The gallbladder and bile ducts

The gallbladder and bile ducts surgery

1. The Gallbladder And Bile Ducts Dr Shaurya Pratap Singh

2. SURGICAL ANATOMY 1. Gallbladder It is a pear-shaped structure, 7.5– 12 cm long, with a normal capacity of about 25–30 mL. The anatomical divisions are a fundus, a body and a neck that terminates in a narrow infundibulum. The muscle fibres in the wall of the gallbladder are arranged in a criss-cross manner, being particularly well developed in its neck. The mucous membrane contains indentations of the mucosa that sink into the muscle coat; these are the crypts of Luschka.

3. SURGICAL ANATOMY 1. Gallbladder The gallbladder is entirely surrounded by peritoneum, and is in direct relation to the visceral surface of the liver. It lies in close proximity to the following structures: Anteriorly and superiorly – inferior border of the liver and the anterior abdominal wall. Posteriorly – transverse colon and the proximal duodenum. Inferiorly – biliary tree and remaining parts of the duodenum.

4. Anatomical Structure

5. Relationship of gallbladder to visceral surface of liver

6. The main variations in gallbladder Phrygian cap

7. SURGICAL ANATOMY 2. Cystic Duct The cystic duct is about 3 cm in length but the length is variable. The lumen is usually 1–3 mm in diameter. The mucosa of the cystic duct is arranged in spiral folds known as the valves of Heister and the wall is surrounded by a sphincteric structure called the sphincter of Lütkens. The cystic duct joins the supraduodenal segment of the common hepatic duct in 80% of cases, however the anatomy may vary and the junction may be much lower in the retroduodenal or even retropancreatic part of the bile duct

8. Schematic view of interior of gallbladder and extrahepatic biliary tract

9. SURGICAL ANATOMY 3. Common Bile Duct The common hepatic duct is usually less than 2.5 cm long and is formed by the union of the right and left hepatic ducts. The common bile duct is about 7.5 cm long and formed by the junction of the cystic and common hepatic ducts. It is divided into four parts: the supraduodenal portion , about 2.5 cm long, runs in the free edge of the lesser omentum; the retroduodenal portion ; the infraduodenal portion lies in a groove, but at times in a tunnel, on the posterior surface of the pancreas; the intraduodenal portion passes obliquely through the wall of the second part of the duodenum, where it is surrounded by the sphincter of Oddi, and terminates by opening on the summit of the ampulla of Vater.

10. The Biliary Tree The biliary tree is a series of gastrointestinal ducts allowing newly synthesized bile from the liver to be concentrated and stored in the gallbladder (prior to release into the duodenum). Bile is initially secreted from hepatocytes and drains from both lobes of the liver via canaliculi, intralobular ducts and collecting ducts into the left and right hepatic ducts. These ducts amalgamate to form the common hepatic duct, which runs alongside the hepatic vein.

11. The Biliary Tree As the common hepatic duct descends, it is joined by the cystic duct – which allows bile to flow in and out of the gallbladder for storage and release. At this point, the common hepatic duct and cystic duct combine to form the common bile duct. The common bile duct descends and passes posteriorly to the first part of the duodenum and head of the pancreas. Here, it is joined by the main pancreatic duct , forming the hepatopancreatic ampulla (commonly known as the ampulla of Vater ) – which then empties into the duodenum via the major duodenal papilla. This papilla is regulated by a muscular valve, the sphincter of Oddi.

12. SURGICAL ANATOMY 2. Bile Ducts

13. Vasculature of Gallbladder

14. Illustration showing Calot’s triangle, epiploic foramen and structures in the hepatoduodenal ligament

15. Vasculature of Gallbladder The arterial supply to the gallbladder is via the cystic artery – a branch of the right hepatic artery (which itself is derived from the common hepatic artery , one of the three major branches of the coeliac trunk ). Venous drainage of the neck of the gallbladder is via the cystic veins , which drain directly into the portal vein . Venous drainage of the fundus and body of the gallbladder flows into the hepatic sinusoids .

16. Vasculature of Gallbladder

17. Schematic illustration of intrahepatic and extrahepatic biliary tract

18. Calot’s triangle, or the hepatobiliary triangle It was initially described by Calot as the space bordered by the cystic duct inferiorly, the common hepatic duct medially and the superior border of the cystic artery. This has been modified in contemporary literature to be the area bound superiorly by the inferior surface of the liver, laterally by the cystic duct and the medial border of the gallbladder and medially by the common hepatic duct.

