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Atlas of Liver Pathology:Vascular Alterations in the
Liver
Diseases Affecting the Vasculature of the
Liver
For Providers
Atlas of Liver Pathology: Chapter 3, Vascular
Alterations in the Liver
Diseases Affecting the Vasculature of the Liver
Frank A. Mitros, M.D.
Peer Review Status: Internally
Peer Reviewed
The vasculature
of the liver is frequently affected in systemic processes.
Thromboses of the major vessels may affect either outflow
(hepatic vein) or inflow (portal vein, hepatic artery). The
exact location and mechanism of thrombosis will lead to vastly
different outcomes. The most important of these diseases is
the group which results in hepatic venous outflow obstruction.
In addition, the
hepatic vessels can be affected directly by such systemic
diseases as vasculitis, or indirectly as a result of an
adverse effect of drugs or the remote effects of neoplasia.
Hepatic Venous Outflow
Obstruction
There are a
number of clinical situations in which the outflow of blood
from the liver is impeded by an obstructive process which may
be thrombotic or nonthrombotic, and affect either extrahepatic
or intrahepatic vessels or both. The two classic clinical
syndromes defining the ends of this spectrum are Budd-Chiari
syndrome and veno-occlusive disease, but many areas of overlap
or variations on this theme exist.
Budd-Chiari Syndrome
In the most
classic form of the syndrome patients present with striking
hepatomegaly, abdominal pain and ascites. There may also be
mild jaundice. This follows the development of obstruction of
either the larger branches of the hepatic vein as they leave
the liver or of the vena cava into which they empty before it
enters the right atrium.. Thus patients with severe right
sided failure, such as might be seen in tricuspid
insufficiency, are not considered to have Budd-Chiari
syndrome, although their clinical appearance may be identical
to that just described. Patients with chronic obstructive
lesions may have developed sufficient collateral circulation
so that ascites may not be present.
In a large
portion of the world, including South Africa, Japan and India,
the most common underlying lesion is a membranous obstruction
in the inferior vena cava. The nature of the fibrous web
within the cava is very poorly understood. It may represent
organization of a prior thrombus. In the United States, such
webs are not commonly seen and more obvious thrombotic events
are the usual underlying event. There are a number of reasons
for thrombosis to occur. The more commonly reported clinical
situations include hematologic diseases (particularly
polycythemia rubra vera, paroxysmal nocturnal hemoglobinurea,
and myelofibrosis), trauma, infection (ameba, hydatid
disease), and coagulopathies (protein C deficiency). Another
cause encountered with some frequency is tumor invasion of the
hepatic vein, particularly hepatocellular or renal cell
carcinoma
In about 25% of cases no cause can be identified.
Of paramount importance, severe cardiac failure must be
excluded, since the gross and microscopic appearance of severe
failure may be indistinguishable from that of Budd-Chiari
syndrome.
Grossly the
liver is enlarged and congested
. With thrombosis of large intrahepatic veins
Zahn's infarcts may develop. The distribution within the liver
may be quite irregular
; the caudate lobe is particularly likely to be
spared since it has some venous branches that empty directly
into the cava.
The changes
histologically are those seen with acute congestion, and are
almost purely those of severe right sided failure. Significant
coagulative necrosis is not regularly seen, although the
massive congestion may lead to significant loss of zone 3
hepatocytes. While one cannot make a definitive diagnosis of
Budd-Chiari syndrome from the histology alone, several
features do favor the diagnosis. The centrilobular congestion
is particularly severe and uniform in the affected areas of
liver
There may be significant loss of centrilobular
hepatocytes
Although the terminal hepatic venules are usually
patent, organizing thrombi may be seen on occasion in the
large sublobular veins
Significant extravasation of blood into the space
of Disse and the hepatocyte cords is very common
. If the underlying cause is not corrected,
fibrosis occurs and the picture of cardiac sclerosis appears.
Veno-occlusive Disease (VOD)
The other major
cause of hepatic venous outflow obstruction is veno-occlusive
disease; it differs from Budd-Chiari syndrome in that it is
the smaller intrahepatic venules within the liver that are
primarily involved usually in a non-thrombotic manner. There
is usually a defined exposure to a toxic agent or physical
injury. In some parts of the world naturally occurring toxins
(pyrrolizidine alkaloids - Jamaican bush tea) remains a
significant cause of hepatic disease, particularly in
children. Presently in the United States bone marrow
transplantation is the most frequent setting for the
development of VOD. This is related to the radiation and
chemotherapeutic agents used in these patients. Renal
transplant patients receiving immunosuppression agents are
also at risk for VOD.
