Ocular survival after intra-arterial chemotherapy for retinoblastoma improves with accrual of experience and programmatic evolution.

BACKGROUND: Intra-arterial chemotherapy (IAC) for the treatment of intraocular retinoblastoma has gained recognition as a method to improve ocular salvage; however, there is a paucity of evidence supporting treatment factors prognosticating ocular survival. METHODS: All patients with retinoblastoma treated with IAC at a single institution between December 2008 and December 2019 were evaluated. Patient demographics, tumor classification, prior treatments, procedural data, other non-IAC therapies, adverse reactions, procedural complications, ocular outcomes, and overall survival were assessed via retrospective chart review. Factors suggestive of increased ocular survival were identified via univariate and multivariate analyses. The impact of accrued treatment experience was evaluated by grouping eyes by the respective year, IAC treatment was initiated. RESULTS: Forty-nine eyes of 43 patients were treated for retinoblastoma with IAC (256 total procedures). At least grade 3 neutropenia was observed following 19% of IAC procedures. The risk of neutropenia was not statistically different between single or multidrug IAC. Comparing those who received balloon-assisted intra-arterial chemotherapy (bIAC) in more than two-thirds of cycles to those who did not, the risk of arterial access site complications was not statistically different. Multivariate analysis revealed a significantly lower risk of enucleation associated with treatment era in years (hazard ratio [HR] = 0.52-1.00, p < .05) and laser therapies (HR = 0.02-0.60, p < .05). CONCLUSIONS: Ocular survival rates in patients treated with IAC for retinoblastoma at our institution have increased over time. Accrued treatment experience and programmatic changes have likely contributed. Larger, prospective series may lead to a better understanding of factors that consistently contribute to better ocular salvage.

Fostering research in pediatric interventional radiology: needs assessment and suggestions for support.

BACKGROUND: Due to the rarity of pediatric diseases, collaborative research is the key to maximizing the impact of research studies. A research needs assessment survey was created to support initiatives to foster pediatric interventional radiology research. OBJECTIVE: To assess the status of pediatric interventional radiology research, identify perceived barriers, obtain community input on areas of research/education/support, and create metrics for evaluating changes/responses to programmatic initiatives. MATERIALS AND METHODS: A survey link was sent to approximately 275 members of the Society for Pediatric Interventional Radiology (SPIR) between May and October 2020. Data was collected using a web-based interface. Data collected included practice setting, clinical role, research experience, research barriers, and suggestions for future initiatives. RESULTS: Fifty-nine surveys were analyzed with a staff physician survey response rate of 28% (56/198). A wide range of practice sizes from 15 countries were represented. Respondents were predominantly staff physicians (95%; 56/59) with an average of 11 years (range: 1-25 years) of clinical experience working at academic or freestanding children's hospitals. A total of 100% (59/59) had research experience, and 70% (41/58) had published research with a mean of 30 peer-reviewed publications (range: 1-200). For job security, 56% (33/59) of respondents were expected or required to publish, but only 19% (11/58) had research support staff, and 42% (25/59) had protected research time, but of those, 36% (9/25) got the time "sometimes or never." Lack of support staff, established collaborative processes, and education were identified as top barriers to performing research. CONCLUSIONS: The needs assessment survey demonstrated active research output despite several identified barriers. There is a widespread interest within the pediatric interventional radiology community for collaborative research.

Multimodal Assessment of Cerebral Autoregulation and Autonomic Function After Pediatric Cerebral Arteriovenous Malformation Rupture.

