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Modern hyperacute reperfusion therapies including intravenous thrombolysis and mechanical thrombectomy have transformed the management of arterial ischemic stroke (AIS) in adults. Multiple randomized clinical trials have demonstrated that these therapies enable remarkable improvements in clinical outcome for properly selected patients with AIS. Because pediatric patients were excluded from predicate clinical trials, there is a conspicuous lack of data to guide selection of therapies and inform age-adjusted and pathology-oriented treatment modifications for children. Specifically, technical guidance concerning treatment eligibility, drug dosing, and device implementation is lacking. This review aims to outline important features that differentiate pediatric AIS from adult AIS and provide practical strategies that will assist the stroke specialist with therapeutic decision making.

In children with arterial ischemic stroke (AIS), the definitive diagnosis of stroke subtype and confirmation of stroke etiology is necessary to mitigate stroke morbidity and prevent recurrent stroke. The common causes of AIS in children are sharply differentiated from the common causes of adult AIS. A comprehensive, structured diagnostic approach will identify the etiology of stroke in most children. Adequate diagnostic evaluation relies on advanced brain imaging and vascular imaging studies. A variety of medical and surgical secondary stroke prevention strategies directed at the underlying cause of stroke are available. This review aims to outline strategies for definitive diagnosis and secondary stroke prevention in children with AIS, emphasizing the critical role of neuroimaging.

OBJECTIVE Torcular dural sinus malformations (tDSMs) are rare pediatric cerebrovascular malformations characterized by giant venous lakes localized to the midline confluence of sinuses. Historical clinical outcomes of patients with these lesions were poor, though better prognoses have been reported in the more recent literature. Long-term outcomes in children with tDSMs are uncertain and require further characterization. The goal of this study was to review a cohort of tDSM patients with an emphasis on long-term outcomes and to describe the treatment strategy. METHODS This study is a single-center retrospective review of a prospectively maintained data bank including patients referred to and cared for at The Hospital for Sick Children for tDSM from January 1996 to March 2019. Each patient’s clinical, radiological, and demographic information, as well as their mother’s demographic information, was collected for review. RESULTS Ten patients with tDSM, with a mean follow-up of 58 months, were included in the study. Diagnoses were made antenatally in 8 patients, and among those cases, 4 families opted for either elective termination (n = 1) or no further care following delivery (n = 3). Of the 6 patients treated, 5 had a favorable long-term neurological outcome, and follow-up imaging demonstrated a decrease or stability in the size of the tDSM over time. Staged embolization was performed in 3 patients, and anticoagulation was utilized in 5 treated patients. CONCLUSIONS The authors add to a growing body of literature indicating that clinical outcomes in tDSM may not be as poor as initially perceived. Greater awareness of the lesion’s natural history and pathophysiology, advancing endovascular techniques, and individualized anticoagulation regimens may lead to continued improvement in outcomes.

Hemorrhage from brain arteriovenous malformations (bAVMs) is estimated at 3% per annum. Features influencing risk of hemorrhage include perforator/posterior circulation supply, associated aneurysms, and deep drainage. Children are more likely to present with bAVM bleeds.To analyze differences in bAVM angioarchitecture between children and adults and describe predictors of poor outcome.Data were collected from adult and pediatric tertiary referral hospitals. Demographic data, bleed location, treatment, and follow-up modified Rankin Scale (mRS) were collected. Angioarchitectural assessment included aneurysm presence, nidus morphology, perinidal angiogenesis, intranidal shunting, steal phenomenon, venous ectasia, venous stenosis, venous reflux, and pseudophlebitic pattern. Regression analyses conducted to determine predictors of mRS 2.A total of 270 adult and 135 pediatric ruptured bAVMs were assessed. Median age was 42 (adults) and 10.9 (children) yr. Intranidal aneurysms were more frequent in children (P = .012), whereas prenidal aneurysms were more common in adults (P .01). Children demonstrated more perinidal angiogenesis (P = .04), whereas steal phenomenon was commoner in adults (P .01). Venous ectasia (P .01), reflux (P .01), and pseudophlebitic pattern (P = .012) were more frequent in adults. Children had better outcome (mRS score ≤ 2) (P .01). Older age (odds ratio [OR] = 1.02), eloquent location (OR = 2.5), multicompartmental hemorrhage (OR = 1.98), venous reflux (OR = 2.5), diffuse nidus (OR = 1.83), pseudophlebitic pattern (OR = 1.96), intranidal shunts (OR = 2), and no treatment (OR = 3.68) were significant predictors of mRS 2.Children are more likely to have intranidal aneurysms and perinidal angiogenesis, whereas adults have more prenidal aneurysms, venous ectasia, corticovenous reflux, and pseudophlebitic pattern. Eloquent location, diffuse nidus, intranidal shunts, venous reflux, and pseudophlebitic pattern predict poorer outcome.

