Perioperative Management of Pediatric Patients with Moyamoya Arteriopathy.

Pediatric patients with moyamoya arteriopathy are at high risk for developing new onset transient or permanent neurologic deficits secondary to cerebral hypoperfusion, particularly in the perioperative period. It is therefore essential to carefully manage these patients in a multidisciplinary, coordinated effort to reduce the risk of new permanent neurologic deficits. However, little has been published on perioperative management of pediatric patients with moyamoya, particularly in the early postoperative period during intensive care unit admission. Our pediatric neurocritical care team sought to create a multidisciplinary periprocedural evidence- and consensus-based care pathway for high-risk pediatric patients with moyamoya arteriopathy undergoing anesthesia for any reason to decrease the incidence of periprocedural stroke or transient ischemic attack (TIA). We reviewed the literature to identify risk factors associated with perioperative stroke or TIA among patients with moyamoya and to gather data supporting specific perioperative management strategies. A multidisciplinary team from pediatric anesthesia, neurocritical care, nursing, child life, neurosurgery, interventional neuroradiology, neurology, and hematology created a care pathway for children with moyamoya undergoing anesthesia, classifying them as either high or standard risk, and applying an individualized perioperative management plan to high-risk patients. The incidence of neurologic sequelae before and after pathway implementation will be compared in future studies.

Cochlear Aqueduct Morphology in Superior Canal Dehiscence Syndrome.

The cochlear aqueduct (CA) connects the scala tympani to the subarachnoid space and is thought to assist in pressure regulation of perilymph in normal ears, however, its role and variation in inner ear pathology, such as in superior canal dehiscence syndrome (SCDS), is unknown. This retrospective radiographic investigation compared CA measurements and classification, as measured on flat-panel computerized tomography, among three groups of ears: controls, n = 64; anatomic superior canal dehiscence without symptoms (SCD), n = 28; and SCDS, n = 64. We found that in a multinomial logistic regression adjusted for age, sex, and BMI, an increase in CA length by 1 mm was associated with a lower odds for being in the SCDS group vs. control (Odds ratio 0.760 p = 0.005). Hierarchical clustering of continuous CA measures revealed a cluster with small CAs and a cluster with large CAs. Another multinomial logistic regression adjusted for the aforementioned clinical covariates showed an odds ratio of 2.97 for SCDS in the small CA cluster as compared to the large (p = 0.004). Further, no significant association was observed between SCDS symptomatology-vestibular and/or auditory symptoms-and CA structure in SCDS ears. The findings of this study lend support to the hypothesis that SCDS has a congenital etiology.

Definitive treatment of seizures due to hemimegalencephaly in neonates and young infants by transarterial embolization: technical considerations for 'endovascular embolic hemispherectomy'.

BACKGROUND: This case series describes the technical considerations and effectiveness of 'endovascular embolic hemispherectomy' for the treatment of medically intractable seizures in neonates and young infants with hemimegalencephaly (HME) and in whom surgical hemispherectomy is not a viable option. METHODS: This is a descriptive review of the endovascular technique used to treat consecutive pediatric patients with serial transarterial embolization for intractable seizures due to HME between 2018 and 2022. Clinical presentation, endovascular procedural details and complications, and efficacy were examined. RESULTS: Three infants (13-day-old, 13-week-old and 15-day-old) with HME and intractable seizures underwent a total of 10 transarterial embolizations. Anticipated intraprocedural events included vasospasm and focal subarachnoid hemorrhage in all three infants, effectively controlled endovascularly, and non-target embolization in one infant. No infants had symptomatic intracranial hemorrhage or femoral artery occlusion. EEG background quiescence and seizure cessation was achieved after the final stage of embolization in all patients. All infants were discharged home from the neonatal ICU (median length of stay 36 days, range 27-74 days) and remain seizure-free to date (4 years, 9 months, and 8 months). None have developed hydrocephalus, required surgical hemispherectomy or other neurosurgical interventions. CONCLUSION: Endovascular hemispherectomy can be safely used to provide definitive treatment of HME-related epilepsy in neonates and young infants when intraprocedural events are managed effectively. This less invasive novel approach should be considered a feasible early alternative to surgical hemispherectomy. Further studies are needed to enhance the safety profile and to assess long-term neurodevelopmental outcome and durability of freedom from seizures.

