Epidemiology
Ep dem olog
SEC T ION FI VE: A DDI T ION A L DISE ASES A ND DISORDERS IN IMMIGR A N TS
CHAPTER 28
Echinococcosis
Pedro L. Moro and Peter M. Schantz
Echinococcosis at a Glance
• Worldwide distribution in association with domestic
livestock populations.
• In endemic areas, prevalence can be as high as 4–
6% for cystic echinococcosis and 4% for alveolar
echinococcosis.
• Tumor-like or cystlike lesions develop most
commonly in the liver followed by the lung. Other
organs affected less frequently.
• Screening for echinococcosis in immigrants
coming from endemic areas is not cost effective.
• Most common treatment is surgery but chemo-
therapy with benzimidazoles is indicated if surgery
is contraindicated, if cysts are small, and as
adjunct to surgery to prevent secondary hydatido-
sis due to accidental spillage of protoscoleces in
peritoneal cavity.
Etiology
Hydatid disease (echinococcosis) is the infection of
humans by the larval stages of taeniid cestodes of
the genus Echinococcus. Four species of Echinococcus
are currently recognized, of which three cause dis-
tinct forms of disease: E. granulosus (cystic hydatid
disease), E. multilocularis (alveolar hydatid disease),
and E. vogeli (polycystic hydatid disease). The fourth
species, E. oligarthrus, has only rarely (fewer than five
cases) been identified as a cause of human disease.
Diverse subpopulations of E. granulosus, distin-
guished by morphologic and biologic characteristics,
have long been recognized; the taxonomic signifi-
cance of these differences remains unresolved and
controversial. However, recent demonstrations of
log
consistent genetic differences has prompted calls for
splitting this species.1 As a cause of morbidity in
humans, Echinococcus species rank high among the
helminths.
Life cycle
The life cycles of Echinococcus species involve carni-
vores as final hosts and herbivores or omnivores as
intermediate hosts (Fig. 28.1). In their adult stage,
these cestodes are small, ranging about 2–12 mm in
length, with three to six segments. They typically
localize in the lower duodenum and jejunum of
the final host. Embryophores containing infective
embryos are expelled in large numbers in the feces
of the final carnivorous host. After ingestion by
the intermediate host, the embryo is released into the
small intestine, which it penetrates, and enters the
portal circulation. The site of localization and devel-
opment of the embryo to the larval or hydatid stage
differs with species of Echinococcus and may be influ-
enced as well by species of the intermediate host.
Humans are an incidental intermediate host, since
further development of these cestodes depends on
ingestion of their larvae (hydatids) by a carnivore.
The microscopic structure of a hydatid cyst is shown
in Figure 28.2.
Epidemiology
Distribution and transmission patterns
Cystic hydatid disease (CHD) is caused by the larval
stage of E. granulosus. Molecular studies using mito-
chondrial DNA sequences have identified nine
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4 6 5
1
2

Adult in Scolex Protoscolex

4 small attached from cyst
4 intestine to intestine
Definitive host
4
(dogs and other canidae)
4
Ingestion
3 of cysts
2 Embryonated
(in organs)
I egg in feces
Ingestion
of eggs
(in feces)
4
Oncosphere hatches: Hydatid cyst in Intermediate host
penetrates intestinal liver, lungs, etc. (sheep, goats, swine, etc.)
wall

I Infective stage 3 D 4
D Diagnostic stage

Fig. 28.1 Life cycle of Echinococcus granulosus. The adult Echinococcus granulosus (3–6 mm long) (1) resides in the
small bowel of the definitive hosts, dogs or other canids. Gravid proglottids release eggs (2) that are passed in the feces.
After ingestion by a suitable intermediate host (under natural conditions: sheep, goat, swine, cattle, horses, camel),
the egg hatches in the small bowel and releases an oncosphere (3) that penetrates the intestinal wall and migrates
through the circulatory system into various organs, especially the liver and lungs. In these organs, the oncosphere
develops into a cyst (4) that enlarges gradually, producing protoscoleces and daughter cysts that fill the cyst interior. The
definitive host becomes infected by ingesting the cyst-containing organs of the infected intermediate host. After ingestion,
the protoscoleces (5) evaginate, attach to the intestinal mucosa (6), and develop into adult stages (1) in 32–80 days. The
same life cycle occurs with E. multilocularis (1.2–3.7 mm), with the following differences: the definitive hosts are foxes, and
to a lesser extent dogs, cats, coyotes and wolves; the intermediate hosts are small rodents; and larval growth (in the liver)
remains indefinitely in the proliferative stage, resulting in invasion of the surrounding tissues. With E. vogeli (up to 5.6 mm
long), the definitive hosts are bush dogs and dogs; the intermediate hosts are rodents; and the larval stage (in the liver,
lungs and other organs) develops both externally and internally, resulting in multiple vesicles. E. oligarthrus (up to 2.9 mm
long) has a life cycle that involves wild felids as definitive hosts and rodents as intermediate hosts. Humans become
infected by ingesting eggs (2), with resulting release of oncospheres (3) in the intestine and the development of cysts (4) in
various organs. (Adapted from Centers for Disease Control and Prevention DPDx.)