Background Given the expanding evidence of clinico-radiological differences between moyamoya disease (MMD) and moyamoya syndrome (MMS), we compared the clinical and radiographic features of childhood MMD and MMS to identify predictors of ischemic event recurrence. Methods We reviewed a pediatric moyamoya cohort followed between 2003 and 2019. Clinical and radiographic characteristics at diagnosis and follow-up were abstracted. Comparisons between MMD and MMS as well as between MMD and two MMS subgroups (neurofibromatosis [MMS-NF1] and sickle cell disease [MMS-SCD]) were performed. Results A total of 111 patients were identified. Patients with MMD presented commonly with transient ischemic attacks (TIAs) (35 % MMD versus 13% MMS-NF1 versus 9.5% MMS-SCD; P = 0.047). Symptomatic stroke presentation (MMD 37% versus MMS-NF1 4% versus 33%; P = 0.0147) and bilateral disease at diagnosis (MMD 73% versus MMS-NF1 22 % versus MMS-SCD 67%; P = 0.0002) were uncommon in MMS-NF1. TIA recurrence was common in MMD (hazard ratio 2.86; P = 0.001). The ivy sign was absent on neuroimaging in a majority of patients with MMS-SCD (MMD 67% versus MMS-NF1 52% versus MMS-SCD 9.5%; P = 0.0002). Predictors of poor motor outcome included early age at diagnosis (odds ratio [OR] 8.45; P = 0.0014), symptomatic stroke presentation (OR 6.6; P = 0.019), and advanced Suzuki stage (OR 3.59; P = 0.019). Conclusions Moyamoya exhibits different phenotypes based on underlying etiologies. Frequent TIAs is a common phenotype of MMD and symptomatic stroke presentation a common feature of MMD and MMS-SCD, whereas unilateral disease and low infarct burden are common in MMS-NF1. In addition, absence of ivy sign is a common phenotype in MMS-SCD.

Thromboembolic events post left ventricular assist devices (LVAD) implantation remain a major cause of morbidity and mortality. Mechanical thrombectomy for the treatment of pediatric intracranial thromboembolic events have been reported in LVADs, but never following HeartMate 3 (HM3) implantation. We present the case of an 8-year-old, 26.5 kg male with dilated cardiomyopathy and decompensated heart failure who presented with extensive intracranial thromboembolism in the early postoperative period following HM3 implantation and underwent successful mechanical thrombectomy with a favorable neurological outcome.

IMPORTANCE: The incidence and natural history of large vessel occlusion (LVO) stroke in children is largely unknown. These knowledge gaps limit the uptake of reperfusion therapies and reduce the efficiency of pediatric acute stroke pathways. OBJECTIVE: To determine the incidence and natural history of pediatric LVO stroke. DESIGN, SETTING, AND PARTICIPANTS: This retrospective population-based cohort study was conducted between January 2010 and December 2019, with a mean (SD) follow-up of 37.0 (28.8) months. Admissions from all pediatric hospitals in the state of New South Wales, Australia, with a final diagnosis of arterial ischemic stroke (AIS) in patients 1 month to younger than 17 years were included. A total of 85 of 251 identified cases were excluded based on selection criteria. Data were analyzed from July 2020 to June 2021. EXPOSURES: One-third of patients with LVO received mechanical thrombectomy with or without intravenous thrombolysis while the remainder were treated conservatively. MAIN OUTCOMES AND MEASURES: The primary outcome was the pediatric modified Rankin Scale (ped-mRS) score 3 months after stroke. Ordinal logistic regression was used to compare non-LVO, LVO without thrombectomy, and LVO with thrombectomy groups. RESULTS: Of 161 included patients, 56 (34.8%) were female, and the mean (SD) age was 6.1 (5.4) years. A total of 166 AIS admissions were studied, and clinical follow-up was available for 164 of 166 admissions. LVO was present in 39 admissions (23.5%). The incidence of LVO stroke was 0.24 per 100 000 patients per year (95% CI, 0.13-0.35). Patients with LVO who did not receive thrombectomy (n = 26) had poor neurological outcomes, with 19 (73.1%) experiencing moderate to severe disability or death (ped-mRS score of 3 to 6) at 3 months (6 of 12 patients receiving thrombectomy [50.0%]; 25 of 38 patients with LVO [65.8%]). Patients with LVO without thrombectomy had significantly worse clinical outcomes than patients with non-LVO at 3 months (odds ratio, 3.64; 95% CI, 1.68-7.87; P = .001). Most patients with LVO presented within time windows suitable for thrombectomy (27 of 39 [69.2%] within 6 hours; 35 of 39 [89.7%] within 24 hours). CONCLUSIONS AND RELEVANCE: In this population-based cohort study, the natural history of pediatric patients with LVO stroke treated conservatively was poor, with most experiencing lifelong disability or death. Nearly 90% of pediatric patients with LVO presented within time windows suitable for thrombectomy.

