Gallbladder

• –      Pear-shaped sac, 7 to 10 cm long, average capacity of 30 to 50 mL.

• –      LOCATION: fossa on the inferior surface of the liver.

• –      4 anatomic areas:

• –        Fundus - rounded, blind end that normally extends 1 to 2 cm beyond the liver’s margin.

•                          contains most of the smooth muscles of the organ, in contrast to the body, which is the main storage area and contains most of the elastic tissue.

• –        Body (corpus)- extends from the fundus and tapers into the neck, a funnel-shaped area that connects with the cystic duct. The neck usually follows a gentle curve, the convexity of which may be enlarged to form the infundibulum or Hartmann’s pouch.

• –        Neck - lies in the deepest part of the gallbladder fossa and extends into the free portion of the hepatoduodenal ligament

•  

• –      Cystic artery that supplies the gallbladder is usually a branch of the right hepatic artery

• –      Venous return is carried either through small veins that enter directly into the liver or, rarely, to a large cystic vein that carries blood back to the portal vein. Gallbladder lymphatics drain into nodes at the neck of the gallbladder.

• –      Nerves of the gallbladder arise from the vagus and from sympathetic branches that pass through the celiac plexus.

•  

Biliary tree

• –       consist of the right and left hepatic ducts, the common hepatic duct, the cystic duct, and the common bile duct

• –       The common bile duct enters the second portion of the duodenum through a muscular structure, the sphincter of Oddi.

•  

• –       The left hepatic duct is longer than the right and has a greater propensity for dilatation as a consequence of distal obstruction.

• –        The two ducts join to form a common hepatic duct, close to their emergence from the liver. The common hepatic duct is 1 to 4 cm in length and has a diameter of approximately 4 mm. It lies in front of the portal vein and to the right of the hepatic artery. The common hepatic duct is joined at an acute angle by the cystic duct to form the common bile duct

•   The sphincter of Oddi, a thick coat of circular smooth muscle, surrounds the common bile duct at the ampulla of Vater.

Physiology (Bile Formation)

• –       The liver produces bile continuously and excretes it into the bile canaliculi.

• –       The normal adult consuming an average diet produces within the liver 500 to 1000 mL of bile a day.

• –       The secretion of bile is responsive to neurogenic, humoral, and chemical stimuli. Vagal stimulation increases secretion of bile, whereas splanchnic nerve stimulation results in decreased bile flow.

• –       Hydrochloric acid, partly digested proteins, and fatty acids in the duodenum stimulate the release of secretin from the duodenum that, in turn, increases bile production and bile flow.

• –       Bile flows from the liver through to the hepatic ducts, into the common hepatic duct, through the common bile duct, and finally into the duodenum. With an intact sphincter of Oddi, bile flow is directed into the gallbladder.

• –       Bile is mainly composed of water, electrolytes, bile salts, proteins, lipids, and bile pigments. Sodium, potassium, calcium, and chlorine have the same concentration in bile as in plasma

• –       The primary bile salts, cholate and chenodeoxycholate, are synthesized in the liver from cholesterol.

• –       Bile salts are excreted into the bile by the hepatocyte and aid in the digestion and absorption of fats in the intestines.

• –       Enterohepatic circulation: Bile acid pool is reabsorbed and returned via the portal venous system to the liver

Function of Gallbladder

• –       The gallbladder, the bile ducts, and the sphincter of Oddi act together to store and regulate the flow of bile. The main function of the gallbladder is to concentrate and store hepatic bile and to deliver bile into the duodenum in response to a meal

Sphincter of Oddi

• –     Regulates flow of bile (and pancreatic juice) into the duodenum, prevents the regurgitation of duodenal contents into the biliary tree, and diverts bile into the gallbladder.

Diagnostic studies

• –       Blood test ( cbc , bilirubin , alk phos )

• –       Ultrasonography ( gallstone ? CBD stone? Liver? Tumor?

• –       Oral Cholecystography

• –       Biliary Radionuclide Scanning (HIDA Scan)

• –       Computed Tomography

• –       Percutaneous Transhepatic Cholangiography

• –       Magnetic Resonance Imaging /MRCP

• –       Endoscopic Ultrasound

Ultrasound

• Ultrasound examination has a sensitivity of 89% and specificity of 88% on the gallstones.
  
  

HIDA SCAN

• –      For a HIDA scan, also known as cholescintigraphy or hepatobiliary scintigraphy, a radioactive tracer is injected into a vein in your arm. The tracer travels through your bloodstream to your liver, where the bile-producing cells take it up. The tracer then travels with the bile into your gallbladder and through your bile ducts to your small intestine.

• –      A nuclear medicine scanner (gamma camera) tracks the flow of the tracer from your liver into your gallbladder and small intestine and creates computer images.

Oral cholecystogram

• –       For an OCG, the patient takes iodine-containing tablets by mouth for one night or two nights in a row. The iodine is absorbed from the intestine into the bloodstream, removed from the blood by the liver, and excreted by the liver into the bile. The iodine, together with the bile, is highly concentrated in the gallbladder. Iodine is used in an OCG because it is dense and radioopague (stops x-rays). It outlines the gallstones that are radiolucent (x-rays pass through them) and that are usually invisible on x-ray.

Gallstone Disease
(Prevalence and Incidence)

• –     Autopsy reports have shown a prevalence of gallstones from 11% to 36%.24 The prevalence of gallstones is related to many factors, including age, gender, and ethnic background.

