Classification

Jaundice is due to elevated bilirubin in blood (upper limit of normal is 17 µmol/L). Jaundice may be pre-hepatic, hepatic or post-hepatic (Table 8.1).

Investigations

Surgery in jaundice

Jaundiced patients are prone to significant postoperative morbidity. Preoperative correction of as many factors as possible is mandatory prior to surgery. Infective complications are common so antibiotic prophylaxis is essential (also used prior to invasive radiology). Jaundice is also associated with poor wound healing with delayed angiogenesis and reduced collagen synthesis. Clotting is corrected by vitamin K. This takes time and levels should be checked just prior to surgery. Preoperative biliary decompression by stenting the common bile duct endoscopically can be helpful. Hepatorenal syndrome is prevented by adequate preoperative hydration, to avoid hypotension (set up an intravenous infusion (IVI) and urinary catheter 24 hours preoperatively to ensure good hydration and urine output).

Biliary atresia

Mild jaundice is not uncommon in the neonatal period but persistent or worsening jaundice after 2 weeks is abnormal and suggests biliary atresia. The cause is unknown. Without surgery, death from liver failure results within 6 months. Treatment requires hepatic portoenterostomy or liver transplantation.

Pyogenic liver abscess

Liver abscess is rare. Common organisms include Streptococcus milleri, E. coli, Enterococcus faecalis, Staphylococcus aureus and Bacteroides (see Box 8.1 for causes).

Symptoms and signs are variable and may reflect underlying disease – fever, rigors, jaundice and upper abdominal pain.

Investigations include WBC, LFTs, blood cultures, ultrasound, CT scan and white cell scan. Differential diagnosis includes tumour and amoebic abscess.

Management requires IV antibiotics. Percutaneous drainage may be used and the collection followed ultrasonically. Sometimes open surgical drainage is required. Morbidity and mortality are high if abscesses are multiple or inadequately drained.

Amoebic liver abscess

Due to infection with Entamoeba histolytica as a complication of parasitic colitis, the amoeba enters the portal circulation through an ulcer in the colonic mucosa. Abscesses due to the parasite may be single (common) or multiple. They may be extensive and are more common in the right lobe of the liver. The pus formed is thick and contains small quantities of blood – the description often used is that it resembles anchovy sauce. Extension of the abscess may lead to pleural effusion, bronchopleural fistula and lung abscess.

Hydatid disease

This is a common condition in the Mediterranean and Middle East, due to the parasite Echinococcus granulosus (tapeworm). The tapeworm develops in the dog intestine after the dog eats contaminated sheep or cattle offal. The ova are shed in the faeces which contaminate grass or vegetables and are then eaten by sheep, cattle or humans. The ova pass to the liver and develop into hydatid cysts. The cysts usually occupy the upper pole of the right lobe and are slow growing. Many become inactive and calcify.

Clinical features are a symptomless mass, abdominal pain and jaundice.

Investigations include plain X-ray, which may show calcification, ultrasound and CT.

Calcified cysts require no treatment. Drug therapy includes albendazole. Obstructive jaundice due to daughter cysts or cholangitis is managed by endoscopic sphincterotomy. Open surgery is rarely needed.

Benign tumours

Benign tumours are common and are usually haemangiomas, focal nodular hyperplasia or hepatic adenomas (occasionally associated with oral contraceptives). CT or MRI scan facilitates diagnosis. Biopsy is contra-indicated for haemangioma and no treatment is needed. Distinguishing between a benign adenoma and a well-differentiated hepatocellular carcinoma may be difficult.

Malignant tumours

Epidemiology and aetiology

Gallstones occur in 10% of the population over 50. They are more common in females but can affect anyone. Cholesterol is the principal constituent of the majority of stones, usually combined with bile pigment, especially bilirubin. Stones composed of pigment alone account for 5% of the total. Eighty per cent of stones are asymptomatic. Gallstones are caused by a combination of excess cholesterol concentration in bile, stasis or increased bilirubin secretion in bile. Bacterial infection may also play a role. Symptoms of gallstones are related to the complications they cause (Box 8.2).

Stones in the gallbladder

Stones in the bile duct (choledocholithiasis)

Common bile duct stones may cause obstructive jaundice, acute cholangitis and acute pancreatitis. The majority of stones originate from the gallbladder but some may form within the duct system de novo. Common bile duct stones are usually removed prior to cholecystectomy at ERCP and sphincterotomy. This is performed using baskets or sweeping the duct with a balloon.

Gallstone ileus

Occasionally in elderly patients a gallbladder containing stones erodes into the duodenum and stones migrate into the GI tract. A stone that has a greater diameter than the narrowest part of the small bowel (terminal ileum) may impact to cause small bowel obstruction (see p. 88). Biliary symptoms may be minimal.

