Pediatric Radiology最新文献

Background: Hindbrain herniation is part of Chiari II deformation and serves as an inclusion criterion for prenatal open neural tube defect (ONTD) repair. Its assessment on fetal MRI remains challenging, due to limited spatial resolution, fetal motion and difficulty obtaining a true mid-sagittal plane.

Objective: We aimed to evaluate the reproducibility of the Hindbrain herniation severity classification, based on repeated consensus readings by two evaluators.

Methods: We retrospectively reviewed fetuses who underwent prenatal ONTD repair at a single center (2011-2024), with pre- and 6 week post-operative T2-weighted fetal brain MRI scans. Hindbrain herniation was graded as: 0 = normal; 1 = effacement of the 4th ventricle and/or cisterna magna without Hindbrain herniation; 2 = Hindbrain herniation above the C1 posterior arch; 3 = Hindbrain herniation bellow C1 posterior arch. Two experienced fetal imaging specialists independently assessed all scans, resolved discrepancies by consensus, and repeated the evaluation two months later, blinded to initial grading. Reproducibility was assessed with weighted Cohen's Kappa overall and separately for pre- and post-operative scans.

Results: 121 fetuses were included. Postoperative MRI was available in 113 (93.4%). Overall reproducibility was excellent (κ = 0.87). Agreement was lower for pre-operative (κ = 0.57) than post-operative (κ = 0.74) scans (p < 0.01).

Conclusion: Hindbrain herniation grading on fetal MRI shows excellent overall reliability. The moderate agreement in preoperative scans reflects the difficulty of assessing Hindbrain herniation before prenatal repair and highlights the need for more standardized imaging criteria, when accurate assessment is necessary for surgical eligibility and parental counseling.

We report a case of Lemierre syndrome complicated by multiple pulmonary artery pseudoaneurysms. A previously healthy 11-year-old girl presented with pansinusitis complicated by internal jugular vein thrombosis consistent with a diagnosis of Lemierre syndrome. She presented an episode of massive hemoptysis secondary to pseudoaneurysm rupture requiring radiologically guided embolization. Blood cultures isolated Streptococcus constellatus. Prolonged antibiotic therapy was administered. The patient was discharged from hospital 1 month later and recovered uneventfully.

Background: Federal regulations require an audible alarm for each passage of 5 min of cumulative x-ray irradiation time during a diagnostic fluoroscopy procedure. In contrast to United States federal requirements, regulations vary amongst individual states.

Objective: Determine whether a shortened fluoroscopy time alarm reduces pediatric patient ionizing radiation exposure for modified barium swallow studies (MBSS).

Materials and methods: Fluoroscopy time alarms were changed from the passage of every 5 min to 2 min. A retrospective analysis of MBSS compared patient reference point air kerma and fluoroscopy time before and after the alarm time was shortened.

Results: A total of 3,875 MBSS performed on patients under the age of 18 years were analyzed. Reductions of 14.9% and 19.6% for average and median reference point air kerma (P<0.001) and 15.5% and 16.2% for average and median fluoroscopy time (P<0.001) were observed at the main campus for MBSS performed before and after changing the fluoroscopy time alarm from 5 min to 2 min. Reductions of 14.3% and 9.1% for average and median reference point air kerma (P<0.001) and 13.9% and 8.3% for average and median fluoroscopy time (P<0.001) were observed at the satellite locations for MBSS performed before and after changing the fluoroscopy time alarm.

Conclusion: This retrospective study suggests a shortened fluoroscopy time alarm can significantly lower pediatric patient dose and fluoroscopy time in MBSS. State and federal regulations should consider establishing flexible fluoroscopic alarm settings, especially when used for the pediatric population.

This case report describes a unique case of hemiconvulsion-hemiplegia-epilepsy syndrome in a paediatric patient with an underlying hypomyelinating leukodystrophy. We present the clinical, neuroimaging and genetic findings of a 3-year-old girl with a myelin deposition disorder who presented with a prolonged febrile status epilepticus, followed by persistent left hemiplegia. Brain magnetic resonance imaging (MRI) revealed a pattern consistent with hemiconvulsion-hemiplegia-epilepsy syndrome, with unilateral cytotoxic oedema and increased cerebral blood flow in the right cerebral hemisphere cortex. The girl was treated with corticosteroid therapy and levetiracetam. Follow-up imaging showed cerebral atrophy ipsilateral to the seizure focus. Incomplete myelination pattern remained unchanged. No prior cases associating hypomyelinating leukodystrophy with hemiconvulsion-hemiplegia-epilepsy syndrome have been reported. This case expands the phenotypic spectrum of hypomyelinating disorders and raises the hypothesis that an underlying white matter vulnerability may predispose to or modify the course of hemiconvulsion-hemiplegia-epilepsy syndrome. Recognition of such associations may have implications for prognosis and management, including seizure control strategies and neurorehabilitation. Further reports are needed to determine the role of myelination in the development of epileptic syndromes.

Background: Diffusion magnetic resonance imaging has emerged as an opportunity to explore brain white matter fiber tracts (WMFTs) through 3D digital reconstruction. This method could be useful in investigating the relationship between positional plagiocephaly and developmental problems; however, this has not been fully explored.

