Schoeneberg Laura,  Bornemeier Renee,  Reemtsen Brian,  Greiten Lawrence,  Greenberg S Bruce,  Lyons Karen,  Renno Markus

Final Pr. ID: Poster #: CR-001

Limited experience with 3D print technology has been reported for surgical planning in infants with congenital heart disease. We present 5 infants with complex intracardiac anatomy for whom 3D cardiac models from gated cardiac CT angiograms facilitated surgical planning.

Case 1: Term 2-day-old infant with d-transposition of the great arteries (TGA), large anterior malalignment ventricular septal defect (VSD), and severe aortic arch hypoplasia. The 3D cardiac model confirmed feasibility of biventricular repair using a Yasui-type operation, with Norwood arch reconstruction, Rastelli VSD closure, and right ventricle to pulmonary artery (RV-PA) conduit placement.

Case 2: 2-month old, ex-34-week premature infant with double-outlet right ventricle (DORV), side-by-side great arteries, large remote predominantly-subaortic VSD, and pulmonary stenosis (PS). The 3D cardiac model highlighted the remoteness of the VSD from the outflow tracts, prompting right ventricular outflow tract patch augmentation without VSD closure when the patient developed hypercyanotic spells.

Case 3: Term 7-day-old infant with Goldenhar syndrome, D-TGA, large posterior malalignment VSD with inlet extension, and PS. The first 3D cardiac model raised concern that VSD closure would be difficult as a newborn due to VSD size, prompting placement of a BT shunt and pulmonary artery band. The second 3D model at 11 months of age demonstrated feasibility of biventricular repair with Rastelli VSD closure and RV-PA conduit placement.

Case 4: Term 2-day-old infant with 22q11.2 deletion syndrome, type B interruption of a right aortic arch, aberrant left subclavian artery, posterior malalignment VSD, small bicuspid aortic valve, and superior-inferior branch pulmonary artery relationships. The 3D cardiac model demonstrated need for VSD enlargement to enable complete repair with Yasui operation with LeCompte maneuver.

Case 5: Term 3-week-old infant with mesocardia, ventricular inversion, DORV, L-malposed great arteries, subpulmonic VSD, multilevel PS, and mitral chordal attachments to the crest of the ventricular septum. The 3D cardiac model suggested that VSD enlargement might enable a double switch operation with Rastelli VSD baffle. However, during the operation at 20 months old, mitral chordal apparatus prevented VSD enlargement, necessitating bidirectional Glenn instead. Read More

Authors:  Schoeneberg Laura , Bornemeier Renee , Reemtsen Brian , Greiten Lawrence , Greenberg S Bruce , Lyons Karen , Renno Markus

Keywords:  3D model, Congenital heart disease, Surgical Planning

Fujiwara Takashi,  Browne Lorna,  Malone Ladonna,  Lu Quin,  Fonseca Brian,  Dimaria Michael,  Barker Alex

Final Pr. ID: Paper #: 040

4D flow MRI with full coverage of the heart and proximal vessels is a powerful tool to understand hemodynamic pathology arising from congenital heart disease (CHD) and subsequent surgery; however, scan times often exceed 10 minutes thereby limiting widespread adoption in pediatrics. Decreasing scan time using compressed-SENSE (CS), a combination of conventional parallel imaging (SENSE) and randomized spatial undersampling, shows promise over SENSE alone. However, both the feasibility and its effect on accurate flow quantification in pediatrics has not been evaluated. This study investigated the feasibility of CS for pediatric 4D flow MRI to quantify flow indices used in risk stratification of CHD such as coarctation or pulmonary regurgitation, in comparison with SENSE. Read More

Authors:  Fujiwara Takashi , Browne Lorna , Malone Ladonna , Lu Quin , Fonseca Brian , Dimaria Michael , Barker Alex

Keywords:  4D flow, Compressed SENSE, Congenital heart disease

Southard Richard,  Ellsworth Erik,  Moe Tabitha,  Augustyn Robyn,  Thorkelson Marrit,  Bardo Dianna

Final Pr. ID: Poster #: EDU-090

The structure of the heart is more complex than 4 major chambers, 4 major valves, venous inflow and arterial outflow.