distinct genetic types (G1–9) within E. granulosus.2,3
These include two sheep strains (G1, G2), two bovid
strains (G3, G5), a horse strain (G4), the camelid
strain (G6), a pig strain (G7), and the cervid strain
(G8). A ninth genotype (G9) has been described in
swine in Poland.2 The sheep strain (G1) is the most
cosmopolitan form that is most commonly associ-
ated with human infections. The other strains appear
to be genetically distinct, suggesting that the taxon
E. granulosus is paraphyletic and may require taxo-
nomic revision.2,3 The ‘cervid,’ or northern sylvatic
genotype (G8), is maintained in cycles involving
wolves and dogs and moose and reindeer in north-
ern North America and Eurasia. Human infection
with this strain is characterized by predominantly
pulmonary localization, slower and more benign
growth, and less frequent occurrence of clinical com-
plications than reported for other forms.2 The pres-
ence of distinct strains of E. granulosus has important
implications for public health. The shortened matu-
ration time of the adult form of the parasite in the
intestine of dogs suggests that, where echinococcidal
drugs are used for controls, the period for adminis-
tering antiparasite drugs to dogs will have to be

412

Fig. 28.2 Histological section showing fibrous wall of the
host (Fw) and parasite’s laminated membrane (Lm),
germinal membrane (Gm), and scoleces (Sc).
Fig. 28.3 Boy with abdominal distention due to cystic
echinococcosis of the liver as shown by ultrasound
imaging.
shortened in those areas where the G2, G5, and G6
strains occur.4
E. granulosus is prevalent in broad regions of
Eurasia, in several South American countries, and in
Africa (Fig. 28.3). Humans become infected through
association with dogs that have been fed viscera
from slaughtered animals or have had access to car-
casses or discarded offal of domestic ungulates in
which the larvae are present.
Most cases of cystic echinococcosis reported
in North America continue to be diagnosed in
immigrants who acquired their infections in their
countries of origin;5 historically, this was mainly
Icelanders, Italians, and Greeks, but in more recent
years increasing numbers of cases are diagnosed in
persons of Middle Eastern and Asian origin [Schantz,
unpublished].
CH A P T ER 28 Echinococcosis
Epidemiology
Hospital record reviews indicate that local trans-
mission continues to occur in Native American
communities in Arizona and New Mexico states [J.
Cheek, Indian Health Service, USPHS, Albuquerque,
NM, 2005, personal communication].
E. granulosus is highly endemic in Argentina,
southern Brazil, Chile, Peru, and Uruguay. In
endemic areas the prevalence of infection in humans
can be as high as 3–6%; 89% of adult livestock may
be infected and 46% of dogs.6 Practices that facilitate
transmission of the parasite include feeding dogs
with infected offal or discarding infected offal in the
field where dogs can have easy access to it. In areas
with no control programs, livestock is commonly
slaughtered in open areas where there is no veteri-
nary supervision.7
Alveolar hydatid disease is caused by E. multilocu-
laris, which has an extensive geographical range in
the northern hemisphere. The natural cycle involves
foxes and small rodents as final and intermediate
hosts, respectively. E. multilocularis is endemic in the
central part of Europe, parts of the Near East, Russia,
and the central Asian republics, China, northern
Japan, and Alaska.6 Recent surveys in central Europe
have extended the known distribution of E. multiloc-
ularis from four countries at the end of the 1980s to
11 countries in 1999, although the annual incidence
of disease in humans remains low.9 There is evi-
dence of parasites spreading from endemic to previ-
ously nonendemic areas in North America and on
the north island, Hokkaido, of Japan, due principally
to the movement or relocation of definitive hosts,
foxes and coyotes. In North America the parasite has
been recorded in two distinct geographic regions:
the north tundra zone (western Alaska) and central
North America.9,10 Despite the presence of infected
definitive and intermediate hosts in 12 of the states
in central North America, only one human case of
alveolar echinococcosis has been described in
Minnesota.11
China is a newly recognized focus of alveolar
echinococcosis (AE) in Asia. E. multilocularis occurs
in three areas: northeastern China including Inner
Mongolia Autonomous region and Heliongjiang
Province; central China including Gansu Province,
Ningxia Hui Autonomous Region, Sichuan Province,
Qinghai Province and Tibet Autonomous Region;
northwestern China including Xingjian Uygur
Autonomous Region.12 The highest prevalence of AE
in the world was found in Qinghai Province with 800
infections per 100 000 inhabitants.13
The infection of humans by the larval E. multilocu-
laris is often the result of association with dogs and
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perhaps cats that have eaten infected rodents. Until
recently, certain villages within the zone of tundra
were hyperendemic foci because of the close interac-
tion between dogs and wild rodents that live as com-
mensals in and around dwellings; however,
transmission has declined as a result of improved
housing and control measures. In central Europe,
rodents inhabiting cultivated fields and gardens
become infected by ingesting embryophores expelled
by foxes and, in turn, may be a source of infection
for dogs and cats. A recent case-control study dem-
onstrated a higher risk of alveolar hydatidosis among
individuals who owned dogs that killed game, dogs
that roamed outdoors unattended, individuals who
were farmers, and individuals who owned cats.14 In
rural regions of central North America, the cycle
involves foxes and rodents of the genera Peromyscus
and Microtus. Allowing pet dogs and cats in these
regions to prey on local rodents may be hazardous.