Objective: Evaluate WMFTs of healthy infants in two age groups with a range of positional plagiocephaly from normal to severe.

Materials and methods: This exploratory study, conducted at a free-standing, quaternary pediatric hospital in the Northeastern United States, utilized an existing database of healthy infants' MRIs obtained between 1 month and 4 months of age. MRIs were included if deemed good quality and had complete T1- and diffusion-weighted sequences and excluded if there were measurement disagreements or MRI data processing problems. Positional plagiocephaly severity was calculated using the Cranial Vault Asymmetry Index (CVAI). A repeated-measures regression model was constructed to assess the association of positional plagiocephaly severity with WMFTs fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD), and radial diffusivity (RD).

Results: Median age of 18 infants was 64.5 (IQR 71) days at the time of MRI. FA had a negative association with CVAI overall (β±SE=-0.53±0.51% per unit CVAI, P=0.32) and in both age groups. MD and RD had a positive association with CVAI overall (β±SE=1.31±0.46% per unit CVAI, P=0.013; β±SE=1.54±0.54% per unit CVAI, P=0.012) and in both age groups and all pathways.

Conclusion: As the severity of positional plagiocephaly increases, differences in WMFT formation are observed, suggesting the need for longitudinal studies with cognitive and behavioral assessments.

Cranial fasciitis is a rare pediatric skull tumor with a challenging radiological diagnosis, particularly when it arises in unusual locations or presents with atypical features. We report a case of cranial fasciitis originating from the ethmoid bone in a 17-month-old girl, presenting as isolated facial asymmetry instead of the usual scalp mass. Cross-sectional and metabolic imaging demonstrated an aggressive-appearing ethmoid sinus mass mimicking rhabdomyosarcoma, with extensive bone destruction and intracranial extension. The patient underwent complete surgical resection of the lesion. This report emphasizes the need to consider cranial fasciitis in the differential diagnosis of any aggressive-appearing skull mass in childhood, and underscores the importance of recognizing the wide anatomical and clinical spectrum of the lesion.

The integration of artificial intelligence (AI) in pediatric radiology requires an interdisciplinary approach that prioritizes transparency, accountability and collaboration between developers, clinicians and regulatory bodies. The development of AI models that are specifically designed to analyze pediatric imaging data has the potential to improve diagnosis and treatment outcomes, but it also requires careful consideration of the ethical implications. This review highlights the importance of the unique challenges posed by AI in pediatric imaging data, including regulatory hurdles, bias mitigation and the need for human oversight. Facing this situation, pediatric radiologists need to be equipped with the skills and knowledge to critically evaluate AI outputs and address potential biases and limitations. This requires ongoing education and training in pediatric radiology as well as AI. The integration of AI in pediatric radiology requires a collaborative approach that involves not only developers and clinicians but also patients and families. Ultimately, the integration of AI in pediatric imaging needs to be a coordinated effort from all stakeholders to prioritize the long-term safety and health of the young patients.

Background: Childhood obesity is rising worldwide, leading to an increased prevalence of metabolic dysfunction-associated steatotic liver disease, the most common pediatric liver disease. Ultrasound-derived fat fraction (UDFF) is a recently developed technique for quantifying hepatic fat that has been validated in children, providing a rapid, reliable, and non-invasive alternative to conventional imaging.

Objective: To evaluate the association between weight status, expressed as body mass index (BMI) z-score, and UDFF in a pediatric population without underlying liver disease.

Materials and methods: Abdominal ultrasounds including a UDFF measurement in patients without liver disease were retrospectively evaluated. We calculated the BMI z-score for all patients and classified them as normal weight (zBMI) or overweight (zBMI≥1). UDFF values were compared across zBMI categories, sex and ethnicity (Hispanic/Latino or non-Hispanic/non-Latino), using chi-square tests (P<0.05). Pearson's correlation was used to assess the relationship between continuous UDFF values and zBMI. Logistic regression analyses were performed to assess associations between elevated UDFF (>6%) and adiposity markers (zBMI and ultrasound-measured abdominal wall thickness (AWT)), as well as ethnicity.

Results: Of 223 subjects, 93 (41 males/52 females, mean age of 8.2 years) met the inclusion criteria. In the normal-weight population (n=46), 41 (89.1%) had normal UDFF, and 5 (10.9%) had elevated UDFF. In the overweight group (n =47), 29 (61.7%) had normal UDFF and 18 (38.3%) had elevated UDFF. UDFF values showed a positive correlation with zBMI, and higher zBMI increased the odds of elevated UDFF. In the multivariable model including zBMI, AWT, and ethnicity, ultrasound-measured abdominal wall thickness was the strongest predictor of elevated UDFF.

Conclusion: BMI z-score was positively associated with hepatic fat content and with higher odds of elevated UDFF. When multiple factors were considered together, ultrasound-measured abdominal wall thickness showed the strongest independent association with elevated UDFF supporting the central role of adiposity in pediatric hepatic fat accumulation. UDFF may serve as a valuable complement to routine clinical markers, using zBMI, for early identification of children with hepatic steatosis. Larger prospective studies are needed to define its role in clinical practice.