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☆
BENIGN CONDITIONS
193
can be divided (after again checking the position of the tail of the pancreas)
and are always oversewn. The upper short gastric vessels are then divided and
the spleen removed ( Fig. 3.89 ). Beware of causing injury to the greater curve of
the stomach while dividing these vessels. After ensuring haemostasis (particu-
larly in the splenic bed which may contain collaterals), two tube drains are
placed in the left upper quadrant and the abdomen closed.
Intravenous antibiotics are given until oral intake is possible (48 hours) and
penicillin (or equivalent in the case of sensitivity) must be given for at least
2 years. The increase in the platelet counts postoperatively, are unpredictable,
but always settle to the upper limit of normal after a few months. During this
period aspirin is used routinely. Long-term results are excellent with complete
resolution of the haematological consequences of the hypersplenism and often
the associated oesophageal varices. We follow these patients long term with
yearly endoscopies but further intervention is almost never necessary.

Budd–Chiari syndrome

The Budd–Chiari syndrome is a collective term for a group of conditions
that impede hepatic venous drainage by occluding major tributaries, hepatic
veins, or the IVC. The first description was from Budd in 1845 (Budd, 1845 )
and in 1899 Chiari published ten cases in German in a pathology journal
Fig. 3.89 Spleen displayed
in preparation for initial
splenectomy and splenic
artery and vein divided in
the splenic hilum.
LIVER
194
(Chiari, 1899 ). In the majority of cases, no cause or previous disposing factor
is identified. Technological advances allowed the recognition of an increasing
number of such factors towards the end of the 20
th
century (particularly those
with an anatomical base such as particularly IVC webs). Many of the other
putative causes are of interest (malignancy, haematological causes, connective
tissue disorders, veno-occlusive disease) but, unlike IVC webs, do not allow
specific treatment options (Menon et al ., 2004 ).
Patients can be treated conservatively but the outcome is uniformly poor
and, in the majority of cases, fatal. Portosystemic shunting has been used and
the preferred approach by most people is the side-to-side portocaval shunt.
The results are better than conservative management but are still disappoint-
ing, especially in the long term (Orloff et al ., 2000 ; Slakey et al . 2001 ). Other
approaches, such as attempts at removal of the venous obstruction and trans-
luminal angioplasty, are less effective than shunting and this persuaded people
to try orthotopic liver transplantation. Unfortunately even transplantation
has disappointing results which are worse than those for shunting (although
these are improving) and this technique should only be used if the patients are
experiencing progressing hepatic failure or a previously performed shunt has
ceased to work (Sakai and Wall, 1994 ; Melear et al ., 2002 ; Srinivasan et al .,
2002 ; Valla, 2009 ).

