Sinovenous Thrombosis Research Articles

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.

Life After Perinatal Stroke

Perinatal strokes are a well-defined group of cerebrovascular diseases that damage the brain early in life and account for most cases of hemiparetic cerebral palsy. The motor deficits are now sufficiently researched to inform early prognostication, the construction of developmental plasticity models, and new clinical trials. Nonmotor outcomes are more complex and are challenging to measure given their emergence with time during developmental maturation. Several fundamental gaps in the understanding of perinatal stroke outcomes are of essential clinical relevance. How can 2 children with virtually identical brain lesions acquired at the same time have extremely different outcomes? How does a child develop new skills that his or her brain has never possessed during stroke recovery? How do we measure outcome as the child continues to grow into deficits with maturation? What are the modulators of developmental plasticity that might be targeted to achieve better functional outcomes? We review the current state of knowledge on perinatal stroke outcomes and attempt to highlight elements that might provide insight into these pressing questions. The most focused lifetime risk for stroke is the week surrounding birth.1 A term newborn carries a risk of ischemic stroke of ≥1:3500, triple the weekly stroke risk of a smoking adult with diabetes mellitus and hypertension.1,2 Adding in populations of neonatal hemorrhagic stroke, sinovenous thrombosis, and late presenting presumed perinatal strokes likely more than doubles the incidence of perinatal stroke.3,4 Outcomes are generally poor with disability often lasting an entire lifetime, resulting in a large global burden of disease. Perinatal stroke is the most common cause of hemiparetic cerebral palsy, and many survivors have additional neurological sequelae including intellectual disabilities, developmental and behavioral disorders, and epilepsy. Identification of a causative factor for perinatal stroke remains elusive in most cases1 with no …

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.

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.

Abstract 74: The Epidemiology of Childhood Stroke in Southern England: A Prospective Study using Multiple Sources of Case Ascertainment

Background: Epidemiological data are vital to quantify the scale of disease and can provide insights into aetiological factors. Objective: To describe the epidemiology of childhood stroke in southern England. Methods: Children (&gt;28 days to &lt;16 years) residing in southern England (5.99 million children) who had arterial ischemic stroke (AIS), cerebral sinovenous thrombosis with venous infarction (CSVT) and hemorrhagic stroke (HS) between July 2008 and June 2009 were identified. Multiple sources including pediatric neurologists, pediatricians, neurosurgeons, radiologists, physiotherapists, a national intensive care audit system, and parents notified cases. Results: 153 cases of stroke were identified (96 cases of AIS, 14 cases of CSVT and 43 cases of HS). The incidence for overall stroke was 2.56 per 100,000 person years (95% CI 2.15 - 2.96). The age distribution is shown in Figure 1. There was no difference between males and females in the risk of ischemic (AIS and CSVT) stroke (RR=1.06, p=0.75), HS (RR=1.46, p=0.22) or overall stroke (RR=1.16, p=0.36). Compared to Whites the relative risk of ischaemic stroke was higher for Asians (RR=2.35, p=0.001) and Blacks (RR=2.52, p=0.003). There was no increased risk of HS for Asians (RR=0.84, p=0.77) or Blacks (RR=1.39, p=0.58). Conclusions: The incidence of childhood stroke found by this study is higher than previously found in the UK. This is the first study to show disparities in the risk of childhood stroke between ethnic groups in Europe. Although an increased risk for males is commonly reported a number of recent population based studies have found no gender disparity.

Extensive Sinovenous Thrombosis and Hemorrhagic Infarction During Therapy for T-Cell Acute Lymphoblastic Leukemia

Sinovenous thrombosis (SVT) is a well-recognized and serious complication in children treated for acute leukemia. This frequently occurs during or immediately upon completion of induction therapy and is commonly attributed to asparaginase therapy.Headache is the first and most common clinical symptom to occur during the early development of SVT. With advancement of the thrombosis, the clinical symptoms can progress to increased sleepiness, focal neurological deficit, seizures, and altered consciousness. We report the case of a 4-year-old girl who presented after several days of headaches and anorexia, which then progressed to seizures, left-sided weakness, and altered consciousness. She was later found to have a widespread and occlusive SVT with right cerebral hemorrhagic infarction. This case is notable for the extensive nature of the cerebral SVT and the child's complete clinical recovery from the neurological event. The report discusses the relation of the thrombosis and leukemia and also emphasizes the importance of early recognition and prompt management, while incorporating a collaborative multidisciplinary approach to prevent long-term consequences.

