MC congenital anomalies = abnormal variants of the gallbladder MC= folded fundus ( Phrygian cap )

Biliary TractBiliary Tract

Congenital AnomaliesCongenital Anomalies

Disorders of gallbladderDisorders of gallbladder

Disorders of Extra hepatic bile ductsDisorders of Extra hepatic bile ducts

TumorsTumors

Acute cholecystitisAcute cholecystitis

Chronic cholecystitisChronic cholecystitis

Choledocholithiasis Choledocholithiasis

Ascending cholangitisAscending cholangitis

Biliary atresia

Choledochal cystsCholedochal cysts

• 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 formation1. supersaturated bile with cholesterol2. gallbladder hypomotility promotes crystal nucleation3. 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





TumorsTumors





Biliary atresia


• 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





TumorsTumors





Biliary atresia


• 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

MC congenital anomalies = abnormal variants of the gallbladder MC= folded fundus ( Phrygian cap )

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