Clinical manifestations and diagnosis of echinococcosis

Author:
Pedro L Moro, MD, MPH
Section Editor:
Peter F Weller, MD, MACP
Deputy Editor:
Elinor L Baron, MD, DTMH

Contributor Disclosures

All topics are updated as new evidence becomes available and our peer review
process is complete.
Literature review current through: Apr 2018. | This topic last updated: Nov 21,
2017.

INTRODUCTION — Echinococcal disease is caused by infection with the metacestode

stage of the tapeworm Echinococcus, which belongs to the family Taeniidae. Four
species of Echinococcus produce infection in humans; E. granulosus and E.
multilocularis are the most common, causing cystic echinococcosis (CE) and alveolar
echinococcosis (AE), respectively. The two other species, E. vogeli and E. oligarthrus,
cause polycystic echinococcosis but have only rarely been associated with human
infection.

The clinical manifestations and diagnosis of cystic and alveolar echinococcal infection
will be reviewed here. The epidemiology, treatment, and prevention of echinococcosis
are discussed separately. (See "Epidemiology and control of
echinococcosis" and "Treatment of echinococcosis".)

CLINICAL MANIFESTATIONS — The Echinococcus species have different

geographic distributions and involve different hosts. E. granulosus and E.
multilocularis also differ in their clinical presentation. (See "Epidemiology and control of
echinococcosis".)

Echinococcus granulosus — The initial phase of primary infection is always

asymptomatic. Many infections are acquired in childhood but do not cause clinical
manifestations until adulthood. Latent periods of more than 50 years before symptoms
arise have been reported. While approximately 50 percent of detected cases occur in
asymptomatic patients, many more cases remain undiagnosed or are found incidentally
at autopsy.

The clinical presentation of E. granulosus infection depends upon the site of the cysts
and their size. Small and/or calcified cysts may remain asymptomatic indefinitely.
However, symptoms due to mass effect within organs, obstruction of blood or lymphatic
flow, or complications such as rupture or secondary bacterial infections can result.

Cysts typically increase in diameter at a rate of one to five centimeters per year.
However, cyst growth rates and time courses are highly variable [1,2]. Hydatid cysts
may be found in almost any site of the body, either from primary inoculation or via
secondary spread. The liver is affected in approximately two-thirds of patients, the
lungs in approximately 25 percent, and other organs including the brain, muscle,
kidneys, bone, heart, and pancreas in a small proportion of patients. Single-organ
involvement occurs in 85 to 90 percent of patients with E. granulosus infection, and
only one cyst is observed in more than 70 percent of cases (image 1).

Liver involvement — E. granulosus infection of the liver frequently produces no

symptoms. The right lobe is affected in 60 to 85 percent of cases. Significant symptoms
are unusual before the cyst has reached at least 10 cm in diameter. If the cysts
become large, hepatomegaly with or without associated right upper quadrant pain,
nausea, and vomiting can result (picture 1).

E. granulosus cysts can rupture into the biliary tree and produce biliary colic,
obstructive jaundice, cholangitis, or pancreatitis. (See "Endoscopic diagnosis and
management of biliary parasitosis".)

Pressure or mass effects on the bile ducts, portal and hepatic veins, or on the inferior
vena cava can result in cholestasis, portal hypertension, venous obstruction, or the
Budd-Chiari syndrome. (See "Etiology of the Budd-Chiari syndrome".)

Liver cysts can also rupture into the peritoneum, causing peritonitis, or
transdiaphragmatically into the pleural space or bronchial tree, causing pulmonary
hydatidosis or a bronchial fistula. Secondary bacterial infection of the cysts can result in
liver abscesses. (See "Pyogenic liver abscess".)

Lung involvement — The most common symptoms of pulmonary cystic

echinococcosis (CE) described in the literature include cough (53 to 62 percent), chest
pain (49 to 91 percent), dyspnea (10 to 70 percent), and hemoptysis (12 to 21 percent).
Less frequent symptoms include malaise, nausea and vomiting, and thoracic
deformations [3,4]. The majority of children and adolescents with lung lesions are
asymptomatic despite having lesions of impressive size, assumedly because of a
weaker immune response and the relatively higher elasticity of the lung parenchyma
relative to older patients [3,5].