Liver abscess

Pyogenic foci in the liver appear to occur at a fairly constant rate of approxi-
mately 1 per 5000 hospital admissions (Branum et al ., 1990 ; Huang et al ., 1996 ;
Lin JC et al ., 2004 ; Cheng et al ., 2008 ) and this show no signs of changing (it
may even be increasing) which is perhaps surprising with the declining rates of
appendicitis. The incidence of different causative organisms does tend to vary,
and reflects changes in aetiology and geographic distribution with significant
differences around the world (Wong et al ., 2002 ).
Aetiology
The majority of cases of liver abscess are due to biliary tract pathology (1/3),
portal spread from the GI tract (1/5), and an amalgam of other unusual aeti-
ologies including the contiguous spread from subphrenic or intra-abdominal
collections or tumours, bacteraemia, secondary to trauma or infective cysts,
and necrotic tumours (often after chemotherapy) (Chou et al ., 1997 ; Perez
et al ., 2001 ). The cause is not found in about 10% of cases and in a number of
these the problem becomes recurrent. Some guidance concerning aetiology
can come from the nature and distribution of lesions. Spread from the portal
circulation usually produces lesions in the right lobe, singly or in low numbers.
BENIGN CONDITIONS
195
Ascending cholangitis from a biliary tract origin generally produces bilateral
numerous lesions which may be diffuse and coalesce If the source has gained
access by the arterial circulation (systemic), the lesions are almost always single
and small but can occur in either lobe (Cheng et al ., 2008 ).
Responsible organisms
Anaerobic organisms are believed (based mainly on experimental studies) to
be important in the development of liver abscesses and although aspiration
frequently does not produce a positive culture, antibiotic treatment should
always cover these organisims (Perez et al ., 2001 ).
In trying to elucidate the predominant organisms, studies are frequently
hampered by the inappropriate collection of samples. Nevertheless although
organisms do change with time, coliforms ( Escherichia coli and Klebsiella ) and
Staphylococcus aureus always predominate. Fungal superinfection has become
increasingly common in patients with a long-standing illness and particularly
prolonged periods on ICU, and must not be overlooked especially in patients
where cultures are obtained, appropriate antibiotics are used and the abscess
does not respond or only initially improves (Karthaus et al ., 1998 ; Metser et al .,
2005 ).
Treatment ( Fig. 3.90 )
Treatment of liver abscesses initially requires identification of the source if
possible, aspiration of the lesion for microbiology and culture, and treatment
with appropriate antibiotics. This appears straightforward but the diagnosis
itself can cause some difficulty. Cysts containing debris, hydatid cysts, necrotic
tumours, and non-infected haematomas (after unrecognized or occasional
trivial trauma), can all be mistaken for abscesses. Frequently the aspirations for
‘the abscess’ resolve the problem but the prognosis for some conditions, par-
ticularly necrotic hepatocellular carcinomas, can be adversely affected by this
procedure. Most of the tumours, which show necrosis and are mistaken for an
abscess, occur in patients with known disseminated malignancies who receive
chemotherapy (particularly large metastases from colorectal cancer and GISTs
treated with Glivec®). The diagnosis of these lesions relies heavily on ultra-
sound and radiology in experienced hands and tumour markers (alpha-
fetoprotein, CEA, CA 19.9) should always be assayed in doubtful lesions.
Unfortunately, even with ultrasound and CT scanning, it can be impossible to
differentiate between a simple abscess and an infected necrotic tumour.
Furthermore response to antibiotics given empirically is unhelpful as most
lesions will, of course, initially improve. With these provisos, however, if there
remains a suspicion of a necrotic HCC then we do prefer to treat with antibiotics
and re-scan. Complete resolution of abscesses can be quite rapid (weeks) and
LIVER
196
Fig. 3.90 Management of a liver abscess.
Liver abscess
Clinically well
Investigate
source and
identify organism
Source identified
and treated
Resolution with
antibiotics
No source
Aspirate and treat
with antibiotics
Refractory
Resolves
Clinically unwell
systemic signs of
sepsis
Blood cultures
aspiration and
drainage
Investigate and treat source
• Diverticula
• Appendix
• Gallstones
• Gastroenteritis
Refractory
Resolution
Surgical
(laparoscopic)
drainage
BENIGN CONDITIONS
197
non-resolving lesions should be treated as a malignancy (Huang et al ., 1996 ;
Yeh TS et al ., 1998 ).
The treatment of confirmed abscesses relies upon aspiration and antibiotics.
If the contents are relatively thin, then they can be aspirated to dryness. This
will speed up complete resolution. With larger abscesses (greater than 5cm) we
ask the radiologist to insert a drain and these can be flushed to prevent block-
age. With this treatment 90–95% of lesions will resolve (Giorgio et al ., 1995 ;
Seeto and Rockey, 1996 ) and while this is occurring the source is sought. In
patients with no history of gastroenteritis, investigation usually demonstrates
gallstones, diverticular disease, or an appendix mass. Gall bladders associated
with stones and liver abscesses should be removed as the problems frequently
recur but diverticular disease and appendicitis, especially in elderly patients,
are treated on their merits.
Recurrent or refractory abscesses
Recurrent abscesses usually occur when the initial lesion was large, abscesses
were multiple, or there is continued communication with the biliary tract
(Bayraktar et al ., 1996 ; Sugiyama and Atomi, 2002 ). It can be difficult in these
patients to know if the lesion has recurred or is new and it is important to
determine this, as the treatment is different. With recurrent lesions, which
were previously aspirated and treated with antibiotics, we would usually insert
a drain rather than trying simply to aspirate them again. The same approach
applies for refractory lesions where a drain should always be inserted and
microbiology repeated. With an external drain left until resolution is com-
plete, subsequent recurrences are rare (Rajak et al ., 1998 ). If this is unsuccess-
ful then occasionally surgery will be required (Lublin et al ., 2002 ). Laparoscopy
has replaced laparotomy as a treatment of choice and allows a full examination
of the peritoneal cavity. This is especially valuable when the source has not
been identified. At laparoscopy we would locate the lesion with ultrasound
(aided often by the percutaneous drain) and insert a 26-French tube drain.
Occasionally with very large lesions, especially those that are immediately
under the liver capsule, two tube drains can be inserted safely to allow irriga-
tion. Subsequent culture in these cases frequently shows fungal superinfection,
which is treated appropriately and it is now a number of years since we had to
resort to a formal laparotomy for an uncomplicated liver abscess (Valla,
2009 ).

