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Advisor : dr. Sjaiful Bachri, SpB Melissa L. Thenata Fakultas Kedokteran Universitas Tarumanagara

Hepatobiliary Disease

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Text of Hepatobiliary Disease

  • Advisor : dr. Sjaiful Bachri, SpB

    Melissa L. Thenata

    Fakultas Kedokteran Universitas Tarumanagara

  • Liver Structure

    Largest gland in the body

    (1.5 Kg)

    Under the diaphragm, within

    the rib cage in the upper

    right quadrant of the


  • Liver Structure

    4 lobes: major (left and right),

    minor (caudate and quadrate)

    Ducts: common hepatic, cystic

    from gall bladder, common bile

    choledochus (join pancreatic

    duct at hepatopancreatic


  • Liver Structure

    Liver lobules hexagonal structures consisting of hepatocytes

    Hepatocytes radiate outward from a central vein

    At each of the six corners of a lobule is a portal triad

    Liver sinusoids

  • Liver Structure

    Hepatocytes produce bile

    Bile flows through canals

    called bile canaliculi to a

    bile duct

    Bile ducts leave the liver

    via the common hepatic


  • Liver Function

    Regulating homeostasis of carbohydrate, lipid and amino

    acid metabolism.

    Storing nutrients such as glycogen, fats and vitamin B12, A

    and K.

    Producing and secreting plasma proteins and lipoproteins,

    including clotting factors and acute phase proteins.

  • Liver Function

    Synthesizing and secreting bile salts for lipid digestion.

    Detoxifying and excreting bilirubin, other endogenous waste

    products and exogenous metal ions, drugs and toxins


    Clearing toxins and infective agents from the portal venous

    blood whilst maintain systemic immune tolerance to antigens

    in the portal circulation.

  • Gall Bladder Structure

    Thin-walled green muscular sac

    On the inferior surface of the liver

    Stores bile that is not immediately needed for digestion

    When the muscular wall of the gallbladder contracts bile is

    expelled into the bile duct

  • Gall Bladder Structure

  • Gall Bladder Function

    Stores 60 mL of bile, released when food containing fat

    enters the digestive tract.

    The bile, produced in the liver, emulsifies (breaks down) fats

    and neutralizes acids in partly digested food.

  • Biliary Atresia

    Obliteration or discontinuity of the extrahepatic biliary system,

    resulting in obstruction to bile flow, in the first few weeks of life.

    Inflammatory process from an unknown cause affects the bile duct

    in the newborn infant.

    Destruction of the extrahepatic bile ducts, causing obstructive

    jaundice and liver failure.

    Kasai procedure, surgical correction of this abnormality before 8

    weeks of age produces the best outcome.

  • Choledochal Cyst

    Cystic dilatation of the intra- or extrahepatic ducts is a rare

    condition, usually presenting before the age of 16 years.

    Symptoms : cholangitis, pancreatitis, stone formation and jaundice.

    Infants may occasionally present with an abdominal mass.

    The cause of this condition is debated.

  • Choledochal Cyst

    Cysts are classified according

    to their site and shape,

    although 80% are fusiform

    abnormalities of the

    extrahepatic bile duct. Type

    II cysts are extremely rare.

  • Choledochal Cyst

    Treated by surgical excision

    of the cyst with the

    formation of a roux-en-Y

    anastomosis to the biliary


  • Hepatobiliary Trauma

    The liver is the most commonly injured solid abdominal organ,

    despite its relative protected location.

    Treatment of traumatic liver injuries is based on patient

    physiology, mechanism and degree of injury, associated

    abdominal and extra-abdominal injuries and local expertise.

  • Hepatobiliary Trauma

    Liver Organ Injury Scale

    Grade Description

    I Hematoma Subcapsular,

  • Hepatobiliary Trauma

    Liver Organ Injury Scale

    Grade Description

    IV Hematoma Parenchymal disruption involving 25% - 75% of hepatic lobe or 1-3 Couinaud segments within a single lobe

    V Laceration Parenchymal disruption involving >75% of hepatic lobe >3 Couinaud segments within a single lobe

    Vascular Juxtahepatic venous injuries; ie. Retrohepatic vena cava/central major hepatic vein

    VI Hepatic avulsion

  • Management of Hepatic Trauma

  • Gall Bladder Cancer

    Highly aggressive malignancy, usually presents at an advanced,

    incurable stage, 5th most common gastrointestinal tumor

    The median survival is less than 6 months after diagnosis

    Women : Men = 2 6 : 1

    The incidence steadily increases with age

    Risk factor : obesity, a high-carbohydrate diet, smoking, and

    alcohol use

  • Gall Bladder Cancer

    Gall bladder cancer arises in the setting of chronic

    inflammation. In the vast majority of patients (>75%), the

    source of this chronic inflammation is cholesterol gallstones.

