United States

Intro Cerebral sinus vein thrombosis (CSVT) is a type of stroke consisting in thrombosis of the dural sinus and/or cerebral veins. The estimated incidence in children is 0.6 per 100,000 children per year however the symptoms and presentations are variable among all age ranges raising the concern for missed diagnosis. Despite new progress on pediatric stroke, CSVT is under-recognized and its risk factors are not well established. Hypothesis To describe risk factors of pediatric CSVT together with its signs and symptoms at presentation. Methods A single-center review of pediatric patients (&lt;18 years) with radiographically confirmed CSVT between 2008 and 2018, seen by the Hematology service at Cook Children&#39;s Medical Center were identified through the Cook Children&#39;s database. Results There were 87 patients identified. Symptoms at diagnosis were nausea/vomiting (33%), headache (32%), fever (29%) and neurologic deficits (25%). The most common neurologic deficits reported were altered mental status (9%) and increased intracranial pressure (9%) followed by focal weakness/hemiparesis (5%), cranial nerve palsy (5%), diplopia (4%), papilledema (4%) and ataxia (2%) (Table 1). Comorbidities included mastoiditis (26%), otitis media (24%), Lemierre&#39;s syndrome (10%), retropharyngeal abscess (5%) and severe anemia (hemoglobin &lt; 7 g/dl; 5%) (Table 2). Results of thrombophilia work up and sinuses involved are included in Figure 1 and Figure 2, respectively. Conclusions Infectious causes such as otitis media, mastoiditis and Lemierre&#39;s syndrome can be common contributors of pediatric CSVT. Anemia is a little known independent risk factor for thrombosis, especially CSVT, and presented in 5% of our patients. Not all patients with CVST underwent a complete thrombophilia evaluation, but thrombophilia was still rare finding. We suggest that if these infections are diagnosed, there should be a high index of suspicion for CSVT even in the absence of neurological deficits/symptoms.

IPSO 2021

Risk factors for pediatric cerebral sinus vein thrombosis

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Dear IPSO Virtual Conference Attendees:

Welcome to IPSO’s first-ever conference! We are delighted to bring together experts from <u>multiple disciplines</u> and <u>six continents</u> to provide a diverse perspective on pediatric cerebrovascular disease.  

A few quick words about what to expect.  Two important goals of this conference are to (1) foster a sense of community and (2) identify global priorities for our field. Hence, the program includes short presentations on an array of clinical controversies and dedicates more time to panel discussions with a live chat function so that everyone can participate. We are excited to announce IPSO is able to offer 8.75 CME units for the virtual conference. If you would like to claim CME credits, please email  <membership@internationalpediatricstroke.org>. 

We want to hear from you!  Planning for the [IPSO Founding Members’ Congress](https://internationalpediatricstroke.org/2022-congress/) in Vienna, April 24-26, 2022, will commence as soon as the Virtual Conference ends. Let us know your priorities through the live chat function, via email, or by submitting a symposium idea. Based on your input, the Scientific Program Committee will select the topics that we will address in greater depth when we meet again in the spring.

We hope you find the Virtual Conference both interesting and educational, and hope to see you in person in Vienna, or at another IPSO meeting in the future.  

Warm regards,

Heather Fullerton, IPSO President

Adam Kirton, IPSO Vice-President

Lori Jordan, IPSO Secretary & Treasurer


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*Header Photo Credit: Helen Carlson, Ph.D, Alberta Children's Hospital*

To access IPSO 2021, you must pre-register for the conference.  Instructions on how to access the event will be provided directly to attendees and included on the IPSO 2021 conference website. [Register now](https://internationalpediatricstroke.org/2021-congress/registration/).

Welcome to the first IPSO Virtual Conference. The mission of the virtual conference is to unify the global community of professionals engaged in research and clinical care for children with cerebrovascular diseases, and chart a path forward for multinational, multidisciplinary efforts.  Please go here to access the virtual conference platform- <a href="https://underline.io/events/157/reception">Reception page</a>. 