Muscular and soft tissue ridges, perforated membranes, tissue flaps, electrical pathways, external grooves, endocardial surface characteristics, and a core architecture structured of fibrous tissue and embryonic tissue remnants form distinguishing anatomic landmarks. These structures provide insight into cardiac development, serve physiologic purpose, define structural and functional elements, as well as offer prognostic information.

Specific details of cardiac anatomy may not be recognized on cardiac MR and CT examinations. Structural anomalies that affect cardiac function and anatomic or physiologic relationships may be abnormal in hearts with congenital malformations. The fine structural details of the heart are typically not part of an imagers search pattern and are therefore overlooked or underreported. Each minute structure and anatomic characteristic provides clues to errors in cardiac formation and may provide clinically relevant diagnostic information. After palliation of CHD defining anatomic structure may be lost, displaced, interrupted or even restored.

Important processes, corresponding anatomic landmarks, and effects of malformation in defining congenital heart disease anatomy, physiology and functional variation from normal include basal structures such as the central fibrous body of the heart, atrial structures including the crista terminalis, the sinus venarum, and eustacian valve, and ventricular structures such as the crista supraventricularis.

Each structure is described along with its normal location, anatomy functional and physiologic importance, the effect of congenital malformation, and imaging findings as in the example of the central fibrous body (table 1).

Minute cardiac anatomy will be shown in illustration as well as in CT and MR images (figure 1). Read More

Authors:  Southard Richard , Ellsworth Erik , Moe Tabitha , Augustyn Robyn , Thorkelson Marrit , Bardo Dianna

Keywords:  Cardiac anatomy, Cardiac MR, Cardiac CT, Congenital heart disease, Embryology

Cielma Tara,  Durfee Teela,  Bulas Dorothy,  Loomis Judyta,  Adeyiga Adebunmi,  Bandarkar Anjum

Final Pr. ID: Poster #: EDU-003 (T)

Bowel ultrasound is a critical component of gastrointestinal evaluation. Serial examination allows real-time assessment of disease progression or improvement, and assists the clinician in therapeutic decision making and clinical management.

The goals of this exhibit are:
1. Describe the technical approach of performing bowel ultrasound.
2. Review tips, and up to date technology that assist in optimizing studies.
3. Discuss sonographic appearance of various pathologies.
4. Review future potential techniques and applications including utility of Doppler flow and contrast enhanced ultrasound. Read More

Authors:  Cielma Tara , Durfee Teela , Bulas Dorothy , Loomis Judyta , Adeyiga Adebunmi , Bandarkar Anjum

Keywords:  gastrointestinal disorder, inflammatory bowel disease, bowel

Golden Eleza,  Simoneaux Stephen,  Dickson Paula

Final Pr. ID: Poster #: CR-049

To describe the radiographic appearance of subclinical calcified brown fat necrosis and to delineate the associated clinical and laboratory findings. While brown fat necrosis has been described in infants with underlying cardiac disease treated with prostaglandins, we emphasize hypotension from cardiac or respiratory arrest as a primary risk factor. Read More

Authors:  Golden Eleza , Simoneaux Stephen , Dickson Paula

Keywords:  fat necrosis, soft tissue calcification, congenital heart disease

Kaloianova Maria,  Naidoo Jaishree,  Thomson Heather,  Bhengu Louisa

Final Pr. ID: Poster #: CR-002

Krabbe disease is an autosomal recessive leukodystrophy that presents clinically with regression of milestones, excessive irritability and inconsolable crying. The pathologic basis of the disease is due to abnormal myelin metabolism resulting from a deficiency in the galactocerebrosidase enzyme with subsequent white matter destruction.
Although optic atrophy is a classic presentation of Krabbe disease, we report two patients who are biological brothers presenting with optic nerve enlargement in addition to other typical MR imaging features of Krabbe disease, thereby confounding the initial diagnosis.

