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OBJECTIVE Proton beam therapy (PBT) is an increasingly used treatment modality for pediatric patients with brain tumors. Moyamoya syndrome (MMS) is well recognized as a complication of traditional photon radiotherapy, however its association with PBT is less well described. The authors discuss their initial experience with the neurosurgical management of MMS secondary to PBT in a large-volume pediatric neurovascular service. METHODS The authors performed a retrospective case review of consecutive children referred for neurosurgical management of MMS after PBT between 2009 and 2022. Patient demographic characteristics, oncological history and treatment, interval between PBT and MMS diagnosis, and MMS management were recorded. Clinical outcome at last review was classified as good if the modified Rankin Scale (mRS) score was ≤ 2 and/or the patient attended mainstream education without additional assistance. Poor outcome was defined as mRS score ≥ 3 and/or the patient received additional educational support. The recorded radiological outcomes included angiographic analysis of stenosis, evidence of brain ischemia/infarction on MRI, and postsurgical angiographic revascularization. RESULTS Ten patients were identified. Oncological diagnosis included craniopharyngioma (n = 6), optic pathway glioma (1), ependymoma (1), Ewing sarcoma (1), and rhabdosarcoma (1). The median (interquartile range [IQR]) age at PBT was 5.1 (2.7–7.9) years. The median (IQR) age at MMS diagnosis was 7.8 (5.7–9.3) years. The median time between PBT and diagnosis of MMS was 20 (15–41) months. Six patients had poor functional status after initial oncological treatment and prior to diagnosis of MMS. All 10 patients had endocrine dysfunction, 8 had visual impairment, and 4 had behavioral issues prior to MMS diagnosis. Four patients had a perioperative ischemic event: 2 after tumor surgery, 1 after MMS surgical revascularization, and 1 after receiving a general anesthetic for an MRI scan during oncological surveillance. Seven children were treated with surgical revascularization, whereas 3 were managed medically. The incidence of ischemic events per cerebral hemisphere was reduced after surgical revascularization: only 1 patient of 7 had an ischemic event during the follow-up period after surgery. No children moved from good to poor functional status after MMS diagnosis. CONCLUSIONS MMS can occur after PBT. Magnetic resonance angiography sequences should be included in surveillance MRI scans to screen for MMS, and families should be counseled about this complication. Management at a high-volume pediatric neurovascular center, including selective use of revascularization surgery, appears to maintain functional status in these children.

Paediatric intracranial aneurysms are rare entities accounting for less than 5% of all age intracranial aneurysms. Traumatic aneurysms are more common in children and have an association with anatomical variations such as arterial fenestrations. Here, we present a case of a child initially presenting with traumatic subarachnoid haemorrhage who returned to baseline and was discharged home only to return within 2 weeks with diffuse subarachnoid and intraventricular re-haemorrhage. A dissecting aneurysm of a duplicated (fenestrated) V4 vertebral artery segment was identified as a rare cause of rebleeding. We describe a course complicated by severe vasospasm delaying aneurysm detection and treatment. Dissecting aneurysms in children should be considered in all cases of delayed post-traumatic cranial rebleeding, particularly where there is anomalous arterial anatomy.

Paediatric intracranial aneurysms are rare entities accounting for less than 5% of all age intracranial aneurysms. Traumatic aneurysms are more common in children and have an association with anatomical variations such as arterial fenestrations. Here we present a case of a child initially presenting with traumatic subarachnoid haemorrhage who returned to baseline and was discharged home only to return within 2 weeks with diffuse subarachnoid and intraventricular re-haemorrhage. A dissecting aneurysm of a duplicated (fenestrated) V4 vertebral artery segment was identified as a rare cause of rebleeding. We describe a course complicated by severe vasospasm delaying aneurysm detection and treatment. Dissecting aneurysms in children should be considered in all cases of delayed post-traumatic cranial rebleeding, particularly where there is anomalous arterial anatomy.

The aim of this study is to describe the outcome and management of all children who have presented with haemorrhagic stroke (HS) secondary to an arteriovenous malformation (AVM) at a single UK centre over a 13-year period. All children with HS managed at our institution (2005–2018) were identified and those with underlying AVMs were studied. Clinical and imaging data were obtained from medical records. Outcome was scored using the Recovery and Recurrence Questionnaire. Ninety-three children (median age 8.8 years; 56 males; 8 neonates) presented with both global and focal features (28 had Glasgow Coma Score

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

Conjoined twins are rare and pose a challenge to radiologists and surgeons. Craniopagus twins, where conjunction involves the cranium, are especially rare. Even in large pediatric centers, radiologists are unlikely to encounter more than one such event in their medical careers. This rarity makes it daunting to select a CT and MRI protocol for these infants. Using the experience of two tertiary pediatric hospitals with six sets of craniopagus twins, this multidisciplinary and multimodal integrated imaging approach highlights the key questions that need addressing in the decision-making process for possible surgical intervention.