Polycystic hydatid disease, caused by E. vogeli, has
been reported infrequently from Central and South
America. The natural hosts of this cestode are the
bush dog, Speothos venaticus, and the paca, Cuniculus
paca.1 The larval stage occurs occasionally in rodents
or other species. Little is known of the epidemiology
of polycystic hydatid disease. The natural final host
of E. vogeli, the bush dog, is a wary and rarely seen
animal that is an unlikely source of infection for
humans. The intermediate host, the paca, is widely
hunted for food in northern South America and local
hunters routinely feed the viscera of pacas to their
dogs; thus, infected dogs may be the primary source
of infection for humans.15
Clinical Manifestations
Cystic hydatid disease
In humans, hydatid cysts of E. granulosus are slowly
enlarging masses comparable to benign neoplasms;
most human infections remain asymptomatic.
Hydatid cysts are frequently observed as incidental
findings at autopsy at rates much higher than the
reported local morbidity rates. The clinical manifes-
tations are variable and are determined by the site,
size, and condition of the cysts.16 Hydatid cysts in the
liver and the lungs together account for 90% of
affected localizations. The average liver-to-lung
infection ratio varies from 2.1:1 in clinical cases to 6:1
and 12:1 in asymptomatic individuals with hydatid
disease.17 The chronic signs of hepatic cystic echino-
coccosis include hepatomegaly with or without the
414
presence of a mass in the upper right quadrant (see
Fig. 28.3). Obstructive jaundice accompanied by
symptoms such as mild epigastric pain, indigestion,
and nausea may occur occasionally. Cysts may also
become secondarily infected with bacteria and man-
ifest as an abscess. Features of lung involvement
include coughing, hemoptysis, dyspnea, and fever.
In about 10% of cases the cysts occur in organs other
than the lungs and liver. Other known complications
include anaphylaxis, secondary spread following
rupture, pathological fracture of bones and forma-
tion of hepatopulmonary fistulae.18 The northern
form (G7 genotype) causes a milder form of the
disease with cysts usually localized in the lungs.
Alveolar hydatid disease
The embryo of E. multilocularis seems to localize
invariably in the liver of the intermediate host.
Development of the larval E. multilocularis is inhib-
ited in humans, so that it persists indefinitely in the
proliferative phase. As a result, the hepatic paren-
chyma is gradually invaded and replaced by fibrous
tissue in which great numbers of vesicles, many
microscopic, are embedded. Proliferation continues
peripherally, with the result that an entire hepatic
lobe may be replaced over a period of years. As the
lesion enlarges, it usually undergoes degenerative
changes that lead to central necrosis, often with lique-
faction, and abscesses with a volume of several
liters may be produced. Uneven calcification of
necrotic tissues is typical in lesions of long standing.
Hepatomegaly is characteristic and may be extreme.
The disease takes a chronic course, with deteriora-
tion of health often occurring around middle age.
Patients eventually succumb to hepatic failure, inva-
sion of contiguous structures, or, less frequently,
metastases to the brain.19 However, instances of
spontaneous death of the cyst during its early stage
of development have been reported in people with
asymptomatic infection.
Polycystic hydatid disease
In human cases, hepatomegaly or tumor-like masses
in the liver have been typical findings. Proliferation
of vesicles may lead to destruction of much of the
liver, and involvement of adjacent structures by
extension does not appear to be unusual. The prog-
nosis in polycystic hydatid disease is poor. The
known cases have been described by D’Alessandro
and associates.15

Diagnosis
Who should be tested?
Routine diagnostic screening of immigrants for echi-
nococcosis is not recommended.
