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Neoplasia

Overview Hepatic neoplasms constitute approximately 2% of all childhood tumors, approximately 6% of pediatric abdominal neoplasms, and are the third most common intra-abdominal tumors in children after Wilms tumor and neuroblastoma. Two-thirds of liver tumors in children are malignant and most of these are hepatoblastomas. The remaining malignant tumors in order of frequency are: hepatocellular carcinoma (HCC) including the fibrolamellar variant, undifferentiated embryonal sarcoma (UES), and…

Infectious Causes of Liver Disease

Introduction A wide range of viruses, bacteria, fungi, and parasites can infect the liver. The liver can be the primary organ of infection (as seen with viral hepatitis), or the liver can be secondarily involved as part of a systemic infection (such as with bacterial or fungal infections). The agents can cause a wide range of disease, ranging from subclinical infection, jaundice and cholestasis, diffuse hepatitis,…

Parenchymal Liver Disease

Introduction Imaging plays a central role in the initial assessment and serial monitoring of chronic pediatric liver disease. While liver biopsy remains the accepted gold standard test for diffuse parenchymal liver disease, a single biopsy specimen only samples 1/50,000 or 0.002% of the liver, and heterogeneity in severity and distribution of disease can result in sampling errors. Liver biopsy is invasive even when done percutaneously, and…

Acquired Biliary Tract Disease

Cholelithiasis and Choledocholithiasis Overview. Cholelithiasis, previously thought rare in children without hemolytic anemia, is diagnosed with increasing frequency since the increased ultrasound use. Gallstones have been reported in the fetus, although most resolve spontaneously. Development of gallstones in infants may be related to immature physiologic regulation of bile salt secretion. Chronic cholestasis likely plays a role in the pathophysiology of cholelithiasis. Although infantile gallstones are often…

Congenital Hepatobiliary Anomalies

Introduction Hepatorenal fibrocystic disorders are a group of associated congenital anomalies of the liver and kidney caused by malformation of the embryonic ductal plates formed by bipotential progenitor hepatoblasts surrounding portal venous ramifications ( Fig. 87.1 ). They include choledochal cysts, Caroli disease, hepatic fibrosis, biliary hamartomas, and cystic liver disease. The specific type of fibrocystic disorder depends on the size of the embryonic duct with…

The Abdominal Wall and Peritoneal Cavity

Peritoneal Cavity Overview. The peritoneum is a thin serosal membrane of mesodermal origin that comprises a single layer of mesothelial cells resting on a basement membrane. It is divided into visceral and parietal components, and the space between the two components constitutes the peritoneal cavity. The layer covering the abdominal viscera, omentum, and the mesenteries is designated visceral, whereas the layer covering the abdominal walls, undersurface…

Prenatal Gastrointestinal and Hepatobiliary Imaging

Overview Fetal abdominal abnormalities evaluated on prenatal imaging can be grouped into one of several general categories: obstructions, ventral wall defects, echogenic bowel and peritoneal abnormalities, and finally masses and solid organ abnormalities. In circumstances when ultrasound (US) cannot accurately determine the extent or nature of an abnormality, fetal magnetic resonance imaging (MRI) can be useful. Sequence selection includes standard single-shot fast spin echo (SSFSE) T2-weighted…

Embryology, Anatomy, and Normal Findings

Abdominal Wall and Peritoneal Cavity During the third week of gestation, the intraembryonic mesoderm differentiates, forming the lateral plates. Mesoderm from paravertebral myotomes invades the lateral plates during the sixth week. The leading edges of the lateral plates will differentiate into the right and left rectus abdominis muscles. The main portion of the mesoderm splits into three layers and forms the external oblique, internal oblique, and…

Acquired Diseases of the Thoracic Great Vessels

This chapter reviews acquired diseases of the thoracic aorta, venae cavae, pulmonary arteries, and pulmonary veins seen in childhood. Acquired pediatric aortic disease is uncommon, but radiologists and imagers play an important role in the care of these patients. Pulmonary embolism (PE) is the most common acquired disease of the pulmonary artery. The most common acquired abnormalities of the pulmonary veins and venae cavae are obstruction…

Cardiovascular Involvement by Systemic Diseases

Numerous systemic diseases can affect the heart and great vessels and are important causes of cardiac dysfunction. These systemic diseases include both prenatal and postnatal toxic and infectious exposures ( e-Fig. 81.1 ); adverse effects of therapeutic agents; and various nutritional, metabolic, inflammatory, granulomatous, and autoimmune entities. Endocrine, circulatory, and blood disorders frequently have secondary cardiac effects. Primary cardiac tumors can occur in association with underlying…

