Peds- GB biliary system Flashcards
3 variants of GB?
- Phrygian cap – fold at fundus
- Hartmann pouch – widening at neck
- Prominent spiral valves of Heister
6 congenital anomalies of GB?
- Ectopia
- Agenesis
- Duplication
- Biliary Atresia
- Choledochal Cyst
- Multiseptate Gb
Multiseptate GB can lead to?
- septations can lead to bile stasis and formation of gallstones
the two major causes of conjugated hyperbilirubinemia in newborns?
- liver diseases (hepatitis)
- biliary atresia
3 types of bilary atresia manifestations?
- Absence of biliary tree
- Rudimentary gb and cystic duct
- Visible gb, cystic duct and CBD
Newborns develop neonatal jaundice at what age?
3-4 weeks
Biliary atresia sono features?
- GB, cystic duct, CBD and IHBD may be seen incompletely, depending on degree of severity
- Early signs of cirrhosis
2 important findings to diagnose biliary atresia?
- triangular cord sign
- GB length <1.5 cm or absent
TRIANGULAR CORD SIGN?
Triangular, cone-shaped, fibrous ductal remnant is positioned anterior and superior to the portal vein and the hepatic artery
Biliary atresia- kasai procedure?
- Surgical intervention where a connection is made between the duodenum and gallbladder, CBD and liver to drain the bile
- Successful if performed early
- Successful in 1/3 of cases
- 2/3 will need liver transplantation
Choledochal cyst symptoms? (3)
- abdo pain
- ando mass
- jaundice
How many types of Choledochal cyst are there?
five
type 1 Choledochal cyst?
- concentric dialation of CBD
- Most common type
Type 2 Choledochal cyst?
- CBD diverticulum
Choledochal cyst type 3?
choledochocele:
- cystic dialation of intraduodenal portion of CBD
Choledochal cyst type 4?
- Ditatation of CBD with IHBD involvement
Choledochal cyst type 5?
- Caroli Disease
- Saccular dilatation of IHBD with calculus formation and bacterial cholangitis
- Minimal dilatation of IHBD, associated with hepatic fibrosis and portal hypertension
Choledochal cyst sono features?
- Cystic structure near GB continuous with bile ducts
- Large cysts containing sludge
- IHBD dilatations (type IV and V)
Choledochal cyst complications?
- Stone formation in cyst, GB, panc duct
- Biliary obstruction
- Chronic cholangitis
- Cirrhosis
- Bilirary rupture with biliary peritonitis (image)
Acquired Pathology of the Gallbladder and Biliary System (7)?
Gallbladder wall thickening Cholelithiasis Cholecystitis Hydropic Gallbladder Biliary Obstruction Sclerosing Cholangitis Rhabdomyosarcoma
Gallbladder Wall Thickness?
Normal up to 3 mm (fasting)
> 3 mm indicates disease
Gallbladder Wall Thickness inflammatory causes?
- acute and chronic cholecystitis
Gallbladder Wall Thickness noninflammatory?
- Viral hepatitis
- cirrhosis
- congestive heart failure
- pancreatitis
What is Cholelithiasis?
Presence of one or more calculi in the GB, cystic duct or CBD
Pediatric cholelithiasis is associated with?
- Cystic fibrosis
- Malabsorption
- Crohn’s disease
- Bowel resection
- Sickle cell anemia
Neonatal cholelithiasis associated with?
- Anomalies of the biliary system
- Dehydration
- TPN (total parenteral nutrition - causes bile stasis)
- Infection
- Hemolytic anemia
cholelithiasis clinical presentations?
Young Children:
- Nonspecific - jaundice, irritability
Older Children:
- RUQ pain
- Intolerance to fatty foods
- N&V
- Neonates may be examined because stones were seen in utero; most resolve within first year of life
cholelithiasis sono?
Mobile echogenic intraluminal structures with PAS, may have twinkle artifact with colour doppler
GB sludge?
- what is it?
- artifact?
- causes?
- Low-level echoes in GB
- May create layer within GB, fill the lumen or create sludge balls
- Slice thickness artifact
Causes:
- Prolonged fasting
- Hyperalimentation/TPN
- Extraheptic bile duct obstruction
Cholecystitis causes?
- Hypoalbuminemia
- Acute Viral Hepatitis
- Heart and renal failure
- Cystic duct obstruction from external source
Cholecystitis symptoms?
- RUQ pain
- Fever
- Vomiting
- Palpable RUQ lump
what Hydropic gallbladder??
- Acutely ill children on TPN
associated with:
- Kawasaki syndrome
- leptospirosis
- chronic biliary obstruction
Hydropic gallbladder clinical presentation?
- RUQ pain
- Fever
- Dehydration
- Abdo distention
- Cause is unclear
Hydropic gallbladder sono features?
GB - enlarged, anechoic, with thin walls
Biliary Obstruction?
- Can be intrahepatic or extrahepatic
examples of intrahepatic:
- Stones, strictures
examples of extrahepatic:
- tumor, LNs. acute pancreatitis
Biliary Obstruction presents as?
- ducts become dilated
- multiple irregular branching of bile ducts
ducts cab rupture in biliary obstruction and cause?
- neonatal jaundice
- biliary ascites
- biloma
Sclerosing Cholangitis?
Chronic disease with inflammatory fibrosis that obliterates the intra and extra hepatic bile ducts
Sclerosing Cholangitis result in? (3)
- biliary cirrhosis
- portal hypertension
- liver failure
Sclerosing Cholangitis associated with?
- IBD (70-80%)
Sclerosing Cholangitis clinical presentation?
- UQ pain
- Jaundice
- Abnormal LFTs and ↑ bilirubin
Sclerosing Cholangitis sono features?
- thickened bile duct walls
- choledocholithiasis
- ductal strictures
Biliary Neoplasm- Rhabdomyosarcoma
- Rare
- Ages 1-5 years
- Arises from biliary tract and causes obstruction
Rhabdomyosarcoma clinical presentation?
- Increasing abdo girth
- Jaundice
- Pain
- Weight loss
Rhabdomyosarcoma sono feature?
Solid
Hyperechoic
Within bile ducts
