54 The gallbladder and bile ducts

R.C.G. RUSSELL Surgical anatomy and physiology The gall bladder is pear-shaped, 7.512cm long, with a normal capacity of about 50 ml, but capable of considerable distension in certain pathological conditions. 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 gall bladder are arranged in a crisscross 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. The cystic duct is about 3 cm in length but variable. Its lumen is usually 13 mm in diameter. The mucosa of the cystic duct is arranged in spiral folds known as the valves of Heister. Its wall is surrounded by a sphincteric structure called the sphincter of Lutkins. While the cystic duct joins the common hepatic duct in its supraduodenal segment in 80 per cent of cases, it may extend down into the retroduodenal or even retropancreatic part of the bile duct before joining. Occasionally the cystic duct may join the right hepatic duct or even a right hepatic sectorial 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 7.5cm 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, running 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. It terminates by opening on the summit of the papilla of Vater. The arterial supply of the gall bladder is critical. It is proposed that arterial damage during cholecystectomy may cause ischaemia and result in postoperative bileduct stricture. The cystic artery, a branch of the right hepatic artery, is usually given off behind the common hepatic duct (Fig. 54.1). Occasionally, an accessory cystic artery arises from the gastroduodenal artery. In 15 per cent of cases the right hepatic artery and/or the cystic artery cross in front of the common hepatic duct and the cystic duct. The most dangerous anomalies are where the hepatic artery takes a torturous course on the front of the origin of the cystic duct, or the right hepatic artery is torturous and the cystic artery short. The tortuosity is known as the caterpillar turn or Moynihans hump (Fig.54.2).This variation is the cause of many problems during a difficult cholecystectomy with inflammation in the region of the cystic duct. Inadvertent damage to the right hepatic artery is most difficult to control lap aroscopically. Lymphatics The lymphatic vessels of the gall bladder (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 gall bladder also connect with the subcapsular lymph channels of the liver, and this accounts for the frequent spread of carcinoma of the gall bladder to the liver. Surgical physiology Bile, as it leaves the liver, is composed of 97 per cent water, 12 per cent bile salts, and 1 per cent pigments, cholesterol and fatty acids. The liver excretes bile at a rate estimated to be approximately 40 ml/hour. The rate of bile secretion is controlled by cholecystokinin which is released from the duodenal mucosa. With feeding there is increased production of bile. Functions of the gall bladder The gall bladder is a reservoir for bile. During fasting resistance to flow through the sphincter is high, and bile excreted by the liver is diverted to the gall bladder. After feeding the resistance to flow through the sphincter of Oddi is reduced, the gall bladder contracts and the bile enters the duodenum. These motor responses of the biliary tract are in part effected by the hormone cholecystokinin. The second main function of the gall bladder is concentration of bile by active absorption of water, sodium chloride and bicarbonate by the mucous membrane of the gall bladder. The hepatic bile which enters the gall bladder becomes concentrated 5 10 times, with a corresponding increase in the proportion of bile salts, bile pigments, cholesterol and calcium. The third function of the gall bladder is the secretion of mucus approximately 20 ml is produced per day. With total obstruction of the cystic duct in a healthy gall bladder, a mucocele develops on account of this function of the mucosa of the gall bladder. Investigation of the biliary tract Plain radiograph The skilfully taken plain X-ray of the gall bladder will show radio-opaque gallstones in 10 per cent of patients (Fig. 54.3). It will also show the rare cases of calcification of the gall bladder, a so-called porcelain gall bladder (Fig. 54.4).The importance of this appearance is that it is premalignant and an indication for cholecystectomy. Limey bile is a curiosity and is frequently related to multiple small stones (Fig. 54.5).This lesion is not a premalignant lesion. Oral cholecystography (GrahamCole test) (Figs 54.6 and 54.7) Iopanoic acid BP is taken as tablets on the night before the examination. A control radiograph is taken before the tablets are given and a series of X-rays is taken on the following day, with further films after a fatty meal. The fatty meal stimulates gallbladder contraction and reveals the adequacy of gallbladder function. This investigation has been discarded by most hospitals because of its inaccuracy except to show diverticulae and polyps, and to assess function; adequate films depend on the patient taking the tablets, and the tablets being absorbed,secreted by the liver and concentrated in the gall bladder after passing into the gall bladder through an unobstructed cystic duct. Thus, a cholecystogram which shows no concentration of contrast can result from many causes and it not diagnostic of gallstone disease. Intravenous cholangiography Intravenous cholangiography (biligrammeglumine ioglycamate) permits radiological visualisation of the bile ducts. The drug is given intravenously and is rapidly secreted by the liver into the biliary tree. Careful radiography with or without tomography can clearly define the ducts and the gall bladder delineating the presence

of stone disease. The contrast agent can cause allergic reactions such that this test has been discarded in most units. Ultrasonography Ultrasonography (Figs 54.8,54.9,54.10,54.11,54.12 and54.13)is noninvasive and is now the standard initial imaging technique for the investigation of the patient suspected of having a gallstone, and is also the prime investigation for the patient presenting with jaundice. It will demonstrate biliary calculi, the size of the gall bladder, the thickness of the gall-bladder wall, the presence of inflammation around the gall bladder, the size of the common bile duct and, occasionally, the presence of stones within the biliary tree. It may even show a carcinoma of the pancreas occluding the common bile duct. Radioisotope scanning Technetium-99m (99mTc)~labelled derivatives of iminodiacetic acid (HIDA, PIPIDA) are excreted in the bile and ate used to visualise the biliary tree. In acute cholecystitis the gall bladder is not seen. The technique is used when biliary enteric anastomoses are functioning inadequately as it will show the extent of obstruction at the anastomosis and indicate the delay in excretion. Computerised tomography (CT) CT is not a useful technique in investigating the biliary tree. Its only value is in the investigation of patients who may have a cancer of the gall bladder or bile ducts, and in these patients will define its extent, the presence of lymphadenopathy and the presence of metastases. Magnetic resonance cholangiopancreatography (MRCP) MRCP is now becoming the standard technique for investigation of the biliary tree. Contrast is not necessary and, with appropriate computing, a clear outline of the biliary tree can be achieved with a sensitive and specific diagnosis of bile-duct stones. This technique will replace alternative diagnostic aids as the appropriate magnets with the specific software become more widely available (Fig. 54.14). Endoscopic retrograde cholangiopancreatography (ERCP) The ampulla of Vater can be cannulated with the aid of a fibre-optic duodenoscope. The bile ducts are visualised after injecting water-soluble contrast. Bile can be sent for cytological and microbiological examination, and brushings can be taken from strictures for cytological studies. Acute cholangitis may follow ERCP when contrast fills a dilated and obstructed duct; antibiotics are given as prophylaxis, and if obstruction is encountered relief of that obstruction by the placement of a stent must be undertaken. If drainage cannot be achieved then percutaneous transhepatic drainage should be performed.