BACKGROUND: Management after cerebral arteriovenous malformation (AVM) rupture aims toward preventing hemorrhagic expansion while maintaining cerebral perfusion to avoid secondary injury. We investigated associations of model-based indices of cerebral autoregulation (CA) and autonomic function (AF) with outcomes after pediatric cerebral AVM rupture. METHODS: Multimodal neurologic monitoring data from the initial 3 days after cerebral AVM rupture were retrospectively analyzed in children (< 18 years). AF indices included standard deviation of heart rate (HRsd), root-mean-square of successive differences in heart rate (HRrmssd), low-high frequency ratio (LHF), and baroreflex sensitivity (BRS). CA indices include pressure reactivity index (PRx), wavelet pressure reactivity indices (wPRx and wPRx-thr), pulse amplitude index (PAx), and correlation coefficient between intracranial pressure pulse amplitude and cerebral perfusion pressure (RAC). Percent time of cerebral perfusion pressure (CPP) below lower limits of autoregulation (LLA) was also computed for each CA index. Primary outcomes were determined using Pediatric Glasgow Outcome Score Extended-Pediatrics (GOSE-PEDs) at 12 months and acquired epilepsy. Association of biomarkers with outcomes was investigated using linear regression, Wilcoxon signed-rank, or Chi-square. RESULTS: Fourteen children were analyzed. Lower AF indices were associated with poor outcomes (BRS [p = 0.04], HRsd [p = 0.04], and HRrmssd [p = 0.00]; and acquired epilepsy (LHF [p = 0.027]). Higher CA indices were associated with poor outcomes (PRx [p = 0.00], wPRx [p = 0.00], and wPRx-thr [p = 0.01]), and acquired epilepsy (PRx [p = 0.02] and wPRx [p = 0.00]). Increased time below LLA was associated with poor outcome (percent time below LLA based on PRx [p = 0.00], PAx [p = 0.04], wPRx-thr [p = 0.03], and RAC [p = 0.01]; and acquired epilepsy (PRx [p = 0.00], PAx [p = 0.00], wPRx-thr [p = 0.03], and RAC [p = 0.01]). CONCLUSIONS: After pediatric cerebral AVM rupture, poor outcomes are associated with AF and CA when applying various neurophysiologic model-based indices. Prospective work is needed to assess these indices of CA and AF in clinical decision support.

Survey of practice patterns and preparedness for endovascular therapy in acute pediatric stroke.

PURPOSE: Endovascular therapy benefits selected adults with acute stroke while data are lacking for children. The purpose of this study was to assess physician practice and institutional preparedness for endovascular therapy in pediatric stroke. METHODS: A link to an anonymous online survey was sent to members of the International Pediatric Stroke Study (IPSS) group about physician experience with endovascular therapy, likelihood of treatment for provided clinical vignettes, and institutional readiness for the delivery of endovascular therapy to children. RESULTS: Thirty-one pediatric physicians with a mean of 11 years (SD 7.1) of experience responded. All but two would consider endovascular therapy in a child, and 20 (64.5%) had recommended endovascular therapy for a child in the preceding year. Most (n = 19, 67.9%) did not commit to an age minimum for endovascular therapy. Sixteen (57.1%) would consider treatment up to 24 h after symptom onset with 19 (67.9%) respondents reporting that their practice changed after the 2018 American Heart Association guidelines extended the time window for endovascular therapy in adults. Seventeen (60.7%) preferred imaging that included perfusion in children presenting beyond 6 h. Nineteen (70.4%) had institutional endovascular therapy criteria. Physicians in larger pediatric groups had more "likely to treat" responses on the clinical vignettes than physicians working in smaller groups (11.7 vs. 6.1, p < 0.05). CONCLUSION: Pediatric stroke physicians are largely willing to consider endovascular therapy with most changing their practice according to adult guidelines, though experience and selection criteria varied. These findings may help to inform consensus guidelines and clinical trial development.

Quantitative Arterial Tortuosity Suggests Arteriopathy in Children With Cryptogenic Stroke.

BACKGROUND AND PURPOSE: Quantitative arterial tortuosity (QAT) is a ratio of vessel length between 2 points to the shortest linear distance between same points. QAT has been reported as an imaging biomarker of arteriopathy in pediatric arterial ischemic stroke (AIS) because of dissection and transient cerebral arteriopathy. We sought to determine whether QAT abnormalities are present in other subtypes of pediatric AIS. METHODS: Children with AIS-absent conventional biomarkers of arteriopathy and case-controls who underwent magnetic resonance angiography were classified by stroke mechanism. The primary study population consisted of cryptogenic AIS cases. AIS with bow hunter physiology and cardiogenic emboli were also evaluated. AIS because of nontraumatic dissection served as positive controls. Patients without vascular risk factors served as negative controls. Segmental QAT of cervicocerebral arteries were measured using automated image processing and differences between groups analyzed. RESULTS: In negative controls, QAT showed significant age-related variability for most arterial segments. Positive controls showed significantly increased QAT of the distal extracranial vertebral arteries (VAs) and decreased QAT of the intracranial VA relative to negative controls. Cryptogenic stroke and bow hunter physiology cases were similar to positive controls showing increased QAT of the distal extracranial VA and decreased QAT of the intracranial VA relative to negative controls. Cardioembolic stroke cases were similar to negative controls showing decreased QAT of the distal extracranial VA and increased QAT of the intracranial VA relative to positive controls. CONCLUSIONS: Pediatric cryptogenic stroke is frequently associated with cervicocerebral arteriopathies expressing altered QAT. QAT may be a diagnostic biomarker of arteriopathy in pediatric AIS.

