Regarding liver cysts, which of the following is false?
They are 3 times more common in females.
Occur more frequently in the African American population.
Account for 1/3 of all hepatic neoplasms in children
Rarely communicate with the biliary tree.
True or False? Lymphangiomas of the liver are classified as vascular lesions, rather than lymphatic malformations
True
False
True or False? Most hepatic cysts are fast-growing and become symptomatic by 2 years of age?
True
False
Regarding benign liver tumors, which of the following scenarios is most correct?
Acute abdominal distention with hypotension in a 6 year old male.
Anemia and abdominal pain in a 14 year old female.
Abdominal fullness with a poorly defined abdominal mass in an 8 year old female
Hematemesis in a 10 year old female.
What is most common location of nonparasitic liver cysts?
Caudate Lobe
Right Lobe
Left Lobe
Bilobar
What is the most common benign liver tumor in childhood?
Focal Nodular Hyperplasia
Adenoma
Hemangioma
Hamartoma
What percentage of polycystic disease of the liver is associated with polycystic disease of the kidney?
15%
30%
50%
80%
Of the following, which is the most likely treatment strategy for polycystic disease of the liver?
No treatment
Operative excision of the cyst
Roux-en-Y drainage of the dominant cyst
Hepatic transplantation
Which type of polycystic kidney disease (PKD) can also involve the liver?
Autosomal dominant PKD.
Autosomal recessive PKD.
Both the dominant and recessive forms.
Neither the dominant or recessive forms.
All of the following are true regarding autosomal recessive polycystic kidney disease (PKD) except which?
It is rare form of inherited PKD.
It is associated with tuberous sclerosis and blindness.
There are 4 different variants determined by what age the patientis when symptoms develop.
Symptoms can begin before birth.
What type of glycogen storage disease is a hepatic adenoma associated with?
Type I glycogen storage disease
Type II glycogen storage disease
Type IV glycogen storage disease
Type V glycogen storage disease
Hepatic adenomas are usually solitary masses that most commonly occur in females during the second decade of life?
True
False
Which of the following laboratory values in a 12 year old with a liver mass is most consistent with adenoma?
Normal AFP, ALT, and AST
Decreased AFP, increased ALT and AST
Increased AFP, ALT, and AST
Increased AFP, decreased ALT and AST
What percentage of patients presenting with hepatic adenoma present with an acute abdominal crisis caused by intraperitoneal hemorrhage?
10%
25%
40%
60%
What is the characteristic appearance of an hepatic adenoma on a radioisotope scan of the liver?
No uptake differential between the adenoma and the normal liver.
Increased adenoma uptake compared to the normal liver.
Decreased adenoma uptake compared to the liver.
Retained radioisotope by the adenoma.
Which of the following is not true regarding hepatic adenomas.
All should undergo segmental or anatomic resections to preserve normal biliary drainage.
Recurrence is uncommon.
There is no evidence that they are premalignant.
They are usually asymptomatic masses found incidentally.
A patient is diagnosed as having focal nodular hyperplasia. What age range would they most likely fall into?
More than 2 years
2 - 5 years
5 - 10 years
More than 15 years
Regarding radiographic evaluation, which is NOT seen in patients with focal nodular hyperplasia?
Tumor uptake on radionucleotide scan
Hypervascular lesions on angiography
Venous pooling during the emptying phase
Solid characteristics on ultrasound
What is the surgical management of focal nodular hyperplasia?
Enucleation
Resection of lobe
Steroids
Observation
What percentage of mesenchymal hamartomas are detected during the first year of life?
20%
40%
60%
80%
Which of the following is true regarding mesenchymal hamartomas?
Easy to histologically differentiate from hemangioma of the liver.
They are premalignant.
Multiple cysts filled with clear fluid or mucoid material.
Resection alone results in recurrences in about 50%
All of the following are true regarding focal nodular hyperplasia except?
Their etiology is unknown.
It usually presents as a palpable mass.
Because they resemble hepatoblastoma, formal lobectomy or extended resection is normally undertaken to ensure cure.
They are solid tumors with extensive vascularization.
Vascular hepatic tumors commonly present after the first decade of life?
True
False
Which is not normal in patients with cavernous hemangioma?
20% will involve both lobes of the liver
Rupture, hemoperitoneum and shock are uncommon.
Large feeding arteries seen on arteriography of the lesion.
A hypervascular lesion seen on nuclear liver scan.
In general, all solitary hepatic vascular tumors should be resected when identified?
True
False
What is the treatment of solitary, asymptomatic liver hemangioma in a child?
Observation
Embolism
Resection
Steroids
What vascular hepatic tumor characteristically is described as multiple and produce nodular deformity of the entire liver?
Capillary hemangioendothelioma
Cavernous hemangioma
Infantile hemangioendothelioma
Both A and C
A six week old male presents with high output congestive heart failure, hepatomegaly, and a bruit over the epigastrium. What is the most likley diagnosis?
Tetrology of Fallot
Hepatic hemangioendothelioma
Hepatic hemangioma
Juvenile hepatic hamartoma
Hepatic arteriovenous malformation
Which is not part of the diagnostic triad of the hepatic hemangioendothelioma?