Diagnostic ERCP is now less commonly performed, but its value is its ability to remove stones and stent strictures, thus becoming a therapeutic rather than a diagnostic technique (Figs 54.15,54.16,54.17,54.18,54.19 and 54.20). This investigation is only undertaken once a bleeding tendency has been excluded and the patients prothrombin time is normal. Antibiotics should be given prior to the procedure. Under fluoroscopic control, a needle (the Chiba or Okuda needle) 15 cm long and 0.7 mm in diameter is advanced into the liver through the eighth intercostal space in the midaxillary line to a point about 2 cm short of the right margin of the vertebral column. The stilette is then removed and while injecting contrast (e.g. meglumine iothalamate 60 per cent, w/v) the needle is slowly withdrawn until contrast is seen entering a bile radical. Addition to 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. Peroperative cholangiography During cholecystectomy a catheter can be placed in the cystic duct and contrast injected into the biliary tree. The technique defines the anatomy and excludes the presence of stones. With improved preoperative imaging and a more careful operative approach the value of this technique is debatable. The limitation of the technique using single plates can be overcome by an X-ray image intensifier with a television monitor which enables a much more accurate diagnosis of biliary pathology (Figs 54.23, 54.24, 54.25). Operative biliary endoscopy (choledochoscopy) At operation a flexible fibre-optic endoscope can be passed down the cystic duct into the common bile duct enabling stone identification and removal under direct vision. The technique can be combined with an X-ray image intensifier to ensure complete clearance of the biliary tree. After exploration of the bile duct, a tube can be left in the cystic duct remnant or in the common bile duct (a T-tube) and drainage of the biliary tree established. After 710 days, a track will be established. This track can be used for the passage of a choledochoscope to remove residual stones in the awake patient in an endoscopy suite. This technique is invaluable in the management of difficult stone disease and prevents the excessive prolongation of an operative exploration of the common bile duct. Congenital abnormalities of the gall bladder and bile ducts Embryology The hepatic diverticulum arises from the ventral wall of the foregut and elongates into a stalk to form the choledochus. A lateral bud is given off which is destined to become the gall bladder and cystic duct. The embryonic hepatic duct sends out many branches which join up the canaliculi between the liver cells. As is usual with embryonic tubular structures, hyperplasia obliterates the lumina of this ductal system; normally recanalisation subsequently occurs and bile begins to flow. During early foetal life the gall bladder is entirely intrahepatic. Absence of the gall bladder Occasionally the gall bladder is absent. Failure to visualise the gall bladder is not necessarily a pathological problem. The Phrygian cap

The Phrygian cap (Fig. 54.26) is present in 26 per cent of cholecystograms and may be mistaken for a pathological deformity of the organ. Phrygian cap refers to hats worn by people of Phrygia, an ancient country of Asia Minor; it was rather like a liberte' cap of the French Revolution. Floating gall bladder The organ may hang on a mesentery which makes it liable to undergo torsion. Double gall bladder Rarely, the gall bladder is twinned. One of the twins may be intrahepatic. Absence of the cystic duct This is 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 The cystic duct opens into the common bile duct near the ampulla. All variations of this anomaly can occur. At operation they are not important. Dissection of a cystic duct which is inserted low in the bile duct should be avoided as removal will damage the blood supply to the common bile duct and can lead to stricture formation. An accessory cholecystohepatic duct Ducts passing directly into the gall bladder from the liver do occur and are probably not uncommon. Nevertheless, larger ducts should be closed but before doing so the precise anatomy should be carefully ascertained (Fig. 54.27). Extrahepatic biliary atresia A etiology and pathology Atresia is present in one per 14 000 live births, and affects male and females equally. The extrahepatic bile ducts are progressively destroyed by an inflammatory process which starts around the time of birth. Intrahepatic changes also occur and eventually result in biliary cirrhosis and portal hypertension. The untreated child dies before the age of 3 years of liver failure or haemorrhage. The inflammatory destruction of the bile ducts has been classified into three main types (Fig. 54.28): 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 include, in about 20 per cent of cases, cardiac lesions, polysplenia, situs inversus, absent vena cava and a pre duodenal portal vein. Clinical features About one-third of cases are jaundiced at birth. In all, however, jaundice is present by the end of the first week and deepens progressively. 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). Pruritis is severe. Clubbing and skin xanthomas, probably related to a raised serum cholesterol, may be present. Differential diagnosis This includes any form of jaundice in a neonate giving a cholestatic picture. Examples are alpha1-antitrypsin deficiency, cholestasis associated with intravenous feeding, choledochal cyst and inspissated bile syndrome. Neonatal hepatitis is the most difficult to differentiate. Both extrahepatic biliary atresia and neonatal hepatitis are associated with giant cell transformation of the hepatocytes. Liver biopsy and radionuclide excretion scans are essential.