• –     Certain conditions predispose to the development of gallstones. Obesity, pregnancy, dietary factors, Crohn’s disease, terminal ileal resection, gastric surgery, hereditary spherocytosis, sickle cell disease, and thalassemia are all associated with an increased risk of developing gallstones. Women are three times more likely to develop gallstones

Gallstone Formation

• –      Gallstones form as a result of solids settling out of solution. The major organic solutes in bile are bilirubin, bile salts, phospholipids, and cholesterol. Gallstones are classified by their cholesterol content as either cholesterol stones or pigment stones. Pigment stones can be further classified as either black or brown.

• –      A. Cholesterol Stones

• –      B. Pigment Stones ( black /brown)

• Cholesterol Stones

  • –     Uncommon and account for <10% of all stones

  • –     Single large stones with smooth surfaces

  • –     Usually multiple, of variable size, and may be hard and faceted or irregular, mulberry-shaped, and soft

  • –     Colors range from whitish yellow and green to black

• Pigment Stones
  ( black / brown)

  • –       Usually dark because of the presence of calcium bilirubinate.

  • –       Black pigment stones are usually small, brittle, black, and sometimes spiculated.

  • –       Formed by supersaturation of calcium bilirubinate, carbonate, and phosphate, most often secondary to hemolytic disorders such as hereditary spherocytosis and sickle cell disease, and in those with cirrhosis.

  • –       In Asian countries such as Japan, black stones account for a much higher percentage of gallstones than in the Western hemisphere.

  • –     Brown stones are usually <1 cm in diameter, brownish yellow, soft, and often mushy.

  • –     May form either in the gallbladder or in the bile ducts

  • –      secondary to bacterial infection caused by bile stasis

Chronic Cholecystitis
(Biliary Colic)

• –     Characterized by recurrent attacks of pain, often inaccurately labeled biliary colic. The pain develops when a stone obstructs the cystic duct, resulting in a progressive increase of tension in the gallbladder wall.

• Clinical Presentation

  • –       The chief symptom associated with symptomatic gallstones is pain.

  • –       Pain is constant and increases in severity over the first half hour or so and typically lasts 1 to 5 hours.

  • –       Location: Epigastrium or right upper quadrant and frequently radiates to the right upper back or between the scapulae.

  • –       Pain is severe and comes on abruptly, typically during the night or after a fatty meal.

  • –      Associated with nausea and sometimes vomiting.

  • –      Pain is episodic

  • –       Discrete attacks of pain, between which they feel well.

  • –      Physical examination may reveal mild right upper quadrant tenderness during an episode of pain. If the patient is pain free, the physical examination is usually unremarkable

  • –       Atypical presentation of gallstone disease is common.

  • –       Pain may be located primarily in the back or the left upper or lower right quadrant. Bloating and belching associated with the attacks of pain.

  • –       When the pain lasts >24 hours, an impacted stone in the cystic duct or acute cholecystitis should be suspected.

  • –       An impacted stone without cholecystitis will result in what is called hydrops of the gallbladder.

  • –       The bile gets absorbed, but the gallbladder epithelium continues to secrete mucus, and the gallbladder becomes distended with mucinous material.

  • –        The gallbladder may be palpable but usually is not tender

• Diagnosis

  • –       Abdominal ultrasound- standard diagnostic test for gallstones

  • –       Gallstones are occasionally identified on abdominal radiographs or CT scans.

  • –       In these cases, if the patient has typical symptoms, an ultrasound of the gallbladder and the biliary tree should be added before surgical intervention.

  • –       Cholesterolosis is caused by the accumulation of cholesterol  in the gallbladder mucosa, either locally or as polyps.

  • –         “strawberry gallbladder.”

  • –       Adenomyomatosis or cholecystitis glandularis proliferans is characterized on microscopy by hypertrophic smooth muscle bundles and by the ingrowths of mucosal glands into the muscle layer (epithelial sinus formation).

  • –       In symptomatic patients, cholecystectomy is the treatment of choice

• Management

  • –       Elective laparoscopic /open cholecystectomy.

  • –       While waiting for surgery, or if surgery has to be postponed, the patient should be advised to avoid dietary fats and large meals.

  • –       Diabetic patients with symptomatic gallstones should have a cholecystectomy promptly, as they are more prone to develop acute cholecystitis that is often severe.

  • –       Pregnant women with symptomatic gallstones who cannot be managed expectantly with diet modifications can safely undergo laparoscopic cholecystectomy during the second trimester.

Acute Cholecystitis (Pathogenesis)

• –       Acute cholecystitis is secondary to gallstones in 90% to 95% of cases.

• –       Obstruction of the cystic duct by a gallstone is the initiating event that leads to gallbladder distention, inflammation, and edema of the gallbladder wall.

• –       Initially, acute cholecystitis is an inflammatory process, probably mediated by the mucosal toxin lysolecithin, a product of lecithin, as well as bile salts and platelet-activating factor.

• –       Secondary bacterial contamination is documented in 15% to 30% of patients undergoing cholecystectomy for acute uncomplicated cholecystitis.

•  

•  

• –       In acute cholecystitis, the gallbladder wall becomes grossly thickened and reddish with subserosal hemorrhages.

• –       Pericholecystic fluid often is present.

• –       The mucosa may show hyperemia and patchy necrosis.