Simple liver cysts

Simple cysts of the liver containing serous fluid and not communicating with
the biliary system were originally thought to be uncommon. The advent of
LIVER
198
routine ultrasound scanning changed this view and the incidental finding of
small lesions is commonplace (Gaines and Sampson, 1989 ; Carrim and
Murchison, 2003 ). Fortunately their appearance on ultrasound and MRI scan-
ning generally allows them to be dismissed and confusion only occurs in situ-
ations where CT scanning is the initial investigation. Even in these situations a
follow-up ultrasound should resolve the issue. Simple cysts are more common
in females (1.5:1) and are larger in patients over 50 years of age. The situation
is different in cysts which are very large, symptomatic, or complex, when they
are much more common in women (10:1) (Moreaux and Bloch, 1971 ; Carrim
and Murchison, 2003 ). Huge cysts, which require treatment are almost unheard
of in males (Regev et al ., 2001 ). Most cysts are asymptomatic and, apart from
occasional transient diagnostic confusion, only cause problems when they do
increase in size or become complex or infected. Increase in size is usually grad-
ual and only causes pain if it is rapid or stretches the capsule. The pain experi-
enced is visceral and often difficult to accurately describe or localize. Other
symptoms will depend on the site of the cyst. Left sided lesions may compress
the stomach if very large and cause early satiety and weight loss. In these
patients, physical examination generally reveals a very large mobile epigastric
mass, which, due to the gradual onset, patients have managed to ignore.
Treatment ( Figs. 3.91 and 3.92 )
Treatment is reserved for symptomatic cysts, which are very large and com-
plex. Small simple cysts probably never cause pain and they should not
be treated unless they are multiple (see ‘Polycystic liver disease’). Prior to
treatment it is important to exclude other causes of the cysts and particularly
parasitic infections. Generally this is straightforward. Single large cysts have an
unremarkable wall and homogenous contents of near-water density. Some of
the larger cysts, however, do have septae and occasionally the appearance can
be confusing. Serology is helpful in excluding parasitic causes such as hydatid
disease and amoebiasis. Unfortunately serology is not infallible being 90%
accurate in amoebiasis (Sharma et al ., 1988 ) and only 70% accurate in hydatid.
This can be improved by the use of immunoelectrophoresis which is more
than 90% accurate for hepatic hydatid but still only 70% for pulmonary
hydatid disease (Varela-Diaz et al ., 1983 ). If doubt remains after CT scanning
(which almost always demonstrates typical features in hydatid disease), and
serology, we have found laparoscopy and ultrasound gives exceptionally good
views and, especially if an experienced radiologist is present, allows treatment
to proceed with confidence.
Very large cysts that require treatment almost invariably present to the sur-
face of the liver and there will be a large area of the wall with no intervening
BENIGN CONDITIONS
199
liver tissue. This is seen well on preoperative CT scanning and can be con-
firmed with ultrasound intraoperatively. Our preferred initial treatment is
now always laparoscopic and we remove the entire cyst wall that contains no
liver tissue (ideally more than 50% to reduce recurrence) (Emmermann et al .,
1997 ; Farges and Bismuth, 1995 ). Bleeding is unusual and generally not prob-
lematic if the diathermy hook or harmonic scalpel is used.
Occasional blood vessels can be clipped and any bile vessel which is seen
must be treated similarly. Simple cysts are supposed to occasionally contain
bile-stained fluid but this has not been our experience. Occasionally, however,
a small radicle will be seen to produce a little bile when the capsule is incised
and these must always be controlled by clip or suture.
After the cyst wall has been excised and haemostasis completed, we do not
routinely attempt to ablate the lining. In the past, when the excised wall was
significantly less than 180 degrees of the circumference, we would mobilize
omentum and insert this into the cyst and clip it to the remnant of the wall.
This made no difference to our results and we now simply insert a large tube
drain and send the resected wall for histological examination.
In very large cysts, patients are sent home and brought back to the clinic to
have the drain removed in 10 days. The aim of this is to produce a track which
will continue to drain if necessary until the cyst collapses completely. This
technique has produced good results and only about 10% of cysts will demon-
strate residual/recurrent fluid when rescanned at 6 months. Few of these
patients, however, have recurrent symptoms (a few have adhesional pain) and
less than half of these will require further treatment.
Fig. 3.91 Incision of the
cyst after confirmation of
simple nature with intra-
operative ultrasound.
LIVER
200
If patients do get further problems, we have repeated the laparoscopic pro-
cedure successfully in about half of these and have treated the other half by
open surgery. The last group are mainly patients where the cyst has become
infected. In these, simple drainage, completing the excision of any cyst residual
wall not containing liver tissue and then ablating the lining with argon cautery
has been effective.