Krabbe disease, also known as globoid cell leukodystrophy, is a lysosomal function disorder which ultimately results in demyelination and dysmyelination of white matter.
Worldwide incidence of Krabbe disease has been shown to be 1 in 100 000-200 000, with 1 in 150 000 live births reported in Europe (1, 2). The incidence in the South African population has not been well established.

Case Report 1:
Patient 1 presented in February 2012 to the paediatric neurodevelopmental clinic at 7 months of age with excessive inconsolable crying, regression of milestones, numerous café au lait macules and large eyes. Birth history was non-contributory.
MRI of the brain was done, demonstrating hypertrophy of the optic nerves and multiple other cranial nerves bilaterally (see figure 1).
High signal intensities were noted within the white matter of the brachium pontis, cerebellar dentate nuclei and corona radiata with a significant background of cortical and white matter atrophy (see figure 2).

Patient 1 died at one year of age with a provisional diagnosis of Neurofibromatosis I (NFI). No specific tests for Krabbe disease were performed prior to his death.

Case Report 2:
Patient 2 presented in November 2014 at 5 months of age with regression of milestones, excessive crying which was difficult to soothe, numerous café au lait macules, large eyes and a relative macrocephaly. Birth history was non-contributory and the child developed normally up until 3 months of age.
Blood tests for amino acids and organic acids, as well as liver and renal functions were normal. CSF lactate and glycine levels were also normal.

Specific enzyme testing for Krabbe disease was performed for patient 2 in Philadelphia, United States. Results revealed the galactocerebrosidase enzyme value to be very low (0.07). This is in the range of patients affected with Krabbe disease.
Patient 2 died shortly after the diagnosis of Krabbe disease was confirmed.

MRI brain scan of patient 2 demonstrated markedly hypertrophied optic nerves (see figures 3 and 4). Increased T2 and T2 Flair signal intensities of the white matter of the brachium pontis and the cerebellar dentate nuclei (see figure 5). The midbrain, pons and cerebellum also demonstrated increased T2 and T2 Flair signal intensities with a significant background of cortical and white matter atrophy (see figure 6).

PLEASE NOTE: the discussion related to Krabbe disease in this case review has not been included here due to charachter limitations. In addition, 6 images in total will be included should this case report be accepted for presentaton. Read More

Authors:  Kaloianova Maria , Naidoo Jaishree , Thomson Heather , Bhengu Louisa

Keywords:  Krabbe disease, leukodystrophy, globoid cell leukodystrophy, optic nerve enlargement

Nakagawa Motoo,  Ozawa Yoshiyuki,  Tanaka Yoshihiro,  Shibamoto Yuta

Final Pr. ID: Poster #: EDU-002

Characteristic chest radiographic findings of patients with congenital heart disease have been reported for some decades ago. For beginner, it may be hard to detect these findings and to understand reasons why these findings depict. Recently, radiologists can interpret specific findings of congenital heart disease because technique of CT have been developed. Read More

Authors:  Nakagawa Motoo , Ozawa Yoshiyuki , Tanaka Yoshihiro , Shibamoto Yuta

Keywords:  congenital heart disease, chest radiograph, dual source CT

Liang Teresa,  Lee Edward

Final Pr. ID: Poster #: EDU-090

Childhood interstitial (diffuse) lung disease (chILD) in infants consists of a rare and heterogeneous group of disorders previously classified with clinical, radiologic, and pathologic features. The purpose of this article is to discuss imaging techniques and provide a pattern-based approach for chILD in infants. Read More

