Intracranial Arteriovenous Shunts Research Articles

Use of levosimendan in hemodynamic management of heart failure in two neonates with intracranial arteriovenous shunts: a case series

BackgroundThe hemodynamic status of newborns with intracranial arteriovenous shunts (AVSs) may be extremely complex. Mini-invasive hemodynamic monitoring through innovative techniques such as Near-Infrared Spectroscopy (NIRS) and Pressure Recording Analytical Method (PRAM) may help in understanding hemodynamics in newborns with AVSs. Levosimendan is a calcium sensitizer and inodilator, and it is known to improve ventricular function, but its use in newborns is limited. In our cases, we evaluated the effect of levosimendan on hemodynamics through NIRS and PRAM.Case presentationHerein, we report the cases of two neonates with intracranial arteriovenous shunts, in whom we used levosimendan to manage cardiac failure refractory to conventional treatment. Levosimendan was used at a dosage of 0.1 mcg/kg/min for 72 h. Combined use of NIRS and PRAM helped in real-time monitoring of hemodynamic effects; in particular, levosimendan determined significant improvement in myocardium contractility as well as a reduction of heart rate.ConclusionIn two neonatal cases of AVSs, levosimendan led to an overall hemodynamic stabilization, documented by the combination of NIRS and PRAM. Our results suggest introducing levosimendan as a second-line treatment in cases of severe cardiac dysfunction due to AVSs without improvement using standard treatment strategies. Future prospective and larger studies are highly warranted.

Intracranial arteriovenous shunts in infants: A decade of experience from a quaternary pediatric center.

Intracranial arteriovenous shunts (IAVS) are rare vascular diseases in infants. They can be categorized into vein of Galen aneurysmal malformation (VGAM), pial arteriovenous fistula (PAVF), and dural arteriovenous fistula associated with dural sinus malformation (DAVF/DSM). We sought to review the clinical presentation, imaging characteristics, endovascular treatment (EVT), and outcomes of IAVS in infants presenting to a quaternary pediatric referral center over one decade. A retrospective review of a prospectively maintained database was performed of all infants diagnosed with IAVS between January 2011 and January 2021 in a quaternary pediatric referral center. For each patient, demographic data, clinical presentation, imaging findings, management strategies, and outcomes were reviewed and discussed. Over the study period, 38 consecutive infants were diagnosed with IAVS. Patients with VGAM (23/38, 60.5%) presented with congenital heart failure (CHF) (14/23), hydrocephalus (4/23), and seizures (2/23), and three patients were asymptomatic. Eighteen patients with VGAM underwent EVT. Among those, 13 patients (72.2%) were successfully treated with an angiographic cure and three patients (3/18, 17%) died. Patients with PAVF (9/38, 23.7%) presented with CHF (5/9), intracranial hemorrhage (2/9), and seizures (2/9), and all of them were successfully treated endovascularly. Patients with Type I DAVF/DSM (4/6, 66.6%) presented with mass effect (2/4), cerebral venous hypertension (1/4), CHF (1/4), and cerebrofacial venous metameric syndrome (1/4). Patients with type II DAVF/DSM (2/6, 33.3%) presented with a thrill behind the ear. Patients with DAVF/DSM were treated endovascularly, five patients were cured, and one with type I DAVF/DSM died. Intracranial arteriovenous shunts are rare but potentially life-threatening neurovascular pathologies in infants. Endovascular treatment is challenging but feasible in carefully selected patients.

Neurodevelopmental and genetic findings in neonates with intracranial arteriovenous shunts: A case series.