19. Calot’s triangle (a) Usual anatomy of Calot’s triangle; (b) tortuous common hepatic artery; (c) tortuous right hepatic artery with short cystic artery; (b) and (c) are examples of so-called ‘caterpillar turn’ or ‘Moynihan’s hump’, which if not recognized can lead to inadvertent arterial injury or bleeding during cholecystectomy.

20. Variability in cystic duct anatomy.

21. Lymphatics The lymphatic vessels of the gallbladder ( subserosal and submucosal) drain into the cystic lymph node of Lund (the sentinel lymph node), which lies in the fork created by the junction of the cystic and common hepatic ducts. Efferent vessels from this lymph node go to the hilum of the liver, and to the coeliac lymph nodes. The subserosal lymphatic vessels of the gallbladder also connect with the subcapsular lymph channels of the liver, and this accounts for the frequent spread of carcinoma of the gallbladder to the liver.

22. Surgical physiology Bile is produced by the liver and stored in the gallbladder, from which it is released into the duodenum. As it leaves the liver it is composed of 97% water, bile salts (cholic and chenodeoxycholic acids, deoxycholic and lithocholic acids), phospholipids, cholesterol and bilirubin. The liver excretes bile at a rate estimated to be approximately 40 mL/hour. About 95% of bile salts are reabsorbed in the terminal ileum (enterohepatic circulation).

23. Functions of the gallbladder Areservoir for bile. During fasting, resistance to flow through the sphincter of Oddi is high, and bile excreted by the liver is diverted to the gallbladder. After feeding, the resistance to flow through the sphincter is reduced, the gallbladder contracts and the bile enters the duodenum. These motor responses of the biliary tract are in part affected by the hormone cholecystokinin. Concentration of bile by active absorption of water, sodium chloride and bicarbonate via the mucous membrane of the gallbladder. The hepatic bile which enters the gallbladder becomes concentrated 5–10 times, with a corresponding increase in the proportion of bile salts, bile pigments, cholesterol and calcium. Secretion of mucus – approximately 20 mL is produced per day. With complete obstruction of the cystic duct in an otherwise healthy gallbladder, a mucocoele may develop as a result of ongoing mucus secretion by the gallbladder mucosa.

24. RADIOLOGICAL INVESTIGATION OF THE BILIARY TRACT 1. Xray Plain radiograph showing radiopaque stones in the gallbladder. Radiopaque stones are rare (10%). Porcelain gallbladder. Gas in the gallbladder and gallbladder wall ( Clostridium perfringens ). Emergency surgery is indicated.

25. RADIOLOGICAL INVESTIGATION OF THE BILIARY TRACT 2. Ultrasonography Transabdominal ultrasonography is the initial imaging modality of choice as it is accurate, readily available, inexpensive and quick to perform. However, it is operator dependent and may be compromised by excessive body fat and intraluminal bowel gas. The size of the gallbladder can be seen, the presence of stones or polyps determined and the thickness of the wall measured. Additionally, the presence of inflammation around the gallbladder, the size of the common bile duct and, occasionally, the presence of stones within the extrahepatic biliary tree can be determined. For the patient who presents with obstructive jaundice, it can identify intra- and extrahepatic biliary dilatation and often the level of obstruction. In addition, the cause of the obstruction may also be determined, such as gallstones in the gallbladder, common hepatic or common bile duct stones, lesions within the wall of the common bile duct suggestive of a cholangiocarcinoma or enlargement of the pancreatic head indicative of a pancreatic carcinoma.

26. RADIOLOGICAL INVESTIGATION OF THE BILIARY TRACT 2. Ultrasonography

27. RADIOLOGICAL INVESTIGATION OF THE BILIARY TRACT 2. Ultrasonography Endoscopic ultrasonography (EUS) utilises a specially designed endoscope with an ultrasound transducer at its tip which allows the gastroenterologist to visualize the liver and biliary tree from within the stomach and duodenum. It is accurate in imaging the bile duct and detecting the presence of choledocholithiasis. In addition, it has been shown to be useful in diagnosing and staging both pancreatic and periampullary cancers. Biopsies can be taken from suspicious areas for either cytological or histopathological analysis.