Abdominal X-ray shows air in the biliary tree, small bowel obstruction and possibly a gallstone in the right iliac fossa. Laparotomy confirms the diagnosis and the stone is extracted via an enterotomy. The gallbladder should be left alone (it is usually buried in adhesions by this stage anyway).

Cholecystectomy (see Fig. 25.8)

Cholecystectomy remains the treatment of choice for patients with proven symptomatic gallstones and is described on page 368-9.

Before operation, consideration should always be given to the possibility of gallstones in the common bile duct, which are only reliably detected by MRCP, preoperative ERCP or perioperative cholangiography. Factors which indicate the possibility of gallstones in the bile duct include a history of jaundice or pancreatitis, abnormal liver function tests or a dilated bile duct greater than 10 mm on ultrasound.

Clinical features

The diagnosis may be difficult in an unconscious patient but should be remembered in a critically ill patient with developed signs of an acute abdomen. Ultrasound may demonstrate oedema in the gallbladder wall which may be dilated. Drainage may be considered percutaneously with ultrasound unless perforation has occurred, in which case laparotomy is required.

Benign

Gallbladder adenoma is uncommon but can predispose to gallbladder cancer. Adenomas less than 1 cm found on ultrasound can be left but larger adenomas should be removed by cholecystectomy.

Carcinoma of the gallbladder

The incidence of carcinoma of the gallbladder is less than 1% in all gallbladders removed in the UK. Peak incidence occurs in the 60–80-year age range and most are associated with gallstones (male to female ratio 1 : 4); 90% are adenocarcinomas, 10% are squamous carcinomas. The disease infiltrates locally into the adjacent liver and has a poor prognosis due to inoperability, with a 5-year survival rate of between 2% and 5%. In early T1-stage disease surgery is potentially curative.

Developmental anomalies

Physiology

The pancreas is an endocrine and exocrine organ. Acinar cells, which synthesise exocrine pancreatic enzymes, drain via intra-glandular ductules into the main pancreatic duct. Secretin and cholecystokinin (produced from the APUD group of cells in the duodenum and upper jejunum) control pancreatic secretion.

Secretin is released into the bloodstream when acid gastric contents enter the first part of the duodenum. This produces a secretion of watery, alkaline pancreatic juice rich in electrolytes. Cholecystokinin (CCK) is released when fatty acids and amino acids enter the duodenum, stimulating contraction of the gallbladder and bile ducts and secretion of pancreatic juice rich in enzymes. These products break down carbohydrates, fats and proteins.

The endocrine portion of the pancreas is arranged as islands (the islets of Langerhans) of endocrine tissue. These have a rich blood supply. The endocrine cells secrete hormones directly into the portal blood.

Investigation of pancreatic function is shown in Box 8.3. The imaging methods for the pancreas are shown in Table 8.3.

Table 8.3 Methods of visualisation of the pancreas

Note: percutaneous biopsy under image control can be done to determine the nature of pancreatic swellings.

MRCP, magnetic resonance cholangiopancreatography.

Pancreatitis

Pancreatitis may be classified as either acute or chronic, based on presentation, aetiology (e.g. gallstones or alcohol) or pathology (e.g. necrotising or oedematous).

Chronic pancreatitis

Permanent structural changes occur, leading to a small, fibrotic gland with either exocrine or endocrine dysfunction. Patients with chronic pancreatitis (i.e. with structural changes including calcification, strictures, stones or cysts) may have episodes that are pain-free but also acute exacerbations of the chronic condition.

Pathology

Mild pancreatitis is characterised by oedema and exudates which, in the more severe forms, can lead to necrosis which may involve surrounding pancreatic tissues. Infection is a common complication. Acute pancreatitis may range from mild and self-limiting to a rapidly fatal disorder with multi-organ failure.

Clinical features

Abdominal pain (mild or severe) localised to the epigastrium, radiating to the back between the scapulae. Nausea and vomiting are common. The patient may be acutely ill and in shock. Variable degrees of tenderness, guarding and rigidity may be present. Body wall ecchymoses around the umbilicus (Cullen’s sign) or in the flanks (Grey Turner’s sign) are due to haemorrhagic fluid tracking from the retroperitoneum. A serum amylase concentration in excess of 1000 IU per litre is highly suggestive of acute pancreatitis (although a number of other acute abdominal emergencies, particularly bowel ischaemia and perforated ulcer, can elevate the levels).

Assessment of severity

Clinical assessment at presentation is unreliable at predicting the course of the illness. Remarkably well-looking patients with serum amylase >1000 can deteriorate suddenly, and desperately sick-looking individuals can recover quickly. For this reason scoring systems have been developed which allow low- and high-risk patients to be identified. The most commonly used scoring system is the Glasgow method, summarised in Table 8.5. All patients with a serum amylase over 1000 should have their Glasgow criteria established and repeated daily to monitor recovery.