Treatment Patent segments of proximal bile duct are found in 10 per cent of type I lesions. A direct Roux-en-Y anastomosis will achieve bile flow in 75 per cent, but progressive fibrosis results in disappointing long-term results. Type II and III 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. 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 per cent of children whose bilirubin falls to within the normal range can be expected to survive for 10 years or more. Early referral for surgery is critical. Postoperative complications include bacterial cholangitis, which occurs in 40 per cent. Repeated attacks lead to hepatic fibrosis and 50 per cent of long-term survivors develop portal hypertension, with one-third having variceal bleeding. Liver transplantation may be considered in the failures. Congenital dilatation of the intrahepatic ducts (Carolis disease) This rare, congenital, nonfamilial condition is characterised by multiple irregular sacular dilatations of the intrahepatic ducts separated by segments of normal or stenotic ducts. Biliary stasis leads to stone formation and cholangitis. The patients present in childhood or in early adult life. Associated conditions include congenital hepatic fibrosis, medullary sponge kidney and, rarely, cholangiocarcinoma. The mainstays of treatment are antibiotics for the cholangitis and the removal of calculi. As the condition can be limited to one lobe of the liver, lobectomy may be indicated. Choledochal cyst Choledochal cyst is due to a specific weakness in a part of or the whole of the wall of the common bile duct. Anomalous junctions of the biliary pancreatic junction are frequently observed and long common channels result in high levels of biliary amylase in 80 per cent of cases. Common pancreatobiliary channels may be associated with repeated attacks of pancreatitis (Fig. 54.29). The patient may present at any age with attacks of jaundice of obstructive type, cholangitis and abdominal signs. In some patients a swelling may be detected in the upper abdomen. Ultrasonography will confirm the presence of an abnormal cyst and a magnetic resonance imaging (MRI) scan will reveal the anatomy; in particular, the relationship between the lower end of the bile duct and the pancreatic duct. It appears that the anomaly is premalignant; carcinoma of the biliary tract is a well-recognised complication and carries a poor prognosis, and therefore excision is the appropriate management. Radical excision of the cyst is the treatment of choice with reconstruction of the biliary tract using a Roux-en-Y loop of jejunum. Other procedures have been shown to be ineffective and associated with recurrent attacks of cholangitis. Trauma Injuries to the gall bladder and extrahepatic biliary tree are rare. They occur as a result of a penetrating wound or a crush injury. Operative trauma is perhaps more frequent than external trauma. The physical signs are those of an acute abdomen. The treatment is cholecystectomy for gall bladder injuries and if the bile duct is damaged then drainage using a T-tube to the bile duct and a drain to the site of damage is appropriate. Many will stricture and these can be secondarily be repaired by a Rouxen-Y anastomosis. Torsion of the gall bladder

This is very rare and relies on a long mesentery which often occurs in an older patient with a large mucocele of the gall bladder. The patient presents with extreme pain and an acute abdomen. Immediate exploration is indicated with cholecystectomy as the only treatment. Gallstones (cholelithiasis) Gallstones are the most common biliary pathology. Indeed, cholecystectomy is the commonest surgical procedure in the abdomen in the Western world. Gallstones are classified according to their chemical composition into cholesterol stones, mixed stones and pigment stones. Cholesterol stones consist almost entirely of cholesterol and are often solitary (cholesterol solitaire). Mixed stones account for 90 per cent of gallstones. Cholesterol is the major component. Other components include calcium bilirubinate, calcium phosphate, calcium carbonate, calcium palmitate and proteins. Usually they are multiple, and they are often faceted. Pigment stones are most common in the Far East and are composed almost entirely of calcium bilirubinate. They are mostly small and multiple. Some are hard and coral-like, whereas others are soft and really concretions of sludge rather than stones (Fig. 54.30). Gas in gallstones Rarely the centre of a stone may contain radiolucent gas in a triradiate or biradiate fissure and this gives rise to characteristic dark shapes on a radiograph the Mercedes Benz or seagull signs. Limey bile Lime-water bile is revealed on a plain radiograph (Fig. 54.5)more clearly than if the gall bladder has been visualised by cholecystography. The opacity is the result of the gall bladder becoming filled with a mixture of calcium carbonate and calcium phosphate, usually the consistency of toothpaste. The condition tends to occur when there is a gradual obstruction of the cystic or common bile duct, for example due to chronic pancreatitis or carcinoma of the pancreas. Organisms are rarely grown from the emulsion. Incidence of gallstones A fat, fertile, flatulent, female of fifty is the classical sufferer from symptomatic gallstones. Useful as this clinical memorandum is, it should be tempered with the knowledge that cholelithiasis occurs in both sexes from childhood to the centenarian. In men the disease tends to occur in the older age groups at which point the incidence is equal to that of women. Stones are rarer in Africa and in southern India, but not in north India. Causal factors in gallstone formation The aetiology of gallstones is probably multifactorial. Factors implicated are: (1) metabolic; (2) infective; and (3) bile stasis. Cholesterol and mixed stones Metabolic Cholesterol, insoluble in water, is held in solution by a detergent action of bile salts and phospholipids with which it forms micelles (Fig. 54.31).Bile containing cholesterol stones has an excess of cholesterol relative to bile salts and phospholipids, thus allowing cholesterol crystals to form. Such bile is termed supersaturated or lithogenic. Bile cholesterol increases with age and is raised in women, particularly those taking the contraceptive pill, in obesity and by clofibrate a drug used in the treatment of certain hyperlipoproteinaemias. The concentration of bile salts in bile is reduced by oestrogens, and also by factors which interrupt the intrahepatic circulation of bile salts (e.g. ileal disease, resection or bypass and cholestyramine therapy). These conditions are all associated with an increased incidence of stones, but there are still