Intro Cerebral sinus vein thrombosis (CSVT) is a type of stroke consisting in thrombosis of the dural sinus and/or cerebral veins. The estimated incidence in children is 0.6 per 100,000 children per year however the symptoms and presentations are variable among all age ranges raising the concern for missed diagnosis. Despite new progress on pediatric stroke, CSVT is under-recognized and its risk factors are not well established. Hypothesis To describe risk factors of pediatric CSVT together with its signs and symptoms at presentation. Methods A single-center review of pediatric patients (<18 years) with radiographically confirmed CSVT between 2008 and 2018, seen by the Hematology service at Cook Children's Medical Center were identified through the Cook Children's database. Results There were 87 patients identified. Symptoms at diagnosis were nausea/vomiting (33%), headache (32%), fever (29%) and neurologic deficits (25%). The most common neurologic deficits reported were altered mental status (9%) and increased intracranial pressure (9%) followed by focal weakness/hemiparesis (5%), cranial nerve palsy (5%), diplopia (4%), papilledema (4%) and ataxia (2%) (Table 1). Comorbidities included mastoiditis (26%), otitis media (24%), Lemierre's syndrome (10%), retropharyngeal abscess (5%) and severe anemia (hemoglobin < 7 g/dl; 5%) (Table 2). Results of thrombophilia work up and sinuses involved are included in Figure 1 and Figure 2, respectively. Conclusions Infectious causes such as otitis media, mastoiditis and Lemierre's syndrome can be common contributors of pediatric CSVT. Anemia is a little known independent risk factor for thrombosis, especially CSVT, and presented in 5% of our patients. Not all patients with CVST underwent a complete thrombophilia evaluation, but thrombophilia was still rare finding. We suggest that if these infections are diagnosed, there should be a high index of suspicion for CSVT even in the absence of neurological deficits/symptoms.

poster

Introduction: Occupational therapy is one of the rehabilitative professions that treats children with hemiplegia secondary to perinatal and pediatric stroke (Rowland, Cooke, & Gustafsson, 2008). There is limited research on the assessment and rehabilitation of pediatric hemiplegia secondary to stroke (Marcroft, Tsutsumi, Pearse, Dulson, Embelton, & Basu, 2018). Aims: The goal is to demonstrate the effectiveness of combining pediatric constraint induced movement therapy (CIMT) with a collection of evidenced based rehabilitation interventions for the treatment of hemiplegia secondary to pediatric and perinatal stroke in occupational therapy. Hypothesis:The combined approach will yield increased quality of movement, more efficient motor patterns and spontaneous use of the affected side for participation in meaningful bilateral and unilateral tasks. Methods: This quasi-experimental, case series study examined children ages 7 to 13 years in a theme-based CIMT camp for 10 or 15 days for two consecutive or alternate years. Each of the six participants received multiple components of intervention combined with CIMT. Pre and post-test measures were taken to examine outcomes using the Abilhand-Kids and Goal Attainment Scaling to compare unilateral and bilateral function from the first round of camp were compared to the second round of intensive camp. Results: The analyses determined that the dosage of CIMT camp for pediatrics can be effective for 5 hours a day for 10 or 15 days especially for first time attendees. Additionally, there is significant sustained benefit to attending CIMT camp the following year or the alternate year. There is value in combining evidenced based practices with CIMT. These participants demonstrated an increase in mean difference for functional participation, range of motion and automaticity of use of the hemiparetic hand for meaningful activities after an intensive combined approach of CIMT, and collective evidence-based rehabilitation. Conclusion: Essential components of evidenced based rehabilitative interventions combined with CIMT can facilitate improvement of motor control for the pediatric stroke population. This work is the first to demonstrate an effective framework for the unilateral treatment of pediatric hemiplegia combining the use of essential multi-components. This report can be used as a guideline for pediatric stroke treatment in the field of occupational therapy for the purpose of knowledge translation.