The clinical
presentation resembles that of Budd-Chiari syndrome with the
sudden onset of ascites and hepatomegaly, although the
clinical background is usually quite different. While it was
originally thought to be a complication limited to the early
period after bone marrow transplantation (the first month) it
is clear that VOD can appear many months after the transplant.
The histology of
VOD is again that seen in severe right sided failure. It
differs from both Budd-Chiari syndrome and failure by the fact
that it is characteristically erratically distributed
throughout the liver
. More importantly, the terminal hepatic venules
show a characteristic histologic lesion. Not all venules are
involved, and the erratic distribution of the involved vessels
matches that of the areas of congestive type change. While the
histology of the venous lesion is characteristic, it can be
easily overlooked. Trichrome or reticulin stains are a must in
these circumstances. There is a narrowing of the lumen, with eventual
complete obliteration of the lumen. In the earliest stages the
intima appears to be strikingly edematous with red blood cells
trapped amongst wisps of loose connective tissue
. As the lesion progresses, the edema is replaced
by more dense fibrous tissue and macrophages with hemosiderin
may be present
. Eventually the lumen can become completely
obliterated
, and the individual lesion cannot be distinguished
from chronic Budd-Chiari syndrome or from cardiac sclerosis.
Extrahepatic Portal Vein
Obstruction
Obstruction of
the portal vein outside the liver does not usually cause
significant parenchymal damage, but may mimic or complicate
hepatic disease. The obstruction is likely thrombotic in
origin in the majority of cases, although the thrombus may not
be recognizable as such at the time of diagnosis. The usual
finding is an area of obliteration in the portal vein
surrounded by a large number of collateral vessels. The liver
is usually histologically normal. The portal vein may be
involved alone, or in combination with the splenic or splenic
and mesenteric veins. Patients present with clinical features
of portal hypertension. Ascites may be present early on, but
often resolves. Hematemesis from varices is the most common
problem, but portal encephalopathy or features of
hypersplenism may also occur.
Extraheptic
portal venous obstruction is seen most commonly in children,
where it was once believed to be a complication of umbilical
sepsis. This view has been questioned since a history of
omphalitis and intra-abdominal sepsis has been documented in
relatively few patients, and there is a seemingly higher than
expected incidence of a variety of congenital abnormalities.
The process can also be seen in adults, where it may
complicate sepsis or pylephlebitis. Infection remains the
overall most commonly identified precipitating factor at all
ages. Abdominal trauma or surgical injury are documented
causes of such obstruction, as is invasion of the portal
venous system in neoplastic processes. The thrombotic and
gynecologic disorders associated with Budd-Chiari syndrome
have also been seen in the setting of portal venous
obstruction, although the portal venous system is much less
frequently involved than the hepatic venous system.
Pancreatitis can give rise to splenic vein thrombosis, which
can then propagate and involve the portal vein; this may
present a particularly difficult diagnostic dilemma in
patients who are alcoholics. A very high percentage of cases
remain idiopathic; of interest is the fact that a large number
of such cases are seen in parts of the world such as India
where idiopathic portal hypertension is frequent (see below).
The main clinical problem is to clearly distinguish patients
with this condition from those whose portal hypertension is
related to parenchymal liver disease. Unfortunately, cirrhosis
of many etiologies may itself be complicated by a secondary
extrahepatic portal vein thrombosis. The relationship amongst
these diseases affecting the hepatic vessels is depicted
diagrammatically
Sinusoidal Dilatation
Most commonly
significant dilatation of the sinusoids is due to hemodynamic
failure or to venous outflow obstruction. There are a number
of situations where the sinusoids themselves appear to be
affected.
Peliosis
hepatis is characterized by blood filled spaces in the
liver which are almost always multiple
The size range is from several millimeters to
several centimeters in diameter; in the latter instance they
may cause confusion with vascular tumors (see Chapter 11).