Individuals with a cystlike mass in liver or lungs
from areas in which E. granulosus is endemic should
be screened for cystic hydatid disease.17 Alveolar
echinococcosis is very rare in North America as only
two cases have been reported in central North
America. Alveolar echinococcosis mimics hepatic
carcinoma and cirrhosis.
Differential diagnosis
Echinococcal cysts must be differentiated from
benign cysts, cavitary tuberculosis, mycoses,
abscesses, and benign or malignant neoplasms.
Alveolar hepatic lesions may be confused with
hepatic carcinoma or cirrhosis.
Diagnosis
A noninvasive confirmation of the diagnosis can
usually be accomplished with the combined use of
radiologic imaging and immunodiagnostic tech-
niques. Chest roentgenography permits the detec-
tion of echinococcal cysts in the lungs; this is the
most common means of diagnosis of the northern
form that most commonly localizes in the lungs (Fig.
28.4). In other sites, calcification is necessary for
roentgenographic visualization by X-ray. Computer-
ized axial tomography (CT), magnetic resonance,
and ultrasound imaging are useful for diagnosing
deep-seated lesions in the liver and other organs and
are further useful for defining the extent and condi-
tion of avascular fluid-filled cysts. The CT image of
E. granulosus larval cysts typically shows sharply
contoured cysts (sometimes with internal daughter
cysts) and marginal calcifications.20,21 Portable ultra-
sonography machines have been used for field
surveys with excellent results.22,23
Serologic tests are useful to confirm presumptive
radiologic diagnoses, although some patients with
cystic echinococcosis do not develop a detectable
immune response.16 Hepatic cysts are more likely to
elicit an immune response than pulmonary cysts;
however, it appears that, regardless of location, the
sensitivity of serologic tests is inversely related to the
degree of sequestration of the echinococcal antigens
inside cysts. Enzyme-linked immunosorbent assay
CH A P T ER 28 Echinococcosis
Diagnosis
Fig. 28.4 Chest radiograph of a Peruvian pastoralist with a
hydatid cyst in the left lung field detected as part of an
imaging survey in an endemic area. The patient was
asymptomatic as is often the case in echinococcosis.
(ELISA) or the indirect hemagglutination test are
highly sensitive procedures for the initial screening
of sera; specific confirmation of reactivity can be
obtained by immunodiffusion (arc 5) procedures or
immunoblot assays (8/12 kDa band).24 Eosinophilia
is present in fewer than 25% of infected persons.
In seronegative patients, a presumptive diagnosis
may be confirmed by demonstrating protoscoleces
or hydatid membranes in the liquid obtained by per-
cutaneous aspiration of the cyst. Although previ-
ously considered taboo because of the potential for
anaphylaxis or dissemination of protoscoleces, with
certain precautions percutaneous aspiration for pur-
poses of diagnosis or treatment is now standard pro-
cedure. Ultrasound guidance of the puncture,
anthelmintic coverage, and anticipation of the pos-
sible need to treat an allergic reaction now minimize
risks. Protoscoleces can sometimes be demonstrated
in sputum or bronchial washings; identification of
hooklets is facilitated by acid-fast stains.
Diagnosis of alveolar echinococcosis (AE) may be
difficult, particularly in regions where its possible
occurrence is not known to clinicians and patholo-
gists, as in central North America; the disease is
typically seen in persons of advanced age in whom
it closely mimics hepatic carcinoma or cirrhosis.
Plain roentgenography shows hepatomegaly and
characteristic scattered areas of radiolucency out-
lined by calcified rings 2–4 mm in diameter. The
usual CT image of E. multilocularis infection is that
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of indistinct solid tumors with central necrotic areas
and perinecrotic plaquelike calcifications.25 Serologic
tests are usually positive at high titers; highly spe-
cific antigens have been identified and synthesized
that, when used in serologic assays, are highly sensi-
tive and specific for diagnosis of AE and can distin-
guish this infection from CE (E. granulosus) and other
forms of echinococcosis.26 Needle biopsy of the liver
may confirm the diagnosis if larval elements are
demonstrated. Exploratory laparotomy is often done
for diagnosis and delineation of the size and extent
of the invasion.
