Pediatric Liver.pptx

Pediatric Abdomen Mrs. Amans Objectives Demonstrate the sonographic scanning techniques, technical considerations, and routine examination for the neonatal and pediatric abdomen to include the prevertebral vessel (aorta and inferior vena cava) evaluation, liver, gallbladder and biliary syste...

Pediatric Abdomen Mrs. Amans Objectives Demonstrate the sonographic scanning techniques, technical considerations, and routine examination for the neonatal and pediatric abdomen to include the prevertebral vessel (aorta and inferior vena cava) evaluation, liver, gallbladder and biliary system, pancreas, gastrointestinal tract, and retroperitoneum. Describe the pathology, etiology, and clinical signs and symptoms for anomalies and pathology of the aorta and inferior vena cava, liver, gallbladder and biliary system, pancreas, gastrointestinal tract, and retroperitoneum in the neonate and pediatric patient. Differentiate between the sonographic appearance of the normal prevertebral vasculature and the sonographic appearance for congenital anomalies and acquired pathology of the prevertebral vessels, liver, gallbladder and biliary system, pancreas, gastrointestinal, tract and retroperitoneum in the neonate and pediatric patient. Identify technically satisfactory and unsatisfactory sonographic examinations of the abdomen on the neonatal and pediatric patient. List the indications for the sonographic evaluation of urinary system and adrenal glands in the pediatric patient. 2 Objectives Explain the protocol process for sonographic evaluation of the urinary system and adrenal glands in the pediatric patient. Identify the normal sonographic appearance of the urinary system and the adrenal glands in the pediatric patient. Describe the pathology, etiology, clinical signs and symptoms, and sonographic appearance of common congenital abnormalities, tumors, and acquired pathology in the upper and lower urinary system in the pediatric patient. Discuss three criteria for sonographic documentation of tumors on pediatric patients to include (1) origin of the mass, (2) extent of the mass, and (3) metastases. Describe the pathology, etiology, clinical signs and symptoms, and sonographic appearance of congenital abnormalities, tumors, hemorrhage, cysts, and abscesses of the adrenal glands in the pediatric patient. Identify technically satisfactory and unsatisfactory sonographic examinations of the urinary system and adrenal glands on the neonatal and pediatric patient. 3 Introduction Sonography is the noninvasive modality of choice to evaluate the neonatal and pediatric abdomen owing to the lack of ionizing radiation, the portability of the equipment, and excellent visualization of the abdominal anatomy in this age group. Critically ill patients who are sensitive to stress (i.e., transport, temperature changes) can easily and safely be examined at the bedside. Pediatric sonography presents many opportunities as well as challenges. Childhood obesity is a serious health care problem, and obese children may be as challenging to examine sonographically as adults. Obese children may also present with some disease processes previously seen only in adults, so the sonographer must have an in-depth knowledge of both pediatric and adult pathology. 4 Sonographic Examination Techniques Scanning the pediatric age group will require sonographic equipment with a wide range of probe frequencies. Depending on the area or organ of interest, a high- frequency (7 to 10 MHz) curved or (8 to 11 MHz) micro- convex or (8 to 15 MHz) linear array transducer is utilized. Scanning infants and small children requires the sonographer to be adept at assessing the anatomy and acquiring images quickly. Distraction techniques are used for this age group rather than sedation. 5 Sonographic Examination Techniques Children with high level of pain, anxiety, or autism spectrum disorder with comorbid developmental delay may or may not be able to cooperate fully during the sonogram. Use of distractions such as headphones, development-appropriate movies, or other resources ensures that the sonographer can complete the examination accurately and in a timely manner. Many health care facilities now employ Certified Child Life Specialists. Professionals may be utilized to assist with therapeutic play and assist with coping skills to manage stressful experiences. 6 Sonographic Examination Techniques Parents are likely be present for the examination. Sonographer must be prepared to professionally interact with the parents and elicit their assistance. Always explain the examination to the patient using age-appropriate terms and answer parent’s questions about the examination in accordance with department policies and procedures. Special precautions should be taken to keep infants warm by placing blankets over all but the scanning surface. 7 Sonographic Examination Techniques Warm gel should always be used on children. Single packets of gel for infection control must be used for neonates and critical care patients. Sterile gel packets should be used whenever a sterile area must be maintained or in cases where infection is of high concern. Sonographer must always follow infection control standards when scanning. Can be even more important when examining pediatric patients Pediatric abdominal sonogram should include an assessment of all the organs, structures, and vessels of the abdomen. 8 Sonographic Examination Techniques Patient Preparation Patient preparation will vary based on the age of the patient and the area or organ of interest. Ideally, the liver and biliary tree are best viewed with the patient in a fasting state. As infants are fed every 3 to 4 hours, the examination should be performed just before a feeding. Children aged 1 to 3 years are best examined 4 hours after fasting and older children 6 hours after fasting. Children with gastronomy (G) or gastronomy-jejunostomy (GJ) tubes are typically fasted between 4 and 6 hours before the procedure. Diabetic patients may require prioritization according to their insulin schedule and may be permitted clear liquids. 9 Review What is the ideal patient preparation for examination of the biliary tree in a 3-year-old? A. No preparation B. 4 hours fasting C. Following a fatty meal D. Well hydrated 10 Prevertebral Vessels Prevertebral Vessel Evaluation Sonographic Examination Technique Patient Preparation Full length of the great vessels can be evaluated from the diaphragm to the bifurcation without patient preparation. NICU patients are often intubated, so turning the patient onto one side or the other may be necessary. Right coronal scanning is most effective in demonstrating the IVC. Left coronal approach is best to visualize the aorta. 12 Prevertebral Vessel Evaluation Sonographic Examination Technique Scan Technique Sonographer should image the aorta and IVC from proximal to distal (bifurcation), including sonographically visible branches and tributaries. Split-screen color Doppler and grayscale imaging can be used to demonstrate the vessels when grayscale alone is insufficient or pathology is present. 13 Prevertebral Vessel Evaluation Sonographic Examination Technique Scan Technique Color and spectral Doppler should also be used to evaluate flow in the aorta, IVC, right and left iliac arteries and veins, and right and left renal arteries and veins. Correct presentation of the abdominal aorta and IVC must be documented so that the correct location and course of these vessels is confirmed. 14 Prevertebral Vessel Evaluation Sonographic Examination Technique Scan Technique Aorta indwelling catheter Relationship to renal arteries must be demonstrated. Umbilical arterial catheter (UAC) Visible on a radiograph, but its location is not reliable. Proper location well above the level of the renal arteries must be documented. 15 Prevertebral Vessel Evaluation A B Umbilical arterial catheter (UAC). A: An indwelling UAC is visualized as two parallel lines (arrow), with an anechoic center representing the catheter lumen on this longitudinal section of the abdominal aorta (A). B: Patent vasculature is appreciated. 16 Prevertebral Vessel Evaluation Sonographic Examination Technique Scan Technique Umbilical venous catheter (UVC) May also be present in the umbilicus next to a UAC (or by itself) Courses through the liver via the umbilical vein and the left hepatic vein Tip should be in the proximal IVC near the junction of the right atrium. UAC and UVC are seen as hyperechoic parallel lines with an anechoic center. Shadowing from the walls may occur with a perpendicular beam. Do not confuse wall shadowing with intrahepatic calcifications. 17 Prevertebral Vessel Evaluation UVC and UAC 18 Prevertebral Vessel Evaluation Figure 20-5 A A: The arrows indicate the correct position on an umbilical venous catheter (UVC). 19 Prevertebral Vessel Evaluation Normal Anatomy Sonographic appearance of the abdominal vessels in the pediatric patient is the same as in an adult. Vessels should have anechoic lumens with hyperechoic walls. Walls of the abdominal aorta may appear more echogenic than the walls of the IVC. Normal spectral Doppler of the aorta shows a pulsatile vessel with a high- resistance flow pattern. Normal spectral Doppler flow pattern of the IVC is monophasic. 