2D Measurements of the Angle of the Vestibular Aqueduct Using CT Imaging.

Recently, Bächinger et al. developed a software that measures the angle between the vestibular aqueduct proximal to the vestibule and the distal vestibular aqueduct on computed tomography (CT) scans and found differences in the vestibular aqueduct angle between the hypoplastic and degenerative categories of Meniere's disease (MD). Hypoplastic radiological findings were associated with the development of bilateral MD and hypoplastic changes were not found outside of fetal temporal bones and individuals with MD. The purpose of this study is to examine how the software developed by Bächinger et al. performs when applied to a large dataset of adult patients with varied otologic diagnoses. Adult patients who underwent high resolution flat panel CT scans without intravenous contrast (n = 301) were retrospectively reviewed. Measurements of the angle of the vestibular aqueduct were made using the previously developed software tool. The tool could be applied to measure the vestibular aqueduct angle in most CT scans of the temporal bones (n = 572 ears, 95%). While the majority of ears fell within the normal range of <120 degrees (n = 462, 80%), fourteen ears (2.3%) in 13 patients were found to have vestibular aqueduct angles that meet criteria for hypoplastic MD (>140 degrees). Only one of the 13 patients had a diagnosis of MD and not in the ear in the hypoplastic category. An inconsistent pattern of other otologic diagnoses were found among the 13 individuals meeting criteria for hypoplastic MD. Although prior reports indicate the software has prognostic value in individuals with MD, these results suggest that the software may have lower positive predictive value when applied to a large population of individuals with varied otologic diagnoses.

Endovascular therapy for acute stroke in children: age and size technical limitations.

Endovascular therapies for acute childhood stroke remain controversial and little evidence exists to determine the minimum age and size cut-off for thrombectomy in children. Despite this, an increasing number of reports suggest feasibility of thrombectomy in at least some children by experienced operators. When compared with adults, technical modifications may be necessary in children owing to differences in vessel sizes, tolerance of blood loss, safety of contrast and radiation exposure, and differing stroke etiologies. We review critical considerations for neurologists and neurointerventionalists when treating pediatric stroke with endovascular therapies. We discuss technical factors that may limit feasibility of endovascular therapy, including size of the femoral and cervicocerebral arteries, which contributes to vasospasm risk. The risk of femoral vasospasm can be assessed by comparing catheter outer diameter with estimated femoral artery size, which can be estimated based on the child's height. We review evidence supporting specific strategies to mitigate cervicocerebral arterial injury, including technique (stent retrieval vs direct aspiration) and device size selection. The importance of and strategies for minimizing blood loss, radiation exposure, and contrast administration are reviewed. Attention to these technical limitations is critical to delivering the safest possible care when thrombectomy is being considered for children with acute stroke.

Interventional neuro-oncology.

Interventional neuro-oncology encompasses an array of image-guided therapies-intra-arterial chemotherapy, regional drug delivery, chemoembolization, tumor ablation-along with techniques to improve therapy delivery such as physical or chemical blood-brain barrier disruption and percutaneous catheter placement. Endovascular and percutaneous image-guided approaches to the treatment of the brain, eye, and other head and neck tumors will be discussed.

Mechanical Thrombectomy for Acute Ischemic Stroke: Considerations in Children.

The use of mechanical thrombectomy for the treatment of acute childhood arterial ischemic stroke with large vessel occlusion is increasing, with mounting evidence for its feasibility and safety. Despite this emerging evidence, clear guidelines for patient selection, thrombectomy technique, and postprocedure care do not exist for the pediatric population. Due to unique features of stroke in children, neurologists and interventionalists must consider differences in patient size, anatomy, collateral vessels, imaging parameters, and expected outcomes that may impact appropriate patient selection and timing criteria. In addition, different causes of stroke and comorbidities in children must be considered and may alter the safety and efficacy of thrombectomy. To optimize the success of endovascular intervention in children, a multidisciplinary team should take into account these nuanced considerations when determining patient eligibility, developing a procedural approach, and formulating a postprocedure neurological monitoring and therapeutic plan.

