The use of sodium MRI in the diagnosis of an anaplastic astrocytoma during immunotherapy: a case report.

Gliomas in the pediatric population are targeted with immune-modulating therapies. The gold standard imaging modality for diagnosis and monitoring treatment response is magnetic resonance imaging (MRI); however, the complex post-therapy-induced changes can make treatment response assessment difficult. These include radiation necrosis, pseudoresponse, and pseudoprogression, as well as more complex responses in the setting of immunotherapy. We report a case of an 11-year-old male with a supratentorial astrocytoma (WHO grade 3) that underwent treatment with immunotherapy. There was a clinical concern for progression due to increased fluid-attenuated inversion recovery (FLAIR) hyperintensity at the site of the primary neoplasm during immunotherapy. However, the Sodium (23Na) MRI continued demonstrating decreased total sodium concentrations, supporting pseudoprogression over true progression, which was confirmed clinicaly. This case reports the capability of 23Na MRI to differentiate between progression, recurrence, and other posttreatment changes.

Neonatal neurosonography practices: a survey of active Society for Pediatric Radiology members.

BACKGROUND: While neonatal brain US is emerging as an imaging modality with greater portability, widespread availability and relative lower cost compared to MRI, it is unknown whether US is being maximized in infants to increase sensitivity in detecting intracranial pathology related to common indications such as hemorrhage, ischemia and ventriculomegaly. OBJECTIVE: To survey active members of the Society for Pediatric Radiology (SPR) regarding their utilization of various cranial US techniques and reporting practices in neonates. MATERIALS AND METHODS: We distributed an online 10-question survey to SPR members to assess practice patterns of neonatal cranial US including protocol details, use of additional sonographic views, perceived utility of spectral Doppler evaluation, and germinal matrix hemorrhage and ventricular size reporting preferences. RESULTS: Of the 107 institutions represented, 90% of respondents were split evenly between free-standing children's hospitals and pediatric departments attached to a general hospital. We found that most used template reporting (72/107, 67%). The anterior fontanelle approach was standard practice (107/107, 100%). We found that posterior fontanelle views (72% sometimes, rarely or never) and high-frequency linear probes to evaluate far-field structures (52% sometimes, rarely or never) were seldom used. Results revealed a range of ways to report germinal matrix hemorrhage and measure ventricular indices to assess ventricular dilatation. There was substantial intra-institutional protocol and reporting variability as well. CONCLUSION: Our results demonstrate high variability in neurosonography practice and reporting among active SPR members, aside from the anterior fontanelle views, template reporting and linear high-resolution near-field evaluation. Standardization of reporting germinal matrix hemorrhage and ventricular size would help ensure a more consistent application of neonatal US in research and clinical practice.

Fetal aqueductal stenosis: Prenatal diagnosis and intervention.

Fetal severe central nervous system ventriculomegaly is associated with poor neurologic outcomes, usually driven by a primary malformation, deformation, or disruption of brain parenchyma. In utero shunting of excess cerebrospinal fluid (CSF) in hopes of improving neurologic outcomes was attempted in the 1980s but was abandoned due to perceived lack of effect, likely due to technological limitations of the time that precluded proper patient selection. Little progress on the antenatal management of severe ventriculomegaly has been made in the intervening decades. A multidisciplinary, evidence-based reassessment of ventriculoamniotic shunting for isolated fetal aqueductal stenosis (FAS), a unique form of severe ventriculomegaly (supratentorial intracranial hypertension), is currently underway. An accurate diagnosis of FAS must precede in utero intervention. Magnetic resonance imaging (MRI) will be an excellent adjunct to high-resolution prenatal ultrasound and next-generation genetic testing to correctly diagnose FAS in a timely fashion while excluding other intracranial and extracranial anomalies. This manuscript will briefly discuss the history, current management, and future directions of the prenatal diagnosis and potential intervention for FAS.

Presenting Characteristics Associated With Outcome in Children With Severe Traumatic Brain Injury: A Secondary Analysis From a Randomized, Controlled Trial of Therapeutic Hypothermia.