Despite the latest advances in prenatal diagnosis and postnatal embolization procedures, intracranial arteriovenous shunts (AVSs) are still associated with high mortality and morbidity rates. Our aim was to evaluate the presentation and clinical course, the neurodevelopmental outcome, and the genetic findings of neonates with AVSs. In this retrospective observational study, medical records of neonates with cerebral AVSs admitted to our hospital from January 2020 to July 2022 were revised. In particular, we evaluated neuroimaging characteristics, endovascular treatment, neurophysiological features, neurodevelopmental outcomes, and genetic findings. We described the characteristics of 11 patients with AVSs. Ten infants (90.9%) required embolization during the first three months of life. In 5/9 infants, pathological electroencephalography findings were observed; of them, two patients presented seizures. Eight patients performed Median Nerve Somatosensory Evoked Potentials (MN-SEPs): of them, six had an impaired response. We found normal responses at Visual Evoked Potentials and Brainstem Auditory Evoked Potentials. Eight patients survived (72.7%) and were enrolled in our multidisciplinary follow-up program. Of them, 7/8 completed the Bayley-III Scales at 6 months of corrected age: none of them had cognitive and language delays; conversely, a patient had a moderate delay on the Motor scale. The remaining survivor patient developed cerebral palsy and could not undergo Bayley-III evaluation because of the severe psychomotor delay. From the genetic point of view, we found a novel pathogenic variant in the NOTCH3 gene and three additional genomic defects of uncertain pathogenicity. We propose SEPs as an ancillary test to discern the most vulnerable infants at the bedside, particularly to identify possible future motor impairment in follow-up. The early identification of a cognitive or motor delay is critical to intervene with personalized rehabilitation treatment and minimize future impairment promptly. Furthermore, the correct interpretation of identified genetic variants could provide useful information, but further studies are needed to investigate the role of these variants in the pathogenesis of AVSs.

Diagnostic performance of dynamic 3D magnetic resonance angiography in daily practice for the detection of intracranial arteriovenous shunts in patients with non-traumatic intracranial hemorrhage.

Identification of treatable causes of intracranial hemorrhage (ICH) such as intracranial arteriovenous shunt is crucial to prevent recurrence. However, diagnostic approaches vary considerably across centers, partly because of limited knowledge of the diagnostic performance of first-line vascular imaging techniques. We assessed the diagnostic performance of dynamic three-dimensional magnetic resonance angiography (dynamic 3D MRA) in daily practice to detect intracranial arteriovenous shunts in ICH patients against subsequent digital subtraction angiography (DSA) as reference standard. We reviewed all adult patients who underwent first-line dynamic 3D MRA and subsequent DSA for non-traumatic ICH between January 2016 and September 2021 in a tertiary center. Sensitivity, specificity, accuracy, positive and negative predictive values of dynamic 3D MRA for the detection of intracranial arteriovenous shunt were calculated with DSA as reference standard. Among 104 included patients, 29 (27.9%) had a DSA-confirmed arteriovenous shunt [19 pial arteriovenous malformations, 10 dural arteriovenous fistulae; median onset-to-DSA: 17 (IQR: 3-88) days]. The sensitivity and specificity of dynamic 3D MRA [median onset-to-dynamic 3D MRA: 14 (3-101) h] for the detection of intracranial arteriovenous shunt were 66% (95% CI: 48-83) and 91% (95% CI: 84-97), respectively. The corresponding accuracy, positive and negative predictive values were 84% (95% CI: 77-91), 73% (95% CI: 56-90), and 87% (95% CI: 80-95), respectively. This study suggests that although first-line evaluation with dynamic 3D MRA may be helpful for the detection of intracranial arteriovenous shunts in patients with ICH, additional vascular imaging work-up should not be withheld if dynamic 3D MRA is negative. Comparative prospective studies are needed to determine the best imaging strategy to diagnose arteriovenous shunts after non-traumatic ICH.

Hemorrhage and Recurrence of Obliterated Brain Arteriovenous Malformations Treated With Stereotactic Radiosurgery.