The aim of this study is to describe the outcome and management of all children who have presented with haemorrhagic stroke (HS) secondary to an arteriovenous malformation (AVM) at a single UK centre over a 13-year period.All children with HS managed at our institution (2005-2018) were identified and those with underlying AVMs were studied. Clinical and imaging data were obtained from medical records. Outcome was scored using the Recovery and Recurrence Questionnaire.Ninety-three children (median age 8.8 years; 56 males; 8 neonates) presented with both global and focal features (28 had Glasgow Coma Score 8). Haemorrhage was intraparenchymal in 72; prior risk factors present in 14. An underlying vascular lesion was identified in 68/93, most commonly AVM (n = 48). A systemic cause was found in 10, cerebral venous thrombosis in three, and 9 remain unidentified despite neuroradiological investigation. Median follow-up was 2.4 years, six died, and one was lost to follow-up. Outcome was rated as good in 60/86. Of the 48 AVMs, 3 were Spetzler-Martin (SM) grade 1, 21 SM 2, 21 SM3 and 3 SM4. One patient was treated conservatively as the AVM was too high risk to treat. At follow-up, 19 with AVM were angiographically cured, all with low SM grade and with the use of a single modality in 9 cases (all low SM grade).Although children with acute HS are extremely unwell at presentation, supportive care results in a good outcome in the majority. Complete obliteration for childhood AVMs is challenging even with low-grade lesions with multimodal treatment.

Purpose Intra-arterial chemotherapy (IAC), delivered directly to the globe via the internal carotid artery is now an established treatment for retinoblastoma. We report a case of anterior segment ischaemia following treatment with multiple intra-arterial chemotherapy (IAC) infusions. Observations A 5 month old female presented with bilateral retinoblastoma and was treated with 12 infusions of IAC. Her right eye was enucleated at diagnosis. After her seventh IAC treatment, she developed ipsilateral sixth and third cranial nerve palsies. After the twelfth IAC, she developed an area of conjunctival and scleral ischaemia between 12 and 3 o'clock meridians in her left eye. However, she maintained visual acuity of LogMAR 0.34. Conclusions and Importance The median number of IAC treatments in large studies is three. It is possible that repeated doses of IAC have an accumulative negative effect on the ocular blood supply, risking anterior segment and neurologic sequelae. This case highlights the significant challenge of balancing the salvage of eyes and vision with the potentially significant morbidity associated with IAC.

Purpose To assess technical success of arterial recanalization in children requiring repeated arterial access and intervention. Materials and Methods Over 14 years, 41 attempts to cross 30 arterial occlusions were made in 22 patients (13 male, 9 female). Median patient age was 12 months (15 days–14 years), and weight was 7.6 kg (3.0–77.3 kg). Techniques and outcomes were recorded. Results Twenty-five of 41 (61%) attempts at crossing an arterial occlusion were successful. Nineteen of 30 (63%) first attempts to cross occlusions were successful, and 6 of 11 (55%) repeat attempts were successful. The occluded segments were combinations of common femoral artery (n = 4), external iliac artery (n = 36), common iliac artery (n = 11), and aorta (n = 1). Complications occurred in 5 of 41(12%) attempts: 3 minor complications (hematoma, extravasation, and transient leg ischemia) and 2 major complications (rupture and thrombosis). Conclusions Arterial access by recanalization of occluded segments is technically feasible in children, with a low complication rate.

Objective Describe the course and outcomes in a UK national cohort of neonates with vein of Galen malformation identified before 28 days of life. Methods Neonates with angiographically confirmed vein of Galen malformation presenting to 1 of 2 UK treatment centers (2006-2016) were included; those surviving were invited to participate in neurocognitive assessment. Results in each domain were dichotomized into "good" and "poor" categories. Cross-sectional and angiographic brain imaging studies were systematically interrogated. Logistic regression was used to explore potential outcome predictors. Results Of 85 children with neonatal vein of Galen malformation, 51 had survived. Thirty-four participated in neurocognitive assessment. Outcomes were approximately evenly split between "good" and "poor" categories across all domains, namely, neurological status, general cognition, neuromotor skills, adaptive behavior, and emotional and behavioral development. Important predictors of poor cognitive outcome were initial Bicetre score ≤ 12 and presence of brain injury, specifically white matter injury, on initial imaging; in multivariate analysis, only Bicetre score ≤ 12 remained significant. Interpretation Despite modern supportive and endovascular treatment, more than one-third of unselected newborns with vein of Galen malformation did not survive. Outcome was good in around half of survivors. The importance of white matter injury suggests that abnormalities of venous as well as arterial circulation are important in the pathophysiology of brain injury. Ann Neurol 2018;84:547-555.

Capillary malformation-arteriovenous malformation syndrome (CM-AVM) is a rare condition associated with mutations in the genes RASA1 and EPHB4. We present a challenging case of CM-AVM in a 17-month-old boy with permanent diplegia from an undiagnosed arteriovenous malformation underlying a large atypical capillary malformation over the lower thoracic spine. This case demonstrates that clinicians should have a low threshold for neuroimaging in the context of new neurologic symptoms in patients with atypical capillary malformations.

