1. Dowling MM, Hynan LS, Lo W, et al. International Paediatric Stroke Study: stroke associated with cardiac disorders. Int J Stroke. 2013;8 Suppl A100:39-44. |
Observational-Dx |
667 children |
To evaluate the patient characteristics, clinical presentations, stroke characteristics from imaging studies, and early outcomes of children with AIS with reported cardiac disorders and compared them to those of children with other causes of stroke from the large population enrolled in the International Paediatric Stroke Study (IPSS) registry. |
Aetiological data were available for 667 children with arterial ischaemic stroke (ages 29 days to 19 years). Cardiac disorders were identified in 204/667 (30.6%), congenital defects in 121/204 (59.3%), acquired in 40/204 (19.6%), and isolated patent foramen ovale in 31/204 (15.2%). Compared to other children with stroke, those with cardiac disorders were younger (median age 3.1 vs. 6.5 years; P < 0.001) and less likely to present with headache (25.6% vs. 44.6%; P < 0.001), but were similar in terms of gender and presentation with focal deficits, seizures, or recent infection. Analysis of imaging data identified significant differences (P = 0.005) in the vascular distribution (anterior vs. posterior circulation or both) between groups. Bilateral strokes and haemorrhagic conversion were more prevalent in the cardiac disorders group. |
3 |
2. Rivkin MJ, deVeber G, Ichord RN, et al. Thrombolysis in pediatric stroke study. Stroke. 2015;46(3):880-885. |
Review/Other-Dx |
N/A |
To provide a special report on the thrombolysis in pediatric stroke study (TIPS). |
No abstract available. |
4 |
3. Steinlin M. Cerebrovascular disorders in childhood. Handb Clin Neurol. 2013;112:1053-1064. |
Review/Other-Dx |
N/A |
To review cerebrovascular disorders in childhood |
No results stated in abstract. |
4 |
4. Golomb MR, Fullerton HJ, Nowak-Gottl U, Deveber G. Male predominance in childhood ischemic stroke: findings from the international pediatric stroke study. Stroke. 2009;40(1):52-57. |
Observational-Dx |
1187 children |
To study gender within a large international series of confirmed cases in pediatric ischemic stroke. |
Among 1187 children with confirmed ischemic stroke, 710 were boys (60%, P<0.0001). Male predominance persisted after stratification by age (61% for neonates, P=0.011; 59% for later childhood, P=0.002) and stroke subtype (58% for arterial ischemic stroke, P=0.004; 65% for cerebral sinovenous thrombosis, P=0.002). The greatest proportion of males occurred among children with arterial ischemic stroke and a history of trauma (75%, P=0.008), although boys were also overrepresented among those with arterial ischemic stroke and no trauma (57%; P=0.07). There were no gender differences in case fatality or deficits at discharge. |
4 |
5. Mackay MT, Wiznitzer M, Benedict SL, Lee KJ, Deveber GA, Ganesan V. Arterial ischemic stroke risk factors: the International Pediatric Stroke Study. Ann Neurol. 2011;69(1):130-140. |
Review/Other-Dx |
676 children |
To describe presumptive risk factors (RFs) for childhood arterial ischemic stroke (AIS) and explore their relationship with presentation, age, geography, and infarct characteristics. |
A total of 676 children were included. No identifiable RFs was present in 54 (9%). RFs in others included arteriopathies (53%), cardiac disorders (CDs) (31%), infection (24%), acute head and neck disorders (AHNDs) (23%), acute systemic conditions (ASCs) (22%), chronic systemic conditions (CSCs) (19%), prothrombotic states (PTSs) (13%), chronic head and neck disorders (CHNDs) (10%), atherosclerosis-related RFs (2%), and other (22%). Fifty-two percent had multiple RFs. There was lower prevalence of arteriopathy in Asia, lower prevalence of CSCs in Europe and Australia, higher prevalence of PTSs in Europe, and higher prevalence of ASCs in Asia and South America. Prevalence of CDs and ASCs was highest in preschoolers, arteriopathies in children 5 to 9 years old, and CHNDs were highest in children aged 10 to 14 years. Arteriopathies were associated with focal signs and ASCs, CHNDs, and AHNDs with diffuse signs. Arteriopathies, CSCs, and ASCs were associated with multiple infarcts and CDs with hemorrhagic conversion. |
4 |
6. Gardner MA, Hills NK, Sidney S, Johnston SC, Fullerton HJ. The 5-year direct medical cost of neonatal and childhood stroke in a population-based cohort. Neurology. 2010;74(5):372-378. |
Review/Other-Dx |
266 children |
To examine acute and 5-year direct costs of neonatal and childhood stroke in a population-based cohort in Northern California. |
Average adjusted 5-year costs for pediatric stroke are substantial: $51,719 for neonatal stroke and $135,161 for childhood stroke. The average cost of a childhood stroke admission was $81,869. The average birth admission cost for a neonatal stroke was $39,613; adjustment for control birth admission costs reduced this by only $4,792, suggesting the stroke accounted for 88% of costs. Even among neonates whose strokes were not recognized until later in childhood ("presumed perinatal strokes"), admission costs exceeded those of controls. Chronic costs were highest in the first year poststroke, but continued to exceed control costs even in the fifth year by an average of $2,016. |
4 |
7. Per H, Unal E, Poyrazoglu HG, et al. Childhood stroke: results of 130 children from a reference center in Central Anatolia, Turkey. Pediatr Neurol. 2014;50(6):595-600. |
Review/Other-Dx |
130 children |
To share our experience of children with arterial ischemic stroke. |
Sixty-eight patients were male. Thirty of the children were aged from 1 to 12 months (seven of them died in this period). Focal neurological signs were the most common presentation, and hemiplegia or hemiparesis were the most common focal signs. Underlying risk factors were detected in 103 patients. Infections and congenital heart disease were the most common risk factors. Seven of the nine children with recurrent arterial ischemic strokes had one or more underlying diseases (moyamoya disease in two children along with factor V Leiden mutation, tuberculous meningitis, congenital heart disease, homocystinuria, and hemiconvulsion-hemiplegia-epilepsy syndrome). The arterial ischemic stroke was located in the middle cerebral circulation in 68 (36 left and 32 right) and in the posterior cerebral artery in 41. Eighteen children died. The neurological outcome was assessed in 98 children. Of these children, 66 children have neurological deficits and 52 children have seizures. Stroke in the first year of life is more often fatal. Recurrent stroke is associated with poor prognosis. |
4 |
8. Gunny RS, Lin D. Imaging of perinatal stroke. Magn Reson Imaging Clin N Am. 2012;20(1):1-33. |
Review/Other-Dx |
N/A |
To discuss general pathophysiological mechanisms and the role of imaging in the following conditions: arterial ischemic stroke (AIS), including perinatal AIS, and "presumed" perinatal AIS; cerebral venous thrombosis, including cortical vein and venous sinus thrombosis and germinal matrix hemorrhage/periventricular hemorrhagic infarction; and intraparenchymal hemorrhage. |
No results stated in abstract. |
4 |
9. Sanelli PC, Sykes JB, Ford AL, Lee JM, Vo KD, Hallam DK. Imaging and treatment of patients with acute stroke: an evidence-based review. AJNR Am J Neuroradiol. 2014;35(6):1045-1051. |
Review/Other-Dx |
N/A |
To summarize the best available evidence for the diagnostic and therapeutic management of patients with acute ischemic stroke. |
No results stated in abstract. |
4 |
10. Sacks D, Black CM, Cognard C, et al. Multisociety consensus quality improvement guidelines for intraarterial catheter-directed treatment of acute ischemic stroke, from the American Society of Neuroradiology, Canadian Interventional Radiology Association, Cardiovascular and Interventional Radiological Society of Europe, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of NeuroInterventional Surgery, European Society of Minimally Invasive Neurological Therapy, and Society of Vascular and Interventional Neurology. J Vasc Interv Radiol. 2013;24(2):151-163. |
Review/Other-Dx |
N/A |
To define quality benchmarks for processes of care and clinical outcomes in this international multi-specialty document. |
In this international multispecialty document, quality benchmarks for processes of care and clinical outcomes are defined. These include process measures of time to imaging, arterial puncture, and revascularization and measures of clinical outcome up to 90 days. |
4 |
11. Bernard TJ, Rivkin MJ, Scholz K, et al. Emergence of the primary pediatric stroke center: impact of the thrombolysis in pediatric stroke trial. Stroke. 2014;45(7):2018-2023. |
Review/Other-Dx |
17 enrolling centers |
To describe the development of primary pediatric stroke centers through preparation of participating centers in the Thrombolysis in Pediatric Stroke (TIPS) trial. |
Before 2004, <25% of TIPS sites had continuous 24-hour availability of acute stroke teams, MRI capability, or stroke order sets, despite significant pediatric stroke expertise. After TIPS preparation, >80% of sites now have these systems in place, and all sites reported increased readiness to treat a child with acute stroke. Use of a 1- to 10-Likert scale on which 10 represented complete readiness, median center readiness increased from 6.2 before site preparation to 8.7 at the time of site activation (P</=0.001). |
4 |
12. Amlie-Lefond C, Rivkin MJ, Friedman NR, Bernard TJ, Dowling MM, deVeber G. The Way Forward: Challenges and Opportunities in Pediatric Stroke. Pediatr Neurol 2016;56:3-7. |
Review/Other-Dx |
N/A |
To review the challenges and opportunities in Pediatric Stroke |
No abstract available |
4 |
13. Rivkin MJ, Bernard TJ, Dowling MM, Amlie-Lefond C. Guidelines for Urgent Management of Stroke in Children. Pediatr Neurol 2016;56:8-17. |
Review/Other-Dx |
N/A |
To provide guidelines for urgent management of stroke in children. |
No results stated in abstract. |
4 |
14. Cobb MIH, Laarakker AS, Gonzalez LF, Smith TP, Hauck EF, Zomorodi AR. Endovascular Therapies for Acute Ischemic Stroke in Children. Stroke 2017;48:2026-30. |
