Liver: Differential Diagnosis of Hepatic Diseases



10.1055/b-0034-87861

Liver: Differential Diagnosis of Hepatic Diseases



Diffuse Parenchymal Disease





















































Table 2.1 Nonhomogeneous liver parenchyma

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.


Fig. 2.1


Fig. 2.2


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).


Fig. 2.3


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.

Fig. 2.1 Liver cirrhosis. High-resolution transverse US of the left hepatic lobe. A heterogeneous liver parenchyma with ill-defined hyperechoic nodules is seen in this 13-year-old boy with Wilson disease and portal hypertension.
Fig. 2.2 Biliary atresia. Transverse US of the right hepatic lobe. Hepatic parenchyma is heterogeneous with hyperechoic areas related to portal spaces.
Fig. 2.3 Neuroblastoma metastases (stage 4S). T2-weighted MRI shows confined left primary adrenal neuroblastoma (N) and hepatomegaly with practically complete liver infiltration by metastases.
































Table 2.2 Homogeneous liver parenchyma

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


Fig. 2.4a, b


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).

Fig. 2.4a, b Starry-sky liver. (a) US of liver (right) and spleen (left) showing a homogeneous hypoechoic parenchyma with relatively hyperechoic portal spaces. The hypoechogenicity of liver has been related with prolonged fasting and/or vomiting. (b) Transverse image of liver.


Focal Parenchymal Abnormalities













































































Table 2.3 Solid heterogeneous lesions

Diagnosis


Findings


Comments


Hepatoblastoma


Fig. 2.5a–d


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


Fig. 2.6a–c


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


Fig. 2.7a, b


Prominent vascularization. Peripheral or heterogeneous enhancement.


Splenic or pulmonary metastases.


Very rare in children (only cases reported).


Lymphoproliferative disorders


Fig. 2.8a, b


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


Fig. 2.10a–c


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


Fig. 2.11a, b


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.

Fig. 2.5a–d Mixed hepatoblastoma. (a) Transverse US shows that calcifications (arrows) and cystic necrotic areas (arrowhead) are dominant features. Axial T2-weighted MRI (b) and T1-weighted MRI (c) show right lobe and segment 4 mass. Note areas of increased signal intensity: hemorrhage (arrow) and hypointense bands (fibrous septations; arrowheads). (d) T1-weighted manganese-enhanced MRI. A 4-year-old boy with resected segment 6 hepatoblastoma. A postoperative inferior vena cava (IVC) thrombus was detected (arrows). Manganese enhancement of the thrombus (equal to liver) confirms its tumoral nature.
Fig. 2.6a–c Undifferentiated embryonal sarcoma in a 12-year-old boy. (a) Doppler US shows avascular, heterogeneous, apparently solid mass. (b) T2-weighted MRI shows septated, heterogeneous, but predominately cystic sarcoma. (c) Gadolinium-enhanced T1-weighted MRI shows enhancing parietal nodules corresponded to solid portions (arrow).
Fig. 2.7a, b Angiosarcoma in a 9-year-old boy. (a) Transverse US shows poorly defined heterogeneous but mainly hyperechoic lesion (arrows). (b) T2-weighted MRI.
Fig. 2.8a, b Multicentric Burkitt lymphoma in a 10-year-old boy with abdominal pain due to ileocolic intussusception. (a) US and (b) coronal contrast-enhanced CT show multiple, homogeneous, lowechogenicity/attenuation nodules (arrowheads). The presence of hepatic nodules and the patient’s age serve to rule out lymphoma.
Fig. 2.9 Posttransplantation lymphoproliferative disease. Contrast-enhanced CT shows multinodular, partially enhanced hepatic lymphoma 2 years after liver transplantation in an 8-year-old girl.
Fig. 2.10a–c Multiple hemangiomas. (a) Transverse US shows multiple hypoechoic lesions, with central echoes, corresponding to vessels (arrow). (b) Doppler US shows vascular rim along the edges of the tumor, with little flow within the tumor itself. (c) Early postcontrast CT: peripheral enhancement of multiple diffusely distributed nodules.
Fig. 2.11a, b Focal nodular hyperplasia. (a) Longitudinal US shows well-defined, practically isoechoic in the right lobe of the liver (cursors). (b) Manganese-enhanced T1-weighted MRI shows lesion (arrows) enhancement (similar to the liver parenchyma) demonstrating hepatocellular origin.
Fig. 2.12a, b Pyogenic abscess in a 6-year-old with liver transplantation. (a) US: heterogeneous, encapsulated fluid collection containing gas (arrows). (b) Contrast-enhanced CT obtained during percutaneous drainage (arrow) confirmed collection was an abscess.
Fig. 2.13 Hepatosplenic candidiasis in a 3-year-old with acute lymphoblastic leukemia and neutropenia. US scan shows multiple hypoechoic hepatic nodules (arrowheads; most frequent US pattern).
Fig. 2.14 Focal fatty liver. Transverse US shows well-marginated hyperechogenicity of liver parenchyma that affects lateral right hepatic segments (arrows), with the rest of the liver preserved.
Fig. 2.15 Angiomyolipoma in a 17-year-old girl with tuberous sclerosis. T1-weighted in-phase MRI shows small hyperintense lesion in the left lateral segment (arrow).
























































Table 2.4 Cystic lesions

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.

Fig. 2.16a, b Echinococcosis. (a) Longitudinal oblique US: single lobular cystic lesion with thin wall, through transmission, and internal daughter cysts (type II) (arrow). (b) Coronal T2-weighted MRI: hyperintense cysts with internal septa (arrowhead).
Fig. 2.17a–c Ciliated foregut cyst in a 7-year-old girl. (a) Transverse US shows irregular, lobulated lesions, with fine internal echoes that increased through transmission. (b) T2-weighted MRI shows hyperin-tense lobular lesions in segment 4. (c) T1-weighted MRI demonstrates high signal intensity content within the lesion (asterisks), corresponding to mucoid material.








































Table 2.5 Liver calcifications

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.

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Jul 12, 2020 | Posted by in PEDIATRIC IMAGING | Comments Off on Liver: Differential Diagnosis of Hepatic Diseases

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