Vessel-targeted compensation of deformable motion in interventional cone-beam CT.

The present standard of care for unresectable liver cancer is transarterial chemoembolization (TACE), which involves using chemotherapeutic particles to selectively embolize the arteries supplying hepatic tumors. Accurate volumetric identification of intricate fine vascularity is crucial for selective embolization. Three-dimensional imaging, particularly cone-beam CT (CBCT), aids in visualization and targeting of small vessels in such highly variable anatomy, but long image acquisition time results in intra-scan patient motion, which distorts vascular structures and tissue boundaries. To improve clarity of vascular anatomy and intra-procedural utility, this work proposes a targeted motion estimation and compensation framework that removes the need for any prior information or external tracking and for user interaction. Motion estimation is performed in two stages: (i) a target identification stage that segments arteries and catheters in the projection domain using a multi-view convolutional neural network to construct a coarse 3D vascular mask; and (ii) a targeted motion estimation stage that iteratively solves for the time-varying motion field via optimization of a vessel-enhancing objective function computed over the target vascular mask. The vessel-enhancing objective is derived through eigenvalues of the local image Hessian to emphasize bright tubular structures. Motion compensation is achieved via spatial transformer operators that apply time-dependent deformations to partial angle reconstructions, allowing efficient minimization via gradient backpropagation. The framework was trained and evaluated in anatomically realistic simulated motion-corrupted CBCTs mimicking TACE of hepatic tumors, at intermediate (3.0 mm) and large (6.0 mm) motion magnitudes. Motion compensation substantially improved median vascular DICE score (from 0.30 to 0.59 for large motion), image SSIM (from 0.77 to 0.93 for large motion), and vessel sharpness (0.189 mm-1 to 0.233 mm-1 for large motion) in simulated cases. Motion compensation also demonstrated increased vessel sharpness (0.188 mm-1 before to 0.205 mm-1 after) and reconstructed vessel length (median increased from 37.37 to 41.00 mm) on a clinical interventional CBCT. The proposed anatomy-aware motion compensation framework presented a promising approach for improving the utility of CBCT for intra-procedural vascular imaging, facilitating selective embolization procedures.

Assessment of arterial supply to the stomach after bariatric surgery using multidetector CT arteriography.

PURPOSE: To describe residual arterial supply to the stomach after bariatric surgery via a systematic arterial-phase CT assessment approach that can aid in diagnosis and treatment of postoperative complications and facilitate planning for future procedures. METHODS: Arterial-phase CT of 46 patients who underwent Roux-en-Y gastric bypass (RYGB) or sleeve gastrectomy (SG) at 3 academic institutions were retrospectively reviewed to assess patency of left gastric artery (LGA), right gastric artery (RGA), gastroepiploic artery (GEA), and left inferior phrenic artery (LIPA) and presence of gastric perforators. RESULTS: In 25 RYGB and 21 SG patients, mean diameters were LGA 2.2 ± 0.4 mm, RGA 1.6 ± 0.5 mm, and GEA 1.7 ± 0.4 mm. On RYGB scans, all LGAs, RGAs, and 24/25 (96%) of GEAs were identified. Excellent to good patency was seen in 20/25 (80%) LGAs, 21/25 (84%) RGAs, and 23/24 (96%) GEAs. On SG scans, all LGAs, 18/21 (86%) of RGAs, and 20/21 (95%) GEAs were identified. Excellent to good patency was seen in 17/21 (81%) LGAs, 15/18 (83%) RGAs, and 20/20 (100%) GEAs. In terms of gastric perforators, LGA supply was seen on 23/25 (92%) of RYGB and 17/17 (100%) of SG scans. RGA supply was seen on 13/21 (62%) RYGB and 9/18 (50%) SG scans. GEA supply was seen on 19/23 (83%) RYGB scans. No gastric supply via GEA was seen on SG scans. CONCLUSION: In this study, arterial supply to the stomach through the LGA was consistently identified in all RYGB and SG cases, indicating an uncomplicated surgical approach with regard to preserving the LGA. Dedicated CT angiography protocol or catheter-directed angiography is recommended for accurate and comprehensive assessment of the gastric blood supply, particularly before surgical re-intervention.

Deformable motion compensation in interventional cone-beam CT with a context-aware learned autofocus metric.