Review/Other-Dx |
N/A |
To perform a retrospective case review of endovascular management of pediatric Acute ischemic stroke (AIS), with afocus on efficacy and safety. |
No abstract available |
4 |
15. Albers GW, Marks MP, Kemp S, et al. Thrombectomy for Stroke at 6 to 16 Hours with Selection by Perfusion Imaging. N Engl J Med. 378(8):708-718, 2018 02 22. |
Experimental-Tx |
182 patients |
To test the hypothesis that patients who were likely to have salvageable ischemic brain tissue as identified by perfusion imaging and who underwent endovascular therapy 6 to 16 hours after they were last known to have been well would have better functional outcomes than patients treated with standard medical therapy. |
The trial was conducted at 38 U.S. centers and terminated early for efficacy after 182 patients had undergone randomization (92 to the endovascular-therapy group and 90 to the medical-therapy group). Endovascular therapy plus medical therapy, as compared with medical therapy alone, was associated with a favorable shift in the distribution of functional outcomes on the modified Rankin scale at 90 days (odds ratio, 2.77; P<0.001) and a higher percentage of patients who were functionally independent, defined as a score on the modified Rankin scale of 0 to 2 (45% vs. 17%, P<0.001). The 90-day mortality rate was 14% in the endovascular-therapy group and 26% in the medical-therapy group (P=0.05), and there was no significant between-group difference in the frequency of symptomatic intracranial hemorrhage (7% and 4%, respectively; P=0.75) or of serious adverse events (43% and 53%, respectively; P=0.18). |
1 |
16. Jones BP, Ganesan V, Saunders DE, Chong WK. Imaging in childhood arterial ischaemic stroke. Neuroradiology. 2010;52(6):577-589. |
Review/Other-Dx |
N/A |
To consider the practical issues related to imaging children and looks at some of the controversies pertaining to aetiology and its implication for stroke management. To give an overview of childhood arterial ischaemic stroke and to highlight the importance of both acute and delayed vascular imaging in the diagnosis, management and stratification of further stroke risk. |
No results stated in abstract. |
4 |
17. Bernard TJ, Goldenberg NA. Pediatric arterial ischemic stroke. Hematol Oncol Clin North Am. 2010;24(1):167-180. |
Review/Other-Dx |
N/A |
To review pediatric arterial ischemic stroke. |
No results stated in abstract. |
4 |
18. Paonessa A, Limbucci N, Tozzi E, Splendiani A, Gallucci M. Radiological strategy in acute stroke in children. Eur J Radiol. 2010;74(1):77-85. |
Review/Other-Dx |
41 pediatric patients |
To estimate the preponderance of patterns of pediatric stroke, ischemic or hemorrhagic, their etiologies and the correct diagnostic protocol for acute management. |
Twenty-three patients underwent magnetic resonance imaging (MRI), 3 cases were studied by computed tomography (CT) without MRI, and 15 underwent both CT and MRI studies. In 9 cases, intra-arterial digital subtraction angiography (IADSA) was performed after non-invasive preliminary assessment. Seventeen hemorrhagic (41%) and 24 ischemic (59%) strokes were found. Among hemorrhagic forms, 5 cases were due to arteriovenous malformation (AVM), 7 to cavernoma, and 2 to aneurysm. Among ischemic forms, 2 were due to sickle-cell disease, 1 to hyperomocysteinemia, 1 to moyamoya syndrome, 1 to pseudoxantoma elasticum, 3 to prothrombotic state, 1 to Fabry's disease, 1 concomitant with CO intoxication, 5 to venous sinus thrombosis, and 4 to cardio-embolic state. Etiology remains unknown in 8 cases (20.5%). |
4 |
19. Schellinger PD, Bryan RN, Caplan LR, et al. Evidence-based guideline: The role of diffusion and perfusion MRI for the diagnosis of acute ischemic stroke: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology. 2010;75(2):177-185. |
Review/Other-Dx |
N/A |
To assess the evidence for the use of diffusion-weighted imaging (DWI) and perfusion-weighted imaging (PWI) in the diagnosis of patients with acute ischemic stroke. |
DWI is established as useful and should be considered more useful than noncontrast CT for the diagnosis of acute ischemic stroke within 12 hours of symptom onset. DWI should be performed for the most accurate diagnosis of acute ischemic stroke (Level A); however, the sensitivity of DWI for the diagnosis of ischemic stroke in a general sample of patients with possible acute stroke is not perfect. The diagnostic accuracy of DWI in evaluating cerebral hemorrhage is outside the scope of this guideline. On the basis of Class II and III evidence, baseline DWI volumes probably predict baseline stroke severity in anterior territory stroke (Level B) but possibly do not in vertebrobasilar artery territory stroke (Level C). Baseline DWI lesion volumes probably predict (final) infarct volumes (Level B) and possibly predict early and late clinical outcome measures (Level C). Baseline PWI volumes predict to a lesser degree the baseline stroke severity compared with DWI (Level C). There is insufficient evidence to support or refute the value of PWI in diagnosing acute ischemic stroke (Level U). |
4 |
20. Haacke EM, Mittal S, Wu Z, Neelavalli J, Cheng YC. Susceptibility-weighted imaging: technical aspects and clinical applications, part 1. AJNR Am J Neuroradiol. 2009;30(1):19-30. |
Review/Other-Dx |
N/A |
To present the technical background for SWI and discuss the concept of gradient-echo images and how local changes in susceptibility can be measured. |
No results stated in abstract. |
4 |
21. Liu AC, Segaren N, Cox TS, et al. Is there a role for magnetic resonance imaging in the evaluation of non-traumatic intraparenchymal haemorrhage in children?. Pediatric Radiology. 36(9):940-6, 2006 Sep.Pediatr Radiol. 36(9):940-6, 2006 Sep. |
Observational-Dx |
50 children |
To retrospectively examine the role of MRI and MRA in children presenting with spontaneous intraparenchymal haemorrhage (IPH). A secondary aim was to compare the value of MRA with conventional catheter angiography (CCA) for the detection and characterization of vascular pathologies in children with IPH. |
An underlying lesion was demonstrated with MR in two-thirds of children (25/38) with IPH. A vascular lesion was the commonest cause, followed by tumour. Three children had false-negative MR scans. |
3 |
22. Robertson RL, Robson CD, Zurakowski D, Antiles S, Strauss K, Mulkern RV. CT versus MR in neonatal brain imaging at term. Pediatr Radiol. 2003;33(7):442-449. |
Observational-Dx |
48 neonates |
To assess the cross-modality agreement and interobserver agreement of CT and MR brain imaging of the term or near-term neonate. |
Ischemic injury was the most common brain abnormality demonstrated. McNemar's test indicated no significant difference between CT and MR test results for reader 1 (P=0.22) or reader 2 (P=0.45). The readers agreed on the presence or absence of abnormality on CT in 40 patients (83.3%) and on MR in 45 patients (93.8%). For CT, the kappa coefficient indicated excellent interobserver agreement (kappa=0.68), although the lower limit of the 95% confidence interval extends to kappa=0.55, which indicates only good-to-moderate agreement. For MR, the kappa coefficient indicated almost perfect interobserver agreement (kappa=0.88) with the 95% confidence interval extending to a lower limit of kappa=0.76, which represents excellent agreement. |
2 |
23. Yock-Corrales A, Barnett P. The role of imaging studies for evaluation of stroke in children. Pediatr Emerg Care. 2011;27(10):966-974; quiz 975-967. |
Review/Other-Dx |
N/A |
To review the role of imaging studies for evaluation of stroke in children. |
No results stated in abstract. |
4 |
24. Nabavizadeh SA, Edgar JC, Vossough A. Utility of susceptibility-weighted imaging and arterial spin perfusion imaging in pediatric brain arteriovenous shunting. Neuroradiology. 2014;56(10):877-884. |
Observational-Dx |
19 pediatric patients |
To investigate the application of susceptibility-weighted imaging (SWI) and arterial spin labeling (ASL) imaging in the assessment of shunting and the draining veins in pediatric patients with arteriovenous shunting and compare the utility of SWI and ASL with conventional MR and digital subtraction angiography (DSA). |
The mean number of detected draining veins on DSA (3.63) was significantly higher compared to SWI phase-filtered image (mean = 2.72), susceptibility magnitude image (mean = 2.92), ASL (mean = 1.76) and conventional MRI (2.47) (p < 0.05). Pairwise comparison of DSA difference scores (i.e., difference between MR modalities in the number of missed draining veins) revealed no difference between the MR modalities (p > 0.05). ASL was the only method that correctly identified superficial and deep venous drainage in all patients. Regarding detection of shunting, ASL, SWI phase-filtered, and magnitude images demonstrated shunting in 100, 83, and 84% of patients, respectively. |
3 |
25. Lehman LL, Danehy AR, Trenor CC 3rd, et al. Transient Focal Neurologic Symptoms Correspond to Regional Cerebral Hypoperfusion by MRI: A Stroke Mimic in Children. AJNR Am J Neuroradiol. 38(11):2199-2202, 2017 Nov. |
Review/Other-Dx |
16 patients (6 females, 10 males) |
To determine if the use of magnetic resonance (MR) imaging with perfusion-sensitive sequences, diffusion weight imaging (DWI), and magnetic resonance angiography (MRA) can help to rapidly distinguish children with conditions mimicking stroke from those with acute stroke. |
Fourteen (88%) children had lateralized MRA evidence of arterial tree pruning without occlusion, all had negative DWI findings, and all showed evidence of hemispheric hypoperfusion by susceptibility-weighted imaging or arterial spin-labeling perfusion imaging at presentation. These findings normalized following resolution of symptoms in all children who had follow-up imaging (6/16, 38%) |
4 |
26. Zimmerman RA. MRI/MRA evaluation of sickle cell disease of the brain. Pediatr Radiol. 2005;35(3):249-257. |
Review/Other-Dx |
N/A |
To understand the sickle cell disease that affects the brain as infarctions, both clinically apparent and silent, and an understanding of how the blood vessels are affected, the way in which both the brain and the blood vessels are imaged by MRI and MRA and the mechanism of injury |