20 Prevertebral Vessel Evaluation Congenital Anomalies Coarctation of the Abdominal Aorta Hypoplasia or coarctation of the abdominal aorta is a rare congenital defect. Proximal descending thoracic aorta is affected in 98% of coarctations, only 2% of them actually affect the abdominal aorta. Renal artery stenosis occurs in more than half of abdominal coarctations. Congenital abdominal coarctation can occur at any time in embryonic development. The earlier it occurs, the more obvious the manifestations. Acquired coarctation of the abdominal aorta has been associated with hypercalcemia, neurofibromatosis, tuberous sclerosis, rubella, and Turner syndrome. 21 Prevertebral Vessel Evaluation A B A: Coarctation of the abdominal aorta (Ao) is demonstrated on this longitudinal section with the interruption of the abdominal aorta and collateral circulation of the superior mesenteric artery (SMA) and renal artery (RA). B: There is hypoplasia of the inferior abdominal aorta with hypertrophied collateral artery, which courses anterior to the midline. 22 Prevertebral Vessel Evaluation Congenital Anomalies Coarctation of the Abdominal Aorta Children present with: Severe hypertension Headaches Fatigue Infants exhibit: failure to thrive 23 Prevertebral Vessel Evaluation Congenital Anomalies Coarctation of the Abdominal Aorta Interrupted abdominal aorta: Produces vascular compromise. Symptoms: Cyanotic, mottled, and discolored limbs Decreased femoral pulses. Can be fatal by the age of 30 years due to severe hypertension Interrupted Aortic Arch 24 Coarctation of the Aorta- Normal Aorta - Fetus fetus Prevertebral Vessel Evaluation Congenital Anomalies Inferior Vena Cava Normal: IVC is located on the right side, receiving the hepatic veins as it enters the right atrium. IVC on the patient’s left side is diagnostic of situs inversus. Interrupted IVC: Drains via an azygous continuation Related to heterotaxy syndrome May lie on either the left or right of the spine Hemiazygous continuation lies more posterior than the aorta. 26 Normal Azygous Vein - fetus Prevertebral Vessel Evaluation Congenital Anomalies Inferior Vena Cava Another abnormal vessel that may be imaged in the long-axis plane is an anomalous venous connection associated with total anomalous pulmonary venous return, which connects to the ductus venosus. Crosses between the aorta and the IVC Displacement or distortion of the IVC or the aorta should alert the sonographer that other anomalies may be present. Sonographers must be cognizant of unusual presentations of the aorta or IVC Anomalous vessels in the lower abdomen may indicate complex congenital heart disease. 28 TPVR - Fetus Normal Pulmonary veins - Fetus Prevertebral Vessel Evaluation Acquired Pathologies Abdominal Aortic Thrombosis in the Neonate Most common reason for evaluating the aorta in the neonate is for aortic thrombus Well-recognized complication of indwelling umbilical artery catheters (UACs). Clinical signs of aortic thrombus Absent femoral pulses Hematuria Cyanosis Hypertension Blanching of the lower extremities Necrotizing enterocolitis Overly distended urinary bladder may cause some of the abovementioned symptoms. 30 Prevertebral Vessel Evaluation Acquired Pathologies Abdominal Aortic Thrombosis in the Neonate Suspected aortic thrombus should be evaluated by a thorough scan of the entire aorta and both kidneys in multiple planes. Thrombus typically appears sonographically as echogenic material within the aortic lumen, which may totally or partially fill the vessel. Clot may be long and thick and is termed extensive if it does one or more of the following: Fills 40% of the aorta in a sectional plane Goes to the level of the renal artery or iliac artery Causes proximal dilatation. 31 Prevertebral Vessel Evaluation Acquired Pathologies Abdominal Aortic Thrombosis in the Neonate As the sonographic appearance of thrombus changes over time, vessel may appear to contain thin linear structures. Color Doppler should be used to demonstrate any blood flow around the thrombus, normal flow reversal, and the presence of any collateral vessels. Grayscale and color Doppler should be used to follow the progression and/or resolution of the thrombus. 32 Prevertebral Vessel Evaluation C: A linear echogenic structure (arrowheads) extending from the mid-aorta through the aortic bifurcation into the right common iliac artery, compatible with nonobstructive thrombus in an ex- 31-week neonate with DiGeorge syndrome on extracorporeal C membranous oxygenation secondary to complex cardiac disease. D: Follow-up ultrasound demonstrates continued resolution of nonocclusive thrombus in the infrarenal aorta, seen on B-flow. D 33 Prevertebral Vessel Evaluation Acquired Pathologies Inferior Vena Cava Thrombosis IVC can be a site of thrombus or calcifications in neonates. IVC thrombosis can also occur secondary to Indwelling catheters (such as UVC) Clotting disorders Dehydration Sepsis Nephrotic syndrome Extension of renal vein thrombosis Extension of pelvic vein thrombosis 34 Prevertebral Vessel Evaluation Acquired Pathologies Inferior Vena Cava Thrombosis Inferior Vena Cava Tumor Invasion Children can have tumor invasion into the IVC from Wilms tumor. Tumor extension can occur from the kidney, adrenal gland (neuroblastoma), retroperitoneum (sarcoma), and from hepatocellular carcinoma (HCC), teratoma, and lymphoma. Important to evaluate the extension of the tumor into the hepatic veins or right atrium and to seek evidence of tumor invasion into the wall of the IVC. 35 Prevertebral Vessel Evaluation B C B: The loss of flow (arrows) is located in the intrahepatic inferior vena cava (IVC) surrounding the UVC, reflecting pericatheter thrombus. C: An echogenic clot (arrow) is visualized within the lumen of the IVC on this longitudinal section seen posterior to the liver. 36 Prevertebral Vessel Evaluation D E D: The dimension of the echogenic clot (arrow) is further identified when it fails to demonstrate blood flow during investigation with Color Doppler, with extension of the clot to the proximal right hepatic vein. E: This image illustrates the aorta (Ao) and the relationship of other vessels. (Images C and D: Courtesy of Primary Children’s Hospital, Salt Lake City, UT.) 37 Prevertebral Vessel Evaluation Acquired Pathologies Inferior Vena Cava Thrombosis Inferior Vena Cava Tumor Invasion Tumor extension appears similar to the solid texture of the tumor itself. Differential diagnosis includes simple thrombus. Computed tomography (CT) is the modality of choice for evaluating IVC wall invasion. Sonography is the best modality for evaluating cephalad extension of IVC tumor invasion. 38 Review Which is characteristic of an anomalous venous connection? A. Associated with arterial blood return B. Crosses between the IVC and the SMV C. Displaces the IVC and/or the aorta D. Associated with normal abdominal vessels 39 Review Where is the tip of the umbilical venous catheter (UVC) imaged? A. Proximal to the left atrium B. Proximal to the right atrium C. Proximal to the left ventricle D. Proximal to the left atrium 40 Review Which type of metastatic tumor is common to invade into the IVC? A. Liver B. Wilms C. Pancreas D. Bone 41 Pediatric Liver Liver Sonographic Examination and Technique Scan Technique Establishing a protocol of longitudinal, coronal, and transverse planes is important to ensure consistency. Examination of the pediatric liver should include assessment of the liver parenchyma, vessels and ligaments in multiple scan planes, and the use of color and/or spectral Doppler to assess blood flow. Patients are most commonly scanned in the supine position. LPO position may be helpful, especially in older and/or obese children. 43 Liver Sonographic Examination and Technique Scan Technique Normal measurement parameters for the liver have been reported and show correlation with age, height, and weight. Longitudinal images demonstrating the lower pole of the right kidney in relationship to the inferior margin of the liver can be helpful. Normal liver appears as a smooth-outlined, homogeneous organ, usually situated in the right upper quadrant of the abdomen. Neonatal liver may appear mildly hyperechoic. 44 Liver Normal Anatomy Liver is divided into Large right lobe in the right side of the abdomen Smaller left lobe extending across the midline Caudate lobe situated on the posterior superior surface of the right lobe Quadrate lobe on the posteroinferior surface of the right lobe Falciform ligament divides the right and left lobes. 45 Liver Normal Anatomy Liver receives a dual blood supply. Liver receives oxygenated blood from the hepatic artery, a branch of the celiac artery. Hepatic artery also enters the liver at the porta hepatis. Blood from the digestive system is carried to the liver via the portal vein, formed by the convergence of the superior mesenteric vein (SMV) and the splenic vein. Portal vein enters the liver at the porta hepatis, where it quickly branches into the right and left portal veins. Major vessels of the liver provide important visual and anatomic landmarks. 