Numerous factors make the initial diagnostic evaluation of children with suspected arterial ischemic stroke (AIS) a relatively unsettling challenge, even for the experienced stroke specialist. The low frequency of pediatric AIS, diversity of unique age-oriented stroke phenotypes, and unconventional approaches required for diagnosis and treatment all contribute difficulty to the process. This review aims to outline important features that differentiate pediatric AIS from adult AIS and provide practical strategies that will assist the stroke specialist with diagnostic decision making in the initial phase of care.

The interpretation of cerebral venous pathologies in paediatric practice is challenging as there are several normal anatomical variants, and the pathologies are diverse, involving the venous system through direct and indirect mechanisms. This paper aims to provide a comprehensive review of these entities, as their awareness can avoid potential diagnostic pitfalls. We also propose a practical classification system of paediatric cerebral venous pathologies, which will enable more accurate reporting of the neuroimaging findings, as relevant to the underlying pathogenesis of these conditions. The proposed classification system comprises of the following main groups: arterio-venous shunting-related disorders, primary venous malformations and veno-occlusive disorders. A multimodal imaging approach has been included in the relevant subsections, with a brief overview of the modality-specific pitfalls that can also limit interpretation of the neuroimaging. The article also summarises the current literature and international practices in terms of management options and outcomes in specific disease entities.

Children with medical complexity and associated neurologic impairment frequently face difficulties with venous access. Intermittently they require urgent intravenous administration of fluids and medication. To analyze the use of implanted port-a-caths in children with medical complexity who have neurologic impairment and difficult venous access. We performed a single-center observational study of port-a-caths placed by interventional radiologists in children with medical complexity with neurologic impairment. We analyzed peripheral intravenous access attempts, peripheral intravenous starts, peripheral intravenous complications, alternative temporary central venous access devices, port-a-cath insertions, catheter days, access days, port-a-cath-related complications, hospital admissions and emergency department visits. We compared the year pre port-a-cath to the year post port-a-cath. Twenty-one children with medical complexity with neurologic impairment (10 boys, 11 girls; median age 4.1 years; median weight 13.7 kg) underwent 26 port-a-cath insertions (median catheter days 787). In the year post port-a-cath compared to pre port-a-cath there was a highly significant reduction (P

BACKGROUND AND PURPOSE: The feasibility and safety of transradial angiography is not established outside the adult literature. The objective of this study was to assess the feasibility and safety of transradial access for neuroangiography in adolescents. MATERIALS AND METHODS: A retrospective case-control study was performed, comparing transradial neuroendovascular procedures in adolescents (age range, 10–18 years) with an age- and procedure-matched cohort of transfemoral neuroendovascular procedures. Clinical and procedural details, including type of procedure, conversion rate, fluoroscopy time, radiation dose, complications, and readmissions, were reported by descriptive statistics or measures of central tendency and compared using a t test or nonparametric equivalent. A P value < .05 was considered statistically significant. RESULTS: Twenty adolescents (mean age, 14.6 [SD, 1.7] years, M/F ratio = 9:11) who underwent transradial neuroangiography were compared against 20 adolescents (mean age, 14.4 [SD, 2.1 ] years, M/F ratio = 12:8) who underwent transfemoral neuroangiography. We found no significant difference in procedural success (0% conversion rate), fluoroscopy times (33.7 [SD, 40.2] minutes versus 23.3 [SD, 26.2] minutes, P = .34) and radiation dose (150.9 [SD, 133.7] Gy×cm(2) and 122.9 [SD, 79.7] Gy×cm,(2) P = .43) There were 2 self-limiting postprocedural complications in the transradial group. There were no major hemorrhages, need for further interventions, or readmissions in either group. CONCLUSIONS: The benefits of transradial angiography described for adults can likely be safely extended to adolescents. These are important data before transitioning to smaller children and should be prospectively evaluated in a larger cohort.

Pediatric neuroendovascular procedures require special considerations. Given small vessel sizes, risk for arterial injury must be weighed against use of larger devices, with the diameter of the sheath being a known association with arterial complications. We recently transitioned to using thin-walled radial sheaths for transfemoral angiography in children, given their lower profile. Here, we report on these sheaths’ technical success and complications, comparing against a historical cohort where regular vascular sheaths were employed. We retrospectively recorded patient and procedural data from 168 consecutive procedures from September 2017 to January 2019 when radial-specific sheaths were exclusively used at our tertiary pediatric hospital. These results were compared to data from September 2015 to January 2017, when regular vascular sheaths were exclusively used in 152 consecutive procedures. Statistical analysis was performed using unpaired t test or chi-square test, with p < 0.05 considered statistically significant. Patient characteristics (age, sex, weight) were not statistically different between the case and control group. No significant differences were found in the procedural data with the exception of heparin use which was higher in the radial-sheath cohort. There was a decrease in the rate of complications in the case group (1.2%) versus control group (2.6%), though not statistically different. Radial-specific sheaths have numerous beneficial characteristics that make them well-suited to the pediatric population. In our study, we show that radial sheaths are equally effective and safe perioperatively. Follow-up research may show if radial sheaths used transfemorally decrease long-term complications such as limb-length discrepancy and mortality.