Authors:  Liang Teresa , Lee Edward

Keywords:  childhood interstitial lung disease, Lung, Infants

Yadav Vikas,  Sudhakar Sniya,  Thomas Maya,  Arunachal Gautham

Final Pr. ID: Poster #: CR-072

A 1-year-old boy with uneventful perinatal history, non-consanguineous parentage presented with global developmental delay. Family history was not contributory. There was no history suggestive of visual or hearing loss, seizures, bladder and bowel involvement or extrapyramidal symptoms. On examination, Head size was large for age with hyper pigmented nevi over right arm. There was delay involving language, motor and cognition. CNS examination revealed stance ataxia with generalized hypotonia. No obvious organomegaly was noted on abdominal examination.
MRI done in August 2014 showed signal abnormality in dorsal brainstem, dentate nuclei, bilateral thalami and basal ganglia. Gyri appeared swollen with subcortical U fiber involvement. Central white matter was spared. MRS revealed elevated borderline elevation of NAA and lactate. CT did not reveal any obvious calcification in these areas.
Based on these findings differential diagnosis of mitochondrial etiology and L2 hydroxyglutaric aciduria were considered. However, urine tests for routine organic acids were negative.
Literature search revealed findings fitting into Canavan variant disease. Consequently, urine examination showed elevated N acetyl aspartate. The ASPA gene mutation was confirmed on genetic testing.
Another case showed similar clinical presentation. However head size was normal in second case. Imaging findings were similar in nature. Genetic mutation confirmed the diagnosis.
Recognition of the Canavan Variant is important, as most of the cases would be diagnosed as mitochondrial (Leigh’s/ LBSL) or L2 hydroxyglutaric aciduria initially. This pattern recognition lead to correct genetic testing and diagnosis. Read More

Authors:  Yadav Vikas , Sudhakar Sniya , Thomas Maya , Arunachal Gautham

Keywords:  Canavan disease, Variant, N acetyl aspartate, ASPA gene

O'donovan Julie,  Murray Becky,  Hu Houchun,  Wallihan Rebecca,  Mejias Asuncion,  Ramilo Octavio,  Krishnamurthy Rajesh

Final Pr. ID: Poster #: SCI-069

CAP is a worldwide contributor to morbidity and mortality in children. Radiogenomics is an emerging specialty which correlates imaging features to gene expression to predict disease severity, therapeutic response and clinical outcomes. A genomic score termed Molecular Distance to Health (MDTH) is a biomarker that measures the global transcriptional perturbation in blood and has been shown to correlate with disease severity in pediatric CAP (Wallihan R, Front. Cell. Infect. Microbiol. 2018). This study was undertaken to determine whether abnormalities on chest radiographs correlate with genomic and clinical markers of disease severity. Read More

Authors:  O'donovan Julie , Murray Becky , Hu Houchun , Wallihan Rebecca , Mejias Asuncion , Ramilo Octavio , Krishnamurthy Rajesh

Keywords:  radiogenomics, disease severity, MDTH

Sharma Priya,  Rajderkar Dhanashree

Final Pr. ID: Poster #: EDU-114

Learning Objectives: This educational exhibit will review glycogen storage diseases and the imaging findings of these disease processes using a multimodality approach. We will also review complications and discuss the treatment and role of imaging as a measure of treatment response. Read More

Authors:  Sharma Priya , Rajderkar Dhanashree

Keywords:  Glycogen Storage Diseases, Hepatic Adenoma, Hepatocellular Carcinoma, Cardiomyopathy, GSD

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

Dong Su-zhen

Final Pr. ID: Poster #: EDU-026

The cardiac system is the part of the fetal anatomy that most frequently suffers from congenital pathology . The fetal heart is routinely studied using first level obstetric ultrasonography. If a fetus is considered to have a cardiac defect, a detailed echocardiographic examination is required. The role of fetal magnetic resonance imaging (MRI) as a tool additional to ultrasound has grown exponentially. Unlike ultrasound imaging, however, MRI is unaffected by maternal and fetal conditions such as obesity and oligohydramnios, which particularly impair sonographic visualization of the fetal heart. In this educational poster, we will provide an overview of fetal cardiac MR imaging methods and their applications in congenital heart disease. This presentation will cover recent technical advances in fetal CMR, the modified anatomic segmental approach to image fetal congenital heart disease, imaging appearances of fetal normal cardiovascular structure and appearances of various structural congenital heart disease on fetal CMR. Multiple imaging examples of fetal congenital heart disease diagnosed by fetal cardiac MR will be presented. Read More