Aim To describe presentation, clinical course, and outcome in postneonatal presentations of vein of Galen malformation (VGM). Method Children older than 28 days presenting with VGM (from 2006-2016) were included. Notes/scans were reviewed. Outcome was dichotomized into 'good' or 'poor' using the Recovery and Recurrence Questionnaire. Logistic regression was performed to explore relationships between clinico-radiological features and outcome. Results Thirty-one children (18 males, 13 females) were included, presenting at a median age of 9.6 months (range 1.2mo-11y 7mo), most commonly with macrocrania (n=24) and prominent facial veins (n=9). Seven had evidence of cardiac failure. VGM morphology was choroidal in 19. Hydrocephalus (n=24) and loss of white matter volume (n=15) were the most common imaging abnormalities. Twenty-nine patients underwent glue embolization (median two per child). Angiographic shunt closure was achieved in 21 out of 28 survivors. Three children died of intracranial haemorrhage (1y, 6y, and 30d after embolization). Ten patients underwent neurosurgical procedures; to treat haemorrhage in four, and hydrocephalus in the rest. Outcome was categorized as good in 20 out of 28 survivors, but this was not predictable on the basis of the variables listed above. Interpretation Postneonatally presenting VGM has distinctive clinico-radiological features, attributable to venous hypertension. Endovascular treatment is associated with good outcomes, but more specific prognostic prediction was not possible within this cohort. What this paper adds Clinical and radiological features in older children with vein of Galen malformation relate to venous hypertension. Outcome is good in most cases with endovascular therapy. Mortality is low but is related to intracranial haemorrhage.

Background Arterio-venous malformations (AVMs) of the ear are a rare entity and their management should be decided in a dedicated multidisciplinary team (MDT) setting. The aim of this study is to describe the distinct anatomical patterns of the auricular AVMs in our unit and propose a combined interventional radiological and surgical approach. Materials and methods All consecutive patients presenting with AVMs of the ear and reviewed by the Vascular Anomalies MDT between 2014 and 2019 were included in this study. Signs, symptoms, diagnostic investigations and operative findings were collected prospectively. Results After reviewing our nine patients, we identified four anatomical patterns of auricular AVMs: I: involves just a component of the ear and should undergo embolization followed by excision and reconstruction without significant loss of form; II: affects the superior two-thirds of the ear, sparing the lobule and part of the conchal bowl; these patients should undergo embolization, excision and monitoring before formal reconstruction of the ear is offered; III: involves the entire ear and should undergo embolization and pinnectomy; if there is no recurrence, the patients can be offered either a carved-rib cartilage reconstruction or a prosthesis, depending on the quality of the surrounding soft-tissues; IV: involves the ear and surrounding tissue, making surgical management and subsequent reconstruction extensive. Conclusion The management of auricular AVMs is based on the extent of the ear involved. We feel that our combined interventional radiological and surgical approach will aid the management of these complex patients.

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.

BACKGROUND AND PURPOSE: Retinoblastoma is the most common pediatric ocular neoplasm. Multimodality treatment approaches are commonplace, and selective ophthalmic artery chemosurgery has emerged as a safe and effective treatment in selected patients. Minimizing radiation dose in this highly radiosensitive patient cohort is critical. We explore which procedural factors affect the radiation dose in a single-center cohort of children managed in the UK National Retinoblastoma Service. MATERIALS AND METHODS: A retrospective review was performed of 177 selective ophthalmic artery chemosurgery procedures in 48 patients with retinoblastoma (2013–2017). Medical records, angiographic imaging, and radiation dosimetry data (including total fluoroscopic screening time, skin dose, and dose-area product) were reviewed. RESULTS: The mean fluoroscopic time was 13.5 ± 13 minutes, the mean dose-area product was 11.7 ± 9.7 Gy.cm2, and the mean total skin dose was 260.9 ± 211.6 mGy. One hundred sixty-three of 177 procedures (92.1%) were technically successful. In 14 (7.9%), the initial attempt was unsuccessful (successful in 13/14 re-attempts). Screening time and radiation dose were associated with drug-delivery microcatheter location and patient age; screening time was associated with treatment cycle. CONCLUSIONS: In selective ophthalmic artery chemosurgery, a microcatheter tip position in the proximal or ostial ophthalmic artery and patient age 2 years or younger were associated with reduced fluoroscopic screening time and radiation dose; treatment beyond the first cycle was associated with reduced fluoroscopic screening time.

Paediatricians commonly encounter neck lumps during their routine clinical practice; vascular abnormalities, such as (pseudo)aneurysms, are a rare cause of these. Pseudoaneurysms of the carotid artery in children are usually the result of blunt or penetrating trauma, infection or vasculitis/connective tissue disorders. They can present with a variety of symptoms including neck pain, as a pulsatile neck mass or with compressive symptoms (for example, cranial nerve palsies or dyspnoea). Pseudoaneurysms carry a risk of rupture in which case they are fatal, unless immediate treatment is provided.We report a 17-month-old male child with idiopathic carotid artery blowout syndrome presenting with acute oropharyngeal haemorrhage leading to asystolic cardiac arrest. He was successfully resuscitated and emergency embolisation controlled the bleeding. Despite extensive left hemispheric infarct, he has survived.Carotid artery blowout syndrome needs to be recognised as a potential cause of major haemorrhage in childhood. The purpose of this case report is to remind readers of the differential diagnosis and work-up of a child presenting with a neck lump, to highlight important aspects of the acute management of major haemorrhage and massive blood transfusion in paediatrics, to describe the aetiology, presentation and management of carotid artery pseudoaneurysm in children and to discuss long term rehabilitation in patients with consequent neurological sequelae (including the need for input from multiple specialty teams).