Polycystic echinococcosis has characteristics
intermediate between those of the cystic and alveo-
lar forms.15 The relatively large cysts are filled with
liquid and contain brood capsules with numerous
protoscoleces. The primary location is the liver, but
cysts may spread to contiguous sites or occur in
other primary locations. Immunodiagnostic and
other techniques useful for diagnosing cystic or
alveolar hydatid disease are also of value in diagnos-
ing polycystic hydatid disease. The hydatid cysts of
E. vogeli can be differentiated from those of other
species based on differences in the dimensions of the
hooks of the protoscoleces.15
ICD-9 Codes
• 122.0 E. granulosus infection of liver
• 122.1 E. granulosus infection of lung
• 122.2 E. granulosus infection of thyroid
• 122.3 E. granulosus infection, other
• 122.4 E. granulosus infection, unspecified
• 122.5 E. multilocularis infection of liver
• 122.6 E. multilocularis infection, other
• 122.7 E. granulosus infection, unspecified
• 122.8 Echinococcosis, unspecified, of liver
• 122.9 Echinococcosis, other and unspecified
ICD-10 Codes
• B67.0 E. granulosus infection of liver
• B67.1 E. granulosus infection of lung
• B67.2 E. granulosus infection of bone
• B67.3 E. granulosus infection, other and multiple
sites
• B67.4 E. granulosus infection, unspecified
• B67.5 E. multilocularis infection of liver
• B67.6 E. multilocularis infection, other and
multiple sites
416
• B67.7 E. multilocularis infection, unspecified
• B67.8 Echinococcosis, unspecified, of liver
• B67.9 Echinococcosis, other and unspecified
Treatment
Until recently, surgery was the only option for treat-
ment of hydatid cysts; however, in the past 15 years
chemotherapy has been introduced and evaluated
and, more recently, combinations of cyst puncture,
aspiration and drainage, with or without injection of
chemicals (called percutaneous aspiration, injection,
re-aspiration, or PAIR), have been evaluated and,
increasingly, are seen to supplement or even replace
surgery as the preferred treatment.27 Surgery remains
the preferred treatment when cysts are large (>10 cm
diameter), secondarily infected, or located in certain
organs, i.e. the brain or heart. The aim of surgery is
total removal of the cyst while avoiding the adverse
consequences of spilling its contents. Pericystectomy
is the usual procedure, but simple drainage, capiton-
nage, marsupialization, and resection of the involved
organ may be used, depending on the location and
condition of the cyst(s). Preoperative albendazole or
mebendazole is indicated to prevent secondary
recurrences following leakage or even rupture of
cyst and spillage of its content and should begin at
least 4 days before surgery and last for 1 month
(albendazole) or 3 months (mebendazole) (Table
28.1).27
Management of patients who fail initial
treatment or in whom surgery is
contraindicated
At times, surgery may be impossible because of the
patient’s general condition and the extent and loca-
tion of the cysts. Under such conditions, treatment
with benzimidazole drugs may be tried; approxi-
mately one-third of patients treated with benzimid-
azole drugs have been cured of their disease (e.g.
complete and permanent disappearance of cysts)
and an even higher proportion have responded with
significant regression of cyst size and alleviation of
symptoms.28 Both albendazole (10–15 mg per kg
body-weight per day) and mebendazole (40–50 mg/
kg) have demonstrated efficacy; however, albenda-
zole, because of its superior pharmacokinetic profile,
which favors intestinal absorption and penetration
into the cyst(s), is slightly more efficacious. Similar
adverse reactions (neutropenia, liver toxicity, alope-
 CH A P T ER 28 Echinococcosis

Treatment

Table 28.1 Treatment of Echinococcosis

Drug Adult Dose Efficacy Adverse events Comments
1
Albendazole 10–15 mg/kg orally Cure in /3 of patients Neutropenia, Not to be used in
two times a day 30–50% show hepatotoxicity pregnancy or in
Should be taken in significant (transient increases patients with chronic
courses of 28 days regression and of aminotransferases), liver disease and
each with 2 weeks alleviation of transient alopecia bone marrow
of rest between symptoms depression.
courses for 3–6 20–40% do not Leukocyte counts and
months or longer respond liver enzymes should
Prophylactic use: 1 No response in 1/3 be assayed during
week before surgery of patients treatment
and 1 month after Imaging examinations
surgical procedure should be carried out
at intervals of
about 3–6 months
for 1–3 years after
termination of
treatment
Mebendazole 40–50 mg/kg daily Less efficacious than Nausea, vomiting, Not to be used in
for 3–6 months or albendazole abdominal pain and pregnancy or in
longer diarrhea patients with chronic
Prophylactic use: 1 liver disease and
week before surgery bone marrow
and 3 months after depression
surgical procedure Not as readily
absorbed as
albendazole
Praziquantel 50 mg/kg per day No clinical trials Mild symptoms such Better results obtained
Once daily or once conducted but cure as dizziness, if used with
weekly reported in case headache, malaise, albendazole
series when abdominal pain,
combined with nausea
albendazole31

cia, and others), reversible upon cessation of treat-
ment, have been noted in most patients treated with
both drugs. A minimum of treatment is 3 months.
