DISEASES OF THE GALL BLADDER AND EXTRAHEPATIC BILE DUCTS Prof Orla Sheils Oct 29 th 2009

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Transcript of DISEASES OF THE GALL BLADDER AND EXTRAHEPATIC BILE DUCTS Prof Orla Sheils Oct 29 th 2009

  • Slide 1
  • DISEASES OF THE GALL BLADDER AND EXTRAHEPATIC BILE DUCTS Prof Orla Sheils Oct 29 th 2009
  • Slide 2
  • GALL STONES (CHOLELITHIASIS) Disorders of the biliary tract affect a significant portion of the worldwide population Majority of cases are attributable to cholelithiasis (gallstones). United States, 20% of persons older than 65 years have gallstones 1 million newly diagnosed cases of gallstones are reported each year. 10% of the Western population. 20% of autopsies.
  • Slide 3
  • Bile exocrine secretion of the liver produced continuously by hepatocytes. contains cholesterol and waste products, bilirubin and bile salts, which aid in the digestion of fats. Half the bile produced runs directly from the liver into the duodenum via a system of ducts, ultimately draining into the common bile duct (CBD). The remaining 50% is stored in the gallbladder. In response to a meal, this bile is released from the gallbladder via the cystic duct, which joins the hepatic ducts from the liver to form the CBD. The CBD courses through the head of the pancreas for approximately 2 cm before passing through the ampulla of Vater into the duodenum.
  • Slide 4
  • Biliary obstruction the blockage of any duct that carries bile from the liver to the gallbladder or from the gallbladder to the small intestine. This can occur at various levels within the biliary system. The major signs and symptoms of biliary obstruction result directly from the failure of bile to reach its proper destination.
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  • TYPES OF GALL STONES Cholesterol mainly cholesterol but also bilirubin, calcium salts, bile salts, proteins. 90% mixed, usually small, hard, multiple, faceted, green or yellow. 10% pure, ie 90% cholesterol, large, solitary, white. - form in the gall bladder. - most common in Western societies. Pigment Black mainly compounds of bilirubin. usually small, irregular in shape, multiple. form in the gall bladder. worldwide but the minority in Western societies. Brown mainly calcium bilirubinate. usually large. form in bile ducts. common in Asia.
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  • Figure 18-50 Cholesterol gallstones. Mechanical manipulation during laparoscopic cholecystectomy has caused fragmentation of several cholesterol gallstones, revealing interiors that are pigmented because of entrapped bile pigments. The gallbladder mucosa is reddened and irregular as a result of coexistent chronic cholecystitis. Downloaded from: Robbins & Cotran Pathologic Basis of Disease (on 5 December 2005 09:32 PM) 2005 Elsevier
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  • Figure 18-51 Pigment gallstones. Several faceted black gallstones are present in this otherwise unremarkable gallbladder from a patient with a mechanical mitral valve prosthesis, leading to chronic intravascular hemolysis. Downloaded from: Robbins & Cotran Pathologic Basis of Disease (on 5 December 2005 09:32 PM) 2005 Elsevier
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  • AETIOLOGY AND PATHOGENESIS Cholesterol gall stones. Supersaturation with cholesterol (lithogenic bile). Due to increase in cholesterol, decrease in bile acids or both. Nucleation - defect in balance between pronucleation factors (eg mucus glycoproteins) and antinucleation factors (eg apolipoproteins. Biliary sludge. Gall bladder hypomotility. Mucus hypersecretion enables crystals to aggregate into stones. Risk Factors: agesex (F:M as 2:1), number of pregnancies, obesity, rapid weight loss,starvation, hyperlipidemia, small bowel disease/resection lipid-lowering drugs, inherited disorders of bile acids, ethnic differences.
  • Slide 9
  • Aetiology & pathogenesis Pigment gall stones. Black - supersaturation with unconjugated bilirubin - calcium bilirubinate. - associated with haemolytic anaemia, cirrhosis, malaria. - bile sterile. Brown - supersaturation with unconjugated bilirubin due to degradation of conjugated bilirubin by bacterial enzymes (beta-glucuronidase). - bile infected and stones contain bacteria. Bile infection usually secondary to stasis in bile ducts. - common in Asia where polymicrobial cholangitis is frequent and biliary obstruction due to flukes (eg Clonorchis, Opisthorchis) are a risk factor.
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  • CLINICAL FEATURES AND COMPLICATIONS 90% are silent. 10% produce symptoms - epigastric or RUQ pain. Complications in the gall bladder:- Acute cholecystitis due to stone blocking the outlet; may lead to empyema, gangrene, perforation, peritonitis. Chronic cholecystitis. Mucocoele. Biliary fistula to duodenum and gall stone ileus. Carcinoma.
  • Slide 11
  • CLINICAL FEATURES AND COMPLICATIONS Complications in the bile duct:- a stone exiting the GB, passes down the bile ducts (choledocholithiasis) and may cause Biliary colic. Extra-hepatic biliary obstruction, jaundice, ascending cholangitis. Pancreatitis. Fibrous stricture. Obstructive jaundice with dilated bile ducts on imaging.