Background CT perfusion (CTP) and diffusion or perfusion MRI might assist patient selection for endovascular thrombectomy. We aimed to establish whether imaging assessments of irreversibly injured ischaemic core and potentially salvageable penumbra volumes were associated with functional outcome and whether they interacted with the treatment effect of endovascular thrombectomy on functional outcome.Methods In this systematic review and meta-analysis, the HERMES collaboration pooled patient-level data from all randomised controlled trials that compared endovascular thrombectomy (predominantly using stent retrievers) with standard medical therapy in patients with anterior circulation ischaemic stroke, published in PubMed from Jan 1,2010, to May 31, 2017. The primary endpoint was functional outcome, assessed by the modified Rankin Scale (mRS) at 90 days after stroke. Ischaemic core was estimated, before treatment with either endovascular thrombectomy or standard medical therapy, by CTP as relative cerebral blood flow less than 30% of normal brain blood flow or by MRI as an apparent diffusion coefficient less than 620 mu m(2)/s. Critically hypoperfused tissue was estimated as the volume of tissue with a CTP time to maximum longer than 6 s. Mismatch volume (ie, the estimated penumbral volume) was calculated as critically hypoperfused tissue volume minus ischaemic core volume. The association of ischaemic core and penumbral volumes with 90-day mRS score was analysed with multivariable logistic regression (functional independence, defined as mRS score 0-2) and ordinal logistic regression (functional improvement by at least one mRS category) in all patients and in a subset of those with more than 50% endovascular reperfusion, adjusted for baseline prognostic variables. The meta-analysis was prospectively designed by the HERMES executive committee, but not registered.Findings We identified seven studies with 1764 patients, all of which were included in the meta-analysis. CTP was available and assessable for 591 (34%) patients and diffusion MRI for 309 (18%) patients. Functional independence was worse in patients who had CTP versus those who had diffusion MRI, after adjustment for ischaemic core volume (odds ratio [OR] 0.47 [95% CI 0.30-0.72], p=0.0007), so the imaging modalities were not pooled. Increasing ischaemic core volume was associated with reduced likelihood of functional independence (CTP OR 0.77 [0.69-0.86] per 10 mL, p(interaction)=0.29; diffusion MRI OR 0.87 [0.81-0.94] per 10 mL, p(interaction)=0.94). Mismatch volume, examined only in the CTP group because of the small numbers of patients who had perfusion MRI, was not associated with either functional independence or functional improvement. In patients with CTP with more than 50% endovascular reperfusion (n=186), age, ischaemic core volume, and imaging-to-reperfusion time were independently associated with functional improvement. Risk of bias between studies was generally low.Interpretation Estimated ischaemic core volume was independently associated with functional independence and functional improvement but did not modify the treatment benefit of endovascular thrombectomy over standard medical therapy for improved functional outcome. Combining ischaemic core volume with age and expected imagingto-reperfusion time will improve assessment of prognosis and might inform endovascular thrombectomy treatment decisions. Copyright (C) 2018 Elsevier Ltd. All rights reserved.

This is the eighth case report of a pediatric dissecting posterior inferior cerebellar artery aneurysm. The authors present the case of a 13-year-old boy who presented with posttraumatic posterior fossa subdural, subarachnoid, and intraventricular hemorrhage with hydrocephalus. Initial vascular imaging findings were negative; however, a high level of suspicion is necessary. The aneurysm was identified on day 20, after recurrence of hydrocephalus, and was treated with endovascular vessel sacrifice. The patient made a good recovery. It is important to consider arterial dissection in pediatric traumatic brain injury, especially with suspicious findings on initial CT scan and clinical presentation out of proportion to the mechanism of injury. Delayed vascular imaging is imperative for appropriate management.

Background and purpose Complete surgical resection of cervical spine tumors is often challenging when there is tumor encasement of major neck vessels. Pre-operative endovascular sacrifice of the major vessels can facilitate safe tumor resection. The use of transarterial detachable coils has been described in this setting, but it can be time-consuming and costly to occlude a patent parent vessel using this method. Our aim was to evaluate the safety and effectiveness of our endovascular detachable balloon occlusion technique, performed without prior balloon test occlusion in the pre-operative management of these tumors. Methods We retrospectively reviewed 18 consecutive patients undergoing pre-operative unilateral permanent endovascular balloon occlusion of tumor-encased vertebral arteries in our institution. Procedure-related ischemic or thromboembolic complication was defined as focal neurologic deficit attributable to the endovascular occlusion which occurs before subsequent surgical resection. Results Successful pre-operative endovascular vertebral artery sacrifice using detachable balloons was achieved in 100% ( n = 18) of cases without prior balloon test occlusion. Procedural complication rate was 5.6% as one patient developed transient focal neurology secondary to a delayed cerebellar infarct at home on day 11 and subsequently made a full recovery. There were no cases of distal balloon migration. Complete macroscopic resection of tumor as reported by the operating surgeon was achieved in 89% of cases. Conclusion Pre-operative endovascular sacrifice of the vertebral artery using detachable balloons and without prior balloon test occlusion is a safe procedure with low complication rates and good surgeon reported rates of total resection.