Cysts can break or develop secondary bacterial infection. The presence of these
complications changes the clinical presentation, either by causing new symptoms or by
increasing the severity of existing symptoms. The principal complication is cyst rupture,
with spilling of cyst material containing fragments of larval tissue and protoscolices into
the bronchial tree or the pleural cavity. Bronchial tree involvement can lead to cough,
chest pain, hemoptysis, or emesis; pleural cavity involvement can cause
pneumothorax, pleural effusion, or empyema. Secondary bacterial infection of the cyst
can manifest as a pulmonary abscess with poorly defined margins [6-8].

Approximately 60 percent of pulmonary hydatid disease affects the right lung, and 50 to
60 percent of cases involve the lower lobes [9]. Multiple cysts are common.
Approximately 20 percent of patients with lung cysts also have liver cysts [10]. The
ratio of lung to liver involvement is higher in children than in adults [10].
Other organs — Involvement of organs outside of the liver or lung is unusual but can
lead to significant morbidity and mortality.

●Infection of the heart can result in mechanical rupture with widespread

dissemination or pericardial tamponade [11-13].

●Central nervous system involvement can lead to seizures or signs of raised

intracranial pressure; infection of the spinal cord can result in spinal cord
compression [14].

●Cysts in the kidney can cause hematuria or flank pain [15]. Immune complex-
mediated disease, glomerulonephritis leading to the nephrotic syndrome, and
secondary amyloidosis have also been described [16,17].

●Bone cysts are usually asymptomatic until a pathologic fracture develops; the
spine, pelvis, and long bones are most frequently affected [18].

●Ocular cysts also occur [19,20].

●Subcutaneous cyst has been described [21,22].

Cyst rupture — Fever and acute hypersensitivity reactions, including anaphylaxis,

may be the principal manifestations of cyst rupture. Hypersensitivity reactions are
related to the release of antigenic material and secondary immunologic reactions.
(See "Anaphylaxis: Emergency treatment".)

Outcome — The outcome of infection varies with the stage of the disease. One study
reported on the long-term outcome of 33 patients with asymptomatic liver hydatid cysts
[1]. The natural history of infection was variable. Approximately 15 percent of patients
had undergone surgery 10 to 12 years after the initial diagnosis. Among patients who
did not undergo surgery, 75 percent remained asymptomatic; 57 percent did not have a
change in the size of the cyst by imaging.

Calcification usually requires 5 to 10 years to develop and occurs most commonly with
hepatic cysts but rarely with pulmonary or bone cysts. Total calcification of the cyst wall
suggests that the cyst may be nonviable.

Echinococcus multilocularis — Infection due to E. multilocularis is usually

symptomatic, although the clinical manifestations are frequently nonspecific. The most
common presenting complaints include malaise, weight loss, and right upper quadrant
discomfort due to hepatomegaly. Cholestatic jaundice, cholangitis, portal hypertension,
and the Budd-Chiari syndrome can also occur. The clinical presentation may mimic that
of hepatocellular carcinoma.

Extrahepatic primary disease is very rare (1 percent of cases). Multiorgan disease was
described in 13 percent of cases in one series in which metacestodes involved the
lungs, spleen, or brain in addition to the liver [23]. Immunodeficiency, such as HIV or
transplantation, may accelerate the manifestations of alveolar echinococcosis [24].
If left untreated, more than 90 percent of patients will die within 10 years of the onset of
clinical symptoms, and virtually 100 percent will die by 15 years [25]. Since treatment
with albendazole has been introduced, the prognosis has improved considerably. Of
117 patients from France who underwent long-term follow-up, the actuarial survival rate
was 88 percent [26]. Life expectancy has improved, and patients undergoing radical
treatment have a better outcome now than 40 years ago. In Switzerland in 1970, the
life expectancy for an average 54-year-old patient with echinococcosis was estimated
to be reduced by 18 and 21 years for men and women, respectively; by 2005, life
expectancy was reduced by approximately 3.5 and 2.6 years for men and women,
respectively [26].