• –       In severe cases, about 5% to 10%, the inflammatory process progresses and leads to ischemia and necrosis of the gallbladder wall.

• –       More frequently, the gallstone is dislodged and the inflammation resolves.

•  

• Acute Cholecystitis (Pathogenesis)

  • –       When the gallbladder remains obstructed and secondary bacterial infection supervenes, an acute gangrenous cholecystitis develops, and an abscess or empyema forms within the gallbladder.

  • –       Rarely, perforation of ischemic areas occurs.

  • –       The perforation is usually contained in the subhepatic space by the omentum and adjacent organs.

  • –       When gas-forming organisms are part of the secondary bacterial infection, gas may be seen in the gallbladder lumen and in the wall of the gallbladder on abdominal radiographs and CT scans, an entity called an emphysematous gallbladder

• Clinical Manifestations

  • –       About 80% of patients with acute cholecystitis give a history compatible with chronic cholecystitis.

  • –       Acute cholecystitis begins as an attack of biliary colic, but in contrast to biliary colic, the pain does not subside; it is unremitting and may persist for several days.

  • –       The pain is typically in the right upper quadrant or epigastrium and may radiate to the right upper part of the back or the interscapular area.

  • –       It is usually more severe than the pain associated with uncomplicated biliary colic.

  • –       The patient is often febrile, complains of anorexia, nausea, and vomiting, and is reluctant to move, as the inflammatory process affects the parietal peritoneum.

  • –       On physical examination, focal tenderness and guarding are usually present in the right upper quadrant.

  • –       A mass, the gallbladder and adherent omentum, is occasionally palpable; however, guarding may prevent this.

  • –       A Murphy’s sign, an inspiratory arrest with deep palpation in the right subcostal area, is characteristic of acute cholecystitis.

  • –       A mild to moderate leukocytosis (12,000–15,000 cells/mm3) is usually present. A high WBC count (above 20,000) is suggestive of a complicated form of cholecystitis such as gangrenous cholecystitis, perforation, or associated cholangitis.

  • –       Serum liver chemistrie sare usually normal, but a mild elevation of serum bilirubin, <4 mg/mL, may be present along with mild elevation of alkaline phosphatase, transaminases, and amylase.

  • –       Severe jaundice is suggestive of common bile duct stones or obstruction of the bile ducts by severe pericholecystic inflammation secondary to impaction of a stone in the infundibulum of the gallbladder that mechanically obstructs the bile duct (Mirizzi’s syndrome).

  • –       The differential diagnosis for acute cholecystitis includes a peptic ulcer with or without perforation, pancreatitis, appendicitis, hepatitis, perihepatitis (Fitz-Hugh–Curtis syndrome), myocardial ischemia, pneumonia, pleuritis, and herpes zoster involving the intercostal nerve

• Diagnostic

  • –       Ultrasonography is the most useful radiologic test for diagnosing acute cholecystitis. It has a sensitivity and specificity of 95%.

  • –       Shows the thickening of the gallbladder wall and the pericholecystic fluid

  • –       Sonographic Murphy’s sign

  • –         Focal tenderness over the gallbladder when compressed

  • –        suggestive of acute cholecystitis.

• Treatment

  • –       Acute cholecystitis: IV fluids, antibiotics, and analgesia.

  • –        Third generationcephalosporin with good anaerobic coverage or a second-generation cephalosporin combined with metronidazole is a typical regimen.

  • –        For patients with allergies to cephalosporins, an aminoglycoside with metronidazole

  • –       Cholecystectomy - definitive treatment

  • –       Laparoscopic cholecystectomy - procedure of choice

  • –       When patients present late, after 3 to 4 days of illness, or if they are unfit for surgery, they can be treated with antibiotics with laparoscopic cholecystectomy scheduled for approximately 2 months later.

Choledocholithiasis

• –       Common bile duct stones may be small or large and single or multiple, and are found in 6% to 12% of patients with stones in the gallbladder.

• –        The incidence increases with age. About 20% to 25% of patients above the age of 60 with symptomatic gallstones have stones in the common bile duct as well as in the gallbladder.

• –       The vast majority of ductal stones in Western countries are formed within the gallbladder and migrate down the cystic duct to the common bile duct. These are classified as secondary common bile duct stones, in contrast to the primary stones that form in the bile ducts

• –       The secondary stones are usually cholesterol stones, whereas the primary stones are usually of the brown pigment type.

• –       The primary stones are associated with biliary stasis and infection and are more commonly seen in Asian populations.

• –       The causes of biliary stasis that lead to the development of primary stones include biliary stricture, papillary stenosis, tumors, or other (secondary) stones.

• Clinical Manifestations

  • –       May be silent and often are discovered incidentally. 

  • –       May manifest with cholangitis or gallstone pancreatitis

  • –        The pain caused by a stone in the bile duct

  • –        Nausea and vomiting are common.

  • –       Physical examination may be normal, but mild epigastric or right upper quadrant tenderness as well as mild icterus are common.

  • –       The symptoms may also be intermittent, such as pain and transient jaundice caused by a stone that temporarily impacts the ampulla but subsequently moves away, acting as a ball valve.