Authors:  Dong Su-zhen

Keywords:  Fetus, Magnetic resonance imaging, Congenital heart disease

Davila Jorge,  Highmore Kerri,  Miller Elka

Final Pr. ID: Poster #: SCI-064

The objective of this prospective study was to determine the accuracy of MR Enterography (MRE) in the assessment of mucosal healing in patients with Pediatric Crohn’s Disease (PCD) after starting treatment. MARIA and Clermont scores are MRE scores that were originally developed in adult population for the assessment of severity of Crohn's Disease (CD) in correlation to Crohn's Disease Endoscopic Index of Severity (CDEIS). MARIA score has shown close correlation to disease severity of colonic CD and Clermont score has shown close correlation to terminal ileum disease. A few reports have shown correlation of mucosal heling when assess by MRE scores and CDEIS in Adult population, but not work is available in pediatrics. Read More

Authors:  Davila Jorge , Highmore Kerri , Miller Elka

Keywords:  MR Enterography, pediatric crohn's disease

Fetzer Daniel,  Aaen Gregory,  Udochukwu Oyoyo,  Achiriloaie Adina

Final Pr. ID: Paper #: 164

Pediatric multiple sclerosis (MS) is difficult to diagnose at presentation, and the clinical features often overlap those of acute disseminated encephalomyelitis (ADEM), neuromyelitis optica or clinically isolated syndrome. Established criteria for diagnosing MS are less reliable in pre-pubescent children. We seek to qualitatively and quantitatively define the MRI features of pediatric MS compared to ADEM at initial presentation. Read More

Authors:  Fetzer Daniel , Aaen Gregory , Udochukwu Oyoyo , Achiriloaie Adina

Keywords:  Multiple sclerosis, ADEM, white matter disease

Adu John,  Watson Tom

Final Pr. ID: Poster #: EDU-024

The inflammatory bowel diseases (IBD), Crohn’s disease (CD) and ulcerative colitis (UC) are multifaceted disorders as a consequence of complex interplay between genetic, environmental and immunological factors, leading to a dysregulated immune response of the host intestinal bacteria. In children both the mucosal immune system and the intestinal microflora are still developing. Taken together, it seems that patients with early onset IBD (EO-IBD) are a unique subset within IBD with particular gene defects, phenotypic appearance, drug responsiveness and immune pathology.
Imaging plays a key role in the diagnosis and follow-up of EO-IBD, with MRI enterography being the gold standard in modern IBD practice, largely because of its ability to provide excellent spatial resolution without ionising radiation. EO-IBD can also be elegantly demonstrated on ultrasound and can be used a complimentary imaging tool in both the diagnostic workup and follow up imaging.
The aims of this educational exhibit are to:
(i) Provide a pictorial review of the key imaging finding of EO-IBD as demonstrated on MRI and ultrasound.
(ii) Review the imaging techniques and protocols for MR enterography as applied to EO-IBD.
(iii) Discuss the role of genetic mutations and innate immune defects in the pathophysiology of EO-IBD. Read More

Authors:  Adu John , Watson Tom

Keywords:  Inflammatory bowel disease, diarrhoea, interleukin-10, crohns disease, ulcerative colitis

Lanier Michael,  Shetty Anup,  Salter Amber,  Khanna Geetika

Final Pr. ID: Poster #: SCI-011

Gadolinium deposition in normal tissues is an increasingly recognized consequence of intravenous gadolinium contrast agents. Children with inflammatory bowel disease (IBD) undergo frequent surveillance imaging with contrast enhanced MR enterography (MRE).
Purpose: To determine the benefit (if any) of IV contrast in evaluation of IBD by MRE. Read More