PURPOSE: Interventional Cone-Beam CT (CBCT) offers 3D visualization of soft-tissue and vascular anatomy, enabling 3D guidance of abdominal interventions. However, its long acquisition time makes CBCT susceptible to patient motion. Image-based autofocus offers a suitable platform for compensation of deformable motion in CBCT, but it relies on handcrafted motion metrics based on first-order image properties and that lack awareness of the underlying anatomy. This work proposes a data-driven approach to motion quantification via a learned, context-aware, deformable metric, VI F D L ${\bm{VI}}{{\bm{F}}}_{DL}$ , that quantifies the amount of motion degradation as well as the realism of the structural anatomical content in the image. METHODS: The proposed VI F D L ${\bm{VI}}{{\bm{F}}}_{DL}$ was modeled as a deep convolutional neural network (CNN) trained to recreate a reference-based structural similarity metric-visual information fidelity (VIF). The deep CNN acted on motion-corrupted images, providing an estimation of the spatial VIF map that would be obtained against a motion-free reference, capturing motion distortion, and anatomic plausibility. The deep CNN featured a multi-branch architecture with a high-resolution branch for estimation of voxel-wise VIF on a small volume of interest. A second contextual, low-resolution branch provided features associated to anatomical context for disentanglement of motion effects and anatomical appearance. The deep CNN was trained on paired motion-free and motion-corrupted data obtained with a high-fidelity forward projection model for a protocol involving 120 kV and 9.90 mGy. The performance of VI F D L ${\bm{VI}}{{\bm{F}}}_{DL}$ was evaluated via metrics of correlation with ground truth VIF ${\bm{VIF}}$ and with the underlying deformable motion field in simulated data with deformable motion fields with amplitude ranging from 5 to 20 mm and frequency from 2.4 up to 4 cycles/scan. Robustness to variation in tissue contrast and noise levels was assessed in simulation studies with varying beam energy (90-120 kV) and dose (1.19-39.59 mGy). Further validation was obtained on experimental studies with a deformable phantom. Final validation was obtained via integration of VI F D L ${\bm{VI}}{{\bm{F}}}_{DL}$ on an autofocus compensation framework, applied to motion compensation on experimental datasets and evaluated via metric of spatial resolution on soft-tissue boundaries and sharpness of contrast-enhanced vascularity. RESULTS: The magnitude and spatial map of VI F D L ${\bm{VI}}{{\bm{F}}}_{DL}$ showed consistent and high correlation levels with the ground truth in both simulation and real data, yielding average normalized cross correlation (NCC) values of 0.95 and 0.88, respectively. Similarly, VI F D L ${\bm{VI}}{{\bm{F}}}_{DL}$ achieved good correlation values with the underlying motion field, with average NCC of 0.90. In experimental phantom studies, VI F D L ${\bm{VI}}{{\bm{F}}}_{DL}$ properly reflects the change in motion amplitudes and frequencies: voxel-wise averaging of the local VI F D L ${\bm{VI}}{{\bm{F}}}_{DL}$ across the full reconstructed volume yielded an average value of 0.69 for the case with mild motion (2 mm, 12 cycles/scan) and 0.29 for the case with severe motion (12 mm, 6 cycles/scan). Autofocus motion compensation using VI F D L ${\bm{VI}}{{\bm{F}}}_{DL}$ resulted in noticeable mitigation of motion artifacts and improved spatial resolution of soft tissue and high-contrast structures, resulting in reduction of edge spread function width of 8.78% and 9.20%, respectively. Motion compensation also increased the conspicuity of contrast-enhanced vascularity, reflected in an increase of 9.64% in vessel sharpness. CONCLUSION: The proposed VI F D L ${\bm{VI}}{{\bm{F}}}_{DL}$ , featuring a novel context-aware architecture, demonstrated its capacity as a reference-free surrogate of structural similarity to quantify motion-induced degradation of image quality and anatomical plausibility of image content. The validation studies showed robust performance across motion patterns, x-ray techniques, and anatomical instances. The proposed anatomy- and context-aware metric poses a powerful alternative to conventional motion estimation metrics, and a step forward for application of deep autofocus motion compensation for guidance in clinical interventional procedures.

Current and future directions in interventional neuro-oncology-are we there yet?