46 Liver A B Figure 20-6 Neonatal liver. A: Transverse image of the liver in a neonatal patient demonstrates the normal homogeneous echo texture of liver and anechoic vasculature. (Image courtesy of Philips Medical Systems, Bothell, WA.) B: Transverse image of the liver in a neonatal patient demonstrating flow in the portal vein and hepatic veins. C (Image courtesy of GE Healthcare, Wauwatosa, WI.) C: A high-frequency linear transducer is used to image the patient. 47 Liver Normal Anatomy Applicable laboratory tests include the standard liver function tests and alpha-fetoprotein (AFP), which, if elevated, may indicate the presence of a hepatoblastoma or other malignant tumor. 48 Congenital Anomalies and Benign Tumors Hemangiomas Arteriovenous malformation (AVF) Forms blood filled spaces Most common vascular tumor in infancy Two Types: Hemangioendotheliomas Multi-layered or hypertrophic lining Cavernous  Congenital Anomalies and Benign Tumors Hemangioma Hemangioendotheli omas Affect infants Boys Noticed around 2 months of age (but may be present at birth) Present with hepatomegaly and/or Congenital Anomalies and Benign Tumors Hemangiomas Cavernous Hemangiomas Complications of large hemangiomas: Obstructive jaundice Fatal rupture Irreversible congestive Kasabach-Merritt syndrome heart failure Affected blood platelets Respiratory Portal hypertension Insufficiency Intravascular coagulation Intestinal Bleeding  Congenital Anomalies and Benign Tumors Hemangiomas Cavernous Less often, Hemangiomas Hypoechoic ​ Sonographic Mimic multiple small Appearance: cysts Well-defined Complex Hyperechoic Irregular walls From multiple Hypoechoic to interfaces of blood anechoic due to  Congenital Anomalies and Benign Tumors Hemangiomas Cavernous Hemangiomas Calcifications or fibrotic changes create a hyperechoic pattern with posterior shadowing Vascular flow within the lesion  Liver Cavernous Hemangioma D: Dual-screen contrast-enhanced ultrasound of an atypical hemangioma in an 8-year-old female. Following contrast administration, there is prompt peripheral nodular enhancement during the early arterial phase that progresses from peripherally to centrally according to a spoke-wheel pattern. Enhancement progresses during the late arterial and portal venous phases and becomes homogeneous, with the exception of nonenhancement of the central scar and central coarse calcifications. During the delayed phase, lesion enhancement is persistently greater than the background liver, consistent with hemangioma. 60  Congenital Anomalies and Benign Tumors Hemangiomas For those with CHF, Cavernous hepatic artery ligation or embolization Hemangiomas Steroid and radiation Biopsy is a last resort for therapy diagnosis due to the risk Differentials: of bleeding in neonates Hepatoblastoma Most involute and resolve Hepatoma on their own For large or symptomatic Metastatic Congenital Anomalies and Benign Tumors Mesenchymal Hamartoma Arises from the connective tissue or mesenchyme of the portal tracts 2nd most common benign hepatic Congenital Anomalies and Benign Tumors Mesenchymal Hamartoma Signs and Symptoms: Presents in the first 2 years of life Painless abdominal swelling Congestive heart failure AV Shunting within tumor Respiratory distress with rapidly growing tumor Normal Liver Labs Congenital Anomalies and Benign Tumors Mesenchymal Hamartoma Ultrasound Appearance: Complex Avascular Internal septations Formed by hepatocytes, bile ducts elements, mesenchyme Usually in the right lobe Congenital Anomalies and Benign Tumors Mesenchymal Differentials: Hamartoma Mesenchymoma Prognosis: Hemangioma Parasitic or congenital Good cyst Resection needed Teratoma Percutaneous drainage Biliary cystadenoma before resection in the those with respiratory Choledochal cyst distress Congenital Anomalies and Benign Tumors FNH Adenoma Other rare benign lesions that present the same in adults and children: Focal nodular hyperplasia Hepatic adenoma Nodular regenerative hyperplasia NRH "Fatty Fatty tumors Tumor" Congenital Anomalies and Benign Tumors Cysts: Congenital Small to large cysts Polycystic liver disease is associated with polycystic kidney disease and von Hippel- Lindua disease Acquired Hydatid cysts Traumatic cysts/blunt force trauma Congenital Anomalies and Benign Tumors Cysts: Symptoms- Simple Cysts: Small lesions – Asymptomatic Large lesions – Impaired liver function Abdominal distention Palpable mass Congenital Anomalies and Benign Tumors Cysts: Sonographic Appearance – Simple Cyst: Smooth-walled Anechoic Posterior enhancement May be completely intrahepatic, partly intrahepatic, or extrahepatic on a stalk Congenital Anomalies and Benign Tumors Cysts: Sonographic Appearance – Hydatid echinococcal: Simple cyst appearance Complex cyst (daughter cysts) Or either with calcifications Congenital Anomalies and Benign Tumors Cysts: Hydatid Cysts - Symptoms: Peak age is 5 to 15 years 25% asymptomatic 60% present with Urticaria RUQ pain Hepatomegaly and/or abdominal distention Associated with hydatid lung cysts Congenital Anomalies and Benign Tumors Cysts: Hydatid Cysts - Symptoms : 40% present with complications: Rupture into the pleural space/peritoneal Anaphylactic shock Pneumonia Organism can pass through the liver and lodge into other areas, causing: Infection Impaired liver function Biliary obstruction Congenital Anomalies and Benign Tumors Cysts: Hydatid Cysts – Treatment: Aspiration Capitonnage or Omentopexy Surgery Differential Ovarian/Mesenteric Cysts Teratoma Mesenchymoma Hepatic granuloma Hepatic Trauma Most commonly injured abdominal organ in children is the liver Right lobe > left lobe Types include: Subcapular Hematomas Lacerations Fractures Hemoperitoneum is often present too Hepatic Trauma Sonographic Appearance – Changes over time: Anechoic in early stages Complex over time Anechoic in late stages Calcifications Necrosis/Air/Gas Possibly in very late stages: Pseudoaneurysms Bilomas (walled off collections of bile) Biloma Infectious and Inflammatory Disease Hepatitis Diffuse infection of the liver Clinical symptoms – Depend Characterized by on the stage of the disease inflammation and hepatic Pain cell necrosis Nausea Usually viral in origin (A, B,C, Fever D, OR E; Cytomegalovirus, Chilles herpes, and Epstein-Barr) Jaundice Type A – fecal-oral Weight loss transmission; Most common in Fatigue children Loss of appetite Noninfectious include toxin exposure, drugs, sclerosing Infectious and Inflammatory Disease Hepatitis Ultrasound Appearance – Dependent on the stage of the disease Acute: Hepatomegaly Decreased echogenicity Increased portal wall echogenicity Chronic: Decreased liver size Increased echogenicity and attenuation Infectious and Inflammatory Disease Hepatitis Ultrasound Appearance – Dependent on the stage of the disease Chronic: Decreased liver size Increased echogenicity and attenuation Can lead to cirrhosis, liver damage, and cancer Severe cases: thickened GB wall, sludge, enlarged portal hepatis lymph nodes Elastography can be used to monitor progression  Transplacen tal Infectious and Inflammatory Disease Abscess Ultrasound appearance: Infants present the same as older patients Varying appearances: Discretely marginated to poorly defines margins Hypoechoic to complex Gas/Air - echogenic with a shadowing or reverb Transplacental – calcifications, hyperechoic lesion with posterior shadowing "Bull's Eye" presentation "Bull's Eye" Infectious and Inflammatory Disease Abscess Pyogenic Liver Abscess Rare in children Can be fatal Secondary to bowel, trauma, or surgery related infections Immunocompromised patients Also related to candida organisms Infectious and Inflammatory Disease Abscess Fungal Abscess Immunocompromised patients Candida albicans Typically seen as Multiple small lesions Irregular walls Spleen and kidney abscess too Sometimes have the "target" appearance Infectious and Inflammatory Disease Abscess Amebic Affects children in areas with poor drinking water Right lobe more commonly affected Hypoechoic, spherical lesion Diffuse Liver Disease Fatty infiltration Hepatic Fibrosis Cirrhosis Hemosiderosis Metabolic diseases Diffuse Liver Disease Fatty infiltration Accumulation of triglycerides and lipids in the hepatocytes Related to childhood obesity, malignancies, metabolic diseases, cystic fibrosis, or toxin exposure Sonographically, hepatomegaly with  Diffuse Liver Disease Metabolic Liver Disease Caused by a defect in enzyme or transport proteins Damages the liver resulting in cirrhosis or liver failure Also caused by metabolic defects in the liver causing damage to other organs Glycogen Storage Disease (Type I von Gierke's most common) Cystic fibrosis Wilson disease Same appearance as Fatty Liver  Diffuse Liver Disease Cirrhosis Parenchymal destruction Scarring fibrosis Nodular regeneration In children, caused by Biliary atresia Cystic fibrosis Chronic hepatitis Metabolic disease Prolonged parenteral nutrition Budd-Chiari syndrome medications Diffuse Liver Disease Cirrhosis Same presentation as in adults Secondary signs Ascites Splenomegaly Portal hypertension Diffuse Liver Disease Hemochromatosis Excessive amounts of iron are stored Video in the liver Genetic, secondary, or transfusional Hemosiderosis is iron