No results stated in abstract. |
4 |
27. Ganesan V, Cox TC, Gunny R. Abnormalities of cervical arteries in children with arterial ischemic stroke. Neurology. 2011;76(2):166-171. |
Observational-Dx |
60 children |
To describe the frequency of cervical arterial abnormalities in children with acute arterial ischemic stroke (AIS), to examine predictors of this, and to characterize observed abnormalities in terms of specific diagnoses. |
Sixty children (31 boys, median age 5 years 3 months) were included. Cerebral infarction was in the anterior circulation only in 50 (25 purely subcortical), the posterior circulation only in 9, and both distributions in 1. Cervical arterial abnormalities occurred in 15/60 (25%) and intracranial abnormalities in 26. There was no significant relationship between the presence of an abnormality in the intracranial and cervical magnetic resonance angiogram (Fisher exact test, p = 0.29). Cervical arterial disease was categorized as definite arterial dissection in 2 cases, probable arterial dissection in 7, nonspecific occlusive arteriopathy in 5, and a migrated vaso-occlusive device in 1. In logistic regression analysis, infarction in the distribution of the posterior circulation significantly predicted the presence of a cervical arterial abnormality (p = 0.04); age, number of risk factors, and antecedent trauma were not predictive. |
3 |
28. Zebedin D, Sorantin E, Riccabona M. Perfusion CT in childhood stroke--initial observations and review of the literature. Eur J Radiol. 2013;82(7):1059-1066. |
Review/Other-Dx |
10 children |
To report the preliminary results of contrast-enhanced perfusion multi-detector CT for diagnoses of perfusion disturbances in children with clinical suspicion of stroke. |
In nine of ten children perfusion CT showed no false positive or false negative results. In one of ten children suffering from migraine focal hypo-perfusion was read as perfusion impairment potentially indicating early stroke, but MRI and MRA follow-up were negative. Overall, perfusion-CT with CT-DSA was rated very good in 80% of cases for the detection of perfusion disturbances and vessel anatomy. |
4 |
29. Albers GW, Thijs VN, Wechsler L, et al. Magnetic resonance imaging profiles predict clinical response to early reperfusion: the diffusion and perfusion imaging evaluation for understanding stroke evolution (DEFUSE) study. Ann Neurol. 2006;60(5):508-517. |
Observational-Dx |
74 consecutive patients |
Prospective multicenter study to determine whether prespecified baseline MRI profiles can identify stroke patients who have a robust clinical response after early reperfusion when treated 3 to 6 hours after symptom onset. |
Early reperfusion was associated with significantly increased odds of achieving a favorable clinical response in patients with a perfusion/diffusion mismatch (OR, 5.4; P=0.039) and an even more favorable response in patients with the Target Mismatch profile (OR, 8.7; P=0.011). Patients with the No Mismatch profile did not appear to benefit from early reperfusion. Early reperfusion was associated with fatal intracranial hemorrhage in patients with the malignant profile. For stroke patients treated 3 to 6 hours after onset, baseline MRI findings can identify subgroups that are likely to benefit from reperfusion therapies and can potentially identify subgroups that are unlikely to benefit or may be harmed. |
3 |
30. Hoffman CE, Santillan A, Rotman L, Gobin YP, Souweidane MM. Complications of cerebral angiography in children younger than 3 years of age. J Neurosurg Pediatr. 2014;13(4):414-419. |
Review/Other-Dx |
87 children |
To evaluate complications after CA in young children to improve the accuracy of counseling of the parents/guardians of these patients and to identify modifiable risk factors. |
The patient population comprised 40 boys and 47 girls; mean age was 14.36 months (range 1-36 months) and mean weight was 10.8 kg (range 3.7-21.0 kg). For 292 of the 309 procedures, intraarterial chemotherapy was administered; the remaining 17 procedures were for vascular malformations, stroke, tumor embolization, and intracranial hemorrhage. The rate of neurological complications was 0.0%. The rate of nonneurological complications was 2.9%: 7 cases of contrast allergy or bronchospasm, 1 groin hematoma (body weight 7 kg), and 1 transient femoral artery occlusion (body weight 10.8 kg). The rate of radiographic complications was 1.3%: 1 case of transient asymptomatic intraarterial dissection and 3 cases of asymptomatic vasospasm. Postprocedural MRI was performed for 33.3% of patients with no evidence of ischemia. There were no delayed complications. Mean follow-up time was 16.6 months. No association was found between complications and age, duration of anesthesia, number of vessels catheterized, size of the sheath, or diagnostic versus interventional procedures. Despite a trend toward a higher rate of complications for patients who weighed less than 15 kg, this finding was not significant (p = 0.35). |
4 |
31. Robertson RL, Chavali RV, Robson CD, et al. Neurologic complications of cerebral angiography in childhood moyamoya syndrome. Pediatr Radiol. 1998;28(11):824-829. |
Review/Other-Dx |
152 children |
To determine the incidence of neurologic complications of cerebral angiography in children with moyamoya syndrome (MMS) as compared to children without MMS. |
There were 2 neurologic complications within 24 hours of angiography, one in the MMS group and one in the non-MMS group. One patient with MMS became mute following angiography. The symptom resolved within 12 hours. One patient without MMS being examined postoperatively for residual arteriovenous malformation developed intracranial hemorrhage requiring reexploration 12 hours after the angiogram. Using a two-tail Fisher's exact test, there was no significant statistical difference in the ischemic (P = 0.3) or hemorrhagic (P = 1.0) complication rates between the group of patients with MMS and the non-MMS groups. |
4 |
32. Lagman-Bartolome AM, Pontigon AM, Moharir M, et al. Basilar artery strokes in children: good outcomes with conservative medical treatment. Dev Med Child Neurol. 2013;55(5):434-439. |
Observational-Tx |
578 children |
To describe, in a consecutive and prospectively enrolled population of children with arterial ischemic stroke, the frequency, characteristics, and outcomes of childhood basilar artery stroke (BAS) including mortality and neurological outcomes, and independent predictors of these outcomes. |
Among 578 children with acute arterial ischemic stroke, 27 had BAS (4.6% including neonates, 6% excluding neonates). Twenty-four (14 males, 10 females) children met study criteria (mean age at stroke was 8 y 10 mo; range 0-17 y). Eleven children had basilar artery occlusion (BAO). Aspirin or anticoagulation was given to 15 children. None received tissue plasminogen activator or endovascular treatments. At mean follow-up (3 y 2 mo, range 1 mo-11 y 8 mo), 12 had a 'good outcome' (seven normal, five insignificant deficit) and 12 had 'poor outcome' (10 moderate or severe deficit, two acute deaths). Larger infarct size (>/=50% of axial brainstem diameter) independently predicted poor outcome (p=0.02; odds ratio 21.2, 95% confidence interval 1.6-274.9) but not BAO, altered level of consciousness, or age. |
2 |
33. Rollins N, Pride GL, Plumb PA, Dowling MM. Brainstem strokes in children: an 11-year series from a tertiary pediatric center. Pediatr Neurol. 2013;49(6):458-464. |
Observational-Dx |
15 children |
To review a decade-long experience with pediatric brainstem strokes examining issues impacting the timely diagnosis, practice patterns with respect to imaging and accuracy of noninvasive imaging at detecting vertebrobasilar pathology in children, and outcomes in a population treated with systemic anticoagulation. |
There were 10 boys and five girls; 9 months to 17 years of age (mean 7.83 years). Symptoms were headaches (eight); visual problems (eight), seizure-like activity (seven), motor deficits (six), and decreased level of consciousness in four. Time since last seen well was 12 hours to 5 days. Pediatric National Institutes of Health Stroke Scale was 1-34; <10 in eight; 3 in 1, 10-20 in two, and >20 in four. Strokes were pontine in 13/15 and involved >50% of the pons in six and <50% in seven; 2/15 had medullary strokes. Magnetic resonance angiography showed basilar artery occlusion in 8/13 patients and vertebral artery dissection in two. Digital subtraction angiography done within 9-36 hours of magnetic resonance angiography in 10/15 patients confirmed the basilar artery occlusion seen by magnetic resonance angiography and showed vertebral artery dissection in four patients. Patients were systemically anticoagulated without hemorrhagic complications. One patient died. Pediatric Stroke Outcome Measures at 2-36 months is 0-5.0/10 (mean 1.25). |
3 |
34. Cowan F, Mercuri E, Groenendaal F, et al. Does cranial ultrasound imaging identify arterial cerebral infarction in term neonates? Arch Dis Child Fetal Neonatal Ed. 2005;90(3):F252-256. |
Observational-Dx |
47 infants |
To evaluate the diagnostic accuracy of cranial ultrasound (CUS) for detection of neonatal arterial territory cerebral infarction in term infants. |
Brain MRI showed infarction in the territory of the middle cerebral artery in 43 of 47 infants, anterior cerebral artery in one, and posterior cerebral artery in three. Twelve of the 47 had minor changes on MRI in the white matter in the contralateral hemisphere, and four infants had bilateral infarctions. The early CUS scans were abnormal in 68% of the infants; the late CUS scans were abnormal in 87%. The late CUS scans were correct for laterality and site of lesion in 25/47 (53%) infants. In six infants with smaller lesions of the cortical middle cerebral artery branch or lesions in the posterior cerebral artery territory, the CUS scans were persistently normal. |
3 |