• –       Elevation of serum bilirubin, alkaline phosphatase, and transaminases

• diagnostics

  • Ultrasound

  • CT scan

  • MRCP (MRI)

  • ERCP

  • MRCP

    • MRCP test is a specialized MRI exam that evaluates the hepatiobiliary and pancreatic systems, including the liver, gallbladder, bile ducts, pancreas and pancreatic duct. MRCP stands for Magnetic Resonance Cholangiopancreatography

    • THE BEST IMAGING FOR BILIARY TREE

• Treatment

  • For patients with symptomatic gallstones and suspected common bile duct stones: preoperative endoscopic cholangiography  ( ERCP) or an intraoperative cholangiogram

  • If an endoscopic cholangiogram reveals stones, sphincterotomy and ductal clearance of the stones is appropriate, followed by a laparoscopic / open  cholecystectomy

  • An intraoperative cholangiogram at the time of cholecystectomy will also document the presence or absence of bile duct stones

  • Laparoscopic common bile duct exploration via the cystic duct or with formal choledochotomy allows the stones to be retrieved in the same setting

  • Open common bile duct exploration is an option if the endoscopic method has already been tried or is, for some reason,not feasible.

  • If a choledochotomy is performed, a T tube is left in place.

  • Roux-en-Y choledochojejunostomy is rarely done

  • ERCP ( DIAGNOSTIC AND THERAPEUTIC!

  • Intraoperative cholangiogram

• Filling defects

  • Filling defect

  • no duodenal / minimal egress  of contrast

  • Choledochotomy / T Tube insertion

  • Treatment

    • ERCP

    • Choledochotomy

    • CBD EXPLORATION

    • CHOLEDOCHOSCOPY

    • T Tube insertion

    • choledochoscopy

    • Hepaticojejunostomy (LEAST DONE)

Cholangitis

• –       Ascending bacterial infection in association with partial or complete obstruction of the bile ducts.

• –       Hepatic bile is sterile

• –       Mechanical hindrance to bile flow facilitates bacterial contamination.

• –       Biliary bacterial contamination alone does not lead to clinical cholangitis; the combination of both significant bacterial contamination and biliary obstruction is required for its development.

• –       Gallstones are the most commoncause of obstruction in cholangitis

• COMPLICATIONS OF CHOLANGITIS

  • –       When the bile ducts get inflamed or blocked, bile can back up into the liver. This can lead to liver damage and other problems. Some types of cholangitis are mild. Other kinds can be serious and life threatening.

  • –       There are two main types of cholangitis:

  • •         Chronic cholangitis happens slowly over time. It can cause symptoms for years.

  • •         Acute cholangitis happens suddenly. It can cause symptoms over a short time period.

  • Liver problems. Cholangitis can cause liver scarring (cirrhosis). This can slow liver function or lead to liver failure. It also increases the risk of liver cancer. It can cause liver swelling and high blood pressure.

  • •         Gallstones. Blocked bile can harden into stones. This may cause pain and infections.

  • •         Enlarged spleen. If the liver isn’t working properly and can’t filter out wastes and toxins, old blood cells can collect in the spleen, causing it to swell.

  • •         Enlarged veins. High blood pressure in the liver may put too much pressure on veins in the stomach. This can lead to swollen and broken veins. It may also cause bleeding.

  • •        Blood infection. Acute cholangitis can lead to sepsis (a blood infection). This can damage several parts of the body and may be life threatening if not treated.

• –       The most common organisms cultured from bile in patients with cholangitis:

  • –         Escherichia coli

  • –         Klebsiella pneumonia

  • –         Streptococcus faecalis

  • –         Enterobacter

  • –         Bacteroides

• Clinical Presentation

  • –       mild, intermittent, and self-limited disease to a fulminant, potentially life-threatening septicemia

  • –       Charcot’s triad

  • –        fever, epigastric or right upper quadrant pain, and jaundice

  • –       Reynolds’ pentad

  • –        The illness may progress rapidly with septicemia and disorientation,

• Diagnosis and Management

  • –       Leukocytosis, hyperbilirubinemia, and elevation of alkaline phosphatase and transaminases

  • –       Utz / MRCP

  • –       CT scanning

  • –       ERCP- definitive diagnostic test

    • –        show the level and the reason for the obstruction, allow culture of the bile, possibly allow the removal of stones if present, and allow drainage of the bile ducts with drainage catheters or stents.

  • –       Magnetic resonance cholangiopancreatography (MRCP). This is a special type of magnetic resonance imaging (MRI) exam that produces detailed pictures of your liver, gallbladder, and bile ducts. It can also show if there are gallstones in your bile duct or any type of blockage.

  • –       Endoscopic retrograde cholangiopancreatography (ERCP). This procedure combines an X-ray and the use of a long, flexible tube with a light and camera on the end, called an endoscope. Your doctor will guide the scope down your throat into your stomach and then into the first part of your intestine. This will allow them to view your organs for any problems. They will then inject a dye into your bile ducts and take an X-ray to see if there is a blockage.

  • –       Percutaneous transhepatic cholangiography (PTC). With this procedure, a contrasting agent (dye) is injected through your skin into your liver or bile duct. Then, you’ll get an X-ray of the ducts to determine if there’s an obstruction. Because of the invasive nature of this procedure, it’s used a lot less frequently than the procedures listed above.

