Hepatic cysts are abnormal fluid-filled spaces in the hepatic parenchyma and biliary tree They are categorized into 3 main types: fibrocystic diseases of the liver cystadenomas and cystadenocarcinomas and hydatid cysts

1. Cystic Liver Disease: Solitary,
Polycystic, hydatid

2. Question
• A 65-year-old farmer presents to the ER with
abdominal pain. He is grasping his right upper
quadrant. The patient reports that he has had the
pain for several months and it has been
worsening. He has no past medical history. Vital
signs are stable. His physical exam is notable for
hepatomegaly. An MRI is performed which
reveals the following (Figure A). If this patient's
symptoms are due to an infectious agent, what
would be the appropriate management?

4. • 1.The patient should be treated with mebendazole
• 2. The cysts should be surgically resected and the patient
treated with praziquantel
• 3. The cysts should be surgically resected after preinjection
with ethanol and the patient treated with praziquantel
• 4.The cysts should be surgically resected and the patient
should receive subsequent mebendazole
• 5.The cysts should be surgically resected after preinjection
with ethanol and the patient treated with mebendazole

5. Hepatic Cyst
• Hepatic cysts are abnormal fluid-filled spaces
in the hepatic parenchyma and biliary tree
• They are categorized into 3 main types:
– fibrocystic diseases of the liver
– cystadenomas and cystadenocarcinomas
– and hydatid cysts

6. • Fibrocystic disorders of the include
– simple hepatic cysts,
– polycystic liver disease (PCLD),
– fibrocystic disease associated with autosomal
recessive polycystic disease,
– von Meyenburg complexes,
– and Caroli’s disease (type V choledochal cyst)

7. Diagnostic approach

8. Simple Hepatic Cyst
• are thought to be congenital in origin and
• have a frequency of about 2.5% of the
population
• are smaller than 5 cm in diameter and can
number up to 3 before being considered part
of PCLD
• The cysts usually are asymptomatic and
discovered incidentally during upper
abdominal imaging

9. • They occur more often in women than in men,
• their prevalence increases with age.
• When symptomatic, they can produce
complications similar to those of PCLD,
including
– intracystic bleeding,
– infection,
– rupture, or compression of adjacent organs.

10. • Typically, initial imaging with US, CT, or MRI
provides an accurate diagnosis and
distinguishes a simple cyst from a hydatid cyst
and cystadenoma.
• Septations, papillary projections, or
calcification should raise suspicion of an
alternative diagnosis
• Asymptomatic solitary hepatic cysts should be
left alone.

12. • If intervention is required because of
symptoms, percutaneous aspiration and
sclerosis with alcohol or doxycycline will
almost always ablate the cyst, but recurrence
is frequent
• An alternative approach is laparoscopic (or
rarely, open surgical) fenestration, which is
seldom followed by recurrence but has
greater morbidity.

13. Polycystic Liver Disease
• is a rare condition in which multiple cysts
form in the hepatic parenchyma
• It usually presents in association with
autosomal dominant polycystic kidney disease
(ADPKD) but can appear as isolated PCLD
• The cysts range in diameter from a few
millimeters to 10 cm or more

14. • They contain clear, colorless, or straw-colored
fluid
• Are lined by a single layer of cuboidal or
columnar epithelium, resembling that of bile
ducts
• The cysts may be lined by squamous
epithelium
• These cysts may be complicated by the
development of squamous cell carcinoma

15. • In addition to the nature of the lining
epithelium, evidence for a biliary origin of
these cysts is suggested by the composition
of the cystic fluid
– low glucose content
– contains secretory immunoglobulin (Ig)A and
GGTP
• The cysts are thought to arise as a result of
ductal plate malformation

16. • This process gives rise to von Meyenburg
complexes, which become disconnected from
the biliary tract during development and
growth and dilate progressively to form cysts

17. Epidemiology
• PCLD is fairly common in patients with ADPKD.
– It occurs in 24% of patients
– In third decade of life to 80% in the sixth decade
of life
– the kidney disease usually dominates the clinical
course
• Cysts also may be present in the
pancreas,
spleen,
and (less often) other organs

19. • Symptomatic liver disease correlates with
advancing age,
severity of renal cysts, and
renal dysfunction
• Women tend to have larger and more
numerous cysts, and a correlation with the
number of pregnancies has been found
• The use of exogenous female sex hormones
may accelerate the rate of growth and size of
the cysts

20. • PCLD may coexist with other fibrocystic liver
diseases, such as
congenital hepatic fibrosis (in which the
patient is likely to present with portal
hypertension),
Caroli’s disease, or
von Meyenburg complexes

21. • PCLD is also associated with other conditions
such as
berry aneurysms,
Mitral valve prolapse,
diverticular disease
inguinal hernias

22. • Isolated PCLD not associated with ADPKD is
rare, representing 7% of all PCLD in autopsy
series
• It usually is asymptomatic
• Like ADPKD-associated PCLD, isolated PCLD is
associated with pregnancy and appears to be
more symptomatic in women than men

23. Etiology and Pathogenesis
• ADPKD is a common genetic disease with a
frequency of 1 : 1000 in whites
• Two genes are responsible
• The gene affected in ADPKD1 is PKD-1, which
is located on chromosome 16q13-q23 and
expresses a ubiquitous protein, polycystin-1
• The gene responsible for ADPKD2 is PKD-2,
which is located on chromosome 4 and
expresses polycystin-2.

