Introduction: While studies have shown an association between post-cardiac arrest hypotension and mortality after conventional CPR in children, there is a lack of evidence regarding optimal mean arterial pressure (MAP) targets after ECPR. Hypothesis: Amongst children with cardiac disease undergoing ECPR, the lowest MAP < 5 th percentile for age during the first 72 hours after return of circulation (ROC) is associated with decreased survival to hospital discharge (SHD) with favorable neurologic outcome (FNO). Methods: This is a single center retrospective cohort study of children admitted to a pediatric cardiac intensive care unit (CICU) from 2010 to 2022 that underwent ECPR. Chi-square test was used to analyze the association of the lowest MAP values, percentile adjusted for age, during the 72 hours post-ROC with SHD with FNO (defined by Pediatric Cerebral Performance Category [PCPC] score of 1,2 or 3 or no change in PCPC from baseline). Our secondary outcomes included acute composite injury (defined by the presence of seizures in the first 72 hours or neuroimaging injury within 14 days post arrest), and abnormal EEG background (defined as moderate or severe). As an exploratory analysis, we used Wilcoxon Rank Sum test to analyze the association of burden of MAP < 5 th percentile for age with our outcomes. Results: We analyzed 83 index ECPR events. SHD with FNO was observed for 35 index events (42%). Table 1 displays the characteristics of patients with SHD with FNO compared to those who died or had SHD without FNO. Patients with SHD with FNO had shorter duration of CPR and lower maximum lactate during the first 6 hours post ROC compared to both survivors without FNO and non-survivors. The majority of the cohort (79.5%) experienced a lowest MAP < 5th percentile for age during the first 72 hours post-ROC. Table 2 and 3 reveal no associations between MAP < 5 th percentile for age and primary or secondary outcomes. Conclusion: This retrospective cohort study of children with cardiac disease undergoing ECPR reveals no association between MAP < 5 th percentile for age during the 72 hours post-ROC and our primary outcome of SHD with FNO as well as secondary outcomes of acute composite injury and abnormal EEG background. Given heterogeneity in the cardiac population as well as the high incidence of hypotension <5 th percentile for age in our cohort, further investigation is warranted into whether certain subgroups may benefit from targeting higher MAP values after ECPR.

Background:Myelin oligodendrocyte glycoprotein antibody associated disease (MOGAD) is a relatively new disease entity in the field of demyelinating disorders. Its first diagnostic criteria have recently been published. Objectives:We evaluated the positive predictive value (PPV) for MOG-IgG testing and report the clinical and radiologic features with respect to the recently published criteria. Methods:A retrospective study was conducted at three centers in Dallas, Texas. Patients with positive MOG-IgG testing on cell-based assays at any time were included. Positive cases were reviewed by at least two neuroimmunologists for fulfillment of the criteria. Results:We included 235 patients. The PPV of seropositivity at any time was 78.3% overall, 52.6% for low titer, and 90.1% for high titer. Children had a higher PPV than adults (93.9% versus 67.2%). Positive predictive value was 6.3% in those without a core clinical demyelinating attack. Children more often have the typical imaging features of MOGAD in optic neuritis than adults. Conclusions:We report a PPV of 78.3% for MOG-IgG testing using the 2023 MOGAD diagnostic criteria. Children had higher PPV and frequency of supporting imaging features. Careful consideration is necessary when assigning patients with no core demyelinating event and low titers a MOGAD diagnosis.

Head and neck emergencies in children are frequent cause of visits to the hospital. Imaging plays a critical role in the management of these patients. This review article aims to familiarize radiologists with the common clinical presentations encountered, imaging characteristics of nontraumatic pediatric head and neck emergencies, and improve their ability to recognize associated complications as well as be aware of common mimics. We researched our database for commonly encountered nontraumatic head and neck emergencies in children. A literature search was done to compare and complete the list of conditions to be discussed in this review. The review was organized according to anatomical location of the emergent condition. Relevant anatomy has been discussed along with clinical presentation, imaging characteristics and complications. We have presented common mimics with each set of disorders. Key imaging characteristics have been delineated using radiology images. Familiarity with the known complications of head and neck emergencies allows the radiologist to actively search for such findings, encourage early institution of appropriate therapy, and improve outcomes.