• Operative Interventions for Gallstone disease

  • –     Cholecystectomy

  • –     Laparoscopic Cholecystectomy

  • –     Open Cholecystectomy

  • –     Intraoperative Cholangiogram or Ultrasound

  • –     Common Bile Duct Exploration

  • –     Common Bile Duct Drainage Procedures

  • –     Choledochoduodenostomy

  • –     Transduodenal Sphincterotomy

  • sphincterotomy

Other Benign Diseases And Lesions

• –       Acalculous Cholecystitis

• –       Biliary Cysts

• –       Sclerosing Cholangitis

• –       Stenosis of the Sphincter of Oddi

• –       Bile Duct Strictures

• Acalculous cholecystitis

  • –       Acalculous cholecystitis is the term for inflammation of the gallbladder that is not due to the presence of gallstones.

  • –     Acalculous cholecystitis tends to occur in patients who are severely ill. It is believed that an underlying disorder that impairs circulation to the gallbladder via the cystic artery then leads to ischemia. Another possibility is that since critically ill patients are not eating, the gallbladder is not stimulated to contract and release bile. Gallbladder dysmotility may also be seen with pregnancy and certain liver disorders. Subsequent bile stasis and sludge formation causes inflammation. Infection is another possible cause of acalculous cholecystitis.

  • –    The symptoms of acalculous cholecystitis are largely the same as acute calculous cholecystitis. However, patients who usually suffer with acalculous cholecystitis are often extremely ill and the symptoms of underlying disorders may be more prominent.

    • –     Unexplained fever

    • –     Abdominal distension

    • –     Tenderness of the abdomen

    • –     Abdominal discomfort or pain, usually in the right upper quadrant (RUQ)

    • –     Elevated white blood cell count (leukocytosis)

    • –     vomiting, loss of appetite, weakness and malaise are non-specific symptoms of acute cholecystitis. In acalculous cholecystitis, these symptoms are more likely due to the underly cause of gallbladder inflammation.

  • causes

    • –     Acalculous cholecystitis tends to occur in patients who are severely ill. It is believed that an underlying disorder that impairs circulation to the gallbladder via the cystic artery then leads to ischemia. Another possibility is that since critically ill patients are not eating, the gallbladder is not stimulated to contract and release bile.

    • –    Gallbladder dysmotility may also be seen with pregnancy and certain liver disorders. Subsequent bile stasis and sludge formation causes inflammation. Infection is another possible cause of acalculous cholecystitis.

  • treatment

    • The treatment for acalculous cholecystitis is surgical removal of the gallbladder (cholecystectomy). However, acalculous cholecystitis often occurs in patients who are severely ill and therefore surgery may not always be advisable at that point in time. Interim measures include :

      • Endoscopic gallbladder stent placement

      • Percutaneous cholecystostomy

        • These procedures facilitate drainage of the gallbladder either directly into the duodenum of the small intestine or externally into the environment through a catheter. Once the patient improves, a cholecystectomy should be done. The procedure can be done laparaoscopically or with open surgery. Ideally a cholecystectomy should not be delayed due to the high risk of complications in acalculous cholecystitis, which are often severe and life threatening.

    • cholecystostomy

  • complications

    •  Severe and untreated cholecystitis can lead to complications like :

    • Perforation of the gallbladder – tear in the gallbladder wall with its contents spilling into the abdominal cavity.

    • Gangrene of the gallbladder – portion of the gallbladder wall dies and decomposes.

    • Patients with these complications may go into shock, experience peritonitis or develop sepsis.

Choledochal cyst

• A choledochal cyst is a cyst (hollow outpouching) of the bile ducts. Choledochal cysts have been classified into several different types depending on where they are located and whether they can be seen as separate structures from the ducts ( diverticulum -like); or whether they can be seen as a localized dilation (enlargement) of the ducts.

  1. What is a choledochal cyst?

  A choledochal cyst is a cyst of the bile ducts.

  2. How are choledochal cysts classified?

  Choledochal cysts are classified into different types based on their location and whether they are separate structures from the ducts or localized dilations.

  3. What are the different types of choledochal cysts based on their structure?

  The different types of choledochal cysts are diverticulum-like cysts and localized dilations of the ducts.

  4. How are diverticulum-like choledochal cysts characterized?

  Diverticulum-like choledochal cysts can be seen as separate structures from the ducts.

  5. How are localized dilations of the ducts choledochal cysts characterized?

  Localized dilations of the ducts choledochal cysts are seen as a localized enlargement of the ducts.

   

• Choledochal cysts are typically diagnosed as one of several types:

  • –       Type I: Cyst of the bile duct. ...

  • –       Type II: Pouching or sac on the bile duct.

  • –       Type III: Cyst within the wall of the duodenum (where the duct connects to the liver) or pancreas.

  • –       Type IV: A Type I cyst that extends into the liver along the bile ducts.

  • –       Type V: Multiple cysts along the bile duct inside the liver, also known as Caroli’s Disease.

  • –       Type I cysts are the most common and represent 80-90% of choledochal cysts. 

• treatment

  • –    the treatment of choice for choledochal cysts is complete excision. Patients with type I, II, or IV cysts are recommended for surgical excision due to the risk of malignancy

  • –     There is an increased risk of cancer in the wall of the cyst. In older individuals, choledochal cysts are more likely to cause abdominal pain and intermittent episodes of jaundice and occasionally cholangitis (inflammation within the bile ducts caused by the spread of bacteria from the intestine into the bile ducts).

Tumors

• Carcinoma of the Gallbladder

  • rare malignancy that occurs predominantly in the elderly.

  •   aggressive tumor, with poor prognosis except when incidentally diagnosed at an early stage after cholecystectomy for cholelithiasis.

  • Overall reported 5-year survival rate is about 5%.

