Biliary Tract Congenital Anomalies Disorders of gallbladder Acute cholecystitis Chronic cholecystitis Disorders of Extra hepatic bile ducts Choledocholithi asis Ascending cholangitis Biliary atresia Choledochal cysts Tumors

MC congenital anomalies = abnormal variants of the gallbladder MC= folded fundus (Phrygian cap) Others= congenitally absent, bilobed or aberrantly located gall bladder Most common congenital anomaly

Cholelithiasis (Gall Stones) 10 to 20% of adults in developed countries Two kinds of stones 1. Cholesterol Stones= crystalline cholesterol monohydrate, Risk factors –Native American –adult in industrialized country –increased age (>40 yrs, Forty) –Females 2:1 –estrogenic influences (Fertile) –Clofibrate –Obesity (Fatty) or rapid weight loss –Gallbladder stasis, in spinal cord injury pregnancy Hypercholesterolemic syndromes

1. Cholesterol Stones contd… Four conditions -necessary for cholesterol stone formation 1.supersaturated bile with cholesterol 2.gallbladder hypomotility  promotes crystal nucleation 3.microprecipitates of calcium salts (inorganic or bilirubin salts) 4.Mucus hypersecretion in the gallbladder Morphology= Pale yellow and hard, ovoid, usually single, Often radiolucent Cholesterol stones arise exclusively in the gall bladder 2. Pigmented Stones= bilirubin calcium salts Risk factors –Asian, Rural –Chronic hemolytic syndromes –Biliary tract infection (E. coli, Ascaris lumbricoides, liver fluke - Opisthorchis) –Ileal disease (resection or bypass) –Cystic fibrosis with pancreatic insufficiency Mechanisms=↑ Unconjugated bilirubin in the biliary tree and precipitation of calcium bilirubin salts Morphology = more often radiopaque, black color Clinical( both types)= 70 to 80% of gallstone patients -asymptomatic

Clinical( both types) Symptoms –spasmodic, colicky pain, owing to obstruction of bile ducts by passing stones, –gallbladder obstruction per se generates right upper abdominal pain, Complications= cholecystitis, Empyema, perforation, fistulas, cholangitis, Obstructive cholestasis or pancreatitis and, gallstone ileus, Mucocele other sites of Mucocele?

Cholelithiasis (Gall Stones)

Acute Cholecystitis =acute Inflammation of the gallbladder Cause = MCC gallstone obstruction of the neck or the cystic duct(90% ) Rarely without gallstone obstruction (10%)- severely ill patients (ex. in the postoperative state, severe trauma, severe burns, multisystem organ failure, sepsis, prolonged hyperalimentation or postpartum state) Symptoms of CBD obstruction = acute right upper quadrant or Epigastric pain, mild fever, anorexia, tachycardia, diaphoresis, nausea and vomiting, jaundice Patho –Mechanisms = bile acids (in cholelithiasis pts.)  chemical irritation of gallbladder  inflammatory mediators (lysolecithin, prostaglandins) –in the severely ill patient= direct result of ischemia Gross= enlarged, tense gallbladder, fibrin on serosal covering Course = mild and intermittent or may be a surgical emergency, –self-limited and mortality is less than 1%, or severely ill patient with mortality is higher, complications –cholangitis and sepsis –gallbladder perforation or rupture –enteric fistula formation

Chronic Cholecystitis Causes= MCC from repeated bouts of symptomatic acute cholecystitis or Morphology = fibrosed, contracted gallbladder, GB wall - thickened and gray- white Microscopy =mucosa is preserved –Cholesterolosis =cholesterol-laden macrophages in the lamina propria –Gallstones –Rokitansky -Aschoff sinuses =Mucosal out pouching –porcelain gallbladder = rarely - dystrophic calcification –xanthogranulomatous cholecystitis =Fibrosed, nodular & histiocytic inflammation of gallbladder Clinically = steady or colicky Recurrent attacks of Epigastric or right upper quadrant pain Complications= same as acute

Cholecystitis

Biliary Tract Congenital Anomalies Disorders of gallbladder Acute cholecystitis Chronic cholecystitis Disorders of Extra hepatic bile ducts Choledocholithi asis Ascending cholangitis Biliary atresia Choledochal cysts Tumors

Choledocholithiasis = Stones within the biliary tree in about 10% of patients with cholelithiasis Western nations - almost all stones are derived from the gallbladder (cholesterol stones) Asia- stones are usually primary and pigmented Symptoms arise from –obstruction –pancreatitis –cholangitis –hepatic abscess –secondary biliary cirrhosis –acute calculous cholecystitis Ascending Cholangitis = Bacterial infection of the bile ducts, Common in the setting of Choledocholithiasis, Ascending bacteria (E. coli, Klebsiella and other enterobacteria) enter the biliary tract through the sphincter of Oddi

Extrahepatic Biliary Atresia = Complete obstruction of bile flow unknown cause Mechanism = destruction or absence of all or part of the extrahepatic bile ducts –Normal at birth, later progressive inflammatory destruction Liver changes (same as cholestasis) –marked bile duct proliferation –portal tract edema and fibrosis –Cirrhosis within 3 to 6 months Clinical features= neonatal cholestasis with normal birth weight & postnatal weight gain Treatment= liver transplantation is curative –if untreated, death occurs within 2 years of birth Choledochal Cysts congenital dilations of the CBD in Children < ten yr. age Clinically = Jaundice, recurrent abdominal pain Complications Predispose to = stone formation, stenosis and stricture, pancreatitis, in the adult life = Obstructive biliary complications and bile duct carcinoma

Extrahepatic Biliary Atresia

Biliary Tract Congenital Anomalies Disorders of gallbladder Acute cholecystitis Chronic cholecystitis Disorders of Extra hepatic bile ducts Choledocholithi asis Ascending cholangitis Biliary atresia Choledochal cysts Tumors

Carcinoma of Gall Bladder (GB )= F>M, in their 60's Gallstones coexist in patients in Western nations critical risk factor=Chronic inflammation of GB MC- adenocarcinomas unresectable when discovered prognosis really is BAD Extahepatic Bile Duct Carcinoma = uncommon malignancies of the extrahepatic biliary tree down to the ampulla of Vater Risk factors –choledochal cysts & Calori disease –ulcerative colitis, –Biliary infection with C. sinensis MC- adenocarcinomas, Klatskin's tumors –tumors arising at the confluence of the right and left hepatic bile ducts –notable for slow growth, –sclerosing behavior and –infrequency of distant metastasis –Good Prognosis

Gallbladder- Carcinoma