Extracorporeal membrane oxygenation (ECMO) is a form of temporary cardiopulmonary bypass for patients with acute respiratory or cardiac failure refractory to conventional therapy. Its usage has become increasingly widespread and while reported survival after ECMO has increased in the past 25 years, the incidence of neurological injury has not declined, leading to the pressing question of how to improve time-to-detection and diagnosis of neurological injury. The neurological status of patients on ECMO is clinically difficult to evaluate due to multiple factors including illness, sedation, and pharmacological paralysis. Thus, increasing attention has been focused on developing tools and techniques to measure and monitor the brain of ECMO patients to identify dynamic risk factors and monitor patients’ neurophysiological state as a function in time. Such tools may guide neuroprotective interventions and thus prevent or mitigate brain injury. Current means to continuously monitor and prevent neurological injury in ECMO patients are rather limited; most techniques provide indirect or postinsult recognition of irreversible brain injury. This review will explore the indications, advantages, and disadvantages of standard-of-care, emerging, and investigational technologies for neurological monitoring on ECMO, focusing on bedside techniques that provide continuous assessment of neurological health.

Extracorporeal life support is lifesaving; however, many survivors suffer neurological injury and current monitoring tools are insufficient to guide therapy. We demonstrate continuous monitoring and serial neuroinflammatory assessment in the first 48 hours, comparing results to neuroimaging.

Various space occupying lesions can arise in the orbit, ranging from developmental anomalies to malignancies, and many of the diseases occurring in children are different from the pathologies in the adult population. As the clinical presentation is frequently nonspecific, radiologic evaluation is essential for lesion detection and characterization as well as patient management. While orbital masses may in some cases involve multiple compartments, a simple compartmental approach is the key for the diagnosis on imaging studies, and MRI is the modality of choice. This pictorial review presents the most common and characteristic non-emergent pediatric orbital lesions, stressing their MRI and CT appearances, including specific differentiating features. The lesions are subdivided into 4 compartments: intraocular, intraconal, extraconal, and orbital walls. Retinoblastoma, Coats disease and persistent fetal vasculature; optic pathway glioma and lymphovascular malformations; rhabdomyosarcoma, infantile hemangioma, neurofibroma and lymphoma; neuroblastoma, leukemia/myeloid sarcoma, Langerhans cell histiocytosis and dermoid are reviewed in their respective compartments.

Background and Objectives Elevated intracranial pressure (ICP) in myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) has been largely unexplored. The objectives of this study were to determine the frequency of increased ICP in MOGAD and its association with disease course and outcomes and to highlight cases requiring medical and/or surgical management of increased ICP. Methods In this retrospective, single-center cohort study, we examined the clinical and paraclinical data from the initial presentation and follow-up data of children diagnosed with MOGAD. In those with opening pressure (OP) measurements, univariate analyses were used to evaluate factors associated with increased ICP, which was defined as OP > 28 cm H2O. We also present a case series of patients with or without OP measurement who required medical and/or surgical management of increased ICP. Results Of 86 children with MOGAD, 43 (50.0%) had an OP recorded and 7 (8.1%) required ICP management. In those with OP recorded, the median (interquartile range) OP for the different MOGAD phenotypes were: 30.0 (22.8-41.6) (acute disseminated encephalomyelitis, ADEM), 20.5 (16.1-23.6) (optic neuritis), 17.0 (17.0-22.5) (myelitis), and 19.5 (16.5-29.3) (other) cm H20. Overall, 20.9% had increased ICP based on an OP > 28 cm H2O, of whom 77.8% presented with ADEM. In a subgroup analysis of those presenting with ADEM, those with an elevated ICP had longer hospital stay (p = 0.007) and neurologic disability (defined as modified Rankin Scale >1) (p = 0.049). In those with or without OP recorded, 7 (6 with ADEM, one with cerebral cortical encephalitis) required ICP-directed therapies. Findings on brain MRI in these 7 children revealed extensive disease burden with bilateral cerebral involvement and evidence of restricted diffusion. While neuropsychological data in this small subset revealed significant variability, all sustained identifiable deficits after discharge, including attention-deficit hyperactivity disorders and language and learning disorders. Discussion In pediatric MOGAD, increased OP and ADEM at initial presentation were associated with longer hospital stays and greater long-term morbidity. Although invasive ICP monitoring has not been specifically advocated in the management of MOGAD, it is important to recognize signs and symptoms of increased ICP in these patients and consider ICP monitoring and management strategies based on clinical and radiologic findings, especially in those presenting with ADEM and with OP > 28 cm H2O.