Advancements in technology and technical expertise increasingly enable neurointerventionalists to deliver safer and more effective endovascular treatments to cancers of the brain, spine, head, and neck. In addition to established neuro-oncological interventions such as pre-surgical tumor embolization and percutaneous ablation, newer modalities focused on direct arterial infusion of chemotherapy, radioisotopes, and radiosensitizers continue to gain traction as complementary treatment options, while stem cell-mediated delivery of theranostic nanoparticles and oncolytic virus are being explored for even greater specificity in targeting cancers across the blood-brain barrier. This article aims to provide an overview of the current state of the art and future directions for the field of interventional neuro-oncology, as well as opportunities and challenges presented by this emerging treatment modality.

Whole-lesion assessment of volume and signal changes after sclerotherapy of extremity venous malformations.

PURPOSE: To investigate quantitative changes in MRI signal intensity (SI) and lesion volume that indicate treatment response and correlate these changes with clinical outcomes after percutaneous sclerotherapy (PS) of extremity venous malformations (VMs). METHODS: VMs were segmented manually on pre- and post-treatment T2-weighted MRI using 3D Slicer to assess changes in lesion volume and SI. Clinical outcomes were scored on a 7-point Likert scale according to patient perception of symptom improvement; treatment response (success or failure) was determined accordingly. RESULTS: Eighty-one patients with VMs underwent 125 PS sessions. Treatment success occurred in 77 patients (95 %). Mean (±SD) changes were -7.9 ± 24 cm3 in lesion volume and -123 ± 162 in SI (both, P <.001). Mean reduction in lesion volume was greater in the success group (-9.4 ± 24 cm3) than in the failure group (21 ± 20 cm3) (P =.006). Overall, lesion volume correlated with treatment response (ρ = -0.3, P =.004). On subgroup analysis, volume change correlated with clinical outcomes in children (ρ = -0.3, P =.03), in sodium tetradecyl sulfate-treated lesions (ρ = -0.5, P =.02), and in foot lesions (ρ = -0.6, P =.04). SI change correlated with clinical outcomes in VMs treated in 1 PS session (ρ = -0.3, P =.01) and in bleomycin-treated lesions (ρ = -0.4, P =.04). CONCLUSIONS: Change in lesion volume is a reliable indicator of treatment response. Lesion volume and SI correlate with clinical outcomes in specific subgroups.

FABRICATION OF MULTILUMEN MICROFLUIDIC TUBING FOR EX SITU DIRECT LASER WRITING.

Among the numerous additive manufacturing or "three-dimensional (3D) printing" techniques, two-photon Direct Laser Writing (DLW) is distinctively suited for applications that demand high geometric versatility with micron-to-submicron-scale feature resolutions. Recently, "ex situ DLW (esDLW)" has emerged as a powerful approach for printing 3D microfluidic structures directly atop meso/macroscale fluidic tubing that can be manipulated by hand; however, difficulties in creating custom esDLW-compatible multilumen tubing at such scales has hindered progress. To address this impediment, here we introduce a novel methodology for fabricating submillimeter multilumen tubing for esDLW 3D printing. Preliminary fabrication results demonstrate the utility of the presented strategy for resolving 743 µm-in-diameter tubing with three lumens-each with an inner diameter (ID) of 80 µm. Experimental results not only revealed independent flow of discrete fluorescently labelled fluids through each of the three lumens, but also effective esDLW-printing of a demonstrative 3D "MEMS" microstructure atop the tubing. These results suggest that the presented approach could offer a promising pathway to enable geometrically sophisticated microfluidic systems to be 3D printed with input and/or output ports fully sealed to multiple, distinct lumens of fluidic tubing for emerging applications in fields ranging from drug delivery and medical diagnostics to soft surgical robotics.

Patient Characteristics Associated With Embolization Versus Hysterectomy for Uterine Fibroids: A Systematic Review and Meta-Analysis.