Hydatid disease

Echinococcus is an organism widely distributed throughout the world (Richards,
1992 ). Most problems occur in sheep farming areas as sheep are one of the
intermediate hosts. Dogs act as a definitive host and perhaps no animal is more
widespread explaining why humans are a common accidental host. Infection
usually occurs after contact with domestic pets or working dogs. The disease in
humans is caused by the larval stage of the organism and there are three varie-
ties Echinococcus granulosus , E. multilocularis , and E. oligarthus. E. granulosus is
the most common (Thompson, 2001 ). With cystic lesions treatment is largely
independent of the species although E. multilocularis (E. alveolaris) is a signifi-
cantly more aggressive organism which often presents as a mass rather than a
cyst (and in the case of spillage, becomes implanted and can be almost impos-
sible to eradicate). Under these circumstances the treatment must be modified
and generally, if technically feasible, a liver resection is required (Bresson-
Hadni et al ., 1999 ).
Fig. 3.92 The wall is sent for histological examination to confirm a simple cyst rather
than cystadenoma.
BENIGN CONDITIONS
201
Diagnosis
The latent period of infestation may be very long and the cysts will usually
remain asymptomatic until mechanical problems or complications occur
(Iscan and Duren, 1991 ; Sozuer et al ., 2002 ; Vuitton, 2004 ). Mechanical prob-
lems are similar to those with any space-occupying lesion, including gastric
compression and biliary obstruction. Complications include infection (most
commonly) and involvement of the biliary tract with occasional obstruction
due to daughter cysts rupturing into a bile duct (Iscan and Duren, 1991 ).
Presentation depends on position, size, stage of development, viability of the
cyst, and the presence of complications, particularly infection. Nevertheless,
symptoms almost always point to a hepatic pathology and scanning (ultra-
sound or CT) will reveal the abnormality. Features are often characteristic
showing well-defined lesions which do not invade the surrounding liver (Beggs,
1983 ; WHO Informal Working Group, 2003 ). They may be solitary or multi-
ple and usually contain visible membranes which may be floating in the cyst
wall (detached germinal layer of the endocyst). Daughter cysts may also be vis-
ible and confusion in non-infected cysts is consequently rare. Infected cysts
may be mistaken for simple abscesses or tumours (see above). Hydatid serol-
ogy can be helpful if positive but false negatives can occur in up to 30% of
patients (Varela-Díaz et al ., 1983 ).
Treatment ( Fig. 3.93 )
Occasionally, elderly patients will present with incidentally discovered heavily
calcified liver lesions with features suggesting previous hydatid infection. These
lesions are almost always asymptomatic and in patients above 60 years of age it
is well recognized that the number of live infestations decreases (Buttenschoen
and Buttenschoen, 2003 ). No treatment is required in these patients apart
from one or two follow-up scans at 6-monthly intervals to demonstrate the
stable nature of the lesions.
Patients who are symptomatic require treatment for these and to prevent
further complications. The decision is sometimes difficult with smaller, deeply
placed lesions which are difficult to access and would require a significant liver
resection for their removal. These lesions can initially be treated conservatively
as hydatid cysts enlarge, and as they do so tend to migrate peripherally and
come to lie under the liver capsule when treatment is considerably more
straightforward and more options are available. Most cysts will be subcapsular
at the time of the presentation and although treatment is easier, the risk of
rupture is significantly increased and surgical intervention justified. The aim
of treatment should be to sterilize the cyst, remove the parasite, and then to
remove or excise the cavity avoiding contamination (if a resection is not
LIVER
202
Fig. 3.93 Management of suspected hydatid disease.
Suspected
hydatid disease
Confirm
• Radiological
appearance
• Exposure to disease
• Serology
Multiple lesions
Single lesion
Observe if
calcified
Depends on size
and symptoms
Large or
symptomatic
Small and
asymptomatic
attempt to treat
medically
Medical
management
Resection if
technically
straightforward
Observation if
calcified
Resection not
possible due to
volume of liver
involved
Drainage
Surgery
Fit
Unfit