some people with cholesterol supersaturation who remain free of stones, suggesting that there are other factors of importance. Infection The role of infection in causing stones is unclear. Often bile from patients with gallstones is sterile, but organisms have been cultured from the centres of gallstones: the radiolucent centre of many gallstones may represent mucus plugs originally formed around bacteria (Moynihans aphorism: A gallstone is a tombstone erected to the memory of the organism within it). Helicobacter pylori antigens have been isolated within gall bladders containing stones. Bile stasis Gall bladder contractility is reduced by oestrogens, in pregnancy and after truncal vagotomy, situations in which the incidence of gallstones is increased. Patients on long-term parenteral nutrition have a high incidence of stones. Lack of good oral intake precludes the release of cholecystokinin, the hormonal stimulant of gallbladder contraction released from the duodenal mucosa. Pigment stones are seen in patients with haemolysis in which bilirubin production is increased. Examples are hereditary spherocytosis, sickle cell anaemia, thalassaemia, malaria and mechanical destruction of red cells by prosthetic heart valves. Pigment stones are found in the ducts of patients with benign and malignant bile duct strictures. Pigment stones in Oriental countries are associated with infestations of the biliary tree by Clonorchis sinensis and Ascaris lumbricoides. Escherichia coli is often found in the bile of these patients. This bacterium produces the enzyme t3glucuronidase which converts the bilirubin into its unconjugated insoluble form. These stones are often present throughout the biliary tree including the intrahepatic ducts. The effects and complications of gallstones Stones are found throughout the biliary tract and their complications relate to obstruction of the cystic duct, of the intrahepatic radicals or of the ampulla of Vater. Obstructive complications may be aggravated by the presence of infection leading to cholangitis and abscess formation. Nevertheless, gallstones can be asymptomatic; it is estimated that between 85 and 90 per cent of patients who have gallstones remain asymptomatic. In the UK the prevalence of gallstones at the time of death is estimated to be 17 per cent and possibly increasing. Thus, the mere presence of gallstones is not an indication for a surgical approach. For this reason symptoms must be analysed with care. A typical patient may fulfil Saints triad having gallstones, diverticulosis of the colon and a hiatus hernia, yet with symptoms that cannot be directly contributed to any of these. When considering management of a patient with gastrointestinal symptoms it is important to take a specific history and consider whether or not the pain from which the patient suffers is typical or not of biliary tract disease. Effects and complications of gallstones In the gall bladder: Silent stones Chronic cholecystitis Acute cholecystitis Gangrene Perforation Empyema Mucocele Carcinoma In the bile ducts:

 Obstructive jaundice Cholangitis Acute pancreatitis In the intestine: Acute intestinal obstruction (gallstone ileus) Cholecystitis It is probably inappropriate, although classical, to subdivide chronic and acute calculous cholecystitis. They are part of the same spectrum of disease and are related to inflammation within the gall bladder secondary to obstruction of the cystic duct by stones. With stones in a gall bladder it appears that there is always some degree of inflammatory change, but there is insufficient evidence to suggest that this is a cause of symptoms. The concept of flatulent dyspepsia being caused by gallstones in the absence of the classic symptoms of biliary colic is probably inappropriate. Nevertheless, some patients do complain of right hypochondrial pain of varying severity in association with nausea and occasional vomiting, and some tenderness in the right subcostal region. Flatulent dyspepsia is common in such patients yet many will not be relieved by cholecystectomy. Numerous investigations have been performed to determine whether those with symptoms of dyspepsia will benefit from cholecystectomy; all have proved ineffective and thus the surgeon must rely on clinical judgment. Clinical features The patient has specific episodes of right subcostal pain radiating to the back and to the shoulder. Occasionally the pain starts on the left subcostal side or even in the epigastrium, but at its most severe it is invariably on the right side. 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. As the pain resolves the patient improves and is able to eat and drink again, often only to suffer further episodes. It is of interest that the patient may have several episodes of this nature over a period of a few weeks and then no more trouble for some months. If the pain does not resolve the patient will become more systemically unwell as infection supervenes. This is associated with a continuous pain, nausea, vomiting and pyrexia. On examination the patient will be tender in the right subcostal area and may develop guarding, even rigidity, and later a mass may be palpable as the omentum walls off an inflamed gall bladder. Fortunately, this process is limited by the stone slipping back into the body of the gall bladder and the contents of the gall bladder escaping by way of the cystic duct. This achieves adequate drainage of the gall bladder and enables the inflammation to resolve. If resolution does not occur the gall bladder may perforate with the development of localised peritonitis or an abscess may form; the abscess may then perforate into the peritoneal cavity with a septic peritonitis this is uncommon, however, because the gall bladder is usually localised by omentum around the perforation. When examining a patient with acute cholecystitis itshould be noted whether the patient is pyrexial, is jaundiced or dehydrated. His or her respiration should be noted to determine whether there is pain on deep inspiration. On examining the abdomen it is important to determine whether the movement is normal or shallow breathing is

present with intestinal distension. The abdomen should be palpated gently, working towards the subcostal area where tenderness with guarding may be noted. In some patients a mass may be palpable. Bowel sounds are usually present but reduced. Differential diagnosis Conditions commonly presenting similarly to acute cholecystitis are appendicitis, perforated peptic ulcer and acute pancreatitis. Occasionally acute pyelonephritis of the right kidney, myocardial infarction and right lower lobe pneumonia may lead to confusion. The diagnosis is confirmed by ultrasonography which should show the presence of stones in an inflamed gall bladder with oedema around the gall bladder wall (Fig. 54.32). The stone can often be observed impacted in the infundibulum. A serum amylase estimation should be performed to exclude pancreatitis, and liver functions tests performed to determine whether or not jaundice is present. A mild elevation of the bilirubin can merely be due to oedema around the portahepatis or obstruction of the biliary tree by a stone escaping into the common bile duct. The distended bile duct should be noted on ultrasonography. A chest X-ray will exclude pneumonia, and if there is doubt concerning a cardiac origin then an electrocardiogram should be performed. Renal disease can be excluded by sending the urine for microscopy and culture. Treatment Conservative treatment followed by cholecystectomy Experience shows that in more than 90 per cent of cases the symptoms of acute cholecystitis subside with conservative measures. Nonoperative treatment is based on four principles: nasogastric aspiration and intravenous fluid administration; 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. cephazolin, cefuroxime or gentamycin); subsequent management when the temperature, pulse and other physical signs show that the inflammation is subsiding, the nasogastric tube is removed and oral fluids followed by a fat-free diet are given. Ultrasonography is performed to ensure that no local complications have developed, that the bile duct is of a normal size and that no stones are contained in the bile duct. Cholecystectomy may either be performed on the next available list, or the patient is allowed home to return later when the inflammation has completely resolved. Conservative treatment is not advised when there is uncertainty about the diagnosis and the possibility of a high retrocaecal appendix or a perforated duodenal ulcer cannot be excluded. Conservative treatment must be abandoned if the pain and tenderness increase; in this case a percutaneous cholecystostomy performed by the radiologist under ultrasound control will rapidly relieve symptoms. Subsequent cholecystectomy will be required. Routine early operations Some surgeons advocate urgent operation as a routine measure in cases of acute cholecystitis. Provided that the operation is undertaken within 48 hours of the onset of the attack, the surgeon is experienced and excellent operating facilities are available, good results are claimed. Nevertheless, the conversion rate in laparoscopic cholecystectomy is five times higher in acute than in elective surgery. Mucocele of the gall bladder