Background: Transradial intervention is increasingly replacing approaches, due to lower access complications, cost, and improved patient satisfaction. There are limited supporting data in the pediatric literature, largely due to concerns regarding arterial size. The objective of this study was to measure radial artery diameters in children across all age groups, to establish reference ranges for clinical use. Methods: This prospective study was carried out in children ≤18 years of age who underwent ultrasound for measuring radial artery diameters from November 2018 to November 2019. The cohort was divided into age groups: ≤2, 3 to 5, 6 to 8, 9 to 11, 12 to 14, 15 to 18 years, and into pre- and post-adolescent (≥12 years) groups. Results: One hundred thirty-four children (M:F=63:71) were included, with bilateral measurements resulting in 268 data points. Mean age was 8.9±5.8 years (range, 29 days to 18 years), mean weight 37.2±27.5 kg (range, 1.7–149.1 kg). Mean-corrected radial artery diameter was 1.86±0.44 mm. There was no difference in arterial diameters between males and females (1.90±0.50 versus 1.81±0.53 mm; P =0.73) or between right and left sides (1.87±0.46 versus 1.87±0.47, P =0.98). There was a strong correlation of diameter with age (R=0.75; P P Conclusions: We provide a reference range for radial artery diameters across childhood ages, which can be used for decision-making. This could be the basis for designing a trial of transradial intervention in children, to establish clinical safety and efficacy.

Radiation-associated aneurysms are rare, difficult to treat, and associated with high morbidity and mortality when ruptured, compared with aneurysms unrelated to radiation treatment. We present a 16-year-old patient with a radiation-induced intracranial aneurysm arising from the left posterior inferior cerebellar artery (PICA), 10 years following radiotherapy for medulloblastoma. The patient successfully underwent endovascular coil embolization of the parent artery across the neck of the aneurysm. CT angiography and MRI in the days following the procedure demonstrated maintained flow in the anterior and lateral medullary PICA segments with no brainstem infarct.

BACKGROUND AND PURPOSE: Conventional angiography is the criterion standard for measuring intracranial arterial stenosis. We evaluated signal intensity ratios from TOF-MRA as a measure of intracranial stenosis and infarct risk in pediatric stroke. MATERIALS AND METHODS: A retrospective study was undertaken in children with intracranial arterial stenosis, who had TOF-MRA and conventional angiography performed within 6 months. Arterial diameters were measured for percentage stenosis. ROI analysis on TOF-MRA measured signal intensity in pre- and poststenotic segments, with post-/pre-signal intensity ratios calculated. The Pearson correlation was used to compare percentage stenosis on MRA with conventional angiography and signal intensity ratios with percentage stenosis; the point-biserial correlation was used for infarcts compared with percentage stenosis and signal intensity ratios. Sensitivity, specificity, and positive and negative predictive values were calculated for determining severe (≥70%) stenosis from MRA and signal intensity ratios against the criterion standard conventional angiography. P 1.00: 97.1%, 77.8%, 71.7%, and 97.4%; combination: 75.5%, 100%, 100%, and 76.8%, respectively. Signal intensity ratios decreased with increasing grade of stenosis (none/mild-moderate/severe/complete, P

BACKGROUND AND PURPOSE: The benefit-risk assessment concerning radiation use in pediatric neuroangiography requires an extensive understanding of the doses delivered. This work evaluated the effective dose of 3D rotational angiography in a cohort of pediatric patients with complex neurovascular lesions and directly compared it with conventional 2D-biplane DSA. MATERIALS AND METHODS: Thirty-three 3D rotational angiography acquisitions were acquired in 24 pediatric patients (mean age, 10.4 years). When clinically indicated, following 2D-biplane DSA, 3D rotational angiography was performed with 1 of 3 technical protocols (2 subtracted, 1 unsubtracted). The protocols consisted of 1 factory and 2 customized techniques, with images subsequently reconstructed into CT volumes for clinical management. Raw projections and quantitative dose metrics were evaluated, and the effective dose was calculated. RESULTS: All 3D rotational angiography acquisitions were of diagnostic quality and assisted in patient management. The mean effective doses were 0.5, 0.12, and 0.06 mSv for the factory-subtracted, customized-subtracted, and customized-unsubtracted protocols, respectively. The mean effective dose for 2D-biplane DSA was 0.9 mSv. A direct intraprocedural comparison between 3D and 2D acquisitions indicated that customized 3D rotational angiography protocols delivered mean relative doses of 9% and 15% in unsubtracted and subtracted acquisitions, respectively, compared with biplane DSA, whereas the factory subtracted protocol delivered 68%. CONCLUSIONS: In pediatric neuroangiography, the effective dose for 3D rotational angiography can be significantly lower than for 2D-biplane DSA and can be an essential adjunct in the evaluation of neurovascular lesions. Additionally, available 3D rotational angiography protocols have significant room to be tailored for effectiveness and dose optimization, depending on the clinical question.