35. Bulas D. Screening children for sickle cell vasculopathy: guidelines for transcranial Doppler evaluation. Pediatr Radiol. 2005;35(3):235-241. |
Review/Other-Dx |
N/A |
To provide screening guidelines for transcranial Doppler evaluation for children with sickle cell vasculopathy. |
No results stated in abstract. |
4 |
36. Amlie-Lefond C, Bernard TJ, Sebire G, et al. Predictors of cerebral arteriopathy in children with arterial ischemic stroke: results of the International Pediatric Stroke Study. Circulation. 2009;119(10):1417-1423. |
Review/Other-Dx |
676 subjects with childhood (nonneonatal) AIS at 30 IPSS centers |
To determine the prevalence and predictors of cerebral arteriopathy and FCA among children with AIS. |
Between January 2003 and July 2007, 30 centers within the International Pediatric Stroke Study enrolled 667 children (age, 29 days to 19 years) with arterial ischemic stroke and abstracted clinical and radiographic data. Cerebral arteriopathy and its subtypes were defined using published definitions; FCA was defined as cerebral arterial stenosis not attributed to specific diagnoses such as moyamoya, arterial dissection, vasculitis, or postvaricella angiopathy. We used multivariate logistic regression techniques to determine predictors of arteriopathy and FCA among those subjects who received vascular imaging. Of 667 subjects, 525 had known vascular imaging results, and 53% of those (n=277) had an arteriopathy. The most common arteriopathies were FCA (n=69, 25%), moyamoya (n=61, 22%), and arterial dissection (n=56, 20%). Predictors of arteriopathy include early school age (5 to 9 years), recent upper respiratory infections, and sickle cell disease, whereas prior cardiac disease and sepsis reduced the risk of arteriopathy. The only predictor of FCA was recent upper respiratory infection. |
4 |
37. Wintermark M, Hills NK, deVeber GA, et al. Arteriopathy diagnosis in childhood arterial ischemic stroke: results of the vascular effects of infection in pediatric stroke study. Stroke. 2014;45(12):3597-3605. |
Observational-Dx |
355 cases |
To study the role of clinical data and follow-up imaging in diagnosing cerebral and cervical arteriopathy in children with arterial ischemic stroke. |
Cases were aged median 7.6 years (interquartile range, 2.8-14 years); 56% boys. The majority (52%) was previously healthy; 41% had follow-up vascular imaging. Only 56 (16%) required adjudication. The gold standard diagnosis was definite arteriopathy in 127 (36%), possible in 34 (9.6%), and absent in 194 (55%). Sensitivity was 79% at step 1, 90% at step 2, and 94% at step 3; specificity was high throughout (99%, 100%, and 100%), as was agreement between reviewers (kappa=0.77, 0.81, and 0.78). |
2 |
38. Wintermark M, Hills NK, DeVeber GA, et al. Clinical and Imaging Characteristics of Arteriopathy Subtypes in Children with Arterial Ischemic Stroke: Results of the VIPS Study. AJNR. American journal of neuroradiology 2017;38:2172-79. |
Review/Other-Dx |
355 patients |
To quantify clinical and imaging characteristics associated with childhood arteriopathy subtypes to facilitate their diagnosis and classification in research and clinical settings. |
Among 127 children with definite arteriopathy, the arteriopathy subtype could not be classified in 18 (14%). Moyamoya disease (n = 34) occurred mostly in children younger than 8 years of age; focal cerebral arteriopathy–inflammatory type (n = 25), in children 8–15 years of age; and dissection (n = 26), at all ages. Vertigo at stroke presentation was common in dissection. Dissection affected the cervical arteries, while Moyamoya disease involved the supraclinoid internal carotid arteries. A banded appearance of the M1 segment of the middle cerebral artery was pathognomonic of focal cerebral arteriopathy–inflammatory type but was present in <25% of patients with focal cerebral arteriopathy–inflammatory type; a small lenticulostriate distribution infarct was a more common predictor of focal cerebral arteriopathy–inflammatory type, present in 76%. It remained difficult to distinguish focal cerebral arteriopathy–inflammatory type from intracranial dissection of the anterior circulation. We observed only secondary forms of diffuse/multifocal vasculitis, mostly due to meningitis. |
4 |
39. Smith ER, Scott RM. Spontaneous occlusion of the circle of Willis in children: pediatric moyamoya summary with proposed evidence-based practice guidelines. A review. J Neurosurg Pediatr. 2012;9(4):353-360. |
Review/Other-Dx |
N/A |
To summarize current studies of pediatric moyamoya, with the objective of providing a framework for construction of evidence-based guidelines for treatment. |
No results stated in abstract. |
4 |
40. Steen RG, Xiong X, Langston JW, Helton KJ. Brain injury in children with sickle cell disease: prevalence and etiology. Ann Neurol 2003;54:564-72. |
Observational-Dx |
146 patients |
To evaluate the relationship between brain injury by magnetic resonance imaging (MRI) and vasculopathy by magnetic resonance angiography (MRA) in children with hemoglobin SS, the most serious form of sickle cell disease. |
Standard MRI criteria were used to identify cystic infarction, leukoencephalopathy, encephalomalacia, or atrophy. Standard MRA criteria were used to identify arterial tortuousity (limited vasculopathy), and stenosis or occlusion (extensive vasculopathy). At an average age of 10 years, the estimated prevalence of infarction, ischemic damage, or atrophy in SS patients was 46%, and of vasculopathy was 64%. Only 28% of patients were normal by both modalities, and patients abnormal by MRA often were abnormal by MRI (p < 0.00001). Patients with cystic infarction had limited vasculopathy, whereas patients with encephalomalacia had stenosis or occlusion (p < 0.0001). Large arteries were affected in 31% of brain injury patients, whereas small arteries are inferred to be abnormal in up to 69% of patients with brain injury. The degree of vasculopathy is closely related to the degree of brain injury, implying that vasculopathy is prodromal to most forms of brain injury in hemoglobin SS. |
3 |
41. Amlie-Lefond C, Flanagan J, Kanter J, Dobyns WB. The Genetic Landscape of Cerebral Steno-Occlusive Arteriopathy and Stroke in Sickle Cell Anemia. J Stroke Cerebrovasc Dis 2018;27:2897-904. |
Review/Other-Dx |
N/A |
To review the genetic landscape of cerebro steno-occlusive arteriopathy and stroke in sickle cell anemia. |
No results stated in abstract. |
4 |
42. Gueguen A, Mahevas M, Nzouakou R, et al. Sickle-cell disease stroke throughout life: a retrospective study in an adult referral center. Am J Hematol 2014;89:267-72. |
Review/Other-Dx |
69 patients |
To better understand the characteristics and follow-up of strokes occurring from childhood to adulthood. |
The mechanisms underlying sickle-cell disease associated strokes were reevaluated and etiologies were determined for first stroke and recurrences, in childhood and adulthood. Sickle-cell disease vasculopathy concerned only SS patients and remains their most frequent stroke etiology. Cardioembolism, vaso-occlusive crisis and triggering factors were other etiologies identified in adults. Recurrences occurred in 19 SS patients only after a first ischemic stroke. SC patients' strokes occurred in adulthood and were associated with cardiovascular risk factors. Our findings provide novel information about cerebrovascular pathologies throughout the lives of sickle-cell disease patients and suggest the need for different diagnostic and therapeutic management approaches in those different settings. |
4 |
43. Gonzalez RG, Schaefer PW, Buonanno FS, et al. Diffusion-weighted MR imaging: diagnostic accuracy in patients imaged within 6 hours of stroke symptom onset. Radiology 1999;210:155-62. |
Observational-Dx |
22 patients |
To evaluate the diagnostic accuracy of diffusion-weighted magnetic resonance (MR) imaging performed within 6 hours of the onset of stroke symptoms. |
Diffusion-weighted images indicated stroke in 14 patients, all of whom had a final diagnosis of acute stroke. Diffusion-weighted images were negative ineight patients, all of whom had a final clinical diagnosis other than stroke (100% sensitivity, 100% specificity, x2 = 23.00, P < .0001). Blinded reviews yielded 100% sensitivity and 86% specificity for diffusion-weighted MR imaging (x2 = 15.43, P < .0005); 18% sensitivity and 100% specificity for conventional MR imaging (x2 = 2.85, P > .2); and 45% sensitivity and 100% specificity for CT (x2 = 4.40, P > .10). Lesion percentage CNRs were 77% for diffusion-weighted imaging, 5.5% for CT, 9.8% for T2-weighted MR imaging, and 3.1% for proton-density–weighted MR imaging (P < .002 for diffusion-weighted imaging vs others). |
2 |
44. Qin Y, Ogawa T, Fujii S, et al. High incidence of asymptomatic cerebral microbleeds in patients with hemorrhagic onset-type moyamoya disease: a phase-sensitive MRI study and meta-analysis. Acta Radiol. 2015;56(3):329-338. |
Meta-analysis |
27 patients for study, 245 patients for meta-analysis |
To investigate the incidence, distribution patterns, and influencing factors of asymptomatic cerebral microbleeds (CMBs) in patients with moyamoya disease. |
Thirty-five asymptomatic CMBs were demonstrated in 14 moyamoya disease patients (51.9%) in our PSI study. Of these, 45.7% were located in the periventricular white matter. In the meta-analysis, the pooled incidence of asymptomatic CMBs in moyamoya disease was 46% (95% confidence interval [CI], 28.2-63.8%) on SWI or PSI and 29.6% (95% CI, 17.4-41.7%) on T2*WI. Statistical analysis showed that PSI or SWI offered better detection of CMBs in moyamoya disease than T2*WI, and 3-T T2*WI offered better detection than 1.5-T T2*WI. Furthermore, hemorrhagic onset-type moyamoya disease correlated with a high incidence of asymptomatic CMBs. |
M |
45. Adams RJ. TCD in sickle cell disease: an important and useful test. Pediatr Radiol. 2005;35(3):229-234. |
Review/Other-Dx |
N/A |
To review the role of transcranial Doppler US in sickle cell disease. |
