Search

Background Vein of Galen malformations (VOGMs) are rare and complex congenital arteriovenous fistulas. The clinical and radiological features of VOGMs and their relation to clinical outcomes are not fully characterized. Objective To examine the clinical and radiological features of VOGMs and the predictors of outcome in patients. Methods We retrospectively reviewed the available imaging and medical records of all patients with VOGMs treated at the University of California, San Francisco between 1986 and 2013. Radiological and clinical features were identified. We applied the modified Rankin Scale to determine functional outcome by chart review. Predictors of outcome were assessed by χ² analyses. Results Forty-one cases were confirmed as VOGM. Most patients (78%) had been diagnosed with VOGM in the first year of life. Age at treatment was bimodally distributed, with predominantly urgent embolization at <10 days of age and elective embolization after 1 year of age. Patients commonly presented with hydrocephalus (65.9%) and congestive heart failure (61.0%). Mixed-type (31.7%) VOGM was more common in our cohort than purely mural (29.3%) or choroidal (26.8%) types. The most common feeding arteries were the choroidal and posterior cerebral arteries. Transarterial embolization with coils was the most common technique used to treat VOGMs at our institution. Functional outcome was normal or only mildly disabled in 50% of the cases at last follow-up (median=3 years, range=0-23 years). Younger age at first diagnosis, congestive heart failure, and seizures were predictive of adverse clinical outcome. The survival rate in our sample was 78.0% and complete thrombosis of the VOGM was achieved in 62.5% of patients. Conclusions VOGMs continue to be challenging to treat and manage. Nonetheless, endovascular approaches to treatment are continuing to be refined and improved, with increasing success. The neurodevelopmental outcomes of affected children whose VOGMs are treated may be good in many cases. [ABSTRACT FROM AUTHOR]

Background and Purpose: A better understanding of the stroke risk factors in children with congenital heart disease (CHD) could inform stroke prevention strategies. We analyzed pediatric stroke associated with CHD in a large community-based case-control study., Methods: From 2.5 million children (aged <20 years) enrolled in a Northern California integrated healthcare plan, we identified children with ischemic and hemorrhagic strokes and randomly selected age- and facility-matched stroke-free controls (3 per case). We determined exposure to CHD (diagnosed before stroke) and used conditional logistic regression to analyze stroke risk factors., Results: CHD was identified in 15 of 412 cases (4%) versus 7 of 1236 controls (0.6%). Cases of childhood stroke (occurring between ages 29 days to 20 years) with CHD had 19-fold (odds ratio, 19; 95% confidence interval 4.2-83) increased stroke risk compared to controls. History of CHD surgery was associated with >30-fold (odds ratio, 31; confidence interval 4-241) increased risk of stroke in children with CHD when compared with controls. After excluding perioperative strokes, the history of CHD surgery still increased the childhood stroke risk (odds ratio, 13; confidence interval 1.5-114). The majority of children with stroke and CHD were outpatients at the time of stroke, and almost half the cases who underwent cardiac surgery had their stroke >5 years after the most recent procedure. An estimated 7% of ischemic and 2% of hemorrhagic childhood strokes in the population were attributable to CHD., Conclusions: CHD is an important childhood stroke risk factor. Children who undergo CHD surgery remain at elevated risk outside the perioperative period and would benefit from optimized long-term stroke prevention strategies., (© 2014 American Heart Association, Inc.)