OBJECTIVES: To identify injury patterns and characteristics associated with severe traumatic brain injury course and outcome, within a well-characterized cohort, which may help guide new research and treatment initiatives. DESIGN: A secondary analysis of a phase 3, randomized, controlled trial that compared therapeutic hypothermia versus normothermia following severe traumatic brain injury in children. SETTING: Fifteen sites in the United States, Australia, and New Zealand. PATIENTS: Children (< 18 yr old) with severe traumatic brain injury. MEASUREMENTS AND MAIN RESULTS: Baseline, clinical, and CT characteristics of patients (n = 77) were examined for association with mortality and outcome, as measured by the Glasgow Outcome Scale-Extended Pediatric Revision 3 months after traumatic brain injury. Data are presented as odds ratios with 95% CIs. No demographic, clinical, or CT characteristic was associated with mortality in bivariate analysis. Characteristics associated with worse Glasgow Outcome Scale-Extended Pediatric Revision in bivariate analysis were two fixed pupils (14.17 [3.38-59.37]), abdominal Abbreviated Injury Severity score (2.03 [1.19-3.49]), and subarachnoid hemorrhage (3.36 [1.30-8.70]). Forward stepwise regression demonstrated that Abbreviated Injury Severity spine (3.48 [1.14-10.58]) and midline shift on CT (8.35 [1.05-66.59]) were significantly associated with mortality. Number of fixed pupils (one fixed pupil 3.47 [0.79-15.30]; two fixed pupils 13.61 [2.89-64.07]), hypoxia (5.22 [1.02-26.67]), and subarachnoid hemorrhage (3.01 [1.01-9.01]) were independently associated with worse Glasgow Outcome Scale-Extended Pediatric Revision following forward stepwise regression. CONCLUSIONS: Severe traumatic brain injury is a clinically heterogeneous disease that can be accompanied by a range of neurologic impairment and a variety of injury patterns at presentation. This secondary analysis of prospectively collected data identifies several characteristics associated with outcome among children with severe traumatic brain injury. Future, larger trials are needed to better characterize phenotypes within this population.

Imaging for Oncologic Response Assessment in Lymphoma.

OBJECTIVE: The purpose of this article is to examine the role of different imaging biomarkers, focusing in particular on the use of updated CT and PET response criteria for the assessment of oncologic treatment effectiveness in patients with lymphoma but also discussing other potential functional imaging methods and their limitations. CONCLUSION: Lymph nodes are commonly involved by metastatic solid tumors as well as by lymphoma. Evolving changes in cancer therapy for lymphoma and metastases have led to improved clinical outcomes. Imaging is a recognized surrogate endpoint that uses established criteria based on changes in tumor bulk to monitor the effects of treatment. With the introduction of targeted therapies and novel antiangiogenic drugs, the oncologic expectations from imaging assessment are changing to move beyond simple morphologic methods. Molecular and functional imaging methods (e.g., PET, perfusion, DWI, and dual-energy CT) are therefore being investigated as imaging biomarkers of response and prognosis. The role of these advanced imaging biomarkers extends beyond measuring tumor burden and therefore might offer insight into early predictors of therapeutic response. Despite the potential benefits of these exciting imaging biomarkers, several challenges currently exist.

Midbrain morphology reflects extent of brain damage in Krabbe disease.