Although peliosis may be associated with hepatomegaly and even
hepatic failure, it is more commonly a lesion found
incidentally. Two types have been described. In one, the
parenchymal variant, there are cystic spaces filled with blood
and without an endothelial lining
. In the other, the phlebectatic variant, the
prominent vascular spaces are in continuity with what appears
to be dilated sinusoids
. The types may coexist; both may result in
thrombosis. Presumably the precipitating condition weakens or
damages the sinusoidal lining in some fashion. Tuberculosis
was formerly the most common underlying disease, but it is now
seen most frequently as a complication of the use of
androgenic-anabolic steroids. It has also been seen
complicating a variety of tumors, and occasionally in
hypervitaminosis A or complicating hemodialysis for renal
failure. Peliosis-like changes can be seen in hepatic adenomas
(see Chapter 11).
Hodgkin's
disease frequently shows a peculiar unexplained sinusoidal
dilatation, and may even show overt peliosis. The reasons for
this phenomenon are not understood.
Pregnant
patients or patients on oral contraceptives may
show a peculiar sinusoidal dilatation showing selective
involvement of zone 2
.
Miscellaneous
causes of sinusoidal dilatation also include AIDS,
chenodeoxycholic acid therapy for gallstones, and the
presence of nearby space occupying lesions.
Systemic Vascular Disease
The liver is
affected in a variety of systemic diseases involving the
vessels; usually the manifestation in other organs are more
important than those in the liver.
Vasculitis
involves the liver not infrequently. Known causes include
polyarteritis nodosa, systemic lupus erythematosis, rheumatoid
arthritis, and temporal (giant cell) arteritis. The
intrahepatic vessels may show fibrinoid necrosis
, and aneurysms and hemorrhage may be found.
Polyarteritis is of particular interest because it may be
related to hepatitis B infection. The liver in such patients
typically show an active vasculitis in the setting of a liver
showing minimal histologic evidence of damage by the chronic
hepatitis
.
Atherosclerotic
cardiovascular disease may affect the intrahepatic vessels.
Cholesterol emboli may be seen in patients will severe
atherosclerosis
. Such lesions are usually of no clinical
significance. Systemic hypertension may cause hyaline
arteriosclerosis in portal triad vessels. Likewise the
vascular changes of diabetes mellitus may be identified in
vessels within the triads.
Patients with
hairy cell leukemia may have cystic spaces lined by the
leukemic cells in the portal areas or in the lobules. These
spaces are filled with red blood cells and may be mistaken for
peliosis.
Patients with
Osler-Weber-Rendu Disease (hemorrhagic hereditary
telangiectasia) may have large thin walled portal vessels that
may extend into the parenchyma and become associated with
fibrosis
. The resulting appearance has been referred to as
pseudo-cirrhosis; nodular regenerative hyperplasia may occur.
Patients with
sickle cell anemia may have a variety of liver problems. In
crisis there may be sequestration of large numbers of sickled
red blood cells in the sinuses
with subsequent mild hepatocyte necrosis. More
typical liver diseases, such as obstructive processes from
complicating gallstones, or intercurrent viral hepatitis are
more common and must be excluded before attributing the liver
dysfunction to the vascular phenomenon.
Disseminated
intravascular coagulation may manifest in the liver with
sinusoidal microthrombi which may extend into central veins on
occasion.
Idiopathic Portal Hypertension
When such causes
of portal hypertension as cirrhosis, extrahepatic portal vein
obstruction, and infections such as Schistosomiasis have been
excluded there remains an enigmatic group of patients who
present with clear-cut evidence of portal hypertension of
uncertain etiology. Most of these patients would fit under the
previously used rubric of Banti's syndrome. As our
understanding of the clinical and pathologic aspects of this
process have progressed, a number of synonyms have appeared in
the world's literature. These include idiopathic portal
hypertension (IPH), noncirrhotic portal hypertension or
fibrosis (NCPF), and hepatoportal sclerosis.