Although complete nidal obliteration of brain arteriovenous malformations (AVM) is generally presumed to represent durable cure, postobliteration hemorrhage, and AVM recurrence have become increasingly recognized phenomena. The goal of the study was to define hemorrhage and nidal recurrence risks of obliterated AVMs treated with stereotactic radiosurgery (SRS). This is a retrospective cohort study from the International Radiosurgery Research Foundation comprising AVM patients treated between 1987 and 2020. Patients with AVM obliteration on digital subtraction angiography (DSA) were included. Outcomes were (1) hemorrhage and (2) AVM recurrence. Follow-up duration began at the time of AVM obliteration and was censored at subsequent hemorrhage, AVM recurrence, additional AVM treatment, or loss to follow-up. Annualized risk and survival analyses were performed. A sensitivity analysis comprising patients with AVM obliteration on magnetic resonance imaging or DSA was also performed for postobliteration hemorrhage. The study cohort comprised 1632 SRS-treated patients with AVM obliteration on DSA. Pediatric patients comprised 15% of the cohort, and 42% of AVMs were previously ruptured. The mean imaging follow-up after AVM obliteration was 22 months. Among 1607 patients with DSA-confirmed AVM obliteration, 16 hemorrhages (1.0%) occurred over 2223 patient-years of follow-up (0.72%/y). Of the 1543 patients with DSA-confirmed AVM obliteration, 5 AVM recurrences (0.32%) occurred over 2071 patient-years of follow-up (0.24%/y). Of the 16 patients with postobliteration hemorrhage, AVM recurrence was identified in 2 (12.5%). In the sensitivity analysis comprising 1939 patients with post-SRS AVM obliteration on magnetic resonance imaging or DSA, 16 hemorrhages (0.83%) occurred over 2560 patient-years of follow-up (0.63%/y). Intracranial hemorrhage and recurrent arteriovenous shunting after complete nidal obliteration are rare in AVM patients treated with SRS, and each phenomenon harbors an annual risk of <1%. Although routine postobliteration DSA cannot be recommended to SRS-treated AVM patients, long-term neuroimaging may be advisable in these patients.

Paediatric intracranial dural arteriovenous shunts: types, clinical presentation and therapeutic management.

Paediatric intracranial dural arteriovenous shunts have clinical presentations and evolutions, with angiographic characteristics that differ from those described in adults. We report our experience concerning their therapeutic management, emphasizing the relevance of early diagnosis and appropriate treatment for satisfactory neurocognitive development. Using a prospective database, we reviewed the clinical and radiological data of all children with dural arteriovenous shunts managed between 2002 and 2020. Dural shunts were categorized into three types: dural sinus malformations with arteriovenous shunts; infantile dural arteriovenous shunts; and adult-type dural arteriovenous shunts. Therapeutic strategies and outcomes were analysed depending on lesional subtypes. Modified Rankin Scale for the paediatric population was assessed pre-treatment and at last follow-up. Twenty-eight patients [16 girls (57.1%); 12 boys (42.9%)] were included: 17 dural sinus malformation [10 boys (58.8%); seven girls (41.2%)], three infantile shunts [three girls (100%)], eight adult-type shunts [four girls (50%)]; four boys (50%)], with a mean age of 19.2 ± 36.6 months at presentation. Twelve (42.9%) had a modified Rankin Scale score of 0–2, four (14.3%) had a score of 3, three (10.7%) had a score of 4 and eight (28.6%) had a score of 5. Embolization was performed in 22 children [78.6%; 12 girls (54.5%); 10 boys (45.5%)]. Fifteen patients could be cured (68.2%): 11 dural sinus malformations (73.3%), four adult-type lesions (100%) but no infantile shunt. Mean post-treatment follow-up was 39.5 months (max. 139 months): 14 patients (63.6%) presented a modified Rankin Scale score of 0–2 and eight (36.4%) had a score ≥3. In the dural sinus malformation group, the modified Rankin Scale score was improved in 11 patients (73.3%) and unchanged in three (20%). Only one patient with infantile subtype (33.3%) improved clinically. In the adult-subtype group, all children (100%) improved. Of six untreated patients [four girls (66.7%); two boys (33.3%)], four with adult-subtype shunts showed uneventful evolutions, one with dural sinus malformation died, and therapeutic abortion was conducted in an antenatally diagnosed dural sinus malformation. Paediatric dural fistulas comprise different subtypes with variable clinical courses. Proper diagnosis is mandatory for optimal therapeutic strategies within appropriate therapeutic windows.