Objective:Explore the relationship between early hypotension after ECPR and survival to hospital discharge (SHD) with favorable neurologic outcome (FNO) in children with cardiac disease. Methods:Retrospective cohort study of patients undergoing ECPR at a single center pediatric cardiac intensive care unit. Hypotension was defined as MAP < 5th percentile for age. Primary and secondary exposure variables were presence and burden of hypotension respectively, during the first 6 h after ECPR. Our primary outcome was SHD with FNO defined by Pediatric Cerebral Performance Category score of 1-3 or no change from baseline. Secondary outcomes included acute central nervous system (CNS) injury via neuroimaging and EEG. Univariate and multivariable logistic regression analyses were performed. Results:We analyzed 82 index ECPR events from 2010 to 2022. Hypotension was observed for at least one MAP value in 36/82 (43.9%) of the cohort. The median [IQR] burden of hypotension was 0 [0,14.3]%. Patients with SHD with FNO had shorter CPR duration, lower number of epinephrine and calcium doses, and lower maximum lactate levels when compared to patients who died or had SHD without FNO. After controlling for potential confounders, there was no association between presence of hypotension or burden of hypotension and SHD, SHD with FNO, or acute CNS injury via neuroimaging and EEG. Conclusion:In children with cardiac disease, there was no association between early hypotension after ECPR and SHD with FNO. Multicenter studies are needed to better understand how early hypotension after ECPR affects neurologic outcomes in children with cardiac disease.

BACKGROUND AND OBJECTIVES:The clinical spectrum of myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is heterogenous and has evolved over time since the commercial availability of the anti-MOG antibody assay. Subclinical disease activity has been previously reported in the visual pathway, but prevalence data remains limited. We investigated subclinical optic neuritis (ON) based on changes on retinal nerve fiber layer (RNFL) thickness on optic coherence tomography (OCT) in pediatric patients who tested positive for the anti-MOG antibody.METHODS:In this retrospective, single-center cohort study, we examined children with MOGAD with at least one complete assessment of the anterior visual pathway. Subclinical ON was defined by structural visual system disease in the absence of a subjective complaint of vision loss, pain (particularly with eye movement), or color desaturation.RESULTS:Records were reviewed from 85 children with MOGAD, 67 of whom (78.8%) had complete records for review. Eleven children (16.4%) had subclinical ON on OCT. Ten had significant reductions in RNFL, of which one had two distinct episodes of decreased RNFL, and one had significant elevations in RNFL. Of the eleven children with subclinical ON, six (54.5%) had a relapsing disease course. We also highlighted the clinical course of three children with subclinical ON detected on longitudinal OCT, including two who had subclinical ON outside of clinical relapses.CONCLUSION:Children with MOGAD can have subclinical ON events that can manifest as significant reductions or elevations in RNFL on OCT. OCT should be used routinely in the management and monitoring of MOGAD patients.

