Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

PS > CH 9 Pediatric Liver and Biliary System > Flashcards

CH 9 Pediatric Liver and Biliary System Flashcards

1
Q

Pediatric liver

A

lies in the RUQ of abdomen and is composed of 3 lobes; right, left and caudate.
normal liver is homogeneous and slightly hyperechoic compared to the normal renal cortex.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Liver sono echogenicity compared to abdominal organs

A

Renal sinus > pancreas > Spleen/Liver > Renal cortex

liver is iso- or hypoechoic relative to the kidneys in neonates. the liver becomes hyperechoic to the kidneys at the end of the first year of life.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Liver size

A

In children the right love of the liver extends to or just below the right kidney. the left lobe should extend no farther than slightly left of the Aorta. In young babies the left lobe is relatively large and extends well over the midline.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Hepatic veins

A

MHV divides the liver into the right and left lobes and runs in the same plane with the IVC and GB fossa.

RHV divides the anterior/posterior segments of the right lobe

LHV divides the medial/lateral segments of the left lobe

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

INTERsegmental vessels

A

Hepatic veins. They course between the lobes and the segments of the Liver.

The HV walls are not echogenic.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

INTRAsegmental vessels

A

include the Portal Triad; MPV, proper HEA and CHD.

These course to the center of each segment of the liver.

PV walls are echogenic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Portal veins

A

RPV: anterior and posterior branch
LPV: medial and lateral branch

normal diameter of the PV:
<10 years old= 8mm
>10 years old= 10 mm

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Fetal circulations

A

umbilical vein > LPV > ductus venosus > MHV/LHV > IVC

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Ligamentum Teres

A

remnant of the umbilical vein. runs from the umbilicus to the LPV. the umbilical vein obliterates after birth to become the ligamentum teres.

runs in the intersegmental fissure along with the falciform ligament.

recannulize with portal HTN and becomes portocystemic collateral.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Ligamentum venosum

A

remnant of the ductus venosus, runs from the LPV to the IVC, separating the left lobe of the liver from the caudate lobe.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Hepatomegaly causes in children:

A

neuroblastoma, hepatoblastoma, cardiac failure, fatty infiltration, hepatitis, leukemia, metaabolic liver disorders, angiomatous lesions, mets from neuroblastoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Hemangiomas

A

primary, benign vascular neoplasms which consist of multiple vascular channels.
Infants may have skin hemangiomas and be asymptomatic, need a liver ultrasound if > 5

*hypoechoic in children/infants

more common in older children and young adolescents.

if multiple and with hemorrhage or cardiac compromise, steroid therapy may be used.

if solitary, hemangioma matures and vessel growth slows. vessels may enlarge and form “lakes” (cavernous hemangiomas) rarely seen in children.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Infantile hemangioendotheliomas

A

*rare benign vascular tumor in the neonate.
80% occur in first 6 months of life and more common in females.

masses appear complex, mostly solid hepatic lesions with variable hypo- or hyperechoic echotexture.

usually regress within the first year of life; life threatening due to CHF (congestive heart failure)

CFD will show blood flow in multiple tortuous vessels within and outside of the mass.

intrahepatic arteries show an increase in velocities.
*AV shunting may occur and cause high-output heart failure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Mesenchymal hamartoma

A

benign hamartomatous growth of mesenchymal tissue of unknown etiology.

more common in infants and children less than 2 years of age.

sono: appear as a large complex mass

surgical resection is required due to the risk of malignancy.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Focal nodular hyperplasia (FNH)

A

RARE in children, thought to be developmental hyperplastic lesion related to an area of congenital vascular malformation in the liver. asumptomatic and are more common in women.

sono: solitary, solid mass of varying echogenicity with a “central scar” sign (fibrous scar)
No risk of malignant transformation.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Liver adenomas

A

very rare in children.
commonly seen in flucogen storage disease, OPC use and steroid therapy ( can evolve into HCC)

may present with pain due to tumor hemorrhage. Surgical resection is recommended.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Liver tumors

A

malignant liver masses are rare in neonates.