24. • The 2 polycystins are transmembrane
glycoproteins that complex and localize in the
primary cilium, a microtubulebased structure
found on renal and biliary tubule epithelium
and thought to act as a flow sensor and
regulator of Ca2+ influx
• Although the mutation is inherited as an
autosomal dominant trait, a second somatic
mutation is thought to be necessary to
produce the monoclonally derived cysts

25. Clinical Features
• The hepatic cysts in PCLD, whether or not they
occur in association with renal cysts, rarely
cause morbidity, and many affected persons
are asymptomatic
• The livers of these patients contain only a few
cysts or cysts smaller than 2 cm in diameter
• Symptoms occur in patients with more
numerous and larger cysts (10% to 15% of
patients, usually women), generally with
markedly enlarged livers

26. Presenting complains
Abdominal discomfort or pain,
postprandial fullness,
awareness of an upper abdominal mass,
a protuberant abdomen,
inability to bend over,
and shortness of breath may be present.
• Severe pain may be experienced with
rupture or infection of a cyst,
bleeding into a cyst, or
torsion of a pedunculated cyst

27. • Jaundice is evident in approximately 5% of
patients and is caused by compression of the
major intrahepatic or extrahepatic bile ducts
• Ascites, if present, is the result of portal
hypertension, which generally is caused by
associated congenital hepatic fibrosis but
occasionally by compression of the hepatic
veins by the cysts
• Gastroesophageal variceal bleeding has rarely
been reported

28. Diagnosis
• Liver biochemical test results generally are
normal,although serum alkaline phosphatase and
GGTP levels may be increased
• The cysts contain high levels of the tumor
marker CA 19-9, and serum levels of CA 19-9
may be elevated
• A raised right hemidiaphragm may be evident on
a plain x-ray of the chest in patients with severe
PCLD
• The diagnosis of PCLD is confirmed by US, CT, or
MRI

29. Treatment
• On the rare occasions when cysts require
treatment, fenestration (unroofing) should
be performed
• Cyst fenestration originally was done at
laparotomy but is now performed
laparoscopically, thereby reducing morbidity
• A high recurrence rate is observed for cysts
treated with fenestration, although
symptomatic relief is usually durable

30. • Cysts have also been treated by percutaneous
injection of a sclerosing substance such as
alcohol or doxycycline
• most patients have too many small cysts, so
percutaneous injection should be reserved for
those with a dominant cyst or excessive risk
for surgery

31. • Patients who fail to respond to cyst
fenestration may be considered for partial
hepatic resection if sufficient relatively
uninvolved liver remains after surgery
• The morbidity of this approach is substantial,
and future liver transplantation may be more
difficult after resection

32. • Liver transplantation (sometimes combined
with renal transplantation) is associated with
excellent outcomes and long-term survival
but, because of the organ donor shortage, is
generally reserved for patients with hepatic
failure or severe symptoms that interfere with
the patient’s quality of life

33. • Several randomized clinical trials have
demonstrated a modest reduction in cyst size
and improvement in quality of life with
treatment with a long acting somatostatin
analog, but the length of follow-up has been
short

34. Echinococcosis
• Infection occurs when humans eat vegetables
contaminated by dog feces that contain
embryonated eggs
• The eggs
– hatch in the small intestine
– liberate oncospheres that penetrate the mucosa
– migrate via vessels or lymphatics to distant sites

35. • The liver is the most common destination
(70%), followed by the
lungs (20%),
kidney,
spleen,
brain, and
bone

37. • In these organs, a hydatid cyst develops by
vesiculation and produces thousands of
protoscolices
• The cyst wall contains 3 layers:
an outer adventitial layer, which is host-
derived and can calcify,
 intermediate acellular
 inner germinal layers, which are worm-
derived

38. • A protoscolex is produced asexually within
small secondary cysts that develop from the
inner layer
• Rupture of the hydatid cyst releases the viable
protoscolices, which set up daughter cysts in
secondary sites

40. • The adult Echinococcus tapeworm consists of
a scolex, which contains a rostellum with 20 to 50
hooklets and 4 suckers,
a neck, and
an immature, mature, and gravid proglottid
• Dogs acquire the infection by consuming
organs of
sheep,
cattle, or
other livestock bearing the hydatid cyst