BACKGROUND: General anaesthesia (GA) during endovascular thrombectomy has been associated with worse patient outcomes in observational studies compared with patients treated without GA. We assessed functional outcome in ischaemic stroke patients with large vessel anterior circulation occlusion undergoing endovascular thrombectomy under GA, versus thrombectomy not under GA (with or without sedation) versus standard care (ie, no thrombectomy), stratified by the use of GA versus standard care.METHODS: For this meta-analysis, patient-level data were pooled from all patients included in randomised trials in PuMed published between Jan 1, 2010, and May 31, 2017, that compared endovascular thrombectomy predominantly done with stent retrievers with standard care in anterior circulation ischaemic stroke patients (HERMES Collaboration). The primary outcome was functional outcome assessed by ordinal analysis of the modified Rankin scale (mRS) at 90 days in the GA and non-GA subgroups of patients treated with endovascular therapy versus those patients treated with standard care, adjusted for baseline prognostic variables. To account for between-trial variance we used mixed-effects modelling with a random effect for trials incorporated in all models. Bias was assessed using the Cochrane method. The meta-analysis was prospectively designed, but not registered.FINDINGS: Seven trials were identified by our search; of 1764 patients included in these trials, 871 were allocated to endovascular thrombectomy and 893 were assigned standard care. After exclusion of 74 patients (72 did not undergo the procedure and two had missing data on anaesthetic strategy), 236 (30%) of 797 patients who had endovascular procedures were treated under GA. At baseline, patients receiving GA were younger and had a shorter delay between stroke onset and randomisation but they had similar pre-treatment clinical severity compared with patients who did not have GA. Endovascular thrombectomy improved functional outcome at 3 months both in patients who had GA (adjusted common odds ratio (cOR) 1·52, 95% CI 1·09-2·11, p=0·014) and in those who did not have GA (adjusted cOR 2·33, 95% CI 1·75-3·10, pINTERPRETATION: Worse outcomes after endovascular thrombectomy were associated with GA, after adjustment for baseline prognostic variables. These data support avoidance of GA whenever possible. The procedure did, however, remain effective versus standard care in patients treated under GA, indicating that treatment should not be withheld in those who require anaesthesia for medical reasons.FUNDING: Medtronic.

OBJECTIVEIdiopathic intracranial hypertension (IIH) is commonly associated with venous sinus stenosis. In recent years, transvenous dural venous sinus stent (DVSS) insertion has emerged as a potential therapy for resistant cases. However, there remains considerable uncertainty over the safety and efficacy of this procedure, in particular the incidence of intraprocedural and delayed complications and in the longevity of sinus patency, pressure gradient obliteration, and therapeutic clinical outcome. The aim of this study was to determine clinical, radiological, and manometric outcomes at 3–4 months after DVSS in this treated IIH cohort.METHODSClinical, radiographic, and manometric data before and 3–4 months after DVSS were reviewed in this single-center case series. All venographic and manometric procedures were performed under local anesthesia with the patient supine.RESULTSForty-one patients underwent DVSS venography/manometry within 120 days. Sinus pressure reduction of between 11 and 15 mm Hg was achieved 3–4 months after DVSS compared with pre-stent baseline, regardless of whether the procedure was primary or secondary (after shunt surgery). Radiographic obliteration of anatomical stenosis correlating with reduction in pressure gradients was observed. The complication rate after DVSS was 4.9% and stent survival was 87.8% at 120 days. At least 20% of patients developed restenosis following DVSS and only 63.3% demonstrated an improvement or resolution of papilledema.CONCLUSIONSReduced venous sinus pressures were observed at 120 days after the procedure. DVSS showed lower complication rates than shunts, but the clinical outcome data were less convincing. To definitively compare the outcomes between DVSS and shunts in IIH, a randomized prospective study is needed.

Childhood intracranial aneurysms differ from those in the adult population in incidence and gender prevalence, cause, location, and clinical presentation. Endovascular treatment of pediatric aneurysms is the suggested approach because it offers both reconstructive and deconstructive techniques and a better clinical outcome compared with surgery; however, the long-term durability of endovascular treatment is still questionable, therefore long-term clinical and imaging follow-up is necessary. The clinical presentation, diagnosis, and treatment of intracranial aneurysms in children are discussed, and data from endovascular treatments are presented.

SummaryObjective To describe our experience with superselective ophthalmic artery chemotherapy (SOAC) in retinoblastoma and to report the serious adverse cardio-respiratory reactions we have observed. Methods SOAC was performed using a standardized protocol for general anesthesia, ophthalmic artery catheterization, and pulsed infusion of melphalan. Adverse reactions were defined as those in which the patient required active treatment to maintain cardio-respiratory stability. Results Between December 2008 and May 2012, 54 eyes in 52 patients were treated. 143 catheterization procedures were performed, with a technical success rate of 93% (n = 133). There were no deaths or major complications. Adverse cardio-respiratory reactions developed during 35 procedures (24%; 95% CI, 18–32%). All reactions occurred during second or subsequent catheterization procedures (39%; 95% CI, .29–49%) and were characterized by hypoxia, reduced lung compliance, systemic hypotension and bradycardia. Adverse events were successfully treated in all patients. One procedure was abandoned due to prolonged hemodynamic instability. Conclusion Adverse cardio-respiratory reactions are commonly observed in SOAC for retinoblastoma. We believe that the adverse clinical signs represent an autonomic reflex response, akin to the trigemino-cardiac or oculo-respiratory reflexes, and all patients should be considered at-risk. Reactions occur only during second or subsequent procedures and can be life-threatening. The routine use of intravenous atropine does not seem to have altered the incidence or severity of these reactions. Anesthetists and interventional neuroradiologists involved in SOAC must be vigilant to ensure adverse reactions, when they develop, are treated quickly and effectively.

