Appropriate imaging is imperative in evaluating children with a primary hepatic malignancy such as hepatoblastoma or hepatocellu lar carcinoma. For use in the adult patient population, the American College of Radiology created the Liver Imaging Reporting and Data System (LIRADS) to provide consistent terminology and to improve imaging interpretation. At present, no similar consensus exists to guide imaging and interpretation of pediatric patients at risk for developing a liver neoplasm or how best to evaluate a pe diatric patient with a known liver neoplasm. Therefore, a new Pediatric Working Group within American College of Radiology LI RADS was created to provide consensus for imaging recommendations and interpretation of pediatric liver neoplasms. The article was drafted based on the most uptodate existing information as interpreted by imaging experts comprising the Pediatric LIRADS Working Group. Guidance is provided regarding appropriate imaging modalities and protocols, as well as imaging interpretation and reporting, with the goals to improve imaging quality, to decrease image interpretation errors, to enhance communication with referrers, and to advance patient care. An expanded version of this document that includes broader background information on pe diatric hepatocellular carcinoma and rationale for recommendations can be found in Appendix E1 (online).

In glycogen storage disease type III (GSD III), liver aminotransferases tend to normalize with age giving an impression that hepatic manifestations improve with age. However, despite dietary treatment, long-term liver complications emerge. We present a GSD III liver natural history study in children to better understand changes in hepatic parameters with age. Methods: We reviewed clinical, biochemical, histological, and radiological data in pediatric patients with GSD III, and performed a literature review of GSD III hepatic findings. Results: Twenty-six patients (median age 12.5 years, range 2-22) with GSD IIIa (n = 23) and IIIb (n = 3) were enrolled in the study. Six of seven pediatric patients showed severe fibrosis on liver biopsy (median [range] age: 1.25 [0.75-7] years). Markers of liver injury (aminotransferases), dysfunction (cholesterol, triglycerides), and glycogen storage (glucose tetrasaccharide, Glc 4) were elevated at an early age, and decreased significantly thereafter (p < 0.001). Creatine phosphokinase was also elevated with no significant correlation with age (p = 0.4). Conclusion: Liver fibrosis can occur at an early age, and may explain the decrease in aminotransferases and Glc 4 with age. Our data outlines the need for systematic follow-up and specific biochemical and radiological tools to monitor the silent course of the liver disease process.

Radiology practitioners - represented by the Society for Pediatric Radiology pediatric radiologists - are using whole-body MRI in the imaging care of pediatric patients for a variety of indications. Survey results reveal some variability in exam utilization and technical performance practices among those pediatric radiologists who perform whole-body MRI.

There are many congenital, neoplastic, inflammatory, and infectious processes in the pediatric patient for which whole-body imaging may be of benefit diagnostically and prognostically. With recent improvements in magnetic resonance imaging (MRI) hardware and software and resultant dramatically reduced scan times, imaging of the whole body with MRI has become a much more practicable technique in children. Whole-body MRI can provide a high level of soft tissue and skeletal detail while avoiding the exposure to ionizing radiation inherent to computed tomography and nuclear medicine imaging techniques. This article reviews the more common current whole-body MRI techniques in children and the primary pathologies for which this imaging modality may be most useful to the radiologists and referring clinicians. J. MAGN. RESON. IMAGING 2016;44:783-793.

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Cystic biliary atresia (CBA) is a relatively uncommon but clinically significant variant of biliary atresia. The presence of a cyst in the hepatic hilum on imaging in an infant with cholestasis supports the diagnosis of CBA, but can also be seen in patients with a choledochal cyst—the main differential diagnosis in patients with CBA. The reported case outlines the clinical presentation and imaging findings in a patient with surgically confirmed and treated CBA and emphasizes the importance of distinguishing CBA from choledochal cyst at diagnostic imaging given the disparate timing and type of surgical treatment necessary for successful management of these distinct entities.