storage in the liver resulting from repeated blood transfusions Presents with decreased echogenicity MRI is the gold standard Malignant Neoplasms Overview: Primary liver neoplasms are Important to: more common in children than Identify the extension of the adults tumor into major blood vessels 2/3rds of pediatric liver tumors Invasion of surrounding are malignant vessels is common Include: Which vessels are invaded Hepatoblastoma determine treatment HCC (hepatoma) Differentiate extension from Mesenchymal thrombus Sarcoma Congenital Neuroblastoma Etc Malignant Neoplasms Vascular invasion can impact the treatment decisions of hepatic malignancies. Sonographically, malignancies usually demonstrate as: Solitary Solid Homogeneous Hyperechoic Less frequently as multiple hyperechoic lesions Sometimes hypoechoic halo or rim may be seen Infrequently may be isoechoic to normal liver tissue Malignant Neoplasms Hepatoblastoma Most common pediatric liver mass Occurring most commonly in boys less than 5 years of age Associated with Beckwith–Wiedemann syndrome (hemihypertrophy, macroglossia, hypoglycemia, organomegaly, and omphalocele) Fetal alcohol syndrome Development of Wilms tumor Dysplastic kidney Meckel diverticulum Malignant Neoplasms Hepatoblastoma Presentation: Hepatomegaly/painless abdominal mass (90%) Elevated AFP Elevated AST/ALP Anemia Thrombocytosis Malignant Neoplasms Hepatoblastoma Presentation: In advanced cases accompanying fever, weight loss, pain, nausea, vomiting, jaundice, anemia, leukocytosis, adenopathy, and fractures due to bone metastases. Elevation of AFP in 84% to 91% of cases Decreases after resection Transaminase elevation, anemia, and thrombocytosis may occur as well Malignant Neoplasms Hepatoblastoma Tumor should be considered resectable if: It does not occupy more than one lobe Has no extrahepatic extension Does not invade the portal vein Unresectable tumors can be biopsied and converted to resectable tumors by chemotherapy. Chemotherapy is applied before surgery to shrink the tumor, resulting in improved operability. Malignant Neoplasms Hepatoblastoma Sonographic Appearance: Solitary Multinodular Heterogenous Hyperechoic Indistinct borders Anechoic foci (necrosis) calcifications Malignant Neoplasms Hepatocellular Carcinoma or Hepatoma (HCC) Children > 3 years Associated with chronic liver diseases Significant differentiating characteristics Daughter nodules Hepatic or portal tumor thrombosis Septa Pseudocapsules Well encapsulated or nonencapsulated Multicentric Malignant Neoplasms Hepatocellular Carcinoma or Hepatoma (HCC) Clinical Presentation Sudden liver failure Hepatomegaly Pain GI bleeding Ascites Anorexia Hypoglycemia Anemia Weakness Fever Elevated AFP (60%-80%) Malignant Neoplasms Hepatocellular Carcinoma or Hepatoma (HCC) Sonographic Appearance Solid Hyperechoic Involves the entire liver Well-defined or ill-defined borders Anechoic areas(necrosis or hemorrhage) Hypoechoic "halo" Tumor thrombi in the portal veins, hepatic veins, and IVC If cirrhosis develops, prognosis is poor. Malignant Neoplasms Hepatocellular Carcinoma or Hepatoma (HCC) Fibrolamellar HCC – subtype Teens and young adults AFP normal or mildly elevated Symptoms are: Pain Weight loss Fever Diarrhea/vomiting Well- marginated, solitary tumor Varying echogenicity Sometimes with calcifications or central scar Biopsy needed to differentiate from other masses Malignant Neoplasms Mesenchymal Sarcoma 5 to 10 years of age Large Fast growing Round Singular Well-defined borders Thick fibrous pseudocapsule Usually in the right lobe Malignant Neoplasms Mesenchymal Sarcoma Can spread to the abdominal cavity, diaphragm, or lungs Subtypes include: Embryonal sarcoma Rhabdomyosarcoma Angiosarcoma Malignant mesenchymoma Malignant Neoplasms Mesenchymal Sarcoma Clinical symptoms include: Abdominal pain and swelling Palpable mass No Jaundice No Increased AFP Ultrasound appearance: Single, hyperechoic mass containing anechoic spaces Or, homogenous and hyperechoic Or, as a complex lesion with calcifications Malignant Neoplasms Metastases Most commonly from neuroblastoma Variable echogenicity Lymphoma of the liver is usually secondary to non- Hodgkin lymphoma Hypoechoic nodules Hepatosplenomegaly Hepatic Vascular Disorders Vascular disorders of the liver include Portal hypertension Budd–Chiari syndrome Portal vein thrombosis Hepatic infarction Peliosis hepatis Portal venous gas Hepatic Vascular Disorders Portal Hypertension Increased resistance to the normal portal venous flow. Clinical symptoms: Splenomegaly Ascites Caput Medusa In severe cases, hematemesis, hepatic encephalopathy, hypersplenism Hepatic Vascular Disorders Portal Hypertension Obstruction to flow can be: Prehepatic Portal or splenic vein thrombosis Intrahepatic Secondary to cirrhosis Hepatic vein obstruction (less common) Post Hepatic Secondary to congestive heart failure or restrictive pericarditis Hepatic Vascular Disorders Portal Hypertension Ultrasound Appearance: Bi-direction or hepatofugal portal vein flow Varices Splenomegaly Ascites Cirrhosis Hepatic Vascular Disorders Portal Hypertension Normal Ultrasound Appearance: Loss of respiration variation in portal vein Increased flow in hepatic artery Abnorm Loss of pulsatility al /monophasic hepatic vein waveform Hepatic Vascular Disorders Portal Vein Thrombosis Caused by tumor invasion or thrombus HCC or hepatoblastoma Nonmalignant causes include Improper UVC placement Dehydration Shock Sepsis Hypercoagulable states Portal Hypertension Hepatic Vascular Disorders Portal Vein Thrombosis Clinical Presentation Acute abdominal pain Splenomegaly Hepatic Vascular Disorders Portal Vein Thrombosis Can be acute or chronic Acute: Chronic: Enlarged vein Cavernous transformation Echogenic (multiple tortuous vessels in the porta hepatis region) Absent flow (thrombus) Non-visualization of the Some flow through the portal vein occlusion (tumor invasion) Hepatic Vascular Disorders Portal Vein Thrombosis Can be acute or chronic Acute: May be anechoic in earliest stages Use color Doppler to confirm no flow Hepatic Vascular Disorders Budd-Chiari Syndromes Idiopathic or neoplastic occlusion of the hepatic veins Idiopathic Hypercoaguable states Trauma Cirrhosis Gaucher diseased Neoplastic Hepatoblastoma HCC Wilms tumor Thrombosis IVC occlusion Congenital membrane Extrinsic compression Hepatic Vascular Disorders Budd-Chiari Syndromes Ultrasound Appearance Hepatomegaly Echogenic clot Lack of Color/PW flow Sometimes: Ascites Pleural effusion Gallbladder wall edema Budd–Chiari syndrome A B A: Irregular, narrowed, and tortuous hepatic vessels. B: Nonvisualized right hepatic vein and diminutive-appearing left and middle hepatic veins compatible history of Budd– Chiari syndrome. 115 Budd–Chiari syndrome C: In another patient, significant ascites is present. 116 Hepatic Vascular Disorders Hepatic Infarction Rare Can be caused by hepatic artery occlusion Usually in the periphery of the Liver Typical anechoic to hyperechoic appearance over time Hepatic Vascular Disorders Portal Venous Gas Air in the portal vein and its branches Associated with: Mesenteric ischemia from small bowel obstruction Important to recognize due to significant mortality High risk population is premature and low birth weight newborns Umbilical venous catherization Bowel surgery Neonatal gastroenteritis Hepatic Vascular Disorders Portal Venous Gas Ultrasound Portal Gas Video Appearance: Multiple echogenic foci moving in the direction of blood flow Usually without shadowing or reverb artifact Portal venous gas A B A: Transverse image of the liver demonstrates multiple echogenic foci without acoustic shadowing or reverberation associated with the portal venous branches. B: Magnified view of the portal vein (arrows) showed real-time mobile foci compatible with portal venous gas. 120 Review Which mass is caused by AV malformation forming blood- filled spaces? A. Budd–Chiari B. Abscess C. Cirrhosis D. Hemangioma 121 Review Which condition presents with hepatomegaly, an echogenic intraluminal clot, the absence of hepatic vein flow, and ascites? A. Budd–Chiari B. Portal vein thrombosis C. Hepatocellular carcinoma D. Hemangioma 122 Gallbladder and Biliary System Overview Gallbladder disease is uncommon in children, but does occur Gallbladder has the same appearance as in an adult: Thin walled Well-defined Anechoic lumen Echogenic walls Gallbladder and Biliary System Congenital Anomalies: Biliary atresia Choledocal cyst Gallbladder ectopia Agenesis Duplication  Congenital Anomalies Biliary Atresia Signs and symptoms: Elevated conjugated bilirubin Jaundice at 3 to 4 weeks of age Early identification improves clinical outcomes Requires surgical intervention Liver transplant or Kasai procedure (creates communication between liver and duodenum)  Congenital Anomalies Biliary Atresia Can range from total absence of the biliary system or visible gallbladder, cystic duct, common bile duct, hepatic bile ducts Most commonly intrahepatic and extrahepatic bile ducts near the porta hepatis are absent If a rudimentary gallbladder is seen, extended fasting (up to 5 hours) can be used to prove lack of further distention A fasting measurement of less