Idiopathic intracranial hypertension (IIH) is characterised by an increased intracranial pressure (ICP) in the absence of any central nervous system disease or structural abnormality and by normal CSF composition. Management becomes complicated once surgical intervention is required. Venous sinus stenosis has been suggested as a possible aetiology for IIH. Venous sinus stenting has emerged as a possible interventional option. Evidence for venous sinus stenting is based on elimination of the venous pressure gradient and clinical response. There have been no studies demonstrating the immediate effect of venous stenting on ICP. Patients with a potential or already known diagnosis of IIH were investigated according to departmental protocol. ICP monitoring was performed for 24 h. When high pressures were confirmed, CT venogram and catheter venography were performed to look for venous stenosis to demonstrate a pressure gradient. If positive, venous stenting would be performed and ICP monitoring would continue for a further 24 h after deployment of the venous stent. Ten patients underwent venous sinus stenting with concomitant ICP monitoring. Nine out of ten patients displayed an immediate reduction in their ICP that was maintained at 24 h. The average reduction in mean ICP and pulsatility was significant (p = 0.003). Six out of ten patients reported a symptomatic improvement within the first 2 weeks. Venous sinus stenting results in an immediate reduction in ICP. This physiological response to venous stenting has not previously been reported. Venous stenting could offer an alternative treatment option in correctly selected patients with IIH.

OBJECTIVE: The Pragmatic Ischaemic Thrombectomy Evaluation (PISTE) trial was a multicentre, randomised, controlled clinical trial comparing intravenous thrombolysis (IVT) alone with IVT and adjunctive intra-arterial mechanical thrombectomy (MT) in patients who had acute ischaemic stroke with large artery occlusive anterior circulation stroke confirmed on CT angiography (CTA).DESIGN: Eligible patients had IVT started within 4.5 hours of stroke symptom onset. Those randomised to additional MT underwent thrombectomy using any Conformité Européene (CE)-marked device, with target interval times for IVT start to arterial puncture of RESULTS: Ten UK centres enrolled 65 patients between April 2013 and April 2015. Median National Institutes of Health Stroke Scale score was 16 (IQR 13-21). Median stroke onset to IVT start was 120 min. In the intention-to-treat analysis, there was no significant difference in disability-free survival at day 90 with MT (absolute difference 11%, adjusted OR 2.12, 95% CI 0.65 to 6.94, p=0.20). Secondary analyses showed significantly greater likelihood of full neurological recovery (mRS 0-1) at day 90 (OR 7.6, 95% CI 1.6 to 37.2, p=0.010). In the per-protocol population (n=58), the primary and most secondary clinical outcomes significantly favoured MT (absolute difference in mRS 0-2 of 22% and adjusted OR 4.9, 95% CI 1.2 to 19.7, p=0.021).CONCLUSIONS: The trial did not find a significant difference between treatment groups for the primary end point. However, the effect size was consistent with published data and across primary and secondary end points. Proceeding as fast as possible to MT after CTA confirmation of large artery occlusion on a background of intravenous alteplase is safe, improves excellent clinical outcomes and, in the per-protocol population, improves disability-free survival.TRIAL REGISTRATION NUMBER: NCT01745692; Results.

Purpose To report the efficacy of super-selective intra-ophthalmic artery melphalan (IAM) for the treatment of refractory retinoblastoma and any associated complications of this treatment. Design A prospective case series. Participants Eyes with retinoblastoma that had been treated with systemic chemotherapy or local therapy and had a relapse of their condition. Methods All patients receiving IAM between May 2009 and September 2010 were included in the study. Intra-ophthalmic artery melphalan was offered to patients who had failed to respond adequately to systemic chemotherapy and local treatment where appropriate or because of a new recurrence of retinoblastoma that could not be treated with local therapies. None of the patients were excluded because of central nervous system abnormalities. Patients received 2 treatments of IAM given 4 weeks apart. All patients received an orthoptic assessment 3 weeks after each treatment and an examination under anesthesia (EUA). A third treatment was given if an unsatisfactory response was observed on EUA after 2 treatments. Main Outcome Measures The response of the retinoblastoma tumor(s) and any associated local side effects from the treatment. Results A total of 15 eyes in 14 patients were treated with IAM during the study period. The mean age at the time of IAM was 31.5 months (median 17.3, range 11.2–150.7 months), and the mean follow-up was 8.7 months (3–16.3 months). Tumor control was achieved in 12 eyes (80%), and 12 eyes (80%) had local side effects that included third cranial nerve palsy in 6 (40%), orbital edema in 3 (20%), permanent retinal detachment in 1 (7%), and vitreous hemorrhage in 4 (27%). Seven eyes (47%) developed significant retinal pigment epithelium changes. Conclusions Intra-ophthalmic artery melphalan is an effective treatment for retinoblastoma, achieving a high level of remission in refractory tumors. It can be associated with significant local side effects that can result in loss of vision and possible amblyogenesis. Clinicians and parents need to consider the benefits and potential local side effects before embarking on treatment. Financial Disclosure(s) The author(s) have no proprietary or commercial interest in any materials discussed in this article.