????The diagnostic performance of the combination of ASL and TOF MRA for the cerebral arteriovenous shunt

Objective: To evaluate the diagnostic performance of the combination of arterial spin labeling (ASL) and time of flight MR angiography (TOF MRA) for intracranial arteriovenous shunt (AVS) detection. Methods: A total of 39 patients with known or suspected with cerebrovascular malformations underwent digital subtraction angiography (DSA) and ASL/TOF MRA imaging in Department of neurosurgery and radiology and nuclear medicine, Xuanwu Hospital from May 1, 2020 to October 31, 2020 were retrospectively analyzed. Patients were divided into either acute cerebral hemorrhage group (n=13) or non-acute cerebral hemorrhage group (n=26) based on the signs of bleeding on imaging findings. According to history of treatment, those patients were divided into treated (n=11) and untreated (n=28) subgroups. The determination of the presence of AVS on images was judged by two radiologists in a blinded and randomized order fashion. The diagnostic performance of ASL or TOF MRA for AVS were evaluated in overall, acute cerebral hemorrhage subgroup and treated subgroup by using the area under receiver operating curve (AUC) with DSA as the reference standard, respectively. The κ coefficients were calculated to determine the interobserver agreement. Results: Among 39 patients, 29 patients were confirmed with AVS by DSA while 10 patients with no AVS. Interobserver agreement was good-excellent (κ=0.83-1.00). In patients with AVM, the detection rates for AVS of ASL or TOF MRA were 93.1% and 86.2% respectively, while the detection rates of the combination of ASL and TOF MRA were 100%. The AUC of ASL, TOF MRA and their combination for diagnosis of AVS in overall were 0.966 (95%CI: 0.909-1.00), 0.914 (95%CI: 0.825-1.00) and 0.983 (95%CI:0.943-1.00), respectively. The AUC of ASL, TOF MRA and their combination for AVS in acute cerebral hemorrhage subgroup were 1(95%CI:1.00-1.00), 0.833(95%CI:0.611-1.00), 1(95%CI:1.00-1.00), respectively. Conclusion: Combination of ASL and TOF MRA can be a non-invasive thchnique for the detection of AVS.

Trends in Incidence of Intracranial and Spinal Arteriovenous Shunts: Hospital-Based Surveillance in Okayama, Japan.

To date, the incidence of intracranial and spinal arteriovenous shunts has not been thoroughly investigated. We aimed to clarify recent trends in the rates of intracranial and spinal arteriovenous shunts in Japan. We conducted multicenter hospital-based surveillance at 8 core hospitals in Okayama Prefecture between April 1, 2009 and March 31, 2019. Patients who lived in Okayama and were diagnosed with cerebral arteriovenous malformations, dural arteriovenous fistulas (DAVFs), or spinal arteriovenous shunts (SAVSs) were enrolled. The incidence and temporal trends of each disease were calculated. Among a total of 393 cranial and spinal arteriovenous shunts, 201 (51.1%) cases of DAVF, 155 (39.4%) cases of cerebral arteriovenous malformation, and 34 (8.7%) cases of SAVS were identified. The crude incidence rates between 2009 and 2019 were 2.040 per 100 000 person-years for all arteriovenous shunts, 0.805 for cerebral arteriovenous malformation, 1.044 for DAVF, and 0.177 for SAVS. The incidence of all types tended to increase over the decade, with a notable increase in incidence starting in 2012. Even after adjusting for population aging, the incidence of nonaggressive DAVF increased 6.0-fold while that of SAVS increased 4.4-fold from 2010 to 2018. In contrast to previous studies, we found that the incidence of DAVF is higher than that of cerebral arteriovenous malformation. Even after adjusting for population aging, all of the disease types tended to increase in incidence over the last decade, with an especially prominent increase in SAVSs and nonaggressive DAVFs. Various factors including population aging may affect an increase in DAVF and SAVS.

