Pathology of extrahepatic biliary tract and pancreas MUDr. Helena Sklov

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Transcript of Pathology of extrahepatic biliary tract and pancreas MUDr. Helena Sklov

  • Slide 1
  • Pathology of extrahepatic biliary tract and pancreas MUDr. Helena Sklov
  • Slide 2
  • Normal biliary tract Liver produces 1 liter of bile per day Storage of concentrated bile in gallbladder Release after meal Bile ducts are essential Gallbladder is not
  • Slide 3
  • Pathology of biliary tract Inborn malformations Cholelithiasis Cholecystitis, cholangoitis PBC, PSC Tumors
  • Slide 4
  • Inborn malformations Atresia of biliary ducts: - narrowing or total closure of the whole lenght of extrahepatic bile ducts or segmetally - ethiopathogenesis variable, often unknown (chromosomal defects, viral infections) - symptoms: cholestatis in days-weeks after birth, progresses to biliary cirrhosis - most frequent cause od death from liver disease in early childhood - therapy: surgery, liver transplantation Cyst of choledochus: - mostly females, up to 10 years old - tumor-like mass - symptoms: asymptomatic, pain, icterus - therapy: surgery
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  • Cholesterolosis Strawberry gallbladder Macrophages with cholesterol Clinically insignificant
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  • Cholelithiasis Bile stones in biliary tract 10-20% of adults in developed countries Risk factors: age, female, pregnancy, obesity, bile stasis, hyperlipidemia, biliary infection Cholesterol stones Cholesterol stones Pigment stones Pigment stones Mixed stones Mixed stones
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  • Cholesterol stones 80% of bile stones Pathogenesis: - supersaturation of bile with cholesterol - slow motility of gallbladder - nucleation into cystals - aggregation into stones in mucous Yellow - white, transparent Solitary / a few
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  • Pigment stones Pathogenesis: - inflammation ( acidicity) - hemolysis (overload with bilirubin) Black, hard or brown, soft Factes, multiple
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  • Cholesterol core, pigmented surface Solitary / multiple Mixed stones
  • Slide 10
  • Location Cholecystolithiasis, hepaticolithiasis, choledocholithiasis
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  • Cholecystolithiasis Most common 70% asymptomatic Cholecystitis, hydrops Carcinoma of gallbladder No icterus Stools and urine normal !
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  • Choledocholithiasis Intrahepatic cholestasis Obstructive icterus Pale stools Dark urine Bile colic / strong constant pain Long-term secondary biliary cirrhosis
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  • Complications of cholelithiasis Acute / chronic cholecystitis, cholangoitis Hydrops of gallbladder Empyema of gallbladder Decubital ulcers Perforation, peritonitis Fistula to duodenum, colon Biliary ileus Obstructive icterus (conjugated hyperbilirubinemia) Biliary cirrhosis Pancreatitis Carcinoma of gallbladder
  • Slide 14
  • Acute cholangoitis Suppurative inflammation Infection + stones or tumor May spread to intrahepatic ducts cholangiogenic abscesses sepsis Obstructive icterus Therapy: restore bile drainage, atb
  • Slide 15
  • Chronic cholangoitis Chronic inflammation accompanying obstruction of bile ducts Prominent fibrosis Stenosis Attacks of icterus, sepsis Secondary biliary cirrhosis
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  • Acute cholecystitis Calculous (90%): - obstruction of gallbladder neck or ductus cysticus ischemia, toxic agents from bile aseptic inflammation infection (E.coli) - rarely primarily bacterial (Salmonella typhi) - older women Acalculous: - severly ill patients (surgery, trauma, burns ) - ischemia Suppurative (empyema) Decubital necrosis, ulcer Gangrenous Complications: rupture (esp. acalculous), peritonitis
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  • Chronic cholecystitis Very common Traumatization by bile stones, repeated mild acute cholecystitis, mild infection Wall thicker (fibrosis, hypertrophy of mucosa) or thinner (atrophy) Mucosa metaplasia (gastric, intestinal), dysplasia (low / high grade) Hydrops chronic obstruction, atrophy, fibrosis, clear secretion Porcelain gallbladder calcified Decubital necrosis RF for carcinoma of gallbladder
  • Slide 18
  • Symptoms of cholecystitis Acute: Sudden onset Pain Signs of sepsis Nausea, vomiting Subsides in 1-10 days Relapses more intensively Therapy: cholecystectomy Chronic: Milder course Recurrent atacks of steady or colicky pain Nausea, vominting Intolerance for fatty food Therapy: cholecystectomy Icterus present ONLY if the inflammation spreads to choledochus or common hepatic duct and obstructs them Icterus present ONLY if the inflammation spreads to choledochus or common hepatic duct and obstructs them