28. 3. Cholescintigraphy Technetium-99m (99mTc)-labelled derivatives of iminodiacetic acid (HIDA, IODIDA) when injected intravenously are selectively taken up by the retroendothelial cells of the liver and excreted into the bile. This allows visualization of the biliary tree and gallbladder. In 90% of normal individuals the gallbladder is visualized within 30 minutes following injection, with 100% being seen within 1 hour. The bowel is seen, usually within 1 hour, in the majority of patients. Non- visualization of the gallbladder is suggestive of acute cholecystitis . If the patient has a contracted gallbladder, as often seen in chronic cholecystitis, the gallbladder visualization may be reduced or delayed. An abnormally low gallbladder ejection fraction may be suggestive of gallbladder dyskinesia; however, the diagnosis and interpretation of cholescintigraphy in this context are controversial. Biliary scintigraphy may also be helpful in diagnosing bile leaks and iatrogenic biliary obstruction.

29. 4. Computed tomography (CT) CT is less affected by body habitus and is not operator dependent. It allows visualisation of the liver, bile ducts, gallbladder and pancreas. It is particularly useful in detecting hepatic and pancreatic lesions and is the modality of choice in the staging of cancers of the liver, gallbladder, bile ducts and pancreas. It can identify the extent of the primary tumour and define the relationship of the tumour to other organs and blood vessels. In addition, the presence of enlarged lymph nodes or metastatic disease may be seen. However, as only 75% of gallstones are identified by CT, it is not used as a screening modality for uncomplicated gallstones.

30. 5. Endoscopic retrograde cholangiopancreatography (ERCP) Using a side-viewing endoscope the ampulla of Vater can be identified and cannulated. Injection of water-soluble contrast directly into the bile duct provides excellent images of the ductal anatomy and can identify causes of obstruction such as calculi or malignant strictures. It is especially useful in determining the cause and level of obstruction. During ERCP, bile aspirates can be sent for cytological and microbiological examination, and endoluminal brushings can be taken from strictures for cytological studies. Therapeutic interventions such as stone removal or stent placement to relieve the obstruction can be performed.

31. 6. Magnetic resonance cholangiopancreatography (MRCP) Images can be obtained of the biliary tree demonstrating ductal obstruction, strictures or other intraductal abnormalities. Images comparable to those obtained using ERCP or PTC can be achieved non-invasively without the potential complications of either technique. By use of the water content of bile, a cholangiopancreatogram can be created, which makes it an excellent modality for cross- sectional imaging of the biliary tree.

32. 7. Percutaneous transhepatic cholangiography (PTC) Under radiological control (either ultrasound or CT) a bile duct is cannulated. Successful entry is confirmed by contrast injection or aspiration of bile. Water- soluble contrast medium is injected to visualise the biliary system. Multiple images can be taken demonstrating areas of strictures or obstruction. Bile can be sent for cytology. In addition, this technique enables placement of a catheter into the bile ducts to provide external biliary drainage or the insertion of indwelling stents. The scope of this procedure can be further extended by leaving the drainage catheter in situ for a number of days and then dilating the track sufficiently for a fine flexible choledochoscope to be passed into the intrahepatic biliary tree in order to diagnose strictures, take biopsies and remove stones In general, if a malignant stricture at the level of the confluence of the right and left hepatic ducts or higher is suspected in a jaundiced patient, a PTC is preferred to ERCP because successful drainage is more likely.

33. CONGENITAL ABNORMALITIES OF THE GALLBLADDER AND BILE DUCTS Absence of the gallbladder The Phrygian cap Floating gallbladder Absence of the cystic duct usually a pathological, as opposed to an anatomical, anomaly and indicates the recent passage of a stone or the presence of a stone at the lower end of the cystic duct, which is ulcerating into the common bile duct. The main danger at surgery is damage to the bile duct, and particular care to identify the correct anatomy is essential before division of any duct. Low insertion of the cystic duct An accessory cholecystohepatic duct

34. EXTRAHEPATIC BILIARY ATRESIA The extrahepatic bile ducts are progressively destroyed by an inflammatory process which starts around the time of birth. The inflammatory destruction of the bile ducts has been classified into three main types: type I: atresia restricted to the common bile duct; type II: atresia of the common hepatic duct; type III: atresia of the right and left hepatic ducts. Associated anomalies occur in about 20% of cases and include cardiac lesions, polysplenia, situs inversus, absent vena cava and a preduodenal portal vein.

35. EXTRAHEPATIC BILIARY ATRESIA Clinical features About one-third of patients are jaundiced at birth. In all, however, jaundice is present by the end of the first week and deepens progressively. Liver function tests show an obstructive pattern with elevated bilirubin and alkaline phosphatase. The meconium may be a little bile stained, but later the stools are pale and the urine is dark. Prolonged steatorrhoea gives rise to osteomalacia (biliary rickets). Pruritus is severe. Clubbing and skin xanthomas, probably related to raised serum cholesterol, may be present.