Extracorporeal membrane oxygenation (ECMO) augments cardiopulmonary function in critically ill children through use of large vessel cannulation and an external pump-oxygenator system. While advances in ECMO have allowed more children to survive otherwise fatal illnesses, the incidence of significant neurologic injury, such as cerebral ischemia, hemorrhage, or brain death has not improved. Documented incidence of neurologic injury in ECMO survivors as determined by neuroimaging vary from 10% to 60%.1–3 The occurrence of neurologic injury among non-survivors is unknown because intra-ECMO head imaging is not uniformly performed. Intra-ECMO neuroimaging is limited to head ultrasound (HUS) or computed tomography (CT) due to incompatibility of the ECMO circuit with magnetic resonance imaging (MRI). CT is the preferred intra-ECMO modality due to its ability to identify both ischemic and smaller hemorrhagic lesions.1 MRI is more sensitive than CT in the detection of small foci of hemorrhage and acute ischemic stroke.4–6 No data exists to compare CT and MRI for the detection of significant neurologic injury among ECMO patients. Therefore, we conducted a retrospective cohort study to test the hypothesis that detection of serious neurologic injury is similar between intra-ECMO CT imaging and post-ECMO MRI among children who required ECMO. Materials and Methods This is a single-center retrospective review conducted in accordance with the Internal Review Board at the University of Texas Southwestern and Children's Health (STU 112015-001). Children under 19 years old who received ECMO during from January 2015 to December 2021 were eligible for study. Patients with both intra-ECMO CT and post-ECMO MRI were included. Neuroimaging Scoring and Classification All intra-ECMO CT and post-ECMO MRI data of patients were scored by two pediatric neuroradiologists who were blinded to the clinical information. The scoring system was adapted from a validated categorical scale first published by Taylor et al.7 and subsequently modified to include commonly seen injuries and validated with long-term outcomes.8 Neuroimaging scores were then grouped into the following two categories: severe injury (score ≥ 10) or not severe injury (score < 10).8 Statistical Analysis Injury scores ≥ 10 were used to indicate severe injury on either MRI or CT, and positive predictive value (PPV) and the negative predictive value (NPV) were calculated using the MRI assessment as the reference standard. Furthermore, PPV and NPV were estimated using two logistic regression models, treating the injury score on MRI (≥ 10 versus < 10) as a binary outcome. In one model, the injury score from CT was used as a continuous predictor. In the other model, the CT score was categorizing as "≥ 10" or "< 10" to treat it as a binary predictor. PPV and NPV were estimated in both models using a 0.5 probability cutoff. Results A total of 26 patients were included in the study. A slight majority of patients enrolled in the study were females (53%, n = 14) and supported on venovenous (VV) ECMO (VV = 15, venoarterial [VA] = 11) (Table 1). The average length of ECMO support was 240 hours (14–1,246 hours) and most patients survived to hospital discharge (84.6%, n = 22). Three patients were cannulated after a cardiopulmonary resuscitation (CPR) event. Neurologic injury was identified in 92% of patients (n = 24). CT had a positive predicted value of 71.4% and a negative predictive value of 68.4% (Table 2) of significant neurologic injury. From the logistic regression model using CT injury score as a binary predictor, estimates of PPV and NPV are the same (Supplemental Table 2B, https://links.lww.com/ASAIO/B55). Using CT injury score as a continuous predictor, estimates are calculated as PPV = 58.3% and NPV = 71.4% (Supplemental Table 2C, https://links.lww.com/ASAIO/B55). When considering patients whose MRI was within 15 days of CT, CT had a PPV = 100% and NPV = 71.4% (Supplemental Table 3A, https://links.lww.com/ASAIO/B55). Using CT injury score as a continuous predictor in this cohort, estimates are calculated as PPV = 70% and NPV = 