Purpose To estimate reference ranges for blood flow volume (BFV) in major splanchnic, thoracoabdominal, and neck vessels by using phase-contrast magnetic resonance (MR) imaging in children and young adults in fasting and postprandial states. Materials and Methods In this institutional research ethics board-approved prospective study, healthy volunteers underwent phase-contrast MR imaging in a fasting state and again after a standardized meal. BFV values were reported as medians and ranges, and postmeal to premeal BFV ratios were calculated. BFVs in volunteers divided into two groups according to age (≤18 years old and18 years old) were compared by using the Mann-Whitney test adjusted for multiple comparisons. Linear regression for internal validation of BFV and Pearson correlation and Bland-Altman analysis for interobserver agreement were used. Results Reference ranges for BFVs were estimated in 39 volunteers (23 male and 16 female; mean age, 21.2 years ± 8.5; range, 9-40 years) and were indexed according to body surface area, with internal validation (R

Intracranial artery-to-artery antegrade revascularization is a poorly recognized entity, more so when it involves main stem arteries. The etiology, appearance, and significance of this condition are not described in the literature. We describe a case of spontaneous revascularization of a chronically occluded middle cerebral arterial branch by collaterals from the proximal segment reconstituting distal flow, mimicking a brain arteriovenous malformation in a 9-year old boy. We discuss the nature of these channels, presumed to be related to artery to artery collaterals that are either dilated adventitial vasa vasorum, or, more likely, leptomeningeal collaterals that are hypertrophied in response to cerebral demand. We review the literature regarding intracerebral vasa vasorum and leptomeningeal collaterals including their imaging. Recognizing the tortuous channels associated with this type of vascular abnormality as normal vessels reconsituting distal flow may prevent unnecessary and potentially dangerous treatments.

The association of perfusion abnormalities with tissue architecture and myocardial function in Kawasaki disease (KD) is poorly understood [(1)][1]. The aims of this study were to quantify myocardial perfusion, measure extracellular volume (ECV) and left ventricular (LV) contractility, and assess

BackgroundThere are few data in the literature on the characteristics and natural history of intracranial arterial infundibular dilatations in children.MethodsAn institutional review board-approved retrospective review was performed of infundibula reported on MR angiography in patients ResultsWe found 60 intracranial infundibula in 60 children (male:female=27:33; mean age 9.7±5.2 years, range 2–18 years,). Family history of aneurysms was present in 2/60 (3.3%). Syndromic association was found in 14/60 (23.3%), most frequently sickle cell disease (4/14=28.6%). Mean infundibulum size was 2.2±0.5 mm, with mean ratio to parent artery of 0.54±0.17. The most common location was on the P1-posterior cerebral artery (34/63=56.7%), whereas posterior communicating infundibula were seen in only 4/60 (6.7%) cases. Other cerebrovascular variants were seen in 12/60 (20%) patients. On follow-up imaging (in 32/60 patients over 86 patient-years, mean 32.3±35.7 months), no significant change in infundibulum:parent artery ratio was noted. None of the infundibular dilatations showed interval evolution to aneurysm.ConclusionWe present the largest reported cohort of pediatric intracranial arterial infundibula, which we found to be distinct from their adult counterparts with regard to location, etiology and temporal evolution. Growth over time and/or aneurysmal formation are rare, not necessitating frequent short-term imaging surveillance during childhood.

Several complex pediatric neurovascular conditions are amenable to endovascular treatment. Given the unique anatomical and physiological challenges in children, there is an ongoing need for tools and techniques that provide accurate information for treatment planning, while minimizing exposure to ionizing radiation and contrast. This is more so for neonates and infants with high-flow arteriovenous (AV) shunts that are challenging to assess using conventional techniques. In this brief report, we describe, through representative cases, the potential role of quantitative color-coded digital subtraction angiography (qDSA) in neuroendovascular procedures in children with high-flow AV shunting lesions. Images were obtained using an ArtisQ biplane system (Siemens Healthineers, Erlangen, Germany). Post-processing was performed at a dedicated workstation (Syngo, Siemens) using the iFlow module to generate color-coded maps of individual digital subtraction angiography runs. Color-coded qDSA provides real-time quantitative information in high-flow AV shunting neurovascular lesions. This can potentially help direct treatment choices, optimize endovascular treatment protocols, monitor outcomes, and determine treatment end points.

