18-6: Structural and Circulatory Diseases

Question 1

what are congenital dilations of the common bile duct called

Answer 1

Cholodochal cysts

Question 2

what are the 3 fibropolycystic diseases of the liver

Answer 2

Von Meyenburg complexes -> hamartomas
Caroli disease -> simple multiple biliary cysts
Caroli Syndrome -> congenital hepatic fibrosis

Question 3

what are some issues that can arise out of choledochal cysts

Answer 3

predisposition to stone formation, stenosis and stricture, pancreatitis, and obstructive biliary complications

Question 4

describe fibropolycystic disease of the liver

Answer 4

heterogeneous group of lesions in which the primary abnormalities are congenital malformations of the biliary tree

Question 5

what is the etiology of fibropolycystic disease of the liver

Answer 5

spectrum of developmental ductal plate malformations, resulting from fetal periportal ductal plates

Question 6

What disease does fibropolycystic disease of the liver occur with

Answer 6

autosomal recessive polycystic renal disease

increased risk of cholangiocarcinoma

Question 7

if blood flow into the liver is impaired what will manifest

Answer 7

esophageal varices
splenomegaly
intestinal congestion

Question 8

if blood flow in the liver is impaired what will manifest

Answer 8

ascites
esophageal varices
hepatomegaly
elevated AST/ALT

Question 9

If blood flow out of the liver is impaired what will manifest

Answer 9

ascites 
Hepatomegaly 
abdominal pain 
Elevated AST/ALT
jaundice

Question 10

what is the result of neonatal umbilical sepsis or umbilical vein catheterization

Answer 10

subclinical portal vein occlusion -> ascites and varices years later

Question 11

what are some conditions that can lead to obstruction of the extrahepatic portal vein

Answer 11

pyelophlebitis 
hypercoagulable states 
trauma 
pancreatitis 
HCC
cirrhosis

Question 12

what is the most common cause of small portal vein branch obstruction

Answer 12

schistosomiasis; egss of the parasites and the associated granulomatous inflammatory response

Question 13

what is the most common cause of intrahepatic blood flow impairment

Answer 13

cirrhosis

also caused by

Sickle cell
DIC
Eclampsia
diffuse intrasinusoidal tumor

Question 14

describe peliosis

Answer 14

peculiar form of sinusoidal dilation that occurs in any condition in which efflux of hepatic blood is impeded

Question 15

The obstruction of major hepatic veins produces a condition known as?

Answer 15

Budd-Chiari syndrome - hepatomegaly, abdominal pain, ascites

Question 16

what is the etiology of sinusoidal obstruction syndrome

Answer 16

allogenic hematopoietic stem cell transplantation

Cancer pts receiving certain chemos