Arterial spin-labeled (ASL) perfusion in children with Sturge-Weber syndrome: a retrospective cross-sectional study.

PURPOSE: Sturge-Weber syndrome (SWS) is a developmental disorder with venous hypertension and associated tissue responses including pial angiomatosis, cortical calcifications, and cerebral atrophy. Arterial spin-labeled (ASL) perfusion is an advanced MR sequence which can assess perfusion, without the need for contrast. We systematically evaluated the potential benefits of using ASL in Sturge-Weber syndrome, to determine the extent of intracranial perfusion abnormality and stage of disease, relevant for prognostication and surgical planning. METHODS: Two pediatric neuroradiologists retrospectively evaluated ASL perfusion imaging of 31 children with confirmed SWS and recorded the presence of hyper-perfusion, hypo-perfusion, or normal perfusion. The presence and distribution of ASL abnormality were compared against the presence and side of atrophy/calcification and pial angiomatosis on standard MR sequences. RESULTS: Thirty-one children (52% female, median age 16.7 months) with SWS had ASL imaging. Seven (23%) had hyper-perfusion, 15 (48%) had hypo-perfusion, and 9 (29%) had no perfusion abnormalities. ASL perfusion abnormality matched the location of SWS findings on conventional imaging in 86% (19/22). ASL demonstrated statistically significant increased perfusion in the early stage of the disease and decreased perfusion when there was atrophy. The parietal lobe was involved in 86% of cases. CONCLUSION: ASL perfusion imaging is an advanced technique which may contribute to earlier diagnosis and more accurate prognostication of Sturge-Weber syndrome, helping guide management and potential surgical planning.

Imaging of Suspected Stroke in Children, From the AJR Special Series on Emergency Radiology.

Pediatric stroke encompasses different causes, clinical presentations, and associated conditions across ages. Although it is relatively uncommon, pediatric stroke presents with poor short- and long-term outcomes in many cases. Because of a wide range of overlapping presenting symptoms between pediatric stroke and other more common conditions, such as migraine and seizures, stroke diagnosis can be challenging or delayed in children. When combined with a comprehensive medical history and physical examination, neuroimaging plays a crucial role in diagnosing stroke and differentiating stroke mimics. This review highlights the current neuroimaging workup for diagnosing pediatric stroke in the emergency department, describes advantages and disadvantages of different imaging modalities, highlights disorders that predispose children to infarct or hemorrhage, and presents an overview of stroke mimics. Key differences in the initial approach to suspected stroke between children and adults are also discussed.

The role of ultrasound in necrotizing enterocolitis.

Ultrasound has proved to be a useful modality for enhancing the diagnostic accuracy of necrotizing enterocolitis and associated complications. The standard imaging algorithm for evaluating necrotizing enterocolitis includes radiographs and clinical symptoms, the combination of which constitutes the Bell criteria. Major limitations of using the Bell criteria for diagnosing and clinically managing necrotizing enterocolitis include low diagnostic accuracy of radiographs and nonspecific symptomatology of preterm infants. In this regard, US can offer additional insights into bowel health by helping to characterize bowel motility, echogenicity, thickness, pneumatosis and perfusion. Extramural findings such as portal venous gas, nature and extent of ascites, and pneumoperitoneum can also be assessed. Recently, contrast-enhanced US was explored in a case series of preterm bowel disease and its diagnostic utility warrants further investigation. This article reviews the US features of necrotizing enterocolitis and highlights the role of US as a complement to radiographs, as well as the emerging use of contrast-enhanced US in necrotizing enterocolitis.

Validation of Sonographic Fronto-Occipital Ratio Based on Anatomical Landmarks Compared to MR/CT-Derived Indexes in Children with Chiari II and Ventriculomegaly.