    10-25% is caused by calcification of the gallbladder (porcelain


    Most common type of gallbladder cancer is adenocarcinoma.

    Gallbladder cancer can spread by direct invasion through the

    gallbladder wall into the liver or peritoneal cavity.

  • Gall Bladder Cancer

    The symptoms of gallbladder cancer overlap with the

    symptoms of gallstones and biliary colic. Abdominal pain may

    be of a more diffuse and persistent nature than the classic

    right upper quadrant pain of gallstone disease.

    Jaundice, anorexia, and weight loss often indicate more

    advanced disease.

  • Gall Bladder Cancer

    Table 2. Summary of the Tumor-Node-Metastasis (TNM) Staging System*

    Stage Description






    Mucosal or muscular invasion (T1N0M0)

    Perimuscular-tissue invasion (T2N0M0)

    Transmural invasion, liver invasion < 2 cm; lymph node metastasis to

    hepatoduodenal ligament (T3N0M0, T13 N1M0)

    Liver invasion > 2 cm (T4N0M0, T4N1M0)

    Distant nodal (outside porta hepatis) or hematogenous metastasis

    (TxN2M0, TxNxM1)

  • Gall Bladder Cancer

    The work-up for right-upper-quadrant pain or biliary colic

    generally starts with an ultrasound examination of the


    Laboratory tests should include liver function tests and

    hematocrit. Advanced cases may demonstrate anemia and

    elevated alkaline phosphatase and bilirubin.

    Tumor markers, CEA and CA 19-9, may be of help and should

    be considered if gallbladder cancer is suspected.

  • Gall Bladder Cancer

    Further radiologic work-up such as CT-scan, MRI, or needle

    biopsy are indicated if gallbladder cancer is suspected.

    The most common and most effective treatment is surgical

    removal of the gallbladder (cholecystectomy) with part of liver

    and lymph node dissection.

    Chemotherapy has not shown significant activity in gallbladder

    carcinoma. Typically, 5-fluorouracil (5-FU) has been used with

    response rates of 10-24% in advanced disease.

  • Gall Bladder Cancer

    Gemcitabine has shown activity in gallbladder cancer. There is

    an increased response rate with gemcitabine combination with

    cisplatinum and capecitabine.

    Currently, no clearly defined standard exists for chemotherapy

    in gallbladder cancer. Patients should be encouraged to

    participate in clinical trials.

  • Cholangiocarcinoma

    Cholangiocarcinomas are malignancies of the biliary duct system

    that may originate in the liver and extrahepatic bile ducts, which

    terminate at the ampulla of Vater.

    The etiology of most bile duct cancers remains undetermined.

    However, one of the most commonly recognized risk factors is

    primary sclerosing cholangitis.

    Cholangiocarcinoma arises from the intrahepatic or extrahepatic

    biliary epithelium. More than 90% are adenocarcinomas.

  • Cholangiocarcinoma

    Cholangiocarcinomas tend to grow slowly and to infiltrate the

    walls of the ducts, dissecting along tissue planes.

    Local extension occurs into the liver, porta hepatis, and regional

    lymph nodes of the celiac and pancreaticoduodenal chains.

    Symptoms of cholangiocarcinoma include jaundice, clay-colored

    stools, bilirubinuria (dark urine), pruritus, weight loss, and

    abdominal pain.

  • Cholangiocarcinoma

    The diagnosis and staging of cholangiocarcinoma require a

    multimodality approach involving laboratory, radiologic,

    endoscopic, and pathologic analysis.

    The most studied serum tumor markers are the CA 19-9, is

    currently the most commonly used tumor marker for


    Surgical resection with curative intent is the treatment of

    choice for extrahepatic cholangiocarcinoma.