INTRODUCTION: Black and underinsured women in the United States are more likely than their counterparts to develop uterine fibroids (UFs) and experience more severe symptoms. Uterine artery embolization (UAE), a uterine-sparing therapeutic procedure, is less invasive than the common alternative, open hysterectomy. To determine whether demographic disparities persist in UF treatment utilization, we reviewed patient characteristics associated with UAE versus hysterectomy for UF among studies of US clinical practices. METHODS: A systematic literature review was conducted via PubMed, Embase, and CINAHL (PROSPERO CRD42023455051), yielding 1,350 articles (January 1, 1995, to July 15, 2023) that outlined demographic characteristics of UAE compared with hysterectomy. Two readers screened for inclusion criteria, yielding 13 full-text US-based comparative studies specifying at least one common demographic characteristic. Random effects meta-analysis was performed on the data (STATA v18.0). Egger's regression test was used to quantify publication bias. RESULTS: Nine (138,960 patients), four (183,643 patients), and seven (312,270 patients) studies were analyzed for race, insurance status, and age as predictors of treatment modality, respectively. Black race (odds ratio = 3.35, P < .01) and young age (P < .05) were associated with UAE, whereas private insurance (relative to Medicare and/or Medicaid) was not (odds ratio = 1.06, P = .52). Between-study heterogeneity (I2 > 50%) was detected in all three meta-analyses. Small-study bias was detected for age but not race or insurance. CONCLUSIONS AND IMPLICATIONS: Knowledge of demographic characteristics of patients with UFs receiving UAE versus hysterectomy is sparse (n = 13 studies). Among these studies, which seem to be racially well distributed, Black and younger women are more likely to receive UAE than their counterparts.

Radiomics Features Extracted From Pre- and Postprocedural Imaging in Early Prediction of Treatment Response in Patients Undergoing Transarterial Radioembolization of Hepatic Lesions: A Systematic Review, Meta-Analysis, and Quality Appraisal Study.

INTRODUCTION: Transarterial radioembolization (TARE) is one of the most promising therapeutic options for hepatic masses. Radiomics features, which are quantitative numeric features extracted from medical images, are considered to have potential in predicting treatment response in TARE. This article aims to provide meta-analytic evidence and critically appraise the methodology of radiomics studies published in this regard. METHODS: A systematic search was performed on PubMed, Scopus, Embase, and Web of Science. All relevant articles were retrieved, and the characteristics of the studies were extracted. The Radiomics Quality Score and Checklist for Evaluation of Radiomics Research were used to assess the methodologic quality of the studies. Pooled sensitivity, specificity, and area under the receiver operating characteristic curve in predicting objective response were determined. RESULTS: The systematic review included 15 studies. The average Radiomics Quality Score of these studies was 11.4 ± 2.1, and the average Checklist for Evaluation of Radiomics Research score was 33± 6.7. There was a notable correlation (correlation coefficient = 0.73) between the two metrics. Adherence to quality measures differed considerably among the studies and even within different components of the same studies. The pooled sensitivity and specificity of the radiomics models in predicting complete or partial response were 83.5% (95% confidence interval 76%-88.9%) and 86.7% (95% confidence interval 78%-92%), respectively. CONCLUSION: Radiomics models show great potential in predicting treatment response in TARE of hepatic lesions. However, the heterogeneity seen between the methodologic quality of studies may limit the generalizability of the results. Future initiatives should aim to develop radiomics signatures using multiple external datasets and adhere to quality measures in radiomics methodology.

Abdominal computed tomography measurements of body composition and waitlist mortality in kidney transplant candidates.

Body mass index is often used to determine kidney transplant (KT) candidacy. However, this measure of body composition (BC) has several limitations, including the inability to accurately capture dry weight. Objective computed tomography (CT)-based measures may improve pre-KT risk stratification and capture physiological aging more accurately. We quantified the association between CT-based BC measurements and waitlist mortality in a retrospective study of 828 KT candidates (2010-2022) with clinically obtained CT scans using adjusted competing risk regression. In total, 42.5% of candidates had myopenia, 11.4% had myopenic obesity (MO), 68.8% had myosteatosis, 24.8% had sarcopenia (probable = 11.2%, confirmed = 10.5%, and severe = 3.1%), and 8.6% had sarcopenic obesity. Myopenia, MO, and sarcopenic obesity were not associated with mortality. Patients with myosteatosis (adjusted subhazard ratio [aSHR] = 1.62, 95% confidence interval [CI]: 1.07-2.45; after confounder adjustment) or sarcopenia (probable: aSHR = 1.78, 95% CI: 1.10-2.88; confirmed: aSHR = 1.68, 95% CI: 1.01-2.82; and severe: aSHR = 2.51, 95% CI: 1.12-5.66; after full adjustment) were at increased risk of mortality. When stratified by age, MO (aSHR = 2.21, 95% CI: 1.28-3.83; P interaction = .005) and myosteatosis (aSHR = 1.95, 95% CI: 1.18-3.21; P interaction = .038) were associated with elevated risk only among candidates <65 years. MO was only associated with waitlist mortality among frail candidates (adjusted hazard ratio = 2.54, 95% CI: 1.28-5.05; P interaction = .021). Transplant centers should consider using BC metrics in addition to body mass index when a CT scan is available to improve pre-KT risk stratification at KT evaluation.