Multi-component occupational therapy intervention in a pediatric constraint induced movement therapy camp setting

Introduction Childhood arterial ischemic stroke (AIS) is rare but associated with significant morbidity. Approximately 30% of new-borns and 50% of children diagnosed with AIS later develop hemiparesis. The relationship between their manual ability and participation at home, school and in the community has not been investigated. Patients and Methods Fourteen children with AIS (diagnosis at least 2 years ago, age 5 to 17 years) and 21 typically developing peers were examined. Participants were contacted by mail and asked to fill in questionnaires regarding their manual ability (ABILHAND-Kids) and participation (Participation and Environment Measure - Children and Youth [PEM-CY]). A Mann-Whitney-U test was used to calculate group differences, correlations were calculated using a Spearman Rank Correlation coefficient. Results There was a significant difference in manual ability between stroke patients and typically developing peers (p<0.001). While there were no significant group differences in participation frequency and involvement at home, school and in the community, parents of AIS patients desired significantly higher participation at school (p=0.022). There was a significant correlation between manual ability and the desired change in participation at school (p=0.026). Conclusion Although children after AIS seem to participate equally often and be equally well involved in participation at home, school and in the community, parents of children diagnosed with AIS appear to desire a higher level of participation at school - especially when manual ability is reduced. Future studies must investigate whether increased parental participation desires in this vulnerable patient group are associated with increased pressure to perform at school.

Participation and manual ability in children diagnosed with arterial ischemic stroke: Do parents of more affected children expect more from their children?

Neuropsychologists play an integral role in a comprehensive pediatric stroke team, and raising a perinatal stroke survivor provides unique insights when working with patients and their families. First, neuropsychologists can help parents understand their child's current and potential cognitive and behavioral outcomes as well as coping with uncertainty regarding their child's future. In addition, depending upon medical factors surrounding their child's stroke, parents may need help coping with their own psychological trauma and feelings of guilt. Parents may also benefit from guidance on how to manage changes in relationships with extended family and friends, and they should be prepared to model responses to questions from others regarding their child. In addition to developmental deficits, pediatric stroke survivors are at increased risk for behavioral dysregulation, and parents may need coaching in behavioral strategies or referrals to psychiatric treatment resources. As a child becomes more self-aware, parents can also benefit from support as they help their child understand his or her medical condition and grieve any newly appreciated losses that their child may experience. Because school is a child's job, parents may need to become their child's academic advocate, and they may be expected to educate school personnel about childhood stroke. In addition to a child's current challenges, parents should also help teachers understand that some stroke-related cognitive deficits may not become apparent until the child is older, and that his or her need for academic support may increase over time. Providing top-quality stroke care does not end with medical stabilization. Stroke survivors and their families should be treated from a lifespan perspective with an understanding of the far-reaching effects that a child's stroke can have upon the entire family system.

Neuropsychological treatment from the perspective of a parent of a perinatal stroke survivor

Background And Aims Existing research supports intensive therapy involving goal-directed training and practice of skills. Such programs are challenging to deliver in the ambulatory setting due to cost and logistics. We aimed to implement a Hand-Arm Bimanual Intensive Therapy (HABIT) program, measure and improve participant-reported outcomes, and expand program size while maintaining quality. Methods HABIT invovled a camp-like environment for 6 hours/day X 10 days, plus home programs. Fourteen campers (ages 6-11) completed the program in 3 groups. Caregiver education improvements were based on a pilot program (2017). Assisting Hand Assessment (AHA) hierarchy guided each camper's treatment (2018-2019). Program capacity increased from 4 to 6 campers. The role of psychology was was intensified yearly. Outcome assessments included AHA, Canadian Occupational Performance Measure (COPM), PROMIS Parent Proxy Upper Extremity Questionnaire, The Pediatric Outcomes Data Collection Instrument (PODCI), and participant surveys. Results: -Group AHA scores improved at program completion (P=.0006) and remained statistically significant at 30 days (P=.0007) despite a decreasing trend. Pattern consistent with literature. -8 of 13 achieved at least the smallest detectable difference (SDD), consistent with literature -Group exceeded criteria for clinically meaningful change -9 of 13 campers achieved clinically meaningful changes in performance and satisfaction -Improvements for 2018, sustained for 2019 Surveys show improved parent and patient-reported outcomes in 2018, sustained for 2019 Conclusions We implemented HABIT in our ambulatory care center with results comparable to the research literature. AHA score improvements were sustained at 30 days in an expanded program that was enhanced based on QI initiatives, with high satisfaction levels reported by participants.