DIAGNOSIS — Both cystic and alveolar Echinococcus may be diagnosed with a

combination of imaging and serology [27]. Serologic assays for E.
multilocularis infection are more sensitive and specific than for E. granulosus infection.
The following discussion primarily deals with the more common E. granulosus infection,
except as indicated. Specific discussion related to the diagnosis of alveolar
echinococcosis follows below.

E. granulosus — Nonspecific leukopenia or thrombocytopenia, mild eosinophilia, and

nonspecific liver function abnormalities may be observed but are not diagnostic.
Eosinophilia is observed in fewer than 15 percent of cases and generally occurs only if
there is leakage of antigenic material.

Imaging — Hydatid cysts may be visualized and evaluated with ultrasonography,

computed tomography (CT), or magnetic resonance imaging (MRI). Ultrasonography is
employed most widely because it is easy to perform and relatively inexpensive.
Portable ultrasound machines are frequently used for screening patients in
communities in which E. granulosus infection is endemic, sometimes with confirmatory
serologic testing to maximize the diagnostic yield [28]. However, CT or MRI may be
useful for circumstances in which greater anatomic detail is needed to establish the
location and number of cysts, the presence or absence of daughter cysts, and
presence of ruptured or calcified cysts, which are important for guiding management
(table 1).

Plain radiography may demonstrate calcification within a cyst but cannot detect
uncalcified cysts so is not adequate for definitive diagnostic evaluation.

Ultrasonography — The sensitivity of ultrasonography for evaluation

of Echinococcus is 90 to 95 percent [29,30]. The most common appearance on
ultrasound is an anechoic, smooth, round cyst, which can be difficult to distinguish from
a benign cyst. In the presence of liver cyst membranes, mixed echoes can be confused
with an abscess or neoplasm. In the presence of daughter cysts, characteristic internal
septation can be seen.

Shifting the patient's position during ultrasonography may demonstrate "hydatid sand,"
which consists predominantly of hooklets and scolexes from the protoscolices. Hydatid
disease is probable in the setting of hydatid sand, inner cyst wall infoldings, and
separation of the hydatid membrane from the wall of the cyst observed on ultrasound
[31].

Ultrasound allows classification of the cyst(s) as active, transitional, or inactive based

on biologic activity; such categorizations may influence the choice of treatment (image
2). Characteristics suggestive of an inactive lesion include a collapsing, flattened
elliptical cyst (corresponds to low pressure within the cyst), detachment of the germinal
layer from the cyst wall ("water lily sign"), coarse echoes within the cyst, and cyst wall
calcification [32,33]. Cysts with a calcified rim may have an "eggshell" appearance.

Several other classification systems are based upon ultrasound appearance (image 2):

●The World Health Organization (WHO) classification characterizes cysts by type

and size (table 1) [34,35].

●The Gharbi classification divides cysts into five types [36]. Type I cysts consist of
pure fluid; type II have a fluid collection with a split wall; type III cysts contain
daughter cysts (with or without degenerated solid material); type IV have a
heterogeneous echo pattern; and type V have a calcified wall [36].

WHO categories CE1 and CE2 are active cysts. Type CE1 is unilocular, and type CE2
is multilocular with daughter cysts (figure 1). Class CE3 consists of cysts that are
thought to be degenerating (transitional group). There are two types of CE3: CE3a,
featuring the "water-lily" sign for floating membranes, and CE3b, which is
predominantly solid with daughter cysts. Establishing whether daughter cysts are
present is important for guiding treatment. In addition, nuclear magnetic resonance has
demonstrated that CE3a and CE3b have different metabolic characteristics [37].
Classes CE4 and CE5 are considered inactive. By ultrasonography, they are
echogenic with increasing degrees of calcification and are nearly always nonviable.

It is important to establish whether daughter cysts (cysts that have involuted from the
wall and reside within the larger cyst) are present and to distinguish them from brood
capsules, which are attached to the wall of the larger cyst. This distinction is important
for guiding treatment.

Lung cysts may be single or multiple, usually do not calcify, rarely lead to daughter cyst
formation, and may contain air if the cyst has ruptured.

