Hydatid cyst

Introduction
 Hydatidosis is a parasitic infection of liver and other organs

 Hippocrates described as liver full of water

 In Greek means drop of water

 Life cycle was elucidated by Haubner in 1855

 Confirmed as zoonosis by Krabbe in 1862

 Currently the most common cause of liver cyst

 Characterized by indolent yet unremitting growth

 Epidemiology
 Occurs throughout the world but endemic in Mediterranean and
various farming regions
 Goel MC, Agarwal MR, Misra A. Percutaneous drainage of renal
hydatid cyst: early results and follow-up. Br J Urol 1995; 75: 724—8.
 Altinors N, Senveli E, Donmez T, Bavbek M, Kars Z, Sanli M.
Management of problematic intracranial hydatid cysts. Infection 1995;
23: 283—7.
 Afflicts all age groups and both sexes
 Incidence of E.Granulosus is 1-220 per 100,000 individuals and
0.03-1.2 cases per 100,000 individuals for E. Multilocularis
 Indian scenario:
 Endemic in India

 Common in southern states of Tamil nadu and Andhra

pradesh
 Human Disease
 Echinococcus granulosus (Most common)
 Echinococcus multilocularis
 Echinococcus oligartus
 Echinococcus vogeli
 Exposure in man is due to ingestion of contaminated
vegetables, or meat.
 Infection may also occur when playing with dogs.
This is most common in children
 Etiology
 Echinococcis parasites are members of
 Order Cestoda (Flatworms)

 Family Taenia

 Adult form- 5mm hermaphrodite infesting the intestines of

carnivorous animals (definitive host)

 Intermediate host- herbivorous animals in E. granulosus and

small rodents in E. multilocularis

 Man is accidental, intermediate host- Terminal event

Adult form

Intermediate host

Larval form

Life cycle of E. Granulosus

 Adult form

Intermediate host

Larval form

Life cycle of E. Multilocularis

Adult Worm
 Morphology of E. Granulosus

 Adults - The adult parasites in the dog represent one of the smallest of the
tapeworms. They measure between 3 and 9mm in length, and usually consist of
only 3 proglottids, an immature, a mature, and a gravid proglottid. The scolex is
globular in shape, and has a prominent rostellum, armed with a double row of
between 30 and 36 hooks. The eggs are very similar to those of the genus Taenia,
and measure between 30 and 40µm in diameter

 Larvae - These Metacestodes (called 'Hydatids') are large, roughly spherical, fluid
filled hollow bladders, containing numerous protoscolices (forming the so-called
hydatid sand), brood capsules, and daughter cysts which are identical in form to
their parent cyst. The cyst wall itself consists of an outer laminated hyaline wall,
supporting the whole cyst. Beneath this there is a nucleated germinal layer, studded
with developing brood capsules, which may eventually break off to float freely in
the fluid filled cyst. The protoscolices are formed within the brood capsules, which
may rupture to give the free protoscolices in the hydatid fluid.
They vary considerably in size depending on where in the body they form, which
may be almost any organ of the body. Those found in the liver (the most common
organ affected) may be approximately 20cm in diameter, but those found in the
peritoneal cavity may sometimes be very much larger, containing several litres of
fluid.
 Morphology of E. Multilocularis

 Adults - The adult parasite is very similar to E. granulosus, being slightly

smaller, with a maximum length of approximately 4mm, and consisting of
4 to 5 proglottids.

 Larvae - The larval E. multilocularis is very different from that of E.

granulosus. In this case the 'cyst' grows invasively by external budding,
forming a diffuse growth through the infected organ, replacing that organs
tissues. The growth itself, (it cannot truly be called a cyst as there is no real
cyst wall), is composed of numerous cavities containing a gelatinous matrix
within which protoscolices and numerous brood capsules are produced, and
which in its behaviour, most closely resembles a malignant neoplasm. In
contrast to E. granulosus this growth is also very rapid, infective
protosocialises being present after only 2 to 3 months, as compared to the 1
to 2 years in the related metacestode.
 Echinococcus vogeli
 This parasite is found in Central and Northern South America,
with bush dogs as the definitive hosts, and pacas and other
rodents as the normal definitive host. The cysts resemble E.
granulosus, but often become septate, forming
multichambered cysts (i.e. polycystic hydatids).
 Lifecycle, Morphology, Pathology of Infection, Larvae - Similar to that of E. granulosus.