There is a
striking geographic variability in incidence. There are also
some relatively subtle differences in the way the disease
manifests itself in different parts of the world. In Japan,
where it is usually known as IPH, the patients are typically
middle aged women; 75% of patients are female, and the mean
age of presentation is 36, although patients in their 50's are
not uncommon. In these patients gastrointestinal bleeding,
splenomegaly, or anemia are equally frequent modes of
presentation. In India, where the process is usually referred
to as NCPF, the usual patient is a young man; 80% of the
patients are male, and the mean age of presentation is 30
years of age. Presentation with gastrointestinal bleeding is
the rule in the vast majority and anemia or symptomatic
splenomegaly are uncommon. Careful physical exam will show
splenomegaly in both groups, and hepatomegaly is present about
half the time. Not infrequently the liver is smaller than
normal. In the United States, the sex incidence is nearly
equal and the patients are slightly older, with
gastrointestinal bleeding being the usual presentation. Liver
function tests are normal or near normal; anemia and
leukopenia are frequent.
Parallel
similarities and differences exist with regards to the
pathology of the these two groups of patients. The liver may
look nodular from the surface, but the nodularity is usually
relatively superficial; the nodularity is more pronounced in
the cases from India. Cirrhosis by definition is not present;
the nodules are similar in appearance and pathogenesis to
those seen in nodular regenerative hyperplasia or partial
nodular transformation. In fact, many believe that the latter
term has been incorrectly used to describe patients who
actually have IPH or NCRF. There is usually significant
fibrosis along the portal vein and its branches particularly
near the hilus . Portal vein thrombosis can occur, but this is
thought to be a complication rather than a cause of IPH since
it usually occurs later in the course of well-established
disease.
Microscopically
there is no evidence of cirrhosis, although a number of
histological clues may be present. The portal vein itself will
usually have a thick sclerotic wall rich in elastic fibers
. There may be stellate or concentric fibrosis in
the portal areas in the advanced cases; the connective tissue
in these areas may have a peculiar wrinkled appearance and
stain strongly for elastic fibers
. There may be diminution or obliteration of portal
vein branches within the triads. Frequently abnormal large
dilated veins may be seen juxtaposed to the portal triads;
these have been mistaken on occasion for terminal hepatic
venules
. Inflammation and necrosis are not prominent
features.
The etiology of
this condition is not known. Exposure to such environmental
toxins such as arsenic has been suspected but not clearly
proven. Infection has also been suspected, perhaps associated
with a peculiar immunologic response to subclinical infection.
It is of interest that the incidence of NCPF is high in
countries like India, where the incidence of extrahepatic
portal vein obstruction (more closely linked to episodes of
infection) is also high.
Patients with
non-cirrhotic portal hypertension have excellent parenchymal
reserve. They tolerate the condition well, having much better
survival than patients with similar degrees of portal
hypertension caused by cirrhosis.
All
contents copyright © 1992-2003 the Author(s) and The
University of Iowa. All rights reserved.
Atlas of Liver Pathology: Chapter 3, Vascular
Alterations in the Liver
Effects of Systemic Vascular Problems on the Liver
For Providers
Atlas of Liver Pathology: Chapter 3, Vascular
Alterations in the Liver
Effects of Systemic Vascular Problems on the Liver
Frank A. Mitros, M.D.
Peer Review Status: Internally
Peer Reviewed
There is a
striking effect on the liver in situations where significant
cardiac dysfunction occurs. These effects will vary greatly
depending on how acutely the hemodynamic changes occur and
whether or not they are predominantly affecting the right or
left side of the circulation (Figure 3-1). In most instances
there will be a combination of events affecting the entire
circulation occurring over a variable period of time, perhaps
modified by pre-existing conditions in the host liver. For
purposes of clarity, classic examples of nearly
"pure" conditions will be considered separately,
although one must be cognizant that these circumstances are
much less common than combined lesions.
Acute Right Heart Failure
The cardinal
hepatic manifestations of significant right sided heart
failure are sinusoidal dilatation and congestion with
subsequent atrophy of hepatocyte cords
. These changes are clearly zonal, with the
centrilobular area (zone 3) being most severely affected; with
time and increasing severity there may also be involvement of
zone 2 and finally of zone 1. This condition has been referred
to as chronic passive congestion and is closely related to
elevated pressures in the right atrium or with other
conditions leading to an increase in systemic venous pressure.
The space of Disse may become more prominent
and may contain red blood cells in the more severe
lesions. The appearance of the sinusoids can be dramatically
altered due to the process of biopsy, particularly with needle
biopsies. Blood may drain out of the specimen collapsing the
sinusoids, but attention to detail such as atrophic cords and
the widened space of Disse provide helpful clues. There is
minimal if any necrosis, correlating with the observation that
such patients may have normal transaminases or, less commonly,
minor elevations (less than 2-fold).