高解像度血管撮影装置による中枢性血管性疾患の血管解剖の分析

高解像度血管撮影装置による中枢性血管性疾患の血管解剖の分析

Evaluation of cerebral arteriovenous shunts: a comparison of parallel imaging time-of-flight magnetic resonance angiography (TOF-MRA) and compressed sensing TOF-MRA to digital subtraction angiography

Time-of-flight (TOF)-MR angiography (MRA) is an important imaging sequence for the surveillance and analysis of cerebral arteriovenous shunt (AVS), including arteriovenous malformation (AVM) and arteriovenous fistula (AVF). However, this technique has the disadvantage of a relatively long scan time. The aim of this study was to compare diagnostic accuracy between compressed sensing (CS)-TOF and conventional parallel imaging (PI)-TOF-MRA for detecting and characterizing AVS. This study was approved by the institutional review board for human studies. Participants comprised 56 patients who underwent both CS-TOF-MRA and PI-TOF-MRA on a 3-T MR unit with or without cerebral AVS between June 2016 and September 2018. Imaging parameters for both sequences were almost identical, except the acceleration factor of 3× for PI-TOF-MRA and 6.5× for CS-TOF-MRA, and the scan time of 5min 19s for PI-TOF-MRA and 2min 26s for CS-TOF-MRA. Two neuroradiologists assessed the accuracy of AVS detection on each sequence and analyzed AVS angioarchitecture. Concordance between CS-TOF, PI-TOF, and digital subtraction angiography was calculated using unweighted and weighted kappa statistics. Both CS-TOF-MRA and PI-TOF-MRA yielded excellent sensitivity and specificity for detecting intracranial AVS (reviewer 1, 97.3%, 94.7%; reviewer 2, 100%, 100%, respectively). Interrater agreement on the angioarchitectural features of intracranial AVS on CS-MRA and PI-MRA was moderate to good. The diagnostic performance of CS-TOF-MRA is comparable to that of PI-TOF-MRA in detecting and classifying AVS with a reduced scan time under 2.5min.

Hypointensity of draining veins on susceptibility-weighted magnetic resonance images might indicate normal venous flow and a lower risk of intracerebral hemorrhage in patients with intracranial arteriovenous shunt(s)

Patients with intracranial arteriovenous shunt(s) have a risk of intracerebral hemorrhage (ICH). We investigated the signal intensity of draining veins on susceptibility-weighted imaging (SWI) and the status of venous drainage shown by digital subtraction angiography (DSA). We then evaluated whether the signal intensity of draining veins on SWI is related to normal venous flow (NVF) and/or ICH. We analyzed SWI and DSA in 10 consecutive patients with intracranial arteriovenous shunt(s). Opacification of draining veins in the normal venous phase by DSA was judged as NVF. We evaluated the relationship between the intensity of draining veins on SWI and the presence of NVF before and after treatment. The relationship between the intensity of draining veins on SWI and the presence of ICH surrounding the draining veins was also evaluated. Of 10 patients with untreated arteriovenous shunt(s), two had arteriovenous malformation and eight had a dural arteriovenous fistula with cortical venous reflux. We analyzed 26 draining veins before treatment. In preoperative analysis, draining veins with hypointensity were significantly more likely to show NVF than were draining veins with isointensity or hyperintensity (45.5% vs. 0.0%, P = 0.007). While 69.2% of the areas surrounding draining veins with isointensity or hyperintensity showed ICH, no veins with hypointensity showed ICH (P = 0.011, odds ratio 0.036; 95% confidence interval 0.0017–0.80). In conclusion, draining veins with hypointensity on SWI may contain NVF, despite arteriovenous shunting. The areas surrounding these veins might have a lower risk of ICH because of less venous hypertension.

Left temporal hemorrhage caused by cerebral venous reflux of a brachio-brachial hemodialysis fistula.

Central vein disease (CVD) is a well-known complication of central venous cannulations, indwelling dialysis catheters, and arteriovenous grafts. Brachiocephalic vein (BCV) stenosis or thrombotic occlusion can occur in dialysis patients, and the presence of an ipsilateral arteriovenous fistula can cause cerebral venous hypertension due to retrograde flow in the ipsilateral jugular vein. A 53-year-old man receiving hemodialysis (left brachiocephalic hemodialysis fistula) presented with impaired consciousness and seizures related to status epilepticus due to left temporal multifocal hemorrhages. Brain computed tomography and angiogram showed left cortical vein congestion without intracranial arteriovenous shunt. Complementary left brachial angiogram showed a left BCV stenosis and jugular and cerebral high-flow venous reflux with cortical venous reflux from the hemodialysis fistula. The left arm shunt resulted in severe cerebral venous hypertension due to ipsilateral stenosis of the BCV. BCV angioplasty immediately resolved the cerebral reflux. Patients with hemodialysis fistulas are at a higher risk of developing these intracerebral hemorrhage complications.