Prospective Study of Polytetrafluoroethylene-Covered Microplugs and Detachable Coils for Embolization of Pulmonary Arteriovenous Malformations: Technical Results, Procedure Times, and Costs.

PURPOSE: To determine time to occlusion and procedure costs of embolization of pulmonary arteriovenous malformations (PAVMs) using a polytetrafluoroethylene-covered microplug compared with embolization using detachable coils. MATERIALS AND METHODS: In this prospective study, 37 patients (mean age, 39.1 years [SD ± 17.6]) with 82 PAVMs underwent embolization with microplug or detachable coils between April 2019 and January 2023. Technical success, procedure time intervals, and costs were analyzed. RESULTS: In 37 patients, 82 PAVMs and 101 feeding arteries were successfully treated (microplug, 64; microplug + another device, 5; detachable coils alone, 32). Time from embolic device inserted into the catheter to device deployed and time to occlusion differed significantly between microplug and detachable coil cohorts (P < .0001 for both). Embolization with ≥1 microplug had a significantly shorter occlusion time than embolization with detachable coils (median, 10.0 minutes saved per feeding artery) (P < .0001). Compared with detachable coil embolization, microplug embolization saved a median of 9.0 minutes per feeding artery (P < .0001) and reduced room cost by a median of $429 per feeding artery (P < .0001). Device costs per feeding artery did not differ significantly between microplug ($2,790) and detachable coil embolization ($3,147) (P = .87). CONCLUSIONS: Compared with coils, microplugs had an equally high technical success rate but significant time to occlusion and room costs savings per feeding artery. Total room cost and device cost together did not differ significantly between microplugs and coils. Microplugs may be considered technically effective and at least cost-neutral for PAVM embolization where clinically appropriate.

Deformable Motion Compensation for Intraprocedural Vascular Cone-beam CT with Sequential Projection Domain Targeting and Vessel-Enhancing Autofocus.

Purpose: Cone-beam CT (CBCT) is used in interventional radiology (IR) for identification of complex vascular anatomy, difficult to visualize in 2D fluoroscopy. However, long acquisition time makes CBCT susceptible to soft-tissue deformable motion that degrades visibility of fine vessels. We propose a targeted framework to compensate for deformable intra-scan motion via learned full-sequence models for identification of vascular anatomy coupled to an autofocus function specifically tailored to vascular imaging. Methods: The vessel-targeted autofocus acts in two stages: (i) identification of vascular and catheter targets in the projection domain; and, (ii) autofocus optimization for a 4D vector field through an objective function that quantifies vascular visibility. Target identification is based on a deep learning model that operates on the complete sequence of projections, via a transformer encoder-decoder architecture that uses spatial-temporal self-attention modules to infer long-range feature correlations, enabling identification of vascular anatomy with highly variable conspicuity. The vascular autofocus function is derived through eigenvalues of the local image Hessian, which quantify the local image structure for identification of bright tubular structures. Motion compensation was achieved via spatial transformer operators that impart time dependent deformations to NPAR = 90 partial angle reconstructions, allowing for efficient minimization via gradient backpropagation. The framework was trained and evaluated in synthetic abdominal CBCTs obtained from liver MDCT volumes and including realistic models of contrast-enhanced vascularity with 15 to 30 end branches, 1 - 3.5 mm vessel diameter, and 1400 HU contrast. Results: The targeted autofocus resulted in qualitative and quantitative improvement in vascular visibility in both simulated and clinical intra-procedural CBCT. The transformer-based target identification module resulted in superior detection of target vascularity and a lower number of false positives, compared to a baseline U-Net model acting on individual projection views, reflected as a 1.97x improvement in intersection-over-union values. Motion compensation in simulated data yielded improved conspicuity of vascular anatomy, and reduced streak artifacts and blurring around vessels, as well as recovery of shape distortion. These improvements amounted to an average 147% improvement in cross correlation computed against the motion-free ground truth, relative to the un-compensated reconstruction. Conclusion: Targeted autofocus yielded improved visibility of vascular anatomy in abdominal CBCT, providing better potential for intra-procedural tracking of fine vascular anatomy in 3D images. The proposed method poses an efficient solution to motion compensation in task-specific imaging, with future application to a wider range of imaging scenarios.