77.8% (Supplemental Table 3B, https://links.lww.com/ASAIO/B55). Among seven patients, MRI identified small, acute ischemic injuries, or microhemorrhages not seen on intra-ECMO CT. Two patients had a CT consensus score that was higher than the MRI consensus score. Both patients had a hemorrhagic injury and more than 20 days between when the CT and MRI were obtained. Table 1. - Characteristics of Children Supported on ECMO Who Received Intra-ECMO and Post-ECMO Neuroimaging Patient Identification Age Gender ECMO Length of Run (Hours) Indications Survival to Hospital Discharge 1 8 months M VA 69 CHD Y 2 Newborn M VV 113 PPHN Y 3 23 months F VV 356 ARDS Y 4 7 y.o. M VV 96 FBO Y 5 6 y.o. F VV 343 ARDS Y 6 13 y.o. F VA 76 ARDS Y 7 6 months M VA 94 ECPR N 8 10 y.o. F VV 402 ARDS N 9 14 y.o. M VV 136 ARDS Y 10 4 y.o. F VA 96 Shock Y 11 17y.o. F VV 720 ARDS Y 12 17 y.o. F VA 93 CHD Y 13 16 y.o. M VV 368 ARDS Y 14 2 months M VV 330 ARDS Y 15 Newborn F VV 14 PPHN N 16 Newborn F VV 106 MAS Y 17 14y.o. M VV 86 ARDS Y 18 15 months M VV 1,262 ARDS Y 19 Newborn F VA 50 ECPR Y 20 Newborn F VA 48 CHD N 21 4 months F VA 150 CHD Y 22 17 months F VV 146 FBO Y 23 Newborn M VV 118 MAS Y 24 Newborn M VA 120 PPHN Y 25 6 months M VA 357 ECPR Y 26 4 months F VA 316 CHD Y ARDS, acute respiratory distress syndrome; CHD, congenital heart disease; ECMO, extracorporeal membrane oxygenation; ECPR, extracorporeal cardiopulmonary resuscitation; F, female; FBO, foreign body obstruction; ID, identification; M, male; MAS, meconium aspiration syndrome; N, no; PPHN, persistent pulmonary hypertension of the newborn; VA, venoarterial; VV, venovenous; Y, yes; y.o., years old. Table 2. - Injury Severity Determined by CT Versus MRI for Children Supported on ECMO (n = 26) Results From CT Sreening Status of Person According to Reference Standard (MRI) Predictive Values Severe Injury No Severe Injury Positive for severe injury 5 2 PPV = 71.4% Negative for severe injury 6 13 NPV = 68.4% CT, computed tomography; ECMO, extracorporeal membrane oxygenation; MRI, magnetic resonance imaging; NPV, negative predictive value; PPV, positive predictive value. Discussion Neurologic injury continues to be a major contributor to morbidity and mortality for patients supported on ECMO. Currently, no guidelines exist for the use of head imaging to assess neurologic health while on ECMO. Presumably, intra-ECMO head CT is obtained primarily in the setting of clinical concern for neurologic injury, but the correlation of this assessment with MRI is not known. Our results suggest intra-ECMO CT is a reasonable modality to assess for significant neurologic injury in children when MRI is not feasible. Clinical decision-making may, then, be guided by CT findings. Notably, in patients who received CT and MRI within 15 days, CT had a 100% PPV for significant neurologic injury (Supplemental Table 3A, https://links.lww.com/ASAIO/B55). Consistent with previous studies, MRI improved identification of smaller ischemic lesions and microhemorrhages than CT.4,6 This study was limited by a small sample size and retrospective design. The precise timing of neurologic injuries is unknown; thus, some patients may have developed an injury in the time period between obtaining intra-ECMO CT and post-ECMO MRI. This may bias our results as MRI findings may indicate injuries which occurred after CT. Therefore, our results may under-estimate the ability of CT to assess significant injuries as compared to MRI. Additionally, information on neurologic symptoms prior to obtaining imaging was not available for analysis. Thus, we are unable to draw any conclusions on clinical management based on neurologic signs. Finally, long-term functional outcomes and quality of life assessments in survivors with neurologic complications was not available for analysis and is an important future area of study. In conclusion, detection of serous neurologic injury with intra-ECMO head CT was correlated with post-ECMO MRI in children who received imaging with both modalities. Intra-ECMO head CT should be considered and utilized to guide clinical management in children on ECMO.