Table 8.5 The Glasgow criteria to gauge severity of pancreatitis

If more than three of the above are positive, the attack is severe.

Imaging

Plain abdominal X-ray and chest X-ray may exclude perforated viscus and demonstrate a sentinel loop of small bowel in the pancreatic region. Ultrasound and CT scan more particularly are used to identify underlying causes, for example gallstones, and to monitor the progress of the disease.

Acute pancreatitis due to gallstones requires early ERCP (within 72 hours) and removal of gallstones from the bile duct. Cholecystectomy is required once the attack has settled to prevent recurrence.

Prognosis

The overall mortality lies between 8 and 10%. Specific treatment for pancreatitis is supportive, with management of complications if and when they occur. Parenteral fluid replacement, nil by mouth and analgesia are the mainstays of treatment, often with antibiotics to prevent super-infection. Large amounts of fluids may be lost into the retroperitoneum and intensive support may be necessary. This may include ventilation and treatment of renal failure. Long-term intravenous nutrition may not be necessary as some studies suggest early enteral nutrition with a nasojejunal tube is more beneficial.

Complications include hypovolaemia, hypoxia, hypocalcaemia, hyperglycaemia and disseminated intravascular coagulation (Table 8.6). Very occasionally, dead infected areas within the pancreas may be treated by surgery (necrosectomy), which can carry a high mortality rate. Pancreatic lavage is sometimes used.

Table 8.6 Complications of acute pancreatitis

ARDS, acute respiratory distress syndrome; SIRS, systemic inflammatory response syndrome.

Pseudocysts

These arise as a result of fluid collections which mature after 4 weeks as a wall of granulation and fibrosis is formed. They usually occur in the lesser sac and often resolve spontaneously. Large pseudocysts (greater than 6 cm in diameter) more often communicate with the ductal system and require internal drainage either percutaneously, laparoscopically or open (cyst gastrostomy or cyst jejunostomy).

History

There is chronic abdominal pain in the epigastrium often punctuated by acute exacerbations of pancreatitis. Some attacks may be precipitated by a bout of heavy drinking.

There may be weight loss and anorexia. Regular analgesic consumption may lead to opiate addiction. When pancreatic lipase secretion is reduced by 90%, steatorrhoea occurs along with the development of diabetes. There is an increased risk for the development of pancreatic cancer. Less commonly, obstructive jaundice and cholangitis may occur as complications. Obstruction of the splenic vein may lead to portal hypertension.

Examination

There may be malnourishment, depression and signs of alcoholic liver disease.

Investigation

The amylase may be normal or only slightly raised. Plain X-ray, ultrasound and CT scan may show atrophy of the gland and duct dilatation with or without calcification. Anatomical abnormalities may be confirmed by ERCP, MRCP or endoscopic ultrasound.

Treatment

Cessation of alcohol and control of pain are mainstays of treatment. Pancreatic supplements may help steatorrhoea with acid suppression. Control of diabetes is important. Indications for surgery include correction of obstructed pancreatic duct, relief of obstructive jaundice or relief of intractable pain. Duodenal-preserving pancreatectomy is occasionally performed. Results are variable. Complications include pancreatic cysts and ascites.

Epidemiology

Pancreatic carcinoma is the fifth most common cause of cancer death in the Western world. Men are more commonly affected than women. The incidence increases with age (peak in the seventh decade). Putative aetiological factors include Western lifestyle, cigarette smoking, high-fat diet and working in chemical industries. Coffee and caffeine have been linked to pancreatic cancer but the connection is uncertain, as is the role of alcohol consumption.

Pathological features

In the pancreas, 95% of cancers are adenocarcinomas arising from the pancreatic ducts; 70% of tumours occur in the pancreatic head. Their appearance may mimic chronic pancreatitis, making histological diagnosis essential. Spread occurs by direct invasion of neighbouring tissues, lymphatic involvement, blood-borne metastases to the liver and beyond or by transcoelomic spread in the peritoneal cavity. Carcinomas in the head often obstruct the lower end of the common bile duct to cause obstructive jaundice (see below). In addition, direct invasion may obstruct the duodenum, resulting in gastric outflow obstruction and vomiting. Local infiltration (particularly portal venous encroachment) determines resectability.

Clinical features

Patients may present with obstructive jaundice and pruritus. There may be weight loss and epigastric pain radiating through to the back. A diagnosis of diabetes may have been made recently. Gastric outlet obstruction presents with vomiting. Carcinoma of the body or tail presents later and may be associated with non-specific symptoms of malaise, weight loss and epigastric pain. The prognosis is extremely poor. The differential diagnosis includes peptic ulcer, oesophagitis, angina and biliary colic.