36. Treatment A simple biliary–enteric anastomosis is not possible in the majority of cases in which the proximal hepatic ducts are either very small (type II) or atretic (type III). These are treated by the Kasai procedure, in which radical excision of all bile duct tissue up to the liver capsule is performed. A Roux-en-Y loop of jejunum is anastomosed to the exposed area of liver capsule above the bifurcation of the portal vein, creating a portoenterostomy. The chances of achieving effective bile drainage after portoenterostomy are maximal when the operation is performed before the age of 8 weeks , and approximately 90% of children whose bilirubin falls to within the normal range can be expected to survive for 10 years or more.

37. Treatment Early referral for surgery is critical. Postoperative complications include bacterial cholangitis, which occurs in 40% of patients. Repeated attacks lead to hepatic fibrosis, and 50% of long-term survivors develop portal hypertension, with one-third having variceal bleeding. Liver transplantation should be considered in children in whom a portoenterostomy is unsuccessful. Results are improving, with 70–80% alive 2–5 years following transplant

38. CONGENITAL DILATATION OF THE INTRAHEPATIC DUCTS (CAROLI’S DISEASE) This rare congenital condition is characterised by multiple irregular saccular dilatations of the intrahepatic ducts, separated by segments of normal or stenotic ducts, with a normal extrahepatic biliary system. In Caroli’s syndrome , the biliary dilatation is associated with congenital hepatic fibrosis

39. CONGENITAL DILATATION OF THE INTRAHEPATIC DUCTS (CAROLI’S DISEASE) The presentation is varied, with many patients presenting with abdominal pain, cholangitis or end-stage liver disease. The majority of patients present before the age of 30. Sex distribution is equal. Management is multidisciplinary: cholangitis or jaundice are treated with appropriate antibiotic therapy and endoscopic or interventional stenting. Malignancy is a complication of long- standing disease.

40. CONGENITAL DILATATION OF THE INTRAHEPATIC DUCTS (CAROLI’S DISEASE) Hepatic resection is indicated for patients with limited disease. Patients with diffuse disease and concomitant hepatic fibrosis are candidates for liver transplantation. Recurrence is common, particularly after resection, and long-term surveillance is required.

41. CHOLEDOCHAL CYST Choledochal cysts are congenital dilations of the intra and/or extrahepatic biliary system. The pathogenesis is unclear. Anomalous junctions of the biliary pancreatic junction are frequently observed, but whether or not these play a role in the pathogenesis of the condition is unclear.

42. Symptoms Patients may present at any age with jaundice, fever, abdominal pain and a right upper quadrant mass on examination; Pancreatitis is not an infrequent presentation in adults. Patients with choledochal cysts have an increased risk of developing cholangiocarcinoma with the risk varying directly with the age at diagnosis.

43. Investigations Ultrasonography will confirm the presence of an abnormal cyst and magnetic resonance imaging (MRI/MRCP) will reveal the anatomy, in particular the relationship between the lower end of the bile duct and the pancreatic duct. CT is also useful for delineating the extent of the intra- or extrahepatic dilatation.

44. Classification of types of choledochal cyst. Type Ia and b: diffuse cystic. Note extension into pancreas of type Ib. Type II: diverticulum of common bile duct. Type III: diverticulum within pancreas. Type IV: extension into the liver. Type V: cystic dilatation only of the intrahepatic ducts.

45. Treatment Radical excision of the cyst is the treatment of choice, with reconstruction of the biliary tract using a Roux-en-Y loop of jejunum. Complete resection of the cyst is important because of the association with the development of cholangiocarcinoma. Resection and roux-en-Y reconstruction are also associated with a reduced incidence of stricture formation and recurrent cholangitis.

46. TRAUMA Iatrogenic injury is perhaps more frequent than external trauma. The physical signs are those of an acute abdomen. Management depends on the location and extent of the biliary and associated injury. In the stable patient a transected bile duct is best repaired by a Roux-en-Y choledochojejunostomy. Injuries to the gallbladder can be dealt with by cholecystectomy

47. TORSION OF THE GALLBLADDER This is very rare and requires a long mesentery, and therefore often occurs in an older patient with a large mucocoele of the gallbladder. The patient presents with extreme pain and an acute abdomen. Immediate exploration is indicated, with cholecystectomy as the only treatment.