• Incidence

  • fifth most common GI malignancy in Western countries.

  • accounts for only 2% to 4% of all malignant GI tumors

  • two to three times more common in females than males

  • peak incidence is in the seventh decade of life.

  • high in native populations of the United States, Mexico, and Chile.

  • annual incidence in Native American females with gallstones approaches 75 per 100,000, compared

• Etiology

  • Cholelithiasis: is the most important risk factor for gallbladder carcinoma

  • 95% of patients with carcinoma of the gallbladder have gallstones.

  • 20-year risk of developing cancer for patients with gallstones is <0.5% for the overall population and 1.5% for high-risk groups.

  • Larger stones (>3 cm) are associated with a 10-fold increased risk of cancer.

  • Polypoid lesions of the gallbladder are associated with increased risk of cancer, particularly in polyps >10 mm.

  • The calcified “porcelain” gallbladder is associated with >20% incidence of gallbladder carcinoma.

  • These gallbladders should be removed, even if the patients are asymptomatic.

  • Porcelain gallbladder refers to the condition in which the inner gallbladder wall is encrusted with calcium. The wall becomes brittle, hard, and often takes on a bluish hue. Other names for this condition are calcified gallbladder, calcifying cholecystitis

• Pathology

  • Histologic subtypes of gallbladder adenocarcinomas:

    • papillary, nodular, and tubular. Less than 10% are of the papillary type, but these are associated with an overall better outcome, as they are most commonly diagnosed

  • Cancer of the gallbladder spreads through the lymphatics, with venous drainage, and with direct invasion into the liver parenchyma.

  • Lymphatic flow from the gallbladder drains first to the cystic duct node (Calot’s), then the pericholedochal and hilar nodes, and finally the peripancreatic, duodenal, periportal, celiac, and superior mesenteric artery nodes.

  • The gallbladder veins drain directly into the adjacent liver, usually segments IV and V, where tumor invasion is common.

  • The gallbladder wall: lacks a muscularis mucosa and submucosa.

  • Lymphatics are present in the subserosal layer only.

  • Therefore, cancers invading but not growing through the muscular layer have minimal risk of nodal disease.

• Clinical Manifestations and Diagnosis

  • Abdominal discomfort, right upper quadrant pain, nausea, and vomiting. Jaundice, weight loss, anorexia, ascites, and abdominal mass are less common presenting symptoms.

  • Laboratory findings are not diagnostic but, if abnormal, are most often consistent with biliary obstruction.

  • Ultrasonography often reveals a thickened, irregular gallbladder wall or a mass replacing the gallbladder.

    • visualize tumor invasion of the liver, lymphadenopathy, and a dilated biliary tree.

    • Sensitivity: ranges from 70% to 100%.

  • CT scan is an important tool for staging and may identify a gallbladder mass or local invasion into adjacent organs.

  • spiral CT scan can demonstrate vascular invasion; however, CT scan is a poor method for identifying nodal spread.

• Clinical Manifestations and Diagnosis

  • Newer MRI techniques MRCP

  • allows complete assessment of biliary, vascular, nodal, hepatic, and adjacent organ involvement.

  • If diagnostic studies suggest that the tumor is unresectable, a CT scan or ultrasound guided biopsy of the tumor can be obtained to provide a pathologic diagnosis.

• Staging of Gallbladder Ca

  I. Introduction

  A. Definition of gallbladder cancer (GBC)

  B. Importance of staging in GBC management

  II. TNM Staging System

  A. Tumor (T) classification

  1. T1: Limited to the mucosa or muscle layer

  2. T2: Invading the perimuscular connective tissue or liver

  3. T3: Invading the liver, serosa, or adjacent structures

  4. T4: Invading the main portal vein or hepatic artery

  B. Node (N) classification

  1. N0: No regional lymph node involvement

  2. N1: Involvement of regional lymph nodes

  C. Metastasis (M) classification

  1. M0: No distant metastasis

  2. M1: Presence of distant metastasis

  III. Stage Grouping

  A. Stage 0: Tis, N0, M0

  B. Stage I: T1, N0, M0

  C. Stage II: T2, N0, M0 or T3, N0, M0

  D. Stage III: T1-T3, N1, M0 or T4, N0-N1, M0

  E. Stage IV: Any T, any N, M1

  IV. Imaging Modalities for Staging

  A. Ultrasound

  B. Computed tomography (CT)

  C. Magnetic resonance imaging (MRI)

  D. Positron emission tomography (PET)

  V. Prognostic Factors

  A. Tumor size and extent of invasion

  B. Lymph node involvement

  C. Presence of distant metastasis

  D. Histological grade

  E. Surgical resectability

  VI. Treatment Options Based on Staging

  A. Stage 0: Cholecystectomy

  B. Stage I: Cholecystectomy with lymphadenectomy

  C. Stage II: Extended cholecystectomy with lymphadenectomy

  D. Stage III: Neoadjuvant therapy followed by surgery

  E. Stage IV: Palliative chemotherapy or targeted therapy

• Treatment

  • Surgery remains the only curative option

  • Palliative procedures: unresectable cancer and jaundice or duodenal obstruction

  • Obstructive jaundice: endoscopic or percutaneously placed biliary stents.

  • Patients without evidence of distant metastasis warrant exploration for tissue diagnosis,  pathologic staging, and possible curative resection.

• Treatment

  • Simple cholecystectomy is an adequate treatment for T1 lesions and results in a near 100% overall 5-year survival rate.