INTRODUCTION: To study the relationships between midbrain morphology, Loes score, gross motor function, and cognitive function in infantile Krabbe disease. METHODS: Magnetic resonance imaging (MRI) scans were evaluated by two neuroradiologists blinded to clinical status and neurodevelopmental function of children with early or late infantile Krabbe disease. A simplified qualitative 3-point scoring system based on midbrain morphology on midsagittal MRI was used. A score of 0 represented normal convex morphology of the midbrain, a score of 1 represented flattening of the midbrain, and a score of 3 represented concave morphology of the midbrain (hummingbird sign). Spearman correlations were estimated between this simplified MRI scoring system and the Loes score, gross motor score, and cognitive score. RESULTS: Forty-two MRIs of 27 subjects were reviewed. Analysis of the 42 scans showed normal midbrain morphology in 3 (7.1%) scans, midbrain flattening in 11 (26.2%) scans, and concave midbrain morphology (hummingbird sign) in 28 (66.7%) scans. Midbrain morphology scores were positively correlated with the Loes score (r = 0.81, p < 0.001) and negatively correlated with both gross motor and cognitive scores (r = -.84, p < 0.001; r = -0.87, p < 0.001, respectively). The inter-rater reliability for the midbrain morphology scale was κ = .95 (95% CI: 0.86-1.0), and the inter-rater reliability for the Loes scale was κ = .58 (95% CI: 0.42-0.73). CONCLUSIONS: Midbrain morphology scores of midsagittal MRI images correlates with cognition and gross motor function in children with Krabbe disease. This MRI scoring system represents a simple but reliable method to assess disease progression in patients with infantile Krabbe disease.

Multiplanar reconstructed CT images increased depiction of intracranial hemorrhages in pediatric head trauma.

INTRODUCTION: The benefits of multiplanar reconstructed images (MPR) of unenhanced axial head computed tomography (CT) data have not been established in trauma patients younger than 3 years old, a population in which a reliable history and physical examination may be most difficult. We retrospectively evaluated unenhanced head CTs in pediatric trauma patients to investigate the various benefits of MPR in this age group. METHODS: A total of 221 unenhanced head CTs performed for any case of head trauma (HT) on children younger than 3 years old were independently reviewed by two radiologists. Studies were reviewed first in the standard axial plane alone and then with the addition of MPR. Reviewers were asked to determine if the MPR affected the ability to make findings of hemorrhage, incidental findings, and artifacts. RESULTS: MPR improved the detection of hemorrhage in 14 cases (6.5 %, p-value < 0.01) and incidental findings in five cases (2.3 %, p-value < 0.05) as well as helped prove artifacts in five cases (2.3 %, p-value < 0.05). CONCLUSION: Routine use of MPR in HT patients younger than 3 years old has the potential to increase the detection of acute and incidental imaging findings.

Fishtail deformity--a delayed complication of distal humeral fractures in children.

BACKGROUND: Concavity in the central portion of the distal humerus is referred to as fishtail deformity. This entity is a rare complication of distal humeral fractures in children. OBJECTIVE: The purpose of this study is to describe imaging features of post-traumatic fishtail deformity and discuss the pathophysiology. MATERIALS AND METHODS: We conducted a retrospective analysis of seven cases of fishtail deformity after distal humeral fractures. RESULTS: Seven children ages 7-14 years (five boys, two girls) presented with elbow pain and history of distal humeral fracture. Four of the seven children had limited range of motion. Five children had prior grade 3 supracondylar fracture treated with closed reduction and percutaneous pinning. One child had a medial condylar fracture and another had a lateral condylar fracture; both had been treated with conservative casting. All children had radiographs, five had CT and three had MRI. All children had a concave central defect in the distal humerus. Other imaging features included joint space narrowing with osteophytes and subchondral cystic changes in four children, synovitis in one, hypertrophy or subluxation of the radial head in three and proximal migration of the ulna in two. CONCLUSION: Fishtail deformity of the distal humerus is a rare complication of distal humeral fractures in children. This entity is infrequently reported in the radiology literature. Awareness of the classic imaging features can result in earlier diagnosis and appropriate treatment.

Fallopian tube torsion in the pediatric age group: radiologic evaluation.