The long-term prognosis in individual patients is
difficult to predict; therefore, prolonged follow-up
with ultrasound or other imaging procedures is
needed to determine the eventual outcome. The
combination of praziquantel and albendazole has
been used successfully in the treatment of hydatid
disease.29,30 Praziquantel used at 50 mg/kg in differ-
ent regimens (once daily, once weekly or once every
2 weeks) in combination with albendazole produced
very effective and rapid results compared with
albendazole therapy alone.29 Further research is
needed to determine the optimum dosage and length
for this form of therapy. A third option for the treat-
ment of echinococcosis cysts in the liver is PAIR
which is based on percutaneous puncture using
ultrasound guidance; aspiration of cyst fluid; injec-
tion of protoscolecidal substances (20% sodium chlo-
ride or 95% ethanol) for at least 15 minutes; and
re-aspiration of the cyst fluid content. PAIR is indi-
cated for univesicular hepatic cysts of >5 cm in diam-
eter, for cysts with daughter cysts, for cysts with
detached membranes and for multiple cysts if acces-
sible to puncture.31 PAIR is contraindicated for inac-
cessible or superficially located liver cysts and lung
cysts. It is also contraindicated for honeycomb-like
cysts, cysts with echogenic lesions, inactive cysts or
calcified lesions, and cysts communicating with the
biliary tree. To avoid sclerosing cholangitis, cysts
should be inspected for bilirubin prior to injection of

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protoscolecidal substances. Presence of bile indicates
direct communication between cyst contents and
biliary ducts. Concomitant drug treatment should be
provided in the form of benzimidazoles before the
procedure and should last for 1 month (albendazole)
or 3 months (mebendazole) after the procedure.
Risks include those associated with any puncture;
anaphylactic shock or allergic reactions caused by
leakage of cyst fluid; and secondary echinococcosis
due to spillage.
Favorable results have been reported from more
than 2000 PAIR interventions. A meta-analysis com-
paring the clinical outcomes for 769 patients with
hepatic cystic echinococcosis treated with PAIR plus
albendazole or mebendazole with 952 era-matched
historical control subjects undergoing surgical inter-
vention found greater clinical and parasitological
efficacy, lower rates of morbidity and mortality and
disease recurrence, and shorter hospital stays than
surgical treatment.32 A policy of conservative man-
agement has been adopted generally in the treat-
ment of infections by the relatively benign northern
form of E. granulosus, and surgical intervention is
considered only in cases of uncertain diagnosis (i.e.
possible neoplasms) or in rare cases of symptomatic
disease.
Until recently, surgery has offered the only pos-
sibility for treatment of alveolar echinococcosis. The
usual procedure has involved removal of the lesion
with part of or the entire affected hepatic lobe. Cases
of advanced disease and those involving multiple
lesions often are inoperable. With or without surgery,
alveolar hydatid disease has a very high mortality
rate. With metastases to the brain, death occurs
within a few months after onset of neurologic disor-
ders. Long-term treatment for several years with
mebendazole (50 mg/kg per day) or albendazole
(10 mg/kg per day) inhibits growth of larval E. mul-
tilocularis, reduces metastasis, and enhances both the
quality and length of survival; prolonged therapy
may eventually be larvicidal in some patients.27 Liver
transplantation has been employed successfully
on otherwise terminal cases.33 In a Swiss study,
therapy for nonresectable alveolar echinococcosis
with mebendazole and albendazole resulted in an
increased 10-year survival rate of approximately 80%
(versus 29% in untreated historical controls) and a
16- to 20-year survival rate of approximately 70%
(versus 0% in historical controls).9
Experience in treatment of polycystic echinococ-
cosis is limited.15 Because the lesions are so extensive,
surgical resection may be difficult and usually
incomplete. A combination of surgery with albenda-
zole is most likely to be successful.
418
Information for patients and providers
Centers for Disease Control and Prevention:
ht t p://w w w.cdc.gov/nc idod/dpd/pa ra site s/
alveolarechinococcosis/default.htm
Prevention
Infection of humans by larval cestodes of the genus
Echinococcus is contingent on ingestion of eggs dis-
tributed in the feces of dogs and perhaps other car-
nivores that harbor the adult worms. Control of
hydatid disease in humans depends on the means to
prevent or to eliminate infection of dogs.
Programs have been based on public education
combined with strict regulations directed particu-
larly toward control of dogs and regulated slaughter
of livestock. Nearly complete control of E. granulosus
in the Greek-controlled area of Cyprus was accom-
plished during the period between 1971 and 1975
through elimination of excess dogs, destruction of
all dogs found to be infected, and regulation of
slaughter. Development of the effective echinococ-
cicidal drug, praziquantel, permitted the effective
use of an anthelmintic in conjunction with other
measures for the control of hydatid disease. The
mass treatment of dogs and strict control of slaugh-
ter is effective under some conditions but of little
value where early reinfection is probable. A
promising advance has been the development of
a recombinant vaccine (EG95) which seems to
confer 96–98 % protection against challenge infec-
tion.Further research is needed to assess the costs
and benefits of this intervention as part of control
programs.