  • When the tumor invades the perimuscular connective tissue without extension beyond the serosa or into the liver (T2 tumors), an extended cholecystectomy should be performed.

  • includes resection of liver segments IVB and V, and lymphadenectomy of the cystic duct and pericholedochal, portal, right celiac, and posterior pancreatoduodenal lymph nodes.

  • Therefore, regional lymphadenectomy is an important part of surgery for T2 cancers.

  • For tumors that grow beyond the serosa or invade the liver or other organs (T3 and T4 tumors), there is a high likelihood of intraperitoneal and distant spread.

  • If no peritoneal or nodal involvement is found, complete tumor excision with an extended right hepatectomy (segments IV, V, VI, VII, and VIII) must be performed for adequate tumor clearance.

• Prognosis

  • Main Branches:

    • 5-year survival rate and median survival

    • Prognosis based on disease stage

    • Recurrence and prognosis after resection

    • Common problems associated with gallbladder cancer

    Sub-Branches:

    5-year survival rate and median survival

    • <5% 5-year survival rate

    • Median survival of 6 months

    Prognosis based on disease stage

    • T1 disease treated with cholecystectomy

      • Excellent prognosis (85%-100% 5-year survival rate)

    • T2 lesions treated with extended cholecystectomy and lymphadenectomy

      • >70% 5-year survival rate

      • Simple cholecystectomy: 25%-40% 5-year survival rate

    • Advanced but resectable gallbladder cancer

      • 20%-50% 5-year survival rate

    • Distant metastasis at presentation

      • Median survival of 1 to 3 months

    Recurrence and prognosis after resection

    • Recurrence commonly in:

      • Liver

      • Celiac nodes

      • Retropancreatic nodes

    • Prognosis for recurrent disease is poor

    Common problems associated with gallbladder cancer

    • Pruritus and cholangitis associated with obstructive jaundice

    • Bowel obstruction due to carcinomatosis

    • Pain

Bile Duct Carcinoma

• Cholangiocarcinoma is a rare tumor arising from the biliary epithelium

• About two thirds are located at the hepatic duct bifurcation.

• Surgical resection

  • Palliative procedures aimed to provide biliary drainage to prevent liver failure and cholangitis are often the only therapeutic possibilities.

• Most patients with unresectable disease die within 1 year of diagnosis.

• Incidence

  • Autopsy incidence: is about 0.3%.

  • Overall incidence of cholangiocarcinoma in the United States is about 1.0 per 100,000 people per year, with about 3000 new cases diagnosed annually.

  • The male-to-female ratio is 1.3:1, and the average age of presentation is between 50 and 70 years.

• Etiology

  • Risk factors: primary sclerosing cholangitis, choledochal cysts, ulcerative colitis, hepatolithiasis, biliary-enteric anastomosis, and biliary tract infections with Clonorchis or in chronic typhoid carriers.

  • Features common: biliary stasis, bile duct stones, and infection.

  • Other risk factors: liver flukes, dietary nitrosamines, Thorotrast, and exposure to dioxin.

• Pathology

  •   Over 95% of bile duct cancers are adenocarcinomas.

  • Morphologically, they are divided into nodular (the most common type), scirrhous, diffusely infiltrating, or papillary.

  • Anatomically, they are divided into distal, proximal, or perihilar tumors.

  • About two thirds of cholangiocarcinomas are located in the perihilar location.

  •  Klatskin tumors- Perihilar cholangiocarcinomas,

  • Type I tumors: confined to the common hepatic duct

  • type II tumors: involve the bifurcation without involvement of the secondary intrahepatic ducts.

  • Type IIIa and IIIb tumors: extend into the right and left secondary intrahepatic ducts, respectively.

  • Type IV tumors: involve both the right and left secondary intrahepatic ducts.

• Clinical Manifestations

  • Painless jaundice: most common presentation.

  • Pruritus, mild right upper quadrant pain, anorexia, fatigue, and weight loss also may be present.

  • Cholangitis is the presenting symptom in about 10% of patients, but occurs more commonly after biliary manipulation in these patients. Except for jaundice, physical examination isusually normal in patients with cholangiocarcinoma.

  • Occasionally, asymptomatic patients are found to have cholangiocarcinoma while being evaluated for elevated alkaline phosphatase and γ-glutamyltransferase levels.

  • Tumor markers such CA 125 and carcinoembryonic antigen can be elevated in cholangiocarcinoma but tend to be nonspecific because they also increase in other GI and gynecologic malignancies or cholangiopathologies.

  • CA 19-9

    • sensitivity of 79% and specificity of 98% if the serum value is >129 U/mL.92

• Clinical Manifestations

  • Ultrasound or CT scan.

  • A perihilar tumor causes dilatation of the intrahepatic biliary tree, but normal or collapsed gallbladder and extrahepatic bile ducts distal to the tumor.

  • Distal bile duct cancer leads to dilatation of the extra- and intrahepatic bile ducts as well as the gallbladder.

  • Ultrasound can establish the level of obstruction and rule out the presence of bile duct stones as the cause of the obstructive jaundice.

  • CT can be used to determine portal vein patency.

  • Biliary anatomy is defined by cholangiography.

  • PTC defines the proximal extent of the tumor, which is the most important factor in determining resectability.

  •   ERC is used, particularly in the evaluation of distal bile duct tumor

  • Tissue diagnosis may be difficult to obtain nonoperatively except in advanced cases.