High output congestive heart failure
Cutaneous hemangiomas
Thrombocytopenia
Anemia
Of the following, which is the best diagnostic test for hepatic hemangioendothelioma?
Computed tomography
Doppler ultrasound
Selective arteriography
Needle biopsy
Regarding symptomatic hepatic hemangiomas, which is the least helpful treatment regimen?
Hepatic artery ligation
Digitalis
Irradiation
Diuretics
Steroids
What is the principal predisposing condition leading to the development of hepatic abscess in children?
Immune suppression during chemotherapy
Chronic granulomatous disease
Bacterial seeding secondary to omphalitis
Congenital hepatitis
Regarding chronic granulomatous disease (CGD), which is true?
It is an acquired disease
There are both granulomatous and suppurative findings within the hepatic parenchyma.
It usually presents after the second year of life.
Polymorphonuclear cell function is normal in children with CGD
Which of the following is/are symptoms of chronic granulomatous disease?
Episodic lymphadenitis, eczema, and pneumonia
Hepatic and splenic enlargement
Increased PMNS, mononuclear cells and histiocytes
Multiple abscesses in the lymph nodes, lungs, and soft tissues
All are symptoms associated with CGD
True or false: The combination of abdominal CT scan, gallium scan, and hepatic scintigraphy will rule out the presence of small hepatic abscess.
True
False
Which of the following is not commonly identified as a cause of pyogenic liver abscesses?
Sickle cell disease
Umbilical vein catheterization
Omphalitis
Cryptogenic abscesses
Which of the following is least often cultured from hepatic abscesses?
Staphlococcus aureus
Streptococcus pyogenes
Bacteroides fragilis
E. coli
In cryptogenic hepatic abscess, what is the most common cause of infection?
Streptococcus species
Anaerobic species
Staphlococcus species
Candida species
What is the most important first step in the management of pyogenic liver abscess.
Surgical resection
Antibiotic administration
Drainage
Steroids
None of the above
What is the expected mortality with appropriate treatment for children with hepatic pyogenic abscess?
20%
40%
60%
80%
Regarding patients with amoebic liver abscess, which is not true?
Amoebic colitis usually preceded the hepatic abscess
Trophozoites implant in the liver via the portal venous circulation
Patients with amoebic colitis that are otherwise healthy rarely develop amoebic liver abscesses.
When present, amoebic abscesses are more commonly found in the left lobe
What is the risk of spontaneous rupture in hepatic amoebic abscesses?
2%
5%
10%
20%
True or false: surgical drainage is often necessary for an amoebic abscess of the liver?
True
False
Of the following, which is not part of the treatment of hydatid disease of the liver?
Drainage of the cyst
Injection of the cyst with hypertonic saline
Albendazole administration
Exteriorzation or resection of the cyst
During the development and diagnosis of hydatid disease of the liver all of the following are true except which?
The cyst is usually under tension
The cyst develop quickly and produce pressure symptoms
Calcifications are commonly seen on plain radiograph
Ultrasound of the liver typically reveals a multicomartented cyst
True or false: The CasoniÂs skin test, which is used to diagnose Hydatid disease, is often negative with Hydatid liver cyst
True
False
Which of the following differentiates amoebic infections from hydatid disease?
Hydatid disease can be safely managed with closed aspiration
Amoebic infections can be diagnosed via a florescent antibody test
Hydatid disease can be diagnosed through immunoelectrophoresis
Metronidazole is effective treatment for both amoebic and hydatid disease
Which is most true regarding biliary atresia?
There is a 10% incidence of associated congenital heart disease
It occurs in approximately 1 in 25,000 live births
Males are more often affected than females
All are true
When does of biliary ductal system develop?
4 weeks
8 weeks
12 weeks
16 weeks
From what embryologic origin does the gallbladder arise?
Caudal component of the hepatic diverticulum
Cranial component of the hepatic diverticulum
Dorsal pancreatic bud
Ventral pancreatic bud
What differentiates the 'correctable' form of biliary atresia from the other forms?
Obliterated proximal intrahepatic ducts
Obliterated proximal and distal ducts
Patent gallbladder and cystic duct
Patent proximal extrahepatic ducts
What percent of infants with biliary atresia have associated anomalies in the other organs that form at the same time?
5%
15%
25%
50%
All of the following describe the untreated natural history of biliary atresia except which?
Biliary cirrhosis secondary to periportal fibrosis and obstruction of intrahepatic veins.
Pneumonia and sepsis
When treated without operation most infants die before 12 months of age
Esophageal varices
Which of the following is the most common cause of neonatal jaundice?
Biliary atresia
Infection (TORCH Syndrome)
Delayed glucuronyl transferase activity
Inspisated bile syndrome
Infants with greater than 20 percent of the total bilirubin as direct (conjugated) have either obstruction or _____?
Cholestasis
Obstruction
Both A and B
Neither A nor B
Of the following which is most consistent with biliary atresia?