Control of E. multilocularis presents a difficult
problem of potentially increasing importance.
Since infection in dogs appears to be the most
important source of infection in humans, educa-
tional measures aimed at preventing dogs from
preying on rodents should be implemented in
endemic areas. Measures for control of the cestode
have involved anthelmintic treatment of dogs
and destruction of stray animals. In Alaska, the
general reduction of numbers of dogs and
improvements in housing probably have had some
effect on the prevalence of E. multilocularis. In
endemic areas, strict controls on the movement of
pet dogs and cats as pets is necessary to prevent
ingestion of infected rodents. Regular anthelmintic
treatment of such animals might be practicable
under some conditions.

Clinical Pearls
• Asymptomatic infection is frequent.
• Presence of a mass in the upper-right quadrant of
the abdomen in an individual coming from an
endemic area is suggestive of hydatid disease.
• Individuals who report expectoration of a salty fluid
(‘hydatid vomit’) and who come from an endemic
area probably harbor a ruptured hydatid cyst in the
lung.
Special Considerations for Immigrants
• It is not cost effective to screen immigrants from
endemic countries for echinococcosis.
• Hydatidosis should be considered if a tumor or
cystlike lesion in the liver or lung with or without
symptoms in an individual coming from an
endemic area (Middle East, South America, Africa)
is observed.
General References
Eckert J, Deplazes P. Biological, epidemiological, and clinical
aspects of echinococcosis, a zoonosis of increasing concern.
Clin Microbiol Rev 2004; 17:107–135.
El-On J. Benzimidazole treatment of cystic echinococcosis. Acta
Trop 2003; 85:243–252.
Craig PS, Rogan MT, Campos-Ponce M. Echinococcosis: disease,
detection and transmission. Parasitology 2003; 127:S5–S20.
McManus DP, Zhang W, Li J, et al. Echinococcosis. Lancet 2003;
362:1295–1304.
Smego RA, Bhatti S, Khaliq AA, et al. Percutaneous aspiration-
injection-reaspiration drainage plus albendazole or
mebendazole for hepatic cystic echinococcosis: a meta-analysis.
Clin Infect Dis 2003; 37:1073–1083.
References
1. Rausch RL. Life-cycle patterns and geographic distribution
of Echinococcus species. In: Thompson RCA, Lymbery AJ,
eds. Echinococcus and hydatid disease. Wallinggford, UK:
CAB International; 1995:89–134.
2. McManus DP, Thompson RCA. Molecular epidemiology of
cystic echinococcosis. Parasitology 2003; 127:S37–S51.
3. Thompson RCA, McManus DP. Towards a taxonomic
revision of the genus Echinococcus. Trends Parasitol 2002;
18:452–457.
4. Rosenzvit MC, Zhang LH, Kamenetzky L, et al. Genetic
variation and epidemiology of Echinococcus granulosus in
Argentina. Parasitology 1999; 118:523–530.
5. Donovan SM, Mickiewicz N, Meyer RD, et al. Imported
echinococcosis in southern California. Am J Trop Med Hyg
1995; 53:668–671.
6. Moro PL, McDonald J, Gilman RH, et al. Epidemiology of
Echinococcus granulosus infection in the central Peruvian
Andes. Bull World Health Organ 1997; 75:553–561.
7. Moro PL, Lopera L, Bonifacio N, et al. Risk factors for
canine echinococcosis in an endemic area of Peru. Vet
Parasitol 2005; 130:99–104.
CH A P T ER 28 Echinococcosis
References
8. McManus DP, Zhang W, Li J, et al. Echinococcosis. Lancet
2003; 362:1295–1304.
9. Eckert J, Deplazes P. Biological, epidemiological, and
clinical aspects of echinococcosis, a zoonosis of increasing
concern. Clin Microbiol Rev 2004; 17:107–135.
10. Eckert J, Conraths FJ, Tackmann K. Echinococcosis: an
emerging or re-emerging zoonosis? Int J Parasitol 2000;
30:1283–1294.
11. Gamble WB, Segal M, Schantz PM, et al. Alveolar hydatid
disease in Minnesota: fi rst human case acquired in the
contiguous United States. JAMA 1979; 241:904–907.
12. Vuitton DA, Zhou H, Bresson-Hadni S, et al. Epidemiology
of alveolar echinococcosis with particular reference to
China and Europe. Parasitology 2003; 127(Suppl):S87–S107.