Fallopian tube torsion is a rare but important cause of acute pelvic pain in young adolescent girls. It is a surgical emergency treated with either detorsion or salpingectomy. The imaging findings can be nonspecific and challenging. However, an accurate early diagnosis is essential for prompt surgical treatment. Our objective was to review whether imaging findings can be specific enough to suggest the diagnosis of tubal torsion prospectively in the appropriate clinical setting. An Institutional Review Board-approved retrospective review of our imaging database from 2005 to 2012 revealed 10 surgically proven cases of fallopian tube torsion. All cases had sonography performed; 5 cases had additional multidetector computed tomography. All 10 patients (9-17 years) presented with acute pelvic pain. Sonographic findings included dilated tubular structures in 6 of 10 cases: adjacent to a normal ipsilateral ovary in 5 of 6 and adjacent to a benign ovarian teratoma in 1. In 4 cases, no dilated tube was identified; 3 of 4 had a cystic mass separate from the ovaries, and 1 had the imaging appearance of a multicystic ovary. Computed tomographic findings in the 5 cases that underwent multidetector computed tomography included a dilated tubular structure in 3 of 5; 2 of 5 had a cystic adnexal mass identified. Although rare, tubal torsion should be considered in female adolescents with acute pelvic pain. Sonography should be the first imaging choice. When a tubular structure or a midline cystic mass associated with a normal ipsilateral ovary is noted, tubal torsion should be considered in the differential diagnosis.

Phenobarbital-enhanced hepatobiliary scintigraphy in the diagnosis of biliary atresia: two decades of experience at a tertiary center.

BACKGROUND: Hepatobiliary scintigraphy is highly sensitive for diagnosing biliary atresia; however, its specificity has varied in the literature from 35% to 97%. OBJECTIVE: The purpose of this study was to re-evaluate the accuracy of phenobarbital-enhanced hepatobiliary scintigraphy in differentiating biliary atresia from other causes of neonatal cholestasis. MATERIALS AND METHODS: We retrospectively reviewed all hepatobiliary scans of infants with cholestasis at our institution from December 1990 to May 2011. Per our routine protocol the scans were obtained after pretreatment with phenobarbital (5 mg/kg/day for 5 days) to achieve a serum level of ≥15 mcg/ml. Normal hepatic uptake with no biliary excretion by 24 h was considered consistent with biliary atresia. RESULTS: One hundred eighty-six infants with 210 hepatobiliary scans composed the study group. Forty-three (23%) infants had the final diagnosis of biliary atresia. Hepatobiliary scintigraphy was 100% sensitive, 93% specific and 94.6% accurate in diagnosing biliary atresia. Of the 186, 39/111 (35.1%) term and 2/68 (2.9%) preterm infants had biliary atresia; two of seven children with unknown gestational age also had biliary atresia. Other diagnoses included neonatal hepatitis, total parenteral nutrition cholestasis, Alagille syndrome, cystic fibrosis, choledochal cyst, hypothyroidism, alpha-1 antitrypsin deficiency and persistent cholestasis of unknown etiology. CONCLUSION: Phenobarbital-enhanced hepatobiliary scintigraphy is highly accurate in differentiating biliary atresia from other causes of neonatal cholestasis. Biliary atresia is rare in premature infants.

Artificial Intelligence Automation of Proptosis Measurement: An Indicator for Pediatric Orbital Abscess Surgery.

INTRODUCTION: To evaluate the ability of artificial intelligence (AI) software to quantify proptosis for identifying patients who need surgical drainage. METHODS: We pursued a retrospective study including 56 subjects with a clinical diagnosis of subperiosteal orbital abscess (SPOA) secondary to sinusitis at a tertiary pediatric hospital from 2002 to 2016. AI computer software was developed to perform 3D visualization and quantitative assessment of proptosis from computed tomography (CT) images acquired at the time of hospital admission. The AI software automatically computed linear and volume metrics of proptosis to provide more practice-consistent and informative measures. Two experienced physicians independently measured proptosis using the interzygomatic line method on axial CT images. The AI software and physician proptosis assessments were evaluated for association with eventual treatment procedures as standalone markers and in combination with the standard predictors. RESULTS: To treat the SPOA, 31 of 56 (55%) children underwent surgical intervention, including 18 early surgeries (performed within 24 h of admission), and 25 (45%) were managed medically. The physician measurements of proptosis were strongly correlated (Spearman r = 0.89, 95% CI 0.82-0.93) with 95% limits of agreement of ± 1.8 mm. The AI linear measurement was on average 1.2 mm larger (p = 0.007) and only moderately correlated with the average physicians' measurements (r = 0.53, 95% CI 0.31-0.69). Increased proptosis of both AI volumetric and linear measurements were moderately predictive of surgery (AUCs of 0.79, 95% CI 0.68-0.91, and 0.78, 95% CI 0.65-0.90, respectively) with the average physician measurement being poorly to fairly predictive (AUC of 0.70, 95% CI 0.56-0.84). The AI proptosis measures were also significantly greater in the early as compared to the late surgery groups (p = 0.02, and p = 0.04, respectively). The surgical and medical groups showed a substantial difference in the abscess volume (p < 0.001). CONCLUSION: AI proptosis measures significantly differed from physician assessments and showed a good overall ability to predict the eventual treatment. The volumetric AI proptosis measurement significantly improved the ability to predict the likelihood of surgery compared to abscess volume alone. Further studies are needed to better characterize and incorporate the AI proptosis measurements for assisting in clinical decision-making.