This occurs when the neck of the gall bladder becomes obstructed by a stone but the contents remain sterile. The bile is absorbed and replaced by mucous secreted by the gall bladder epithelium. The gall bladder may be palpable. Enormous sizes and shapes have been encountered. A mucocele also occurs in those cases of malignancy which occlude the cystic duct, for instance a cholangiocarcinoma. Empyema of the gall bladder The gall bladder appears to be filled with pus but, surprisingly in over half of cases, bacteria cannot be cultured from this pus. It may be a sequel of acute cholecystitis or the result of a mucocele becoming infected. The treatment is drainage and, later, cholecystectomy (Fig. 54.33). Acalculous cholecystitis Acute and chronic inflammation of the gall bladder can occur in the absence of stones and give rise to a clinical picture similar to calculous cholecystitis. Some patients have nonspecific inflammation of the gall bladder, whereas others have one of the cholecystoses. Oral cholecystography is more useful than ultrasonography in the diagnosis of those patients presenting with chronic symptoms, and radioisotope scanning in those presenting acutely. The identification of cholesterol crystals in a duodenal aspirate may also help. Acute acalculous cholecystitis is particularly seen in patients recovering from major surgery, trauma and burns. In these patients the diagnosis is often missed and the mortality rate is 20 per cent. The cholecystoses (cholesterosis, polyposis, adenomyomatosis and cholecystitis glandularis proliferans) This is a not uncommon group of conditions affecting the gall bladder in which there are chronic inflammatory changes with hyperplasia of all tissue elements. Cholesterosis (strawberry gall bladder) In the fresh state the interior of the gall bladder looks something like a strawberry; the yellow specks (submucous aggregations of cholesterol crystals and cholesterol esters) correspond to the seeds (Fig. 54.34).It may be associated with cholesterol stones. Cholesterol polyposis of the gall bladder Cholecystography shows negative shadows in a functioning gall bladder, or on ultrasound there is a well-defined polyp present. These are either cholesterol polyposis or adenomatous change. With improving ultrasonography they are seen more frequently and surgery advised only if they change in size. Figure 54.35 summarises the varieties of this condition. A polyp of the mucous membrane is fleshy and granulomatous. All layers of the gall bladder wall may be thickened but sometimes an incomplete septum forms which separates the hyperplastic from the normal. Intraparietal mixed calculi may be present. These can be complicated by an intramural, and later extramural, abscess. If symptomatic, the patient is treated by cholecystectomy. Diverticulosis of the gall bladder Diverticulosis of the gall bladder is usually manifest as black pigment stones impacted in the out-pouchings of the lacunae of Luschka. Diverticulosis of the gall bladder may be demonstrated by cholecystography, especially when the gall bladder contracts after a fatty meal. There are small dots of contrast medium just outside the gall bladder (Fig. 54.36).A septum may also be present (to be distinguished from the Phrygian cap Fig. 54.26) and the treatment is cholecystectomy. Typhoid gall bladder Salmonella typhi (Typhoid Mary, a cook-general who passed Salmonella typhi in her faeces and urine, was responsible for nearly a score of epidemics of typhoid in and around New York City) or, occasionally, Salmonella typhimunium can infect the gall

bladder. Acute cholecystitis can occur. Mote frequently, chronic cholecystitis occurs, the patient being 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 gall bladder to chronic infection. Salmonellae can, however, frequently be cultured from these stones. Ampicillin and cholecystectomy are indicated. Cholecystectomy Preparation for operation After appropriate history taking and assessment of the patients fitness for the procedure, which will include investigation of the cardiovascular and respiratory systems if history suggests these to be a risk factor, a full blood count and biochemical profile are done to exclude abnormal liver function tests or anaemia. Blood coagulation is checked if there is a history of jaundice. The patient is given prophylactic antibiotics either with the premedication intramuscularly, or intravenously at the time of induction. A second-generation cephalosporin is appropriate. Subcutaneous heparin or antiembolus stockings are prescribed. A consent form is signed ensuring that the patient is fully aware of the procedure being undertaken, the risks involved and complications that may occur. Laparotomy A short right upper transverse incision is made centred over the lateral border of the rectus muscle. A full laparotomy, inspecting all abdominal organs, is undertaken and the diagnosis of gallstones confirmed. The gall bladder is appropriately exposed and packs are placed on the hepatic flexure of the colon, the duodenum and the lesser omentum to ensure a clear view of the anatomy of the porta hepatis. These packs may be retracted using the hand of the assistant (It is the left hand of the assistant that does all the work Moynihan) or a stabilised ring retractor used to keep the packs in position (Fig. 54.37).An artery forceps is placed on the infundibulum of the gall bladder and the peritoneum overlying Calots triangle is placed on a stretch. The peritoneum is then divided close to the wall of the gall bladder and the fat in the triangle of Calot carefully dissected away to expose the cystic artery and the cystic duct. The cystic duct is cleaned down to the common bile duct whose position is clearly ascertained. The cystic artery is tied and divided. The whole of the triangle of Calot is displayed to ensure that the anatomy of the ducts is clear and the cystic duct is then divided between ligatures. The gall bladder is then dissected away from the gall-bladder bed. Some golden rules in case of difficulty. When the anatomy of the triangle of Clot is unclear, blind dissection should stop. Bleeding adjacent to the triangle of Calot should be controlled by pressure and not by blind clipping or clamping. When there is doubt about the anatomy a fundus-flrst cholecystectomy dissecting on the gall-bladder wall down to the cystic duct can be helpful. If the cystic duct is densely adherent to the common bile duct and there is the possibility of a Mirizzi syndrome (the stone ulcerating through into the common duct), the infundibulum of the gall bladder should be opened, the stone removed and the infundibulum oversewn. A cholecystostomy is almost never indicated, but if it has to be done as many stones as possible should be extracted and a large Foley catheter (14 French) placed in the