48. GALLSTONES (CHOLELITHIASIS) Gallstones are the most common biliary pathology. Gallstones can be divided into three main types: cholesterol, pigment (brown/black) or mixed stones. Pigment stone is the name used for stones containing <30% cholesterol. There are two types: black and brown. Black stones are largely composed of an insoluble bilirubin pigment polymer mixed with calcium phosphate and calcium bicarbonate . Overall, 20–30% of stones are black. The incidence rises with age. Black stones are associated with haemolysis , usually hereditary spherocytosis or sickle cell disease. Brown pigment stones contain calcium bilirubinate , calcium palmitate and calcium stearate , as well as cholesterol . Brown stones are rare in the gallbladder. They form in the bile duct and are related to bile stasis and infected bile.

49. Factors associated with gallstone formation.

50. Clinical presentation Gallstones may remain asymptomatic, being detected incidentally as imaging is performed for other symptoms. If symptoms occur, patients typically complain of right upper quadrant or epigastric pain, which may radiate to the back. This may be described as colicky but more often is dull and constant. Other symptoms include dyspepsia, flatulence, food intolerance particularly to fats and some alteration in bowel frequency. Biliary colic is typically present in 10–25% of patients. This is described as a severe right upper quadrant pain which ebbs and flows, associated with nausea and vomiting. Pain may radiate to the chest. The pain is usually severe and may last for minutes or even several hours. Frequently, the pain starts during the night and wakes the patient. Minor episodes of the same discomfort may occur intermittently during the day. Dyspeptic symptoms may coexist and be worse after such an attack.

51. Effects and complications of gallstones ● ● Biliary colic ● ● Acute cholecystitis ● ● Chronic cholecystitis ● ● Empyema of the gallbladder ● ● Mucocoele ● ● Perforation ● ● Biliary obstruction ● ● Acute cholangitis ● ● Acute pancreatitis ● ● Intestinal obstruction (gallstone ileus)

52. Differential diagnosis of acute cholecystitis Common ● ● Appendicitis ● ● Perforated peptic ulcer ● ● Acute pancreatitis Rare ● ● Acute pyelonephritis ● ● Myocardial infarction ● ● Pneumonia – right lower lobe

53. Diagnosis In the acute phase the patient may have right upper quadrant tenderness that is exacerbated during inspiration by the examiner’s right subcostal palpation (Murphy’s sign). A positive Murphy’s sign suggests acute inflammation and may be associated with a leukocytosis and moderately elevated liver function tests. A mass may be palpable as the omentum walls off an inflamed gallbladder. While the presentation and examination may suggest acute cholecystitis, a definitive diagnosis can only be made following appropriate imaging studies (US or CT).

54. Treatment Prophylactic cholecystectomy may be considered for diabetic patients, those with congenital haemolytic anaemia and those patients who are undergoing bariatric surgery for morbid obesity because it has been found in these groups that the risk of developing symptoms is increased. For patients with symptomatic gallstones, cholecystectomy is the treatment of choice if there are no medical contraindications. Experience shows that, in more than 90% of cases, the symptoms of acute cholecystitis subside with conservative measures.

55. Treatment Non-operative treatment is based on four principles: ● ● Nil per mouth (NPO) and intravenous fluid administration until the pain resolves. ● ● Administration of analgesics. ● ● Administration of antibiotics. As the cystic duct is blocked in most instances, the concentration of antibiotic in the serum is more important than its concentration in bile. A broad-spectrum antibiotic effective against gram-negative aerobes is most appropriate (e.g. cefazolin, cefuroxime or ciprofloxacillin ). ● ● Subsequent management. When the temperature, pulse and other physical signs show that the inflammation is subsiding, oral fluids are reinstated, followed by a regular diet. .

56. Treatment Ultrasonography is performed to confirm the diagnosis. If jaundice is present MRCP is performed to exclude choledocholithiasis. If there is any concern regarding the diagnosis or presence of complications such as perforation CT should be performed. Cholecystectomy may be performed on the next available list, or the patient may be allowed home to return later when the inflammation has completely resolved

57. Tokyo Consensus Guidelines for severity grading of acute cholecystitis.

58. EMPYEMA OF THE GALLBLADDER Empyema may be a sequel to acute cholecystitis or the result of a mucocoele becoming infected. The gallbladder is distended with pus. The optimal treatment is drainage (cholecystostomy) and, later, cholecystectomy.