Authors:  Lanier Michael , Shetty Anup , Salter Amber , Khanna Geetika

Keywords:  Enterography, Crohn disease, inflammation

Zhong Yumin,  Sun Aimin,  Gao Wei

Final Pr. ID: Poster #: SCI-086

Tracheobronchial anomalies including tracheobronchial stenosis, tracheal bronchus, cardiac bronchus and bronchial isomerism are common in congenital heart disease. Cardiovascular anomaly is the principal extrinsic lesion causing tracheobronchial stenosis. MRI has the advantage of being non-ionizing and providing excellent soft tissue contrast for the diagnosis of congenital heart disease and tracheobronchial anomalies
Purpose: To define diagnostic accuracy of three-dimensional turbo field echo (3D-TFE) to detect tracheobronchial anomalies in patients with congenital heart disease Read More

Authors:  Zhong Yumin , Sun Aimin , Gao Wei

Keywords:  Tracheobronchial Anomalies, congenital heart disease, Magnetic Resonance Imaging Sequence

Joshi Hena,  Alazraki Adina,  Rostad Bradley

Final Pr. ID: Poster #: EDU-024

Ulcerative colitis (UC) and Crohn disease are chronic, immune-mediated, inflammatory disorders of the gastrointestinal tract collectively referred to as inflammatory bowel disease (IBD). As many of 20-25% of patients with IBD initially present in childhood or adolescence, and the incidence of pediatric IBD is increasing. IBD primarily affects the bowel, but other organs can be involved. Nearly one-third of patients will have at least one extra-intestinal manifestation. Some extra-intestinal manifestations, such as that between UC and primary sclerosing cholangitis (PSC), are well-established. Others are less understood and may mimic more common pathology, particularly infection. Therefore, pediatric radiologists must become familiar with these extra-intestinal manifestations and consider the diagnosis of IBD as the etiology for their pathology. Read More

Authors:  Joshi Hena , Alazraki Adina , Rostad Bradley

Keywords:  inflammatory bowel disease, ulcerative colitis, Crohn disease

Griffin Lindsay,  Gupta Aditi,  Berhane Haben,  Husain Nazia,  Rigsby Cynthia

Final Pr. ID: Paper #: 011

Three dimensional magnetic resonance angiography (3DMRA) and two-dimensional phase contrast are commonly used for assessment of anatomy and flow in congenital heart disease. Time-resolved, three-dimensional phase contrast MRI (4D flow) provides complete volumetric coverage of the chest with flow assessment in any plane. How the addition of ferumoxytol (Fe), a blood pool contrast, changes 4D flow magnitude image quality relative to extracellular gadolinium-based contrast agents (GB) and non-contrast (NC) exams is unknown. We compare image quality measurements in Fe, GB and NC 4D flow with ECG-gated, respiratory-navigated 3DMRA. Read More

Authors:  Griffin Lindsay , Gupta Aditi , Berhane Haben , Husain Nazia , Rigsby Cynthia

Keywords:  4D flow, Ferumoxytol, Congenital Heart Disease

Bardo Dianna,  Lindblade Christopher,  Wishah Fidaa,  Patel Mittun,  Cornejo Patricia,  Ellsworth Erik,  Awerbach Jordan,  Goncalves Luis

Final Pr. ID: Paper #: 100

Purpose is to describe how FCMR is instrumental in diagnosis of congenital heart disease (CHD) and to present imaging findings and correlation in 50 fetuses with CHD. Read More

Authors:  Bardo Dianna , Lindblade Christopher , Wishah Fidaa , Patel Mittun , Cornejo Patricia , Ellsworth Erik , Awerbach Jordan , Goncalves Luis

Keywords:  fetal cardiac MRI, congenital heart disease

Bardo Dianna,  Lindblade Christopher,  Wishah Fidaa,  Cornejo Patricia,  Patel Mittun,  Ellsworth Erik,  Awerbach Jordan,  Goncalves Luis

Final Pr. ID: Poster #: EDU-006

The purpose of this educational exhibit is to show FCMR images of congenital heart disease (CHD) and anatomical findings associated with CHD. We will describe currently available FCMR image acquisition techniques and use a case-based approach to show a variety of CHD diagnosis with teaching points to highlight the added value of FCMR in diagnosis and prognosis. Read More