fundus of the gall bladder with a direct track externally. By so doing, should stones be left behind in the gall bladder, these can be extracted with a choledochoscope. The technique of open cholecystectomy has largely been superseded by laparoscopic cholecystectomy. Nevertheless, no surgeon undertaking laparoscopic cholecystectomy should lose the different technique of undertaking an open cholecystectomy as this may be required when difficulties are encountered laparoscopically. Laparoscopic cholecystectomy The preparation and indications for cholecystectomy are the same whether it is performed by laparoscopy or by open techniques. However, a laparoscopic cholecystectomy should only be performed by a surgeon who is frequently undertaking laparoscopic procedures as the skills are different from those required in undertaking an open cholecystectomy. The patient is positioned either in a Lloyd-Davies position or flat on the table depending on whether the French or American approach is used by the surgeon. A pneumoperitoneum is created and four ports are placed in the abdomen, usually at the umbilicus and the epigastrium, with two 5-mm ports laterally. The triangle of Calot is laid widely open by dividing the peritoneum on the posterior and on the anterior aspect. The cystic duct is carefully defined as is the cystic artery which is divided. Once the triangle of Calot has been laid widely open the cystic duct is clipped and divided. The gall bladder is then removed from the gall bladder bed and once free removed via the umbilicus (Fig. 54.40). Indications for choledochotomy In an environment where the modern diagnostic armamentarium described at the beginning of this chapter is not available and neither is peroperative cholangiography, it is well to rehearse the traditional indications for choledochotomy, which are: (1) palpable duct stones; (2) there is jaundice or a history of jaundice or cholangitis; (3) the common bile duct is dilated; and (4) the liver function tests are abnormal, in particular, the alkaline phosphatase is raised. In centres where adequate facilities are available it is probably inadvisable to do a choledochotomy laparoscopically, but rather to rely on endoscopic techniques unless particular expertise has been achieved in laparoscopic exploration of the bile duct. The incidence of symptomatic stones in the bile duct varies from 5 to 8 pet cent. These can, in the main, be dealt with endoscopically without resort to opening the duct. However, current trials suggest that in experienced hands the morbidity of the two techniques is identical. Symptoms persisting after cholecystectomy In 15 per cent of patients cholecystectomy fails to relieve the symptoms for which the operation was performed. Such patients may be considered to have a postcholecystectomy syndrome. However, such problems are usually related to the preoperative symptoms and are merely a continuation of those symptoms. Full investigation should be undertaken to exclude the presence of a stone in the bile duct, a stone in the cystic duct stump or operative damage to the biliary tree. This is best performed by an MRCP or an ERCP which has the added advantage that if a stone remains it can be removed. Management of bile-duct obstruction Patients with symptoms after a cholecystectomy, particularly if jaundice is present, demand urgent investigation. Any patient who does not make an uneventful recovery following gall-bladder surgery should be investigated by ultrasound scanning as a

matter of immediacy. If jaundice (either biochemical or clinical) is present emergency measures are required. The first step in management is to undertake an immediate ultrasound scan. If there is evidence of fluid in the subhepatic space or obstruction of the biliary tree as shown by bile-duct dilatation then an immediate ERCP should be performed to ascertain whether a stone is present or there is obstruction of the common bile duct due to damage at the time of surgery (Fig. 54.41). If a stone is present immediate removal endoscopically is indicated. If the common bile duct is obstructed by clips or there is leakage from the biliary tree due to a cystic duct leak then a drain should be placed in the subhepatic space percutaneously and a stent placed in the bile duct where possible. Small leaks will usually resolve spontaneously. Should the common bile duct be damaged the patient should be referred to an appropriate expert for reconstruction of the duct. Stones in the bile duct Duct stones may occur many years after a cholecystectomy or be related to the development of new pathology, such as infection of the biliary tree or infestation by Ascaris lumbricoides or Clinorchis sinensis. Any obstruction to the flow of bile can give rise to stasis with the formation of stones within the duct. The consequences of duct stones are either obstruction to bile flow or infection-cholangitis. Stones in the bile ducts are more often associated with infected bile (80 per cent) than are stones in the gall bladder. Symptoms The patient may be asymptomatic but usually has bouts of pain, jaundice and fever. The patient is often ill and feels unwell. The term cholangitis is given to the triad of pain, jaundice and fevers sometimes known as Charcots triad. Signs Tenderness may be elicited in the epigastrium and the right hypochondrium. In the jaundiced patient it is useful to remember Courvoisiers law in obstruction of the common bile duct due to a stone, distension of the gall bladder seldom occurs; the organ usually is already shrivelled. In obstruction from other causes distension is common by comparison. However, if there is no disease in the gall bladder and the obstruction is due to a cancer of the ampulla, pancreas or bile duct, then the gall bladder may well be distended. Management It is essential to determine whether the jaundice is due to liver disease, disease within the duct such as sclerosing cholangitis or obstruction. Ultrasound scanning, liver function tests, liver biopsy if the ducts are not dilated, and MRI or ERCP will demarcate the nature of the obstruction. The patient may be ill. Pus may be present within the biliary tree and liver abscesses may be developing. Full supportive measures are required with rehydration, attention to clotting, exclusion of diabetes and starting the appropriate broad-spectrum antibiotics. As soon as resuscitation has taken place, relief of the obstruction is essential. Endoscopic papillotomy is the preferred first technique with a sphincterotomy, removal of the stones using a Dormia basket (Fig. 54.42) or the placement of a stent if stone removal is not possible. If this technique fails, a percutaneous transhepatic cholangiogram can be performed to provide drainage and subsequent percutaneous choledochoscopy. Surgery, in the form of choledochotomy,