59. Acalculous cholecystitis Acute acalculous cholecystitis is particularly seen in critically ill patients and those recovering from major surgery, trauma and burns. The diagnosis is often missed and the mortality rate is high.

60. THE CHOLECYSTOSES (CHOLESTEROSIS, POLYPOSIS, ADENOMYOMATOSIS AND CHOLECYSTITIS GLANDULARIS PROLIFERANS ) Cholesterosis (‘strawberry gallbladder’) In the fresh state, the interior of the gallbladder looks something like a strawberry in patients with this condition; the yellow specks (submucous aggregations of cholesterol crystals and cholesterol esters) correspond to the seeds. It may be associated with cholesterol stones. Cholesterol polyposis of the gallbladder Ultrasound may show a non-mobile defect in the gallbladder lumen which does not exhibit an associated acoustic shadow. The differential is an adenomatous polyp, and interval follow-up is indicated to ensure stability. Surgery is only advised if there is a diagnostic dilemma. Cholecystitis glandularis proliferans (polyp, adenomyomatosis and intramural diverticulosis) A polyp of the mucous membrane is fleshy and granulomatous. All layers of the gallbladder wall may be thickened, but sometimes an incomplete septum forms that separates the hyperplastic from the normal. Intraparietal ‘mixed’ calculi may be present. These can be complicated by an intramural, and later extramural, abscess and potentially fistula formation. If symptomatic, the patient is treated by cholecystectomy

61. Types of cholecystitis glandularis proliferans (polypus, intramural or diverticular stones and fistula).

62. Diverticulosis of the gallbladder Diverticulosis of the gallbladder is usually manifest as black pigment stones impacted in the outpouchings of the lacunae of Luschka. Diverticulosis of the gallbladder may be demonstrated by cholecystography, especially when the gallbladder contracts after a fatty meal. There are small dots of contrast medium just within and outside the gallbladder. A septum may also be present, to be distinguished from the Phrygian cap. The treatment is cholecystectomy

63. Typhoid infection of the gallbladder Salmonella typhi or Salmonella typhimurium can infect the gallbladder. Acute cholecystitis can occur. More frequently chronic cholecystitis occurs, the patient becoming a typhoid carrier excreting the bacteria in the bile. Gallstones may be present (surgeons should not give patients their stones after their operation if there is any suspicion of typhoid!). It is debatable whether the stones are secondary to the Salmonella cholecystitis or whether pre-existing stones predispose the gallbladder to chronic infection. Salmonellae can, however, frequently be cultured from these stones. Treatment with ampicillin and cholecystectomy are indicated. In cases of penicillin allergy a quinolone antibiotic can be used.

64. Stricture of the bile duct Causes of benign biliary stricture Congenital ● ● Biliary atresia Bile duct injury at surgery ● ● Cholecystectomy ● ● Choledochotomy ● ● Gastrectomy ● ● Hepatic resection ● ● Transplantation Inflammatory ● ● Stones ● ● Cholangitis ● ● Parasitic ● ● Pancreatitis ● ● Sclerosing cholangitis ● ● Radiotherapy Trauma Idiopathic

65. Stricture of the bile duct Radiological investigation of biliary strictures ● ● Ultrasonography ● ● Cholangiography via T-tube, if present ● ● ERCP ● ● MRCP ● ● Percutaneous transhepatic cholangiography ● ● CT scan

66. PRIMARY SCLEROSING CHOLANGITIS Primary sclerosing cholangitis is an idiopathic fibrosing inflammatory condition of the biliary tree that affects both intrahepatic and extrahepatic ducts. It is of unknown origin but the association of hypergammaglobulinaemia and elevated markers such as smooth muscle antibodies and antinuclear factor suggest an immunological basis. The majority of patients are between 30 and 60 years of age. There appears to be a male predominance and a strong association with inflammatory bowel disease, especially ulcerative colitis.

67. PRIMARY SCLEROSING CHOLANGITIS Common symptoms include right upper quadrant discomfort, jaundice, pruritus, fever, fatigue and weight loss. Investigation reveals a cholestatic pattern to the liver function tests with elevation of the serum alkaline phosphatase and γ-glutamyl transferase and smaller rises in the aminotransferases. Bilirubin values can be variable and may fluctuate. Imaging studies such as MRCP or ERCP may demonstrate stricturing and beading of the bile ducts . A liver biopsy is helpful to confirm the diagnosis and may help guide therapy by excluding cirrhosis. The important differential diagnoses are secondary sclerosing cholangitis and cholangiocarcinoma. The latter may be very difficult to diagnose and a high index of suspicion is required especially in the setting of unexplained clinical deterioration.