Balancing Safety and Efficacy to Determine the Most Suitable Size of Imaging-Visible Embolic Microspheres for Bariatric Arterial Embolization in a Preclinical Model.

OBJECTIVES: To identify the most suitable size of imaging-visible embolic agents with balanced safety and efficacy for bariatric arterial embolization (BAE) in a preclinical model. MATERIALS AND METHODS: Twenty-seven pigs were divided into 3 cohorts. In Cohort I, 16 pigs were randomized to receive (n = 4 each) 40-100-µm microspheres in 1 or 2 fundal arteries, 70-340-µm radiopaque microspheres in 2 fundal arteries, or saline. In Cohort II, 3 pigs underwent renal arterial embolization with either custom-made 100-200-µm, 200-250-µm, 200-300-µm, or 300-400-µm radiopaque microspheres or Bead Block 300-500 µm with microsphere distribution assessed histologically. In Cohort III, 8 pigs underwent BAE in 2 fundal arteries with tailored 100-200-µm radiopaque microspheres (n = 5) or saline (n = 3). RESULTS: In Cohort I, no significant differences in weight or ghrelin expression were observed between BAE and control animals. Moderate-to-severe gastric ulcerations were noted in all BAE animals. In Cohort II, renal embolization with 100-200-µm microspheres occluded vessels with a mean diameter of 139 µm ± 31, which is within the lower range of actual diameters of Bead Block 300-500 µm. In Cohort III, BAE with 100-200-µm microspheres resulted in significantly lower weight gain (42.3% ± 5.7% vs 51.6% ± 2.9% at 8 weeks; P = .04), fundal ghrelin cell density (16.1 ± 6.7 vs 23.6 ± 12.6; P = .045), and plasma ghrelin levels (1,709 pg/mL ± 172 vs 4,343 pg/mL ± 1,555; P < .01) compared with controls and superficial gastric ulcers (5/5). CONCLUSIONS: In this preclinical model, tailored 100-200-µm microspheres were shown to be most suitable for BAE in terms of safety and efficacy.

EMBIO trial study protocol: left gastric artery embolisation for weight loss in patients living with obesity with a BMI 35-50 kg/m2.

INTRODUCTION: Left gastric artery embolisation (LGAE) is a well-established treatment for major upper gastrointestinal (GI) bleeding when control is not established via upper GI endoscopy and recently has shown promising results for weight loss in small single arm studies. LGAE could be a treatment option in between our current tier-3 and tier-4 services for obesity. EMBIO is a National Institute for Health Research funded trial, a multicentre double-blinded randomised controlled trial between Imperial College National Health Service Trust and University College London Hospital, comparing LGAE versus Placebo procedure. The key aims of the trial is to evaluate LGAE efficacy on weight loss, its mechanism of action, safety profile and obesity-related comorbidities. METHODS AND ANALYSIS: 76 participants will be recruited from the existing tier-3 database after providing informed consent. Key inclusion criteria include adults aged 18-70 with a body mass index 35-50 kg/m2 and appropriate anatomy of the left gastric artery and coeliac plexus on CT Angiogram. Key exclusion criteria included previous major abdominal and bariatric surgery, weight >150 kg, type 2 diabetes on any medications other than metformin and the use of weight modifying medications. Participants will undergo mechanistic visits 1 week prior to the intervention and 3, 6 and 12 months postintervention. Informed consent will be received from each participant and they will be randomised in a 1:1 ratio to left gastric artery embolisation and placebo treatment. Blinding strategies include the use of moderate doses of sedation, visual and auditory isolation. All participants will enter a tier-3 weight management programme postintervention. The primary analysis will estimate the difference between the groups in the mean per cent weight loss at 12 months. ETHICS AND DISSEMINATION: This trial shall be conducted in full conformity with the 1964 Declaration of Helsinki and all subsequent revisions. Local research ethics approval was granted by London-Central Research Ethics Committee, (Reference 19/LO/0509) on 11 October 2019. The Medicines and Healthcare products Regulatory Agency (MHRA) issued the Letter of No Objection on 8 April 2022 (Reference CI/2022/0008/GB). The trial's development and progress are monitored by an independent trial steering committee and data monitoring and ethics committee. The researchers plan to disseminate results at conferences, in peer- reviewed journals as well as lay media and to patient organisations. TRIAL REGISTRATION NUMBER: ISRCTN16158402.