An 8-year-old boy is currently an inpatient in your paediatric unit having had a sudden onset of left sided weakness after a roller-coaster ride. Brain MRI has shown this to be due to a right-sided pontine infarct; brain and neck magnetic resonance angiography (MRA) were normal. Assessment has not identified any risk factors for arterial ischaemic stroke (AIS). Transfer has been arranged to the tertiary centre, where the paediatric neurologists are planning to request diagnostic catheter cerebral angiography (CA) to exclude vertebral artery dissection. The child's parents are apprehensive about this and would like to know the risks of this procedure, especially as they have heard these potentially include neurological complications. In a child with suspected cerebral arteriopathy (patient) undergoing diagnostic catheter angiography (intervention), what is the risk of neurological complications (outcome)? Using the PubMed, Medline and EMBASE databases, the terms ‘CA’, ‘digital subtraction angiography’ and ‘cerebral arteriography’ were sequentially paired with the qualifiers ‘adverse effects’, ‘complications’, ‘side effects’ and ‘safety’. The results were filtered by age, to include subjects under the age of 19 years. In total this yielded 149 papers, the abstracts of which were reviewed. The majority of these featured adults and so were excluded. Papers detailing complications of interventional CA were also excluded. Four relevant studies remained, each published within the last 15 years and documenting the complications of diagnostic CA in children. We also identified several other papers which were of interest without directly contributing to our structured clinical question. These will be briefly …

A 55 year old man presented with sudden onset right sided weakness and aphasia. Computed tomography angiogram showed a left internal carotid artery occlusion and thrombus in the proximal left middle cerebral …

The optimal strategy for monitoring the stability of ruptured intracranial aneurysms following coil embolisation is unclear. The value of delayed follow-up angiography in detecting new recurrences or progression of residual lesions visualised on earlier angiographic studies was determined in the light of the increasing use of non-invasive imaging techniques such as time of flight magnetic resonance angiography (TOF-MRA) for the evaluation of intracranial aneurysm occlusion. Ninety-seven patients with 105 ruptured aneurysms treated with detachable coils in 2005 and 2006 were included. The presence of a residual neck or aneurysm was assessed on catheter angiograms performed at 6 months and 2 years using the Raymond criteria (Class I = completely occluded, class II = small residual neck, class III = aneurysm sac filling). At 6-month follow-up, 32% of class I aneurysms progressed to class II and 6% of these aneurysms required re-treatment. A further 2-year angiogram was obtained in 59 patients with 65 aneurysms. Ninety-six per cent of class I, 100% of the class II and class III aneurysms remained unchanged at 2 years compared to 6 months. In our series, most recurrences were apparent at 6-month follow-up. The vast majority of coiled ruptured aneurysms that were class I or II at 6 months remained stable at 2-year follow-up. In the absence of a residual lesion in the early angiographic study, a further delayed catheter angiogram may not be warranted. The use of non-invasive strategies such as TOF-MRA should be considered.

Patients with poor grade (World Federation of Neurosurgeons (WFNS) Grades 4 and 5) subarachnoid hemorrhage (SAH) were historically considered to have a poor neurological outcome and therefore not traditionally offered aggressive treatment. In recent years there has been increasing evidence that early aggressive treatment of this patient group can result in a good outcome. Aim of this study is to identify the outcome of patients with WFNS Grade-4 and -5 SAH treated acutely with endovascular detachable coil embolization (DCE) and aggressive neurocritical care within our institution. We retrospectively reviewed the records of patients with SAH WFNS Grades 4 and 5 treated with DCE within 7 days of admission between 1st January 2004 and 1st January 2008. Data collected included age, sex, grade SAH, position/number of Aneurysms, coiling complications, time spent on the neurosurgical critical care unit (NCCU), and 6-month outcome assessed by Glasgow outcome scale (GOS). GOS was dichotomized into good outcome (good recovery/moderate disability) and poor outcome (severe disability, vegetative, dead). A total of 193 acute SAH patients were admitted and treated within this time period, of these, 47 patients were classified as poor grade and included: 70% were female and 30% were male. The mean age was 56 years (33–88 years range). A total of 56 aneurysms were noted at angiography, 52 aneurysms were coiled. Complications of SAH Vasospasm was noted in 18 patients (38%), cerebral infarction in 13 patients (28%), seizures in 7 patients (15%), hydrocephalus in 25 patients (53%). Complications of DCE occurred in 2 patients (4% of total) these were an aneurysmal rupture and a peri-procedure thrombosis. Incomplete coiling occurred in another 5 patients (10.6% of total) due to technical difficulties. The median length of stay on the NCCU was 12 days (1–52 days range). Of the 47 poor grade patients coiled, 25 (53%) had a good outcome (good recovery/moderate disability) and 22 (47%) had a poor outcome (severe disability, vegetative, dead) by the time of the 6-month follow-up. Potentially, more than half the patients with WFNS Grade-4 and -5 SAH who are treated aggressively with coil embolization in association with supportive neurocritical care can achieve a good quality neurological outcome. However, it should be anticipated that these patients will spend a significant period of time in neurocritical care.

Sixty percent of vascular anomalies in children are found in the head and neck. These lesions can present throughout antenatal, perinatal and childhood development. They broadly fall into two categories: vascular tumours and vascular malformations. Their clinical and, often, psychological impact is determined by both pathological type and location: many lesions follow an uncomplicated natural course and other more complex, extensive or progressive lesions can present a threat to life from mass effect, haemorrhage or large volume arteriovenous shunting. Vascular tumours include infantile haemangioma (IH), congenital haemangioma (CH) and kaposiform hemangioendothelioma (KH); of which IH is the most common. Management options for vascular tumours include conservative approaches, oral medications and surgical intervention as determined by tumour type, location and associated complications. Vascular malformations can be categorised into low flow and high flow lesions. Low flow lesions include capillary, venous and lymphatic malformations (LMs). High flow lesions describe the arteriovenous malformations (AVMs), a highly heterogeneous group of lesions which can present in a variety of ways—the mainstay of treatment for these dynamic lesions is endovascular or surgical obliteration. We provide a practical framework for clinical classification of vascular anomalies of the head and neck in children. We also explore principles of their clinical and radiological assessment along with management, highlighting the importance of a multi-disciplinary approach.