Authors:  Bardo Dianna , Lindblade Christopher , Wishah Fidaa , Cornejo Patricia , Patel Mittun , Ellsworth Erik , Awerbach Jordan , Goncalves Luis

Keywords:  Fetal cardiac MRI, congenital heart disease

Dong Su-zhen

Final Pr. ID: Poster #: EDU-010

1. Review the safety, limitations and advantages of fetal cardiac MR (CMR)
2. Discuss potentialindications of fetal CMR
3. Describe the technique of fetal CMR, including imaging sequences, imaging planes, the method
of overcoming motion artifact
4. Illustrate the appearance of normal fetal cardiac structures and present a modified anatomic
segmental approach of congenital heart disease (CHD) at prenatal CMR
5. Demonstrate examples of fetal CMR in the evaluation of various cardiovascular anomalies Read More

Authors:  Dong Su-zhen

Keywords:  Fetus, cardiac magnetic resonance, congenital heart disease

Shelmerdine Susan,  Semple Thomas,  Spencer Helen,  Pillai Anjay,  Owens Catherine

Final Pr. ID: Poster #: CR-004

To evaluate the radiographic and CT appearances of filamin A (FLNA) mutation related lung disease and review the available medical literature regarding this new entity. Read More

Authors:  Shelmerdine Susan , Semple Thomas , Spencer Helen , Pillai Anjay , Owens Catherine

Keywords:  Lung, CT, Interstitial Lund Disease, ChILD classification, Emphysema

Rees Mitchell,  Suman Garima,  Krishnamurthy Ramkumar,  Krishnamurthy Rajesh

Final Pr. ID: Paper #: 010

This study compares the performance of a single free-breathing GRASP sequence to a full C-MRE protocol in pediatric small bowel CD. The high k-trans of affected bowel in CD may allow high spatiotemporal GRASP dynamic contrast-enhanced technique to provide comprehensive diagnostic value in CD, especially in patients with limited breath-hold capacity. Read More

Authors:  Rees Mitchell , Suman Garima , Krishnamurthy Ramkumar , Krishnamurthy Rajesh

Keywords:  Crohn disease, enterography, GRASP

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

Alis Jonathan,  Levin Terry,  Kurian Jessica,  Mahadeo Kris,  Taragin Benjamin

Final Pr. ID: Poster #: EDU-092

Sickle Cell Disease (SCD) affects approximately 100,000 individuals in the US. Despite medical advances, morbidity is high and their lifespan remains shortened. Hematopoietic stem cell transplantation (HSCT) is the only curative treatment to date and as outcomes improve its use has increased. Complications of HSCT are frequent and some literature suggests there is a higher incidence in the SCD population. In addition, preexisting vasculopathy in this population may contribute to an increased incidence of post-transplant CNS toxicity. We present the imaging findings of HCST complications in children with SCD. Read More

Authors:  Alis Jonathan , Levin Terry , Kurian Jessica , Mahadeo Kris , Taragin Benjamin

Keywords:  Graft versus host disease, bone marrow transplant, HSCT

Aslan Mine,  Kalyoncu Ucar Ayse,  Arioz Habibi Hatice,  Namdar Yesim,  Kurugoglu Sebuh,  Adaletli Ibrahim

Final Pr. ID: Poster #: CR-027

Wolman disease is a rare autosomal recessive inherited disease characterized by storage of cholesterol esters and triglycerides in lysosomes due to a deficiency of lysosomal acid lipase. Clinical signs such as persistent vomiting, diarrhea, hepatosplenomegaly, growth retardation and liver dysfunction occur in the first weeks of life. Most cases die in the first year of life. Here, we aim to present radiological findings of Wolman disease.
An asymptomatic girl two months of age was admitted to the pediatric genetic disease service with the history of Wolman disease in two brothers who had died in the neonatal period. Abdominal X-ray imaging displayed calcification of the bilateral adrenal gland regions. Hepatosplenomegaly and bilateral adrenal gland posterior acoustic shadowing due to calcifications were revealed by abdominal sonography. Magnetic resonance imaging showed enlarged hypointense adrenal glands in all sequences and hepatosplenomegaly. The lysosomal acid lipase levels were low compatible with Wolman disease.
Multimodality radiologic imaging methods should be performed to display hepatosplenomegaly, hepatosteatosis, bilateral adrenal gland enlargement and calcification in Wolman disease. Read More