Assessment of Brain Magnetic Resonance and Spectroscopy Imaging Findings and Outcomes After Pediatric Cardiac Arrest.

Importance: Morbidity and mortality after pediatric cardiac arrest are chiefly due to hypoxic-ischemic brain injury. Brain features seen on magnetic resonance imaging (MRI) and magnetic resonance spectroscopy (MRS) after arrest may identify injury and aid in outcome assessments. Objective: To analyze the association of brain lesions seen on T2-weighted MRI and diffusion-weighted imaging and N-acetylaspartate (NAA) and lactate concentrations seen on MRS with 1-year outcomes after pediatric cardiac arrest. Design, Setting, and Participants: This multicenter cohort study took place in pediatric intensive care units at 14 US hospitals between May 16, 2017, and August 19, 2020. Children aged 48 hours to 17 years who were resuscitated from in-hospital or out-of-hospital cardiac arrest and who had a clinical brain MRI or MRS performed within 14 days postarrest were included in the study. Data were analyzed from January 2022 to February 2023. Exposure: Brain MRI or MRS. Main Outcomes and Measures: The primary outcome was an unfavorable outcome (either death or survival with a Vineland Adaptive Behavior Scales, Third Edition, score of <70) at 1 year after cardiac arrest. MRI brain lesions were scored according to region and severity (0 = none, 1 = mild, 2 = moderate, 3 = severe) by 2 blinded pediatric neuroradiologists. MRI Injury Score was a sum of T2-weighted and diffusion-weighted imaging lesions in gray and white matter (maximum score, 34). MRS lactate and NAA concentrations in the basal ganglia, thalamus, and occipital-parietal white and gray matter were quantified. Logistic regression was performed to determine the association of MRI and MRS features with patient outcomes. Results: A total of 98 children, including 66 children who underwent brain MRI (median [IQR] age, 1.0 [0.0-3.0] years; 28 girls [42.4%]; 46 White children [69.7%]) and 32 children who underwent brain MRS (median [IQR] age, 1.0 [0.0-9.5] years; 13 girls [40.6%]; 21 White children [65.6%]) were included in the study. In the MRI group, 23 children (34.8%) had an unfavorable outcome, and in the MRS group, 12 children (37.5%) had an unfavorable outcome. MRI Injury Scores were higher among children with an unfavorable outcome (median [IQR] score, 22 [7-32]) than children with a favorable outcome (median [IQR] score, 1 [0-8]). Increased lactate and decreased NAA in all 4 regions of interest were associated with an unfavorable outcome. In a multivariable logistic regression adjusted for clinical characteristics, increased MRI Injury Score (odds ratio, 1.12; 95% CI, 1.04-1.20) was associated with an unfavorable outcome. Conclusions and Relevance: In this cohort study of children with cardiac arrest, brain features seen on MRI and MRS performed within 2 weeks after arrest were associated with 1-year outcomes, suggesting the utility of these imaging modalities to identify injury and assess outcomes.

Brain and spine MRI findings in children presenting with TMCO1 mutation.

