Moyamoya disease in a 3-year-old boy presenting with a focal motor seizure provoked by hyperventilation

A previously healthy, 3-year-old boy presented to the emergency department with an afebrile focal motor seizure. He was found crying and having a seizure 30 minutes earlier. During this seizure, he was jerking his head and right extremities. Subsequent magnetic resonance imaging showed acute infarction in the bilateral frontal lobes, chiefly in the left. After hospitalization, conventional angiography demonstrated bilateral stenosis of the distal internal carotid arteries with development of lenticulostriate collaterals, which confirmed the diagnosis of moyamoya disease. It is vital to recognize focal motor seizures and situations related to hyperventilation in children with a seizure, which imply a structural lesion and a provoked cerebral ischemia in preexisting moyamoya disease, respectively

Adrenal Cortical Neoplasm with Uncertain Malignant Potential Arising in the Heterotopic Adrenal Cortex in the Liver of a Patient with Beckwith-Wiedemann Syndrome

Patients with Beckwith-Wiedemann syndrome (BWS) are predisposed to developing embryonal tumors, with hepatoblastoma being the most common type. Our patient showed hemihypertrophy, macroglossia, and paternal uniparental disomy in chromosome 11 and was diagnosed with BWS. When the patient was 9 months old, a 2.5×1.5 cm oval hypoechoic exophytic mass was detected in the inferior tip of his right liver. Preoperative imaging identified it as hepatoblastoma; however, histologic, immunohistochemistry, and electron microscopic findings were compatible with adrenal cortical neoplasm with uncertain malignant potential. The origin of the adrenal tissue seemed to be heterotopic. Here, we describe for the first time an adrenal cortical neoplasm with uncertain malignant potential arising in the heterotopic adrenal cortex located in the liver of a patient with BWS

Prognostic Factors for Event-Free Survival in Pediatric Patients with Hepatoblastoma Based on the 2017 PRETEXT and CHIC-HS Systems

This study aimed to evaluate the prognostic value of variables used in the 2017 PRE-Treatment EXTent of tumor (PRETEXT) system and the Children’s Hepatic tumors International Collaboration-Hepatoblastoma Stratification (CHIC-HS) system in pediatric patients with hepatoblastoma. A retrospective analysis of data from the pediatric hepatoblastoma registry of a tertiary referral center was conducted to evaluate the clinical and imaging variables (annotation factors) of the PRETEXT staging system. The primary outcome was event-free survival (EFS). Data from 84 patients (mean age: 2.9 ± 3.5 years) identified between 1998 and 2017 were included. Univariable Cox proportional hazards analysis revealed that PRETEXT annotation factors P (portal vein involvement), F (multifocality of tumor), and M (distant metastasis) showed a significant negative association with EFS. Multivariable Cox proportional hazard analysis showed that factor F was the strongest predictor (HR (hazard ratio), 2.908; 95% CI (confidence interval), 1.061–7.972; p = 0.038), whereas factor M showed borderline significance (HR, 2.416; 95% CI, 0.918–6.354; p = 0.074). The prediction model based on F and M (F + M) showed good performance to predict EFS (C-statistic, 0.734; 95% CI, 0.612–0.854). In conclusion, the PRETEXT annotation factor F was the strongest predictor of EFS, and the F + M model showed good performance to predict EFS in pediatric patients with hepatoblastoma

Accuracy and precision of ultrasound shear wave elasticity measurements according to target elasticity and acquisition depth: A phantom study.

ObjectiveTo investigate the accuracy and precision of ultrasound shear wave elasticity measurements as a function of target elasticity and acquisition depth.Materials and methodsUsing five ultrasound systems (VTQ, VTIQ, EPIQ 5, Aixplorer, and Aplio 500), two operators independently measured shear wave elasticities in two phantoms containing five different target elasticities (8±3, 14±4, 25±6, 45±8, and 80±12 kPa) at depths of 15, 30, 35, and 60 mm. Accuracy was assessed by evaluating measurement errors and the proportions of outliers, while factors affecting accuracy were assessed using logistic regression analysis. Measurement errors were defined as differences between the measured values and 1) the margins of the target elasticity, and 2) the median values of the target elasticity. Outliers were defined as measured values outside the margins of the target elasticity. Precision was assessed by calculating the reproducibility of measurements using the within-subject coefficient of variation (wCV).ResultsMean measurement errors and the proportions of outliers were higher for high than for low target elasticities (p0.05) did not. The wCV for the 80±12 kPa target (31.33%) was significantly higher than that for lower elasticity targets (6.96-10.43 kPa; pConclusionsTargets with high elasticity showed lower accuracy and lower precision than targets with low elasticity, while acquisition depth did not show consistent patterns in either accuracy or precision

Factors Associated with Occurrence of Atelectasis during Sedation for Imaging in Pediatric Patients: A Retrospective Single Center Cohort Study

Sedation can induce atelectasis which may cause suboptimal image quality. This study aimed to identify factors associated with the occurrence of atelectasis during sedation for imaging in pediatric patients. Patients < 18 years who had undergone whole-body magnetic resonance imaging (MRI) under sedation with propofol or dexmedetomidine were included in this study. The development of atelectasis was visually and quantitatively assessed by coronal short tau inversion recovery images of the thoracic level. Multivariable logistic regression was performed to identify the independent factors associated with the development of atelectasis. Ninety-one patients were included in the analysis. In the multivariable analysis, administration of supplemental oxygen was the only factor significantly associated with the occurrence of atelectasis (adjusted odds ratio, 4.84; 95% confidence interval, 1.48–15.83; p = 0.009). Univariable analysis showed that the use of dexmedetomidine was associated with a lower incidence of atelectasis; however, this could not be verified in the multivariable analysis. Among the pediatric patients who had undergone imaging under sedation, additional oxygen supplementation was the only independent factor associated with atelectasis occurrence. A prospective clinical trial is required to identify the cause-effect relationship between oxygen administration and occurrence of atelectasis during sedation