Hepatoblastoma, hepatocellular carcinoma (HCC), liver mets

*hepatoblastoma’s are the most common malignant liver tumor in childhood.

hepatocellular carcinoma (HCC)are the 2nd most common malignant liver tumor in childhood.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Hepatoblastoma

A

*most common malignant liver tumor in childhood.
usually occur before the age of 3. present with asymptomatic enlargement of the liver. increase in serum AFP is seen.

mass is usually 10-12 cm at diagnosis. sono: solitary well-defined lesion which is more common in the right lobe. *PV invasion and lung mets are common.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q
  • Hepatoblastomas are associated with:
A

Beckwith-Wiedemann syndrome, hemihypertrophy and familial adenomatous polyposis.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Hepatocellular carcinoma (HCC)

A

peaks at 4-5 years and again at 12-14 years of age.
half of patients affected have a preexisting condition :glycogen storage disease, hepatitis or cirrhosis following biliary atresia.

Solid masses of variable echogenicity are seen sonographically. PV invasion is common and serum AFP levels are elevated.

2nd most common malignant liver tumor in childhood.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Liver metastases

A

usually seen with neuroblastoma, wilms tumor, leukemia and lymphoma in children.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Hepatic cysts

A

may be congenital or acquired.

congenital hepatic cysts: occur when an intrahepatic biliary duct fails to involute.

acquired hepatic cyst: usually due to trauma or infection. typically solitary, usually benign and do not require follow-up. may appear complex due to hemorrhage or infection.

> 10, evaluate for PCKD.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Hepatic abcessses:

A

pyogenic

amebic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Pyogenic abscess

A

localized collection of pus in the hepatic parenchyma.
more common in the right lobe of the liver and are commonly multiple in children.

generally associated with: sepsis, leukemia
may be due to drug therapy, appendicitis, cholangitis, or immunocompromised children.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Amebic abscess

A

occur when a parasite from the intestines invades the liver via the PV. right liver more commonly affected.
*clinical history is important ?? travel

amebic abscesses are endemic to the tropics and spread person to person. Most frequent extraintestinal complication of an ambeic infection.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Echinococcal cyst (hydatid cyst)

A

due to parasitic tapeworm infestation associated with sheep and cattle raising countries. Eggs are swallowed and pass into the portal venous system where they hatch and move into the liver.

average size is 5cm and can grow 2-3 cm a year. calcifications appear during healing phase.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Echinococcal cyst (hydatid cyst) sono:

A

appearances vary depending on the stage of disease:
simple cyst
cyst within a cyst (cyst with detached endocyst)
cyst with “daughter cysts”
calcified mass

*sonographic “water lily” sign

28
Q

Schistomsomiasis

A

invasion of the PV by the schistosoma ova.
It leads to portal HTN without cirrhosis.
PV becomes very echogenic due to fibrotic changes due to invasion. Lay eggs in PV wall.
RARE in children.

29
Q

Schistosoma ova:

A

freshwater parasitic worms occurring in tropical and subtropical regions.

usually contracted from contaminated food and water.

30
Q

Cirrhosis

A

usual forms in children: biliary and post-necrotic.
cirrhosis leads to portal HTN. the liver becomes nodular due to regenerating nodules. vascularity is not seen due to the abundance of connective tissue in the liver.

sono: shrunken with a nodular surgace and an enlarged caudate lobe (has its own blood supply, so it is spared)

31
Q

Hepatitis

A

Inflamation of the liver.
may be due to: TORCH infections, biliary atresia, cystic fibrosis, or toxin accumulation in inherited metabolic diseases.

sono: enlarged hypoechoic liver and PV more echogenic giving a * “starry night” appearance.

32
Q

Fatty Infiltration (steatosis)

A

accumulation of fat in the hepatocytes (liver cells). The liver appears hyperechoic and there is poor sound penetration.

reversible in most cases except when the steatosis leads to cirrhosis.