42. Classification

44. Clinical Features
• Most patients with a hydatid cyst in the liver
have no symptoms
• As the cysts of E. granulosus grow within the
liver, they begin to cause
low-grade fever,
pain,
tender hepatomegaly (usually affecting the right
hepatic lobe), and
eosinophilia

45. • If the cysts grow large enough, they may
rupture spontaneously or after trauma into
the lungs, thereby leading to
dyspnea and
hemoptysis
• More extensive rupture into the peritoneum
or lungs may lead to a life threatening
anaphylactic reaction to the cyst contents

46. • Rupture into the biliary tract can cause
cholangitis and obstruction; marked
eosinophilia may be present
• Superinfection of the hepatic cysts can lead to
pyogenic liver abscesses in up to 20% of
patients with hepatic disease

47. • Rare complications of hydatid cysts or cyst
rupture include
pancreatitis,
portal hypertension,
Budd-Chiari syndrome, and
rupture into the pericardial sac

48. • E. multilocularis is highly invasive; infection
leads to formation of solid masses in the liver
that are easily confused with cirrhosis or
carcinoma
• Alveolar hydatid disease is the term applied to
hepatic nodules that appear on microscopy as
alveoli-like microvesicles
• Daughter cysts bud from the germinal membrane
in an uncontrolled manner, with “invasion” of the
surrounding liver parenchyma by the scolices

49. • Infection of bile ducts and vessels and necrosis
of parenchyma may result in
cholangitis,
liver abscess,
sepsis,
portal hypertension,
hepatic vein occlusion, and
biliary cirrhosis

50. Diagnosis
• A history of exposure in a patient with
hepatomegaly and an abdominal mass is
highly suggestive of hepatic echinococcosis
• the most important diagnostic tools are
imaging and serology
• Ring-like calcifications in up to one fourth of
hepatic cysts are visible on plain abdominal
films in patients infected with E. granulosus

52. • The sensitivity and specificity of both US and
CT in confirming the diagnosis are high
• Both modalities can demonstrate intracystic
septations and daughter cyst formation in
about half of the cysts
• Contrast-enhanced CT may display avascular
cysts with ring enhancement

53. • Percutaneous aspiration of the cyst had
traditionally been discouraged because of
concern about anaphylactic reactions
• Encouraging reports, however, suggest that under
carefully controlled conditions, with use of thin
needles and concomitant antihelminthic therapy,
percutaneous aspiration for diagnosis and
therapy may be safe
• The detection of protoscolices or acid-fast
hooklets in the cyst fluid confirms the diagnosis

54. • An ELISA is the best serologic assay for
diagnosis, with a sensitivity of 84% to 90%
• The Casoni skin test, used in the past, is
nonspecific and no longer recommended
• E. multilocularis infection can be diagnosed with
a combination of ELISA and CT, which often
shows scattered areas of calcified necrotic tissue
• In E. vogeli infection, CT demonstrates polycystic
lesions in the liver or peritoneal space

57. Treatment
• Accessible cysts in younger persons were
always treated surgically
• surgery is still considered the preferred
treatment in many cases
• The goal has been removal of the cestode
without disruption of cyst contents
• Care must be taken to isolate the cyst and to
inject cidal agents before the cyst is aspirated

58. • Successful approaches have included
cystectomy,
endocystectomy,
omentoplasty, and
marsupialization
• A laparoscopic approach is feasible in some cases
• In complicated cases, hepatic lobectomy or
hemihepatectomy may be necessary
• Calcified cysts need not be removed

59. • Promising data indicate that careful
percutaneous drainage is a safe and effective
alternative to surgery for the treatment of
complicated cysts
• In addition to surgery or drainage,
administration of an antihelminthic, such as
albendazole, 10 mg/kg/day for 8 weeks, is
recommended

60. • Puncture, aspiration, injection (of a scolicidal
agent), and re-aspiration (PAIR) can be
performed safely with long-term control of
echinococcal cysts
• Injection of hydatid liver cysts with
albendazole has also been described
• Therefore, nonsurgical approaches are now
available for management of hydatid cysts

61. • The decision between surgical and nonsurgical
techniques depends on the extent and type of
lesions
• Cysts that cannot be treated surgically or
percutaneously should be treated with
albendazole, or
mebendazole

62. • Large doses and prolonged treatment are
required (e.g., albendazole 10 mg/kg daily in 2
divided doses for 28 days, repeated 3 or 4
times, with 2-week breaks between courses)

63. • Surgical resection is curative in up to one third of
cases of E. multilocularis infection
• In most cases the disease is advanced when the
diagnosis is made
• In such cases, palliative drainage procedures or
long-term treatment with albendazole or other
benzimidazole carbamates may prolong survival
• Surgery appears to be the most effective
approach to the management of E. vogeli
infection

64. THE END