Authors:  Aslan Mine , Kalyoncu Ucar Ayse , Arioz Habibi Hatice , Namdar Yesim , Kurugoglu Sebuh , Adaletli Ibrahim

Keywords:  Wolman Disease, Sonography, Adrenal gland, calcification, magnetic resonance imaging

Singh Jaspreet,  Tuburan Smyrna

Final Pr. ID: Poster #: CR-077

We report the case of a neonatal male with alveolar capillary dysplasia with misalignment of the pulmonary veins (ACD/MPV). ACD/MPV is a rare, fatal disorder of the development of the alveolar vasculature. In this disorder, alveolar capillaries are reduced in number and are improperly located within the walls of the alveoli impeding oxygen exchange. In addition to alveolar capillary abnormalities, there are other associated abnormalities with the cardiovascular, gastrointestinal tract, and genitourinary systems. In this report, we describe the case of a term male neonate with a prenatal diagnosis of hypoplastic left heart who developed respiratory distress shortly after birth. The patient required prolonged ECMO and underwent evaluation with CT angiography of the chest. CT findings included diffuse bilateral interstitial thickening with enhancing airspace disease scattered in the left lung. The respiratory distress continued to be unresponsive to treatment, with difficulty weaning ECMO and respiratory support. Life support was withdrawn on day 30. Pathologic examination at autopsy demonstrated ACD/MPV. Our case adds to the limited literature available in describing imaging findings of ACD/MPV and support for early diagnosis with lung biopsy as it can prevent aggressive futile interventions. Read More

Authors:  Singh Jaspreet , Tuburan Smyrna

Keywords:  Interstitial Lung Disease, diffuse lung developmental disease

Degnan Andrew,  Serai Suraj,  Ho-fung Victor,  Barrera Christian,  Wang Dah-jyuu,  Ahrens-nicklas Rebecca,  Ficicioglu Can

Final Pr. ID: Poster #: EDU-057

Gaucher disease is an inherited metabolic disorder due deficiency of the lysosomal enzyme β-glucocerebrosidase that results in the accumulation of abnormal macrophages (“Gaucher cells”) within multiple organs, most conspicuously affecting the liver, spleen and bone marrow. As the diagnosis is increasingly made during childhood and young adulthood, pediatric radiologists should be familiar with imaging features of Gaucher disease and its complications.

Visceromegaly consisting of hepatosplenomegaly is a hallmark of Gaucher disease and uniformly present in cohorts of pediatric patients. In addition, bone marrow involvement with ‘Erlenmeyer flasks’ have been long recognized as part of this disease, although the classic radiographic finding is not present until adulthood. Marrow involvement confers significant morbidity for these patients with pain, bone infarcts and pathologic fracture.

Traditionally, imaging of disease severity has been based on hepatic and splenic visceral organ enlargement and/or qualitative assessment of bone marrow involvement. However, advances in the understanding of Gaucher disease and observations of elevated ferritin levels and increased risk of hepatic fibrosis emphasize the importance of more comprehensive assessment of liver involvement beyond simple enlargement. Moreover, quantitative MRI assessment of bone fat-fractions also may have a role in assessing marrow involvement. These methods of disease assessment are important in addressing management decisions regarding enzyme replacement and substrate reduction therapy. Read More

Authors:  Degnan Andrew , Serai Suraj , Ho-fung Victor , Barrera Christian , Wang Dah-jyuu , Ahrens-nicklas Rebecca , Ficicioglu Can

Keywords:  Gaucher disease, bone marrow, hepatic involvement