Computed tomography — Many reports suggest that CT has higher overall sensitivity
than ultrasonography (95 to 100 percent) [29,30,38]. CT is the best mode for
determining the number, size, and anatomic location of the cysts and is better than
ultrasound for detection of extrahepatic cysts. CT may also be used for monitoring
lesions during therapy and to detect recurrences (image 1) [39].

CT may be superior to ultrasonography in assessing for complications such as infection

and intrabiliary rupture [40]. In one study, ultrasound performed better than CT in the
investigation of the cyst wall, hydatid sand, daughter cysts, and splitting of the cyst
wall, while CT was superior for detecting gas and minute calcifications within the cysts,
in attenuation measurement, and in anatomic mapping [33,41].

Magnetic resonance imaging — MRI has no major advantage over CT for evaluation
of abdominal or pulmonary hydatid cysts, except in defining changes in the intra- and
extrahepatic venous system [42]. MRI may delineate the cyst capsule better than CT
and may be better at diagnosing complications, particularly for cysts with infection or
biliary communication. However, MRI is usually not required and, in most instances, is
not cost effective [43-45].

Both CT and MRI are useful in diagnosing echinococcal infection in other sites such as
in the brain [46].

Other — Other imaging techniques such as cholangiography may be indicated to

diagnose biliary involvement, particularly in patients with cholestatic jaundice.
Endoscopic retrograde cholangiopancreatography (ERCP) or magnetic resonance
cholangiopancreatography (MRCP) is frequently performed in patients with liver cysts
prior to intervention to ascertain potential involvement of the biliary system and to guide
the treatment approach. (See "Endoscopic diagnosis and management of biliary
parasitosis".)

Serologic and antigen assays — Serology is useful for primary diagnosis and for
follow-up after treatment [23,47,48]. Antibody detection is more sensitive than antigen
detection for diagnosis of E. granulosus [23].

Laboratory serologic tools — Diagnostic serologic techniques include:

●Complement fixation

●Indirect hemagglutination (IHA)

●Indirect immunofluorescence

●Latex agglutination

●Double diffusion immunoelectrophoresis

●Counter-current immunoelectrophoresis (CIEP)

●Radioimmunoassay (RIA)

●Enzyme-linked immunosorbent assay (ELISA)

●Enzyme-linked immunoelectrodiffusion assay (ELIEDA)

●Time-resolved fluoroimmunoassay (TR-FLA)

●Immunoblot
The sensitivity and specificity of a number of the serologic tests have been compared
(table 2). ELISA appears to be the most sensitive and specific of the available assays
[49-54]:

●One study of 79 patients with surgically confirmed pulmonary hydatidosis

demonstrated that immunoglobulin (Ig)G ELISA was the most sensitive (84
percent), followed by IgM ELISA (62 percent), passive hemagglutination (61
percent), latex agglutination (58 percent), immunoelectrophoresis (51 percent),
and specific IgE ELISA (44 percent). The specificity of all tests was 98 to 100
percent. Specific IgG ELISA had the highest negative predictive value (93 percent)
[49].

●One study compared eight serologic tests among 131 patients with E.
granulosus infection [52]. IgG ELISA was the most sensitive (94 percent) and
specific (99 percent) for the majority of cyst locations.

●One report compared six different serologic tests for the diagnosis of cystic
hydatid disease among 243 patients with surgically confirmed infection [53]. The
two ELISA tests gave identical results, with a sensitivity of 89 percent for liver
cysts and 78 percent for lung cysts. In the 39 patients with false-negative results,
the use of immunoblotting only increased the yield by 8 percent; ELIEDA did not
identify any additional cases.

The methods most frequently employed for initial screening tests (using crude antigens
such as hydatid fluid or protoscolex extracts) are ELISA and IHA. Confirmatory tests
using specific antigens can then be performed, such as immunoelectrophoresis and
immunoblotting [51]. Additional tests using recombinant or purified species-specific
antigens may also be useful diagnosis [55].

Simple, heat-stable, inexpensive tests, such as hydatid antigen dot immunoassays, are
often used for field testing and population screening [56]. The dot-ELISA has a
reported sensitivity of 88 to 96 percent and a specificity of 90 to 98 percent [57-59].