68. PRIMARY SCLEROSING CHOLANGITIS Medical management with antibiotics, vitamin K, cholestyramine, steroids and immunosuppressant drugs such as azathioprine is generally unsuccessful. Endoscopic stenting of dominant strictures and, in selected patients with predominantly extrahepatic disease, operative resection may be worthwhile. For patients with cirrhosis, liver transplantation is the best option. Five-year survival following transplantation in high-volume centres is in excess of 80%.

69. Immunoglobulin (Ig)G4-related cholangitis This recently recognised entity presents with diffuse or segmental narrowing of the intra- or extrahepatic bile ducts. Its features may make differentiation from primary sclerosing cholangitis (PSC), cholangiocarcinoma or pancreatic cancer difficult. However, patients often have elevated serum IgG4 levels and concomitant autoimmune pancreatitis, IgG4- related sialadenitis or retroperitoneal fibrosis. Biliary biopsies show lymphoplasmacytic sclerosing cholangitis. Treatment is with systemic steroids. Failure to respond to steroid therapy should make one reconsider the diagnosis and exclude an underlying malignancy.

70. Biliary ascariasis The roundworm Ascaris lumbricoides commonly infests the intestines of inhabitants of Asia, Africa and Central America. It may enter the biliary tree through the ampulla of Vater and cause biliary pain. Complications include strictures, suppurative cholangitis, liver abscesses and empyema of the gallbladder. In the uncomplicated case, antispasmodics can be given to relax the sphincter of Oddi and the worms will return to the small intestine to be dealt with by anthelminthic drugs. Operation may be necessary to remove the worms or deal with complications. Worms can be extracted via the ampulla of Vater by ERCP.

71. Clonorchiasis (Asiatic cholangiohepatis) This disease is endemic in the Far East. The fluke, up to 25 mm long and 5 mm wide, inhabits the bile ducts, including the intrahepatic ducts. Fibrous thickening of the duct walls occurs. Many cases are asymptomatic. Complications include biliary pain, stones, cholangitis, cirrhosis and bile duct carcinoma. Choledochotomy and T-tube drainage and, in some cases, choledochoduodenostomy are required. Because a process of recurrent stone formation is set up, a choledochojejunostomy with a Roux loop fixed to the adjacent abdominal wall is performed in some centers to allow easy subsequent access to the duct system.

72. Hydatid disease A large hydatid cyst may obstruct the hepatic ducts. Sometimes, a cyst will rupture into the biliary tree and its contents cause obstructive jaundice or cholangitis, requiring appropriate surgery

73. Benign tumours of the bile duct Benign neoplasms causing biliary obstruction may be classified as follows: ● ● papilloma and adenoma; ● ● multiple biliary papillomatosis; ● ● granular cell myoblastoma ; ● ● neural tumours; ● ● leiomyoma; ● ● endocrine tumours.

74. Malignant tumours of the biliary tract Bile duct cancer (cholangiocarcinoma) ● ● Rare, but incidence increasing ● ● Most patients present with abnormal liver function tests or frank jaundice ● ● Diagnosis by ultrasound, CT or MRCP scanning ● ● The majority of patients receive palliative care only ● ● Complete surgical excision possible in <10% ● ● Prognosis poor: 90% die within 1 year, from liver failure or biliary sepsis ● ● Adjuvant chemoradiation therapy has a limited role

75. Cholangiocarcinoma Risk factors for cholangiocarcinoma Chronic inflammatory conditions ● ● Primary sclerosing cholangitis (PSC) ● ● Oriental cholangiohepatitis ● ● Hepatitis C infection Parasitic infections ● ● Opisthorchis viverrini ● ● Clonorchis sinensis Congenital ● ● Choledochal cysts ● ● Caroli’s disease Chemical agents ● ● Thorium dioxide ( Thorotrast ) ● ● Vinyl chloride ● ● Dioxin ● ● Asbestos Post surgical ● ● Biliary–enteric anastomosis

76. Clinical features Early symptoms of cholangiocarcinoma are often non-specific, with abdominal pain, early satiety, anorexia and weight loss commonly seen. Symptoms associated with biliary obstruction ( puritus and jaundice) may be present in a minority of patients. In these patients, examination often demonstrates clinical signs of jaundice, cachexia is often noticeable and a palpable gallbladder present if the obstruction is in the distal common bile duct (Courvoisier’s sign).