Increased direct bilirubin, increased alkaline phosphatase
Decreased transaminases, increased total bilirubin
Increased transaminases, normal bilirubin
Phenobarbital pretreatment before the DISIDA scan will increase biliary flow. Which should be avaoided in a child as a result of phenobarbital pretreatment?
Succinylcholine
Halogenated anesthetics
Non-halogenated anesthetics
Aminoglycosides
What percent of infants with obstruction on DISIDA scan will actually have biliary atresia?
20%
40%
60%
80%
What is the best approach to delineate biliary atresia from neonatal hepatitis?
DISHIDA Scan
Percutaneous biopsy
Open cholangiogram
Magnetic resonance cholangiopancreatography (MRCP)
An infant is diagnosed with biliary atresia at 12 weeks of age. He should undergo operative repair immediately to optimize long-term hepatic function?
True
False
What percentage of patients have the Âcorrectable form of biliary atresia?
5%
20%
40%
60%
Of the following, which postoperative medication is not commonly utilized in infants who have undergone operative correction of biliary atresia?
Steroids
Trimethoprim/sulfamethoxazole
Rapamycin
Vitamin K
What is the expected five-year survival for infants with biliary atresia undergoing liver transplantation?
10%
25%
50%
75%
What percentage of pediatric liver tumors are malignant?
10 %
25 %
50 %
70 %
More than 90% of benign pediatric liver tumors occur before what age?
6 months
1 year
2 years
5 years
What fraction of hepatoblastomas occur before the age of 24 months?
10%
33%
66%
90%
What factors are associated with an increased incidence of hepatoblastoma?
Beckwith-Weidemann and hemihpertrophy
Fanconi's anemia
Cirrhosis
Type 1 glycogen storage disease
All
Which factor is not associated with an increased incidence of hepatoblastoma?
11p and 5q abnormalities
Beckwith-Weidemann and hemihpertrophy
Fetal alcohol syndrome
Choledochal cyst
TPN cholestasis
What viral infection is hepatocellular carcinoma associated with?
Epstein-Barr virus
Hepatitis A
Hepatitis B
Cytomegalovirus
True or False? Both hepatoblastoma and hepatocellular carcinoma are what type of malignancies?
Epithelial
Mesenchymal
Kuppfer cell
APUD cell
Which variant of hepatocellular carcinoma has a better prognosis?
Fibrolamellar
Embryonal
Fetal
Anaplastic
Which subclass of hepatoblastoma has the best prognosis?
Fetal
Embryonal
Mixed epithelial and mesenchymal (spindle cells)
Anaplastic (sheets of cells)
The most common symptom of a malignant liver tumor is what?
Mass which moves with respiration
Jaundice
Anemia
Anorexia and weight loss
Rarely hepatoblastoma presents as precocious puberty in boys; this is due to what?
Tumor limiting estrogen production
Tumor producing HcG
Tumor production of 5 alpha reductase
Tumor metastasis to the adrenal gland
Which of the following is most representative of a the serology of hepatoblastoma?
Increased AFP, bilirubin, and alkaline phophatase
Normal AFP, bilirubin and alkaline phosphatase
Increased alkaline phosphatase and AFP, normal bilirubin
Normal AFP and alklaine phosphatase, increased bilirubin
What percent of patients with hepatocellular carcinoma will have elevation in serum ferritin?
25%
50%
75%
95%
True or false? Both alpha-fetoprotein and ferritin can be used as tumor markers.
True
False
True or false? Hepatocellular carcinoma often has calcifications which can be seen on plain x-rays or CT scans.
True
False
Which of the following metastatic studies are part of the evaluation for hepatocellular carcinoma or hepatoblastoma?
Bone scan
Bone Marrow
Gallium scintigraphy
Celiac axis artreriography
Which of the following describes a Stage 2 hepatoblastoma?
Complete resection, tumor less than 5 cm
Resected with microscopic residual disease
Resected with gross residual disease
Ipsilateral nodal involvement
At initial presentation, what percent of hepatoblastomas are amenable to complete resection?
25 percent
40 percent
60 percent
80 percent
Given a primary tumor in one lobe and multicentric nodules in the contralateral lobe without metastatic disease, how many multicentric nodules can be resected with the primary without compromising outcome?
One
Upto three
Upto five
All can be resected regardless of number
A patient undergoes a left lateral hepatic segmentectomy, which hepatic vein(s) is/are divided?
Left and middle vein
Right and middle vein
Left vein only
Right vein only
Of the following, which is not a common metabolic disorder encountered after major liver resection?
Hypoalbuminemia
Coagulopathy
Hyperglycemia
Hypoprothrombinemia
Following hepatic resection, how long should you expect for hepatic regeneration to be complete?
One week
Three weeks
Two months
Four months
Which of the following is NOT a poor prognostic indicator for hepatoblastoma?
Embryonal histology
Multifocal disease
Diploidy
AFP more than 1 million
Which of the following accurately describes the survival of hepatoblastoma (HB) when compared to hepatocellular carcinoma (HCC)?
HB more than 50%, HCC under60%
HB under 10%, HCC more than 50%
HB more than 90%, HCC under 10%
HB more than 60%, HCC under 20%
What is the anticipated overall survival after liver transplantation for hepatoblastoma?