To review our experience with the Etomidate speech test (EST) for lateralizing language in children undergoing epilepsy surgery evaluation METHODS: This retrospective study included children (18 years) with drug refractory focal epilepsy undergoing EST for bilateral or poorly reliable language representation on functional MRI. Data for consecutive children who underwent EST between January 2013 to June 2017 were reviewed.Twenty-one children (mean age at EST, 13.1 ± 4.4 years) were studied, with 19-right hemispheric and 20 left hemispheric injections. Six patients had neurological co-morbidities. Duration of ipsilateral EEG slowing was sufficient for speech testing in all children with a single bolus of Etomidate per carotid artery. Language was lateralized to one hemisphere in 17 (80.9%) and bilateral in two cases. EST was unsuccessful in two patients because of diffuse EEG slowing. Contralateral transient frontal EEG slowing was seen in 14 (73.7%) cases. EST was well tolerated in all the patients.The EST was found to be successful and safe in lateralizing language in most of our drug refractory pediatric epilepsy cohort.

The purpose of this study was to determine the additional information provided by Magnetic Resonance Angiography (MRA) in pediatric sickle cell disease (SCD) patients with normal Transcranial Doppler imaging (TCDI) examinations.This cohort study included all pediatric SCD patients over an 18-year period who had no history of stroke and had normal TCDI examinations and subsequently underwent MRA. Routine TCDI inclusive of time-averaged mean of maximum velocities (TAMMV) were assesses and compared with tortuosity on MRA and silent infarct on MRI.86 children (52.3% female; mean age 8.7 ± 3.5years) were included. There were 77 patients (89.5%) with Hb-SS disease and 9(10.4%) with HB-S beta-thalassemia. All patients had normal TAMMV (170 cm/s) on TCDI. 76/86 (88.3%) patients also had one or more velocity readings70 cm/s, albeit none in the middle cerebral arteries. Posterior cerebral arteries had the lowest velocities,70 cm/s in 51.7% (right) and 60.9% (left). Silent MRI infarcts were seen in 27/86 (31.4%) patients. No new lesions were identified on follow-up MRI. Although mild vascular tortuosity was appreciated in 31/86 (36.0%) of the patients, there were no steno-occlusive lesions in the circle of Willis.TCDI and MRA are routinely performed for non-invasively evaluating intracranial vascular abnormalities in children with SCD. In SCD children with no history of TIA or stroke, MRA following a normal TCDI examination is unlikely to show vascular abnormality. However, almost a third of these patients show silent infarcts on MRI, unassociated with MRA changes.

OBJECTIVEPediatric rolandic arteriovenous malformations (AVMs) present a treatment challenge given the lifetime risk of hemorrhage, rehemorrhage, and associated long-term morbidity. Microsurgical resection has been recommended as the optimal treatment for AVMs in general, but there is no dedicated literature on the outcomes of resection of pediatric rolandic AVMs. Here, the study objective was to review the outcomes of microsurgical resection of pediatric rolandic AVMs in the modern era, together with the utilization of surgical adjuncts including navigation, intraoperative angiography, and neurophysiological monitoring.METHODSThe authors performed a retrospective review of patients 18 years of age and younger with cerebral AVMs microsurgically treated between January 2000 and May 2016 at The Hospital for Sick Children. Only those patients with an AVM whose nidus was located within the rolandic region were analyzed. A descriptive analysis was performed to identify patient demographics, preoperative AVM characteristics, and postoperative obliteration rates and neurological complications.RESULTSA total of 279 AVMs were evaluated in the study period. Twenty-three of these AVMs were rolandic, and the median age in the 11 microsurgically treated cases was 11 years (range 1–17 years). AVM hemorrhage was the most common presentation, occurring in 8 patients (73%). Lesions were either Spetzler-Martin grade II (n = 8, 73%) or grade III (n = 3, 27%). The postoperative obliteration rate of AVMs was 100%. The mean imaging follow-up duration was 33 months (range 5–164 months). There was no documented recurrence of an AVM during follow-up. One patient developed a transient postoperative hemiparesis, while another patient developed right fingertip hyperesthesia.CONCLUSIONSMicrosurgical resection of rolandic pediatric AVMs yields excellent AVM obliteration with minimal neurological morbidity in selected patients. The incorporation of surgical adjuncts, including neurophysiological monitoring and neuronavigation, allows accurate demarcation of functional cortex and enables effective resection.

Moyamoya is a chronic, progressive steno-occlusive arteriopathy that typically affects the anterior circulation arteries of the circle of Willis. A network of deep thalamoperforating and lenticulostriate collaterals develop to by-pass the occlusion giving rise to the characteristic angiographic "puff of smoke" appearance. Moyamoya confers a lifelong risk of stroke and neurological demise, with peak age of presentation in childhood ranging between five and 10 years. Moyamoya disease refers to patients who do not have a comorbid condition, whereas moyamoya syndrome refers to patients in whom moyamoya occurs in association with an acquired or inherited disorder such as sickle cell disease, neurofibromatosis type-1 or trisomy 21. The incidence of moyamoya disease and moyamoya syndrome demonstrates geographic and ethnic variation, with a predominance of moyamoya disease in East-Asian populations. Antiplatelet therapy and surgical revascularization procedures are the mainstay of management, as there are no available treatments to slow the progression of the arteriopathy. Future research is required to address the major gaps that remain in our understanding of the pathologic basis, optimal timing for surgery, and determinants of outcome in this high-stroke risk condition of childhood.

