Congenital Abnormalities Flashcards
1
Q
Congenital abnormalities of the liver
A
- Fibrocystic disease of the liver
- Congenital hepatic fibrosis
- Caroli disease
- Polycystic liver disease - Neonatal cholestasis
- neonatal hepatitis
- cholangiopathies (biliary atresia)
2
Q
Features of congenital hepatic fibrosis
A
- broad bands of collagenous fibrous septa dividing liver into irregular islands
- distorted bile ducts within fibrous bands - persistence of embryonic form of biliary tree
- fibrous bands obstruct blood flow - portal HTN - bleeding, varices
- also stasis - predispose to infection + bile exposure - carcinogenic
3
Q
Features of caroli disease
A
- larger ducts of intrahepatic biliary tree segmentally dilated
- stasis - stone formation, infection
- complications: cholelithiasis, cholangitis + abscess, cholangiocarcinoma
4
Q
Features of polycystic liver disease
A
- multiple cysts in the liver, not communicating w biliary tract
- clinically asymptomatic
5
Q
Causes of neonatal hepatitis
A
- Infections - bact sepsis, syphilis, toxoplasmosis, hep B, CMB, rubella, HSV
- Metabolic/genetic - α1-antitrypsin def, Wilson’s disease, galactosemia
- Toxic - drugs
- Idiopathic
6
Q
Features of neonatal hepatitis
A
- cholestasis, jaundice
- mononuclear infiltration
- necrosis, focal
- multinucleated hepatocytic giant cells
- bile ductule proliferation
7
Q
Features of biliary atresia
A
- complete/partial obstruction of the lumen of the extra hepatic biliary tree
- (A) foetal - aberrant intrauterine development (B) perinatal - normally developed biliary tree destroyed following birth (viral, autoimmune)
8
Q
Morphological features of biliary atresia
A
- severe cholestasis
- marked bile duct proliferation
- inflammatory cellular infiltration (obstruction - stasis - infection - neutrophils)
- progressive destruction of intrahepatic bile ducts (usually chronic)
- biliary fibrosis
- biliary cirrhosis