than 1.5cm suggests atresia Small Gallbladder in Neonate  Congenital Anomalies Biliary Atresia Associated findings: The liver is enlarged and hyperechoic Microcysts in the porta hepatis may be seen Increased hepatic artery diameter (>2mm) Splenomegaly (>6mm) or polysplenia Patients may have other findings such as abnormal vessel origins or situs anomalies Do not confuse a choledochal cyst for a normal gallbladder Microcyst at the Porta Hepatis Absent gallbladder may be the only sonographic sign of atresia Congenital Anomalies Biliary Atresia "Triangular cord sign" Echogenic tubular focus Found near the anterior branch of the right portal vein Measures >4mm in thickness Important sonographic finding for biliary atresia Triangular Cord Sign Congenital Anomalies Choledochal Cyst Congenital dilation of the common bile duct Presents as: Abdominal pain Mass Jaundice Choledochal Cyst Congenital Anomalies Choledochal Cyst There are five main types: Type I Fusiform dilation of the common bile duct Most common form found in infants and children Choledochal Cyst Type I Congenital Anomalies Choledochal Cyst There are five main types: Type I Fluid-filled well- defined mass in the porta hepatis adjacent to the gallbladder The right, left, and common bile ducts may be seen entering the cyst Cyst may contain Choledochal Cyst Type I sludge Congenital Anomalies Choledochal Cyst There are five main types: Type II Diverticulum of the common bile duct Second most common Appears as one or more fluid-filled structures coming off the common bile duct Choledochal Cyst Type II Congenital Anomalies Choledochal Cyst There are five main types: Type III Choledochocele Cystic dilation of the intraduodenal portion of the common bile duct Choledochal Cyst Type III Congenital Anomalies Choledochal Cyst There are five main types: Type IV Concentric dilations of the common bile duct with extrahepatic ductal dilation Choledochal Cyst Type IV Congenital Anomalies Choledochal Cyst There are five main types: Type V Caroli disease Peripheral hepatic ducts are affected Choledochal Cyst Type V Congenital Anomalies Choledochal Cyst Complications if untreated: Stone formation in the cyst, gallbladder, or pancreas Chronic biliary obstruction Chronic cholangitis Cirrhosis Biliary rupture/peritonitis Neoplasia Pancreatitis Stones in a Choledochal Cyst Abnormal Gallbladder Size Small gallbladder after fasting: Biliary atresia Congenital hypoplasia AVH Cystic fibrosis Chronic cholecystitis (uncommon in children) Small gallbladder Abnormal Gallbladder Size Large gallbladder: Prolonged fasting Hydrops Cystic or common bile duct obstruction Scan after a fatty meal to observe if the gallbladder empties. Cystic duct is patent if it empties Large Gallbladder Abnormal Gallbladder Size Non-visualization could be due to: Biliary atresia Viral hepatitis Agenesis Ectopia Sludge Normal contracted state Sludge filled GB Gallbladder Wall Thickening Non-specific finding Normal wall measurement in children is 2 mm 2mm to 5mm(or more) indicates disease Diffuse wall thickening has numerous causes including: Cholecystitis Congestive Heart Failure Renal Disease Etc Focal wall thickening is associated with: Cholecystitis Adenomyomatosis Cholecystitis Cholelithiasis Incidence is children is rising due to childhood obesity Any treatment that suppresses bile acid formation in children greatly increases their risk of stones Pigmented stones are more common in children  Cholelithiasis Most children with stones are predisposed due to an underlying disease Children with sickle cell have double the risk for developing. The formation of sludge is associated with prolonged fasting, hyperalimentation, and extrahepatic bile duct obstruction Children with sickle cell disease and Sludge in the cystic fibrosis are predisposed to the GB formation of sludge Cholelithiasis Clinical Presentation: Young children- Jaundice, irritability Older children – Classic symptoms as in adults The most common complication is pancreatitis Childhood gallstones often resolve without treatment Cholelithiasis Neonates: May be examined because of gallstones on a fetal ultrasound Most resolve spontaneously Fetal Gallstones Cholelithiasis Mirizzi Syndrome: Cystic duct stone that compresses the adjacent common bile duct Causes bile duct dilation and jaundice Cholecystitis Patients present with: RUQ pain Fever Vomiting Palpable mass in the RUQ Differentials include: Appendicitis RUQ abscess Pancreatitis Cholecystitis Gallbladder torsion Cholecystitis Remember, on ultrasound: The wall will be thickened wall (>2mm in children) Irregular Highly reflective A hypoechoic halo will surround the gallbladder Sludge is sometimes seen Cholecystitis Hydropic Gallbladder Develops in children: That are acutely ill Receive total parineal nutrition Hyperalimentation therapy In association with: Group B Strep CHF Shock Chronic biliary obstruction Kawasaki disease Etc. Hydropic Gallbladder Hydropic Gallbladder Patient presents with: RUQ pain Fever Dehydration Abdominal distention Hydropic gallbladder usually resolves spontaneously Hydropic Gallbladder Hydropic Gallbladder On ultrasound: The gallbladder is dramatically enlarged Anechoic with thin walls Gallstones/sludge may be present Will not contract well after a fatty meal Hydropic Gallbladder Biliary Obstruction Intrahepatic and extrahepatic ducts can be obstructed due to: Neoplasm Enlarged lymph nodes in the porta hepatis Acute pancreatitis Biliary calculi Biliary stricture (uncommon) Obstructed Bile Ducts Biliary Obstruction Patients present with: Jaundice Ultrasound Appearance Intrahepatic Multiple, anechoic irregular branching structures Larest at the porta hepatis Extrahepatic Round, tubular anechoic structures Found at the porta hepatis and pancreatic head Obstructed Bile Ducts Biliary Obstruction The sonographer should search for the point of obstruction Demonstrate the presence of a mass or calculus causing the obstruction Pancreatitis – the duct will taper significantly at the pancreas head If caused by a mass/stone - abrupt change from a dilated to narrow or absent duct Obstructed Bile Ducts Biliary Obstruction Dilated bile ducts may spontaneously causing: Neonatal jaundice Bile ascites Biloma Most common place for rupture is at the junction the cystic and common bile ducts Biloma Biliary Obstruction Patients usually present In the first three months of life Ascites Mild jaundice Failure to thrive Abdominal distention Elevated bilirubin values All other labs normal Helps distinguish obstruction from hepatis syndrome or Biloma biliary atresia Biliary Obstruction Bile plug Syndrome: Causes obstruct the bile ducts Liver abnormalities are not present in this syndrome Affects full-term newborns Risk factors include: Massive hemolysis TPN Hirschsprung disease Cystic fibrosis Bile Plug Intestinal atresias Biliary Obstruction Bile plug Syndrome: Ultrasound Appearance: Echogenic material found in dilate ducts Sludge in the gallbladder Bleeding into the ducts from trauma/surgery Bile Plug can mimic it  Sclerosing Cholangitis A chronic disease that obliterates the intrahepatic and extrahepatic bile ducts Leads to: Biliary cirrhosis Portal hypertension Liver failure 70% to 80% of children also have inflammatory Hyperechoic bile duct bowel disease Sclerosing Cholangitis Clinical presentation: RUQ pain Jaundice Abnormal liver function test Elevated bilirubin Sonographic Appearance Thickened ductal walls Choledocholithiasis Cholelithiasis Bile Plug Ductal strictures  Aids-Related Cholangitis In children with AIDS, the most common biliary tract finding is: Calculus cholecystitis Cholangitis Similar appearance and presentation as Sclerosing type Edema at the ampulla of Vater may demonstrate at hyperechoic nodule  Biliary Neoplasm Rhabdomyosarcoma Soft tissue tumor Rare Occurs between 1 and 5 years of age 2nd most common cause of obstructive jaundice in children/neonates Rhabdomyosarcoma  Biliary Neoplasm Clinical signs: Increasing abdominal girth Vomiting Pain Weight loss Elevated total serum bilirubin Lab Values Markedly increased ALP Normal of mildly elevated ALT Increased WBC (due to subsequent cholangitis Rhabdomyosarcoma May not have an elevated AFP  Biliary Neoplasm Ultrasound appearance: Predominately solid Hyperechoic formations Focal areas of necrosis/hemorrhage No posterior shadows Stones within (sometimes) Lobulated appearance Usually at the hilum of the Liver Dilated bile ducts surround the mass Frequently misdiagnosed as a Rhabdomyosarcoma choledochal cyst  Biliary Neoplasm Treatment: Surgery Chemotherapy Radiation Rhabdomyosarcoma Pediatric Pancreas Pancreas Sonography is currently the diagnostic procedure of choice for the examination of children with symptoms of pancreatic disease. Real-time sonography of the pancreas in infants and children is easily performed. Compared to adults, the pancreas is more easily seen in children because most are lean and have a large left hepatic lobe, which serves as an excellent window for visualizing the pancreas. Drawbacks of pancreatic sonography include Technically unsatisfactory studies due to obesity or excessive bowel gas Limited scanning surfaces when surgical dressings or ostomy