Pediatric intracranial arteriovenous shunts: Advances in diagnosis and treatment.

Pediatric intracranial arteriovenous shunts (IAVSs) comprise a wide range of lesions, including pial arteriovenous malformation (AVM) and arteriovenous fistula (AVF), dural arteriovenous fistula (DAVF) and vein of Galen aneurysmal malformation (VGAM). We provide an overview of pediatric IAVSs, encompassing both diagnosis and endovascular treatment. We include references from pertinent literature and representative cases from our hospital. Pediatric IAVS are frequently associated two broader conditions, such as capillary malformation-arteriovenous malformation (CM-AVM) and hereditary hemorrhagic telangiectasia (HHT). These conditions and the associated genetic mutations have only recently been described. Their impact on the brain will be different in the prenatal period, in neonates, in infants, and in children, with variable symptoms according to each age group and the current strategies of endovascular treatment in the management of these vascular lesions will be discussed. This review could improve the understanding of pediatric IAVSs and their diagnosis and treatment.

Endovascular Treatment for Pediatric Intracranial and Spinal Arteriovenous Shunt Disease

Endovascular Treatment for Pediatric Intracranial and Spinal Arteriovenous Shunt Disease

High sensitivity and specificity of 4D-CTA in the detection of cranial arteriovenous shunts

PurposeIn a prospective cohort study, we evaluated the diagnostic accuracy of time-resolved CT angiography (4D-CTA) compared to digital subtraction angiography (DSA) for detecting cranial arteriovenous shunts.Material and methodsPatients were enrolled if a DSA had been ordered querying either a dural arteriovenous fistula (dAVF) or a cerebral arteriovenous malformation (bAVM). After enrolment, both a DSA and a 4D-CTA were performed. Both studies were evaluated using a standardized form. If a dAVF or bAVM was found, its classification, angioarchitectural details, and treatment options were recorded.ResultsNinety-eight patients were enrolled and 76 full datasets were acquired. DSA demonstrated a shunting lesion in 28 out of 76 cases (prevalence 37%). 4D-CTA demonstrated all but two of these lesions (sensitivity of 93%) and produced one false positive (specificity of 98%). These numbers yielded a positive predictive value (PPV) of 96% and a negative predictive value (NPV) of 96%. Significant doubt regarding the 4D-CTA diagnosis was reported in 6.6% of all cases and both false-negative 4D-CTA results were characterized by such doubt.Conclusions4D-CTA has very high sensitivity and specificity for the detection of intracranial arteriovenous shunts. Based on these results, 4D-CTA may replace DSA imaging as a first modality in the diagnostic workup in a large number of patients suspected of a cranial dAVF or bAVM, especially if there is no doubt regarding the 4D-CTA diagnosis.Key Points• 4D-CTA was shown to have a high diagnostic accuracy and is an appropriate, less invasive replacement for DSA as a diagnostic tool for cranial arteriovenous shunts in the majority of suspected cases.• Doubt regarding the 4D-CTA result should prompt additional DSA imaging, as it is associated with false negatives.• False-positive 4D-CTA results are rare, but do exist.

Quantifying the Intra-Operative Hemodynamic Effects of Glue Embolization in Vein of Galen Malformations.

Vein of Galen malformation is a rare congenital pathological intracranial arteriovenous shunt which carries 30% risk of death before 28 days-of-age. Treatment is by high risk minimally invasive endovascular glue embolization of shunt feeding arteries under angiographic control. A tool to support intra-operative decision making would be useful. We present a novel method for visualizing angiography data to demonstrate the effect of the intervention based upon change the after embolization in the delay in time of peak contrast density relative to the injected artery and a novel method for quantifying the immediate effect of embolization on the hemodynamics of the shunt. The method is demonstrated on the angiograms of five neonates who underwent embolization. We show consistent results including a post-embolization increase in the delay in time of peak contrast density relative to the injected artery at the venous outflow in keeping with reduced shunting and redistribution of blood following embolization.

Nationwide survey of pediatric intracranial arteriovenous shunts in Japan: Japanese Pediatric Arteriovenous Shunts Study (JPAS).

The authors performed a nationwide study in Japan to evaluate the annual detected rate of pediatric intracranial arteriovenous (AV) shunts such as brain AV malformations (BAVMs), pial AV fistulas (PAVFs), vein of Galen aneurysmal malformations (VGAMs), and dural AV fistulas (DAVFs). These rates were revealed for the first time and showed that VGAM, DAVF, and PAVF were relatively common but that BAVMs were extremely rare in neonates and infants.

Non contrast, Pseudo-Continuous Arterial Spin Labeling and Accelerated 3-Dimensional Radial Acquisition Intracranial 3-Dimensional Magnetic Resonance Angiography for the Detection and Classification of Intracranial Arteriovenous Shunts.

The aim of this study was to assess the sensitivity and specificity of pseudo-continuous arterial spin labeling (PCASL) magnetic resonance angiography (MRA) with 3-dimensional (3D) radial acquisition for the detection of intracranial arteriovenous (AV) shunts. A total of 32 patients who underwent PCASL-MRA, clinical magnetic resonance imaging (MRI)/MRA exam, and digital subtraction angiography (DSA) were included in this retrospective analysis. Twelve patients presented with AV shunts. Among these were 8 patients with AV malformations (AVM) and 4 patients with AV fistulas (AVF). The clinical MRI/MRA included 3D time-of-flight MRA in all cases and time-resolved, contrast-enhanced MRA in 9 cases (6 cases with AV shunting). Research MRI and clinical MRI were independently evaluated by 2 neuroradiologists blinded to patient history. A third radiologist evaluated DSA imaging. A diagnostic confidence score was used for the presence of abnormalities associated with AV shunting (1-5). The AVMs were characterized using the Spetzler-Martin scale, whereas AVFs were characterized using the Borden classification. κ Statistics were applied to assess intermodality agreement. Compared with clinical MRA, noncontrast PCASL-MRA with 3D radial acquisition yielded excellent sensitivity and specificity for the detection of intracranial AV shunts (reader 1: 100%/100%, clinical MRA: 91.7%, 94.4%; reader 2: 91.7%/100%, clinical MRA: 91.7%/100%). Diagnostic confidence was 4.8/4.66 with PCASL-MRA and 4.25/4.66 with clinical MRA. For AVM characterization with PCASL-MRA, intermodality agreement with DSA showed κ values of 0.43 and 0.6 for readers 1 and 2, respectively. For AVF characterization, intermodality agreement showed κ values of 0.56 for both readers. Noncontrast PCASL-MRA with 3D radial acquisition is a potential tool for the detection and characterization of intracranial AV shunts with a sensitivity and specificity equivalent or higher than routine clinical MRA.

Endovascular treatment of pediatric intracranial arteriovenous shunt.

Intracranial arteriovenous shunts (ICAVS) in young children are characterized by frequent high-flow fistulas. In association with high-flow fistulas and the physiological condition of the developing brain and heart, each ICAVS type tends to present at a certain age with unique symptoms. Vein of Galen aneurysmal malformation (VGAM) and dural sinus malformation with arteriovenous (AV) shunt tend to present in the neonate with high output cardiac failure. In infancy, VGAM, pial arteriovenous fistula (AVF) and infantile dural AVF (DAVF) tend to present with hydrodynamic disorder such as macrocephaly, ventriculomegaly, prominent facial veins, and developmental delay. Pial AVF, AV malformation, and infantile DAVF can present with focal neurological signs such as seizure or hemorrhage at older ages. Endovascular treatment is currently the first choice of treatment for most pediatric ICAVS. The treatment goal should be defined on a patient-by-patient basis, according to the unique physiological condition of the child.

Intracranial Arteriovenous Shunts in Pediatrics : Clinical Presentation and Endovascular Treatment

Intracranial Arteriovenous Shunts in Pediatrics : Clinical Presentation and Endovascular Treatment