TCD results were classified as normal, conditional, abnormal or inadequate based on velocity readings in specific arterial segments. The MCA and ICA were carefully searched to find the highest velocity, and this was used to stratify patients as to stroke risk. Those with abnormal or high-risk TCD received either monthly blood transfusions or no transfusions, and those randomized to transfusion had a much better outcome in terms of stroke (one stroke, versus ten in the control group) and also fewer other medical problems while transfused. TCD screening is recommended for SCD patients to begin at 24 months of age and should be repeated every 6-12 months during early childhood. Transcranial Doppler imaging (TCDI) can be easier to use and learn and with a few adjustments can provide equivalent predictive power. |
4 |
46. Goetti R, Warnock G, Kuhn FP, et al. Quantitative cerebral perfusion imaging in children and young adults with Moyamoya disease: comparison of arterial spin-labeling-MRI and H(2)[(15)O]-PET. AJNR Am J Neuroradiol. 2014;35(5):1022-1028. |
Observational-Dx |
13 patients |
To evaluate the correlation of quantitative CBF measurements performed with arterial spin-labeling-MR imaging and H2[(15)O]-PET in children and young adults with Moyamoya disease. |
Interreader agreement was good (kappa = 0.67-0.69), and strong and significant correlations were found between arterial spin-labeling-MR imaging and H2[(15)O]-PET for both qualitative perfusion scoring (rho = 0.77; P < .001) and quantitative perfusion assessment of relative CBF with cerebellar normalization (r = 0.67, P < .001). |
2 |
47. Dlamini N, Yau I, Muthusami P, et al. Arterial Wall Imaging in Pediatric Stroke. Stroke; a journal of cerebral circulation 2018;49:891-98. |
Observational-Dx |
26 patients (16 males, 10 females) |
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. |
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. |
3 |
48. Ishii Y, Nariai T, Tanaka Y, et al. Practical clinical use of dynamic susceptibility contrast magnetic resonance imaging for the surgical treatment of moyamoya disease. Neurosurgery. 2014;74(3):302-309. |
Review/Other-Dx |
69 patients |
To explore whether dynamic susceptibility contrast magnetic resonance imaging could predict the effects and risk of indirect bypass surgery on moyamoya disease. (MMD). |
The hemispheres that caused the ischemic events (responsible hemisphere) had a significantly longer preoperative MTT delay than the nonresponsible hemispheres (2.66 +/- 1.34 vs 1.57 +/- 1.09 seconds). The postoperative MTT delay fell significantly in the patients whose symptoms disappeared (preoperative, 2.61 +/- 1.35 seconds; postoperative, 1.35 +/- 0.96 seconds). Perioperative infarction occurred in 4 hemispheres (3.5%), and the MTT delay was significantly longer in those hemispheres than in the others (3.97 +/- 1.20 vs 2.38 +/- 1.34 seconds). The MTT delay was significantly longer in patients with higher angiographic stages. Indirect bypass surgery ameliorated the MTT delay to the same degree in adults and children. Digital subtraction angiography revealed that the induced revascularization was far superior in areas with longer MTT delays. |
4 |
49. Noguchi T, Kawashima M, Nishihara M, Hirai T, Matsushima T, Irie H. Arterial spin-labeling MR imaging in Moyamoya disease compared with clinical assessments and other MR imaging findings. Eur J Radiol. 2013;82(12):e840-847. |
Observational-Dx |
71 patients |
To identify the causal factors for the perfusion distribution obtained with ASL-MRI by comparing ASL-MRI with clinical information and other MRI findings in moyamoya disease. |
Seventy-one patients with moyamoya disease underwent ASL-MRI and other MRI, including fluid-attenuated inversion recovery imaging (FLAIR) and three-dimensional time-of-flight magnetic resonance angiography (MRA) on 3.0-Tesla MRI system. Cerebral blood flow (CBF) values (ASL values) for the cerebral hemispheres (142 sides) were measured on CBF maps generated by ASL-MRI. Relationships between the ASL values and the following 9 factors were assessed: sex, family history, revascularization surgery, age at MR exam, age at onset, the steno-occlusive severity on MRA (MRA score), degree of basal collaterals, degree of leptomeningeal high signal intensity seen on FLAIR, and size of ischemic or hemorrhagic cerebrovascular accident lesion (CVA score). Patients with a family history had significantly higher ASL values than those without such a history. There were significant negative correlations between ASL values and age at MR exam, MRA score, and CVA score. |
3 |
50. Debette S, Compter A, Labeyrie MA, et al. Epidemiology, pathophysiology, diagnosis, and management of intracranial artery dissection. Lancet Neurol. 2015;14(6):640-654. |
Review/Other-Dx |
N/A |
To review the epidemiology, pathophysiology, diagnosis, and management of intracranial artery dissection. |
No results stated in abstract. |
4 |
51. Gemmete JJ, Toma AK, Davagnanam I, Robertson F, Brew S. Pediatric cerebral aneurysms. Neuroimaging Clin N Am. 2013;23(4):771-779. |
Review/Other-Dx |
N/A |
To review pediatric cerebral aneurysms. |
No results stated in abstract. |
4 |
52. Stence NV, Fenton LZ, Goldenberg NA, Armstrong-Wells J, Bernard TJ. Craniocervical arterial dissection in children: diagnosis and treatment. Curr Treat Options Neurol. 2011;13(6):636-648. |
Review/Other-Dx |
N/A |
Review diagnosis and treatment of craniocervical arterial dissection in children. |
No results stated in abstract. |
4 |
53. Tan MA, DeVeber G, Kirton A, Vidarsson L, MacGregor D, Shroff M. Low detection rate of craniocervical arterial dissection in children using time-of-flight magnetic resonance angiography: causes and strategies to improve diagnosis. J Child Neurol. 2009;24(10):1250-1257. |
Observational-Dx |
13 patients |
To assess the role of time-of-flight magnetic resonance angiography in diagnosing craniocervical arterial dissection. |
We included children (1 month to 18 years) with craniocervical arterial dissection and ischemic stroke from January 1998 to June 2007. Institutional protocol required magnetic resonance imaging (MRI)/ magnetic resonance angiography for all ischemic stroke cases and conventional cerebral angiography if magnetic resonance angiography showed any arteriopathy. Among 233 ischemic stroke cases, craniocervical arterial dissection was diagnosed in 13 patients (5.6%; 10 males), with a mean age of 7.5 years. Initial time-of-flight magnetic resonance angiography missed dissection in 8 patients (61.5%) because the neck vessels were not scanned (n = 2), of suboptimal technique (n = 3), and of diagnostic error (n = 3). T1 fat-saturated MRI and contrast-enhanced magnetic resonance angiography were not performed in 12 patients. If suspicion for arterial dissection is high, T1 fat-saturated neck imaging and contrast-enhanced magnetic resonance angiography should be considered to maximize detection. |
3 |
54. Benseler S, Pohl D. Childhood central nervous system vasculitis. Handb Clin Neurol. 2013;112:1065-1078. |
Review/Other-Dx |
N/A |
To discuss childhood central nervous system vasculitis |
No results stated in abstract. |
4 |
55. Elbers J, Halliday W, Hawkins C, Hutchinson C, Benseler SM. Brain biopsy in children with primary small-vessel central nervous system vasculitis. Ann Neurol. 2010;68(5):602-610. |
Review/Other-Dx |
13 patients |
To characterize the clinical and histopathological features of brain biopsies in small-vessel primary angiitis of the central nervous system in childhood (cPACNS) (SVcPACNS). |
A total of 13 SVcPACNS patients were included. Ages ranged from 5 to 17 years. Presenting features included seizures (85%), headache (62%), and cognitive decline (54%). Brain biopsy confirmed SVcPACNS in 11 patients with intramural lymphocytic infiltrate. Two had nonspecific perivascular inflammation only. All 6 nonlesional biopsies yielded a diagnosis of SVcPACNS. Lack of specific histological features correlated with prolonged time to biopsy, prior steroid treatment, and inadequate specimen sampling. |
4 |
56. Benseler SM, deVeber G, Hawkins C, et al. Angiography-negative primary central nervous system vasculitis in children: a newly recognized inflammatory central nervous system disease. Arthritis Rheum. 2005;52(7):2159-2167. |
Review/Other-Dx |
4 children |
To study 4 children with primary CNS vasculitis in whom results of magnetic resonance imaging studies were abnormal but results of conventional angiography were normal. |
We determined that angiography-negative, biopsy-confirmed primary small-vessel CNS vasculitis is a previously unrecognized distinct disease entity in children. The diagnosis must be considered in a child with a progressive, acquired diffuse or focal neurologic deficit, even if the results of conventional angiography are normal. A lesional brain biopsy is required to confirm the diagnosis. |
4 |
57. Blauwblomme T, Bourgeois M, Meyer P, et al. Long-term outcome of 106 consecutive pediatric ruptured brain arteriovenous malformations after combined treatment. Stroke. 2014;45(6):1664-1671. |
Observational-Tx |
106 children |
To assess the clinical and angiographic outcome and to analyze risk factors for rebleeding during and after combined treatment of pediatric brain ateriovenous malformations (bAVMs). |
One hundred six children with ruptured bAVMs were followed up for a total of 480.5 patient-years (mean, 4.5 years). Thirteen rebleeding events occurred, corresponding to an annual bleeding rate of 2.71+/-1.32%, significantly higher in the first year (3.88+/-1.39%) than thereafter (2.22+/-1.38%; P<0.001) and in the case of associated aneurysms (relative risk, 2.68; P=0.004) or any deep venous drainage (relative risk, 2.97; P=0.002), in univariate and multivariate analysis. Partial embolization was associated with a higher annual bleeding rate, whereas initial surgery for intracerebral hemorrhage evacuation was associated with a lower risk of rebleeding. |
2 |
58. Lo WD. Childhood hemorrhagic stroke: an important but understudied problem. J Child Neurol. 2011;26(9):1174-1185. |
Review/Other-Dx |
N/A |
To describe the estimated frequency, clinical presentation, acute management of hemorrhagic stroke, and an overview of rehabilitation techniques. |
No results stated in abstract. |
