Structural and Circulatory Disorders Flashcards
What are choledochal cysts?
congenital dilations of the biliary tree
How do choledochal cyst often present?
(epi)
commonly before age of 10 (~20% present in adults still)
-most common in females
Presentation:
- jaundice
- biliary colic
What complications occur with cholecochal cysts?
- stone formation
- stenosis
- stricture
- pancreatitis
- obstructive biliary complications in the liver
What is fibropolycystic liver disease?
group of diseases resulting in malformation of the biliary tree due to dutal plate malformations
What are the types of fibropolycystic disease?
- Von Meyenburg complexes (hamartomas)
- cysts (Caroli disease/syndrome)
- congenital fibrosis
What is difference between Caroli disease and syndrome?
Caroli disease:
- single/multiple intra/extrahepatic biliary cysts
- absence of fibrosis
Caroli syndrome:
-single/multiple intra/extrahepatic biliary cysts w/ congenital hepatic fibrosis
What disease frequently occurs with fibropolycystic disease?
-polycystic renal disease (autosomal recessive; polycystin gene)
What complications can occur with fibropolycystic disease?
- ascending cholangitis
- cholangiocarinoma
What are the main vascular conditions that affect the liver?
Impaired blood inflow:
- hepatic artery compromise
- portal vein obstruction
Impaired intrahepatic flow:
- cirrhosis (**main cause**)
- sinusoidal occulsion
Impaired blood outflow:
- hepatic vein occlusion (Budd-Chiari syndrome)
- sinusoidal obstructive syndrome
Why is liver infarction uncommon?
dual blood supply from hepatic artery and portal vein
How do circulatory disorders of the liver present depending on location?
Inflow (particularly portal vein):
- portosystemic shunts (varicies, caput medusa, hemorrhoids)
- splenomegaly
Intrahepatic:
-hepatomegaly
-elevated AST/ALT
-ascites
-portosystemic shunts
Outflow:
- abdominal pain
- jaundice
- hepatomegaly
- elevated AST/ALT
- ascites
What are the main causes of hepatic artery compromise?
- thrombosis
- neoplasia -> compression
- polyarteritis nodosa
What conditions are associated with portal obstruction?
- neonatal umbilical sepsis or umbilical vein catheteization (presents years later)
- pyleophlebitis
- hypercoagulable states (polycythemia vera)
- trauma/surgery
- pancreatitis/pancreatic cancer
- HCC
- thrombosis
What conditions are associated with sinusoidal occlusion?
- sickle cell disease
- DIC
- eclampsia
- hematogenous metastasis
What is peliosis hepatitis?
(appearance, cause)
sinusoid dilation due to impeded blood efflux
- multiple blood filled cystic spaces
- risk of rupture -> fatal hemorrhage
- cause unknown but associated with sex-hormones
What is Budd-Chiari syndrome?
(presentation)
obstruction of at least two major hepatic veins
Presentation (triad):
- abdominal pain
- hepatomegaly
- ascites
What conditions are associated with Budd-Chiari syndrome?
- polycythemia vera
- hypercoagulative disorders
- anitphospholipid antibody syndrome
- paroxysmal nocturnal hemoglobinuria
- intraabdominal cancers
What is sinusoidal obstructive syndrome?
(causes)
obliteration of of terminal hepatic venules
Causes:
- hematopoietic stem cell transplantation (HSCT)
- cancer pt’s on chemo
-Jamacian bush tea (pyrrolizidine alkaliod)
What features are seen in sinusoidal obstruction syndrome?
- characteristic traits with Masson tirchrome stain
- reveresed venous blood flow
What causes passive congestion of the liver and how does appear?
- right-sided cardiac failure -> passive congestion
- left-sided cardiac failure -> hypoperfusion (also causes R-sided failure)
results in centrilobular (zone 3, it is most vulnerable to ) hemorrhagic necrosis -> gross appearance of nutmeg liver