 Echinococcus oligarthus –
 This parasite is found in Panama and Argentina. It is unusual
compared with the other species in this genus as wild felids (
e.g. puma, jaguar) act as the definitive host, whilst the agouti
acts as the intermediate host where it forms a polycystic cyst
similar to that of E. vogeli.
 Pathology
 Three layered cyst and slow growing

 Single cyst in 75% of which it may be multilocular with

daughter cyst in 50%

 Right lobe- 80% and left lobe in 20%

 Three layers
 Pericyst

 Laminated layer

 Germinal layer
 Protoscolex with retractile hooklets

Laminated and germinal layer

 Pathology
 Pericyst-
 Host derived fibrous layer in liver and spleen but absent in
lung and brain
 Provides mechanical support. The internal pressure 70 cms
of water
 Not demonstrable on US or CT unless thickened or
calcified
 Calcification occurs in a minority and does not necessarily
mean death
 Laminated membrane
 1-2mm acellular, proteinaceous membrane
 Germinal layer
 Single cell layer
 Forms daughter cysts, cyst fluid, brood capsules
 Hydatid sand- aggregates of scolices floating freely in the
cyst fluid
 Cyst fluid- thick, clear and slightly alkaline
 Opaque cyst- dead cyst or secondarily infected
 Matrix-
 Parts of endocyst gets isolated and degenrate
 Gelatinous amber coloured
 Psuedotumoral appearance on US and CT
 Most common in older patients
 Cyst of E. multilocularis
 Atypical
 Smaller, thin walled and lacking a definitive
capsule
 Appear jelly like, spongy often at the hilum
 Branches into the liver parenchyma like a
malignant disease
 Produces liver pathology by space occupying
process, local encasement or invasion of vascular,
lymphatic or biliary structures.
 Pathogenesis
 Humans are accidental intermediate hosts

Ingested eggs hatch in the duodenum (Oncospheres)

Burrow into the jejunum

Into the veins and lymphatics

Reach the liver and the lungs

And then to virtually anywhere in the body

 Distribution
 Liver - 65-75%
 Lung - 25%
 Others - 5-10% McGreevy PB et al Larval
cestode infections. In:
 Kidney - 1-4% Strickland GT, Hunter’s
Tropical Medicine. Toronto,
 Spleen - 2-3% Canada: WB Saunders;
1984:771
 Brain - 1-2%
 Uterus and adnexa - 0.5-1.5%
 Retroperitoneum -0.5-1.5%
 Pancreas - 0.3-0.8%
 Cardiac involvement with echinococcosis is uncommon
(0.02%-2%); the left ventricular wall is the most frequent site,
but the interventricular septum, right ventricle and left or right
atrium may also be involved
 Major complications of cardiac hydatid disease result from
rupture of the cyst either into the heart or pericardium and
death may occur subsequent to anaphylactic shock, cardiac
tamponade and systemic or pulmonary hypertension
 Pancreatic involvement has been reported in 0.25—0.75% of
adult cases and the mode of infestation is presumed to be
haematogenous, although local spread via the pancreatic or
bile ducts has been suggested, as well as peripancreatic
lymphatic invasion
 Pre-operative diagnosis of hydatid cysts of the pancreas may
be difficult, because it may be confused with
pseudopancreatic, cystadenocarcinoma and true congenital and
post-traumatic pancreatic cysts
 Clinical presentation
 75% are asymptomatic abdominal mass or a suspicious
calcification on a plain x ray

 Parasite load, the site, and the size of the cysts are important
determinant

 Symptomatic if more than 5 cms

 Age group- third and fourth decade

 Abdominal pain is the most common symptom- usually mild

but severe pain indicates rupture, biliary complication and
secondary bacterial infection
 Clinical presentation
 Urticaria in the presence of history of trauma is diagnostic

 Polyarthritis due to circulating IgE antibodies and bronchobilia

are rare presentation

 Other symptoms if liver is involved: obstructive jaundice and the

classical triad of biliary colic, jaundice and urticaria if biliary

rupture occurs. Cholangitis can also occur

 Hydatid emesis and hydatid enterica are also rare

  Routine hematology
 Casoni’s intradermal tests
 Immunological study
 Indirect hemagglutination tests
 Complement fixation test
 Latex agglutination test
Bentonite flocculation test
Investigations 