Background and Purpose— Arteriopathy is common in childhood arterial ischemic stroke (AIS) and predicts stroke recurrence. Currently available vascular imaging techniques mainly image the arterial lumen rather than the vessel wall and have a limited ability to differentiate among common arteriopathies. We aimed to investigate the value of a magnetic resonance imaging-based technique, namely noninvasive arterial wall imaging (AWI), for distinguishing among arteriopathy subtypes in a consecutive cohort of children presenting with AIS. Methods— Children with confirmed AIS and magnetic resonance angiography underwent 3-Tesla AWI including T1-weighted 2-dimensional fluid-attenuated inversion recovery fast spin echo sequences pre- and post-gadolinium contrast. AWI characteristics, including wall enhancement, wall thickening, and luminal stenosis, were documented for all. Results— Twenty-six children with AIS had AWI. Of these, 9 (35%) had AWI enhancement. AWI enhancement was associated with anterior circulation magnetic resonance angiography abnormality and cortical infarction in 8 of 9 (89%) children and normal magnetic resonance angiography with posterior circulation subcortical infarction in 1 (1 of 9; 11%) child. AWI enhancement was not seen in 17 (65%), 10 (59%) of whom had an abnormal magnetic resonance angiography. Distinct patterns of pre- and postcontrast signal abnormality were demonstrated in the vessel wall in the region of interest in children with transient cerebral arteriopathy, arterial dissection, primary central nervous system angiitis, dissecting aneurysm, and cardioembolic stroke. Conclusions— AWI is a noninvasive, high-resolution magnetic resonance AWI technique, which can be successfully used in children presenting with AIS. Patterns of AWI enhancement are recognizable and associated with specific AIS pathogeneses. Further studies are required to assess the additional diagnostic utility of AWI over routine vascular imaging techniques, in childhood AIS.

Complex neurovascular lesions in children require precise anatomic understanding for treatment planning. Although 3DRA is commonly employed for volumetric reformation in neurointerventional procedures, the ability to reconstruct this data into CT-like images (3DRA-CT) is not widely utilized. This study demonstrates the feasibility and usefulness of 3DRA-CT and subsequent MRI fusion for problem solving in pediatric neuroangiography. This retrospective study includes 18 3DRA-CT studies in 16 children (age 9.6 ± 3.8 years, range 2–16 years) over 1 year. After biplane 2D-digital subtraction angiography (DSA), 5-second 3DRA was performed with selective vessel injection either with or without subtraction. Images were reconstructed into CT sections which were post-processed to generate multiplanar reformation (MPR) and maximum intensity projection (MIP) images. Fusion was performed with 3D T1 MRI images to precisely demonstrate neurovascular relationships. Quantitative radiation metrics were extracted and compared against those for the entire examination and for corresponding biplane 2D-DSA acquisitions. In all 18 cases, the 3DRA procedure and MRI fusion were technically successful and provided clinically useful information relevant to management. The unsubtracted and subtracted 3DRA acquisitions were measured to deliver 5.9 and 132.2%, respectively, of the mean radiation dose of corresponding biplane 2D-DSA acquisitions and contributed 1.2 and 12.5%, respectively, to the total procedure dose. Lower radiation doses, high spatial resolution, and multiplanar reformatting capability make 3DRA-CT a useful adjunct to evaluate neurovascular lesions in children. Fusing 3DRA-CT data with MRI is an additional capability that can further enhance diagnostic information.

We aimed to evaluate the role of post-procedural hemoglobin (hb), without pre-discharge ultrasound (US), after US-guided renal biopsy in children.A retrospective review was conducted of consecutive outpatient native kidney biopsies over an 8-year period. Procedures were performed under real-time US guidance. Data collected included number of passes, presence and size of perinephric hematoma, age, body mass index (BMI), blood pressure (BP), fasting status, hb and platelets. Continuous variables were expressed as mean ± standard deviation, group differences were tested with Student's unpaired t test and analysis of variance and correlations were assessed using Pearson's r. Significance was defined as p 0.05. Hb changes (g/L), percentage hb (%hb) change, hematoma size and positive and negative predictive values (PPV, NPV, respectively) were analyzed.A total of 330 procedures in 300 children (141 females, mean age 11.2 ± 4.30 years) were analyzed. Post-procedural hematoma occurred in 63%. There was a significant (p = 0.0001) post-procedural hb decrease of 6.3 ± 5.5 g/L and %hb decrease of 4.56 ± 4.01%. Fasting during pre-procedural hb estimation and procedural hematoma was associated with a greater hb drop. A ≥10% hb drop had 73% PPV for repeat blood work and US and a 17% PPV for admission, whereas a10% hb drop had a NPV of 84% and 98%, respectively. Hb change showed a weak correlation with age, and hematoma size showed a weak inverse correlation with platelet count, but no correlation with BMI, number of passes or BP.Bleeding complications from US-guided native kidney biopsies in pediatric outpatients can be safely followed by a complete blood count at 6 h post procedure. A 10% hb decrease is strongly associated with requirement for further testing and/or admission.

