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    Biliary Tract

    Kimiko Suzue MD, Ph.D.

    Department of Pathology

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    Biliary Tract

    Extrahepatic biliary tract

    Gallbladder

    Cystic duct

    Common hepatic duct

    Common bile duct

    Intrahepatic biliary tract

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    Biliary Disease

    Gallbladder Disorders

    -Cholelithiasis

    -Cholesterolosis

    -Cholecystitis

    Extrahepatic Bile Duct Disorders

    -Choledocholithiasis

    -Cholangitis-Biliary Atresia

    -Choledochal cysts

    Tumors

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    GALLBLADDER

    Storage and

    concentration of

    bile

    Contraction of the

    muscle induced by

    cholecystokinin

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    Gallbladder

    Gallstones (cholelithiasis) Afflict 10% of adult population

    in Western countries

    Costs of management: $6 billion

    a year 20 million patients are estimated

    to have gallstones totalling

    several tons

    Made of cholesterol, bilirubinand calcium salts with different

    concentrations

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    CholelithiasisIn West, >90% are cholesterol stones

    Usually radiolucent (10% radiopaque due to calcium)

    Risk factors:

    -Native Americans-Women > Men

    -Advancing Age (25-30% of people over 80)

    -Estrogenic influence

    Pregnancy, Oral contraceptives-Clofibrate

    -Obesity

    -Gallbladder stasis (neurogenic or hormonal)

    -Hereditary (bile acid metabolism)

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    Cholelithiasis

    Pigmented bilirubin stonesUsually radiopaque (calcium bilirubinate)

    Risk factors:

    -Chronic hemolytic states (increased bilirubin in bile)Sickle cell, hereditary spherocytosis, cardiac valve

    replacement, malaria)

    -Ileal disease (bile salt malabsorption)

    Crohns disease, bowel resection

    -Biliary tract infection/infestation

    Ascaris lumbricoides, Clonorchis sinensis

    -Liver disease (alcoholic cirrhosis)

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    Hepatolithiasis

    Choledocholithiasis

    Cholelithiasis

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    Cholelithiasis

    Asymptomatic: 70%

    Obstruction: Biliary Colic

    Spasmodic right upper quadrant pain

    Symptoms are relieved if stone passesInfection: Cholecystitis, cholangitis

    Acute Pancreatitis

    Gallbladder cancer

    Principal therapeutic modality:

    Laporascopic cholecystectomy (late 1980s)

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    Cholesterolosis

    Aggregates of lipid-containingmacrophages in lamina propria of

    gallbladder

    Debated clinical relevance Strawberry gallbladder

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    Cholesterolosis

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    Polypoid cholesterolosis

    Abundant foamy macrophages forming polypoid

    excrescences

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    Cholecystitis

    General Features

    Present in autopsy series in half of the population

    Female, obese, multiparous, 5th

    and 6th

    decades Acute (Chemical irritation and inflammation)

    Suppurative (Empyema, purulent contents)

    Emphysematous (clostridia and coliforms)

    Gangrenous (Necrotic) Chronic

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    CholecystitisClinical Picture

    Acute: RUQ pain referred to right shoulder

    Rigidity of abdominal wall (RUQ)

    Fever, nausea, vomiting, leukocytosis Jaundice (25% of cases)

    Increased WBC count

    Increased serum alkaline phosphatase (duct damage)

    Chronic: Fatty food intolerance

    Epigastric distress, nausea

    Vague RUQ pain

    Complications: Ca or obstructive jaundice

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    Acute Cholecystitis

    Gross Pathology The gallbladder is enlarged (x2 or x3)

    The gallbladder wall is thickened (x10)

    The serosa is covered by fibrin with subserosalhemorrhages

    The lumen is filled with turbid bile, fibrin or pus

    The mucosa is hyperemic, ulcerated or franklynecrotic

    Gallstones are present in 80% of cases

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    Acute Cholecystitis

    Histology

    Edema and neutrophilic inflammation

    Vascular congestion

    Abscess formation & gangrenous necrosis

    Later neutrophils eosinophils (subacute)

    Ca++

    deposition:porcelain gallbladder

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    Porcelain

    Gallbladder

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    PorcelainGallbladder withStones