is now rarely used for this situation as most patients can be managed by minimally invasive techniques (Fig. 54.43). Choledochotomy If a stone (or stones) is present in the common bile duct ,removal should have priority over cholecystectomy. Should the patient be unfit for cholecystectomy, or even cholecystostomy, the gall bladder should be removed on a future occasion (a living problem is better than a dead cert Grey Turner). In particular, this may be the case in suppurative cholangitis. Recent evidence suggests that subsequent cholecystectomy may not be necessary. After endoscopic removal of stones, only 10 per cent of patients will have subsequent problems with their gall bladder. Supraduodenal choledochotomy Most stones in the common bile duct can be removed by this route. If, as is often the case, a stone can be felt, an attempt is made to manoeuvre it into a position midway between the entrance of the cystic duct and the superior border of the duodenum. The stone is steadied between the finger and thumb. The duct is opened longitudinally directly on to the stone, enabling it to be removed by a malleable scoop or Desjardins gallstone forceps. The interior of the duct is then explored upwards and downwards with the scoop for further stones. When the stone cannot be felt, or cannot be manipulated into the optimum position just described, 2 cm of the common bile duct is exposed, two stay sutures are placed in the duct and a longitudinal incision into the duct is made between them. Escaping bile is mopped up or removed by suction. Through this opening it may be possible to identify the stones and remove them with a scoop or forceps (Fig. 54.44). A balloon catheter, similar to that used for embolectomy, and irrigation of the ducts with saline are useful additional methods. Choledochoscopy may be employed to confirm that all calculi have been removed. Usually drainage ofthe common bile duct is carried out by means of a T-tube (Fig. 54.45);T-tubes should be made of latex or rubber and used only once plastic tubes are hardened by the bile and are difficult to remove. Latex and rubber stimulate fibrinous adhesion of the omentum to liver and colon to form a safe track. There is very little reaction to a plastic tube and therefore the risk of biliary peritonitis is greater. The transverse limb, shortened if necessary to about 5 cm long, is inserted in the duct which is closed snugly about the vertical limb, using fine catgut on an atraumatic needle. The long limb is brought out through a separate stab wound laterally, as this facilitates the Burhenne procedure should it subsequently be required for a retained stone. The bile draining from the tube is collected in a plastic bag by the side of the bed, its amount and character being noted. After 10 days the tube may be clamped for increasing periods, and the absence of pain and jaundice and the presence of bile in the stools indicate satisfactory flow into the duodenum. Sodium diatrizoate is injected down the tube to obtain a cholangiogram, and if there are no filling defects in a well-outlined duct, and the contrast enters the duodenum freely, the T-tube can be removed. Subsequent bile drainage is minimal and does not usually persist for more than 1 day. Closure of the common duct without a T-tube If this procedure is attempted, it is essential to provide drainage placed in apposition to the common duct. Transduodenal sphincterotomy Transduodenal sphincterotomy is indicated when a stone is found to be impacted near the ampulla of Vater (Fig. 54.46) and it cannot be retrieved from above. Other indications are when the common bile duct is dilated and contains multiple stones and biliary sludge, and when the papilla is fibrosed and stenosed secondary to the passage

of stones through it. Some surgeons prefer the method to supraduodenal choledochotomy to remove all duct stones. If the supraduodenal approach fails to clear the duct, it is preferable to place a large T-tube in the duct (14 or 16 Fr) and close the abdomen. Subsequently, the stone can be removed by the Burhenne procedure or endoscopically. The combination of a supraduodenal approach with a transduodenal approach frequently leads to complications. The duodenum is opened in its second part between stay sutures and the region of the ampulla brought into the opening by traction using tissue forceps. Removal of the stone or stones requires division of the duodenal papilla and the sphincter. A grooved director is passed through the papillary opening and up into the bile duct where it must be palpated. The papilla and part of all the sphincter are now divided at 10 oclock. If the bile-duct mucosa is sutured to that of the duodenum, the procedure is called a sphincteroplasty. Before sutures are placed in the papilla, it is essential to identify the pancreatic duct. Choledochoduodenostomy Choledochoduodenostomy is an alternative to transduodenal sphincterotomy when the common bile duct is dilated and contains multiple stones and sludge, particularly in elderly people (Fig. 54.47). The operation is contraindicated if the common duct is not 15 mm or more in diameter, or it is impossible to make a stoma of 23 cm. The convalescence is usually surprisingly placid. It is, indeed, a procedure which has commanded much support. Stricture of the bile duct BenignStricture (1)Postoperative (80%) (2) Inflammatory (20%) Malignant Stricture Postoperative stricture Postoperative strictures concern either the common bile duct or the common hepatic duct. In a few cases only the right hepatic duct is implicated. The stricture is the result of a preventable error in technique during the performance of cholecystectomy. Blind plunge application of a haemostat to a bleeding cystic or accessory cystic artery, or to the right hepatic artery, is likely to damage the common hepatic duct (Fig. 54.48). The prevention of this tragic happening is standardised. All unexpected haemorrhage in this region should be controlled initially by inserting an index finger into the foramen of Winslow, and pinching the free edge of the gastrohepatic omentum between the finger and thumb. Temporary compression of the hepatic artery in this way allows the bleeding point to be visualised and ligated securely (Hogarth Pringles manoeuvre). Should cholecystectomy be performed by dissecting from the fundus (fundus-first procedure) too much traction applied to the freed gall bladder may so tent the common bile duct that any forceps intended for the cystic duct grasp the angulated main channel (Fig. 54.49)(forceps should not, in fact, ever be used for grasping the cystic duct prior to ligature Fig. 54.38). Failure to identify the anatomy in Calots triangle when there is much inflammation. The common hepatic duct is tied instead of the cystic duct. Ignorance of the anatomical anomalies of the bile ducts. Laceration of the common bile duct while exploring it for stones. In 3 per cent of cases of stricture of the common bile duct, injury occurs during related surgical procedures.