Cerebral sinovenous thrombosis in pediatric practice

Cerebral sinovenous thrombosis (CSVT) in the pediatric population is a relatively uncommon yet under-appreciated and potentially life-threatening neurological condition. Early symptoms and signs are often vague and the clinician requesting a cranial imaging study might not even suspect sinovenous thrombosis. If left undiagnosed, or if the diagnosis of CSVT is delayed, progressive neurological deterioration, coma and death can follow. The purpose of this review is to highlight pertinent development of the cerebral venous system, discuss the causal factors of cerebral sinovenous thrombosis in the pediatric population, review practical imaging strategies using cranial sonography augmented with color and pulsed Doppler, unenhanced brain CT, CT venography, cerebral MRI, and MR venography (MRV). Finally, this review will illustrate the imaging features of sinovenous thrombosis, including a discussion of the common causes of false-positive and false-negative CT and MRI studies.

Cerebral Sinovenous Thrombosis Related to Iron Deficiency Anemia As a Result of Severe Menorrhagia: A Case Report

AbstractAbstract 5143Cerebral sinovenous thrombosis (CSVT) is a rare condition with a wide spectrum of non-specific symptoms, which makes it occasionally difficult to diagnose. CSVT has been associated with various etiologies including dehydration, hypercoagulable states, neoplasmic invasion of a venous sinus, intracranial and systemic infections, use of oral contraceptives, puerperium and pregnancy. Although iron deficiency anemia (IDA) has been reported as a cause of CSVT in several pediatric cases, this association is extremely rare. To our knowledge, IDA-associated CSVT in teenagers has been rarely reported.Here, we present the case of a 13-year old female with complete thrombosis of the vein of Galen, the straight sinus, and the left internal cerebral vein related to iron deficiency anemia as a result of severe menorrhagia. She initially presented with three-week history of headaches, nausea and vomiting. She was initially diagnosed and treated for bacterial sinusitis by her primary pediatrician, without symptom relief. She then suffered a syncopal episode, reason why she was evaluated at our institution. Initial laboratories revealed a slightly increased PT (15. 6 secs), hemoglobin of 5. 1 g/dL, MCV 63. 6 and D-dimer 1. 31. Non-contrast brain CT demonstrated no evidence of stroke, hemorrhage or mass. However, an abnormal hyperdensity in the straight sinus, inferior sagittal sinus, vein of Galen, and deep cerebral veins, were suggestive of venous thrombosis. Findings were then confirmed by brain MRI/MRV/MRA and CT angiogram. A bleeding work-up performed based on her history of heavy menses was normal. Her ferritin level was significantly decreased (6 ng/mL). Iron supplementation was initiated as well as progestin-only hormonal therapy for menstrual cycle regulation. The patient's clinical status improved back to baseline during her 8-day admission. She was discharged home on anticoagulation with scheduled follow up. Brain MRI/MRV six months later showed patency of the cerebral veins and dural venous sinuses.IDA should be considered as an underlying cause of CSVT in pediatric patients, particularly in patients with other risk factors for thrombosis including dehydration, regardless of their age. Early recognition and management of CSVT is critical to minimize the possibility of permanent neurologic damage. Physicians should be aware of the non-specific signs and symptoms of CSVT and should have a high index of suspicion in patients with anemia. Disclosures:No relevant conflicts of interest to declare.

Trombosis de senos venosos cerebrales en una niña con leucemia linfoblástica, portadora de la variante de la protrombina G20210A

Although cerebral venous thrombosis is rare, it is more commonly associated with children suffering from acute lymphoblastic leukaemia.We report the case of a 7-year-old girl who developed massive cerebral sinovenous thrombosis on day 22 of induction therapy for high-risk acute lymphoblastic leukaemia. Clinical symptoms were gradual onset of headache, decreasing consciousness, and ensuing left hemiplegia. A subsequent prothrombotic study revealed a heterozygous prothrombin G20210A variant in the child and mother. We analysed the prothrombotic factors found in the case before and after thrombosis.We confirm the importance of early exploration of patients for clinical predisposing risk factors of thrombosis and primary prothrombotic states in children with acute lymphoblastic leukaemia. This might help identify patients at particular risk from thrombosis and so administer thromboprophylaxis.

C0240 Safety and efficacy of anticoagulation in cerebral sinus thrombosis in children: A single center experience

Cerebral sinovenous thrombosis (CSVT) is a rare and serious disorder that involves high morbi-mortality and neurological sequelae. Prognosis can be improved with an early diagnosis and an optimal treatment. Although anticoagulation is recommended, the treatment of CVST in children and neonates remains controversial. Noclinical trials are available on the safety and efficacy of anticoagulation in children.