INTRODUCTION: Ultrasound (US)-based indexes such as fronto-occipital ratio (FOR) can be used to obtain an acceptable estimation of ventricular volume. Patients with colpocephaly present a unique challenge due to the shape of their ventricles. In the present study, we aimed to evaluate the validity and reproducibility of the modified US-FOR index in children with Chiari II-related ventriculomegaly. METHODS: In this retrospective study, we evaluated Chiari II patients younger than 1 year who underwent head US and MR or CT scans for ventriculomegaly evaluation. MR/CT-based FOR was measured in the axial plane by identifying the widest diameter of frontal horns, occipital horns, and the interparietal diameter (IPD). US-based FOR (US-FOR) was measured using the largest diameter based on the following landmarks: frontal horn and IPD in the coronal plane at the level of the foramen of Monro, IPD just superior to the Sylvian fissures, and occipital horn posterior to the thalami and inferior to the superior margins of the thalami. Intraclass correlation coefficients (ICCs) were used to evaluate inter-rater reliability, and Pearson correlation coefficients and Bland-Altman plots were applied to assess agreement between US and other two modalities. RESULTS: Sixty-three paired US and MR/CT exams were assessed for agreement between US-FOR and MR/CT-FOR measurements. ICC showed an excellent inter-rater reliability for US-FOR (ICC = 0.99, p < 0.001) and MR/CT-FOR (ICC = 0.99, p < 0.001) measurements. The mean (range) values based on US-FOR showed a slight overestimation in comparison with MR/CT-FOR (0.51 [0.36-0.68] vs. 0.46 [0.34-0.64]). The Pearson correlation coefficient showed high cross-modality agreement for the FOR index (r = 0.83, p < 0.001). The Bland-Altman plot showed excellent concordance between US-FOR and MR/CT-FOR with a bias of 0.05 (95% CI: -0.03 to 0.13). CONCLUSION: US-FOR in the coronal plane is a comparable tool for evaluating ventriculomegaly in Chiari II patients when compared with MR/CT-FOR, even in the context of colpocephaly.

Dynamic contrast-enhanced magnetic resonance lymphangiography.

Lymphatic flow disorders include a broad spectrum of abnormalities that can originate in the lymphatic or the venous system. The development of these disorders is multifactorial and is most commonly associated with congenital heart diseases and palliative surgeries that these patients undergo. Central lymphatic disorders might be secondary to traumatic leaks, lymphatic overproduction, conduction abnormalities or lymphedema, and they can progress to perfusion anomalies. Several imaging modalities have been used to visualize the lymphatic system. However, the imaging of central lymphatic flow has always been challenging. Dynamic contrast-enhanced magnetic resonance lymphangiography (DCMRL) allows for visualization of central lymphatic flow disorders and has been recently applied for the assessment of plastic bronchitis, protein-losing enteropathy, chylothorax and chylopericardium, among other lymphatic disorders. The hepatic and mesenteric accesses are innovative and promising techniques for better identification and understanding of these abnormalities. The main objectives of this review are to discuss the physiology and anatomy of the lymphatic system and review the current uses of DCMRL in the diagnosis and management of lymphatic flow disorders.

Ultrasound imaging of preterm brain injury: fundamentals and updates.

Neurosonography has become an essential tool for diagnosis and serial monitoring of preterm brain injury. Preterm infants are at significantly higher risk of hypoxic-ischemic injury, intraventricular hemorrhage, periventricular leukomalacia and post-hemorrhagic hydrocephalus. Neonatologists have become increasingly dependent on neurosonography to initiate medical and surgical interventions because it can be used at the bedside. While brain MRI is regarded as the gold standard for detecting preterm brain injury, neurosonography offers distinct advantages such as its cost-effectiveness, diagnostic utility and convenience. Neurosonographic signatures associated with poor long-term outcomes shape decisions regarding supportive care, medical or behavioral interventions, and family members' expectations. Within the last decade substantial progress has been made in neurosonography techniques, prompting an updated review of the topic. In addition to the up-to-date summary of neurosonography, this review discusses the potential roles of emerging neurosonography techniques that offer new functional insights into the brain, such as superb microvessel imaging, elastography, three-dimensional ventricular volume assessment, and contrast-enhanced US.

Congenital amusia and academic performance among Colombian university students.

BACKGROUND: Congenital amusia is a rare neurogenetic and neuropsychological condition which hinders the ability to recognize variations in all aspects of a musical piece. Although previous studies have determined the prevalence of congenital amusia in the general population, few have studied its presence among university students. Findings regarding the association between this condition and academic performance are equivocal, although evidence suggests that musical training improves scholastic achievement. METHODS: We conducted a cross-sectional study on a sample of 383 university students, all pursuing health-related degrees, comparing their class rank with their performance on the BRAMS Online Test for amusia. RESULTS: We found a prevalence of 0.52% for pitch-based amusia. When applying the Off-Scale test failure criterion for the definition of amusia in our sample, we found a prevalence of 4.4%. Logistic models showed an increase in risk of poor academic performance (lowest quartile) in subjects who failed the off-scale test (Odds Ratio: 7.14 95% CI 2.59-19.6) and who met any of the described definitions of amusia (Odds Ratio: 4.89 95% CI 2.24-10.7). CONCLUSIONS: Both musical training and self-report of musical ability significantly affected test results. Although musical education shows some effect over academic performance, further studies are required to determine if this is due to differential effects in subjects with and without amusia.