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 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.

Otogenic Lateral Sinovenous Thrombosis in Children: A Case Series from a Single Centre and Narrative Review

AimWe aimed to describe otogenic lateral sinovenous thrombosis (OLST), a rare, potentially life-threatening complication of otomastoiditis. MethodsChildren diagnosed with OLST in a tertiary-care Hospital from 2014 to 2019 was retrospectively selected. Clinical and radiological features, timing of diagnosis, treatment and outcome are reported. ResultsSeven children (5 males) were studied. Fever and neurological symptoms (headache, lethargy, diplopia, dizziness and papilledema) were always present. Otalgia and/or otorrhea were found in 6 children; none had signs of mastoiditis. Diagnosis was reached after 7 days (median) from clinical onset. Brain CT-scan was performed in 5 children being diagnostic for 3. Venography-MRI detected OLST and mastoiditis in all cases without parenchymal lesions. Treatment was based on intravenous rehydration, antibiotic and low-molecular weight heparin; acetazolamide was added in 3 children. Mastoidectomy and ventriculoperitoneal-shunting were selectively performed. Patients were discharged after 26 days (median). Follow-up neuroimaging showed sinus recanalization after a median time of 6 months. ConclusionA multidisciplinary approach is needed to optimize diagnostic-therapeutic protocols of pediatric OLST.

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.

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.

Management of Stroke in Infants and Children

The purpose of this statement is to review the literature on childhood stroke and to provide recommendations for optimal diagnosis and treatment. This statement is intended for physicians who are responsible for diagnosing and treating infants, children, and adolescents with cerebrovascular disease. The Writing Group members were appointed by the American Heart Association Stroke Council's Scientific Statement Oversight Committee. The panel included members with several different areas of expertise. Each of the panel's recommendations was weighted by applying the American Heart Association Stroke Council's Levels of Evidence grading algorithm. After being reviewed by panel members, the manuscript was reviewed by 4 expert peer reviewers and by members of the Stroke Council Leadership Committee and was approved by the American Heart Association Science Advisory and Coordinating Committee. We anticipate that this statement will need to be updated in 4 years. Evidence-based recommendations are provided for the prevention of ischemic stroke caused by sickle cell disease, moyamoya disease, cervicocephalic arterial dissection, and cardiogenic embolism. Recommendations on the evaluation and management of hemorrhagic stroke also are provided. Protocols for dosing of heparin and warfarin in children are suggested. Also included are recommendations on the evaluation and management of perinatal stroke and cerebral sinovenous thrombosis in children.

Cerebral sinovenous thrombosis in children

Cerebral sinovenous thrombosis (CSVT) is a rare but increasingly recognized disease process in children. In the pediatric population, the etiologies of CSVT are multiple and the outcomes are variable. The current therapies for CSVT include anticoagulation, thrombolysis, hydration, surgery, and supportive care. Adult studies, pediatric case studies, and expert opinion form the basis for these treatment strategies. International cooperative efforts are currently being undertaken to collect data on pediatric stroke patients, including CSVT. These multicenter collaborations are needed to increase our understanding of the incidence, risk factors, treatment modalities, and outcomes of CSVT in pediatric populations.

Cerebral Sinovenous Thrombosis in Children: Another Reason to Treat Iron Deficiency Anemia

Iron deficiency anemia is a rare cause of cerebral sinovenous thrombosis in children. We report three cases of cerebral sinovenous thrombosis and iron deficiency anemia treated at Primary Children's Medical Center in Salt Lake City, Utah, between 1998 and 2001. The children were 9, 19, and 27 months old at the time of admission. Hemoglobin levels ranged from 6.6 to 7.0 g/dL, mean corpuscular volume levels from 45 to 56 fL, and platelet counts from 248,000 to 586,000/microL. Magnetic resonance imaging and magnetic resonance venography revealed thrombosis of the straight sinus and internal cerebral veins in all three children, with the addition of the vein of Galen, left transverse and sigmoid sinuses, and upper left internal jugular vein in one child. Recovery ranged from excellent to poor in 3 months to 3 years of follow-up. Four additional cases, ages 6 to 22 months, were found in the English-language literature. Evaluation for prothrombotic disorders was negative in all children, including the current cases. Treatments have included thrombectomy, corticosteroids, mannitol, heparin, low-molecular-weight heparin, warfarin, aspirin, blood transfusion, and iron supplementation, but there is no consensus regarding therapy, other than to correct the anemia and treat iron deficiency. Iron deficiency anemia, a preventable cause of cerebral sinovenous thrombosis, deserves consideration when cerebral sinovenous thrombosis is detected in young children.

Cerebral sinovenous thrombosis in children.

Cerebral sinovenous thrombosis in children is a serious disorder, and information is needed about its prevention and treatment. The Canadian Pediatric Ischemic Stroke Registry was initiated in 1992 at the 16 pediatric tertiary care centers in Canada. Children (newborn to 18 years of age) with symptoms and radiographic confirmation of sinovenous thrombosis were included. During the first six years of the registry, 160 consecutive children with sinovenous thrombosis were enrolled, and the incidence of the disorder was 0.67 cases per 100,000 children per year. Neonates were most commonly affected. Fifty-eight percent of the children had seizures, 76 percent had diffuse neurologic signs, and 42 percent had focal neurologic signs. Risk factors included head and neck disorders (in 29 percent), acute systemic illnesses (in 54 percent), chronic systemic diseases (in 36 percent), and prothrombotic states (in 41 percent). Venous infarcts occurred in 41 percent of the children. Fifty-three percent of the children received antithrombotic agents. Neurologic deficits were present in 38 percent of the children, and 8 percent died; half the deaths were due to sinovenous thrombosis. Predictors of adverse neurologic outcomes were seizures at presentation and venous infarcts. Sinovenous thrombosis in children affects primarily neonates and results in neurologic impairment or death in approximately half the cases. The occurrence of venous infarcts or seizures portends a poor outcome.