Jin Annie,  Bao Shashan

Final Pr. ID: Poster #: CR-015

Rhabdomyosarcoma is a common pediatric soft tissue cancer with about 31% of cases in the urinary tract and 25% of cases in the head and neck. Although rhabdomyosarcomas are common, primary intrahepatic rhabdomyosarcomas are very rare and have a poor prognosis. In fact, there have only been 20 reported cases from 1956 through 2017, and the mean survival time of 9.25 months.

A 3-year-old male with a history of constipation presented with a 3-days of low-grade fevers of 99-100 F, periumbilical pain, and anorexia. On physical exam, there was a palpable, tender RUQ mass. Labs showed an elevated ALT of 159, AST of 179, and alkaline phosphatase of 748. A radiograph of the abdomen showed a soft tissue opacity in the right upper quadrant which displaced the large and small bowel loops. Further imaging included an ultrasound, CT, and MRI. The ultrasound confirmed a large mostly solid mass in the right hepatic lobe. CT imaging showed a large heterogeneous liver mass measuring 10.5 x 10.0 x 14.0 cm. The mass extended superiorly to the dome of the liver, involved the caudate lobe, and compressed the IVC. An indeterminate lucency of the right femoral neck was also noted. A follow up MRI showed that the liver mass displaced the hepatic veins, main portal vein and right portal vein. Additionally, innumerable lesions were found on the axial skeleton and periportal lymphadenopathy was seen suggesting metastasis. PET scan further confirmed bony metastasis of the right femur. Initially, hepatoblastoma was highest on the differential. However, liver wedge biopsy showed myxoid spindle cell areas and small round blue cell areas. These findings favored the diagnosis of rhabdomyosarcoma as opposed to hepatoblastoma which would have had an epithelial component. Bone marrow biopsy further confirmed metastasis. As a result of this diagnosis, the patient was started on vincristine, dactinomycin, cyclophosphamide. Pain was controlled with morphine and acetaminophen. Nausea was controlled with Ativan, Zofran, Reglan, and Benadryl. The patient’s abdominal distension and constipation responded well to a stool regimen and multiple enemas. The patient was discharged in stable condition with oncology follow up.

Hepatic rhabdomyosarcoma is a rare malignancy, and early diagnosis and treatment has been beneficial in prolongation of mean survival time. Diagnostic imaging modalities and pathology findings are an essential part of the work up and can be used to guide the treatment and management. Read More

Authors:  Jin Annie , Bao Shashan

Keywords:  Hepatic Rhabdomyosarcoma, Spindle Cell, Diagnostic Imaging

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

Schaefer Carrie,  Aria David,  Beydoun Tammam,  Kaye Robin,  Towbin Richard

Final Pr. ID: Poster #: CR-071

In 2014, 3 patients including a 28-week preemie, 18 day-old neonate, and 11 month-old infant, were treated for large hepatic AVMs. Symptoms included CHF in both the 890-gram preemie and neonate and no symptoms in the infant with imaging revealing extensive shunting. Embolization of the AVMs was performed using various agents, entry sites, and arterial and venous techniques. Agents utilized included detachable and non-detachable coils and vascular plugs. Both direct puncture and intra-vascular techniques, including the use of the umbilical artery, were utilized. Immediate hospital course and short-term clinical and radiographic follow-up was conducted.

Technical success was achieved in all 3 patients without immediate complications. The two symptomatic patients improved clinically on short-term follow up with resolution of the preemie’s and neonate’s CHF, confirmed on post-embolization imaging. Conventional angiography demonstrated complete resolution of the AVM in the infant. Read More

Authors:  Schaefer Carrie , Aria David , Beydoun Tammam , Kaye Robin , Towbin Richard

Keywords:  Hepatic AVM, Venous Embolization

Shore Matthew,  Riedesel Erica

Final Pr. ID: Poster #: EDU-128

Hepatic sinusoidal obstruction syndrome (SOS) - previously termed hepatic veno-occlusive disease (VOD) - is a serious cause of acute liver failure in patients undergoing hematopoietic cell transplantation (HCT).

Hepatic SOS results from injury to the hepatic venous endothelium during intensive myelopablative chemotherapy or radiation therapy prior to HCT. Endothelial injury eventually leads to fibrous obliteration of the terminal hepatic venules with sinusoidal obstruction, hepatocyte necrosis, and hemorrhage, typically seen in the first 3-6 weeks post-transplant.

Early identification of this disease process is critical given high incidence of multi-organ failure and significant morbidity and mortality. Ultrasound is extremely valuable in accurate early diagnosis as imaging findings often precede clinical signs of disease. Spectrum of findings on gray-scale ultrasound includes hepatomegaly, heterogeneous hepatic parenchymal echotexture, ascites, and gallbladder wall thickening. Findings on Doppler ultrasound - increased mean hepatic artery resistive index (RI) and decreased or reversed portal venous flow - are highly sensitivie for hepatic SOS.