Spectrum of cerebral arterial and venous abnormalities in Alagille syndrome.

BACKGROUND: Alagille syndrome is a pediatric multisystem disease with increased prevalence of cerebrovascular disease. The spectrum of cerebrovascular disease in Alagille syndrome includes cerebral aneurysms, moyamoya arteriopathy and dolichoectasia. The prevalence of cerebrovascular disease in Alagille syndrome varies widely in the literature. OBJECTIVE: To determine the prevalence of cerebrovascular disease in our institution's Alagille patient population by employing a full primary review of all available neuroimaging. MATERIALS AND METHODS: An institutional review board-approved retrospective review of all Alagille syndrome patients seen at a tertiary care children's hospital from January 2000 to January 2014 was performed. All neuroimaging studies were reviewed for arterial or venous abnormalities. The prevalence of arterial and venous abnormalities was calculated and clinical outcomes were determined. RESULTS: Fifty-two patients with Alagille syndrome ranging in age from 11 months to 27 years were studied. Nineteen (37%) had dedicated vascular neuroimaging. Six (32%) had cerebral arterial disease, 4 with dolichoectasia, 3 with aneurysm(s) and 2 with moyamoya arteriopathy. Three of the four patients with dolichoectasia had associated aneurysm(s). Venous anomalies were present in 4 (21%) patients. One patient with moyamoya arteriopathy underwent revascularization procedures. No deaths were attributable to cerebrovascular disease. CONCLUSION: Cerebral vasculopathy is an important feature of Alagille syndrome and includes dolichoectasia, cerebral aneurysms and moyamoya arteriopathy. The high prevalence identified in our study suggests noninvasive vascular neuroimaging screening should be performed in this patient population. In addition to cerebral arterial abnormalities, alterations of venous development may be a feature of Alagille syndrome.

A comprehensive institutional overview of intrathecal nusinersen injections for spinal muscular atrophy.

BACKGROUND: Spinal muscular atrophy (SMA) is an autosomal-recessive neuromuscular disorder resulting in progressive muscle weakness. In December 2016, the U.S. Food and Drug Administration approved the first treatment for SMA, a drug named nusinersen (Spinraza) that is administered intrathecally. However many children with SMA have neuromuscular scoliosis or spinal instrumentation resulting in challenging intrathecal access. Therefore alternative routes must be considered in these complex patients. OBJECTIVE: To investigate routes of drug access, we reviewed our institutional experience of administering intrathecal nusinersen in all children with spinal muscular atrophy regardless of spinal anatomy or instrumentation. MATERIALS AND METHODS: We reviewed children with SMA who were referred for intrathecal nusinersen injections from March to December 2017 at our institution. In select children with spinal hardware, spinal imaging was requested to facilitate pre-procedure planning. Standard equipment for intrathecal injections was utilized. All children were followed up by their referring neurologist. RESULTS: A total of 104 intrathecal nusinersen injections were performed in 26 children with 100% technical success. Sixty procedures were performed without pre-procedural imaging and via standard interspinous technique. The remaining 44 procedures were performed in 11 complex (i.e. neuromuscular scoliosis or spinal instrumentation) patients requiring pre-procedural imaging for planning purposes. Nineteen of the 44 complex procedures were performed via standard interspinous technique from L2 to S1. Twenty-two of the 44 complex procedures were performed using a neural-foraminal approach from L3 to L5. Three of the 44 complex procedures were performed via cervical puncture technique. There were no immediate or long-term complications but there was one child with short-term complications of meningismus and back pain at the injection site. CONCLUSION: Although we achieved 100% technical success in intrathecal nusinersen administration, our practices evolved during the course of this study. As a result of our early experience we developed an algorithm to assist in promoting safe and effective nusinersen administration in children with spinal muscular atrophy regardless of SMA type, abnormal spinal anatomy and complex spinal instrumentation.