Objective: In a population-based case-control study, we examined whether the timing and number of minor infections increased risk of childhood arterial ischemic stroke (AIS)., Methods: Among 102 children with AIS and 306 age-matched controls identified from a cohort of 2.5 million children in a large integrated health care plan (1993-2007), we abstracted data on all medical visits for minor infection within the 2 years prior to AIS or index date for pairwise age-matched controls. We excluded cases of AIS with severe infection (e.g., sepsis, meningitis). Using conditional logistic regression, we examined the effect of timing and total number of minor infections on stroke risk., Results: After adjusting for known pediatric stroke risk factors, the strongest association between infection and AIS was observed for infectious visits ≤3 days prior to stroke (odds ratio [OR] 12.1, 95% confidence interval [CI] 2.5, 57, p = 0.002). Respiratory infections represented 80% of case infections in that time period. Cases had more infectious visits, but not significantly so, for all time periods ≥4 days prior to the stroke. A greater cumulative number of infectious visits over 2 years did not increase risk of AIS., Conclusions: Minor infections appear to have a strong but short-lived effect on pediatric stroke risk, while cumulative burden of infection had no effect. Proposed mechanisms for the link between minor infection and stroke in adults include an inflammatory-mediated prothrombotic state and chronic endothelial injury. The transient effect of infection in children may suggest a greater role for a prothrombotic mechanism., (© 2014 American Academy of Neurology.)

Objective: To determine incidence rates and predictors of epilepsy after childhood stroke and compare these to published estimates of 3 to 5% cumulative epilepsy incidence by 5 years poststroke in adults., Methods: In a retrospective population-based study of children with stroke (29 days-19 years) in an integrated health care system (1993-2007), poststroke seizures were identified through electronic searches and confirmed by chart review. Stroke and seizure characteristics were abstracted from medical records. Survival analysis was used to determine rates and predictors of remote seizures and active epilepsy (anticonvulsant treatment for remote seizure within prior 6 months) at last follow-up., Results: From a population of 2.5 million children, we identified 305 stroke cases. Over a median follow-up of 4.1 years (interquartile range = 1.8-6.8), 49 children had a first unprovoked remote seizure. The average annual incidence rate of first remote seizure was 4.4% (95% confidence interval [CI] = 3.3-5.8) with a cumulative risk of 16% (95% CI = 12-21) at 5 years and 33% (95% CI = 23-46) at 10 years poststroke. The cumulative risk of active epilepsy was 13% (95% CI = 9-18) at 5 years and 30% (95% CI = 20-44) at 10 years. Acute seizures at the time of stroke predicted development of active epilepsy (hazard ratio = 4.2, 95% CI = 2.2-8.1). At last follow-up, ⅓ of the children with active epilepsy had a recent breakthrough seizure despite anticonvulsant usage., Interpretation: Unlike adults, children are uniquely vulnerable to epilepsy after stroke. Children with acute seizures at the time of stroke are at particularly high risk., (© 2013 American Neurological Association.)

Objectives: Predictors for perinatal arterial ischemic stroke include both maternal and intrapartum factors, but predictors of perinatal hemorrhagic stroke have not been studied. We sought to determine both the prevalence and predictors of perinatal hemorrhagic stroke within a large, multiethnic population., Patients and Methods: We performed a case-control study nested within the cohort of all infants born from 1993 to 2003 in the Northern California Kaiser Permanente Medical Care Program, a health maintenance organization providing care for >3 million members. Cases of symptomatic perinatal hemorrhagic stroke and perinatal arterial ischemic stroke in neonates (28 weeks' gestational age through 28 days of life) were identified through electronic searches of diagnosis and radiology databases and confirmed by medical chart review. Three controls per case were randomly selected and matched on birth year and facility. This analysis included cases of perinatal hemorrhagic stroke (intracerebral hemorrhage or subarachnoid hemorrhage, excluding pure intraventricular hemorrhage) and all controls. Predictors of perinatal hemorrhagic stroke were assessed by using logistic regression, adjusting for the matching criteria., Results: Among 323 532 live births, we identified 20 cases of perinatal hemorrhagic stroke (19 intracerebral hemorrhage and 1 subarachnoid hemorrhage), which yielded a population prevalence for perinatal hemorrhagic stroke of 6.2 in 100 000 live births. Cases presented with encephalopathy (100%) and seizures (65%). Perinatal hemorrhagic stroke was typically unifocal (74%) and unilateral (83%). Etiologies included thrombocytopenia (n = 4) and cavernous malformation (n = 1); 15 (75%) were idiopathic. Univariate predictors of perinatal hemorrhagic stroke included male gender, fetal distress, emergent cesarean delivery, prematurity, and postmaturity but not birth weight. When entered into a multivariate model, fetal distress and postmaturity continued to be independent predictors., Conclusions: Fetal distress is an independent predictor of perinatal hemorrhagic stroke, perhaps suggesting a prenatal event. Postmaturity also predicts perinatal hemorrhagic stroke, an association not explained by large birth weight in our study.

