Echinococcosis

What is Ech
Tapeworm type of flatworm.
Has no disgestive tract
Each tapeworm has both male and female sex organs
Morphology
Tapeworm is long and flat
Consists of chain of box like segments called
proglottids
Morphology
Morphology
Scolex – the most anterior segment of the tape worm
which has suckers and sometimes hooks.
Immature proglottids
Mature proglottids – have both male & female sex
organs
Gravid proglottids – contain fertilized eggs
Type of Echinococcus
Echinococcus Granulosus
Echinococcus Multilocularis
Echinococcus Vogeli
Echinococcus Oligarthrus

E. Vogeli & E. Oligarthrus are less frequently

associated with human infection.
Life Cycle & Transmission
Dogs and Sheep perpetuate the life cyle of E.
Granulosus
Human is only a dead-end in the cycle
Human ingesting the eggs
Eggs hatch in the intestine and develop into larvae.
After penetrating thru the instestinal wall the larvae
disseminate throughut the body.
Life Cycle and Transmission
Most larvae are concentrated in the liver, but they may
also infect the lungs, kidney and brain.
Each larva form a single round fluid filled hydatid
cyst.
The cysts can undergo asexualy budding to form
daughter cysts and protoscolices inside the original
cyst.
E. Multilocularis cysts undergo lateral budding and
spread
Life Cycle and Transmission
E. Granulosus (E. Granulosus causes cystic echincoccosis)

Epidemiology
Cystic Hydatidosis is significant public health problem in
South & Central America, Middle East, some Sub-Saharan
African countries, China and the former the Soviet Union.
In endemic rural areas prevalance rates of 2-6% or higher
have been recorded.
In a Chinese region endemic for both E. Granulosus & E.
Multilocularis village prevalance rate for cystic & alveolar
echincoccosis were 6.8 and 6.2% respectively.
In the U.S. most cases occur among immigrants from
endemic countries. Local transmission has been observed in
California, Arizona, New Mexico, Utah & Alaska
E. Granulosus
Pathology
Hydatid cyst is usually filled with fluid. The inner layer is
germinative layer which gives rises to the hydatid fluid & brood
capsules (secondary cysts) which bud internally from this layer.
Fragmentation of the germinative layer and brood capsules give
rise to daughter cysts.
10-12 following infection protoscolices are produced within the
brood capsules.
Cysts may contain liters of fluid and thousands of protoscolices.
Such cysts fertile.
External to the germinative layer is an acellular, laminated
membrane of variable thickeness, a host granulomatous reaction
occurs around this membrane.
E. Granulosus
Immunity
Intermediate and incidental hosts mount both humoral &
celluluar immune responses to the organism.
The initial immune response against oncospheres that
penetrate the G.I. mucosa.
Subsequently the host mount an immune response
against the hydatid cysts.
Clinical Features
Initial phase of primary infection is always
asymptomatic.
Many infections are acquired in childhood but don’t
cause clinical manifestations until adulthood.
Latent periods of more than 50 years before symptoms
arise have been reported.
Clinical presentation depends upon the site of the cysts
and their size.
Small and calcified cysts remain asymptomatic
indefinitely.
Clinical Features
Cysts typically increase in diameter at a rate of 1-
5cm/year.
Cysts may be found in almost any site of the body.
However liver is affected in approxmiately 2/3rd of the
patients and the lungs approximately 25% and other
orgrans organs including brain, muscle, kindey, bone,
heart and pancreas in small proportion of the patients.
Clinical Features
Liver Involvement
E. Granulosus infection of the liver frequently produces no
symptoms.
The right lobe is affected in 60-85% of the cases.
Symptoms are unusual until cyst reaches in 10cm in diameter. If
cysts become large hepatomegaly with or without associated right
upper quadrant pain, nausea & vomiting can result.
E. Granulosus cyst can rupture in biliary tree and produce biliary
colic, obstructive jaundice, cholangitis or pancreatitis.
Pressure of mass effects on the bile ducts, portal and hepatic or on
the inferior vena cava can result in cholestasis, portal HTN,
venous obstruction or Budd-Chiari syndrome.
Liver cysts can rupture into peritoneum causing peritonits
Clinical Features