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  • ACUTE CHOLECYSTITIS 90% associated with gall stones, F>M. 10% acalculous, M>F. Calculous cholecystitis. Clinical:- Pain RUQ, referred to shoulder or interscapular. Anorexia, nausea, vomiting. Fever, tenderness RUQ. Neutrophil polymorphonuclear leukocytosis. Pathology:- swollen, tense, possible exudate surface. Microscopically necrosis +/- haemorrhage; later a neutrophil polymorphonuclear infiltrate. Bacterial cultures eventually positive. Complications:- empyema, gangrene, perforation with walled off abscess or generalised peritonitis.
  • Slide 13
  • Acute cholecystitis Acalculous cholecystitis. Following:- Severe burns. Severe trauma. Major surgery. Prolonged labour. Ischaemia in PAN, SLE, embolism. Salmonella typhi & other organisms gas-forming organisms giving rise to bubbles in the wall - emphysematous gall bladder. Insidious clinical features.
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  • Figure 18-52 Acute calculous cholecystitis; the stone was not photographed. Downloaded from: Robbins & Cotran Pathologic Basis of Disease (on 5 December 2005 09:32 PM) 2005 Elsevier
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  • CHRONIC CHOLECYSTITIS Much more common than acute form. F>M. Gall stones in 95%. May be a chemical damage due to supersaturated bile. Bacteria seldom present. Obstruction to the outlet not required. Clinical features: epigastric or RUQ pain; often at night or after a heavy meal. Intolerance to fatty foods. Pathology: thickened firm wall; almost always gallstones in the lumen. Microscopically chronic inflammation and fibrosis. Rokitansky-Aschoff sinuses are outpouchings of mucosal epithelium through the wall and are characteristic. Thought to be due to intra-luminal pressure effect. The wall may become calcified when it is known as a porcelain gall bladder and can be seen on X-ray.
  • Slide 16
  • Figure 18-53 Chronic cholecystitis with cholesterol stones. The gallbladder wall is thickened and gray-white, owing to fibrosis and inflammation. The mucosa is effaced. Multiple faceted cholesterol gallstones are present within the lumen. The exterior of the specimen is black as a result of India ink application. Downloaded from: Robbins & Cotran Pathologic Basis of Disease (on 5 December 2005 09:32 PM) 2005 Elsevier
  • Slide 17
  • BENIGN STRICTURES OF BILE DUCTS Causes. Injury - Surgery. Non-surgical trauma. Post-inflammatory - Passage of a gall stone -> ulcer/fibrosis. Healed pyogenic cholangitis. Spread from adjacent inflammation eg DU. Radiation. Primary sclerosing cholangitis. Results Obstructive jaundice. Secondary biliary cirrhosis.
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  • NEOPLASMS Benign - very rare. Malignant - 3% of malignancies. 5th commonest in GIT. GB to BD as 3:1. Carcinoma. GB - F>M. BD M>F. Peak age 7th decade. Aetiology unknown. Associations - GB - gall stones in 90%. BD 32%. But only 0.5% of patients with gall stones develop carcinoma. BD - parasites (Clonorchis,Opisthorchis). - primary sclerosing cholangitis. - choledochal cyst. Pathology - GB - diffuse or polypoid. An adenocarcinoma. Spread to regional lymph nodes, peritoneum, liver. BD - slow growing desmoplastic adenocarcinoma. Spread to contiguous organs, lymph nodes, liver.
  • Slide 19
  • Figure 18-55 Gallbladder adenocarcinoma. A, The opened gallbladder contains a large, exophytic tumor that virtually fills the lumen. B, Malignant glandular structures are present within a densely fibrotic gallbladder wall. Downloaded from: Robbins & Cotran Pathologic Basis of Disease (on 5 December 2005 09:32 PM) 2005 Elsevier
  • Slide 20
  • Figure 18-55 Gallbladder adenocarcinoma. A, The opened gallbladder contains a large, exophytic tumor that virtually fills the lumen. B, Malignant glandular structures are present within a densely fibrotic gallbladder wall. Downloaded from: Robbins & Cotran Pathologic Basis of Disease (on 5 December 2005 09:32 PM) 2005 Elsevier
  • Slide 21
  • Neoplasms Clinical features and prognosis - GB. A silent area so no symptoms until advanced. When symptomatic, gives rise to upper abdominal pain, anorexia, weight loss. Jaundice a late feature.5 year survival 1%. BD.Give rise to obstructive jaundice earlier. Most die < 1 year from diagnosis. 75% have already spread to contiguous organs or metastasised by the time of diagnosis. Sites. Clinical and pathological findings differ in carcinomas arising at the ampulla of Vater, common bile duct, hepatic duct, or confluence of hepatic ducts at the hilum (Klatskin tumour). Peri-ampullary carcinomas could be from the ampulla, lower CBD (both have bet