Acute Left Heart Failure
Significant
failure of the left side of the circulation is characterized
by necrosis of hepatocytes in the centrilobular area
. This necrosis is coagulative rather than lytic.
It may appear to be asymmetric in zone 3, owing to the
variability of blood flow through the various acini
It is clear that the presence of centrilobular
necrosis correlates closely with the severity of hypotension,
whether sustained or episodic. Overt clinical shock need not
be present for severe necrosis to develop. When such necrosis
is present, there may be dramatic increases in serum
transaminases, sometimes leading to a mistaken clinical
impression of a viral hepatitis
; this has been referred to by some as ischemic
hepatitis. In most cases with significant necrosis there
will also be seen evidence of co-existing right sided failure
with prominent congestive changes. As necrosis progresses,
there may be replacement of hepatocytes within the cords by
red blood cells. Associated with this zone 3 necrosis the
adjoining intact hepatocytes have been known to contain PAS
positive spherical inclusions. While these resemble alpha-1-antitrypsin
deficiency inclusions they differ in their location (zone 3
rather than zone 1); they represent damaged enlarged lysosomes.
Chronic Passive Congestion
(Cardiac Sclerosis)
Active
deposition of perisinusoidal collagen has been noted with
right sided failure, and both passive collapse of the
reticulin framework as well as active deposition of stromal
connective tissue are seen in left sided failure. With
long-standing circulatory failure some degree of fibrosis will
develop. This is most pronounced in zone 3. This phenomenon of
centrilobular fibrosis with sparing of periportal hepatocytes
leads to an appearance referred to as "reverse lobulation"
The central congestion and fibrosis with pale
periportal areas (and perhaps some degree of fatty change in
the zone 2 hepatocytes) combine to give an appearance which
has commonly been referred to as "nutmeg liver"
. If the scarring is allowed to progress, the
centrilobular collagen will eventually link with the portal
connective tissue. At this stage the condition has been
referred to as cardiac cirrhosis
, but the term cardiac sclerosis is more accurate
in that overall architectural integrity is maintained (see
Chapter 9). Even at such a late stage clinical manifestations
are usually minor with regards to signs of liver disease,
there being minor elevations of serum bilirubin and even less
impressive elevations of alkaline phosphatase and
transaminases. The clinical picture is dominated by the signs
of the underlying cardiovascular disease. In fact, with modern
pharmacologic agents, valve replacement technology, and
availability of cardiac transplantation, it is quite unusual
to see significant hepatic sclerosis based on purely
cardiovascular problems.
Shock Liver
The liver can be
significantly damaged in shock from a variety of causes. With
severe hemorrhage, burns, etc. the type of centrilobular
necrosis described in left sided failure is seen. Such lesions
are uncommon if shock is transitory (less than 10 hours) but
are almost invariable if shock persists for 24 hours or more.
Similar centrilobular necrosis occurs with severe hyperpyrexia
or with heat stroke; in such circumstances the hepatocytes may
contain small vacuoles. With septic shock, there may be
striking cholestasis and even a prominent cholangitis (see
Chapter 8).
Hepatic Infarction
Because of the
dual blood supply of the liver (hepatic artery and portal
vein) true infarction of the liver has been considered to be
rare. This may not be true; there is considerable variation in
the incidence of infarction as reported in several autopsy
series. In at least one careful series, hepatic infarction, as
defined by coagulation necrosis occupying more than one
hepatic lobule, was seen in 14 of 700 autopsies. Most other
investigators encounter hepatic infarction much less
frequently. The underlying defect is usually hepatic arterial
occlusion (usually thrombotic). Portal vein occlusion, or
combined hepatic and portal vein occlusion, are occasionally
found. Even less commonly, no vascular occlusion is
identified. Recent abdominal surgery, particularly
cholecystectomy, is frequently present historically in
patients with arterial or combined arterial and venous
thrombosis. Pure venous thrombosis is commonly related to
tumor emboli. Patients without demonstrable vascular occlusion
frequently have significant cardiovascular disease and have
had significant recent hypotensive episodes. Arteritis has
been the underlying mechanism in some cases. Recently a number
of reports have documented hepatic infarction in liver
transplant recipients secondary to the arteriopathy of chronic
rejection (see Chapter 12).