Removal of Antiscatter Grids for Spinal Digital Subtraction Angiography: Dose Reduction without Loss of Diagnostic Value.

Background Spinal digital subtraction angiography (DSA) exposes patients and operators to substantial amounts of radiation. Antiscatter grid (ASG) removal is used to decrease radiation exposure but may reduce image quality. Purpose To determine whether ASG removal during spinal DSA in adults reduces radiation dose while maintaining diagnostic image quality and whether dose reduction is related to body mass index (BMI). Materials and Methods This Health Insurance Portability and Accountability Act-compliant prospective study included adults undergoing spinal DSA between January and December 2016. Each procedure included an additional angiographic acquisition performed twice, once with and once without ASG, either documenting the artery of Adamkiewicz (no pathology group) or the condition leading to the procedure (pathology group). Dose differences between study acquisitions and the influence of BMI were evaluated via paired t test. Two neurointerventionalists blinded to acquisition protocols were asked to independently evaluate a sample of 40 study acquisitions (20 with ASG, 20 without ASG) from 20 randomly selected participants to (a) rate image quality, (b) categorize findings, and (c) determine whether images had been obtained with or without ASG. Percentage agreement on image quality, findings categorization, and ability to correctly identify the acquisition protocol was calculated for both readers. Results Fifty-three participants (mean age ± standard deviation, 51 years ± 15.2; 32 men) were evaluated. ASG removal reduced the mean dose per acquisition by approximately 33% (mean dose-area product and air kerma decreased from 202 to 135.6 µGy/m2 and from 35.3 to 24 mGy, respectively; P < .001) independently of BMI (P = .3). Both readers evaluated all images (40 of 40) as being of diagnostic quality and correctly categorized findings in 19 of 20 (95%) cases. Overall percentage agreement for correct protocol identification was 60% (12 of 20) for grid-in and 45% (nine of 20) for grid-out images. Conclusion Antiscatter grid removal during spinal digital subtraction angiography decreased participants' radiation exposure while preserving diagnostic image quality. © RSNA, 2020.

Atlanto-occipital ligament calcification: a novel imaging finding in pediatric rotational vertebral artery occlusion.

We describe a 2-year-old girl with bow hunter syndrome complicated by vertebral artery dissection and multiple ischemic infarcts. Pediatric bow hunter syndrome is a rare and likely under-recognized disorder. Interestingly, our patient had atlanto-occipital ligament calcification on CT scan, an imaging finding that has not been reported in association with bow hunter syndrome and one that might help increase recognition of this dynamic disorder of the posterior circulation.

Susceptibility weighted imaging in infants with staged embolization of vein of Galen aneurysmal malformations.

BACKGROUND AND PURPOSE: The vein of Galen aneurysmal malformation (VGAM) is a rare congenital vascular malformation with a higher morbidity and mortality, especially in neonates. Ultrasound, CT and MR are usually used in diagnosis and treatment monitoring of these disorders. In this current study, we aim to examine utility of SWI in evaluation of treatment response in infants with VGAM. MATERIALS AND METHODS: We performed a retrospective chart analysis of children with VGAM in our institution between January 2008 and December 2016. Inclusion criteria included; confirmed VGAM on DSA; available SWI sequence at baseline and at follow up after at least a single embolization session; age at initial MR of 18 years or younger. Signal intensity and Angioarchitecture of VGAM and cerebral veins on SWI, as well as hydrocephalus and clinical outcome were evaluated. RESULTS: Of 11 patients identified with VGAM in our institution, 5 children (3 males and 2 females) satisfied the inclusion criteria. The average age at initial MR was 29 days (range 1-120). Fourteen MRI were available for review. All children had VGAM of mural type. Intramedullary veins were dilated and SWI-hypointense in all children, while subependymal and sulcal veins were dilated and SWI-hypointense in 4 patients on initial MRI. On the first available follow up MRI, cerebral veins have mostly normalized in 4 children and remained mostly dilated and SWI-hypointense in 1 child; even after complete treatment of the VGAM. CONCLUSION: Our preliminary findings show that SWI seems to offer a beneficial non-invasive tool in evaluating passive venous congestion patterns in pediatric patients with VGAM. It remains to be determined in larger studies, the clinical significance of these SWI changes.