77. Investigations Biochemical investigations will confirm the presence of obstructive jaundice (elevated bilirubin, alkaline phosphatase and γ- glutamyltransferase ). The tumour-marker CA 19-9 may also be elevated. Imaging studies such as ultrasound, MDR-CT and MRI/MRCP are essential for diagnosis and staging. These studies allow the level of biliary obstruction to be defined and determine the locoregional extent of disease and the presence of metastases

78. Investigations Direct cholangiography using ERCP or PTC is also used following non-invasive studies. Both can define the level of obstruction and allow access to the biliary system for biopsy and placement of endobiliary stents for biliary drainage. The choice between the modalities depends on local availability and the anatomical site of the tumour. PTC preferred for more proximal lesions and ERCP favoured for distal tumours. Cytology can be obtained from either procedure but it is often non-diagnostic. Positron emission tomography (PET) is useful in detecting lymph node and distant metastases but has limited value in assessment of local resectability .

79. Bismuth-Corlette Classification for cholangiocarcinoma.

80. Treatment A multidisciplinary approach is required in all cases. The choice of treatment depends on the site and extent of the disease. Unfortunately, the majority of patients present with advanced disease. Whether or not the disease is resectable depends on patient factors (comorbidities, presence or absence of chronic liver disease), and tumour factors (extent of disease within the biliary tree, vascular involvement, presence or absence of metastatic disease). Depending on the site of disease, surgery may involve either a standard or extended hepatic resection with en bloc lymphadenectomy and reconstruction of the biliary tree. Distal common duct tumours may require a pancreaticoduodenectomy (Whipple procedure). Local resection should be avoided. In selected patients, liver transplantation has been recommended for those with locally unresectable disease without evidence of distant metastases. Transplantation is often combined with neoadjuvant chemoradiation therapy. Biliary obstruction can be relieved by either endoscopic (ERCP) or percutaneous (PTC) methods. Surgical bypass rarely has a role apart from in patients who present with a distal bile duct lesion and are found to have unresectable disease at operation.

81. Cancer of the gallbladder Highest incidence is among Chileans, American Indians and residents in parts of northern India. ● ● Similar presentation to benign biliary disease i.e., gallstones ● ● Diagnosis by ultrasound, CT, MRI/MRCP ● ● Most patients present with advanced disease ● ● Surgical resection in less than 10% – remainder receive palliative treatment ● ● Prognosis is poor – median survival approximately 6 months Patients may be asymptomatic at the time of diagnosis. Jaundice and anorexia are late features. A palpable mass is a late sign.

82. Investigation Serum CA19-9 is elevated in approximately 80% of patients. The preoperative diagnosis is often made on ultrasonography, and confirmed by a CT scan or MRI/MRCP. Preoperative staging should aim to determine the local extent of disease and exclude the presence of distant metastases. A percutaneous biopsy under radiological guidance is often done to obtain tissue for pathological examination. In selected cases, laparoscopic examination is useful in staging the disease. Laparoscopy can detect peritoneal or liver metastases which would preclude further surgical resection PET scanning also has a role in detecting metastatic disease.

83. Aims of staging gallbladder cancer ● ● Assessment of local disease ● ● Detection of metastatic disease: Liver Peritoneal Lymphatic Extra-abdominal disease

84. Treatment and prognosis Cholecystectomy should be performed for all gallbladder polyps greater than 1 cm. Polyps less than 1 cm can be followed with serial ultrasonography to detect any change in size or character as the incidence of malignancy in polyps less than 1 cm is extremely low. Radical en bloc resection that may include segmental or extended hepatectomy, bile duct resection and regional lymphadenectomy should be considered in selected patients.

85. Treatment and prognosis The aim is to remove the tumour entirely and achieve negative histopathological margins. Patients can have the disease diagnosed following histopathological examination of the gallbladder removed for presumed benign disease. In these cases, the need for further surgery is determined by the stage of disease. For early-stage disease, confined to the mucosa or muscle of the gallbladder, no further treatment is indicated.

86. Treatment and prognosis However, for transmural disease, a radical en bloc resection of the gallbladder fossa and surrounding liver along with the regional lymph nodes should be performed. If the initial procedure was performed laparoscopically, the surgeon should examine the laparoscopic port sites. Routine resection of port sites is no longer recommended. However, it is recognised that the finding of disease at the port sites is a sign of generalised peritoneal disease and carries a very poor prognosis.

87. Thankyou…