Predicting symptomatic cerebral vasospasm after aneurysmal subarachnoid hemorrhage with an artificial neural network in a pediatric population.

PURPOSE: Artificial neural networks (ANN) are increasingly applied to complex medical problem solving algorithms because their outcome prediction performance is superior to existing multiple regression models. ANN can successfully identify symptomatic cerebral vasospasm (SCV) in adults presenting after aneurysmal subarachnoid hemorrhage (aSAH). Although SCV is unusual in children with aSAH, the clinical consequences are severe. Consequently, reliable tools to predict patients at greatest risk for SCV may have significant value. We applied ANN modeling to a consecutive cohort of pediatric aSAH cases to assess its ability to predict SCV. METHODS: A retrospective chart review was conducted to identify patients < 21 years of age who presented with spontaneously ruptured, non-traumatic, non-mycotic, non-flow-related intracranial arterial aneurysms to our institution between January 2002 and January 2015. Demographics, clinical, radiographic, and outcome data were analyzed using an adapted ANN model using learned value nodes from the adult aneurysmal SAH dataset previously reported. The strength of the ANN prediction was measured between - 1 and 1 with - 1 representing no likelihood of SCV and 1 representing high likelihood of SCV. RESULTS: Sixteen patients met study inclusion criteria. The median age for aSAH patients was 15 years. Ten underwent surgical clipping and 6 underwent endovascular coiling for definitive treatment. One patient experienced SCV and 15 did not. The ANN applied here was able to accurately predict all 16 outcomes. The mean strength of prediction for those who did not exhibit SCV was - 0.86. The strength for the one patient who did exhibit SCV was 0.93. CONCLUSIONS: Adult-derived aneurysmal SAH value nodes can be applied to a simple AAN model to accurately predict SCV in children presenting with aSAH. Further work is needed to determine if ANN models can prospectively predict SCV in the pediatric aSAH population in toto; adapted to include mycotic, traumatic, and flow-related origins as well.

Imaging patterns of venous-related brain injury in children.

Venous-related brain injury is a common form of cerebrovascular injury in children and encompasses a diverse group of cerebrovascular diagnoses. The purpose of this pictorial essay is to introduce the relevant anatomy, pathophysiology and various imaging patterns of venous-related cerebral injury in children. Unifying concepts to better understand the effects of venous hypertension in the developing brain will be emphasized. These unifying concepts will provide the imaging professional with a conceptual framework to better understand and confidently identify imaging patterns of venous-related cerebral injury.

Selective Ophthalmic Artery Infusion Chemotherapy for Advanced Intraocular Retinoblastoma: CCHMC Early Experience.

Selective ophthalmic artery infusion chemotherapy (SOAIC) is increasingly used to treat retinoblastoma. We report the toxicities and outcome of 19 eyes in 17 patients with retinoblastoma receiving SOAIC treatment between 2008 and 2013. From the 87 treatments, mild local reactions were common. Myelosuppression was more common after triple-agent SOAIC (melphalan, carboplatin, and topotecan) than single-agent melphalan. Ocular salvage was achieved in 11 of 19 eyes and associated with triple-agent therapy. SOAIC is a effective therapy for some retinoblastoma with manageable toxicity; however, systemic toxicity increases with increasing therapeutic intensity of SOAIC.

Developmental venous anomalies of the brain in children -- imaging spectrum and update.

Developmental venous anomalies (DVAs) are the most common vascular malformation of the brain and are commonly identified on routine imaging of the brain. They are typically considered incidental findings, usually with no clinical significance. However the increasing identification of DVAs as a result of improved imaging technology has led to recognition of their association with a variety of abnormal imaging findings and clinically important conditions. This pictorial essay explores the suspected embryological origin, associated imaging features, and proposed pathophysiological mechanisms of DVAs in the pediatric population. This paper emphasizes newer physiological imaging data, which suggest that DVA drainage has less physiological flexibility than otherwise normal venous drainage development.

Comparison of pediatric radiation dose and vessel visibility on angiographic systems using piglets as a surrogate: antiscatter grid removal vs. lower detector air kerma settings with a grid - a preclinical investigation.