 Indirect fluorescent antibody

test
 Immunoelectrophoresis
 Counterimmune electrophoresis
 Double diffusion tests
 ELISA
 Radioallergosorbant test
 Basophil degranulation test
 Routine hematology
 Total count is usually normal
 Eosinophilia (>3%) can occur in 25-45% of patients
but it is a non specific finding in endemic areas
 LFT is usually normal unless the biliary tree is
involved
 Elevated IgE levels are non specific for prior or
recent infection
 Elevated IgM class specific to echinococcal organism
may sensitive for recurrent disease
 Serology
 Often necessary for confirmation
 IEP is the most sensitive with 90% sensitivity remaining
positive for 1 year
 IHA has a sensitivity of 85% but remains postive for several
years decreasing the usefulness
 CFT is 70% sensitive and remains positive for 6 months
 ELISA- low cost, good sensitivity and good for population
surveys
 Serology not very sensitive for E. multilocularis
 Casoni’s test has a sensitivity and specificity of 80% and 70%
respectively
 Imaging tests
 Cornerstone for diagnosis
 Plain X- ray – Calcification in 50%
 USG and CT are excellent modalities for hydatid cyst.
Daughter cyst and hydatid sand are specific
 Ultrasonography- detects in 90%
 Cost effective in endemic areas
 Less effective than CT foe delineating and localizing the cyst
 CT- Cyst wall calcification, cyst infection
and peritoneal seeding, 100% sensitive
 MRI- Characteristic low signal intensity rim of
 the hydatid cyst on T2- weighted images
 ERCP- In biliary complications. Allows delineation of the
biliary communication
 Endoscopic sphincterotomy can be performed
 Classification of Hydatid cyst

 Gharbi classification
 WHO classification
 Gharbi classification
 Type I- Pure fluid collection- univesicular cyst

 Type II- Fluid collection with split wall- detached

laminated wall- “ water lily sign”

 Type III- Fluid collection with septa and daughter cyst

 Type IV- Heterogenoeus appearance- presence of matrix

and mimics solid mass

 Type V- Reflecting thick walls- calcification

 WHO Classification of Hydatid
cyst
 CL (Cystic lesion) : Unilocular cystic lesions with anechoic

content without any limiting membrane. Usually indicate

active cyst but are not fertile in the initial stage.

 CE1 (Cystic Echinococcosis 1) : Unilocular simple cyst with

uniform anechoic content with hydatid sand at the floor or may

shows fine echoes due to shifting of brood capsule which is

called as “Snow flake sign”.

 CE2 (Cystic Echinococcosis2) : Multivesicular, multiseptated
cysts producing ' Wheel like' structures and presence of
daughter cysts are indicated by' rosette like' or' honey-comb
like' structure. Cyst wall normally visible. Cyst is active and
fertile.

 CE3 (Cystic Echinococcosis 3) : Unilocular cyst which

contains daughter cysts. Echo free content with detached
laminated membrane from cyst wall give rise to the' water lily'
sign, which indicates many detached membrane floating in the
cystic fluid. It is the transitional stage of the cyst which may
degenerate or give rise to further daughter cyst.
 CE4 (Cystic Echinococcosis 4) : Cyst contains mixed hypo and

hyperechoic degenerative contents. No daughter cysts. Contents may

give' ball of wool' sign which is indicative of degenerating

membranes. The cyst is inactive and infertile.

 CE5 (Cystic Echinococcosis 5) : Cyst is characterized by Arch like

thick calcified wall producing cone shaped shadow. Calcifications

may occur partially or completely. Cyst is inactive and not fertile in

most cases.
Typical multilocular cyst
Large hydatid cyst with floating membrane sign and daughter cyst
Snow storm sign due to hydatid sand
CT image of Hydatid cyst in liver
 Complications
 Occur in 1/3 rd of patients
 Rupture- most common
 Internal
 more common; due to trauma and pressure necrosis

 laminated membrane gets seperated and forms multilocular cyst

 External -
 Intrabiliary- obstrucive jaundice, cholangitis, secondary cyst

infection
 Intrathoracic rupture occurs when cyst reaches posterior or superior

liver capsule- right shoulder pain

 Hepatobronchial and subsequent bronchobilia

 Intraperitoneal- abdominal echinococcosis

 Complications
 Pressure effects- obstructive jaundice
 Organ dysfunction- liver failure
 Secondary infection accounts for 25% of all complication
 Spread- more common with E. multilocularis
 Severe cholestasis, PHT, GI hemorrhage, dissemination of the
disease- associated with E. multilocularis
 Allergic reaction- anaphylactic shock most acutely life
threatening complication
 Medical management
 Indications