Cerebrofaciothoracic dysplasia (CFTD) is a developmental disorder characterized by distinctive craniofacial dysmorphism, global developmental delay, and skeletal anomalies. CTFD is the result of biallelic autosomal recessive loss of function mutations in the transmembrane and coiled-coil domains one protein (TMCO1) gene. Based on a population of 27 molecularly confirmed cases, classic brain morphologies associated with CFTD have been described in the literature. Previous studies have demonstrated only mild ventriculomegaly, corpus callosum abnormalities, frontotemporal atrophy, and three cases of associated epilepsy. We present previously undescribed brain MRI findings in two children presenting with seizures due to TMCO1 mutation. MR Imaging demonstrated hippocampal malrotation, olfactory bulb agenesis and olfactory sulcus hypoplasia in both children, pontine hypoplasia, and cochlear nerve agenesis in one child. We demonstrate that TMCO1 may play a more extensive and previously undescribed role in neurodevelopment thereby expanding the phenotype associated with CFTD.

The clinical presentation and magnetic resonance imaging characteristics of an infant with idiopathic orbital inflammation.

Idiopathic orbital inflammation is rare in the pediatric population, particularly infants. It can be mistaken either for orbital infection or malignant tumors. We report the case of a 9-month-old previously healthy white boy who presented with right upper eyelid swelling. Magnetic resonance imaging revealed an abnormal T2-hypointense enhancing lesion within the extraconal superior right orbit.

Arterial Spin Labeling in Pediatric Neuroimaging.

Perfusion imaging using arterial spin labeling noninvasively evaluates cerebral blood flow utilizing arterial blood water as endogenous tracer. It does not require the need of radiotracer or intravenous contrast and offers unique complimentary information in the imaging of pediatric brain. Common clinical applications include neonatal hypoxic ischemic encephalopathy, pediatric stroke and vascular malformations, epilepsy and brain tumors. Future applications may include evaluation of silent ischemia in sickle cell patients, monitor changes in intracranial pressure in hydrocephalus, provide additional insights in nonaccidental trauma and chronic traumatic brain injury (TBI) and in functional Magnetic resonance imaging (MRI). The purpose of this review article is to evaluate the technical considerations including pitfalls, physiological variations, clinical applications and future directions of arterial spin labeling imaging.

Staging of colorectal cancer using contrast-enhanced multidetector computed tomographic colonography.

INTRODUCTION: Preoperative staging is essential for the optimal treatment and surgical planning of colorectal cancers. This study was aimed to evaluate the accuracy of colorectal cancer staging done using contrast-enhanced multidetector computed tomographic colonography (CEMDCTC). METHODS: We recruited 25 patients with 28 proven colorectal cancers. A 16-slice multidetector computed tomography scanner was used to generate two-dimensional multiplanar reformatted sagittal, coronal and oblique coronal images, and three-dimensional virtual colonography (endoluminal) images. Axial and reformatted views were analysed, and TNM staging was done. Patients underwent surgery and conventional colonoscopy, and surgical histopathological correlation was obtained. RESULTS: The diagnostic accuracies for TNM colorectal cancer staging were 92.3% for T staging, 42.3% for N staging and 96.1% for M staging using CEMDCTC. There was excellent positive correlation for T staging between CEMDCTC and both surgery (κ-value = 0.686) and histopathology (κ-value = 0.838) (p < 0.0001), and moderate positive correlation for N staging between CEMDCTC and surgery (κ-value = 0.424; p < 0.0001). The correlation between CEMDCTC and histopathology for N staging was poor (κ-value = 0.186; p < 0.05); the negative predictive value was 100% for lymph node detection. Moderate positive correlation was seen for M staging between CEMDCTC and both surgery (κ-value = 0.462) and histopathology (κ-value = 0.649). No false negatives were identified in any of the M0 cases. CONCLUSION: CEMDCTC correlated well with pathologic T and M stages, but poorly with pathologic N stage. It is an extremely accurate tool for T staging, but cannot reliably distinguish between malignant lymph nodes and enlarged reactive lymph nodes.

Duodenoduodenal intussusception.

Duodenoduodenal intussusception is a rare event which is usually caused by the presence of a tumor. We present a case of duodenoduodenal intussusception secondary to a large tubulovillous adenoma causing gastric outlet and biliary obstruction in a 50-year-old female. The imaging features on ultrasonography, CT, and MRI are described.