Intensive bimanual therapy for pediatric chronic hemiparesis: implementing research and optimizing caregiver and patient-reported outcomes

Hemorrhage (HI) accounts for about half of all strokes in children. In total, vascular brain abnormalities accounted for 43.5% of all hemorrhages. Aim: to evaluate the incidence of HI in the primary pediatric stroke center of the Morozov Children City Clinical Hospital; revealed cerebral vascular anomalies without rupture, neurosurgical treatment options. Material: children who were treated in the primary pediatric stroke center from 2017 to 2019, with cerebrovascular diseases, and are included in the Moscow Pediatric Stroke Register. Results: 142 children were enrolled in the register in 2017: 62.2% of ischemic stroke and transient ischemic attacks (IS and TIA), 8.1% of sinus thrombosis (ST) and 29.7% of HI; in 2018, 282 children - 58% IS + TIA, 5.6% ST, 36.4% HI (7% cerebral vessel anomalies without rupture); in 2019, 235 children - 52.7% of IS and TIA, 12.8% of ST, 34.5% of HI (12.8% cerebral vessel anomalies without rupture). Cerebral angiography was performed in 90 patients, of which 45 (50%) patients did not have vascular pathology, 45 (50%) -pathology of cerebral vessels, of which: AVM-in 28 (62.2%) patients, arterial aneurysm - 6 (13.3%), venous angioma i- 4 (8.8%), arteriovenous fistula - 3 (6.6%) patients, ST - 2 (4.4%), Moya-Moya-2 (4.4%). Neurosurgical treatment of 14 patients (2017-2018): 11 (78.6%) with arteriovenous malformations, 2 (14.2%) with cavernomas, 1 (7.1%) aneurysm. Age ranged from 7 to 17 years. Ten of them (71.43%) were operated on: microsurgical -5 (50%), endovascular5 (50%). Conclusion: The neurosurgical service is an important part of the work of primary pediatric stroke centers. Improving diagnostic methods has improved the early detection of cerebral vascular anomalies with the optimal treatment tactics. According to the Moscow pediatric stroke Center, annually the number of first diagnosed cerebral vascular anomalies increases by 30-40%, with the predominance of AVM.

Neurosurgical service in the primary pediatric stroke center in Moscow

Introduction: Brainstem location of arteriovenous malformation occurs in 2-6% of all intracranial AVMs and more commonly presents as hemorrhage in adults with mean age of 25-38.5 years. This is the first local report of two cases presenting during the adolescent period with headaches. Case and Discussion The first case is a 13-year old male who had a one-year history of occipital headache eventually developing left hemiparesis, slurred speech and right-sided ataxia (mRS 3). Brain MRI revealed brainstem AVM extending to the right cerebellum (Figure 1) confirmed by angiography (Figure 2). After instituting medical supportive management, patient was sent home. Gradual improvement of the neurologic deficits (mRS 2) were noted three months after discharge. The second is an 18-year old female taking risperidone for Major Depressive Disorder and Post-Traumatic Stress Disorder who had recurrent episodes of migrainous-like headaches but consulted for galactorrhea. Cranial CT scan showed a midbrain AVM (Figure 3) confirmed by angiography (Figure 4). Endocrine work-up revealed risperidone-induced galactorrhea which resolved after drug withdrawal. Patient was discharged while waiting for definitive management. However, her AVM ruptured four months later manifesting as Weber syndrome (mRS 3). Patient was stabilized and eventually sent home. There is gradual improvement of the left hemiparesis (mRS 2) five months from the hemorrhagic stroke. Considering the poor prognosis of brainstem hemorrhage, these two cases illustrate the need for high-index of suspicion in seemingly benign headaches as early detection and management of this rare condition could spell the difference in their outcomes. Currently, treatment option for these patients is stereotactic radiosurgery. Keywords: pediatric hemorrhagic stroke, brainstem AVM.

