18.6 Flashcards
what are choledochal cysts
congenital dilations of common bile duct (CBD)
more common in F pts and often seen in kids around the age of 10
present w/ jaundice and recurrent abd pain (biliary colic).
20% of cases will become symptomatic in adulthood.
predisposes the pt to stone formation, stenosis, strictures, pancreatitis and obstructive biliary compilation w/i the liver.
what is fibropolycystic dz
heterogeneous groups of lesions that are congenital in origin, affecting the biliary tree.
All of these lesions are ductal plate malformations,resulting from persistence of the fetal periportal ductal plate
can be associated w/ AR-polycystic renal dz and have an increased risk for cholangiocarcinoma
ex: Von Meyenburg complexes, Simple multiple biliary cysts, Congenital hepatic fibrosis
what are von meyenburg complexes
hamartomas: this is disorganized tissue in NL tissue
If these are diffuse/multiple then they are associated w/ fibropolycystic dz
what is an example of simple multiple biliary cysts
caroli dz:
this is multifocal cystic dilation of large intrahepatic bile ducts
what is an example of congenital hepatic fibrosis
caroli syndrome
= caroli dz + congenital hepatic fibrosis
what is the normal O2 sat of the hepatic A
O2 sat of the hepatic A is > 95% usually, but portal bloodO2 sat can reduce to 85% during fasting. This drops lower after food consumption.
what occurs w/ of hepatic A compromise
what can cause this
will NOT always produce ischemic necrosis
However it may lead to local infarct (pale and anemic) or hemorrhagic (if suffusion w/ portal blood)
Common causes : neoplasms, polyarteritis nodosa-vasculitis that affects renal/visceral vessels (seen in hep B), and sepsis
what is the manifestation of portal V obstruction
= esophageal varices (prone to rupture), splenomegaly and intestinal congestion
spectrum between well-tolerated to catastrophic
presentation includes abd pain and portal htn
may be idiopathic or from neonatal umbilical sepsis/umbilical V catheterization
when and how neonatal umbilical sepsis/umbilical V catherization occur
present years later as ascites and variceal bleed due to subclinical occlusion of the portal V
give examples of extrahepatic obstructions
intraabd infxn by acute diverticulosis or appendicitis → pylephlebitis in splanchnic circulation
inherited hypercoag state (myeloprolific neoplasm- polycythemia vera)
trauma
pancreatitis & pancreatic CA-associated splenic V thrombosis
HCC (invasion of portal V)
cirrhosis (25%, may w/ underlying thrombophilic genotype)
what is the MCC of small portal obstruction
schistosomiasis- the eggs of the parasite
associated granulomatous inflam response that obstruct the smallest branches of the portal V
what is the association of idiopathic noncirrhotic portal HTN in small portal V branches
how does it present
what is the prevalence
This is associated w/ drugs, toxin, infections, prothrombotic states, immune def, chronic biliary obstruction and AI dz.
The pt presents w/ UGIB.
India
Japan, F >M and these pts present w/ splenomegaly associated w/ rheumatic dz
untreated HIV dz, pts on ART (complication of therapy)
Liver transplant may be needed to avoid sequelae.
what is the MCC of intrahepatic cause of blood flow obstruction
cirrhosis
how may occlusion of sinusoidal flow occur
sickle cell dz, DIC, eclampsia or diffuse intrasinusoidal tumors
With all of these the obstruction can lead to massive necrosis of the hepatocyte and cause acute liver failure
who will present w/ sinusoidal obstruction syndrome
Jamaicans that drink pyrrolizidine-alkaloid-containing bush tea (veno-occlusive dz, originally)
can be found in allogeneic transplant patients w/i 1st 3 weeks or CA pt on certain chemo