4 |
59. Toma AK, Davagnanam I, Ganesan V, Brew S. Cerebral arteriovenous shunts in children. Neuroimaging Clin N Am. 2013;23(4):757-770. |
Review/Other-Dx |
N/A |
To describe cerebral arteriovenous shunts in children. |
No results stated in abstract. |
4 |
60. Saraf R, Shrivastava M, Siddhartha W, Limaye U. Intracranial pediatric aneurysms: endovascular treatment and its outcome. J Neurosurg Pediatrics. 10(3):230-40, 2012 Sep. |
Review/Other-Dx |
23 pediatric patients |
To analyze the location, clinical presentation, and morphological characteristics of pediatric aneurysms and the safety, feasibility, and durability of endovascular treatment. |
During the study period, 23 pediatric patients (mean age 13 years, range 2 months-18 years) were referred to the authors' department and underwent endovascular treatment for aneurysms. The aneurysms were saccular in 6 cases, dissecting in 4, infectious in 5, and giant partially thrombosed lesions in 8. Fourteen of the aneurysms were ruptured, and 9 were unruptured. Thirteen were in the anterior circulation and 10 in the posterior circulation. The most common location in the anterior circulation was the anterior communicating artery; in the posterior circulation, the most common location was the basilar artery. Saccular aneurysms were the most common type in the anterior circulation; and giant partially thrombosed and dissecting aneurysms were the most common types in the posterior circulation. Coil embolization was performed in 7 cases, parent vessel sacrifice in 10, flow reversal in 3, glue embolization in 2, and stent placement in 1. Immediate angiographic cure was seen in 21 (91%) of 23 patients. Complications occurred in 4 patients, 3 of whom eventually had a good outcome. No patient died. Overall, a favorable outcome was seen in 22 (96%) of 23 patients. Follow-up showed stable occlusion of aneurysms in 96% of the patients. |
4 |
61. Linscott LL, Leach JL, Jones BV, Abruzzo TA. Developmental venous anomalies of the brain in children -- imaging spectrum and update. [Review]. Pediatr Radiol. 46(3):394-406; quiz 391-3, 2016 Mar. |
Review/Other-Dx |
N/A |
To review developmental venous anomalies of the brain in children. |
No results stated in abstract. |
4 |
62. Aeron G, Abruzzo TA, Jones BV. Clinical and imaging features of intracranial arterial aneurysms in the pediatric population. Radiographics. 2012;32(3):667-681. |
Review/Other-Dx |
N/A |
To report on the clinical and imaging features of intracranial arterial aneurysms in the pediatric population. |
No results stated in abstract. |
4 |
63. Hetts SW, Narvid J, Sanai N, et al. Intracranial aneurysms in childhood: 27-year single-institution experience. AJNR Am J Neuroradiol. 30(7):1315-24, 2009 Aug. |
Review/Other-Dx |
77 patients |
To characterize the clinical, imaging, treatment, and outcome data of patients younger than 19 years diagnosed with intracranial aneurysms at a tertiary care institution. |
We evaluated 77 patients (mean age, 12 years; 40 female, 37 male) with 103 intracranial aneurysms. Patients presented with headache (45%), cranial neuropathies (16%), nausea/vomiting (15%), vision changes (13%), trauma (13%), seizure (4%), or sensory changes (3%). Subarachnoid hemorrhage occurred in 25 patients. Thirty-one fusiform aneurysms occurred in 25 patients. Forty-seven saccular aneurysms occurred in 35 patients. Twelve infectious aneurysms occurred in 6 patients. Fifteen traumatic aneurysms occurred in 12 patients. Fifty-nine patients underwent treatment of their aneurysms; 18 patients' conditions were managed conservatively. Nineteen patients underwent primary endovascular coiling, 1 patient had endovascular stent-assisted coiling, 11 patients underwent endovascular parent artery occlusion, 19 patients underwent surgical clipping, and 10 patients had aneurysms trapped and bypassed. Mortality was 1.3%. Morbidity included 8% infarction and 4% new-onset seizures. Six patients developed new aneurysms or had enlargement of untreated aneurysms. |
4 |
64. Hetts SW, English JD, Dowd CF, Higashida RT, Scanlon JT, Halbach VV. Pediatric intracranial aneurysms: new and enlarging aneurysms after index aneurysm treatment or observation. AJNR Am J Neuroradiol. 2011;32(11):2017-2022. |
Observational-Dx |
83 patients |
To identify comorbidities and angiographic features in children that predict new aneurysm formation or enlargement of untreated aneurysms. |
Of 83 patients harboring 114 intracranial aneurysms not associated with brain arteriovenous malformations or intracranial arteriovenous fistulas, 9 (8.4%) developed new or enlarging brain aneurysms an average of 4.2 years after initial presentation. Comorbidities that may be related to aneurysm formation were significantly higher in patients who developed new aneurysms (89%) as opposed to patients who did not develop new or enlarging aneurysms (41%; RR, 9.5; 95% CI, 1.9%-48%; P = .0099). Patients with multiple aneurysms at initial presentation were more likely than patients with a single aneurysm at presentation to develop a new or enlarging aneurysm (RR, 6.2; 95% CI, 2.1%-185; P = .0058). Patients who initially presented with at least 1 fusiform aneurysm were more likely to develop a new or enlarging aneurysm than patients who did not present with a fusiform aneurysm (RR, 22; 95% CI, 3.6%-68%; P = .00050). Index aneurysm treatment with parent artery occlusion also was associated with higher risk of new aneurysm formation (RR, 4.2; 95% CI, 1.3%-13%; P = .024). New aneurysms did not necessarily arise near index aneurysms. The only fatality in the series was due to subarachnoid hemorrhage from a new posterior circulation aneurysm arising 20 months after index anterior circulation aneurysm treatment in an immunosuppressed patient. |
4 |
65. Lu L, Zhang LJ, Poon CS, et al. Digital subtraction CT angiography for detection of intracranial aneurysms: comparison with three-dimensional digital subtraction angiography. Radiology 2012;262:605-12. |
Observational-Dx |
513 patients |
To evaluate the diagnostic accuracy of digital subtraction computed tomographic (CT) angiography in the detection of intracranial aneurysms compared with three-dimensional (3D) rotational digital subtraction angiography (DSA), as reference standard, in a large cohort in a single center. |
Of 513 patients, 106 (20.7%) had no aneurysms , while 407 patients (79.3%) had 459 aneurysms at 3D DSA. Digital subtraction CT angiography correctly depicted 456 (99.3%) of the 459 aneurysms. By using 3D DSA as the standard of reference, the sensitivity and specifi city of depicting intracranial aneurysms were 97.8% (398 of 407) and 88.7% (94 of 106), respectively, on a per-patient basis, and 96.5% (443 of 459) and 87.8% (94 of 107), respectively, on a per-aneurysm basis. Digital subtraction CT angiography had sensitivities of 91.3% (42 of 46), 94.0% (140 of 149), 98.4% (186 of 189), and 100% (75 of 75) in depicting aneurysms of less than 3 mm, between 3 mm but less than 5 mm, between 5 mm but less than 10 mm, and 10 mm or greater, respectively, and of 95.8% (276 of 288) and 97.7% (167 of 171) in depicting anterior circulation and posterior circulation aneurysms, respectively. Excellent inter- and intrareader agreement was found on a per-patient ( k = 0.900 and 0.939, both P < .001) and per-aneurysm basis ( k = 0.846 and 0.921, both P < .001) for the detection of intracranial aneurysms with digital subtraction CT angiography. |
2 |
66. Thaker NG, Turner JD, Cobb WS, et al. Computed tomographic angiography versus digital subtraction angiography for the postoperative detection of residual aneurysms: a single-institution series and meta-analysis. J Neurointerv Surg 2012;4:219-25. |
Meta-analysis |
11 studies |
To compare the diagnostic accuracy of Computed tomographic angiography (CTA) with the current ‘gold standard’, digital subtraction angiography (DSA), in the postoperative detection of residual cerebral aneurysms (RA). |
This institutional series reports sensitivity, specificity, positive (PPV) and negative predictive values (NPV) of 100%. Eleven studies met the inclusion criteriafor the meta-analysis. A total of 427 patients with 513 aneurysms were included, with 61 RA detected by DSA and 40 detected by CTA. Unweighted analysis resulted in pooled sensitivity of 73.8%, specificity of 96.3%, PPV of 91.0% and NPV of 86.1%. Stratified analysis of studies using 16-slice CTA versus 2D DSA reported pooled sensitivity of 92.6%, specificity of 99.3%, PPV of 95.8%, and NPV of 97.8%. |
Good |
67. Sailer AM, Wagemans BA, Nelemans PJ, de Graaf R, van Zwam WH. Diagnosing intracranial aneurysms with MR angiography: systematic review and meta-analysis. [Review]. Stroke. 45(1):119-26, 2014 Jan.Stroke. 45(1):119-26, 2014 Jan. |
Meta-analysis |
12 studies |
To evaluate the sensitivity and specificity of MR angiography (MRA) in the diagnosis of ruptured and unruptured intracranial aneurysms. |
Included studies were of high methodological quality. Studies with larger sample size tended to have higher diagnostic performance. Most studies used time-of-flight MRA technique. Among the 960 patients assessed, 772 aneurysms were present. Heterogeneity with reference to sensitivity and specificity was moderate to high. Pooled sensitivity of MRA was 95% (95% confidence interval, 89%-98%), and pooled specificity was 89% (95% confidence interval, 80%-95%). False-negative and false-positive aneurysms detected on MRA were mainly located at the skull base and middle cerebral artery. Freehand 3-dimensional reconstructions performed by the radiologist significantly increased diagnostic performance. Studies performed on 3 Tesla showed a trend toward higher performance (P=0.054). |
Good |
68. Crawley F, Clifton A, Brown MM. Should we screen for familial intracranial aneurysm? Stroke. 1999;30(2):312-316. |
Meta-analysis |
N/A |
To establish whether individuals with a family history of >/=2 first-degree relatives with intracranial aneurysm should be offered screening for aneurysm. |
