19 Feb 24 Flashcards
Biliary Cyst pathogenesis
Cystic dilation of biliary tree
Most common:
Single , Extra hepatic dilation of CBD (type 1)
Biliary Cyst CF
🧣Incidental finding
🧣Classic triad (children/adults)
Abd pain
RUQ mass
Jaundice
🧣Neonates : jaundice , acholic stools , dark urine , hepatomegaly
🧣N , V
DX of biliary Cyst
USG. Or CT or MRCp
Complications of biliary Cyst
Cholangiocarcinoma
Acute Cholangitis
Pancreatitis
Stone formation
Ttt of biliary Cyst
Cyst resection (to dec risk for malignancy)
Roux en Y hepaticojejunoatomy
(To allow for biliary drainage)
Pt with obstructive SS should be treated for acute condition first and cyst resection later.
Differnt prsentations of biliary Cysts
🥾A/S
🥾Symptomatic :
RUQ abd pain , jaundice and RUQ mass
🥾Acute complications:
Pancreatitis, cholangitis , stone formation.
Biliary cysts and pancreatitis
Biliary cysts inc the risk of pancreatitis due to association with
ANOMALOUS pancreaticobiliary junction
🏣 abnormally long common channel connecting pancreatic duct and CBD
This lengthy channel is predisposed to OBSTRUCTION from plugs and stones which can cause reflux of pancreatic fluid and CHOLESTASIS (conjugated hyperbili).
Common etiologies of Acute Cholangitis
👘Choledocholithiaisis
👘Malignancy
👘PSC
👘Biliary interventions causing incomplete bile drainage
Ttt of acute cholangitis
😉BS antibiotic
😉ERCP with sphincterotomy for biliary drainage in 24-48hrs
😉PTC (percutaneous trashepatic cholangiography) with drain placement
open surgical decompression
CO of acute cholangitis
🗻Charcot triad : fever RUQ pain jaundice
🗻Reynold pentad : AMS , hypotension
🗻Leukocytosis
🗻Cholestasis : inc ALP and direct hyperbili
🗻AGMA with Lactic acidosis (with severe sepsis)
Acute cholangitis def
Life threatening infection that develops due to biliary obstruction , which enables bacteria to enter ampulla and biliary tree.
How to approach neonatal cholestasis
(Dark urine ,acholic stools , conjugated hyperbili)
🥁 ABD ultrasound
⬇️
↪️If abnormal/absent GB ➡️ biliary atresia
↪️If cystic dilation of biliary tree ➡️ biliary Cyst
↪️Isolated hepatomegaly or Normal USG
1️⃣ infectious evaluation
CMV , Toxoplasma , HSV , UTI /sepsis
2️⃣ genetic/metabolic evaluation
AAT def
Dubin johnson
Galactosemia
Cystic Fibrosis
Gall stone pancreatitis complicated by Acute cholangitis
Gallstone pancreatitis develops when gallstone passes thru bikiary tree and obstructs ampulla or flow from pancreatic duct allowing bile to reflux into pancreas.
Pts have pancreatitis CF plus cholestasis.
If stone remains in biliary tract resultant bile stasis allows bacteria to ascend from duodenum ➡️ Acute cholangitis.
So CF combined are ;
Pancreatitis plus cholestasis plus reynolds pentad.
Ttt: IV fluids
AB
ERCP (to relieve biliary obs - stone extraction/sphincterotomy/stent placement)
Pathogenesis of PBC
Autoimmune destruction of Intrahepatic bile ducts.
(Leading to bile stasis and cirrhosis)
CF of PBC
❇️Affects middle aged women
❇️Insidious onset of fatigue and pruritis
❇️Progressive jaundice , hepatomegaly , Cirrhosis
❇️Cutaneous XANTHOMAS, XANTHELASMAS.
Labs of PBC
🛞Cholestatic pattern of liver injury
🛞⬆️⬆️ALP
🛞⬆️aminotransferases
🛞Antimitichondrial Antibody
🛞Severe Hypercholesterolemia
(Hyperlipidemia has elevated of HDL out of proportion to LDL and doesnt increase the risk of atherosclerosis)
Ttt of PBC
Urosodeoxycholic acid (delays progression)
Liver transplant
Complications of PBC
🥁Malabsorption, Fat soluble Vitamin def
🥁Metabolic Bone disease (osteoporosis , osteomalacia)
🥁HCC
🥇osteomalacia (dec bone mineralization) is related to vitamin D def and fat malabsorption.
How to manage suspected PBC
🦷Do labs
ALP , cholesterol, LFTs , bilirubin
🦷RUQ USG (distinguishes intrahepatic cholestasis vs Extrahepatic cholestasis-gall stones causing biliary tract dilation)
If USG suggests intraheptic cholestasis
🦷Antimitochondrial antibody titres
How to evaluate a patient with high ALP
1️⃣ check GGT. If Normal then ALP is from bone
2️⃣ if GGT is high ➡️ ALP is biliary
3️⃣ do RUQ USG and AMA
↪️ positive AMA or Abnormal USG
Liver biopsy
↪️ dilated bile ducts on USG
ERCP
↪️ both normal
Liver biopsy or ERCP or observe
Benefits of Ursodeoxycholic acid UDCA in PBC
Drug of choice
Increases biliary secretion and has anti inf and immunomodulatory effects
UDCA delays histologic progression in PBC and may improve SS and survival.
Should be given as soon as dx is made even to A/S patients.
Less effective ttt for more advanced stage of Dx.
Acute cholangitis etiology
👗Occurs in any setting of Biliary Stasis
Gall stones
Malignancy
Strictures /stenosis
👗Elevated intrabiliary pressure allows for disruption of Bile -blood -barrier
And translocation of bacteria from hepatobiliary system into bloodstream.
USG / CT findings Of AC
Dilation of intrahepatic ducts and CBD
Malignant biliary obstruction causes
Cholangiocarcinoma
Pancreatic cA / HCC
Metastasis (colon , gastric)