Two major E. granulosus antigens utilized in serologic testing include antigen 5 and
antigen B:

●Antigen 5 is a major parasite antigen found on the inner aspect of the germinal
layer, brood capsule, and protoscolices. Only a few studies have assessed the
value of tests based on recombinant antigen 5, which has relatively low specificity,
although it is used quite extensively for diagnosis in clinical practice [51].

●Antigen B is a highly immunogenic polymeric lipoprotein. Studies have showed

that antigen B shows a high degree of genetic variability [60]. It offers greater
specificity than detection of antigen 5; however, neither antigen is specific for E.
granulosus per se despite a very high specificity for echinococcal infection [61,62].

The sensitivity of these antigens in ELISA assays is 60 to 90 percent and the specificity
is usually approximately 90 percent [63]. The sensitivity in immunoblot and gel diffusion
assays is approximately 90 percent with a specificity of 97 to 100 percent [62,64]. One
study demonstrated that the immunoblot with the antigen B–rich fraction was positive in
92 percent of patients with E. granulosus but was also positive in 79 percent of patients
with E. multilocularis [65]. No cross-reactivity was observed with sera from patients with
other parasitic diseases, malignancies, or healthy controls.

A variety of issues influence the rates of false-positive and false-negative serologic

results. First, there is a lack of standardization among different laboratories; for
example, one study showed that ELISA using antigen B had a sensitivity of 63 percent,
whereas immunoblotting using the same antigen had a sensitivity of 80 percent [66].
Second, methods of antigen isolation and purification can influence the results. Third,
serological assays with high sensitivity and specificity for diagnosis of cystic
echinococcosis in clinical settings can be less useful in epidemiological studies; these
assays may detect only half of liver cysts in field surveys and as few as 20 percent of
lung cysts [67,68].

The utility of serology can be improved by using a combination of tests or sequential

testing [69]. Tests employing a number of recombinant antigens are being evaluated to
improve the sensitivity and specificity of the commercially available serologic tests. A
highly sensitive assay, usually an ELISA or indirect hemagglutination test, is commonly
used as an initial screen, followed by a highly specific immunoblot or gel diffusion
assay for confirmation. Testing for specific antibodies, such as specific IgG1 or IgG4
rather than total IgG, may improve specificity [66,70-72].

Clinical factors — A negative serologic test generally does not rule out
echinococcosis. There is no consistent correlation between serologic results and the
number or size of cysts [49]. In general, liver cysts elicit an antibody response more
frequently than lung cysts. Overall, approximately 85 to 95 percent of liver cysts and 65
percent of lung cysts are associated with positive serology, although this varies with the
specific serologic test used and cyst activity [50]. Brain, eye, and splenic cysts often do
not produce detectable antibodies, whereas bone cysts frequently are associated with
positive serology. Serology is less likely to be positive with cysts at any site if the cysts
are intact, calcified, or nonviable.

The likelihood of false-negative results is variable depending on the site of the lesion
and the integrity and viability of the cyst. Antigen-antibody complexes that "mop up" all
antibodies may lead to false-negative reactions. The sensitivity of serologic assays is
often assessed in human patients whose cysts are not intact, leading to high rates of
positive serology [73]. The sensitivity of the same assays are much lower in human
cases detected as part of epidemiologic studies (eg, ultrasound surveys) [2,67]. False-
positive reactions are more likely in the presence of other helminth infections (such
as Taenia saginata, Taenia solium, and particularly neurocysticercosis), cancer, and
immune disorders.

Antigen assays — A variety of purified or recombinant diagnostic antigens have been

evaluated. Demonstration of antigens in cystic fluid or serum can also be used for
diagnosis of primary infection or relapse [69,74,75]. However, up to 50 percent of
patients with echinococcal cysts do not have circulating antigens. Latex agglutination or
a dot-ELISA to detect echinococcal antigens from cyst fluid have excellent sensitivity
and specificity [76-78]. Antigen assays and tests for circulating immune complexes are
not widely available but may become useful secondary tests in the future [79].