sites are present 167 Pancreas Sonographic Examination Technique Scan Technique Standard examination of the pancreas includes transverse and longitudinal scans of the supine patient. Transverse scans may require some initial survey to determine the exact position of the gland because it generally lies oblique in the middle portion of the body, with the head lower than the body and tail. Longitudinal scans should be oriented to the true longitudinal axis of the pancreas, as determined by the transverse scans. Common to examine the patient in different positions (i.e., upright, decubitus) to adequately visualize the pancreas Important in the presence of disease because the scan must demonstrate the lesion’s relationship to surrounding pancreatic structure and adjacent organs 168 Pancreas Sonographic Examination Technique Scan Technique Another helpful technique is to use the water-filled duodenum to outline the pancreas. Patient is given approximately 16 oz of water, and the progress of the water into the duodenum is checked. When the duodenum is appropriately distended, the patient is repositioned until the water-filled duodenum outlines the area of the pancreas that is of interest. Fluid-filled stomach may also be helpful to outline the pancreatic tail. 169 Pancreas Sonographic Examination Technique Scan Technique This technique, however, has several drawbacks Many patients suffer severe nausea, and large amounts of water may induce vomiting Fluid filling is contraindicated for fasting patients receiving intravenous fluid Method can be time-consuming 170 Pancreas Sonographic Examination Technique Technical Considerations During pancreatic sonography, gain control is usually at settings comparable to those used for scanning the liver. Determination of the normal pancreatic sonographic pattern is based on a comparison to the liver parenchymal pattern. In children, normal pancreatic parenchyma is relatively homogeneous, with even high- and medium-level echo distribution. In contrast to the irregular echotexture, or “cobblestone” appearance, considered normal for adults 171 Pancreas Sonographic Examination Technique Technical Considerations Normal pancreas is similar to or more echogenic than the liver parenchyma Vascular structures abound in this area and should have a clearly echo- free pattern and not filled in by too high a gain setting Transducer frequency selections should be made to obtain the highest resolution with adequate penetration 172 Pancreas Normal Anatomy Maximum AP diameters of the head, body, and tail are measured in transverse. Entire gland can usually be seen in one image if it is oriented transversely across the abdomen. Oblique lie may require additional images. Pancreatic duct is not always seen in normal patients. Appears as a single echogenic line less than 1 mm located in the pancreatic body in a plane cephalad to the splenic vein 173 Pancreas Developmental and Congenital Anomalies Very small pancreas (head only) is due to agenesis of the dorsal pancreas during the embryonic stage and is associated with polysplenia. Annular pancreas is the result of a bifid pancreatic head, which encases the duodenum. Anomaly is associated with duodenal atresia or stenosis 174 Pancreas Developmental and Congenital Anomalies Cystic Fibrosis Recessively inherited disease with a prevalence in the United States 1 in 3,500 in Caucasians 1 in 17,000 in African Americans Affects the exocrine glands in the lungs and GI tract, which produce abnormal highly viscous mucus Small pancreatic ductules are obstructed by mucoid secretions, leading to pancreatic exocrine dysfunction. Tissue destruction and atrophy occur with eventual replacement of the pancreatic tissue with fibrosis and fat Normal function is compromised and pancreatic insufficiency results 175 Pancreas Developmental and Congenital Anomalies Cystic Fibrosis Hyperechoic pancreas Normal tissue is replaced by fibrosis and fatty tissue Sonographically Apparent cysts may be found. Intraluminal calcifications can be found Cystic Fibrosis Pancreas 176 Pancreas Developmental and Congenital Anomalies Congenital Cysts von Hippel–Lindau disease and autosomal polycystic disease are autosomal dominant disorders that can cause cysts in the pancreas as well as other organs (liver, kidney, adrenal glands, and spleen). Patients with von Hippel–Lindau disease also present with cerebellar, medullary, and spinal hemangioblastomas and pheochromocytomas 177 Pancreas Pancreatic Neoplasms Pancreatic Carcinoma Pancreatic carcinoma is a nonfunctioning tumor Tumor is often large when discovered and may metastases to Liver Lymph nodes Lung Appears as a localized, hypoechoic mass Focal enlargement is common Lesions less than 2 cm are difficult to detect 178 Pancreas Pancreatic Neoplasms Islet Cell Tumors Approximately two-thirds of islet cell tumors are functional. Producing a hormone that provokes the clinical suspicion of tumor early in the course of disease Most islet cell tumors are small when first detected. Diagnosis is usually made by analyzing serum hormone levels. Diagnostic imaging is used to localize rather than to diagnose. Remaining one-third of islet cell tumors are nonfunctional. Owing to a lack of hormone secretion, they remain silent until they grow large enough to produce a palpable mass that obstructs the biliary system or the GI tract. Sonographically, islet cell tumor is a well-circumscribed, anechoic mass. 179 Pancreas Pancreatic Neoplasms Insulinoma Round, firm, and encapsulated, and located in the body or tail (75% of the time) Patients present with hypoglycemia. Appear as small hypoechoic well-circumscribed masses Intraoperative sonography is most accurate for visualization and localization of masses. 180 Pancreas Pancreatic Neoplasms Lymphoma Rare neoplasm Involvement of the pancreas in a lymphatic process is usually secondary to primary lymph node disease. Diagnosis should be considered when A pancreatic mass develops in a patient with disseminated lymphoma, biopsy of a pancreatic mass reveals lymphocytes without evidence of carcinoma, a pancreatic mass is associated with chylous ascites. Focal infiltration of the pancreas in lymphoma appears as a large, solitary, hypoechoic lesion. 181 Pancreas Pancreatitis Significantly less common in children than in adults but is not all that rare Pediatric pancreatitis may have a variety of causes Trauma Blunt abdominal trauma secondary to childhood accident, motor vehicle accidents, and child abuse Most common cause of pancreatitis is blunt abuse trauma. Infection Usually viral, such as mumps or mononucleosis Toxicity Secondary to drugs such as prednisone and L-asparaginase Heredity An autosomal dominant disorder beginning in childhood) Idiopathic 182 Pancreas Pancreatitis Acute Pancreatitis Inflammation causes the escape of pancreatic enzymes from the acinar cells into surrounding tissues. Can usually be diagnosed with combined clinical and laboratory information without requiring pancreatic imaging Diagnostic imaging may be necessary when a wide variety of clinical symptoms, possible causes, and complications cause confusion. Nausea and vomiting are common and may precede or follow the onset of abdominal pain. Other frequent physical findings include fever, tachycardia, abdominal distention due to ileus, and abdominal tenderness. 183 Pancreas Pancreatitis Acute Pancreatitis Primary sonographic appearance Enlarged, edematous gland that is less echogenic than the liver parenchyma Characteristics are most obvious during the first hours after an acute attack. Dilated pancreatic duct is another indication of pancreatitis. Pancreatic ductal diameter of 1.5 mm should be considered abnormal. 184 Pancreas A B Acute pancreatitis. A: The pancreas is enlarged in size and heterogeneous in echotexture, consistent with acute pancreatitis. B: In another patient, there is a markedly dilated pancreatic duct at 0.9 cm. 185 Pancreas Pancreatitis Complications of Acute Pancreatitis Pseudocysts Causes pain or bowel/biliary obstruction and can become infected Often located in or adjacent to the pancreas. May be single or multiple and many resolve within 4 to 12 weeks Children’s pseudocysts are less likely to recur following sonography-guided drainage. Usually anechoic, with a sharp back wall and increased through transmission May contain internal echoes emanating from pus and cellular debris 186 Pancreas A B Pancreatic pseudocyst (PC). A 17-year-old male with type 2 diabetes mellitus, fatty liver, hyperlipidemia, and pancreatitis ×2 presents with 3 days of abdominal pain. A: Large left-sided pancreatic PC, medial to the spleen (S). B: A septated pancreatic PC replaces the distal body and tail of the pancreas, measuring approximately 11 cm × 8 cm × 10 cm on computed tomography. 187 Pancreas Pancreatitis Complications of Acute Pancreatitis Hemorrhage Results from disruption of one or more pancreatic blood vessels Potentially lethal and may produce a large pancreatic hematoma Appears as an inhomogeneous mass Initially, acute hemorrhage into the pancreas may appear anechoic. It becomes moderately echogenic as organization occurs. 