13. Schantz PM, Wang H, Qiu J, et al. Echinococcosis on the
Tibetan Plateau: prevalence and risk factors for cystic
echinococcosis in Tibetan populations in Qinghai Province,
China. Parasitology 2003; 127(Suppl):S109–S120.
14. Kern P, Ammon A, Kron M, et al. Risk factors for alveolar
echinococcosis in humans. Emerg Infect Dis 2004;
10:2088–2893.
15. D’Alessandro A. Polycystic echinococcosis in tropical
America: Echinococcus vogeli and E. oligarthrus. Acta Trop
1997; 67:43–65.
16. Kammerer WS, Schantz PM. Echinococcal disease. Infect
Dis Clin North Am 1993; 7:605–618.
17. Pawlowski ZS, Eckert J, Vuitton DA, et al. In: WHO/OIE
manual on echinococcosis in humans and animals: a public
health problem of global concern. Echinococcosis in
humans: clinical aspects, diagnosis and treatment. Paris,
France, World Health Organization Office International des
Epizooties. World Organization for Animal Health,
2001:20–66.
18. Wilson JF, Diddams AC, Rausch RL. Cystic hydatid disease
in Alaska. A review of 101 autochthonous cases of
Echinococcus granulosus infection. Am Rev Respir Dis 1968;
98:1–15.
19. Wilson JF, Rausch RL. Alveolar hydatid disease: a review of
clinical features of 33 indigenous cases of Echinocccus
multilocularis infection in Alaskan Eskimos. Am J Trop Med
Hyg 1980; 29:340–349.
20. Morris DL, Richards KS. Hydatid disease: current medical
and surgical management. Oxford: Butterworth-Heineman;
1992.
21. Craig PS, Rogan MT, Campos-Ponce M. Echinococcosis:
disease, detection and transmission. Parasitology 2003;
127(Suppl):S5–S20.
22. MacPherson CNL, Bartholomot B, Frider B. Application of
ultrasound in diagnosis, treatment, epidemiology, public
health and control of Echinococcus granulosus and
Echinococcus multilocularis. Parasitology 2003; 127(Suppl):
S21–S35.
23. Larrieu E, Del Carpio M, Salvitti JC, et al. Ultrasonographic
diagnosis and medical treatment of human cystic
echinococcosis in asymptomatic school age carriers: 5 years
of follow-up. Acta Trop 2004; 91:5–13.
24. Maddison SE, Slemenda SB, Schantz PM, et al. A specific
diagnostic antigen of Echinococcus granulosus with an
apparent molecular weight of 8 kDA. Am J Trop Med Hyg
1989; 40:337–383.
25. Didier D, Weiler S, Rohmer P, et al. Hepatic alveolar
echinococcosis: Correlative US and CT study. Radiology
1985; 154:179–186.
26. Ito A, Sako Y, Yamasaki H, et al. Development of Em18-
immunoblot and Em18-ELISA for specific diagnosis of
alveolar echinococcosis. Acta Trop 2003; 85:173–182.
27. Anon. Guidelines for treatment of cystic and alveolar
echinococcosis in humans. Bull World Health Org 1996;
74:231–243.
28. Davis A, Pawlowski ZS, Dixon H. Multicentre clinical trials
of benzimidazole carbamates in human echinococcosis. Bull
World Health Organ 1986; 64:383–387.
29. Mohamed AE, Yasawy MI, Al Karawi MA. Combined
albendazole and praziquantel versus albendazole alone in
419
 SEC T ION FI V E:
A DDI T IO N A L DISE A SES A ND DISO R DERS IN IMMIGR A N T S
the treatment of hydatid disease. Hepatogastroenterology
1998; 45:1690–1694.
30. Cobo F, Yarnoz C, Sesma B, et al. Albendazole plus
praziquantel versus albendazole alone as a pre-operative
treatment in intra-abdominal hydatidosis caused by
Echinococcus granulosus. Trop Med Int Health 1998; 3:462–466.
31. World Health Organization Informal Working Group of
Echinococcosis. 2001. Puncture, aspiration, injection, re-
aspiration. An option for the treatment of cystic
echinococcosis. Document WHO/CDS/CSR/APH/2001.6.
Geneva, Switzerland: World Health Organization; 1–40.
420
32. Smego RA, Bhatti S, Khalij AA, et al. Percutaneous
aspiration-injection-reaspiration-drainage plus albendazole
or mebendazole for hepatic cystic echinococcosis: a meta-
analysis. Clin Infect Dis 2003; 27:1073–1083.
33. Koch S, Bresson-Hadni S, Miguet JP, et al. European
collaborating clinicians. Experience of liver transplantation
for incurable alveolar echinococcosis: a 45-case
European collaborative report. Transplantation 2003;
75:856–863.