  • Gallstones

    Two major types of gallstones: cholesterol and

    pigment stones. Cholesterol gallstones contain50%

    cholesterol monohydrate. Pigment stones have 20%

    cholesterol and are composed primarily of calcium


    Predisposing factors include demographic/genetics,

    obesity, weight loss, female sex hormones, age, ileal

    disease, pregnancy, type IV hyperlipidemia, and


  • Gallstones

    Many gallstones are

    silent. Symptoms occur

    when stones produce

    inflammation or

    obstruction of the cystic

    or common bile ducts.

  • Gallstones

    Major symptoms: (1) biliary colica severe steady ache in the

    RUQ or epigastrium that begins suddenly; often occurs 3090

    min after meals, lasts for several hours, and occasionally

    radiates to the right scapula or back; (2) nausea, vomiting.

    Physical exam may be normal or show epigastric or RUQ


    Mild and transient elevations in bilirubin [85 mol/L (5 mg/dL)]

    accompany biliary colic.

  • Gallstones

    Only 10% of cholesterol gallstones are

    radiopaque. USG is best diagnostic test.

    Elective cholecystectomy should be

    reserved for: (1) symptomatic patients;

    (2) persons with previous complications

    of cholelithiasis; and (3) presence of an

    underlying condition predisposing to an

    increased risk of complications (calcified

    or porcelain gallbladder).

  • Gallstones

    Patients with gallstones 3 cm or with an anomalous gallbladder

    containing stones should be considered for surgery.

    Laparoscopic cholecystectomy is minimally invasive and is the

    procedure of choice for most patients undergoing elective


    Oral dissolution agents (ursodeoxycholic acid) partially or

    completely dissolve small radiolucent stones in 50% of selected

    pts within 624 months.

  • Gallstones

    Extracorporeal shockwave lithotripsy followed by medical

    litholytic therapy is effective in selected patients with solitary

    radiolucent gallstones. Because of the frequency of stone

    recurrence and the effectiveness of laparoscopic surgery, the

    role of oral dissolution therapy and lithotripsy has been

    reduced to selected patients who are not candidates for

    elective cholecystectomy.

  • Acute Cholecystitis

    Acute inflammation of the gallbladder usually caused by cystic

    duct obstruction by an impacted stone.

    90% calculous; 10% acalculous.

    Acalculous cholecystitis associated with higher complication rate

    and associated with acute illness (i.e., burns, trauma, major

    surgery), fasting, hyperalimentation leading to gallbladder stasis,

    vasculitis, carcinoma of gallbladder or common bile duct, some

    gallbladder infections but in > 50% of cases an underlying

    explanation is not found.

  • Acute Cholecystitis

    Signs and symptoms :

    Attack of bilary colic (RUQ or epigastric pain), progressively worsens

    Nausea, vomiting, anorexia


    Examination typically reveals RUQ tenderness

    Palpable RUQ mass found in 20% of patients

    Murphys sign is present when deep inspiration or cough during

    palpation of the RUQ produces increased pain or inspiratory arrest.

  • Acute Cholecystitis

    Laboratory : Mild leukocytosis; serum bilirubin, alkaline

    phosphatase, and AST may be mildly elevated.

    Imaging : Ultrasonography is useful for demonstrating

    gallstones and occasionally a phlegmonous mass surrounding

    the gallbladder. Radionuclide scans may identify cystic duct


  • Acute Cholecystitis

    No oral intake, nasogastric suction, IV fluids and electrolytes,

    analgesia (meperidine or NSAIDS), and antibiotics

    (ureidopenicillins, ampicillin sulbactam, third-generation

    cephalosporins; anaerobic coverage should be added if

    gangrenous or emphysematous cholecystitis is suspected;

    consider combination with aminoglycosides in diabetic patient

    or others with signs of gram-negative sepsis).

  • Acute Cholecystitis

    Acute symptoms will resolve in 70% of patient.

    Optimal timing of surgery depends on patient stabilization and

    should be performed as soon as feasible.

    Urgent cholecystectomy is appropriate in most patients with a

    suspected or confirmed complication.

    Delayed surgery is reserved for patients with high risk of

    emergent surgery and where the diagnosis is in doubt.