We derived 3 theoretical models and calculated the outcomes of screening with magnetic resonance angiography (MRA) followed by digital subtraction angiography (DSA) if MRA was positive (model 1), screening with DSA alone (model 2), and not screening (model 3). Screening was repeated at intervals of 10 years, and aneurysms detected were treated surgically. We assumed a prevalence of aneurysm of 9.8% (95% CI, 8.9% to 10.6%) in the population screened, an annual rupture rate of asymptomatic aneurysm of 0.8% (95% CI, 0.4% to 1.5%), and a 75% chance of poor outcome from rupture. We assumed the sensitivity and specificity of MRA were each 90% and the risk of DSA was 0.1%. The risk of surgery was taken as 5.1%. Screening 1000 individuals on 3 occasions with MRA and DSA or with DSA alone followed by surgery resulted in poor outcome in 14 and 18 individuals, respectively, over 30 years. Without screening, poor outcome occurred in 15 individuals over the same period of time. |
M |
69. Hetts SW, Cooke DL, Nelson J, et al. Influence of patient age on angioarchitecture of brain arteriovenous malformations. AJNR Am J Neuroradiol. 2014;35(7):1376-1380. |
Observational-Dx |
203 children |
To determine whether clinical and angioarchitectural features of brain AVMs differ between children and adults. |
Results often differed when age was treated as a continuous variable as opposed to dividing subjects into children (18 years or younger; n = 203) versus adults (older than 18 years; n = 630). Children were more likely to present with AVM hemorrhage than adults (59% versus 41%, P < .001). Although AVMs with a larger nidus presented at younger ages (mean of 26.8 years for >6 cm compared with 37.1 years for <3 cm), this feature was not significantly different between children and adults (P = .069). Exclusively deep venous drainage was more common in younger subjects when age was treated continuously (P = .04) or dichotomized (P < .001). Venous ectasia was more common with increasing age (mean, 39.4 years with ectasia compared with 31.1 years without ectasia) and when adults were compared with children (52% versus 35%, P < .001). Patients with feeding artery aneurysms presented at a later average age (44.1 years) than those without such aneurysms (31.6 years); this observation persisted when comparing children with adults (13% versus 29%, P < .001). |
3 |
70. Kouznetsov E, Weill A, Ghostine JS, Gentric JC, Raymond J, Roy D. Association between posterior fossa arteriovenous malformations and prenidal aneurysm rupture: potential impact on management. Neurosurg Focus. 2014;37(3):E4. |
Review/Other-Dx |
233 cases |
To evaluate the relative proportions of cases of infra- and supratentorial AVMs in which patients presented with prenidal aneurysm rupture. |
Of 233 AVMs, 25 (11%) were in the posterior fossa, and in 22 (88%) of these cases, the patients presented with hemorrhage, including 9 patients (41%) who presented with hemorrhage due to prenidal aneurysm rupture. Of 208 patients with supratentorial AVMs, 107 (51%) presented with hemorrhage, including 5 patients (4.7%) in whom the hemorrhage was associated with a prenidal aneurysm (p < 0.01). All 9 patients with posterior fossa AVMs and prenidal aneurysm rupture were treated with early embolization of the offending aneurysm. There was no early rebleeding or clinical complication related to this approach. At the end of follow-up (mean 46.8 months), 2 patients had an mRS score of 0, 1 had a score of 1, 3 had a score of 2, 2 patients were dead (mRS score of 6), and 1 patient was lost to follow-up. |
4 |
71. Gaballah M, Storm PB, Rabinowitz D, et al. Intraoperative cerebral angiography in arteriovenous malformation resection in children: a single institutional experience. J Neurosurg Pediatr. 2014;13(2):222-228. |
Observational-Tx |
17 patients |
To evaluate the use of cerebral intraoperative angiography in children undergoing arteriovenous malformations (AVM) resection. |
A total of 21 intraoperative angiography procedures were performed for 18 AVM resections in 17 patients. The technical success rate was 94%. In 2 cases (11%), intraoperative angiography demonstrated a residual AVM, and repeat resections were performed. In both cases, no recurrent disease was noted on postoperative follow-up. One procedure-related complication (4.8%) occurred in 1 patient who was positioned prone. Recurrence to date was noted in 2 (14%) of the 14 cases with available postoperative follow-up at 3.5 and 4.7 months following resection with intraoperative angiography. The median follow-up time from intraoperative angiography to the most recent postoperative angiography was 1.1 years (range 4.3 months to 3.8 years). |
2 |
72. Gross BA, Du R, Orbach DB, Scott RM, Smith ER. The natural history of cerebral cavernous malformations in children. J Neurosurg Pediatr. 2015:1-6. |
Observational-Dx |
167 patients |
To elucidate the natural history of newly diagnosed cerebral cavernous malformations (CMs) in the pediatric population to determine their eventual clinical course, with an emphasis on risk factors for hemorrhage. |
In a cohort of 167 patients with 222 CMs, the mean patient age at the time of diagnosis was 10.1 years old (SD 6.0). Ninety patients (54%) were male. One hundred four patients (62%) presented with hemorrhage from at least 1 CM, 58 (35%) with seizures with or without CM hemorrhage, and 43 (26%) with incidental lesions. Twenty-five patients (15%) had multiple CMs, 17 (10%) had a family history of CMs, and 33 (20%) had radiologically apparent developmental venous anomalies (DVAs). The overall annual hemorrhage rate was 3.3%. Permanent neurological morbidity was 29% per hemorrhage, increasing to 45% for brainstem, thalamic, or basal ganglia CM and decreasing to 15% for supratentorial lobar or cerebellar lesions. The annual hemorrhage rate for incidental CMs was 0.5%; for hemorrhagic CMs, it was 11.3%, increasing to 18.2% within the first 3 years. Hemorrhage clustering within 3 years was statistically significant (HR 6.1, 95% CI 1.72-21.7, p = 0.005). On multivariate analysis, hemorrhagic presentation (HR 4.63, 95% CI 1.53-14.1, p = 0.007), brainstem location (HR 4.42, 95% CI 1.57-12.4, p = 0.005), and an associated radiologically apparent DVA (HR 2.91, 95% CI 1.04-8.09, p = 0.04) emerged as significant risk factors for hemorrhage, whereas age, sex, CM multiplicity, and CM family history did not. |
4 |
73. D'Angelo R, Marini V, Rinaldi C, et al. Mutation analysis of CCM1, CCM2 and CCM3 genes in a cohort of Italian patients with cerebral cavernous malformation. Brain Pathol. 2011;21(2):215-224. |
Review/Other-Dx |
95 patients |
To represent the first extensive research program for a comprehensive molecular screening of the three known genes in an italian cohort of cerebral cavernous malformations (CCMs) patients and their at-risk relatives. |
Sixteen mutations in 16 unrelated CCM patients were identified,nine mutations are novel: c.413T > C; c.601C > T; c.846 + 2T > G; c.1254delA; c.1255-4delGTA; c.1682-1683 delTA in CCM1; c.48A > G; c.82-83dupAG in CCM2; and c.395 + 1G > A in CCM3 genes [corrected].The samples, negative to direct exon sequencing, were investigated by MLPA to search for intragenic deletions or duplications. One deletion in CCM1 exon 18 was detected in a sporadic patient. Among familial cases 67% had a mutation in CCM1, 5.5% in CCM2, and 5.5% in CCM3, whereas in the remaining 22% no mutations were detected, suggesting the existence of either undetectable mutations or other CCM genes. |
4 |
74. Zawistowski JS, Stalheim L, Uhlik MT, et al. CCM1 and CCM2 protein interactions in cell signaling: implications for cerebral cavernous malformations pathogenesis. Hum Mol Genet. 2005;14(17):2521-2531. |
Review/Other-Dx |
N/A |
To discuss the implications for cerebral cavernous malformations pathogenesis with CCM1 and CCM2 protein interactions in cell signaling. |
Here, we use co-immunoprecipitation, fluorescence resonance energy transfer and subcellular localization strategies to show that the CCM1 gene product, KRIT1, interacts with the CCM2 gene product, malcavernin/OSM. Analogous to the established interactions of CCM1 and beta1 integrin with ICAP1, the CCM1/CCM2 association is dependent upon the phosphotyrosine binding (PTB) domain of CCM2. A familial CCM2 missense mutation abrogates the CCM1/CCM2 interaction, suggesting that loss of this interaction may be critical in CCM pathogenesis. CCM2 and ICAP1 bound to CCM1 via their respective PTB domains differentially influence the subcellular localization of CCM1. Furthermore, we expand upon the established involvement of CCM2 in the p38 mitogen-activated protein kinase signaling module by demonstrating that CCM1 associates with CCM2 and MEKK3 in a ternary complex. |
4 |
75. Nikoubashman O, Di Rocco F, Davagnanam I, Mankad K, Zerah M, Wiesmann M. Prospective Hemorrhage Rates of Cerebral Cavernous Malformations in Children and Adolescents Based on MRI Appearance. AJNR Am J Neuroradiol. 2015;36(11):2177-2183. |
Review/Other-Dx |
70 children and adolescents |
To provide a morphologic classification that reflects hemorrhage rates. |
Overall, there were 199 MR imaging examinations with 1558 distinct cavernous malformation observations during a cumulative observation period of 1094.2 lesion-years. The mean hemorrhage rate of all 355 cavernous malformations was 4.5% per lesion-year. According to Kaplan-Meier survival models, Zabramski type I and II cavernous malformations had a significantly higher hemorrhage rate than type III and IV lesions. The presence of acute or subacute blood-degradation products was the strongest indicator for an increased hemorrhage risk (P = .036, Cox regression): The mean annual hemorrhage rate and mean hemorrhage-free interval for cavernous malformations with and without signs of acute or subacute blood degradation products were 23.4% and 22.6 months and 3.4% and 27.9 months, respectively. Dot-sized cavernous malformations, visible in T2* and not or barely visible in T1WI and T2WI sequences, had a mean annual hemorrhage rate of 1.3% and a mean hemorrhage-free interval of 37.8 months. |
4 |
76. Lynch JK, Pavlakis S, Deveber G. Treatment and prevention of cerebrovascular disorders in children. Curr Treat Options Neurol. 2005;7(6):469-480. |