E. multilocularis — Nonspecific leukopenia or thrombocytopenia, mild eosinophilia,

and nonspecific liver function abnormalities may be detected but are not diagnostic.
Hypergammaglobulinemia and elevated serum IgE levels are present in more than 50
percent of cases.

Imaging — The diagnosis of E. multilocularis is generally made by imaging techniques

in conjunction with serology. On ultrasound or CT, the lesions usually have an irregular
contour with no well-defined wall, central necrosis, and irregular intralesional and wall
calcifications. They may be difficult to distinguish from a tumor, but the patient's overall
condition is usually better than would be expected for a malignancy.

Obstruction of the inferior vena cava or of the portal venous system may be evident,
which may be more easily appreciated on MRI. Lung, brain, and bone lesions may also
be detected.

Serology — Serologic tests are more reliable for diagnosis of E. multilocularis infection
than for E. granulosus infection; sensitivity and specificity rates are 95 to 100 percent
[80]. A specific E. multilocularis antigen such as the affinity purified Em2 antigen from
AE metacestodes is often used; the Em2-ELISA can discriminate between E.
granulosus and E. multilocularis in 95 percent of cases. Serology usually remains
positive indefinitely; following complete surgical resection, serology may normalize
within a few years [81]. The Em2-ELISA frequently becomes negative within four years
of surgery and becomes positive again in the setting of a recurrence [80,82,83]. An
Em2plus-ELISA assay uses additional species-specific antigens; it has sensitivity and
specificity of 97 and 99 percent, respectively, and is also useful for monitoring
recurrence following surgical resection [84-86]. These tests may not be readily
available and may be found only in specialized centers.

ELISA and immunoblot studies using Em 18, an 18-kD protoscolex antigen, are
sensitive and highly species specific [65,87-91]. The antigen is also useful for
differentiating between active and inactive infection and is useful for follow-up of
patients on treatment [86,87,92,93].

Clinical recurrence is frequently associated with rising serologic titers. IgG1 and IgG4
antibodies are the most sensitive isotypes for monitoring success of therapy [87].

Cyst aspiration or biopsy — In the absence of a positive serologic test, percutaneous

aspiration or biopsy may be required to confirm the diagnosis by demonstrating the
presence of protoscolices, hooklets, or hydatid membranes (picture 2). Percutaneous
aspiration of liver cyst contents is associated with very low rates of complications, but
this method of diagnosis is generally reserved for situations when other diagnostic
methods are inconclusive because of the potential for anaphylaxis and secondary
spread of the infection [94-97].
Active cysts have clear, watery fluid containing scolices and elevated pressure; inactive
cysts have cloudy fluid without detectable scolices and do not have elevated pressure
[32]. In the setting of lung cysts, protoscolices or degenerated hooklets may be
demonstrable in sputum or bronchial washings. A variety of staining methods to detect
parasitic material can be used. Stains for visualization of hydatid elements include
Ryan trichrome blue stain and modified Baxby stain. Ziehl-Neelsen stain is also useful;
under green excitation light (546 nm), the hydatid elements have a fluorescent bright
red appearance [98].

If aspiration is required, it should be performed under ultrasound or CT guidance;

complications can be minimized by concurrent administration
of albendazole and praziquantel [99]. (See "Treatment of echinococcosis".)

Polymerase chain reaction — Polymerase chain reaction techniques are limited to

research settings but may play a diagnostic role in the future [100]. DNA probes using
Southern hybridization tests are also being developed [84].

DIFFERENTIAL DIAGNOSIS — In general, any mass occupying lesion may clinically

resemble an echinococcal cyst. The differential diagnosis of cystic echinococcosis
includes [101]:

●Simple benign cyst – Patients with symptomatic liver cyst may present with
abdominal discomfort, pain, or nausea. Liver cysts are distinguished
from Echinococcus by ultrasonography. (See "Diagnosis and management of
cystic lesions of the liver".)

●Hemangioma – Hemangioma is usually an incidental finding identified in the

setting of radiographic imaging or laparotomy; the most common symptoms are
abdominal pain and right upper quadrant fullness. The diagnosis of hemangioma
is generally established radiographically. (See "Hepatic hemangioma".)