Brainstem arteriovenous malformation in two adolescent Filipinos

Introduction: Management after hemorrhagic stroke from cerebral arteriovenous malformation (AVM) rupture remains controversial due to strategies to prevent hemorrhagic expansion while maintaining cerebral autoregulation (CA). We investigated associations of hemodynamics on outcomes after pediatric AVM rupture. Hypotheses: Measures of CA, baroreflex sensitivity (BRs) and heart rate variability (HRV) are associated with outcome at 12 months after pediatric cerebral AVM rupture. Methods: Children (<18 years old) with ruptured cerebral AVMs at a single critical care unit over 5 years were retrospectively analyzed. Continuous measurements of data including arterial blood pressure (ABP), intracranial pressure (ICP), cerebral perfusion pressure (CPP) and heart rate (HR) were evaluated. CA measures include pressure reactivity index (PRx), weighted pressure-reactivity index (wPRx), pulse-amplitude index (PAx), and correlation coefficient between ICP pulse amplitude (AMP) and CPP (RAC). Model-based indices of HRV, BRs, CA and estimations of optimal CPP (CPP-Opt) were computed along with percent of time below lower limits of autoregulation (LLA). Outcomes were determined using Pediatric Glasgow Outcome Score - Extended (GOSE-Peds) at 12 months. Patients with scores below 5 were grouped as "unfavorable outcomes". Association of biomarkers with outcome was determined with Wilcoxon singed rank test. Results: 14 children who underwent multimodal monitoring (6 female; mean [IQR] age, 10.57 [1.5]) were included. 8/14 children underwent intraparenchymal ICP monitoring from which CA was measured. Unfavorable outcomes were associated with lower median BRs values as compared to favorable outcomes (p=0.001) and lower HRV as compared to favorable outcome (p=0.026). Favorable outcome was consistent with decreased percent time below LLA based upon PAx and RAC, although this study was not powered for statistical significance (p=0.057). Conclusion: Decreased BRs and HRV were associated with unfavorable outcomes in children after hemorrhagic stroke from AVM rupture. Prospective studies are warranted to understand CA and its impact on outcomes.

Multimodal assessment of cerebral hemodynamics after pediatric hemorrhagic stroke secondary to cerebral arteriovenous malformation rupture

Introduction: Understanding non-traumatic pediatric intracerebral hemorrhage (pICH) etiological spectrum is key to diagnostic work-up and care pathway orientation. We aimed to evaluate the etiologic spectrum of diseases underlying pICH. Patients and Methods Children treated at our institution for a pICH were included in an inception cohort initiated in 2008, retrospectively inclusive to 2000 and analyzed in October 2019. We then conducted a systematic review of relevant articles published between 1990 and 2019, identifying cohorts with pICH patient. Identified populations, and patients from our cohort were pooled in multi category diagnosis meta-analysis. Results A total of 243 children with pICH were analyzed in the cohort study. The final primary diagnosis was an intracranial vascular lesion in 190 patients (78.2%), a complication of a cardiac disease in 17 (7.0%), a coagulation disorder in 14 (5.8%). Hematologic and cardiologic etiologies were disproportionately more frequent in children younger than 2 years old (p<0.001). The systematic review identified 1309 children in 23 relevant records, pooled in meta-analysis. Overall there was important heterogeneity. The dominant etiology were vascular lesions, with an aggregate prevalence of 0.59 [95% confidence interval: 0.45-0.64, p=0,001 Q:302.8, I²: 92%]. In eighteen studies reporting detailed etiological spectrum, arterio-venous malformation represented the dominant etiology (68.3% of all vascular causes, [95%CI 64.2 - 70.9%), followed by cavernoma (15.7% [95%CI 13.0 - 18.2%]). Conclusion The most frequent etiology of pICH are brain arteriovenous malformations. The probability of an underlying vascular etiology increases with age, and conversely hematologic and cardiac causes are dominant causes in children younger than 2 years old.