188 Pancreas Pancreatitis Complications of Acute Pancreatitis Phlegmon Solid inflammatory mass composed of necrotic tissue mixed with inflammatory exudate and tissue edema. May resolve spontaneously whenever the necrotic process is not progressive In more severe episodes, a necrotizing process may predominate and provoke further complications. Patient is at risk for developing a pancreatic abscess by bacterial seeding into necrotic tissue. Appears as an anechoic mass in the pancreatic bed 189 Pancreas Pancreatic phlegmon with splenic artery aneurysm/pseudoaneurys m. A: Large phlegmon in the left upper quadrant extending to the splenic hilum consistent with necrotizing pancreatitis of the distal body and tail with surrounding phlegmon (cursors). 190 Pancreas B C B: There is a focal 1.4 cm × 1.7 cm dilatation of the splenic artery (arrow) at the superior aspect of the phlegmon with pulsatile swirling blood products (C). 191 Pancreas Pancreatitis Complications of Acute Pancreatitis Abscess Formation likely in severe cases of pancreatitis with extensive necrosis Marked by spiking fevers, chills, and a recurrence of abdominal pain 10 to 14 days after the initial episode, drainage is required in all cases Appears as a large anechoic mass in the pancreatic bed, and the appearance varies with the amount of suppurative material and debris Walls are usually thick, irregular, and echogenic. When air bubbles are present, the pancreatic area appears highly echogenic with occasional shadowing. 192 Pancreas Pancreatitis Chronic Pancreatitis Clinical condition caused by repeated attacks of acute pancreatitis Causes fibrosis and destruction of pancreatic cells Majority of the childhood cases of chronic relapsing pancreatitis are hereditary. Likelihood that patients with “hereditary pancreatitis” will develop pancreatic carcinoma is increased, particularly for those with pancreatic calcifications. 193 Pancreas Pancreatitis Chronic Pancreatitis Sonographically In early stages of chronic pancreatitis, the pancreas is less echogenic than the normal liver parenchyma. Appearance is similar to that of acute pancreatitis. May be dilatation of the main pancreatic duct or common bile duct Advanced stages Pancreas shrinks, develops irregular borders, more echogenic than usual. Calcifications and dilated ducts may also be present. May be so pronounced and cause so much acoustic shadowing that pancreatic identification is difficult 194 Review Which describes the normal pancreatic parenchyma in children? A. Homogeneous echo distribution B. Irregular echotexture C. Less echogenic than the liver D. Echogenic vascular structures 195 Review Which pathology is inherited and characterized by the production of highly viscous mucus? A. Congenital cysts B. Cystic fibrosis C. Islet cell tumors D. Lymphoma 196 Review Which solid inflammatory mass is composed of necrotic tissue mixed with inflammatory exudate and tissue edema? A. Phlegmon B. Hemorrhage C. Insulinoma D. Cystic fibrosis 197 Pediatric Spleen Spleen Most common indications for imaging the pediatric spleen are trauma and evaluating for congenital anomalies, such as polysplenia or asplenia Spleen size is most often determined by measuring the length in a longitudinal, coronal plane from the dome to the inferior margin Normal spleen size (based on length) has been established by age and weight As a general guideline, inferior margin of the spleen should not extend past the lower pole of the left kidney 199 Spleen Most common indications for imaging the pediatric spleen are: Trauma Evaluating for congenital anomalies (polysplenia or asplenia) Spleen size Determined by measuring the length in a longitudinal, coronal plane from the dome to the inferior margin Normal spleen size (based on length) has been established by age and weight As a general guideline, inferior margin of the spleen should not extend past the lower pole of the left kidney 200 Spleen 201 Spleen Developmental and Congenital Anomalies Numerous syndromes and conditions are associated with asplenia and polysplenia Important to differentiate between asplenia and polysplenia Associated with significant differences in anomalous systemic venous connections and tracheobronchial anomalies Abnormal renal vein confluence is frequently observed in asplenia Congenital heart disease is typically more complex when associated with asplenia than polysplenia 202 Spleen Developmental and Congenital Anomalies Asplenia When evaluating for suspected asplenia: Identify the vascular hilum of the spleen to differentiate it from the left lobe of the liver Evaluate the entire abdomen for splenic tissue aka “wandering spleen” Polysplenia Multiple splenules without a parent spleen Typically located on the left side May be bilateral Most common anomaly affiliated with polysplenia is an interrupted IVC with azygous or hemiazygous continuation 203 Spleen Splenomegaly Enlargement of the spleen Associated with: Sickle cell disease Portal hypertension Lymphoma Infectious mononucleosis Cardiac disease Infection Abscess Fungal microabscess ECMO Sepsis 204 Spleen Splenomegaly Splenic cysts may be a cause for enlargement or palpable mass in the LUQ Congenital cysts are considered “primary” and have an epithelial lining Acquired or “secondary” cysts do not have a lining Are also known as pseudocysts In children, are typically the result of prior trauma or affiliated with severe pancreatitis 205 Spleen A B Splenic cyst. A: Large 18-cm cystic structure within the spleen (S) in a 14-year-old male. B: Rupture of preexisting very large splenic cystic lesion in the setting of blunt abdominal trauma in the same patient. Sonography now demonstrates a large multiloculated cyst within the S. 206 Spleen Infection Results in abscesses associated with a variety of processes, including trauma, sickle cell disease, bacterial and fungal organisms, infarction, and pancreatitis More common in immunocompromised children Fungal microabscesses should be suspected in children who are: Neutropenic with fever Have abdominal pain Spleen is enlarged 207 Spleen Infection Appearance of fungal infection is multiple “bull’s-eye” or “wheel within a wheel” lesions High-frequency linear transducer should be used to visualize these microabscesses, which can be less than 2 cm in diameter Infection with Bartonella henselae can result from being scratched by a cat or kitten Microabscesses are characterized as hypoechoic on ultrasound, may calcify, and often take months to resolve 208 Spleen Tumors Lymphoma Most common splenic malignant mass Masses appear as hypoechoic, solitary, or multiple and may or may not have associated splenomegaly Enlargement of retroperitoneal lymph nodes can help with diagnosis Patients with leukemia may present with diffuse splenomegaly 209 Spleen Tumors Hamartomas Most common primary neoplasm of the spleen in children, although rare and benign Sonographically, they are hypoechoic, avascular, and solid but may have a cystic component Hemangiomas Variable appearance May occur as solitary lesions or in association with a syndrome, such as Beckwith–Wiedemann or Klippel–Trenaunay–Weber 210 Spleen Tumors Lymphangioma, leiomyoma, and angiosarcoma Uncommon in children and have a sonographic depiction similar to that of the adult population Metastasis Although rarely present in childhood, most often related to neuroblastoma 211 Spleen Sickle Cell Anemia Patients develop acute splenomegaly secondary to congestion of red pulp, causing intense pain Acute splenic sequestration can be life-threatening Do to rapid drop in hematocrit and hypotensiveness Sonographically, the spleen is very large with hyperechoic and hypoechoic areas as a result of autoinfarction In older children with sickle cell disease, the spleen becomes small and fibrotic from chronic autoinfarction and may calcify 212 Spleen A B Sickle cell disease. A: Acute splenic sequestration. The spleen is moderately enlarged in size and demonstrates diffusely heterogeneous echotexture. The spleen measures 16.3 cm in maximum length and 7.6 cm in maximum width. Splenic parenchyma is diffusely heterogeneous with a mottled appearance characterized by innumerable tiny hypoechoic areas interspersed in the parenchyma. There is a relatively more hypoechoic and peripherally located area up to 4 cm × 3.5 cm × 3 cm size in the interpolar region, without any intrinsic vascularity (arrows). B: Atrophic echogenic spleen in a 14-year-old female with sickle cell disease. 213 Spleen Gaucher Disease Most common lysosomal storage disorder Predominantly affects those with Ashkenazi Jewish heritage Hepatosplenomegaly and bone marrow involvement are hallmarks of the most common (nonneuropathic) form of this disease Contribute to anemia and thrombocytopenia Spleen is: Markedly enlarged compared to the liver Echogenic May have small areas of hypoechogenicity or hyperechoic foci Extent of splenic fibrosis and infarction is best depicted on MRI. 214 Review Which malignancy is associated with enlarged retroperitoneal lymph nodes and splenomegaly caused by multiple hypoechoic splenic masses? A. Hemangioma B. Gaucher disease C. Lymphoma D. Hamartoma 215 Review Which pathology results from a lysosomal storage disorder? A. Sickle cell anemia B. Gaucher disease C. Lymphoma D. Polysplenia 216 Pediatric Retroperitoneum Retroperitoneum Sonographic evaluation of the retroperitoneum is performed primarily to examine the kidneys, ureters, and urinary bladder Retroperitoneum also includes the muscles (psoas, quadratus lumborum), the crura of the diaphragm, and lymph nodes. Retroperitoneal hemorrhage, retroperitoneal fibrosis (rare in children), and presacral tumors can be evaluated with sonography. 218 Retroperitoneum Muscles Retroperitoneal muscles may become involved in diseases that originate in the lymph nodes, kidneys, pancreas, duodenum, colon, and spinal column. Abscess can occur from bacteremia or adjacent inflammatory conditions; extension of lymphoma, Wilms tumor, Ewing sarcoma; and rhabdomyosarcoma originating in the muscle. Psoas muscle is a common location for abscess or changes related to neoplasm as well as hematoma in the hemophiliac patient. Correlation with clinical history is important. 219 Retroperitoneum Lymph Nodes When multiple or large retroperitoneal lymph nodes are found during abdominal sonography of an infant or child, they are abnormal and are generally located near the aorta and IVC. Enlargement nodes are most commonly associated with lymphoma, Wilms tumor, and neuroblastoma. Appears as one or more hypoechoic homogeneous structures that may combine to appear as one large mass Enlarged nodes can displace vessels and bowel anteriorly and the kidneys laterally. 220 Retroperitoneum A B Lymphadenopathy. Transverse (A) and longitudinal (B) images demonstrate multiple hypoechoic lymph nodes (N) seen anterior to the aorta (Ao), adjacent to the pancreatic head (P) and porta hepatis. IVC, inferior vena cava. 221 Retroperitoneum Neoplasms Arise from lymph channels, nerves, and connective tissues Benign tumors Teratoma, hemangiomas, lipomatosis, lymphangioma, and neural tumors More likely hyperechoic with mature teratomas having a complex appearance Malignant tumors Rhabdomyosarcoma, fibrosarcoma, neuroblastoma, leiomyosarcoma, malignant germ cell tumor, malignant schwannoma, and Ewing sarcoma Usually solid with variable echogenicity and complexity due to hemorrhage or necrosis 222 Retroperitoneum A Retroperitoneal tumor in an ex-FT male born with large thoracolumbar mass. A: Ultrasound demonstrates a complex, hypervascular (B) structure to the right of midline. B 223 Retroperitoneum C: Large right-sided paraspinal soft-tissue tumor, invading the spinal canal from the lower T9 to S1, with severe cord compression. A diagnosis of fibrosarcoma was made by biopsy. 224 Retroperitoneum Neoplasms Presacral masses in children include Sacrococcygeal teratoma (the most common) Neuroblastoma Soft-tissue sarcomas Lymphoma Lipoma Anterior meningocele Sacral bone tumors Abscess Rectal duplication 225 Retroperitoneum Neoplasms Sacrococcygeal teratoma Type I is predominately external. Type II is external with significant internal components. Type III is predominately internal. Type IV is entirely presacral without external component or extension. Benign teratoma is mostly cystic with varied amounts of solid components. Malignant teratomas are mostly solid but may contain cystic areas. Hydronephrosis or urine ascites from bladder rupture may be found. In most cases, CT and/or MRI are needed for a more accurate preoperative assessment. 226 Retroperitoneum Trauma Hemorrhage from renal trauma first surrounds the kidney before extending into the retroperitoneal space Sonography can detect the presence of fresh hemorrhage (anechoic) and follow the progress of the hemorrhage as it ages and changes in echogenicity 227 Retroperitoneum Trauma Sonography has become one of the most important imaging modalities used for the evaluation of the pediatric urinary tract and adrenal glands. Unlike excretory urography and CT, sonography does not expose the child to ionizing radiation and does not carry the risk of a life-threatening anaphylactic contrast reaction Sonography can be performed portably, without sedation, and may be used for serial follow-up examinations Examination cost is typically lower than the abovementioned modalities, and the ultrasound examinations are usually tolerated well by pediatric patients 228 Retroperitoneum Trauma One of the most common ultrasound examinations ordered for the pediatric patient is the renal ultrasound Usually due to a suspected renal anomaly, which could be associated with abnormalities found in other body systems Findings in prenatal sonography may also raise the question of renal anomalies For older infants and younger children, the most common reasons for ultrasound imaging referral are a workup for a urinary tract infection, enuresis, urgency, dysuria, and urinary reflux Less common clinical indications of this examination include suspected mass palpation, unexplained hypertension, tuberous sclerosis, hemihyperplasia, prerenal or postrenal transplant evaluation, multicystic kidneys, polycystic kidneys, Prune Belly syndrome, and the need for renal biopsy guidance 229 Review Where are abnormal retroperitoneal lymph nodes commonly located? A. Surrounding the pancreatic head B. Near the aorta and IVC C. Behind the sternum D. Adhered to the sacrum 230 Review When blunt trauma to the kidney occurs, where is blood first accumulated? A. Surrounding the kidney B. Within the retroperitoneal space C. Within the pericolic gutter D. Above the diaphragm 231 Pediatric GI Tract Gastrointestinal Tract Sonography of the GI tract is readily performed in infants and children using a high-frequency linear transducer. Graded compression is utilized to displace bowel gas and permit visualization of the underlying structures. Hypertrophic pyloric stenosis (HPS), intussusception, and acute appendicitis are primarily evaluated using sonography. 233 Gastrointestinal Tract Stomach Normal gastric wall, including the mucosa and muscularis muscle layer, measures from 2.5 to 3.5 mm. Gastric wall’s thickness can be assessed with the patient lying in the supine and RLD positions before and after ingestion of fluid. Child should fast for preparation. 234 Gastrointestinal Tract Stomach Wall Thickening Stomach has an echogenic submucosa well seen with a fluid-filled stomach and an outer hypoechoic rim of muscle. Measurements of thick wall should remain unchanged when water is ingested. Mucosa is thickened and echogenic, especially in the fundus and antrum. Variety of abnormalities have been reported to cause gastric wall thickening of 5 to 15 mm. Ménétrier disease or transient protein losing gastropathy displays hypertrophy of the mucosa. 235 Gastrointestinal Tract Stomach Wall Thickening In children with gastric ulcer disease, the thickening occurs in the antropyloric mucosa. Moderate or generalized thickening up to 5 mm was present with lymphoid hyperplasia, varioliform gastritis, and Crohn disease involving the stomach. Greatest amount of thickening (up to 10 mm) occurs in children with chronic granulomatous disease. 236 Gastrointestinal Tract A B Figure 20-36 A and B Stomach. A: In this image of a normal neonatal stomach filled with fluid, the hyperechoic submucosa can be clearly seen surrounded by the hypoechoic muscle layer. B: This image of the stomach demonstrates thickened submucosal and muscular layers. 237 Gastrointestinal Tract Figure 20-36 C C: This transverse image of the stomach demonstrates a markedly thickened hypoechoic muscular layer. (Images courtesy of Phillip Medical Systems. Bothell, WA.) 238 Gastrointestinal Tract Stomach Pyloric Stenosis Often affects first-born male infants between 2 and 10 weeks of age with most patients presenting at 1 to 2 months of age Caused by abnormal thickening of the antropyloric region of the stomach Patients present with dehydration and frequent episodes of projectile nonbilious vomiting. Sonography provides direct visualization of the pyloric muscle and lack of passage of fluid through the pylorus. With pyloric stenosis, stomach is often not empty even if the patient has been fasting. 239 Gastrointestinal Tract Stomach Pyloric Stenosis Patients are examined in the supine and RPO positions. If possible, patient should be fasting for 2 hours. Transverse plane demonstrates the long axis of the pylorus, and the longitudinal plane demonstrates its transverse axis. Scans should be made in the transverse plane, descending along the lesser curvature of the stomach through the left lobe of the liver, just to the right of the midline. Antrum of the stomach appears just medial to the gallbladder in the transverse plane, and the pylorus is continuous with the stomach. 240 Gastrointestinal Tract Stomach Pyloric Stenosis If the pylorus is not well visualized, the patient may be given water to better display the gastric lumen. Pylorus should be examined periodically while doing so, as an overly full stomach can reposition the pylorus posteriorly, making it more difficult to evaluate. Images or video clips documenting fluid passing through the pyloric channel and into the duodenal bulb rules out HPS. Mass presents as a doughnut sign: An anechoic to hypoechoic muscle mass with a central lumen of increased echogenicity 241 Gastrointestinal Tract Stomach Pyloric Stenosis Diagnosis of pylor