The purpose of this study was to reduce pediatric doses while maintaining or improv-ing image quality scores without removing the grid from X-ray beam. This study was approved by the Institutional Animal Care and Use Committee. Three piglets (5, 14, and 20 kg) were imaged using six different selectable detector air kerma (Kair) per frame values (100%, 70%, 50%, 35%, 25%, 17.5%) with and without the grid. Number of distal branches visualized with diagnostic confidence relative to the injected vessel defined image quality score. Five pediatric interventional radiologists evaluated all images. Image quality score and piglet Kair were statistically compared using analysis of variance and receiver operating curve analysis to define the preferred dose setting and use of grid for a visibility of 2nd and 3rd order vessel branches. Grid removal reduced both dose to subject and imaging quality by 26%. Third order branches could only be visualized with the grid present; 100% detector Kair was required for smallest pig, while 70% detector Kair was adequate for the two larger pigs. Second order branches could be visualized with grid at 17.5% detector Kair for all three pig sizes. Without the grid, 50%, 35%, and 35% detector Kair were required for smallest to largest pig, respectively. Grid removal reduces both dose and image quality score. Image quality scores can be maintained with less dose to subject with the grid in the beam as opposed to removed. Smaller anatomy requires more dose to the detector to achieve the same image quality score.

Geographic access to acute stroke care in the United States.

BACKGROUND AND PURPOSE: Only 3% to 5% of patients with acute ischemic stroke receive intravenous recombinant tissue-type plasminogen activator (r-tPA) and <1% receive endovascular therapy. We describe access of the US population to all facilities that actually provide intravenous r-tPA or endovascular therapy for acute ischemic stroke. METHODS: We used US demographic data and intravenous r-tPA and endovascular therapy rates in the 2011 US Medicare Provider and Analysis Review data set. International Classification of Diseases-Ninth Revision codes 433.xx, 434.xx and 436 identified acute ischemic stroke cases. International Classification of Diseases-Ninth Revision code 99.10 defined intravenous r-tPA treatment and International Classification of Diseases-Ninth Revision code 39.74 defined endovascular therapy. We estimated ambulance response times using arc-Geographic Information System's network analyst and helicopter transport times using validated models. Population access to care was determined by summing the population contained within travel sheds that could reach capable hospitals within 60 and 120 minutes. RESULTS: Of 370,351 acute ischemic stroke primary diagnosis discharges, 14,926 (4%) received intravenous r-tPA and 1889 (0.5%) had endovascular therapy. By ground, 81% of the US population had access to intravenous-capable hospitals within 60 minutes and 56% had access to endovascular-capable hospitals. By air, 97% had access to intravenous-capable hospitals within 60 minutes and 85% had access to endovascular hospitals. Within 120 minutes, 99% of the population had access to both intravenous and endovascular hospitals. CONCLUSIONS: More than half of the US population has geographic access to hospitals that actually deliver acute stroke care but treatment rates remain low. These data provide a national perspective on acute stroke care and should inform the planning and optimization of stroke systems in the United States.

Significant dose reduction for pediatric digital subtraction angiography without impairing image quality: preclinical study in a piglet model.

OBJECTIVE: The purpose of this study was to validate the hypothesis that image quality of digital subtraction angiography (DSA) in pediatrics is not impaired when using a low-dose acquisition protocol. MATERIALS AND METHODS: Three piglets corresponding to common pediatric population sizes were used. DSA was performed in the aorta and renal, hepatic, and superior mesenteric arteries using both the commonly used reference standard and novel radiographic imaging noise reduction technologies to ensure pairwise radiation dose and image quality comparison. The air kerma per frame at the interventional reference point for each DSA acquisition was collected as a radiation dose measure, and image quality was evaluated by five interventional radiologists in a randomized blinded fashion using a 5-point scale. RESULTS: The mean air kerma (± SD) at the interventional reference point with the novel x-ray imaging noise reduction technology was significantly lower (1.1 ± 0.8 mGy/frame) than with the reference technology (4.2 ± 3.0 mGy/frame, p = 0.005). However, image quality was statistically similar, with average scores of 3.2 ± 0.4 and 3.1 ± 0.5 for the novel and reference technologies, respectively (p = 0.934); interrater absolute agreement was 0.77. CONCLUSION: The DSA radiation dose for pediatrics can be reduced by a factor of four with a novel x-ray imaging noise reduction technology without deterioration of image quality.

Microvascular flow ultrasound imaging for retinoblastoma.