  • Slide 19
  • Diseases involving intrahepatic bile ducts Primary biliary cirrhosis (PBC): Autoimmune nonsupurrative destruction of small and medium-sized intrahepatic bile ducts, portal inflammation Fibrosis, biliary cirrhosis Middle-aged women Primary sclerosing cholangitis (PSC): Etiopathogenesis unknown Association with IBD (70% of patients have UC) Inflammation and obliterative fibrosis of intra- and extrahepatic larger bile ducts Dilatation of preserved segments Biliary cirrhosis Middle-aged men
  • Slide 20
  • Tumors of gallbladder Adenoma: - tubular, vilous, tubulovilous - low / high grade dysplasia Adenocarcinoma: - 7th decade, slightly more women - 80% associated with gallstones (chronic inflammation) - Asia: higher % of pyogenic and parasitic diseases (without gallstones) - infiltrating (scirrhotic) / exophytic - fundus, neck - invades directly into bile ducts, liver, peritoneum, LN - metastases: LN, peritoneum, GIT, lungs - symptoms: similar to cholecystitis - diagnosis: late, after cholecystectomy
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  • Adenocarcinoma of extrahepatic bile ducts Uncommon Older age, slightly more men 30% associated with gallstones RF: PSC, UC, choledodal cyst, fluke infection (Asia) Symptoms: painless, progressive jaundice, nausea, vomiting, weight loss, hepatomegaly, palpable gallbladder Diagnosis: early, but tumor is usually not resectable Spreads along bile ducts, metastases in LN Klatskin tumor: - slowly growing sclerotizing tumor from large ducts at liver hilus, rare metastases Carcinoma of the ampula of Vater: - origin may be also in pancreas or duodenum
  • Slide 22
  • Normal pancreas Exocrine component (80%): - Acinar cells cca 20 digestive enzymes (trypsin, chymotripsin, aminopeptidases, lipasis, amylasis, fosfolipasis ) - Ductules, dutcs bicarbonate - Regulation by secretin and cholecystokinin (produced by duodenal mucosa) and n. vagus - 1-3 l of pancreatic juice / day - Protective mechanisms (inactive precursors, inhibitors) Endocrine component: - Langerhans islets (insulin, glucagon, somatostatin)
  • Slide 23
  • Inborn malformations Agenesis - rare, associated with widespread malformations Pancreas divisum - common, failure of fusion of dorsal and ventral part Annular pancreas - ring around duodenum Ectopic pancreas - common in stomach, duodenum Inborn cysts solitary, multiple Polycystosis kidney, liver, pancreas
  • Slide 24
  • Cystic fibrosis = mucoviscidosis, cystic pancreatofibrosis White rase, incidence in CR 1:2500 AR, mutation in CFTR gene 2-5% of people are heterozygots (carriers) Defective ion (chloride) transportation Highly viscous mucous obstruction of ducts in exocrine glands dilatation of terminal ducts and acini (cysts) atrophy fibrosis
  • Slide 25
  • Symptoms of cystic fibrosis Pancreas: malabsorption, steatorrhea, hypovitaminosis, DM (10%) Pancreas: malabsorption, steatorrhea, hypovitaminosis, DM (10%) Intestine: meconium ileus in newborns Bile ducts: cholestasis, biliary cirrhosis Salivary, lacrimal glands: xerostomia, xerophtalmia Epidydimis: infertility Skin: salty sweat (diagnostic) Skin: salty sweat (diagnostic)
  • Slide 26
  • Cystic fibrosis in lungs
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  • Cystic fibrosis Therapy: - substitution of pancreatic enzymes - vaccination, atb, NSAID - mucolytics, oxygenotherapy - lung transplantation Prognosis: - without therapy death in childhood - with advanced therapy between 30-40 years
  • Slide 28
  • Acute pancreatitis Common (Western countries) Etiology: Biliary diseases Biliary diseases Alcoholism Alcoholism (exacerbation of chronic pancreatitis) Others: obstruction of pancreatic ducts, drugs, infections, parasites, ischemia, trauma, genetics Types of acute pancreatitis: Intersticial nonsuppurative accompanies systemic infections (e.g. endemic parotitis) Intersticial suppurative after hemorrhagic necrotizing, hematogenous Hemorrhagic necrotizing Hemorrhagic necrotizing
  • Slide 29
  • Pathogenesis and morphology of acute hemorrhagic necrotizing pancreatitis Obscure Key role of activation of tripsinogen, which then activates other enzymes Autodigestion necrosis and liquefaction of pancreas Lipase fatty tissue necroses Hypocalcemia precipitation of Ca soaps in fat necroses Elastase vascular destruction, hemorrhage Coagulative cascade DIC Enzymes enter blood circulation: - Lipase - fatty tissue necroses in distant sites - Phospholipids surfactant destruction, ARDS Loss of blood volume, electrolyte disturbance, release of cytokines, vasoactive factors - shock
  • Slide 30
  • Symptoms of full-blown acute hemorrhagic necrotizing pancreatitis Severe constant abdominal pain, vomiting Rapidly progresses to shock and circulatory failure, DIC, acute tubular necrosis, ARDS Lab: amylase, lipase in plasma, leucocytosis, hypocalcemia 20% mortality Therapy: total restriction of