Imaging of Kidney Cysts and Cystic Kidney Diseases in Children: An International Working Group Consensus Statement.

Kidney cysts can manifest as focal disease (simple and complex kidney cysts), affect a whole kidney (eg, multicystic dysplastic kidney or cystic dysplasia), or manifest as bilateral cystic disease (eg, autosomal recessive polycystic kidney disease [ARPKD] or autosomal dominant polycystic kidney disease [ADPKD]). In children, as opposed to adults, a larger proportion of kidney cysts are due to genetic diseases (eg, HNF1B nephropathy, various ciliopathies, and tuberous sclerosis complex), and fewer patients have simple cysts or acquired cystic kidney disease. The purpose of this consensus statement is to provide clinical guidance on standardization of imaging tests to evaluate kidney cysts in children. A committee of international experts in pediatric nephrology, pediatric radiology, pediatric US, and adult nephrology prepared systematic literature reviews and formulated recommendations at a consensus meeting. The final statement was endorsed by the European Society of Pediatric Radiology, the European Federation of Societies for Ultrasound in Medicine and Biology, the European Society of Pediatric Nephrology, and reviewed by the European Reference Network for Rare Kidney Diseases. Main recommendations are as follows: US is the method of choice when assessing pediatric kidney cysts, with selected indications for MRI and contrast-enhanced US. CT should be avoided whenever possible because of ionizing radiation. Renal US yields essential diagnostic information in many cases. In patients with ARPKD or other ciliopathies, abdominal US is needed for diagnosis and screening of portal hypertension. US is usually sufficient for follow-up kidney imaging, but MRI can be valuable for clinical trials in patients with ADPKD or in older children with tuberous sclerosis complex to evaluate both kidney cysts and angiomyolipomas.

European Society of Paediatric Radiology abdominal imaging task force: statement on imaging in very early onset inflammatory bowel disease.

Very early onset inflammatory bowel disease (VEO-IBD) is defined as disease presenting before the age of 6. These children require a tailored imaging approach because conventional imaging studies can be difficult to perform at such a young age. Unlike inflammatory bowel disease in older children and adults, colonic disease predominates in VEO-IBD, and small-bowel disease is rare. Distinguishing Crohn disease from ulcerative colitis is challenging both clinically and on histology. Radiology offers the greatest utility for detecting small-bowel disease because it helps to distinguish the two main disease entities and guide clinical management. Small-bowel ultrasound is recommended as the first-line investigation because it requires relatively little preparation, is readily available and is generally well tolerated in young children. We present these recommendations, based on the current evidence for radiologic management in this group, and propose an imaging algorithm for investigating VEO-IBD.

Correction to: European Society of Paediatric Radiology abdominal imaging task force: statement on imaging in very early onset inflammatory bowel disease.

The above article was published online with incorrect author name. The right spelling should be Damjana Kljucevsek instead of Damjana Kjucevsek. The correct name is presented here.

Update on congenital nephrotic syndromes and the contribution of US.

The clinical classification of nephrotic syndrome (NS) is based on age at presentation. However, this classification is arbitrary because the majority of early onset NS has a genetic origin and has a widespread age of onset (from fetal life to several years). The aims of this review are to illustrate the knowledge accumulated on congenital nephrotic syndrome (CNS) in terms of genetics, classification, findings at histology and US-based on a review of the literature.

Postmortem fetal magnetic resonance imaging: where do we stand?

Postmortem fetal magnetic resonance imaging (PMFMRI) is increasingly used thanks to its good overall concordance with histology paralleling the rising incidence of parental refusal of autopsy. The technique could become a routine clinical examination but it needs to be standardized and conducted by trained radiologists. Such radiologists should be aware of not only the (congenital and acquired) anomalies that can involve the fetus, but also of the "physiological" postmortem changes. In this article, we intend to focus on the contribution of PMFMRI based on the existing literature and on our own experience, as we presently perform the technique routinely in our clinical practice. KEY POINTS: • Concordance rates between PMFMRI and autopsy are high for detecting fetal pathologies. • PMFMRI is more acceptable for parents than traditional autopsy. • PMFMRI is becoming widely used as a part of the postmortem investigations. • A dedicated radiologist needs to learn to interpret correctly a PMFMRI. • PMFMRI can be easily realized in daily clinical practice.

MR urography in children.

Thanks to the development of rapid sequences with better resolution, applications of uro MR have rapidly increased in children. Difficulties that remain are related to the variable ages of the patients. It is therefore mandatory to standardize as much as possible the techniques that are used in order to obtain reproducible results. In this review, the examination protocols will be explained. In a second part the current applications in children will be illustrated and discussed, especially in comparison with the other imaging techniques.

Type 1 primary hyperoxaluria in pediatric patients: renal sonographic patterns.

OBJECTIVE: Our aim was to review the sonographic features of type I primary hyperoxaluria in children and to correlate the sonographic patterns with the clinical development of end-stage renal disease (ESRD). MATERIALS AND METHODS: We performed a retrospective analysis of the clinical and imaging files of 13 patients with type I primary hyperoxaluria who were treated in one institution and of the sonographic patterns and the clinical follow-up reports. RESULTS: We encountered the following two sonographic patterns: medullary nephrocalcinosis in eight patients and cortical nephrocalcinosis in five patients. The sonographic appearance of cortical nephrocalcinosis is quite specific: a hyperechoic peripheral renal cortex with acoustic shadowing behind it. Medullary nephrocalcinosis is less specific because there are many other causes of hyperechoic pyramids. All patients with medullary nephrocalcinosis developed lithiasis during the course of the disease. All patients with cortical nephrocalcinosis but only two of eight with medullary nephrocalcinosis developed ESRD. CONCLUSION: Sonography can be used differentiate the two patterns of type 1 primary hyperoxaluria. The cortical nephrocalcinosis type carries a higher risk of developing ESRD.