PURPOSE: To present the results of a pilot study of microvascular flow imaging (MFI) in characterizing tumor vasculature of retinoblastoma. METHODS: The medical records of consecutive patients with retinoblastoma presenting at our institution between July 2019 and June 2022 that were imaged using MFI were reviewed retroactively. Each patient underwent diagnostic evaluation according to standard of care by examination under anesthesia with fluorescein angiography and ocular ultrasound imaging, including color Doppler and MFI. RESULTS: Thirteen eyes of 10 patients with retinoblastoma were included. MFI showed a prominent feeder vessel in 8 eyes, basket vasculature in 6 eyes and tumor bed vascularity in 10 eyes. MFI showed a more extensive vascular branching pattern that was not visible on color Doppler and fluorescein angiography in all eyes. CONCLUSIONS: MFI of retinoblastoma patients could add information about tumor vascularity not detectable by color Doppler or fluorescein angiography. Further study is needed to determine whether this information could be used to predict prognosis for ocular salvage and tumor response to treatment.

Reversal of Cerebral Arteriopathy Post-Hematopoietic Stem Cell Transplant for Sickle Cell Disease.

Sickle cell disease (SCD) is a chronic hematologic disorder which causes progressive cerebral arteriopathy beginning in childhood. As a result, arterial ischemic stroke is a major cause of morbidity and mortality in SCD, and SCD is a leading cause of childhood stroke worldwide. Allogenic hematopoietic stem cell transplant (HSCT) may be curative for individuals with SCD. Long-term outcomes and effects are currently being studied. In this report, we describe a child with SCD who presented with arterial ischemic stroke at 6 years of age and was found to have a severe form of cerebral large vessel arteriopathy by catheter-directed angiography. The patient initially underwent revascularization surgery by indirect superficial temporal artery to middle cerebral artery bypass, and 1 year later, he underwent curative HSCT. Approximately 3 years after HSCT, repeat catheter-directed angiography revealed a striking reversal of cerebral large vessel arteriopathy. This article reveals a previously unrecognized and potentially beneficial effect of HSCT that may ameliorate cerebral large vessel arteriopathy and improve cerebrovascular health for children with SCD.

Changes in autonomic function and cerebral and somatic oxygenation with arterial flow pulsatility for children requiring veno-arterial extracorporeal membrane oxygenation.

Background: Veno-arterial extracorporeal membrane oxygenation (VA-ECMO) carries variability in arterial flow pulsatility (AFP). Research question: What changes in cerebral and somatic oxygenation, hemodynamics, and autonomic function are associated with AFP during VA-ECMO? Methods: This is a prospective study of children on VA-ECMO undergoing neuromonitoring. AFP was quantified by arterial blood pressure pulse amplitude and subcategorized: no pulsatility (<1 mmHg), minimal pulsatility (1 to <5 mmHg), moderate pulsatility (5 to <15 mmHg) and high pulsatility (≥15 mmHg). CVPR was assessed using the cerebral oximetry index (COx). Cerebral and somatic oxygenation was assessed using cerebral regional oximetry (rSO2) or peripheral oxygen saturation (SpO2). Autonomic function was assessed using baroreflex sensitivity (BRs), low-frequency high-frequency (LF/HF) ratio and standard deviation of heart rate R-R intervals (HRsd). Differences were assessed across AFP categories using linear mixed effects models with Tukey pairwise comparisons. Univariate logistic regression was used to explore risk of AFP with brain injuries. Results: Among fifty-three children, comparisons of moderate to high pulsatility were associated with reductions in rSO2 (p < 0.001), SpO 2 (p = 0.005), LF/HF ratio (p = 0.028) and an increase in HRsd (p < 0.001). Reductions in BRs were observed across comparisons of none to minimal (P < 0.001) and minimal to moderate pulsatility (p = 0.004). Comparisons of no to low pulsatility were associated with reductions in BRs (p < 0.001) and ABP (p < 0.001) with increases in SpO2 (p < 0.001) and HR (p < 0.001). Arterial ischemic stroke was associated with higher pulsatility (p = 0.0384). Conclusion: During VA-ECMO support, changes toward high AFP are associated with autonomic dysregulation and compromised cerebral and somatic tissue oxygenation.

Three-Dimensional Ultrasound Imaging of Retinoblastoma and Its Correlation With Pathology.

