Chronic liver conditions

There are several chronic liver conditions which, although rare, are important to recognise because they require a specific plan for investigation and treatment, and may present mimicking a more common clinical condition.

Budd—Chiari syndrome

This is a condition principally affecting young females in which the venous drainage of the liver is occluded by hepatic venous thrombosis or obstruction from a venous web. As a result of venous outflow obstruction the liver becomes acutely congested with the development of impaired liver function and subsequently portal hypertension, ascites and oesophageal varices. In an acute thrombosis the patient may rapidly progress to fulminant liver failure, but in the majority of cases abdominal discomfort and ascites are the main pre­senting features. If chronic the liver progresses to established cirrhosis. The cause of the venous thrombosis needs to be established and an underlying myeloproliferative disorder or procoagulant state is commonly found, such as antithrombin 3, protein C or protein S deficiency. The diagnosis is com­monly suspected in a patient presenting with ascites in whom CT scan shows a large congested liver (early stage, Fig. 52.14) or a small cirrhotic liver in which there is gross enlargement of segment I (the caudate lobe). This feature is due to preservation and hypertrophy of the segment with direct venous drainage to the IVC in the face of atrophy of the rest of the liver due to venous obstruction (Fig. 52.15). IVC compression or occlusion from the segment I hyper­trophy is also commonly a feature, as is thrombosis of the portal vein. Confirmation of the suspected diagnosis is by hepatic venography via a transjugular approach which demonstrates occlusion of the hepatic veins and may allow a transjugular biopsy. Treatment of BCS must be tailored to the individual patient and, in particular, the stage of disease at presentation. Patients presenting in fulminant liver failure should be considered for liver transplantation, as should those with established cirrhosis and the complications of portal hypertension. Those in whom cirrhosis is not established may be considered for portosystemic shunting by TIPPS, portocaval shunt or mesoatrial shunting. IVC com­pression may be relieved by the insertion of a retrohepatic expandable metallic stent. If the BCS is treated satisfactorily the prognosis of this patient group is largely dependent on the underlying aetiology and whether this is amenable to treatment. Patients are usually left on life-long anti-coagulation with warfarin.

Primary sclerosing cholangitis (PSC)

This condition often presents in young adults with mild non­specific symptoms, and biliary disease is suggested by the finding of abnormal liver function tests. Rarely the first pre­sentation is with jaundice due to biliary obstruction. The dis­ease process results in progressive fibrous stricturing and obliteration of both the intrahepatic and extra hepatic bile ducts. Although the aetiology is unknown, a genetic predisposition is likely owing to its association with chronic ulcerative colitis (UC). In patients with PSC and UC the condition usually progresses even if the diseased colon is removed. The diagnosis is principally based on the findings at cholangiography in which irregular, narrowed bile ducts are demonstrated in both the intrahepatic and extrahepatic biliary tree (Fig. 52.16). If the radiological appearances are equivocal a liver biopsy is required to demonstrate the fibrous obliteration of the biliary tracts. There is no specific treatment which can reverse the ductal changes, and the patients usually slowly progress to progressive cholestasis and death from liver failure. There is a strong prediposition to cholangiocarcinoma and this should be considered in any patient with PSC in whom a new or dominant stricture is demonstrated on cholangiography. Diagnosis of cancers in PSC is greatly facilitated by biliary brush cytology, as imaging rarely shows evidence of a mass lesion even in patients with advanced cancers. Patients with good liver function, no dominant strictures and negative biliary cytology may simply be monitored for disease progression. The only useful treat­ment modality is liver transplantation, which is associated with excellent results if carried out before bile duct cancer has developed. Temporary relief of obstructive jaundice due to a dominant bile duct stricture can be achieved by biliary stenting, although there is considerable risk of cholangitis from the introduction of bacteria to the biliary tract.

Primary biliary cirrhosis (PBC)

As with PSC, the presentation of patients with PBC is often hidden with general malaise and lethargy prior to the development of clinical jaundice or the finding of abnormal liver function tests. The condition is largely confined to females. Diagnosis is suggested by the finding of circulating anti-smooth muscle antibodies and, if necessary, is confirmed by liver biopsy. The condition is slowly progressive with deterioration in liver function resulting in lethargy and malaise. It may be complicated by the development of portal hypertension and the secondary complications of ascites and variceal bleeding. The mainstay of treatment is liver trans­plantation, which should be considered when the patient’s general condition starts to deteriorate with inability to lead a normal lifestyle.

.Caroli’s disease

This is congenital dilatation of the intrahepatic biliary tree which is often complicated by the presence of intrahepatic stone formation. Presentation may be with abdominal pain or sepsis. Imaging is usually diagnostic with the finding on ultrasound or CT of intrahepatic biliary lakes containing stones. Biliary stasis and stone formation combine to predipose to biliary sepsis which may be life threatening. Another well-recognised complication is the development of carcinoma. No specific treatment is available. Acute infective episodes are treated with antibiotics. Obstructed and septic bile ducts may be drained radiologically or surgically with some improvement. Malignant change within the ductal system results in cholangiocarcinoma which may be amenable to resection. Segmental involvement of the liver by Caroli’s disease may be treated by resection of the affected part, although the ductal dilatation is usually diffuse. Transplanta­tion is a radical but definitive treatment for a patient whose liver function is generally well preserved.

Simple cystic disease

Liver cysts are a common coincidental finding in patients undergoing abdominal ultrasound. Radiological findings to suggest that a cyst is simple are that it is regular, thin walled, unilocular, with no surrounding tissue response and no variation in density within the cyst cavity. If these criteria are confirmed and the cyst is asymptomatic no further tests or treatment are required. Large cysts may be associated with symptoms of abdominal discomfort, possibly related to stretching of the overlying liver capsule. Aspiration of the cyst contents under radiological guidance provides a sample for culture, microscopy and cytology, and allows the symptomatic response to cyst drainage to be assessed. Aspiration alone is usually associated with cyst and symptom recurrence, in which case more definitive treatment is required. Open or more recently laparoscopic deroofing is the treatment of choice for large symptomatic cysts and is associated with good long-term symptomatic relief.

Polycystic liver disease

This is a congenital abnormality associated with cyst forma­tion within the liver and often other abdominal organs, principally the pancreas and kidney. Those associated with renal cysts may have autosomal dominant inheritance. The cysts are often asymptomatic and incidental findings on ultrasound. They usually have no effect on organ function and require no specific treatment. Occasionally, multiple liver cysts give rise to liver discomfort. This often responds to treatment with simple analgesics. Severe pain often indicates haemorrhage into a cyst which may be confirmed by ultrasound or CT scan. Cyst discomfort which is not adequately controlled by oral analgesics may be treated by open or laparoscopic fenestration of the liver cysts, although the results are less favourable than with simple cysts.