Most neurologists, vascular neuroradiologists and stroke physicians encounter carotid artery dissection regularly, yet its management remains challenging. The main goal is the prevention of ischaemic stroke in the territory of the affected artery. Such strokes usually result from thromboembolism, though they can also occur due to critically reduced flow in the dissected arterial segment, a so-called haemodynamic stroke. Unfortunately, we do not have randomised controlled trial evidence for the optimal medical treatment of extracranial arterial dissection: antiplatelet agents or anticoagulants are considered reasonable options.1 Why is carotid dissection still a treatment challenge? First, like stroke itself, carotid dissection is not one disease. The aetiology (traumatic versus spontaneous), site of dissection (intracranial versus extracranial), degree of luminal stenosis and extent of intracranial collateral circulation all vary from patient to patient, with profound effects on the risk, distribution and extent of any resultant cerebral infarction. Second, although carotid artery dissection accounts for about 20% of …

Dural sinus malformation (DSM) is a rare pediatric cerebrovascular malformation, presenting in antenatal or early postnatal life with massive dilation of the dural sinuses. Barbosa’s 2003 seminal paper describes two forms of the disease: a midline subtype spanning the confluence of the sinuses with giant dural sinus lakes (so-called ‘torcular DSM’ (tDSM), the focus of Yang’s two JNIS papers and a lateral subtype involving the jugular bulb with otherwise normal sinuses and associated high flow sigmoid sinus arteriovenous fistula.1 The cause of DSM is unknown. As with all rare pediatric conditions, literature is sparse, based on single cases and small case series, many historical and numbering less than 100 in total. As part of normal venous development there is ‘ballooning’ of the transverse sinuses toward the end of the second trimester, reflecting increased venous flow from the rapidly growing cerebral hemispheres. Normally, remodeling to a more normal ‘adult’ pattern occurs around birth, with formation of the jugular bulbs.2 However, …

Tandem internal carotid and middle cerebral artery occlusion after carotid dissection predicts poor outcome after systemic thrombolysis. Current treatments include the use of endovascular carotid stenting, which carries with it a high risk of propagating further embolic events and worsening the dissection. New strategies for avoiding the aforementioned side-effects include recanalization using cross-collaterals for delivery of intra-lesional tissue plasminogen activator (tPA). We present two cases that provide further support for this novel approach. Both patients presented with a National Institute of Health Stroke Scale of 20, received intra-arterial tPA via cross-collateralization, and made full recoveries without the need for stenting.

Background Preoperative embolization of meningiomas remains contentious, with persisting uncertainty over the safety and efficacy of this adjunctive technique. Objective To evaluate the safety of presurgical embolization of meningiomas and its impact on subsequent transfusion requirement with respect to the extent of embolization and technique used. Methods One hundred seventeen consecutive patients between 2001 and 2010 were referred for embolization of presumed intracranial meningioma before surgical resection. Glue and/or particles were used to devascularize the tumor in 107 patients, all of whom went on to operative resection. The extent and nature of embolization-related complications, degree of angiographic devascularization, and the intraoperative blood transfusion requirements were analyzed. Results Mean blood transfusion requirement during surgery was 0.8 units per case (range, 1-14 units). Blood transfusion was significantly lower in patients whose meningiomas were completely, angiographically devascularized (P = .035). Four patients had complications as a direct result of the embolization procedure. These included intratumoral hemorrhage in 2, sixth cranial nerve palsy in 1, and scalp necrosis requiring reconstructive surgery in 1 patient. Conclusion The complication rate was 3.7%. No relationship between the embolic agent and the degree of devascularization was observed. Achieving a complete devascularization resulted in a lower blood transfusion requirement, considered an indirect measure of operative blood loss. This series demonstrates that preoperative meningioma embolization is safe and may reduce operative blood loss. We present distal intratumoral injection of liquid embolic as a safe and effective alternative to more established particle embolization techniques.

Brain arteriovenous malformations (AVMs) are rare and heterogeneous vascular abnormalities. AVMs are formed of a tangled anastomosis of arteries and veins without intervening capillaries located within the brain parenchyma (90 % supratentorial) with pathologic shunting of blood from the arterial to the venous side. Prevalence is approximately 18 per 100,000 adults with an incidence of approximately 1.3 per 100,000 adults per year. Most cases are sporadic – cause unknown and probably multifactorial with genetic and environmental factors.

The goal of treatment of cerebral aneurysms is complete, permanent aneurysmal occlusion with preservation of parent artery and its branches (constructive techniques). Developments in endovascular and neurosurgical techniques mean most aneurysms are treatable by constructive techniques. Constructive techniques remain inappropriate for a small number of aneurysms (i.e., fusiform, giant, or blister aneurysms). Parent artery occlusion or trapping aneurysmal segment (deconstructive) techniques remain the safest option in some settings.