Lung Involvement
Can lead to variety of symptoms including chronic
cough, chest pain, dyspnea.
Rupture of a cysts into bronchus may lead to hemoptysis,
respiratory distress & asthma like symptoms.
If cysts rupture into pleural space, a pleural effusion or
empyema may develop, lung abscesses can occur.
Clinical Features
Other Organs
Infection of the heart can result in mechanical rupture with widespread
dissemination or pericardial tamponade.
CNS involvement can lead to seizures or signs of raised ICP, infection
of the spinal cord can result in spinal cord compression.
Cysts in the kidneys can cause hematuria or flank pain, immune
complex mediated disease, glomerulonephritis leading to the nephrotic
syndrome.
Bone cysts are usually asymptomatic until a pathological fracture
develops.
Ocular cysts also occur
If cyst rupture occurs, fever and acute hypersensitivity reactions
including anaphylaxis, may be principle manifestations of cyst rupture.
E. Multilocularis (causes Alveolar
Echinococcosis)
Epidemiology
E. Multilocularis occurs only in the northern hemisphere,
in parts of central Europe, Russia, Western China, area of
North America and Northern Africa.
The incidence of human infection with E. Multilocularis
is not known but is significantly less common than
infection with E. Granulosus.
E. Multilocularis
Pathology
E. Multilocularis can cause severe infection in humans. Hydatid
cyst tissue behaves like a malignancy which invades and
destroys tissue, extend beyond organ borders into adjacent
structures and can metastasize to distant sites.
The lesions are composed of numerous irregular cyts of various
sizes with no sharp demarcation from surrounding organ tissue.
The lack of limiting membrane allows exogenous budding,
proliferation and infiltration into adjacent tissues result in
necrosis of surround host tissue.
Microscopically the cyts are composed of a thin laminated layer
with minimal or germinative layer. Reproduction occurs by
asexual lateral budding.
E. Multilocularis
Immunity
Most patients who become infected with E.
Multilocularis develop antibodies, although it is
uncertain whether these antibodies play a role in
controlling hydatid cyst proliferation
T lymphocyte responses maybe more prominent in
controlling infection.
E. Multilocularis
Clinical Features
E. Multilocularis infections are less likely to be asymptomatic,
although the clinical manifestation are frequently nonspecific.
Most common presenting complaints inlcude malaise, weight loss, and
right upper quadrant discomfort due to hepatomegaly.
Cholestatic jaundice, cholangitis, portal HTN and Budd-Chiari
syndrome can also occur.
Extrahepatic primary disease is very rare, 13% of the cases present as
multi-organ disease where hydatid cysts involve the lungs, spleen or
brain in addition to the liver.
Immunodeficiency such as HIV or transplantation may accelrate the
manifestations of alveolar echinococcosis.
If left untreated more than 90% of patients will die within 10yrs of the
onset of clinical symptoms and virtually 100% by 15yrs.
Diagnosis
The combination of imaging and serology usually
make the diagnosis of both cystic and alveolar
echinococcosis, although serological assays are more
sensitive and specific for E. Multilocularis compared
to E. Granulosus.
Routine lab tests
Non specific leukopenia or thrombocytopenia, mild
eosinophila and non specific liver function abnormalities
maybe detected but are not diagnostic.
Diagnosis
Imaging
1. Ultrasound
 has sensitivity of approximately 90-95%.
 The most common appearance on ultrasound is an
anechoic smooth, round cyst, which can be difficult to
distinguish from a benign cyst
 When the liver cyst contains membranes, mixed echoes
will appear that can be confused with an abscess or
neoplasm
 When ultrasound reveals infoldings of the inner cyst
walls, separation of the hydatid membrane from the
wall of cyst or hydatid sand, a diagnois of hydatid
disease is made
Diagnosis
Imaging
1. CT Scan
 has sensitivity of approximately 95-100%.
 CT is the best mode for determining the number, size,
and anatomic location of the cysts, and is also better
than ultrasound in detecting extrahepatic cysts
 CT maybe superior to ultrasound in determining
complications such as infection and intrabiliary rupture.
Diagnosis
Imaging
MRI