●Hepatocellular carcinoma – Patients with hepatocellular carcinoma are usually

asymptomatic in the early stages; they are distinguished from patients with
cystic Echinococcusbased on clinical history and imaging. (See "Clinical features
and diagnosis of primary hepatocellular carcinoma".)

●Abscess – A liver or lung abscess may resemble an Echinococcus cyst clinically

and radiographically. Liver abscess is evaluated by aspiration; lung abscess may
be evaluated via bronchoscopy or aspiration. In the setting of suspicion for
echinococcosis, percutaneous aspiration or biopsy should be reserved for
situations when other diagnostic methods are inconclusive because of the
potential for anaphylaxis and secondary spread of the infection. (See "Pyogenic
liver abscess" and "Lung abscess".)

●Tuberculosis – A cavitary tuberculosis lesion may resemble

an Echinococcus cyst on radiographic imaging. The diagnosis of tuberculosis is
established based on the presence of acid-fast bacilli on smear and culture.
(See "Clinical manifestations and complications of pulmonary tuberculosis".)
The differential diagnosis of alveolar Echinococcus includes:

●Cirrhosis – Patients with cirrhosis may have anorexia, weight loss, weakness,
and fatigue; patients with alveolar Echinococcus may have malaise, weight loss,
and right upper quadrant discomfort. The two are distinguished based on
radiographic imaging and laboratory data. (See "Cirrhosis in adults: Etiologies,
clinical manifestations, and diagnosis".)

●Malignancy (hepatocellular carcinoma or liver metastases) – Patients with liver

tumors are generally distinguished from those with alveolar Echinococcus based
on radiographic imaging. (See "Solid liver lesions: Differential diagnosis and
evaluation".)

SUMMARY

●Echinococcus granulosus infection is initially asymptomatic and may remain so

for many years. Subsequent clinical features and complications depend upon the
site and size of the cyst(s). The liver and lungs are affected in approximately 67
and 25 percent of cases, respectively. Most patients have single-organ
involvement, and a single cyst is present in more than 70 percent of cases. The
long-term outcome is variable and many patients remain asymptomatic.
(See 'Echinococcus granulosus' above.)

●Echinococcus multilocularis infection is more likely to be symptomatic than E.

granulosus infection. The most common clinical manifestations include right upper
quadrant discomfort, malaise, and weight loss, and the picture may mimic that
associated with hepatocellular carcinoma. In the absence of treatment, more than
90 percent of patients with alveolar echinococcosis die within 10 years of the
onset of clinical symptoms. (See 'Echinococcus multilocularis' above.)

●The diagnosis of echinococcosis is typically established by ultrasound imaging

(table 1 and image 2) in combination with serologic testing (usually enzyme-linked
immunosorbent assay). Hydatid disease is probable in the setting of ultrasound
demonstrating infoldings of the inner cyst wall, separation of the hydatid
membrane from the wall of the cyst, or hydatid sand. E. multilocularis lesions may
have an irregular contour and may be difficult to differentiate from tumor.
(See 'Ultrasonography' above.)

●Diagnostic judgment must take into consideration the limitations of serologic

testing. The likelihood of a positive serology depends on cyst location and viability.
Patients with liver cysts are more likely to be seropositive than patients with lung
cysts. Serologic assays are less likely to be positive in the setting of calcified or
nonviable cysts. In addition, the sensitivity and specificity of serology is greater
for E. multilocularis than for E. granulosus. (See 'Serologic and antigen
assays' above.)

●Percutaneous aspiration or biopsy should be reserved for situations when other

diagnostic methods are inconclusive because of the potential for anaphylaxis and
 secondary spread of the infection. If aspiration is required, it should be performed
under ultrasound or CT guidance. Complications can be minimized by concurrent
administration of albendazole and praziquantel. (See 'Cyst aspiration or
biopsy' above.)

ACKNOWLEDGMENT — The editorial staff at UpToDate would like to acknowledge

Dr. Karin Leder and Dr. Peter Weller, who contributed to an earlier version of this topic
review.