BACKGROUND AND OBJECTIVE: Monitoring the response of retinoblastoma to globe-salvaging therapies is based on subjective assessments of changes determined by fundoscopy, ultrasound, and optical coherence tomography. Advances in organ-preserving therapies have increased the need for objective, quantitative estimates of tumor response to treatment. Primary tumor volume is a metric that can be objectively determined as a surrogate measure of treatment response. PATIENTS AND METHODS: We evaluated the correlation of objective, quantitative estimates of tumor volume made with two-dimensional (2D) and three-dimensional (3D) ultrasound with gold standard pathological tumor volumes derived by analysis of enucleation specimens. RESULTS: Twelve eyes in 12 patients undergoing primary enucleation were evaluated by 2D and 3D ultrasound during ophthalmic examination under anesthesia prior to enucleation. 2D- and 3D-ultra-sound measurements of tumor volume were both strongly correlated with pathological estimates of tumor volume (r = 0.69, P = 0.018; and r = 0.66, P = 0.027, respectively). CONCLUSIONS: 2D- and 3D-ultrasound measurements of retinoblastoma primary tumor volume are highly correlated with pathological estimates. 3D measurements are easy to perform with volumetric probes and consider the irregular morphology of the tumor. Further study should be undertaken to evaluate the performance of these metrics as surrogate markers of tumor response to treatment. [Ophthalmic Surg Lasers Imaging Retina 2024;55:136-140.].

Safety and efficacy of dynamic catheter-directed cerebral digital subtraction angiography for diagnosis of bowhunter syndrome spectrum disorders: A systematic review of the literature.

INTRODUCTION: Dynamic catheter-directed cerebral digital subtraction angiography (dcDSA) is the gold standard for diagnosing dynamic vascular occlusion syndromes such as bowhunter syndrome (BHS). Nonetheless, concerns about its safety exist and no standardized protocols have been published to date. METHODS: We describe our methodology and insights regarding the use of dcDSA in patients with BHS. We also perform a systematic literature review to identify cases of typical and atypical presentations of BHS wherein dcDSA was utilized and report on any procedural complications related to dcDSA. RESULTS: Our study included 104 cases wherein dcDSA was used for the diagnosis of BHS. There were 0 reported complications of dcDSA. DcDSA successfully established diagnosis in 102 of these cases. Thirty-eight cases were deemed atypical presentations of BHS. Fourteen patients endorsed symptoms during neck flexion/extension. In eight cases, there was dynamic occlusion of bilateral vertebral arteries during a single maneuver. Three patients had multiple areas of occlusion along a single vertebral artery (VA). An anomalous entry of the VA above the C6 transverse foramen was observed in four patients. One patient had VA occlusion with neutral head position and recanalization upon contralateral lateral head tilt. CONCLUSION: Our study highlights the safety and diagnostic benefits of dcDSA in characterizing the broad spectrum of BHS pathology encountered in clinical practice. This technique offers a powerful means to evaluate changes in cerebral blood flow and cervical arterial morphology in real time, overcoming the constraints of static imaging methods. Our findings pave the way for further studies on dcDSA to enhance cross-sectional imaging methods for the characterization of BHS and other dynamic vascular occlusion syndromes.

Is intra-arterial thrombolysis beneficial for M2 occlusions? Subgroup analysis of the PROACT-II trial.

BACKGROUND AND PURPOSE: The role of endovascular therapy for acute M2 trunk occlusions is debatable. Through a subgroup analysis of Prolyse in Acute Cerebral Thromboembolism-II, we compared outcomes of M2 occlusions in treatment and control arms. METHODS: Solitary M2 occlusions were identified from the Prolyse in Acute Cerebral Thromboembolism-II database. Primary endpoints were successful angiographic reperfusion (TICI 2-3) at 120 minutes and functional independence (mRS 0-2) at 90 days. RESULTS: Forty-four patients with solitary M2 occlusions, 30 in the treatment arm and 14 in the control arm, were identified. Successful reperfusion (TICI 2-3) was achieved in 53.6% and 16.7% of patients in the treatment and control arms, respectively (P=0.04). A favorable clinical outcome (mRS 0-2) was observed in 53.3% and 28.6%, respectively (P=0.19). Baseline characteristics were similar between the 2 groups. CONCLUSIONS: Intra-arterial thrombolysis may lead to a 3-fold increase in the rate of early reperfusion of solitary M2 occlusions and could potentially double the chance of a favorable functional outcome at 90 days. Clinical Trial Registration- This trial was not registered because enrollment began before July 1, 2005.