Background and Purpose: Prior population-based studies of pediatric hemorrhagic stroke (HS) had too few incident cases to assess predictors of cerebral aneurysms, a HS etiology that requires urgent intervention., Methods: We performed a retrospective cohort study of HS (intracerebral, subarachnoid [SAH], and intraventricular hemorrhage) using the population of all children <20 years of age enrolled in a large Northern Californian healthcare plan (January 1993 to December 2003). Cases were identified through electronic searches and confirmed through independent chart review by 2 neurologists with adjudication by a third; traumatic hemorrhages were excluded. Logistic regression was used to examine potential predictors of underlying aneurysm., Results: Within a cohort of 2.3 million children followed for a mean of 3.5 years, we identified 116 cases of spontaneous HS (overall incidence, 1.4 per 100000 person-years). Cerebral aneurysms were identified in 15 (13%) of HS cases. Among 21 children with pure SAH, 57% were found to have an underlying aneurysm compared with only 2% of 58 children with pure intracerebral hemorrhage and 5% of 37 children with a mixed pattern of hemorrhage (intracerebral hemorrhage and SAH). Independent predictors of an underlying aneurysm included pure SAH (OR, 76; 95% CI, 9 to 657; P<0.001) and late adolescent age (15 to 19 years versus younger age groups; OR, 6.4; 95% CI, 1.0 to 40; P=0.047)., Conclusions: Cerebral aneurysms cause the majority of spontaneous SAH in children and account for more than 10% of childhood HS overall. Children, and particularly teenagers, presenting with spontaneous SAH should be promptly evaluated with cerebrovascular imaging.

Objective: Few data exist regarding rates and predictors of recurrence after childhood arterial ischemic stroke. We sought to establish such rates within a large, multiethnic population and determine whether clinical vascular imaging predicts recurrence., Patients and Methods: In a population-based cohort study, we collected data on all documented cases of arterial ischemic stroke among 2.3 million children (<20 years old) enrolled in a northern Californian managed care plan from January 1993 to December 2004. Perinatal strokes were those that occurred by 28 days of life. Data on cerebrovascular imaging (conventional or magnetic resonance angiography), including presence of vascular abnormalities, were abstracted from official radiology reports. We used Kaplan-Meier survival-analysis techniques to determine rates and predictors of recurrent stroke., Results: Among 181 incident childhood stroke cases (84 perinatal; 97 later childhood), there were 16 recurrent strokes (1 after a perinatal stroke) at a median of 2.7 months. The 5-year cumulative recurrence rates were 1.2% after perinatal stroke and 19% after later childhood stroke. Of the 97 children with later childhood strokes, 52 received cerebrovascular imaging, predominantly magnetic resonance angiography (n = 36) and conventional angiography (n = 26). Although there were no recurrences among children with normal vascular imaging, children with a vascular abnormality had a 5-year cumulative recurrence rate of 66%., Conclusions: Strokes recur in one fifth of cases of later childhood arterial ischemic stroke but are rare after perinatal stroke. Among the later childhood cases, cerebrovascular imaging identifies those at highest risk for recurrence.