 Has no major advantage over CT for abdominal and

pulmonary hydated cysts, except in defining changes in
the intra and extrahepatic venous system.
Diagnosis
Imaging
Other
 Other imaging techniques such as cholangiography maybe
indicated to diagnose biliary involvement.
Diagnosis
Sereology – Immunodiagnosis is useful for primary
diagnosis and for follow-up after treatment. Detection
of circulating E. Granulosus antigen in serum is less
sensitive than antibody detection which remains the
method of choice. As a general rule serologic testing
for alveolar echinococcosis is more reliable than for
cystic echinococcosis
SEROLOGY
False positve reactions are more likely in the presence
of other helminth infections, cancer, and immune
disorders
False negative results occur with varying frequency
depending upon the site of lesions and the cyst
integrity & viability. Antigen-antibody complexes that
“mop-up” all antibodies may lead to false negative
reactions. Thus a negative serologic test generally
does not rule out echinococcosis
Serology - Site of Involvement
There is no consistent correlation between the number
or size of cysts and serologic results. However cysts in
the liver more commonly elicit an antibody response
than cyst in the lungs.
Overall, approximately 85-95% of liver cysts and 65%
of lung cysts will be associated with positive serology.
Brain, eye and splenic cysts often do not produce
detectable antibodies. Whereas bone cysts frequently
are associated with positive serology.
Serologic Methods
The complement fixation test was the first immunologic method used
for serodiagnosis of hydatid disease, but a number of other techniques
currently are employed including
Indirect hemagglutination
Indirect immunofluorefcence
Latex agglutination
Double diffusion immunoelectrophoresis
Counter-current immunoelectrophoresis
Radioimmunoassay
Enzyme-linked immunosorbent assay (ELISA)
Enzyme-linked immunoelectrotransfer blots (EITB)
Enzyme-linked immunoelectrodiffusion assay (ELIEDA)
Time-resolved fluoroimmunoassay (TR-FLA)
Immunoblot
Serology – Antibody Detection
Cystic echinococcal disease (Echinococcus granulosus).  Indirect
hemagglutination (IHA), indirect fluorescent antibody (IFA) tests, and
enzyme immunoassays (EIA) are sensitive tests for detecting
antibodies in serum of patients with cystic disease; sensitivity rates
vary from 60% to 90%, depending on the characteristics of the cases. 
Crude hydatid cyst fluid is generally employed as antigen.  At present,
the best available serologic diagnosis is obtained by using
combinations of tests.  EIA or IHA is used to screen all specimens; a
positive reaction is confirmed by immunoblot assay or any gel
diffusion assay that demonstrates the echinococcal "Arc 5."  Although
these confirmatory assays give false-positive reactions with sera of
5% to 25% of persons with neurocysticercosis, the clinical and
epidemiological presentation of neurocysticercosis patients should be
rarely confused with that of cystic echinococcosis. 
Serology – Antibody Detection
Alveolar echinococcal disease (Echinococcus multilocularis).  Most patients
with alveolar disease have detectable antibodies in serologic tests using
heterologous E. granulosus or homologous Echinococcus multilocularis
antigens.  With crude Echinococcus antigens, nonspecific reactions create the
same difficulties as described above, however, immunoaffinity-purified E.
multilocularis antigens (Em2) used in EIA allow the detection of positive
antibody reactions in more than 95% of alveolar cases.  Comparing serologic
reactivity to Em2 antigen with that to antigens containing components of both
E. multilocularis and E. granulosus permits discrimination of patients with
alveolar from those with cystic disease.  Combining two purified E.
multilocularis antigens (Em2 and recombinant antigen II/3-10) in a single
immunoassay optimized sensitivity and specificity.  These antigens are sold in
a commercial EIA kit in Europe, but not in the United States.  As in cystic
echinococcosis, Em2 tests are more useful for postoperative follow-up than
for monitoring the effectiveness of chemotherapy.
Diagnosis
Cyst aspiration or biopsy – maybe required to confirm
the diagnosis for situations when other diagnostic
methods are inconclusive, complications associated
with the procedure (such as potential for anaphylaxis
and secondary spread of the infection) can be
minimized by concurrent administration of
benzimidazole therapy
Polymerase Chain Reaction – currently PCR
techniques are only being used for research purposes.
Treatment
Open Surgery – Is generally the preferred option for large liver cysts,
superficially located single liver cysts which have risk of rupture,
complicated cysts such as those accompanied by infection, compression
or obstruction, or cysts in the lungs, kidney, bone, brain or other organs.
Contraindications
 Patients whose general condition is very poor
 Patients at extremes of age
 Pregnant women
 Patients with multiple cysts or cysts that are difficult to access
 Patients with dead or totally calcified cysts

 Surgical Techniques – Main goal of surgical therapy is to evacuate the

cyst while avoiding spillage of its contents, neutralization of the cyst,
and obliteration of the residual cavity.
Treatment
Liver Cysts – Cystectomy, total pericystectomy, and
partial hepatic resections are all potential resection
procedures for liver cysts.
Lung Cysts – The surgical options for lung cysts
include lobectomy, wedge resection, pericystectomy,
intact endocystectomy.
Protoscolicidal agents - Although it used to be a
common practice to instill a protoscolicidal agent such
as hypertonic saline, cetrimide, or ethanol into the cysts
during surgery, this has not been definitively shown to
reduce the incidence of secondary recurrence.
Treatment
Chemotherapy – Adjunctive chemotherapy before and after surgery
appears to reduce the risk of recurrence by inactivating protoscolices and
lessening the tension of the cysts for easier cyst removal. If spillage of
cyst contents actually occurs (either spontaneously or following
latrogenic manipulation) it is recommended that either mebendazole or
albendazole be given to reduce the risk of secondary hydatidosis.
Laparoscopy – Laparoscopic treatment includes partial or total
pericystectomy and cyst drainage with omentoplasty. Laparoscopic
techniques in appropriately selected patients, such as those with
anteriorly located hepatic cysts, have high sucess rates, low complication
rates, and low recurrence rates. The main exclusion criteria for
laparoscopic intervention are deep intraparenchymal cysts or posterior
cysts situated close to the vena cava, more than 3 cysts with thick and
calcified walls.
Treatment
PAIR Proceedure - PAIR stands for puncture,
aspiration, injection and reaspiration. Percutaneous
puncture of the cysts is performed under ultrasound or
CT guidance followed by aspiration of substantial
amounts of cyst fluid and injection of a protoscolicidal
agent into the cyst cavity. The cyst is then reaspirated
after a period of 15 minutes.
Treatment
Medical Chemotherapy – Benizmidazoles are commonly
used for the medical therapy of echinococcosis, often as
supplementary therapy with either surgery or the PAIR
proceedure. Medical therapy is recommended for
Patients with inoperable disease or those unfit for surgery
Patients with multiple cysts in two or more organs
Multiple small liver cysts or cysts deep in liver parenchyma
Peritoneal cysts
Patients following incomplete surgery or relapse
Prevention of secondary spread of echinococcal infection
following spontaneous rupture or aspiration of cysts.