Risk factors and clinical features associated with basal ganglia manganese deposition in patients with hereditary hemorrhagic telangiectasia.

BACKGROUND: T1-hyperintensity of the basal ganglia (BG) due to manganese deposition is a known radiologic finding in patients with hereditary hemorrhagic telangiectasia (HHT), but risk factors and associated clinical manifestations are unclear. This study conducted a quantitative analysis of the association of T1-hyperintensity in HHT patients with specific risk factors, signs, and symptoms. METHODS: Patients seen at our center between 2005 and 2020 with a definitive diagnosis of HHT who had an available non-contrast T1-weighted brain MRI were included. Hyperintensity was evaluated using oval regions of interest measurements. The BG: thalamus intensity ratio was used to quantitatively evaluate T1-hyperintensity. Patient laboratory values and clinical findings were collected from electronic medical records. Hyperintensity was analyzed for its association with laboratory values, and clinical findings. Variables were analyzed through regression analysis. RESULTS: A total of 239 patients were included in this study. On 1.5 T scanners, values that were significant on multivariable regression analysis were age (p < .001), hepatic AVMs (p < .001), iron deficiency anemia (p = .0021), and cirrhosis (p = .016). On 3 T scanners, values that were significant on multivariable analysis were hepatic AVMs (p = .0024) and cirrhosis (p = .0056). On 3 T scanners, hyperintensity was significantly associated with tremor (OR = 1.17, p = .033), restless leg syndrome (OR = 1.22, p = .0086), and memory problems (OR = 1.17, p = .046). CONCLUSIONS: BG hyperintensity due to manganese deposition is significantly associated with hepatic risk factors on 1.5 T and 3 T scanners and iron deficiency anemia on 1.5 T scanners. On 3 T scanners, T1-hyperintensity is associated with neuropsychiatric signs and symptoms, such as tremor, restless leg syndrome, and memory problems.

Intraoperative Neuromonitoring during Peripheral Arteriovenous Malformation Embolization.

PURPOSE: To evaluate whether intraoperative neuromonitoring (IONM), including pre-embolization lidocaine injection challenge ("provocative testing") is associated with reduced risk of irreversible nerve injury during embolization of peripheral arteriovenous malformations (AVMs). MATERIALS AND METHODS: Medical records of patients with peripheral AVMs who underwent embolotherapy with IONM with provocative testing between 2012 and 2021 were reviewed retrospectively. Data collected included patient demographic characteristics, AVM location and size, embolic agent used, IONM signal changes after lidocaine and embolic agent injections, postprocedural adverse events, and clinical outcomes. Decisions regarding whether embolization would proceed at specific locations were based on IONM findings after the lidocaine challenge and as embolization proceeded. RESULTS: A cohort of 17 patients (mean age, 27 years ± 19; 5 women) who underwent 59 image-guided embolization procedures with adequate IONM data was identified. No permanent neurologic deficits occurred. Transient neurologic deficits were observed in 3 patients (4 sessions), comprising skin numbness (2 patients), extremity weakness (1 patient), and extremity weakness and numbness (1 patient). All neurologic deficits resolved by postoperative day 4 without additional treatment. CONCLUSIONS: IONM, including provocative testing, during AVM embolization may minimize potential nerve injury.

Venous malformation may be a feature of EXT1-related hereditary multiple exostoses: A report of two unrelated probands.

Hereditary multiple exostoses (HME), also known as hereditary multiple osteochondroma (HMO), is an autosomal dominant disorder caused by pathogenic variants in exostosin-1 or -2 (EXT1 or EXT2). It is characterized by the formation of multiple benign growing osteochondromas (exostoses) that most commonly affect the long bones; however, it may also occur throughout the body. Although many of these lesions are clinically asymptomatic, some can lead to chronic pain and skeletal deformities and interfere with adjacent neurovascular structures. Here, we report two unrelated probands that presented with a clinical and molecular diagnosis of HME with venous malformation, a clinical feature not previously reported in individuals with HME.