Objective: Birth asphyxia is recognized as an important cause of neonatal morbidity and mortality. Whether advances in perinatal care have altered the incidence of birth asphyxia is unknown. We determined the incidence of birth asphyxia diagnoses made over a 10-year period in California., Methods: In a population-based retrospective cohort study of 5,364,663 live births, we determined the incidence and case fatality of birth asphyxia between 1991 and 2000. Using a statewide administrative hospital discharge database, we identified all newborn admissions that generated a diagnosis of birth asphyxia (International Classification of Diseases, Ninth Revision, Clinical Modification codes 768.5, 768.6, or 768.9) or a diagnosis that overlaps with birth asphyxia, such as congenital encephalopathy or fetal distress. We determined incidence and in-hospital case fatality rates adjusted for birth weight and demographic characteristics and stratified by associated perinatal complications., Results: The 24 330 newborns who received a diagnosis of birth asphyxia yielded a population incidence of 4.5 per 1000 live births. Black ethnicity (relative risk [RR]: 1.3; 95% confidence interval [CI]: 1.2-1.3), male gender (RR: 1.2; 95% CI: 1.1-1.2), and low socioeconomic status (RR: 1.2; 95% CI: 1.1-1.2) all were associated with increased risk. The diagnosis of birth asphyxia decreased by 91% from 14.8 to 1.3 per 1000 live births during the study years. This decrease could not be explained by an increased diagnosis of overlapping conditions. Overall case fatality was 4%, and the majority of deaths in infants >2000 g occurred in the presence of congenital anomalies, cord abnormalities, or maternal hemorrhage. In newborns <2000 g, case fatality was highest in the presence of chorioamnionitis (48%)., Conclusion: The diagnosis of birth asphyxia has decreased dramatically in recent years. The factors that are responsible for this decline are unclear and deserve additional investigation.

Although the Stroke Prevention Trial in Sickle Cell Anemia (STOP) demonstrated the efficacy of blood transfusions for primary stroke prevention in high-risk children with sickle cell disease (SCD) in 1998, the impact of this trial on public health has not been studied. Our objective was to determine whether stroke rates in Californian children with SCD have declined since 1998. Using a California-wide hospital discharge database, we identified all first admissions for stroke in children with SCD from 1991 through 2000. Annual stroke incidence rates were calculated as the number of admissions divided by the estimated population of Californian children with SCD in that year. For 1991-2000, 93 children with SCD were admitted to Californian hospitals with a first stroke during 12 030 person-years of follow-up; 92.5% were ischemic and 7.5% hemorrhagic. Overall, the rate of first stroke was 0.77/100 person-years. For the study years 1991-1998, the rate for first stroke was 0.88/100 person-years compared to 0.50 in 1999 and 0.17 in 2000 (P <.005 for trend). Since the publication of the STOP study in 1998, annual rates of admissions for first stroke for Californian children with SCD have declined.

Methods: Using a California-wide hospital discharge database, the authors analyzed all first admissions for stroke in children 1 month through 19 years of age from 1991 through 2000. Incidence rates were estimated as the number of first hospitalizations divided by the person-years at risk; case fatality rates were based on in-hospital deaths., Results: The authors identified 2,278 first admissions for childhood stroke, yielding an annual incidence rate of 2.3 per 100,000 children (1.2 for ischemic stroke, 1.1 for hemorrhagic stroke). Compared with whites, black children were at higher risk of stroke (for ischemic stroke, relative risk [RR] 2.59, 95% CI 2.17 to 3.09, p < 0.0001; subarachnoid hemorrhage [SAH], RR 1.59, CI 1.06 to 2.33, p = 0.02; intracerebral hemorrhage [ICH], RR 1.66, CI 1.23 to 2.13, p = 0.0001). Hispanics, however, had a lower risk of ischemic stroke (RR 0.70, CI 0.60 to 0.82, p < 0.0001) and ICH (RR 0.77, CI 0.64 to 0.93, p = 0.0004), whereas Asians had similar risks as whites. Boys were at higher risk for all stroke types than girls (ischemic stroke, RR 1.25, CI 1.11 to 1.40, p = 0.0002; SAH, RR 1.24, CI 1.00 to 1.53, p = 0.047; ICH, RR 1.34, CI 1.16 to 1.56, p = 0.0001). After eliminating cases with coexisting sickle cell disease, excess stroke risk persisted in blacks; after elimination of trauma, excess stroke risk persisted in boys. Case fatality rates were similar among different ethnic groups. Compared with girls, boys had a higher case fatality rate for ischemic stroke (17 vs 12%; p = 0.002) but not for ICH or SAH., Conclusions: Rates of hospitalization for stroke are higher among black children and boys; sickle cell disease and trauma do not fully account for these findings.