  • Percutaneous fine-needle aspiration biopsy, biliary brush or scrape biopsy, and cytologic examination have a low sensitivity in detecting malignancy.

• Treatment

  • –       Surgical excision is the only potentially curative treatment for cholangiocarcinoma.

  • –       Patients should undergo surgical exploration if they have no signs of metastasis or locally unresectable disease.

  • –       For these patients, surgical bypass for biliary decompression and cholecystectomy to prevent the occurrence of acute cholecystitis should be performed

  • –       For unresectable perihilar cholangiocarcinoma, Roux-en-Y  cholangiojejunostomy to either segment II or III bile ducts or to the right hepatic duct can be performed.

  • –       For curative resection, the location and local extension of the tumor dictates the extent of the resection.

  • –       Perihilar tumors involving the bifurcation or proximal common hepatic duct (Bismuth-Corlette type I or II) with no signs of vascular involvement are candidates for local tumor excision with portal lymphadenectomy, cholecystectomy, common bile duct excision, and bilateral Roux-en-Y hepaticojejunostomies.

  • –       If the tumor involves the right or left hepatic duct (Bismuth-Corlette type IIIa or IIIb), right or left hepatic lobectomy, respectively, should also be performed

  • Distal bile duct tumors are more often resectable

  • Whipple procedure

    • pylorus-preserving pancreatoduodenectomy

  • Distal bile duct cancer found to be unresectable

  • Roux-en-Y hepaticojejunostomy, cholecystectomy, and gastrojejunostomy to prevent gastric outlet obstruction should be performed.

  • Nonoperative biliary decompression is performed for patients with unresectable disease on diagnostic evaluation.

  • Percutaneous placement of expandable metal stents or drainage catheters is usually the appropriate approach for proximal tumors.

  • bypass

  • A Whipple procedure - also known as a pancreaticoduodenectomy - is a complex operation to remove the head of the pancreas, the first part of the small intestine (duodenum), the gallbladder and the bile duct.

  • Patients with unresectable disease

    • 5-fluorouracil alone or in combination with mitomycin C and doxorubicin, but the response rates are low, <10% and <30%, respectively.  ( chemotherapy)

    • The use of interstitial (intraoperative) radiation, brachytherapy with iridium-192 via percutaneous or endoscopic stents, and combined interstitial and external-beam radiation for unresectable cholangiocarcinoma has been reported with some encouraging results.

  • interstitial radiation therapy: Radiation treatment given by placing radioactive material directly into the target,

    • Brachytherapy is a procedure used to treat certain types of cancer and other conditions. It involves placing radioactive material inside the body. This is sometimes called internal radiation.

  • Another type of radiation, called external radiation, is more common than brachytherapy. During external radiation, a machine moves around you and directs beams of radiation to specific points on the body.

• Prognosis

  • Unresectable disease have a median survival between 5 and 8 months.

  • Most common causes of death: hepatic failure and cholangitis.

  • The overall 5-year survival rate for patients with resectable perihilar cholangiocarcinoma is between 10% and 30%, but for patients with negative margins, it may be as high as 40%.

  • The operative mortality for perihilar cholangiocarcinoma is 6% to 8%.

  • The overall 5-year survival rate for resectable disease is 30% to 50%, and the median survival is 32 to 38 months.

  • The greatest risk factors for recurrence after resection are the presence of positive margins and lymph node–positive tumors.

  • Therapy for recurrent disease is palliation of symptoms.

  • Surgery is not recommended for patients with recurrent disease.

  • Summary questions

    1. What is cholangiocarcinoma?

    Answer: Cholangiocarcinoma is a rare tumor arising from the biliary epithelium.

    2. Where are about two-thirds of cholangiocarcinomas located?

    Answer: About two-thirds of cholangiocarcinomas are located at the hepatic duct bifurcation.

    3. What are the therapeutic possibilities for patients with unresectable cholangiocarcinoma?

    Answer: Palliative procedures aimed to provide biliary drainage to prevent liver failure and cholangitis are often the only therapeutic possibilities.

    4. What is the incidence of cholangiocarcinoma in the United States?

    Answer: The overall incidence of cholangiocarcinoma in the United States is about 1.0 per 100,000 people per year, with about 3000 new cases diagnosed annually.

    5. What are the risk factors for cholangiocarcinoma?

    Answer: Risk factors for cholangiocarcinoma include primary sclerosing cholangitis, choledochal cysts, ulcerative colitis, hepatolithiasis, biliary-enteric anastomosis, and biliary tract infections with Clonorchis or in chronic typhoid carriers.

    6. What is the most common type of bile duct cancer?

    Answer: Over 95% of bile duct cancers are adenocarcinomas.

    7. What is the most common presentation of cholangiocarcinoma?

    Answer: Painless jaundice is the most common presentation of cholangiocarcinoma.

    8. How can the level of obstruction and the presence of bile duct stones be determined in cholangiocarcinoma?

    Answer: Ultrasound can establish the level of obstruction and rule out the presence of bile duct stones as the cause of obstructive jaundice.

    9. What is the only potentially curative treatment for cholangiocarcinoma?

    Answer: Surgical excision is the only potentially curative treatment for cholangiocarcinoma.

    10. What is the prognosis for patients with unresectable cholangiocarcinoma?

    Answer: Patients with unresectable disease have a median survival between 5 and 8 months.