Pituitary Adenoma Apoplexy of the Orbit, Diagnosis, and Management With Presurgical Embolization.

Orbital invasion of pituitary adenomas has been previously reported. In this report, the authors describe a 71-year-old female with a prolactinoma that presented with invasion of and apoplexy within the orbit. The patient underwent exenteration, followed by rapid tumor recurrence and growth. Given the hemorrhagic nature of the tumor, she subsequently underwent preoperative embolization and surgical resection. This case is notable in that it illustrates both apoplexy of a pituitary tumor within the orbit and the benefit of presurgical embolization.

Reducing Radiation Dose for High-resolution Flat-panel CT Imaging of Superior Semicircular Canal Dehiscence.

OBJECTIVE: High-resolution flat panel computed tomography (FPCT) is useful for the evaluation of temporal bone pathologies. While radiation exposure remains a concern, efforts have been devoted to reduce dose, while maintaining image quality. We hypothesize that removing the antiscatter grid (ASG) results in decreased radiation exposure, while maintaining diagnostic image quality for the evaluation of superior semicircular canal dehiscence (SSCD). METHODS: Ten patients with clinical suspicion for SSCD participated in this prospective study. Two sequential collimated 20-second FPCT acquisitions were performed (first: grid in; second: grid removed) in all patients. Secondary reconstructions were created by manually generating the volume of interest to include the middle ear using a voxel size of 0.1 mm and 512 × 512 matrix. Radiation dose parameters (air kerma (Ka,r) in mGy and dose area product (DAP) in µGym) were recorded. Three reviewers analyzed images for the ability to diagnose SSCD, to identify the stapes crurae, and to determine if an ASG was present. RESULTS: The average Ka,r and DAP for the grid-in acquisitions were 246.7 mGy (SD 47.9) and 2838.0 µGym (SD 862.8), versus 160.2 mGy (SD 33.2) and 2026.3 µGym (SD 644.8) for the grid-out acquisitions, respectively (p<0.001 for both Ka,r and DAP). Radiation exposure was reduced by approximately 30% solely by removing the ASG. All reviewers correctly identified all patients with SSCD (confirmed at surgery), with mean AUC of 0.99 (κ = 0.90). CONCLUSION: Removing the antiscatter grid during FPCT imaging of the temporal bones is a simple and effective way to reduce radiation exposure while maintaining diagnostic image quality for the evaluation of SSCD.

Embolization of a traumatic arteriovenous fistula between the middle meningeal artery and middle meningeal vein in a child with pulsatile tinnitus.

Traumatic arteriovenous fistulas (AVFs) involving the middle meningeal artery (MMA) are uncommon lesions and rare in the pediatric population. An adolescent with headaches developed a bruit and a rhythmic "swooshing" noise in the left ear 9 weeks after a traumatic brain injury involving a left temporal bone fracture. An MRA of the brain demonstrated an enlarged left MMA and arterialized flow in the left middle meningeal vein (MMV), suggestive of an AVF. Cerebral angiography confirmed and characterized the left MMA-MMV fistula, which drained into the pterygoid plexus. The patient underwent transarterial coil embolization, with successful obliteration of the fistulous connection, and her tinnitus resolved. This case illustrates the importance of recognizing a post-traumatic AVF in a pediatric patient with pulsatile tinnitus, and that endovascular treatment by coil embolization is a safe and effective treatment option.