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    Chronic Cholecystitis

    Histology Mucosa is preserved or atrophic

    Mononuclear cell infiltration

    Subepithelial and subserosal fibrosis

    Severe fibrosis with replacement of smooth

    muscle

    Outpouchings of mucosal epithelium

    through wall Rokitansky-Aschoff sinuses

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    Choledocholithiasis

    Passage of gallstones into common bile duct occurs in10-15% of patients with cholelithiasis

    Majority are cholesterol stones formed in gallbladder

    Primary calculi arising de novo in ducts are typicallypigmented stones in pts with:

    -Chronic recurrent cholangitis

    -Congenital anomalies of bile ducts (Carolis disease)

    -Dilated, sclerosed or strictured ducts-MDR3 gene defect leading to impaired biliary phospholipid

    secretion

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    Choledocholithiasis

    May remain asymptomatic May pass spontaneously into duodenum

    May present as biliary colic or complications

    -Cholangitis

    -Obstructive jaundice

    -Pancreatitis

    -Secondary biliary cirrhosis

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    Cholangitis

    Acute or chronic

    Bacteria present in 75% of pts with acute

    cholangitis Charcots triad

    Intermittent abdominal pain

    Spiking fevers with chills Jaundice

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    Biliary Atresia

    Complete or partial obstruction of lumen of

    extrahepatic biliary tree within first 3 months of life Perinatal type (80% to >90%)

    -Presumed normal biliary tree is destroyed at birth

    -Jaundice develops within 2 weeks

    -No associated congenital anomalies

    -Etiology unknown(?viral, ?autoimmunity)

    Fetal type (less common)

    -Jaundice at birth or within a day or 2

    -Assoc cardiac and vascular malformations, sinus inversus,polysplenia, midgut malrotation

    -Presumed aberrant intrauterine development of extrahepaticbiliary tree

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    Biliary Aresia

    Histologic features:

    Bile ductular proliferation

    Portal fibrosis

    Bile plugs in bile ducts/ductules

    Parenchymal cholestasis

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    Biliary Atresia

    Type I Disease limited to common bile duct

    Type II Disease limited to hepatic bile ducts

    Type III Obstruction of ducts above porta hepatis

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    Biliary Atresia

    Normal birth weight and postnatal weight gain

    Initially normal stools but become acholic stools

    Present with neonatal cholestasis

    Timing of biopsy Liver biopsy shows nonspecific changes at less than 4 weeks

    Fairly good sensitivity/specificity at 6-8 weeks

    Diagnostic at >8 weeks

    Cirrhosis develops within 3 to 6 months if not recognized

    Kasai procedure for types I and II Liver transplantation for rest

    Without surgery, death within 2 years

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    Choledochal Cysts

    Congenital dilatations of bile duct

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    Choledochal Cysts

    Present most often before 10 yrs of age

    Jaundice, abdominal pain, abdominal mass

    Delayed diagnosis can lead to complications:

    - Pancreatitis

    - Spontaneous perforation

    - Cholelithiasis

    - Cholangitis- Secondary biliary cirrhosis

    - Portal hypertension

    - Carcinoma

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    Carcinoma of the Gallbladder

    Clinical Symptoms Pain 70-80%

    Weight loss 60%

    Jaundice 50%

    Gallstones 50-80%

    Palpable mass 60%

    Ascites 20%

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    Carcinoma of the Gallbladder

    Histologic Types Adenocarcinoma

    Adenosquamous carcinoma

    Squamous cell carcinoma Intracystic or intraductal papillary neoplasm with

    an associated invasive carcinoma

    Mucinous cystic neoplasm with an associated

    invasive carcinoma Undifferentiated carcinoma

    Neuroendocrine neoplasms

    Mesenchymal tumors

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    Dysplasia

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    Adenoca. of Gallbladder

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    Adenoca of Gallbladder

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    Adenoca of Gallbladder

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    Adenoca of Gallbladder

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    Biliary Disease

    Gallbladder Disorders

    -Cholelithiasis

    -Cholesterolosis

    -Cholecystitis

    Extrahepatic Bile Duct Disorders

    -Choledocholithiasis

    -Cholangitis

    -Biliary Atresia

    -Choledochal cysts

    Tumors