  • Chronic Cholecystitis

    Chronic inflammation of the gallbladder; almost always

    associated with gallstones. Results from repeated

    acute/subacute cholecystitis or prolonged mechanical

    irritation of gallbladder wall.

    May be asymptomatic for years, may progress to symptomatic

    gallbladder disease or to acute cholecystitis, or present with


  • Chronic Cholecystitis

    Laboratory tests are usually normal.

    Ultrasonography preferred; usually shows gallstones within a

    contracted gallbladder.

    Surgery indicated if patient is symptomatic.

  • Liver Infections Pyogenic Abscess

    Pyogenic or bacterial abscess may be caused by several factors.

    Infections may arise from the biliary tract, portal vein and hepatic

    artery or by direct extension.

    Symptoms : pyrexia and rigours associated with right upper

    quadrant pain, general malaise and anorexia.

    Examination may reveal tender hepatomegaly. A pleural effusion

    may be present. Occasionally, hypotension and cardiovascular

    collapse may be the presenting symptoms.

  • Liver Infections Pyogenic Abscess

    Laboratory tests : hyperbilirubinemia, raised alkaline

    phosphatase and transaminase levels, blood cultures are

    frequently positive, leucocytosis.

    USG / CT scan abdomen : to determine the size, characteristics,

    number and anatomical location of the liver abscesses.

    Chest X-ray : elevated hemidiaphragm or a pleural effusion.

    ERCP or a colonoscopy : to determine the cause of pyogenic

    liver abscesses.

  • Liver Infections Pyogenic Abscess

    Treatment :

    Analgesics and attention to adequate nutrition and hydration

    Antimicrobial therapy

    Drainage of the abscess

    Frequent clinical, biochemical, microbial and radiological

    follow-up is required to assess progress and detect relapses

  • Liver Infections Amoebic Liver Abscess

    Amoebic infestation is caused by the organism Entamoeba


    Transmission is by passage of cysts in the stool, the cysts then

    being ingested orally as a result of poor hygienic practices.

    Risk factors include malnutrition, depressed immunity and low

    socioeconomic status.

    Complications of amoebic abscess include rupture into the

    peritoneal cavity or hollow viscus such as colon or stomach.

  • Liver Infections Amoebic Liver Abscess

    The onset of the disease may be sudden or gradual.

    The most common symptoms : right upper quadrant pain,

    general malaise, weight loss, pyrexia and sweating.

    Signs : tender hepatomegaly and, occasionally, jaundice.

    Full blood examination : leukocytosis and eosinophilia.

    Amoebic serology and stool cultures are usually positive.

    The antibiotic of choice is metronidazole.

  • Benign Liver Tumors - Adenoma

    Hepatocellular adenomas occur most commonly in women in

    the third or fourth decades who take birth control pills.

    The major concern is their tendency to rupture with massive

    haemorrhage; therefore, this condition must be considered in

    young women presenting with abdominal pain, signs of

    hypovolaemic shock and features of haemoperitoneum.

    After resuscitation, the treatment is resection of the affected

    liver segment.

  • Benign Liver Tumors Focal Nodular Hyperplasia

    Focal nodular hyperplasia (FNH) is not a true neoplasm but is

    probably due to a fibrous reaction to vessel ingrowth.

    It is most common in young women.

    It appears as a nodular firm vascular mass.

    There may be symptoms of right upper quadrant pain.

    No specific treatment is required and the main purpose of

    management is to distinguish the lesion from neoplasms.

  • Hepatocellular Cancer (Hepatoma)

    Worldwides most common tumor.

    Male : female = 4 : 1; tumor usually develops in cirrhotic liver

    in persons in fifth or sixth decade.

    High incidence in Asia and Africa is related to etiologic

    relationship between this cancer and hepatitis B and C


  • Hepatocellular Cancer (Hepatoma)

    Aflatoxin exposure contributes to etiology and leaves a molecular

    signature, a mutation in codon 249 of the gene for p53.

    Surgical resection or liver transplantation is therapeutic option but

    rarely successful.

    Hepatitis B vaccine prevents the disease. Interferon may prevent

    liver cancer in persons with chronic active hepatitis C disease and

    possibly in those with hepatitis B.

    Ribivarin / interferon (IFN) is most effective treatment of chronic

    hepatitis C.