Review/Other-Dx |
N/A |
To review the treatment and prevention of cerebrovascular disorders in children. |
No results stated in abstract. |
4 |
77. Hedlund GL.. Cerebral sinovenous thrombosis in pediatric practice. [Review]. Pediatr Radiol. 43(2):173-88, 2013 Jan. |
Review/Other-Dx |
N/A |
To highlight pertinent development of the cerebral venous system, discuss the causal factors of cerebral sinovenous thrombosis in the pediatric population, review practical imaging strategies using cranial sonography augmented with color and pulsed Doppler, unenhanced brain CT, CT venography, cerebral MRI, and MR venography (MRV). And to illustrate the imaging features of sinovenous thrombosis, including a discussion of the common causes of false-positive and false-negative CT and MRI studies. |
No results stated in abstract. |
4 |
78. Lynch JK. Cerebrovascular disorders in children. Curr Neurol Neurosci Rep. 2004;4(2):129-138. |
Review/Other-Dx |
N/A |
To review cerebrovascular disorders in children. |
No results stated in abstract. |
4 |
79. Moharir MD, Shroff M, Stephens D, et al. Anticoagulants in pediatric cerebral sinovenous thrombosis: a safety and outcome study. Ann Neurol. 2010;67(5):590-599. |
Observational-Tx |
162 pediatric patients |
To assess the safety and outcome of anticoagulant therapy (ACT) in pediatric cerebral sinovenous thrombosis (CSVT). |
Among 162 pediatric patients, 85 received ACT at diagnosis, including 29/83 (35%) neonates and 56/79 (71%) children. Major hemorrhage occurred in 6% (6/99) of treated patients, including 14% (3/21 neonates, 2/15 children) with and 2% (0/17 neonates, 1/46 children) without pretreatment ICH. ACT-associated bleeds were all nonfatal, and clinical outcome was favorable in 50%, similar to the remaining patients (53%). Early follow-up imaging demonstrated thrombus propagation in 11/57 neonates (10/35 [28%] without and 1/22 [4%] with ACT [p = 0.037]) and 10/63 children (7/19 [37%] without and 3/44 [7%] with ACT [p = 0.006]). Propagation was associated with new venous infarcts in 10% neonates and 40% children and worse clinical outcome in children (p = 0.053). Recanalization occurred earlier and more completely in neonates (p = 0.002). Clinical outcome was unfavorable in 47%. |
1 |
80. Bracken J, Barnacle A, Ditchfield M. Potential pitfalls in imaging of paediatric cerebral sinovenous thrombosis. Pediatr Radiol. 2013;43(2):219-231. |
Review/Other-Dx |
N/A |
To present a pictorial essay of the pitfalls of diagnosis of CSVT on CT and MRI. |
No results stated in abstract. |
4 |
81. Sari S, Verim S, Hamcan S, et al. MRI diagnosis of dural sinus - Cortical venous thrombosis: Immediate post-contrast 3D GRE T1-weighted imaging versus unenhanced MR venography and conventional MR sequences. Clin Neurol Neurosurg. 134:44-54, 2015 Jul. |
Observational-Dx |
30 patients |
To compare the diagnostic value of contrast-enhanced 3D GRE T1-weighted sequences with unenhanced MR venography and conventional magnetic resonance imaging (MRI), in detection of dural venous sinus (DVS) and cortical venous thrombosis; secondary aim is to determine the relationship between DVS thrombosis/site and gender, age, infarction or hemorrhage. |
Final diagnosis of cortical venous and/or dural sinus thrombosis was established in 24 (80%) of 30 cases and 67 (22.3%) out of 300 segments. For detection of the thrombotic segment, sensitivity, specificity, and accuracy were 83.6%, 95.3%, and 92.7% by conventional MR sequences, 89.6%, 91.8%, and 91.3% byunenhanced MR venography, and 92.5%, 100%, and 98.3% by contrast-enhanced 3D GRE T1-weighted sequence, respectively. Infarction and hemorrhage were more frequent in cases with cortical venous thrombosis, while gender and age had no significant relation with DVS thrombosis or its site. ConventionalMR sequences and unenhanced MR venography were helpful due to additional information they provided in some cases with isolated cortical venous thrombosis, with hyperintense thrombus material and with associated hemorrhage or infarction. |
2 |
82. Ohene-Frempong K, Weiner SJ, Sleeper LA, et al. Cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood. 1998;91(1):288-294. |
Review/Other-Dx |
4,082 patients |
To describe the rates and risk factors of cerebrovascular accidents in sickle cell disease. |
Patients were followed for an average of 5.2 +/- 2.0 years. Age-specific prevalence and incidence rates of CVA in patients with the common genotypes of sickle cell disease were determined, and the effects of hematologic and clinical events on the risk of CVA were analyzed. The highest rates of prevalence of CVA (4.01%) and incidence (0.61 per 100 patient-years) were in sickle cell anemia (SS) patients, but CVA occurred in all common genotypes. The incidence of infarctive CVA was lowest in SS patients 20 to 29 years of age and higher in children and older patients. Conversely, the incidence of hemorrhagic stroke in SS patients was highest among patients aged 20 to 29 years. Across all ages the mortality rate was 26% in the 2 weeks after hemorrhagic stroke. No deaths occurred after infarctive stroke. Risk factors for infarctive stroke included prior transient ischemic attack, low steady-state hemoglobin concentration and rate of and recent episode of acute chest syndrome, and elevated systolic blood pressure. Hemorrhagic stroke was associated with low steady-state hemoglobin and high leukocyte count. |
4 |
83. Sheehan VA, Hansbury EN, Smeltzer MP, Fortner G, McCarville MB, Aygun B. Transcranial Doppler velocity and brain MRI/MRA changes in children with sickle cell anemia on chronic transfusions to prevent primary stroke. Pediatr Blood Cancer. 2013;60(9):1499-1502. |
Observational-Tx |
27 children |
To analyze serial pre-transfusion TCD measurements, laboratory values including %HbS, and brain MRI/MRA findings in a consecutive cohort of children with SCA receiving chronic transfusion therapy to prevent primary stroke.. |
For the whole cohort, the average pre-transfusion HbS on transfusions was 31.7 +/- 12.3%. The most significant decline in TCD velocities occurred within 10 months of starting transfusions. Follow-up TCD values trended upward with increasing pre-transfusion %HbS levels while on treatment. Half of the children had persistent conditional/abnormal TCD velocities despite transfusions and 28% had new/progressive stenosis on MRA, but none had primary stroke during 73 patient-years of follow-up. |
3 |
84. Moser FG, Miller ST, Bello JA, et al. The spectrum of brain MR abnormalities in sickle-cell disease: a report from the Cooperative Study of Sickle Cell Disease. AJNR Am J Neuroradiol. 1996;17(5):965-972. |
Review/Other-Dx |
312 children |
To define the spectrum of abnormalities in sickle-cell disease, including infarction, atrophy, and hemorrhage, that are identified by brain MR imaging. |
Seventy patients (22%) had infarction/ischemia and/or atrophy, infarction/ischemia was noted in 39 children (13%) who had no history of a stroke (the "silent" group). The prevalence rates for silent lesions were 17% for sickle-cell anemia and 3% for hemoglobin sickle-cell disease. For patients with sickle-cell anemia and a history of cerebrovascular accident, infarction/ischemia lesions typically involved both cortex and deep white matter, while silent lesions usually were confined to deep white matter. Within the age range studied, the prevalence of infarction/ischemia did not increase significantly with age, although older patients with lesions had more lesions than did younger patients with lesions. |
4 |
85. Helton KJ, Adams RJ, Kesler KL, et al. Magnetic resonance imaging/angiography and transcranial Doppler velocities in sickle cell anemia: results from the SWiTCH trial. Blood. 124(6):891-8, 2014 Aug 07. |
Observational-Dx |
161 patients |
To evaluate if cerebrovascular disease by magnetic resosnance angiography (MRA) and (transcranial Doppler) USTCD would correlate with MRI abnormalities. |
A novel MRA vasculopathy grading scale demonstrated frequent severe baseline left/right vessel stenosis (53%/41% >/=Grade 4); 31% had no vessel stenosis on either side. Baseline parenchymal injury was prevalent (85%/79% subcortical, 53%/37% cortical, 50%/35% subcortical and cortical). Most children had low or uninterpretable baseline middle cerebral artery TCD velocities, which were associated with worse stenoses (incidence risk ratio [IRR] = 5.1, P </= .0001 and IRR = 4.1, P < .0001) than normal velocities; only 2% to 12% had any conditional/abnormal velocity. Patients with adjudicated stroke (7) and transient ischemic attacks (19 in 11 standard/8 alternative arm subjects) had substantial parenchymal injury/vessel stenosis. At exit, 1 child (alternative arm) had a new silent infarct, and another had worse stenosis. |
1 |
86. Adams RJ, McKie VC, Hsu L, et al. Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography. N Engl J Med. 1998;339(1):5-11. |
Experimental-Tx |
130 children |
To prevent first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial doppler ultrasonography |
A total of 130 children (mean [+/-SD] age, 8.3+/-3.3 years) were enrolled; 63 were randomly assigned to receive transfusions and 67 to receive standard care. At base line, the transfusion group had a slightly lower mean hemoglobin concentration (7.2 vs. 7.6 g per deciliter, P=0.001) and hematocrit (20.4 vs. 21.7 percent, P=0.002). Ten patients dropped out of the transfusion group, and two patients crossed over from the standard-care group to the transfusion group. There were 10 cerebral infarctions and 1 intracerebral hematoma in the standard-care group, as compared with 1 infarction in the transfusion group -- a 92 percent difference in the risk of stroke (P<0.001). This result led to the early termination of the trial. |
1 |
87. American College of Radiology. ACR Appropriateness Criteria® Radiation Dose Assessment Introduction. Available at: https://www.acr.org/-/media/ACR/Files/Appropriateness-Criteria/RadiationDoseAssessmentIntro.pdf. |
Review/Other-Dx |
N/A |
To provide evidence-based guidelines on exposure of patients to ionizing radiation. |
No abstract available. |
4 |