopment and the history of seizures were assessed at follow-up in the outpatient clinic in all 14 survivors (age 2-9years). Whole-night or sleep-deprived EEG recordings were obtained to assess the prevalence of interictal epileptiform activity (EA) and calculate a sleep-induced spike and wave index (SWI). Three children were diagnosed with classic ESES (SWI >85%). Two children had ESES spectrum disorder (SWI between 50% and 85%), and in two children significant sleep-induced epileptiform activity (SIEA) was noted (SWI between 25% and 50%). Two other children were diagnosed with focal epilepsy, in the absence of sleep-induced epileptiform EEG abnormalities. Five children (age 2-7years) had normal EEG recordings at follow-up. Deficits in neurodevelopment were seen significantly more often in children with ESES, ESES spectrum, or SIEA. Neonates with thalamic hemorrhage associated with straight sinus thrombosis, without evidence of more widespread cerebral damage, are at high risk of developing ESES (spectrum) disorder (35%), SIEA (14%), or focal epilepsy (14%). Electrographic abnormalities may already be present prior to recognition of cognitive deficits. Early diagnosis may guide parents and caregivers, and subsequent treatment may improve neurodevelopmental outcome. Routine annual sleep EEG recordings in children with neonatal thalamic injury following CSVT may improve recognition of ESES.