Etiology of intracerebral hemorrhage in children:cohort study, systematic review & meta-analysis

Introduction: The clinical outcome of pediatric intracerebral hemorrhage (pICH) is scantly reported in a comprehensive way. In this cohort study of patients with pICH, systematic review and meta-analysis, we aimed at describing the basic clinical outcomes after pICH. Methods Children treated at our institution for a pICH were included in an inception cohort initiated in 2008, retrospectively inclusive to 2000 and analyzed in October 2019. We then conducted a systematic review of relevant articles published since 1990, Identified populations, and patients from our cohort were pooled in multi category diagnosis meta-analysis, and analyzed with regards to clinical outcomes. Results Amongst 243 children screened for inclusion, 231 patients were included and analysed. Median age at ictus was 9.6 years [interquartile range: 4.6 -12.5], and 128 (53%) patients were male. After a median of 33 months of follow-up (interquartile range 13-63), 132 patients (57.4%) had a favourable clinical outcome, of which 58 (44%) had no residual symptom. Amongst 2622 records screened, we retained 24 studies with available data for the final analyses. Overall, the aggregate proportion of children with complete recovery was 26% [95% confidence interval: 16.4-34.2%, Q:170, I²: 89%], and of those with residual deficits 52.7% [95% confidence interval: 39.7-60.3%, Q:170, I²: 89%]. When pooled with our cohort, the aggregate case fatality at last follow up was 15.3% [95% confidence interval: 11.2-19.9%, Q:191, I²: 87%]. Conclusion Here we showed that mortality affects 1 in 6 children after pICH, and that the majority of children have residual neurological deficits at latest follow up. Complementary data from the cohort study are to be presented.

Mortality and functional outcome after pediatric intracerebral hemorrhage: a cohort study, systematic review and meta-analysis

Introduction: Hemorrhage expansion (HE) is a known predictor of clinical deterioration, and poor clinical outcome following admission after an intracerebral hemorrhage (ICH) in adults, but remains poorly reported in children. In a large inception cohort of children with ICH, we aimed to explore the prevalence of HE, its associations with clinical outcomes and its clinical-imaging predictors. Patients and Methods The report is based on an inception registry of children presenting with an ICH at a single quaternary university pediatric hospital (Necker Enfants Malades, Paris, France), retrospectively inclusive to January 2000. The study sample was restricted to children with two CT scans within 72 hours of symptoms onset available for re-asessement, and a clinical follow-up duration of more than 12 months. Hemorrhage expansion was defined as being clinically significant if the difference between follow-up and baseline ICH volumes exceeded 12.5cc or 33% of the baseline volume. Results Fifty two children met inclusion criteria, amongst which 8 (15%) demonstrated hemorrhage expansion. Children with hemorrhage expansion had more frequent coagulation disorders (25,0% vs 2,3%, p=0,022), focal deficits at presentation (75,0% vs 43,2%, p=0,08) and infra-tentorial ICH (25,0% vs 9,1%, p=0,01). After adjustment for variables associated with HE in univariable analyses at a p<0.1, only the presence of coagulation disorders at baseline remained independently associated with HE (adjusted OR 14,4 95%CI [1,04-217], p=0,048). HE was independently associated with poor (KOSCHI < 5A, adjusted OR 5,77 95%CI [1,01-38,95], p=0,043) as well as severe (KOSCHI < 4A, aOR 20,22 95%CI [3,06-133,68], p= 0,002) clinical outcomes Conclusion Hemorrhage expansion frequency is unexpectedly high after a pediatric ICH, affecting over 15% of children in our sample, and was found to be strongly associated with poorer clinical outcome, regardless of its definition.

Risk factors for pediatric cerebral sinus vein thrombosis

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