Most hepatic
infarcts are discovered incidentally at the time of autopsy.
Clinical and laboratory findings are poorly defined, probably
because they are masked by the overwhelming manifestations of
the underlying disease. Sharp elevations of transaminases and
mild hyperbilirubinemia and a mild increase in the alkaline
phosphatase have been attributed to sublethal infarcts.
Grossly the
infarcts are sharply demarcated and a uniform dark red; there
is a mottled peripheral zone of congestion
. With time the central area becomes pale. The size
ranges from 1 to 15 cm in greatest diameter. Typical
coagulative necrosis affects the parenchyma, but mesenchymal
elements such as triads and central veins appear relatively
viable
True hepatic
infarction must be distinguished from the atrophic red
"infarct' of Zahn. These are areas of severe acute
passive congestion with minimal parenchymal necrosis and
atrophy (zone 3) resulting from focal thrombotic obstruction
of portal vein branches. They present as sharply delimited
areas of hyperemia grossly
Pylephlebitis
Suppurative
thrombophlebitis involving the portal vein and its
intrahepatic branches was formerly much more common. It was
most commonly seen complicating delayed treatment of
appendicitis in the pre-antibiotic era. Multiple hepatic
abscesses resulting from this seeding of the liver was
frequently lethal
. Abdominal sepsis still remains the most likely
underlying mechanism for pylephlebitis, but the spectrum of
disease has shifted. Clinically silent diverticulitis is the
most common mechanism; complicated inflammatory bowel disease
can also lead to pylephlebitis. The involved portal triad may
have its architecture obscured by the suppurative process, but
earlier lesions will show clear evidence of a fibrin
neutrophil coagulum in the intrahepatic branches of the portal
vein
For Providers
Case-Based Learning in Gastroenterology and Hepatology:
Hepatic Disorders
Hepatic Hydrothorax
Klaus Bielefeldt, M.D., Ph.D.
Piush Gupta, M.D.
Peer Review Status:
Internally Peer Reviewed
Creation Date: October 2000
Last Revision Date: February 2001
63 year-old Caucasian female with biopsy-proven cirrhosis
secondary to alpha-1-antitrypsin deficiency complained of
shortness of breath and cough productive of scant amounts of
mucoid phlegm for 1 week. She denied fevers or chills. In the
post, she had undergone a total abdominal hysterectomy and
radiation therapy for uterine cancer as well as a sigmoid
resection for radiation colitis. Follow up evaluations had not
shown signs of cancer recurrence. She did not smoke or drink
alcoholic beverages. On physical examination, she appeared in
moderate respiratory distress. The right hemithorax was dull
to percussion and the vocal fremitus was markedly diminished.
Shifting dullness was present on abdominal exam. She had 2+
pitting edema up to her shins bilaterally.
A chest X-ray
revealed a large right pleural effusion. The
combination of ascites and right-sided pleural effusion in a
patient with cirrhosis raised the possibility of a hepatic
hydrothorax. A TC99 study
demonstrated accumulation of tracer in right
hemi-thorax after injection into peritoneal cavity.
The patient was placed on salt and fluid restriction and
received diuretics, resulting in dramatic improvement of her
symptoms. Serial chest X-rays showed a progressively
decreasing size of the pleural effusion. Two weeks after
discharge the hepatic hydrothorax had completely resolved
.
Discussion:
Hepatic-hydrothorax occurs in 1-5% of cirrhotic patients.
Most cases affect the right side. These effusions are usually
due to the passage of ascitic fluid into the pleural space
through minute defects in the diaphragm. Hepatic-hydrothorax
has been reported even in the absence of clinical ascites.
Treatment is primarily medical with salt and fluid
restriction, diuretics and if necessary drainage of ascites.
Intractable cases may require a more aggressive approach.
Transjugular intrahepatic portosystemic shunts (TIPS) is a
viable option in selected patients. Chemical pleurodesis by
thoracoscopy carries a failure rate in excess of 33%.
Thoracotomy to repair the diaphragmatic defects has been tried
with very limited success. Chest tube insertion is not
indicated, as it does not address the underlying abnormality.
References:
1.
Mouroux J, Perrin C, Richelme H. Management of pleural
effusion of cirrhotic origin. Chest. Vol 109. No. 4. April
1996
2.