33
Q

Causes of fatty infiltration in children:

A

early cirrhosis, chemotherapy, obesity, nephrotic syndrom, glycogen storage disease, hyperalimentation, malnutrition, alcohol abuse, steroid use,

*Reye’s syndrome, * cystic fibrosis (most common)

34
Q

Liver transplants in children

A

chronic hepatitis, liver cancer, familial cholestasis, alpha-1- antitrysin deficiency, primary sclerosing cholangitis, glycogen storage disease, neonatal hemochromatosis, sudden acute failure (due to infection, drugs, poisons), tyrosinemia (metabolism error cuased by an enzyme defect

*biliary atresia (leads to liver failure), *wilson’s disease (copper accumulation in the liver.

35
Q

Most common complication post liver transplant

A

*hepatic artery stenosis.

Pneumobilia and fluid collections are also commonly seen.

36
Q

Rex shunt/bypass

A

used to manage portal HTN in children when TIPS may be technically difficult.

Rex bypass involves linking the SMV to the LPV and redirects flow into the liver.

portal flow is restored by bringing the mesenteric venous and splenic blood around the extrahepatic portal obstruction and restores hepatopedal flow.

37
Q

Situs inversus

A

congenital condition in which the major visceral organs are reversed or mirrored from their normal position.

38
Q

Biliary tract

A

composed of the gallbladder and the biliary ducts.
basic function is to drain the liver of bile and store the bile until needed to aid in digestion. GB holds bile, the bile is concentrated by the GB secreting mucous and absorbing water.

39
Q

course of bile from GB to duodenum

A

GB composed of a fundus, neck and body. Neck narrows into the cystic duct which joins the common hepatic duct (CHD) to form the common bile duct (CBD). The CBD joins with the duct of wirsung to form the ampulla of vater. The flow of bile into the duodenum is controlled by the sphincter of Oddi.

40
Q

Neonatal jaundice

A

result of elevated bilirubin in bloodstream.

2 types: unconjugated and conjugated.

41
Q

Unconjugated bilirubin (indirect)

A

when bilirubin is released from the red blood cells that have been broken down (hemolysis)
common in newborns, if persists for more than 2 weeks it is consisdered persistent.
*unconjugated “toxic” hyperbilirubinemia

42
Q

causes of unconjugated bilirubin

A 
breast milk jaundice
UTI
hemolytic anemia
hypothyroidism
high GI obstruction
43
Q

*breast milk jaundice

A

associated with breast feeding. an increase in indirect bilirubin in a breastfed newborn after the first 4-7 days of life. no identifiable cause.

44
Q

*breastfeeding jaundice

A

first 3 days of life, caused by an insufficient production or intake of breastmilk.

45
Q

Conjugated bilirubin (direct)

A

when the released bilirubin is converted by liver cells to a water-soluble form. This aids in fat digestion and turns stools dark.
*conjugated huperbilirubinemia is persistent jaundic caused by liver disease (bile duct obstruction) non-toxic

46
Q

Causes of conjugated biliruin (direct)

A 
biliary atresia (main cause)
choledochal cyst
neonatal hepatitis
metabolism errors
cystic fibrosis
choledocolithiasis
TPN
intrahepatic biliary hypoplasia (alagille syndrome)
47
Q

Biliary atresia

A

*progressive sclerosis of the extra- and proximal intrahepatic bile ducts and gallbladder.
acquired inflammation of the bile ducts or an inherited viral infection at birth which results in damage to segments of the biliary tree.

GB very small or absent

suspected if hyperbilirubinemia persists after 14 days of life. *more common in females.

  • perinatal biliary atresia most common, presents 2-4 weeks of life
  • fetal biliary atresia is rare
48
Q

Kasai classification of biliary atresia

A

type I: obliteration of CBD, proximal ducts patent.
type IIa: atresia of hepatic duct, with cystic and common bile duct patent
typeIIb: cystic, common, and hepatic ducts obliterated
type III: (90%) discontinuity of right and left hepatic ducts to the level of the porta hepatis.

49
Q

Infants with biliary atresia:

A

failure to thrive, cholestatic jaundice, pale stools and dark urine.
*more common in females
cirrhosis and portal HTN result and it is fatal without Kasai procedure (surgical drain) or liver transplant within the first 3 months of life.