Pediatric lumbar puncture can be challenging or unsuccessful for several reasons. At the same time, the excellent sonographic window into the pediatric spine provides a distinct opportunity for ultrasound-guided lumbar puncture. Minimal cerebrospinal fluid and thecal displacement by subdural or epidural hematomas are common after failed clinical attempts. Ultrasound is useful for determining a safe infraconal level for subarachnoid access. Real-time guidance increases not only the success rate but also the safety of diagnostic lumbar puncture and injections for chemotherapy and myelography. In this article, we discuss clinical and technical factors for ultrasound-guided pediatric lumbar puncture.

Thoracic outlet syndrome is caused by compression of the neurovascular bundle as it passes from the upper thorax to the axilla. The neurovascular bundle can be compressed by bony structures such as the first rib, cervical ribs or bone tubercles, or from soft-tissue abnormalities like a fibrous band, muscle hypertrophy or space-occupying lesion. Thoracic outlet syndrome commonly affects young adults but can be seen in the pediatric age group, especially in older children. Diagnosis is based on a holistic approach encompassing clinical features, physical examination findings including those triggered by various maneuvers, electromyography, nerve conduction studies and imaging. Imaging is performed to confirm the diagnosis, exclude mimics and classify thoracic outlet syndrome into neurogenic, arterial, venous or mixed causes. MRI and MR angiography are useful in this process. A complete MRI examination for suspected thoracic outlet syndrome should include the assessment of anatomy and any abnormalities using routine sequences, vessel assessment with the arms in adduction by MR angiography and assessment of dynamic compression of vessels with abduction of the arms. The purpose of this paper is to describe the anatomy of the thoracic outlet, causes of thoracic outlet syndrome, the MR imaging techniques used in its diagnosis and the principles of image interpretation.

The use of MRI in isolated pediatric nystagmus remains a gray area in clinical management. Many clinicians prefer to order an MRI to rule out intracranial pathology despite the lack of clinically significant findings in most cases. To assess the yield of MR imaging in isolated pediatric nystagmus and define a management algorithm to minimize avoidable MRI referrals and streamline MRI protocols. We reviewed the charts of 148 children who underwent neuro MRI for isolated nystagmus between January 2008 and September 2014. We noted nystagmus onset and clinical characteristics and compared them with the MRI features and visual electrophysiology results. We included 85 boys and 63 girls (total 148, average age at MRI 4.24 ± 4.19 years). Twenty-three (15.5%) children had abnormal intracranial findings on MRI including abnormal signal lesions (4.1%; n=6), Chiari I malformations (3.4%; n=5) and optic pathway glioma (2.0%; n=3). The time of onset of nystagmus was not associated with an abnormal MRI (P=0.2). Seventy children underwent visual electrophysiology testing but this test could not predict abnormality at MRI, either (P=0.12). Among children with isolated nystagmus, 15.5% had abnormalities on neuroimaging. Neither clinical characteristics of nystagmus nor the visual electrophysiology results allowed prediction of intracranial pathology. We were unable to formulate a management algorithm for the optimal sequence of investigations (MRI preceding visual electrophysiology or vice versa), but we discuss the use of gadolinium contrast agent and orbital sequences in isolated pediatric nystagmus.

Conventional MRI protocols are an integral part of routine clinical imaging in pediatric patients. The advent of several newer MRI techniques provides crucial insight into the structural integrity and functional aspects of the developing brain, especially with the introduction of 3T MRI systems in clinical practice. The field of pediatric neuroimaging continues to evolve, with greater emphasis on high spatial resolution, faster scan time, as well as a quest for visualization of the functional aspects of the human brain. MR vendors are increasingly focusing on optimizing MR technology to make it suitable for children, in whom as compared to adults the head size is usually smaller and demonstrates inherent neuroanatomical differences relating to brain development. The eventual goal of these advances would be to evolve as potential biomarkers for predicting neurodevelopment outcomes and prognostication, in addition to their utility in routine diagnostic and therapeutic decision-making. Advanced MR techniques like diffusion tensor imaging, functional MRI, MR perfusion, spectroscopy, volumetric imaging and arterial spin labeling add to our understanding of normal brain development and pathophysiology of various neurological disease processes. This review is primarily focused on outlining advanced MR techniques and their current and potential pediatric neuroimaging applications as well as providing a brief overview of advances in hardware and machine design.