Johnson D, FD Gordon, Gitlin N. TIPS for patients with
refractory hydrothorax: what is the take home message? Am. J.
of Gastroenterology Vol 92. No. 11. November 1997
3.
Runyon BA, Greenblatt M, Ring MHC. Hepatic hydrothorax
is a relative contraindication to chest tube insertion. Am J
Gastroenterology 1986; 81:566-7
For Providers
Case-Based Learning in
Gastroenterology and Hepatology: Hepatic Disorders
Hepatic Abscess from Streptococcis Milleri
Klaus Bielefeldt, M.D., Ph.D.
Piush Gupta, M.D.
Peer Review Status:
Internally Peer Reviewed
Creation Date: October 2000
Last Revision Date: February 2001
A 58-year-old Caucasian male complained about rash over the
legs and lower back, arthralgias and soaking night sweats
which had started about one week before his clinic visit. Five
weeks prior to presentation, he had been admitted to an
outside hospital for evaluation of weakness, dizziness,
night-sweats and a 25-pound weight loss. A CT scan had
revealed a liver abscess, which was aspirated.
A"streptococcus species" was isolated from the
aspirate. He was treated with IV ceftriaxone for about 3 weeks
and was discharged after resolution of his symptoms.
His past history was significant for chronic alcoholic
pancreatitis with pancreatic duct strictures and stones which
had been treated with dilation and stone extraction 4 years
ago. He had reduced his alcohol consumption since and quit
completely 6 months ago. On physical examination, the patient
appeared disheveled and ill. He had poor oral hygiene with few
remaining, badly decaying teeth
. He had RUQ tenderness without rebound or
guarding. The liver span was estimated to be around 13 cm in
the midclavicular line. He had 1+ pitting edema up to his
shins. He had a skin rash that was most prominent over his
feet, hands and the sacral area
Based on his history and the physical findings, recurrent
hepatic abcesses were suspected. An ultrasound examination
demonstrated multiple hypoechoic lesions in the liver
measuring up to 4.3x3.3 cm with increased blood flow to the
periphery. On contrast-enhanced CT scan, these lesions
appeared hypodense. In addition, scattered calcifications in
the pancreatic head and body, minimal ascites and portal vein
thrombosis with cavernous transformation were noted. Two
drains were placed in the largest abscesses in the right and
left lobes under ultrasound guidance
. Only one organism, streptococcus milleri, was
identified in the aspirated material. To further delineta the
nature of the skin rash, biopsies were obtained which revealed
leucocytoclastic vasculitis.
Discussion:
The two major mechanisms for development of pyogenic liver
abscesses are local spread from contiguous infections within
the peritoneal cavity or hematogenous seeding of the liver
either through the portal vein or hepatic artery. However, in
many situation, no obvious cause can be found for the
formation of hepatic abscesses. The average patient age is
most often between 50-60 years. Biliary disease with
cholangitic spread remains the major cause of pyogenic liver
abscesses. Typically, multiple abscesses are present in
patients with suppurative cholangitis.
The organisms recovered from the liver abscesses are varied
and often reflect the origin of the infectious process. With
hematogenous spread of infection, a single organism is most
commonly isolated, including Staphyllococcus aureus or a
streptococcal species such as Streptococcus milleri.
Clinical symptoms and signs can be varied and most patients
do not have symptoms directly referable to the right upper
quadrant. Fever occurs in upto 90% of patients. Nonspecific
symptoms include chills, anorexia, weight loss, nausea and
vomiting and malaise.
Laboratory abnormalities are nonspecific with an elevated
serum alkaline phosphatase level, which is the single most
reliable abnormality for liver abscess. Ultrasonography and
computed tomography are the imaging studies of choice. The
traetment combines drainage with sufficiently long antibiotic
therapy. Percutaneous drainage has virtually replaced open
surgical drainage.
The duration of antibiotic therapy may be weeks to months
till repeat CT scans show complete resolution of the abscess
cavity. An identifiable focus of primary infection should also
be addressed when possible. In this case isolation of
Streptococcus milleri helped localize the site of infection.
The patient underwent removal of his few remaining decaying
teeth. The presence of leucocytoclastic vasculitis in this
case was an incidental though interesting finding.
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