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
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.
.
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.