50
Q

Triangular cord sign

A

triangular echogenic cord of fibrous tissue seen in the porta hepatis. useful in evaluation of infants with cholestatic jaundice, helps with differential diagnosis of biliary atresia from neonatal hepatitis.

*more that 4mm thickness of the echogenic anterior wall of the right portal vein measured on longitudinal scan.

51
Q

biliary atresia associated with:

A

polysplenia
absent IVC
situs inversus
some cardiac anomalies

52
Q

Intrahepatic biliary hypoplasia (alagille syndrome)

A

autosomal dominant condition.
child has intrahepatic biliary hypoplasia, severe itching, and failure to thrive.

also have: characteristic triangular face, skeletal abnormalities,peripheral pulmonary stenosis, renal tubular disorders and eye defects. without transplant, only half survive into adult life.

53
Q

Caroli disease

A

*non-obstructive, congenital, multifocal segmetal dilatation of the intrahepatic bile ducts.

ducts form cystic dilatations and are prone to sludge and calculi which puts the patient at risk for cholangitis.

associated with: congenital hepatic fibrosis, renal medullary cystic disease and choledochal cyst.

complications: cholangitis, choledocholithiasis, cholangicarcinoma and hepatic abscesses and sepsis.

54
Q

Congenital hepatic fibrosis

A

Liver initially appears normal but eventually enlarges, especially the left lobe. Peri-portal fibrosis appears and increases with time. Cystic dilatations of the biliary tree are seen; this is the most diagnostic appearance.

**congenital hepatic fibrosis is one of the fibropolycystic diseases which include:
Caroli disease
ADPCKD
ARPCKD

55
Q

Choledochal cysts

A

congenital biliary anomaly of the bile ducts. *There are 5 types, the most common is type I (80-90%)

*more common in Japanese females.
symptoms usually occur before age of 10

children present with: fever, abdominal pain, jaundice and palpable abd mass.

56
Q

Choledochal cysts sono:

associated with:

A

sono: well-defined, fluid-filled cystic masses in the porta hepatis, and the GB and CBD and intrahepatic bile ducts are dilated.
associated: pancreatitis, cholangitis, hepatic abscess, cirrhosis, portal HTN, and cholangicarcinoma.

57
Q

Cholecystitis

A

inflammation of the GB which may be acute or chronic. The GB wall appears thickened due to infection of the GB wall. RARE in pediactrics.

*GB disease is more common in children with sickle cell anemia

58
Q

Thickened GB wall may be seen with:

A 
Cholecystitis
ascites due to heart,liver, or kidney failure
veno-occulusive disease
peritonitis and sepsis
pancreatitis
cholangitis
leukemia
kawasaki's dz
59
Q

Cholelithiasis

A

stones in the GB, sludge is more common in children

60
Q

Causes of gallstones in pediatric patient:

A 
*sickle cell
crohn's dz
choledochal cysts
biliary tree obstruction
metabolic dz
hemolysis
sepsis
hemolytic anemia
TPN
RH problems
blood rransfusions
dehydration
obesity
bile duct anomalies
wilsons dz (too much copper in body; inherited)
*cystic fibrosis ("sticky" bile)
61
Q

Gallbladder sludge

A

thickened bile caused by bile stasis. Mostly seen in children on TPN or who have been extremely ill and not eating. May also be caused by an obstructed biliary tree and GB

62
Q

Gallbladdr hydrops

A

*GB mucocele. the GB is massively dilated due to obstruction of the cystic duct.
The bile is reabsorbed and GB mucosa secretions or watery fluid fill the GB.
*Commonly seen in Kawasaki dz in children.

sono: large GB will be seen with anechoic contents and no ductal dilatation.

63
Q

CBD sizes:

A

< 1 mm = neonates and infants < 1 year
<2 mm = <2 years
<4 mm = 2-12 years
<5 mm= adolescents

64
Q

GB wall in children:

A

1-3 mm (no different than adults)

65
Q

Pneumobilla:

A

air in the biliary tract which is associated with sphincter of Oddi incompetency, emphysematous cholecystitis or due to and ERCP, liver transplants.
Echogenic foci are seen in the ducts with comet-tail or reverberation artifacts.

PS (15 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions