Liver: Differential Diagnosis of Hepatic Diseases
Diffuse Parenchymal Disease
Diagnosis | Findings | Comments |
Cirrhosis: Regenerative changes in biliary atresia, chronic active hepatitis, cystic fibrosis, hepatic fibrosis, Budd-Chiari syndrome, Alagille syndrome, chronic biliary obstruction—biliary cirrhosis, glycogen storage disease type IV, alpha1-antitrypsin deficiency, Wilson disease, galactosemia, tyrosinemia, etc. | Cirrhotic liver shows a small right lobe. The caudate lobe and lateral segment of the left lobe are enlarged. The margins of the liver are irregular. Hepatic parenchyma is heterogeneous because of fatty infiltration, fibrosis, and regenerative nodules. The hepatic vessels may be diffcult to see because of compression by fibrosis. Findings of portal hypertension are often seen. | Differentiation of regenerative nodules from hepatocellular carcinoma may be diffcult by imaging techniques. |
Storage diseases | Other viscera are also involved. | Glycogen storage disease, Gaucher disease, mucopolysaccharidoses, tyrosinosis, alphal-antitrypsin deficiency, Niemann–Pick disease |
Malignant diseases: multifocal or diffuse hepatocellular carcinoma, diffuse metastatic disease (neuroblastoma stage 4 and 4S). | Diffuse involvement of the liver in neuroblastoma stage 4 and 4S: “salt and pepper” pattern (good prognosis). | Compression and distortion of normal vascular anatomy. |
Acute hepatitis | Nonhomogeneous echo pattern; hepatomegaly; periportal edema with increased periportal echogenicity. | Thickening of gallbladder wall; portal lymph-adenopathy. |
Chronic granulomatous disease | Multiple, poorly defined hepatic abscesses. Lesions may resolve or calcify with treatment. | Recurrent infections of the lung, bones, lymph nodes, or liver. |
Diffuse hemangioma | Near-total replacement of hepatic parenchyma by the tumor. Below the celiac axis, the aorta has a marked decrease in caliber as a result of increased hepatic arterial flow. | In diffuse hemangiomas, cardiac failure secondary to high volume shunting, hypothyroidism, fulminant hepatic failure, abdominal compartment syndrome, and even death may occur. |
Irradiated liver | In the chronic stage (after 6 wks), the liver is typically small, contracted, and fibrotic. | |
Liver transplantation | A periportal area of low echogenicity (dilatation of lymphatic channels) is often seen after transplantation. On computed tomography (CT) peri-portal edema is seen as a central or peripheral low-attenuation area and has been called the “periportal collar” sign. | |
Chronic congestion in cardiac disease | Ultrasound (US): echogenic liver parenchyma. | The hepatic veins adjacent to the vena cava are often enlarged. |
Chemotherapy | US: enlarged liver, echogenic in comparison to the normal renal parenchyma due to steatosis. Differential diagnosis (DD): veno-occlusive disease, excluded by Doppler. | Toxic effect of chemotherapy. |
Diagnosis | Findings | Comments |
Diffuse fatty infiltration | US: Increased parenchymal echogenicity. Appreciation and evaluation of the hepatic echotexture is often diffcult and operator-dependent. The degree of acoustic attenuation is easier evaluated when comparing the liver to the healthy kidney. An enlarged liver suggests fatty change. Further work-up: magnetic resonance imaging (MRI) in obese patients and biopsy. | Fatty liver in obesity, hyperalimentation with high fat content, hepatitis, long-term steroid therapy, Cushing disease, diabetes mellitus, severe malabsorption, protein-deficiency malnutrition, toxic and drug reactions, hyperlipidemia, familial hyperlipoproteinemia, cystic fibrosis, Reye syndrome, Wilson disease, glycogen storage disease. |
Starry sky liver | Hyperechoic portal spaces related to diffuse hypoechogenicity. | Related to fasting and/or vomiting; reverses after meals. |
Edema in acute hepatitis | Hepatomegaly and lymphatic periportal edema. Progressive gallbladder wall thickening or small amounts of clear fluid in the perihepatic space and in the gallbladder fossa. Frequently enlarged lymph nodes in the hepatic ilium. | |
Acute congestion in cardiac disease | US: hypoechoic liver parenchyma. | Dilated hepatic veins. |
Iron deposition in the liver | MRI is the best imaging modality. Marked decrease in signal intensity on T2- and T1-weighted images. | Either in the hepatocytes (primary hemochromatosis, cirrhosis) or in the reticuloendothelial cells (hemosiderosis). |
Focal Parenchymal Abnormalities
Diagnosis | Findings | Comments |
Hepatoblastoma | Large, solid mass with cystic areas (hemorrhage). Manganese-enhanced MRI may be used for increasing detection of small satellite lesions and elucidating tumoral or nontumoral thrombus. | Mean age is 0–3 y. Most common primary malignant hepatic tumor (50%). Possible venous involvement (intravascular solid material, venous encasement). Calcifications (40%). |
Hepatocellular carcinoma | US: Sometimes thin hypoechoic halo (tumor capsule) and hypoechoic areas secondary to necrosis. Rare calcifications (frequent in fibrolamellar scar). CT or MRI: central scar on fibrolamellar subtype. | Mean age is 12–14 y. Underlying liver disease (50%): tyrosinemia, biliary atresia, familial cholestasis, Alagille syndrome, glycogen storage disease type 1, chronic hepatitis. Growth patterns: solitary, multifocal, or diffuse. |
Undifferentiated embryonal carcinoma | Well delineated by a fibrotic pseudocapsule. Multi-septated cystic or inhomogeneous solid appearance. Discordance between US imaging (heterogeneous solid mass) and CT or MRI (cystic appearance due to myxomatous tissue). | Mean age is 8–12 y. Most frequent pediatric hepatic sarcoma, though uncommon (5%). |
Angiosarcoma | Prominent vascularization. Peripheral or heterogeneous enhancement. Splenic or pulmonary metastases. | Very rare in children (only cases reported). |
Lymphoproliferative disorders Fig. 2.9, p. 148 | Growth patterns: solitary, multifocal, diffuse, perihepatic, or periportal infiltration (rare but characteristic). Hepatomegaly (not necessarily due to lymphomatous infiltration). | Mean age is 2–16 y. Twelve percent of lymphomas affect the liver. Burkitt lymphoma most frequent. Posttransplantation lymphoproliferative disease: Epstein-Barr-virus–related. |
Hepatic metastases Fig. 2.3, p. 143 | Imaging: depends on some degree on the nature of the primary tumor. Typically multiple and well circumscribed masses. | Mostly Wilms tumor and neuroblastoma (neuroblastoma stage 4 and 4S). |
Hemangioma | Focal: Hepatic form of the cutaneous rapidly involuting congenital hemangioma (RICH). Fast spontaneous involution. Multiple hepatic lesions (multifocal) or near-total replacement of hepatic parenchyma (diffuse). | Neonates and infants, some diagnosed prenatally. Most common benign pediatric liver tumor (50%). In diffuse hemangiomas, cardiac failure, hypothyroidism, and fulminant hepatic failure may occur. Twenty to forty percent have skin hemangiomas. glucose transporter (GLUT)-1 positive in diffuse forms and GLUT-1 negative in focal forms. |
Nodular regenerative hyperplasia | US: multiple nodules of variable size (frequently hyper-echoic). MRI: manganese enhancement (regenerative hepatocytes uptake manganese). Absence of fibrous septa. | Associations: portal low flow conditions (Abernethy malformation, Budd-Chiari syndrome, postchemotherapy, collagen vascular diseases, hematologic disorders). |
Focal nodular hyperplasia | US: mass with echogenicity similar to the liver. Central scar in 33% of the cases. T2 with ferumoxide: lesion decreased signal except scar, manganese-enhancement. | Unusual, 5% of all benign hepatic tumors. Associations: type I and type VI glycogen storage disease, Hurler syndrome, galactosemia. |
Hepatic adenoma | Imaging depends on the attenuation of the surrounding liver. Low attenuation (fat, old hemorrhage) or high attenuation (recent hemorrhage, glycogen, surrounding fatty liver). No uptake of superparamagnetic iron oxide particles. | Associations: intake of androgens, type I glycogen storage disease, human immunodeficiency virus infection, and oral contraceptives. |
Pyogenic abscess Fig. 2.12a, b, p. 150 | Complex mass with internal debris, air-fluid, or debris-fluid levels. Increased peripheric vascularization. | Staphylococcus aureus. Presentation: abdominal pain, fever, septicemia. |
Amebic abscess | Chocolate-like content with leukocytes or amebae (may only be seen in abscess wall). Peripherally located (near or in contact to Glisson capsule). Peripheral “halo.” Doppler: no prominent peripheral vascularization (differential finding compared to pyogenic abscess). | In 3%–7% of patients with Entamoeba histolytica infection. More frequent in patients younger than 3 y. |
Hepatosplenic candidiasis Fig. 2.13, p. 150 | US: “wheel-within-a-wheel,” “bull’s eye,” or hypoechoic multiple nodules. | Candida albicans is the most frequent pathogen. In immunocompromised patients (prolonged neutropenia). |
Liver infarct | In acute phase, solid, hypoechoic lesions, mainly in the periphery of the liver. | Posttraumatic, after partial resection, or in liver transplant after an acute ischemic episode (hepatic artery stenosis or thrombosis). |
Cat-scratch disease | Granulomas either resolve or calcify. | Hepatic and splenic granulomas. |
Lipomatous lesions: focal fatty liver, lipoma, angiomyolipoma Fig. 2.14, p. 150 Fig. 2.15, p. 151 | Imaging: hyperechoic on US, homogenous low-attenuation lesion (less than —20 Hounsfield units [HU]) on CT, and high signal intensity on in-phase, drop in signal intensity on out-phase T1-weighted and iso- or hyperintense on T2-weighted MRI. | Rare in children. |
Diagnosis | Findings | Comments |
Normal variants: loops of small bowel in the porta hepatis, caudate lobe, Riedel lobe | If a loop of bowel is suspected in the porta hepatis, US must be performed. In case of doubt, CT after oral contrast administration may be necessary. | |
Echinococcal cysts Fig. 2.16a, b, p. 152 | Unilocular or multilocular well-defined cystic lesions. Calcification in cyst wall or complete calcium replacement. Internal debris (hydatid sand), daughter cysts, undulating membrane (water-lily sign). | Liver is the most commonly involved organ (75%–80%) in echinococcosis. Possible superinfection and anaphylaxis secondary to rupture. |
Ciliated hepatic foregut cysts Fig. 2.17a–c, p. 152 | Well-delineated small cyst anechoic or with fine echoes (due to mucoid content) on US and hypoattenuating or isoattenuating relative to surrounding liver parenchyma on CT. MRI: hyperintense on T2-weighted, variable on T1-weighted (due to variable content). | Solitary cyst. Congenital (embryonic foregut remnant in the liver). Often < 3 cm and located in segment 4. Rarely, malignant transformation through squamous metaplasia. |
Benign cystic tumors (multiple cysts): mesenchymal hamartoma | Multiseptated cystic mass, although a single dominant cyst may be seen. Echogenic material within the cyst fluid secondary to blood. Doppler: avascular. On MRI, signal intensity may vary depending on stromal, protein, or hemorrhage contents. | Patients under 2 y. Hamartoma is the second most common benign hepatic tumor (22%). Predominantly located in the right hepatic lobe. |
Posttraumatic hematomas or bilomas | Intrahepatic bilomas usually present on US or CT as intrahepatic peripheral cystic lesions that communicate with the bile duct. | Image-guided aspiration of a fluid collection can be necessary to determine composition. |
Mucocele | On US, mucoceles appear as cystic masses near the porta hepatis. These abnormalities are readily seen as cystic structures on MR cholangiography. | Cystic duct remnant mucocele is an uncommon complication of liver transplant that occurs when the donor cystic duct remnant becomes distended with mucus. |
Post-Kasai procedure (hepatojejunostomy) | The jejunal loop is anastomosed with the bile ducts in the porta hepatis. The anastomosis may appear cystic. | |
Choledochal cysts | Hepatobiliary. US and radionuclide studies usually suggest the correct diagnosis, which can be confirmed by MR cholangiography. Frequently, the intrahepatic ducts are normal. Sludge or stones may be identified within the dilated ducts. | The most common form (80%–90% of cases) is Todani type I (dilatation of the common bile duct). A characteristic triad of abdominal pain, obstructive jaundice, and fever is only seen in a minority of patients. |
Caroli disease | Recognition of the connection of the ectatic ducts with one another and with the rest of the ductal system is critical in distinguishing Caroli disease from polycystic liver disease. Central dot sign. | Represents segmental or diffuse nonobstructive dilatation of the intrahepatic ducts. |
Polycystic liver disease | Hepatobiliary cysts may be intrahepatic or peribiliary. Occasionally, echogenic debris can be seen if hemorrhage has occurred. | |
Posttraumatic cysts and posthepatic infarct cysts (liver transplant) | In late phase posthepatic infarct, cystic image is similar to posttraumatic or echinococcal cysts, mainly in the periphery of the liver. Usually after hepatic artery stenosis or thrombosis in transplanted liver. | In posttraumatic cysts, the clinical history of trauma is the diagnostic key. |
Diagnosis | Findings | Comments |
Metastases after chemotherapy | Neuroblastoma lesions may have calcifications, especially postchemotherapy. | |
Hepatic tumors: hepatoblastoma, hepatocellular carcinoma, hemangiomas, focal nodular hyperplasia | Hepatoblastoma: more evident in mixed type after chemotherapy. Hepatocellular carcinoma: calcifications frequent in fibrolamellar scar. Hemangiomas: calcifications with involution. Nodular regenerative hyperplasia: calcification is seen in only 1% of patients. | |
Granulomas | After acute disease, abscesses finally resolve into a calcified granuloma. | In infectious diseases. |
Echinococcal cysts | Calcification in cyst wall or complete calcium replacement. | |
Postinfarct calcifications | After partial hepatic resection or in liver transplant after an ischemic episode (hepatic artery thrombosis or stenosis). The larger areas of infarction may occasionally calcify. | |
Portal venous system or umbilical veins | In preterm infants. | |
Calcified hematoma (sequela of biopsy or trauma) | Calcification in the site of biopsy. |