Physical findings include jaundice and scratch marks secondary to pruritus. Weight loss is common. Other findings include Virchow’s gland in the left supraclavicular fossa, ascites, a palpably enlarged gallbladder (Courvoisier’s law). Differential diagnoses include drug-induced cholestasis, carcinoma of the duodenum, carcinoma of the bile duct, Mirizzi’s syndrome (cholecystitis and gallstone in Hartmann’s pouch causing compression of the common bile or hepatic duct or a stone in the lower end of the common bile duct).

Investigations

LFTs, full blood count, coagulation studies, ultrasound (excluding gallstones, confirming dilated intra- and extrahepatic biliary tree, a mass in the head of the pancreas). ERCP ± EUS may be used in addition to confirm the diagnosis with cytological brushings or biopsy, and therapeutic stenting may be done at the same time. Percutaneous transhepatic cholangiography is performed when ERCP has failed. MRC cholangiography is increasingly being used. CT scan is valuable for determining operability, demonstrating the relationship of the tumour to the superior mesenteric vessels and portal vein. Visceral angiography may show encasement of the portal vein by tumour, suggesting irresectability. Laparoscopy is used to exclude peritoneal spread before an attempt at resection. Serum tumour markers (CEA and CA19–9) are both elevated in pancreatic cancer and may support a clinical diagnosis.

Treatment

Resection of the primary lesion for cure is possible in less than 20% of patients and therefore management is most often palliative to alleviate the jaundice, control pain and treat exocrine and endocrine failure, often combined with radiation and chemotherapeutic palliation. Whipple’s operation (pancreatico-duodenectomy) should only be carried out in specialist centres. Tumours irresectable at operation are treated by palliative biliary bypass. Alleviation of obstructive jaundice is achieved by stenting at ERCP or, if unsuccessful, a percutaneous cholangiogram (PTC) can be performed. Surgical decompression may be undertaken using cholecysto-jejunostomy or hepatico-jejunostomy, often combined with a gastro-enterostomy to manage duodenal obstruction. Intractable pain from invasion of the coeliac plexus is managed by plexus nerve block and opiate analgesics. Chemotherapy, including doxorubicin (adriamycin), epirubicin and mitomycin, has demonstrated some palliative response (10–30%). Less toxic derivatives of platinum-based compounds and metalloproteinase inhibitors are being evaluated. External beam radiotherapy has been shown to prolong survival but is not curative.

Prognosis

Overall prognosis is dismal, with a median survival after diagnosis of 6 months.

Splenomegaly

The spleen must be enlarged to three times its normal size before it becomes clinically palpable. The lower margin may feel notched to palpation. Massive splenomegaly in the United Kingdom is likely to be due to chronic myeloid leukaemia, myelofibrosis or lymphoma. Splenomegaly may lead to hypersplenism (pancytopenia) due to cells being trapped and destroyed in an over-active spleen; anaemia, infection and haemorrhage result.

Causes of hypersplenism and possible indications for splenectomy are shown in Table 8.7. Other indications for splenectomy include trauma or as part of other operative procedures, for example gastrectomy, tumours, cysts or more rarely diagnostic procedures such as colonoscopy.

Ruptured spleen

The immediate management of traumatic ruptured spleen (resuscitation) is covered in Chapter 4, p. 66-68. The majority of ruptures are associated with blunt trauma, although splenic hypertrophy may make injury more likely. In the haemodynamically unstable patient laparotomy and splenectomy are indicated, but there is a trend towards conservative management in the stable patient. A CT grading system has developed (see American Association for the Surgery of Trauma guidelines) with Grade 1 = <10% area, subcapsular haematoma to Grade V = ‘Shattered spleen’. Stable patients >55y with isolated Grade 1 or 2 injury can often be managed with close observation.

Overwhelming post-splenectomy infection (OPSI)

OPSI is a serious late complication of splenectomy. Patients are prone to severe infection from encapsulated organisms such as pneumococci and meningococci and Haemophilus influenzae. Post-splenectomy sepsis may start insidiously but can rapidly develop into a fulminant infection with fever, vomiting, dehydration and collapse. Early treatment with antibiotics is essential. Prophylactic immunisation should take place before an elective splenectomy and within two weeks after an emergency splenectomy. Prophylactic antibiotics should be taken after an emergency operation, and usually are continued for life (a typical regimen would be penicillin V 250 mg b.d.). All UK hospitals have guidance available for patients who have undergone splenectomy. Any patient who develops a viral or bacterial illness should have antibiotic treatment without delay.