 Widely disseminated hydatid disease

 Multiple or recurrent disease

 Inaccessible liver cysts

 Localized disease with poor surgical risk

 Ruptured cyst

 Significant intraoperative spillage

 Medical management
 Mebendazole: 60mg/kbw/day for 6-24 months

 Albendazole: 10mg/kbw/day for 6 months

 Praziquantel: 40mg/ kg body weight once a week

 May arrest spread in E. Multilocularis

 Chemotherapy is effective in 30-40% patients

 More effective for pulmonary disease and less for liver hydatid
 Complications of imidazoles
 Alopecia

 Granulocytopenia

 Neurotoxicity

 GI ulcers

 Hepatic dysfunction
 Non operative management
 PAIR- Puncture Aspiration Injection Re-aspiration

 PAIRD- Puncture Aspiration Injection Re-aspiration Drainage

 PPDC- Puncture Aspiration Drainage Curettage

 Scolicidal agent used are hypertonic saline (20%) or absolute

alcohol.

 Response to treatment is best assessed by serial imaging.

 Non operative management
 PAIR
 Puncture, Aspiration, Injection, Reaspiration
 Proposed by a Tunisian team in 1986
 Found efffective as an alternative treatment
 Chernyshev VN, Panfilov KA, Bogdanov VE. Khirurgiia (Mosk).

2005;(9):39-44.

 PAIR is a safe and effective procedure of choice for

patients with hepatic echinococcosis
 Smego RA Jr, Sebanego P Int J Infect Dis. 2005 Mar;9(2):69-76
 Dziri C, Haouet K, Fingerhut A. World J Surg. 2004 Aug;28(8):731-6.
Epub 2004 Aug 3.
 Indication
 Non echoic lesion < 5 cms
 Cyst with daughter cyst or detached membranes
 Multiple or infected cyst
 Pregnant women
 Patient failing medical therapy
 When surgery is contraindicated
 Who relapse after surgery
 Technique
Prophylaxis

Puncture and parasite exam

10-15 cc aspirated

Bilirubin present No bilirubin

Stop aspiration scolicidal

Reaspiration
 Contraindication

 Non cooperative patient

 Inactive or calcified cyst

 Communication with biliary tree

 Cyst opening into the abdominal cavity

 Advantages

 Minimal invasion

 Confirmation of diagnosis

 Improved efficacy of chemotherapy

 Removal of protoscolices with fluid

 Reduced hospital stay and cost

 Risks

 Hemorrhage

 Anaphylaxis

 Secondary echinococcosis by spillage

 Chemical cholangitis

 Persistence of daughter cyst

World J Surg. 2004 Aug;28(8):731-6. Epub 2004 Aug 3.Related Articles, Links

Treatment of hydatid cyst of the liver: where is the evidence?

Dziri C, Haouet K, Fingerhut A.

Treatment of hydatid cyst of the liver ranges from surgical intervention (conventional or
laparoscopic approach) to percutaneous drainage and to medical therapy. The aim of this
systematic review was to provide "evidence-based" answers to the following questions:
Should chemotherapy be used alone or in association with surgery? What is the best
surgical technique? When is the percutaneous aspiration injection and reaspiration
technique (PAIR) indicated? An extensive electronic search of the relevant literature
without limiting it to the English language was carried out using MEDLINE and the Cochrane
Library. Key words used for the final search were "hydatid cyst," "liver," "treatment," "meta
analysis," "randomized controlled trial," "prospective study," "retrospective study." All
relevant studies reporting the assessment of one modality of treatment or a comparison of two
or several therapeutic methods to treat hydatid cyst of the liver and published in a peer-
reviewed journal were considered for analysis. This systematic review allowed us to conclude
that chemotherapy is not the ideal treatment for uncomplicated hydatid cysts of the liver
when used alone (level II evidence, grade B recommendation). The level of evidence was too
low to help decide between radical or conservative treatment (level IV evidence, grade C
recommendation). Omentoplasty associated with radical or conservative treatment is efficient
in preventing deep abscesses (level II evidence, grade A recommendation). The laparoscopic
approach is safe (level IV evidence, grade C recommendation). Drug treatment associated
with surgery (level II evidence, grade C recommendation) requires further studies.
Percutaneous drainage associated with albendazole therapy is safe and efficient in
selected patients (level II evidence, grade B recommendation). The level of evidence is low
concerning treatment of complicated cysts.
Thank you