In this education exhibit we will review the epidemiology and pathophysiology of hepatic sinusoidal obstruction syndreom (SOS) and discuss imaging findings that aid in accurate diagnosis. Special attention will be given to Doppler ultrasound findings and additional diagnostic information provided by B-flow Doppler ultrasound. Read More

Authors:  Shore Matthew , Riedesel Erica

Keywords:  Hepatic Sinusoidal Obstruction Syndrome, Doppler Ultrasound, B-flow Ultrasound, Educational Exhibit

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

Koujok Khaldoun,  Bilal Ahmed,  Abdeen Nishard

Final Pr. ID: Poster #: EDU-141

The purpose of this educational exhibit is to demonstrate the imaging characteristics of several conditions that cause multifocal liver lesions in children that are immunocompromised, have a history of malignancy, or being treated for a malignancy using a radiological multi-modality method. Read More

Authors:  Koujok Khaldoun , Bilal Ahmed , Abdeen Nishard

Keywords:  hepatic, pediatric, neoplasm

Hanquinet Sylviane,  Morice Claire,  Merlini Laura,  Cousin Vladimir,  Mclin Valérie,  Anooshiravani Mehrak

Final Pr. ID: Poster #: SCI-008

The diagnosis of hepatic encephalopathy is mainly detected by neuropsychological tests. These tests, however, do not always apply depending on the status of the child (age, social and cultural environment) and are therfore not reliable for subtle changes. We propose to detect minimal hepatic encephalopathy (minHE) in children with chronic liver disease and/or porto-systemic shunting, using MRI with Diffusion-weighted (ADC) and ¹H- spectroscopy in the globus pallidum. Read More

Authors:  Hanquinet Sylviane , Morice Claire , Merlini Laura , Cousin Vladimir , Mclin Valérie , Anooshiravani Mehrak

Keywords:  hepatic encephalopathy, 1H- spectroscopy, brain MRI, chronic liver disease, porto-systemic shunting

Mcbee Morgan,  Towbin Alexander,  Dillman Jonathan,  Trout Andrew

Final Pr. ID: Poster #: EDU-004

Cystic liver lesions in the pediatric population are relatively uncommon but encompass a wide range of pathologies. It is important for radiologists to be familiar with the differential diagnosis and imaging characteristics of each entity because prognosis and treatment vary widely. This educational exhibit will review the appearance of various cystic liver lesions in the pediatric population by ultrasound, CT, and MRI and will review clinical presentation and genetic/biologic causes. Read More

Authors:  Mcbee Morgan , Towbin Alexander , Dillman Jonathan , Trout Andrew

Keywords:  hepatic cyst, Cystic mass, Fluid lesion

Lee Samantha,  Cajigas-loyola Stephanie,  Acord Michael

Final Pr. ID: Poster #: EDU-031

Pediatric primary liver tumors, including hepatoblastoma and hepatocellular carcinoma (HCC), are staged according to the PRETEXT system (PRE-Treatment EXTent of Tumor). Although primary hepatic tumors are rare in the pediatric population, hepatoblastoma is increasing in prevalence due to its association with prematurity and the prolonged survival of this patient population. Therefore, it is prudent for radiologists to refamiliarize themselves with these malignancies and how to accurately describe their imaging appearance using descriptors defined by PRETEXT.

Various imaging modalities offer a role in the evaluation of primary hepatic tumors, including ultrasound (US), magnetic resonance (MR), and computed tomography (CT). Contrast enhanced ultrasound (CEUS) is not currently a primary modality but has potential to evaluate vascular involvement and satellite lesions. PRETEXT is the standard to describe a tumor’s imaging features on CT or MR; MR is preferred due to improved soft tissue detail, and emphasis is placed on use of a hepatobiliary contrast agent.

For PRETEXT staging, the liver is divided into four sections: right posterior, right anterior, left medial, and left lateral. Depending on the number of continuous, uninvolved sections, patients are assigned a group ranging from I-IV. Tumors are often large at presentation, and determination of anatomic boundaries can be challenging for the radiologist. Other special circumstances discussed include variant hepatic venous anatomy and pedunculated tumors. PRETEXT also considers “annotation factors”, such as vascular involvement, tumor rupture, and metastases, among others.

In combination with clinical factors, such as the patient's age and alpha-fetoprotein (AFP) level, PRETEXT is also used to stratify patient risk and ultimately influence patient management. If the tumor is not resectable upfront, percutaneous biopsy is recommended to confirm the diagnosis. First line therapy includes surgical resection and systemic chemotherapy. In cases not amenable to this treatment, palliative options offered by pediatric interventional radiology include transarterial chemoembolization (TACE) and percutaneous ablation.

The goal of this educational exhibit is to provide a case-based illustration of PRETEXT staging and annotations factors for the pediatric radiologist. Read More

Authors:  Lee Samantha , Cajigas-loyola Stephanie , Acord Michael

Keywords:  PRETEXT, Primary Hepatic Tumor, Staging

Hakim Ibrahim,  Halabi Safwan

Final Pr. ID: Poster #: EDU-085

Fetal MRI (FMR) has been particularly helpful in the evaluation of abnormalities observed on antenatal ultrasound (US). In this educational exhibit, the aim is to present the spectrum of liver abnormalities encountered in the fetus with US and FMR imaging. The most common entities encountered in utero include hemochromatosis, cystic lesions, hepatoblastoma, hemangioma, and metastatic disease. Antenatal identification of liver abnormalities contributes to accurate diagnosis and recognition of complications, ultimately guiding delivery, postnatal imaging, therapeutic planning, and treatment. Read More

Authors:  Hakim Ibrahim , Halabi Safwan

Keywords:  antenatal, MRI, ultrasound, hepatic, tumors