About 15 per cent of injuries to the bile ducts are not recognised at the time of operation. In 85 per cent of cases the injury declares itself postoperatively by: (1) a profuse and persistent leakage of bile if drainage has been provided, or bile peritonitis if such drainage has not been provided; and (2) deepening obstructive jaundice. When the obstruction is incomplete, jaundice is delayed until subsequent fibrosis renders the lumen of the duct inadequate. Radiological investigation of biliary strictures Ultrasonography Cholangiography via I-tube, if present ERCP Transhepatic cholangiography Treatment In the debilitated patient, temporary external biliary drainage may be achieved by passing a catheter percutaneously into an intrahepatic duct. Also, stents may be passed through strictures at the time of ERCP and left to drain into the duodenum. When the general condition of the patient has improved, definitive surgery can be undertaken. However, both of these methods may be complicated by cholangitis and are not recommended for all cases. For benign stricture or duct transection, the preferred treatment is immediate Rouxen-Y choledochojejunostomy by a surgeon well versed in managing benign postoperative strictures. For a stricture of recent onset through which a guidewire can be passed, balloon dilatation with insertion of a stent is an acceptable alternative provided that the services of an experienced endoscopist are available. Primary sclerosing cholangitis This is a chronic fibrosing inflammatory condition of the biliary tree which affects both intrahepatic and extrahepatic ducts, and may involve the gall bladder and pancreas. It is of unknown origin and must be distinguished from secondary sclerosing cholangitis associated with choledocholithiasis. It is associated with inflammatory bowel disease, usually ulcerative colitis, in 5070 per cent of cases, and these patients may be at greater risk of developing a bile-duct carcinoma. The patients are commonly young, being less than 50 years old. They present with a progressive cholestatic disorder and right upper quadrant discomfort, jaundice, pruritis and fever. Investigation reveals a considerable elevation of the alkaline phosphatase, and on cholangiography stricturing and beading of the bile ducts (Fig. 54.50).As the majority of patients has both intrahepatic and extrahepatic biliary tree involvement, surgical treatment is not appropriate. Primarily, these patients are managed with antibiotics, vitamin K, cholestyramine, steroids and azothiaprine, but with little benefit. Repeated dilatation of the strictures is helpful. Many go on to develop cirrhosis due to obstruction. If liver failure supervenes these patients are suitable candidates for liver transplantation. Parasitic infestation of the biliary tract Biliary ascariasis The round worm, A. lumbnicoides, commonly infests the intestine 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 gall bladder. In the uncomplicated case, antispasmodics can be given to relax the sphincter of Oddi and the worm will return to the small intestine to be dealt with by antihelminthic drugs. Operation may be necessary to remove the worm or deal with complications. Worms can be extracted via the ampulla of Vater by ERCP

Clonorchiasis (asiatic cholangiohepatis) The disease is endemic in the Far East. The fluke, up to 25 mm long and 5mm wide, inhabits the bile ducts, including the intrahepatic ducts. Fibrous thickening of the duct walls occur. 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 Roux loop affixed to the abdominal parietes is performed in some centres to allow easy subsequent access to the duct system. 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 (see Chapter 52). Tumours of the bile duct Papillomatosis This rare condition is characterised by the presence of multiple mucoussecreting tumours of the biliary epithelium. These tumours are low-grade papillary carcinomas and should be treated by choledochoscopy with obliteration of the papillary lesions. The malignancy tends to develop over a period of years and may require liver resection. If both lobes are affected then liver transplantation may be required. Carcinoma of the bile duct Incidence and aetiology The incidence of bile-duct carcinoma varies markedly in different parts of the world. It is very rare in the UK, but high rates are found in parts of the world where biliary infection is common. It is associated with the presence of bile-duct stones in a quarter of patients, and is more frequent in patients with sclerosing cholangitis and ulcerative colitis. Anatomical abnormalities, particularly intrahepatic cystic change and choledochal cyst, are also associated with an increased incidence. Clinorchis sinensis is a recognised aetiological factor. Pathology Macroscopically, the tumour arises as a small nodule which rapidly encases the bile duct causing jaundice. The tumour invades locally spreading via the nerves, the lymphatics and the portal venous trunk. It may directly invade the portal vein or the surrounding liver. Microscopically, the majority of bile duct tumours are well to poorly differentiated adenocarcinomas. Clinical features The patient presents with jaundice. The diagnosis is suspected when obstruction in the biliary tree with a characteristic stricture is demonstrated. Local spread is frequent and metastases are common at the time of presentation. The diagnosis is confirmed by biopsy, either by the percutaneous transhepatic route or endoscopically, or by cytological brushings taken at endoscopy. If the patient is under 70 years of age and the tumour is localised either to one lobe of the liver or to the extrahepatic bile duct then resection is possible in 1020 per cent of patients. Such resections may involve excision of a lobe of the liver and reconstruction of the biliary tree. With the improved techniques associated with modern liver surgery the mortality is now 5 per cent. The median survival is 18 months, with 20 per cent of the patients surviving for 5years. Carcinoma of the gall bladder

This is rare in the Western world but there are areas where the incidence is high (in Patna, India, the incidence reaches as high as 9 per cent of biliary tract disease). The tumour is found in less than I per cent of gall-bladder operations. In over 90 per cent of instances gallstones are present. The patients are usually in their late 70s, with a female to male ratio of 5:1. The tumour is usually scirrhous, but squamous cell and mixed squamous adenocarcinomas are found. Spread is by direct invasion through the mucosa to the serosa and into the liver, the lymphatics and the veins. Clinical features Most present either with an extensive mass in the liver during investigations for jaundice, or at cholecystectomy at the time the histology is received, the tumour being unrecognised by the surgeon. Treatment and prognosis Those that are diagnosed at cholecystectomy and confined to the mucosa have a good prognosis. It is debated whether such patients should have a wide excision with resection of adjacent liver and lymph nodes. For those tumours that involve the serosa, the prognosis is poor and chemoradiotherapy is all that can be offered. The survival rate is less than 5 per cent at 5 years and the median survival is 12 months. Further reading Blumgart, L.H. (ed.) (1988) Surgery of the Liver and Biliary Tract, Churchill Livingstone, Edinburgh. Carter, D.C., Russell, R.C.G., Pitt, H.A. and Bismuth, H. (eds) (1996) Rob & Smiths Operative Surgery: Hepatobiliary and Pancreatic Surgery, Chapman and Hall, London. Sherlock, S. and Dooley, J. (eds) (1997) Diseases of the Liver and Biliary System, 10th edn, Blackwell Science, Oxford.