Cardiac and Pericardial Tumors

Primary cardiac tumors are rare in children, with a reported prevalence of up to 0.32%. Use of echocardiography has resulted in more frequent detection of cardiac tumors in the fetus and neonate. More than 90% of pediatric cardiac tumors are benign. Symptoms are variable and usually depend on tumor location and size. Intracavitary cardiac tumors can cause cardiac valve obstruction or spread of tumor emboli, and…

Myocardial, Endocardial, and Pericardial Diseases

Myocardial Diseases Cardiomyopathies include various diseases of the myocardium, often chronic or progressive, and associated with cardiac dysfunction. These are rare but serious disorders; only 25% of children survive more than 5 years after the onset of symptoms. Although our understanding of the causes of pediatric cardiomyopathy has advanced, the prognosis has not changed considerably in the past 30 years and is the same in developing…

Syndromes and Chromosomal Anomalies

A large number of syndromes, dysplasias, and chromosomal anomalies are associated with congenital or acquired cardiac and vascular disease. This chapter discusses the cardiovascular features of some of the more commonly encountered lesions. A more extensive list is provided in Tables 78.1 to 78.3 later in this chapter. Syndromes Situs and Cardiosplenic (Heterotaxy) Syndromes Overview. The heterotaxy syndromes, comprising right and left isomerism, feature abnormalities of…

Coronary Artery Disease in Children

The author would like to acknowledge Frandics Chan, MD, PhD, for his contributions to prior editions of this chapter. Coronary artery disease in children can be acquired, but the majority of anomalies are congenital. Among acquired coronary abnormalities, Kawasaki disease (KD) is the most common. Other causes include sequelae from trauma, vasculitides, radiation injury after oncologic treatment, and rare cases of familial hyperlipidemia and idiopathic infantile…

Congenital Anomalies of the Thoracic Great Arteries

This chapter covers congenital anomalies of the great arteries that result in a vascular ring or sling. Overall, anomalies of the aortic arch and the cervical vessels are relatively common, with an estimated prevalence of 0.5% to 3%. They can be understood as the result of abnormal persistence and/or regression of branches in the totipotential arch, which is derived from the theoretical double aortic arch proposed…

Conotruncal Anomalies

Conotruncal anomalies are a group of congenital heart defects involving the outflow tracts of the heart. The conotruncal anomalies include tetralogy of Fallot (TOF), transposition of the great arteries (TGA), double-outlet ventricles, truncus arteriosus, and interrupted aortic arch (IAA) type B. Conotruncal abnormalities are the result of abnormal division or rotation of the primitive truncus during embryologic development. The common outlet of the embryonic univentricular heart…

Right Heart Lesions

Imaging of the Right Heart The algorithms used for left ventricular assessment cannot always be applied to the right ventricle (RV), which differs in morphology, shape, and function. The RV has a complex geometrical shape with a thinner and more trabeculated myocardium than the left ventricle (LV). The chest x-ray is a preliminary imaging tool, with the lateral view best suited to detect RV enlargement. Echocardiography…

Left Heart Lesions

Hypoplastic Left Heart Syndrome Overview. Hypoplastic left heart syndrome (HLHS) is a spectrum of disease characterized by underdevelopment of the left ventricle with obstruction or atresia of ventricular inflow and outflow. HLHS accounts for approximately 2% to 3% of all cases of congenital heart disease, with a slight male predominance. HLHS is most commonly isolated, but extracardiac anomalies occur in up to 30% and a variety…

Systemic to Pulmonary Arterial Shunts

Atrial Septal Defect Overview. An atrial septal defect (ASD) is a defect in the atrial septum that allows communication between the right atrium (RA) and left atrium (LA). Isolated ASDs account for 6% to 10% of all congenital heart disease. Etiology. Two primary types of ASDs occur and are classified by their relationship to the fossa ovalis ( Fig. 72.1 ). Secundum-type defects (80%–90% of all…

Abnormal Pulmonary and Systemic Venous Connections

Partial Anomalous Pulmonary Venous Connection and Scimitar Syndrome Overview. Partial anomalous pulmonary venous connection (PAPVC) is present when one or more pulmonary veins drain into a systemic vein. Because a single anomalous connection may be unrecognized, the incidence is difficult to establish, but it has been reported to be present in 1 in 200 postmortem examinations. Etiology, Pathophysiology, and Clinical Presentation. All pulmonary veins from one…