Deboer Emily,  Deterding Robin,  Lynch David,  Humphries Stephen,  Jacob Joseph,  Devaraj Anand,  Ley-zaporozhan Julia,  Griese Matthias,  Schiwek Marilisa,  Stowasser Susanne,  Clerisme-beaty Emmanuelle

Final Pr. ID: Paper #: 034

Children’s interstitial lung diseases (chILD) comprise a large spectrum of rare diffuse lung disorders, with a subset of patients developing chronic fibrosing lung disease. While imaging criteria for lung fibrosis are established for adults and correlate with prognosis and outcome, there are limited data on imaging for diagnosis, prognosis, and outcomes in children with fibrotic lung disease. To fill this gap, a group of ILD specialists aimed to define the imaging features for diagnosis of lung fibrosis for a forthcoming randomized trial of nintedanib versus placebo in pediatric patients with fibrosing ILD (1199-0337; NCT04093024). Read More

Authors:  Deboer Emily , Deterding Robin , Lynch David , Humphries Stephen , Jacob Joseph , Devaraj Anand , Ley-zaporozhan Julia , Griese Matthias , Schiwek Marilisa , Stowasser Susanne , Clerisme-beaty Emmanuelle

Keywords:  childhood interstitial lung disease, computed tomography, fibrosis criteria

Lam Christopher,  Chavhan Govind

Final Pr. ID: Poster #: EDU-019

Gadolinium-based contrast agents (GBCAs) are widely used in clinical practice. However, there are growing concerns regarding the safety of GBCAs with recent findings of gadolinium deposition in patients with normal renal function. This educational exhibit reviews the use of gadolinium in children, including the safety profile, existing controversies, and potential solutions. Read More

Authors:  Lam Christopher , Chavhan Govind

Keywords:  nephrogenic systemic fibrosis, gadolinium deposition disease, gadolinium toxicity

Supakul Nucharin,  Wanner Matthew,  Marine Megan,  Karmazyn Boaz

Final Pr. ID: Poster #: EDU-032

To summarize our experience in diagnosis neonatal bowel obstruction in cystic fibrosis (CF) patient using contrast enema study. This pictorial review will illustrate and discuss several aspects of imaging findings in non-complicated and complicated meconium ileus as well as the mimicker.

Key imaging findings, pearls and pitfalls in diagnosis and guided treatment will be made, emphasizing what radiologists need to know. Correlation with intraoperative findings and follow-up images will also be provided. Read More

Authors:  Supakul Nucharin , Wanner Matthew , Marine Megan , Karmazyn Boaz

Keywords:  microcolon, cystic fibrosis, delayed pass meconium

Abu Ata Nadeen,  Dillman Jonathan,  Gandhi Deep,  Dudley Jonathan,  Trout Andrew,  Miethke Alexander

Final Pr. ID: Paper #: 095

Multiple noninvasive magnetic resonance imaging (MRI) methods have been described to detect liver fibrosis, including diffusion-weighted imaging (DWI). The purpose of our study was to evaluate predictors of liver MRI DWI apparent diffusion coefficient (ADC) measurements in pediatric patients with autoimmune liver disease, including the relationship between ADC measurements and liver stiffness.
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Authors:  Abu Ata Nadeen , Dillman Jonathan , Gandhi Deep , Dudley Jonathan , Trout Andrew , Miethke Alexander

Keywords:  Liver fibrosis, Liver stiffness

Priya Sarv,  Handa Atsuhiko,  Ferreira Da Silva Renato,  Lai Lillian,  Khanna Geetika,  Sato T Shawn

Final Pr. ID: Poster #: EDU-017

Immunoglobulin4 related disease (IgG4RD) is an inflammatory condition involving multiple regions of the body resulting in fibrosis which can lead to eventual organ failure. This entity was originally described with autoimmune pancreatitis. Recently many other previously described lesions have been brought under the umbrella of IgG4RD. These include a spectrum of conditions involving the head and neck region (orbits, salivary and lacrimal glands), thyroid gland (Riedel’s thyroiditis), vasculature (periaortitis), kidneys, lungs, retroperitoneum, mesentery, pituitary gland, biliary tract, pericardium, lymph nodes and pachymeninges.
Reports of IgG4RD are quite rare in the pediatric literature, however this may be due to potential unawareness about the condition as well as the variable presentations and non-specific imaging features of IgG4RD. The prevalence in pediatric population is poorly described.
The exact pathophysiology of IgG4RD is yet to be completely elucidated. The imaging manifestations are non-specific, and primarily consist of tumefactive enlargement of involved organs and homogenous contrast enhancement and associated lymphadenopathy. IgG4RD may manifest in single organ or may present as widespread disease involving multiple organs. These features overlap with other mass forming conditions like malignancy or lymphoma. However, the presence of multifocal disease with more than one organ involvement may point towards possible IgG4 related disease. Other than IgG4 related autoimmune pancreatitis, there is no consensus on diagnostic criteria based upon imaging. Definitive diagnosis of IgG4RD is made with biopsy and the histology characterized by infiltration of lymphocytes and IgG4 plasma cells with storiform fibrosis and obliterative phlebitis. According to Boston consensus, the ratio of IgG4 /IgG in tissue should be more than 0.4 with more than 10 IgG4+ cells per high power field. Serum IgG4 levels range from normal to elevated. Steroids are effective as first line treatment in majority of patients.
Our aim in this presentation is to familiarize radiologists with the spectrum of imaging features, and areas of involvement in IgG4 related disease using cases of IgG4RD collected at three different pediatric hospitals. It is important for pediatric radiologists to be familiar with this relatively newly described disease entity and be aware of the spectrum of manifestations of IgG4RD, ensuring prompt recognition and early treatment. Read More

Authors:  Priya Sarv , Handa Atsuhiko , Ferreira Da Silva Renato , Lai Lillian , Khanna Geetika , Sato T Shawn

Keywords:  IgG4, fibrosis

Alhashmi Ghufran,  Gupta Anita,  Trout Andrew,  Dillman Jonathan

Final Pr. ID: Alt #: 001

Ultrasound shear wave elastography (US SWE) can be used to noninvasively measure liver stiffness. US SWE is a potentially useful technique for diagnosing and monitoring liver fibrosis, obviating the need for liver biopsy in some patients. When compared to MR elastography (MRE), US SWE is lower cost, portable, and does not need sedation/anesthesia. US SWE measurements can vary by method (point vs. 2D) and vendor. Few studies have defined cut-off values for detecting fibrosis in pediatric patients using US 2D SWE. This study aims to evaluate the diagnostic performance and define cut-off values of US 2D SWE for the detection of liver fibrosis in pediatric patients, using Toshiba (Canon) Aplio 500 and Aplio i800 systems. Read More

Authors:  Alhashmi Ghufran , Gupta Anita , Trout Andrew , Dillman Jonathan

Keywords:  2D US shear wave elastography, Liver fibrosis, Pediatric